Paeds 👶🏼 Flashcards
1
Q
What is the classic 4 Px of Henloch-Schonlein Purpura?
A
- Rash (palpable purpura usually on legs and bum)
2.Abdominal pain - Arthralgia (joint pain - mostly knees and ankles here)
- Renal involvement
If rash on trunk, think meningiococcal infection, if rash elsewhere, think HSP
2
Q
What conditions can Henloch-Schonlein Purpura (HSP) predispose you to?
A
Nephrotic syndrome (CKD in 1-5%)
Intussusception (when part of the intestine slides/telescopes into an adjacent part of the intestine and cuts off blood supply to the effected area)
Bowel infarction
GI haemorrhage
3
Q
What are the most common infection causes in babies?
A
Group B streptococcus,any gram-ve bacteria and listeria
4
Q
What abx covers listeria?
A
Amoxicillin
5
Q
Which abx covers gram -ve bacteria (broad spectrum)?
A
Co-Amoxiclav
6
Q
What is the abx used in neutropenic sepsis?
A
Tazocin
7
Q
What is the treatment for IJA (Idiopathic Juvenile Arthritis)?
A
Disease modifying anti-rheumatic drugs (DMARDs) Eg Methotrexate, sulfasalazine and leflunomide.
Can also give NSAIDs if mild or steroids in oligoarthritis IJA
If severe, can give biologic inhibitors Eg etanercept, infliximab and adalimumab.
8
Q
Haemolytic uraemic syndrome Px in kids?
A
Non-blanching rash assoc. with oliguria (very low urine output), and signs of anaemia.
Often recent diarrhoea.
9
Q
What is Henloch-Schonlein Purpura?
A
An IgA mediated small vessel vasculitis affecting the skin, kidneys and GI tract.
Often triggered by an URTI or gastroenteritis.
Mostly kids under 10.
Can be differentiated from other DDs by the purpura being palpable
10
Q
Henloch-Sconlein Purpura (HSP) management?
A
Analgesia, rest + hydration
Monitor urine dip and BP
Steroid use is debatable
11
Q
What is Juvenile Idiopathic Arthritis?
A
JIA is a condition affecting kids and adolescents where autoimmune inflammation occurs in joints.
12
Q
How is Juvenile Idiopathic Arthritis diagnosed?
A
JIA is diagnosed when there is arthritis with no other known cause lasting over 6 weeks in a pt under 16 yrs old.
13
Q
What is the main complication of Juvenile Idiopathic Arthritis (JIA)?
A
Macrophage activation syndrome (MAS), where there is severe activation of the immune system + massive inflammatory response.
Presents as an acutely unwell child with DIC, anaemia, thrombocytopenia, bleeding, and a non-blanching rash.
It is life-threatening.
Low ESR!
14
Q
What is the Px of Still’s disease (systemic JIA)?
A
Salmon-pink rash, fever and joint pain.
in a child with a fever lasting over 5 days, the key non-infective differentials are Kawasaki’s dx, Still’s dx, Rheumatic fever, and Leukaemia
15
Q
Polyarticular IJA Px?
A
Affects 5+ joints.
Symmetrical
Joints of all sizes can be affected
Equivalent of a paediatric Rheumatoid arthritis
Can get reduced growth, mild fever and anaemia
16
Q
Oligoarticular JIA (AKA pauciarticular JIA) Px?
A
Involves 4 joints or less. Can be just 1 affected joint (monoarthritis), usually a larger joint like knee or ankle.
Frequently affects younger females under 6.
Classic feature is anterior uveitis.
ANA +ve.
17
Q
What is Enthesitis-Related Arthritis?
A
The paediatric version of seronegative spondyloarthropathy (conditions like ankylosing spondylitis, psoriatic arthritis, reactive arthritis and IBD related arthritis).
Patients have inflammatory arthritis + enthesitis (inflammation of the point where the tendon of a muscle inserts into the bone)
MRI diagnoses enthesitis
18
Q
What is Kawasaki’s disease?
A
AKA muco-cutaneous lymph node syndrome.
It is a systemic medium vessel vasculitis.
Affects young kids under 5.
No clear cause.
More common asian males.
19
Q
What is the key complication of Kawasaki’s disease?
