Rhabdomyolysis with myoglobin-induced acute kidney injury: A case series of four cases.

Rhabdomyolysis is a potentially life-threatening clinical syndrome characterized by the breakdown of skeletal muscle cells and release of creatine kinase (CK), lactate dehydrogenase (LDH), and myoglobin into the plasma and interstitial space. Rhabdomyolysis can occur due to a variety of causes and acute kidney injury (AKI) is one of its most dreaded complications occurring in 33%-50% patients. The main pathophysiology of renal injury is due to vasoconstriction, intraluminal casts, tubular obstruction, and direct myoglobin toxicity. As the symptoms are nonspecific, a high level of suspicion is required in the mind of the treating physician. Early diagnosis and prompt management with fluid resuscitation, initiation of renal replacement therapy (RRT), and elimination of causative agents can help prevent complications. We hereby report four interesting cases of this clinical syndrome with emphasis on the causative agents.

Systemic Lupus Erythematosus and Myasthenia Gravis: A Rare Association.

Systemic lupus erythematosus (SLE) and myasthenia gravis (MG) are two autoimmune diseases that have a higher incidence in young females, relapsing-remitting course, and positive antinuclear antibodies. SLE and MG are two different clinical syndromes, which can coexist or precede each other; however, their occurrence in the same patient is rare. We report a 38-year-old female with biopsy-proven lupus nephritis on steroids and cyclophosphamide, later developed MG. Nerve conduction studies showed the decremental response of 15%-25% over facial muscles with no decremental response over limb muscles. Although antianticholinesterase receptor (AchR) antibodies were negative, she was treated with oral pyridostigmine 60 mg twice daily and clinical improvement of ocular symptoms was seen within 48 h. At present, she is on oral prednisolone and mycophenolate mofetil with follow-up creatinine of 1.4 mg/dl and no neurological symptoms.

Spectrum of Hypokalemic Paralysis from a Tertiary Care Center in India.

Hypokalemic paralysis is an important and reversible cause of acute flaccid paralysis. The treating physician faces unique diagnostic and therapeutic challenges. We did a prospective study and included all patients with acute flaccid weakness and documented serum potassium of <3.5 mEq/L during the period between January 2009 and August 2015. We studied the incidence, etiology, clinical profile, and acid-base disturbances in patients presenting with hypokalemic paralysis and analyzed the significance of periodic and non-periodic forms of hypokalemic paralysis on patient's outcome. Two hundred and six patients were studied with a mean follow-up of 3.6 ± 1.2 years. Mean age was 37.61 ± 2.2 years (range 18-50 years). Males were predominant (M:F ratio 2.1:1). The nonperiodic form of hypokalemic paralysis was the most common (61%). Eighty-one (39%) patients had metabolic acidosis, 78 (38%) had normal acid-base status, and 47 (23%) patients had metabolic alkalosis. The most common secondary cause was distal renal tubular acidosis (RTA) (n = 75, 36%), followed by Gitelman syndrome (n = 39, 18%), thyrotoxic paralysis (n = 8, 4%), hyperaldosteronism (n = 7, 3%), and proximal RTA (n = 6, 4%). Patients with non-periodic paralysis had more urinary loss (40.1 vs. 12.2 mmol, P = 0.04), more requirement of potassium replacement (120 vs. 48 mmol, P = 0.05), and longer recovery time of weakness (48.1 vs. 16.5 h, P = 0.05) than patients with periodic paralysis. Non-periodic form of hypokalemic paralysis was the most common variant in our study. Patients with periodic paralysis had significant incidence of rebound hyperkalemia.

Crescentic infection related glomerulonephritis in adult and its outcome.

The epidemiology of infection-related glomerulonephritis (IRGN) is changing in recent times both in developed and developing nations. Although published studies showed renal outcome in adult IRGN was not as benign as in children, literature regarding clinical profile and outcome of crescentic form of adult IRGN is scarce; hence, we aimed to study the clinical profile of crescentic IRGN. We conducted a retrospective observational study in patients with crescentic IRGN in adults at the Department of Nephrology, Madras medical college, Chennai between 2009 and 2014. A total of 47 patients were included with a mean follow-up of 9.9 ± 4.2 months. The mean age was 42 ± 13.5 years. About 19.1% of patients had diabetes. The skin was the most common site of infection (38.3%) with methicillin-resistant Staphylococcus acareas (MRSA) as the most common organism. Hypocomplementemia was present in 100% in our study. Hemodialysis (HD) was required in 53.2% of patients and oral steroids were given in 78.7%. Complete renal recovery was seen only in 25.5%, progression to chronic kidney disease in 40.4%, seven patients reached end-stage renal disease, and nine patients died during follow-up. On univariate analysis, MRSA infection, the unidentified source of infection, nonisolation of organisms presence of interstitial fibrosis and tubular atrophy in renal biopsy and requirement of HD were found to be significant risk factors for poor renal outcome. In our study, crescentic form of IRGN is associated with poor renal outcome.

