Paeds Flashcards
(183 cards)
1
Q
Double bubble on an abdominal x-ray refers to and represents what:
A
Duodenal atresia
Represents the normal gastric bubble and duodenal dilation proximal to the obstruction
2
Q
Neonates with trisomy 21 (Down syndrome) are at increased risk for developing which GI condition:
A
duodenal atresia
3
Q
Pyloric stenosis refers to thickening of what muscle and what type of vomiting is it?
A
Refers to thickening of the gastric smooth muscle at the pylorus, and this prevents food from passing out of the stomach and into the duodenum
These pts present with forceful, non-bilious vomiting within the first month of life
4
Q
What can be felt on an abdominal examination for pyloric stenosis?
A
A palpable mass on examination, ‘Olive-shaped’ mass
5
Q
A neonate that does not pass meconium within the first few days of life is suggestion of what:
A
Imperforate anus
6
Q
Biliary atresia is often associated with trisomy 21, these pts suffer from what?
A
- Suffer from indigestion due to bile sludging
- Impaired fat absorption
- They can become jaundiced due to bile retention
7
Q
Tracheo-oesophageal fistula can be diagnosed:
A
Bronchoscopy and surgical correction will be required
8
Q
What are the respiratory distress?
A
- Nasal flaring
- Grunting
- Intercostal recession
- Increased respiratory effort
- Cyanosis
9
Q
Acute bronchiolitis has the causative agent (RSV) has which symptoms:
A
- Prodome of coryzal symptoms and respiratory distress
10
Q
A history of polyhydroamnios suggests what the fetus was unable to swallow what?
A
Amniotic fluid
11
Q
A baby who is blue, and has ventricular septum and a slightly enlarged right ventricle, can lead to:
A
pulmonary hypertrophy with shunt reversal leading to congestive heart failure
12
Q
The investigation Chest X-ray would only be appropriate if an infective cause (pneunomia) was suspected, what would the qn mention?
A
- Acute onset of a productive cough
2. Fever
13
Q
The investigation CFTR gene analysis would only be appropriate if CF is suspected, what would the qn mention?
A
- History of recurrent chest infections
2. Growth depression
14
Q
6yr old child has coughs at night, wheezing when running, also has eczema and hay fever, what investigation should be used?
A
Spirometry should be performed in all children with suspected asthma
You would expect to see an obstructive pattern on spirometry
15
Q
What is the second step in the management of asthma in >5 yrs old?
A
Trial of salmeterol inhaler as a LTRA
16
Q
First line treatment management for asthma for children?
A
Step 1: Inhaled SABA PRN
and
consider monitored initiation of very low to low dose ICS (Inhaled corticosteroid)
17
Q
A child presents with few days of fever with since not recurred followed by a widespread rose-pink macular rash with surrounding pale white halos with minimal spread and cervical lymphadenopathy, with the cause being human herpes virus 6?
(Note it’s a very common childhood infection)
A
Roseola Infantum
18
Q
Roseola Infantum is caused by:
A
Human herpes virus 6 is the pathogen responsible for roseola
19
Q
Roseola infantum, a common disease in children characterised by an initial high fever (which settles) but followed by a maculopapular rash.
It is caused by human herpes virus 6, what is the most common complication of this disease?
A
Febrile convulsions occur in up to 15% of children with the disease
20
Q
Other than febrile convulsions, which is another recognised complication with human herpes virus 6 causing roseola infantum?
A
Meningitis
21
Q
Orchitis is a common complication of:
A
mumps
mumps, a viral infection, usually presents with fever, malaise, muscular pain and parotitis
22
Q
Sensorineural hearing loss is a common complication of:
A
bacterial menigitis
23
Q
Sensorineural hearing loss is a common complication of:
A
bacterial meningitis
24
Q
Otitis media is the most common complication following and which other long term complication:
A
-measles
Measles would present with conjunctivitis, fever, irritability, Koplik spots, as well as a rash which starts behind the ear before spreading to the whole body
Subacute sclerosing panencephalitis is a possible long-term complication, which can occur up to 10 years after initial measles infection.
25
A child who's short of breath, used a salbutamol inhaler, and used a nebulised ipratropium bromide?
- The management of acute severe asthma is approached in a step-wise fashion
- Inhaled and nebulised salbutamol and nebulised ipratropium bromide should be tried before moving to intravenous salbutamol
26
At what stage of O2 sats would high flow oxygen be given to an asthmatic?
- Maintaining oxygen saturations of 96%, so unless oxygen saturations fall to <92%, there is no indication to give high flow oxygen
27
For the management of asthma, and if the O2 saturations <92% add magnesium sulphate?
If O2 saturations <92% add magnesium sulphate
28
If it's severe or life-threatening acute asthma and it's not responsive to inhaled therapy, what can be added?
aminophylline
29
Pneumothorax and anaphylaxis can be differentials for asthma, what would be the presentations?
