Normal QT interval in a 6 months old baby is < 0.44 seconds. A duration exceeding this value is referred to as QT interval prolongation, which is associated with some important congenital syndromes that include Brugada syndrome which is characterized by vasovagal syncope and sudden cardiac death, Jervell and Lange-Nielsen syndrome which is associated with congenital deafness, Andersen syndrome which is marked by bone deformities, and Timothy syndrome with associated heart disease and immunodeficiency. Romano-Ward syndrome is the commonest cause of congenital QT prolongation with no associated extra-cardiac manifestations. Lown-Ganong-Levine syndrome is characterized by a shortened PR interval and a normal QRS duration.

A peripheral blood film in iron deficiency anaemia can reveal the following morphologically variant RBCs:- Microcytic cells- Hypochromic cells- Pencil cells- Target cellsOther options:- Schistocytes can be caused by mechanical heart valves. – Rouleaux can be seen in chronic liver disease and malignant lymphoma. – Tear-drop poikilocytes can be seen in myelofibrosis. – Acanthocytes can be seen in liver disease and McLeod blood group phenotype.Pathological red cell forms include:- Target cells: Sickle-cell/thalassaemia, iron-deficiency anaemia, hyposplenism, and liver disease.- Tear-drop poikilocytes: Myelofibrosis- Spherocytes: Hereditary spherocytosis and autoimmune haemolytic anaemia- Basophilic stippling: Lead poisoning, thalassaemia, sideroblastic anaemia, and myelodysplasia- Howell-Jolly bodies: Hyposplenism- Heinz bodies: G6PD deficiency and alpha-thalassaemiaSchistocytes (‘helmet cells’): Intravascular haemolysis, mechanical heart valve, and disseminated intravascular coagulation- Pencil poikilocytes: Iron deficiency anaemia- Burr cells (echinocytes): Uraemia and pyruvate kinase deficiency – Acanthocytes: Abetalipoproteinemia

Compared to the rest of the options, Meckel’s diverticulum with ectopic gastric mucosa seems to be the most probable diagnosis, as it can lead to fresh bleeding.Fresh red bleeding can be caused by haemorrhoids, polyps or a massive GI bleed.

The level of statistical significance is often expressed as a p-value between 0 and 1. The smaller the p-value, the stronger the evidence that you should reject the null hypothesis.A p-value less than 0.05 (typically ≤ 0.05) is statistically significant. It indicates strong evidence against the null hypothesis, as there is less than a 5% probability the null is correct (and the results are random). Therefore, we reject the null hypothesis, and accept the alternative hypothesis.However, this does not mean that there is a 95% probability that the research hypothesis is true. The p-value is conditional upon the null hypothesis being true is unrelated to the truth or falsity of the research hypothesis.A lower p-value is sometimes interpreted as meaning there is a stronger relationship between two variables. However, statistical significance means that it is unlikely that the null hypothesis is true (less than 5%).To understand the strength of the difference between two groups (control vs. experimental) a researcher needs to calculate the effect size.

Cataracts in infants are most commonly identified by an abnormal red reflex. Due to the high risk of amblyopia in unilateral cataracts, prompt referral to a paediatric ophthalmologist is indicated. Bilateral cataracts may occur in association with several syndromes or diseases, and these children require evaluation for these systemic disorders.Retinoblastoma is rare, but it is the most common primary intraocular tumour in children. About 80% of cases are diagnosed before age 4, with a median age at diagnosis of 2 years. It most frequently presents due to an abnormal red reflex. It is one of the few life-threatening disorders encountered in paediatric ophthalmology. Intraocular retinoblastoma is very treatable, but the mortality for metastatic disease is high. Identification of tumours before systemic spread is critical. Most children with large unilateral tumours will require enucleation (surgical removal of the eye), but the eye and vision may sometimes be preserved if the tumours are identified when they are small.

Guidelines suggest that when hypothermia manifests in the context of a shockable rhythm, it might get harder to defibrillate. Therefore, defibrillation is recommended to occur at temperatures below 30°C, and to be limited in three shocks.

Pyloric stenosis affects infants, typically in the second to fourth weeks of life and is caused most commonly by hypertrophy and thickening of the pylorus. It usually presents with projectile vomiting and failure to thrive. The infant usually has a normal appetite. Features include: ‘projectile’ vomiting, typically 30 minutes after a feed, constipation and dehydration may also be present and a palpable mass may be present in the upper abdomen.

