Ortho/rheum Flashcards

Question

childhood torticollis - congenital x2 causes and what might complicate, 9 acquired causes inc a syndrome

Answer 1

common congen or acquired congen may be due to intrauterine positioning, or sternomastoid haematoma after traumatic delivery may fibrose causing contracture of ipsi scm muscle acquired inc most commonly idiopathic and self resolving but also BG disorders, neuro tumours, cervical spine bony abnorms/tumours/infs, post-traumatic, peritonsillar abscess or phayryngeal infection, strabismus (esp trochlear palsy), rarely associated with severe GORD (sandifer syndrome), drug induced dystonia (antipsychs, DAr antag antiemetics)

Answer 2

80% in girls, may be postural (they can correct by standing differently) or structural; may see one shoulder higher than the other or prominent scapula usually to the right in thoracic region and left in lumbar - more pronounced if child bends forwards 80-85% idiopathic, rest eg hemivertebrae, neuromusc problems like dystrophies, marfan syndrome, mucopolysaccharidoses refer severe for poss surgical correction

Answer 3

Non-structural lateral curvature can masquerade as scoliosis. Common causes include: Poor posture Leg length discrepancy Trauma Tumour Infection structural/true scoliosis causes include: cerebral palsy Friedreich’s ataxia Polio Spinomuscular atrophy Spina bifida Myopathic Muscular dystrophy Neurofibromatosis Skeletal dysplasia Metabolic disorders Collagen disorders Congenital Or in 75% of time, idiopathic - adolescent if onset >10yo and early onset otherwise hallmark of a structural curve is axial rotation, which clinically manifests as a rib prominence; this is generally not seen in the non structural causes diagnosis of idiopathic scoliosis is dependent on excluding the other causes typical adolescent idiopathic scoliosis patient is a female with a convex right thoracic curve or convex left lumbar curve, right shoulder elevated, right rib prominence, left lumbar loin bolster, no abnormal neurology and no significant pain. Deviation from typical features should prompt a search for underlying causes red flags: Structural scoliosis in a male patient Significant pain, particularly night pain Left-sided thoracic curves Abnormal neurological signs/symptoms Rapidly progressive curves Onset in childhood rather than adolescence While scoliosis has cosmetic implications, its clinical significance is related to growth potential and respiratory function. Thoracic idiopathic curves may be associated with decreased respiratory function when >50° and increased shortness of breath when >80°. Scoliosis in children under 10 years of age may result in pulmonary hypoplasia and long-term respiratory failure important to document curve location as well as shoulder and pelvis symmetry. Leg length discrepancy can be assessed by palpating both iliac crests while the patient is standing. Rib prominence, resulting from rotational deformity, is measured by the Adam’s forward bend test. A detailed neurological examination is required to assess tone, power, reflexes and sensation of upper and lower limbs standing whole-spine plain posteroanterior and lateral radiograph should be obtained for patients with structural scoliosis. The Cobb angle measures the most significant magnitude of the curve from the superior endplate of the upper vertebral body to the inferior endplate of the lower vertebral body involved in the curve; Cobb angle <25° is unlikely to progress, 25–50° will likely progress during skeletal immaturity and >50° will likely progress even after skeletal maturity; Peak growth velocity (growth of 5–6 cm in six months) usually occurs 6–12 months before menses. This growth spurt represents the period of highest risk of curve progression Early referral to a paediatric spinal specialist is recommended, especially for skeletally immature patients with curves >20°, patients with significant rotation or those with red flags Observation is appropriate for curves <20° in patients with high growth potential (Risser 0–2) and curves <40° in patients with minimal growth potential (Risser 3–5). If the curve is <20° without rotation, it is appropriate for the general practitioner to repeat plain radiograph imaging in six months’ time to assess progression. At each consultation, the patient should perform an Adam’s forward bend test. Bracing is appropriate for patients with a curve of 20–40° with high growth potential. Brace prevents progression but doesn't cure Symptomatic treatment includes an exercise regimen for core strengthening and posture control. Physiotherapy can help obtain and maintain muscle condition Indications for the surgical management of scoliosis vary considerably depending on patient and curve factors; however, surgery can be indicated when the curve is >40–50°.

Answer 4

Postural kyphosis is the most common form of kyphosis and tends to occur in teenagers. As its name implies, this curve is due to bad posture and becomes more apparent during the teenage growth spurt. It is more common in boys than girls. Requires PT. Scheuermann's kyphosis usually occurs during a growth spurt. It is when the back of the vertebrae in the upper spine grow faster than the front. On an x-ray, the vertebrae, instead of looking like rectangular building blocks, look wedged at the front so that the spine curves forward. Congenital kyphosis is rare but occurs when a baby is born with an abnormal spine. The spine has not developed properly and the curve can get worse as the child grows a condition of hyperkyphosis that involves the vertebral bodies and discs of the spine identified by anterior wedging of greater than or equal to 5 degrees in 3 or more adjacent vertebral bodies thoracic spine is most commonly involved, although involvement can include the thoracolumbar/lumbar region as well Likely, genetic inheritance results in discordant vertebral endplate mineralization and ossification during growth, causing disproportional vertebral body growth with the resultant classic wedge-shaped vertebral bodies that lead to kyphosis the deformity is typically appreciated in the early-mid teenage years by the child or parents; there may be subacute thoracic pain; the curve is rigid, ie does not resolve with an extension or lying prone/supine although neurologic deficits are uncommon, a thorough neurologic exam must be completed obtain AP/lateral radiographs, then diagnostic criteria include the following: Rigid hyperkyphosis, greater than 40 degrees Anterior wedging, greater than or equal to 5 degrees in three or more adjacent vertebral bodies stretches, NSAIDs when needed, PT if kyphosis less than 60 degrees and generally asymptomatic above + extension bracing if kyphosis 60 to 80 degrees plus/minus symptomatic surgery if: Kyphosis greater than 75 degrees causing unacceptable deformity Kyphosis greater than 75 degrees with associated pain Neurologic deficit/spinal cord compression Severe refractory pain ddx Postural kyphosis (flexible postural deformity) Hyperkyphosis attributable to another known disease state Postsurgical kyphosis Ankylosing spondylitis Scoliosis

Answer 5

minor self limiting injury, usually due to lifting heavy load, prolonged uncomfortable posture, a fall, or sudden unexpected motion as in accident pain usually lower back, either in midline, across waist, or just on one side; localised tenderness and increased pain in postural changes supports diagnosis as does history; pain should resolve in few days to a week strain of sacroiliac lig will have tenderness over that joint and pain radiating into buttock and post thigh, usually worsened by abduction of thigh against resistance and pain may refer to symphysis pubis or groin; week or two and will heal, be sure to distinguish it from sciatica bed rest doesnt help, get active slowly with no more than 48hrs off, do trunk strengthening exercises esp abs; while pain initially bad lying on side with knees and hips flexed or supine with pillows under knees will help relieve, or hyperextend legs by lying prone or with pillow under lumbar spine if sacroiliac sprain; ice or heat and massage help, NSAIDs in first few days but not for too long; chiropractic can help some pts return to work sooner; yoga is beneficial in both acute and chronic back pain for many people aching and stiffness may become chronic and radiate into buttocks and post thigh but no motor, sensory reflex abnorms; same treatments as for acute can help end an attack but often recur; long term chiropractor helps some ppl but not all; changing firmness of mattress often helps; worth considering arthropathy; metastatic disease if percussion tenderness over one vertebra, OA, spinal stenosis

Answer 6

stiff pain often worst in the morning with progressive mobilization making better in contrast with some other back pain causes; straight leg raising tests dont give pain unlike radiculopathy facet syndrome due to degen of a facet joint, will be tender on palpation with no signs of nerve root compression, NSAIDs helpful

Answer 7

fairly common, may be attributed to posture or injury, or else to mental illness; low back pain may occur in anxiety, depression but always consider bio causes first sometimes ppl will play up pain as seeking eg compensation: usually describe pain vaguely and prefer to discuss extent of their disability; description may vary from time to time or region and radiation not make sense; pt may wince over slightest pressure, even over sacrum, or may fail to bend forward despite lack of muscle spasm

Answer 8

Age <20 >65 * Constant progressive pain * Thoracic pain * PMH HIV drug abuse * Widespread neurological signs * Sphincter disturbance

Answer 9

5 types of backache: transient after muscular activity, suggests simple strain needing some rest then increasing activity; sudden acute pain and sciatica may be infection or spondylolisthesis (if under 20 these most likely cause), acute disc prolapse (20-40yo, recent lifting), compression fractures but possibly mets or multiple myeloma if a bit older intermittent low back pain after exertion poss relieved by rest, may be OA of facet joints, lumbar spondylosis, ankylosing spondylitis, chronic infection, mult myeloma back pain plus pseudoclaudication may be spinal stenosis severe constant localised pain may be fracture, paget's disease, tumour, or infection and remember osteoporosis in man <60 suspicious and needs raft of test for all the poss causes chronic backache syndrome from self perpetuating illness behaviour may have non physiological signs, variable responses to tests, exaggerated behaviours like falling, hypervent etc; exclude all organic causes, important to address psychosocial need, then consider completementary therapies, pain clinic referral

Answer 10

mechanical back pain most common but diagnosis of exclusion vertebral fracture if onset coincides with injury or if bony tenderness, LXR prolapsed disc, straight leg test renal colic from loin to groin, micro haematuria in 95% cases, loin tenderness common but abdo tenderness rare so if thats present consider other options pyelonephritis symptomatic aortic aneurysm: shock and sudden pain if noncontained bleed, pain if contained bleed, rapidly growing w/o rupture hurt due to stretching of the a wall; beware esp in those >55, and often they present with what seems to be left sided renal colic due to bleed contained in left retroperitoneal space, but may have abdo tenderness; abdo uss to rule out as exam alone often doesnt pick up bony mets - constant, chronic, unremitting, worse at night; radionucleotide bone scan if suspect; myeloma can also be a cause, diagnosis from plasma electrophoresis and urinary bence jones protein test pancreatitis relatively common but usually abdo pain radiating to back, check serum amylase discitis, osteomyelitis, epidural abscess, cauda equina or spinal cord compression; prostatitis often has severe pain in lower back and perineum, prostate exam v tender, sometimes fevers and rigors, often associated uti OA, lumbar stenosis red flags for cauda equina: saddle area paraesthesia, urinary retention or incontinence, faecal incontinence red flags malignancy - constant pain inc at rest and worst at night red flags infection - fever, constant pain inc at rest aaa red flags - abdo pain, sudden cv collapse fracture red flags - history of trauma w/ pain starting soon after; fractures unlikely if pain free after accident then pain devs, or if no bony tenderness; mechanical cause unlikely if pain not made worse by movement

Answer 11

usually from violent impact eg fall or jump from height or vehicle accident; osteoporosis often the cause, esp in older ppl, inc due to hyperparathroidism, prolonged corticosteroid use; osetomalacia, myeloma, bony met pain usually immediate but may be delayed up to days after; all lumbar movements limited, spasm of lower lumbar muscles high impact rotary injury can break transverse process and tear paravert muscle giving limited movements, deep tenderness, local haematoma, and poss bleeding into retroperitoneal space giving groin pain, prox leg weakness and loss of patellar reflex, and grey turner sign body fractures usually need bed rest and good analgesia; discuss with neurosurg or T&O; get a standing XR once pain allows to rule out secondary kyphosis or collapse; brace generally only for comfort if needed but can get neurosurg or T&O opinion

Answer 12

a narrowing in the vertebra, in the areas of the central canal, lateral recess, or the neural foramen degenerative spondylosis is a significant etiology of lumbar spinal stenosis; degenerative changes can also lead to posterior vertebral osteophyte formation (uncinate spurs), facet hypertrophy, synovial facet cysts, and ligamentum flavum hypertrophy; Degenerative spondylolisthesis is another cause of lumbar spinal stenosis; also more rarely things like SOLs, post-surgical fibrosis, rheumatoid conditions like AS, hyperostosis, achondroplasia presents as pain exacerbated by prolonged ambulation, standing, and with lumbar extension, and is relieved by forward flexion and rest. Neurogenic claudication is an important feature of lumbar spinal stenosis. Symptoms are typically bilateral, but usually asymmetric. Low back pain, numbness, and tingling are present in a majority of patients. Numbness and tingling in lumbar spinal stenosis typically involve the entire leg and rarely affect only a single nerve root distribution may also report walking upstairs being easier than walking downstairs, as the back is forward flexed with stairs climbing if present with new-onset bowel or bladder dysfunction, saddle anesthesia, bilateral lower extremity weakness, and/or increased lower extremity pain, the patient may have developed cauda equina or conus medullaris syndromes Patients presenting with radicular pain will not have their pain exacerbated by Valsalva as is the case with intervertebral disc prolapse. check pedal pulses due to dd of vascular claudication

Answer 13

majority are due to preventable causes such as falls (40%), road traffic collisions (35%), or sport injuries (12%). TSCI can be classified* as complete or incomplete: A complete injury is damage occurring across the whole spinal cord width, leading to complete loss of sensation and paralysis below the level of injury An incomplete injury is the injury is spread across part of the spinal cord thereby only partially affecting sensation or movement below the level of injury Trauma causes injury to the spinal cord from (1) the initial acute impact, resulting in a concussion on the spinal cord (2) compression on the spinal cord from increased pressures from nearby rigid structures In the latter cases, such changes result in increased tissue pressure, which can block venous return and result in oedema around the spinal cord. Arterial blood supply to the spinal cord can also be compromised, and therefore lead to ischaemia Canadian C-spine rules can help further stratify risk of cervical spine injury and therefore potential imaging modality. rules apply to patients who are alert (Glasgow Coma Scale score 15/15) and are in a stable condition following trauma where C-spine injury is a possibility. Patients who have a high-risk factor require immediate radiological imaging, which includes either age ≥ 65yrs, a dangerous mechanism, or paraesthesia in extremities. dangerous mechanism: fall from 3feet+, load to head eg from diving, vehicle rollover/ejection or 100kph speed+, if in any motorized recreational vehicle, bicycle accident If none of these are present, those who have a low-risk factor present do not require radiological imaging prior to assessment. These factors include those involved in a simple rear-end motor vehicle collision, those who are waiting in a sitting position, those ambulatory at any time, presence of delayed onset neck pain, or the absence of midline C-spine tenderness. An assessment of range of motion can then be carried out, if imaging deemed not required Suspected Cervical Spine Injury Perform a CT scan in adults, if suggested by Canadian C-spine rules Perform MRI for children, if suggested by Canadian C-spine rules Consider a plain film radiograph in those who do not fulfil the criteria for MRI but clinical suspicion remains after repeated clinical assessment Suspected Thoracic or Lumbosacral Spine Injury Perform a plain film radiograph as the first‑line investigation for those with suspected spinal column injury without abnormal neurological signs or symptoms Perform a CT scan if the radiograph is abnormal or there are clinical signs or symptoms suggestive of a spinal column injury If a new spinal column fracture is confirmed, image the rest of the spinal column *Whole body imaging should be considered with blunt major trauma and suspected multiple injuries Restricting movement of the spine is recommended to prevent further damage to the spinal cord, with the patient is initially strapped to a backboard prior to further assessment or imaging Conservative management includes a combination of bed rest, cervical collars/motion restriction devices, and traction, followed by early mobilisation and rehabilitation absolute indications for surgical management of a traumatic spinal cord injury are evidence of a progressive neurological deficit or a dislocation-type injury to the spinal column (displaced and unstable) Cervical spine surgery aims to realign the spine, decompress the neural tissue, and stabilise the spine with internal fixation (screws, plates, cages) Thoracolumbar spine surgery typically involves spinal decompression, discectomy, spinal fixation, or spinal cord simulation Physiotherapy and other specialist therapy input (e.g. speech and language or occupation therapy) should be utilised early (as soon as deemed safe), as TSCI patients often require extensive rehabilitation both as inpatient and outpatient

Answer 14

For people 16 and over who have sustained a head injury (including people with delayed presentation), do a CT cervical spine scan within 1 hour of the risk factor being identified if any of these high-risk factors apply: the GCS score is <15 on initial assessment* the person has been intubated* a definitive diagnosis of a cervical spine injury is urgently needed (for example, if cervical spine manipulation is needed during surgery or anaesthesia)* there has been blunt polytrauma involving the head and chest, abdomen or pelvis in someone who is alert and stable there is clinical suspicion of a cervical spine injury (pain, tenderness, stiffness) and any of these factors: age 65 or over a dangerous mechanism of injury (that is, a fall from a height of more than 1 m or 5 stairs, an axial load to the head such as from diving, a high-speed motor vehicle collision, a rollover motor accident, ejection from a motor vehicle, an accident involving motorised recreational vehicles or a bicycle collision)** focal peripheral neurological deficit* paraesthesia in the upper or lower limbs.* if none of the above, but neck pain/tenderness is present, then do CT c-spine if cannot actively rotate neck 45 deg to left and right**, or condition putting them at risk eg axial spondyloarthritis (collagen vascular disease, or osteogenesis imperfecta)**, safe assessment not possible** * = within 1hr in <16yos **= 3 view plain x-rays, follow up with CT if they show an abnormality Do MRI in addition to CT if there are neurological signs and symptoms suggesting injury to the cervical spine Do CT or MRI angiography of the neck vessels if there is a suspicion of vascular injury, for example, because of: vertebral malalignment a high-risk fracture (that is, a high-grade or complex facial fracture or a base of skull fracture likely to involve the internal carotid artery or vertebral artery) posterior circulation syndrome

Answer 15

Among younger patients, cervical fractures are usually the result of high-energy trauma, whilst older patients can develop cervical fractures from low impact injuries Patients can present with neck pain, but this is not always the case; may be varying degrees of neurological involvement present, depending on the level of spinal cord involvement and besides potential sensory and motor deficits, innervation to the diaphragm and vasomotor tone can also be affected; injury to the vertebral artery from a cervical fracture (especially in high cervical fractures) may present with a posterior circulation stroke Differentials for patients presenting with cervical neck pain, with or without neurology, following injury include cervical spondylosis, cervical dislocation, or whiplash injury Jefferson fracture is the eponymous name given to a burst fracture of the atlas; caused by axial loading of the cervical spine resulting in the occipital condyles being driven into the lateral masses of C1; Hangman’s fracture, also termed as traumatic spondylolisthesis of the axis, describes a fracture through the pars interarticularis of C2 bilaterally, sually with subluxation of the C2 vertebra on C3. These are caused by cervical hyperextension and distraction; Odontoid peg fractures are common cervical fractures, most common in older patients 3-point C-spine immobilisation, CT in adults or MRI in children (sometimes XR based on c-spine rules), discuss with spine specialists; Non-operative management can be appropriate for stable injuries - rigid collar during initial assessment, then possibly a halo vest; unstable get surgical fusion

Answer 16

Rigid collars do restrict neck motion compared with soft collars but only for flexion, extension, and rotation. Side bending was the same for both types of collars; thought they may restrict motion less than previously believed, hence things like halo used for long-term immobilisation or conservative management of unstable fracture, where collars can be used for stable injuries, esp of lower c-spine Collars may still be beneficial for patients with neck pain that goes down the arm. The collar will restrict neck motion and take the pressure off the nerve roots that are irritated or compressed causing pain. Soft collars are more comfortable, allow necessary motion needed for daily function, and may increase patient compliance after cervical spine surgery cervical orthoses are effective for short term pain relief, they are not an alternative to physiotherapy treatment collars can be used for people who are in less pain, but who need the collar to immobilise the neck and for a sense of security. In this case, the collars act primarily as proprioceptive guides to regulate the movement of the cervical spine rather than as a restraint to physically impede motion collar should be worn constantly for one week only then gradually increased, to prevent soft tissue contractures, muscular atrophy and deconditioning, and psychological dependence There is conflicting research regarding the effectiveness of cervical collars in treating patients, especially following trauma or injury, but they do seem to be effective for pain