A
Coronary artery aneurysm
20
Q
Clinical features of Kawasaki’s?
A
Persistent high fever (over 39) for more than 5 days.
Kids unhappy and unwell.
Also get widespread erythematous maculopapular rash and desquamation (skin peeling) on the palms and soles.
May also see strawberry tongue, cracked lips, cervical lymphadenopathy and bilateral conjunctivitis
21
Q
What blood and urinalysis results are indicative of Kawasaki’s?
A
Anaemia, leukocytosis and thrombocytosis, also hypoalbuminaemia and elevated liver enzymes and high ESR.
Urine shows raised WBCs without infection
22
Q
How do we manage Kawasaki’s disease?
A
High dose aspirin to reduce risk of thrombosis.
IV Immunoglobulins to reduce the risk of coronary artery aneurysms.
Kawasaki disease is one of the few scenarios where aspirin is used in children. Aspirin is usually avoided due to the risk of Reye’s syndrome (dx that causes brain and liver damage).
23
Q
What is Spinal Muscular Atrophy (SMA)?
A
SMA is a rare autosomal recessive disorder that causes a progressive loss of motor neurones leading to progressive muscle weakness.
Affects the lower motor neurones of the spinal cord.
24
Q
What is the px of Spinal Muscular Atrophy?
A
LMN signs - fasciculations, reduced muscle bulk, reduced tone and power, and reduced or absent reflexes.
25
What are the categories of Spinal Muscular Atrophy?
SMA is numbered from most to least severe.
Type 1: onset in 1st few months of life, death within 2 yrs
Type 2: onset within first 18 months, never walk, but survive to adulthood (most common)
Type 3: onset AFTER 1st yr of life. Most walk w/o support but then lose the ability. Respiratory muscles are less affected and life expectancy is close to normal.
Type 4: onset in 20s. Most can walk short distances but need wheelchair for mobility. Normal activities = v. fatigued. Respiratory muscles + life expectancy not affected.
26
Spinal Muscular Atrophy management?
No cure.
Physio to maximise strength + retain respiratory function.
NIV may be needed to prevent hypoventilation and respiratory failure, esp during sleep.
Pts with SMA type 1 may need tracheostomy and mechanical ventilation.
Percutaneous endoscopic gastrostomy (PEG) feeding may be required when a weak swallow makes swallowing unsafe
27
What are the most common types of nephrotic syndrome in paeds?
Congenital nephrosis and minimal change disease (0-10 yrs)
Focal Segmental Glomeruloscerosis (FSGS)
28
What is the Px of Congenital Nephrotic Syndrome?
Rare
Majority born prematurely but high birth weight
High alpha-feto protein
Low albumin
Muscular hypotonia
Umbilical hernia
Widened cranial structures
29
What is the Rx of Congenital Nephrotic Syndrome?
Central line inserted for albumin and thyroxin infusions
Supplements + nutrition
Chemical nephrectomy (reduce GFR to give renal failure)
Nephrectomy - usually bilateral
30
What is Erlers-Danlos Syndrome?
Erlers-Danlos Syndrome is an umbrella term for a group of genetic conditions that cause defects in collagen, resulting in hypermobility of the joints and connective tissue abnormalities (of the blood vessels, skin, bones and organs). There are several types.
Diagnosis made with Beighton score.
31
What are the types of Erlers-Danlos Syndrome?
Hypermobile, classical, vascular and kyphoscoliotic
32
What is Hypermobile Erlers-Danlos Syndrome?
The most common and least severe type of Erlers-Danlos Syndrome.
The key feature is hypermobility of joints, and pts have soft and stretchy skin.
33
What is Classical Erlers-Danlos Syndrome?
Main feature is very smooth and stretchy skin that feels velvety.
Also have severe joint hypermobility, joint pain and impaired wound healing.
They develop lumps over pressure points eg elbows.
Prone to hernias, prolapse, mitral regurgitation, and aortic root dilation.
Autosomal dominant inheritance.
34
What is Vascular Erlers-Danlos Syndrome?
The most dangerous form of EDS.
BVs are fragile due to defective collagen.
Pts hae thin, translucent skin.
Skin, organs + arteries are prone to rupturing.
Pts are monitored for vascular abnormalities.
