Nephrology

251. A 65-year-old man, who has been on haemodialysis for 12 years, presents with gradually increasing bone pain. He underwent subtotal parathyroidectomy 4 years ago. Current medications include alfacalcidol 1 g/day and calcium carbonate 500 mg three times daily. His serum calcium concentration is 2.97 mmol/l, phosphate is 2.7 mmol/l and intact parathyroid hormone level is 90 pmol/l. What is the most probable cause of his symptoms? Adynamic bone disease Dialysis amyloid Hyperparathyroid bone disease Your answer Osteoporosis Vitamin D-induced hypercalcaemia

Adynamic bone disease is increasing in prominence as a cause of skeletal pathology in patients with renal failure, especially those on peritoneal dialysis. It is characterised by a low bone-turnover state, with PTH levels usually < 8 pmol/l, absence of excess osteoid formation (distinguishing it from osteomalacia), a tendency towards hypercalcaemia and easy fracture. However, despite his previous parathyroidectomy, this patient clearly has grossly elevated PTH levels in the presence of hypercalcaemia and hyperphosphataemia. This indicates tertiary hyperparathyroidism due to hyperplasia of retained parathyroid tissue. Indeed, even after total parathyroidectomy in end-stage renal failure, only 10% of patients have a total lack of parathyroid activity postoperatively. Residual parathyroid tissue may be detected using technetium-99 sestamibi scanning.

252. A 70-year-old man with a 6-month history of malaise and back pain is admitted to A&E with severe loin pain. Physical examination is unremarkable except for pallor. An Xray of the lower abdomen shows a ureteric calculus and lytic lesions and osteoporosis of the lumbar vertebrae. Blood urea, creatinine and uric acid levels are raised. What is the most likely diagnosis? Chronic renal failure Hyperparathyroidism Myeloma Acute pyelonephritis Chronic myeloid leukaemia

Your answer

Myeloma is a plasma-cell neoplasm that produces diffuse bone marrow infiltration with a tendency to form localised osteolytic deposits. Anaemia, hypercalcaemia and raised urea, uric acid and creatinine levels are all features of this condition. Back pain is common. In chronic myeloid leukaemia, splenomegaly is often massive. Uric acid levels may be raised. Hyperparathyroidism presents with raised serum calcium levels and increased bone turnover seen as subperiosteal resorption (especially on hand X-rays). Lytic lesions do not occur in this condition. The clinical features are not suggestive of acute pyelonephritis. Hypocalcaemia is a characteristic feature of chronic renal failure. Urolithiasis does not occur in this condition.

253. A 28-year-old woman is reviewed by her GP. She had glycosuria identified on new patient screening. Her fasting blood glucose concentration was revealed to be 4.8 mmol/l (3.06.0). Body mass index (BMI) is 23 (18.524.9). Serum lipid screen, liver function testing and full blood count were normal. What diagnosis fits best with this clinical picture? Type-2 diabetes mellitus Type-1 diabetes mellitus Renal glycosuria Your answer Impaired glucose tolerance Impaired fasting glucose Glycosuria is unusual where no other urine abnormality is identified, the blood glucose concentration is normal and no other metabolic abnormalities are identified. Either there is a defect in the tubular threshold for glucose reabsorption in the proximal tubule (a so-called splayed reabsorption curve) or a defect in the maximum tubular reabsorption of glucose. The abnormality appears to run in families in some cases and both autosomal-dominant and -recessive inheritance patterns have been described. In this case, renal glucose excretion appears to have no clinical significance. However, where glycosuria is present it is important to rule out diabetes mellitus or other tubular disorders such as the Fanconis syndrome.

254. A 27-year-old Asian man presents with frequency, dysuria, haematuria and night sweats. He has lost weight over the past few months and his girlfriend noticed that he has a chronic cough. Which investigation would be most helpful in reaching a diagnosis? Plain X-ray chest Excretion urography Renal ultrasound Computed tomography Micturating cystourethrography

Your answer

Tuberculosis of the urinary tract should be kept in mind in patients presenting with frequency, dysuria, haematuria and fever, particularly in the Asian immigrant population of the UK. Excretion urography may show cavitating lesions in the renal papillary areas, commonly with calcification. There may also be evidence of ureteral obstruction with hydronephrosis. Plain X-ray of the chest may show evidence of pulmonary tuberculosis, which is usually the original focus of the infection.

