Abstract:
:Since the classic description of this disease by Harold Hirschsprung's. In 1886 just over a century ago, there has been a marked evolution in the diagnostic modalities, understanding of the pathophysiology, unraveling of genetic association and the treatment approach of this disease. Recent research on the molecular biology of the disease has bestowed us with a clearer understanding of the pathogenesis of the disease. Genetic deletions have been identified which explain the familial incidence of 3.6%-7.8%, and in some kindreds the inheritance rate approaches 50%. Improvement in the diagnostic approaches has evolved from full thickness rectal biopsy (under GA) to suction rectal biopsy. The development of the histochemical staining for acetylcholinesterase, allows rapid identification in the lamina propria of hypertrophied extrinsic nerve fibers which have proliferated in the absence of intrinsic ganglion cells. Frozen sections now permit screening for normal ganglia in the myenteric plexus from seromuscular biopsies during the definitive pull-through procedures or leveling colostomy. This service, however, requires significant infrastructure and an expert pathologist, not widely available. Hirschsprung's disease (HD) variants like intestinal neuronal dysplasia(IND), hypoganglionosis are better identified and treated with availability of histochemical staining. The three-stage procedure till date remains the gold standard; however, increasingly, the world over, two stage and even single stage procedures are being tried. In the era of minimally invasive surgery, perineal pull through procedure has been attempted with or without laparoscopic assistance.
journal_name
Indian J Pediatrjournal_title
Indian journal of pediatricsauthors
Lall A,Gupta DK,Bajpai Mdoi
10.1007/BF02758486keywords:
subject
Has Abstractpub_date
2000-08-01 00:00:00pages
583-8issue
8eissn
0019-5456issn
0973-7693journal_volume
67pub_type
杂志文章,评审abstract:OBJECTIVE:The present study was conducted to evaluate physical growth and nutritional status of 214 school-going girls ranging in age from 5 to 12 years of rural area in Pauri Garhwal district of Uttaranchal. METHODS:Physical growth was evaluated using eleven standard anthropometric measurements viz., height, body wei...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02724181
更新日期:2005-07-01 00:00:00
abstract:OBJECTIVE:To find out the efficacy of continuous fetal heart monitoring by analyzing the cases of cesarean section for nonreassuring fetal heart in labor, detected by cardiotocography (CTG) and correlating these cases with perinatal outcome. To evaluate whether a 30 minute decision to delivery (D-D) interval for emerge...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-008-0245-9
更新日期:2008-12-01 00:00:00
abstract::The natural history of the univentricular physiology is determined primarily by the presence (severity) or absence of obstruction to pulmonary blood flow and secondarily by the single ventricular cardiomyopathy that develops in response to chronic volume overload. Important obstruction to pulmonary blood flow will aff...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02760487
更新日期:2000-07-01 00:00:00
abstract::Every 5 years the American Heart Association (AHA) updates the Guidelines for CPR and Emergency Cardiovascular Care (ECC). The ECC Guidelines 2000 incorporates all the current consensus of experts, from not only a variety of disciplines, but also a variety of countries and cultures and is evidence based. The new Guide...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章,评审
doi:
更新日期:2003-03-01 00:00:00
abstract::The study was conducted to assess copper and zinc levels in neonate's serum, mother's serum, neonate's hair and urine and to ascertain association between them. It is of concern whether zinc and copper deficiency is present at birth and maternal blood and breast milk zinc and copper levels have any effect on this. The...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02726255
更新日期:1999-09-01 00:00:00
abstract:OBJECTIVES:To evaluate the efficacy and safety of nitazoxanide in intestinal parasitic infections in children. METHODS:Four databases, PubMed, EMBASE, Web of Science and Cochrane Library, have been systematically searched from the inception of each database up to March 1st, 2019. The enrolled studies were limited to r...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章,meta分析
doi:10.1007/s12098-019-03098-w
更新日期:2020-01-01 00:00:00
abstract:OBJECTIVE:To study the new ISPAE guidelines in neonates with congenital hypothyroidism and present authors' experience in managing these neonates. METHODS:A retrospective review of all babies who were screened for congenital hypothyroidism in the institution over a period of 5 y was conducted. Details pertaining to ma...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-020-03555-x