Answer 17

for classifying spinal cord injury, based on examination of sensory and motor performance (dermatomes and myotomes) to identify the sensory and motor level each sensory level scored with: 0 = Absent 1 = Altered - Impaired or Partial Appreciation, including Hyperesthesia 2 = Normal or Intact - Similar as on the Cheek sensory level is most caudal, intact dermatome for both light touch and pin prick (sharp/dull discrimination) sensation. The sensory level is determined by performing an examination of the key sensory points within each of the 28 dermatomes on each side of the body, as above, and may be different for the right and left side max score 56 for each modality or 112 overall, can be used to track recovery each motor level scored with normal power grading of 10 Paired Myotomes C5 - T1 and L2 - S1 Motor Level is defined by the lowest key muscle function that has a grade of at least 3 (on supine testing), providing the key muscle functions represented by segments above that level are judged to be intact (graded as a 5) Max of 25 for each extremity so 50 for upper limbs and 50 for lower limbs (should consider separately) Grade A: No Sensory or Motor Function is preserved in the Sacral Segments S4-S5 Grade B: Sensory but not Motor Function is preserved below the neurological level and includes the Sacral Segments S4-S5 Grade C: Motor Function is preserved below the Neurological Level AND More than half of key muscle functions below the Neurological Level of Injury have a muscle grade less than 3 Grade D: Motor function is preserved below the neurological level AND At least half (half or more) of key muscle functions below the NLI have a muscle grade ≥ 3 Grade E: Normal if had prev deficits (no AIS grade given if no SCI) Incomplete further subclassified: Brown-Sequard Syndrome Anterior Cord Syndrome Posterior Cord Syndrome Conus Medullaris Syndrome Cauda Equina Syndrome

Answer 18

infection of intervertebral disc space relatively scarce blood supply to this region compared to the vertebra themselves makes it difficult both for infection to reach the area but also to then treat infection here frequency of discitis is more common in pediatric patients than in the adult population, which is thought to be due to the vascular supply of the intervertebral discs, which diminish later in life: in kids the blood vessels extend from the cartilaginous endplates into the nucleus pulposus, whereas in adults these vascular supplies degenerate, and only extend into the annulus fibrosis back pain, refusal to walk, even abdo pain in younger kids; in adults back pain, fever, anorexia, neurological sx rare but poss; pain normally localises to the disc without radiation, and will have point tenderness; commonest in lumbar region blood cultures, MRI, biopsy for histology and culture; treat with abx for 4-6 weeks (so PICC line needed)

Answer 19

infection within the epidural space anywhere in the brain or spinal cord; part of collection of disease with vertebral OM and discitis Acute SEA is usually less than 2 weeks in duration with fever and signs of systemic inflammation from a hematogenous source. This is in contrast with subtle, afebrile, and long-standing chronic SEA that has resulted from a direct extension of vertebral osteomyelitis. Both present with back and radicular pain, but leukocytosis (in serum and CSF) is more likely in the acute form and not so much in the chronic form intracerebral form 9x rarer than spinal, and 3rd commonest purulent intracerebral disease after brain abscess and subdural empyema; was historically a result of head and neck infections such as sinusitis, mastoiditis, and otitis; but nowadays is mostly a complication of neurosurgical procedures early symptoms of backache may be indolent and persist for weeks; severe back pain usually progresses to root pain within 3 to 4 days. This pain is followed by advanced signs of spinal cord dysfunction within the next 4 to 5 days. The neurologic deficits at this stage are often still reversible; yet, rapid surgical intervention may be needed; Most patients will also develop fevers above 38 C. Other nonspecific findings may also be present such as generalized malaise, fatigue, headaches, irritability, or vomiting progression of neurologic deterioration to severe spinal cord dysfunction can occur in a matter of just a few hours, making the diagnosis and treatment imperative; neurological deficit can become permanent blood cultures, CT or MRI, and all abscess needs neurosurg discussion; may need CT guided aspiration to decompress or else surgery; abx for 4-6 weeks, 6-8 if also osteomyelitis get imaging within 6 hrs for sure if neuro signs, otherwise just as quickly as possible; MRI with and without contrast of the complete spine is the preferred imaging study; CT myelogram if MRI contra'd, if not possible then CT with contrast abx for this, vertebral osteomyelitis, or discitis hold off until biopsy if haem stable and no neuro sx otherwise start broad spectrum immediately

Answer 20

aka infantile cortical hyperostosis, usually seen in first year of life tender swelling of flat bones like mandible and tubular bones like radius/ulna; intense periosteal reaction on x-ray due to inflam mx may need steroids, and can be mistaken for osteomyelitis or healing fractures after physical abuse

Answer 21

acquired dislocation: usually either pyogenic (septic) arthritis, muscle imbalance (cerebral palsy, polio etc), or trauma hip OA: pain oft in groin radiating down to knee, starting off after activity but may become constant and disturb sleep; stiffness inc over time, leg held in adduction so appears short, trendelenburg sign pos eventually; lose weight, keep active but try to reduce eg uphill walking, carrying heavy things etc

Answer 22

hip capsule boundaries: capsule of the hip joint attaches to the edge of the acetabulum proximally. Distally, it attaches to the intertrochanteric line anteriorly and the femoral neck posteriorly (so more proximal on the posterior aspect) Most of the femoral head blood supply is done by the extracapsular arterial ring, which is formed by the lateral femoral circumflex and the medial femoral circumflex arteries, both of which arise from the profunda femoris artery; thus, intracapsular fractures will disrupt the blood supply causing osteonecrosis of the head, and so the joint is replaced; THR is preferred, unless poor functional status or v old in which case hemiarthroplasty is done in children the artery running in the ligamentum teres of the hip supplies blood and if disrupted leads to perthes disease of the hip; after 8 years of age the MFCA is most important with negligible flow through ligamentum teres artery and LFCA #NOFs can be split into intra and extra capsular; intracapsular may be head, subcapital (most common), transcervical, basicervical subcapital fractured NOFs are further classified using the garden system: stage 1 is undisplaced incomplete, stage 2 is undisplaced complete (both of these stages can have internal fixation), then stage 3 is complete fracture incompletely displaced and stage 4 is complete fracture completely displaced extracapsular tend to be intertrochanteric or subtrochanteric displaced subcapital get arthroplasty; non-dsiplaced intracapsular get cannulated hip screws; inter-trochanteric and basicervical get dynamic hip screws or short IM nail; subtrochanteric get IM nail

Answer 23

commonly used for #NOF fracture, after regular paracetamol and opioids used (as tolerated/not contraindicated) idea is to give large volume of LA under fascia iliaca to block femoral nerve, lateral femoral cutaneous nerve, and obturator nerve (last one oft only partially blocked); rather than injecting, you can insert a catheter to the nerve site allowing for infusion of LA (eg bupivicaine) - note if pt pulls this out can try and control pain using conventional analgesia, and only bleep anaesthetics to replace if this is not successful

Answer 24

numerous possible routes of administration (IV, IM, SC, topical, neb, etc) pKa determines their lipid solubility, which in turn determines potency; at physiological pH they become more lipid soluble which allows them to penetrate the cell and bind to the intracellular part of the voltage-gated sodium channel, which is their site of action; this is bc the LA solution will be pH 5-6 so more water soluble, then enters body and this solution buffered to physiological pH of 7.35-7.45 preferentially bind to the channel in its open state, stabilising it in the inactive state which gives rise to use-dependent block, where repeated stimulation of the axon makes more open channels available, and increases the blockade effect; preferentially affect pain and temperature fibres ("Differential block"), possible because they are largely unmyelinated (C-fibres) but experiments are inconclusive bupivacaine is common long acting, with slower time of onset and longer half life, where lidocaine has faster onset and shorter half life being a common short acting LA

Answer 25

lidocaine toxic dose is 4.5mg/kg; bupivacaine is 2.5mg/kg; note if given with adrenaline then max dose is higher as the vasoconstriction slows systemic redistribution allowing more to be excreted while more is absorbing inhibitory neuronal activity is preferentially suppressed first by systemic local anaesthetics; initially see perioral paraesthesia and visual disturbances, light headedness and tinnitus, then shivering, twitching/tremors, slurred speech, and GTC seizures CNS is more sensitive than CVS, but with large doses may see bradycardia, short QTc, depressed contractility, hypotension and cardiac arrest give lipid infusion to treat, as this will bind the lipophillic LA and as plasma conc falls this will pull more from the affected organs, then the lipid and LA will be metabolised in the liver give intralipid bolus 1.5 mL/kg then continuous infusion 0.25ml/kg/min (double if BP remains low)

Answer 26

perthe's disease - limp, often pain in hip and groin, sometimes referred to thigh/knee; affected leg may be shorter; worsen over weeks, hip stiffens and muscles weaken; hip joint regrows and remodels but can take 2 years or so, and incs risk of OA; hip examination and x ray; observation, physio, basic analgesia; crutches may be needed if bad; surgery maybe if badly affected or older children; younger the child, better the outcome ddh - 80% cases in girls; breech presentation incs risk; ortolani and barlow tests; if 3-6mo then asym abduction and galeazzi sign; older children may have painless limp or walk on toes of affected side; uss in under 4.5mo may help, x rays after that as femoral epiphysis ossified by then (4-6mo); most stabilise by 2-6weeks of age, if dont then bracing if under 6mo; ideally dynamic harness, can start up to 4.5-6mo but best before 6 weeks; if not respond or older than 6mo then surgery; surg comes with risk of avn

Answer 27

affects around 1-3% of newborns. Risk factors female sex: 6 times greater risk breech presentation positive family history firstborn children oligohydramnios birth weight > 5 kg congenital calcaneovalgus foot deformity DDH is slightly more common in the left hip. Around 20% of cases are bilateral. Screening for DDH the following infants require a routine ultrasound examination first-degree family history of hip problems in early life breech presentation at or after 36 weeks gestation, irrespective of presentation at birth or mode of delivery multiple pregnancy all infants are screened at both the newborn check and also the six-week baby check using the Barlow and Ortolani tests Clinical examination Barlow test: attempts to dislocate an articulated femoral head Ortolani test: attempts to relocate a dislocated femoral head other important factors include: symmetry of leg length level of knees when hips and knees are bilaterally flexed - galeazzi sign restricted abduction of the hip in flexion Imaging ultrasound is generally used to confirm the diagnosis if clinically suspected however, if the infant is > 4.5 months then x-ray is the first line investigation - you can see interruption of shentons line (imaginary line following superior pubic ramus down inferomedial nof - also disrupted in #nof) All breech babies at or after 36 weeks gestation require USS for DDH screening at 6 weeks regardless of mode of delivery Management most unstable hips will spontaneously stabilise by 3-6 weeks of age Pavlik harness (dynamic flexion-abduction orthosis) in children younger than 4-5 months older children may require surgery

Answer 28

Stage Features Stage 1 Clinical and histological features only Stage 2 Sclerosis with or without cystic changes and preservation of the articular surface Stage 3 Loss of structural integrity of the femoral head Stage 4 Loss of acetabular integrity Management To keep the femoral head within the acetabulum: cast, braces If less than 6 years: observation Older: surgical management with moderate results Operate on severe deformities <6yo eg fracture or joint collapse Prognosis Most cases will resolve with conservative management

Answer 29

femoral head and acetabulum dont fit together properly (socket too deep/retroverted so head rubs on its ant edge, or head/neck junction too thick so neck jams against ant acet) pt usually in 30s/40s, complains of pain in groin esp after activity, hip movements may be restricted or provoke pain leads to damage/degen of joint cartilage and is big cause of OA MRI needed to diagnose

Answer 30

perthe - containment of femoral head w abduction bracing, maybe osteotomy; healing can take upto 2 years; may get OA later SUFE - consider an emergency, epiphysiodesis if mild slip and open reduction + fixation or osteotomy for larger slips; child needs to be kept non weight bearing in mean time, give analgesia, and obtain AP and frog leg XR views Increased risk in adolescence because: -the perichondrial ring thins and weakens -physis is still vertical in this age group (160° at birth to 125° at skeletal maturity), which results in increased shearing forces -the epiphyseal tubercle can provide a rotational pivot point - this structure shrinks with skeletal maturity

Answer 31

traumatic avulsion of the ASIS due to a sudden and forceful contraction of the sartorius and tensor fascia lata that occurs in young athletes occurs during hip extension (sprinting or swinging a baseball bat) athlete will often report a pop or snap at the time of injury may complain of weakness may be confused or misdiagnosed as an acute muscle strain may see weakness to hip flexion and knee extension severe injuries may result in a limp XR will generally show unless non-displaced, in which case CT/MRI might be needed after ortho discussion rest, protected weight bearing with crutches and routine/VFC follow up if >3cm displacement will need ORIF AIIS avulsion is also possible, usually as hip extends and knee is flexed eg in kicking sports, also seen in adolescents; conservative mx with hip rest in flexion then protected weight bearing another ddx is adductor strain: a result of forceful hip extension & external rotation of an abducted leg, usually in sports and esp football and hockey pain is immediate and severe in the groin region with tenderness is at the site of injury along the subcutaneous border of the pubic ramus and pain and/or decreased strength with resisted leg adduction compared to the other leg XR won't show anything unless avulsion injury also present but useful to rule those out MRI can confirm generally rest, ice, protected weight bearing

Answer 32

commonest cause of limp in children, usually between age 2-12yo, often after URTI, affects hip sudden onset of limp + pain (may refer to knee), child may not be weight bearing; no local inflam signs; septic arth more painful and immobile vs reduced movements in transient synovitis; may be tender to palpation anteriorly, esr and wcc usually normal or only mildly elevated vs septic arth; may be fluid in joint space but less than in SA, but aspirate if you're not sure - may be cloudy but not pustulent usually improves in couple of weeks with bed rest, analgesics; 6% of cases turn out to actually be perthes

Answer 33

20% of polytrauma cases esp older ppl (may be non-traumatic in them too) and younger ppl (think motorbikes, falls etc); generally high energy with multiple other injuries to thorax, long bones, spine, intracranial etc presacral venous plexus sits in pelvic fascia ant to sacrum and may bleed heavily in these fractures corona mortis common variant anastomosis between ext iliac/deep inf epigastric and obturator, post to sup pubic ramus and so also vulnerable to injury and bleeding (is found in 1/3 ppl) ext/int iliac arteries potentially in danger too, and lumbar/sacral plexi membranous urethra in men may be torn as prostate forced back but mem urethra fixed so tears - bleeds and haematoma may cause retention, later incontinence and sexual dysfunction

Answer 34

Chondromalacia patellae Softening of the cartilage of the patella Common in teenage girls Characteristically anterior knee pain on walking up and down stairs and rising from prolonged sitting Usually responds to physiotherapy Osgood-Schlatter disease (tibial apophysitis) Seen in sporty teenagers Pain, tenderness and swelling over the tibial tubercle; generally clinically diagnosed if atraumatic, localised to tubercle, knee exam otherwise normal, settles with rest and at night, and systemically well; settles over weeks-months (para/NSAIDs and ice pack in meantime + continue but modify exercise); if not settling refer to PT, if unsure of diagnosis refer to T&O Osteochondritis dissecans Pain after exercise Intermittent swelling and locking Patellar subluxation Medial knee pain due to lateral subluxation of the patella Knee may give way Patellar tendonitis More common in athletic teenage boys Chronic anterior knee pain that worsens after running Tender below the patella on examination

Answer 35

acute swelling: reactive synovitis dev within hours of mod severe injury eg lig/men tear, swelling immediately after means haemarthrosis with painful warm knee (x ray to see if fracture, if not suspect lig damage), also non traum haemarth from clotting disorder, may also be acute septic arthritis (systemic symptoms too, aspirate pus from joint and send for culture before drainage and iv antibiotics); acute swelling without sign of infection nor trauma may be gout or pseudogout, also reactive arthritis is poss after urogen/GI infection chronic swelling: OA and RA most common reasons, TB is poss, rarer causes too swellings not involving joint: pre/infapat bursitis, pop/bakers cyst or aneurysm (pop cyst most commonly caused by RA or OA as post capsule bulges and synovium herniates, cyst may rupture causing painful swollen calf (rule out DVT, it is self resolving with time, elevation, analgesia) RA causes chronic swelling of knee, wasting of quad, restricted movements and valgus deformity OA causes joint to be stiff after rest and hurt to get going again, giving way and locking may occur, quad wasting and varus deform (usually), no fluid or warmth but crepitus present

Answer 36

septic arth, lyme, syphilis, TB, crystal, reactive arth, IBD, osteoarth flare, autoimmune, injury (fracture, ligament) get XR: AP, lateral +/- axial views bloods: looking mainly at inflam markers but also check urate and calcium levels; consider ASOT, lyme serology, RPR for syphilis, and consider autoimmune screen Arthrocentesis and subsequent synovial fluid analysis should be done in all cases of unexplained knee effusion; may show crystals, raised white cells, culture or gram stain bugs, blood if haemarthrosis, fat drops if fracture saline load test may be utilized to determine if a wound near a joint communicates with the joint. In the knee, 155 mL of saline is needed colchicine/NSAID + ice pack; brace and hot knee clinic if ligament injury, rheum referral if suspect autoimmune; abx for septic arthritis + T&O for washout

Answer 37

locked knee: mechanical block preventing knee fully extending pseudolocking: appears locked but due to pain meaning pt holds knee in place most common cause of a locked knee is a tear in the cartilage that cushions the knee joint, or loose body from a fracture or arthritis damage, or from remnants of surgery; pseudolocking may be caused by anything that makes the knee painful or due to patella dislocation/maltracking a true locked knee may require MRI to find the cause and surgery to remove/fix it

Answer 38

sudden quad contraction while knee valgus and extern rotated, commonly when runner dodges to one side; may feel tearing sensation and like knee has gone out of joint, may collapse; patella may spring back, or remain displaced (it is lateral and hard to see, but med fem condyle is prominent) can push it back in, 15-20% chance of rec dislocation; risk higher if patella alta, general lax joint, marked valgus knee, flat lat condyle, small patella - in these risky cases initial dislocate may not have obvious trauma behind it; but pos apprehension test often medial part of patellofemoral lig may tear too, repair doesnt seem to help reduce odds of it happening again

Answer 39

ACL tear typically occurs in an athlete with a history of twisting the knee whilst weight-bearing. The majority of ACL injuries occur without contact and result from a sudden change of direction twisting the flexed knee. The patient is usually unable to weight bear. An ACL tear will typically present with a rapid joint swelling* and significant pain. If the presentation is delayed, instability may also be evident, in which the patient describes the leg ‘giving way’. A plain film radiograph of the knee (AP and lateral) should be taken to exclude bony injuries, any joint effusion, or a lipohaemarthrosis present. A Segond fracture (bony avulsion of the lateral proximal tibia) is pathognomic of ACL injury. An MRI scan of the knee is gold-standard to confirm the diagnosis (>90% sensitivity), also picking up any associated meniscal tears* *50% of ACL tears will also have a meniscal tear, with the medial meniscus the more commonly affected RICE, then Conservative treatment involves rehabilitation, which utilises strength training of the quadriceps to stabilise the knee In the emergency setting, inpatient admission is rarely required; the patient can often partially weight bear and a cricket pad knee splint can be applied for comfort. Surgical reconstruction of the ACL involves the use of a tendon or an artificial graft This is not performed acutely but following a period of ‘prehabilitation’, whereby the patient will engage with a physiotherapist for a period of months prior to the surgery Acute surgical repair of the ACL is possible in some cases dependent on the location of the tear within the ligament If the imaging on MRI is favourable, the patient can be further assessed under GA knee arthroscopy, proceeding to an acute repair where possible Post-traumatic osteoarthritis is a well-established complication of both ACL injury and ACL reconstructive surgery.

