Nephrology

201. A 72-year-old man with acute myelogenous leukaemia on doxorubicin and cytarabine presents with severe left flank pain and oliguria. An abdominal radiograph shows a left ureteric calculus. What is the most probable cause for this problem? Doxorubicin toxicity Exacerbation of AML Hyperuricaemia Hypercalcaemia Hyperphosphataemia

Your answer

The patient has most probably developed acute hyperuricaemic nephropathy. This causes renal failure with marked hyperuricaemia that follows on commencement of chemotherapy, when there is rapid lysis of malignant cells, release of large amounts of nucleoprotein and increased uric acid production. Uric acid crystals may deposit in the collecting ducts, pelvis and ureters. Ultrasound may demonstrate extrarenal obstruction due to stones. Hyperphosphataemia may also occur due to tumour lysis. However, it does not cause renal failure nor does it form stones.

202. A 64-year-old patient in end-stage renal disease wonders whether he is a suitable candidate for renal transplantation. He asks you, as his doctor, for advice. In which of the following conditions is renal transplantation advisable? Generalised bronchiectasis Primary oxalosis Severe diabetes mellitus Congestive heart failure Bilateral polycystic kidney disease

Your answer

Bilateral polycystic kidney disease is the only one in the list where a renal transplant is advisable. In primary oxalosis and diabetes mellitus, there is a risk of disease recurrence. Vascular disease in diabetes may render anastomoses difficult and compromise blood flow to the lower limb. Both generalised bronchiectasis and congestive heart failure are likely to limit survival and the degree of rehabilitation after transplantation. 203. A 30-year-old man complains of weakness in his upper limbs and has digital infarcts involving the middle and ring fingers of his right hand. On examination his blood pressure is 160/140 mmHg. Investigations show Hb 10.0 g/dl, WCC 14 109/l, platelets 450 109/l,

ESR 69 mm/1st h. Urinalysis shows proteinuria and microscopic haematuria with no casts. What is the most likely diagnosis? Polyarteritis nodosa Your answer Systemic lupus erythematosus Wegeners granulomatosis Polymyositis Cryoglobulinaemic renal disease This patient has polyarteritis nodosa. Renal and visceral arterioles are most often affected. In Wegeners granulomatosis, symptoms of respiratory tract involvement (otitis media, sinusitis and pulmonary) are seen in 95% of cases. Systemic lupus erythematosus is much more common in women and may present with fever, malaise, myalgia and Raynauds phenomenon. The full blood count usually shows leucopenia and/or thrombocytopenia. It may cause glomerulonephritis, in which case there would be red cell casts in the urine. Renal involvement is uncommon in polymyositis. Cryoglobulinaemic renal disease usually presents in the fourth or fifth decades of life, and women are more frequently affected. Systemic features include purpura, arthralgia, leg ulcers, Raynauds phenomenon, polyneuropathy and hepatic involvement.

204. A 72-year-old psychiatric patient is admitted to casualty complaining of thirst. He has with him a large number of shopping bags and has spent a considerable amount of money. Biochemical blood testing reveals a urea of 21.0 mmol/l, creatinine of 185 mol/l and a sodium of 146 mmol/l. Urinary osmolality is low. What diagnosis fits best with this clinical picture? Chronic reflux nephropathy Cranial diabetes insipidus Nephrogenic diabetes insipidus Your answer Syndrome of inappropriate ADH secretion Psychogenic polydipsia Nephrogenic diabetes insipidus may be a long-term consequence of lithium therapy due to direct toxicity. He appears hypomanic judging by his extreme spending, and lithium is therefore his most likely maintenance medication. Some improvement may be seen by switching his lithium to an alternative maintenance drug for manic depression, but this should only be considered after consultation with a psychiatrist. Alternatives include lamotrigine and valproate. Diagnosis is made on low urine osmolality (< 200 mOsm/kg) in the presence of raised serum osmolality, hypernatraemia and evidence of dehydration. Treatment centres around adequate hydration, reduced sodium. Patients with lithium-related DI may also benefit from taking amiloride 510 mg/day. Even after lithium withdrawal there may be some permanent impairment of renal concentrating function. Nephrogenic diabetes insipidus may also be inherited as a sex-linked recessive condition.

205. A 45-year-old man had recurrent nephrolithiasis. Renal function tests and serum calcium measurements were normal. A 24 hour urine collection revealed (normal range in brackets):

Volume 3 litres Calcium 15 mmol/24 hours (2.57.5) Oxalate 200 mmol/24 hours (90450) Uric acid 3 mmol/24 hours (1.484.45) Citrate 2 mmol/24 hours(0.33.4)

What is the most useful therapy to reduce stone formation? Allopurinol Dietary calcium restriction Penicillamine Potassium citrate Thiazide diuretic

Your answer

This unfortunate man is likely to be suffering from idiopathic hypercalciuria. Severe dietary restriction of calcium is an ineffective treatment as this in itself leads to hyperabsorption of oxalate. The advice of a specialist dietician is advised if patients are to undertake dietary measures. Key advice is to maintain a high fluid intake, and where patients live in a hard water area they may benefit from switching to drinking softened water. Supplements of vitamin D should obviously be avoided. Thiazide diuretics are the drug treatment of choice as they act directly on the renal tubule to reduce urinary calcium excretion.

206. An asymptomatic 8-year-old boy during a routine medical examination is found to have bacteriuria > 105/ml. What is the most appropriate initial management in this case? Antibiotic treatment No treatment Prophylactic antibiotics for 3 months Alkalinise the urine with sodium bicarbonate Excretion urography Your answer

The presence of a bacterial count of more than 105/ml indicates the need for antibiotic treatment. In all men and children, excretion urography should be carried out following a

first proven episode of bacteriuria to identify complicating factors. However, in women, excretion urography is indicated only if there are further attacks or if post-treatment urinalysis is abnormal.

