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Nephrology

A 15-year-old male presents with fever, cough and
weight loss. He is of Indian origin and has recently
returned to the United Kingdom after four months in
India with his parents where he developed these
symptoms.
He is diagnosed with pulmonary tuberculosis and is
commenced on rifampicin, isoniazid, and
pyrazinamide.
Four weeks later he presents with fever, myalgia, and
lethargy.
Investigations:
Haemoglobin

145 g/L

(120-160)

WBC

7.5 ×109/L

(4-11)

Neutrophils

5.5 ×109/L

(1.5-7)

Lymphocytes

0.8 ×109/L

(1.5-4)

Monocytes

0.05 ×109/L

(0-0.8)

Eosinophils

1.14 ×109/L

(0.04-0.4)

Basophils

0.01 ×109/L

(0-0.1)

Platelets

350 ×109/L

(150-400)

Serum sodium

141 mmol/L

(137-144)

Serum potassium

5.1 mmol/L

(3.5-4.9)

Serum urea

27.9 mmol/L

(2.5-7.5)

Serum creatinine

400 µmol/L

(60-110)

Serum bilirubin

7 µmol/L

(1-22)

Serum aspartate transaminase

31 U/L

(5-35)

Serum alkaline phosphatase

97 U/L

(45-105)

Serum albumin

39 g/L

(37-49)

Urinalysis

Protein +
Leucocytes +

no bacteria
Urine culture

Negative

Which of the following is the most likely cause of his
renal impairment?
(Please select 1 option)
Acute interstitial nephritis

Correct

Isoniazid toxicity
Plasmodium vivax malaria
Renal tuberculosis
Rhabdomyolysis

Interstitial nephritis may be caused by infection,
autoimmunity or glomerular disease as well as
hypersensitivity to medicines.
A large number of medicines are reported to cause
acute interstitial nephritis.
The agents most commonly implicated are:











Lactams
Vancomycin
Rifampicin
Co-trimoxazole
Sulphonamides
Ciprofloxacin
NSAIDs
Ranitidine
Cimetidine
Furosemide
Thiazides, and
Phenytoin.

An acute allergic reaction, with infiltration of immune
cells, occurs in response to the causative drug,
causing direct cytotoxicity.
Typical clinical features include:

2Kidney ultrasound This 9-month-old boy presented with fever for four days.    Renal failure with fever Arthralgia Eosinophilia and Eosinophiluria. and his fluid intake . He has gone off his food.

1 mmol/L (3. oedema or lymphadenopathy. He has not been admitted previously and is on no regular medications. anaemia. Abdomen soft. His immunisations are up to date.98 kg and there were no neonatal problems. with no neck stiffness. Mother felt that his urine was very concentrated and smelly. clubbing. Mother suffered from post-natal depression. .5-4. He was a full term normal vaginal delivery weighing 2.5°C. Chest and ENT clear.5) Sodium 135 mmol/L (137-144) Potassium 4. On examination his temperature 39.9) Creatinine 56 µmol/L (60-110) 3+ protein Urine dipstix 3+ blood + nitrites The picture is from his ultrasound scan of kidneys. cyanosis. Investigations revealed: Haemoglobin White cell count 112 g/L (130-180) 15.has been poor today. Dry mucous membranes. Alert and cooperative.7 ×109/L (4-11) 78% neutrophils 14% lymphocytes Platelets 322 ×109/L (150-400) Serum urea 7. There is no other family history of note. Heart rate 130/min. No jaundice. with slight tenderness in the left renal angle.5-7. respiratory rate 25/min.2 mmol/L (2.

His fever responds rapidly to IV cefotaxime. settling within 24 hours and with improvement in his feeding. What is the most likely diagnosis? (Please select 1 option) Nephrocalcinosis Incorrect answer selected Nephronophthiasis Perinephric abscess Pyelonephritis This is the correct answer Renal calculus What is the single most important abnormality shown in the image? (Please select 1 option) Enlarged kidney Incorrect answer selected Hyperechoeic areas This is the correct answer Hypoechoeic areas Nephrocalcinosis Shrunken kidney What single item of management is most important? .