Answer 40

most commonly fractures following high-energy trauma, such as a fall from height or a road traffic accident, from the impaction of the femoral condyle onto the tibial plateau. Less commonly they can occur in elderly patients following a fall, especially those with osteoporosis. It is typically a varus-deforming force, meaning that the lateral tibial plateau is more frequently fractured than the medial side. They are often found alongside other bony and soft tissue injuries, such as meniscal tears or cruciate or collateral ligament injury. It is important to recognise that this is a significant injury, as there is disruption of the congruence of the articular surface that, if left, will lead to rapid degenerative change within the knee Patients will present with sudden onset pain in the affected knee, being unable to weight-bear, and swelling of the knee*. On examination, significant swelling will be evident, alongside tenderness over the medial or lateral aspects of the proximal tibia, with potential ligament instability (albeit not clinically assessed initially, due to the pain it would cause). Ensure to check peripheral neurovascular status of the patient, as neurovascular injuries (such as popliteal vessel dissection or common fibular nerve damage) are an important association of high grade injuries. For patients presenting with knee pain following trauma, other differentials to consider are knee dislocation, other knee fractures (including patella or distal femur), meniscal injuries, ligamentous injuries, patella dislocation, or patella/quadriceps tendon rupture first line investigation for a suspected plateau fracture is plain film radiographs (anteroposterior and lateral), often features on radiograph are subtle. There will also be a lipohaemarthrosis present. CT scanning is needed in almost all cases, apart from undisplaced fractures can be classified through the Schatzker Classification Non-operative management can be trialled in uncomplicated tibial plateau fractures (including no evidence of ligamentous damage, tibial subluxation, or articular step <2mm) These can typically be treated with a hinged knee brace and non- or partial-weight bearing for around 8-12 weeks, alongside ongoing physiotherapy and suitable analgesia Operative management is typically warranted in complicated tibial plateau fractures*, or any evidence of open fracture or compartment syndrome. Any form of medial tibial plateau fractures may also require surgical intervention, even if undisplaced, as they have a great potential for displacement. Open reduction and internal fixation (ORIF) is the mainstay of most tibial plateau fractures, with the aim to restore the joint surface congruence and ensure joint stability Postoperatively, a hinged knee brace is fitted with early passive range of movement but limited or non-weight bearing for around 8-12 weeks months is typically required. External fixation with delayed definitive surgery is indicated in cases of significant soft tissue injury, polytrauma and highly comminuted fractures where an immediate ORIF may not be suitable main long-term complication following a tibial plateau fracture is post-traumatic osteoarthritis to the affected joint

Answer 41

menisci rest on the tibial plateau and have two main functions (1) shock-absorbers of the knee joint (2) increase articulating surface area. The medial meniscus is less circular than the lateral and is attached to the medial collateral ligament, whilst the lateral meniscus is not attached to the lateral collateral ligament most common causes for meniscal tears are trauma-related injury and degenerative disease (the latter more common in older patients). In traumatic tears, the mechanism typically involves a young patient who has twisted their knee whilst it is flexed and weight-bearing, with the onset of symptoms following soon after Patients often report a ‘tearing’ sensation in their knee, associated with an intense sudden-onset pain. The knee invariably swells slowly subsequently over a period of 6-12 hours. In cases where the meniscal tear results in a free body within the knee (typically the bucket-handle type), it may be locked in flexion and unable to extend. On examination, there is a joint line tenderness, significant joint effusion, and limited knee flexion x-ray and MRI immediate management of an acutely swollen knee is for rest and elevation with compression and ice. Most small (<1cm) meniscal tears will initially swell however the pain will subside over the next few days as the tear heals. For larger tears or those remaining symptomatic, arthroscopic surgery is indicated: If the tear is in the outer third of the meniscus (where it has a rich vascular supply), then the tear can often be repaired using sutures If the tear is in the inner third, then the tear is usually trimmed to reduce locking symptoms (and middle third tears may either be repaired or trimmed) meniscal tear is a risk factor for developing secondary osteoarthritis. Knee arthroscopy carries a risk of deep vein thrombosis and damage to local structures, such as the saphenous nerve and vein, the peroneal nerve, and the popliteal vessels.

Answer 42

medial collateral ligament (MCL) is the most commonly injured ligament of the knee*. The MCL primary function is to act as a valgus stabiliser of the knee and is most often injured when external rotational forces are applied to the lateral knee, such as a impact to the outside of the knee. MCL injuries can be graded from one to three: Grade I – mild injury, with minimally torn fibres and no loss of MCL integrity Grade II – moderate injury, with an incomplete tear and increased laxity of the MCL Grade III – severe injury, with a complete tear and gross laxity of the MCL patient may report hearing a ‘pop’ with immediate medial joint line pain. Swelling tends to follow after a few hours (unless there is an associated haemarthrosis, in which case it will occur within minutes). The main clinical finding on examination will be increased laxity when testing the MCL*, via the valgus stress test. The patient will be extremely tender along the medial joint line, but may be able to weight bear. *A Grade II and III tear can be distinguished clinically on medial stress testing; Grade II is lax in 30 degrees of knee flexion but solid in full extension, whereas Grade III is lax in both these positions. Any patient following trauma with significant knee pain and swelling should have a plain film radiograph to exclude any fracture. The gold-standard investigation to confirm the diagnosis for an MCL tear is via MRI scanning management of an MCL injury is dependent on the grade of injury: Grade I Injury: Rest, Ice, Compression, and Elevation (RICE) with analgesia (typically NSAIDs) as the mainstay. Strength training as tolerated should be incorporated, with an aim to return to full exercise within around 6 weeks. Grade II Injury: Analgesia with a knee brace and weight-bearing/strength training as tolerated. Patients should aim to be able to return to full exercise within around 10 weeks Grade III Injury: Analgesia with a knee brace and crutches, however any associated distal avulsion then surgery is considered. Patients should aim to be able to return to full exercise within around 12 weeks main complications following a MCL tear are instability in the joint and damage to the saphenous nerve

Answer 43

occur when a posteriorly directed sheer force is applied to a flexed knee. One example is the classic "dashboard injury" in which the anterior knee, including the proximal tibia, of the driver or a front seat passenger collides with the car's dashboard

Answer 44

liotibial band (also termed the iliotibial tract) is a branch of longitudinal fibres that form the shared aponeurosis of tensor fasciae latae and the gluteus maximus. It extends from the iliac tubercle to the anterolateral tubercle of the tibia most common cause of lateral knee pain in athletes thought to be linked to repetitive flexion and extension of the knee, causing impingement of the band against the lateral femoral condyle common in those who have regular exercise involving repetitive flexion and extension of the knee, commonly runners, weightlifters, or cyclists Anatomical risk factors include genu varum, excessive internal tibial torsion, foot pronation, and hip abductor weakness Lateral knee pain is the classical clinical feature in iliotibial band syndrome, exacerbated by exercise, often found in patients who take part in frequent exercise or with a sudden increase in intensity (such as training for a marathon). Pain may also be worsened running downhill or on an excessive camber. In the history, ensure to assess for any previous trauma (more suggestive of ligamentous knee injury) or features of osteoarthritis. Examination may often be unremarkable, only with pain localising to lateral aspect of the knee Nobles test – the patient lies in a supine position and the examiner places a finger on the lateral femoral condyle, with the knee slowly extends. A positive test is indicated when pain is felt at 30 degrees, when the ITB passes over the lateral femoral condyle. Renne test – the examiner stands in front of the affected knee and puts pressure on the lateral epicondyle, with the patient then asked to squat. A positive test is indicated by the presence of pain at 30 degrees of flexion. Clinical diagnosis, x-ray and MRI to exclude other things if you're not sure Most patients can be advised to modify their activity accordingly and use of regular simple analgesics during periods of acute pain. Longer-term management can often include local steroid injections, in aid to reduce localised inflammation, and physiotherapy, including a period of stretching followed by strengthening of the muscles around the knee joint. Surgical Management Surgery is only indication if patients remain symptomatic or functionally limited after 6 months, despite all other non-medical treatment. Surgical management involves release of the iliotibial band from its attachments from the patella, allowing for a greater range of movement

Answer 45

a misnomer as the pains are often not related to growth - the current term used in rheumatology is 'benign idiopathic nocturnal limb pains of childhood' Growing pains are equally common in boys and girls and occur in the age range of 3-12 years. Features of growing pains never present at the start of the day after the child has woken no limp no limitation of physical activity systemically well normal physical examination motor milestones normal symptoms are often intermittent and worse after a day of vigorous activity

Answer 46

flat foot: long arch collapse so med border of foot in contact (or nearly) with floor, foot turned outward on weight bearing; arch support and good shoes, if rigid (ie arch doesnt appear when go up on toes or dorsiflex great toe passively) then consider if some kind of arthritis, neurological condition etc could be at play RA common, OA almost always secondary to something eg previous injury, gout common in toes but also in ankle joints, TB is poss achilles tendinitis: common in runners, hikers etc, is painful and tendon feels thick (suspect rupture if onset acute and felt like was hit in the calf); RICE, NSAID, then stretching and strength building exercises when recovered painful ankle: trauma or RA poss? if not consider tenosynovitis (move exacerbates, tendon tender), rup of tib pos tendon (med mall tender, pt may have felt pop); pain and slight movement limitation might be fracture of dome of talus giving small fragment of free bone, x ray may help and MRI too, avascular necrosis of talus poss, dont forget gout etc painful foot: traction injury, calcaneal bursitis caused by shoe rubbing, plantar fasciitis (pain under ball of heel or slightly ant, worse on weight bearing, tenderness of distal edge of heel; painful ridge of bone may dev on med cuneiform and first metatarsal, gives hard lump which may be tender; pain in forefoot from faulty weight distribution if flat foot, claw toes, pes cavus etc; stress fracture of 2nd/3rd MT in young ppl after unaccustomed activity; exostosis can grow under nail and lift it up; onychogryposis happens for no apparent reason, chripodist can help but sometimes just have to remove nail

Answer 47

leg divided into 4 compartments by: interosseous membrane of the leg transverse intermuscular septum anterior intermuscular (crural) septum anterior compartment contains tibialis anterior, EHL, EDL, ant tibial vessels, deep peroneal nerve; lat compartment contains peroneus longus and brevis and superficial peroneal nerve; superficial post compartment contains gastroc, soleus, plantaris, and sural nerve; deep post compartment contains tibialis post, FHL, FDL, popliteus, posterior vessels and tibial nerve deep peroneal nerve supplies skin between 1st/2nd toes and does ant comp muscles and some foot muscles (which combined do dorsiflexion of foot, important for swing phase and heel strike and toe extension - damage will give footdrop, tibialis anterior also does inversion with TP in deep post comp) superficial peroneal nerve does rest of dorsum of foot as well as anterolat distal leg and muscles in lat comp (which evert foot and slightly plantarflex); ankle sprains/twisting and fracture of the fibula often damage this tibial nerve comes from sciatic in popliteal fossa and gives branches to superficial post comp for muscles and to form sural nerve, while rest continues in deep post comp; sup post comp does plantarflexion (and knee flexion for gastroc), deep does flexion of toes, plantaflexion, TP does plantarflexion and inversion, and popliteus unlocks knee by lat rotation of femur; tibial nerve also innervates intrinsic foot muscles except EDB which is deep peroneal sural nerve formed from branches of tibial and common peroneal nerves, and innervates posterolat leg and lateral foot tibial nerve also innervates sole of foot; in tarsal tunnel syndrome compression of TP branches at medial malleolus gives paraetshesia in sole of foot and weakness/wasting of intrinsic foot muscles

Answer 48

Clinical examination is facilitated by the Ottawa ankle rules to try and minimise the unnecessary use of x-rays. These state that x-rays are only necessary if there is pain in the malleolar zone and: 1. Inability to weight bear for 4 steps 2. Tenderness over the distal tibia 3. Bone tenderness over the distal fibula Weber: Related to the level of the fibular fracture. Type A is below the syndesmosis Type B fractures start at the level of the tibial plafond and may extend proximally to involve the syndesmosis Type C is above the syndesmosis which may itself be damaged A subtype known as a Maisonneuve fracture may occur with spiral fibular fracture that leads to disruption of the syndesmosis with widening of the ankle joint, surgery is required. Mx: All ankle fractures should be promptly reduced to remove pressure on the overlying skin and subsequent necrosis Young patients, with unstable, high velocity or proximal injuries will usually require surgical repair. Often using a compression plate. Elderly patients, even with potentially unstable injuries usually fare better with attempts at conservative management as their thin bone does not hold metalwork well

Answer 49

tarsometatarsal fracture dislocation characterized by traumatic disruption between the articulation of the medial cuneiform and base of the second metatarsal, which should be aligned; you may also see 2nd metatarsal sitcking upwards on weight bearing XR; medial side of 4th MT may also not align with medial cuboid bone like it should the lisfranc ligament is on the plantar side of foot and responsible for holding the structure together and supporting the midfoot arch pain will oft be severe/prevent weight bearing; may see bruise on plantar surface of foot (always look); get AP, lateral, oblique foot XRs and weight bearing if poss; CT for subtle injuries and pre-op planning stable, weight bearing candidates or not suitable for surgery get cast for 8 weeks; otherwise ORIF or arthrodesis

Answer 50

friedreich ataxia, duchenne, spina bifida, tethered cord syndrome, CMT, idiopathic, and familial

Answer 51

intrinsic muscles originate from the scapula and/or clavicle, and attach to the humerus and include deltoid, teres major, and the four rotator cuff muscles (supraspinatus, infraspinatus, subscapularis and teres minor) [vs extrinsic muscles which come from torso to one of the three bones above] deltoid: Anterior fibres – flexion and medial rotation. Posterior fibres – extension and lateral rotation. Middle fibres – the major abductor of the arm (takes over from the supraspinatus, which abducts the first 15 degrees). Innervation: Axillary nerve. teres major: Adducts and extends at the shoulder, and medially rotates the arm, innervated by lower subscapular nerve rotator cuff resting tone pulls head of humerus into glenoid fossa; then: supraspinatus Abducts the arm 0-15o, and assists deltoid for 15-90o, innervated by suprascap nerve which also innervtes infraspinatus that laterally rotates the arm subscapularis medially/internally rotates arm and is innervated by upper/lower subscapular nerves teres minor laterally rotates arm and is innervated by axillary nerve axillary nerve: direct continuation of the posterior cord from the brachial plexus – and therefore contains fibres from the C5 and C6 nerve rootsexits the axilla at the inferior border of subscapularis via the quadrangular space, often accompanied by the posterior circumflex humeral artery and vein. The axillary nerve then passes medially to the surgical neck of the humerus, where it divides into three terminal branches: articular to the glenohumeral joint, posterior to posterior deltoid and teres minor and upper lat cut nerve of arm to skin over regimental patch area; ant terminal branch to ant deltoid and more cut branches if damaged: unable to abduct the affected limb beyond 15 degrees + loss of sensation over inf deltoid; also compressed at quadrangular space if trauma, SOL, or muscle hypertrophy (eg athletes doing overhead activities) giving weak deltoid/teres minor; borders of quadrang space: teres minor (top) major (bottom) surgical neck of humerus (lat) oong head of tricpes (medial) subscapularis (roof)

Answer 52

rotator cuff syndrome: (subacute tendinitis, chronic tendinitis, rotator cuff tears); pain or weakness during movement of shoulder; may have started suddenly after particular exertion; painful arc (60-120deg) SaT after eg weekend of decorating, swimming etc, tender along ant acromion worst in ext and may disappear in flex; CT commonly rec attacks of SaT like thing, better with rest or NSAIDs and starting again with demanding movement, oft worse at night; history of this inc over time may be tear developing, but can occur acutely too with sudden pain and inability to abduct: above 90deg can keep it up with deltoid, suddenly drop as lower it again; MRI shows well, x ray not until late with sclerosis of insertion and thinning of acromion; USS can show tear adhesive capsulitis: prog pain and stiffness, resolves oft after 18mo, prob starts like chronic tendinitis but spreads to involve whole joint capsule; pt 40-60 with oft trivial, sometimes forgotten trauma, then pain inc over time and maybe cant sleep on that side as with CT, pain subsides but stiffness starts small then incs then bad for up to 12mo; movements stiff, x rays normal biceps tendon: tendinitis sharply localsied to bicipital groove, with flexion/supination against resistance inc pain; tendon may degen, feel a snap while lift something heavy, bruising on ant arm, then popeye syndrome on flexion

Answer 53

ant oft from arm force into abduction/ext rotation; may give ant instability after where keeps slipping out when abducting or extending arm, or get subluxation with catching feeling and weakness when eg throw ball or serve at tennis, apprehension sign can also get post instability, or atraumatic often linked to eg connective tissue problems x ray may show damage to humerous head from repeated subluxations

Answer 54

narrowing of space between acromion and humerus (subacromial space), like exostosis, OA of ACJ, or variation in acromion shape; also thickening of coracoacromial lig; arms int rotated and elevated above shoulder height exercise eg upright row might cause acute or insidious onset depending on cause of the narrowing (eg trauma could cause), but results in tendonitis esp of supraspinatus but could be all rotator cuff; pain, weakness, reduced movement, often worse when arm raised above head or at night if lying on affected shoulder pain often dull rest, physio, nsaids, local steroids/LA

Answer 55

over half of major joint dislocations which present to emergency departments; if not managed correctly they can lead to chronic joint instability and chronic pain anterior dislocation is classically caused by force being applied to an extended, abducted, and externally rotated humerus is 95% of cases; posterior typically caused by seizures or electrocution, but can occur through trauma (a direct blow to the anterior shoulder or force through a flexed adducted arm) present with a painful shoulder, acutely reduced mobility, and a feeling of instability; often there is a loss of shoulder contour (from a ‘flattened deltoid’) and an anterior bulge from the head of the humerus may also be seen; check neurovasc status pre- and post-reduction - axillary and suprascapular nerves esp at risk look for bony and ligamentous bankart lesion, rotator cuff injuries, hill sachs lesions, and fractures of greater tuberosity or surgical neck trauma shoulder series: PA, Y view +/- axial view; while normal for humeral head to overlap glenoid (due to angle it points at) on PA view, Y view will make things more clear (should line up nicely with blade/coracoid/acromion); may see light bulb sign in posterior dislocation; MRI if suspect labral or rotator cuff injury; post reduction XR, and if bankart lesion then CT and ortho discussion analgesia, reduce, sling for 2ish weeks, early PT; surgery if failed reduction, or large bankart or hill sachs lesions; entonox is general first choice analgesia, with morphine and midazolam for sedation if this fails; entonox is N2O and O2 mix, N2O has analgesic and calming effects, acts on many receptors but biggest is NMDAr antag

Answer 56

median: C6-T1 Innervates the flexor and pronator muscles in the anterior compartment of the forearm - superficial and intermediate layers directly and deep flexors via anterior interosseous branch (except the flexor carpi ulnaris and medial part of the flexor digitorum profundus, innervated by the ulnar nerve). Also supplies innervation to the thenar muscles (via recurrent branch) and lateral two lumbricals in the hand (by digital branch). In general: flexion of fingers and wrist and pronation of forearm; hand of benediction sign when make a fist if damaged due to these lumbricals not working properly Gives rise to the palmar cutaneous branch, which innervates the lateral aspect of the palm, and the digital branch, which innervates the lateral three and a half fingers on the anterior (palmar) surface of the hand palmar cutaneous branch does not travel through the carpal tunnel - so palm spared in CTS ulnar nerve C8-T1 flexor carpi ulnaris and medial half of flexor digitorum profundus + Intrinsic muscles of the hand (apart from the thenar muscles and two lateral lumbricals) Sensation of medial one and half fingers and the associated palm area passes posterior to the elbow through the ulnar tunnel (small space between the medial epicondyle and olecranon) where gives branch to elbow joint froment's sign positive if can't adduct thumb to hold paper between thumb and index may see claw hand - appearance reduced if proximal injury due to denervation of FDP radial nerve C5 – T1. wraps around the humerus laterally, and supplies a branch to the triceps brachii as well as brachoradialis and ECRL. During much of its course within the arm, it is accompanied by the deep branch of the brachial artery ant to lat epicondyle though cubital fossa, then deep branch to remaining post forearm muscles (supinate, extend wrist and fingers) - note after penetrating supinator, deep branch is called post interosseous gives 4 sensory branches: Lower lateral cutaneous nerve of arm – Innervates the lateral aspect of the arm, inferior to the insertion of the deltoid muscle. Posterior cutaneous nerve of arm – Innervates the posterior surface of the arm. Posterior cutaneous nerve of forearm – Innervates a strip of skin down the middle of the posterior forearm Superficial branch - innervates the dorsal surface of the lateral three and half digits and the associated area on the dorsum of the hand can be damaged in the axilla region by a dislocation at the shoulder joint, or a fracture of the proximal humerus, or pressure from a crutch or eg arm over back of a chair while sleeping; can also be damaged in radial groove (same signs but triceps spared) or in forearm (sensation if superficial branch damaged and motor if deep); motor function knocked out gives wrist drop due to unopposed flexion, and if higher level also unable to extend elbow

