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Question 1

Chew Farn Ye
1) A 60 yr old smoker presents with exertional
dyspnoea for 2 years. He has complained of ankle
swelling recent 1 month
On examination, there is bilateral pitting pedal
oedema, central cyanosis and elevated JVP. There is
scattered ronchi heard on both lung. Cardiac
examination reveal left parasternal heave and RV S3

• Mention the probable diagnosis and justify it

• Describe the investigation and treatment of this


patients
Answer
• Diagnosis: Cor pulmonale secondary to chronic
obstructive airway disease
• Justification:
• COAD because of chronic smokers, exertional
dyspnoea for 2 years and scattered ronchi on both lung
• Cor pulmonale as signs and symptoms suggestive of
right ventricular failure such as recent onset of ankle
swelling (1 month), bilateral pitting pedal oedema,
central cyanosis, raised JVP, left parasternal heave and
S3
Investigation
a) 12 lead ECG:
-to look structural heart disease (hypertrophy/enlargement)
b) CXR
- to identify pulmonary congestion, cardiac size and shape,
- look for COPD
c) Blood tests:
- FBC, renal function, liver function, serum glucose, ABG
d) SpO2
e) ) Echocardiography and lung function test
- when patient is stable to assess the condition of heart and lung
Other investigation
• Sputum: culture and sensitivity, gram stain
Treatment
-Admit the patient (due to presence of peripheral odema)
-Supplementary Oxygen therapy if SpO2 < 92% (24-28%
via nasal mask)
-Start nebulised bronchodilater (SABA eg: salbutamol
2.5mg and SAAC eg: iptratropium bromide 0.5 mg) 4-
6hourly
- Oral corticosteroid of 30 mg/day for 7-14 days
- start patient on IV furosemide 40 mg/day
- start patient on ACE inhibitor
Subsequent managment in ward
- review SpO2 and ABG after 30 -60 minutes (if improving,
increasing monitoring time interval)
- monitor the patients conditions
- if not improving:
• increase the frequency of nebulised SAAC and SABA
• Administer IV aminophylline 250 mg over 20 minutes
followed by infusion (250 mg in 500 ml of NS) at rate of
0.1-0.3 mg/kg/hr (in heart failure patient)
• Look for possible cause of deteriorating conditions eg:
pneumonia
(sputum culture and sensitivity, repeat CXR, repeat FBC and
ABG) and treat accordingly eg: amoxicillin 1 g three times a
day (complicated COPD)
- If patient conditions continue to deteriorate and
subsequent (or initial) ABG showing type II respiratory
failure:
• • Start patient on non-invasive ventilation
Moderate to severe dyspnoea with use of accessory muscles and
paradoxical abdominal
motion.
• Respiratory rate > 25 breaths per minute.
• Moderate to severe acidosis (pH 7.25 - 7.35) and/or hypercapnia
[PaCO2 > 6.0 kPa (45 mmHg)].

• If NIV failure, start on mechanical ventilation


- other managements:
• monitoring of fluid balance, deep vein thrombosis
prophylaxis with S/C heparin in immobilise patient
• supplementary nutrition
• sputum clearance
Discharging criteria
- Patients with AECOPD can be discharged when:
• Inhaled bronchodilator therapy is required not more frequently
than every 4 hours
• Patient, if previously ambulatory, is able to walk across the room
• Patient is able to eat and sleep without frequent awakening by
dyspnoea
• Patient has been clinically stable for 12-24 hours
• ABG or SpO2 have been stable for at least 12-24 hours
• Patient (or home caregiver) understands the disease and its
management (including correct use of medications) at home
• Follow-up has been organised. (within 8 weeks of discharge)
PBL 6
(2) A 42 year old female presents
with bilateral swelling of the feet
and facial puffiness of 1 month
duration.
Clinically, there is bilateral pitting
oedema, ascites, and right
hydrothorax. There is normal JVP.
What are the differential diagnoses?

• Chronic Kidney disease


• Chronic Liver disease
• Congestive Cardiac failure
• Chronic lung disease, eg : emphysema
What further questions should be asked in history to aid her
diagnois?