207. A 62-year-old woman visits the diabetes clinic for review. She has had type-2 diabetes for 9 years and is now on insulin therapy. She has diabetic nephropathy, as exemplified by hypertension and proteinuria; a recent creatinine level was 205 mol/l. Which of the following options best fits her prognosis or management? If she has the DD-ACE phenotype it may be associated with a more rapid Your decline in renal function answer Intensive glucose control will not affect prognosis Tight blood pressure control is unlikely to affect prognosis Her systolic BP target should be 140 mmHg Non-dihydropyridine calcium antagonists have no effect on proteinuria The UKPDS showed positive effects for tight blood pressure and glucose control, in terms of slowing the slope of decline in renal function in diabetic nephropathy. It is suggested that the target for systolic blood pressure should be 130 mmHg or lower. (Angiotensin-converting enzyme (ACE) inhibitors or A2RBs (angiotensin 2-receptor blockers) are the treatment of choice for hypertension in diabetic nephropathy, but non-dihydropyridine calcium antagonists, eg diltiazem, may have additional effects in reducing proteinuria. Cross-sectional studies in type-2 diabetes suggest a prevalence of microalbuminuria of between 15 and 50% depending on ethnicity. There is a strong association between nephropathy and markers of increased cardiovascular risk such as insulin resistance, dyslipidaemia, left ventricular hypertrophy, endothelial dysfunction and absence of nocturnal blood pressure dipping. The DD-ACE phenotype is thought to be associated with raised circulating levels of angiotensin-2 and a more rapid decline in renal function.

208. Chronic renal failure is an irreversible and usually long-standing loss of renal function. Of the following, which condition is most likely to cause chronic renal failure? Leptospirosis Haemolytic Uraemic syndrome

Vancomycin treatment Myoglobinuria IgA nephropathy Your answer All the other conditions cause acute renal failure. Bergers disease (IgA nephropathy) is associated with chronic renal failure in up to 30% of adult cases. It usually follows a viral upper respiratory tract infection. Heavy proteinuria, renal failure and hypertension indicate a poor prognosis. Leptospirosis, also called Weil's disease, is spread by contact with infected rat urine. Features include meningitis, myocarditis, acute renal failure and haemolytic anaemia. Acute renal failure follows muscle trauma or necrosis due to the appearance of myoglobin, which may cause tubular damage or blockage. Vancomycin is a bactericidal antibiotic. Indications are limited to serious infections such as endocarditis and septicaemia. It is nephrotoxic and can precipitate acute renal failure. Haemolytic uraemic syndrome is associated with acute damage and occlusion of small blood vessels (arterioles and capillaries) in the kidney. Thrombotic microangiopathy associated with Escherichia coli infection (especially O157 serotypes) is now the most common cause of acute renal failure in children in developed countries.

209. A 28-year-old woman presents with painless lymphadenopathy in her neck region. She has had fevers and night sweats intermittently over the past few weeks, weight loss and generalised malaise. She has abandoned her Friday nights out after work due to abdominal pain after drinking alcohol. On examination there is neck lymphadenopathy, abdominal fullness (which may be ascites) and peripheral oedema. Routine initial bloods reveal a decreased serum albumin concentration, and a 24-h urine collection reveals a protein excretion of 4.5 g over a 24-h period (< 0.2 g/24 h). Initial renal biopsy is unremarkable. What is the most likely cause underlying her renal pathology? Membranous glomerulonephritis Sarcoidosis Polyarteritis nodosa Minimal-change disease Your answer Membranoproliferative glomerulonephritis This woman has evidence of possible Hodgkins disease, given her history of painless

lymphadenopathy, night sweats and pain on drinking alcohol. Her renal biopsy is unremarkable, ruling out membranous glomerulonephritis as a cause. Minimal-change disease is associated with Hodgkins disease and is also the commonest cause of nephrotic syndrome in children; other associations include non-steroidal anti-inflammatory agents. Membranous glomerulonephritis is the commonest cause in adults and is associated with Hodgkins disease, carcinomatous disease, gold therapy and systemic lupus erythematosus (SLE). Diagnostic work-up includes renal biopsy, which appears normal in minimal-change disease, 24-h urine collection and urinary examination for oval fat bodies (tubular epithelial cells containing cholesterol esters). Minimal-change disease in this case is likely to improve when chemotherapy for the Hodgkins disease is commenced; otherwise there is usually a response to prednisolone at a dose of 1 mg/kg per day. The response to treatment of membranous glomerulonephritis is generally poorer.

210. A young man presents with dysuria and urethral discharge. Gram stain shows numerous pus cells but no microorganisms. The culture is negative on routine laboratory media. What is the most likely causative agent? Chlamydia trachomatis Haemophilus ducreyi Treponema pallidum Neisseria gonorrhoeae Bacteroides species Your answer

Chlamydia trachomatis is a Gram-negative organism that is too small to be visualised on staining. This is an obligate intracellular parasite and hence cannot grow on any cell-free media. All the other organisms may be identified on light microscopy.

211. A 15-year-old boy presents with dark discolouration of urine. There is a history of upper respiratory tract infection two weeks earlier. If untreated, he may go on to develop: Nephrotic syndrome Immune deficiency with normal complement (C3) levels

Acute renal failure Fits or a stroke Your answer Nephroblastoma Bacterial infections, usually subacute, may cause a variety of histological patterns of glomerulonephritis, but usually with plentiful immunoglobulin deposition and often with evidence of complement consumption (low C3). A low C3 level is indicative of poor prognosis in nephrotic syndrome, but is common in glomerulonephritis. Renal failure occurs in less than 1% of cases of poststreptococcal nephritis. Hypertensive encephalopathy may occur and result in fits, coma and neurological events like stroke.

212. A 26-year-old man presents with bilateral loin pain and frank haematuria. He has had a sore throat for the preceding 24 hours. His BP is 138/78 and urinalysis reveals RC ++++, protein +. What is the most likely cause? IgA nephropathy Microscopic polyarteritis Nephrolithiasis Post-streptococcal glomerulonephritis Septicaemia Your answer

IgA nephropathy commonly present in young males in the first few days of, or after, an upper respiratory tract infection. Recurrent frank haematuria is a common presentation and appreciable haematuria will often be accompanied by a small amount of proteinuria. Loin pain may occur in IgA nephropathy due to renal capsular swelling. Post-streptococcal glomerulonephritis occurs 10-21 days after streptococcal infection and commonly presents with the nephritic syndrome of haematuria, hypertension, oedema and oliguria. Microscopic polyarteritis is one of the vasculitides usually presenting with acute renal failure, pulmonary involvement and a vasculitic, purpuric rash. Nephrolithiasis may present with haematuria and loin pain. The loin pain may be the predominant feature as renal colic which is often unilateral.

