Nephrology Best r

8/13/2019 Nephrology Best r

1/6

A 14-year-old female presents with a two week history of deteriorating puffiness of the face.She has been aware of tiredness and lethargy over the last two months since having acquireda throat infection. Over this period she has gained at least 5 kg in weight over this time. Shehad a similar throat infection aged 10 from which she made an uneventful recovery. There isnothing else of note in the history and she takes no medication. Examination revealsgeneralised puffiness with pitting oedema of the lower limbs. Her blood pressure is 133/86

mmHg with a pulse of 88 bpm. Chest, cardiovascular and abdominal examinations are normal.Her urine dip stick produces +++ protein.

Other investigations reveal:

Haemoglobin 14.6g/dl

White cell count 6 x 1 09/l

Platelets 250 x 109/l

Sodium 136 mmol/l

Potassium 4.0 mmol/l

Chloride 103 mmol/l

Bicarbonate 24 mmol/l

Urea 4.2 mmol/l

Creatinine 93 micromol/l

Serum albumin 25 g/l

24 hour urine protein 4.3 g/l

Which is the best treatment for this patient?

Available marks are shown in brackets

1 ) Observe [0]

2 ) Salt poor albumin [0]3 ) Intravenous frusemide [0]

4 ) High dose corticosteroids [100]

5 ) Cyclophosphamide [0]

Comments:

This patient has the nephrotic syndrome as defined by a triad of hypoalbuminaemia,proteinuria > 3g/24hrs and oedema. The commonest cause of nephrotic syndrome in a child isminimal change disease, which usually responds to a course of high dose corticosteroids.Cyclophosphamide may hasten a remission, but given its cytotoxic profile, should be reservedfor steroid-resistant cases. If possible it should be avoided in childhood. Salt poor albumin and

iv frusemide may be useful adjuncts for managing oedema, but is not curative of proteinuria. Ifthe nephrotic syndrome is left unchecked, complications include Streptococcal sepsis, venousthromboembolism and hypercholesterolaemia

A 6 year old girl with global developmental delay presents with fever and gross haematuria,with visible pink urine and clots. Full term normal delivery with no neonatal complications.Diagnosed with developmental delay at 9 months, and receives special schooling.Immunisations up to date. There is no family or social history of note.

On examination she has a temperature of 38.9C, respirattory rate of 18/min and heart rate of90/min. She has suprapubic tenderness. Urine dipstix testing shows 4+ haematuria, 3+proteinuria and 3+ nitrites.

What is the most likely diagnosis?


2/6


1 ) Benign familial haematuria [0]

2 ) Glomerulonephritis [0]

3 ) Haemorrhagic cystitis [100]

4 ) Henoch-Schonlein Disease [0]

5 ) Urinary tract infection [0]

Comments:

The history of fever, gross haematuria with clots and suprapubic tenderness in a child

with global delay suggests a delayed presentation of UTI resulting in haemorrhagic

cystitis. This should be treated aggressively with antibiotics. A renal USS should be

performed to look for structural abnormalities and to confirm resolution.

Which ONE of the following is true concerning Antidiuretic hormone (ADH)?

Available marks are shown in brackets1 ) Carbamazepine potentiates it's release [100]

2 ) Ethanol potentiates it's release [0]

3 ) It circulates in the blood bound to neurohypophysin [0]

4 ) It is a cyclic octapeptide [0]

5 ) It is synthesised in the posterior pituitary [0]

Comments:ADH is a nonapeptide manufactured in the paraventricular and supra-optic nuclei of thehypothalamus and released from the posterior pituitary. It acts on the collecting ductsimproving water permeability and hence water retention. Carbamazepine as well as otheragents such as thiazides and SSRIs may potentiate its release. Ethanol usually inhibits

release.

A 10 year old boy presents with a history of passing smoky coloured urine for 3 monthsintermittently. On the day of admission he is admitted with severe right loin pain radiating tothe groin coming in spasms every few minutes. Full term normal delivery with no neonatalcomplications. Immunisations up to date. There is no family and social history of note.

On examination he is intermittently writhing around the bed and crying. He is apyrexial butsweating. Respiratory rate is 15/min and heart rate 95/min. He has slight tenderness in theright loin.

What is the most likely diagnosis?


1 ) Benign familial haematuria [0]

2 ) Glomerulonephritis [0]

3 ) Hydronephrosis [0]

4 ) Renal Claculi [100]

5 ) Urinary tract infection [0]

Comments:

The history suggests renal colic. This, combined with the history of probable haematuria,suggest renal stone. There is sometimes a family history. Timed collections for calcium andoxalate should be performed. An USS KUB and plain film X-ray will locate radio-opaquestones. Lithotrypsy or surgery may be required, followed by special diet depending on the


3/6

underlying cause.

