Determination of 'free' bilirubin, erythrocyte-bound bilirubin and unconjugated bilirubin was used to test the effects of ceftriaxone on the binding of bilirubin to albumin. This study, performed on blood samples from icteric neonates, showed that the addition of ceftriaxone produced an increase of free bilirubin and erythrocyte-bound bilirubin and a decrease of unconjugated bilirubin. Ceftriaxone displays a significant displacing effect at concentrations obtained during therapeutic use and should be used with caution in high-risk jaundiced infants.
We report the effects of recombinant human erythropoietin (rHuEPO) in the treatment of late hyporegenerative anemia in 2 neonates with Rh hemolytic disease who had received several in utero exchange transfusions. In both cases anemia occurred at 6 weeks of age and we started therapy at approximately 70 days of age. We used rHuEPO at 250 U/kg three times a week. We also used high-dose intravenous immunoglobulin therapy. One week after initiation of erythropoietin treatment, an increase in reticulocyte count and Hb level was noted in our 2 patients. They did not require further erythrocyte transfusions but they already had received two transfusions after birth. There were no side effects attributable to rHuEPO treatment.
The twins' neuropsychological outcome was not more marked than that of the singletons. Birth weight discordance and chorionicity were the only predictive perinatal factors with worse outcome in the twin population.
The keratitis, ichthyosis, and deafness (KID) syndrome is a rare congenital disorder of the ectoderm characterized by diffuse hyperkeratotic erythroderma, keratitis with neovascularization of the cornea, and severe neurosensory hearing loss. A familial occurrence of this syndrome has been mentioned in four reports including three of vertical transmission and one of two affected sisters born from consanguineous, unaffected parents. We report for the first time a familial case of KID syndrome in two half siblings born to the same unaffected mother. This new observation allows us to propose various hypotheses about its mode of inheritance.The keratitis, ichthyosis, and deafness (KID) syndrome, so-called by Skinner et al. in 1981 (l), is a rare disorder including an extensive hyperkeratotic eruption, neurosensory deafness, and keratitis with neovascularization of the cornea. It is a congenital ectodermal disorder without a clear mode of inheritance. However, three previous reports of vertical transmission have suggested a dominant pattern of transmission ( 2 4 ) . In contradiction of this speculation are the cases of two sisters born of consanguineous parents (cousins four generations before) which are suggestive of recessive transmission (5). We report herein for the first time a familial case of KID syndrome affecting two half siblings (same unaffected mother) of different sex. This new observation allows us to exclude the possibility of an Xchromosomal recessive mode of inheritance. CASE REPORTS Patient 1A 5-year-old black boy was referred for an extensive chronic pruritic hyperkeratotic dermatosis. He was born to unrelated healthy parents (Malagasy mother with dark skin and Comorian father with pale skin) after a full-term pregnancy and normal delivery. Skin lesions began at the age of 3 months and his hair was lost by 6 months. At his first physical examination in our unit he had multiple vermcoid lesions, diffuse ichthyosis with palmoplantar keratoderma, and total alopecia including the scalp, eyebrows, and eyelashes (Figs. 1, 2). A hard crust was present on his scalp and nipples; moreover, his skin was thickened and hyperkeratotic over the cheeks, knees and elbows, as well as his perineum and extremities. The palms and soles showed a reddish orange keratoderma. His nails were dystrophic, with both leukonychia and hypertrophy. He had moderate growth retardation (-2 SD) and his development was impaired by sensory handicaps including complete deafness and severe loss of visual acuity with permanent photophobia. An audiogram indicated neurosensory deafness. The ophthalmoscopic examination disclosed a thickened, almost opaque corneal epithelium with superficial neovascularization and linear opacities. Histologic findings on specimens from venucoid hyperkeratotic areas of the integument
The keratitis, ichthyosis, and deafness (KID) syndrome is a rare congenital disorder of the ectoderm characterized by diffuse hyperkeratotic erythroderma, keratitis with neovascularization of the cornea, and severe neurosensory hearing loss. A familial occurrence of this syndrome has been mentioned in four reports including three of vertical transmission and one of two affected sisters born from consanguineous, unaffected parents. We report for the first time a familial case of KID syndrome in two half siblings born to the same unaffected mother. This new observation allows us to propose various hypotheses about its mode of inheritance.
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