213. A 2-year-old boy has vitamin D-resistant rickets. Investigations show: serum calcium 2.6 mmol/l, phosphate 0.5 mmol/l and alkaline phosphatase 1040 U/l. Parathyroid hormone and bicarbonate levels are normal. What is the most probable diagnosis? Distal renal tubular acidosis Hypophosphataemic rickets Vitamin D-dependent rickets Proximal renal tubular acidosis Hyperparathyroidism Disorder Hypophosphataemic rickets Vitamin D-dependent rickets Hyperparathyroidism Nutritional rickets Serum calcium Normal or decreased Decreased Increased Decreased Serum phosphate Decreased Normal or decreased Decreased Decreased

Your answer

Alkaline phosphatase Increased Increased Increased Increased

Parathyroid hormone Normal Increased Increased Increased

Proximal tubular acidosis also causes hypophosphataemic rickets but the bicarbonate level will be reduced. Distal tubular acidosis causes vitamin D-dependent rickets.

214. A young man presents with swelling of his face and feet, haematuria and proteinuria. He had measles 6 weeks earlier from which he had recovered uneventfully. Renal biopsy shows mesangial cell proliferation with electron-dense, linear intramembranous deposits that stain for C3 only. What is the most probable diagnosis? Focal glomerulonephritis Membranoproliferative glomerulonephritis Minimal-change nephropathy Nodular sclerosis Proliferative glomerulonephritis

Your answer

The features are highly suggestive of type-2 mesangiocapillary (membranoproliferative) glomerulonephritis. This type may be idiopathic or may occur after measles. Nephrotic syndrome or renal failure may occur in this condition.

215. A 75-year-old man visits his GP for a new-patient screen. His only previous complaints have been type-2 diabetes and mild long-standing back pain. Screening tests reveal an elevated serum creatinine of 215 mol/l, and anaemia with Hb of 10.1 g/dl. A

back X-ray shows collapse of the lumbar spine and there is a monoclonal band on serum protein electrophoresis. What is the most likely cause of his deteriorated renal function? Diabetic nephropathy Membranous glomerulonephritis Metformin toxicity Amyloidosis Your answer Urinary tract infection Amyloidosis presents predominantly between the ages of 60 and 70 years. The cause in this case is likely to be related to light-chain deposition from a myeloma, exemplified by the monoclonal banding on electrophoresis and lumbar spine collapse. Symptoms of generalised amyloidosis include fatigue and dyspnoea, diarrhoea, macroglossia, hepatomegaly and weight loss. Cardiac involvement with amyloid may lead to a restrictive picture with rightsided heart failure and jugular venous distension. In this case, renal amyloidosis has precipitated development of the nephrotic syndrome. Other causes of amyloid, apart from myeloma, are hereditary forms of amyloidosis including familial Mediterranean fever, and those related to chronic disease, eg rheumatoid arthritis. Amyloidosis associated with myeloma carries a very poor prognosis (often less than 1 year), familial forms are associated with much better outcomes, with a prognosis up to 1015 years.

216. A 68-year-old man has backache and hypercalcaemia, plasma globulins are elevated at 52 g/l and he has a normocytic anaemia. His 24- hour urinary protein excretion is 0.5 grams. He develops diarrhoea and vomiting and presents with acute renal failure. A renal biopsy is performed. What is the most likely diagnosis? Acute tubular necrosis Amyloidosis Interstitial nephritis Intraglomerular thrombi Light chain nephropathy

Your answer

The history is typical of myeloma. Amyloidosis would be heralded by more significant proteinuria (often nephrotic range). Intratubular casts, composed of light chain, can crystallise within the tubule leading to oliguric acute renal failure. This tendency would be exacerbated by hypovolaemia due to a gastroenteric illness.

217. A 68-year-old diabetic patient complains of feeling unwell. Investigations reveal that he has developed dilutional hyponatraemia. Which medication is most likely to cause this complication? Chlorpropamide Metformin Acarbose Rosiglitazone Glimepiride Your answer

Sulphonylureas, especially chlorpropamide, induce hyponatraemia by potentiating the effects of antidiuretic hormone on the renal collecting ducts. This complication occurs in 5% of patients on chlorpropamide. Metformin may cause lactic acidosis in patients with severe hepatic or renal disease. Gastrointestinal disturbances, such as abdominal discomfort, flatulence and diarrhoea, are seen with acarbose. Rosiglitazone and other thiazolidinediones tend to cause weight gain and salt and water retention. Glimepiride is usually not associated with hyponatraemia.

218. Renal osteodystrophy is a metabolic bone disease that accompanies chronic renal failure. Of the following, which is the most likely to be responsible for renal osteodystrophy? Diminished activity of renal 1--hydroxylase Hypophosphataemia Increased intestinal absorption of calcium Hypoalbuminaemia Increased levels of 1,25-dihydroxy-cholecalciferol Your answer

This metabolic bone disease, which accompanies chronic renal failure, consists of a mixture of osteomalacia, hyperparathyroid bone disease (osteitis fibrosa), osteoporosis and osteosclerosis. Osteomalacia results from diminished acitivity of renal 1--hydroxylase with failure to convert cholecalciferol to its active metabolite 1,25dihydroxychole-

calciferol. This leads to reduced intestinal absorption of calcium, hypocalcaemia, hyperphosphataemia and stimulation of the parathyroid glands. 219. A 30-year-old man with Marfans syndrome presents with severe left-sided loin pain and haematuria. Blood tests reveal hypokalaemia and acidosis. The anion gap is 14 mmol/l. There is hypercalciuria. Abdominal radiographs show a left ureteric calculus. What is the most likely cause for this condition? Uraemic acidosis Diabetic ketoacidosis Renal tubular acidosis Lactic acidosis Salicylate poisoning

Your answer

Distal renal tubular acidosis (type-1 RTA) may occur in Marfans syndrome. This acidosis is associated with a normal anion gap (ie 816 mmol/l). Salicylate poisoning, which can occur due to excessive ingestion of salicylates for the renal colic, would cause an increased anion gap, as would all the other listed conditions.

