Genitourinary

A 2 year old child presents with a palpable abdominal mass. The following renal pathology should be considered: A Autosomal recessive polycystic disease B Tuberous sclerosis C Wilm's tumour D Nephroblastoma E Autosomal dominant polycystic kidney disease (True) (True) (True) (True) (False)

Comments: It is essential that all abdominal masses are promptly investigated using an ultrasound scan. Causes of palpable kidneys include:

Unilateral: multicystic kidneys, compensatory hypertrophy, obstructive hydronephrosis, Wilm's tumour, renal vein thrombosis. Bilateral: autosomal recessive polycystic disease, autosomal dominant polycystic disease (presents in adulthood), tuberous sclerosis, renal vein thrombosis.

Copyright 2002 Dr Colin Melville

A 2 year old child presents with a palpable abdominal mass. The following renal pathology should be considered: A Autosomal recessive polycystic disease B Tuberous sclerosis C Wilm's tumour D Nephroblastoma E Autosomal dominant polycystic kidney disease (True) (True) (True) (True) (False)

Comments: It is essential that all abdominal masses are promptly investigated using an ultrasound scan. Causes of palpable kidneys include:

Unilateral: multicystic kidneys, compensatory hypertrophy, obstructive hydronephrosis, Wilm's tumour, renal vein thrombosis. Bilateral: autosomal recessive polycystic disease, autosomal dominant polycystic disease (presents in adulthood), tuberous sclerosis, renal vein thrombosis.

Copyright 2002 Dr Colin Melville

A 4 month old girl presents with Nephrotic Syndrome. The following suggest a diagnosis of cystinosis:

A Urinary cystine of 80000 times the normal level. B Characteristic urinary smell. C Growth failure. D Blonde hair and fair complexion. E Crystalline deposits in the cornea. Comments:

(False) (False) (True) (True) (True)

In cystinosis, there is increased cellular uptake of cystine inherited as an autosomal recessive. This accumulates in the reticular endothelial cells, particularly the spleen, liver, lymph nodes, bone marrow, but not the muscle or brain. It is also deposited in the renal tubular cells, the cornea, and the conjunctivae. The renal deposition causes Fanconi's Syndrome, and on renal biopsy, biorefringent crystals may be seen in the interstitiam. Renal failure supervenes with shrunken, contracted kidneys. Presentation may be with an aggressive infantile form, an intermediate adolescent form, or a benign adult form in which renal disease is absent. Confirmation is by measuring cystine content of white cells or fibroblasts which are 80000 times normal. Split lamp examination shows crystals in the cornea. Prenatal diagnosis is available. Isolated cystinuria is not found, though a generalised amino aciduria will be found in association with Fanconi's Syndrome. Treatment is with cystine. Copyright 2002 Dr Colin Melville

If dilatation fo the renal pelvis is found on antenatal ultrasound scan in a male child: A Intra-uterine surgery is mandatory. B Posterior urethral valves should be considered in bilateral cases. C Mild defects often resolve by the time of birth. D MCUG should be performed if a unilateral defect is severe and persists after birth. E Prophylactic antibiotics should be given until evaluation is complete. Comments: Dilatation of the renal pelvis is a fairly frequent finding and varies from mild to severe. In the first instance, antibiotic prophylaxis should be started at birth, and an ultrasound scan in the first week arranged. Should there be no abnormality, or a unilateral abnormality, then prophylaxis should be continued and the ultrasound repeated 4-6 weeks later. If there is any abnormality at this latter scan then an MCUG and a DMSA scan should be arranged. If the abnormality is bilateral, then an urgent MCUG should be performed in a male child because of the possibility of posterior urethral valve. Copyright 2002 Dr Colin Melville (False) (True) (True) (True) (True)

Regarding congenital Nephrotic Syndrome (Finnish type):

A It is characterised by cysts visible on ultrasound scan. B Presentation is usually within the first 3 months of life. C It may be associated with a small placenta. D It is inherited as an autosomal recessive disorder. E May be associated with respiratory distress in the newborn. Comments:

(False) (True) (False) (True) (True)

Congenital Nephrotic Syndrome is autosomal recessive, and commonest in Scandinavian genotypes. There is dilatation of the proximal convoluted tubules visible on microscopy (microcystic disease). Proteinuria is present at birth, and the children may be born prematurely with enlarged placenta, respiratory distress, and separation of the cranial sutures. They usually present within the first 3 months of life, and tend to have persistent oedema and recurrent infections resulting in death by the age of 5 years. The disease is not steroid responsive, with renal transplantation the only hope. Antenatal diagnosis by measuring alphafeta protein levels is possible on amniocentesis. Copyright 2002 Dr Colin Melville

A girl of 2 years has 3+ proteinuria. The following should be considered in the differential diagnosis: A Post-streptococcal GN B Idiopathic nephrotic syndrome C Urinary tract infection D Ectopic ureter E Ureterocele (True) (True) (False) (False) (False)

Comments: Non-pathological proteinuria may be postural, due to a fever or secondary to a exercise. Pathological proteinuria may be divided into:

Tubular: Hereditary: cystinosis, Wilson's Disease, Lowe's Syndrome, proximal RTA, galactosaemia. o Acquired: antibiotics, interstitial nephritis, acute tubular necrosis, cystic diseases, heavy metal poisoning. Glomerular: o Persistent asymptomatic. o Nephrotic Syndrome: a) Idiopathic: minimal change, mesangial proliferation, focal sclerosis. b) Glomerular nephritis. c) Tumours. d) Drugs. e) Congenital Tubular proteinuria rarely exceeds 1g/day, whereas glomerular ranges from <1->30g/24 hours.

Glomerular may be selective (loss of plasma proteins of molecular weight up to and including albumin), or non-selective (loss of albumin of larger molecular weight proteins such as IgG). The majority of cases are non-selective, selective proteinuria being seen primarily in minimal change Nephrotic Syndrome. Urine testing on 1+ is equivalent to 0.3g/L, 2+ to 1g/L, 3+ to 3g/L, and 4+ to >20g/L. 3 and 4+ proteinuria are therefore suggestive of glomerular proteinuria. Copyright 2002 Dr Colin Melville

Recognised causes of nephrotic syndrome include: A Henoch-Schonlein purpura B Renal vein thrombosis C Quartan malaria D SLE E Renal amyloidosis (True) (False) (False) (True) (True)

Comments: Nephrotic Syndrome is defined by the clinical association of heavy proteinuria, a low plasma albumin, and oedema. The proteinuria is usually heavy, secondary to glomerular involvement. Causes include:

Idiopathic Nephrotic Syndrome: minimal change (85%), mesangial proliferation, focal sclerosis. Glomerular nephritis. Tumours. Drugs. Congenital.