Sepsis is a medical emergency that describes the body’s systemic immunological response to an infectious process that can lead to end-stage organ dysfunction and death. The fever, alongside the clinical presentation, make sepsis the likely diagnosis. A temperature >38 degrees in an infant <3months of age warrants investigation.

The thyroid gland is one of the first endocrine glands to develop, and is formed from the endoderm of the epithelial tissue of the primitive pharynx. The mature gland is functionally made up of follicles with colloid which contain the pro hormone thyroglobulin. Thyroid hormone is synthesised at a cellular level and is stored as thyroglobulin, before its release as thyroid hormone, a major modulator of metabolism. In between the follicular cells are parafollicular, or C-cells which secrete parathyroid hormone or calcitonin which are responsible for calcium regulation in the body.

The most probable diagnosis based on the clinical presentation is myocarditis secondary to Coxsackie virus infection.Myocarditis is an important cause of acquired heart failure. The other infective causes of myocarditis are influenza and adenoviruses, and bacterial causes as seen with Borrelia burgdorferi (Lyme disease). Rheumatic fever is unlikely if the ASO titres are within normal limits. While pancarditis may occur as part of Kawasaki disease; the patient is unlikely to present in failure.

This child has pseudo hypoparathyroidism. It is a heterogeneous group of rare endocrine disorders characterized by normal renal function and resistance to the action of parathyroid hormone (PTH), manifesting with hypocalcaemia, hyperphosphatemia, and increased serum concentration of PTH.Patients with pseudo hypoparathyroidism type 1a present with a characteristic phenotype collectively called Albright hereditary osteodystrophy (AHO). The constellation of findings includes the following:Short statureStocky habitusObesityDevelopmental delayRound faceDental hypoplasiaBrachymetacarpalsBrachymetatarsalsSoft tissue calcification/ossificationThe goals of therapy are to maintain serum total and ionized calcium levels within the reference range to avoid hypercalcaemia and to suppress PTH levels to normal. This is important because elevated PTH levels in patients with PHP can cause increased bone remodelling and lead to hyper-parathyroid bone disease.The goals of pharmacotherapy are to correct calcium deficiency, to prevent complications, and to reduce morbidity. Intravenous calcium is the initial treatment for all patients with severe symptomatic hypocalcaemia. Administration of oral calcium and 1alpha-hydroxylated vitamin D metabolites, such as calcitriol, remains the mainstay of treatment and should be initiated in every patient with a diagnosis of pseudo hypoparathyroidism.

Vitamin A is a fat-soluble compound occurring in various forms. The RDA for 0-12 months old babies is 400-600micrograms/day. Most of the vitamin A is stored in the liver, making animal liver an excellent source of vitamin A. Vitamin A deficiency is characterized by the deposits of keratin in the conjunctiva, known as Bitot’s spots, keratomalacia, night blindness, and anaemia.Vitamin A toxicity or excess is characterized by hyperkeratosis, hypothyroidism, hypo/hyperpigmentation, etc.According to WHO guidelines, children under two years of age who develop measles should be given two additional doses of vitamin A supplementation 24 hours apart, which is necessary for preventing eye damage, blindness, and reducing mortality by 50%.

Turner syndrome (TS) is one of the most common genetic disorders; occurs with an incidence of I: 2,500 female live births. It results from complete or partial chromosome X monosomy. TS is associated with abnormalities of the X chromosome and characteristic clinical features of short stature, gonadal dysgenesis, sexual developmental deficiencies, cardiac and/or renal defects, webbed neck, low-set ears, skeletal deformities including cubitus valgus, a propensity to ear infections and hearing deficits.

Milestones of 6 monthsSocial and EmotionalKnows familiar faces and begins to know if someone is a stranger Likes to play with others, especially parents Responds to other people’s emotions and often seems happy Likes to look at self in a mirror Language/CommunicationResponds to sounds by making sounds Strings vowels together when babbling (ah,ť eh,ť ohť) and likes taking turns with the parent while making sounds Responds to own name Makes sounds to show joy and displeasure Begins to say consonant sounds (jabbering with m,ť bť) Cognitive (learning, thinking, problem-solving)Looks around at things nearby Brings things to mouth Shows curiosity about things and tries to get things that are out of reach Begins to pass things from one hand to the other Movement/Physical DevelopmentRolls over in both directions (front to back, back to front) Begins to sit without support When standing, supports weight on legs and might bounce Rocks back and forth, sometimes crawling backwards before moving forward

Umbilical vein catheterization utilizes the exposed umbilical stump in a neonate as a site for emergency central venous access up to 14 days old. Umbilical vein catheterization can provide a safe and effective route for intravenous delivery of medications and fluids during resuscitation.The indication for umbilical vein catheterization is when there is a need for IV access in a neonate for resuscitation, transfusions, or short-term venous access when otherwise unobtainable.