220. Water excretion in the kidneys is influenced by: Proximal tubule Vasopressin Your answer Distal tubule Ascending limb of loop of Henle Integrity of collecting ducts Regulation of water secretion is by the distal tubule and the collecting ducts under the influence of vasopressin. The relative hyperosmolality of the medulla is maintained by a counter-current mechanism and is responsible for the flux of water across the renal tubule.

221. A patient has been complaining of a facial rash and arthralgia for the last six months. She is hypertensive and has proteinuria. What is the most likely diagnosis? IgA nephropathy Minimal change disease Lupus nephritis Rapid progressive glomerulonephritis Proliferative glomerulonephritis

Your answer

10 to 20% of patients with systemic lupus erythematosus have evidence of renal involvement, and this develops in about 4050% of patients, typically during the first five years after diagnosis. While renal disease is a major complication of systemic lupus erythematosus, it is always important to recognise that lupus is a systemic disease and that nephritis typically occurs in patients with extrarenal symptoms such as a rash, arthralgia, Raynauds phenomenon, and pleuropericarditis. Other major organ systems may be involved, including the central nervous system, heart, and lungs. Proteinuria is found in all patients with lupus nephritis and in 5060% of cases is heavy enough to lead to a nephrotic syndrome. Microscopic haematuria accompanies the proteinuria in about 80% of patients; hypertension is found at presentation in 2050%; and 2030% present with rapidly deteriorating renal function that may occasionally be severe enough to lead to acute renal failure.

222. A 45-year-old woman with longstanding rheumatoid arthritis presents with fever, arthralgia, skin rashes and oliguria. She has been taking diclofenac on a regular basis for the past 2 years. Renal biopsy shows an intense interstitial infiltrate, often including eosinophils, with variable tubular necrosis. What is the most characteristic feature of this side-effect of diclofenac? It is reversible It is not dose-related It is mediated by increased PGI2 synthesis It results in acute tubular necrosis It is counteracted by the use of ACE inhibitors Your answer

Chronic consumption of large amounts of analgesics (especially those containing phenacetin) and non-steroidal anti-inflammatory drugs (NSAIDs) leads to chronic tubulointerstitial nephritis and papillary necrosis. The higher the dose, the more the damage to the kidneys. It is mediated by inhibition of prostacyclin (PGI2) synthesis. Stopping the drug may result in arrest of the disease and even in improvement in function.

223. Which is the most appropriate first line management in a 17-year-old female presenting with recurrent lower urinary tract infection? Attention to lifestyle measures (eg fluid intake, pericoital hygiene) Cystoscopy Long term low dose trimethoprim therapy Micturating cystogram Renal ultrasound scan Your answer

Recurrent cystitis may often accompany the onset of sexual activity in young females. The appropriate first line management is to advise strict attention to personal hygiene and an increase in fluid intake and subsequent urine flow around times of sexual activity. Further investigations would be indicated if such simple measures fail to alleviate the problem.

224. Patients with sickle cell disease, especially those aged 40 or older, have a high risk of developing which complication? Renal carcinoma Chronic renal failure Hepatic fibrosis Lung cancer Urinary tract infection

Your answer

Chronic renal failure is an important contributor to illness and death among adults with sickle cell disease, especially those over 40 years of age. It is usually insidious in onset, manifested initially by a falling haemoglobin level attributable to lowered erythropoietin levels. The renal function of older patients should therefore be monitored regularly. Serum creatinine levels tend to be lower than normal in steady-state sickle cell disease and creatinine levels within the accepted normal range should not be interpreted as indicating normal renal function, indeed in sickle cell disease it is likely that levels of 60 to 70 mol/l reflect significant renal damage.

225. During the course of a routine medical examination, a 26-year-old man is found to have 3+ haematuria. This is confirmed on microscopy and on a second urinalysis 9 months later. He remembers two episodes during the past year when

his urine was red, both occurred in association with an upper respiratory tract infection. The family history is negative for renal disease. His blood pressure is 150/100 mmHg and he has no oedema. Plasma creatinine is 65 mol/l. What would a renal biopsy most probably show? Cast nephropathy IgA nephropathy Your answer KimmelstielWilson lesions Minimal-change disease Thin basement membranes

IgA nephropathy (Bergers disease) is the glomerular lesion most frequently found on renal biopsy. On light microscopy, diffuse mesangial proliferation is typical, with prominent IgA deposits evident on immunostaining. It usually presents in the manner illustrated above or with recurrent macroscopic haematuria, often in close temporal relationship with an upper respiratory tract infection. The principal differential diagnosis is thin-membrane disease (pathogenetically related to Alports syndrome, also termed benign familial haematuria). The presence of hypertension and a negative family history militates against this. In those with haematuria unexplained by bleeding elsewhere in the urinary tract, approximately 50% will have a glomerular lesion. The presence of red cell casts or dysmorphic red cells in the urine may point towards this. About 20% of those with unexplained haematuria will have a tendency towards renal stone formation and crystalluria. Most centres would not perform renal biopsy for isolated glomerular haematuria with normal renal function.

226. A 7-year-old boy has clinical features of the nephrotic syndrome. However, a blood test shows that his serum cholesterol level is normal. Urine microscopy is normal. What is the most probable cause of nephrotic syndrome in this patient?

Lupus nephritis Minimal-change glomerulonephritis Membranous glomerulonephritis Focal glomerulosclerosis Drug-induced nephrotic syndrome

Your answer

Minimal-change glomerulonephritis (nephropathy) accounts for most of the cases of nephrotic syndrome in childhood. It also is responsible for 2025% of cases of adult nephrotic syndrome. Serum cholesterol levels generally remain within normal limits in patients with the nephrotic syndrome caused by minimal-change nephropathy. Minimalchange lesions do not usually result in the presence of red cells or red cell casts in the urine.