更新日期:2020-11-20 00:00:00
abstract:OBJECTIVES:Type 1 diabetes (T1D) adolescents often do not achieve good glycemic control. In the context of growing number of technologically savvy adolescents, this study was done to examine the effectiveness of a motivational smartphone app to improve their glycemic control. METHODS:Eleven to eighteen year old adoles...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-019-03035-x
更新日期:2019-12-01 00:00:00
abstract::Biotin Thiamine responsive Basal Ganglia Disease (BTBGD) is a rare treatable autosomal recessive metabolic disorder caused by mutations in SLC19A3 gene. It usually presents with encephalopathy and dystonia; if not treated, can progress to quadriparesis and death. Two Indian siblings born to a consanguineous marriage p...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-017-2471-5
更新日期:2018-02-01 00:00:00
abstract:OBJECTIVES:To examine the prevalence of school bullying and to investigate the behavioral, emotional, socio-economic and demographic correlates of bullying behaviors among Indian school going adolescents. METHODS:Self-reports on bullying involvement were collected from 9th to 10th class students (N = 209; Mean = 14.82...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-014-1378-7
更新日期:2014-11-01 00:00:00
abstract::Tuberous sclerosis complex (TSC) is an autosomal dominant disorder, caused due to mutations in the TSC1 and TSC2 genes. Mutations in TSC2 gene are more common than in TSC1 gene and mostly they are in the form of large genomic deletions or duplications. The authors report on a novel deletion in TSC2 gene, prenatal diag...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-011-0408-y
更新日期:2012-10-01 00:00:00
abstract::Hypertransfusion and regular chelation therapy have allowed improved survival in patients with thalassemia major (TM). Despite medical advances, growth failure and hypogonadism remain significant clinical problems in these patients in adolescence. Disproportionate truncal shortening which is common especially among ad...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/BF02760702
更新日期:2005-02-01 00:00:00
abstract::Staff of the neonatal intensive care unit (NICU) at the L.T.M.G. Hospital in Bombay, India exclusively feeds all infants breast milk. A lactation management nurse oversees its formal milk banking system by encouraging health mothers of NICU infants to donate milk and by assessing daily milk demand. Breast milk dono...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02727917
更新日期:1990-05-01 00:00:00
abstract:OBJECTIVE:To find out whether gDNA methylation can be used as a diagnostic/prognostic method for neonatal sepsis. METHODS:The study was conducted in the neonatal division of a tertiary care referral hospital. Fifty one newborns as cases and thirty seven newborns as controls were enrolled in the study. Using 5-mC DNA E...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-014-1574-5
更新日期:2015-04-01 00:00:00
abstract::Sedation is being used increasingly in children to allay anxiety and discomfort. Sedation can also increase the efficiency of performing both diagnostic and therapeutic procedures in children. There are a wide array of available sedation methods that are used by radiologists, gastroenterologists, hematologists/oncolog...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/BF02823860
更新日期:1996-01-01 00:00:00
abstract::A fifteen-month-old boy, born to consanguineously married couple, presented with asymptomatic hepatomegaly. Investigations revealed mildly deranged liver functions, necroinflammatory changes and cirrhosis on liver biopsy, a markedly raised alpha feto protein and tyrosine levels in plasma and a generalized aminoaciduri...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02820214
更新日期:2006-02-01 00:00:00
abstract:OBJECTIVE:To find out the incidence and natural history of IgMN in India. METHODS:Renal biopsies of children ≤12 y age of last 6 y were retrospectively evaluated. Their clinical and biochemical presentation were correlated. Patients with systemic diseases/disorders were excluded from the study. RESULTS:Immunoglobulin...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-012-0693-0
更新日期:2012-08-01 00:00:00
abstract:OBJECTIVES:To investigate factors involved in causing hypocalcemia in critically ill patients. METHODS:The patients aged 1 mo to 18 y, admitted to PICU at Nemazee Hospital, from May through November 2012, were reviewed. Those with impaired calcium hemostasis or on vitamin-D supplement were excluded. Calcitonin and par...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-014-1536-y
更新日期:2015-03-01 00:00:00
