Portal Vein Thrombosis: A Rare Complication of Nephrotic Syndrome.

Deep vein thrombosis, renal vein thrombosis, and cerebral venous sinus thrombosis in children are frequently described complications of nephrotic syndrome (NS). Early diagnosis and treatment with anticoagulants is the key for a good outcome. There are a few reported cases of portal vein and superior mesenteric thrombosis in adults in association with NS. Here, we describe two cases of portal vein thrombosis with variable extent of involvement of superior mesenteric vein in association with relapse of NS. A high degree of suspicion, ultrasonography of the abdomen along with Doppler study of abdominal vessels, and computed tomography angiography can only pick up such unusual sites of thrombosis and facilitate early management.

De Novo Collapsing Glomerulopathy in Renal Allograft in Association with BK Virus Nephropathy in a Child and Stabilization of Renal Function by Elimination of Viremia.

Well-recognized association between HIV 1 infection and collapsing glomerulopathy (CG) raises the possibility that intrarenal infection by other viruses may also contribute to the development of this lesion in native or post-transplant kidneys. There is evidence in literature about association of these lesions with cytomegalovirus, Epstein-Barr virus, hepatitis C virus, and parvovirus B19 infections. Here, we present a case report of post-transplant BK virus nephropathy in a male child who was found to have CG in subsequent biopsy 2 months later. His renal function and proteinuria were stabilized on elimination of viremia.

Posterior reversible encephalopathy syndrome in children with kidney disease.

Posterior reversible encephalopathy syndrome (PRES) is a clinic-radiographic entity of heterogeneous etiologies that are grouped together because of similar findings on neuro-imaging and associated symptom complex of headache, vision loss, altered mentation, and seizures. Although usually considered benign and reversible, characteristics of this syndrome in pediatric patients remain obscure. This case series included 11 patients (8 males, 3 females, age 3-15 years) of PRES during September 2010 to February 2012 out of a total 660 renal pediatric patients (1.66%). We studied their clinical profile, contributory factors, and outcome. Presenting symptoms were headache in 73%, dimness of vision or cortical blindness in 36%, seizures in 91%, and altered mentation in 55%. The associated renal diseases were acute renal failure (55%), chronic renal failure (9%), and 36% had normal renal function. The contributory factors were uncontrolled hypertension (100%), severe hypoproteinemia (9%), persistent hypocalcemia (9%), hemolytic uremic syndrome (36%), cyclosporine toxicity (9%), lupus nephritis (9%), high hematocrit (9%), and pulse methylprednisolone (9%). Brain imaging showed involvement of occipito-parietal area (100%) and other brain areas (63%). All but one patient of hemolytic uremic syndrome had complete clinical neurological recovery in a week, and all had normal neurological imaging after 4-5 weeks. PRES is an underdiagnosed entity in pediatric renal disease patients. Associated hypertension, renal disease, and immunosuppressive treatment are important triggers. Early diagnosis and treatment of comorbid conditions is of prime importance for early reversal of syndrome.

Successful renal transplantation from a brain-dead deceased donor with head injury, disseminated intravascular coagulation and deranged renal functions.

Deceased donors (DDs) with the brain death due to head injury are the major source of organs for transplantation. The incidence of post-head injury disseminated intravascular coagulation (DIC) ranges from 24% to 50%. Many centers do not accept organs from donors with DIC due to increased risk of primary graft non-function and/or high chances of morbidity/mortality. We performed two successful renal transplants from a DD with head injury with DIC and deranged renal function. One of the recipients developed transient thrombocytopenia, but there was no evidence of DIC or delayed graft functions in either of the recipients. Over a follow-up of 1 month, both are doing well with stable graft function and hematological profile. Thus, a carefully selected DD with severe DIC even with deranged renal function is not a contraindication for organ donation if other risk factors for primary non-function are excluded. This approach will also help in overcoming organ shortage.

Chronic type B aortic dissection in association with Hemolyticuremic syndrome in a child.

Aortic dissection (AD) is a potentially life-threatening medical emergency usually encountered in the elderly. Here, we report a 9-year-old child who was incidentally detected to have asymptomatic chronic type B dissecting aneurysm of aorta when he presented with relapse of Hemolytic uremic syndrome (HUS) without any genetic abnormalities like Marfan or Ehler-Danlos syndrome. To the best of our knowledge, this is the first case of AD associated with HUS in a child without any known associated genetic or inherited risk factors.

Cerebral venous sinus thrombosis in children with nephrotic syndrome.

Nephrotic syndrome in infancy and childhood can be associated with thromboembolic complications. We describe two cases of nephrotic syndrome who presented with non-specific findings. Magnetic resonance imaging brain with magnetic resonance venography showed thrombosis of multiple venous sinuses. Patients were successfully treated with anticoagulants and there was complete resolution of thrombosis on repeat imaging.

