Syndrome In Indian Children Research Articles

Predictive value of WHO vs. IAP BMI charts for identification of metabolic risk in Indian children and adolescents.

Owing to increase in prevalence of obesity and metabolic syndrome in Indian children and adolescents, this study is conducted to assess the predictive value of IAP 2015 and WHO 2007 BMI for age cut-offs in identifying metabolic risk in Indian children. Cross-sectional multicentric school-based study on 9-18-year-old healthy children (n=1,418) randomly selected from three states of India. WHO 2007 and IAP 2015 charts classified 222 (15.7%) and 271 (19.1%) as overweight/obese, respectively. A total of 192 (13.5%) subjects had metabolic risk. Of these 47 (25%) and 36 (18.75%) were classified as having normal body mass index (BMI) by WHO and IAP, respectively. In identifying metabolic risk, IAP 2015 and WHO 2007 charts showed a sensitivity of 81.3 and 75%, negative predictive value 96.5% as against 94.8%, positive predictive value 57.5 and 64.8%, and specificity of 89.7 and 91.6%, respectively. Owing to obesity epidemic and high metabolic risk in Indians, IAP 2015 charts (as against the WHO 2007 references) which had a higher sensitivity in identifying metabolic risk may be more suitable in Indian children and adolescents.

Changing trend in sporadic acute encephalitis syndrome in Indian children – A retrospective cohort study

Background: Acute encephalitis syndrome (AES) is a common cause of admission to the pediatric intensive care unit (PICU). In the past decade, noninfective etiologies are emerging as important contributor of AES.Materials and Methods: A retrospective chart review (clinical, laboratory, and neuroradiological parameters; etiology; and outcome) of children with AES in the age group of 1 month–18 years admitted to a tertiary referral hospital PICU over 6 years was done. For inclusion, AES was defined as an acute onset of fever and encephalopathy with/without seizures or other neurologic signs. Children with febrile seizures and inborn errors of metabolism were excluded from the study.Results: A total of 121 cases of AES were included in the study with a mean age of 5.74 ± 4.76 years and male-to-female ratio of 2:1. Infection (67.8%) was found to be the most common etiology, followed by autoimmune (21.5%) and undetermined/inflammatory (10.7%). Probable viral encephalitis (37.18%), pyogenic meningitis (18.18%), and tubercular meningitis (TBM) (6.66%) were common among infective etiology. Autoimmune encephalitis was diagnosed in 14 (11.57%), acute disseminated encephalomyelitis (ADEM) in 10 (8.26%), and acute necrotizing encephalopathy (ANE) in 10 (8.26%) cases. Abnormal behavior, reduced speech, sleep disturbances, and seizures were the common presenting features of autoimmune encephalitis. Neuroimaging was available in 113 (93.38%) cases, and 42 (34.71%) were reported as abnormal. In all cases of ANE and 80% of ADEM, neuroimaging was diagnostic. Initial magnetic resonance imaging of the brain was normal in all autoimmune cases except one with limbic encephalitis. Among all cases, 62.81% of the cases recovered completely, 33.06% had sequelae, and 4.13% died.Conclusion: Although central nervous system infections still remain the most common cause of AES, noninfective etiologies such as autoimmune encephalitis, ADEM, and ANE should also be considered early in the differential diagnoses of AES to tailor appropriate investigations and institute early immunotherapy to reduce morbidity.

Characterization of NPHS2 gene polymorphisms associated to steroid resistance nephrotic syndrome in Indian children

Nephrotic syndrome (NS) is the common glomerular disease in children. These children are treated with steroids, depending upon their behavior. They are either steroid sensitive (SSNS) or steroid resistant (SRNS). NPHS2 gene mutants are linked to the risk of autosomal recessive SRNS and in some cases to SSNS. The present study has been performed to screen single nucleotide polymorphisms (SNPs) of the NPHS2 gene in a group of 90 Indian children suffering with NS (30 SSNS, 30 SRNS and 30 Controls) by PCR method followed by direct exon sequencing. Effect of SNPs on fold expression changes at transcript level of podocin was checked using quantitative real time PCR (qRT-PCR). SNPs identified through sequencing helps to carry out in-silico analysis. Overall 17 SNPs were identified in NPHS2 gene where 6 were found novel. Three missense SNPs p.R299Q, p.P20L and p.G35D were also identified in this population where SNP, p.G35D was found novel. In addition to sequencing analysis, results of in silico analysis shows that a mutant with these three missense SNPs has least ligand binding efficiency compared to native model. Moreover the significant observation of this study included two intronic SNPs c.451+23C>T and c.451+58A>T present in SRNS group of patients. These SNPs has shown high level of clinical significance within genomic and allelic frequency along with haplotypes and linkage disequilibrium count. The qRT-PCR analysis shows, down expression of podocin protein at transcript level in SRNS patients compared to SSNS patients. All these results support the fact that SNPs present in this population could affect the protein structural stability. Thus it is concluded that the polymorphisms predicted in this study might be disease causing in the NPHS2 gene and may have influence on the therapeutic response of NS patients.