Autosomal dominant inheritance.
35
What is Kyphoscoliotic Erlers-Danlos Syndrome?
Characterised by initial hypotonia (poor tone) in infancy followed by kyphoscoliosis as they grow.
Significant joint hypermobility.
Pts are tall + thin.
Risk of rupture of medium arteries.
Autosomal dominant inheritance.
36
Erlers-Danlos Syndrome Px?
Hypermobility and joint pain are the main features.
Can also have: soft + stretchy skin, easy bruising, headaches, GORD, IBS, abdo pain, urinary incontinence or pelvic organ prolapse.
37
What is the score for assessing hypermobility?
Beighton score.
Sores on both sides, with max score of 9:
Palms flat on floor with straight legs (score 1)
Elbows hyperextend
Knees hyperextend
Thumb can bend to touch the forearm
Little finger hyperextends past 90 degrees
38
When diagnosing Erlers-Danlos Syndrome, which other syndrome is important to exclude?
Marfan's syndrome.
Exclude by examining features such as high arch palate, arachnodactyly and arm span, which are present in Marfan's but NOT Erlers-Danlos.
39
Erlers-Danlos Syndrome management?
No cure, management focuses on maintaining joint health, monitoring for complications and minimising symptoms.
Physio and OT help.
40
What is Postural Orthostatic Tachycardia Syndrome (POTS)?
Postural orthostatic tachycardia syndrome (POTS) can occur with hypermobile Erlers-Danlos syndrome, and is a result of autonomic dysfunction.
Causes inappropriate tachycardia on sitting or standing up, resulting in presyncope, syncope, headaches, disorientation, nausea and tremor.
41
What is acute Rheumatic Fever?
Rheumatic fever is an autoimmune condition triggered by streptococcal bacteria.
It is a multi-system disorder that affects joints, heart, skin and nervous system.
Rare in UK.
42
What is the pathophysiology of rheumatic fever?
Rheumatic fever is caused by Group A beta-haemolytic streptococcal bacteria, esp. streptococcus pyogenes (that causes tonsillitis).
Results in a Type 2 Hypersensitivity reaction.
Onset 2-4 wks after initial infection.
43
Rheumatic Fever Px?
Occurs 2-4wks after a streptococcal infection Eg tonsillitis.
Fever
Rash
SOB
Chorea (involuntary, irregular, unpredictable muscle movements)
Nodules
Joint pain
44
Rheumatic Fever involves the joints, heart, skin and nervous system. How does it effect the joints?
RF causes migratory arthritis affecting large joints, causing hot, swollen and painful joints that improve and worsen at different times, giving the impression that the arthritis is migrating.
45
Rheumatic Fever involves the joints, heart, skin and nervous system. How does it effect the heart?
Carditis (peri-, myo-, and endo-)
Leads to HR change, murmurs, pericardial rub, and heart failure.
46
Rheumatic Fever involves the joints, heart, skin and nervous system. How does it effect the skin?
2 key skin findings in RF:
Subcutaneous nodules - firm, painless nodules develop over extensor surfaces of joints eg elbows
Erythema margitanum rash - pink rings of various sizes over the torso and proximal limbs.
47
Rheumatic Fever involves the joints, heart, skin and nervous system. How does it effect the nervous system?
Chorea is the main NS symptom. This is irregular, uncontrolled and rapid movements of the limbs.
AKA Sydenham chorea
48
How do you assess a pt with suspected rheumatic fever?
Throat swab for bacterial cultures
ASO abs (Anti-Streptococcal abs) - indicate recent strep infection, repeat after 2 wks to confirm
Echo, ECG or CXR can determine heart involvement
49
What does the Jones Criteria diagnose?
Rheumatic fever.
Need evidence of recent strep infection + either 2 major criteria or 1 major + 2 minor.
Mneumonic JONES FEAR
Major:
Joint arthritis
Organ inflammation -eg carditis
Nodules
Erythema margitanum rash
Sydenham chorea
Minor:
Fever
ECG changes (long PR interal) w/o carditis
Arthralgia w/o arthritis
Raised inflammatory markers (ESR and CRP)
50
How do you manage streptococcal infections eg tonsillitis?
