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Biomedical subjects

H Mishalany

Publications and source records attributed to H Mishalany.

17 recordsLinked to original sources

Seven years' experience with idiopathic unremitting chronic constipation.

Two hundred sixty patients with severe chronic constipation were studied by rectal biopsy, barium enema, and anorectal manometry. None had any medical, neurologic, or anatomic cause for their complaint. All biopsies showed presence of ganglion cells and absence of hypertrophied nerves. Barium enemas showed a dilated anorectum (otherwise normal colon) in 183 (70%) and a normal anorectum and colon in 77 (30%). Manometry showed three patterns. One hundred seventy-eight patients (68%) had pressures between 0 and 20 mmHg with normal anorectal reflex, 69 (26%) had pressures between 0 and 20 mmHg with absence of anorectal reflex, and 13 (16%) had high pressures (20 to 60 mmHg) with normal anorectal reflex. One hundred seventy-eight patients (68%) were treated conservatively by diet and/or laxatives and did well. Eighty-two (32%) required a posterior internal sphincter myectomy after failure of prolonged conservative therapy. All patients were given senna postoperatively (one tablet or one teaspoon every night). Thirty-five patients (43%) did not require the senna after a few months and have daily bowel movements. Forty-two patients (51%) are still using senna. Ten (12%) experienced occasional impaction relieved by enemas. Two recurrences required reoperation. Five (6%) did not benefit from the operation. Sixteen had immunoperoxidase antineurofilament studies that showed no distinctive pattern as described by Kluck et al.

Barium Sulfate

Deficient neurogenic innervation of the myenteric plexus with normal submucous plexus involving the entire small and large bowel.

A newborn presented with a picture of intestinal obstruction. Multiple biopsies of the small and large bowel showed an unusual neurogenic innervation. The myenteric plexus of Auerbach was severely depleted of ganglion cells and nerve fibers, while the submucous plexus of Meissner was normally innervated. An ileostomy failed to function and extended trial with experimental smooth muscle stimulant (Cisapride) was equally ineffective. The patient was finally treated by a myectomy from the duodenum to the descending colon with a sigmoid colostomy. This procedure, coupled with a Nissen fundoplication, stopped the vomiting and allowed normal defecation through the colostomy. The patient is presently taking increasing increments of oral fluids with a concomitant decrease in the volume of parenteral nutrition. The myectomy initiated marked hypertrophy of the muscularis mucosa. Could this muscular hypertrophy account for the improvement in bowel function? Possible etiology will be discussed. We caution that rectal submucosal suction biopsy alone may be misleading if normal ganglion cells and nerve fibers are found, yet the patient's clinical symptoms fail to improve. A full thickness bowel wall biopsy is then recommended.

Ganglia, Parasympathetic

Congenital diaphragmatic hernia in monozygotic twins.

This is the third reported instance of identical twins, each with left-sided posterolateral congenital diaphragmatic hernia (CDH) (Bochdalek type), who have been operated upon successfully. The associated anomalies were mirror image undescended testicles. Comparative review of familial and sporadic cases of CDH revealed that males were more commonly affected in the former, while females were more commonly affected in the latter. There was no known etiology in either type. Both familial and sporadic cases shared a high incidence of associated anomalies (40% to 50%). Three anomalies were equally found in both types, mainly pulmonary hypoplasia, intestinal malrotation, and patent ductus arteriosus. Central nervous system anomalies were highly prevalent in the sporadic cases (55% to 75%), while cardiovascular and genitourinary anomalies (30% each) were the more common anomalies encountered in the familial cases.

Diseases in Twins

Congenital asplenia and anomalies of the gastrointestinal tract.

Congenital asplenia has been traditionally described in association with cardiopulmonary anomalies (Ivemark syndrome). A case of congenital asplenia with duplication of the hindgut and genitourinary tract, myelomeningocele, situs inversus abdominis, and imperforate anus generated interest in review of the gastrointestinal anomalies of 36 patients who died of the disease in Childrens Hospital of Los Angeles. A significant number were found to have situs inversus, malrotation of the bowel, esophageal varices, duplication and hypoplasia of the stomach, Hirschsprung's disease, imperforate anus, and duplication of the hindgut. These patients present with symptoms referable to their gastrointestinal anomalies and may not draw attention to the concomitant cardiopulmonary anomalies or to the absence of the spleen. It becomes important, therefore, to establish the presence of the spleen in infants suffering from various anomalies of the gastrointestinal tract because of the overwhelming septicemia that is known to complicate the clinical management of these patients.

Adolescent

Ten-year experience with childhood rhabdomyosarcoma.

The ten-year (1965-1975) experience of the Pediatric Tumor Clinic with 14 cases of proven CRM was reviewed. Seven cases seen prior to 1970 acted as control. They were treated by surgery and X--ray therapy alone (one received one chemotherapeutic agent). Seven other cases seen after 1970 adhered to a strict protocol of therapy. After surgery (resection or biopsy), roentgenological and hematological survey for diagnosis and staging, they received curative radiotherapy locally in doses of 5,200 r to 6,000 r. Concomitantly a regimen of multiple chemotherapeutic agents (vincristine, dactinomycin and cyclophosphamide) was administered according to the protocol followed at the M.D. Anderson Hospital, Houston. Only one of the seven controls is alive and free of disease for 9 years. Only one of the seven cases who adhered to the protocol died. The other six are alive and free of disease for periods of one to two and a half years (one has a 4 months follow-up so far). One of the six cases presenting with a solitary pulmonary metastasis had a lobectomy 2 and a half years after start of treatment, as no new metastasis developed. Another, presenting with a local recurrence after excision is alive and free of disease 2 years after the star of treatment with the protocol. The results of treatment of CRM with the protocol adopted show a significant improvement in terms of local recurrences, distant metastasis and survival rates.

Child

Pyloro-duodenal atresia. A report of three families with several similarly affected children.

Seven patients born with pyloro-duodenal atresia are reported, one had an additional atresia of the small and large bowel, and another had a pneumoperitoneum probably secondary to rupture of the stomach. All patients had the same radiological findings and had no air distal to the pylorus. Consanguinity was elicited in four and a familial incidence of an identical malformation in siblings in six. 6 of the 7 patients were operated on and 4 are living and well 6 months to 5 years later. This report raises the total number of published cases of pyloro-duodenal atresia to 46, and provides further evidence for an autosomal recessive mode of inheritance.

Duodenum