Clinical and Histopathological Profile of Adolescent onset Idiopathic Nephrotic Syndrome in North Indian Children.

Adolescent onset idiopathic nephrotic syndrome (INS) is marked by increased incidence atypical features and non-minimal change disease in histopathology. The objective of the study was to analyze the clinical features and histopathological spectrum of adolescent-onset INS. It was conducted in a Pediatric nephrology clinic of a tertiary care hospital in North India. We retrospectively evaluated clinical features, biochemical investigations and histopathology of 33 adolescents with idiopathic NS registered in pediatric nephrology clinic. Twenty-three (70.0%) adolescents had steroid resistant nephrotic syndrome. Hematuria was present in 39%, hypertension 36% and acute kidney injury (AKI) in 27%. Three-fourth of adolescents who underwent biopsy had non-minimal change disease in histopathology. Adolescent onset INS have increased incidence of AKI, hypertension, and non-minimal change disease.

Hepcidin and proinflammatory markers in children with chronic kidney disease: A case-control study
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BACKGROUND: Hepcidin is the main regulator of hepcidin-ferroportin axis and is elevated in children with chronic kidney disease (CKD). Anemia of CKD and its relation to hepcidin, interleukin-6 (IL-6), tumor necrosis factor-α (TNF-α), and high-sensitivity C-reactive protein (hs-CRP) in iron- and erythropoietin (EPO)-naïve, non-dialyzed children with CKD is under-studied. MATERIALS AND METHODS: This case-control study aimed to study the levels of hepcidin and other proinflammatory markers (IL-6, TNF-α, hs-CRP) and their relation with anemia in iron- and erythropoietin-naïve, non-dialysis CKD (stage 3 - 5) patients. 32 pediatric CKD stage 3 - 5 patients aged 2 - 18 years without previous iron or EPO therapy were compared with 32 gender- and age-matched healthy controls. The CKD cases were also divided into three categories based on their serum ferritin levels and transferrin saturation (%TSAT): true iron deficiency, impaired iron trafficking, and no iron deficiency. The baseline iron status was then correlated with the serum hepcidin levels. RESULTS: Serum hepcidin, IL-6, and TNF-α levels were significantly elevated compared to controls. As CKD stage progressed, hemoglobin levels decreased, while serum hepcidin, IL6, TNF-α and hs-CRP levels increased significantly. Serum hepcidin levels correlated positively with IL-6 (r = 0.57, p = 0.001), TNF-α (r = 0.34, p = 0.05), hs-CRP (r = 0.36, p = 0.03), and ferritin (r = 0.07, p = 0.001), while being inversely correlated with Total iron binding capacity (TIBC) (r = -0.50, p = 0.003), hemoglobin (r = -0.52, p = 0.001), and glomerular filtration rate (GFR) (r = -0.71, p = 0.000). Serum hepcidin levels were highest in those with impaired iron trafficking, followed by those with no iron deficiency, followed by those with absolute iron deficiency (55.16 vs. 49 vs. 11.8, p = 0.005). Amongst those with no iron deficiency, hepcidin correlated negatively with hemoglobin (r = -0.752, p-value = 0.007). CONCLUSION: A positive correlation between hepcidin and other inflammatory biomarkers in non-dialyzed, iron- and EPO-naïve pediatric CKD patients suggests a role of these markers in higher hepcidin production and its contribution to iron-restricted erythropoiesis across the spectrum of CKD. Median hepcidin levels were highest in those with impaired iron trafficking, followed by those with no iron deficiency, followed by those with absolute iron deficiency, suggesting that in an iron-replete state, high hepcidin levels inhibit iron absorption from the gut and release from iron storing cells, thus restricting erythropoiesis leading to anemia.
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Mycophenolate Sodium for Children with Frequently Relapsing or Steroid Dependent Nephrotic Syndrome.

In this retrospective study, patients with idiopathic frequently-relapsing nephrotic syndrome (FRNS) (n=27) and steroid dependent nephrotic syndrome (SDNS) (n=13) who received enteric coated mycophenolate sodium (ECMS) for at least 6 months, were included for analysis. Primary outcome was response to ECMS, which was defined as complete if there were no relapses, partial response if there was 1 relapse and no response if there were 2 or more relapses within 6 months of initiation. The mean (SD) dose of ECMS was 985.24 (190.82) mg/m2/day. Thirty patients(75%) had complete response, eight (20%) had partial and two (5%) patients did not respond at 6 months. ECMS seems to be a safe and effective as steroid sparing agent in children with FRNS/SDNS.