Answer 57

majority of proximal humeral fractures are low energy injuries occurring in elderly patients falling onto an outstretched hand from standing - oft in context of osteoporosis; high energy/traumatic in younger ppl (soft tissue/neurovasc damage); generally if distal half then put in backslab, if proximal half then put in sling/brace pain around the upper arm and shoulder, with restriction of arm movement and an inability to abduct their arm; bruise, swelling; close anatomical relationship with the axillary nerve and the circumflex vessels - check neurovasc status; risk of AVN due to damage to circumflex arteries meaning hemiarth or reverse shoulder arth needed trauma bloods, osteoporosis type bloods, can consider myeloma screen; AP, lateral, axillary views XR polysling, gravity helps reduce the fragments, early mobilisation guided by PT; if displaced, neurovasc compromise, or open then ORIF if head or nail if neck/shaft shaft fractures bimodal distribution, occurring in both younger patients due to high energy trauma and in elderly patients following low impact injuries. Due to the location of the radial nerve within the spiral groove, there is a reasonably high risk of injury; the overall incidence is around 10% risk factors as above inc osteoporosis, may be pathological If the radial nerve is involved, the patient may also complain of reduced sensation over the dorsal 1st webspace and weakness in wrist extension or even wrist drop - urgent surgery needed; otherwise 90% nerve contusion will improve within 3 months AP/lat XR; generally mx with brace; ORIF with plate sometimes, and rarely nail if trauma or pathological/osteoporosis supracondylar common paediatric elbow injury, but are almost never seen in adults; most commonly FOOSH on extended elbow; see deformity, swelling, bruising; look carefully for open wound study median nerve, the anterior interosseous nerve (the deep motor branch of the median nerve), the radial nerve, and the ulnar nerve; check vascular function of hand;; get urgent T&O review AP/lat XR of elbow; subtle signs inc displacement of ant humeral line (should be in middle 3rd of capitellum), or lucnecy in posterior fat pad immediate closed reduction if neurovasc compromise (children will need theatre); gartland classification based on displacement to guide mx, after reduction get K wire fixation

Answer 58

proximal humerus is marked by a head, anatomical neck, surgical neck, greater and lesser tuberosity and intertubercular sulcus greater tuberosity - attachment site for three of the rotator cuff muscles – supraspinatus, infraspinatus and teres minor (subscap inserts onto lesser tuberosity) Pectoralis major, teres major and latissimus dorsi insert on the lips of the intertubercular sulcus and long head of biceps runs through it surgical neck extends from just distal to the tuberosities to the shaft of the humerus and is where ax nerve and circumflex vessels run shaft has spiral groove in which radial nerve and profunda brachii artery lie lateral and medial borders of the distal humerus form medial and lateral supraepicondylar ridges distal to which are the epicondyles; the trochlea sits medially and articulates with the trochlea notch of the ulna, and the capitulum (aka capitellum) sits laterally and articulates with the head of the radius supracondylar fractures: brachial artery at risk (comp syndrome, volkmanns contracture) as well as ant interosseous, ulnar, radial nerves; gartland classification: type 1, non displaced, usually above elbow cast; type 2 displaced with intact post cortex and type 3 completely displaced generally need int fixation with K wire look for anterior humeral line (ant humerus margin should form line running through middle third of capitellum) and radiocapitellar line (likewise - line running through middle of radial neck) to assess for elbow dislocation pulled elbow: subluxation as radial head pulled through annular ligament; forearm held pronated and in slight flexion, painful and supination/pronation will worsen; no joint bruising, swelling, erythema, or point tenderness on bone - if there is then XR; give analgesia then manipulate back into place

Answer 59

ossification centre for the capitellum develops at one year and then is followed by the radial head, medial epicondyle, trochlea, olecranon and lateral epicondyle. This order of appearance is specified in the mnemonic C-R-I-T-O-E each appears 2 years after the last this helps you tell whether something is a fractured bone or an ossification centre (eg if something might be the lateral epicondyle ossification centre but trochlea hasn't appeared yet then it is highly likely to be a fracture - however, some ppl do show a degree of variation)

Answer 60

cubitus varus or valgus from malunion of supacon or lat cond fractures; latter may give ulnar nerve palsy can get TB affect elbow as with shoulder or any joint, long chronic pain and stiffness history with marked wasting, warmth and swelling RA affects elbow in >50% pts; nodules over olecranon, tenderness around head of radius can get OA of elbow but uncommon olecranon bursitis: linked to gout or RA tennis and golfers elbow: often history of occupational stress or new activity eg house painting, carpentry etc; also often spont; pain on outside (lat epicon) for tennis but can radiate widely, aggravated by eg pouring tea, turning door handle, passive flexion to stress extensors may provoke pain; golfers around med epicon but can radiate widely, passive extension to stretch common flexor origin when arm supinated should provoke pain; rest, splint and physio, inject steroid and LA, surgery (step up as needed)

Answer 61

bursas in areas where skin and bone move in opposite directions, purpose is to reduce friction Common types of bursitis include prepatellar, olecranon, trochanteric, and retrocalcaneal; ICE, rest, NSAIDs usually if septic bursitis (based on bloods which you should get for everyone) then give abx steroid injections, or aspiration of pus can be done but doesn't heal well so try with just abx first; needs admission for iv abx if extensive cellulitis, suspect septic joint (joint rom limited and pain etc), or systemically unwell

Answer 62

TB and RA can both affect; OA doesnt often affect wrist proper except if previous AVN sec to fracture, but OA of thumb CMC joint common w/pain, swelling, tenderness, oft bilat, heberden's nodes of fingers common, eventually may get fixed adduction of the thumb giving characteristic deformity DQ tenosynovitis: pain and swelling on dorsal radial wrist, tenderness at tip of radial styloid, tendon sheath may feel thick; fairly common after childbirth or eg pruning roses, cutting with scissors, wringing out clothes etc; ulnar deviation passive while holding thumb tucked to palm will provoke sharp radial pain; can get tenosynovitis in other extensor compartments more ulnarlly too but DQT most common ganglion cysts: cystic degen of joint or tendon sheaths, most common dorsally; painless or slight ache, not tender, well defined; oft goes away after a few months carpal tunnel - menopause, RA, pregnancy, myxoedema all have this commonly occur so often 40-50yo woman; pain or paraesthesia in median nerve distribution, woken by burning pain at night, hang arm out of ebd or shake to relieve; tinel (tap) or phalen (hold flexed for 1 min) tests can reproduce symptom; late on thenar wasting, weakness of thumb abduction; splint to prevent wrist flexion, steroid injection, division of trans carp lig If there are mild or moderate symptoms, offer a 6-week trial of conservative treatment(s) in primary care, if available. Options include: Use of a wrist splint in a neutral position at night. These may be fitted by the local musculoskeletal service, depending on local referral pathways. A single corticosteroid injection into the carpal tunnel. refer if The diagnosis is unclear. There are persistent symptoms despite a trial of conservative treatment(s) in primary care. There are progressive symptoms or clinical features of severe disease seccare may do nerve conduction studies to rule out polyneuropathy, radiculopathy, do carpal tunnel surgery

Answer 63

can use various body parts based on known sequence of ossification, but wrist and knee areas represent the gold standard bone maturation process can be better characterized by the evaluation of the knee region in children under the age of 3, while in those older than 3 years, the assessment of hand and wrist bones is the most appropriate - usually via XR of left hand/wrist Capitate and uncinate bones are the first to show ossification centers (2nd to 4th month) while the pisiform is the last (9–12th year); can also assess progress of ossification of the phalanges process is strongly affected by numerous factors, including GH and insulin-like growth factor-1 (IGF-1). Moreover, a deficit of thyroid hormones or an excess of corticosteroids causes a cell division reduction in the proliferation zone, inducing a growth delay. Not only hormones but also gender might affect this process. In particular, bone age is more advanced in female than in male

Answer 64

mallet finger: can't straighten DIP, passive movement poss, due to injury to insertion of extensor tendon, splint for 8 weeks; can occur after stubbing finger when eg catching ball or making bed trigger finger: usually ring, mid, thumb, gets stuck in flexion when clench and unclench then snap to full extension with more force; due to thickening of fibrous tendon sheath or from tenosynovitis; usually wait for it to get better, can inject steroid infection: oft limited to one capsule or space, often staph, often from trauma which could be as little as a thorn prick; throbbing pain, swollen, tender, red tense tissue, movement restricted; assume all human or animal bites infected until proven otherwise OA: DIP joints commonly affected with knobbly heberden's nodes, sometimes Bouchards nodes on PIP joints if affected too, not MCP usually so if thats affected think RA

Answer 65

Pyogenic flexor tenosynovitis (PFT), also known as septic or suppurative flexor tenosynovitis or a flexor sheath infection, is a closed-space infection of the flexor tendon sheath of the hand can be haematogeneous but usually due to local trauma and connections between compartments allows for rapid spread proximally; severe cases can lead to tendon rupture, tissue necrosis, and require amputation of finger staph aureus and other commensals are most common causative organisms; kanavel signs used for scoring likelihood: a finger held in slight flexion, fusiform swelling of the affected digit, tenderness along the flexor tendon sheath, and pain with passive extension of the digit; start IV abx for any suspicion and refer for T&O assessment, if high suspicion or not improving on abx in 24 hrs then surgical irrigation and debridement

Answer 66

causes: peripheral nerve damage (ulnar, median palsies, cubital tunnel syndrome, guyon's canal syndrome), higher level nerve damage (brachial plexopathy inc klumpke's palsy, cervical radiculopathy), dupuytrens contracture, volkmann contracture, trigger finger dupuytrens: bnormal myofibroblastic growth in the hand, predominantly composed of type III collagen, cytokines linked inc PDGF, EGF etc; often eventually stabilises and may even regress nodules in hand then flexion contractures, rarely pain;; 4th finger most commonly affected then 5th; observation every 6mo if no functional problems; steroid injection if painful may work (risk of fat atrophy, tendon rupture), surgery if severe; associated with smoking, diabetes, alcohol, epilepsy, and positive family history; incidence increased in liver disease

Answer 67

any forearm fractures have risk of comp syndrome; open internal fixation oft best for adults (closed works better in kids) monteggia - upper 1/3 ulna breaks, radial head dislocates so no longer points at capitulum (can be missed), usually foosh/other force with pronation forced; ORIF mx galeazzi - much more common, distal 1/3 radius breaks (oft v tender), inf radioulnar joint subluxes or dislocates; ORIF mx colles - most common fracture in old ppl due to osteopor in post menopau women with transverse fracture of dist radius and dorsal displacement of dist fragment smith - Smith fracture is a break to the end of the radius with volar angulation if cant see scaphoid fracture but suspect one then splint and repeat x ray after 2 weeks; dont accept diagnosis of sprained wrist until more serious things definitely ruled out

Answer 68

in wrist commonest are: Os ulnostyloideum - unfused ulnar styloid process, 1.5-2.5% ppl Os centrale, between the scaphoid, the trapezoid and capitate (independent bone in most primates except african apes in humans where ti fused to scaphoid as adaptation to knuckle walking), 1.5-2.5% ppl in foot (sesamoids): sesamoid bones of first MTP - always present sesamoid of 5th MTP - 5% sesamoids of IP of hallux - up to 13% sesamoids possible for other joints too in foot (other): os trigonum - 7-25% (failure of fusion of lat tubercle of talus, can get trapped between calcaneus and ankle in some injuries causing irritation) os peroneum - up to 26% os naviculare - 2-21% (from failure to fuse properly, normally asymp but can strain posterior tibialis tendon giving chronic pain, and if tendon ruptures flat foot - NSAIDs/steroids, PT, surgery) fabella: in lat head of gastroc behind lat condyle of femur in 35% ppl, can cause posterolat knee pain if degenerates

Answer 69

as part of pmh always include recent illnesses; gals screen good for these pt septic arthritis - short history of hot, swollen, painful joint with restricted movement, consider this until proven otherwise; usually mono but can present poly; may see fever, rashes, even septic shock history of trauma; HIV, DM, prosthetic joints, extremes of age, bacteraemia (dentistry or injecting), immunocompromise, preexisting inflam arth are all risk factors; also sexual history as gonorrhoea can (rarely in dev'd countries) spread to the joints; TB might cause too so assess for that crystal arthropathy - gout is commonest inflam arth in men >40; usually monoarth early in morning in lower limb; pain often excruciating; oft have fever haemarthrosis - from trauma, minor trauma if inc'd tendency to bleed; intense pain so holds joint immobile reactive arthritis - usually healthy young adults in response to infection, often of git or GUT so again look for recent infection; period of incubation up to a month; asymmetric and oligoarticular, from mild to very disabling, freq associated with extra-articular features like iritis, tendonitis, urethritis, cervicitis first presentation of inflam arth - eg RA or spondyloarthropathy, or presentation of connective tissue disease like sle uncommon causes - lyme disease, brucellosis, acute HIV (social and travel history may help), tumour, sarcoidosis, whipples disease, rheumatic fever etc and consider bursitis

Answer 70

In a young patient presenting with a single acutely swollen joint always think of gonococcus septic arthritis until proven otherwise; patient may have urinary or genital symptoms a la reactive arthritis but remember that it is important to exclude gonococcal septic arthritis first staph aureus most common cause of sep arth though, others eg GAS, Haem inf, e coli Aspirate the joint prior to antibiotics and send the sample for gram staining, crystal microscopy, culture and antibiotic sensitivities Empirical IV antibiotics should be given until the sensitivities are known. Antibiotics are usually continued for 3 – 6 weeks in total. Choice of antibiotic depends on the local guidelines. Example regimes are: Flucloxacillin plus rifampicin is often first line Vancomycin plus rifampicin for penicillin allergy, MRSA or prosthetic joint Typically, reactive arthritis is caused by a sexually transmitted infection (STI), such as chlamydia, or an infection of the bowel; also after glandular fever or slapped cheek syndrome nsaids, maybe steroids if severe, and clear triggering infection

Answer 71

may occur secondary to trauma (inc surgery) or bacteremia common causes are staphylococcus aureus, strep, Hib pseudomonas and enterococci Patients with sickle cell anaemia have a higher risk of osteomyelitis, which is typically caused by Salmonella (intestinal infarction due to vaso-occlusion allows into blood, clearance then suppressed due to poor spleen function) Diabetes is a risk factor for poor healing and these patients therefore have a higher risk of progression of foot wounds/ulcers to osteomyelitis Patients may present with severe pain in a limb/foot/hand, as well as fevers; may be superficial warmth, erythema, swelling and in some cases a clear source of entry of an infection ix/mx: ◦ Bloods, blood cultures ◦ Swab of any ulcers/blisters etc. ◦ X-ray of affected area ◦ MRI is definitive imaging ◦ Prolonged course of antibiotics – typically 4-6 weeks but may be up to 12 ◦ Empirical antibiotics then guided by culture ◦ If the infection is around a prosthetic joint/bone will need stronger combo oft eg vanc + taz ◦ Good blood sugar control in diabetics ◦ If severe/does not resolve/start to improve with antibiotics = amputation may be required

Answer 72

bone has many rigid compartments so more susceptible to vasc damage and cell death when pressure builds up in acute inflam compared to soft tissue acute osteomyelitis usually in children (if in adults often due to dec immune resistance or trauma); infection into blood from trauma/abrasion, boil/abscess, infected umbilical cord (and so often staph aureus) or from catheter, vasc access/line, dirty syringe; settle in vasc metaphysis infection gives pressure so severe pain, malaise, fever, local signs of inflam, resist movement of joint if pus tracks into it; pus spreads along volkmans channels and out poss forming subperiosteal abscess and spreading along bone or into joint if metaphysis intracapsular (vasc epiphysis in infants makes easy to spread into joint for them) adults most common site for haemat spread is spine so backache and mild fever no early x ray sign, bone ragged over time as necrosis occurs with osteolytic appearance; MRI can spot locus of infection (T1 imaging) bloods, cultures, aspirate pus from subperi abscess, send to microbio might also be cellulitis (MRI if unsure), sickle cell crisis, tumour, TB, haematoma, caffey disease subacute form often child who's had pain in dist femur, prox/dist tibia for several weeks, lab tests may be neg but xray shows sclerotic bone can get chronic form with rec bouts of redness, tenderness etc at site of infection due to bacti remaining dormant (sometimes for years) in pockets of sclerosed bone, draining sinuses at infection site may be present

Answer 73

staph aureus, strep rarer but important cause (h inf if not vaccinated against it); penetrating wound, explosion of adjacent abscess (from eg osteomyelitis), or haematogeneous articular cartilage eroded, whole epiphysis in infants as is all still cartilage pain, warmth, and swelling of joint, oft hip in children and knee in adults but can be any joint; rapid pulse and swinging fever, movements restricted by pain infants may not move around on the joint normally anyway so present with septicaemia (irritable, not feeding, rapid pulse, fever) pt with RA, esp if on steroids, may get silent joint infection with unexplained general deterioration/sepsis - make sure to examine joints as possible source; check bloods etc for infection if unsure whether joint inflamed, if it is then aspirate the joint; other immunosuppressed eg poorly controlled DM may also present like this, may find a history of fall(s) septic bursitis common at olecranon in labourers and those with RA; pain, swelling, redness of bursa; aspirate bursal fluid

Answer 74

TB: affects bones/joints in 5% cases; haemat seeding from lung or gut; chronic caseating granuloma formation presenting feature is chronic monoarthritis of large joint, sometimes get secondary pyogenic infection; common in vertebrae which can cause disc to collapse forward giving kyphosis in local segment of spine besides pain may see muscle wasting, joint stiffness and deformity of joint, oft little pain if spine, abscess may form and track into loin, along psoas into leg, or to surface bone erosion and joint space narrowing on x ray

Answer 75

most at risk: femoral head, condyles, head of humerus, prox poles of scaphoid and talus fractures of hip, scaphoid, talus; also seen in Perthe's disease, sickle cell disease, Gaucher's disease, high dose corticosteroids, alcohol abuse, decompression sickness due to cap obstruction by gas bubbles and changes in marrow pt usually presents with pain in affected region and also stiffness, joint may be tender and/or swollen and movements restricted x ray will show subarticular region of increased density, then fracture and collapse of necrotic segment; joint space remains normal until very late unlike in OA; MRI is only way to pick it up early (x ray needs several months before change appears) - hypointense band in T1 outlining the ischaemic segment

Answer 76

Minimal early joint pain, Increased joint pain as bone and joint begin to collapse Limited range of motion due to pain fracture eg nof; alcoholism; high dose steroid use; sickle cell, transplants (inc bone marrow), kids perthes, radiation or chemo, hypercoag state, HIV; bisphosphonates can give osteonecrosis of jaw; decompression sickness, gaucher disease

Answer 77

typically seen in young people, is autoimmune; mean age 10yo, dx oft delayed as rare (1 in 1 million) Interleukin-6, interleukin-10, and TNF-a are thought to play a part in the development of the disease possible genetic contribution to CRMO with deficiency of interleukin-1 receptor antagonist (DIRA) due to mutation in IL1RN (interleukin-1 receptor antagonist gene) Typically, patients present with dull pain and swelling on the site of bone involvement. Local skin changes include tenderness and redness; may have history of similar episodes before; commonest locations include long bone metaphysis and vertebral bodies typically raised inflammatory markers like ESR, CRP, and leukocytosis; X-rays are the primary imaging modality and show lesions to be osteolytic in nature with surrounding sclerosis. The imaging modality of choice is MRI and it shows sites and extent of disease. Few of the important findings of MRI include soft tissue and bone marrow edema, periostitis, and extension across the physis NSAIDs or steroids first line, DMARDs if fail with these

