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J Pediatr Surg. 1998 Jan;33(1):130-2.
Abnormal colonic interstitial cells of Cajal in children with anorectal malformations.

Kenny SE, Connell MG, Rintala RJ, Vaillant C, Edgar DH, Lloyd DA.

Department of Paediatric Surgery, Alder Hey Children's Hospital, Liverpool, England.

BACKGROUND/PURPOSE: Constipation is a frequent functional problem in children after operation for all types of anorectal malformations. Although this has been assumed to be caused by hypomotility of the rectosigmoid colon, recent studies have demonstrated generalized colonic hypomotility in children with high or intermediate anomalies. The cause of this disorder is unknown. The aim of this study was to determine whether the observed colonic hypomotility seen in patients with anorectal malformations was caused by defects in distribution or density of interstitial cells of Cajal (ICC), recently identified as 'intestinal pacemaker cells'. METHODS: Colostomy specimens from 12 patients with high anorectal anomalies (ARM group; age 0 to 14 months) were compared with colostomy specimens from five control patients with nonmotility-related gastrointestinal pathology (age, 1 to 4 months). Specimens were immunohistochemically labelled with antibodies to PGP9.5, a marker for neural tissue, and antibodies to c-kit, a recently characterized marker for interstitial cells of Cajal (ICC). RESULTS: Ganglion cells were present in all histological specimens. Abnormalities in distribution and density of c-kit-positive ICC were present in 7 of 12 ARM patients. In two ARM patients, ICC were completely absent, and in five patients, ICC density was markedly reduced in circular muscle and at the submucosal border of circular muscle. Only five ARM patients had a distribution of ICC similar to that of control patients. CONCLUSION: Defects in the population of intestinal pacemaker cells may underlie the colonic hypomotility seen in high anorectal malformations and hence may contribute to refractory constipation.

Laxative online source: www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=9473118&dopt=Abstract constipation laxative [PubMed - ind

J Pediatr Surg. 1998 Jan;33(1):133-7.
Bowel management for fecal incontinence in patients with anorectal malformations.

Pena A, Guardino K, Tovilla JM, Levitt MA, Rodriguez G, Torres R.

Department of Surgery, Long Island Jewish Medical Center, New Hyde Park, NY 11040, USA.

BACKGROUND/PURPOSE: Fecal incontinence is common in patients operated on for anorectal malformations. Treatment with enemas, laxatives, and medications are often given by clinicians in an indiscriminate manner and without a demonstrated benefit. A systematic diagnostic approach and bowel management program was developed for patients suffering from fecal incontinence, and a retrospective analysis of the results is presented. METHODS: Three hundred forty-eight patients were seen in consultation for fecal incontinence after repair of imperforate anus at other institutions. Clinical and radiological evaluation helped determine different types of patients. Group I consisted of 147 patients who were considered candidates for reoperation and forms the basis of a future report. Group II included 172 patients who had no potential for bowel control and were therefore candidates for bowel management. These patients fell into two categories; group IIA included 44 patients with incontinence and constipation. The bowel management involved the use of daily large enemas only. Group IIB included 128 patients with incontinence and a tendency to diarrhea. Group III consisted of 29 patients who had pseudoincontinence. They had an original defect with good prognosis, good sphincters, good sacrum, and a well-located rectum. They suffered from severe constipation, megasigmoid, chronic fecal impaction, and overflow pseudoincontinence and were treated with laxatives or sigmoid resection. RESULTS: Bowel management was successful in 93% of patients in the constipation group (IIA) and 88% in the diarrhea group (IIB). Ninety-seven percent of patients in group III became fecally continent. CONCLUSION: Bowel management consisting of enemas, laxatives

Sante. 1997 Sep-Oct;7(5):291-4.
[Clinical characteristics of primary hypothyroidism in Dakar. Apropos of 37 cases]

[Article in French]

Sidibe el-H, Fall L, Sow AM.

Clinique medicale II, Centre Marc-Sankale, Dakar-Fann, Senegal.

Primary hypothyroidism, other than cases of endemic goiter, has rarely been described in Africa. We conducted a retrospective study of the patients admitted to our hospital unit between 1985 and 1996. The inclusion criteria were clinical signs of hypothyroidism and low levels of thyroid-stimulating hormone. We investigated socio-demographic, clinical (hypometabolic syndrome, cutaneomucal syndrome, muscular syndrome) and etiological (spontaneous thyroid atrophy, thyroidectomy, multinodular goiter) factors. Overall, our study population contained 37 cases, 8 men and 29 women. The mean age of the men was 40.8 +/- 19.2 years and that of the women was 41.5 +/- 14.5 years. Eighteen patients (about 50%) lived in the suburbs, 25% of patients were from urban areas and 25% from rural areas. The associated clinical signs were: 1) hypometabolism: constipation (51% of cases), bradycardia (45%), physical asthenia (40%), sleeping during the day (32%), frilosity (35%); 2) cutaneomucal syndrome: hoarseness (48%), alopecia (32%), facial puffiness (27%), macroglossia (24%), hypoacousia (21%), weight gain (18%), dry skin (16%), pallor (2%); 3) muscular syndrome was rare: myalgia (4 cases), muscle weakness (2 cases). Mean total cholesterol concentration was 2.54 +/- 0.75 g/l; mean total T3 was 1.027 +/- 0.84 nmol/l; mean total T4 was 16.70 +/- 16.89 nmol/l; mean TSH concentration, measured by radiometry, was 63.74 +/- 51.01 mIU/l. The etiology was goiter in 13 cases, thyroidectomy (11 cases) and spontaneous thyroid atrophy (13 cases). Thus, primary hypothyroidism does occur in African hospitals, particularly in Senegal. This disease, which has traditionally been reported in public health studies of endemic goiter, also occurs in cosmopolitan African environments.

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