255. A 52-year-old diabetic patient on metformin complains of feeling unwell and is found to have a blood glucose of 4.2 mmol/l and an anion gap of 20 mmol/l. What is the most likely cause for his illness? Hypoglycaemia Diabetic ketoacidosis Lactic acidosis Diabetic nephropathy Type-1 distal renal tubular acidosis

Your answer

This patient most probably has lactic acidosis as a side-effect of metformin therapy. His blood sugar levels are not suggestive of either hypoglycaemia or diabetic ketoacidosis. Diabetic nephropathy is unlikely to cause metabolic acidosis unless there is renal failure. In type-1 distal renal tubular acidosis the anion gap is within normal limits.

256. A middle-aged man is brought to the hospital. He appears drowsy and has altered consciousness. His blood glucose is 5.2 mmol/l, chloride 116 mmol/l, bicarbonate 15

mmol/l, sodium 131 mmol/l, potassium 3.2 mmol/l, calcium 3.6 mmol/l, phosphate 0.4 mmol/l and anion gap 3 mmol/l. What is the most probable diagnosis? Lactic acidosis Hyperparathyroidism Ethylene glycol poisoning Alcohol intoxication Salicylate poisoning

Your answer

This patient has symptoms of hypercalcaemia. Immediate treatment is mandatory in this case as his serum calcium concentration is over 3.5 mmol/l (ie 3.6 mmol/l). This is the only listed condition that can cause metabolic acidosis with a normal anion gap.

257. Following a motorcycle accident, a 30-year-old man has suffered considerable blood loss and is oliguric. In a urine sample, what finding will point to acute tubular necrosis that may not be reversed by fluid replacement? Raised urinary chloride excretion Increased urinary sodium excretion Decreased urinary urea excretion Decreased urinary osmolality Decreased urine volume

Your answer

Oliguria is common in the early stages of acute tubular necrosis (ATN). Urine osmolarity is usually low and urinary sodium is high in ATN, allowing distinction from hypovolaemic pre-renal failure. Particularly in the early stages of management, assessment of extracellular fluid volume is essential to guide fluid and electrolyte replacement. After inappropriate attempts to initiate a diuresis by infusion of normal saline without adequate monitoring of the patients volume status, pulmonary oedema due to salt and water retention is not uncommon.

258. A 10-year-old boy is hospitalised because of a recent malaena and fever

(38C). The patient also complains of arthralgia involving the knees and the ankles. On examination he has a purpuric rash involving the legs. Urinalysis discloses proteinuria with microscopic haematuria. A biopsy of the purpuric lesion reveals leucocytoclastic vasculitis in the small vessels. Which one of the following statements is true about this boys illness?

The purpuric skin rash is due to associated thrombocytopenia Active urinary sediment with RBC casts indicates glomerulonephritis Identification of anti-glomerular basement membrane antibodies is expected in half of the cases pANCA is positive in two thirds of the cases Renal granulomas are pathognomonic for this disorder Henoch-Schnlein Purpura (HSP) is recognised as a systemic small vessel vasculitis mainly involving the blood vessels of the skin, GI tract, kidneys, and joints. HSP affects mainly children between the ages of 3 10 years. Males are affected more often (1.5:1) than females and in approximately two thirds of children an upper respiratory tract infection precedes the onset of HSP by one to three weeks. The hallmark of the disease is the characteristic palpable purpura, which is seen in almost 100% of patients. It is due to inflammation of dermal blood vessels and not thrombocytopenia. Apart from raised circulating IgA the immunology profile including pANCA and anti-glomerular basement membrane antibody test is usually negative. HSP nephritis becomes clinically manifest in only 2030% of cases. It usually presents as macroscopic haematuria and proteinuria lasting days to weeks. Most glomeruli may look normal by light microscopy with only a few showing mesangial proliferation. The most consistent findings are the deposits of IgA in the mesangium. Other cases may show focal and segmental intra-capillary and extra capillary proliferation with adhesions in small crescents. Granuloma formation is not a feature of HSP.

Your answer

259. A renal biopsy from a 56-year-old woman with progressive renal failure shows glomerular and vascular deposition of a pink amorphous material. This shows apple-green birefringence under polarised light after Congo red staining. The deposits are positive for lambda light chains. What disease is she most likely to be suffering from? Rheumatoid arthritis

Tuberculosis Systemic lupus erythematosus Multiple myeloma Secondary amyloidosis

Your answer

This patient has renal amyloidosis. Lambda light chains are present in the AL type of amyloid protein. The AL type of amyloid protein occurs in multiple myeloma. Rheumatoid arthritis and tuberculosis cause secondary amyloidosis, which is associated with the AA type of amyloid protein. Systemic lupus erythematosus is not usually associated with renal amyloidosis.