Answer 78

unexplained lump increasing in size - urgent 2 week uss in adults, very urgent 48 uss in kids unexplained bone swelling or pain in kids - very urgent 48hrs x ray x ray suggests bone cancer in adults -2ww referral, in kids 48hrs referral uss suggests soft tissue sarcoma or is uncertain but clinical concern: 2ww in adults, 48hrs in kids generally bone sarcoma causes pain, maybe reduced function of the limb, maybe painless swelling; rapid mets to lung soft tissue sarcoma in connective tissue, generally presents as mass which is oft painless and can be quite large; biopsy to diagnose; most common form in kids is rhabdomosarcoma, others inc liposarcomas, fibrosarcomas, mesenchymomas; 40% rhabsarcs in head (esp paranasal sinuses and around nose), others in eg bladder, arms, chest, abdo etc surgery, radiotherapy postop (maybe preop to shrink), chemo (vincristine, doxorubicin, cyclophos, actinomycin D)

Answer 79

lump that may be painful or not, may be warm or not; x ray useful, also CT/MRI and biopsy mimics: chronic osteomyelitis (pain, swelling, bone destruction on x ray); stress fractures; haematoma, sickle cell, TB fibroma: common, benign nest of fibrous tissue in bone which lasts for a few years then ossifies; radioluscent area on xray near cortex, oval shaped; before spont healing a pathological fracture is poss chondroma: island of cartilage which persists in bone, can cause path fracture, contains tiny flecks of calcification within radioluscent area, can enlarge or be painful and these two things can (but arent always) be signs of a malignant change, which occurs in rare circumstances osteochondroma: cart overgrowth at end of physis, endochond ossif into bone with cart cap; any cart dev bone can have this, iliac crest and ends of long bones most common; hereditary multiple exostosis too; rarely malig change, spot if keeps growing after bones themselves stopped chondroblastoma: aching and tenderness of joint due to immature cart cells in the epiphysis (distinguishing feature)

Answer 80

osteosarcoma: highly malig tumour start in bone, spread to periosteum and soft tissue; 2 peak distribution, with incidence peaks 10-14yo (corresponds to growth spurt) and second smaller peak >65yo often secondary to eg pagets disease of bone or prev bone infarction; esp in long bone metaphys - constant pain, worse at night, increasing, sometimes a lump, often tenderness; often lytic, sometimes inc'd bone density, sunburst of new bone formation when cortex breached (on xray, hazy aura); CT for extent of tumour, pulm CT as 10% pt will have lung mets, biopsy; osteosarc is associated with hereditary retinoblastoma, li fraumeni, and others codmans triangle: sign where aggresive tumour growth lifts periosteum off the bone without chance for new bone to be laid down, seen with any aggressive bone tumour but esp osteosarcoma, ewings, chondosarcoma, mets chondrosarcoma: slow growing mass over many months, dull ache, usually pt in 40s/50s, radiolucent area with central flecks of calcification oft in metaphy of tubular long bones, or can arise from cart cap of osteochondroma multiple myeloma: weakness, backache, bone ache, osteoporosis and lytic lesions throughout bones, hypercalc leading to thirst, polyuria, abdo pain; anaemia, vertebral collapse may cause cord/root compression; myeloma one of commonest causes of osteoporosis and vert compress fractures in men >45yo; later stage renal dysfunction, viscous blood giving retinopathy and headaches; normocytic anaemia, ESR high; fractures bone mets: more common than all prim malig combined; breast then prostate, kidney, lung, thyroid, bladder, GI, myeloma, lymphoma; vert, pelvis, prox femur, humerus most likely to get; osteolysis giving fractures; pain commonest, sometimes only, feature; sudden appearance of back or thigh pain in pt >50 always suspicious, esp if had carcinoma before; hypercalcaemia may occur

Answer 81

Commoner in males Incidence of 0.3 / 1, 000, 000 Onset typically between 10 and 20 years of age Location by femoral diaphysis is commonest site Histologically it is a small round blue cell tumour Blood borne metastasis is common and chemotherapy is often combined with surgery Tends to cause severe pain associated with t(11;22) translocation which results in an EWS-FLI1 gene product x-ray shows 'onion skin' appearance may present with bone fracture entities with similar clinical presentations include osteomyelitis, osteosarcoma

Answer 82

OM is common tumour mimic so consider it (also eg TB, haematoma, stress fracture) infection/other dd or tumour -> if tumour then prim or sec -> if sec then carcinoma met (breast, prostate, lung, kidney, thyroid), haemat (MM, lymphoma), or melanocytic (melanoma); if prim then benign (osteochondroma/blastoma, GCT etc) or malig (osteo/fibro/chondrosarcoma) MGUS->10% pts will dev MM in 10 years; paraproteinaemia from CTD, lymphoma, HIV, cryoglobulinaemia, MGUS etc hypercalc -> PTH (prim), malignancy, granulomatous disease, vit D toxicity (macros in granuloma disease may make excess activated vit D) familial hypocalciuric hypercalcaemia (creatinine calcium clearance ratio helps you tell from prim hyperpara, also it's rarer and auto dom) hypocalc - hypopara, vit D def, renal impairment/CKD, drug induced

Answer 83

history from child and carers looking for red flags; developmental including walking history and neurodev; any recent illnesses and trauma paediatric GALS and general exam: fever/tachy, pallor, lethargy/irritability, bruising (maltreatment?), lymphadenopathy or rash red flags: pain waking at night (malig poss); redness, swelling, stiffness (joint inflam/infection); weight loss, anorexia, fever, night sweats, fatigue (malig or inflam/infect); unexplained rash/bruising (haemat or inflam disease, or maltreatment); limp and stiffness worse in the morning (inflam); severe pain, anxiety, or agitation after traumatic injury (comp syndrome) refer from GP for urgent assessment: under 3yo, >9 w/ restricted/painful hip movements, unable to weight bear, fever or red flags; possible neurovasc compromise in hospital setting management of child with limp: if trauma then X rays, consider referral to orthopaedics and/or involving CPS if no trauma: assess as above; if symptoms for <72 hrs or >72 but improving, child is mobile, well overall, no red flags then analgesia (ibuprof/paracet), consider if safeguarding problem, rule out slipped femoral epiphysis; send home and review in 48-72 hrs if not improving if symptoms >72hrs with no improvement but no red flags then get senior review, to consider need for X rays and bloods (FBCs and film, CRP, culture); if infection red flags (which can include inability to weight bear, pain on passive movement) then urgent AP and frog lat X ray, bloods as above, ortho review; if malig red flags then urgent paediatrics review

Answer 84

age <3: dev dysplasia; NAI; tranisent synovitis rarer in this age group but poss age 3-10: transient synovitis common (onset after viral infection, acute, no systemic upset, no pain at rest, passive movements only hurt at extreme ends, recurs in 15% of cases, oral analgesia); perthe's disease age 10+: perthe's; slipped upper femoral epiphysis (usually 11-14, risk inc'd by being boy and or obese, may be bilat, may present as knee pain, same day x ray essential; groin pain usually, or just hip or knee, may hold hip flexed and externally rotated with reduced rom, waddling gait and/or leg turned out, pain can be bad enough to not bear wait); is salt har t1 any age: septic arthritis, osteomyelitis, fracture, malig inc leukaemia; sickle cell, haemophilia, rickets, cerebral palsy, spina bifida, leg length discrep, JIA

Answer 85

Full history and examination after analgesia +/- plain x-rays of focussed area if clear diagnosis eg toddlers fracture implement specific mx for that condition, which might involve onwards referral to ortho if unclear and systemically unwell inc fever, or refusing to walk, recurrent, limp for >2 weeks then consider bloods and refer to ortho if unclear but systemically well, afebrile with no history of fever and able to do partial weight bearing if >8 and localised to hip get frog lateral view if not obtained and refer if abnormal, if normal or localised to hip and <8 or site unclear then: advise to rest limb, use regular NSAID, f/up in 1 week with advice to return to ED immediately if:  becomes unwell  develops a fever  becomes completely non-weight bearing  develops red / hot then at review if systemically well and afebrile and improvement in symptoms / signs then review at 14 days or discharge if unwell/fever/worsening/not improving then refer to ortho

Answer 86

Transient synovitis: idiopathic inflammation of hip joint synovium with effusion. Typical age is 3 – 10 years. There is usually a history of preceding minor illness or trauma. Children tend to weight bear with a limp and have mild restriction of hip movements. They may have a tender groin. They are almost always afebrile. Will gradually improve over 10 days. Treat with rest and NSAIDs and arrange follow-up (can be with GP) in 7-10 days. Infections: usually have severe pain and do not weight bear at all. The children are usually febrile and will often be systemically unwell and irritable. Inflammatory markers are usually raised – WBC > 12; CRP is an early sensitive marker; ESR is specific but less sensitive than CRP. Septic hip: usually acute onset with severe hip pain, limited hip movement and spasm with passive movements. Gold standard investigation is aspiration of the joint but USS is often used. Osteomyelitis: usually haematogenous spread to metaphyses of growing bones. There is often local swelling, erythema and tenderness. Bony changes on plain films are not evident until 14 –21 days (periosteal elevation then lucencies). Diagnose with bone scan or MRI DDH - If it is not picked up in neonatal period DDH usually presents as a painless limp. Examination usually reveals a short leg if unilateral or a classical waddling gain if bilateral. There is usually limited abduction of the affected hip(s). Plain x-ray will be diagnostic Cerebral palsy: The child will have a painless limp and may toe walk. Limbs will be hypertonic and hyper-reflexic. Toddlers fracture: a spiral fracture of the distal tibia. There may be a history of minor trauma (usually twisting of the foot, for example getting leg caught going down a slide) or no trauma at all. Often the child refuses to weight bear. There may be bony tenderness over the tibia. Plain xray may show a fracture or frequently does not. Treat with analgesia and conservative management unless parents are keen for a cast - will walk sooner if not immobilised idiopathic avascular necrosis of the femoral head. It usually affects 4 – 10 year olds and is more common in boys. There is usually a history of chronic limp and pain. Hip movements are restricted. Plain x-rays are usually normal in the early stages. The first x-ray sign is increased density of the femoral head SUFE - head of the femur slips posteriorly and inferiorly relative to the neck at the epiphysis. There is usually a history of chronic limp and pain but it may present acutely as non weight bearing. Children with SUFE often complain of knee pain due to activation of medial obturator nerve. They are commonly overweight and have limitation of hip movements most commonly atraumatic, although some present after an injury pain has often been present for weeks to several months Iliopsoas muscle inflammation: from intra-abdominal or retroperitoneal disease (eg appendicitis) or psoas muscle abscess. Typically the child holds their hip in flexion and complains of pain with passive extension of the hip. Psoas muscle abscess is diagnosed with USS or CT. Overuse syndromes: usually in physically active children or sports players. Pain and limping typically occurs with activity. The usual management is rest (+/- crutches +/- cast or splint) and NSAIDs. Stress fractures – repetitive loading of the lower limbs. Usually affects the tibia or fibula. May be seen on x-rays as sclerotic lines or a periosteal reaction. Confirm on bone scan. Calcaneus apophysitis (Sever’s disease) – child complains of heel pain. Can be chronic or intermittent. Other causes of heel pain include calcaneal spurs, plantar fasciitis, and peroneal tendinopathy. Patellar tendonitis Apophysitis of tibial tubercle (Osgood-Schlatter disease) – classically occurs in footballers. The child complains of knee pain and tenderness over tibial tubercle. Osteochondritis dissecans – a portion of subchondral bone within the joint becomes avascular. Typically occurs around the knee. Tarsal coalitions – fusion of some of the tarsal bones which is usually asymptomatic but may cause pain, pes cavus, or recurrent ankle sprains. Diagnose on x-ray

Answer 87

ALP is produced by osteoblasts and used in mineralisation of bone, so it is raised when bone is being laid down produced by osteoblasts to provide a high PO4 concentration at the osteoblast cell surface during bone mineralization (though some argue this view outdated and main role is to remove inhibitory pyrophosphate [inhibitory by preventing hydroxyapatite formation, which is the Ca phos salt used in bone mineralisation)) see it high in bone growth, pagets, healing fracture, acromegaly, osteogenic malignancies (some sarcoma, bone metastases, leukaemia, myelofibrosis, and rarely myeloma); also hyperthyroidism after fracture ALP rises, peaking up to 3 weeks after

Answer 88

insidious symmetrical polyarthritis, usually hands and feet, also early morning stiffness mentioned spontaneously, and lack of general well being warmth and tenderness of prox finger joints and wrists, may be swollen too, joint movements limited, tendon sheaths rupture periarticular osteoporosis and soft tissue swelling then narrowing of articular space usually hands first then other joint: elbows, shoulders, knees, ankles, feet; occasionally will begin in a larger joint and even seem like a monoarthritis like TB subcut nodules over olecranon in 25% (but major hallmark if present) ulnar deviation of fingers, radial displacement of wrist, valgus knees and feet, clawed toes; pain and stiffness in cervical spine in 1/3 patients muscle wasting, lymphadenopathy, skin atrophy or ulceration, scleritis, lung fibrosis/effusion esr and crp up, anaemia often from disrupted erhytropoi; RF pos in 80% pts, antinuc factors in 30%, anti-CCP rises early min diagnostic crit: bilat symmet polyarth in prox joints of hands or feet for at least 6 weeks oral steroids for initial bridging when start DMARDs (methotrexate, sometimes eg sulfasalazine, leflunomide), can offer multiple then biologics; reconstructive surgery after joint destruction, fixed deform dev etc NSAIDs with PPI to control pain if reg treatment not enough, be careful of risk factors inc if on aspirin

Answer 89

in RA the joint erosions are much closer to the margin than gout where it's a bit further away anti-CCP best earlier marker, better than RF (though only 70% sensitivity); ant-ccp goes up few months before symptoms even appear RF only reaches 75% sensitivity; esr and crp may be negative RA management: First line is monotherapy with methotrexate, leflunomide or sulfasalazine. Hydroxychloroquine can be considered in mild disease and is considered the “mildest” anti rheumatic drug. Second line is 2 of these used in combination. Third line is methotrexate plus a biological therapy, usually a TNF inhibitor. Fourth line is methotrexate plus rituximab DAS28 used to monitor Anti-TNF (adalimumab, infliximab, etanercept); reactivation of dormant TB, HBV etc; also immunosup'd Leflunomide: Hypertension and peripheral neuropathy Sulfasalazine: Male infertility (reduces sperm count), headache, *agranulocytosis*, drug induced lupus, pancreatitis Hydroxychloroquine: Nightmares and reduced visual acuity also be aware of drug induced lupus

Answer 90

felty syndrome: rheum arth, splenomegaly, neutropenia (combo gives rec infections) (splenomeg may cause thrombocyto, anemia) manage the rheum, also consider eg splenectomy to improve neutropenia as need to control rec infections

Answer 91

Methotrexate is a folic acid antagonist and is classified as an antimetabolite cytotoxic agent. Dose initially 10-15mg/m2 once weekly oral or subcutaneous injection Folic acid should be prescribed when weekly Methotrexate treatment is initiated - Folic acid should not be taken on the same day as methotrexate Prescribing of methotrexate with co-trimoxazole or trimethoprim is an ABSOLUTE CONTRAINDICATION and MUST NOT occur under any circumstances as methotrexate and trimethoprim have the same mode of action other contras: * Active infection for example TB * Ascites * Immunodeficiency syndromes for example SCID or HIV * Significant pleural effusion * Pregnancy and breast feeding. before starting: Baseline bloods including FBC, CRP and CRP, liver transaminase levels, serum creatinine/calculated GFR Consider pregnancy testing in Females of childbearing age. Check measles and varicella vaccine/immunity status monitoring bloods: * Full blood count * Urea and electrolytes, * Liver function tests (ALT/AST) * Bone Profile. * CRP and PV - Check every four weeks for the first 2 months after starting methotrexate treatment or after changing the dose of methotrexate. - If stable at 2 months check, check 6 weekly for three months. - Thereafter check every two months or 3 monthly maximum When to consider suspending methotrexate: * Platelet count less than 150x109/L. * White cell count less than 3x109/L. * Neutrophils less than 1.5x109/L. * Lymphocytes less than 0.5x109/L * Haemoglobin less than 90mg/dL (without another identifiable cause). * AST or ALT three times the upper limit of the normal range Children on low dose Methotrexate treatment are safe to receive all inactivated vaccinations. Live vaccinations should not be given apart from the nasal influenza vaccine most common adverse effects are gastrointestinal manifestations such as nausea, vomiting, mucosal ulcers, and loss of appetite - ondansetron can help, and taking the folic acid mucosal ulceration, alopecia, fatigue, fever, increased risk of infection, leukopenia, GI bleeding, pancreatitis, cirrhosis, aplastic anaemia, malignancy (lymphoproliferative disorders), infections, interstitial pneumonitis, renal impairment are more serious side effects - if pt presents, give folinic acid, the reduced form of folic acid that bypasses the stage MTX acts on to rescue downstream function; alkalinisation of urine helps remove MTX from system, and pt may also need IVF, g-CSF, blood products

Answer 92

anti-ds, anti-sm = sle; anti-histone = drug induced lupus anti-scl-70 - systemic sclerosis; anti-centromere - limited cut sys scler (crest) ro/la is sjogrens; jo (RNA synthetase), polymyositis, often also dermato Mi2 is dermatomyositis immunoflourescence - homogenous or speckled, speckled shows specific extractable nuclear antibodies present as above ANA is very sensitive for CTDs, but people without condition may also have SLE gives no joint erosions, but periarticular osteopaenia

Answer 93

Antibody titres are essentially based upon how many dilutions it takes to dilute your original sample of antibodies to a predetermined point (pretend it’s zero). The more antibodies you have, the more dilutions (denominator value) it takes 1:500 means you can dilute the sample 500x and still detect the "thing". That would mean there's a lot of it. 1:20 means after 20 dilutions you lost sight of the "thing" because there's less of it. Basically the higher the second number, the higher the titre

Answer 94

SLE in all ages, commoner in girls esp after menarche, and in afro-caribbean pop malaise, fever, weight loss, tiredness, lymphad, splenomeg, sym usually small joint polyarth butterfly rash (diffs inc dermatomyositis, malar flush of mitral stenosis, eczema, contact dermatitis) - in SLE oft photosensitive, nail-fold infarcts, splinter haemorrahges, raynauds, petechiae (from platelets low), panniculitis (may look like erythe nod) renal involvement commonest way to present in kids, usually as nephrotic syndrome but GN can occur, or slow prog to renal failure CNS involvement may give periph neuropathy, nerve palsies, fits, changes in behaviour, psychosis; can get perdicarditis, cardiac failure, libman-sacks endocard; can get autoimmune leucopenia, thrombocytopenia, coombs pos haem anaem; can get lower lobe pulm fibrosis, pleuritis, effusion drug induced lupus syndrome similar w constitutional symptoms dominating and arthritis maybe even larger joints; kidney, neuro, malar rash rare onset after starting SHIPP (sulfasalazine, hydralazine, isoniazid, phenytoin, procainamide)

Answer 95

esr raised, ANA in 95%, dsDNA in 75%, RF in 30%; low levels of C3/4, esp if renal involvement; renal biopsy if suspect involved transfusion, analgesics, abx, (all as required), steroids, maybe immunosuppressants (hydroxychloroquine, stronger only if needed) for drug induced antihistone antibodies often seen; discontinue causative medication and consider eg hydroxychloroquine maternal SLE -> transplacental IgG giving skin rash (pink maculopapular), 50% have complete heart block (permanent, will see bradycardia in post-natal exam), thrombocytopenia, anaemia; ix baby (and mum if asymp) for ANA, anti-ro, anti-la maternal SLE also linked to rec miscarriage and stillbirth