Copyright 2002 Dr Colin Melville

The following confirm a urine infection in an infant: A A bag urine specimen containing 300 white cells and >105 E. Coli per mm3. B White cells and 103 E. Coli per mm3 in a suprapubic aspirate. C Two bag urines growing >105 E. Coli. D Catheter specimen of urine containing 60 white cells and >105 E. Coli. E Positive nitrite in a bag urine specimen. Comments: The diagnosis of a urinary tract infection is confirmed by a positive catheter or suprapubic aspirate of urine. Bag urines are only useful in excluding infection if negative. Any number of gram negative bacteria and an SPA indicates infection, though a small number of gram positive cocci may represent skin contaminants. Even the absence of leukocytes does not exclude infection. In infancy, 33% will have positive blood cultures, and a few may have meningitis concurrently. A negative nitrite test is a good way of ruling out infection, but not diagnosing it. Copyright 2002 Dr Colin Melville (False) (True) (False) (True) (False)

The following functions are correctly described:

A The proximal tubule reabsorbs amino acids. B Approximately 20 times blood volume is filtered each day by the glomeruli. C Ammonia is generated at the distal tubule. D The distal tubule is important in acid-base balance. E Atrial naturiuretic peptide is produced by the adrenal cortex in response to decreased circulating volume. Comments:

(True) (False) (True) (True) (False)

The proximal tubule is responsible for reabsorption of glucose, amino acids, phosphate, urate, bicarbonate, and calcium. The distal tubule is responsible for chloride and water reabsorption and hydrogen iron secretion. The control of acid base balance involves the production of bicarbonate and ammonia. Approximately 120 times the blood volume is filtered each day, and ANP is produced in the cardiac atria. Copyright 2002 Dr Colin Melville

The following are characteristic of Bartter's Syndrome: A Secondary hyperaldosteronism B Hyperkalaemia C Metabolic acidosis D Reduced renal concentrating ability E 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. Copyright 2002 Dr Colin Melville (True) (False) (False) (False) (False)

Concerning childhood urinary tract infection:

A It is commonest in girls under one month of age. B It may present with nausea and vomiting. C It is usually caused by E.Coli. D It is commonly associated with vesico-ureteric reflux. E The prophylactic antibiotic of choice is ampicillin. Comments:

(False) (True) (True) (True) (False)

In infancy, urinary tract infection is commonest in boys, particularly those who are uncircumcised. Over this age there is a predominance in girls. The usually aetiology is E.Coli, and in approximately 50% of cases with proven urinary tract infection, vesico-ureteric reflux or some other structural abnormality of the kidneys is present. The symptoms are very variable, but include nausea and vomiting. Copyright 2002 Dr Colin Melville

A 2 year old boy presents with 4+ proteinuria and periorbital oedema. The following suggest that a diagnosis of mesangiocapillary glomerulonephritis should be considered: A Non-response to oral steroids B Gross haematuria C Low C3 level D Previous history of hepatitis B infection E Family history of Nephrotic Syndrome Comments: Minimal change Nephrotic Syndrome usually responds to steroids, and this makes up about 85% of the total number of cases. Non-response is commoner in more complex pathologies. Focal segmental glomerular sclerosis is associated with a family history, and 30% progress to end stage renal failure in 5 years. 20% respond to cyclophosphamide or other immunosuppressives. (True) (True) (True) (False) (False)

Mesangiocapillary (membranoproliferative): commoner in older children, and associated with haematuria and low complement levels. There is a gradual decline in renal function over many years. Membranous: associated with hepatitis B infection. Most remit within 5 years. May be associated with SLE. Post-Streptococcal nephritis may also be associated with low C3 levels, and these return to normal after 3-4 weeks. This usually presents with haematuria rather than proteinuria.

Copyright 2002 Dr Colin Melville

Regarding undescended testes: A It occurs in 10% of male infants at birth. (False)

B Bilateral cases occur more frequently than unilateral cases. C Retractile testes can be brought down to the base of the scrotum without tension. D Palpable undescended testes are usually ectopic. E If impalpable, the testis may be intra-abdominal. Comments:

(False) (True) (False) (True)

In term infants, 2% of males have bilateral undescended testes, and 3% unilateral. The incidence is higher in preterm infants, with descent primarily in the third trimester. By 3 months of age, the rate is 1.5%. Retractile testes can be brought down without tension, and these cases should be followed-up as occasionally the testes ascends into the inguinal canal. Palpable testes are usually in the inguinal canal, but occasionally ectopic. They cannot be manoeuvred into the scrotum. Impalpable testes may be absent, intra-abdominal or inguinal. Copyright 2002 Dr Colin Melville

In Prune Belly Syndrome: A The bladder is neuropathic. B Bilateral severe hydronephrosis is always found. C Only two vessels are usually present in the umbilical cord. D Bilateral cyptorchidism is present in most cases. E Bladder extrophy may be associated. Comments: In the Prune Belly Syndrome there is absence of abdominal musculature, with visible intestinal pattern, and a thin lax protruding abdominal wall which is wrinkled. The bladder is distended and there is fetal and postnatal urinary tract obstruction, often with posterior urethral valves, hydronephrosis, hydroureter and cryptorchidism. The mode of inheritance is unclear. Copyright 2002 Dr Colin Melville (False) (True) (False) (True) (False)

The following investigations are useful in locating an ectopic testis: A Radionucleotide scan B Ultrasound scan C Testosterone response to HCG D Abdominal x-ray E Chromosomes Comments: (False) (True) (False) (False) (False)

If the testis is ectopic then it may be palpable outside the normal line of descent. However, in obese boys, ultrasound may be useful in identifying its location. Radionucleotide scans and x-rays should be avoided, and hormonal testing may be useful in cases of bilateral impalpable testes to detect the presence of testicular tissue within the body. Laparoscopy is the investigation of choice if the testes are impalpable to determine whether they are absent or intra-abdominal. Copyright 2002 Dr Colin Melville

