[Management of autoimmune thrombocytopenic purpura: a prospective study of 147 children from the regional network RHémaP]. / Purpura thrombocytopénique auto-immun: à propos d'une cohorte prospective de 147 enfants pris en charge dans le réseau d'hématologie pédiatrique des régions PACA et Corse (RHémaP).

AIM: Assessment of the impact of guidelines from a regional pediatric network to standardize the management of childhood immune thrombocytopenic purpura (ITP). MATERIALS AND METHODS: Consensus guidelines were drawn up in centers of the pediatric network for hematological diseases, RHémaP, and a cohort of children referred for ITP in these centers was set up. A 1-year follow-up was recorded for each patient over a 43-month period. RESULTS: We report data from a cohort of 147 children. At diagnosis, we recorded severe thrombocytopenia (median=8G/l) and 141 children had hemorrhagic symptoms (96%). Only 23 children had a bone marrow aspiration (BMA) at diagnosis (16.3%), which meant a high level of implementation of the RHémaP recommendations (96%) since indications of BMA were limited to rare indications. For 135 children (91.8%), treatment fulfilled the RHémaP guidelines that were mainly based on the platelet count: 121 received intraveinous immunoglobulin (IVIG) and 14 were not treated. Among those who received IVIG, 110 were good responders (91%) at the 96-h evaluation (platelet count greater than 20G/l), nine (7.4%) were poor responders, and 1 died of intracranial hemorrhage. At 6 months, chronic ITP was observed in 40 children (32.8%). Chronic ITP was associated with a higher platelet count at diagnosis and an older age (p<10(-3) and p=10(-3), respectively). CONCLUSION: The practices recorded over a 43-month period in our cohort fulfilled the RhémaP guidelines and we conclude that we managed to standardize regional practices for children with ITP. We observed conventional epidemiological characteristics in this cohort. Older children and higher platelet count at diagnosis were significantly associated with higher frequency of chronic ITP.

[Star anise poisoning in infants]. / Intoxication aiguë par la badiane chez le nourrisson.

UNLABELLED: Star anise is used as herbal tea, for the treatment of colicky pain in infants. It may cause neurological troubles. CASE REPORTS: We report 2 cases of star anise poisoning in infants before 6 months of age. Star anise herbal tea was given by parents. Tremors or spasms, hypertonia, hyperexcitability with crying, nystagmus, and vomiting were observed. Contamination or adulteration of Chinese star anise (Illicium verum Hook), with Japanese star anise (Illicium religiosum) was proved in one child. CONCLUSION: Confusion or blending between Chinese and Japanese star anise may cause poisoning. Japanese star anise is a neurotoxic plant indeed, because it contains sesquiterpenic lactones. From November 2001, star anise products are theoretically prohibited in France, but they may be still available in some small groceries, or imported by families themselves.

Relapsing acute encephalopathy: a complication of diphtheria-tetanus-poliomyelitis immunization in a young boy.

UNLABELLED: Neurological complications of immunizations are rare. We report the case of relapsing acute encephalitis in a boy after two subsequent diphtheria-tetanus-poliomyelitis vaccinations. First the clinical signs were those of acute disseminated encephalitis. During the second episode, the boy experienced optic neuritis. Recovery was complete after both events. Because of the close temporal relationship of both these demyelinating episodes with the immunizations, we favour a cause and effect relationship. CONCLUSION: The observation of a 7-year-old boy who developed relapsing acute encephalitis after two diphtheria-tetanus-poliomyelitis vaccinations and a similar case in the literature suggests that this neurological manifestation may occur as a very rare complication of diphtheria-tetanus-poliomyelitis vaccination.

[Vitamin D-resistant rickets type II: apropos of 2 cases]. / Rachitisme vitamino-résistant de type II: à propos de deux observations.

We present two cases of vitamin D-dependent rickets type II, a rare disease caused by a hereditary disorder of the receptor for 1,25 dihydroxyvitamin D (1,25 (OH) 2 D). The first patient was seen for the first time at the age of 2 years with florid rickets, almost complete alopecia, and an elevated plasma level of 1,25 (OH) 2 D; there was no improvement following treatment with 1 alpha-hydroxy-vitamin D3). The second patient, a boy whose parents are first cousins, was seen at the age of 7 years with severe rickets, partial alopecia and an elevated serum level of 1,25 (OH) 2 D; the rickets disappeared after administering high doses of 1 alpha hydroxyvitamin D3 and vitamin D.