- Pneumothorax and anaphylaxis will be very sudden onset (with pneumothorax being associated with chest pain and possible deviation of the trachea and anaphylaxis being associated with antigen exposure)
- Inhalation of a foreign body and pneumothorax will give unilateral chest signs and cardiac arrhythmia is suggested by chest pain or palpitations, tachycardia or changes in blood pressure
- Acute asthma is suggested by widespread wheeze, with exacerbation developing over minutes to hours and is confirmed by a reduced peak expiratory flow rate and FEV1 which improves with treatment
30
When will you consider adding nebulised Magnesium sulphate?
Unless oxygen saturations fall to <92%, there is no indication to add nebulised magnesium sulphate.
According to the British Thoracic Society guidelines, you should consider adding 150 mg magnesium sulphate to each nebulised salbutamol and ipratropium in the first hour in children with a short duration of acute severe asthma symptoms presenting with an SpO2 <92%
31
A child with persistent ductus arteriosus
| (Treatment of a persistent ductus arteriosus is only required if the baby is symptomatic), what is the treatment?
About 1/3 of patients with a patent ductus arteriosus require medical treatment with indomethacin
Indomethacin is an NSAID that inhibits prostaglandin synthesis. Inhibition of prostaglandin causes vasoconstriction which closes the ductus arteriosus
32
A child with persistent ductus arteriosus
| (Treatment of a persistent ductus arteriosus is only required if the baby is symptomatic), what is the treatment?
About 1/3 of patients with a patent ductus arteriosus require medical treatment with indomethacin
Indomethacin is an NSAID that inhibits prostaglandin synthesis. Inhibition of prostaglandin causes vasoconstriction which closes the ductus arteriosus
33
Patent ductus arteriosus has which features:
On examination, the pulse is bounding, collapsing pulse and there is a continuous machine-like murmur heard loudest at the upper left sternal edge.
34
Coarctation of the aorta, the pulse is:
noticeably weaker in the legs or groin than in the arms or neck
35
What can be used for the diagnosis of fungal skin infections like Tinea (ringworm)...commonly presents as an annular erythematous plaque with a cleared centre?
Skin scraping
36
A child, with a fever for >5 days, conjunctivitis, erythema and edema of hands and feet with peeling and cervical lymphadenopathy most likely has:
Kawasaki disease
37
Management of Kawasaki disease is:
IV immunoglobin (IV Ig) and oral aspirin
Aspirin is usually avoided in children due to the risk of Reyes syndrome (liver and brain damage).
38
Kawasaki disease common complication:
Coronary artery aneurysms
(can occur in 20-25% untreated cases)
Echocardiogram
39
Criteria for diagnosis of Kawasaki disease include fever for >5 days, and 4/5 of the 'CREAM' features:
1. Conjunctivitis
2. Rash
3. Edema/Erythema of hands and feet
4. Adenopathy (cervical, commonly unilateral)
5. Mucosal involvement (strawberry tongue, oral fissures etc)
40
CT head strict guidelines with indications for CT head in children, for example, in certain circumstances such as:
Head trauma
41
What the most important feature of a rash that should be considered in the first instance?
Blanching or non-blanching
42
Grey spots inside the cheeks are associated with what:
These are koplik's spots (grey spots inside the cheeks) are associated with measles, but measles is a self-limiting illness with no specific treatment, it's important to rule out any possible features of meningococcal illness first
43
Hand, foot and mouth disease presents with which features:
Presents with blisters on the hands and feet and ulcerations on the tongue and a fever
44
Hand, foot and mouth disease is caused by:
Coxsackie virus A16
45
Hand, foot and mouth disease management:
It is self-limiting and usually resolves within one week
46
A child presents with scarlet fever, which features:
Scarlet fever presents with a course red rash and other non-specific symptoms such as:
- Sore throat
- Headache
- Fever
It has a characteristic 'sandpaper' texture and the tongue appears bright red.
47
Scarlet fever is treated with:
Antibiotics (usually 10 days of phenoxymethylpenicillin)
48
For scarlet fever, children remain infectious until when:
24hrs after the first dose of antibiotics
49
A child presents with raised, red rashes which occurs as an allergic reaction, is called what?
Hives
50
Features of Gastro-oesophageal reflux in children?
As acid refluxes back up the oesophagus, babies commonly present with 4 features:
1. Milky vomits after feeds.
2. Crying/irritability
3. Arching of the back
4. Drawing up of the knees into the chest
51
A child of 6 months presents with milky vomits after feeding has a history and examination most consistent with what:
gastroesophageal reflux disease (GORD)
52
Vomiting after being laid flat and crying with arching of the back and drawing up the knees to the chest is characteristic of .
reflux
53
A child who's been presenting with symptoms of reflux, and is well and has no signs of dehydration (moist mucous membranes, normal capillary refill time) and there are no red flags (normal blood gas, no palpable abdominal mass, no fever, no diarrhoea).
The chid is well, what safety netting advice should be given before being discharged?
Advice on conservative treatments (keep upright and burp after feeds, put their cot on slight incline) and infant gaviscon
Management of GORD in infants can be:
Conservative (advice on keeping baby upright and burping after feeds, put their cot on a slight incline for sleeping)
Medical (infant gaviscon, omeprazole)
Surgical in severe cases (fundoplication)
54
Which of the following is reserved for the treatment of severe episodes of this pt presentation with Tet spells?