The following investigations are useful in locating an ectopic testis: A Radionucleotide scan B Ultrasound scan C Testosterone response to HCG D Abdominal x-ray E Chromosomes Comments: If the testis is ectopic then it may be palpable outside the normal line of descent. However, in obese boys, ultrasound may be useful in identifying its location. Radionucleotide scans and x-rays should be avoided, and hormonal testing may be useful in cases of bilateral impalpable testes to detect the presence of testicular tissue within the body. Laparoscopy is the investigation of choice if the testes are impalpable to determine whether they are absent or intra-abdominal. Copyright 2002 Dr Colin Melville (False) (True) (False) (False) (False)

The following are recognised complications of circumcision: A Delayed healing B Bleeding C Pain D Damage to the glans E Infection Comments: There are great cultural differences in the rates of circumcision. In the UK, the above reasons are used to justify not circumcising unless specifically indicated. In America, circumcision is encouraged as it slightly reduces the risk of urinary tract infections and the already low risk of penile cancer. Copyright 2002 Dr Colin Melville (True) (True) (True) (True) (True)

Regarding the diagnosis of urinary tract infection in infants: A A bag urine will confirm it. B Ultrasound scan of kidneys, ureters and bladder should be performed in all suspected cases. C DMSA scan should be performed in confirmed cases. D IVP is the only investigation required in most cases. E MCUG should be performed in suspected cases. (False) (False) (True) (False) (False)

Comments: The diagnosis of urinary tract infection in infants is difficult. A bag specimen is only useful when negative to exclude infection. At least 2 bag urines growing a pure growth of >105 of the same organism with the same sensitivities, accompanied by pyuria can be taken as presumptive evidence of an infection. However, the gold standard remains the suprapubic aspirate or catheter specimen of urine. In the former, any number of gram negative bacteria indicates infection, though a small number of gram positive cocci may represent skin contaminants. The absence of pyuria does not rule out infection. In infancy, 33% of blood cultures are positive in those with proven urinary tract infection, and meningitis can also occur simultaneously. An ultrasound scan should be performed in all confirmed cases soon after the diagnosis. This is helpful in ruling out a pyonephrosis or renal abscess. After resolution, an MCUG is done to exclude reflux, and a DMSA scan can help in diagnosing pyelonephritis or scarring. An IVP is only done in selected cases, though historically this was the popular investigation. Copyright 2002 Dr Colin Melville

The following are recognised causes of acute renal failure: A Burns B Dermatomyocytis C Duchenne muscular dystrophy D Penicillin therapy E Alport's Syndrome Comments: Causes of acute renal failure can be divided into pre-renal, renal and post-renal. (True) (False) (False) (False) (False)

Pre-renal: o Hypovolaemia (gastroenteritis, burns, sepsis, haemorrhage, Nephrotic Syndrome). o Circulatory failure. Renal: o Vascular: HUS, vasculitis, embolus, renal vein thrombosis. o Tubular: acute tubular necrosis, ischaemic, toxic, obstructive. o Glomerular: glomerulonephritis. o Interstitial: interstitial nephritis, pyelonephritis. o Acute chronic renal failure. Post-renal: obstruction, either congenital or acquired. Although Alport's Syndrome is associated with end

stage renal failure, this usually progresses gradually so that it occurs in adult life. Copyright 2002 Dr Colin Melville

In moderate (not end stage) chronic renal failure: A The level of blood insulin is inappropriately high compared to the level of blood glucose. B The main cause of growth failure is growth hormone insufficiency. C Polyuria is more common than oliguria. D Typically, there is hypercalcinuria. E Treatment and control of hypertension will improve GFR. Comments: Regardless of the cause of kidney damage, once a critical level of renal functional deterioration is reached, progression to end stage renal failure is inevitable. Factors that may be important include: ongoing immunological injury, hyperfiltration in surviving glomeruli, dietary protein and phosphate intake, persistent proteinuria, and systemic hypertension. Growth retardation is partly due to protein calorie deficiency, partly to acidosis and anaemia, and partly to renal osteodystrophy. In renal osteodystrophy there is abnormal mineral and bone metabolism, including malabsorption of calcium, phosphate retention, and hyperparathyroidism. There is cutaneous vascular and visceral calcification, and impairment of 1-hydroxylation of Vitamin D. There is osteomalacia, and later osteitis fibrosa. The hyperfiltration theory is borne out by the fact that hypertension control improves GFR. Copyright 2002 Dr Colin Melville

(False) (False) (True) (True) (True)

The following are indications for peritoneal dialysis: A Hyperkalaemia B Aspirin overdose C Hepatic encephalopathy D Hypercalcaemia E Fluid overload Comments: Indications for peritoneal dialysis include: (True) (False) (False) (False) (True)

Acute renal failure (especially HUS, where it can remove toxins). Hypernatraemia and hyperkalaemia. Metabolic diseases such as urea cycle defects/hyperammonaemia, and maple syrup urine disease. Drugs: especially theophylline toxicity. Unfortunately, it is useless in aspirin toxicity, though charcoal haemoperfusion may be useful in this condition.

Copyright 2002 Dr Colin Melville

The following are indications for peritoneal dialysis: A Hyperkalaemia B Aspirin overdose C Hepatic encephalopathy D Hypercalcaemia E Fluid overload Comments: Indications for peritoneal dialysis include: (True) (False) (False) (False) (True)

Acute renal failure (especially HUS, where it can remove toxins). Hypernatraemia and hyperkalaemia. Metabolic diseases such as urea cycle defects/hyperammonaemia, and maple syrup urine disease. Drugs: especially theophylline toxicity. Unfortunately, it is useless in aspirin toxicity, though charcoal haemoperfusion may be useful in this condition.

