Archives françaises de pédiatrie
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Case Reports
[Indifference to pain secondary to congenital sensory neuropathy. Apropos of a new case].
The authors report a new case of indifference to pain secondary to hereditary sensory neuropathy in a 3 year 9 month-old boy. This child presented with isolated diffuse deficiency of pain and heat sensitiveness with preserved touch without any other neurologic involvement or anhidrosis. Nerve biopsy showed the complete lack of amyelinic fibers. P substance, which might act as a mediator or modulator of the nociception, was absent from the cutaneous nerve endings.
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Ten homozygous sickle cell patients, aged from 4 to 20 years, underwent a periodic red blood cells transfusion program. Indications were = one stroke or more in 4 cases, repeated severe painful crisis in 2 cases, and for others = chronic anaemia induced by hypersplenism, priapism, chronic leg ulceration, dwarfism with delayed sexual maturation. Transfusion program lasted from 6 months to 6 years. ⋯ No red cell antibodies were found. An HIV contamination occurred in 3 children. All transfused patients during a period exceeding one year developed iron overload, needing a daily chelation by intramuscular deferoxamine in two of them.
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Five neonates born to women who had had varicella late in pregnancy or in the post-partum were admitted to our unit during the last year. In utero transmission of varicella-zoster virus occurred in 2 cases. One of them had no clinical eruption but specific IgM at a titer of 1/200. ⋯ It may develop severe neonatal varicella (mortality: 20-30%). A neonate in critical condition was successfully given a prophylactic treatment by Aciclovir IV (15 mg/kg/8 h for 5 days) and varicella-zona immunoglobulins (2 ml on days 1, 2, 3). This approach may be the best treatment for babies at risk for severe neonatal varicella.
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The authors report the case of an 8 year-old girl admitted for an erysipelas-like rash of the lower limbs and an episode of Henoch-Schönlein purpura. These manifestations were subsequently attributed to familial mediterranean fever. This case report illustrates the polymorphism of cutaneous manifestations in this disease.
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A new lethal case of nemaline myopathy is reported. Muscle biopsy at 20 days of age permitted the diagnosis but this boy died at the 35th day of life. In a review of the literature (15 similar cases) the authors analyse the diagnostic, histopathogenic, genetic and evolutive aspects of this heterogeneous disorder, apparently less "benign" than previously thought.