Answer 96

chronic disease, so patient education really important! then sun blocks/protection for photosensitivity; hydroxychloroquine for immunomodulation; if worrying diease then azathioprine, methotrexate, prednisolone, cyclophos, mycophenolate; if not responding then rituximab hydroxychloroquine -> increases lysosomal pH

Answer 97

Compared to adult-onset SLE, jSLE is more aggressive, with higher disease activity and medication burden only a few patients diagnosed with SLE carry disease-causing mutations in single genes. Most individuals have a genetically determined risk for the development of SLE (e.g. disease-associated risk alleles) that requires additional factors to be present or accumulate over time to result in clinical disease EULAR score used to diagnose, only after raised ANA titre identified; it encompasses the following points, so suspect SLE if sufficient of these present: fever, leucopenia, thrombocytopenia, haemolysis, delirium, psychosis, seizures, alopecia, oral ulcers, discoid rash, butterfly rash, pericarditis, pleural or pericardial effusion, arthritis, proteinuria or nephritis, low C3 +/- C4, anti-dsDNA, anti-smith, or antiphospholipid syndrome antibodies (lupu anticoagulant, anti-cardiolipin mx in acute setting is with pulsed methylpred +/- other immunosuppresants +/- plasmapharesis maintenance then under rheum with DMARDs

Answer 98

autoimmune disease that takes place due to the passive maternal transfer of autoantibodies ro and la across the placenta (so mum can have sjogren syndrome, SLE, RA and others inc having the antibodies without clinical disease) skin lesions in 40% of pts, resemble those seen in older ppl with photosensitive, discoid rashes that generally remit within a year can present with anaemia, neutropenia or thrombocytopenia; asymptomatic elevation of aminotransferases, cholestasis, or hepatomegaly/splenomegaly in 15% to 25% of the cases classic and distinctive cardiac manifestation is a congenital atrioventricular block that can present as first, second, and especially third-degree AV block; rarely associated with signs of congestive heart failure (diaphoresis, pallor, peripheral oedema, prominent jugular veins and crackles on auscultation of the lungs) Non-cardiac reversible manifestations resolve spontaneously. Therefore, observation is advised of these clinical symptoms. Photoprotection is the key to management of cutaneous manifestations. Topical corticosteroids and antimalarial drugs are not indicated, and if there are residual telangiectasia laser therapy can be used. Anaemia and thrombocytopenia that become symptomatic can be managed with blood and platelets transfusion; Congenital heart block is usually detected when the fetus is between 18 and 24 weeks old. The condition does not disappear, and affected infants will eventually need a pacemaker Neonatal lupus erythematosus causes 80 to 95 percent of the cases of severe atrioventricular block in the neonatal period (younger than 28 days of life)

Answer 99

systemic autoimmune disorder commonly presenting with dryness involving the eyes and mouth due to inflammation and resultant pathology of the lacrimal and salivary glands. Up to one-half of affected individuals also develop extra-glandular involvement in organs such as the joints, skin, lungs, gastrointestinal (GI) tract, nervous system, and kidneys Extraglandular involvement often manifests in polyarthritis, lower extremity purpura, and/or evidence of peripheral neuropathy Other glands affected giving nasal dryness, vaginal dryness, dry skin etc; smell and taste may be altered due to less saliva etc frequently associated with other autoimmune disorders including rheumatoid arthritis (RA), scleroderma, and systemic lupus erythematosus (SLE) in which case it is called secondary sjogrens lymphocyte-rich mononuclear cells infiltrate exocrine glandular tissue causing gland dysfunction; mostly T cells but other WCC lines seen too; Rarely, malignant transformation of B lymphocytes can result in non-Hodgkin lymphoma. Immune complexes may deposit autoimmune studies, salivary gland functional assessment, slit lamp assessment for conjunctivitis sicca, and biopsy of salivary gland; salivary glands will often be enlarged; assess lymph nodes for swelling given increased NHL risk artificial tears, can give ciclosporin eye drops or occlude puncta; drink water, chew gum, artificial saliva, pilocarpine may help; nasal dryness can have humidifier or nasal saline irrigation; extra-glandular effects may benefit from systemic steroids - DMARDs also sometimes used

Answer 100

chronic, autoimmune connective tissue disorder In localized scleroderma, the skin is the main organ system involved and muscles and bones may or may not be. In systemic scleroderma (aka systemic sclerosis), internal organs such as the digestive tract, heart, lungs, and kidneys may be affected localised: 2 types, morphea (giving erythematous -> white plaques surrounded by purple, oft itchy, not painful, then atrophy and become pale, generally on trunk or prox limb) or linear (bands of tight skin, oft face or limbs and commonest form in kids systemic sclerosis: cutaneous disease may be limited (CREST syndrome - calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia and often PAH leading to cor pulmonale - skin involvement limited to hands/arms/face), diffuse if rises above level of elbow/knees, get ILD, dysmotility (predisposes to SIBO), cardiac conduction system defects, pericardial involvement, myalgia, arthritis, renal involvement inc scleroderma renal crisis (acute AKI and HTN)

Answer 101

autoantibodies, esp anti centromere and anti scl 70; echo, high resolution chest CT, lung function tests inc TLCO/DLCO, hand XR, oesophageal manometry, ECG, baseline bloods, NT-pro BNP immunosuppression done if recent onset; CCBs for raynauds, iloprost IV if severe, avoid beta blockers/cold and quit smoking; prokinetics and PPI, abx if SIBO; ACEi if renal crisis is v effective often; PAH managed with normal pulm HTN drugs and possibly LTOT; steroids can be used for inflam joint/myopathy but may trigger renal crisis morphea gets topical steroids; generalised morphea gets systemic steroids, maybe methotrexate or phototherapy; linear form generally needs sys steroids + methotrex

Answer 102

for 2 weeks: Bilateral shoulder pain that may radiate to the elbow, worse on movement Bilateral pelvic girdle pain Stiffness for at least 45 minutes in the morning weight loss, fatigue, low grade fever and low mood highly responsive to steroids, so if they dont work reconsider if actually weak not just stiff/achey, or if <55, probably inflam myositis (dermatomyosit etc) new onset raynauds but w/o other symptoms of SLE might be systemic sclerosis initial presentation

Answer 103

similar to RA but spine and sacroiliac joints; back stiffness and pain oft worse in morning or after inactivity, commonly in young 15-25yo male and relatives may have this or psoriatic arth 10% pain in other joints too, also painful achilles may have uveitis, aortitis, aortic regurg, heart block from carditis, apical fibrosis of lung fuzziness or erosion of sacroiliac joint on x ray exercises and stretching, NSAIDs oft v effective, TNF inhibitors can have good response if needed note related condition is reactive arthritis: after urogenital or bowel infection, gout like symptoms in asym selection of knee, ankle, tarsal and toe joints, achilles tenderness, backache; history of urethritis, cervicitis, prostatitis, diarrhoea; rec attacks poss

Answer 104

group of conditions with inflam of spine and joints reactive, ank spond, psoriatic, enteropathic (IBD); HLA-B27 associated, esp ank spond; asymmetrical, inflam targets where tendons/ligaments attach RA uses B and T cells - rituximab helps; PA is T cells only, so rituximab not helpful, but NSAIDs good irregular widening, erosions and sclerosis at sacrum -> anky spond; bridging calcification -> bones fuse; shiny corners on vert show inflam where the ligaments attach; spinal ligs ossify leaving a bamboo spine and dagger sign histo of RA shows panus: protrusions of inflamed, oedematous tissue with congested vessels, rather than smooth synovium polyarticular inflam arth can be caused by many viruses, may look a bit like RA but lasts < 6 weeks; hep, parvo etc, oft quite acute onset and symet reactive arth: acute onset fever/myalgia, asym oligoarth, usually lower joints, aspirate to rule out septic arth see proliferative enthesitis: whiskery new bone formation where tendons attach; give nsaids, maybe steroid injections etc

Answer 105

rheum fever: molecular mimicry from GAS; sore throat, carditis, polyarthritis, subcut nodules, erythema marginatum, chorea; last 3 jones criteria incs above as major, fever, arthritis, raised esr/crp as minor arth itself is symet oligoarth: knees, ankles, elbows, wrists; one joint after another (additive), inflam so red/warm etc; usually settles in 4 weeks whipples disease: chronic diarrhoea, weight loss, and arthritis in middle aged men; can look like IBD with diarrhoea, malabsorb, weight loss; small bowel biopsy -> PCR for bacti signs, histology showing pas positive macros 2 step serology for lyme disease -> elisa, then confirmatory western blot chronic long term lyme disease can present with oligoarthritis (may not remember being bitten but have history of bells palsy, heart block etc) sarcoidosis acutely can cause reactive arth looking picture (often ankle), may see erythema nodosum, bilat hilar lymph etc

Answer 106

gout risks: dietary (beer, proteins), haemat cancers; dec'd excretion: insulin resistance, renal impairment, diuretics feet in men, knees in women (obvs can be either for both); rat bite, extra-art erosion, preserved bone density negatively birefringence needles on synovial fluid polarised light microscopy; attacks last days; oft have fever acute: NSAIDs first line, Colchicine is used in patients that are inappropriate for NSAIDs, such as those with renal impairment or significant heart disease. NSAID for 1-2 days after attack finishes. Or can do oral pred for 3-5 days off label as safer alternative to colchicine, and if NSAID/colchicine not safe or effective another option is IM depo-medrone (methylpred); ice + paracetamol as adjuncts proph: allopurinol or febuxostat - continue during flares; assess for and manage risk factors including checking for CKD; start 2-4 weeks after flare unless frequent, start low and titrate up until target urate levels reached; when starting or titrating cover with colchicine/NSAID/steroid in gout can get tophi: a deposit of monosodium urate crystals due to hyperuricemia, pathognomic for gout; appear yellow and chalk like if break through skin, otherwise like large mass in joint/bone/cartilage (inc ears), usually 3-15 years after first attack pseudogout: knees, shoulders, elbows, wrists; oft affects ppl with florid OA (ca phos dep as healing occurs) attacks last week, quite often in elderly ppl with chronic OA; weakly positively birefringent rhomboid crystals in younger pts: joint trauma, hypercalcaemia, haemochromatosis, wilson's disease RICE and anti-inflam as above

Answer 107

large (GCA, aortitis, takayasus, steroids), medium (PAN, associated with HCV), medium/small is ANCA associated (GPA, eGPA, MPA, steroids +/- immunosuppression), small is HSP ANCA associated - often come in young, quite sick; asthma, neuropathy, renal, skin, eosins eGPA; ENT, pulmonary, renal, skin GPA; renal, pulmonary, skin, pANCA MPA investigations -> biopsy (eg renal) -> steroids +/- cyclophos or DMARDs Libmann-Sacks endocarditis is a type of non-bacterial endocarditis where there are growths (vegetations) on the valves of the heart. The mitral valve is most commonly affected. It is associated with SLE and antiphospholipid syndrome Takayasu’s arteritis is a form of large vessel vasculitis. It mainly affects the aorta and it’s branches. It also affect the pulmonary arteries. These large vessels and their branches can swell and form aneurysms or become narrowed and blocked. This leads to it’s other name of “pulseless disease”. It usually presents before the age of 40 years with non-specific systemic symptoms, such as fever, malaise and muscle aches, or with more specific symptoms of arm claudication or syncope. It is diagnosed using CT or MRI angiography.

Answer 108

systemic necrotizing vasculitis typically affects medium-sized arterial vessels but may affect small-sized arterial vessels. Unlike other small-sized arterial vessel vasculitides, polyarteritis nodosa is not typically associated with anti-neutrophil cytoplasmic antibodies (ANCA) Primary cases of PAN are idiopathic. Secondary PAN is less frequent and often seen in hepatitis B, hepatitis C, and malignancies such as hairy cell leukemia also linked to eg Klebsiella, Toxoplasma, Pseudomonas, trichinosis, parvovirus B-19, and Yersinia as well as RA and sjogren Affected vessels become thickened and inflamed through intimal proliferation. This leads to vessel narrowing and decreased blood flow, predisposing affected vessels to thrombosis. Inflammation not only leads to vessel narrowing but the weakening of the vessels which leads to aneurysm development. affects multiple systems including renal, skin, neurologic, cardiac, gastrointestinal, and muscular. The kidney is the most commonly affected Patients with PAN and renal involvement most often present with hypertension - if longstanding may dev HF; generalized fatigue, weight loss, fevers, arthralgias, and skin lesions (purpura, erythema nodosum, livedo reticularis, ulcers); abdo pain, oft after meals (intestinal angina), N&V; neuro sx, esp mononeuritis multiplex (radial, ulnar, peroneal commonest sites) dip urine for protein; ESR up, need U&Es, LFTs, CK, hep serology, ANCA, ANA, complement levels, cryoglobulins, rheumatoid factor, HIV serology, renal/abdo artery USS or arteriography, organ biopsy prednisone over months; sometimes eg azathioprine, methotrex, cyclophos; ACEi and monitor U&Es for HTN (CCB is second line); antivirals if pos for infection; surgery if aneurysms, ischaemic gut etc; medical mx for months-years

Answer 109

vexas = vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (mutation is somatic not inherited); V: Vacuoles are often identified in the bone marrow stem cells of patients presenting with VEXAS. E: The E1 ubiquitin conjugating enzyme encoded by the UBA1 gene is mutated in patients. monogenic disease of adulthood (onset >50 yo) caused by somatic mutations in UBA1 in hematopoietic progenitor cells. Patients develop inflammatory and hematologic symptoms inflammation involving the skin, lungs, blood vessels, and cartilage often leads to the assignment of various clinical diagnoses, including Sweet syndrome, relapsing polychondritis, polyarteritis nodosa, and giant cell arteritis; hematologic problems, including macrocytic anemia, thrombocytopenia, thromboembolic disease, and progressive bone marrow failure, which can evolve to hematologic malignancy (myelodysplastic syndrome esp) should be considered in patients with treatment-refractory inflammatory disease with associated progressive hematologic abnormalities

Answer 110

1-alpha hydroxylation normally occurs in the kidneys → CKD leads to low vitamin D the kidneys normally excrete phosphate → CKD leads to high phosphate This, in turn, causes other problems: the high phosphate level 'drags' calcium from the bones, resulting in osteomalacia low calcium: due to lack of vitamin D, high phosphate secondary hyperparathyroidism: due to low calcium, high phosphate and low vitamin D aim is to reduce phosphate and parathyroid hormone levels. Overview reduced dietary intake of phosphate is the first-line management phosphate binders vitamin D supplements: alfacalcidol, calcitriol parathyroidectomy may be needed in some cases Phosphate binders aluminium-based binders are less commonly used now calcium-based binders problems include hypercalcemia and vascular calcification sevelamer a non-calcium based binder that is now increasingly used binds to dietary phosphate and prevents its absorption

Answer 111

cancerous plasma cells invade the bone marrow. This is described as bone marrow infiltration. This causes suppression of the development of other blood cell lines leading to pancytopenia Myeloma bone disease is a result of increased osteoclast activity and suppressed osteoblast activity. Osteoclasts absorb bone and osteoblasts deposit bone. This results in the metabolism of bone becoming imbalanced as more bone is being reabsorbed than constructed. This is caused by cytokines released from the plasma cells High levels of immunoglobulins (antibodies) can block the flow through the tubules Plasma viscosity also increased B – Bence–Jones protein (request urine electrophoresis) L – Serum‑free Light‑chain assay I – Serum Immunoglobulins P – Serum Protein electrophoresis Bone marrow biopsy is necessary to confirm the diagnosis of myeloma Imaging is required to assess for bone lesions. The order of preference to establish this is: Whole body MRI Whole body CT Skeletal survey (only need one, whichever highest they can tolerate) chemo (and may need VTE proph on certain regimes) Myeloma bone disease can be improved using bisphosphonates. These suppress osteoclast activity. Radiotherapy to bone lesions can improve bone pain. Orthopaedic surgery can stabilise bones

Answer 112

enlargement and deformation of a bone, can be any, in bad cases can be multiple; alternating phases of rapid resorption and formation; early on resorption predominates giving easily deformed bone, later the bone is sclerotic and brittle increasingly common as pt gets over age of 50; pelvis and tibia most common sites, then femur, skull, spine, clavicle often found incidentally on x ray or when raised ALP noticed (so if see raised ALP w/o another cause you should suspect this as a possibility); pain not always, may be dull and constant; in lower limb it may look bent and feel thick; skull may enlarge so eg old hats dont fit anymore; kyphosis may occur and cranial nerves at risk of compression so dysfunctions eg hearing loss, facial palsy etc; x ray shows thick, sclerotic, often bent bone that may have coarse trabeculation; path fracture poss ca and phos usually normal but dec mobility may lead to hypercalc; there is risk of malignant change to osteosarcoma: suspect if known site becomes swollen, painful, tender (1% cases have malig change)

Answer 113

secondary osteoporosis - suspect if osteoporotic fracture in men <60yo or women premenopause malabsorption/nutrition, TB, rheumatoid disease inc ank spond; corticosteroids, alcohol abuse (common and oft overlooked cause), carbamazepine and phenytoin etc; hyperpara, thyrotox, cushings, gonad insuff, chronic renal disease, COPD and always consider metastatic bone disease (high esr, anaemia, generalised osteoporosis may suggest) inc leukaemia and multiple myeloma; thus eg steroid treatment for RA is esp high risk and bones need protecting osteomalacia - defects in vit D eg malab/nut, liver or kidney disease, underexposure to sunlight; years of bone pain, backache, muscle weakness, and eventually a fracture; note osteopor is reduction in bone mass, osteomal is softening of bone; after diagnosis be sure to investigate for cause; signs pointing to osteomal instead of osteopor: generalised chronic ache, muscle weak, vit D levels down chronic kidney disease: liable to have some combo of osteomalacia, secondary hyperpara (phos retention), osteoporosis

Answer 114

major risk factors for osteoporosis are age and female gender. Other risk factors include: corticosteroid use smoking alcohol low body mass index family history Rheumatoid arthritis Guidelines recommend using a screening tool such as FRAX or QFracture to assess the 10-year risk of a patient developing a fragility fracture. A patient who has sustained a fragility fracture (e.g. following a Colles' wrist fracture) should also be assessed for osteoporosis. tool such as FRAX or QFracture to assess a patients 10 year risk of developing a fracture. This is analogous to the cardiovascular risk tools such as QRISK; start with BMD if: before starting treatments that may have a rapid adverse effect on bone density (for example, sex hormone deprivation for treatment for breast or prostate cancer). in people aged under 40 years who have a major risk factor, such as history of multiple fragility fracture, major osteoporotic fracture, or current or recent use of high-dose oral or high-dose systemic glucocorticoids To assess the actual bone mineral density a dual-energy X-ray absorptiometry (DEXA) scan is used. The DEXA scan looks at the hip and lumbar spine. If either have a T score of < -2.5 then treatment is recommended. (T score is a normal distribution, so a score of < -2.5 means more than 2.5 standard deviations below mean; this also means more than -1 is considered normal density; wrist can be used if eg hip replacements make it harder to assess) The first-line treatment for osteoporosis is an oral bisphosphonate such as alendronate; important to note that we should manage patients in an anticipatory, i.e. if it likely that the patient will have to take steroids for at least 3 months then we should start bone protection straight away, vitamin D and calcium supplementation should be offered to all women unless the clinician is confident they have adequate calcium intake and are vitamin D replete strontium ranelate and raloxifene are recommended if patients cannot tolerate bisphosphonates Always take bisphosphonates on an empty stomach with a full glass of water. Stand or sit upright for 30 minutes after taking them. You'll also need to wait between 30 minutes and 2 hours before eating food or drinking any other fluids