A 16 year-old feamle presents with ankle swelling 4 days after having had a sore throat. Onexamination she had a blood pressure of 125/80 mmHg and ankle oedema. Investigationsreveal:

creatinine 90 umol/L (50-100)

albumin 25 g/L (37-45)24 hour urinary protein 9g (3g/day- as in this case) an 8-12 week course of prednisoloneshould be prescribed. If the proteinuria is


4/6

1 ) a tendency to relapse [0]

2 ) full renal recovery [100]

3 ) permanent renal impairment [0]

4 ) persistent hypertension [0]

5 ) persistent proteinuria [0]

Comments:30-40% of children achieve spontaneous remission and 90% achieve remission following 8weeks treatment with high dose steroids. However in adults only around 50% achieveremission.

Which of the following is characteristic of Bartter's Syndrome?


1 ) Secondary hyperaldosteronism [100]

2 ) Hyperkalaemia [0]

3 ) Metabolic acidosis [0]

4 ) Reduced renal concentrating ability [0]

5 ) Diarrhoea [0]

Comments:

Barter's Syndromeis a rare form of renal potassium wasting characterised by hypokalaemia,normotension, and elevated renin and aldosterone levels. It is occasionally autosomalrecessive. There is hyperplasia of the juxtaglomerular apparatus in most cases. It is postulatedthat the primary defect is in chloride reabsorption in the ascending limb, resulting in sodiumchloride excessively presented to the distal tubule, with sodium reabsorption in exchange forpotassium, resulting in urinary sodium wasting. There is secondary stimulation ofprostaglandin synthesis, which activates the renin angiotensin aldosterone system which

exacerbates the renal potassium wasting. Growth failure, muscle weakness, constipation,polyuria and dehydration are typical in younger children with muscle weakness, cramps orcarpopedal spasms present in older children. The potassium is


5/6


1 ) Metformin [0]

2 ) Metformin plus Gliclazide [0]

3 ) Rosiglitazone [0]

4 ) Sliding scale IV insulin infusion [100]

5 ) Subcutaneous insulin mixture [0]

Comments:This patient has a metabolic acidosis with pH of 7.3 and low bicarbonate likely due to sepsis.She is a type 2 diabetic with uncontrolled hyperglycaemia but is ulikely to have diabeticketoacidosis because the urine is negative for ketones.It is important that her glycaemia is controlled to promote recovery from the sepsis this is bestachieved with intravenous insulin initially.

Which of the following is associated with hypernatraemia.

Available marks are shown in brackets1 ) Cholera [0]

2 ) Pyloric stenosis [0]

3 ) Inappropriate ADH secretion [0]

4 ) Diabetes insipidus [100]

5 ) Diabetes mellitus [0]

Comments:

Cholera results in excessive water and sodium losses and hence hyponatraemia. Pyloricstenosis typically results in hypokalaemic, hypochloraemic, metabolic alkalosis and not causehypernatraemia. Inappropriate ADH secretion causes hyponatraemia. In Diabetes insipidusfailure to concentrate urine results in hypernatraemic dehydration with hyperosmolarity. Poorlycontrolled diabetes results in osmotic diuresis and renal sodium loss.

Which of the following is a known risk factor for the development of chronic rejection of kidneytransplantation :-


1 ) Age [0]

2 ) Anti-smooth muscle antibodies [0]

3 ) Presence of anti-HLA antibodies [100]

4 ) Smoking [0]

5 ) Toxoplasma infection [0]

Comments:

Chronic rejection is characterised by fibrosis of normal organ structures. The pathogenesis ofchronic rejection is not clear- some prefer the term "chronic allograft dysfunction" since bothimmunological (antigen-dependent and antigen-independent) and non-immunological factorshave been identified.

Cell-mediated and humoral immune mechanisms have been implicated in this form of graftrejection. It has also been suggested that rejection is a response to chronic ischaemia causedby injury to endothelial cells. Proliferation of intimal smooth muscle is observed leading tovascular occlusion. The fact that chronic rejection is rare in transplants between HLA-identicalsiblings suggests that HLA-antigen dependent immunological factors are important. Riskfactors include: number of previous acute rejection episodes, presence of anti-HLA antibodies,anti-endothelial antibodies, CMV infection, dyslipidaemia, hypertension, functional mass of the


6/6

donor kidney and delayed graft function (a clinical manifestation of ischaemia/reperfusioninjury)

A 2 week old male child is brought to casualty by his concerned parents with diarrhoea andvomiting. He is the first child of a young couple. Examination reveals few features besidesobvious dehydration. He is noted to have a penile length of 3.5cms. Which of the following isthe most appropriate inital treatment for this patient?


1 ) Cow's milk allergy is the most likely diagnosis [0]

2 ) gluten-enteropathy should be excluded [0]

3 ) Requires urgent treatment with oral steroids [0]

4 ) Requires urgent treatment with IV normal saline [100]

5 ) Rota virus gastroenteritis is the most likely diagnosis [0]

Comments:The history suggests a diagnosis of classical congenital adrenal hyperplasia which is

commonly due to 21 hydroxylase deficiency. A variable presentation is typical but neonatalpresentations include salt losing crisis, penile development in the male virilisation andambiguous genitialia in females. Patients should initially be resuscitated with fluid, usuallysaline and if suspicious, urgent biochemistry requested for cortisol, 17OHP etc prior toadministration of intravenous steroids.

Documents

Nephrology Best r