Tet spells can be managed with analgesia and supplemental oxygen. A last line medication is a vasoconstrictive agent (e.g. phenylephrine). Vasoconstriction will help to increase systemic vascular resistance, reducing the right-to-left shunt and improving cyanosis.
LAST LINE: Phenylephrine (a vaso-constrictive agent) as a last line medical therapy
Vasoconstriction will help to increase systemic vascular resistance, reducing the right-to-left shunt and improving cyanosis
55
What is the surgical management of Tetralogy patients?
They will need definitive surgery to correct the ventricular septal defect and widen the right ventricular outflow tract
56
What are the features of patients with Tetralogy of Fallot?
Tetralogy of Fallot is a relatively rare form of congenital cardiac disease. The clinical features of Tetralogy of Fallot are:
- Pulmonary stenosis
- Right ventricular hypertrophy
- Overriding aorta
- Ventricular septal defect.
PROV
57
What happens during a tet spell?
Tetralogy of Fallot is most commonly diagnosed antenatally, or on detection of a murmur in the first few months of life. Babies can present with tet spells, which are acute episodes of cyanosis.
During a tet spell, pulmonary stenosis causes a right-to-left shunt of deoxygenated blood across the ventricular septal defect. This results in hypoxia, causing pain (inconsolable crying) and cyanosis.
58
What is used to stabilise the myocardium in severe hyperkalaemia?
Calcium gluconate
59
Common congenital heart disease in Down's syndrome?
- ASDs and atrioventricular septal defects (AVSD) are a common type of congenital heart disease seen in patients with Down's syndrome
- It causes an ejection systolic murmur at the upper left sternal edge
- The first heart sound is normal but there is wide fixed splitting of the second heart sound.
(VSDs are common in Down's syndrome however they cause a pansystolic murmur heard in lower left sternal edge often all over the praecordium and do not usually radiate to the back
Also, the second heart sound is normal in VSD, but wide fixed splitting occurs in ASD)
60
What is the most common associated condition for Down syndrome?
Main concern is CHD risk
61
What are the three heart conditions associated with down syndrome?
Of these, atrioventricular septal defects (AVSD) are the most common, followed by ventricular septal defects (VSD)
Patients with trisomy 21 are also more likely to have a patent ductus arteriosus (PDA)
62
What is the most serious complication of untreated jaundice in babies?
Kernicterus is a rare but serious complication of untreated jaundice in babies
It's caused by excess bilirubin damaging the brain or central nervous system
Treatment for kernicterus involves using an exchange transfusion.
63
When would you request an urgent chest x-ray for a baby presenting with jaundice?
There's no infective respiratory cause of jaundice (e.g. Coughing, nasal flaring, cyanosis, etc.)
64
What days will breast feeding jaundice occur?
Between 2-14 days
65
A 27 day old neonate not feeding well. The baby is exclusively breast fed and has not had any problems with latching on before and previously was putting on weight.
The baby overall is jaundice and unwell, on examination, the baby looks jaundiced and is lethargic. The stool is of a normal colour and consistency. The mother says that there have been fewer wet nappies over the last 12 hours. There is some loss of skin turgor. There is no hepatomegaly on palpation of the abdomen.
- In a jaundiced unwell baby, sepsis is the first thing which has to be investigated due to the devastating consequences (organ failure, kernicterus, death)
- A urinary tract infection (UTI) is a common serious cause of jaundice in the newborn, and can often present with vague symptoms of lethargy or difficulty feeding
- UTI in a newborn is particularly serious as it can rapidly progress to sepsis.
- Remember to check glucose as hypo can be another sign of sepsis
66
What are the causes of jaundice of a baby less than 24 hrs?
Haemolytic disorders (Rh incompatibility, ABO incompatibility, G6PD, spherocytosis), Infection (TORCH Screen is indicated)
67
What are the causes of jaundice of a baby 24 hours- 14 days?
Physiological jaundice, breast milk jaundice, dehydration, infection, haemolysis, bruising, polycythaemia, Crigler-Najjar Syndrome
68
What are the causes of jaundice of a baby >14 days (>21 if preterm)?
Physiological jaundice, breast milk jaundice, infection (esp UTI), hypothyroidism, G6PD, pyloric stenosis, bile obstruction (biliary atresia), neonatal hepatitis
69
What causes physiological jaundice?
Marked physiological release of Hb from breakdown of red cells due to high Hb at birth
Red cell life span of newborns is shorter than adults
Hepatic bilirubin metabolism less efficient in first few days of life
70
This child with sudden onset respiratory distress, lip and tongue swelling, and inspiratory stridor immediately after eating demonstrates acute anaphylaxis, the dose of adrenaline of child under 6:
150 micrograms of 1:1000 is for children under 6
71
This child with sudden onset respiratory distress, lip and tongue swelling, and inspiratory stridor immediately after eating demonstrates acute anaphylaxis, the dose of adrenaline of any child between 6-12:
300 micrograms is for children (6-12)
72
This child with sudden onset respiratory distress, lip and tongue swelling, and inspiratory stridor immediately after eating demonstrates acute anaphylaxis, the dose of adrenaline of children over 12 and adults
500 micrograms is for children over 12 and adults
73
What equipment is used for an anaphylaxis?