Common neurological symptoms of Wilson disease that may appear and progress with time include tremor, involuntary movements, difficulty swallowing (dysphagia), difficulty speaking and poor articulation (dysarthria), lack of coordination, spasticity, dystonic postures, and muscle rigidity.

The spinal cord ends at L3 in the new-bornmigrates cephalad during childhood to end at L1 – L2 and reaches adult size by the age of 10.

The most probable diagnosis for this patient would be torsion of a hydatid cyst of the testis.Note:The differential diagnoses in acute testicular pain/swelling are listed in the possible answers. Testicular torsion typically affects adolescent males and presents with acute painful swelling and a horizontally lying testicle. Other options:- Epididymo-orchitis is diagnosed during surgery when a thickened erythematous epididymis is noted. Ectopic ureteric insertion into the vas must be excluded by ultrasound scan on an out-patient basis. The hydatid cyst or cyst of Morgagni represents the remnants of the Mullerian structure. In the pre-pubertal child, a surge in hormones can stimulate growth and chance of torsion. It is diagnosed by surgical exploration or by a blue dot sign. Idiopathic scrotal oedema tends to be within the scrotum itself, and the testis can be examined in the groin to exclude pain in the testis. All acute scrotal pain must be explored to exclude testicular torsion unless a surgical registrar is convinced of a torted hydatid cyst.- An 8-year-old is more likely to present with a torted hydatid cyst. The blue dot sign is diagnostic and can negate the need for surgical exploration. The age of the child also makes epididymo-orchitis less likely.- Idiopathic scrotal oedema presents with an erythematous and thickened scrotum. This can cross the midline and involve the whole scrotum.- Finally, an inguinal hernia would present with a mass in the groin extending to the scrotum that you cannot get above.

The sensitivity of a screening test can be described in a variety of ways, typically such as sensitivity being the ability of a screening test to detect a true positive, being based on the true positive rate, reflecting a test’s ability to correctly identify all people who have a condition, or, if 100%, identifying all people with a condition of interest by those people testing positive on the test.The specificity of a test is defined in a variety of ways, typically such as specificity is the ability of a screening test to detect a true negative, being based on the true negative rate, correctly identifying people who do not have a condition, or, if 100%, identifying all patients who do not have the condition of interest by those people testing negative on the test.Sensitivity=[a/(a+c)]x100Specificity=[d/(b+d)]x100a: True positiveb: False Positivec: False negatived: True negative

For Otorhinolaryngologist, removal of foreign bodies (FB) from the ear, nose and throat is one of the common emergency procedures done. Most of the cases especially of the ear and nose can be managed without General Anaesthesia (GA). But in some cases GA may be needed. As the child is mentally unstable, he is unlikely to be able to hold still while the foreign body (pea) is being removed with a forceps, which could result in instrumental damage to the ear canal. Pea is an organic foreign body and not metallic hence the use of magnets would not be of any help either. From the options provided, general anaesthesia would be the best option in this child.

The most probable cause for the patient’s presentation would be intra-abdominal adhesions, and the most appropriate management for this patient would be nasogastric decompression and intravenous fluids.Malrotation typically presents in the first month of life with bilious vomiting. There is a lifetime risk of intra-abdominal adhesions. This presents with bilious vomiting and dilated bowel loops on plain abdominal film. 2/3 of adhesional obstructions resolve by conservative management with nasogastric decompression and intravenous fluids. If this fails to resolve after 24-48 hours or if there are signs of peritonism, a laparotomy is indicated.Other options:- Recurrent volvulus, urgent laparotomy required: The child is now 8-years-old and recurrent volvulus is unlikely as bowel should be fixed by intra-abdominal adhesions.- Intra-abdominal adhesions, surgery for division of intra-abdominal adhesions: Although surgery may be required, the majority of adhesional obstructive cases respond to conservative management.- Gastroenteritis with incompetent pylorus, intravenous fluids: While it is true that patients with malrotation can have bilious vomiting in gastroenteritis, the abdominal X-ray is suggestive of obstruction.- Likely sepsis with bilious vomiting, intravenous fluids and antibiotics: While a septic ileus can give bilious vomiting, this patient has no inflammatory markers suggestive of sepsis.