Clinicopathological study of nephrotic syndrome in Indian children :A tertiary care experience

Background: Nephrotic syndrome is an important chronic disorder in children with clinical manifestation of different histopathological subtypes. An aim of the study was to determine incidence of renal biopsy in paediatric nephrotic syndrome, correlations between the clinical and histomorphological patterns in Indian ethnicity children at our tertiary care institute. Methods: : A retrospective study of kidney biopsies with, immunofluorescence and electron microscopy with clinical outcome of the children with nephrotic syndrome was done over a period of 15 years. Biopsies were stained with Hematoxylin & eosin, Periodic acid Schiff and silver impregnation stain and also processed for immunofluorescence and electron microscopy as necessary. All renal biopsy findings were correlated with clinical response to steroid therapy, immunosuppressant, and clinical parameters. Result: Frequencies in results were calculated by Chi square test for categorical variables. Mann-Whitney U test was used for non parametric variables. Predominantly cases were seen in the age group 8-12 years. Atypical nephrotic syndrome was the commonest presenting feature followed by steroid resistant nephrotic syndrome. Minimal change disease was the commonest histomorphological pattern followed by focal segmental glomerular sclerosis. All the cases of membranous glomerulonephritis in study were secondary in nature. Light microscopy findings correlated with electron microscopy, in 80.95% cases. Conclusion: Minimal change disease occurs up to 6 times more commonly in Indian children than in Europian counterpart, but rise in FSGS cases as a cause for nephrotic syndrome is now being encountered. In India the proportion of cases with Membrano-proliferative glomerulonephritis is high, attributable to the high prevalence of infectious diseases like tuberculosis, chronic suppurative infections and malnutrition. DOI: 10.21276/apalm.2017.995

Clinical, endoscopic, and pathologic characteristics of colorectal polyps in Indian children and adolescents.

Colorectal polyps are among the common causes for rectal bleeding in children. We studied the clinical, colonoscopic, and histopathological features of colorectal polyps and polyposis syndrome in Indian children and adolescents. Medical records of children and adolescents with colorectal polyps and polyposis syndrome were retrospectively reviewed from 2001 to 2014 at Department of Gastroenterology, in large tertiary care center of Mumbai. A total of 120 patients were found to have colonic polyps during study period. Mean age of presentation in children was 7.31 ± 4.05 years (range 2 to 19 years), with male-to-female ratio of 2.16:1. Rectal bleeding was presenting symptom in 95.8 % with mean duration of 12.6 ± 15 months. Majority of polyps (77.5 %) were juvenile, and 97.2 % were located in left colon. Solitary polyps were seen in 76.6 %, multiple polyps in 11.6 %, juvenile polyposis syndrome in 6.6 %, familial adenomatous polyposis in 4.2 %, and Peutz-Jeghers syndrome in 0.8 % of the children. The polyposis syndrome group had higher age at presentation (p = 0.00006), greater likelihood of anemia, abdominal pain, and diarrhea (p = 0.0001, 0.0002, and 0.0051, respectively). Likelihood of adenomatous change in polyps was higher in polyposis syndrome group (p = 0.0003). Left colonic polyps were more common in non-polyposis group, whereas pan-colonic polyps were more common in polyposis syndrome group (p < 0.00001). Presence of anemia, abdominal pain, diarrhea, higher age at presentation (more than 10 years), and history of polypectomy are clinical indicators of polyposis syndrome.

Parameters of metabolic syndrome in Indian children with epilepsy on valproate or phenytoin monotherapy.