Phenoxymethylpenicillin (penicillin V) for 10 days
This helps avoid Rheumatic fever development
51
Rheumatic Fever Management?
Refer to specialist.
NSAIDs for joint pain
Aspirin and steroids for carditis
Prophylactic abx to prevent recurrence of rheumatic fever
Monitor and manage complications
52
What are the most common complications of rheumatic fever?
Chronic heart failure
Valular heart dx, esp Mitral stenosis
Recurrence of rheumatic fever
53
At what age are babies weaned off breast/bottle milk and on to puree food eg baby rice?
6 months old
54
What is the definition of failure to thrive?
Poor growth and development of a child
55
What are the causes of Failure to Thrive?
Inadequate nutritional intake eg neglect
Difficulty feeding eg cleft lip or cerebral palsy causing poor suck
Malabsorption eg coeliacs, cystic fibrosis + IBD
Increased energy requirements eg hyperthyroidism, chronic dx or malignancy
Inability to process nutrition eg T1DM
56
How do we assess faltering growth?
Urine dip for UTI
Coeliac screen (anti-TTG or anti-EMA abs)
If signs, can do tests for cystic fibrosis or pyloric stenosis
57
What are the causes of short stature in children?
- Familial short stature
- Constitutional delay in growth and development
- Malnutrition
- Chronic diseases, such as coeliac disease, inflammatory bowel disease or congenital heart disease
- Endocrine disorders, such as hypothyroidism
- Genetic conditions, such as Down syndrome
- Skeletal dysplasias, such as achondroplasia
58
What are the 4 major domains of childhood developmental milestones?
Gross motor
Fine motor
Language
Personal and social
59
What are the red flag signs that suggest a problem with development?
- Not able to hold an object at 5 months
- Not sitting unsupported at 12 months
- Not standing independently at 18 months
- Not walking independently at 2 years
- Not running at 2.5 years
- No words at 18 months
- No interest in others at 18 months
60
What is dyslexia?
Specific difficulty with reading, writing, and spelling.
61
What is dysgraphia?
A specific difficulty with writing.
62
What is dyspraxia?
AKA developmental co-ordination disorder.
Specific difficulty in physical co-ordination.
More common in boys.
Presents as delayed gross and fine motor skills, and the child appears clumsy
63
What is an auditory-processing disorder?
A specific difficulty in processing auditory information
64
What is a non-verbal learning disability?
A specific difficulty in processing non-verbal information, such as body language and facial expressions
65
What is a profound and multiple learning disability?
Severe difficulty in multiple areas, often requiring help with all aspects of everyday life.
66
What is the staging system used to determine a pt's pubertal stage?
Tanner Staging
67
What is hypogonadism?
A lack of the sex hormones, oestrogen and testosterone, resulting in delayed puberty.
It is either due to hypogonadotrophic hypogonadism (a deficiency is FSH or LH), or hypergonadotrophic hypogonadism (a lack of response to LH or FSH in the gonads/ovaries/testes)
68
What is APGAR Scoring?
The score used to assess newborns.
Assesses colour, HR, reflex irritability, muscle tone, respiration.
Higher score is better!
Do it 1m and 5m after birth.
69
What are the 4 conditions that co-exist to make up Tetralogy of Fallot?
Ventricular septal defect (VSD)
Overriding aorta
Pulmonary valve stenosis
Right ventricular hypertrophy
70
What is the pathology of Tetralogy of Fallot?
The 4 cardiac abnormalities cause a right to left cardiac shunt.
This means blood bypasses the child’s lungs.
Blood bypassing the lungs does not become oxygenated.
Deoxygenated blood entering the systemic circulation causes cyanosis.
The degree to which this happens is related mostly to the severity of the patients pulmonary stenosis.
71
What are the risk factors for Tetralogy of Fallot?
Rubella infection
Mother aged over 40
Alcohol in pregnancy
Diabetic mother
72
Ix for Tetralogy of Fallot?
Echo ( + doppler flow studies to determine the severity of the abnormalities + shunt)
CXR may show the characteristic "boot-shaped" heart due to right-ventricular thickening.
73
Px of Tetralogy of Fallot?
