Clinical, cytogenetic & hormonal profile in extreme hypospadias with bilaterally descended testes.
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Biomedical subjects
Publications and source records attributed to M Rohatgi.
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A new method for accurate positioning of the tip of the atrial catheter in the middle of the right atrium in ventriculoatrial shunt operations is described. The correct length of the catheter is obtained from a consideration of the surface anatomy of the heart in each patient. The position of the tip of the catheter was checked by postoperative chest radiographs in postero-anterior and lateral views. The technique was used in 50 cases of hydrocephalus in which ventriculoatrial shunts were performed. The approach is simple and reliable, and is useful not only for elective operations but also in emergency situations where specialized monitoring equipment is not readily available.
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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.
The clinical and myelographic evaluation in 24 children with spina bifida occulta has been correlated with the operative findings. Myelograms were done using Myodil in 11 patients and Metrizamide in 13 patients. The diagnostic quality of myelograms done with Metrizamide was comparatively better. Myodil myelography failed to demonstrate diastematomyelia in one case and a dural sac in 3 cases of lipomeningomyelocele, whereas, Metrizamide failed to demonstrate a sac in one case of lipomeningomyelocele only. Worsening of the neurological status was observed in only 3 cases in whom myelography was done with Myodil. The radiographic and operative correlation has been discussed.
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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.