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 the presence of lymphoid follicles in the wall of the appendix. Our early experience with this technique shows that antiperistaltic appendiceal conduit works quite satisfactorily. The procedure has been successfully utilised to reduce the problems contributing post-operative morbidity and mortality in cases with the advanced disease. The HAJ procedure provides a conduit for bilioenteric drainage, that closely resembles to normal anatomy.

Anastomosis, Roux-en-Y↗

PubMedDate not supplied

Esophageal atresia and tracheo-esophageal fistula: a review.

The improved survival of neonates with esophageal atresia and tracheo-esophageal fistula reflects the advancement in neonatal care and anaesthesia over the years. Chick embryo studies have given new insights in the embryopathy of esophageal atresia. It is now apparent that the various types of esophageal atresia could be explained due to selective discrepancy in the growth of the 3 folds in the region of tracheo-esophageal separation. The early disturbances in organogenesis which result in esophageal atresia also lead to other associated anomalies, the incidence of which varies from 40 to 55%. These anomalies have an important bearing on the survival outcome. The physiological aspects of esophageal atresia such as esophageal dysmotility and gastro-esophageal reflux are also vital in the long term and proper treatment of the associated defects. The criteria for an ideal esophageal substitute in long gap esophageal atresia have been determined and several options are now available with good results, such as: gastric transposition, colon, gastric tube and small intestine. IN developing countries, however, a high mortality is still attributed to late referrals, low birth weight, hypothermia and chest infection.

Algorithms↗

PubMedDate not supplied

Spectrum of congenital anomalies associated with biliary atresia.

Several congenital anomalies have been reported in association with biliary atresia. We have analysed the type and frequency of anomalies observed over a 10-year period in consecutive patients operated for extrahepatic biliary atresia at our institution. Of the 107 infants who underwent a laparotomy and surgical correction of biliary atresia, 9 (8.4%) showed significant associated anomalies. Among them, 5 (55.5%) had splenic malformations, 6 (66.6%) had digestive anomalies in the form of malrotation, Meckel's diverticulum and jejunal atresia. One patient had the classical polysplenia syndrome. Our follow-up period was limited (i.e. 5 months) during which time 3 of the 9 (33.3%) patients with associated anomalies became jaundice-free. A higher incidence of malformations found in association with biliary atresia supports the congenital theory and a more thorough search for these anomalies is recommended.

Abnormalities, Multiple↗

PubMedDate not supplied

Congenital hypertrophic pyloric stenosis at birth.

Explore the source record for details and available documents.

Follow-Up Studies↗

PubMedDate not supplied

Mixed gonadal dysgenesis and dysgenetic male pseudohermaphroditism--a critical analysis.

A 10 year prospective study of 14 patients with mixed gonadal dysgenesis (MGD) and six patients with dysgenetic male pseudohermaphroditism (DMP) is reported. All of them had internal mullerian structures, along with unilateral or bilateral dysgenetic testes, ambiguous external genitalia. Twelve had been brought up as male, nine of whom had a unilateral descended testis. Eight had been reared as females, as they had bilaterally undescended gonads, and ambiguous genitalia. Clinical examination, retrograde genito-urethrography and cytogenetic studies suggested the diagnosis in 16 patients, while four were diagnosed on inguino-abdominal exploration for undescended testis. This report delineates more clearly the clinical profile of these orders. All the patients reared as male were assigned the male gender following abdominal gonadectomy, retention of scrotal testis and male genitoplasty. The eight patients who were reared as females underwent bilateral salpingo-gonadectomy and female genitoplasty. This management differs from the usual recommendation that all such children should be reared as females. Ten patients (50%) had maternal history of previous abortion/stillbirth, or drug intake in the first trimester of pregnancy suggesting a role of these factors in the etiology. All cases of DMP had a 46,XY karyotype, while eight of 14 cases of MGD had mosaicism with 45X/46,XY cell lines in blood or gonadal cultures. The clinicopathological features of patients of MGD and DMP were similar. It is suggested that these two disorders represent different spectra of the same disorder. A unifying concept of etiopathogenesis is proposed.

Child↗

PubMedDate not supplied

Congenital adrenal hyperplasia: experience at intersex clinic, AIIMS.

During 1981-88, 63 cases of female pseudohermaphroditism (FPH) were seen at the Intersex clinic at AIIMS, of whom 34 (54%) were diagnosed as due to congenital adrenal hyperplasia (CAH). Though ambiguity was present at birth in most cases, only one child was brought immediately after birth, while 14 presented after one year. Family history of affected siblings and fetal wastage was present in 10. Salt wasting symptoms were present in 13 (38.2%), evidence of early virilization in 10 (29.4%) and generalised hyperpigmentation in 7 (20.6%). Clitoromegaly was present in 30 children with labial fusion in 10 and scrotalisation of labia in 6. The urogenital opening was single in 25 (73.5%). Buccal smear was positive for sex chromatin in 19. Chromosomal pattern showed 46 XX in 33. Dyselectrolytemia was present in 16 children. Bone age was advanced in all. Adrenal hyperplasia could be documented in 3 on CT scan. All the girls were put on hydrocortisone or prednisolone, and fluodrocortisone was given only to children with salt wasting CAH. Children with CAH are being brought to medical attention much too late and investigative and therapeutic facilities are grossly inadequate. There is a need to educate primary care physicians for early case detection and provide minimum diagnostic and therapeutic facilities in regional centres.

Adrenal Hyperplasia, Congenital↗

PubMedDate not supplied

Estimation of tissue DNA content in Wilm's tumour.

DNA genome, is affected by malignant processes. Estimation of DNA content was carried out biochemically using Gile's and Myer's technique to assess its prognostic value in 21 cases of Wilm's tumour. The DNA content of tumour tissue and normal adjoining renal tissue was estimated in each case. The DNA content was significantly higher in Wilm's tumour tissue as compared to normal renal tissue (p < 0.05). As the disease advanced, the DNA content of the tumour further increased. The tumours having unfavourable histology had significantly higher DNA content as compared to favourable histology cases (p < 0.001). Similarly, the patients who died because of the disease, had a very high DNA content as compared to those who survived. Further, it was seen that two fold increase in DNA content indicated advanced stage (III & IV), whereas a three fold or more increase indicated grave prognosis as it was usually seen in advanced stage with unfavourable histology and such cases died early. Thus, the DNA content estimation has a prognostic significance in Wilm's tumour.

Child, Preschool↗

PubMedDate not supplied

Ambiguous genitalia--an Indian perspective.

Ambiguity of genitalia in the newborns and children still remains a poorly understood subject and even during the postgraduate teaching programmes, very little attention has been paid to make this subject popular. There is also no chapter "without tears" on the subject for the proper management and to prevent the agony of the parents resulting from the ignorance and the social stigma. The purpose of this brief communication is to project the practical management of Children with intersex disorders, based on our clinical experience from the pediatric intersex clinic at AIIMS.

Adolescent↗

PubMedDate not supplied

Acute suppurative thyroiditis in children.

Explore the source record for details and available documents.

Acute Disease↗