abstract:OBJECTIVE:The study was designed to investigate the acute respiratory disease incidence (DI) in deprived infants and test the hypothesis if maternal deprivation effects the processes of immunoregulation in infants. METHODS:The prospective study during 1 year in Tbilisi Infant's House was performed. Cohort of 136 infan...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02758305
更新日期:2006-11-01 00:00:00
abstract:OBJECTIVE:To evaluate the utility of Indian adaptation of IMCI algorithm. METHODS:Children presenting to outpatient department (n=169) or casualty (n=140) among 309 cases were assessed and classified as per IMCI algorithm, the final diagnosis made after detailed evaluation and relevant investigations, served as the go...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-008-0103-9
更新日期:2008-08-01 00:00:00
abstract:OBJECTIVE:To determine pulmonary function abnormalities in children with Sickle Cell Disease (SCD) from Western India. METHODS:In this cross sectional study conducted at Surat, Gujarat, India; equal number of age and gender matched children i.e., 99 in the age group of 6-18 y was recruited in case (children with SCD) ...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-016-2065-7
更新日期:2016-08-01 00:00:00
abstract::Functional follicular ovarian cysts are frequently reported in girls with peripheral precocious puberty (PP). These cysts are usually self-limiting and resolve spontaneously. Several drugs like antiestrogens (tamoxifen) and new aromatase inhibitors are seldom used for treatment. Here we report a girl with peripheral P...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-009-0190-2
更新日期:2009-09-01 00:00:00
abstract::In neonatal period anemia is a complex problem owing to the unique blood picture. The erythrocytic system undergoes serial adaptation to meet progressively changing demands of oxygen in the embryo, the fetus and neonate. This leads to rapid change in normal hematological change in post-birth period. Definition of anem...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02730596
更新日期:2003-11-01 00:00:00
abstract::Fainting attack or syncope in children is a common occurrence, with vasovagal syncope being the commonest cause for majority of pediatric syncope. The aim of emergency room evaluation is not to miss the rare serious underlying disorder causing syncope. A complete detailed history of the event followed by physical exam...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-011-0497-7
更新日期:2012-03-01 00:00:00
abstract::It is a common clinical practice to estimate FSH, LH and testosterone levels in patients with unilateral undescended testis (U/L UDT) as a prognostic pointer to fertility potential. Is this practice correct? To ascertain this aspect, new born rats were operated to create experimental U/L UDT by gubernaculectomy and an...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02761205
更新日期:1999-03-01 00:00:00
abstract::A two-and-a-half-year-old male child presented with recurrent attacks of intractable vomiting, psychomotor retardation since 14 months of age. He had also lower cranial nerve palsy and corticospinal involvement. Magnetic resonance imaging had shown multiple well circumscribed areas of hypointusity in T1 weighted image...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02730822
更新日期:1996-09-01 00:00:00
abstract:OBJECTIVES:To retrospectively assess growth of children with congenital adrenal hyperplasia (CAH) with special reference to puberty and to assess longitudinal growth and final height of subset of children with CAH. METHODS:A retrospective analysis of 30 children (14 boys) with classic CAH (11 salt wasters, 19 simple v...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-018-2753-6
更新日期:2019-02-01 00:00:00
abstract::The terms trend and cerebral palsy are defined emphazing the non-diagnostic nature of the cerebral palsy label. Criteria necessary for valid estimation of trends include constant methods of estimating population based numerators and denominators over a number of years, which render them hard to obtain, particularly in...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02723024
更新日期:2001-05-01 00:00:00
abstract::There is increased interest in the surgical management of epilepsy in children in the last decade or two. The plasticity of the developing brain and realization of the deleterious effects of uncontrolled seizures on the brain have prompted earlier surgery where indicated. All patients with uncontrolled partial seizure...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章,评审
doi:
更新日期:2000-01-01 00:00:00
abstract:OBJECTIVE:To evaluate the concerns the parents have with the development of their children and related conditions and investigate how often these concerns are addressed at all levels of the healthcare system. Determining parental perceptions of these issues and highlighting missed opportunities is valuable for improvin...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-020-03302-2
更新日期:2021-01-01 00:00:00