227. A 46-year-old woman is managed with long-term haemodialysis. The cause of her chronic renal failure is long-standing type 1 diabetes. She is treated with a steady dose of erythropoietin (EPO) yet on recent dialysis sessions you have noticed a decrease in her haemoglobin (Hb) from 11.1 g/dl to 8.4 g/dl at her last dialysis session. Which of the following would be the most appropriate investigation? Serum ferritin Upper gastrointestinal (GI) endoscopy Lower GI endoscopy Measurement of EPO antibodies Bone marrow aspiration Your answer

Patients with chronic renal failure often have co-existent low iron stores. Adequate total body iron is required for erythropoietin to work as an effective treatment for renal anaemia. Options for screening include serum ferritin, percentage hypochromia and transferring saturation. Intravenous iron replacement is no established as a treatment of choice for iron replacement. Untreated, chronic anaemia is thought to predispose to worsening of left ventricular hypertrophy in this group of patients.

228. A 33-year-old man has hepatosplenomegaly, serum calcium 2.95 mmol/l, creatinine 60 mol/l and normal sized, non-obstructed kidneys at ultrasound. He has a urine output of 1.5 l/day with 2.2 g proteinuria. Which of the following is the most likely diagnosis? Non-Hodgkins lymphoma Weils disease

Multiple myeloma Sarcoidosis Alports syndrome

Your answer

Sarcoidosis can lead to an acute or chronic interstitial nephritis. The classical feature is the presence of non-caseating granulomata (with giant cells). It may occasionally present with a glomerulonephritis, most often membranous. The renal disease usually responds well to steroids. Lymphoma could also present in the manner described, but hypercalcaemia would be expected less often, and the renal ultrasound may show evidence of infiltration (although lymphoma can also be associated with glomerulonephritis). Myeloma would only rarely lead to hepatosplenomegaly, and Weils disease (due to leptospirosis) results in oliguric acute renal failure.

229. A 19-year-old woman complains of stiff joints and a facial rash. Her blood pressure is 145/95 mmHg, she has +1 oedema, slightly swollen metacarpophalangeal joints and some ulceration of her buccal mucosa. Plasma creatinine is 92 mol/l, Hb is 8.9 g/dl and platelet count is 92 x 1012/l. Urinalysis shows +3 blood and +3 protein. High titres of double-stranded DNA antibodies are detectable in her serum. What would histological examination of renal tissue demonstrate? Diffuse proliferative glomerulonephritis with deposits of IgG, IgM and C3 Your answer Fibrillary glomerulonephritis Focal segmental glomerulosclerosis Pauci-immune diffuse proliferative glomerulonephritis Pauci-immune focal necrotising crescentic nephritis This woman clearly has lupus nephritis. The most common (and most severe) form, one which is entirely compatible with the clinical history, is type IV proliferative GN affecting > 50% of glomeruli, often with crescents present. This form of lupus nephritis frequently progresses to cause renal failure when untreated and is associated with extensive immune deposits on

immunofluorescence staining. Therefore, patients with type IV (and sometimes type III, where < 50% of glomeruli are involved) should be treated with a combination of cyclophosphamide and steroids, a treatment combination that is frequently extremely emotive in this population. Additional types of lupus nephritis are type I (abnormal urine sediment with normal light microscopy), type II (mesangioproliferative GN), type V (membranous nephropathy) and type VI (advanced sclerosed). The latter classification was added in 2003. Pauciimmune proliferative glomerular lesions, with and without crescents, tend to be associated with antineutrophil cytoplasmic antibody (ANCA)-associated smallvessel vasculitis.

230. A 2-year-old boy has a phimosis. What is the most appropriate treatment for this patient? Wait and watch Circumcision Dorsal slit Release of preputial adhesions Dilatation of urethral meatus Your answer

It would be appropriate to watch and wait in this case. Phimosis is common in 2 year olds and most will slowly dilate. In those who have persistent problems into teenage years, around 85% will respond to topical steroids, reducing the need for circumcision. Where there is obvious infection, a dorsal slit may be considered.

231. You are asked to review a 56-year-old man with myocardial infarction who is on the third day of his admission. There is a past history of arthritis for which he uses NSAIDs, but nil else of note. During his admission he had a short period of atrial fibrillation that reverted spontaneously. It has been noted that his urine output is tailing off. His serum creatinine level has risen from 156 mol/l (60110) on admission to 195 mol/l now, and his urea is 12 mmol/l (2.57.5). His urine osmolality has been measured at 520 mOsmol/kg (3501000), with a low urine sodium at 15 mmol/l. What diagnosis do you suspect? Acute tubular necrosis Prerenal uraemia Chronic interstitial nephritis Acute interstitial nephritis Renal artery embolus

Your answer

His urine results suggest prerenal uraemia, with raised urine osmolality and appropriately low urine sodium. It is likely that he received large amounts of diuretics at the time of admission and that this, coupled with any period of hypotension, is likely to have led to a drop in the glomerular filtration rate (GFR) and prerenal uraemia. Drugs that impair renal autoregulation (such as angiotensin-converting enzyme (ACE) inhibitors and non-steroidal anti-inflammatory drugs (NSAIDs)), may increase the tendency to develop the problem. All causes of prerenal uraemia may lead to the development of established parenchymal kidney damage if not correctly managed. This mans fluid status should be assessed in the context of his myocardial infarction, and if he looks dry then some fluid replacement should be instigated. Measurement of urinary sodium is useful in differentiating between prerenal and intrinsic renal dysfunction. Prerenal failure is considered likely if urinary sodium excretion is less than 20 mmol/l, whereas intrinsic renal failure (ATN) is considered if urinary sodium excretion is above 40 mmol/l. Depending on the status and position of his myocardial infarction, this may be undertaken with the benefit of a central line to assess central venous pressure.

232. A patient is diagnosed with membranous nephropathy. With which other condition is this condition associated? Asthma Malignancy Hepatic fibrosis Liver cancer Diabetes mellitus

Your answer

Membranous nephropathy is associated with malignancy, especially in the elderly. Series have reported rates of malignancy ranging from 511%, with the greatest risk in those at the upper end of the age range. However, different inclusion criteria have sometimes been used to assess risk. For example, some series have included tumours recognised long after a diagnosis of renal disease has been made, when the association may be coincidental. Most reported tumours have been of solid organs but haematological malignancies are also implicated. Very often, the disease is advanced and obvious by the time that nephrotic syndrome or a heavy proteinuria is recognised. In some cases, effective treatment of the malignancy has led to an improvement in the nephrotic syndrome or proteinuria. The use of alkylating agents or corticosteroids to treat membranous nephropathy is not recommended in this setting, unless it would be appropriate for treatment of the malignancy itself.