- Administer oxygen and manage airway
- IV fluid challenge
- Administer chlorphenamine and hydrocortisone
- Attach patient to monitoring
74
What is measured to confirm a diagnosis of anaphylaxis?
Serum levels of mast cell tryptase
75
A 15 year old male presents to his GP with a widespread maculopapular rash that developed over the past day. He recently recovered from a flu-like illness and sore throat 1 week ago, for which he was given a course of antibiotics. There are no other abnormalities on physical examination. What's the cause?
Epstein Barr virus (EBV)
This is the causative agent of infectious mononucleosis, which presents as a fever, sore throat and lymphadenopathy. If given amoxicillin, patients may develop a widespread maculopapular rash due to a hypersensitivity reaction.
Management of EBV infection is supportive
76
What are the two features to note of scarlet fever?
rash has a sandpaper texture
| strawberry tongue of scarlet fever
77
A child presents with meningitis suspected, the child should be given what immediately?
Immediate intramuscular benzylpenicillin and sent to hospital
78
What few things looked at a newborn screening?
Newborn screening checks are undertaken by a doctor or midwife before discharge from maternity units
They screen for various congenital abnormalities including of the:
eyes (cataracts, retinoblastoma)
heart (murmurs)
hips (developmental dysplasia of the hip)- with Barlow and Ortolani manoeuvres screens- In DDH, the acetabulum of the hip does not articulate properly with the ball of the femur and the joint does not develop properly. This can cause mobility issues and early onset arthritis if not properly recognised and managed
checking that the testes are descended in boys
79
What genetic condition who parents with it often report that their child will eat anything and everything and will be consistently hungry?
Prader-Willi Syndrome
This girl with progressive obesity, hyperphagia, short stature and learning difficulties on a background of hypotonia which would have caused difficulty breastfeeding as an infant, most likely has a diagnosis of Prader-Willi syndrome.
80
Duchenne muscular dystrophy is an x-linked recessive condition and is almost exclusively seen in males, and it causes what:
- Progressive proximal muscle weakness and therefore problems with mobility
- The main features of Duchenne muscular dystrophy are delayed walking, calf pseudohypertrophy and a positive Gowers' sign
81
Fragile x characteristic appearance is a:
long thin face and large low-set ears
82
Does Angelman Syndrome cause childhood obesity like Prader-Willi syndrome?
No it does not
However this is caused by genomic imprinting like Prader willi syndrome
Children with Angelman syndrome often have a happy disposition and severe intellectual disability
83
Does Angelman Syndrome cause childhood obesity like Prader-Willi syndrome?
No it does not
However this is caused by genomic imprinting like Prader willi syndrome
Children with Angelman syndrome often have a happy disposition and severe intellectual disability
84
What are the features of congenital diaphragmatic hernia?
Diaphragmatic hernias cause opacification of a hemithorax (typically left) and a scaphoid abdomen, and occasionally distended loops of bowel
85
Appendicitis has which classic x-ray findings:
A perforated appendix may cause an acutely peritonitic abdomen and a shocked infant, however it would show air in the abdomen without generalised pneumointestinalis.
86
How is Necrotising Enterocolitis diagnosed and what is the typical presentation of NEC?
Necrotising enterocolitis is diagnosed with abdominal X-ray
Classic X-ray findings are of grossly distended bowel loops with air within the bowel wall (pneumointestinalis)....As the abdomen becomes significantly distended, there are dilated loops of bowel see on X-ray.
There may also be air within the portal tract and biliary tree
Surgery is required for perforations
Other characteristic abdominal X-ray findings include air in the biliary tree and air in the bowel wall (thin black lines in the white bowel wall), which confirm the diagnosis
87
Management of NEC?
Necrotising enterocolitis is managed with broad-spectrum antibiotics (to cover both anaerobic and aerobic bacteria) and parenteral nutrition (to rest the bowel)
Supportive treatment with IV fluids and ventilation are also crucial
Surgery to resect necrotic sections of bowel may be necessary, and is essential in cases of bowel perforation.
88
What's a positive Gowers sign? And is seen where?
Gowers' sign describes a patient that has to use their hands and arms to 'walk' up their own body in order to stand up from a supine position. This is classically seen in Duchenne muscular dystrophy (DMD) due to proximal muscle weakness
All boys that are not walking by the age of 1 1/2 years should have creatinine levels measured, to rule out DMD
89
On assessment, the boy has a waddling gait with a tendency to walk on his toes. He also struggles to stand up from lying without walking his arms up his legs. Likely diagnosis?
