Originals
Calcium-sensing receptor gene polymorphism affects the parathyroid response to moderate hypercalcemic suppression in patients with end-stage renal disease
K. Yokoyama, T. Shigematsu, T. Tsukada, S. Hara, A. Yamada, Y. Kawaguchi and T. Hosoya
Volume 57 (2002) p. 131 - 135
Abstract
K. Yokoyama, T. Shigematsu, T. Tsukada, S. Hara, A. Yamada, Y. Kawaguchi and T. Hosoya
1The Division of Nephrology and Hypertension, Jikei University School of Medicine, Tokyo, 2Nephrology and Dialysis Unit, Department of Internal Medicine, Sakura National Hospital, Chiba, 3Department of Clinical Physiology, Toranomon Hospital, Tokyo, 4Kidney Center, Toranomon Hospital, Tokyo, Japan
Aim: The basic mechanism of secondary hyperparathyroidism is still unclear, but a change in Ca2+ sensing by parathyroid cells is possibly involved in this uremic complication. A rightward shift of the calcium set-point and an increase of the minimum secretion rate have been found in secondary hyperparathyroidism, indicating abnormal calcium sensing. Methods: We evaluated the effect of calcium sensing receptor (CaR) gene polymorphism (codon G990R) on the response of the parathyroid gland to moderate hypercalcemic suppression in 77 ESRD patients on regular hemodialysis (HD using 2.5 mEq/l Ca2+ dialysate). All patients underwent an HD session with 3.0 mEq/l Ca2+ dialysate to suppress parathyroid hormone (PTH). Then we investigated the effect of CaR gene polymorphism on the parathyroid response to hypercalcemic stimulation. Results: Patients were divided into 3 groups on the basis of genotype (GG = 33 patients (42.9%), GR = 39 patients (50.6%), RR = 5 patients (6.5%)). Baseline intact PTH levels in patients without the R allele were not significantly different from those in patients with the R allele (GG group, 181.4 ± 31.1 pg/ml vs. GR and RR groups, 230 ± 51.2 pg/ml: mean ± SEM). The significant effect of moderate hypercalcemic suppression on the intact PTH level was observed in the GG group (p < 0.01) but not in the GR and RR groups, despite the identical increase in Ca2+. Conclusion: Our results suggest that CaR gene polymorphism (codon G990R) influences the responsiveness of the parathyroid gland to changes of extracellular Ca2+ in ESRD patients. The glands of patients with the GG genotype of the CaR gene may be more sensitive to extracellular Ca2+ changes.
Case Reports
A pediatric occurrence of crescentic glomerulonephritis associated with antineutrophil cytoplasmic antibodies and mesangial IgA deposits
R. Jimbo, Y. Ubara, T. Tagami, Y. Higa, T. Suwabe, S. Nakanishi, Y. Sogawa, K. Nomura, H. Kadoguchi, J. Hoshino, N. Sawa, H. Katori, F. Takemoto, S. Hara, S. Hara, K. Ohashi and K. Takaichi
Volume 68 (2007) p. 104 - 108
Abstract
R. Jimbo, Y. Ubara, T. Tagami, Y. Higa, T. Suwabe, S. Nakanishi, Y. Sogawa, K. Nomura, H. Kadoguchi, J. Hoshino, N. Sawa, H. Katori, F. Takemoto, S. Hara, S. Hara, K. Ohashi and K. Takaichi
1Nephrology Center, 2Health Care Center, 3Department of Pathology, Toranomon Hospital, Tokyo, Japan
Antineutrophil cytoplasmic antibody- (ANCA) associated glomerulonephritis usually shows histopathologic features of pauciimmune crescentic glomerulonephritis and occurs late in life. We report a 14-year-old Japanese girl presenting with proteinuria, hematuria and mildly elevated serum creatinine. A renal biopsy specimen demonstrated crescentic glomerulonephritis, immunofluorescence showed mesangial IgA staining. Electron microscopic examination disclosed paramesangial deposits. Serum ANCA against myeloperoxidase(MPO) were detected at high titers. Myeloperoxidase-ANCA-related nephritis accompanied by IgA nephropathy is considered rare in childhood and teen years. Yet, if ANCA assays and detailed electron microscopic examination of renal specimens were performed routinely in patients with rapidly progressive glomerulonephritis, the diagnosis might be more frequent in young patients.Correspondence to:
Dr. Y. Ubara
Nephrology Center
Toranomon Hospital Kajigaya
1-3-1 Kajigaya, Takatsu
Kawasaki, 213-0015, Japan
Email: [email protected]
Case Reports
Multicentric Castleman disease with secondary AA renal amyloidosis, nephrotic syndrome and chronic renal failure, remission after high-dose melphalan and autologous stem cell transplantation
M. Ogita, J. Hoshino, Y. Sogawa, N. Sawa, H. Katori, F. Takemoto, Y. Ubara, S. Hara, S. Miyakoshi and K. Takaichi
Volume 68 (2007) p. 171 - 176
Abstract
M. Ogita, J. Hoshino, Y. Sogawa, N. Sawa, H. Katori, F. Takemoto, Y. Ubara, S. Hara, S. Miyakoshi and K. Takaichi
1Kidney Center, 2Department of Hematology, Toranomon Hospital, Tokyo, Japan
Multicentric Castleman disease is a systemic lymphoproliferative disease with incomplete understood etiology. The various renal complications of this disease may include minimal change disease, mesangial proliferative glomerulonephritis, membranous glomerulonephritis and nephrotic syndrome, caused by secondary amyloidosis. In several reported cases of localized Castleman disease associated with renal amyloidosis and nephrotic syndrome, resection of organs involved by lymphoid proliferation resulted in complete remission. However, therapy of multicentric Castleman disease with renal amyloidosis is not well-established. We treated a case of a 39-year-old woman with multicentric Castleman disease complicated by nephrotic syndrome caused by secondary AA amyloidosis. The patient underwent autologous peripheral blood stem cell transplantation (auto-PBSCT), achieving complete remission. Autologous stem cell transplantation may be an attractive choice in therapy for refractory multicentric Castleman disease.Correspondence to:
