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Volume 218 (2009)
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Volume 218, Issue 1
May
Displaying 1-12 of 12 articles from this issue
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Regular Contributions
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Yasuyo Kashiwagi, Hisashi Kawashima, Naoki Kato, Kouji Takekuma, Akino ...2009 Volume 218 Issue 1 Pages 1-3
Published: 2009
Released on J-STAGE: April 25, 2009
JOURNAL FREE ACCESSSevere combined immunodeficiency (SCID) is an inherited disease with profoundly defective T cells, B cells, and NK cells. X-linked severe combined immunodeficiency (X-SCID) is its most common form. In this report, we describe a 4-month old male with X-SCID who also showed opisthotonic posturing. Opisthotonus represents abnormal motor posturing and is defined as the posturing, in which the neck and back are arched posteriorly. The patient was referred to our hospital with liver dysfunction, respiratory distress, anal abscess, poor feeding and wasting; the patient appeared to suffer from severe and persistent infections. In fact, circulating T cells were not detectable, despite that the number of B cells was maintained in the normal ranges. Diagnosis of X-SCID was established by DNA analysis of the interleukin (IL)-2 receptor γ chain gene; namely, we detected the novel mutation within exon 2 (221 C→A), which leads to the substitution of tyrosine codon for stop codon (Y69stop). Computed tomography of the brain revealed mild atrophy, but no hemorrhage and no malformation. There were no pathological findings in the cerebrospinal fluid. Thus, the cause of opisthotonic posturing remains unknown. The patient died due to severe infection at the age of 7 months. It remains to be investigated to clarify the relationship between the mutation and clinical manifestations. In conclusion, we have identified the novel mutation in the IL-2 receptor γ chain gene, which is associated with X-SCID. Furthermore, this is the first report that describes the patient with X-SCID accompanying opisthotonus.View full abstractDownload PDF (318K) -
Yong Man Kim, Min Hyung Jung, Dae-Yeon Kim, Jong Hyeok Kim, Young Tak ...2009 Volume 218 Issue 1 Pages 5-9
Published: 2009
Released on J-STAGE: April 25, 2009
JOURNAL FREE ACCESSPrimary fallopian tube cancer (PFTC) is a rare tumor (< 1% of all female genital tract cancers) and remains poorly characterized. Histologically and clinically, PFTC resembles epithelial ovarian cancer (EOC). The similarities in pathological features and patterns of spread between EOC and PFTC have led clinicians to treat these two malignancies in a similar manner. However, these malignancies may be biologically distinct and may follow different clinical courses. Lymphadenectomy is of crucial importance in patients with EOC, because it improves disease-free survival even in advanced stages. The lymph node metastasis is relatively frequent even in early-stage PFTC. We therefore hypothesized that lymphadenectomy could improve the prognosis in PFTC, as observed in EOC. The purpose of this study is to evaluate the prognostic role of lymphadenectomy in PFTC with a special focus on advanced-stage PFTC. The medical records of 18 patients treated in a single institute from April 1997 to August 2004 were reviewed retrospectively. For advanced stages (III and IV, 10 patients), mean overall survival for patients with positive lymph node involvement is 62.00 ± 4.00 months, while the patients with negative lymph node involvement were alive at last follow-up performed in July 2008 (log-rank; P < 0.05). Furthermore, the mean disease-free survival for patients with positive lymph node involvement is 46.67 ± 5.14 months. Based on our analysis, lymphadenectomy could improve overall survival and disease-free survival in patients with advanced-stage PFTC.View full abstractDownload PDF (277K) -
Yukihiko Kawasaki, Kazuhide Suyama, Yusaku Abe, Yumiko Ushijima, Nobuk ...2009 Volume 218 Issue 1 Pages 11-16
Published: 2009
Released on J-STAGE: April 25, 2009
JOURNAL FREE ACCESSPrimary immunoglobulin A (IgA) nephropathy is characterized by microhematuria and proteinuria and by the deposition of IgA in the glomerular mesangium. Steroid was a main drug for treatment of IgA nephropathy. However, some of children with IgA nephropathy are resistance to steroid treatment, but the therapy for steroid-resistant IgA nephropathy was not established. There have been reports on the efficacy of tonsillectomy as an initial treatment for IgA nephropathy in adults and children. We examined whether tonsillectomy with methylprednisolone pulse therapy (tonsillectomy pulse therapy) was effective as rescue treatment for steroid-resistant pediatric IgA nephropathy. We studied 11 patients (age at onset and duration of follow-up, 11.7 ± 2.0 and 6.2 ± 1.1 years) who had been diagnosed with steroid-resistant IgA nephropathy. Clinical features, laboratory data, and pathological findings were