Abstract:
OBJECTIVE:Hirschsprung's disease may be associated with a number of congenital anomalies of which Down's syndrome and intestinal atresias are commonly encountered. The study aimed to assess the impact of rare associated anomalies on the diagnosis and management of Hirschsprung's disease. METHODS:A retrospective review of the clinical presentation, diagnosis and outcome of thirty five consecutive newly diagnosed cases of Hirschsprung's disease encountered over two years was performed. RESULTS:Besides Down's syndrome (two), intestinal atresia (one) and pigmentary ocular defects (two), three rare anomalies (Occipital meningocele, Calcific meconium cyst with anal stenosis, Malrotation) were encountered in four of thirty five cases. The clinical features, radiologic anatomy and gross morphology of the bowel were unconventional and the diagnosis was supported by intraoperative acetylcholinesterase staining of biopsies. Though the diagnosis was relatively delayed in these cases, the outcome has been comparable to the rest. The dilemma in their diagnosis and management and their possible pathoembryology is discussed. CONCLUSION:Awareness of such associations and a specific investigative protocol is imperative for timely diagnosis and minimal morbidity in complex presentations of Hirschsprung's disease.
journal_name
Indian J Pediatrjournal_title
Indian journal of pediatricsauthors
Das K,Alladi A,Kini U,Babu MK,D'Cruz AJdoi
10.1007/BF02762106keywords:
subject
Has Abstractpub_date
2001-09-01 00:00:00pages
835-7issue
9eissn
0019-5456issn
0973-7693journal_volume
68pub_type
杂志文章abstract::Tumor lysis syndrome (TLS) refers to the constellation of deranged metabolic state, characterized by hyperkalemia, hyperphosphatemia, hyperuricemia, hypocalcemia, and/or azotemia, secondary to rapid breakdown of tumor cells. It is a life threatening emergency that typically follows administration of chemotherapy or ma...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-012-0824-7
更新日期:2013-01-01 00:00:00
abstract::A large number of complications in thalassemia major are due mainly to iron overload. Deferoxamine in iron-overloaded patients has established that chelation therapy, when given at an adequate dose, reduces iron-related complications. Parenteral administration and the daily nuisance of an infusion pump hinder the opti...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s12098-007-0134-7
更新日期:2007-08-01 00:00:00
abstract:OBJECTIVE:To identify the risk factors for small for gestational age (SGA) babies. METHODS:The present study was a nested case control study which was carried out by two Medical Officers of Health areas (The field level administrative unit responsible for provision of preventive and promotive health services) in Colom...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-014-1382-y
更新日期:2014-10-01 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::Gastrointestinal tumours are rare in children and leiomyoblastoma of small intestine is still rarer. We report a case of leiomyoblastoma of small intestine in a child who presented with acute intestinal obstruction. ...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02831363
更新日期:1998-11-01 00:00:00
abstract::Locked-in syndrome is characterized by upper motor neuron quadriplegia, paralysis of lower cranial nerves, bilateral horizontal gaze palsy and anarthria, with preserved consciousness. It is due to a ventral pontine lesion following a basilar artery occlusion. We report the first Indian case report of locked-in syndrom...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-009-0200-4
更新日期:2009-10-01 00:00:00
abstract:OBJECTIVES:To determine the incidence, pattern, causality, preventability, severity and predictors of adverse drug reactions (ADRs) in pediatric population. METHODS:It was a prospective, observational study that included patients of either sex, of any age treated in the pediatric wards of a tertiary care hospital. Stu...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-015-2002-1
更新日期:2016-05-01 00:00:00
abstract::Caroli's disease is a rare congenital disorder and occasional cases have been reported from Japan and other parts of Asia. It comprises of congenital dilation of the lower (segmental) intrahepatic bile duct. Cholangitis liver, cirrhosis and cholangiocarcinoma are its potential complication. A case of caroli's disease ...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02825490
更新日期:2006-03-01 00:00:00