Objectives:The prevalence of obesity is rapidly increasing among Indian children, who, in general, are more prone to develop metabolic complications at an early age. Valproate and phenytoin are commonly used antiepileptic drugs in children. This study aimed to assess the parameters of the metabolic syndrome in Indian children with epilepsy on valproate or phenytoin monotherapy.Methods:This cross-sectional study recruited children from the Pediatric Epilepsy Clinic, Department of Pediatrics, Kalawati Saran Children Hospital, New Delhi from March 2012 to September 2012. All consecutive children diagnosed with epilepsy as per International League Against Epilepsy definition aged 3–18 years on valproate or phenytoin monotherapy for at least 6 months were enrolled at a tertiary care children's hospital in Northern India. After clinical and anthropometric evaluation (including body mass index [BMI] and waist circumference), the blood samples were analyzed for fasting serum glucose, total cholesterol, high-density lipoprotein-cholesterol, and serum triglyceride.Results:Children with BMI >95th centile and waist circumference >90th centile were not significantly different among children on valproate and phenytoin monotherapy. Children on valproate had significantly higher mean serum triglyceride (96.9 mg/dL vs. 77.6 mg/dL; P < 0.001) and total cholesterol (148.3 mg/dL vs. 132.8 mg/dL; P = 0.002) levels as compared to children on phenytoin monotherapy.Conclusions:The lipid abnormalities may be observed in children on valproate or phenytoin therapy and may warrant periodic screening.

Clinical features and endocrine profile of Laron syndrome in Indian children.

Introduction:Patients with growth hormone (GH) insensitivity (also known as Laron syndome) have been reported from the Mediterranean region and Southern Eucador, with few case reports from India. We present here the clinical and endocrine profile of 9 children with Laron syndrome from India.Material and Methods:Nine children diagnosed with Laron syndrome based on clinical features of GH deficiency and biochemical profile suggestive of GH resistance were studied over a period of 5 years from January 2008 to January 2013.Results and Discussion:Age of presentation was between 2.5-11.5 years. All children were considerably short on contemporary Indian charts with mean (SD) height Z score -5.2 (1.6). However, they were within ± 2 SD on Laron charts. No child was overweight [mean (SD) BMI Z score 0.92 (1.1)]. All children had characteristic facies of GH deficiency with an added feature of prominent eyes. Three boys had micropenis and 1 had unilateral undescended testis. All children had low IGF-1 (<5 percentile) and IGFP-3 (<0.1 percentile) with high basal and stimulated GH [Basal GH mean (SD) = 13.78 (12.75) ng/ml, 1-h stimulated GH mean (SD) = 46.29 (25.68) ng/ml]. All children showed poor response to IGF generation test.Conclusion:Laron syndrome should be suspected in children with clinical features of GH deficiency, high GH levels and low IGF-1/IGFBP-3. These children are in a state of GH resistance and need IGF-1 therapy.

Nephrotic syndrome in indian children.

Nephrotic syndrome in indian children.

Histopathological spectrum of childhood nephrotic syndrome in Indian children.

Nephrotic syndrome in children is a clinical manifestation of different histopathological subtypes. There is a paucity of recent large studies dealing with the histopathological spectrum from developing countries. A prospective study was performed from January 1990 to December 2000 at our center, involving 600 children (with age of onset up to 16 years) with idiopathic nephrotic syndrome (INS). The objectives were: (1) to study the histopathological distribution of different subtypes of INS and (2) to compare the clinical and biochemical parameters at the time of diagnosis of minimal change disease (MCD) with non-MCD subtypes. For the purpose of this study we analyzed only those children with INS who underwent biopsies. The study group included 290 children in which adequate biopsy reports were available. There were 213 males and 77 females. Mean age at onset of INS was 7.9+5.1 years. Facial edema was found in 286 (98.6%), microhematuria in 120 (41.3%), gross hematuria in 7 (2.5%), and hypertension in 77(26.8%) patients. All patients of the study group were seronegative for HBsAg and HIV. Focal and segmental glomerulosclerosis (FSGS) was the most common histopathological subtype, occurring in 110 of 290 children (38%). Other subtypes included MCD in 95 children (32%), membranoproliferative glomerulonephritis (MPGN) in 44 children (15%), mesangioproliferative glomerulonephritis in 33 children (11%), membranous glomerulonephritis in 5 children (2%), and diffuse mesangial sclerosis in 3 children (1%). In children under 8 years of age, MCD was the most common entity, whereas FSGS predominated in children with age at onset greater than 8 years. The age at onset of nephrotic syndrome was significantly higher in the non-MCD group than the MCD group. The incidence of hypertension, microhematuria, and gross hematuria was significantly lower in the MCD group. MCD remains the most common histopathological subtype in Indian children with INS and onset under 8 years of age. The incidence of MPGN continues to be high. MCD can be differentiated from non-MCD subtype by younger age at onset, absence of hypertension, and absence of microscopic hematuria.

Anti-oxidant vitamins and steroid responsive nephrotic syndrome in Indian children.

