2. CASE PRESENTATION By
Dr.Syed Hunain Riaz
PGR

3. Presenting Complaints
A 25 year old male presented in the ER with
Shortness of breath------>1 day
2. Oliguria >1-2 days
3. Body swellings >2 months

4. History of Presenting Ilness
Shortness of breath initially with exertion, later on developed with lying down also. Also developed cough on lying down.
Had oliguria for 1 day, with very small amount of urine eveyday despite adquate intake, no hematuria, no dysuria.
Body swellings developed gradually over 2 months, initially over hands and feet, later on extened to elbows and knees resepectively.

5. No hemoptysis, no chest pain, no h/o palpitations, no h/o wheeze.
No h/o flank pain
Patient developed abdominal distention and complained of fullness.
No h/o malena, hematemesis, fits, or unconsciousness

6. No h/ joint pains or skin rash.
No h/o sore throat preceding these events.

7. Past History
Non-hypertensive, non diabetic, non-asthmatic, no cardiac issue in the past.
Was diagnosed with Pulmonary T.B 2 years back, took ATT for 9 months.
Was not taking any medication, allopathic or homeopathic, prior to these events. And did not take any medication in the past.

8. Personal and Family History
Non smoker and has no addiction, be it I/V, snorting or sniffing
Is a student by occupation.
Has 2 healthy brothers, and 3 healthy sisters, no one has ever suffered from such ailment.
Parents are both healthy and do not have chronic disease

9. General Physical Exam
A young man of average built with a puffy face and swollen limbs, with an IV line in his right forearm, lying propped up in bed, and is drowsy.
Vital Signs:
B.P: 140/70
Pulse: 100/min, regular.
Temp: Afebrile
R/R: 20/min

10. General Exam:
Jaundice: -
Pallor: ++
Cyanosis: -
Clubbing: -
Pedal Edema: +++ ( Pitting, and extending above shins,
while both upper limbs are also have pitting edema ) JVP: Not raised
Nail infarcts: -
Rash: -
Lymph Nodes: No nodes palpable throughout the body.
No prick marks seen on limbs
Neck Veins: Distended

11. SYSTEMIC EXAMINATION

12. Cardiovascular Exam No obvious deformity of Precodium
Apex Beat in left 5th ICS 2 cm medial to midclavicular line. Normal character of apex beat.
S1+S2+0
Heart sounds of normal intensity, no murmur heard.

13. Gastrointestinal Exam
Abdomen distended, with fullness in flanks, and a slit like umbilicus.
Fluid Thrill + ( Ascites )
Liver upper border percussed in right 5th ICS.
Lower border not palpated by dipping method.
Spleen impalpable
Kidneys not palpable.
Bowel Sounds audible, normal frequency.

14. Respiratory Exam Chest has no obvious deformity
Chest movements bilaterally symmetrical
Fine inspiratory crepts in both lungs extending upto midzones
Breath sounds reduced on right base.

15. ENT EXAM
Palatine tonsils not enlarged, no hyperemia or exudates seen on tonsils and post-pharyngeal wall.
No Faucial flare.

16. On History+Exam…Possibilities are?

17. Provisional On History+Exam
Acute on chronic renal faliure ( with underlying nephrotic syndrome )
Cardiomyopathy???
Chronic liver disease?

18. INVESTIGATIONS

19. CBC:
Hb: 6.5 g/dl
TLC: 11,000 /mm3
Platelets: 105,000 /mm3

20. Urine C/E: Sp.Gravity of 1 Revealed No RBC’s or casts
No WBC’S or bacteria
pH of 6
+++Proteinuria

21. RFT’S B/UREA: 286 mg/dl S/ELECTROLYTES S/CREATININE: 6.2
S/Na: 121 mmol
S/K: mmol
S/Ca+: 6.2
S/PO4: 10.4

22. SPOT PROTEIN CREATINIINE RATIO: 9
LFT’S
ALT: 19 units
S/Bilirubin: 0.5
PT/APTT: 14/13 and 35/33
S/Albumin: mg/dl
SPOT PROTEIN CREATINIINE RATIO: 9
HEP B AND C SEROLOGY: - P

23. S/COMPLEMENT LEVELS: Normal RA FACTOR: - ANA: -
ABG’S:
pH: 7.3
HCO3: 16
CO2: 35 mm Hg
O2 SAT: 99 %
pO2: 88mmHg
S/COMPLEMENT LEVELS: Normal
RA FACTOR: -
ANA: -
S/CRYOGLOBULINS: Normal

24. CXR:

25. USG ABDOMEN PELVIS: 14 cm liver, normal echotexture Spleen 11.5 cm
Both Kidneys upto 14 cm in size, swollen with increased parenchymal echogenecity
Gross ascites
No abdominal lymph nodes enlarged

26. USG GUIDED RENAL BIOPSY:
SHOWED PROLIFERATIVE CHANGES IN THE ENDOTHELIUM, EPITHELIUM AND TO SOME EXTENT IN THE MESANGIUM IN ALMOST ALL THE GLOMERULI UNDER LIGHT MICROSCOPY
DIFFUSE PROLIFERATIVE GLOMERULONEPHRITIS