233. An 18-year-old girl presents with peripheral oedema and polyuria. Investigations show pulse 90bpm, BP 130/80, bloods show LFTs normal, albumin 23, Hb normal, U&Es normal, cholesterol9. What is the most appropriate next investigation? Renal biopsy Renal ultrasound 24 hour urine collection Autoantibodies Dipstick

Your answer

This girl has nephrotic syndrome, the combination of proteinuria (>3g/24 hours), hypoalbuminaemia (<30g/l) and oedema. In practise, a 24 hour urine collection is required to quantify the proteinuria. However, heavy proteinuria on urine dipstick would be sufficient to confirm the need for a renal biopsy. A renal ultrasound would always be required prior to a renal biopsy to ensure the presence of two kidneys and confirm kidney size and position before the biopsy. Autoantibodies would also be done to aid diagnosis but the initial confirmatory investigation would be the dipstick. In children and young adults the most likely renal biopsy finding would be minimal change glomerulonephritis which may be steroid responsive and has a good prognosis.

234. You are asked to review a man who is being considered for peritoneal dialysis for chronic renal failure. He has a number of concerns before proceeding to surgery and placement of a Tenckhoff catheter. Regarding concomitant pathologies, which of the following best describes a point to consider when thinking of peritoneal dialysis? Diabetes treatment may need to be adjusted once dialysis is commenced Your answer Abdominal hernias do not need to be repaired before commencing dialysis Stomas do rarely present a problem when considering catheter placement Staphylococcus epidermidis is a rare cause of CAPD peritonitis Fungal infections commonly cause CAPD peritonitis Peritoneal dialysis fluid contains a high concentration of glucose (standard fluid at a concentration of 1.36%). More concentrated dialysis fluid may contain glucose at concentrations of up to 6.36%, this means that patients with diabetes may require significantly more diabetes treatment to reduce their blood glucose once dialysis is commenced.

Due to the large fluid volume expansion once fluid is introduced, abdominal hernias may significantly worsen once dialysis treatment is commenced, and should be surgically repaired. Stomas may be associated with significant adhesions and changes within the abdominal cavity making catheter placement impossible. Staphylococcus epidermidis is the commonest cause of CAPD (continuous ambulatory peritoneal dialysis) peritonitis (4050% of cases); fungal infections such as candida are responsible for only 2% of cases.

235. A 48-year-old nun presents to her GP with malaise, anorexia and weight loss. Screening blood samples reveals a urea of 50.1 mmol/l and a serum creatinine of 690 mol/l. Her past history includes frequent headaches, but nil else of note. She has, however, failed to attend her routine well-woman appointments. Ultrasound reveals bilateral hydronephrosis and a suspicion of a central pelvic mass. What diagnosis is most likely to be responsible for this womans hydronephrosis? Retroperitoneal fibrosis Ovarian carcinoma Cervical carcinoma Your answer Analgesic nephropathy Chronic urinary reflux There is a suspicion that this woman has been non-compliant with standard womens healthscreening measures, eg cervical screening. This, with the central pelvic mass on ultrasound examination and bilateral hydronephrosis accompanied by renal failure, makes cervical carcinoma the most likely cause. Although cervical cancer is fairly rare in virgins (eg nuns), it can still occur spontaneously. Neurogenic bladder (eg after spinal injury) is another possible cause, and pharmacological agents such as -blockers and anticholinergics may inhibit bladder emptying, leading over time to ureteric reflux. Treatment in this case will involve insertion of bilateral nephrostomy tubes to relieve the obstruction. Specialist referral and gynaecological opinion will be required for her cervical carcinoma.

236. Which one of the folowing statements is true regarding retroperitoneal fibrosis? Low back pain is the most common presenting symptom Your answer

Bilateral swelling of the legs is often due to inferior vena-caval obstruction Pizotifen (migraine treatment) is implicated in causing similar disease Renal failure is due to fibrous tissue infiltrating the kidneys Hashimoto's thyroiditis is a recognised association Retroperitoneal fibrosis is one of the multifocal fibrosclerotic syndromes, which also includes mediastinal fibrosis, scerosing cholangitis and Riedel's thyroiditis. The process usually begins over the promontory of the sacrum and extends laterally across the ureers and as high as the second or the third lumbar vertebra. It is more common in males (2:1), peak incidence in the 5th and 6th decades. Low back pain is the most common symptom, which may be accompanied by fever and weight loss. Methysergide a 5HT2-antagonist used to treat migraine headache can cause a similar syndrome. Other drugs such as beta-blockers, (methyldopa, hydralazine) are implicated. Pizotifen is an antihistamine; its use is not associated with restroperitoneal fibrosis. Systemic diseases associated with retroperitoneal fibrosis include SLE, scleroderma and carcinoid syndrome. Diagnosis is suggested by the finding at intravenous pyelography (IVP) of displacement of the ureters toward the midline. the fibrosing process may surround the inferior vena cava, but obstruction of that vessel is uncommon. Thromboembolism and hypertension are recognised complications. the fibrous tissue does not infiltrate the kidneys or the ovaries.

237. A 39-year-old female is diagnosed as having chronic renal failure. When considering long-term dialysis for this patient, what is the most common complication that may occur? Congestive heart failure Squamous cell cancer of the skin

Carpal tunnel syndrome Your answer Gastrointestinal malignancy Non-Hodgkins lymphoma

Long-term haemodialysis is associated with carpal tunnel syndrome. High output congestive heart failure due to anaemia is rare nowadays due to erythropoietin therapy. Non-Hodgkins lymphoma and skin cancers are more frequent in kidney transplant recipients owing to prolonged immunosuppressive therapy. There is no known increased risk of gastrointestinal malignancy in patients on long-term dialysis.