Collapsing Glomerulopathy and Thrombotic Microangiopathy in Postpartum Period: Two Case Reports.

Collapsing glomerulopathy (CG) is a distinct histopathologic pattern of glomerular injury characterized by global/segmental wrinkling of the glomerular basement membrane with podocyte hyperplasia and hypertrophy along with tubulointerstitial changes. There is no specific treatment for CG due to etiological heterogeneity, and newer insights into the pathogenesis may lead to the development of targeted therapy. The most common form of CG is the primary or idiopathic followed by secondary (due to viral infections, autoimmune disease, drugs, etc.) and genetic causes. Thrombotic microangiopathy (TMA) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ failure of variable severity. We here present two young women with preeclampsia who presented with acute kidney injury, anemia, and schistocytes in peripheral smear suggestive of TMA. Renal biopsy showed interesting histopathology of CG in addition to TMA in the first patient and CG alone in the second. Both the patients received supportive therapy while the first patient also received plasmapheresis. One patient had complete recovery, and other had partial recovery of renal function at last follow-up. Combined histopathological lesion of CG with TMA has never been reported in postpartum period so far in literature.

Unusual cause of crystalline nephropathy.

Adenine phosphoribosyltransferase deficiency is a rare, inherited autosomal recessive disease presenting with 2,8-dihydroxyadenine (DHA) urolithiasis, DHA nephropathy, and chronic kidney disease. The presence of DHA crystals in urine and renal biopsy is pathognomonic of the disease. We report a 23-year-old female with acute renal failure and nephrotic proteinuria. Urinalysis showed reddish brown, round crystals with dark outline, and central spicules consistent with 2,8-DHA crystals. Renal biopsy showed membranous nephropathy and 2,8-DHA nephropathy. Our patient improved with liberal fluid intake, restriction of high adenine content foods, and oral xanthine dehydrogenase inhibitor febuxostat. Early diagnosis and initiation of treatment prevent renal complications.

Paraneoplastic glomerulopathies associated with hematologic malignancies.

Paraneoplastic glomerulopathy (PG) includes a group of glomerular lesions that are not directly related to tumor burden, invasion, or metastasis, but induced by products from tumor cells such as hormones, growth factors, cytokines, and tumor antigens. PG was first described by Galloway in 1922, in which nephrotic syndrome (NS) secondary to Hodgkin's lymphoma was reported. They can present before, at the time of diagnosis, during remission, or at relapse of the hematological malignancies. We present three cases of paraneoplastic NS associated with hematological malignancies. Early diagnosis and treatment can alter prognosis of these patients. Paraneoplastic syndromes would remit only with proper treatment of the neoplasia.

Proliferative Glomerulonephritis with Monoclonal IgG Deposits.

Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) is a recently described disorder that belongs to the class of monoclonal gammopathy of renal significance with the incidence of 0.17%. Monoclonal IgG deposits in glomeruli can produce a proliferative glomerulonephritis that mimics immune-complex mediated glomerulonephritis by light microscopy (LM) and electron microscopy (EM). Proper recognition of this disease requires confirmation of monoclonality by immunofluorescence (IF) staining. We present here a 50 year old female patient presented with nephrotic range proteinuria and normal renal function. Renal biopsy showed endocapillary and mesangial proliferation by LM. IF showed Ig G (+2) and C3 (+2) with positivity for Lambda light chain and Kappa light chain is negative consistent with PGNMID.

Infection-related glomerulonephritis in a renal allograft.

Infection-related glomerulonephritis (IRGN) is an immune-mediated glomerulo-nephritis, most commonly caused by bacterial infections. Although there is an increased incidence of infectious episodes in renal transplant recipients, IRGN as a cause of de novo glomerulonephritis is rarely seen probably due to impaired immunity. We hereby report a 28-year-old male renal transplant recipient, who developed IRGN following impetigenous skin lesions after six years of transplant. He developed rapid worsening of allograft function and was started on hemodialysis. Allograft renal biopsy showed diffuse exudative endocapillary proliferation with crescents. Electron microscopy revealed large subepithelial hump-like deposits. Despite pulse steroid therapy, he became dialysis dependent. Our patient is unique in the way that poststreptococcal glomerulonephritis in an adult after renal transplantation has not been reported so far. We conclude that IRGN after renal transplant, though rare is a possible etiology for allograft dysfunction. There is no definitive treatment protocol for this de novo glomerulonephritis which has an overall poor prognosis.