The renal pelvis is minimally dilated.(Please select 1 option) IV cefotaxime Correct Meticulous monitoring of fluid balance PO trimethoprim Surgery What single investigation should be done? (Please select 1 option) DMSA scan in 2 weeks DMSA scan in 4 months Incorrect answer selected This is the correct answer MAG3 scan in 2 weeks MAG3 scan in 4 months MCUG in 2 weeks Repeat ultrasound scan of kidneys. A MAG3 scan is not as sensitive as a DMSA scan in the detection of scars. A micturating cystourethrogram (MCUG) would be indicated if he had presented below the age of 6 months and the fever had not settled within 48 hours (atypical UTI). It may be combined with an . non-scar photopaenic areas may be present. This should be treated with IV antibiotics. At 6 months of age should be investigated for possible scarring using a dimercaptosuccinic acid (DMSA) scan in a few months time. If this is done too early. Gentamicin or cefotaxime would be appropriate. The appearances are consistent with acute pyelonephritis. but the wall appears thickened. ureters and bladder in 3 months The image shows an enlarged kidney with several hyperechoic areas within the cortex.

but this is only possible when the child can pass urine on demand (usually about 3 years). Further Reading: NICE. Image: © 2007 Staffordshire General Hospital 3Kidney A Duplex kidney B Multicystic dysplastic kidney C Pelvi-ureteric junction obstruction D Polycystic kidney .indirect CUG. Urinary tract infection in children (CG54).

Some would advocate surgical excision to eliminate the risk of malignancy or hypertension. The report states there is a 3cm diameter cyst in the right kidney. presenting with renal cysts and hypertension in young adulthood or recessive. A 14-year-old girl has an ultrasound during an episode of abdominal pain. Polycystic kidneys are a different entity and can be autosomal dominant. Duplex kidney Incorrect . causing loin pain.The correct answer is Simple renal cyst This is a simple benign cyst and requires no follow up. Duplex kidney Incorrect . Duplex kidney Incorrect . presenting with multiple small cyst and renal failure in infancy.The correct answer is Multicystic dysplastic kidney This is the typical appearance of a multicystic dysplastic kidney. The MAG-3 shows no functioning renal tissue on that side. A 15-year-old boy presents with loin pain and haematuria.E Renal agenesis F Simple renal cyst G Vesico-ureteric reflux From the given list what is the most likely diagnosis? A 3-month-old baby boy sees you with the results of a MAG-3 scan following antenatal scans showing multiple cysts in his left kidney.The correct answer is Pelvi-ureteric junction obstruction This is the typical picture of Dietl's crisis when a fluid load overwhelms the kidney's ability to drain because of an obstruction at the PUJ. . He admits drinking beer at a party the night before.

What is the most appropriate management option? (Please select 1 option) Check U&E Incorrect answer selected Operative pyeloplasty on the basis of current information Repeat MAG-3 This is the correct answer Repeat ultrasound in 3 months Watchful waiting This girl has pelvi-ureteric junction obstruction with stable USS findings (only intra-renal pelvis measurements are relevant as there is too much variability in extra-renal measurement). This should now be repeated. The differential function on MAG-3 needs to be lower than 40% on the affected side for most urologists to consider operative intervention. A MAG-3 scan at 6 months of age showed delayed drainage on the affected side with a differential function of 42:58. . if the differential function on the affected side is less than 40% then that is an indication for pyeloplasty. her MAG-3 showed delayed drainage and a degree of functional impairment. there is no definite indication for surgery on the information given. the intrarenal diameter is 14 mm (previously 13 mm).4- A 16-month-old girl who is being followed up for antenatally diagnosed unilateral hydronephrosis has a recent ultrasound scan showing an extra-renal pelvic diameter of 24 mm (previously 19 mm). However.