Inferior vena cava thrombosis in a case of amoebic liver abscess: is hypercomplementemia responsible for this rare entity?

Among the liver abscesses, thrombosis of the inferior vena cava (IVC) has been reported mainly in amoebic liver abscess (ALA) caused by Entamoeba histolytica (E.H). It is an unusual complication especially in paediatric age group. Association of hypercomplementemia and IVC thrombosis has not been discussed previously. Published data suggest that E.H can activate the complement system and can cause hypercomplementemia. A very few studies suggest that complement activation and hypercomplementemia are associated with thrombus formation. We describe a paediatric case of ALA complicated by IVC thrombosis extending to the right atrium and discuss the possible role of hypercomplementemia in causation of IVC thrombosis in cases of ALA.

Frequency and characteristics of infections caused by extended-spectrum beta-lactamase-producing organisms in neonates: a prospective cohort study.

This prospective cohort study was conducted to determine the frequency of infections caused by extended-spectrum beta-lactamase- (ESBL-) producing organisms, various bacteria producing ESBL, antibiotic susceptibility of these organisms, and the risk factors associated with these infections in a neonatal intensive care unit in a tertiary care hospital in North India. Of the 150 neonates enrolled in the study, 47 culture-positive neonates were included in the study cohort and were divided into two groups: ESBL-positive (8 neonates) and ESBL-negative (39 neonates) cohorts. Various organisms were isolated from 72 culture samples in these 47 neonates. Of these, 10 culture samples grew ESBL-positive organisms and 62 samples grew ESBL-negative organisms. The frequency of ESBL-producing organisms was found to be 5.3%. ESBL infection incidence densities were found to be 3.4 per 1000 patient-days. Klebsiella (60%) was the most common organism producing ESBL followed by Escherichia coli (30%) and Pseudomonas (10%). Eighty percent of the ESBL-producing organisms were sensitive to piperacillin-tazobactam. Risk factors found significant by univariate analysis (P < 0.05) were preterm, low birthweight, perinatal asphyxia, respiratory distress syndrome, anaemia, metabolic acidosis, prolonged mechanical ventilation (>7 days), length of hospitalization, length of level 3 stay, prior antibiotic use, central venous catheter duration, peripherally inserted central venous catheter duration, and total parenteral nutrition duration. Factors that retained significance in the logistic regression model were duration of hospital stay (adjusted OR: 0.958, CI: 0.920-0.997, and P value = 0.037) and gestational age (adjusted OR: 1.39, CI: 1.037-1.865, and P value = 0.028). There was no significant difference in the mortality between the two groups.

Congenital vivax malaria: rare or underdiagnosed infection.

Congenital malaria is a rare disease both in endemic and non-endemic areas. It is seldom suspected due to its rarity and the fact that its signs and symptoms may be similar to those with neonatal sepsis. Furthermore, clues such as a history of maternal travel to an endemic area during pregnancy or any malaria symptoms may not always be revealed. The situation is further complicated by subjective smear tests and an expensive rapid diagnostic test, especially in developing countries where affordability is an issue. We call attention to the need to consider the diagnosis of malaria in neonates who present with signs and symptoms often confused with sepsis, to enable a quick diagnosis and treatment in order to reduce mortality.

Do interventions in an ICU affect the predictive ability of pediatric index of mortality and pediatric index of mortality-2 scores in a tertiary care hospital?