260. A 55-year-old man complains of swollen feet and puffy eyelids. On examination, he is found to have a left-sided varicocele. His blood pressure is 160/120 mmHg. Which type of glomerular disease is most likely to have caused this problem? Minimal-change nephropathy Membranous glomerulonephritis Diffuse proliferative glomerulonephritis IgA nephropathy Focal segmental glomerulosclerosis

Your answer

This man most probably has a left renal vein thrombosis, which can occur as a complication of nephrotic syndrome. As the left testicular vein drains into the left renal vein, a left-sided varicocele may develop in these individuals. Renal vein thrombosis is a complication, rather than a cause, of nephritic syndrome because of the increased coagulability of blood and sluggish circulation due to hypovolaemia. It is particularly likely to complicate membranous glomerulonephritis.

261. A patient undergoing cancer chemotherapy complains of increased urinary frequency and suprapubic pain. Investigations reveal that he has microcytic, hypochromic anaemia, leukopaenia, thrombocytopaenia and haematuria. What could be the possible cause for his symptoms? Bladder metastases Transitional cell carcinoma of bladder

Cyclophosphamide therapy Your answer Urinary tract infection Bleeding diathesis Cyclophosphamide is inactive unless metabolised by the liver to 4-hydroxyl cyclophosphamide, which decomposes into alkylating species as well as to chloroacetaldehyde and acrolein. The latter causes chemical cystitis and therefore excellent hydration must be maintained during therapy with cyclophosphamide. The investigations do not suggest bladder infection while bladder metastases and bleeding diathesis are usually asymptomatic.

262. A 28-year-old woman presents to casualty with flank pain and a 48-h history of dysuria. Her past medical history includes polycystic ovarian syndrome. She is not in a steady relationship at present. There is haematuria on urine dipstick testing. Examination reveals a pyrexia of 38.1 C and flank pain. What diagnosis fits best with this clinical picture? Nephrolithiasis Pelvic inflammatory disease Ovarian cyst pain Pyelonephritis Your answer Acute glomerulonephritis The two peaks of incidence in adults for pyelonephritis occur in young sexually active women and in men > 50 years of age. Symptoms include fever, rigors, flank pain, dysuria, polyuria, haematuria, nausea and vomiting, headache and diarrhoea. Gram-negative bacilli such as Escherichia coli or Klebsiella species are responsible in more than 95% of cases. Unusual organisms may be responsible if there has been a history of urethral instrumentation. Staphylococcal urinary sepsis is usually indicative of haematological seeding of infection. Work-up includes urea and electrolytes measurement, a full blood count, blood cultures and renal ultrasound in compromised patients. In young women with a first infection, urine culture may be all that is required. Standard antibiotic treatment is with oral trimethoprim or ciprofloxacin. Surgical opinion may be required for recurrent infections or where there is evidence of vesicoureteric reflux on scanning.

263. A 6-month-old boy is found to have primary grade-V vesicoureteric reflux involving both kidneys. What would be the most appropriate management in this case? Antibiotic prophylaxis Ureteric implantation Cystoscopy followed by subureteric injection of Teflon Bilateral ureterostomies Bilateral percutaneous nephrostomies Your answer

As this boy is under 1 year of age, antibiotic prophylaxis may be given first. Grade V V V V V Age (years) <1 15 15 15 >5 Scarring No No No Yes Initial treatment Follow-up Antibiotic prophylaxis Surgery If unilateral: antibiotic prophylaxis Surgery If bilateral: surgery Surgery Surgery

Grading of vesicoureteric reflux: Grade I II III IV V Description Reflux into a non-dilated ureter Reflux into the upper collecting system without dilatation Reflux into a dilated ureter and/or blunting of calyceal fornices Reflux into a grossly dilated ureter Gross dilatation of the ureter, renal pelvis and calyces; calyces show loss of papillary impression

264. Following a road traffic accident, a man is brought to A&E. He is found to have oliguria and a diagnosis of acute renal tubular necrosis is made. What is the most common complication and cause of death in this condition? Infection Electrolyte abnormalities Nephrotic syndrome Congestive heart failure Persistent hypertension Your answer

Sepsis, particularly Gram-negative septicaemia, is the most frequent complication and cause of death in acute renal tubular necrosis while awaiting spontaneous recovery of renal function. Infections, when detected, should be treated promptly, bearing constantly in mind the need to avoid nephrotoxic drugs and to use drugs excreted by the kidneys with appropriate precautions such as alteration of the dosage and monitoring of blood levels. Neither the use of prophylactic antibiotics nor barrier nursing is considered appropriate.

