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Severe primary immunodeficiencies (PID) are rare; their global incidence is comparable to that of childhood leukemia; they include more than 100 different entities. Clinical manifestations are: unusually severe or frequent infections or infections that do not respond to adequate treatment; an increased risk of certain malignancies; sometimes auto-immune manifestations. Delayed diagnosis and management of PID can lead to severe and irreversible complications or to death. PID can become manifest only in the adult; in common variable immune deficiency, the median age at diagnosis is between the 2nd and the 3rd decade of life. PID are often transmitted genetically; recent progresses in molecular biology have allowed more precise and earlier, including antenatal, diagnosis. Molecular treatment of 3 infants with a severe immunodeficiency has recently been achieved in April 2000. Those progresses were mostly based on the study of immunodeficiency databases. We present here the work of a Belgian group specialized in PID; meetings have started in June 1997. This group establishes guidelines for the diagnosis and treatment of PID, adapted to the local situation. The elaboration of a national register of PID is also underway; this has to provide all guaranties of anonymity to patients and families. Such a register already exists at the European level; it has provided the basis for new diagnostic and therapeutic possibilities. The inclusion of Belgian data in this register should allow essential progresses essential for our patients.

Between 1966 and 1986, 14 patients with histologically verified cerebellar medulloblastomas were seen at J. Bordet Institute after total (4 cases) or partial (10 cases) tumor removal. Twelve received postoperative irradiation after some courses of chemotherapy (Vincristine, Procarbazine, Methotrexate) for 5. In 10 cases the X ray treatment was given to the whole cerebrospinal axis with approximately 50 Gy delivered to the primary tumor site, 25-35 Gy to the remaining brain and spinal cord. As in other series of the literature the long term prognosis is not too good, with approximately 50% of the patients surviving more than 5 years. Failure at the primary site happened in 4 cases. As a consequence of the small number of patients treated, no conclusion can be drawn from risk factor analysis. Yet it appears that younger patients fared better than older ones. A detailed description of the side effects of the treatment is therefore given for the five children treated at prepubertal age: some growth impairment seems to be the rule, a consequence of GH deficiency--if not compensated--and of the direct effect of ionizing radiation on the vertebral bodies. While discussing the progress of neurosurgery and radiotherapy for the treatment of medulloblastoma, the authors emphasize the need of more efficient adjuvant chemotherapy regimens and the interest of multicenter trial for evaluating them.