1-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? BOF 1 ) Benign familial haematuria 2 ) Glomerulonephritis 3 ) Haemorrhagic cystitis 4 ) Henoch-Schonlein Disease 5 ) Urinary tract infection

3 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

2-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? BOF 1 ) idiopathic membranous nephropathy 2 ) IgA nephropathy 3 ) membranoproliferative glomerulonephritis 4 ) minimal change nephropathy 5 ) post-streptococcal glomerulonephritis

2 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.

3-A 17 year old girl is admitted with a 2 day history of rigors due to a urinary tract infection. On examination she appears unwell, has a Body Mass Index of 31kg/m2, 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 Which one of the following is the best initial treatment for her hyperglycaemia?

BOF 1 ) Metformin 2 ) Metformin plus Gliclazide 3 ) Rosiglitazone 4 ) Sliding scale IV insulin infusion 5 ) Subcutaneous insulin mixture

4 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.

4-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? BOF

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

4 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.

5-Which of the following is a known risk factor for the development of chronic rejection of kidney transplantation :BOF 1 ) Age

2 ) Anti-smooth muscle antibodies 3 ) Presence of anti-HLA antibodies 4 ) Smoking 5 ) Toxoplasma infection

3 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 donor kidney and delayed graft function (a clinical manifestation of ischaemia/reperfusion injury)

6-The following regarding Atrial Natriuretic Peptide are correct: T/F A It is secreted in response to right atrial distension B It inhibits aldosterone release C It is a direct inotrope D It is a direct vasodilator E It is inactivated by endopeptidase

TTFTT Comments: ANP is a 28 aa peptide that is synthesised and released by atrial myocytes in response to distension, angiotensin II and endothelin. It causes salt and hence water losses, is a vasodilator (through possible direct and indirect mechanisms) and inhibits aldosterone release. Such actions reduce blood volume and cardiac output. ANP is degraded by neutral endopeptidase.

7-Hypernatraemic dehydration is commonly associated with the following:T/F A Cholera B Pyloric stenosis C Inappropriate ADH secretion D Diabetes insipidus

E Diabetes mellitus

FFFTF 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.

8-Nephrogenic diabetes insipidus is commonly associated with:T/F A Recurrent fevers B Craving for table salt C Abnormal growth D Urinary osmolality of 200 MOsmols/kg H20 or less E XY Karyotype

TFTTT Comments:

Clinical manifestations of nephrogenic DI include thirst, dehydration and hyperthermia. Patients crave water and ice cold drinks rather than salt. Inadequate caloric ingestion associated with incessant water intake can cause growth retardation and repeated bouts of hypernatraemia can cause mental impairment. Urine osmolarity of less than 200 mOsmols/kg H20, hypernatraemia and urine specific gravity of 1.005 or less are characteristic. Familial nephrogenic DI is commonly X linked and males present more often

9-Theme: Urinary discoloration A Myoglobulinaemia B Tetracyclines C Phenolphthalein D Phenylketonuria E Laxative abuse F Levodopa G Isoniazid H Iron I Rifampicin J Acute intravascular haemolysis Match these aetiological causes of a discolored urine to the colour of the urine observed.

Scenario 1 Which one of the above aetiological factors causes a yellow urine? Scenario 2 Which one of the above aetiological factors causes a pink/orange urine? Scenario 3 Which one of the above aetiological factors causes a black urine?

BIH Scenario 1 Which one of the above aetiological factors causes a yellow urine? B Tetracyclines A yellow urine can result from taking tetracyclines.

Scenario 2 Which one of the above aetiological factors causes a pink/orange urine? I Rifampicin Causes of a pink urine include rifampicin, dyes and urates.

Scenario 3 Which one of the above aetiological factors causes a black urine?