265. A 26-year-old woman with epilepsy and a low IQ presents with flank pain, hypotension and anaemia. A CT scan of her abdomen shows a large haemorrhage into a mass in her right kidney, which is composed primarily of fat. There are two similar lesions (without haemorrhage) in her left kidney. Renal function is normal. What is the underlying condition? Adult polycystic kidney disease Renal-cell carcinoma Tuberous sclerosis von HippelLindau disease Xanthogranulomatous pyelonephritis

Your answer

The CT scan appearance is typical of angiomyolipoma, which when bilateral and associated with epilepsy, is highly suggestive of a diagnosis of tuberous sclerosis. It is inherited as an autosomal-dominant trait, often with incomplete penetrance. Two genetic defects have been identified, one on chromosome 9 (TSC1) and one on chromosome 16 (TSC2). Interestingly, the latter is adjacent to the PKD1 gene, which is deficient in adult polycystic kidney disease. The renal lesions are hamartomas, which tend to grow slowly over time and occasionally undergo malignant transformation. Bleeding into the angiomyolipoma is the most common means of presentation. The principal differential diagnosis is von HippelLindau disease, which is manifested by retinal and cerebellar haemangioblastomas, pancreatic cysts, phaeochromocytoma and renal cysts. The latter frequently undergo malignant transformation such that these patients often end up on dialysis because of bilateral nephrectomy.

266. A 1-year-old boy presents with a poor urinary stream since birth. Which of the following investigations would be most helpful in this case? Micturating cystourethrography Ultrasound of the bladder Excretion urography Uroflowmetry Your answer

Computed tomography A poor urinary stream suggests a urinary tract obstruction (usually infravesical). The most common cause in a male child is posterior urethral valves. The best diagnostic method is a micturating cystourethrography. The other option is endoscopy. Both these investigations would clearly show the site of obstruction.

267. A 65-year-old man with congestive heart failure who has received an excess amount of furosemide is now found to have hypochloraemic alkalosis. Which of the following investigative results would be most characteristic of this disorder? Low serum bicarbonate level Expanded extracellular fluid volume Poor response to ammonium chloride Fall in pa(CO2) Hypokalaemia

Your answer

Hypochloraemic alkalosis is associated with severe hypokalaemia and increased bicarbonate reabsorption. Potassium depletion leads to increased urinary acid excretion as ammonium, which in turn enhances bicarbonate reabsorption. There is an increase in pa(CO2). Diuretics acutely diminish the extracellular fluid volume. The alkalosis is maintained by persistence of contraction of the extracellular fluid volume and secondary hyperaldosteronism. Oral ammonium chloride may achieve acidification.

268. A 60-year-old man presents with heart failure and pedal oedema. The oedema occurs due to: Hypoalbuminaemia Increased release of renin Your answer Decreased filtration fraction Increased proximal tubular sodium reabsorption

Left heart failure

Decreased cardiac output stimulates renin release. Increased release of renin from juxtaglomerular cells in the kidney leads to the release of angiotensin I from angiotensinogen. This is converted to angiotensin II which is a potent vasoconstrictor and stimulant for aldosterone secretion. The major target of aldosterone is the distal tubult of the kidney, where it stimulates exchange of sodium and potassium. This results in three primary physiologic effects:

Increased resorption of sodium: sodium loss in urine is decreased under aldosterone stimulation. Increased resorption of water: with consequent expansion of extracellular fluid volume. This is an osmotic effect directly related to increased resorption of sodium. Increased renal excretion of potassium.

Activation of the renin-angiotensin system as a result of decreased renal blood flow (usually due to decreased vascular volume) results in release of angiotensin II, which stimulates aldosterone secretion. There is an increased filtration fraction caused by renal vasoconstriction in response to the diminished cardiac output. Right heart failure is associated with peripheral oedema, which affects the ankles in ambulant patients.

269. You are asked to see the relatives of a 68-year-old man who has undergone surgery for repair of a ruptured aortic aneurysm. Prior to surgery he was well, with a creatinine of 119 micromol/l at baseline. Although the operative procedure went well, the period of acute hypotension has resulted in an episode of acute tubular necrosis (ATN) and he has undergone renal dialysis on a number of occasions in the past week. Which of the following most accurately represents the chance of recovery of renal function to the level where dialysis is not required? 0% 95% 5% 50% 25%

Your answer

ATN usually occurs after an acute ischaemic or toxic event, in this case a period of severe hypotension resulting from the ruptured aneurysm. Only around 5% of patients require long-term renal replacement therapy. Around 50% have some impairment of renal function

on a permanent basis; around another 5% undergo a slow deterioration in their renal function. Prognosis of ATN is poorer in patients with severe sepsis or trauma, where mortality rates can approach 5060%. Infections are the leading cause of death in acute renal failure.