Renal :
• swelling ; onset, duration, extension, progression, tenderness
• symptoms ; easy fatiguablity, exertional palpitations, chest pain,
generalised weakness, malaise, orthopnea, paroxysmal
nortunal dyspnea, tinnitus, blackouts, itchiness, muscle cramps
& twitches, vomiting, nausea, headache
• signs ; anemia, decreased mental alertness, loss of weight
• bladder habbits ; oliguria, frothy, retention, incontinence,
colour changes, hematuria, frequancy, tenderness
• co-morbitidies ; diabetes mellitus, hypertension
• diet control, compliance to medications
What are the importance of general and
systemic examination in her.
• To look for uraemic encephalopathy and uraemic
dermopathy and underlying essential hypertension
• To prevent fall into coma
 Drowsiness and flapping tremors (uraemic encephalopathy)
 Pallor (anemia)
 White nails and half nails – proximal white and distal half of
nail pigmented brown-Lindsay’s nail (hypoalbunemia)
 Uraemic frost (flaky skin deposits of nitrogenous waste
products)
 Kussmaul’s respiration (metabolic acidosis)
 Itch marks (pruritis)
 Hyperpigmentation (Kyrle’s disease- Idiopathic perforating
dermatosis: papular lesions with central keratin & necrosis)
 Easy bruising (platelet dysfunction and thrombocytopenia)
 Recurrent infections (decreased immunity and pancytopenia
due to bone marrow suppression-uraemic toxicity)
 Restless leg syndrome (Eckbom’s disease- due to anemia
&hyperphosphataemia)
 Bony pains (renal osteodystrophy)
• Hypertension
 Pulse and blood pressure
 Peripheral signs for CCF
 CVS : displaced apical impulse, heaving in
nature, loud A2 at aortic area
Question 3:
A 12 years old boy present with smoky
urine of 1 week duration. This is
associated with multiple skin lesions both
his lower limbs recent 2 weeks.
1.What is the most probable
diagnosis?
• Acute glomerulonephritis
(Post-streptococcus glomerulonephritis)
– 12 years old (common in pediatric)
– boy
– smoky urine(hematuria)
– multiple skin lesions both his lower
limbs(impetigo)
2.What is the pathogenesis of the
disease?
3.What investigations you would
like to do?
• Blood culture
– Beta hemolysis
• transparent
• Gram stain-gram positive cocci
Renal biopsy immunofluorescenc
e•
Stary sky
• granular

Light microscope
• Hypercellularity
• Thickening Electron microscope
• Hump
• Subendothelial
deposit
4.What are the complications of
this condition?
 Severe proteinuria
• Pulmonary edema
• generalised anasarca
 Hypertension
• Hypertensive retinopathy
• Hypertensive encephalopathy
 Rapidly progressive GN
 Chronic renal failure
 Nephrotic syndrome(5 %)
 Sclerosis(rare)
Prognosis

• Usually good in children


• In small number of adult develop
hypertension and chronic kidney disease
PBL 6- Question 4

Nasheel Kaur Gill


Group E1
A 70 year old lady presented with 1 day history
of breathlessness. This was preceded by severe
bout diarrhoea and vomiting 2 days ago, from
which she recovered with home medicine. She
complains of passing reduced amount of urine.
1. What is the likely diagnosis
- Acute kidney injury secondary to dehydration
- Acute: symptoms were within a duration of days
- AKI: oliguria
- Pre renal: volume loss due to vomiting and
diarrhoea
2. Describe the investigations to arrive at the
diagnosis
- Blood
- Urea and creatinine: Sudden increase (compared with baseline)
- Electrolytes: if potassium is >6mmol/L, treat urgently
- Calcium and phosphate (CKD)
- Albumin (nephrotic)
- Full blood count (anemia in CKD)
- Urinalysis and microscopy
- Renal ultrasound
- Culture (blood,urine,sputum): treat all infections
- Chest X-ray: pulmonary edema
- ECG: electrolyte abnormalities
3. Describe the principles of the management of her condition

i. Haemodynamic status
- Replace with IV fluid (Hartmann’s/Ringer’s lactate) or blood
- Monitor using central venous line
- Critically ill patients may require inotropic agents to restore BP
ii. Hyperkalemia and acidosis
- If serum potassium >6.5mmol/L, give glucose and insulin
- Severe acidosis: give sodium bicarbonate
iii. Cardiopulmonary complications
- Pulmonary edema can develop due to increased administration of fluid
while urine output is still low
iv. Treat electrolyte imbalance
v. Adequate nutritional support
vi. Haemodialysis in AKI
Recovery from AKI
- Begins with gradual return of urine output
- Steady improvement of plasma biochemistry
4. Describe pathogenesis of acute renal
failure

