RESUMO
Hyaline fibromatosis syndrome (HFS) is rare autosomal recessive disease characterized by the deposition of amorphous hyaline material in skin and visceral organs. It represents a disease spectrum with infantile systemic hyalinosis (ISH) being the severe form and juvenile hyaline fibromatosis (JHF) being the mild form. Dermatologic manifestations include thickened skin, perianal nodules, and facial papules, gingival hyperplasia, large subcutaneous tumors on the scalp, hyperpigmented plaques over the metacarpophalangeal joints and malleoli, and joint contractures. ISH shows a severe visceral involvement, recurrent infections, and early death. We report a case of 2.5-year-old female patient who presented with HFS who had overlapping features of both ISH and JHF. To the best of our knowledge, very few cases of HFS have been reported in Indian literature till date.
RESUMO
This study examined acceptability among pregnant women and their husbands for HIV testing within the antenatal clinic (ANC) and delivery room (DR) of a government hospital in Pune, India from September 2000 to November 2001. Acceptance of HIV counselling and testing was high with 83% of eligible women in the antenatal clinic (851 of 1025) and 68% of eligible women in the delivery room (417 of 613) getting tested on the same day. Structured interviews were conducted on 94 pregnant women in the ANC 50 women in the DR, and 100 husbands who accompanied their wives in the ANC. These data indicated that the majority of women agreed to be tested independently without the need for further consultation with family members, a view that was strongly supported in this sub-sample of accompanying husbands. For delivering women who were not progressing in their labour, counselling in the DR allowed for individual attention to questions and concerns thereby making counselling in the DR feasible.