270. A 33-year-old woman presents with polydipsia and polyuria. These symptoms started soon after a road traffic accident 6 months ago. Her blood pressure is 120/80 mmHg with no postural drop. The daily urine output is 68 litres. Blood tests show: sodium 130 mmol/l, potassium 3.5 mmol/l, urea 5.5 mmol/l and glucose 4.4 mmol/l. The plasma osmolality is 268 mOsmol/l and urine osmolality is 45 mOsmol/l. What is the most likely diagnosis? Central diabetes insipidus Nephrogenic diabetes insipidus Diuretic phase of acute tubular necrosis Primary polydipsia SIADH

Your answer

Primary or hysterical polydipsia is a psychiatric disturbance that causes a fall in plasma sodium and osmolality and a concomitant low urine osmolality. Diabetes insipidus, whether central or nephrogenic, causes an increase in plasma osmolality and serum sodium, whereas both are decreased in this case. During the resolving or diuretic phase of acute tubular necrosis, serum sodium is increased. In the syndrome of inappropriate antidiuretic hormone (SIADH) secretion, there is low plasma osmolality with inappropriate urine osmolality, which is higher than plasma osmolality.

271. The plasma biochemistry of a patient presenting with severe right flank pain is as follows: sodium 135 mmol/l, potassium 2.5 mmol/l, urea 3.5 mmol/l, chloride 115 mmol/l, bicarbonate 15 mmol/l. The urinary pH is 6.5. What is the most probable diagnosis in this case? Type-1 renal tubular acidosis Type-2 renal tubular acidosis Type-4 renal tubular acidosis Chronic renal failure Ureteric stone Your answer

The presence of acidosis, hypokalaemia, low serum bicarbonate levels and relatively high urinary pH are characteristic of distal renal tubular acidosis. The problem here is an inability to maximally acidify the urine. Typically urine pH remains > 5.5 despite severe acidaemia ([HCO3] < 15 mmol/l). Some patients with less severe acidosis require acid loading tests (eg with NH4Cl) to assist in the diagnosis. If the acid load drops the plasma [HCO3] but the urine pH remains > 5.5, this establishes the diagnosis.Renal stone formation and nephrocalcinosis are common in this condition.

272. A 34-year-old woman presents with vomiting, malaise and weakness 5 weeks after delivering a healthy male infant. The pregnancy had been uncomplicated with no hypertension evident. Her blood pressure is now 210/110 mmHg, plasma creatinine is 650 mol/l and potassium is 6.9 mmol/l. Haemoglobin is 7.6 g/dl, associated with a platelet count of 65 109/l, reticulocytes of 7%, normal clotting indices and lactate dehydrogenase levels of 800 IU/l. Which of the following most accurately describes the pathological process? Crescentic glomerulonephritis Disseminated intravascular coagulation Malignant hypertension Pre-eclampsia Thrombotic microangiopathy

Your answer

The history is typical of postpartum haemolyticuraemic syndrome (HUS), which usually occurs 46 weeks postpartum and is characterised by microangiopathic haemolytic anaemia, severe irreversible renal failure and hypertension. Schistocytes are evident on the blood film but the clotting indices are normal, the latter helping to distinguish it from the HELLP (haemolysiselevated liver enzymeslow platelets) syndrome, which is far more common. The differential diagnosis of acute renal failure in the setting of pregnancy includes postpartum HUS, thrombotic thrombocytopenic purpura (which occurs invariably antepartum and usually before 24 weeks, and is associated with relatively mild renal failure) and the HELLP syndrome (which is usually preceded by a history of pre-eclampsia and is characterised by evidence of disseminated intravascular coagulation). All may be complicated by acute cortical necrosis, resulting in oligoanuria, haematuria and flank pain. In cases of postpartum HUS it is important to consider the possibility of retained products of conception.