H Iron Causes of a black urine include iron, homogentisic acid and melanotic sarcomas. Red urine is the result of acute haemolysis, and red blood cells may be noted on microscopy. Brown smoky urine may be due to a disease of the glomerulus, and the cells on microscopy are distorted, small and fragmented. Red cell casts may be seen. Haematuria from the bladder or urethra may show normal cells on microscopy. Causes of a brown urine include levodopa, methyldopa, myoglobinuria, Alport syndrome with associated glomerular sclerosis, congenital biliary atresia, bowel disease, acute porphyria (porphobilinogen), porphyria variegata and congenital porphyria. A red urine can arise from rifampicin, dyes, laxative abuse, phenolphthalein, acute intravascular haemolysis, haemoglobinuria (urobilinogen), beetroot, acute intermittent porphyria,Serratia marcescens, haematuria associated with hereditary telangiectasia and Wilms tumour. Causes of a pink urine include rifampicin, dyes and urates. A yellow urine can result from taking tetracyclines. Causes of blue urine, especially noted in nappies, include idicanuria. Drugs which show no change in the urinary colour include isoniazid, and disorders with the same effect include phenylketonuria.

10-Theme: Renal tract malformations Options A. Autosomal dominant polycystic kidney disease B. Autosomal recessive polycystic kidney disease C. Bilateral renal agenesis D. Bladder exstrophy E. Multicystic dysplastic kidney F. Posterior urethral valves

G. Unilateral hydronephrosis H. Vesico-ureteric reflux Instructions: For each of the following case scenarios, select the most likely diagnosis from the list above. Each option may be used once, more than once or not at all. Scenario 1 A 2-year-old girl who is asymptomatic had been found to have a right-sided cystic kidney on antenatal scanning. Postnatally, the mass was confirmed on ultrasound and was found on DMSA isotope scan to have no function at 3 months of age. A recent ultrasound scan has shown that the mass is reducing in size. Scenario 2 A 3-month-old boy presents with failure to thrive and acute renal failure. Ultrasound scan shows an enlarged bladder. A surgical registrar passes a catheter and urine is produced. Scenario 3 A 6-year-old girl who is under the care of the hepatology team for hepatic fibrosis with an enlarged liver and spleen is found to be markedly hypertensive, with a systolic blood pressure of 140 mmHg. Renal ultrasound shows bilaterally enlarged and echogenic kidneys.

EFB Scenario 1 A 2-year-old girl who is asymptomatic had been found to have a right-sided cystic kidney on antenatal scanning. Postnatally, the mass was confirmed on ultrasound and was found on DMSA isotope scan to have no function at 3 months of age. A recent ultrasound scan has shown that the mass is reducing in size. Correct answer E Multicystic dysplastic kidney The differential diagnosis of a cystic kidney in a neonate includes hydronephrosis due to obstruction or vesico-ureteric reflux, and urinary tract ultrasound helps to distinguish these. No

function on a DMSA isotope scan is characteristic of multicystic dysplastic kidney and most, but not all, involute with age.

Scenario 2 A 3-month-old boy presents with failure to thrive and acute renal failure. Ultrasound scan shows an enlarged bladder. A surgical registrar passes a catheter and urine is produced. Correct answer F Posterior urethral valves Frequently, but not always, posterior urethral valves are detected antenatally. If valves have been missed they can present with poor urinary flow and varying degrees of renal impairment. Unless there is significant renal dysplasia associated, renal function will improve with drainage. Repeated attempts at urethral catheterisation should be avoided as urethral damage can occur.

Scenario 3 A 6-year-old girl who is under the care of the hepatology team for hepatic fibrosis with an enlarged liver and spleen is found to be markedly hypertensive, with a systolic blood pressure of 140 mmHg. Renal ultrasound shows bilaterally enlarged and echogenic kidneys. Correct answer B Autosomal recessive polycystic kidney disease Autosomal recessive polycystic kidney disease is typically associated with varying degrees of hepatic fibrosis. Marked hypertension is a characteristic feature. In contrast to autosomal dominant polycystic kidney disease, in which discrete cysts are seen, the cysts in the autosomal recessive type are so small and diffuse that they give the appearance of enlarged and echogenic kidneys.