Denys-Drash syndrome Incorrect . generalised oedema and hypoalbuminaemia. a Wilms' tumour is discovered. Both parents are aware that they are carriers for the disease. Whilst this is being investigated.The correct answer is Finnish-type congenital nephrotic syndrome . Denys-Drash syndrome Correct A 6-month-old girl who was found to have nephrotic syndrome at 3 weeks of age and has now developed end stage renal disease requiring transplantation. He also has a hypospadias.Even in severe unilateral cases the urea and electrolytes (U&Es) are normal as the contralateral kidney has sufficient reserve. 5Types of Nephrotic syndrome A Denys-Drash syndrome B Finnish-type congenital nephrotic syndrome C Henoch-Schonlein purpura D Nail-patella syndrome E Oculocerebrorenal syndrome F Steroid sensitive nephrotic syndrome G Steroid resistant nephrotic syndrome H Secondary congenital nephrotic syndrome Which of the given options is the most likely answer in the following scenarios? A 12-month-old boy who develops proteinuria.

It is almost always idiopathic in childhood. Age at onset Sustained hypertension Microscopic haematuria Renal function Long term prognosis Usual histology Steroid sensitive nephrotic syndrome Steroid resistant nephrotic syndrome (SSNS) (SRNS) Toddler. Denys-Drash syndrome Incorrect . from historical data known to be minimal change Poor . If it were to be biopsied. Has had several relapses of her nephrotic syndrome. whereas SRNS is normally focal segmental glomerulosclerosis. intermittent Persistent Normal Often reduced Excellent. On biopsy was found to have minimal change disease. SSNS usually shows minimal change disease.The correct answer is Steroid sensitive nephrotic syndrome A 9-year-old girl with hypertension. proteinuria and hypoalbuminaemia. No other signs on physical examination.A 2-year-old boy with normal renal function and mild haematuria associated with his nephrotic syndrome.significant risk of long term hypertension and renal failure Focal segmental glomerulosclerosis . even if frequently relapsing Usually not biopsied. pre-school <1 year or >8 year No Often Mild. Idiopathic nephrotic syndrome is divided into steroidsensitive nephrotic syndrome (SSNS) and steroidresistant nephrotic syndrome (SRNS) because response to steroids has a high correlation with histological subtype and prognosis. Denys-Drash syndrome Incorrect .The correct answer is Steroid resistant nephrotic syndrome Nephrotic syndrome is characterised by a triad of oedema. persistent microscopic haematuria and focal segmental glomerulosclerosis on biopsy.

and intersex disorders resulting from mutations in the Wilms' tumour suppressor (WT1) gene. Medscape. 6- . Pastest: 2012.Denys-Drash syndrome (DDS) is a rare disorder consisting of the triad of congenital nephropathy. 2. Champion MC (eds. At this time. Denys-Drash Syndrome. Nephropathy is a constant feature. Pediatric Nephrotic Syndrome.) Essential Revision Notes in Paediatrics for the MRCPCH. 3. Congenital nephrotic syndrome of Finnish type is inherited in an autosomal recessive fashion and is caused by mutations in the NPHS1 gene. congenital nephrotic syndrome of Finnish type has been reported worldwide. in the incomplete forms of the syndrome. Medscape. Congenital nephrotic syndrome of Finnish type presents in utero or within the first 3 months of life. It is characterised by nephrotic syndrome which progresses rapidly to end-stage kidney disease. Wilms' tumour. Beattie RM. the nephropathy is present with either Wilms' tumour or intersex disorders. (3rd ed). kidney transplantation seems to be the only treatment available for this condition Reference: 1. but the vast majority of patients with Denys-Drash syndrome are destined to develop Wilms' tumour in any residual renal tissue. Cheshire. Although more commonly seen in individuals of Finnish descent.

A 4-year-old boy is referred to the nephrologist with faltering growth and polyuria and polydipsia. and have a hypochloraemic hypokalaemic alkalosis. What is the most likely diagnosis? (Please select 1 option) Bartter syndrome Correct Gitelman syndrome Liddle syndrome Nephrogenic diabetes insipidus Pseudo-Bartter syndrome Bartter syndrome is characterised by a hypokalaemic. unlike Gitelman's syndrome. Very high levels of urinary chloride and sodium are seen with normal urinary calcium. Urinary calcium is normal or high. Symptoms are      Polyuria Polydipsia Episodes of dehydration Faltering growth and Constipation. The underlying renal abnormality results in excessive urinary losses of sodium. He is found to be normotensive. chloride. It is caused by an autosomal recessive defect in the sodium-potassium-chloride co-transporter in the thick ascending limb of loop of Henle. and potassium. leading to sodium chloride and water wasting. hypochloraemic alkalosis and hyperreninaemia with normal blood pressure. The resultant ECF volume contraction causes secondary renin secretion and sodium and water reabsorption in distal tubule and .