Pre renal Azotemia ( inadequate renal plasma


flow and intraglomerular hydrostatic pressure
to support normal GFR)

Acute tubular necrosis

Inflammatory changes, focal breaks in tubular


basement membrane and interstitial edema
Renal Causes
- Sepsis
- Ischaemia
- Post operative
- Burns and acute pancreatitis
- Nephrotoxins

Post renal AKI occurs when the flow of urine is acutely


blocked (completely/partially)

Increase retrograde hydrostatic pressure and interfere


with glomerular filtration
5. How do you differentiate acute renal failure from chronic renal
failure
AKI Features CKD
Sudden, reversible loss of Definition Irreversible deterioration in
renal function renal function

Days to weeks Duration Years


Pre renal, renal, post renal Causes DM, Hypertension, SLE,
Glomerular disease

Sudden oliguria Urine output Polyuria in early stages


Absent Anemia Present
Normal Kidney size Decreased
Normal Baseline Cr Abnormal
Normal PTH Increased
Absent Renal osteodystrophy Present
Q5
A 60 y/o diabetic and hypertensive patient for
20 years, who is currently on insulin and
enalapril, undergoes routine blood
investigations. He is otherwise well

Report is as follow
– BUN – 30mmol/l
– Serum creatinine 450umol/L
– Potassium 6mmol/L
– Sodium 140mmol/L
Interpret his report and explain his condition
• BUN increased
• sCR increased
• K+ increased
• Sodium increased

• BUN:sCR ration = 106:1 (>20:1)


– Pre-renal cause of kidney disease
• Electrolyte imbalance 2o to chronic kidney
disease
• How do you counsel him
– Recognise symptoms of electrolyte imbalance
– Importance of control of blood pressure
(130/80mmHg)
– Importance of blood sugar level control(&HbA1c)
– Importance of compliance to medication
– Diet control (protein and salt restriction)
– Counsel for dialysis if required
Calculate the eGFR in this patient
• GFR = 186 x (sCR/88.4)-1.154 x (age)-0.203
x 0.742(F) x 1.212(N)
= 186 x (450/88.4)-1.154 x (60)-0.203
= 12.39ml/min/1.73m2
6. A 70 year-old known hypertensive
of 10 years duration presents with
difficulty in passing urine, pruritus and
anorexia. His investigations revealed
BUN – 36 mmol/L, creatinine
380umol/L
a) What other physical signs will you look
for in this patient?
• Kussmaul breathing (due to metabolic
acidosis)
• Uraemic frost
• Scratch marks
• Ecchymosis
• Hands : Half-and-half nails, ‘Brown line’
pigmentation of nails
• Conjunctival pallor
• Yellow complexion
• Raised JVP, pulsus paradoxus (pericardial
tamponade)
• Pedal oedema, Ascites
• CVS : Pericardial friction rub
• RS : Pulmonary oedema, Pleural effusion
• Peripheral neuropathy : Absent reflexes,
Reduced sensation, paraesthesia
b) What is the diagnosis?
• Chronic kidney disease secondary to
hypertension
c) What is the complication of the
condition?
i) Immune dysfunction (impaired cellular &
humoral immunity)
ii) Haematological
 Impaired platelet function  Bleeding
tendency
 Anaemia
iii) Electrolyte abnormalities
• Hyperkalemia
• Metabolic acidosis  ↑ tissue catabolism, ↓ protein
synthesis, exacerbate bone disease, decline in renal
function
• Fluid retention

iv) Neurological & muscle function


• Generalised myopathy (poor nutrition, Vit. D deficiency,
electrolyte abnormalities)
• Muscle cramps
• Restless leg syndrome
• Sensory & motor neuropathy
v) Cardiovascular disease
• Pericarditis  Pericardial
tamponade/Constrictive pericarditis
• Heart failure
• Vascular calcification

vi) Metabolic bone disease


• Renal osteodystrophy : Osteitis fibrosa cystica,
Adynamic bone disease, Osteomalacia
d) What is the indication of dialysis?