Most picked up in antenatal screening or by hearing an ejection systolic murmur in the newborn baby check. But otherwise:
-Cyanosis
-Clubbing
-Poor feeding
-Poor weight gain
-Ejection systolic murmur heard best in the pulmonary area
-'Tet spells'
74
What is a 'tet spell'?
Intermittent sporadic periods where the right to left shunt in a pt with Tetralogy of Fallot gets temporarily worse, precipitating a cyanotic episode.
This happens eg when a child is exercising, waking, and crying.
Severe spells may lead to LOC, seizures + potentially death.
Older kids squat during the spells, younger pull knees to chest.
Squatting increases systemic vascular resistance, encouraging blood to enter the pulmonary vessels.
75
Rx of a 'tet spell'?
-O2
-B-blocker
-IV Fluid
-Morphine
-Sodium bicarb if acidosis
-Phenylephrine infusion
76
Management of Tetralogy of Fallot?
In neonates, prostaglandins can maintain the ductus arteriosus, allowing blood to flow back from the aorta into the pulmonary veins.
Total surgical repair is definitive treatment.
77
What is a Hemiplegic migraine?
A rare type of migraine in which pts experience weakness in one 1/2 of their body (hemiplegia) as a form of aura, in addition to normal migraine symptoms.
78
What is a Pseudomyogenic hemangioendothelioma (PMHE)?
Pseudomyogenic hemangioendothelioma (PMHE) is a rare vascular tumour that affects teens and young adults.
Commonly in lower limbs.
79
What are the important DDs of headaches in children?
Tension headaches
Migraines
Ear, nose and throat infection
Analgesic headache
Problems with vision
Raised intracranial pressure
Brain tumours
Meningitis
Encephalitis
Carbon monoxide poisoning
80
What are the triggers of tension headaches in young kids?
Stress, fear or discomfort
Skipping meals
Dehydration
Infection
Theses cause a mild symmetrical band-like headache of the forehead etc
81
Tension headache management in kids?
Reassurance, analgesia, avoiding dehydration, regular meals + avoiding stress.
82
Migraine px in kids?
Unilateral, more severe +throbbing
Take longer to resolve
Associated with Visual aura, photophobia and phonophobia, nausea and vomiting, and abdominal pain
*Kids are more likely to px with abdominal migraines, where the pt has seere central abdo pain lasting >1hr along with the typical migraine symptoms. Examination is normal.
When a patient presents with possible migraines ask about recurrent central abdominal pain as a child. They may have a history of abdominal migraine that started before the headaches.
83
What is the management of migraines in children? (Not prophylaxis)
-Rest, fluids and low stimulus environment
-Paracetamol
-Ibuprofen
-Sumatriptan
-Antiemetics, such as domperidone (unless contraindicated)
84
What is the prophylactic management of migraines in kids?
-Propranolol (avoid in asthma)
-Pizotifen (often causes drowsiness)
-Topiramate (girls with child bearing potential need highly effective contraception as it is very teratogenic).
85
What is Juvenile dermatomyositis?
Juvenile dermatomyositis is a rare childhood disease that causes muscle weakness and skin rash.
Causes inflammation of the BVs, muscles and skin.
Most cases between ages 5-10.
86
What is Hirschsprung’s disease?
A congenital disease where the parasympathetic ganglion cells of the myenteric plexus (Auerbach’s plexus) are absent in the distal bowel and rectum.
The affected area doesn't relax and is constantly constricted, leading to obstruction of the bowel. The unaffected portion becomes distended and full.
When the entire colon is affected it is called total colonic aganglionosis.
87
What conditions are associated with Hirschsprung's disease?
-Downs syndrome
-Neurofibromatosis
-Waardenburg syndrome (a genetic condition causing pale blue eyes, hearing loss and patches of white skin and hair)
-Multiple endocrine neoplasia type II
88
Px of Hirschsprung’s disease?
Depends on the proportion of the bowel affected.
Can present with acute intestinal obstruction shortly after birth, or gradually. Symptoms include:
-Delay in passing meconium (more than 24 hours)
-Chronic constipation since birth
-Abdominal pain and distention
-Vomiting
-Poor weight gain and failure to thrive
89
What is Hirschsprung-Associated Enterocolitis (HAEC)?
Hirschsprung-Associated Enterocolitis is inflammation + obstruction of the intestine occuring in 20% of noenates with Hirschsprung disease.