Presentation of Duchenne's muscular dystrophy
(In Duchenne's muscular dystrophy, children may have bulky-appearing muscles, as degenerated muscle is replaced by fat. Parents may notice that the child 'slips through their hands' when they pick them up (due to loose muscles in the shoulder)
90
Children with muscular dystrophies most commonly die from:
respiratory complications or dilated cardiomyopathy
91
The features of 'innoSent' murmurs all start with the letter S:
Soft Systolic murmur at the left Sternal edge in an a Symptomatic patient
92
When a child who has a fever, when would think the infection to likely be a mild viral upper respiratory tract infection:
On ENT examination:
- Throat is pink with no swelling or exudate of the tonsils
- Ears- tympanic membranes are visible with no bulging or effusion
- On auscultation of the chest, you hear a soft systolic murmur at the left sternal edge
This is evidenced by a lack of tonsillar exudate, angry red throat and no sign of ear infection (bulging tympanic membrane with effusion) so infection is likely to be a mild viral upper respiratory tract infection
93
Those with Down's syndrome have an increased risk of developing:
Acute lymphocytic leukaemia (ALL)
Acute lymphocytic leukaemia (ALL) is the most common childhood malignancy and often presents with anaemia (fatigue- sleeping more than usual and pallor), thrombocytopenia (bruising and epistaxis- experienced recurrent nose bleeds and bruising on his arms and legs) and neutropenia (recurrent infections and fever)
The testicular swelling is due to infiltration
Individuals with a diagnosis of Down syndrome have a four-fold increased risk of developing ALL
94
Non-hodgkins lymphoma is commonly associated with viral infections such as:
H. Pylori or Ebstein-Barr virus
Non-Hodgkins lymphoma does present with constitutional symptoms (eg. fever, weight loss, fatigue) and lymphadenopathy; however, in children, acute lymphocytic leukaemia is far more common
95
Henoch-Schonlein Purpura features:
Henoch-Schonlein purpura (HSP) presents with the classical triad of abdominal pain, arthralgia (joint pain) and purpura on the extensor surfaces and buttocks following a viral illness
HSP can rarely cause scrotal swelling; however, it does not cause lymphadenopathy
96
Acute Lymphocytic Leukaemia in children, what's the most common sign?
Lymphadenopathy
97
ALL is diagnosed definitively through
bone marrow biopsy
98
ALL has a bimodal distribution typically affecting
children under 6 years of age and adults over 80.
99
Schistocytes cells are typically seen in:
These are fragments of red blood cells seen on blood smears in patients with haemolytic anaemia
100
Reed Sternberg cells is typically seen:
These are giant B cells seen in patients with Hodgkin's lymphoma
They are typically multinucleated and contain inclusions
101
Orphan Annie Nuclei are seen:
These are typical of papillary thyroid cancer and are seen on fine needle aspiration of the thyroid
102
Smudge cells are seen in:
These are fragile B cells which are broken on smears that are found in chronic lymphocytic leukaemia
103
Caseating granulomas are seen in:
This is seen in Tuberculosis, which is an important differential in this case
Depending on location however, it is less likely than Hodgkin's lymphoma
104
Hodgkin's lymphoma presents with B symptoms:
fever, night sweats, and weight loss
occur in 30% of patients
It typically presents in young adults with cervical or supraclavicular non-tender lymphadenopathy, though the location of diseased nodes can vary.
105
Initial management and other lines management of primary nocturnal enuresis:
Conservative measures such as parents made adjustments to his sleeping routine and fluid intake in the evening and A star reward chart
Second line is enuresis alarm
Third line Desmopressin is a synthetic anti-diuretic hormone analogue which is licensed for use in children over the age of 7 in whom reward systems and an enuresis alarm have failed......This drug increases water re-absorption and reduces urine production overnight....man-made form of vasopressin and is used to replace a low level of vasopressin
Fourth line: Imipramine is a tricyclic antidepressant that can be used to treat nocturnal enuresis, however this is after a trial of Desmopressin.
106
For a child who is wetting the bed at night, when would you give oxybutynin:
Oxybutynin would only be indicated if there were symptoms of detrusor instability, such as frequency or daytime urgency
107
What's the difference between primary and secondary nocturnal enuresis:
Nocturnal enuresis (bedwetting) can be primary or secondary.
Primary nocturnal enuresis is seen in children who have never achieved urinary continence overnight.
Secondary nocturnal enuresis is seen in children who have previously achieved urinary continence overnight.
108
Nocturnal enuresis can have a variety of underlying physical causes, such as:
Diabetes mellitus
Urinary tract infections
Constipation
109
Investigation for nocturnal enuresis:
A full history and examination and a urine dip should always be performed.
Secondary nocturnal enuresis requires more in-depth investigation to ensure there is no underlying physical cause, with a urine dip, urine osmolarity and renal ultrasound scan.
110
Type 1 is an autoimmune condition which presents in childhood with:
Polyuria, polydipsia and weight loss
Diagnosis can be made based off a random plasma glucose measurement > 11.0 mmol/L if the patient has symptoms
Type 2 diabetes is associated with weight gain and obesity rather than weight loss
111
What's the most associated condition to type 1 diabetes?
Thyroid disease so screening is recommended
112
List some complications and associations with type 1 diabetes?
```
Complications
Growth and pubertal development (delay in puberty and obesity)
Hypertension
Renal disease
Retinopathy
Feet
```
Associated illnesses:
Thyroid disease (most associated; screening recommended)
Coeliac disease
113
Definitive management of Type 1 diabetes:
- Definitive management of type 1 diabetes is through insulin replacement with a personalised regimen of short-acting (after meals and snacks) and long-term (at night) insulin replacement therapy
- Insulin can be delivered through subcutaneous injections with a pen device, or with a continuous automatic infusion pump in older children.
