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A 14-year-old female presents with a two week history of deteriorating puffiness of the face.

She has been aware of tiredness and lethargy over the last two months since having acquired a throat infection. Over this period she has gained at least 5 kg in weight over this time. She had a similar throat infection aged 10 from which she made an uneventful recovery. There is nothing else of note in the history and she takes no medication. Examination reveals generalised puffiness with pitting oedema of the lower limbs. Her blood pressure is 133/86 mmHg with a pulse of 88 bpm. Chest, cardiovascular and abdominal examinations are normal. Her urine dip stick produces +++ protein. Other investigations reveal: Haemoglobin White cell count Platelets Sodium Potassium Chloride Bicarbonate Urea Creatinine Serum albumin 24 hour urine protein 14.6g/dl 9 6 x 10 /l 9 250 x 10 /l 136 mmol/l 4.0 mmol/l 103 mmol/l 24 mmol/l 4.2 mmol/l 93 micromol/l 25 g/l 4.3 g/l

Which is the best treatment for this patient? Available marks are shown in brackets 1) Observe 2) Salt poor albumin 3) Intravenous frusemide 4) High dose corticosteroids 5) Cyclophosphamide Comments: This patient has the nephrotic syndrome as defined by a triad of hypoalbuminaemia, proteinuria > 3g/24hrs and oedema. The commonest cause of nephrotic syndrome in a child is minimal change disease, which usually responds to a course of high dose corticosteroids. Cyclophosphamide may hasten a remission, but given its cytotoxic profile, should be reserved for steroid-resistant cases. If possible it should be avoided in childhood. Salt poor albumin and iv frusemide may be useful adjuncts for managing oedema, but is not curative of proteinuria. If the nephrotic syndrome is left unchecked, complications include Streptococcal sepsis, venous thromboembolism and hypercholesterolaemia A 6 year old girl with global developmental delay presents with fever and gross haematuria, with visible pink urine and clots. Full term normal delivery with no neonatal complications. Diagnosed with developmental delay at 9 months, and receives special schooling. Immunisations up to date. There is no family or social history of note. On examination she has a temperature of 38.9C, respirattory rate of 18/min and heart rate of 90/min. She has suprapubic tenderness. Urine dipstix testing shows 4+ haematuria, 3+ proteinuria and 3+ nitrites. What is the most likely diagnosis?

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Available marks are shown in brackets Benign familial haematuria 1) 2) Glomerulonephritis 3) Haemorrhagic cystitis 4) Henoch-Schonlein Disease 5) Urinary tract infection

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Comments: The history of fever, gross haematuria with clots and suprapubic tenderness in a child with global delay suggests a delayed presentation of UTI resulting in haemorrhagic cystitis. This should be treated aggressively with antibiotics. A renal USS should be performed to look for structural abnormalities and to confirm resolution.
Which ONE of the following is true concerning Antidiuretic hormone (ADH)? Available marks are shown in brackets 1 ) Carbamazepine potentiates it's release 2 ) Ethanol potentiates it's release 3 ) It circulates in the blood bound to neurohypophysin 4 ) It is a cyclic octapeptide 5 ) It is synthesised in the posterior pituitary

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Comments: ADH is a nonapeptide manufactured in the paraventricular and supra-optic nuclei of the hypothalamus and released from the posterior pituitary. It acts on the collecting ducts improving water permeability and hence water retention. Carbamazepine as well as other agents such as thiazides and SSRIs may potentiate its release. Ethanol usually inhibits release. A 10 year old boy presents with a history of passing smoky coloured urine for 3 months intermittently. On the day of admission he is admitted with severe right loin pain radiating to the groin coming in spasms every few minutes. Full term normal delivery with no neonatal complications. Immunisations up to date. There is no family and social history of note. On examination he is intermittently writhing around the bed and crying. He is apyrexial but sweating. Respiratory rate is 15/min and heart rate 95/min. He has slight tenderness in the right loin. What is the most likely diagnosis? Available marks are shown in brackets 1) Benign familial haematuria 2) Glomerulonephritis 3) Hydronephrosis 4) Renal Claculi 5) Urinary tract infection Comments: The history suggests renal colic. This, combined with the history of probable haematuria, suggest renal stone. There is sometimes a family history. Timed collections for calcium and oxalate should be performed. An USS KUB and plain film X-ray will locate radio-opaque stones. Lithotrypsy or surgery may be required, followed by special diet depending on the