It typically presents within 2-4 weeks of birth with fever, abdominal distention, diarrhoea (often with blood) and features of sepsis.
It is life threatening and can lead to toxic megacolon and perforation of the bowel.
It requires urgent antibiotics, fluid resuscitation and decompression of the obstructed bowel.
90
How do you diagnose Hirschsprung's disease?
Rectal biopsy shows an absence of ganglionic cells.
91
Management of Hirschsprung's disease?
IV fluid, management of the intestinal obstruction, then surgical removal of the aganglionic portion of the bowel.
92
What are the main DDs for a compromised airway in a paediatric pt?
Anaphylaxis
Foreign body aspiration
Epiglottitis
Croup
93
Anaphylaxis Px in kids
Urticaria
Itching
Angio-oedema, with swelling around eyes and lips
Acute onset
Skin changes
Abdo pain
94
What is DiGeorge Syndrome?
A 22q11 micro-deletion syndrome that is autosomal dominant and leads to a developmental delay in the third pharyngeal pouch and third branchial cleft.
95
What is the Px of DiGeorge Syndrome?
CATCH 22
C - Cardiac abnormality (Eg Tetralogy of Fallot, VSDs)
A - Abnormal facies (characteristic facial appearance)
T - Thymic hypoplasia --> Immunodeficiency as where T cells mature
C - Cleft palate (+lip) --> may cause unsafe swallow
H - Hypocalcaemia (due to parathyroid dysfunction)
22q11 microdeletion
96
How do you diagnose DiGeorge Syndrome?
FISH to confirm deletion
97
What is Meckel's Diverticulum?
Congenital embryological remnant of vitelline duct (which connects the yolk sac to the midgut lumen), often comprising of acid-secreting gastric mucosa.
Effects males x2 as much as females
98
What are the common complications of Meckel's Diverticulum?
Bowel obstruction as a consequence of:
Intussusception, midgut volvulus +/or adhesions or strictures from chronic inflammation.
99
Meckel's Diverticulum Px?
- Often asymptomatic.
- Painless, bright red PR bleeding
- Diarrhoea
- Umbilical pain radiating to RIF
- Obstruction Px = severe constipation, abdo pain,N+V, red current jelly stools (if intussusception)
100
Meckel's diverticulum Ix?
Technetium-99m pertechnetate scintigraphy
Abdo XR + CT
If unstable, skip imaging + do laparoscopy/laparotomy
101
What is the management of Meckel's Diverticulum?
If symptomatic, laparoscopic resection.
If asymptomatic, no Rx needed.
102
What is the main cause of UTIs in kids?
E-coli
103
What is Eisenmenger Syndrome?
Eisenmenger is the syndrome that occurs when there is a right --> left shunt across a structural heart lesion, bypassing the lungs.
104
What are the 3 main underlying lesions that can result in Eisenmenger syndrome?
- Atrial septal defect
- Ventricular septal defect
- Patent ductus arteriosus
105
When does Eisenmenger Syndrome usually Px?
1-2 yrs old with large shunts or in adulthood with smaller shunts.
Can develop faster in pregnancy, so women with a PMH of small shunts need monitoring with echos
106
What are the main examination findings in Eisenmengers Syndrome?
Pulmonary hypertension:
Right ventricular heave: forceful contraction against high pulm pressure
Loud P2: forceful shutting of the pulmonary valve
Raised JVP
Peripheral oedema
Underlying septal defect:
Atrial septal defect: mid-systolic, crescendo-decrescendo murmur loudest at the upper left sternal boarder
Ventricular septal defect: pan-systolic murmur loudest at the left lower sternal boarder
Patent ductus arteriosus: continuous crescendo-decrescendo “machinery” murmur
Arrhythmias
Right to left shunt and chronic hypoxia:
Cyanosis
Clubbing
Dyspnoea
Plethoric complexion (a red complexion related to polycythaemia)
107
What is the management of Eisnmenger's Syndrome?
Ideally, the defect should be corrected before the development of Eisenmenger's.
Once Eisenmengers develops, there is no way to medically reverse it. The only cure is a heart-lung transplant, which has a high mortality.
108
Who is usually affected by Cow's milk protein allergy?