273. A 32-year-old man is referred to the renal clinic by his GP after a second episode of gross haematuria. Past history of note includes coeliac disease. On both occasions the haematuria appears to have been closely associated with an upper respiratory tract infection. Blood pressure is 125/80 mmHg. Light

microscopy of a renal biopsy specimen reveals diffuse mesangial proliferation and extracellular matrix expansion. IgA deposits are seen on immunofluorescence. Which of the following diagnoses fits best with this clinical picture? Alports syndrome Lupus nephritis IgA nephropathy Your answer Goodpastures syndrome Wegeners granulomatosis This patient gives a typical clinical picture of episodic haematuria associated with respiratory tract infection, and the light microscopy result is in keeping with IgA nephropathy, sometimes known as Bergers disease. IgA nephropathy is seen in up to one-third of patients with gluten enteropathy and is also associated with cirrhosis, HIV infection and has a familial form. As well as presenting with episodic gross haematuria, other presentations include nephrotic syndrome with proteinuria and acute renal failure. Chronic renal failure occurs in up to 2% of patients per year who have IgA nephropathy. Medical treatment of IgA nephropathy includes angiotensin-converting enzyme (ACE) inhibition which as been shown to delay progression to renal failure, and use of prednisolone in patients with severe disease may also be of benefit.

274. An 80-year-old man with a long history of poor urinary stream and nocturia presented with malaise and vomiting. His plasma creatinine concentration was 1242 mol/l and his urea 65 mmol/l. Renal ultrasound showed gross bilateral hydronephrosis with thin cortices. A urinary catheter was placed and he passed 7.6 litres of urine over the next 24 hours, during which time he received no supplemental iv fluid. His blood pressure is now 80/50 mmHg, he has no oedema and his neck veins are flat. His serum Na+ is 150 mmol/l and urine osmolality is

198 mOsm/l. Which of the following most accurately describes the hormonal milieu? High ADH, high aldosterone, high renin Your answer High ADH, high aldosterone, low renin High ADH, low aldosterone, low renin Low ADH, high aldosterone, high renin Low ADH, low aldosterone, high renin In the setting of long-standing urinary tract obstruction, as evidenced by the severity of uraemia and thin cortices, the ability of the kidney to concentrate urine becomes severely impaired, resulting in partial nephrogenic diabetes insipidus. Much of the polyuria that occurs after urinary tract obstruction is relieved is an appropriate attempt by the kidneys to deal with salt and water overload. However, a rise in serum osmolality to > 300 mOsm/l, as in this case, in the presence of a low urine osmolality indicates that the polyuria is pathological. The ability of the kidney to conserve Na+ is also impaired, as the patient became clinically volume-depleted. Therefore, one can infer that there is severe distal tubular damage with effective unresponsiveness to both ADH and aldosterone. Hypovolaemia is a potent stimulus for renin production from the juxtaglomerular apparatus and hypernatraemia is a potent stimulus for ADH production from the posterior pituitary. As there is nothing in this case to suggest adrenal disease, secondary hyperaldosteronism would be present. Obviously, this situation should never be allowed to occur in practice. A reasonable approach in postobstructive diuresis is to replace half the volume of urine with 0.45% saline or alternating dextrose 5% and normal saline, with frequent assessment of fluid status. Replacing urine ml for ml, or even output minus 30 ml/h, prolongs the duration of diuresis.

275. A 22-year-old student commences chemotherapy for B-type acute lymphoblastic leukaemia. She suffers from vomiting, but 36 h later her condition worsens and her bloods reveal a corrected calcium of 2.0 mmol/l and serum potassium of 6.7 mmol/l. Which of the following options is the best way to avoid this problem from occurring?

Pretreatment with furosemide therapy Adequate hydration pre-chemotherapy Allopurinol pre-chemotherapy Hydration and allopurinol pre-chemotherapy Furosemide and allopurinol pre-chemotherapy

Your answer

This is the tumour-lysis syndrome, which occurs rapidly after the commencement of chemotherapy due to necrosis of leukaemic cells. There is rapid electrolyte derangement with hypocalcaemia, hyperphosphataemia and hyperkalaemia leading to a life-threatening situation, for which the only treatment is often haemodialysis. The rapid electrolyte changes exceed the rate of renal clearance. The risk of developing the tumour-lysis syndrome can be reduced by prehydration and treatment with allopurinol to reduce serum urate levels. Furosemide may precipitate dehydration and actually increase the risk of tumour-lysis syndrome.