hypertension associated with low plasma renin activity. as the hyperreninaemia is a compensatory response to maintain normal blood pressure in the presence of chronic volume depletion. Gitelman syndrome is considered to be a variant of Bartter syndrome and is also an autosomal recessive defect but this time in the distal tubule sodium chloride co-transporter. Liddle syndrome (also called pseudoaldosteronism) is a syndrome of low-renin hypertension. and frequently severe. metabolic alkalosis due to hypokalaemia and hypoaldosteronism (low secretion of aldosterone). Nephrogenic DI can be . Thiazide diuretics produce some biochemical changes. leading to a decrease in the ability of the kidney to concentrate the urine by removing free water. Loop diuretics produce some biochemical changes. Liddle syndrome involves abnormal kidney function. amiloride). raised renin and aldosterone are present like Bartter's but there is also hypocalciuria and hypomagnesaemia which helps distinguish it from Bartter's. It is often asymptomatic with transient episodes of weakness and tetany with abdominal pain and vomiting. It is an autosomal dominant disorder characterised by early. A hypokalaemic metabolic alkalosis. Nephrogenic diabetes insipidus (DI) is caused by an improper response of the kidney to ADH.reciprocal potassium and hydrogen secretion into urine. Blood pressure is normal. with excess reabsorption of sodium and loss of potassium from the renal tubule and is treated with a combination of low sodium diet and potassium-sparing diuretic drugs (for example.

urinary calcium would be low. Pseudo-Bartter syndrome is associated with extrarenal losses of sodium and chloride. It has the same plasma biochemistry as Bartter syndrome but appropriately low levels of urine sodium and chloride. The boy in the question was normotensive. as the hyperreninaemia is a compensatory response to maintain normal blood pressure in the presence of chronic volume depletion. the urinary sodium and chloride would be appropriately low. but in this patient they are very high. Nephrogenic diabetes insipidus is incorrect in this case. If it were nephrogenic diabetes insipidus all urinary electrolytes would be low. reflecting the defect in the thick ascending loop of Henle. Since the urinary calcium is normal. Main causes are cystic fibrosis. Bartter syndrome and loop diuretics work in the same site in the thick ascending loop of Henle. Gitelman syndrome is incorrect in this case. .acquired or inherited. Pseudo-Bartter syndrome is incorrect. cyclical vomiting and laxative abuse. If it were pseudo-Bartter's syndrome. Liddle syndrome is a low-renin hypertension. Urinary osmolality is low as are urinary electrolytes. If it were Gitelman's.

. What does this show? (Please select 1 option) Central diabetes insipidus Incorrect answer selected Desmospressin must be administered to distinguish primarypolydipsia from central diabetes insipidus Desmospressin must be administered to distinguish primarypolydipsia from nephrogenic diabetes insipidus Nephrogenic diabetes insipidus Primary polydipsia This is the correct answer Nephrogenic diabetes insipidus (DI) is caused by an improper response of the kidney to ADH.014 or greater. Nephrogenic DI can be acquired or inherited.7- A 9-year-old boy presents with new onset enuresis and polydipsia. Urinary osmolality is low as are urinary electrolytes. Monitor urine specific gravity hourly. which can be used both to confirm the diagnosis and to distinguish between central DI and nephrogenic DI on the basis of response to a vasopressin analogue. After water deprivation a urine osmolality of >750 mOsm was recorded. leading to a decrease in the ability of the kidney to concentrate the urine by removing free water. The water deprivation test is performed as follows:   Obtain baseline urine and blood for osmolality and electrolytes.  Weigh patient every two hours and limit dehydration to 2-5% loss of body weight. The definitive diagnostic study for diabetes insipidus is the water deprivation test. if the specific gravity is 1. terminate the test and obtain urine and blood specimens for osmolality. Deprive the patient of water after breakfast until significant dehydration occurs. His GP is concerned that he may have diabetes insipidus but is unsure what type so he performed a water deprivation test.