- Overall, T1DM is a complex condition that requires a multidisciplinary team approach
- It is essential to make sure the family, patient and school are educated and engaged with monitoring diet, glucose levels and insulin therapy in this lifelong condition
114
What are the symptoms and management of hypoglycaemia?
Symptoms
Hypoglycaemia presents with irritability, abdominal pain, sweating, dizziness, loss of consciousness and even fits. It should be managed according to the condition of the child.
Management
If the hypoglycaemic child is alert, sugary drinks, gels and snacks should be encouraged. If unconscious and out of hospital, intramuscular glucagon should be given. In hospital, an unconscious child should be started on intravenous dextrose.
115
Sturge-Weber syndrome has which distinct feature:
distinctive port-wine stains on the forehead, face or scalp
and an abnormality of the brain
116
Features of Tuberous sclerosis:
The ash leaf macules (hypopigmentation) and shagreen patch- orange peel cover- (leathery plaque) are common cutaneous features of tuberous sclerosis
The abnormal repetitive movements are suggestive of infantile spasms, another feature of tuberous sclerosis complex.
117
What is the most common cogential cardiac defect?
Ventricular septal defect (VSD) which accounts for about 30-60% of all congenital heart defects
118
Ventricular septal defect (VSD) has what type of murmur:
Pan systolic murmur most likely associated with VSD
119
Transposition of the great vessels is a defect where the aorta and pulmonary artery are swapped over, it presents with what:
early-onset cyanosis and is associated with type 1/2 diabetes in the mother
120
For VSDs that are causing signs and symptoms of heart failure, what may be useful to use?
ACE-Is such as captopril may be useful. Corrective surgery may be required in very severe cases
Diagnosis
VSDs are definitively diagnosed with echocardiogram scans, which allow visualisation of the defect and characterisation of its severity.
121
Vomiting more frequent and forceful in nature (projectile) and not passed stools yet today, what is the condition and what is the most definitive treatment:
Laparoscopic pyloromyotomy
Pyloric stenosis
122
What are the complications of pyloric stenosis? e.g. k+ levels, h+, cl- levels
Severe vomiting in pyloric stenosis may lead to an acid base abnormality of hypochloremic hypokalemic metabolic alkalosis.
This is because of loss of stomach hydrochloric acid. Loss of Cl- causes hypochloremia.
Loss of H+ causes alkalosis.
Furthermore, loss of H+ results in increased renal reabsorption of H+ in exchange for K+
123
Fluid resuscitation regime to start for this patient based on weight is calculated by:
Fluid resuscitation regime to start for this patient based on his weight, with fluids of:
20/ml/Kg being appropriate in children for the first 10Kg of their weight
124
What's the Prophylaxis for a child with Bronchiolitis?
Palivizumab prophylaxis in high risk patients
125
child has had coryzal symptoms and a sharp non-productive cough. The past medical history is unremarkable. On examination the child is tachypnoeic and tachycardic, the chest is hyperinflated and there are fine crackles. Given the likely diagnosis, what is the most common causal organism? Common symptoms of bronchiolitis:
```
Cough
Laboured breathing
Wheeze
Tachypnoea
Intercostal recession, grunting, and nasal flaring
```
126
XXY karyotype causes:
Klinefelter's syndrome
Klinefelter's syndrome presents in patients who are phenotypically male and it may only be recognised on investigation of infertility
Features of Klinefelter's include testicular atrophy, gynaecomastia and a female distribution of hair, all caused by an underlying increase in oestrogen production
127
Trisomy 18 causes what and what symptoms:
Trisomy 18 causes Edward's syndrome, which presents with low-set ears, micrognathia and microcephaly, overlapping 4th and 5th fingers, and rocked bottomed feet
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Trisomy 21 causes:
Down's syndrome
Trisomy 21 causes Down's syndrome, which presents with prominent epicanthal folds, flat facial features, a single palmar crease, a sandal gap and brushfield spots in the eyes and learning difficulties, among other abnormalities.
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Trisomy 13 causes:
Trisomy 13 causes Patau syndrome, which presents with serious congenital defects including holoprosencephaly, cleft palate and polydactyly
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XO karyotype causes what:
teenager who has never menstruated with minimal breast development and widely-spaced nipples most likely has Turner's syndrome. Turner's syndrome is caused by a chromosomal abnormality of an XO karyotype
Turner's syndrome is caused by an XO karyotype, in which only one sex chromosome is inherited: 45XO.
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Complications from Turner's syndrome:
Patients with Turner's syndrome have an increased risk of cardiovascular disease, and specifically increased risks of aortic stenosis (from bicuspid valve) and aortic dissection (from coarctation of the aorta)
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Diagnosis of intussusception investigation choice:
Ultrasound is the investigation of choice for intussusception
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Blood stained stool (red currant jelly) is a late sign for:
Intussusception
Bilious vomitting
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Management for Intussusception:
Management
the majority of children can be treated with reduction by air insufflation under radiological control, which is now widely used first-line compared to the traditional barium enema
if this fails, or the child has signs of peritonitis, surgery is performed
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Intussusception describes the invagination of one portion of bowel into the lumen of the adjacent bowel, most commonly around
the ileo-caecal region.
