HbA1c stands for glycated haemoglobin. In patients with haemolytic diseases associated with a shortened RBC survival, HbA1c levels can be unusually low despite controlled diabetes mellitus.These haemolytic diseases can include glucose-6-phosphate dehydrogenase deficiency and sickle cell anaemia.HbA1c:It is a measure of non-enzymatic glycation which occurs due to haemoglobin’s exposure to plasma glucose. As plasma glucose increases so does HbA1c.The level of HbA1c quantitively assesses the control of diabetes mellitus over the last 120 days (as this is the life span of a red blood cell).Haemoglobin type A is separated on cation exchange chromatographyOther subsections include HbA1O, HbA1a, HbA1b.According to the updated NICE recommendations (2015):The target level of HbA1c in children with T1DM is 48 mmol/mol (6.5%) or lower. It is monitored every three months. Better control is associated with fewer long term complications

Acute epiglottitis is a life-threatening disorder with serious implications to the anaesthesiologist because of the potential for laryngospasm and irrevocable loss of the airway. There is inflammatory oedema of the arytenoids, aryepiglottic folds and the epiglottis; therefore, supraglottitis may be used instead or preferred to the term acute epiglottitis.Acute epiglottitis can occur at any age. The responsible organism used to be Hemophilus influenzae type B (Hib), but infection with group A b-haemolytic Streptococci has become more frequent after the widespread use of Hemophilus influenzae vaccination. The typical presentation in epiglottitis includes acute occurrence of high fever, severe sore throat and difficulty in swallowing with the sitting up and leaning forward position in order to enhance airflow. There is usually drooling because of difficulty and pain on swallowing. Acute epiglottitis usually leads to generalized toxaemia. The most common differential diagnosis is croup and a foreign body in the airway. A late referral to an acute care setting with its serious consequences may result from difficulty in differentiation between acute epiglottitis and less urgent causes of a sore throat, shortness of breath and dysphagia.

Based on the clinical scenario, the boy is going through normal pubertal changes.In the 3 years before puberty, low pulsatile LH levels become detectable during sleep. LH and FSH are produced in the anterior pituitary and released due to pulsatile gonadotrophin-releasing hormone (GnRH) secreted by the hypothalamus. There is an increase in the amplitude and frequency of LH secretion as puberty approaches, which causes enlargement of the gonads. In boys, the testicles produce testosterone, and in girls, the ovaries produce oestradiol and ovarian androgens, which, with the adrenal androgens, produce secondary sexual characteristics.Note:The average age at onset of puberty is 11 years in girls. The first sign is breast bud development, followed by the appearance of pubic hair 6-12 months later. Menarche usually occurs 2-2.5 years after breast bud development. Peak height velocity in girls occurs at breast stage 2-3 and virtually always precedes menarche. The onset of puberty in boys is at 11.5 years. The first sign is testicular enlargement (>3 ml) and thinning of the scrotum. This is followed by the pigmentation of the scrotum and growth of the penis, and pubic hair follows. Peak height velocity (growth spurt) is two years later in boys than in girls and occurs at testicular stage 4-5 (i.e. testicular volume 10-12 ml), which is around 13-14 years of age. Breast enlargement occurs in 40-60% of boys (rarely, significant enough to cause social embarrassment in 10%) and is a result of oestradiol produced by the metabolism of testosterone. It usually resolves within three years. During puberty, elongation of the eye often occurs, causing short-sightedness.

Ebstein’s anomaly is characterised by apical displacement and adherence of the septal and posterior leaflets of the tricuspid valve to the underlying myocardium, thereby displacing the functional tricuspid orifice apically and dividing the right ventricle into two portions. The main haemodynamic abnormality leading to symptoms is tricuspid valve incompetence. The clinical spectrum is broad; patients may be asymptomatic or experience right-sided heart failure, cyanosis, arrhythmias and sudden cardiac death (SCD). Many Ebstein’s anomaly patients have an interatrial communication (secundum atrial septal defect (ASD II) or patent foramen ovale). Other structural anomalies may also be present, including a bicuspid aortic valve (BAV), ventricular septal defect (VSD), and pulmonary stenosis. The morphology of the tricuspid valve in Ebstein anomaly, and consequently the clinical presentation, is highly variable. The tricuspid valve leaflets demonstrate variable degrees of failed delamination (separation of the valve tissue from the myocardium) with fibrous attachments to the right ventricular endocardium.The displacement of annular attachments of septal and posterior (inferior) leaflets into the right ventricle toward the apex and right ventricular outflow tract is the hallmark finding of Ebstein anomaly.