Infants and young children under 3.
Formula-fed babies or those with personal/FH of atopic conditions.
Most pts grow out of it by 3
109
What is Cow's milk protein allergy?
Hypersenitivvity to cow's milk protein.
Mostly IgE-mediated, when there is a rapid reaction within 2 hrs of ingestion.
Can also be non-IgE-mediated, when the reaction is slower over a few days.
110
Cow's milk protein allergy Px?
- Bloating and wind
- Abdominal pain
- Diarrhoea + Vomiting
- General allergic Symptoms --> Urticarial rash (hives), angio-oedema (facial swelling), cough or wheeze, sneezing, watery eyes, eczema
111
What is the management of cow's milk protein allergy?
Breast-feeding mothers should avoid dairy.
Formulated milk should be replaced with hydrolysed formulas.
Hydrolysed formulas contain cow’s milk, but proteins have been broken down so no immune response.
In severe cases infants may require elemental formulas made of basic amino acids (e.g. neocate).
Go up milk ladder slowly until can drink milk on its own.
112
What is the most common predisposition to developing Cerebral Palsy?
HIE (Hypoxic Ischaemic Encephalopathy)
Usually pre-natal or early post-natal birth trauma
113
What is William's Syndrome?
A deletion on chromosome 7.
The distinctive features are v.sociable personality, starburst eyes (a star-like pattern on the iris), and a wide mouth with a big smile.
Assoc. w/ supravalvular aortic stenosis and hypercalcaemia
114
William Syndrome Px?
Broad forehead
Starburst eyes (a star-like pattern on iris)
Flattened nasal bridge
Long philtrum
Wide mouth with widely spaced teeth
Small chin
V. sociable + trusting personality
Mild learning disability
115
What conditions are William Syndrome associated with?
Supravalvular aortic stenosis (narrowing just above the aortic valve)
Attention-deficit hyperactivity disorder
Hypertension
Hypercalcaemia
116
William Syndrome Rx?
Regular echos and BP monitoring to assess for aortic stenosis.
Low calcium diet to control hypercalcaemia (+ avoid calcium and vit D supplements)
117
When does the ductus arteriosus stop functioning and when does it close completely?
The ductus arteriosus stops functioning 1-3 days after birth.
It closes completely within first 2-3 wks of life.
118
What predisposes you to a patent ductus arteriosus?
Rubella infection
Prematurity
Genetics
119
How would a patent ductus arteriosus that has remained asymptomatic during childhood present in adulhood?
Signs of heart failure.
120
What is the pathophysiology of a patent ductus arteriosis?
- Pressure in aorta >pressure in pulm artery
- Blood shunted through patent ductus arteriosus from aorta to pulm artery
- Pulm HTN
- Right-sided heart strain + :. right ventricular hypertrophy
- Increased blood flow back into LV from lungs leads to left ventricular hypertrophy
121
Px of a patent ductus arteriosus?
- SOB
- Difficulty feeding
- Poor weight gain
- worse LRTIs (pulm oedema means that when they get LRTIs they have less pulmonary reserve to fight infection)
- Continuous crescendo-decrescendo “machinery” murmur that may continue into second heart sound.
122
How do you diagnose a patent ductus arteriosus?
Confirm with Echo
Doppler to identify extent of shunt
123
Patent ductus arteriosus management?
Monitor with echos until 1yr.
If hasn't closed by 1yr, do trans-catheter or surgical closure.
Symptomatic pts/those with heart failure evidence treated earlier
124
What are the features of neonatal sepsis?
- Respiratory distress
- Tachycardia
- Apnoea
- Altered mental status/lethargy
- Jaundice
- Poor feeding
- Abdo distension
- Vomiting
125
How does a neonate usually acquire neonatal sepsis?
Transplacentally, via descent from vagina, during birth or from the environment
126
What are the risk factors for neonatal sepsis?
Mother:
- previous baby with GBS
-current GBS colonisation
- current bacteraemia
-intrapartum fever
- membrane rupture >18hrs
-chorioamnionitis
Baby:
- premature
-low birth weight
127
What are the 2 main causative organisms of neonatal sepsis?