M. Ogita, MD
Kidney Center, Toranomon Hospital, 2-2-2 toranomon, Minato-ku, Tokyo, Japan, 105-8470
Email: [email protected]
Case report
A patient with pregnancy-related acute abdomen after hemodialysis for over 18 years
T. Saito, Y. Ubara, T. Suwabe, Y. Higa, Y. Higa, S. Nakanishi, J. Hoshino, N. Sawa, H. Katori, F. Takemoto, R. Marui, M. Nakamura, S. Tomikawa, S. Hara, H. Tohbai and K. Takaichi
Volume 71 (2009) p. 345 - 349
Abstract
T. Saito1, Y. Ubara1, T. Suwabe1, Y. Higa1, Y. Higa1, S. Nakanishi, J. Hoshino1, N. Sawa1, H. Katori1, F. Takemoto1, R. Marui1, M. Nakamura1, S. Tomikawa1, S. Hara1, H. Tohbai2 and K. Takaichi1
1Nephrology Center and Department of Obstetrics and 2Gynecology, Toranomon Hospital, Tokyo, Japan
Because pregnancy is rare in women with end-stage renal disease, dialysis patients have not been reported to present with acute abdominal symptoms related to pregnancy including ectopic pregnancy. A 41-year-old woman treated with hemodialysis for over 18 years was brought to the emergency room at our institution because of acute abdominal pain. Ultrasonography detected an abdominal fluid collection, and her anemia had worsened (hematocrit 18%). Emergency laparoscopic exploration disclosed a hemorrhagic corpus luteum of pregnancy, causing ovarian bleeding on the left. Coagulation of bleeding points was carried out. At this time, pregnancy at 7 weeks of gestation was discovered. After the procedures, hemodialysis frequency was increased to 5 times weekly, and an erythropoietin derivative was administered to maintain a hematocrit above 30%. The patient developed no hypertension. At 33 weeks of gestation, cesarean section was performed because of a decrease in amniotic fluid and frequent late deceleration of the fetal heart rate. A live baby girl weighing 1,422 g was born. The successful pregnancy reflects remarkable progress in dialysis technology. Pregnancy, then, can underlie an acute abdomen in childbearing-age women (14 – 44 years old) undergoing long-term dialysis.Correspondence to:
Y. Ubara, MD, PhD; Nephrology Center, Toranomon Hospital Kajigaya, 1-3-1, Kajigaya, Takatsu, Kawasaki, Kanagawa 213-0015, Japan
Email: [email protected]
Nephrology Education
A case of familial lecithin-cholesterol acyltransferase deficiency on hemodialysis for over 20 years
Y. Tsuchiya, Y. Ubara, R. Hiramatsu, T. Suwabe, J. Hoshino, K. Sumida, E. Hasegawa, M. Yamanouchi, N. Hayami, Y. Marui, N. Sawa, S. Hara, K. Takaichi and K. Oohashi
Volume 76 (2011) p. 492 - 498
Abstract
Clinical Nephrology, Vol. 76 – No. 6/2011 (492-498)
A case of familial lecithin-cholesterol acyltransferase deficiency on hemodialysis for over 20 years
Y. Tsuchiya1, Y. Ubara1, R. Hiramatsu1, T. Suwabe1, J. Hoshino1, K. Sumida1, E. Hasegawa1, M. Yamanouchi1, N. Hayami1, Y. Marui1, N. Sawa1, S. Hara1, K. Takaichi1 and K. Oohashi2
1Nephrology Center and 2Department of Pathology, Toranomon Hospital Kajigaya, Takatsu, Kanagawa, Japan
We trace the 34-year history of a member of the first Japanese family in which lecithin-cholesterol acyltransferase (LCAT) deficiency was diagnosed. Marriage between cousins with low LCAT activity was responsible for familial LCAT deficiency (FLD). In 1976, a 27-year-old Japanese man was noted to have FLD based on proteinuria, hematuria, grayish corneal opacity and low LCAT activity (9.83%). Genetic analysis showed insertion of G-G-C coding glycine at codon 141. Total cholesterol (C) was low at 108 mg/dl and the ratio of C-ester to total C was very low (12%), while the lecithin (phosphatidylcholine) level was very high (97.3%). When his serum creatinine reached 2.6 mg/dl at the age of 41 years (in 1991), renal biopsy was performed. This showed expansion of the mesangial matrix and irregularly thickened capillary walls with a bubble-like appearance because of lipid deposits consisting of two components (partly lucent vacuolated areas and partly deeply osmiophilic areas). Magnification of the latter deposits showed curvilinear and serpiginous striated membranous structure. Hemodialysis was started in 1990 and has been continued for over 20 years until August 2010. Clinical problems have included AV shunt failure requiring 4 operations and 13 percutaneous transcatheter angioplasty procedures, as well as episodes of hemolytic anemia that subsided after infusion of fresh frozen plasma. Cardiovascular events have not yet occurred, although severe calcification of abdominal aorta has been detected by computed tomography.Correspondence to:
Y. Tsuchiya, MD
Nephrology Center
Toranomon Hospital Kajigaya
1-3-1, Kajigaya, Takatsu
Kawasaki, Kanagawa, 213-8587, Japan
Email: [email protected]
