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CASE PRESENTATION

CASE PRESENTATION. By Dr.Syed Hunain Riaz PGR. 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. History of Presenting Ilness.

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CASE PRESENTATION

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  1. CASE PRESENTATION By Dr.Syed Hunain Riaz PGR

  2. 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

  3. 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.

  4. 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

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

  6. 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.

  7. 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

  8. 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

  9. 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

  10. SYSTEMIC EXAMINATION

  11. 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.

  12. 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.

  13. 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.

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

  15. On History+Exam…Possibilities are?

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

  17. INVESTIGATIONS

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

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

  20. RFT’S B/UREA: 286 mg/dl S/CREATININE: 6.2 • S/ELECTROLYTES • S/Na: 121 mmol • S/K: 5.6 mmol • S/Ca+: 6.2 • S/PO4: 10.4

  21. LFT’S ALT: 19 units S/Bilirubin: 0.5 PT/APTT: 14/13 and 35/33 S/Albumin: 0.8 mg/dl • SPOT PROTEIN CREATINIINE RATIO: 9 • HEP B AND C SEROLOGY: -

  22. 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

  23. CXR:

  24. 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

  25. 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

  26. DIFFUSE PROLIFERATIVE GLOMERULONEPHRITIS  NORMAL GLOMERULUS DIFFUSE PROLIFERATIVE

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

  28. 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:

  29. 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

  30. 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

  31. 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

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

  33. 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

  34. STEROID THERAPY: • 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.

  35. 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

  36. MEMBRANOUS GLOMERULONEHPRITIS: • Commonest type in adults • Causes can be due to drugs, infections and certain tumors • Spontaneous remissions in 20-30 precent • Results of Steroid therapy are conflicting, those with heavy proteinuria benefit more from steroids • Cyclophosphamide can be used in steroid non responders

  37. 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

  38. 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

  39. 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

  40. 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

  41. 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.

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