Meckel’s diverticulum is a true diverticulum and is the most common congenital anomaly of the GI tract, occurring in about 2% of people. It is caused by incomplete obliteration of the vitelline duct and consists of a congenital sacculation of the antimesenteric border of the ileum. Symptoms are uncommon but include lower GI bleeding, bowel obstruction, and inflammation (diverticulitis). Causes of upper GI bleeding in neonates:Swallowed maternal bloodCoagulopathies including haemorrhagic disease of the new-bornStress gastritis & stress ulcersDrugs (NSAIDs, heparin, indomethacin used for patent duct closure, dexamethasone)Causes of upper GI bleeding in children 1 month to 1 year:GORDGastritis (with or without Helicobacter pylori infection)Crohn diseaseDrugs (NSAIDs)Zollinger-Ellison syndromeCauses of upper GI bleeding in children 1 -2 years:Peptic ulcer diseaseZollinger-Ellison syndromeBurns (Curling ulcer)Head trauma (Cushing ulcer)SepsisCauses of upper GI bleeding in older than 2 years:Oesophageal varices secondary to portal hypertension (most commonly portal vein thrombosis and biliary atresia)Duodenal ulcersOesophagitisGastritisMallory-Weiss tears

Answer: Acute Epstein Barr virus infectionThe Epstein-Barr virus is one of eight known human herpesvirus types in the herpes family, and is one of the most common viruses in humans. Infection with Epstein-Barr virus (EBV) is common and usually occurs in childhood or early adulthood.EBV is the cause of infectious mononucleosis, an illness associated with symptoms and signs like:fever,fatigue,swollen tonsils,headache, andsweats,sore throat,swollen lymph nodes in the neck, andsometimes an enlarged spleen.Although EBV can cause mononucleosis, not everyone infected with the virus will get mononucleosis. White blood cells called B cells are the primary targets of EBV infection.

Ventilatory rates of 40-60 breaths/min should be provided initially, with proportionally fewer assisted breaths provided if the infant’s spontaneous respiratory efforts increase. Although this practice has not been extensively studied, initial inflation of the new-born’s lungs with either slow-rise or square-wave inflation to a pressure of 30-40 cm H2 O for approximately 5 seconds has been reported to result in more rapid formation of Functional Residual Capacity (FRC).At the moment of delivery and first breath, the neonatal lung is converting from a fetal non-aerated status to a neonatal status. The neonatal lung requires gas exchange, and this necessitates the development of FRC with the resorption of lung fluid and the resolution of most of the atelectasis. Therefore, initial slow ventilation with more prolonged inspiratory times may be useful to assist in this task, balanced against the need to avoid inappropriate inspiratory pressures.Flow-controlled, pressure-limited mechanical devices are acceptable for delivering PPV. These mechanical devices control flow and limit pressure and be more consistent than bags. Self-inflating and flow-inflating bags remain a standard of care. Laryngeal mask airways are effective for assisted ventilation when bag-mask ventilation and intubation are unsuccessful.

Spontaneous intestinal perforation (SIP) of the new-born, also referred to as isolated perforation or focal intestinal perforation (FIP) of the new-born, is a single intestinal perforation that is typically found at the terminal ileum. SIP occurs primarily in preterm infants with very low birth weight (VLBW, birth weight <1500 g) and extremely low birth weight (ELBW, birth weight <1000 g), umbilical catheter placement, maternal drug use and twin-to-twin transfusion syndrome. SIP is a separate clinical entity from necrotizing enterocolitis, the most severe gastrointestinal complication of preterm infants.

Tinea corporis is a type of dermatophytosis, caused by Trichophyton or Microsporum. The disease is highly contagious and rapidly spreads to all the areas of the body, including the glabrous skin. It produces an erythematous itchy skin rash with a central area of clearance surrounded by raised scaly borders. Lesions can appear as concentric circles that overlap, referred to as tinea imbricate.

The child most likely has nephritic syndrome which is characterised by a reduced renal function, proteinuria of non-nephrotic range, haematuria, erythrocyte casts, and oedema. Polyarteritis nodosa usually occurs in middle-aged men but can also be seen in young children. It is accompanied by severe systemic manifestations such as fever, malaise, weight loss and myalgia.Renal cell carcinoma usually presents around 55 years with the classic triad of haematuria, loin pain and a unilateral mass in the flank.Polycystic kidney disease usually presents in adult life with acute loin pain and palpation of masses in the flanks.