• GFR < 15 mL/min/1.73m2 in the presence of


refractory metabolic acidosis, uraemic
symptoms, decline in nutritional status and
volume overload not responding to
medications
e) Describe the principles of his
investigation and treatment
Investigation principle :
• Identify underlying cause, reversible factors
that may worsen renal function
• Screen for complications
• Screen for CVS risk factors
Treatment principle:
• Treat underlying cause
• Slow progression of kidney damage
• Management of complications
• Preparation for renal replacement therapy &
referral to nephrologist (if indicated)
Slow disease progression
1. Antihypertensive therapy target :
• 130/80 mmHg for uncomplicated CKD
• 125/75 mmHg for CKD complicated by
significant proteinuria > 1g/day

2. Reduction of proteinuria : ACEi, ARBs


• Also reduces BP and risk of cardiovascular
events
3. Lipid-lowering therapy

4. Dietary and lifestyle intervention


• Prevent excessive consumption of protein,
adequate calorific intake
• Stop smoking
• Exercise and weight loss
Management of complications
Complications Treatment
Anaemia (Aim for Hb 10 – 20 Check haematinics & replace
g/dL) iron/B12/folate
Recombinant human erythropoietin

Acidosis Maintain plasma bicarbonate > 22 mmol/L


by giving sodium bicarbonate
supplements (start 1g TDS, increase as
required)
Alternative : CaCO3 (up to 3g daily)
Oedema Loop diuretics (e.g. furosemide 250 mg-
2g/24 hr)
Restriction of fluid & sodium intake (about
100 mmol/day)

Hyperkalaemia Limiting potassium intake (70 mmol/day)


Potassium-binding resins
Complications Treatment
Renal bone disease Active Vit. D metabolites (1-a-
(Maintain phosphate values at 1.8 hydroxyvitamin D / 1,25-
mmol/L [5.6 mg/dL]) dihydroxyvitamin D)
Restrict diet with high phosphate
content
Phosphate binding drugs : CaCO3,
Al(OH)3
Parathyroidectomy : For 30
hyperparathyroidism

Restless legs/cramps Check ferritin


Clonazepam (0.5 – 2 mg daily) or
gabapentin
Quinine (300 mg) for cramps
Referral to nephrologist
PBL QUESTION 7

As a house surgeon, you get a call from the


ward nurse that a patient's serum potassium
is reported as 6.8 mmol/L

* Normal serum Potassium = 3.5-5.5 mmol/L


1) How to approach this problem?

Symptoms- (MURDER)
1. Muscle weakness
2. Urine(Oliguria/ Anuria)
3. Respiratory distress
4. Decreased cardiac
contractility
5. ECG changes
6. Reflex(hyperreflexia/anrefle
xia)
Also ask for ;
1. Underlying co-morbid - kidney disease/ HF
2. Drugs - Beta-blocker/ Digitalis/ NSAIDs/ ACEI/ ARBs/ Heparin /
Potassium-sparing anti diuretics-----> Stop medications immediately
3. Diet history - evidence of excess dietary potassium intake
* Melon/bananas/citrus juice(unusual habits that contains
exclusively these)
* Traditional medications / herbal supplements
* Sport drinks/ salt substitute
INVESTIGATIONS
1. FBC- Thrombocytosis/ leukocytosis may cause hyperkalemia
2. Electrolytes(potassium/calcium), creatinine & bicarbonate level
- the rapidity change in the potassium level influences the symptoms
observed at various potassium levels
- for evaluation of renal status
- if patient has renal failure(hypocalcemia can exacerbate cardiac
rhythm disturbance)
2) Blood Glucose level- in pts with DM which complicate with DKA
3) Digoxin level - if pts is on digitalis medications
4) Arterial/venous blood gases - if acidosis is suspected
5) Urinalysis- if signs of renal insufficiency without an already known
cause are present(to look for evidence of glomerulonephritis)
2) Describe the ECG changes in the above
abnormalities
ECG changes in Hyperkalemia
1. Tall, symmetrically peaked T waves
2. Prolonged PR segment
3. Loss of P waves
3) How do you treat the condition in A&E ?