Acute renal failure in the pediatric age group - single center prospective study of 180 cases.

Acute renal failure (ARF) is one of the common emergencies in pediatric practice. In the Indian subcontinent, its etiology, clinical features and outcome vary from other parts of the world. We decided to perform a prospective study of ARF in 180 pediatric patients admitted to our institute between August 2006 and March 2008. Our study included children, neonates 7.8%, <1 year 16.7%, 1-5 years 30.5% and >5 years comprised 52.8%. The male:female ratio was 2.3:1. Acute tubular necrosis remains the major cause of ARF; other intrinsic renal disease accounted for almost 30% of the patients. In all patients of ARF who required dialysis, peritoneal dialysis was offered as the first-line management. Six patients were offered hemodialysis. Mortality below one year age was higher compared with those who were more than one year of age (40% vs 11.3%). The overall mortality in the present study was 17.7%. ARF in pediatric nephrology is not uncommon. In our setup, peritoneal dialysis (PD) is an effective and safe modality of renal replacement therapy in most of the cases. Delayed referral, malnutrition, infections, age less than one year and multiorgan involvement were bad prognostic features.

Mucormycosis in renal transplant recipients: predictors and outcome.

Mucormycosis, though uncommon, is associated with high mortality in transplant recipients. This study was conducted to assess the incidence and risk factors associated with mucor infection and its outcome. We retrospectively reviewed the hospital records for evidence of mucor infection in patients transplanted between January 2005 and December 2009 at the Department of Nephrology and Clinical Transplantation (IKDRC), Civil Hospital Campus, Asarwa, Ahmedabad, Gujarat, India. The patient demographics, symptoms, diagnostic techniques and outcomes were analyzed. Out of a total of 1,330 transplants, 16 patients (1.20%) had evidence of mucor infection, including 14 males and two females. The mean age of the patients was 43.8 years. The time interval between transplantation and disease onset varied greatly (range: 1 month to 7 years; median 13.8 months). The presenting symptoms were fever (87.5%), severe headache (56.2%), facial swelling (56.2%), watering of eyes (56.2%), cough (31.2%), respiratory distress (18.7%) and pain abdomen (12.5%). Suspected patients were evaluated by computerized tomographic (CT) scan/magnetic resonance imaging (MRI), bronchoalveolar lavage (BAL) and biopsy, and the diagnosis was confirmed by culture. Of the 16 patients studied, nine had rhinocerebral mucormycosis, five had pulmonary mucormycosis and one case each had infection at the graft anastmosis site and disseminated mucormycosis. Early and intensive treatment with liposomal amphotericin-B was instituted in all patients, and extensive debridement was performed in addition in 11 cases, and one patient was subjected to graft nephrectomy; 10 patients (62.5%) survived. Our study suggests that rhinocerebral is the most frequent site of mucormycosis and it can occur very early or late in the post-transplant period. Early diagnosis and combined surgical debridement and parenteral liposomal amphotericin-B along with reduction of immunosuppression improve the patient survival.

Pregnancy-related acute renal failure: A single-center experience.

Pregnancy-related acute renal failure (ARF) is a common occurrence and is associated with substantial maternal and fetal mortality. It also bears a high risk of bilateral renal cortical necrosis. We conducted this study to evaluate the contributing factors and to assess the frequency of cortical necrosis. In this prospective study, of the 772 patients with ARF admitted at our institute between January 2004 and May 2006, 70 had ARF associated with pregnancy complications. ARF was diagnosed by documenting oliguria (urine output <400 ml/d) or mounting azotemia in the presence of normal urine output. (serum creatinine >2 mg%). Renal biopsy was performed if a patient was found to be oliguric or required dialysis support at the end of three weeks. The incidence of pregnancy-related ARF was 9.06%. Approximately 20% cases occurred due to postabortal complications in early pregnancy and 80% following complications in late pregnancy. Puerperal sepsis was the most common etiological factor in 61.42% of the patients. Preeclampsia accounted for 28.57% of ARF. Two-thirds of patients recovered with dialysis and supportive care. The incidence of biopsy proven renal cortical necrosis was 14.8% (10 of the 70 patients). The incidence of renal cortical necrosis was 28.57% in the early pregnancy group and 10.71% in the late pregnancy group. Postabortal sepsis was the most common precipitating event for renal cortical necrosis. Maternal mortality was 18.57%. Sepsis accounted for a majority of deaths (61.53%). Pregnancy-related ARF is common in western India. Puerperal sepsis is the most frequent etiological factor. Renal cortical necrosis is common and postabortal sepsis was the most common precipitating event. Sepsis accounted for a majority of maternal mortality.