abstract::Assay of hexosaminidase A and B enzymes in four cases with developmental regression and cherry red spot on fundus examination confirmed that three cases had Tay-Sachs disease, and one case had Sandhoff disease. Prenatal diagnosis was carried out by hexosaminidase enzyme assay in amniotic fluid and cells in one family,...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02755072
更新日期:1995-07-01 00:00:00
abstract:OBJECTIVE:Acute respiratory infection is a leading cause of morbidity and mortality in under five children in developing countries. Hence, the present study was undertaken to identify various modifiable risk factors for acute lower respiratory tract infections (ALRI) in children aged 1 mth to 5 yr. METHODS:104 ALRI ca...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-007-0081-3
更新日期:2007-05-01 00:00:00
abstract:OBJECTIVES:Hydrocarbons are a common cause of accidental poisoning in children, with kerosene being the most implicated agent in rural parts of India. However, lately, liquid mosquito repellent ingestion is emerging as an important cause of hydrocarbon (kerosene) poisoning in urban households. METHODS:This is a retros...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-019-03088-y
更新日期:2020-01-01 00:00:00
abstract:OBJECTIVES:To analyze the results after surgery for spinal cord lipomas. METHODS:The authors report their results of management of 63 non consecutive random children with spinal cord lipomas treated over a period from 2001 to 2005, at the All India Institute of Medical Sciences, New Delhi, India, a tertiary care neuro...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-007-0060-8
更新日期:2007-04-01 00:00:00
abstract:OBJECTIVES:To evaluate if cardiac dysfunctions are important in assessing the outcome in newborns with Bronchopulmonary Dysplasia (BPD), by evaluating cardiac functions with N-terminal prohormone of brain natriüretic peptide (NT-proBNP) levels, M-mode and tissue doppler echocardiography at 6-12 mo of age. METHODS:Twen...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-013-0994-y
更新日期:2013-11-01 00:00:00
abstract::With improved survival of very low birth infants in India, Retinopathy of Prematurity (ROP) is emerging as a significant problem. The most important risk factor in the pathogenesis of ROP is prematurity. Other factors like, problems with oxygenation frequent blood transfusions, sepsis and apnea have also been implicat...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s12098-008-0011-z
更新日期:2008-01-01 00:00:00
abstract::Systemic infection in the newborn is the commonest cause of neonatal mortality. Data from National Neonatal Perinatal Database 2000 suggest that Klebsiella pneumoniae and Staphylococcus aureus are the commonest causes of neonatal sepsis in India. Two forms of clinical presentations have been identified. Early onset se...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/BF02722932
更新日期:2001-12-01 00:00:00
abstract::The idea that nutrition in early life (such as before conception, during pregnancy and in infancy) can influence, or programme, long-term health, known as the 'Developmental Origins of Health and Disease Hypothesis', has generated great scientific interest. This concept is particularly relevant for the development of ...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s12098-017-2554-3
更新日期:2018-06-01 00:00:00
abstract::The authors report a preterm infant with Rh-isoimmunization, who had persistent hepato-splenomegaly with conjugated hyperbilirubinemia, transaminitis, and hyperammonemia. Ultrasound abdomen revealed an intrahepatic portosystemic venous shunt (PSVS). The child was managed conservatively. On follow up at 2.4 y of age, t...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-013-0972-4
更新日期:2013-12-01 00:00:00
abstract::Asthma is fairly common in pediatric age group and the suffering due to asthma continues to increase despite excellent treatments available. One of the four major components of asthma management is patient education and is critical to the success of asthma management. Reasons for continued suffering include that our m...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章,病人教育讲义,评审
doi:
更新日期:2001-09-01 00:00:00
abstract:OBJECTIVE:To study the etiological profile of patients with acute febrile encephalopathy syndrome focusing chiefly on the viral etiology, and to correlate clinical and radiological features of patients with viral encephalitis. METHODS:A prospective hospital based study conducted on the consecutive patients admitted in...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-008-0150-2
更新日期:2008-08-01 00:00:00