3 main aims :-
1. Stabilise the cell
membrane
potential- by IV
Calcium Gluconate
(10mL of 10%
solution)
2) Shift K+ into the cells -
* Inhaled beta-adrenoceptor agonist( Salbutamol)
* IV glucose (50mL of 50% solution) & insulin(5 U
Actrapid)
* IV sodium bicarbonate
3) Remove K from body by,
A. IV Furosemide & Normal Saline
- if renal function reasonably
preserved
- accompanied by normal saline if
hypovolemia present.
B. Ion exchange resin ( eg. Resonium
orally/rectally) ,acting through the GIT
C. Urgent dialysis
Question 8
NORA ELYNA BINTI ANUAR
J1

A 30 year old woman comes with history


of burning and frequent micturition with
shaking chills of 1 day duration.
What physical sign do you look for?
Fever
Hematuria
Suprapubic tenderness
Cloudy urine with an offensive smell
Sensation of bladder fullness or lower abdominal
discomfort
Loin pain

What is the most likely diagnosis?


Urinary tract infection
What are the potential complications?
• Recurrent infections
• Permanent kidney damage due to an
untreated UTI
• Pregnant woman: Increased risk of
delivering low birth weight or premature
babies
• Urethral stricture in males from recurrent
urethritis
• Sepsis
Describe her treatment.
1. Fever: Paracetamol 500 mg qd
2. Antibiotics
• While awaiting for urine culture result, treatment can be
started.
• Treatment for 3 days is the norm and less likely to alter
the bowel flora however severe infection may require 7-
14 days.
• Trimethoprim is the usual choice.
• Alternatives for organisms that are resistant to
trimethoprim: Nitrofurantoin, ciprofloxacin
• Safe in pregnancy: Penicillin and cephalosporin
• Unsafe in pregnancy: Trimethoprim, sulfonamides,
quinolones, tetracyclines
• Trimethoprim: 200 mg bd
• Nitrofurantoin: 5o mg qds
• Ciprofloxacin: 500 mg bd
• Amoxicillin: 250 mg tds
Question 9

Thanushiya Mageswaran
Group J1
• A 29 y/o man presents with hx of severe
burning micturition & increased frequency of
micturition. It is associated with pus-like
urethral discharge
What other history would you like to take?
• Elaboration of CC: onset, progression, duration of
burning micturition, description of discharge
• Any associated factors: suprapubic pain, intense
desire to pass more urine after micturition,
presence of any hematuria, fever with chills & rigor,
any itching
• To rule out other possible Dx: presence of any loin
to groin pain, any perineal tenderness, any
tenderness on ejaculation, any joint pain, visual
changes, rashes
• Past Medical Hx: known diabetic? Any previous
catheter insertion? Any neurological disorder
diagnosed before? Any radiation done to the
pelvic region? Any cancers diagnosed prior and is
the patient on cyclophosphamide?

• Personal Hx: any sexual promiscuity? Partner


known to have any STD diagnosed before?
What is the likely diagnosis?
• UTI with cystitis or urethritis
• Urethritis, either Gonococcal or non-
Gonococcal urethritis
• Prostatitis or prostatic abscess
• Depending on duration of complaint, bladder
outflow obstruction has to be ruled out
What investigation would you like to do?
URINE
• Dipstick: nitrite, leucocyte esterase, glucose
• UFEME
• C&S
• NAAT for N. gonorrheae, Chlamydia

BLOOD
• FBC
• Cultures if sepsis is suspected

IMAGING
• Renal ultrasound
• USG of the prostate and biopsy (if required)

CYSTOSCOPY
*may be required to investigate persistent problem/ TRO bladder outflow obstruction
What Tx would you like to initiate?
PROBABLE DX DRUG REGIMEN
Cystitis Trimethoprim 200mg BD for 3 days, can go up to 7-10
days
Pyelonephritis Co-amoxiclav 500/125mg TID for 14 days
Ciprofloxacin 500mg BD for 7 days
Acute prostatitis Trimethoprim 200mg BD for 28 days

OTHER MEASURES:
1. Advice fluid intake of at least 2L/day
2. Regular, complete emptying of bladder
PBL Question 10

Suniljeet Singh Sandhu


J1
131303212
1. An elderly woman has been on treatment for
her knee pain for years. She presents with
facial swelling and unusual tiredness of 1
month duration.
1. Mention the differential diagnosis?