IgA-dominant Infection-related Glomerulonephritis in India: A Single-center Experience.

IgA-dominant infection-related glomerulonephritis (IRGN) is a distinct morphologic variant of IRGN, characterized by dominant or codominant glomerular deposits of IgA, mostly in elderly and patients with diabetes. More cases are being reported in recent times due to increased awareness of the disease entity and increased rate of Staphylococcus infection. It usually presents as rapidly progressive renal failure with proteinuria, and treatment guidelines for this disease entity are not well defined. We report here 12 cases of IgA-dominant IRGN seen over a period of 5 years from a single center. Clinical features, biopsy findings, treatment, and outcomes were analyzed. Out of 12 patients, eight were males. The mean age of presentation was 52.4 ± 21 years. Skin was the most common site of infection seen in six patients. Gross hematuria was seen in 4 patients and 11 had nephrotic proteinuria. Eleven had low serum C3. Only two patients had diabetes. Methicillin-resistant Staphylococcus aureus (MRSA) was the most common organism isolated in six patients. Most common histopathology was crescentic glomerulonephritis seen in seven patients, followed by endocapillary proliferation in three and diffuse proliferative glomerulonephritis in two. Hemodialysis was done in eight patients and six patients received steroid therapy. End-stage renal disease developed in three patients, chronic kidney disease in three, and three patients died due to sepsis. Various infections including MRSA and Escherichia coli were associated with IgA-dominant IRGN both in patients with diabetes and nondiabetics. Suspicion and recognition of the disease is important as it has therapeutic and prognostic implications.

Scrub typhus meningitis in a renal transplant recipient.

Scrub typhus is a rickettsial infection commonly seen in Asia. The clinical presentation ranges from nonspecific febrile illness to potentially fatal multiorgan involvement such as liver, kidney, or lung. Central nervous system involvement is uncommon. We report a 45-year-old female renal transplant recipient who presented with fever, headache, meningeal signs, graft dysfunction, and eschar. IgM antibodies against Orientia tsutsugamushi were positive by enzyme-linked immunosorbent assay. Despite oral doxycycline therapy for 5 days, she did not improve but responded well to intravenous azithromycin. To the best of our knowledge, scrub typhus as a cause of meningitis in a renal transplant recipient has not been reported so far.

Thrombotic microangiopathy due to Viperidae bite: Two case reports.

Snake bite is mainly an occupational hazard and causes serious health problems in rural India. Acute kidney injury (AKI) occurs in 5-30% cases. Renal pathologic findings include acute tubular necrosis, cortical necrosis, interstitial nephritis, glomerulonephritis, and vasculitis. Thrombotic microangiopathy (TMA) occurrence after a snake bite is reported rarely. Here, we present two patients who developed TMA after viper bite treated with hemodialysis and plasmapheresis. Renal biopsy showed fibrin thrombi in glomeruli and arterioles with cortical necrosis. One patient progressed to end-stage renal disease and other was lost to follow-up. TMA should be considered as a possible pathogenesis of AKI after snake bite. The role of plasma exchanges in snake bite TMA is yet to be defined.

Thrombotic microangiopathy: An unusual cause of renal failure in rheumatoid arthritis.

Rheumatoid arthritis (RA) is one of the commonest rheumatological diseases. Renal involvement is not common but can occur as a result of chronic inflammation as part of disease process or drug toxicity. Thrombotic microangiopathy (TMA) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ failure of variable severity. Only a few cases of TMA in patients with RA were reported to date. We describe a 45-year-old female patient with RA who presented with oliguria and edema. Renal biopsy showed TMA with patchy cortical necrosis. She improved with hemodialysis and plasmapheresis.

Deceased donor renal transplantation: A single center experience.

Deceased donor renal transplantation (DDRT) constitutes less than 5% of all kidney transplantats in India. A retrospective analysis of 173 deceased donor renal transplants performed in a public funded government hospital was done. Mean age of the recipients was 36 years (male:female ratio 2.4:1), and that of the donors was 32.3 years (male:female ratio 6:1). The cold ischemic time was 340 ± 170 minutes. Mean follow-up period was 36 months. Forty one patients died, 75% of them in the first post - transplant year. Sepsis and cardiovascular disease were the most common causes of death. Twenty two percent had acute rejection. There was no significant difference in the incidence in the rate of acute rejection, bacterial, fungal infections and death rate between the cohorts of induction and non induction immunosuppression. The patient and death censored graft survival at 1 year were 80 and 82.6% and at 5 years were 76 and 80% respectively.