Copyright 2002 Dr Colin Melville

The following may be associated with posterior urethral valve syndrome: A Polyhydraminos B Bilateral hydronephrosis on antenatal ultrasound scan C Hypospadias D Undescended testis E Adult polycystic disease Comments: Posterior urethral valves are membranes with an eccentric opening, causing varying degrees of obstruction. The prostatis urethra dilates, and the detrusor muscle and bladder neck hypertrophy. There may be vesico-ureteric reflux from chronic distension. This may result in mild hydronephrosis to severe renal dysplasia. Oligohydraminos and pulmonary hypoplasia may also occur. It may be diagnosed prenatally, when the prognosis is worse, or at the time of birth because of a palpable bladder and weak urinary stream. Later presentations include failure to thrive or urine infection or enuresis. There is a recognised association with prune belly syndrome. Copyright 2002 Dr Colin Melville (False) (True) (False) (False) (False)

Quantitative estimates of urinary protein loss can be made using: A 24 hour urine collection B Protein/ creatinine ratios in the urine C Albumin/ creatinine ratios in the serum D Urine dipstix E Urine microscopy Comments: Urine dipsticks gives a rough and ready semi-quantitative method of assessing protein loss. 1+ is equivalent to 0.3g/L, 2+ to 1g/L, 3+ to 3g/L, and 4+ to >20g/L. Urine microscopy may reveal granular casts if proteinuria is prolonged, but this is not a quantitative method of estimation of urine protein loss. A 24 hour urine collection remains the gold standard, but more commonly protein or albumin is measured as a ratio against creatinine, excretion of which is assumed to be constant through the day. Ratios are, obviously, measured in the urine rather than the serum. Copyright 2002 Dr Colin Melville (True) (True) (False) (True) (False)

A 3 year old girl is being investigated for a urinary tract infection. The following can be reliably diagnosed using a dynamic nuclear medicine scan (MAG3): A Pelvi-ureteric junctional obstruction B Glomerular filtration rate C Relative renal uptake D Bladder wall thickening E Spina bifida occulta Comments: A dynamic nuclear medicine scan (MAG3) involves injecting isotope which is taken up by the kidneys and excreted down the ureters. If the child is old enough to co-operate with micturition it can provide indirect micturating cystourethrography. In a 3 year old girl it can, therefore, assist in the diagnosis of renal obstruction, and can also give the percentage renal uptake by each kidney. It can give no direct information about the structure of the bladder or the nerve supply to it. It is excreted by a glomerular filtration and can therefore give information about blood flow and renal function, but it is not usually used to obtain an estimate of glomerular filtration rate. Copyright 2002 Dr Colin Melville (True) (False) (True) (False) (False)

Concerning chloride depletion: A It is usually associated with an alkaline urine. (True)

B There is an inappropriate pottasium loss in the kidney. C It is associated with hyperparathyroidism. D It occurs with agents acting on the ascending loope of Henle. E It is associated with hypertriglyceridaemia. Comments:

(True) (False) (True) (True)

Under most circumstances, chloride concentration in the blood parallels sodium. Most often these changes occur secondary to diarrhoea with secondary dehydration. Hypochloraemia is typically seen in metabolic alkalosis. Chloride depletion as a cause of metabolic alkalosis occurs when chloride is lost from the body in excess of sodium losses (e.g. vomiting, gastric drainage, chloride diarrhoea, cystic fibrosis). Urinary losses of chloride may exceed those of sodium during the correction of metabolic acidosis, and also in potassium deficiency. Decreased filtered chloride increases bicarbonate reabsorption in the proximal tubule, leaving less available in the ascending limb, which reduces the amount of sodium reabsorbed. The increased sodium load at the distal nephron enhances potassium and hydrogen iron exchange. Chloride administration is therefore necessary to correct most cases of metabolic alkalosis. The urine is classically alkaline, but in the case of severe depletion of potassium, the urine potassium level and paradoxical aciduria exists. Copyright 2002 Dr Colin Melville

A 3 year old child has bloody diarrhoea and then becomes anuric with a serum potassium of 8.5mmol/l. The following statements are true: A D+ haemolytic uraemic syndrome is the most likely cause. B Treatment with dextrose and insulin may be required. C Calcium resonium resin given rectally may be beneficial. D The ECG monitor may show flattened T waves. E Broad-spectrum antibiotics should be given. Comments: The history given is highly suggestive of HUS associated with haemorrhagic diarrhoea. The likeliest organism is verotoxin-producing E.Coli 0107:H7. Hyperkalaemia reduces transmembrane potential towards threshold levels, producing delayed depolarisation, faster repolarisation, and slower conduction velocity. The heart is particularly vulnerable, with the ECG showing peaking of the T waves, lengthening of the PR interval, and widening of the QRS complex, the latter heralding the development of VF. Hyperkalaemia is usually seen when renal excretory mechanisms fail. It may occur in acute or chronic renal failure, in adrenal insufficiency, in hyporeninaemic hyperaldosteronism, and with the use of potassium-sparing diuretics. Treatments of hyperkalaemia include: calcium resonium resin, dextrose and insulin, and salbutamol infusion. Antibiotics do not help in HUS, and may prolong the condition. Copyright 2002 Dr Colin Melville (True) (True) (True) (False) (False)

Regarding undescended testes:

A For optimal fertility, the testes should be 1C below body temperature. B Undescended testes should be corrected by the age of 10 years to optimise fertility. C If one testis is absent, a prosthesis should be inserted as soon as possible to minimise psychological problems. D Intramuscular HCG is a reliable method of promoting testicular decent. E The risk of testicular malignancy at puberty for an intra-abdominal testis may be as high as 10%. Comments: The indications for treatment of undescended testes are:

(True) (False) (False) (False) (True)

Improved fertility: the testes should be 1C below body temperature to optimise fertility, and correction should occur before 2 years of age. Reduced risk of malignancy: the highest risk for testicular malignancy may be around 10% for testes intra-abdominal at puberty. The risk is increased with bilateral undescended testes. Although early orchidopexy may not reduce this risk, it does at least make the testes palpable. Cosmetic and psychological: prostheses are usually inserted around puberty, once the adult size of the normal testis is known. IM HCG or intranasal LHRF is used in some centres, but it rarely prevents the need for surgery. Orchidopexy is the usual procedure and is performed at 12 years of age as a day case. Orchidectomy should be done if the intra-abdominal testis cannot be mobilised sufficiently. Microvascular procedures or staged orchidopexy may be alternatives to preserve the testes if they are bilaterally intra-abdominal.

Copyright 2002 Dr Colin Melville

Nephrotic syndrome is associated with: A Cholesterol emboli B Highly selective proteinuria C Venous thrombosis D Hyperkalaemia E Pneumococcal infection Comments: Complications of Nephrotic Syndrome include: (False) (True) (True) (True) (True)

Infections: decreased immunoglobulins, oedema acting as a culture medium, protein deficiency, decreased bactericidal activity of leukocytes, immunosuppressive therapy, decreased perfusion of the spleen, loss of properdine and factor B have all been proposed as reasons for this. Spontaneous peritonitis is the commonest, with septicaemia, pneumonia, cellulitis, and urinary tract infections also occurring. Strep. Pneumoniae is the commonest cause of peritonitis, though gram negatives are also seen. Steroids may mask symptoms. In remission, all patients with Nephrotic Syndrome should receive polyvalent pneumococcal vaccine. Arterial and venous thrombosis: partly due to elevated plasma levels of coagulation factors and inhibitors of fibreinlisis, decreased plasma level of anti-thrombin 3, and increased platelet

aggregation. Deficiencies of coagulation factors 9, 11 and 12. Reduced serum levels of Vitamin D.