a) Cushings syndrome caused by long term


corticosteroid use. (around 15 mg/day for 3
mnths)
b) Hypothyroidism due to corticosteroid use.
(decrease TSH secretion by anterior pituitary)
c) NSAID toxicity (renal failure leading to
sodium and water retention worsening CCF)
2. What investigations would you like to
perform?
For Cushings Syndrome
- late-night salivary cortisol, 24 hour urine free
cortisol, low-dose dexamethasone suppression
test or over night dexamethasone suppression
test.
To determine cause of Cushings:
- Plasma ACTH 1.1pmol/3.3pmol
- HDDST (pituitary dependant causes : ACTH
supressed)
• Thyroid function test – T3,T4,TSH
• Renal Function Tests – To assess kidney
function
• Cardiac function – X-ray, echo
Question 11
A 79 years old hypertensive woman was brought to A&E due to
increasing confusion .She has been on bendroflumethiazide 2.5 mg
once a day. Her vital sign were stables
• BUSE
• Na 113 mmol/L ( 136-145 mmol/L ) impression : hyponatremia
• Potassium 3.3mmol/L (3.5 -5.1 mmol /L) : hypokalemia
• Urea 3.4mmol /L (3.2-8.2 mmo l/L) : Normal
• Creatinine 64mmol/L ( 44-97 mmol /L) :Normal
• Glucose 5mmol/L :Normal
• Urine osmolality 318 mosmol /L (360 -1200mosmol/L )
• Urinary Na 55 mmol /L ( 5-300mmol/L)
• Urine FEME no protein ,no blood
• CXR NL
• A )What is the likely cause of this patient’s
confusion ?

• Cerebral oedema due to hyponatremia.


• When hyponatremia occurs, the resulting
decrease inplama osmolality causes water
movement into the brain in response to the
osmotic gradient, thus causing cerebral
oedema.
• B) What are the potentials causes ?

• Diuretics ( Bendroflumethiazide ).
• Diuretics cause the kidneys to remove more
sodium and water from kidney, thereby
lowering blood pressure. This cause the
hyponatremia.
• C) how would you correct this patient ?

• Stop taking drug


• Infusion of 3% sodium chloride (hypertonic
saline) 2g/kg
• Osmotheraphy using mannitol
12. A 24 year-old man present with h/o
fever, cough, and pleuritic chest pain for 1
day. The next day, he becomes drowsy and is
brought to the hospital. On examination,
there is bronchial breathing, with coarse
crepitation over the right mid and lower
zone of the lungs. He was drowsy. His
biochemical investigations were normal
except sodium, which was 110mmol/L.
(a) What is the cause for this abnormality

- Syndrome of inappropriate antidiuretic


hormone ( SIADH ) secondary to pneumonia.
(b) How to confirm it?
-Plasma and urine electrolytes and osmolarity are usually the only tests to classify the
hyponatremia.

Features of the plasma and urine


(i)Low plasma sodium concentration (typically < 130 mmol/L)

(ii)Low plasma osmolality (< 270 mmol/kg)

(iii) Urine osmolality not minimally low (typically > 150 mmol/kg)

(iv)Urine sodium concentration not minimally low (> 30 mmol/L)

(v)Low-normal plasma urea, creatinine, uric acid

(vi) Exclusion of other causes of hyponatraemia

(vii) Appropriate clinical context ( below)


(c) How to treat him?
(i) Treat the underlying cause (pneumonia)

(ii) Fluid intake should be restricted to 500–1000 mL daily.If


tolerated, and complied with, this will correct the biochemical
abnormalities in almost every case.

(iii) Plasma osmolality, serum sodium and body weight should be


measured frequently.

(iv) If water restriction is poorly tolerated or ineffective,


demeclocycline (600–1200 mg daily) is given; this inhibits the
action of vasopressin on the kidney, causing a reversible form of
nephrogenic diabetes insipidus. It often, however, causes
photosensitive rashes.
(v) When the syndrome is very severe (i.e.
acute and symptomatic), hypertonic saline may
be indicated but this is potentially dangerous
and should only be used with extreme caution

(vi) Vasopressin V2 antagonists, e.g. tolvaptan


15 mg daily, are being used with good results.
13. A 20 year-old woman presents with history
of smoky urine since 1 month. She had one
episode of haemoptysis. Her urine examination
reveals plenty of RBCs on microscopy
(a) Mention a likely diagnosis.
-Acute Glomerulonephritis (Goodpasture’s
disease)
(b) How to investigate her further?
1. Urine analysis
Shows low grade proteinuria
Gross or microscopic hematuria and red blood cell casts
Increase level of urea

2. Full blood count


Increase in white cell count. Leukocytosis
Anaemia may be observed
Increased creatinine levels
Elevated blood urea nitrogen

3. Imaging
Kidney X ray, Ultrasound or CT scan

4. Kidney biopsy
Question 14
• A 33 year old male presents to the ED complaining of
shortness of breath and cough of 10days duration. He
must sleep in chair due to orthopnea.He also complains of
severe fatigue and a mild diffuse headache. He reports no
prior medical priblems and surgeries. He quit smoking 1
year ago and denies alcohol and drug use. He has a strong
family history of hypertension. The review of systems is
otherwise negative.

