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Interesting Case Presentation. Rajesh NG Department of Pathology JIPMER Puducherry. Clinical presentation. 58 year old male manual labourer presented with loose stools – 4 days Several episodes of watery stools, non blood stained ↓ ed urine output – 1 day
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Interesting Case Presentation Rajesh NG Department of Pathology JIPMER Puducherry
Clinical presentation • 58 year old male manual labourer presented with loose stools – 4 days • Several episodes of watery stools, non blood stained • ↓ed urine output – 1 day • Minimal breathing difficulty • No h/o cough, expectoration • Frequent NSAID intake for arthritis • Not a known diabetic or hypertensive
Clinical course • Day 1 – conscious, oriented • Elevated JVP, minimal bilateral basal creps • Provisional diagnosis – Acute Kidney Injury (ATN) in oliguric phase • Probable Etiology : Acute Gastroenteritis • Serum creatinine (pre dialysis) : 15.2mg/dl • Urine – plenty of pus cells, [No RBC Casts] • Culture (Blood, Urine & tracheal aspirate) : E. coli • 2 hour Hemodialysis on day 1 • Oliguria + Volume overload + Azotemia • Unresponsive to fluid & diuretic challenge
Initial Investigations • Hemoglobin – 12.8 g/dl • Total WBC count – 8700 • Platelet count – 179000 • Blood urea – 105 mg/dl • Serum creatinine – 5.6 mg/dl (Post Dialysis) • Na+ - 139 mEq.L, K+ - 3.3mEq/L • Total protein 6.4 g/L • Serum Albumin 3.2 g/dl • SGOT – 39 IU/L, SGPT – 17 IU/L, ALP -120 IU/L • Blood glucose – 86 mg/dl
Clinical course – Day 3 • Developed worsening breathlessness • Persisted after second session of hemodialysis on day 3 • Worsening tachypnea & hypoxic on O2mask • Patient intubated with mechanical ventilation • Chest X ray – bilateral infiltrates L>R • Total WBC count elevated with fever spikes • USG abdomen – • Bilateral normal sized kidneys with maintained cortico-medullary differentiation • Echocardiogram – normal, no vegetations
Clinical course – Day 5 • Hematologic profile over ICU stay • Hb dropped to 7.7 g/dl • Thrombocytopenia with lekocytosis • P. Smear – normocytic, normochromic with no evidence of hemolysis/ Schistocytes • Started on piperacilin & Tazobactum (adjusted for creatinine clearance) and Levofloxacin for nosocomial infection • Alternate day hemodialysis • Urine output gradually improved • Ventilatory requirements static (intermittent sedation)
Clinical course – Day 7 • Fever reappeared • Blood culture – Staph aureus sensitive to vancomycin • Urine pyuria – persisted • C Xray – new infiltrates, presumed to be ventilator associated pneumonia • Started on Meropenam & Linezolid • Inotropes for sepsis/SIRS/septic shock • Sensorium worsened • Taken up for slow efficiency dialysis (SLED) as he was on inotropes
Clinical course - Day 9 • Urine output – normal • But worsening azotemia • Renal biopsy planned in view of non recovery • Deferred due to sepsis with positive urine culture • Inotrope requirement increased • Could not be dialysed due to hemodynamic instability. • Developed refractory hyperkalemia & sustained cardiac arrest. • Post mortem kidney biopsy performed
Kidney biopsy • Revealed e/o fibrin thrombi occluding glomerular capillaries & hilum • Tubules revealed neutrophilic casts • Tubules, interstitium and blood vessels revealed infiltration of fungal hyphae • Branching, aseptate, broad fungal hyphae of zygomycosis • Dense infiltration of neutrophils, lymphocytes and eosinophils in interstitium
Final Diagnosis • Thrombotic microangiopathy with invasive mucormycosis and acute pyelonephritis
Review of Literature • Acute renal failure secondary to systemic mucormycosis is extremely rare • Most commonly associated with immunosuppression (primary or secondary) • Mucormycosis is rarely reported secondary to aggressive antibiotic use • Thrombotic microangiopathy • Is a medical emergency • Most patients recover completely • Approximately 3-5% die during acute phase of illness due to CVS or neurological complications • Poor prognostic factors – marked leukocytosis and older age of onset
Conclusion • Biopsy is indicated early in diagnosis of unknown cause of AKI • Systemic invasive fungal infection in non immuno-suppressed patient extremely rare • High index of clinical suspicion needed to suspect and diagnose fungal infections • Clinical diagnosis of thrombotic micro-angiopathy can be difficult • With variable hematologic and systemic findings
References • KL Gupta et al, Papillary necrosis with invasive fungal infections: a case series of 29 patients. Clin Kid J. 2013;6:390-394 • Marie Scully et al, Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic Microangiopathies. Br J Hematology 2012; 1-13 • KL Gupta et al. Renal zygomycosis: an underdiagnosed cause of acute renal failure. NDT 1999;14:2720-2725 • KL Gupta, Fungal infections and the Kidney. Indian J Nephrol 2001;11: 147-154 • KL Gupta et al, Disseminated mucormycosis presenting as acute renal failure. Postgrad Med J 1987;63:297-299 • Melnick JZ et al, Systemic mucormycosis complicating acute renal failure: case report and review of the literature. Ren Fail. 1995;17:619-27.