Copyright 2002 Dr Colin Melville

The following are associated with an increased risk of UTI: A Duplex ureter B Myelomeningocele C Oral contraceptive pill D Constipation E Vesico-ureteric reflux Comments: Urinary tract infection occurs in 0.1% of newborns. Infections are more frequent in males, and more severe than in older children, and the clinical manifestations are vaguer. Predisposing factors include: P blood group secretor status, vesico-ureteric reflux, obstructive uropathy, low birth weight, meningomyelocele, bladder catheterisation, and being uncircumcised. 75% of urinary tract infections are caused by E.Coli. The route of infection is usually ascending, and rarely haematogenous. Infections in the newborn can cause renal scarring, and renal growth retardation. In older children, infections are more common in females, occurring in 1.5% of school aged females most commonly in the 7-10 year old age group. Sexually active females are at increased risks from cystitis. 80% of infections are caused by E.Coli, with Klebsiella and Proteus being rarer. Viral infections may also occur. In older children, immunosuppression, diabetes, urinary tract obstruction, and chronic granulomatous disease, presence of calculi, and urinary stasis all predispose to infection. Copyright 2002 Dr Colin Melville (True) (True) (False) (True) (True)

A 9 month old boy presents with an acute scrotal swelling. The following diagnoses are likely: A Epididymitis B Orchitis C Torsion of the testicular appendage D Irreducible inguinal hernia E Acute idiopathic scrotal oedema Comments: With any acute swollen scrotum, the fear is of torsion of testis. This is uncommon in the neonate, and is much commoner around puberty. Presentation is with a hard tender testis and spermatic cord -/+ a red scrotum. Torsion of the appendage of testis has a peak incidence of between 4 and 8 years of age, as does acute idiopathic scrotal oedema. In the former, there is a tender upper pole of testis with a blue spot (False) (False) (False) (True) (False)

on transillumination. In the latter, the erythema extends beyond the scrotum, and the testis is minimally tender. Epididymitis is rare before puberty, and presents with a tender epididimus (urological investigations are needed, as it is associated with reflux of infected urine via the vas deferens). Inguinal herniae that are irreducible have a peak incidence below 2 years of age. A firm immobile tender swelling is found in the scrotum, which becomes inflamed as the strangulation occurs. Occasionally, acute hydroceles can also present with a mobile blue transilluminating swelling at 1-3 years of age. Copyright 2002 Dr Colin Melville

An 18 month old child presents with capillary pH 7.25, PCO2, 3.9 kPa, BE -8, serum Na 130mmol/l, serum K 7.9mmol/l, urine pH of 6.5. The following diagnoses should be considered: A Proximal renal tubular acidosis B Distal renal tubular acidosis C Congenital adrenal hypoplasia D Congenital adrenal hyperplasia E Conn's Syndrome (Congenital hyperaldosteronism) Comments: The picture is one of a metabolic acidosis, partly compensated by respiratory alkalosis (low PCO2). The sodium is low, the potassium is high, and the urine pH is relatively high given the degree of acidosis. Renal tubular acidosis both proximal and distal can give metabolic acidosis, which can also occur if there is under-production of aldosterone, which can occur in CAH. Proximal RTA is usually associated with a low potassium. In Conn's Syndrome there is metabolic alkalosis and hypokalaemia because of increased renal reabsorption of bicarbonate. Copyright 2002 Dr Colin Melville (False) (True) (True) (True) (False)

The following may present with pulmonary haemorrhage and glomerulonephritis: A IgA nephropathy B Goodpasture's Syndrome C Congenital Nephrotic Syndrome D Pulmonary haemosiderosis E HSP Comments: The combination of pulmonary haemorrhage and glomerulonephritis may occur in: Goodpasture's Syndrome, SLE, HSP, polyarteritis nodosa, Wegener's granulomatosis. Goodpasture's Disease is pulmonary haemorrhage and glomerulonephritis associated with antibodies against the lung and glomerular basement membrane, and should be distinguished from Goodpasture's Syndrome (pulmonary (False) (True) (False) (False) (True)

haemorrhage plus glomerulonephritis). Copyright 2002 Dr Colin Melville

Recognised side effects of thiazides: A Hypercholesterolaemia B Hypochloraemia C Impotence D Agranulocytosis E Peripheral neuropathy Comments: Side effects of thiazides include: (True) (True) (True) (True) (False)

Postural hypotension. GI upsets. Reversible impotence. Electrolyte disturbance: hypokalaemia, hypomagnesaemia, hyponatraemia, hypochloraemic alkalosis, hypercalcaemia, hyperglycaemia, hyperuricaemia, hypercholesterolaemia. Rashes, photosensitivity. Blood disorders, including exacerbation of SLE.

Copyright 2002 Dr Colin Melville

A one hour old baby has capillary blood gases showing pH 7.25, PCO2 7.1kPa, PO2 5.4 kPa and BE -12. The following statements are true: A There is evidence of respiratory alkalosis. B There is evidence of metabolic acidosis. C The result may be artefactual from poor peripheral perfusion. D An arterial blood gas should be taken to confirm the results. E Oxygen should be given immediately and endotracheal intubation considered. Comments: Soon after birth, infants can have a haemoglobin as high as 21g/dl. This may result in sluggish peripheral perfusion, and this can result in "metabolic acidosis" as an artefact. Although there is evidence of metabolic acidosis on this sample, it is therefore sensible to confirm the results with an arterial blood gas should the child's general condition be good. If the child looks ill then steps to resuscitate him should be initiated. Copyright 2002 Dr Colin Melville (False) (True) (True) (False) (False)

Distal renal tubular acidosis type IV: A Is characterised by hyperchloraemic acidosis and hypokalaemia. B Is characterised by a large anion gap and aminoaciduria. C A decreased glomerular filtration rate. D Is characterised by the presence of hyponatraemia. E Is due to increased reabsorption of hydrogen ions. (True) (False) (False) (True) (False)

Comments: Renal tubular acidosis is a clinical state of systemic hyperchloraemic acidosis resulting from impaired urinary acidification. Three types exist:

Distal RTA (type I). Proximal RTA (type II). Mineralacorticoid deficiency (type IV) (a proposed type III was found to be a variant of type I).