• On physical examination, his temperature is 36.8C, pulse


104, respiratory rate 16, and blood pressure 200/118.
Oxygen saturation on the room air is 98%. On chest exam,
there are bibasilar crackles and dullness to percussion at
the lung bases. Cardiac auscultation is unremarkable.
Abdominal examination reveals bilateral palpable kidneys.
• His chest xray shows cardiomegaly and
bilateral small pleural effusions. An ECG shows
sinus tachycardia with left atrial enlargement
and left ventricular hypertrophy.
• His labarotory tests are as follows, troponin
negative, hemoglobin 9.1g/dL, WBC count and
platelets normal, Na 136meq/L, K 5.4 meq/L,
Cl 96 meq/L, HCO3 19meq/L, BUN 108
mmol/L, Cr 1100.9umol/L
Mention the likely diagnosis

Chronic Kidney Disease due to Adult


Polycystic Kidney Disease with underlying CCF
How to further investigate him?
• Urine analysis and microscopy
• Urine biochemistry
• Urea and creatinine
• Electrolytes to identify hyperkalaemia
• Calcium, phosphate and parathyroid hormone for the assessment
of renal osteodystrophy
• Serum albumin
• Full blood count to check for anaemia
• Lipids, glucose with HBA1c for the risk of cardiovascular diseases
• Echocardiography
• Renal ultrasound
Describe principles of his treatment
• Antihypertensive therapy
• Sodium nitroprusside 0.3 mcg/kg/min
• Various targets have been suggested, such as 130/80 mmHg for
uncomplicated CKD and 125/75 mmHg for CKD complicated by
significant proteinuria

• Reduction of proteinuria- diet restriction of protein to 60g/day with


adequate intake of calories to prevent malnutrition.
• ACE inhibitors and ARBs which also helps to reduce fluid overload and
improve cardiovascular functions.

• Treatment of anemia
• Recombinant human erythropoietin ( epotin a)
• Target haemoglobin is between 10-12 g/dL
• 50U/kg of epotin over 1-5mins, 3 times weekly
• Dietary sodium intake limited to about 100 mmol/day and
potassium to 70mmol/day
• If hyperkalaemia occurs drug therapy should be reviewed,
to reduce or stop potassium sparing diuretics
• Correction of acidosis. Plasma bicarbonate should be
maintained above 22mmol/L by giving sodium bicarbonate

• Lipid lowering therapy


• Statins

• Maintaining fluid and electrolyte balance. Around 3L/day


of fluid intake is desirable.
• Dietary and lifestyle interventions
• Advice patients to stop smoking

• Renal replacement therapy


-Hemodialysis or peritoneal dialysis

• Renal transplantation
Complications
• Anemia
• renal osteodystrophy
• skin disease- pruritus, dry skin, porphyria cutanea tarda
• Nephrogenic systemic fibrosis
• Gastrointestinal complications – peptic ulceration, reduced gastric
emptying, reflux esophagitis,acute pancreatitis
• Metabolic abnormalities – gout, insulin and lipid metabolism
abnormalities
• Endocrine abnormalities – hyperprolactinemia, decreased serum
testosterone levels, increased LH hormones in both sexes ,
abnormal thyroid hormone
• Muscle dysfunction
• CNS- seizures,asterixis, tremors, myoclonus
due to severe uraemia.
- dialysis dementia due to aluminium
intoxication
- psychoses, anxiety, depression
• Peripheral nervous system
- median nerve compression
- restless leg syndrome
• Calciphylaxis
- calcific uremic arteriolopathy, life
threathening
-painful non healing eschars
• CVS- increased incidence of cardiovascular
disease
• Malignancy
How do u screen the family members?

• Mutation screening of ADPDK1 or ADPDK2 for


adult polycystic kidney disease

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