Answer 115

if >75yo and NOF then no need for DEXA, if limb or vertebral fracture, or if <75 then DEXA scan for T score (v high score may need referral to secondary care osteoporosis service) check U&Es, bone profile, and vitamin D plus consider secondary causes inc multiple myeloma, mets, PTH disease etc replace Ca and vit D as required calculate Cr clearance: if >30 (and eGFR >35) then alendronic acid once a week or zoledronic acid annually, if eGFR 30-35 then risedronate weekly, if Cr cl <30 then will need osteoporosis specialist guidance after 5 years rpt DEXA scan and FRAX calculation; if mx has been effective and not high risk (pred 7.5mg+ OD, >75, prev fracture) then drug holiday, if high risk keep going with mx, if ineffective change bisphos or refer one specialist medication is denosumab, a RANKL inhibitor, that is given every 6mo -> so TTOs need to say when it was given if given in hospital also note post fracture hold off starting denosumab or bisphos for first 1-2 weeks to allow for greater strength of callus formation, but studies are mixed and certainly long term fracture healing isn't affected by early bisphos etc, certainly not in first 1-2 weeks after

Answer 116

check vit D levels: if suspect osteomalacia, if suspect osteoporosis, if need to know vit D levels prior to treatment starting (antiresorptive agents like denosumab, zoledronate; prior to treating paget's with bisphosphonate) If rapid correction of vitamin D deficiency is needed, for example in people with symptoms or about to start treatment with a potent antiresorptive agent then load with 300,000 units of vit D over 6 weeks (various ways to do this, but eg 50,000 units once a week), followed by maintenance of 800-2000 units daily (max 4000 in specific circumstances); this is to minimise risk of hypocalcemia, as antiosteoclast action means less bone resorption -> less ability to mobilise Ca reserves No need to load if less urgent, or starting oral bisphos (alendronic acid, risedronate), instead just start maintenance dose specialist advice if severe renal or kidney disease (may need activated form of vit D giving, as standard supplements have D2 and cholecalciferol (D3), which liver turns into 25OHD and kidneys into calcitriol (1,25(OH)2D)), if malabsorption (may need intense supplements with specialist supervision), renal stones (risk of hypercalciuria -> stones) or diseases that inc hypercalc risk (granulomatous diseases like TB, sarcoidosis, prim hyperpara, bone mets) vit D toxicity: causes hypercalcemia, potentially severe; needs fluids and potentially bisphosphonates and calcitonin, dialysis may be needed if renal function impaired

Answer 117

Test if: Symptoms and signs of rickets:  progressive bowing of legs (bowing of legs can be a normal finding in toddlers)  progressive knock knees  wrist swelling  rachitic rosary (swelling of the costochondral junctions)  craniotabes (skull softening with frontal bossing and delayed fontanelle closure)  delayed tooth eruption and enamel hypoplasia. 2. Other symptoms or conditions associated with vitamin D deficiency:  long-standing (> three months), unexplained bone pain  muscular (proximal) weakness (e.g. difficulty climbing stairs, waddling gait, difficulty rising from a chair or delayed walking, gower sign)  tetany due to low plasma calcium  seizures due to low plasma calcium (usually in infancy)  infantile cardiomyopathy. red, dry and itchy rashes 3. Abnormal investigations:  low plasma calcium or phosphate, high alkaline phosphatase (greater than the local age-appropriate reference range)  radiographs – showing osteopenia, rickets or pathological fractures revealed by radiographs. 4. Chronic disease that may increase risk of vitamin D deficiency:  chronic renal disease, chronic liver disease  malabsorption syndromes (e.g. coeliac disease, Crohn’s disease, cystic fibrosis).  Children with limited mobility (e.g. wheelchair bound) who sustain repeated fractures with/without radiological evidence of osteopenia 5. Treatment with bone-targeted drugs that require vitamin D sufficiency such as bisphosphonates (used in conditions affecting bones such as osteoporosis due to steroids, immobility or inflammatory disorders). 6. Certain group of children and young people are at ‘high risk of vitamin D deficiency’ so consider a lower threshold for testing in the presence of symptoms in this group:  Diets insufficient in calcium (e.g. vegan or low dairy intake) or with generally poor diets  Limited sun exposure (e.g. veiled and photosensitive patients and patients who are advised to apply high factor sun block due to photosensitive medications or malignancy risk e.g. cancer survivors)  Those who spend very little time outdoors (e.g. those with limited mobility)  Those with dark skin, for example people of African, African-Caribbean or South Asian origin, as they may not get enough vitamin D from sunlight.  Those who take anticonvulsants that induce liver enzymes such as phenytoin, carbamazepine, primidone or phenobarbitone  Family members with proven vitamin D deficiency Bloods: Calcium, Phosphate, Magnesium Alkaline Phosphatase – can be normal or high 25 Hydroxy Vitamin D level Parathyroid Hormone Consider FBC/ferritin as oft associated with IDA In the first stage, 25OHD and Ca levels decrease but PO4 and PTH remain unchanged. In the second stage, 25OHD decrease further but PTH acts to raise Ca levels. PO4 levels decrease. In the most severe third stage, 25OHD levels continue to decrease but even the raised levels of PTH are not able to keep Ca levels normal so they begin to decrease along with PO4 25(OH)D > 50 nmol/L – treatment not recommended  25(OH)D < 25 nmol/L – treatment recommended  25 (OH) D 25-50 nmol/L – treatment recommended if child at ‘high risk of vitamin D deficiency’ (the bottom 6 of criteria to test) 0 - 6 months age 3000 units once daily 6 months to 12 years 6000 units once daily 12-18 years 10000 units once daily for 6 weeks if <6mo, 8 weeks if older Then repeat bloods 7 times the daily dose given as once weekly dose for the same duration of length can be advised if compliance is an issue, and there is an option to give entire thing in one dose in hospital but this should be avoided if possible If 25(OH)D > 50 nmol/L – provide lifestyle/dietary advice If 25(OH)D < 50 nmol/L – consider compliance, chronic disease, malabsorption, drug interaction – a longer course or higher dose may be required If sx not improved but levels corrected consider other explanation for sx A daily intake of 500 mg elemental oral calcium (either dietary or supplemental) with vitamin D treatment, irrespective of age or weight is recommended during treatment of Vitamin D deficiency Advice vitamin D supplements in winter, dietary calcium and vitamin D intake, sunlight advice – provide information leaflet which covers this information Advice all year round Vitamin D supplements if at ‘high risk' – as above

Answer 118

For bone maturation, the organic component of the bone matrix, the osteoid, must be mineralized by calcium salts. In rickets, this process is hampered and results in amassing of osteoid beneath the growth plate leading to softness in the bone over a gradual period of time can be broadly classified into calcipenic and phosphopenic calcipenic: Vit D deficiency Genetic vitamin D dependent rickets (VDDR1 due to defect in hydroxylation, VDDR2 due to end organ resistance) Ca resistance CKD phosphopenic: Phosphate deficiency/malabsorption (inc due to antacids, PPIs) Fanconi syndrome Genetic hypophosphatemic rickets (X-linked, AR, AD, and hypophos rickets with hypercalciuria) In calcipenic rickets will see: Skeletal symptoms include swollen wrists and ankles, delayed tooth eruption, leg deformity, rachitic rosary, frontal bossing, craniotabes, delayed growth, and bone pain as well as extraskeletal findings include muscle weakness and hypocalcemic seizures VDDR1 presents similarly but with normal vit D intake; labs show show low calcium, low phosphate, elevated PTH, and high alkaline phosphatase, but in contrast to nutritional rickets they will have normal or high 25-hydroxyvitamin levels and low calcitriol levels; hese children will not respond to high doses of cholecalciferol but respond to physiologic doses of calcitriol or 1α-hydroxyvitamin D; radiological healing occurs within 6 to 8 weeks of therapy; should be monitored for potential side effects of hypercalcemia, hypercalciuria, and nephrocalcinosis secondary to calcitriol therapy including blood work (serum creatinine and calcium, phosphate), urine examination for urine calcium and creatinine ratio, and kidney ultrasound examination VDDR2 presents early in life and may have hypocalcemia, rickets, growth failure, seizures, enamel hypoplasia, and dental caries. Alopecia also occurs in two-thirds of cases due to a lack of vitamin D receptor activity within keratinocytes and is a marker of disease severity; has no proven treatment but try to saturate the normal receptors through mega-doses of calcitriol and calcium, may need Ca infusion CKD - patients with chronic kidney disease cannot convert the calcidiol into the active form calcitriol, vitamin D supplementation alone is therefore ineffective for renal rickets. Instead, a low-phosphate diet, dietary phosphate binders, and oral administration of 1 alfacalcidol or calcitriol is advised XLHPR - craniotabes and rachitic rosary are not common. One of the initial clinical findings is frontal bossing, which may appear as early as 6 months of age; will have other sx similar to above, and dental problems often presentation; AD and AR forms present similarly and with similar lab tests (hypophosphatemia and renal phosphate loss without deficiency of vitamin D or calcium); those with HHRWH have above but also predisposed to nephrocalcinosis; generally all managed with phosphate supplementation, and may benefit from calcitriol; Serum calcium, phosphorus, creatinine, and spot urinary calcium/creatinine should be monitored every 3–4 months. PTH levels should be checked annually. Nephrocalcinosis and tertiary hyperparathyroidism are the potentially serious complications of therapy. Renal ultrasound should be done at the baseline and yearly thereafter.

Answer 119

Ehlers-Danlos syndrome features remarkably stretchy skin that feels smooth and velvety to touch. They also have severe joint hypermobility, joint pain and abnormal wound healing. They often develop lumps over pressure points, such as the elbows. They are prone to hernias, prolapses, mitral regurgitation and aortic root dilatation. Inheritance is autosomal dominant. Vascular Ehlers-Danlos syndrome is the most dangerous form of EDS, where the blood vessels are particularly fragile as a result of defective collagen. Patients have characterise thin, translucent skin that you can almost see through. The skin, internal organs and arteries are fragile and prone to rupturing

Answer 120

There are 13 types of EDS, most of which are rare. Hypermobile EDS (hEDS) is the most common type. Other types of EDS include classical EDS, vascular EDS and kyphoscoliotic EDS. hypermobile: joint hypermobility loose, unstable joints that dislocate easily joint pain and clicking joints extreme tiredness (fatigue) skin that bruises easily digestive problems, such as heartburn and constipation dizziness and an increased heart rate after standing up classical: type 5 collagen mutation characterized by extremely elastic skin that is fragile and bruises easily and hypermobility of the joints. Molluscoid pseudotumors (calcified hematomas that occur over pressure points) and spheroids (cysts that contain fat occurring over forearms and shins) are also often seen. vascular: type 3 collagen identified by skin that is thin, translucent, extremely fragile, and bruises easily. It is also characterized by fragile blood vessels and organs that can easily rupture. Affected people are frequently short, and have thin scalp hair. It also has characteristic facial features, including large eyes, an undersized chin, sunken cheeks, a thin nose and lips, and ears without lobes assess hypermobility with beighton score positive Beighton score is any score greater than or equal to 5/9 points in adults, 6/9 points in children (before puberty), and 4/9 points in adults over age 50 diagnosis is by collagen typing of a skin biopsy, except for hEDS where it doesn't show anything; genetic testing also won't show anything for hEDS echo should be considered looking for aortic root dilatation and mitral valve prolapse mx: nonoperative physical therapy, OT, orthotics, supportive measures for pain inc CBT arthrodesis and posterior spinal fusion may be needed

Answer 121

Collagen is main component of EXM an makes up 25-35% of all protein in the body, over 28 types but 5 main ones as follows: Collagen I: skin, tendon, vascular ligature, organs, bone (main component of the organic part of bone) * Collagen II: cartilage (main component of cartilage) * Collagen III: reticulate (main component of reticular fibers), commonly found alongside type I. * Collagen IV: forms basal lamina, the epithelium-secreted layer of the basement membrane. * Collagen V: cell surfaces, hair and placenta type 1 - osteogenesis imperfecta type 2 -chondrodysplasia type 3 - vascular EDS type 4 - alport syndrome type 5 - classical EDS

Answer 122

trauma commonest, inc NAI septic arth (in neonates may be GBS or g- baci), lyme disease, gonococcal reactive arth (sterile, follows illness like mumps, mono, mycoplasma, gastroent), may form part of reiter syndrome HSP, JCA, SLE, rhem fever, sickle cell, haemarthrosis, psoriatic (most commonly large joint monoarth), IBD, malignancy (osteosarcome can present as swollen/painful knee) FBC - raised wcc, diff count may be helpful here, plats oft raised during acute phase; CRP and ESR, blood culture, viral/lyme serology, throat swab (streptococ), C3/4, joint asp (uss first may be helpful), joint x-ray

Answer 123

v commonly comes up in exams, is somewhat diagnosis of exclusion and the arth needs to be present for at least 6 weeks; as it progresses over 6mo will fall into one of three categories: sys onset, pauciarticular, polyarticular (<5 former, 5+ latter) must consider and eliminate acute leukaemia, neuroblastoma, reactive, rheumatic fever, and other causes of arth from other flashcard 60-70% of children outgrow disease but prog depends on the particular type; often relapses and remits mdt mx - initially NSAIDs, and consider DMARDs like sulfasalazine, hydroxychloroquine, gold steroids can control systemic sx, intraart ones for severe arthritis but steroids dont halt progression (though can abort flares sith iv pred, and eye drops for uveitis); if all tried ans hasnt helped methotrexate, maybe ciclosporin instead can be used and helps w joints but less w sys sx physio, hydrotherapy, splints, occipational therapy, surgery may be needed to release soft tissue orreplace joints; in pauciart needs slit lamp every 6mo in ANA+ and yearly in ANA- to spot uveitis

Answer 124

sys is 10%, pauci 60%, poly 30% sys any time in childhood but commonest <5yo, type 1 pauci 1-5yo and type2 >8; t1 poly mean 6yo and t2 teenagers sys doesnt present with arth but later get polyarth; intermitten high fevers, blotchy pink non-pruritis rash, lymphad, organomeg, serositis, growth retardation t1 pauci lower limb esp knee/ankle, asym and 1/3 have iridocyclitis; t2 asym lower limb inc hips, sacroilium, and malaise, fatigue, acute uveitis t1 poly any joint but esp hands/feet, t2 similar but more aggressivel both types may have fever, malaise but less severe then sys JCA, growth retardation more common in severe disease sys has anaemia, inc'd plats, neutrophilia (usually), raised esr, rh and ANA neg, chronic erosive arth in 25% and rest resolve t1 pauci may have normal ix, 80% of those w uveitis are ANA+ rh-, oft resolves spont but mx uveitis early; t2 normal ix except raised acute phase reactants, sometimes ESR; rh-, esp if HLA-B27 haplotype may go on to dev seroneg arthro like ank spond t1 poly rh-, ANA may be +, generally good prognosis; t2ANA+ in 50%, rh+' poorer prognosis w more disability and more likely to continue into adulthood

Answer 125

Think of Still’s disease (systemic JIA) when a patient presents with a salmon-pink rash, fevers and joint pain. In children that have fevers for more than 5 days, the key non-infective differentials to remember are Kawasaki disease, Still’s disease, rheumatic fever and leukaemia Polyarticular JIA involves idiopathic inflammatory arthritis in 5 joints or more. The inflammatory arthritis tends to be symmetrical and can affect the small joints of the hands and feet, as well as the large joints such as the hips and knees. There are minimal systemic symptoms, but there can be mild fever, anaemia and reduced growth pauciarticular JIA. It involves 4 joints or less. Usually it only affects a single joint, which is described as a monoarthritis. It tends to affect the larger joints, often the knee or ankle. It occurs more frequently in girls under the age of 6 years. A classic associated feature with oligoarticular JIA is anterior uveitis

Answer 126

essentially a diagnosis of exclusion and therefore a high index of suspicion for other differential diagnoses is necessary. The chief differentials for a swollen joint will be infection (septic arthritis or osteomyelitis), malignancy (particularly leukaemia or bone tumours) and trauma Note: More than one swollen joint will always require rheumatology referral In an otherwise well chid with swollen joint(s) the most likely diagnosis is JIA features to inc suspicion: joint swelling, stiffness worse in the morning, duration >6 weeks (but dont wait 6 weeks to start ix/rx), family history of B-27 linked conditions do pGALS to check all joints fever or acute severe pain points towards septic arth/OM, nocturnal/severe pain or B features suggest leukaemia as would eg pallor, recent viral illness in well child can be reactive arth Initial laboratory tests should include CBC, ESR, CRP, ANA, RF, anti-cyclic citrullinated peptide antibodies (anti-CCP), and HLA-B27. Ferritin, fibrinogen, AST, triglyceride are recommended when there is a concern of macrophage activation syndrome (MAS) oligoarticular JIA (used to be called pauciarticular) affects ≤4 joints in the first 6 months of illness peak age: 1-6 years mainly affects medium and large joints polyarticular JIA (pJIA) affects ≥5 joints peak age: 1-4 years; 7-10 years mainly affects small and medium joints above 2 if mild need rheum OPA and daily ibuprofen per BNFc until appointment At diagnosis all patients should receive either intra-articular steroids to all affected joints, or systemic (preferably IV) corticosteroids. Apart from the two specific circumstances of Macrophage Activation Syndrome or Sacroiliitis all patients should start treatment with Methotrexate (MTX) as the first line DMARD and be re-assessed every 3/12. ‘Flares’ of arthritis should be treated with steroids systemic JIA (stills disease) 1 or more joints  Daily fever for at least 3 days over a period of 2 weeks  Including at least one of the following: o Evanescent erythematous rash o Generalised lymphadenopathy o Hepatosplenomegaly and or splenomegaly o Serositis High risk of macrophage activation syndrome Admit, speak urgently to rheum May need tocilizumab +/- anakinra

Answer 127

Interleukin production is a self-limited process. The messenger RNA encoding most interleukins is unstable and causes a transient synthesis. Interleukins have redundant functions IL1: macros, fibroblasts, B cells, astrocytes, endothelium secretes; causes lymphocyte activation, macrophage stimulation, increased leukocyte/endothelial adhesion, fever due to hypothalamus stimulation, and release of acute phase proteins; corticosteroids increase production of interleukin 1 receptor antagonist to reduce interleukin 1 effect IL2: produced by T cells, primary effects are Th-cell proliferation and differentiation, increased cytokine synthesis, potentiating Fas-mediated apoptosis, and promoting regulatory T cell development. also proliferation of NK cells and B cells; primary IL-2 function in vivo is the suppression of T responses IL3: T cells and stem cells make IL-3. It functions as a multilineage colony-stimulating factor IL4: CD4+T cells (Th2) synthesize IL-4, and it acts on both B and T cells. It is a B-cell growth factor and causes IgE and IgG1 isotype selection. It causes Th2 differentiation and proliferation, and it inhibits IFN gamma-mediated activation on macrophages IL5: CD4+T cells (Th2) produce IL-5, and its principal targets are B cells. It causes B-cell growth factor and differentiation and IgA selection IL6: T and B lymphocytes, fibroblasts and macrophages make IL-6. B lymphocytes and hepatocytes are its principal targets. IL-6 primary effects include B-cell differentiation and stimulation of acute phase proteins IL7: Bone marrow stromal cells produce, causes B-cell and T-cell proliferation IL8: Monocytes and fibroblasts make IL-8; targets are neutrophils, basophils, mast cells, macrophages; causes neutrophil chemotaxis, angiogenesis, superoxide release, and granule release overall there are 33 interleukins