276. A 52-year-old woman with a history of recurrent urinary tract infection presents with a 3-week history of increasing left flank pain, fever, anorexia and weight loss. On examination a left-sided abdominal mass is palpable. Her white cell count is 17.5 109/l, Hb is 8.1 g/dl and plasma creatinine is 190 mol/l. There is a significant growth of Klebsiella spp in her urine. What is the probable diagnosis? Acute pyelonephritis Renal abscess Renal-cell carcinoma Transitional-cell carcinoma Xanthogranulomatous pyelonephritis Your answer This is the typical presentation of xanthogranulomatous pyelonephritis (XPN), which, although rare, is increasing in incidence. It develops as an abnormal macrophage response to infection, particularly in the presence of urinary tract

obstruction, and is pathologically related to malacoplakia. A flank mass is usually palpable, thereby distinguishing it from simple acute pyelonephritis or renal abscess, and occasionally mimicking renal cancer. The disease is almost invariably unilateral. Patients with XGP often appear chronically ill. Symptoms include anorexia, fevers, weight loss, and flank pain. The relatively rapid history, leucocytosis, renal impairment and positive urine culture make XPN much more probable than cancer. The course may also be indolent and protracted, extending over months or years. In these cases, AA amyloid may develop, resulting in the onset of nephrotic syndrome. CT scan is the investigation of choice to confirm the diagnosis. It will show the replacement of renal parenchyma by rounded, low-density areas surrounded by a ring of enhancement, and it establishes the extent of the lesion (which may involve surrounding structures).

277. A 23-year-old man has had recurrent attacks of hepatitis. He now presents with bone pain, muscle weakness, polyuria and polydipsia. Slit-lamp examination shows a greenish-brown discoloration at the corneoscleral junctions. What is the most common association with this condition? Hyperkalaemia Metabolic alkalosis Hypophosphataemia Your answer Increased serum bicarbonate Hypercalcaemia

This patient has Wilsons disease, a rare inborn error of copper metabolism. The condition can cause type-2 proximal renal tubular acidosis (RTA). The cardinal features are acidosis, hypokalaemia, and hypophosphataemia. An inability to lower the urine pH below 5.5 despite systemic acidosis and the appearance of bicarbonate in the urine despite subnormal plasma bicarbonate helps differentiate between RTA type 1 and type 2. If the urine can be acidified, then this is more suggestive of Type 2.

278. An 18-year-old man was admitted to the emergency department having collapsed with occipital headache. A scan revealed a massive subarachnoid haemorrhage. Further investigation during the following days revealed multiple bilateral renal cysts. A defect on which chromosome is likely to be responsible for this clinical syndrome? Chromosome 16 Your answer Chromosome 4 Chromosome 12 Chromosome 5 Chromosome 3 This is one presentation of autosomal-dominant polycystic kidney disease, characterised by the development of multiple renal cysts and cardiovascular abnormalities. It is the commonest inherited nephropathy, with a prevalence rate of between 1:400 and 1:1000. It accounts for 310% of patients undergoing regular dialysis in the UK. Symptoms may include loin pain due to a cyst haemorrhage or infection, abdominal discomfort due to increasing kidney size or presentation with extrarenal manifestations such as subarachnoid haemorrhage, as in this case. Other presentations may include the complications of hypertension or complications of associated liver cysts. The commonest chromosomal defect is on chromosome 16 (85%); a second gene defect has been identified on chromosome 4, which is responsible for the majority of other cases.

279. A 45-year-old woman with type 2 diabetes presents for review. She currently takes metformin and gliclazide and has an Hb A1c of 6.8%. Blood pressure is 142/82 mmHg on no antihypertensive medication. Total cholesterol is 5.2 mmol/l, but high-density lipoprotein (HDL) level is only 0.8 mmol/l; serum creatinine is 92 mol/l. Urine microalbumin stix are positive. Which one of the following would be the most appropriate intervention in this case? Stop metformin Stop gliclazide Start atorvastatin 10 mg daily Your answer Transition to insulin therapy

Start bendroflumethiazide 2.5 mg This patient has microalbuminuria, which puts her in a patient group at high risk of cardiovascular complications. The CARDS study of atorvastatin 10 mg in patients with type 2 diabetes and average cholesterol has demonstrated benefit in cardiovascular event reduction. Additionally, the HOPE study and a number of similar studies such as LIFE, showed the benefit of blockade of the renin angiotensin system in reducing both cardiovascular events and renal progression. Hence it would be correct to begin atorvastatin and also ramipril. Her Hb A1c is already near target and there would be no additional benefit in converting her to insulin therapy. The treatment of microalbuminuria in type 1 diabetes where the blood pressure is normal is a source of some debate. The consensus at the moment is that even this patient group may benefit from angiotensin-converting enzyme (ACE) inhibitor or angiotensin-receptor blockers (ARB) treatment.

