Successful treatment of systemic juvenile xanthogranulomatosis with cytarabine and 2-chlorodeoxyadenosine: case report and review of the literature.

The non-Langerhans cell histiocytosis (LCH) juvenile xanthogranulomatosis (JXG) is usually a benign disease limited to the skin. Only a few cases of systemic disease with at least two affected organs and lethal outcomes have been reported to date. Treatment is controversial and no standard protocol is available. We report the rare case of a 22-month-old boy presenting multiple erythematous brownish papules of the head, trunk and legs, which had developed starting from his 6th month of life. Additional symptoms were delayed psychomotor development, hydrocephalus and hepatosplenomegaly. Further diagnostics revealed a systemic JXG with involvement of the skin, central nervous system, liver and spleen. The patient did not respond to initial therapy with prednisone and vinblastine according to protocol III for LCH. However, further therapy with cytarabine and 2-chlorodeoxyadenosine followed by a consolidation phase with 2-chlorodeoxyadenosine alone was successful and the patient is in his 4th year of remission. We provide a comprehensive review of the reported cases of systemic JXG to date.

[The current standing of obstetrical analgesia and anesthesia. A survey of North Rhein-Westfalia]. / Stand der Analgesie und Anästhesie in der Geburtshilfe. Eine Umfrage aus Nordrhein-Westfalen.

BACKGROUND: The purpose of this study was to determine obstetrical pain management practices for labour for spontaneous and operative vaginal deliveries since there were no comparative German data available for the past 10 years. METHODS: A mail survey was sent to the chief anaesthetists of all hospitals in North Rhine-Westfalia designated to have obstetrical beds. The confidential and standardised questionnaire consisted of 24 mainly multiple-choice questions relating to general issues and methods of analgesia and anaesthesia for vaginal deliveries. RESULTS: In all, 118 completed replies to 258 sent questionnaires were received, giving a response rate of 46%. Among the 118 hospitals there were 79,157 vaginal deliveries annually. All participating hospitals practiced either systemic analgesics/spasmolytics and/or regional-anaesthetic methods (Table 2). Perineal local infiltration (23.7% of vaginal deliveries, in 99% of cases performed by the obstetrician) and epidural analgesia (23.2% of vaginal deliveries, in 81% of cases performed by an anaesthetist) were the commonest regional-anaesthetic methods. Pudendal nerve blocks were performed in 18.5% of vaginal deliveries (Table 1). Of all participating hospitals, 97% provided a 24-h epidural service. The method of epidural anaesthesia was widely homogeneous in all 118 hospitals (Table 3). Other methods of regional analgesia (i.e., epidural infusions or patient-controlled epidural analgesia) were performed only rarely. CONCLUSIONS: The methods of obstetrical analgesia and anaesthesia are on a high level and show a broad homogeneity in all hospitals. Overall, the results indicate, in comparison to former studies and in common with other countries, steadily increasing use of regional anaesthesia performed by anaesthetists in contrast to decreasing numbers of local infiltrations performed by obstetricians.