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underlying cause. A 16 year-old feamle presents with ankle swelling 4 days after having had a sore throat. On examination she had a blood pressure of 125/80 mmHg and ankle oedema. Investigations reveal: creatinine 90 umol/L (50-100) albumin 25 g/L (37-45) 24 hour urinary protein 9g (<0.2) What is the most likely diagnosis? Available marks are shown in brackets 1 ) idiopathic membranous nephropathy 2 ) IgA nephropathy 3 ) membranoproliferative glomerulonephritis 4 ) minimal change nephropathy 5 ) post-streptococcal glomerulonephritis

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Comments: Idiopathic membranous nephropathy (A) accounts for 2-5% of cases of nephritic syndrome in children, and 20-30% of cases in adults. The immune mechanism that leads to the development of membranous nephropathy is unknown. Histologically, it is characterised by diffuse thickening of the glomerular basement membrane (GBM) on light microscopy. On immunofluorescence, the thickening is caused by immune deposits of IgG and C3, on the subepithelial surface of the GBM. When not idiopathic, it is associated with AI diseases (SLE, Rheumatoid arthritis, thyroid disease), Drugs (Gold, penicillamine, captopril), Malignancy (Bronchus, breast, stomach, colon, prostate), Infections (Hep B, Syphilis, Leprosy, Filiarasis), and diabetes mellitus. Membranoproliferative (or mesangiocapillary) glomerulonephritis (C) can be classed into three types (I, II, and III) depending on which complement pathway is activated. It is associated with SLE, cryoglobulinaemia with or without Hep C, chronic infections (SBE) or with neoplasms. It is not associated acutely with upper respiratory tract infections. Minimal change nephropathy (D) is the most common form of nephrotic syndrome in children. The histological findings on light microscopy are normal or small looking glomeruli. On electron microscopy there is effacement of the epithelial cell foot processes over the outer surface of the GBM. It tends to be steroid responsive in children, but over 60% of children will have further relapses. In adults, it is associated with Hodgkins lymphoma, and other carcinomas. Post streptococcal GN (E) as the name implies occurs 10-14 days after an acute infection. The typical case occurs following infection with group A Lancefield streptococci (haemolytic strep, S pyogenes) either causing pharyngitis or skin infections. It is more common in the developing world. The histology shows diffuse proliferative GN, with infiltration by neutrophil polymorphs. The main treatment is eradication of the infection (10/7 course of penicillin) and symptomatic relief of the acute nephritis. The need for dialysis is uncommon, and complete recovery of renal function should occur. The correct answer is IgA nephritis (B). IgA nephritis is most common during the second and third decade of life. It commonly occurs within two days of an onset of an upper respiratory tract infection, or less commonly infection of other mucous membranes (e.g. GI, Bladder, Breast). It should be diagnosed by a renal biopsy, where IgA is seen deposited in the mesangium. The treatment of IgA nephritis is variable. In a patient with haematuria only, the treatment is conservative. When there is nephrotic range proteinuria (>3g/day- as in this case) an 8-12 week course of prednisolone should be prescribed. If the proteinuria is <3g/day an ACE inhibitor can be used. In all patients, careful control of blood pressure should be achieved, by using ACE inhibitors in the first instance, and regular follow up of renal function and urinalysis. 30% of children will have a spontaneous remission within 10 years, but 25% will go on to develop ESRF within 20 years. What is the most likely outcome of minimal change nephropathy at 16 year of age? Available marks are shown in brackets

1) 2) 3) 4) 5)

a tendency to relapse full renal recovery permanent renal impairment persistent hypertension persistent proteinuria