Psoriasis is a chronic inflammatory skin disease that is mainly due to the interplay between keratinocytes, dermal vascular cells, and antigen-presenting cells. There are five main types of psoriasis based on the type and severity of the lesions it produces. A useful tool to determine the extent and severity of psoriasis is the Psoriasis Area and Severity Index (PASI) score, which takes into account the percentage of a specific area affected by psoriasis in combination with severity, which in turn is assessed by the presence of desquamation, induration, and erythema.

Cluster headaches present with unilateral severe headache that may be associated with ipsilateral cranial autonomic symptoms including; lacrimation, rhinorrhoea, ophthalmic injection, and occasionally, Horner syndrome (ipsilateral miosis, ptosis, and facial anhidrosis).

The symptoms and signs described are suggestive of otitis externa. The key treatment for otitis externa is ear cleaning and antibiotic drops with steroid.

This patient has Wilson’s disease. They Kayser-Fleischer ring (ring that encircles the iris) is diagnostic of this. Low serum copper is seen in Wilson’s disease. With the Kayser-Fleischer ring, this makes all of the other answer choices incorrect.

Diagnostic criteria of PCOsAccording to the American Association of Clinical Endocrinologists, at least two of three of the criteria below are required for diagnosis of PCOS after excluding other causes of irregular bleeding and elevated androgen levels.Hyperandrogenism (clinical or laboratory)Oligo- and/or anovulationPolycystic ovaries on ultrasoundDiagnosis of PCOS is possible without the presence of ovarian cysts.Rule out any other causes of hyperandrogenism and anovulation.Blood hormone levels:Testosterone (both total and free) or free androgen indexLH (LH:FSH ratio > 2:1)Oestrogen is normal or slightly elevated A clinical picture of hyperandrogenism overrules any normal hormone levels and can fulfil a diagnostic criterium of PCOS.

Hirschsprung’s disease is characterized by congenital absence of the autonomic plexus (Meissner’s and Auerbach’s plexus) in the intestinal wall. Usually limited to the distal colon, it can occasionally involve the entire colon or even the small bowel. There is abnormal or absent peristalsis in the affected segment, resulting in continuous spasm of smooth muscle and partial/complete obstruction. This causes accumulation of intestinal contents and dilatation of proximal segment. Skip lesions are highly uncommon. This disease is seen early in life with 15% patients presenting in first month, 60% by 1 year of age and 85% by the age of 4 years. Symptoms include severe and complete constipation, abdominal distension and vomiting. Patients with involvement of ultra-short segments might have mild constipation with intervening diarrhoea. In older children, symptoms include failure to thrive, anorexia, and lack of an urge to defecate. On examination, an empty rectum is revealed with stool palpable high up in the colon. If not diagnosed in time, it can lead to Hirschsprung’s enterocolitis (toxic megacolon), which can be fulminant and lead to death. Diagnosis involves a barium enema or a rectal suction biopsy. Barium enema shows a transition in diameter between the dilated, normal colon proximal to the narrowed, affected distal segment. It is to be noted that barium enema should be done without prior preparation, which can dilate the abnormal segment, leading to a false-negative result. A 24-hour post-evacuation film can be obtained in the neonatal period -if the colon is still filled with barium, there is a high likelihood of Hirschsprung’s disease. Full-thickness rectal biopsy is diagnostic by showing the absence of ganglion cells. Acetylcholinesterase staining can be done to highlight the enlarged nerve trunks. Abnormal innervation can also be demonstrated by rectal manometry.

Klinefelter syndrome is a chromosomal disease that does not lead to congenital cataracts. A congenital cataract is the clouding of the cornea that is present from birth. Causes can be categorized as genetic, infectious, and metabolic. The most common infectious cause is congenital rubella syndrome. Galactosemia is a metabolic cause of congenital cataracts. Down’s syndrome represents a genetic cause of congenital cataract.

Age (years) Respiratory rate (per minute) Heart rate (per minute) Systolic blood pressure12 15-20 60-100 100-120Adapted from Advanced Paediatric Life Support Manual

The anti-B antibodies in a patient with blood group A belong to the IgM class of immunoglobulins.Note:IgM is the largest antibody formed of 5 antibodies attached together. This functions to agglutinate or clump antigens. The associated anti-A and anti-B antibodies are usually IgM produced in the first years of life by sensitisation to environmental substances such as food, bacteria, and viruses.Other options:- IgG is the most common antibody. It is a single antibody complex.- IgD is found on the surface of B-lymphocytes.- IgE is bound to tissue cells, especially mast cells and eosinophils.