Proximal RTA results from reduced proximal tubular reabsorption of bicarbonate, presumably owing to deficient carbonic and hydrase production or hydrogen iron secretion. There is, therefore and increased load of bicarbonate reaching the distal tubules, overwhelming the capacity of the distal tubule to reabsorb it. Up to 25% of a normal filtered load of bicarbonate can be lost in the urine, and proximal RTA is therefore more severe than distal RTA. Causes of proximal RTA include: cystinosis, Lowe's Syndrome, galactosaemia, hereditary fructose intolerance, tyrosinaemia, Wilson's Disease, medullary cystic disease. Distal RTA is a deficiency of hydrogen iron secretion by the distal tubule and collecting duct. There must thus be a loss of bicarbonate in the urine of upto 10% of the filtered load. The urine pH cannot be reduced below 5.8 despite severe systemic acidosis. The loss of sodium bicarbonate results in hyperchloraemia and hypokalaemia. Mineralacorticoid deficiency RTA may result from diseases of the adrenal gland (Addison's, CAH, primary hypoaldosteronism) in which aldosterone production is deficient. In these disorders renal function is normal, urine sodium wasting is common, and the plasma renin level is high. Copyright 2002 Dr Colin Melville

In cases of non-gonococcal urethritis: A Chlamydia trachomatis is the commonest organism B Association with septic arthritis is common C It is usually treated with Septrin D Cystitis is typical E Chronic conjunctivitis is a recognised sequela. Comments: Most non-gonococcal urethritis is due to chlamydia, and more rarely due to mycoplasma, ureaplasma, (True) (False) (False) (False) (True)

trichomonas or meningococcal disease. These may occur together. Non-gonococcal urethritis is also part of Reiter's Syndrome (arthritis, conjunctivitis, urethritis). This can be caused by gonococcus and campylobacter. Complications of NGU: Salpingitis, perihepatitis, conjunctivitis, sterility. Treatment of NGU: Doxycyclin or Erythromycin. Copyright 2002 Dr Colin Melville

In analgesic nephropathy: A A history of renal colic is recognised. B There is an equal incidence in males and females. C There is an association with an increased incidence of urothelial transitional cell carcinoma. D There is an association with loss of ability to concentrate urine. E Diagnosis is by percutaneous renal biopsy. Comments: Analgesic nephropathy occurs secondary to prolonged relatively high exposure of the kidney to drugs, particularly analgesics such as aminopyridine, paraminosalicylate, phenacitin, phenylbutazone, nonsteroidal anti-inflammatories. It can also occur with sulphonamides and methicillin. The pathology is one of interstitial nephritis -/+ capillary necrosis, and this can result in a renal concentrating defect similar to that seen in sickle cell disease. Copyright 2002 Dr Colin Melville (False) (False) (False) (True) (True)

The following are recognised drug side effects: A Diarrhoea with Trimethoprim. B Haemolysis in G6PD deficiency with nitrofurantoin. C Benign intracranial hypertension with nitrofurantoin. D LE-like syndrome with Cephalexin. E Aseptic meningitis with Trimethoprim. Comments: Virtually all drugs can cause GI upset such as nausea, vomiting and diarrhoea. The vast majority have also been reported as causing skin rashes. Drugs with a definite risk of haemolysis in most G6PD deficient subjects include: dapsone and other sulphones, methenamine, nitrofurantoin, primaquine, quinilones (including ciprafloxacin, nalidixic acid), sulphonamides. (True) (True) (True) (True) (False)

Pseudotumour cerebri (benign intracranial hypertension) is a clinical syndrome that mimics brain tumours, and is characterised by increased intracranial pressure with a normal CSF cell count and protein content, normal ventricular size, anatomy and position. Causes include:

Metabolic disorders (galactosaemia, hypoparathyroidism, pseudohyperparathyroidism, hypophosphatasia, prolonged steroid therapy, possibly growth hormone treatment, hypervitaminosis A, Vitamin A deficiency, Addison's Disease, obesity, menarche, oral contraceptives, pregnancy). Infections (roseola infantum, chronic otitis media, mastoiditis, Guillain-Barre). Drugs (nalidixic acid, tetracyclines in addition to steroids). Haematological disorders (polycythaemia, haemolytic and iron deficiency anaemia, WiskottAldrich Syndrome). Obstruction of intracranial drainage by venous thrombosis (lateral sinus or posterior sagittal sinus thrombosis, head injury, obstruction of the superior vena cava).

Copyright 2002 Dr Colin Melville

In a 6 year old girl, the following are recognised causes of microscopic haematuria: A Paroxysmal nocturnal haemoglobinuria B Alport's Syndrome C Henoch-Schnlein purpura D Vaginal foreign body E Urinary tract infection (False) (True) (True) (True) (True)

Comments: Haematuria may be gross (visible to the naked eye) or microscopic (detected by dipstick or microscopic examination of the urine sediment). Gross haematuria may originate from the kidney (usually brown or cola coloured, containing red cell casts) or from the lower tract (red to pink clots). Gross haematuria may be associated with oedema, hypertension and renal insufficiency ("acute nephritis"). Asymptomatic microscopic haematuria is found in 1% of school age children. Causes:

Glomerular: Recurrent gross haematuria: a) IgA nephropathy, benign familial, Alport's Syndrome. b) Acute Streptococcal glomerulonephritis. c) Membranous glomerulopathy. d) SLE. e) Membranoproliferative glomerulonephritis. f) Nephritis of chronic infection. g) Rapidly progressive glomerulonephritis. h) Goodpasture Syndrome. i) Anaphylactoid purpura. j) HUS. Infection: Bacterial, TB, viral. Haematological: coagulopathies, thrombocytopenia, sickle cell disease, renal vein thrombosis. Stones and hypercalcinuria. Anatomical abnormalities: congenital anomalies, trauma, polycystic kidneys, vascular abnormalities, tumours. Exercise. Drugs.

