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 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.

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

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

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.

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

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

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

SYSTEMIC EXAMINATION

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.

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.

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.

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

On History+Exam…Possibilities are?

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

INVESTIGATIONS

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

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

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

SPOT PROTEIN CREATINIINE RATIO: 9 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: - P

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

CXR:

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

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

DIFFUSE PROLIFERATIVE GLOMERULONEPHRITIS  NORMAL GLOMERULUS DIFFUSE PROLIFERATIVE

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

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:

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

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

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

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

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

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.

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

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

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

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

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

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

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.