Vitamin K deficiency during the early neonatal period has been a major hemorrhagic disorder of thenewborn for a long time. On the other hand, vitamin K deficiency occurring in infants over 2 weeks of age was first reported in the second half of the 1970's. Infantile vitamin K deficiency frequently occurred in breast-fed infants, and attracted public attention as a negative factor of breast feeding because it induced intracranial hemorrhage in many cases, and resulted in death or sequelae in more than half of the cases. Although the incidence of these diseases was sharply reduced by preventive vitamin K administration at birth, discharge from the maternity ward, and theone month of age health checkup, a considerable number of infants still develop vitamin K deficiency bleeding. In EU countries such as France and England, weekly or daily oral administration of a vitamin K preparation was introduced, and the disease has not developed in any infants to whom a vitamin K preparation is administered by these prophylactic regimens. Thus, in Japan, new guidelines (draft) have been proposed in order to eradicate the vitamin K deficiency in infancy.

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Pediatric Transfusion Medicine Conference conducted a survey of pediatric hematologists to obtain consensus for the Guideline on Transfusion Practice announced in September 2005. Most approved of the guideline and agreed on the proposed cutoff level for hemoglobin of 7 g/dl, for a platelet count of 10, 000-20, 000 / μl, and for FFP of an INR of 2.0 or PT of 30%, and APTT of twice the standard value or below 25%. They desired additional criteria in relation to the platelet parameters, with over half of them seeking more detailed criteria in relation to the disease and pathophysiology. They also desired fresh criteria in relation to albumin and immunoglobulin levels. We need prospective randomized trials to develop criteria for granulocyte transfusions and donor lymphocyte infusion. Other options, e.g., administration of erythropoietin, G-CSF, rFVIIa and tranexamic acid, are expected to replace blood transfusion and decrease the frequency of blood transfusion. We would like to organize our pediatric community to conduct welldesigned clinical studies for the purpose of developing evidence-based guidelines for transfusion.

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We investigated factors that affected final height and the response to a growth hormone (GH) provocation test in survivors of childhood acute lymphoblastic leukemia (ALL) who underwent cranial irradiation (CRI). Medical records of 51 patients (33 males, 18 females) who had undergone treatment for childhood ALL were retrospectively examined. Though the expected final height is based on parental height (target height), which is determined by genetic predisposition, we analyzed final height using the height standard deviation score (HSDS) and by calculating the ratio of final height to target height (RFT). HSDS values were within the normal distribution (between-2 and +2 standard deviations) in 46 of 51 patients. The RFT of 3 male and 4 female survivors (13.7%) was below 0.95, and that of 6 male and 8 female survivors (27.5%) was below 0.975. RFT was associated with age at diagnosis and cumulative doses of corticosteroid therapy but not associated with gender or dose of cranial irradiation. Eleven of 12 survivors with RFT < 0.975 showed a normal response to the GH provocation test, whereas 3 of 17 survivors with RFT≥1.0 showed a low response to GH provocation tests. Thus, there was no relationship between the results of the GH provocation test and final height.

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Congenital syphilis is a rare and serious disease that continues to be a major healthcare problem. Its clinical spectrum ranges from an asymptomatic infection to fulminating sepsis and death. Here, we report a case of a onemonth-old infant diagnosed with congenital syphilis. The initial symptoms of the disease mimicked those of acute leukemia, e.g., hepatosplenomegaly, high white blood cell count, anemia, and thrombopenia. With the changing social environment, the prevalence of sexually transmitted diseases, including syphilis, has increased. Therefore, provision of appropriate medical care services is recommended for the prevention, diagnosis, and treatment of congenital syphilis.

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Omenn Syndrome is a form of severe congenital combined immunodeficiency characterized by erythrodermia, hepatosplenomegaly, lymphadenopathy, and alopecia. Most patients with Omenn Syndrome are threatened due to severe infections; thus, they are generally treated with cord blood or bone marrow stem cell transplantation in early childhood. We here report a patient with Omenn Syndrome who suffered Mycobacterium avium complex (MAC) infection in the neck and axillary lymph nodes after cord blood stem cell transplantation. Thispatient was mainly treated with ethambutol, rifampicin, clarithromycin, and azithromycin, and the repeated incisions and drainages of lymph node lesions were performed for 2 years after the onset of MAC infection. These results suggested that administration of antituberculosis drugs and macrolide antibiotics, and surgical treatment are effective for systemic MAC infections after hematopoietic cell transplantation.

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