After four hours (two hours in infants). Due to the significant nature of the UTI the infant has a renal USS performed six weeks after the urinary tract infection. Therefore. The urine culture grows Escherichia coli. His fever has been up to 40°C. obtain urine and blood for osmolality. If urine osmolality exceeds 750 mOsm this excludes diabetes insipidus and you do not need to give desmopressin (DDAVP). 7- A 9-month-old male infant presents to the children's assessment unit with a history of fever and vomiting for the last 24 hours. that is. administering desmospressin to distinguish primarypolydipsia from central diabetes insipidus or nephrogenic diabetes insipidus is incorrect. nephrogenic diabetes insipidus. no diabetes insipidus. this shows a  defect in the kidney's response to ADH production. This shows a left sided grade 4 hydronephrosis but no ureteral dilation.000 organisms. A very high urine osmolality after water deprivation shows an appropriate response of the kidney to ADH. central diabetes insipidus. If urine osmolality is >750 mOsm after fluid deprivation. administer intranasal desmopressin and replace urine output  with fluids. . that is. Urine microscopy shows >100 WCC and 50 RBC with >100. If urine osmolality is <300 mOsm after fluid deprivation but >750 mOsm after desmopressin this shows a defect in ADH production. that is. If urine osmolality is <300 mOsm before and after desmopressin. If polyuria persists. central diabetes insipidus or nephrogenic diabetes insipidus are not shown in this case. primary polydipsia can be  diagnosed. Therefore.

however should diminished function become apparent in the affected kidney then surgical intervention is also required. As such it will give the appearance on ultrasound of a dilated renal pelvis but not a dilated ureter as the obstruction is superior to the ureter. PUJ obstruction may be diagnosed antenatally on ultrasound scan.What is the most likely diagnosis? (Please select 1 option) Congenital obstructive megaureter Incorrect answer selected Pelvo-ureteric junction obstruction This is the correct answer Posterior urethral valves Unilateral muticystic dysplastic kidney Wilms' tumour Pelvo-ureteric junction (PUJ) obstruction is the most common obstructive lesion in children and is usually a result of a congenital intrinsic stenosis at the junction between the renal pelvis and the ureter. Children with known PUJ obstruction should receive antibiotic prophylaxis with co-trimoxazole (trimethoprim and sulfamethoxazole). PUJ obstruction is twice as common in males as in females. If the PUJ obstruction is bilateral and there is severe hydronephrosis with renal compromise then urgent surgical correction is required. In milder or unilateral cases the situation can be managed by observation and follow up ultrasound scans. In this case a unilateral multicystic dysplastic kidney and Wilms' tumour should have been identified on the . but if not often presents with a palpable renal mass at newborn examination or a febrile UTI later in childhood.

9- A 12-year-old boy presents to the surgery with periorbital and mild ankle oedema which has increased over the past few weeks. He has been feeling increasingly tired and lethargic over the past few weeks. Other history of note is a recent upper respiratory tract infection.USS with a cystic mass or complex renal mass as opposed to just straight hydronephrosis. He has periorbital oedema and pitting ankle oedema. Congenital obstructive megaureter would have shown a dilated ureter on USS and posterior urethral valves would also cause dilated ureters if obstructing flow significantly enough to cause hydronephrosis. On examination his BP is 118/72 mmHg. Investigations show: Haemoglobin 124 g/L (135-180) .

White cell count 7.6 mmol/L (3. and IgA nephropathy. The lack of haematuria counts against post stretococcal glomerulonephritis. The important point about making the diagnosis is that the condition responds to corticosteroid therapy in 90% or more of sufferers in childhood within two weeks. The condition is much more common in this age range than membranous nephropathy.5-5) Creatinine 104 μmol/L (60-120) Serum albumin 28 g/L (35-50) Urine Protein ++ Which of the following is the most likely cause? (Please select 1 option) IgA nephropathy Incorrect answer selected Alport's syndrome Membranous nephropathy Minimal change nephropathy This is the correct answer Post streptococcal glomerulonephritis The history of periorbital oedema. but proteinuria and low albumin is typical of minimal change disease. . which also causes proteinuria. although treatment is usually continued for a period of eight weeks.8 ×109/L (4-10) Platelets 191 ×109/L (150-400) Sodium 141 mmol/L (134-143) Potassium 4. normal blood pressure and creatinine.