OBJECTIVE: Our objective was to evaluate the effect of interventions in the initial period of stabilization (i.e., at 4 hrs) on the predictive ability of Pediatric Index of Mortality and Pediatric Index of Mortality-2 scores and to evaluate their performance in our ICU. DESIGN: Prospective observational study. SETTING: PICU of a tertiary care teaching hospital. PATIENTS: Consecutive children aged 2 months to 17 yr admitted to our ICU from June 2010 to July 2011 were included. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We prospectively recorded the baseline characteristics, variables of Pediatric Index of Mortality and Pediatric Index of Mortality-2 at 1 and 4 hrs after admission, and the outcome data in a predesigned proforma. We compared the performance of the scores at these two time points by calculating their discriminative ability and calibration as measured by the area under curve of the receiver operating characteristic curves and the Hosmer-Lemeshow goodness-of-fit test, respectively.Of the 282 children enrolled, 93 (32.9%) died. The median (interquartile) age of the study patients was 3.5 yr (0.8, 10). The major reasons for ICU admission as well as mortality were sepsis/severe sepsis and cardiac and neurological illnesses. The area under curves for Pediatric Index of Mortality at 4 and 1 hrs were 0.73 (95% confidence interval 0.66-0.79) and 0.70 (0.63-0.77), respectively. The corresponding values for Pediatric Index of Mortality-2 were 0.72 (0.66-0.79) and 0.71 (0.64-0.78), respectively. The goodness-of-fit test showed a good calibration across deciles of risk for the two scores at both the time points (p > 0.1 for all). The calibration across different age and diagnostic subgroups was also good. CONCLUSION: Interventions in the first 4 hrs did not affect the predictive ability of Pediatric Index of Mortality and Pediatric Index of Mortality-2 scores. The 4-hr scores may be used in place of the 1-hr score, particularly in units where scoring is not possible with in the 1-hr time frame.

Membranoproliferative glomerulonephritis associated with autoimmune thyroiditis.

We present a rare case of membranoproliferative glomerulonephritis (MPGN) associated with autoimmune hypothyroidism in a child. The exact pathogenesis of glomerulonephritis remains unclear. Thyroxine replacement therapy along with steroids may lead to significant decrease in proteinuria and resolution of edema. Thyroid status should be evaluated in all cases with MPGN.

Infantile trauma due to a rat bite.

We present two cases of extreme neglect with injuries. These are perfect examples of gender bias. Our first case is a 20-day-old female neonate was brought to the pediatric emergency department with multiple rat bites to the face. A 9-month-old female infant was brought to the emergency care division with multiple rat bites on the eyes and upper extremities. These cases point towards the existing gender bias and extreme social neglect of females in the Indian society.

Multiminicore disease with respiratory failure.

Multiminicore disease is a rare form of slowly progressive or nonprogressive myopathy, characterized by multiple cores within the muscle fibers. Respiratory failure in multiminicore disease rarely occurs. We describe a 5-year-old girl with multiminicore disease and early-onset respiratory failure after an episode of bronchopneumonia. The child received mechanical ventilation for 280 days and was discharged on home ventilation. The relevant literature is reviewed.

Lung resections in children for congenital and acquired lesions.

We reviewed a single-center experience of pediatric lung resections for various congenital and acquired benign lung conditions. Thirty-five children underwent lung resections between 1998 and 2006, their age ranging from 8 days to 12 years (mean 3 years), with a male:female ratio of 4:1. Twelve patients were neonates. Antenatal diagnosis was available in only one patient. The presenting symptoms were respiratory distress and respiratory tract infections. Imaging with chest X-ray with/without a CT scan picked up the lesion in all cases. Preoperative ventilation was required for five patients. One patient had pneumothorax at presentation; however, ten patients had inadvertent intercostal tube insertion before surgical referral. The surgical procedures performed included lobectomy (28), segmentectomy (3), and pneumonectomy in 4 cases. Twenty-one patients underwent emergency surgery. Six patients required postoperative ventilation. The histopathological diagnosis was congenital lobar emphysema (CLE) (9), congenital cystic adenomatoid malformation (CCAM) (9), bronchiectasis (9), sequestration (3), atelectasis (1), lung abscess (1), unilobar tuberculosis (1), hydatid cyst (1), and foreign body with collapse (1). There was considerable discrepancy between the preoperative diagnosis based on imaging and the postoperative histopathological diagnosis. Postoperative complications included atelectasis (2), pneumothorax (2) and fluid collection (4 cases). Three patients died, one from compromised cardiac function, one from overwhelming sepsis and one from respiratory failure due to severe bilateral CCAM; the rest of the patients made a satisfactory recovery. At short-term follow-up all patients were doing well. Pulmonary resections are necessary for various congenital and acquired lung lesions in children and can be done safely in a pediatric hospital setup. Proper preoperative diagnosis can avoid inadvertent intercostal tube insertion in patients with congenital cystic lung lesions. The histopathological diagnosis often differs from the radiological diagnosis. Emergency lobectomies for acute respiratory distress, even in neonates, result in a satisfactory outcome.