The next step in the management of this patient is starting a normal saline fluid bolus, following which we should send samples for blood culture, lactate and C-reactive protein. The patient is probably a case of anorexia nervosa (AN).Note:Children with AN are at higher risk of developing infections, and this needs to be considered in this case. Patients with AN may not respond in the usual fashion to sepsis as they may not mount a fever in response to infection. Hypoglycaemia (especially when severe or persistent despite correction) should raise the alarm for an infection as should tachycardia and cool peripheries. Children with severe AN are more likely to be bradycardic. Therefore, a tachycardia should cause the clinician to consider that another process is going on.Other options:- Correcting electrolyte abnormalities is, of course, very important. However, while these are awaited, one needs to consider that this patient is septic and managed accordingly. Children with anorexia nervosa (AN) may have a whole host of electrolyte abnormalities, some of which can be treated with oral or nutritional supplementation although sometimes intravenous correction is required. Electrolytes need to be checked frequently during admission because of the risk of refeeding syndrome, and dietetic input is required to advise on appropriate starting intake.- While maintenance fluids like dextrose are likely to be needed in this case, consideration needs to be given to the underlying cause of the hypoglycaemia. As the sugar has come up following a glucose bolus, a 10% dextrose infusion would be excessive.- An ECG should form a part of any assessment of a child with AN. In this case, it would not be the first thing; however, as part of the acute management of this child, cardiac monitoring should be commenced. One would usually expect a child with AN to be bradycardic so a tachycardia should raise suspicion that another process is going on.- Dietary input will be vital for this child’s ongoing management. However, their acute issues take precedence in this case. Nevertheless, a dietician should be informed and involved from an early stage.

Blood culture and sensitivity will distinguish the bacteria that is responsible for the infection and the effective antibiotic treatment to which the bacteria is sensitive.

Unconjugated bilirubin is conjugated to glucuronic acid in the hepatocyte. Conjugated bilirubin passes into the enterohepatic circulation and the bilirubin which evades this system is metabolised by bacteria, primarily in the large intestine, to urobilinogen, then stercobilinogen and eventually oxidised to stercobilin. Stercobilin gives faeces its brown colour.

Most experts suggest that parents reassurance in case of infantile gastro-oesophageal reflux (GOR) is a sufficient initial measure that involves education about regurgitation and lifestyle changes.

A three-year child’s typical developmental milestones include walking up and down the stairs with alternating feet, jumping in a place with two feet together, and jumping forward for about 2 feet. The child can pedal a tricycle and can throw a ball over and underhand. An average child should start walking around 12-14 months of age and should be investigated as early as 18 months if they do not begin to walk. Skipping can be done by a child of 5 years of age. A child of 6-7 years of age can ride a bike with stabilizers and can balance on one foot for 20 seconds.

Deficiency of 21-hydroxylase, resulting from mutations or deletions of CYP21A, is the most common form of Congenital Adrenal Hyperplasia, accounting for more than 90% of cases.Females with mild 21-hydroxylase deficiency are identified later in childhood because of precocious pubic hair, clitoromegaly, or both, often accompanied by accelerated growth and skeletal maturation (simple virilizing adrenal hyperplasia)Diagnosis of 21-hydroxylase deficiency: High serum concentration of 17-hydroxyprogesterone (usually >1000 ng/dL) and urinary pregnanetriol (metabolite of 17-hydroxyprogesterone) in the presence of clinical features suggestive of the disease; 24-hour urinary 17-ketosteroid levels are elevated

Polycythaemia is a commonly occurring neonatal disorder, which is characterized by a venous haematocrit ≥ 65%. It increases the blood viscosity and causes microcirculatory flow impairment in the end-organs. Causes of polycythaemia are multi-factorial, but the significant risk factors are maternal diabetes, SGA, LGA or post-term birth, infants with chromosomal abnormalities, and delayed clamping of the cord. Jaundice is not a recognized risk factor for polycythaemia.

One-third of patients with Guillain-Barre syndrome suffer from diaphragm weakness which can lead to further respiratory complications if there is involvement of the tongue, palate, and neck muscles. Forced vital capacity (FVC) is the best way to monitor respiratory muscle function by assessing it repeatedly. Admission for ITU is suggested when FVC is below 20ml/kg and intubation recommended when FVC is 15ml/kg or below. FVC is used in any neurological disorders wherein the respiratory muscles are affected.

Based on the clinical scenario, the most likely nerve injured in this case is the ulnar nerve.Ulnar Nerve:It arises from the medial cord of brachial plexus (C8, T1).It supplies motor fibres to the following structures:- Medial two lumbricals- Adductor pollicis- Interossei- Hypothenar muscles: abductor digiti minimi, flexor digiti minimiflexor carpi ulnarisIt carries sensation from the palmar and dorsal aspects of the medial 1 1/2 fingers.Patterns of damageIf the nerve is damaged at the wrist, the following features are observed:- Claw hand’: Hyperextension of the metacarpophalangeal joints and flexion at the distal and proximal interphalangeal joints of the 4th and 5th digits.- Wasting and paralysis of intrinsic hand muscles (except lateral two lumbricals)- Wasting and paralysis of hypothenar muscles- A sensory loss in the medial 1 1/2 fingers (palmar and dorsal aspects)If the nerve is damaged at the elbow or above the ulnar paradox is observed – the fourth and fifth fingers are simply paralyzed and claw hand is less severe.

