Home-based subcutaneous immunoglobulin after switch from intravenous immunoglobulin improved quality of life in pediatric patient with common variable immunodeficiency: A case report

　Common variable immunodeficiency (CVID) is one of the primary immunodeficiency. Regular immunoglobulin G (IgG) replacement therapy is often performed for patients with CVID.　We experienced a patient who was hospitalized in our hospital for repeated pneumonia and diagnosed CVID at the age of 10 years. He had often been absent from school due to infectious diseases. We were administered intravenous IgG (IVIG) two times and his serum level of IgG became over 1,000 mg/dL. Afterward, he was affected the hand-foot-and-mouth disease one week after discharge. At that time, his IgG level decreased to 751 mg/dL. To maintain stable IgG trough levels, we introduced subcutaneous IgG (SCIG). Since then, his IgG levels remained around 1,000 mg/dL, he has lived without suffering from infectious diseases.　There are some reports that IVIG and SCIG were compared and SCIG was able to obtain a stable IgG trough levels to prevent infection. In addition, because our patient is a mother and child family, it was difficult to visit the outpatient department frequently, so it was desirable to infuse at home.　We experienced a patient who had a stable trough levels with SCIG and improved quality of life, so we report this case with literature reviews

Long-term survival with RAS-associated autoimmune leukoproliferative disorder with somatic KRAS mutation:A case report

　RAS -associated autoimmune leukoproliferative disorder (RALD) is a recently reported rare nonmalignant autoimmune disorder. The characteristic clinical findings of RALD include monocytosis, leukocytosis, lymphoproliferation, and autoimmune phenomena. RALD is defined by somatic mutations in KRAS or NRAS . It is a new disease that was reported by Niemela and Takagi in 2011. The prognosis and incidence are currently unknown and the treatment strategy has not yet been established.　Here we describe the long-term survival of a patient with who displayed a somatic KRAS G12D mutation. His clinical features and labolatory data were overlapped with juvenile myelomonocytic leukemia and chronic myelomonocytic leukemia. Mercaptopurine hydrate, hydroxycarbamide and azacitizine were administered to control white blood cell count and improve clinical symptoms. He had a long survival time without hematopoietic stem cell transplantation

Case reports of pregnancies complicated with kidney disease and their fetal prognosis

　The number of patients with chronic kidney disease (CKD) has been increasing every year, with a current prevalence of one in eight adults. Although the frequency of complications due to kidney disease before pregnancy is not high (0.02–0.12%), frequency of pregnancy with CKD, including cases receiving continuous dialysis therapy is expected to increase in the future. The fertility and birth rates among dialysis patients are low, and perinatal management in these patients is currently difficult. However, even under such circumstances, the probability of having a live-born baby in pregnant women on dialysis has increased due to improvements in dialysis technology, perinatal management, and neonatal care. There are some case reports written about them, and I think that it is possible to approach term delivery with careful care through the cases experienced this time.　In this study, we examined the pregnant patients, on dialysis or requiring postpartum dialysis, at Kawasaki Medical School Hospital between January 2005 and March 2018. Six patients (86%) had a live-born baby, while one had a miscarriage. One patient underwent two pregnancies on dialysis; one case gave a full-term birth, while the rest had a premature delivery. The modes of delivery were vaginal delivery (n = 1), elective cesarean section (n = 3), and emergency cesarean section (n = 2). Five patients delivered successfully and had a good prognosis, while in one case, the neonate died.　Over the years, owing to continuous improvement at our hospital, we have achieved better pregnancy prognosis and longer gestation periods in the patients. In particular, one case, which had a natural second pregnancy, 9 years after the beginning of dialysis, was worthy of note; we were able to manage her second pregnancy using the process followed during her first pregnancy as reference