27. DIFFUSE PROLIFERATIVE GLOMERULONEPHRITIS
 NORMAL GLOMERULUS
DIFFUSE
PROLIFERATIVE

28. FINAL DIAGNOSIS ACUTE ON CHRONIC RENAL FALIURE DUE TO
NEPHROTIC SYNDROME
( DIFFUSE PROLIFERATIVE GLOMERULONEPHRITIS )

29. WHAT IS NEPHROTIC SYNDROME?
A SYNDROME CONSISTING OF:
PROTEINURIA >3.5 gms/3.75 m²
PERIPHERAL EDEMA
HYPOALBUMINEMIA <3g/dl
Casued by glomerulonephritis which may:
DIVIDED INTO:
PRIMARY ( IDIOPATHIC )
SECONDARY:

30. KEY PROBLEMS IN NEPHROTIC SYNDROME
PROTEINURIA:
Causes hyperfilteration injury to glomeruli
ACE inhibitors have a role in reducing proteinuria
PROTEIN RESTRICTION when gfr falls below 25 ml/min
INFECTIONS:
Due to immunoglobulin loss in urine

31. HYPERCOAGULABLE STATE:
Due to Hyperlipidemias
Urinary loss of anithrombin III
Increased stickiness of platelets
Hypercoagulable especially with S/Albumin below 2 g/dl
EDEMA:
Salt water restriction is required

32. GLOMERULONEPHRITIS PROLIFERATIVE: DIVIDED INTO ENDOCAPILLARY TYPE
EXTRACAPILLARY TYPE
MESANGIAL
DIFFUSE PROLIFERATIVE AFFECTS ALL OF THE ABOVE
SYSTEMIC DISEASES CAUSING THIS ARE:
SLE
POST-SREPTOCOCCAL
IgA NEPHROPATHY
CRYOGLOBULINEMIC RENAL DISEASE

33. IN OUR CASE, THE CAUSE COULD NOT BE FOUND,
LABLLED TO BE THE IDIOPATHIC TYPE

34. MANAGEMENT
Hi dose i/v diuretics for peripheral edema and fluid overload if present
Salt water restriction and protein restriction to some extent
Treat the systemic disease if present
Anticoagulation prophylactically
Dialysis is rarely required, renal functions improve with treatment of systemic disease or when managed conservatively if is of the idiopathic variety
Proteinuria or hematuria ( if nephritic syndrome ) may persist and tendency to develop CKD increases

35. STEROID THERAPY: IMMUNOSUPPRESSANTS: PULSE THERAPY:
1g/day methylprednisolone for 3 days, then 1mg/kg for 4-6 weeks
5-10 mg/kg for 6 months
Alternative is predniosolone 1mg/kg for 6 months not exceeding 80 mg/day
IMMUNOSUPPRESSANTS:
Mycofenolate mofetil can be usd in refractory cases.

36. BRIEFLY ABOUT OTHER GLOMERULONEPHRITIDIES
MINIMAL CHANGE DISEASE:
Most common type in children, and most steroid responsive type
No changes on light microscopy in glomeruli
REMISSIONS RELAPSES AND RESPONSES
UPTO 80 percent show remission with steroids
DOSE: DAILY 60 mg or ALTERNATE DAY 120mg/day
Steroid dependant and non responders are treated with cyclophosphamide or cyclosporin

37. MEMBRANOUS GLOMERULONEHPRITIS:
Commonest type in adults
Causes can be due to drugs, infections and certain tumors
Spontaneous remissions in precent
Results of Steroid therapy are conflicting, those with heavy proteinuria benefit more from steroids
Cyclophosphamide can be used in steroid non responders

38. MEMBRANOPROLIFERATIVE:
Is an uncommon type
Disease of children and young adults
Etiology includes infectious agents i.e Hep C and autoimmmunity i.e SLE
Treatment with steroids and immunosupressants have not revealed good results in adults
Steroids may prove effective in children u

39. FOCAL SEGMENTAL: Associated with HIV and HEROIN abuse
There is progressive proteinuria decline in GFR
Very less chances of spontaneous remission
Treatments is with steroids and intensive courses of immunosupressants

40. PROGRESS DURING MANAGEMENT
With symptomatic treatment, his urine output became adequate
The anasarca gradually improved
His S/Creatinine came down to 4 mg/dl and urea to below 180 mg/dl
But he developed produtive cough and a massive loculated right sided pleural effusion, along with fever
TLC of 20,000 and neutrophilia

41. Pleural tap was hemorrhagic and its examination is as follows:
Glucose: 150 mg/dl
Protein: 2.5 g/dl
LDH: 70 u/l
RBCS: 50,000 /cmm
WBCS: 2040 /cmm
NEUTROPHILS: 80 %
LYMPHOCYTES: 20%
NO AFB OR MICRO-ORGANISM SEEN

43. THE PATIENT IS BEING MANAGED AS A PARA-PNEUMONIC EFFUSION WITH LOCULATIONS
THE INFECTION, IS ATTRIBUTABLE TO NEPHROTIC SYNDROME, IN WHICH THE CHANCES OF INFECTION INCREASE.