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during paroxysm the infant will characteristically draw their knees up and turn pale with vomiting called:
Intussusception
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Meconium aspiration syndrome refers to respiratory distress in the newborn as a result of meconium in the trachea. It occurs in the immediate neonatal period. It is more common in
post-term deliveries, with rates of up to 44% reported in babies born after 42 weeks. It causes respiratory distress, which can be severe. Higher rates occur where there is a history of maternal hypertension, pre-eclampsia, chorioamnionitis, smoking or substance abuse
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What acute medication/medications should he be prescribed on discharge for a child who's had an asthma attack?
salbutamol inhaler + 3 days prednisolone PO
this acute presentation combined with interval symptoms (exertional breathlessness and nighttime cough) suggests that this patient has suffered an acute exacerbation of asthma.
For this, a salbutamol inhaler + 3 days prednisolone PO are the correct acute medications to prescribe on discharge.
For all children suffering an acute exacerbation of asthma 3-5 days of oral prednisolone should be given.
It is important to ensure that all patients have an adequate supply of their salbutamol inhaler with advice on when and how to use it.
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Beclomethasone
Beclomethasone may be a useful medication for the longer-term prophylactic management of this child's asthma but it is not used in short courses (e.g. 7 days) after acute exacerbations.
Similarly, oral steroids should be given to all children with acute asthma irrespective of whether they are usually on an inhaled corticosteroid.
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Kawasaki disease features:
Typical findings, as seen in this case, include an acute febrile illness lasting over 5 days, bilateral non-purulent conjunctivitis, unilateral cervical lymphadenopathy, a polymorphic rash, and mucosal erythema with a strawberry tongue.
Swelling of the hands and feet can occur in the acute stage with desquamation in the second week.
Coronary aneurysms can develop in up to one-quarter of untreated patients.
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Kawasaki disease treatment:
The standard treatment in the UK is intravenous immunoglobulin and high dose aspirin (despite the fact that it is usually contraindicated in children) due to the additional anti-inflammatory effects this provides
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Kawasaki disease is a systemic vasculitis that generally presents in
the under 5's
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attention deficit hyperactivity disorder (ADHD). Studies have shown that in patients with ADHD, there is reduced function of the
Frontal lobe
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ADHD is managed with behavioural techniques and stimulant medicines such as
methylphenidate. These medicines increase function of the frontal lobe to increase executive function to increase attention and reduce impulsivity.
Methylphenidate is first line in children and should initially be given on a six-week trial basis. It is a CNS stimulant which primarily acts as a dopamine/norepinephrine reuptake inhibitor. Side-effects include abdominal pain, nausea and dyspepsia. In children, weight and height should be monitored every 6 months
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Hydrocephalus
This is an important diagnosis to investigate in macrocephaly. There can be potentially life-threatening consequences of missing this. This is a condition in which there is an accumulation of cerebrospinal fluid in the brain. Acute causes...
include meningitis, traumatic brain injury and haemorrhage (intra-ventricular or sub-arachnoid). In this case, meningitis is hinted at in this question (febrile, poor feeding)
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Macrocephaly is defined as
OFC ≥98th percentile
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Plagiocephaly is a change in shape of a baby’s head (flatter at the back) due to babies
sleeping on their back
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To consider hydrocephalus, what's to consider?
age of the baby
pre-term risk factor
acute presentation
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Magnesium sulphate can be given to mothers with pre-eclampsia to reduce the likelihood of
eclamptic seizures
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This preterm baby with signs of respiratory distress including
cyanosis, grunting, tachypnoea, nasal flaring and subcostal recessions with a ground glass appearance on chest x-ray has neonatal respiratory distress syndrome
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If preterm delivery is suspected, maternal intramuscular..........can help to boost foetal surfactant production and thus reduce the likelihood of developing neonatal respiratory distress syndrome.
steroid injection, such as dexamethasone,
(Surfactant is made from around 26 weeks gestation, although adequate levels are not achieved until about 35 weeks. This means that premature babies do not have enough surfactant)
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Treatment of neonatal respiratory distress syndrome is with
intratracheal instillation of artificial surfactant
Additionally, if preterm delivery is suspected, giving the mother glucocorticoids before delivery can increase surfactant production in the baby. Glucocorticoids bind to nuclear steroid receptors to trigger surfactant synthesis
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Mother history of bipolar, baby born with large right atrium and small right ventricle, usually due to low insertion of the tricuspid valve, which also causes tricuspid incompetence?
Ebstein's anomaly
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Ventricular septal defect (VSD) present with a
blowing pansystolic murmur (+/- thrill), and on echocardiogram, a shunt would be visible between the two ventricles.
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Atrial septal defect (ASD) would present with
a systolic murmur and a split S2. It is also not associated with bipolar disorder
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Coarctation of the aorta
his is narrowing of the aorta and would present with an ejection systolic murmur, radio-femoral delay, and absent or weak femoral pulses
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Transposition of the great arteries (TGA) usually presents shortly after birth
with cyanosis, and occurs when the pulmonary artery originates from the left ventricle, and the aorta from the right ventricle.
