[Ritual tooth mutilation among Aleutians].
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OBJECTIVE: To assess the reliability of self reported form of female genital mutilation (FGM) and to compare the extent of cutting verified by clinical examination with the corresponding World Health Organization classification. DESIGN: Cross sectional study. SETTINGS: One paediatric hospital and one gynaecological outpatient clinic in Khartoum, Sudan, 2003-4. PARTICIPANTS: 255 girls aged 4-9 and 282 women aged 17-35. MAIN OUTCOME MEASURES: The women's reports of FGMthe actual anatomical extent of the mutilation, and the corresponding types according to the WHO classification. RESULTS: All girls and women reported to have undergone FGM had this verified by genital inspection. None of those who said they had not undergone FGM were found to have it. Many said to have undergone "sunna circumcision" (excision of prepuce and part or all of clitoris, equivalent to WHO type I) had a form of FGM extending beyond the clitoris (10/23 (43%) girls and 20/35 (57%) women). Of those who said they had undergone this form, nine girls (39%) and 19 women (54%) actually had WHO type III (infibulation and excision of part or all of external genitalia). The anatomical extent of forms classified as WHO type III varies widely. In 12/32 girls (38%) and 27/245 women (11%) classified as having WHO type III, the labia majora were not involved. Thus there is a substantial overlap, in an anatomical sense, between WHO types II and III. CONCLUSION: The reliability of reported form of FGM is low. There is considerable under-reporting of the extent. The WHO classification fails to relate the defined forms to the severity of the operation. It is important to be aware of these aspects in the conduct and interpretation of epidemiological and clinical studies. WHO should revise its classification.
A 7-month-old infant, son of consanguinous Indian parents, presented with recurrent chewing of his digits in a median nerve distribution as the primary manifestation of carpal tunnel syndrome, in conjunction with features consistent with congenital insensitivity to pain. Electromyography (EMG) demonstrated severe median nerve entrapment at the wrist bilaterally, but other nerves were normal. In spite of clinical evidence of diffuse pain insensitivity, sural nerve and skin biopsies were normal, and he had no evidence of autonomic dysfunction. Hand findings evolved with scarring and infection of median innervated digits and loss of fine motor skills. Carpal tunnel release resulted in complete clinical resolution and significant EMG improvement. Milder symptoms and EMG evidence of median nerve entrapment were demonstrated in both parents, paternal grandparents, and several of his father's siblings. We hypothesize this child may be homozygous for a mutant allele that in its heterozygous state predisposes to familial autosomal dominant carpal tunnel syndrome. Homozygosity for this or another mutant allele may be responsible for his congenital insensitivity to pain.
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