Copyright 2002 Dr Colin Melville

The following are presentations of urinary tract infection in infancy:

A Unexplained fever B Acute diarrhoea and vomiting C Chronic diarrhoea D Urinary frequency E Febrile convulsion Comments: Clinical manifestations of urinary tract infection in infancy are protean and include:

(True) (True) (True) (True) (True)

GI: failure to thrive, weight loss, poor feeding, jaundice, diarrhoea. General: fever (low grade to acute septicaemia). There may be a palpable abdominal mass or weak urinary stream suggesting obstruction. An increased white cell count, raised creatinine, and acidosis may be found. The suprapubic aspirate or urinary catheter specimen can be used to confirm the diagnosis. Urine cultures collected by adhesive bag are only useful when negative to exclude infection. The absence of pyuria does not rule out infection. A urinary tract infection may be complicated by septicaemia or meningitis.

Copyright 2002 Dr Colin Melville

The following hereditary diseases frequently cause end stage renal disease in childhood: A Nephronophthiasis B Lesch-Nyhan Syndrome C Down's Syndrome D Cystinosis E Nail Patella Syndrome Comments: The most frequent hereditary metabolic diseases that cause end stage renal disease are: (True) (False) (False) (True) (True)

Nephronopthiasis (medullary cystic disease) Congenital Nephrotic Syndrome Alport's Syndrome Cystinosis Primary oxylosis and oxilurea Infantile and adult polycystic disease Nail Patella Syndrome.

Copyright 2002 Dr Colin Melville

Regarding the prepuce:

A Forcible retraction of non-retractile foreskin should be avoided. B Non-retractile prepuce is present in 14% of infants at birth. C May be affected by lichen planus. D Recurrent balanitis may be an indication for circumcision. E Ballooning of the prepuce is an indication for circumcision.

(True) (False) (False) (True) (False)

Comments: At birth, most foreskins are non-retractile, dropping to 8% at 6 years and 1% at 16 years. Some preputial adhesions are common in pre-school children. Indications for circumcision include:

Religious reasons. Phimosis with scarring. Recurrent balanitis. Recurrent urinary tract infection.

Copyright 2002 Dr Colin Melville

Regarding inguinal herniae in children: A They are commoner in girls. B They usually present with a persistent groin swelling. C 60% of herniaes in infants are irreducible at presentation. D They are invariably indirect hernias. E Treatment is surgical with division and closure of the processus vaginalis. Comments: Inguinal herniae are invariably indirect, due to a patent processus vaginalis. They are commoner in preterms, boys, and on the right. They present as intermittent swelling worsened by crying. 30% in infants are irreducible, at least until analgesia is given. If reduction is impossible, urgent surgery is needed, but if reduction is achieved, then surgery can be delayed for 24-48 hours to allow settling of the oedema. Division and closure of the processus repairs the defect. Copyright 2002 Dr Colin Melville (False) (False) (False) (True) (True)

The following are characteristic of renal osteodystrophy: A Elevated PTH B Alkalosis C Hypernatraemia D Hyperkalaemia E Hyperphosphataemia (True) (False) (False) (True) (True)

Comments: Renal osteodystrophy refers to the alterations in skeletal growth and remodelling occurring in chronic renal disease. These include malabsorption of calcium, phosphate retention, hyperfunction of the parathyroid glands, cutaneous vascular and visceral calcification, an impairment of renal production of Vitamin D. Renal osteodystrophy can occur with tubular dysfunction even though glomerular filtration remains intact, but usually follows progressive loss of nephrons associated with glomerular insufficiency and uraemia. The earliest sign is usually growth failure with anaemia, metabolic acidosis, protein calorie malnutrition, hormonal disorders and trace mineral deficiencies associated. In later disease, muscle weakness, bone pain and deformity, metastatic calcification and pruritis may occur with genu varum frontal bossing and dental abnormalities. Tetany is rare. X-rays of the hands and wrists show sub-periostial erosions of the middle and distal phalanges. Treatment involves controlling hyperphosphataemia, supplying adequate calcium intake, and providing extra Vitamin D. Copyright 2002 Dr Colin Melville

Concerning membranous glomerulonephritis: A It is one form renal disease in SLE. B One third of patients progress to chronic renal failure. C Complete remission is expected with steroids. D Immune complex deposition of IgG and C3 occurs in the glomerulus. E Blood C3 levels are reduced. Comments: Membranous glomerulopathy is the commonest cause of Nephrotic Syndrome in adults, but it is rare in childhood, and is a rare cause of haematuria. There is diffuse thickening of the glomerular basement membrane, and on IF there are granular deposits of IgG and C3, which on EM are shown to be on the epithelial side of the GBM. This is therefore an immune complex mediated disease. Onset is usually in the second decade of life, and the usual presentation is as Nephrotic Syndrome. Microscopic haematuria is almost invariable, with occasional gross haematuria. Blood pressure and C3 levels are normal. Possible precipitants include: SLE, cancer, gold or penicllinae therapy, syphilis and hepatitis B virus infections. Patients are at an increased risk of renal vein thrombosis. Usually, it resolves spontaneously, although some patients have persistent proteinuria. Nephrosis is controlled by salt restriction and diuretics. Copyright 2002 Dr Colin Melville (True) (False) (False) (True) (False)

In a 2 year old boy with meningomyelocele: A Prophylactic antibiotics are mandatory. B Detrusor instability is common. (True) (False)

C Proteus urinary tract infections may occur. D Intermittent catheterisation is usually necessary. E Vesico-ureteric reflux is extremely common. Comments:

(True) (True) (True)

Neurogenic bladder dysfunction is usually caused by a congenital abnormality, such as meningomyelocele, lipomeningocele, sacral agenesis, or other spinal abnormality. This can result in upper tract deterioration and urinary incontinence. The lack of co-ordination between detrusor contraction and sphincter relaxation (a brain stem function) results in functional bladder outlet obstruction, leading to high intrabladder pressures, VUR, and rapid deterioration of the upper tracts. VUR is present in 30% of neonates with myelomeningocele, and develops later in a further 20%. Urodynamic studies are necessary for accurate evaluation of each case. If the bladder is atonic or sphincters are dennovated then bladder pressure is low and VUR is unlikely. However, urinary stasis increases the chance of infection, and intermittent catheterisation (and therefore the introduction of more unusual organisms causing urinary tract infection) increases in frequency. Prophylactic antibiotics are almost always necessary. Copyright 2002 Dr Colin Melville

Recognised causes of haematuria include: A Group A Streptococcal infection B Schistosoma japonicum infection C Sickle Cell Disease D Hypocalciuria E IgA nephropathy Comments: Haematuria can be caused by non-glomerular or glomerular abnormalities: (True) (False) (True) (False) (True)

Non-glomerular: infection (bacterial, viral, TB, schistosoma haematobium), trauma (genital, urinary tract, kidneys), stones, tumours, Sickle Cell Disease, bleeding diathesis, renal vein thrombosis, hypercalcinuria. Glomerular: acute or chronic glomerular nephritis, IgA nephropathy, familial nephritis, thin basement membrane disease.