In recent years, it has been proposed that nephrotic syndrome is a consequence of an imbalance between oxidant and anti-oxidant activity. In the present study, the levels of micronutrient anti-oxidant vitamins (vitamin E, vitamin C, carotene and riboflavin) in Indian children with steroid responsive nephrotic syndrome were investigated. Their levels were measured during the acute proteinuric phase of the disease, as well as during clinical recovery (remission), in order to understand the possible role of nutritionally modifiable anti-oxidants in the aetiopathogenesis of the disease. The study was a hospital based, prospective cohort study. Serum and erythrocyte vitamin E, leucocyte vitamin C, serum carotene, erythrocyte riboflavin activity and serum malonyldialdehyde (MDA) levels were measured in 30 consecutive cases of children with nephrotic syndrome (International Study of Kidney Diseases in Children (ISKDC) criteria) during the proteinuric phase of the disease and at 4 weeks after remission was induced by steroid therapy. The same biochemical parameters were measured in healthy siblings (controls) of the 30 patients. Mean vitamin E (serum and erythrocyte), vitamin C and carotene were significantly lower during the proteinuric phase of the disease, and there was decreased erythrocyte riboflavin activity. There was significant elevation in the serum level of MDA during this phase. In addition, all these parameters tended to improve during remission, although complete normalization did not occur. These vitamins were active in performing their anti-oxidant function, as indicated by significant depression in their levels during the acute (proteinuric) phase, followed by partial recovery during remission. It may be concluded that steroid responsive nephrotic syndrome in children is associated with oxidative stress.

Spectrum of adolescent-onset nephrotic syndrome in Indian children

There are few data regarding adolescent-onset nephrotic syndrome (NS) and no guidelines for biopsy criteria and treatment protocol. This study was conducted to analyze the clinical spectrum of adolescent-onset NS and evaluate possible biopsy criteria in these children. A prospective analysis was carried out on all patients with idiopathic NS (fulfilling the ISKDC criteria) with onset between 1 and 18 years of age. They were evaluated clinically, followed by biochemical investigations and kidney biopsy. These characteristics of patients with onset between 1 and 12 years (group A) were compared with the same parameters in patients with onset between 12 and 18 years of age (group B) referred to our hospital over the same period. Among all clinical parameters, microhematuria was significantly more prevalent in adolescents (P<0.001). Kidney biopsy was performed in 88% of adolescent patients. Focal segmental glomerulosclerosis (FSGS) was the most-common histopathology in group B (46.3%) compared with minimal change disease (MCD) in group A (42.9%). Group B had a significantly higher frequency of membranoproliferative glomerulonephritis (MPGN) (P<0.005) and a significantly lower frequency of MCD (P<0.001). The biochemical parameters at the onset were similar. On comparing microhematuria, hypertension, and renal insufficiency at presentation, we observed that two or more of these features were present in all patients with MPGN and only in 19.6% of adolescents with MCD, mesangioproliferative glomerulonephritis, and FSGS. The frequency of steroid resistance was significantly higher in group B (P<0.001). In conclusion, adolescent-onset NS differs from the childhood variety in having a significantly higher frequency of hematuria, steroid resistance, and evidence of non-MCD, especially MPGN, on histopathology. Kidney biopsy can be restricted to those adolescents who have at least two abnormal clinical/biochemical features or are steroid non-responders.

Nephrotic syndrome in indian children.

A clinicopathological study of 206 Indian children with nephrotic syndrome showed a primary renal cause in 195 (96%), of which 77% were boys. In 126 children (96 boys, 30 girls) onset of the disorder occurred before the age of 5 years. Renal biopsy showed minimal lesions in 150 patients (77%); in 85 of these biopsy was done 3 months to 16 years after onset of the nephrotic syndrome. Significant renal histological abnormalities in 45 cases were labelled as mesangiocapillary 8, mesangioproliferative 4, proliferative with extensive crescents 2, membranous 3, focal segmental glomerulosclerosis 9, focal global glomerulosclerosis 2, advanced nonspecific 8, and mild proliferative 9. Nephritic manifestations were mainly associated with significant renal lesions, which were more frequently encountered when the onset of disease was after the age of 5 years. Clearance of proteinuria with corticosteroid therapy was practically confined to patients with minimal or mild renal histological changes. Our findings suggest that the pattern of idiopathic nephrotic syndrome in Indian children is similar to that reported from Western countries.

Nephrotic syndrome in children

Observations on fortyfive cases of nephrotic syndrome in Indian children have been reported. Clinical and biochemical data have been presented. The significance of some of the observations have been discussed.