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Coeliac disease (paediatrics), Serological markers include:
Anti-tissue transglutaminase
Anti-endomysial autoantibodies
Antigliadin autoantibodies
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untreated coeliac disease is associated with an increased risk of
lymphoma
(Long-standing history of loose foul-smelling stools, abdominal pain- particularly after eating bread), a background of autoimmune disease (type 1 diabetes) and family history of food intolerance, and signs of anaemia and abdominal distention most likely has a diagnosis of coeliac disease
If not properly managed with a gluten-free diet long-term, there is an increased risk of lymphomas (such as EATL - enteropathy associated T-cell lymphoma) and small bowel adenocarcinomas
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nitrogen washout test (also known as the hyperoxia test) may be used to differentiate....
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Down's syndrome which can be associated with sleep apnoea and snoring. This is due to...
- the low muscle tone in the upper airways and large tongue/adenoids
- There is also an increased risk of obesity which in people with Down's syndrome which is another predisposing factor to snoring
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Snoring in children...
Causes
- obesity
- nasal problems: polyps, deviated septum, hypertrophic nasal turbinates
- recurrent tonsillitis
- Down's syndrome
- hypothyroidism
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......... is one of the most common childhood malignancies. It typically presents in children under 5 years of age, with a median age of 3 years old.
Wilms' nephroblastoma
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Meningitis in children < 3 months give?
IV amoxicillin in addition to cefotaxime to cover for Listeria
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For patients with meningococcal septicaemia, what is contraindicated?
For patients with meningococcal septicaemia a lumbar puncture is contraindicated - blood cultures and PCR for meningococcus should be obtained
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Initial empirical therapy for meningitis if more than 3 months of age,
....give IV 3rd generation cephalosporin
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Which are considered to be red flags?
- reduced skin turgor
- age <3 months with temperature >=38°C
- Respiratory rate >60 breaths/minute is a red flag symptom
- Heart rate >160 beats/minute, age <12 months
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Perform the following investigations in infants younger than 3 months with fever:
- Full blood count
- Blood culture
- C-reactive protein
- Urine testing for urinary tract infection
- Chest radiograph only if respiratory signs are present
- Stool culture, if diarrhoea is present
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NSAIDs can increase the risk of ....... in patients with chicken pox
necrotising fasciitis
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A boy noted to have Hypotonia,
| Hypogonadism, Obesity?
Prader-willi syndrome
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Acute epiglottitis is caused by
Haemophilus influenzae type B
- x-ray will show a thumb sign
endotracheal intubation may be necessary to protect the airway
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What are the signs and symptoms of necrotising enterocolitis?
And what investigation?
This is of the leading causes of death among premature infants.
It presents with abdominal distension, feeding intolerance and bloody stool.
The child can suffer from vomit bouts that are usually bilious
Abdominal x-ray will show dilated bowel loops, pneumatosis intestinalis (intramural gas) and occasionally portal venous gas.
The treatment is with total gut rest and total parenteral nutrition, babies with perforations will require laparotomy.
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What is the choice of investigation for intussusception?
Abdominal ultrasound
- This condition is characterised by paroxysmal abdominal colic pain and red currant jelly stool
Rmb the difference between necrotising enterocolitis, is the child will have abdo distention, feeding intolerance and bloody stool, vomit bouts usually bilious (abdo x-ray)
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Pyloric stenosis can be diagnosed via a
test feed
This condition is characterised by projectile vomiting, typically 30 minutes after a feed.
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What is the investigation of choice in evident cases of perforation? Patient has a fever with other gasto issues
Necrotising enterocolitis can eventually lead to perforation, so the child should be treated immediately to avoid so
Laparotomy is the investigation of choice
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An upper gastrointestinal tract contrast study is used to diagnose malrotation, what are the symptoms
This condition features exomphalos, congenital diaphragmatic hernia, intrinsic duodenal atresia. This child was born healthy making this diagnosis unlikely.
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What is the hallmark feature on the AXR for necrotising enterocolitis?
Initial symptoms can include feeding intolerance, abdominal distension and bloody stools, which can quickly progress to abdominal discolouration, perforation and peritonitis.
pneumatosis intestinalis (intramural gas)
and
dilated bowel loops (often asymmetrical in distribution)
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What is rigler sign on the axr?
air both inside and outside of the bowel wall (Rigler sign)
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Evidence of bowel sounds in a respiratory exam of a neonate in respiratory distress should make you consider...
a diaphragmatic hernia
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SDLD (also known as respiratory distress syndrome) occurs in premature births before ........... as there is not enough surfactant production due to the immaturity of the lungs.
31 weeks gestation
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SDLD (also known as respiratory distress syndrome) occurs in premature births before ........... as there is not enough surfactant production due to the immaturity of the lungs.
31 weeks gestation
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The most common cause of respiratory distress in the newborn period and when is most common?
Transient tachpneoa of the newborn
TTNB is the most common cause of respiratory distress in the newborn period and is caused by delayed reabsorption of fluid from the lungs.
It is more common following caesarean sections. A congenital diaphragmatic hernia is more likely in this case as bowel sounds can be heard during the respiratory examination.
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What is a congenital heart disease that can cause respiratory distress in the newborn period?
Tetraology of fallot
It is causes the newborn to be cyanotic, which is not the case in this patient.
