Immunoglobulin-like receptors (IgLR), which have immunoglobulin (Ig) -like domains in their extracellular portions, are of two types : Activating type receptors which activate cellular signaling, and inhibitory type receptors which repress cellular activation. Analysis of mice lacking Fc receptors (FcRs), one of the IgLRs, has revealed their essential roles in the regulation of various immune responses. Recent reports suggest that novel IgLR family receptors as well as FcRs are involved in the development of various human diseases. This review provides an overview of IgLR-mediated immune regulation, highlighting the implication of FcRs in the development of autoimmune disease.

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Chronic granulomatous disease (CGD) is an inherited immunodeficiency characterized by severe recurrent bacterial and fungal infections and by a granuloma formation. Phagocyte NADPH oxidase (NOX) which is deficit in CGD, consists of gp91-, p22-, p47-, p67-, p40-phox (phagocyte oxidase), and Rac p21. Recently, based on homology of the gp91-phox oxidase domain structure, novel NOX proteins (NOX 1-5and Duox1/2) have been identified. Studies of these physiological functions and tissue localizations are in progress. Clinically the prognosis of CGD patients is expected to be ameliorated due to a development of novel anti-fungal drugs and prophylactic administration of inter-feron-gamma. Bone marrow transplantation (BMT) has been applied in 18 cases and also is expected to be done under non-myeloablative conditions. Although the medical treatment has progressed, standard precaution for the bacterial and fungal infections has not been proposed yet in Japan. We are going to prescribe a manual for patients' quality of lives to prevent life-threatening infections.

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The patient was born by vacuum delivery at 35 weeks and 3 days' gestation with a birth weight of 2, 750 g. He was transferred to our NICU suffering from severe respiratory distress and cyanosis with marked hydrops. On ad-mission, he had anemia, thrombocytopenia and liver dysfunction and subsequently was treated with antibiotics, γ-globulin and aciclovir, and the treatment for heart failure and disseminated intravascular coagulation (DIC) was also initiated. However, the symptoms were not improved. On day 11, serum ferritin, serum sIL-2R, serum IL-6 and urinary β₂-MG were markedly elevated. Bone marrow examination revealed hemophagocytic findings. Therefore, we diagnosed hydrops fetalis associated with hemophagocytic syndrome and started to treat with prednisolone and high-dose y-globulin. But, the liver dysfunction was not declined and heart failure and respiratory distress progressed. He died on day 22. It seems that the pathogenesis of hydrops in this case was related to hemophagocytic syndrome with hypercytokinemia.

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We report a case with congenital thrombotic thrombocytopenic purpura (cTTP) who was followed up for more than 10 years before being diagnosed at the age of 14. Recurrent hemolytic and thrombocytopenic attacks occurred since her neonatal period, and deteriorated with age and worsened in winter. She also suffered from chronic headache and abdominal pain since her pubertal period. Both thrombocytopenic attacks and chronic complaints have been dramatically improved by FFP infusion. Activity of von Willebrand factor-cleaving protease (VWF-CP) and inhibitor to VWF-CP in her plasma were both under detectable levels. DNA analysis revealed that the patient is a compound heterozygous for ADAMTS13 mutation, the gene responsible for VWF-CP. Based on these findings, the patient was diagnosed as cTTP (Upshaw-Schulman syndrome, USS). This is one of the rare USS cases with a chronic relapsing course. Long-term sequelae of these patients is unknown. The recent advances in diagnostic procedure will certainly allow us to identify more USS patients including those with atypical clinical courses. We report the characteristic features of this patient found by the long-term follow-up and discuss the latest findings of USS.

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1. Activities of the QOL committee in Japan Association Childhood Leukemia Study (JACLS) : We are preparing “The Handbook for a Family, ” which deals with a concise explanation of leukemia, and “Daily Records, ” which enables the family to record treatment course. We conduct an assessment of the QOL during treatment of children with leukemia, and survey various factors which influence QOL during leukemia treatment. We provide information on the JACLS group by opening a homepage. 2. In order to create long-term follow-up system : We propose the patient's regime “For the Long-term Follow-up” in order to inform a new attending doctor what therapy has been done for the patient. 3. Assessment of late effects : We are planning the prospective assessment of late effects by two-step investigations : a screening and some detail examinations between 5 years and 20 years after the diagnosis with the in-formed consent of the patient and family.

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Because of improvements in the treatment of childhood cancer in the past several decades, the survival rate of patients is between 60 and 90%. With the increase of long-term survivors, the late effects have become a new problem. We have found that it is difficult to grasp the state of the physical and psychological problem, because of the construction of the regular long-term follow-up system off-treatment. As the result of questionnaires, we understood the patient's and family's anxiety about late effects and the necessity for construction of long-term follow-up systems as soon as possible.

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[in Japanese]

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A long-term follow-up clinic for survivors of childhood cancer was started in 1998 in order to give care for late effects and psycho-social problems. The patients are seen at the department of general medicine rather than at the department of pediatrics. They are seen mainly by a pediatric oncologist in close collaboration with a social worker. Half of them are suffering from late effects and are referred to adult-care providers if necessary. The long-term follow-up period should be recognized by patients, family and pediatric oncologists as a transition from pediatric to adult-oriented health care. However the pediatric oncologist who knows the detailed clinical history of the patients continues play a vital advisory role after the transition to adult care.

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