10Causes of Haematuria A Alport's syndrome B Anaphylactoid purpura C Goodpasture's syndrome D Haemolytic uraemic syndrome E Idiopathic haematuria F IgA nephropathy G Membranous glomerulopathy H Polycystic kidneys I Renal vein thrombosis J Systemic lupus erythematosus From the given list of options.. . choose the single most likely diagnosis which best fits the description below.Alport's syndrome is a familial nephritis associated with haematuria and progressive sensorineural hearing loss.

Alport's syndrome Correct Alport's syndrome is an x-linked condition although inheritance can also be autosomal dominant with variable expression and autosomal recessive. haematuria. Dialysis and transplantation are common in the second and third decades. Treatment is supportive and dialysis may be required.A 13-year-old boy presents with macroscopic haematuria. pallor and oliguria. It affects boys more than girls and tends to present in the second decade with renal involvement. It often follows a prodromal diarrhoeal illness. She has a seizure on day 2. Salmonella. A 5-year-old boy is admitted to hospital with moderate dehydration secondary to diarrhoea and vomiting. Genetic counselling is recommended.The correct answer is Haemolytic uraemic syndrome Haemolytic uraemic syndrome is the commonest cause of acute renal failure in children. Shigella and viruses have been implicated. and acute renal failure. The report states that there is no growth but there is a microscopic haematuria. Alport's syndrome . apart from holding a statement of special educational needs for his severe hearing impairment and short sightedness. Laboratory investigations reveal a microangiopathic haemolytic anaemia. Urinalysis is carried out to exclude infection. develops haematuria and her urine output tails off. Alport's syndrome Incorrect . A baby is born prematurely at 36 weeks gestation following an antepartum haemorrhage. He is otherwise well. Sensorineural deafness and ocular manifestations are common features. resulting in lethargy. Its aetiology is unknown although Escherichia coli 0157.

In the older child it may complicate nephrotic syndrome and congestive cardiac failure. Urine dipstix shows 4+ proteinuria. On examination he has swollen eyelids. 11Proteinuria A Finnish-type nephrotic syndrome B Glomerulonephritis C Henoch-Schonlein purpura D Idiopathic nephrotic syndrome E Interstitial nephritis F Isolated asymptomatic proteinuria G Reflux nephropathy H Shunt nephritis I Systemic lupus erythematosus For each scenario choose the most likely diagnosis: A 2-month-old boy presents with weight gain and decreased frequency of wetting nappies. In neonates. haematuria and have signs of an enlarged kidney.Incorrect . Treatment is supportive although if there is bilateral involvement thrombolytic agents or thrombectomy may be required. abdomen and scrotum. Finnish-type nephrotic syndrome . Patients may present with pain.shock and dehydration. other causes include sepsis.The correct answer is Renal vein thrombosis This describes a baby with a history of asphyxia presenting with renal vein thrombosis.

Recumbent and standing dipstix testing will exclude the latter. Examination (including blood pressure) are normal. In evaluating the child with proteinuria first establish whether the child is symptomatic. and urine contains 3+ proteinuria and 2+ haematuria. . Repeat testing two weeks later confirms the finding. complicated by posthaemorrhagic hydrocephalus requiring VA shunt. This is most likely nephrotic syndrome. Finnish-type nephrotic syndrome Incorrect . Finnish-type nephrotic syndrome Incorrect .The correct answer is Shunt nephritis In the ex-prem with VA shunt there is symptomatic proteinuria and hypertension.84 kg has a stormy neonatal course. A 12-year-old girl presents with 2+ proteinuria discovered incidentally when she registered with her new family doctor.Correct The 2-month-old boy has severe proteinuria with symptoms of fluid retention. A 27/40 infant weighing 0. This is most likely to represent isolated asymptomatic proteinuria or orthostatic proteinuria. most likely shunt nephritis.The correct answer is Isolated asymptomatic proteinuria The 12-year-old girl has persistent asymptomatic proteinuria. At 8 months of age she presents with blood pressure of 120/90 mmHg. and at this age Finnish-type is commonest. If not then repeat the test to see whether the proteinuria is persistent.