retrospectively compared between before and after tonsillectomy pulse therapy. Urinary protein excretion was significantly decreased at 24.7 ± 7.3 months after tonsillectomy pulse therapy. On renal pathologic examination of 6 patients who underwent renal biopsy at 17.1 ± 6.9 months after tonsillectomy pulse therapy, the activity index, an index of inflammation, was lower compared to the index evaluated before the therapy, but the chronic index, an index of renal sclerosis, remained unchanged. At 24.7 ± 7.3 months after tonsillectomy pulse therapy, seven patients had normal urine and four had minor urinary abnormalities; namely, none had active renal disease or renal insufficiency. Our findings suggest that tonsillectomy pulse therapy may be effective as rescue treatment for steroid-resistant IgA nephropathy in childhood.View full abstractDownload PDF (940K) -
Terry Ting-Yu Chiou, Po-Hui Chiang, Morgan Fuh, Rue-Tsuan Liu, Wei-Chi ...2009 Volume 218 Issue 1 Pages 17-24
Published: 2009
Released on J-STAGE: April 25, 2009
JOURNAL FREE ACCESSPrimary aldosteronism is an important cause of secondary hypertension, because it is potentially curable, especially in case of unilateral aldosterone-producing adrenal adenoma (APA). However, the information is limited concerning the cardiovascular and renal outcomes in this patient population. We studied 52 patients with APA in order to determine the pre-operative and post-operative factors predicting cardiovascular and renal outcomes. All 52 patients were hypertensive before the operation. Among 35 patients who underwent pre-operative electrocardiogram, 23 patients had left ventricular hypertrophy (LVH). Patients with LVH had lower estimated glomerular filtration rate (eGFR). Adrenalectomy successfully normalized or improved hypertension, hypokalemia, and aldosterone excess. One month after the adrenalectomy, 32 patients (62%) became normotensive, but 20 patients (38%) remained hypertensive. However, after an average follow-up period of 51 months, only 18 patients remained normotensive, while 34 patients were hypertensive. Thus, the rate of recurrent hypertension after adrenalectomy was high (14/32, 43%). Pre-operative systolic blood pressure (BP), diastolic BP, and post-operative plasma aldosterone concentrations were the only variables significantly different between the hypertensive and normotensive patients. Using pre-operative BP 165/110 mmHg as a cutoff has good positive predictive values (73-92%) for post-operative long-term hypertension. Patients whose renal function worsened after adrenalectomy had significantly higher pre-operative plasma active renin levels. Thus, in patients with APA, the presence of LVH is correlated with impaired renal function (lower eGFR). In conclusion, pre-operative BP and post-operative plasma aldosterone are important in predicting post-adrenalectomy hypertension, and a lower pre-operative plasma renin predicts the improvement in renal function after adrenalectomy.View full abstractDownload PDF (399K) -
Feng Xu, Yaning Sun, Yuguo Chen, Yi Sun, Ruijian Li, Chunxi Liu, Cheng ...2009 Volume 218 Issue 1 Pages 25-33
Published: 2009
Released on J-STAGE: April 25, 2009
JOURNAL FREE ACCESSIschemic manifestations of atherosclerosis are mainly due to thrombus formation upon superficially eroded (denudation of luminal endothelium) plaques or deeply ruptured (fibrous cap rupture) plaques. Eroded plaques, atherosclerotic plaques without rupture, are found common in young patients of sudden death with coronary thrombosis. Our study aimed to investigate the role of endothelial cell (EC) apoptosis in eroded plaque with thrombosis using an atherosclerotic rabbit model. Atherosclerotic plaques were established by post-balloon-injury high-cholesterol feeding in 33 rabbits. After three-month feeding, a 2-cm segment of plaque-rich abdominal aorta for each animal was clamped in vivo and filled with staurosporine, which induces endothelial apoptosis, or saline for 20 minutes. Three days later, serum lipids and high sensitive C-reactive protein (hs-CRP), a valuable inflammatory parameter, were quantified, and abdominal aorta angiography was conducted. In addition, immunohistochemistry staining was performed for all processed aortae. In the staurosporine-treated aorta, endothelium integrity of plaques was disrupted partially or in large areas, but fibrous cap rupture was not observed, the findings of which were similar to eroded plaques detected in human subjects. As compared to saline controls, staurosporine-treated rabbits showed higher apoptosis scores and thrombotic scores, and more angiographic overt thrombosis and histological thrombosis (P < 0.01, respectively), despite the similar serum levels of lipids and hs-CRP. We further confirmed that apoptosis score was linearly associated with thrombotic score. These results suggest that endothelial apoptosis may be an independent risk factor for thrombosis. In conclusion, the increase in endothelial apoptosis is involved in the formation of thrombotic eroded plaques.View full abstractDownload PDF (2018K) -