Evaluation of renal lesions and clinicopathologic correlation in rheumatoid arthritis.

The most common causes of renal disease in rheumatoid arthritis (RA) are glomerulonephritis (GN), amyloidosis, tubulo-interstitial nephritis, and drug toxicity. Our aim was to evaluate the clinicopathologic correlation of renal lesions and to assess the course and prognosis of renal disease in patients with RA. We conducted a prospective observational study in all adult patients with RA between July 2010 and June 2015. The total number of patients studied was 90, with a female:male ratio of 2.3:1. Mean follow-up duration was 30 ± 6.5 months. About 54 patients (60%) were asymptomatic. The most common symptom was edema legs (30%), followed by oliguria (10%). About 18 patients (20%) presented with the nephrotic syndrome, 15 patients (16.6%) with nephritic syndrome, and 30 (33%) with asymptomatic urinary abnormalities. Chronic kidney disease (CKD) was seen in 48 of 90 patients (53%).The most common renal pathology noted was mesangioproliferative GN followed by membranous nephropathy (MN). IgM with C3 deposits was the most common immunofluorescence pattern observed. Among the patients who had glomerular diseases, complete remission was seen in nine patients, partial remission in 15, and persistent proteinuria in 14. Duration of RA and a high erythrocyte sedimentation rate correlated significantly with persistent proteinuria. Only one patient in the glomerular disease group progressed to dialysis-dependent renal failure. On followup, 11 out of 48 CKD patients showed a significant decrease in estimated glomerular filtration rate and worsened to the next stage of CKD. Renal disease in RA presents with varied renal pathology. MN was seen frequently and was not associated with gold or penicillamine usage. Relatively high incidence of CKD was noted. Hence, it is important to monitor renal function abnormalities periodically in these patients.

Clinicopathological correlation and treatment response of primary focal segmental glomerulosclerosis in adults and adolescents.

The incidence of focal segmental glomerulosclerosis (FSGS) is approximately 10% in children <6 years, 20% in adolescents, and 20-25% in adults. A retrospective observational study was done to document clinicopathological correlation, treatment response, and risk factors in the progression of chronic kidney disease (CKD) of primary FSGS in adults and adolescents. A total of 170 patients were studied with a mean follow-up of 4.32 ± 1.2 years. FSGS not otherwise specified was the most common subtype (56%) followed by tip variant (24%). About 32% had complete remission (CR) at a mean time of 6.4 months, 23% had partial remission (PR) at a mean time of 5.7 months, and 45% had no response to steroids. Persistent nephrotic proteinuria at 3rd and 6th month and presence of interstitial fibrosis and tubular atrophy >30% in renal biopsy are the independent predictors of poor response to treatment. Presence of anemia, interstitial fibrosis, and tubular atrophy of >30% in renal biopsy and the absence of remission after treatment were the independent predictors of CKD progression. Overall renal survival was 78% at 3 years and 54% at 5 years. Renal survival difference with or without nephrotic proteinuria at onset was 39% and 69% at 5 years. Renal survival was higher in patients with normal renal function (66%) compared with those who had renal failure (42%) at 5 years. Renal survival at 5 years for CR was 69%, PR was 49%, and no remission was 42%.

Two consecutive recurrences of crescentic immunoglobulin A nephropathy in a renal transplant recipient.

We report a 21-year-old male who developed end-stage renal disease, probably due to immunoglobulin A nephropathy (IgAN), received a renal transplant from his mother, which was lost due to crescentic IgAN after 18 months. Two years later, he received a second transplant from a deceased donor. He developed rapidly progressive graft dysfunction 3 years later. Allograft biopsy revealed crescentic IgAN, which was successfully treated with intravenous steroids and cyclophosphamide. Recurrence of IgAN in two successive allografts in one patient has not been reported previously.

Familial Lecithin Cholesterol Acyl Transferase Deficiency with Chronic Kidney Disease.

Familial lecithin-cholesterol acyltransferase (LCAT) deficiency is a rare autosomal recessive (AR) disease caused by mutation in the LCAT gene. LCAT enzyme esterifies cholesterol molecules in high-density lipoprotein(HDL) and low density-lipoprotein (LDL) particles. This enzyme deficiency is characterised by progressive corneal opacification, glomerulopathy, mild - moderate haemolytic anaemia and very low plasma levels of HDL. We here report a 34 year-old lady who presented with hypertension, nephrotic proteinuria, renal failure, corneal ring opacities, anemia and dyslipidemia. The diagnosis of familial LCAT deficiency was confirmed by clinical examination, characteristic dyslipidemia, undetectable LCAT levels in plasma and positive family history.