Group B Strep (within 72hrs of birth)
Staph. Epidermidis or gram -ve bacteria (7-28 days of life)
128
What is the management of neonatal sepsis?
1st line: IV Benzylpenicillin with gentamicin
*Cefotaxime +aciclovir if meningitis
Fluids + O2 maintenance
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What is Hypoxic Ischaemic Encephalopathy (HIE)?
HIE is a clinical syndrome of brain injury secondary to perinatal hypoxic ischaemic insult in term babies.
The baby is typically unwell at birth, requiring resus.
Encephalopathy develops within 24hrs of birth
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How do you manage a patient with HIE (Hypoxic ischaemic encephalopathy)?
Resuscitation
Monitoring and treating seizures
Cooling --> therapeutic hypothermia reduces death and disability. TOBY cooling criteria.
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What is the Px of Disseminated intravascular coagulation (DIC)?
Petechiae
Bleeding (cutaneous, mucosal, umbilical, GI)
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What would you see on a blood film from someone with DIC?
low platelets
schistocytes (fragmented RBCs)
high INR
high APTT
Low fibrinogen
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How would you manage a child with DIC?
- Treat the underlying cause (could be HIE, RDS, NEC, sepsis)
- 1mg of vit K
- blood products
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What causes neonatal respiratory distress syndrome (NRDS)?
Insufficient surfactant production and structural immaturity of the lungs.
Commonly seen in premature infants - as gestational age increases, the risk of NRDS decreases.
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What are the risk factors for having NRDS?
-male sex
- maternal diabetes
- C section
- 2nd born of premature twins
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How would a baby with NRDS Px?
-Respiratory distress
- Tachypnoea
- Intercostal recession
- Expiratory grunting
- Cyanosis
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What features on a CXR are diagnostic of NRDS?
'Ground glass' appearance with an indistinct heart border
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What is the management of a neonate with NRDS?
During pregnancy: Maternal steroids
Post-natal: O2, assissted ventilation, exogenous surfactant given via ET.
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How do Neonatal seizures Px?
Occur 12-48hrs after birth. Can be any type of seizure.
Look for lip smacking, limb-climbing, eye deviation, apnoeas.
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What are the causes of neonatal seizures?
HIE
Infection
Intracranial bleed
CNS lesions
Metabolic disturbances
Drug withdrawal
Kernicterus
Benign neonatal convulsions (5th day fits)
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What is Kernicterus?
Kernicterus is a type of brain damage that can result from high levels of bilirubin in a baby's blood.
It can cause cerebral palsy and hearing loss.
Kernicterus also causes problems with vision and teeth and sometimes can cause intellectual disabilities.
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What is the management of neonatal seizures?
-Check glucose and turn on side if aspiration risk.
-1st line: phenobarbital 20mg/kg IV as slow injection
-2nd line: phenytoin 18mg/kg IV
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What neonatal infection/condition would you suspect if you saw the following:
-Abdominal distension
-Bilious aspirates
-FOB
- Temp instability
- Lethargy
- Discolouration
-Mucosal sloughing
- DIC
Necrotising Enterocolitis (NEC)
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How would you diagnose NEC?
Abdo XR and USS
- See gas in the gut/pneumatosis intestinalis
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How do you manage a neonate with necrotising enterocolitis?
-Make them NBM
- Insert an NG tube
- IV Abx
- Surgery: laparotomy if progressive distension or perforatio (1/2 of perfs die)
- Monitor platelets for dx progression
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What is the cause of Meconium aspiration syndrome (MAS)?
Meconium-stained amniotic fluid occurs in 8-25% of births when meconium passed in utero due to foetal maturity/distress.
MAS occurs in 5% of these cases.
Aspiration of meconium occurs in utero with prolonged hypoxia.
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What are the features of meconium aspiration syndrome?
- Respiratory distress
- Airway obstruction
- Surfactant dysfunction
- Pulmonary vasoconstriction or persistent pulmonary HTN
- Chemical pneumonitis
- Pneumothorax
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What. is the management of MAS?
Surfactant, ventilation, inhales NO, Abx, chest drains and ECMO
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What is transient tachypnoea of the newborn (TTN)?
TTN is increased RR +/- mild WOB starting in the first 4hrs of birth due to slow clearance of lung fluid/delayed resorp
