[Prader-Willi syndrome: a 5 cases report]

The Prader-Willi syndrome is a rare genetic syndrome. Its diagnosis is currently well codified, based on clinic and paraclinic criteria. Its management has to be precocious and multidisciplinary. The authors relate 5 cases, and they insist on the difficulty of the diagnosis and of the management of this syndrome. Method: 5 cases of Prader-Willi syndrome were diagnosed in the Unit of Endocrinology and Metabolic Diseases of Rabat Children Hospital. These cases were collected according to criteria's of diagnosis of the American Society of Human Genetics and the French association "AFPW". The mean age of diagnosis was 6 year-old. The circumstances of diagnosis were a behavior trouble in 1 case, a short stature in 1 case, a neonatal hypotonia 1 case and a suspicion of Cushing syndrome in 2 cases. In 4 patients the diagnosis was made according to the criteria of the AFPW, and by molecular biology in 1 case. 4 children had a facial dysmorphism and a psycho-motor retardation. The treatment was based on dietary management, and the treatment of other hormonal deficiencies. Conclusions: only an early diagnosis and a multidisciplinary management can improve the prognosis of this syndrome

Infant cancers: epidemiological study of 90 cases

The cancer of infant is a rare event. The differences of infant cancer types lead to a different approach of diagnosis and treatment. This study is a retrospective analysis of all cases of cancer in infants less than 1 year-old in the Paediatric Oncology Unit of Rabat Children Hospital. From January 1992 to December 2002, 90 infants were followed-up for cancer. Neuroblastoma was the most frequent tumour [34%], followed by nephroblastoma [15%], leukaemia [13%], sarcoma [11%] and retinoblastoma [8%]. Germinal cell tumours were found in 6 cases and hepatoblastoma in 3 cases. Treatment consisted in chemotherapy in 73 cases. Surgery alone was done in 7 cases and radiotherapy in 5. The event free survival rate at 130 months was 47.2% +/- 9% and the overall survival 66.8% +/- 7%. In conclusion, the distribution of cancer in the young infant is different of the older child. The diagnostic and therapeutic approach must take these characteristics into account

Autoimmune haemolytic anemia and Hodgkin disease: a 2 cases report

Hodgkin's disease can be associated to autoimmune disorders including autoimmune hemolytic anemia. This association is rare. We report a Hodgkin's disease revealed by haemolytic anaemia in two children. Case 1: a 13 year-old boy presented acute hemolysis one month before with abdominal lymph nodes. The Coombs test and IgG were positive. In spite of corticosteroid therapy during two months, the hemolysis has persisted. Abdominal lymph nodes biopsy was achieved. This biopsy revealed a Hodgkin's disease. The chemotherapy has permitted a complete remission of the Hodgkin's disease and the cure of hemolysis. The follow-up is 27 months. Case 2: a 11 year-old girl presented an hemolytic anemia two months before. She had an abdominal mass and a splenomegaly. The Coombs test and IgG were positives. The abdominal mass biopsy diagnosed a Hodgkin's disease. The chemotherapy cured the hemolysis. The Hodgkin's disease was in complete remission after a follow-up of 26 months. This patient developed a vitiligo in the beginning of the treatment. Hodgkin's disease is a possible diagnosis when auto-immune hemolytic anemia is associated with lymphoproliferative syndrome, and only chemotherapy can stop the hemolysis