238. A 27-year-old man, who has a history of hypertension and intermittent loin pain, presents to his new GP to register after moving house. Urine testing on registration has revealed evidence of haematuria. There is a family history of subarachnoid haemorrhage. What diagnosis fits best with this clinical picture? Autosomal-dominant polycystic kidney disease (ADPKD) Your answer Glomerulonephritis Renal-cell carcinoma Urinary tract infection Nephrolithiasis The history of hypertension, painless haematuria, intermittent loin pain and family history of subarachnoid haemorrhage is highly suggestive of ADPKD. Patients often present in young adult life with hypertension. Its prevalence rate varies from 1:400 to 1:1000 in Caucasian populations, accounting for 310% of patients in the West undergoing haemodialysis. Abnormal genes responsible have been mapped to chromosome 16 and chromosome 4. Patients may present acutely with haemorrhage or infection into a cyst. Around 8% of patients with ADPKD have asymptomatic intracranial aneurysms, but this prevalence rate is doubled in families where there is a history of subarachnoid haemorrhage. Physical examination usually reveals large irregularly shaped kidneys. Progressive renal failure means that by the age of 70 years 30% of sufferers require renal replacement therapy. For this reason control of hypertension is crucial, and ACE inhibitors are the first-line choice of treatment.

239. A worker in a factory making torch batteries has developed swelling of his face and feet. Urinalysis shows proteinuria. What would be the most characteristic finding in a blood test in this patient? Normal serum creatinine levels Hyperalbuminaemia Raised serum transferrin levels Normal serum ceruloplasmin levels Low serum calcium and vitamin D levels

Your answer

This patient most probably has mercury or cadmium poisoning. Creatinine levels are raised in the early stages of the disease. Serum albumin levels are reduced due to massive proteinuria, a characteristic feature of nephrotic syndrome. Serum transferrin levels are reduced due to transferrin loss via the kidneys, which may cause microcytic hypochromic anaemia. Ceruloplasmin levels are also reduced. The combination of hypocalcaemia and vitamin D deficiency is due to secondary hyperparathyroidism and the enhanced urinary excretion of cholecalciferol-binding protein.

240. The presence of which one of the following features is MOST helpful in distinguishing chronic from acute renal failure? Seizures Bilateral small kidneys Your answer Hypocalcaemia Dilute urine with high urine sodium Acute pulmonary oedema

When a patient presents for the first time with moderate renal impairment, it is very important to identify the duration of renal failure, as it will influence the line of management and prognosis. Anaemia, hypocalcaemia and dilute urine can be encountered in both acute and chronic renal failure. Acute pulmonary oedema and seizures are a consequence of volume overload in both acute and

chronic renal faliure. Bilateral small kidneys on imaging and evidence of renal osteodystrophy on plain X-ray reflect a chronic long-standing pathology more suggestive of chronic renal failure. Furthermore, skin pigmentation and peripheral neuropathy are the result of long-standing metabolic abnormality such as chronic renal failure.

241. You are asked to review a 36-year-old woman who has presented to the casualty department with renal colic. KUB X-ray has revealed the presence of multiple renal stones. Her past history of note includes extensive surgical resection for Crohns disease. What is the most likely chemical composition of her renal stones? Calcium phosphate Uric acid Magnesium ammonium phosphate Cysteine Calcium oxalate Your answer Patients with short bowel syndrome due to surgical resection are particularly prone to the formation of oxalate stones. It is likely that her surgery for Crohns has precipitated the problem. Calcium oxalate stones occur in around 65% of patients in the UK with renal stones, calcium phosphate in around 15% and magnesium ammonium phosphate (struvite) stones occur in around 1015%. Oxalate stones occur in patients who ingest excess amounts of foods high in oxalate, such as spinach, rhubarb and tea, those on a calciumrestricted diet, and those with gastrointestinal disease such as Crohns who have increased colonic oxalate absorption.

242. A 25-year-old woman presents with a renal calculus. She has recurrent episodes of headache and sweating. Physical examination reveals a thyroid nodule but no clinical sign of thyrotoxicosis. Which of the following investigations would be most useful in arriving at a diagnosis? T3, T4 and TSH levels Serum calcium level Serum alkaline phosphatase 24-hour urine collection for 5-hydroxyindoleacetic acid excretion 24-hour urine collection for metanephrines

Your answer

This is a case of MEN (multiple endocrine neoplasia)-type II. It is associated with medullary carcinoma of the thyroid, hyperparathyroidism and phaeochromocytoma. The most useful investigation would be a 24-hour urine collection for metanephrines. Plasma and urinary catecholamines can also be measured directly. Detection of

hyperparathyroidism would not lead to the diagnosis of MEN-type II, thus serum calcium and alkaline phosphatase levels would not be helpful in this regard. Thyroid functions are normal in this disorder. Estimation of 5-hydroxyindoleacetic acid is done for carcinoid syndrome.

243. A 57-year-old man with a long history of gout presents with loin pain. Other past history of note includes ileostomy after bowel surgery. There is no history of weight loss of malabsorption syndrome after his bowel surgery. Excretion urography reveals evidence of bilateral renal stones. What is the most likely composition of his renal stones? Calcium oxalate Calcium phosphate Uric acid stones Magnesium ammonium phosphate stones Cysteine stones

Your answer

This man has a long history of gout. Although uric acid stones only account for 35% of presentations with renal stones in the UK (calcium oxalate stones are the commonest), his history of hyperuricaemia is a give-away. It is likely that his ileostomy has contributed due to acidification of urine and dehydration. Both of these conditions make urate stone formation more likely, since uric acid is more soluble in an alkaline medium. Calcium stones may also occur in patients with hyperuricaemia and hyperuricosuria: it is believed that calcium crystals precipitate around an initial nidus of uric acid in these patients. Avoidance of large amounts of dietary purines, coupled with adequate hydration should be advised.