280. A 12-year-old boy is referred for evaluation of nocturnal enuresis and short stature. His blood pressure is normal. Blood urea is 14 mmol/l, serum creatinine 145 mol/l, sodium 120 mmol/l, potassium 4.2 mmol/l, phosphate 2.5 mmol/l and alkaline phosphatase 400 U/l. Urinalysis shows trace proteinuria with hyaline casts. Ultrasound shows bilateral small kidneys. A micturating cystourethrogram is normal. What is the most likely diagnosis? Alports syndrome Medullary sponge kidney Chronic glomerulonephritis Nephronophthisis Cystinosis

Your answer

Nephronophthisis is an autosomal-recessive disorder. The clinical features and investigations are suggestive of this disorder. Alports syndrome presents with microscopic haematuria, sensorineural hearing loss and bilateral lenticonus. In medullary sponge kidney, the kidneys are normal or increased in size. The age of presentation is usually in the third or fourth decade. Patients with chronic glomerulonephritis usually have heavy proteinuria, frank or occult haematuria and hypertension. Cystinosis presents in the first year of life with failure to thrive. This results from renal tubular defects caused by cystine deposits in the kidney. Renal damage progresses and visual impairment occurs as a result of cystine deposits in the retina and cornea.

281. A 14-year-old boy presents with proteinuria, haematuria and nephrotic syndrome. Blood testing shows the presence of circulating immune complexes. Which of the following disorders is he most likely to be suffering from? Goodpastures syndrome Pernicious anaemia Allergic rhinitis Minimal-change glomerulonephritis Mesangiocapillary glomerulonephritis Your answer Circulating immune complexes are seen in mesangiocapillary (membranoproliferative) glomerulonephritis. Antiglomerular basement membrane antibodies that bind mainly to the non-collagenous domain of the a-3 chain of type-IV collagen cause Goodpastures syndrome. Parietal cell and intrinsic factor antibodies are present in pernicious anaemia. These act locally on the parietal cells and inhibit the production and action of intrinsic factor. In minimal-change nephropathy, there are no immune complexes. The pathogenesis is unclear, but it may be possible that a factor produced by T lymphocytes increases the glomerular permeability to protein. Allergic rhinitis is, again, a local interaction between the inhaled allergen and adjacent molecules of the IgE antibody present on the surface of mast cells found in the nasal secretions and nasal epithelium.

282. A 62-year-old woman has a history of chronic otitis media and mastoiditis over the past few months. There have been one or two episodes of haemoptysis. She smokes 30 cigarettes per day. Urinalysis reveals haematuria, proteinuria and red-cell casts. Chest Xray reveals cavitating mass lesions and evidence of multiple nodules with diffuse alveolar changes. Urea and creatinine are both markedly raised. ESR and rheumatoid factor are elevated. What diagnosis fits best with this clinical picture? Rheumatoid arthritis with typical lung changes Tuberculosis Bronchial carcinoma Wegeners granulomatosis Your answer Goodpastures syndrome She has evidence of chronic upper respiratory tract changes associated with Wegeners granulomatosis, these may include sinusitis, otitis, mastoiditis, nasal crusting, epistaxis and eventually saddle-nose deformity. Lung changes may include multiple nodules and cavitating lesions with diffuse alveolar changes. Renal changes include a focal glomerulonephritis, associated with necrotising glomerular lesions. c-ANCA positivity is the

confirmatory immunology test for Wegeners granulomatosis. Management is with iv methylprednisolone and cyclophosphamide therapy. Renal function may deteriorate to the stage where renal replacement therapy is required. Unfortunately, in these cases some residual renal insufficiency after recovery is common.

283. A 56-year-old diabetic man came in for acute breathlessness in the middle of the night. There was no previous history of retinopathy. Chest X-ray revealed evidence of pulmonary oedema. His HbA1c is 8.4%, and his blood pressure (BP) was 180/112mmHg. Investigation: creatinine (Cr) 251 mol/l, urine dipstick protein 1+, blood 1+. Which of the following would best explain the above findings? Chronic glomerulonephritis Renal artery stenosis Nephrotic syndrome Renal vein thrombosis Acute urinary tract infection Your answer

Diabetic nephropathy does not normally present with microscopic haematuria as well as proteinuria, hence the combination of haematuria and proteinuria suggests glomerulonephritis as a possible cause. In the absence of retinopathy, the presence of haematuria and proteinuria is strongly predictive of a non-diabetic cause (positive predictive value 94%). Assessment of aetiology involves urine microscopy for red-cell casts, assessment of renal function by estimation of creatinine clearance and quantification of proteinuria and renal imaging, initially by excretion urography. Renal biopsy may be useful to further elucidate the underlying cause. Crucial to the management of this patient is adequate control of blood pressure. Presentation with pulmonary oedema is secondary to cardiac failure related to hypertension.