Hitoaki Sato, Masaaki Ueki, Takehiko Asaga, Kousuke Chujo, Nobuhiro Ma ...2009 Volume 218 Issue 1 Pages 35-40
Published: 2009
Released on J-STAGE: April 25, 2009
JOURNAL FREE ACCESSThe ischemia/reperfusion (I/R) represents a common pathological mechanism that causes renal injuries. A monosaccharide D-allose has been shown to inhibit neutrophil activation, which is involved in the I/R-induced organ injuries. We therefore examined the role of D-ribose in the I/R-induced renal injury using a rat model. D-ribose, a monosaccharide found in all living cells, serves as a key component of adenosine-5'-triphosphate and nicotinamide adenine dinucleotide. Male Wistar rats were divided into the sham, control and D-ribose groups. In the control and D-ribose groups, rats were subjected to 45 min of left renal ischemia, followed by 24 h of reperfusion, while the I/R procedure was not performed in the sham group. Rats were intravenously administered D-ribose (sham group and D-ribose group, 400 mg/kg) or saline (control group) 30 min before ischemia. Blood urea nitrogen (BUN), serum creatinine and urinary N-acetyl β-D-glucosaminidase (NAG) were measured as indicators of glomerular function and proximal tubular function. We also measured cytokine-induced neutrophil chemoattractant-1 (CINC-1) and myeloperoxidase concentrations to assess neutrophil activation and infiltration, respectively. The tissue sections were scored to evaluate the tubular injury. In the control group, BUN, creatinine, NAG, CINC-1, myeloperoxidase, histological severity score, and number of infiltrating neutrophils were increased following I/R insult, as compared with the sham group. Such increases in biochemical markers, severity score, and infiltrating neutrophils were significantly inhibited in the D-ribose group. Thus, D-ribose ameliorates the I/R-induced renal injury probably by inhibiting neutrophil activation, and may be useful in attenuating the renal injury associated with renal ischemia.View full abstractDownload PDF (745K) -
Isao Uemaetomari, Keiji Tabuchi, Mariko Nakamagoe, Syuho Tanaka, Hidek ...2009 Volume 218 Issue 1 Pages 41-47
Published: 2009
Released on J-STAGE: April 25, 2009
JOURNAL FREE ACCESSExcessive calcium entry into cells leads to cell death, and voltage-gated calcium channels (VGCCs) are responsible for the calcium entry in the central nervous system. VGCC blockers inhibit excessive calcium entry and protect the central nervous system against various types of injury. The purpose of the present study was to identify the type of calcium channels that is responsible for acoustic injury of the cochlea. The effects of L- and T-type VGCC blockers on acoustic injury were examined. Female ddY mice, at 8 weeks of age, were used in this study. The animals were subjected to a 4-kHz pure tone of 128-dB sound pressure level (SPL) for 4 hours through an open field system inside a sound-exposure box. A L-type or T-type VGCC blocker was administered immediately before acoustic overexposure. The hearing ability was evaluated using the auditory brainstem response (ABR). ABR is an electrical signal evoked from the brainstem by the sound. After the final ABR measurement at two weeks after acoustic overexposure, cell nuclei in the organ of Corti were stained with propidium iodide, and hair cell loss was calculated in a region 3.66 mm from the apex. Each of four L-type VGCC blockers tested, i.e. diltiazem, verapamil, nicardipine and nimodipine, significantly improved shifts of the ABR threshold from the pre-exposure levels. In addition, each L-type VGCC blocker consistently decreased hair cell loss, but not a given T-type calcium blocker. The present findings suggest that the L-type VGCC is involved in the pathogenesis of acoustic injury in the cochlea.View full abstractDownload PDF (376K) -
Yukihiko Kawasaki, Kazuhide Suyama, Hayato Go, Takashi Imamura, Yumiko ...2009 Volume 218 Issue 1 Pages 49-55
Published: 2009
Released on J-STAGE: April 25, 2009
JOURNAL FREE ACCESSMyeloid-related protein (MRP) 8 is a calcium-binding protein of the S100 family. The renal accumulation of macrophages expressing MRP8 is associated with the inflammatory activity of glomerulonephritis. We evaluated the renal accumulation of macrophages expressing MRP8 in children with IgA nephropathy (IgAN). We collected data on 25 IgAN children who had been treated with prednisolone and divided these patients into two groups: Favorable group, consisting of 11 patients with normal urine and 6 with minor urinary abnormalities at 4.3 ± 1.3 years after initial treatment; and Unfavorable group, consisting of 8 patients with persistent nephropathy. The pathological renal findings were compared between both groups. The second biopsy was performed at two years after first biopsy at 5.5 ± 4.9 months from onset. In Favorable group, the glomerular accumulation of macrophages expressing MRP8, and mesangial cells expressing α-smooth muscle actin (α-SMA) were lower in the second biopsy specimens than those of the first biopsy specimens. In Unfavorable group, the glomerular accumulation of macrophages expressing MRP8 detected in the second biopsy specimens was similar to that of the first biopsy, while the number of mesangial cells expressing α-SMA and the index of renal sclerosis were higher in the second biopsy than in the first biopsy. The indexes of renal sclerosis were higher in children with more macrophages expressing MRP8 than in children with less macrophages expressing MRP8. Our results suggest that renal macrophages expressing MRP8 may be involved in the progression of sclerotic changes in children with IgAN.View full abstractDownload PDF (1208K) -