abstract:OBJECTIVES:To study the etiology and burden of comorbidities across stages of chronic kidney disease (CKD). METHODS:Children, 2-16 y of age with CKD Stages II- IV were recruited over 12 mo. The etiology, clinical presentation and severity of complications were studied. RESULTS:Among 78 children [Stage II (n = 21), St...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-017-2413-2
更新日期:2017-11-01 00:00:00
abstract::Moebius syndrome is characterized by congenital complete or partial facial nerve palsy with or without paralysis of cranial nerves and often associated with other malformations. Cardiac anomalies though known are very rare and till date only 4 cases have been reported. We present a case of Moebius syndrome with suprac...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02758568
更新日期:2006-05-01 00:00:00
abstract::Paracetamol (acetaminophen) has become an antipyretic drug of choice. Due to its widespread use, toxicity secondary to overdose has increased in recent years. Children are especially vulnerable to accidental exposure due to non availability of child proof containers in India. The main clinical features of acute toxici...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/BF02761132
更新日期:1998-05-01 00:00:00
abstract:OBJECTIVE:To assess the prevalence of peripheral neuropathy in HIV infected children (>5 y) receiving stavudine-based combination anti-retroviral treatment (ART) for more than 3 mo in a cross-sectional study. METHODS:History, detailed neurological examination and nerve conduction studies were performed. RESULTS:Forty...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-014-1477-5
更新日期:2015-02-01 00:00:00
abstract:OBJECTIVE:To explore the independent role of age at menarche on menstrual abnormalities among adolescents. METHODS:The present study was a multicenter cross-sectional study on a large sample (n = 3782) of Italian girls aged 13-21 y attending secondary school who already had menarche. Girls were asked to fill in a ques...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s12098-018-2822-x
更新日期:2019-01-01 00:00:00
abstract::Antiperistaltic appendiceal conduit based on its vascular pedicle was used as hepaticoporto-appendico-jejunostomy (HAJ) in 9 infants with advanced stage of biliary atresia. Operative procedure was simple and less time consuming. The postoperative cholangitis was conspicuously absent, possibly due to the role played by...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02722419
更新日期:1989-07-01 00:00:00
abstract::The magnitude of work and existence of multiple agencies makes the task of providing school health services to all pupils of the State of Delhi with uniform standards, a very complex job. The present article looks at the matter from the provider's end, illustrates the structural and organizational features of various ...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02723659
更新日期:2000-03-01 00:00:00
abstract::Malignant tumours of the chest wall are rare in the pediatric age group. Askin tumour, a primitive neuroectodermal tumour with aggressive nature, is extremely rare. Clinico-pathological features make it quite distinct from Ewing's sarcoma. Aggressive nature of the tumour results in short clinical presentation of the p...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02726235
更新日期:2000-11-01 00:00:00
abstract:OBJECTIVE:To assess the trends and outcomes in referrals of 'phimosis for circumcision' to a tertiary care pediatric surgical department. METHODS:This is a prospective study of 100 consecutive children of presumed phimosis referred for circumcision. They were assessed and classified by the senior author as having eith...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s12098-009-0120-3
更新日期:2009-08-01 00:00:00
abstract::A bleeding child is a cause of great concern and often, panic, for parents and pediatricians alike. Causes of bleeding could be trivial or secondary to an underlying bleeding disorder or a potentially serious systemic illness. Based on etiology, they can be categorized into disorders affecting platelets or the coagula...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s12098-012-0918-2
更新日期:2013-05-01 00:00:00
abstract::The authors present a case of a preterm newborn with congenital infection of herpes simplex virus type 2. The patient was treated with newly recommended high intravenous doses of acyclovir. It can be supposed that it reduces mortality, but the high morbidity continues to be a problem. ...
journal_title:Indian journal of pediatrics
pub_type: 杂志文章
doi:
更新日期:2004-12-01 00:00:00