Copyright 2002 Dr Colin Melville

The following are standard treatments for vesico-ureteric reflux disease: A Ureteric reimplantation for grade I B Teflon injection for grade II C Prophylactic antibiotics for grade II (False) (False) (True)

D Intermittent catheterisation for grade II E Partial nephrectomy if a duplex system is present. Comments:

(False) (True)

Reflux of urine from the bladder to the ureter and renal pelvis results from competence of the valvular mechanism at the ureterovesical junction. This can damage the kidneys by exposing the renal pelvis to increased pressure during voiding, and facilitates the passage of bacteria from the bladder to the kidneys. The ureter can dilate and renal scars can form particularly in association with urinary tract infection. Intrarenal reflux is important in the development of the latter. Reflux nephropathy accounts for 20% of all end stage renal failure in children , and is an important cause of hypertension. Normally, the ureter has an angled path through the bladder wall. A more right angled path is associated with incompetence of the valve mechanism, and more severe reflux. Five grades are described:

I. Normal ureter calibre, partial reflux only. II. Normal ureteric calibre, reflux to renal pelvis. III. Slightly dilated ureter, clubbed calyces. IV. Markedly dilated ureter with clubbed calyces. V. Severe ureteric dilatation and tortuosity with effacement of the calyceal system.

80% of grades I and II reflux resolve spontaneously with age, and these are usually treated conservatively with prophylactic antibiotics. The chances of spontaneous disappearance decrease to 10% in grades III and IV. Grade III is often treated with Teflon injection to try to improve the performance of the valve, while reimplantation may be used for more severe grades. In the case of a duplex system, the ectopic ureter usually arises from the upper pole, and implants below the normal ureter and is invariably associated with severe reflux. If the other kidney is functioning normally, then a partial nephrectomy and excision of the ectopic ureter is performed. Copyright 2002 Dr Colin Melville

At first examination, a neonate is noted to have hypospadias: A The defect occurs in the anterior penile area in 65% of cases. B Surgery should be undertaken around puberty when penile growth is complete. C A chordee is usually present. D The incidence of hypospadias is approximately 0.3% of male infants. E Circumcision should be performed to minimise the risk of urinary tract infection. Comments: The abnormalities associated with abnormal opening of the urethral meatus include: (True) (False) (False) (True) (False)

A hooded prepuce (foreskin formed completely only on the dorsum). Chordee: ventral curvature, present only in the worst cases. The incidence is 0.3% of males, and surgery is usually undertaken before the age of 2 in order to create a terminal urethral meatus, and a straight erection.

The foreskin is essential for surgical correction, and should always be preserved. Copyright 2002 Dr Colin Melville

Useful measurements to distinguish between acute renal failure and chronic renal failure include: A X-ray of the wrist B Ultrasound scan of the kidneys C Serum phosphate level D Anthropometry E Serum potassium Comments: In acute renal failure, there may be metabolic acidosis with hyperphosphataemia and hyperkalaemia. The kidneys may be swollen, for example with renal vein thrombosis. Because of the acute onset, there is unlikely to be any changes on the x-ray and growth failure will be absent. In chronic renal failure, there may be evidence of renal osteodystrophy on x-ray, with growth failure evident on anthropometry. Ultrasound scan may show a shrunken scarred kidney with evidence of structural malformation. Serum potassium and phosphate, as in the acute case, are often elevated, and associated with acidosis, and secondary hypoparathyroidism. Copyright 2002 Dr Colin Melville (True) (True) (False) (True) (False)

The following are characteristic of Lowe Syndrome (occulocerebrorenal dystrophy): A Congenital cataracts B Hypertonia C Renal osteodystrophy D Normal development E Chorioretinitis Comments: Lowe Syndrome is a rare X-linked recessive. There is Fanconi's Syndrome, organic aciduria, heavy proteinuria. Congenital cataracts, glaucoma, buphthalmos lead to severe visual impairment. Children are hypotonic and develop severe progressive mental retardation with behavioural problems. Renal failure occurs later. Copyright 2002 Dr Colin Melville (True) (False) (True) (False) (False)

The following are usual in Potter's Syndrome:

A Beaked nose B Low-set ears C Pulmonary hypoplasia D Neonatal death E Severe talipes Comments:

(True) (True) (True) (True) (True)

Potter syndrome is caused by renal agenesis or oligohydramnios from other causes. This produces the following features: FACIAL: flat nose, recessed chin, epicanthic folds, low-set abnormal ears, limb abnormalities LUNG: pulmonary hypoplasia. Copyright 2002 Dr Colin Melville

Most patients with minimal change neuropathy have: A Normal BP B Microscopic haematuria C Poorly selective proteinuria D No change in the glomeruli on electron microscopy E Spontaneous peritonitis Comments: Idiopathic Nephrotic Syndrome is caused by: (True) (True) (False) (False) (False)

Minimal change disease (85%) - normal glomeruli, negative IF findings, retraction of epithelial foot process on EN. Mesangial proliferation (5%) - diffuse increase in mesangial cells, with IgM and C3 seen on IF. Focal sclerosis (10%) - segmental scarring in the juxtamedullary areas, often progressive. Peak incidence is 2-6 years, and is commonest in boys. Episodes may follow viral infections. There is pitting oedema particularly around the eyes and the legs, with later ascites and/or pleural effusions and declining urine output. Oedema accumulates in dependent sites. Anorexia, abdominal pain and diarrhoea are common, but hypertension is rare. There is 3 or 4+ proteinuria and microscopic haematuria may be present. Renal function is normal or reduced. Serum cholesterol and triglyceride levels are raised, and the serum albumin is usually <20g/L. C3 is normal. The mechanism of increased permeability of the glomerular capillary wall is thought to be related to the loss of negatively charged glycoproteins. Protein loss generally exceeds 2g/24 hours, and is mainly albumin (selective).

Copyright 2002 Dr Colin Melville