The hypothalamus and pituitary gland control how much testosterone the testes produce and secrete. The hypothalamus sends a signal to the pituitary gland to release gonadotrophic substances (follicle stimulating hormone and luteinizing hormone). Luteinizing hormone (LH) stimulates testosterone production.

Constipation is often associated with nocturnal enuresis in children. The palpable mass is the impacted stool. The decreased appetite is common in those with constipation.

Omega-shaped epiglottis is a characteristic feature in the X-ray of a patient with laryngomalacia.

Blalock-Thomas-Taussig shunt is a surgical procedure done to increase pulmonary blood flow in cases like pulmonary atresia and results in a continuous murmur.The type of murmur which is heard during the systole and remains continuous till the second heart sound is known as a continuous murmur. This murmur is often intense and considered rough. It can also be accompanied by quivering. There are numerous causes of a continuous murmur that differ depending on the location and components of this murmur. It is caused by the shunting of blood from a high-pressure circulation to a low-pressure circulation. Pathological causes of a continuous murmur include patent ductus arteriosus, aortopulmonary window, AV malformation, coarctation of aorta, any acquired trauma, and iatrogenic cause like interventional or surgical procedures.

All the given development milestones are compatible with the given age.

Among the given options, pituitary adenoma is an acquired anterior pituitary disorder which represent between 10 and 25% of all intracranial neoplasms.Other options:- Agenesis of the corpus callosum is characterised by the partial or complete absence of the corpus callosum. This condition is associated with pituitary hormone deficiencies.- Congenital disorders such as hydrocephalus or arachnoid cysts may be a cause of precocious puberty.- Pituitary hypoplasia and septo-optic dysplasia are congenital disorders of the pituitary gland.

The characteristic cells in granulomatous inflammation are giant cells, formed from merging macrophages and epithelioid cells elongated with granular eosinophilic cytoplasm. Granulomatous reactions are seen in patients with tuberculosis. A tuberculous/caseating granuloma is characterised by a zone of central necrosis lined with giant multinucleated giant cells (Langhans cells) and surrounded by epithelioid cells, lymphocytes and fibroblasts. The caseous zone is present due to the damaged and dead giant cells and epithelioid cells. Mast cells are only few in number and fibroblasts lay down collagen. Basophils are not present. The giant cell made up of macrophages are the most abundant cells in this inflammatory process.

Transfusion with blood products carries a risk of acute and more chronic adverse reactions. These reactions can either be immune mediated due to a component mismatch, or non immune, underpinned by bacterial or viral contamination. Reactions range from very mild such as urticaria, to life threatening in the case of transfusion-related acute lung injury. In transfusion with packed red blood cells, the most common adverse event is pyrexia, while urticaria is the most common adverse event that follows infusion with FFP.

In the final result of this double blind study, 25% or one in four of patients who received the treatment responded positively. Though the difference is significant at P<0.05, it is better to present this as a significant difference at 0.4%. The number needed to treat (NNT) is defined as the inverse of the absolute risk reduction. Taking this as 0.25 (25%), the NNT is 1/0.25 = 4. However given the 95% confidence interval is between 10-50% for absolute risk reduction, the NNT with 95% CI is between 2 and 10. Therefore we cannot say with certainty that the NNT is at least 4.

Transient tachypnoea of the new-born is a condition associated with the delayed clearance of amniotic fluid from the new-born. The X-ray findings are typical of this condition. As the name implies and was noted in this patient, it is not a lasting condition and resolves within 24-72 hours after birth. The differential diagnoses usually present with different chest X-ray findings:- Aspiration pneumonia shows infiltrates in the lower lobes of the lungs- Congenitally acquired pneumonia shows patchy, asymmetrical densities- Meconium aspiration shows hyperinflation and patchy asymmetric airspace disease- Pulmonary oedema shows cephalization of pulmonary veins and indistinctness of the vascular margins.

Due to breakdown of the red blood cell membrane in haemophilic patients, cholesterol crystals are formed by the lipids. On the other hand lipofuscin deposition does not occur in haemolysis or haemorrhage. Neutrophil accumulation suggests acute inflammation. Anthracotic pigment is an exogenous carbon pigment that deposits in the lung from dust. Russell bodies are intracellular accumulations of immunoglobins in plasma cells. Curschmann’s spirals and Charcot Leyden crystals are pathognomonic of asthma.