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Comments: 30-40% of children achieve spontaneous remission and 90% achieve remission following 8 weeks treatment with high dose steroids. However in adults only around 50% achieve remission. Which of the following is characteristic of Bartter's Syndrome? Available marks are shown in brackets 1) Secondary hyperaldosteronism 2) Hyperkalaemia 3) Metabolic acidosis 4) Reduced renal concentrating ability 5) Diarrhoea Comments: Barter's Syndrome is a rare form of renal potassium wasting characterised by hypokalaemia, normotension, and elevated renin and aldosterone levels. It is occasionally autosomal recessive. There is hyperplasia of the juxtaglomerular apparatus in most cases. It is postulated that the primary defect is in chloride reabsorption in the ascending limb, resulting in sodium chloride excessively presented to the distal tubule, with sodium reabsorption in exchange for potassium, resulting in urinary sodium wasting. There is secondary stimulation of prostaglandin synthesis, which activates the renin angiotensin aldosterone system which exacerbates the renal potassium wasting. Growth failure, muscle weakness, constipation, polyuria and dehydration are typical in younger children with muscle weakness, cramps or carpopedal spasms present in older children. The potassium is <2.5mmol/L, there is metabolic alkalosis, and hyperammonaemia with hyperaldosteronism. There are high levels of urinary potassium and chloride. The high urinary chloride level is helpful in distinguishing it from similar presentations which have low urinary chloride levels, such as liquorice, laxative, or diuretic use, persistent vomiting or diarrhoea, pyelonephritis, or diabetes insipidus. Oral potassium and indomethacin may be used. A 17 year old girl is admitted with a 2 day history of rigors due to a urinary tract infection. On 2 examination she appears unwell, has a Body Mass Index of 31kg/m , a temperature of 39C; examination is otherwise normal. Initial biochemistry revealed: Potassium 4 mmol/L (3.5-5) Urea 7 mmol/L (2.5-7) Glucose 33 mmol/L (3.0-6.0)arterial blood gases pH 7.3 (7.36-7.44) Standard bicarbonate 14 mmol/l Base deficit -10 urinalysis negative for ketones

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Which one of the following is the best initial treatment for her hyperglycaemia?

Available marks are shown in brackets 1) Metformin 2) Metformin plus Gliclazide 3) Rosiglitazone 4) Sliding scale IV insulin infusion 5) Subcutaneous insulin mixture

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Comments: This patient has a metabolic acidosis with pH of 7.3 and low bicarbonate likely due to sepsis. She is a type 2 diabetic with uncontrolled hyperglycaemia but is ulikely to have diabetic ketoacidosis because the urine is negative for ketones. It is important that her glycaemia is controlled to promote recovery from the sepsis this is best achieved with intravenous insulin initially. Which of the following is associated with hypernatraemia. Available marks are shown in brackets 1) Cholera 2) Pyloric stenosis 3) Inappropriate ADH secretion 4) Diabetes insipidus 5) Diabetes mellitus

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Comments: Cholera results in excessive water and sodium losses and hence hyponatraemia. Pyloric stenosis typically results in hypokalaemic, hypochloraemic, metabolic alkalosis and not cause hypernatraemia. Inappropriate ADH secretion causes hyponatraemia. In Diabetes insipidus failure to concentrate urine results in hypernatraemic dehydration with hyperosmolarity. Poorly controlled diabetes results in osmotic diuresis and renal sodium loss. Which of the following is a known risk factor for the development of chronic rejection of kidney transplantation :Available marks are shown in brackets 1) Age 2) Anti-smooth muscle antibodies 3) Presence of anti-HLA antibodies 4) Smoking 5) Toxoplasma infection Comments: Chronic rejection is characterised by fibrosis of normal organ structures. The pathogenesis of chronic rejection is not clear- some prefer the term "chronic allograft dysfunction" since both immunological (antigen-dependent and antigen-independent) and non-immunological factors have been identified. Cell-mediated and humoral immune mechanisms have been implicated in this form of graft rejection. It has also been suggested that rejection is a response to chronic ischaemia caused by injury to endothelial cells. Proliferation of intimal smooth muscle is observed leading to vascular occlusion. The fact that chronic rejection is rare in transplants between HLA-identical siblings suggests that HLA-antigen dependent immunological factors are important. Risk factors include: number of previous acute rejection episodes, presence of anti-HLA antibodies, anti-endothelial antibodies, CMV infection, dyslipidaemia, hypertension, functional mass of the

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donor kidney and delayed graft function (a clinical manifestation of ischaemia/reperfusion injury) A 2 week old male child is brought to casualty by his concerned parents with diarrhoea and vomiting. He is the first child of a young couple. Examination reveals few features besides obvious dehydration. He is noted to have a penile length of 3.5cms. Which of the following is the most appropriate inital treatment for this patient? Available marks are shown in brackets 1 ) Cow's milk allergy is the most likely diagnosis 2 ) gluten-enteropathy should be excluded 3 ) Requires urgent treatment with oral steroids 4 ) Requires urgent treatment with IV normal saline 5 ) Rota virus gastroenteritis is the most likely diagnosis

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Comments: The history suggests a diagnosis of classical congenital adrenal hyperplasia which is commonly due to 21 hydroxylase deficiency. A variable presentation is typical but neonatal presentations include salt losing crisis, penile development in the male virilisation and ambiguous genitialia in females. Patients should initially be resuscitated with fluid, usually saline and if suspicious, urgent biochemistry requested for cortisol, 17OHP etc prior to administration of intravenous steroids.