Answer 128

group of autoinflammatory disorders presenting (generally in children) as recurrent episodes of fever accompanied by inflammation of the eyes, joints, skin, or serosal surfaces; repetitive episodes of fever with no symptoms in between the episodes. These episodes generally last for a few days to weeks up to several months can be categorized into Inflammasomopathies or IL-1 β-activation syndromes including familial Mediterranean fever (FMF), cryopyrin-associated periodic syndromes (CAPS), and mevalonate kinase deficiency (MKD), and protein folding disorders such as tumor necrosis factor (TNF) and receptor-associated periodic fever syndrome (TRAPS) FMF episodes from 12 hours to three days. Abdominal pain may occur suddenly before the fever, mimics appendicitis, and could be accompanied by diarrhea. Asymmetrical monoarthritis, which compromises the ankle, knee, or wrist joint, usually resolves in 5-14 days. An erysipelas-like rash may be present over the affected joint-in about 25% affected, leading to the misdiagnosis of juvenile idiopathic arthritis. The rash is unilateral and disappears within two to three days. Pleuritic chest pain (may be associated with difficulty in breathing), peritoneal abdominal pain, and pericarditis (in <1%) can also be present. Splenomegaly may be present in 30-50% of the affected. Amyloidosis is the most common complication and cause of mortality of untreated familial Mediterranean fever and can progress to end-stage renal disease PFAPA is the most common cause of periodic fever; findings like faltering growth would suggest a different diagnosis; specific genetic test (PFAPA doesnt have one) careful ix and mx from pediatric immunologist/infectious diseases specialist or rheumatologist note that IgD is associated with periodic fever syndromes, and that one form is even called hyper IgD syndrome

Answer 129

disease presents at a median age of four years with perennial fever, exudative redness with negative throat culture, cervical lymphadenopathy, and oral aphthae. It less frequently presents with abdominal pain, arthralgia, myalgia, and headache Regardless of antibiotics or antipyretics, each step lasts four to six days and is repeated every three to five weeks leukocytosis and high C-reactive protein (CRP) levels predominate during a flare, with normalization between episodes; rx with steroids +/- anakinra, and if multiple attacks colchicine prophylaxis Familial Mediterranean fever is one of the most prevalent inheritable inflammatory diseases. The main sequence in patients with familial Mediterranean fever is the MEFV gene, a secret writing pyrin macromolecule. Pyrin is a physiological protein within the nucleotide-binding oligomerization domain, leucine-rich repeat, and pyrin domain-containing three (NLRP3) inflammasome complex; mutation of this protein causes excess inflammation via caspase-1 activation and IL-1b production main symptoms of FMF are fever, abdominal pain, and arthritis. The temperature rises to about 40 degrees celsius, associated with chills, and lasts from 12 hours to three days. Abdominal pain may occur suddenly before the fever, mimics appendicitis, and could be accompanied by diarrhea. Asymmetrical monoarthritis, which compromises the ankle, knee, or wrist joint, usually resolves in 5-14 days. An erysipelas-like rash may be present over the affected joint-in about 25% affected, leading to the misdiagnosis of juvenile idiopathic arthritis. The rash is unilateral and disappears within two to three days. Pleuritic chest pain (may be associated with difficulty in breathing), peritoneal abdominal pain, and pericarditis (in <1%) can also be present. Splenomegaly may be present in 30-50% of the affected. Amyloidosis is the most common complication and cause of mortality; should be on prophylactic colchicine to protect against this CAPS pathophysiology is related to NLRP3 mutation, coding cryopyrin. NLRP3 may be a key supermolecule of the NLRP3 inflammasome; common clinical options are fever, urticarial rash, system symptoms, elevated acute part reactants, and conjunctivitis; anakinra is usual mx, note that CAPS is v rare

Answer 130

most common form of amyloidosis is reactive AA amyloidosis due to chronic inflammatory diseases amyloidosis secondary to chronic infectious diseases was common in the early 20th century. However, due to the advances in the management of chronic infections such as tuberculosis, this is no longer the case. Juvenile idiopathic arthritis (JIA) was also a cause of secondary amyloidosis, however, with better recognition and treatment, as well as the introduction of biologics, secondary amyloidosis rarely occurs in JIA in this decade. The main cause of childhood amyloidosis is familial Mediterranean fever (FMF) and the other autoinflammatory diseases precursor of amyloid A protein is serum amyloid A (SAA), an acute phase protein produced in response to inflammation; persistent augmentation of an inflammatory pathway through the innate immune system might be crucial in the deposition of the amyloid protein amyloid fibrils are most commonly deposited in the kidneys, but may also attack the heart, peripheral nerves, thyroid, gastrointestinal system, and bone marrow - in secondary amyloidosis typically only the kidney is involved; Asymptomatic proteinuria is the most common initial presentation of kidney disease. Thus, urinalysis should be routinely examined in patients with a disease associated with the risk of secondary amyloidosis. The most common and earliest sign of amyloidosis in FMF is proteinuria, gradually progressing to nephrotic syndrome and/or renal dysfunction Systolic or diastolic dysfunction, and arrhythmia are the main presenting features of heart involvement Gastrointestinal presentations are frequently nonspecific and may include dyspepsia, abdominal pain, nausea, diarrhea, gastrointestinal bleeding, malabsorption, constipation, and obstruction; may get hepatomegaly or splenomegaly; leeding diathesis due to factor X deficiency, liver disease, or infiltration of blood vessel; thrombosis due to nephrotic syndrome or underlying disease may be seen Mixed sensory and motor peripheral neuropathy and autonomic neuropathy may occur in amyloidosis albeit rare in the secondary forms. Neuropathy is a prominent feature in some of the hereditary amyloidoses diagnosis of amyloidosis is based on the demonstration of amyloid fibrils in the biopsy of the involved tissue; Congo red staining is necessary for diagnosis. Amyloid fibrils appear faintly red on Congo red staining and show the characteristic apple-green birefringence under polarized light

Answer 131

dactylitis esp in black child think of sickle cell first, but also consider JCA, other CTDs

Answer 132

sickle cell, acute leukaemia, neruoblastoma (with bone marrow invasion), JCA, TB arth, SLE, chronic arthropathy treated with NSAIDs (which then cause GI bleed), methotrexate use

Answer 133

all ages of childhood, commoner in girls marked vasculitis affecting skin, muscles, and viscera; may see gradually onset symmetrical proximal myopathy (varies from mild to bed-bound and can affect muscles of speech, swallowing, and ventilation) skin features: non-pitting oedema, periorbital oedema, vilaceous heliotrope rash around eyes and cheeks which may extend to upper trunk, gottron patches on knuckles/elbows/knees/ankles (extensor surfaces), telangiectasias in nail bed, poss calcinosis in skin/joints/subcut tissues visceral sx mainly from GI tract and get abdo pain (even acute abdo), bleeding or perf dx from raised esr, cpk, characteristic EMG (polyphasic short motor potentials w spont fibrillations), and muscle biopsy showing inflam changes MRI can be used to define degree of inflam and monitor treatment response (which incs physio and oral or iv steroids, resistant cases poss needing methotrexate, gammaglobulin, and maybe other immunosupp)

Answer 134

Chronic infantile neurological cutaneous and articular aka neonatal onset multisystemic inflammatory disease triad of (1) neonatal onset of cutaneous symptoms, (2) chronic meningitis, and (3) joint manifestations with recurrent fever and inflammation Persistent and migratory skin rash associated with skin perivascular polymorphonuclear infiltrates is present in patients, starting at birth. A progressive neurological impairment results from chronic meningitis caused by polymorphonuclear cell (PMNC) infiltration. A progressive visual defect and perceptive deafness frequently occurs with increasing age. Joint symptoms manifest as recurrent joint flares with or without severe radiologically evident modifications involving the growth cartilage or bone epiphysis; common morphology of frontal bossing, protruding eyes, short stature, short distal limbs majority of above result from the deleterious effect of PMNC infiltration

Answer 135

female >1yo fusiform aneurysms aneurysm diamter <4mm low plat count

Answer 136

bucket handle metaphyseal fractures of long bones, spiral fractures, mid-shaft fractures, fractures of various ages in x-ray or in ribs/skull/pelvis many sig bruises <1yo, or characteristic pattern like fingertip, hand slap, bite mark, or bruises in unusual places like perineum, ears also cig burns, scalds, reintal haemorrhages, SDH, torn frenulum, alert bu anxious (frozen watchfulness), failure to thrive, underachievement at school, dev delay; vaginal bleeding/discharge, rec abdo pain, STIs or pregnancy; reflex anal dilatation (also in chronic constipation) other features in history: delay in seeking med attention, inconsistencies in history, either too much or too little parental concern, abnormal parental affect, anger from parent if admission wanted you should exclude bleeding and clotting problems and eg osteogenesis imperfecta, including doing a skeletal survey/bone scan as well as clotting studies detailed history and exam - document everything and treat injuries - admit child if suspicion high - look up at risk register and contact social services

Answer 137

SHH activates TF to transcribe HoxD, giving diversity across limb bud through sequential HoxD expression to create sub-regions; ectopic SHH can give eg ectopic HoxD13; HoxD family and TFs determine structures formed with mutation in HoxD13 giving syndactyly corrected by a zigzag flap surgery; BMP signalling proteins like GDF5, BMP4 regulate formation of cartilage condensations in limb buds by increasing adhesiveness between cells so more recruited, over expression can result in increased size/shape of skeletal elements in chicken embryos; chondrodysplasia grebe-type CGT or Hunter-Thompson type CHTT due to mutation in GDF5 or partner proteins so skeletal elements shorten, more severe distally with joints possibly lost; BMP can cause programmed cell death to shape digits, Noggin acts to block this; HoxD13 involved in expression of BMP proteins, explaining syndactyly FGF placed between upper/lower limb buds can give another one; Hox genes define position of limb bud with knockout HoxB5 mice growing forelimbs more anteriorly; appropriate Hox genes lead to Tbx/FGF production with abnormal Tbx5 expression causing Holt-Oram syndrome with associated limb deformities such as absent forearm bones, polydactyly as well as heart problems as Tbx5 has a role in septation; both limbs form in week 4 but lower limb 1-2 days behind upper limb; distal flattens into hand/foot plate in week 6 with limbs rotating 90 degrees in week 7 (upper laterally, lower medially, defining position of compartments, major entrances to limbs etc); cartilage model also forms in week 6; chondrogenesis arrested in cartilage models in some places with cell death forming joint cavity

Answer 138

AD inheritance short stature, frontal bossing, mid face hypoplasia, lumbar lordosis, genu varum; incidence incs with paternal age at birth or in first year with large head, normal body, short limbs regular follow up as 10% dev complications inc obs sleep apnoea and rec middle ear infections gross motor skills dev later - 50% sit alone by 9mo, 50% walk by 18mo leg lengthening surgery sometimes an option, and may need a brace for the lordosis in first year of life final height 80-150cm, life expectancy normal; obesity and OA common problems, may dev spinal stenosis later in life

Answer 139

B5 - behcets B27 - reactive arth, ank spond, psoriatic arth, IBD arth DQ2/8 - coeliac disease DR2 - MS, SLE, goodpastures DR3 - T1DM, graves, hashimotos, addisons, SLE DR4 - RA, T1DM, addisons DR5 - hashimotos

Answer 140

skeletal survey is required if there are concerns that a child has been subjected to physical abuse In the context of suspected NAI, a skeletal survey should be carried out in children under 24 months with a fracture and any of the following: Confessed abuse/injury during domestic violence Delayed attendance (>24 hours in a child with obvious distress) History of impact from a toy or other object causing a fracture Other injuries on assessment that are not related to the fracture No trauma history to explain fracture (not an exception is made for ambulatory children >12 months old with a distal buckle fracture of the radius/ulna, or a distal spiral or buckle fracture of the tibia/fibula) A skeletal survey should be carried out in children 12-23 months with any of the following fracture types: Rib fracture Classic metaphyseal lesion Complex or ping-pong skull fracture Humeral fracture with epiphyseal separation attributed to a short (≤3 feet) fall A skeletal survey should be carried out in all children 0-11 months old with any fracture except the following (with no additional concerns): Distal radius/ulna buckle fracture or toddler fracture of tibia/fibula in a cruising child≥9 months old with a history of fall. Linear, unilateral skull fracture in a child >6 months with a history of a significant fall (height >3 feet or fall with caregiver landing on the child). Clavicle fracture likely due to birth trauma (acute fracture in baby <22 days old or healing fracture in baby <30 days old. undertaken as planned event when requested by a consultant - need to explain why doing it and obtain written consent, with court order if refused All children undergoing skeletal survey for suspected physical abuse should have the following blood tests: Calcium and phosphate, alkaline phosphatase Serum 25-hydroxyvitamin D Parathyroid hormone Minimum Projections Required for a Skeletal Survey are as follows: Head, chest, spine, and pelvis: - AP and lateral skull. - AP chest (to include shoulders) - Oblique views of ribs both sides (to include all ribs, 1-12) - AP abdomen and pelvis - Lateral whole spine (on one view is possible. For larger children separate views will be required) Upper limbs: - AP of the whole arm (centred at the elbow) - Coned lateral elbow - Coned lateral wrist - PA hand and wrist In larger children where a single whole arm view is not possible: - AP humerus (including the shoulder and elbow) - AP forearm (including the elbow and wrist) - Coned lateral elbow - Coned lateral wrist - DP hand and wrist Lower limbs: - Whole AP lower limb, hip to ankle - Coned lateral knee and ankle - Coned AP ankle (mortise view) - DP foot For larger children: - AP femur - AP tibia and fibula - AP knee - AP ankle - Coned lateral knee - Coned lateral ankle - DP foot Brain CT should be conducted for all children under the age of 1 year in whom NAI is suspected; Children >1yr with external evidence of head trauma or abnormal neurology also need CT Follow-up imaging 11- 14 days after the initial skeletal survey and no later than 28 days after the initial skeletal survey should be performed

Answer 141

aka myalgic encephalomyelitis may or may not be triggered by a virus. There are many potential aetiologies for CFS/ME, including neurological, endocrine, immunological, genetic, psychiatric and infectious causes which have been investigated, but the diverse nature of the symptoms cannot yet be fully explained Consider the possibility of CFS/ME if a C&YP has:  Profound fatigue with all of the following features: o new or had a specific onset (that is, it is not lifelong) o persistent and/or recurrent o unexplained by other conditions o has resulted in a substantial reduction in activity level o characterised by post-exertional malaise and/or fatigue (typically delayed, for example by at least 24 hours, with slow recovery over several days) And  one or more of the following symptoms: o difficulty with sleeping, such as insomnia, hypersomnia, unrefreshing sleep, a disturbed sleep–wake cycle o muscle and/or joint pain that is multi-site and without evidence of inflammation o headaches o painful lymph nodes without pathological enlargement o sore throat o cognitive dysfunction, such as difficulty thinking, inability to concentrate, impairment of short-term memory, and difficulties with word-finding, planning/organising thoughts and information processing o physical or mental exertion makes symptoms worse o general malaise or 'flu-like' symptoms o dizziness and/or nausea o palpitations in the absence of identified cardiac pathology o abdominal pain, loose stools o reported less often include feeling too hot or cold, cough, eye pain/increased sensitivity to light (photophobia), vision or hearing disturbances (hyperacusis), weight loss or gain frequently misdiagnosed as a behavioral or emotional problem, in particular school phobia. Unlike children with school phobia, children with ME/CFS are typically ill on weekends as well as during the school week. following tests should usually be done:  urinalysis for protein, blood and glucose  FBC, U&Es, LFTs (and thyroid peroxidise antibodies), ESR, CRP  random blood glucose  coeliac screen (tissue transglutaminase antibodies and total serum IgA)  serum calcium  creatine kinase  Vitamin D levels  serum ferritin levels  autoimmune screen  GF screen, EBV serology Extensive serology shouldn't be done but based on clinical picture could do:  chronic bacterial infections, such as borreliosis  chronic viral infections, such as HIV or hepatitis B or C  latent infections, such as toxoplasmosis, cytomegalovirus diagnosis should be made after other possible diagnoses have been excluded and the symptoms have persisted for 3 months keep an activity diary and plan to build up slowly emphasise the importance of eating regularly, and including slow-release starchy foods in meals and snacks nausea should be managed conventionally, including giving advice on eating little and often, snacking on dry starchy foods and sipping fluids. The use of anti-emetic drugs (e.g. Buccastem SL) should be considered only if the nausea is severe some benefit from exclusion diets, if want to try they should do so with a dietician sleep hygiene measures, if appropriate melatonin can be used under specialist guidance but advise that excessive sleep does not generally improve physical or mental functioning, and excessive periods of daytime sleep or frequent napping may further disrupt the sleep–wake cycle rest periods of up to 30mins at a time can be implemented and can use relaxation techniques refer to PT to support graded activity programmes For patients with prolonged school absence it would be useful to formulate a graded re-entry timetable in conjunction with the C&YP, parents and school may need psych support and refer to specialist childrens ME clinic (bristol and UCLH) within 6 months of presentation for people with mild CFS/ME  within 3–4 months of presentation for people with moderate CFS/ME symptoms  immediately for people with severe CFS/ME symptoms; keep reviewing regularly in secondary clinic too prognosis in children and young people is generally positive and most will make a full recovery but this may be over several months

Answer 142

Amplified musculoskeletal pain syndrome (AMPS) is a term that encompasses the spectrum of manifestations of chronic pediatric musculoskeletal pain. The common thread underlying these different subtypes is central and/or peripheral sensory pain amplification, hence the name amplified musculoskeletal pain causes may include diffuse idiopathic pain syndrome aka juvenile fibromyalgia; complex regional pain syndrome (aka reflex sympathetic dystrophy or localised idiopathic pain) CRPS I criteria: 1) The presence of an initiating noxious event, or a cause of immobilization. 2) Continuing pain, allodynia, or hyperalgesia with which the pain is disproportionate to any inciting event. 3) Evidence at some time of edema, changes in skin blood flow, or abnormal sudomotor activity in the region of the pain. 4) This diagnosis is excluded by the existence of conditions that would otherwise account for the degree of pain and dysfunction. Exercise and physical therapy (PT) are the main treatment cornerstones of all paediatric CRPS patients + psychological therapies; NSAIDs, paracetamol, gabapentin, amitriptyline may be used as adjuncts fibromyalgia criteria: widespread pain in at least four of five body regions for at least three months: Left upper region: Including shoulder, arm, or jaw Right upper region: Including shoulder, arm, or jaw Left lower region: Including hip, buttock, or leg Right lower region: Including hip, buttock, or leg Axial region: Including neck, back, chest, or abdomen and may see: Feeling unrefreshed after sleep Cognitive difficulties, such as problems thinking or remembering Fatigue assess for tender points (maps available that show where these are) - the pressure to assess tenderness with digital examination is the pressure needed to see the examiner’s own nail bed blanch, look for at least 5 management of JFMS is centered on the issues of education, behavioral and cognitive change (cognitive-behavioral therapy (CBT)) with a strong emphasis on physical exercise), and a relatively minor role for pharmacological treatment with medications such as muscle relaxants, analgesics and tricyclic agents

Answer 143

Primary prevention aims to prevent disease or injury before it ever occurs. This is done by preventing exposures to hazards that cause disease or injury, altering unhealthy or unsafe behaviours that can lead to disease or injury, and increasing resistance to disease or injury should exposure occur. Examples include: legislation and enforcement to ban or control the use of hazardous products (e.g. asbestos) or to mandate safe and healthy practices (e.g. use of seatbelts and bike helmets) education about healthy and safe habits (e.g. eating well, exercising regularly, not smoking) immunization against infectious diseases Secondary prevention aims to reduce the impact of a disease or injury that has already occurred. This is done by detecting and treating disease or injury as soon as possible to halt or slow its progress, encouraging personal strategies to prevent reinjury or recurrence, and implementing programs to return people to their original health and function to prevent long-term problems. Examples include: regular exams and screening tests to detect disease in its earliest stages (e.g. mammograms to detect breast cancer) daily, low-dose aspirins and/or diet and exercise programs to prevent further heart attacks or strokes suitably modified work so injured or ill workers can return safely to their jobs Tertiary prevention aims to soften the impact of an ongoing illness or injury that has lasting effects. This is done by helping people manage long-term, often-complex health problems and injuries (e.g. chronic diseases, permanent impairments) in order to improve as much as possible their ability to function, their quality of life and their life expectancy. Examples include: cardiac or stroke rehabilitation programs, chronic disease management programs (e.g. for diabetes, arthritis, depression, etc.) support groups that allow members to share strategies for living well vocational rehabilitation programs to retrain workers for new jobs when they have recovered as much as possible

Ortho/rheum Flashcards

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