244. A peritoneal dialysis patient presents with a 1-week history of abdominal pain. She has been on dialysis for 2 years and also has a history of coronary artery disease. Clinically, her abdomen is rigid and distended with absent bowel sounds, blood pressure is 85/50 mmHg, pulse rate is 120/min and the dialysate appears very cloudy. Culture of the dialysis fluid reveals a mixed growth of Escherichia coli, Bacteroides spp and Enterobacter spp. What is the most likely cause of this presentation? Acute pancreatitis Diverticulitis

Your answer

Myocardial infarct Primary peritoneal-dialysis peritonitis Ulcerative colitis The most common cause of acute peritonitis in peritoneal dialysis (PD) patients is primary peritonitis, which is due to contamination of the PD fluid or catheter with skin organisms such as coagulase-negative staphylococci or Staphylococcus aureus. The presence of mixed Gram-negative and/or anaerobic organisms is highly suggestive of secondary peritonitis due to a perforated large bowel or appendicitis. Diverticulitis is the most frequent cause of this presentation. Other clues to the presence of intra-abdominal pathology are slow resolution of peritonitis, air under the diaphragm on a plain X-ray (erect chest and/or abdominal views) - although this may occur during the course of normal PD) and elevated PD fluid amylase. It is essential to examine the hernial orifices carefully to look for evidence of strangulated hernia. The outcome from secondary peritonitis is much worse than that from primary peritonitis, primarily because of the delay in diagnosis.

245. A patient presents with metabolic acidosis but has a normal anion gap. What is the most likely diagnosis? Alcohol excess Lactic acidosis Diarrhoea Salicylate poisoning Diabetic ketosis

Your answer

The anion gap is calculated by subtracting the measured plasma anionic concentration (bicarbonate and chloride) from the added serum sodium and potassium value, and should be in the range 1016 mmol/l. In the presence of metabolic acidosis, a normal or low anion gap would indicate hyperchloraemic acidosis (such as with diarrhoea or renal tubular acidosis), whereas an enlarged gap would signify the presence in plasma of a normally undetected anion that is contributing to the acidosis (eg lactic, salicylic or ketoacids, unmeasured uraemic toxins).

246. A 10-year-old boy has a history of tonsillitis followed by haematuria and hypertension. What would be the characteristic blood test finding in this condition? Normal C3 level Depressed CH 50 level Low C4 level Increased cryoglobulins

Your answer

Elevated antinuclear antibody This boy in all probability has post-streptococcal glomerulonephritis. Serum C3 and CH 50 levels are depressed within 2 weeks of the occurrence of the disease. C4 levels remain normal, indicating activation of the alternate complement pathway. Cryoglobulin levels are increased only in cryoglobulinaemic renal disease. Antinuclear antibody is raised in systemic lupus erythematosus.

247. A 21-year-old registered drug addict is brought in unconscious to the emergency department. Its not known how long he was on the floor of his apartment. Blood tests on admission revealed a bicarbonate of 19 mmol/l, creatinine of 280 mol/l, urinary sodium of 60 mmol/l and a urine osmolality of 220 mOsmol/l. There is evidence of acute sepsis. What diagnosis fits best with this clinical picture? Pre-renal failure due to a prolonged period of unconsciousness Acute sepsis Acute tubular necrosis Your answer Glomerulonephritis Renal artery embolus secondary to drug injecting Heroin addicts are particularly prone to acute tubular necrosis, precipitated in this case by intravenous drug injection and the prolonged period of unconsciousness and inactivity that followed. Pre-renal failure is distinguished by a urine osmolality > 500 mOsmol/l and a urine sodium excretion of less than 20 mmol/l. Intrinsic renal failure is characterised by a urine osmolality < 350 mOsmol/l and a urine sodium excretion greater than 40 mmol/l. There are multiple causes of acute tubular necrosis, these include: haemorrhage; burns; diarrhoea and vomiting; pancreatitis; diuretics; myocardial infarction; endotoxic shock; some snake bites; myoglobinaemia; hepatorenal syndrome; drugs, including aminoglycosides; and pre-eclampsia and eclampsia.

248. A 32-year-old man attends the transplant clinic with his brother who has end-stage renal failure due to autosomal dominant polycystic kidney disease (ADPKD). He would like to donate a kidney to his brother if possible. Which of the following is the most appropriate modality for screening? Intravenous urography Computed tomography (CT) scan Genetic screening Micturating cystogram

Your answer

Abdominal ultrasound scan The major indication for genetic screening in ADPKD is for subjects who are considering donating a kidney to a relative affected by the disease. Although ultrasound has a high degree of sensitivity for ADPKD type 1, the sensitivity for ADPKD type 2 is much lower and it is possible that a subject may be cleared to donate a kidney yet still have the disease. Genetic probes exist for both mutations, the causative mutation for Type 1 being found on chromosome 16, type 2 on chromosome 4. Type 1 disease makes up around 8590% of the cases; type 2 disease makes up the remainder.

249. A patient with chronic renal failure has a creatinine of 350 mmol/l. She has persistent proteinuria. Which drug is most likely of benefit to her renal prognosis? ACE inhibitors Aspirin Clopidogrel Doxazosin Methotrexate Your answer

Patients with proteinuria of any cause have their renal prognosis improved if they are treated with angiotensin-converting enzyme (ACE) inhibitors. They are at increased cardiovascular risk, and attention should be paid to other modifiable risk factors, especially cigarette smoking and hyperlipidaemia.

250. A 36-year-old man who has been suffering from gouty arthritis for the past 5 years now presents with features of nephrotic syndrome. Which medication is the most likely cause for his condition? Methotrexate Oral NSAIDs Hydroxychloroquine d-Penicillamine Sulfasalazine

Your answer

d-Penicillamine can precipitate the nephrotic syndrome and is used as therapy for severe gouty arthritis. The drug combines with a plasma protein to form an antigenic hapten that induces an immune-complex mediated membranous glomerulonephritis. If proteinuria exceeds 2 g/24 hours, the drug must be stopped. Sulfasalazine mainly causes marrow

suppression, skin rashes, mouth ulcers and abnormal liver biochemistry. Non-steroidal antiinflammatory drugs (NSAIDs) may cause acute tubulointerstitial nephritis. Patients present with fever, arthralgia, skin rashes and acute oliguric or non-oliguric renal failure. Hydroxychloroquine is associated with corneal deposits and retinopathy. Methotrexate causes neutropenia, thrombocytopenia and renal impairment. Nephrotic syndrome is not a side-effect of this drug.