[DRESS syndrome, a drug reaction which remains bad known from paediatricians]. / Le DRESS syndrome, une réaction d'hypersensibilité aux médicaments, qui reste mal connue des pédiatres.

The DRESS syndrome (Rash with Eosinophilia and Systemic Symptoms) is a drug hypersensitivity reaction poorly known by paediatricians. It occurs within 1 to 8 weeks of treatment. Clinical features associate in variable patterns, fever, rash, lymphadenopathies, arthritis and potentially life-threatening damage (hepatitis, nephritis, pneumonitis), hyperleucocytosis and eosinophilia. This condition must be early recognized in order to immediately stop suspect drugs. A 6.5 year old girl had a febrile rash, hyperleucocytosis, lymph nodes and cytolitic hepatitis probably due to phenobarbital. Diagnosis of DRESS syndrome was performed only 13 days after the beginning of the eruption. Evolution was favorable but characterized by the recurrence of the febrile eruption with pleuritis. DRESS syndrome is a well described disease that occurs during treatment with a number drugs, particularly anti-epileptic drugs. Steroid therapy and immunoglobulins are proposed for treatment but have not been evaluated.

[Polymyositis, dermatomyositis and pregnancy: high-risk pregnancy. A further case report and review of the literature]. / Polymyosites, dermatomyosites et grossesse: grossesse à haut risque. Nouvelle observation et revue de la littérature.

The authors report a new case where polymyositis/dermatomyositis and pregnancy were associated. The polymyositis and dermatomyositis appeared in the last third of the pregnancy. A caesarean operation had to be performed for fetal distress after 37 weeks of amenorrhoea. An apparently dead small-for-dates baby was born. Progress, however, after resuscitation of the baby was good. At 20 months after delivery the polymyositis and dermatomyositis also were improved. Two and a half years later there was no recurrence. In the light of cases that have previously been reported in the literature, it has been established that on the one hand pregnancy may trigger off the condition and worsen the PM/DM and on the other hand the condition has a bad effect on the progress of the pregnancy. 50% of the babies are born dead or die in the week after delivery and 55% are born prematurely. We discuss the possibility that PM/DM is of viral origin. A confirmation of this is the presence of viral inclusion bodies on electron microscopy found in the muscle cells of the patients who have PM/DM. However, in the previous history of these patients an acute viral myositis in childhood is a troublesome factor. Pregnancy, because it involves alterations in the immune responses of the patients, may lead to a recrudescence of a "sleeping" virus and so be responsible for the appearance or aggravation of polymyositis and dermatomyositis.

[Study of erythrocyte bilirubin. Evaluation of the risk of kernicterus]. / Etude de la bilirubine érythrocytaire. Appréciation du risque d'ictère nucléaire.

351 analysis of bilirubin binding by erythrocytes (BE) and of non conjugated bilirubin (NCB) are realised. 15 analysis have an increasing of the value: 6 with NCB and BE high with EST; 3 with NCB high and BE normal with EST; 6 with NCB high and BE normal without EST and any complication.

[Analysis of bilirubin binding by erythrocytes. Study of kernicterus risk (author's transl)]. / Etude de la bilirubine érythrocytaire. Appréciation du risque d'ictère nucléaire.

351 analysis of bilirubin binding by erythrocytes (BE) and of non conjugated bilirubin (NCB) are realised. 331 analysis. 15 analysis have an increasing of the value : 6 with NCB and BE high with EST ; 3 with NCB high and BE normal with EST ; 6 with NCB high and BE normal without EST and any complication.

[Bone-marrow biopsy in chronic myeloid leukemia. Value of the initial examination]. / La biopsie médullaire dans la leucémie myéloïde chronique. I.- Intétêt lors de l'examen initial

In 31 patients with chronic myeloid leukemia, the initial bone marrow biopsy constantly showed considerable cell richness. The maturation of the granulocytes was harmonious in 52% of cases, with a predominance of myelocytes in 32% of cases, and groups of myeloblasts in 13% of cases. Eosinophilia was frequent and fairly intense. Fairly numerous loaded cells and Charcot-Leyden crystals were signs of granulocyte failure in the bone marrow. The erythroblasts were reduced, the megakaryocytes increased. Myelofibrosis was present in 45% of cases. This gave a very characteristic histological appearance. It was not possible to draw up correlations between the type of granulocyte maturation and/or the presence of myelofibrosis and, on the other hand, the clinical course of the disease, but the small number of case studied both in our series and in the literature do not permit one to carry out a proper statistical study.

[Bone-marrow biopsy in chronic myeloid leukemia. Significance during development]. / La biopsie médullaire dans la leucémie myéloïde chronique. II. - Intérêt au cours de l'évolution

In 29 patients bone marrow biopsy carried out during the course of chronic myeloid luekemia, permitted the authors to divide up the patients into 5 histologically different groups according to the association of 3 parameters: the richness in granulocytes, the state of the matrix, the degree of leukoblastosis. There was, in most cases, correspondence between the histological appearance and the clinical and laboratory symptoms. This was particularly clear when the patient entered the terminal phase of the disease. 4 of the 5 histological groups had an unfavourable short-term prognosis, i.e., granulocyte hyperplasia with myelofibrosis, aplasia with normal matrix or with myelofibrosis and massive leukoblast invasion.