Orhan Ozer, Vedat Davutoglu, Suleyman Ercan, Murat Akcay, Ibrahim Sari ...2009 Volume 218 Issue 1 Pages 57-62
Published: 2009
Released on J-STAGE: April 25, 2009
JOURNAL FREE ACCESSIn developing countries, rheumatic valve disease (RVD) is still prevelant. Management of RVD depends on symptomatology, physical examination and echocardiographic evaluation, all of which, however, might be inadequate. Reliable biomarkers to establish severity of RVD and predict complications would be highly beneficial. Urotensin II is regarded as a cardiovascular autacoid/hormone, and its role in cardiovascular diseases is emerging. We hypothesized urotensin II might have pathophysiological roles in RVD. We investidated 71 patients with RVD (mean age 40 ± 12 years, 17 female patients) and 25 normal subjects (mean age 40 ± 7 years, 8 female patients). We assessed their New York Heart Association (NYHA) functional class, RVD severity and pulmonary artery pressure (PAP), and measured plasma urotensin II levels. Mitral regurgitation (r = 0.226, p = 0.02), tricuspid regurgitation (r = 0.238, p = 0.02), PAP (r = 0.320, p = 0.01), and NYHA class (r = 0.213, p = 0.03) correlated positively with urotensin II levels. There was positive correlation between urotensin II levels and severity of mitral regurgitation (r = 0.248, p = 0.01) and tricuspid regurgitation (r = 0.326, p = 0.001). In linear regression analysis, only PAP was predictive of urotensin II (β = 0.3; p = 0.02). In conclusion, this is the first study showing that plasma urotensin II is elevated in chronic RVD, associated with severe mitral and tricuspid valve regurgitation. Furthermore, urotensin II level is correlated with NYHA functional class, and the increase in PAP is predictive of plasma urotensin II.View full abstractDownload PDF (291K) -
Chiharu Ito, Tetsu Akimoto, Takashi Ioka, Takahisa Kobayashi, Eiji Kus ...2009 Volume 218 Issue 1 Pages 63-71
Published: 2009
Released on J-STAGE: April 25, 2009
JOURNAL FREE ACCESSOrganogenesis accompanies the establishment of the vascular system which begins with sprouting angiogenesis. Vascular endothelial growth factor (VEGF) provides the primary stimulation in the vascular sprouting process but the negative regulation of this process remains unclear. This study examined the role of the transforming growth factor-β (TGF-β) superfamily in vascular sprouting using a three-dimensional dorsal aorta culture system, in which the dissected tissue was embedded in type I collagen gel. The cultures were maintained under hypoxic conditions to enhance the expression of Flk-1, a receptor for VEGF, thereby ensuring the responsibility to VEGF. Under the culture conditions employed, the dorsal aorta formed many cord-like structures in response to VEGF. To examine the role of TGF-β in vascular sprouting, each member of the TGF-β superfamily was applied to this culture system. TGF-β1, as well as TGF-β2 and TGF-β3, inhibited capillary formation. Likewise, activin A, another member of TGF-β superfamily, also abolished vascular sprouting, but bone morphogenetic protein 2 did not noticeably change the morphology. Both neutralizing anti-TGF-β1 antibody and TGF-β type I receptor (ALK5) inhibitor partially reversed the inhibitory effect of TGF-β1. Furthermore, down-regulation of ALK5 with small interfering RNA rather than activin receptor-like kinase-1 (ALK1) reversed the effect of TGF-β1. These data suggest that TGF-β superfamily may act as an inhibitor of vascular sprouting mainly through ALK5 signaling pathway. We propose that VEGF may antagonize the TGF-β autoregulatory action to initiate vascular sprouting.View full abstractDownload PDF (1374K) -
Shiho Terai, Katsunori Iijima, Kiyotaka Asanuma, Nobuyuki Ara, Kaname ...2009 Volume 218 Issue 1 Pages 73-79
Published: 2009
Released on J-STAGE: April 25, 2009
JOURNAL FREE ACCESSNitric oxide produced endogenously in vagal neurons modulates gastrointestinal motor activity as an important non-aderenergic and non-cholinergic neurotransmitter. Other than through endogenous biosynthesis, a high concentration of nitric oxide also occurs by chemical reactions within the stomach in the presence of gastric acid through the entero-salivary re-circulation of dietary nitrate. Although dietary nitrate can be a potential source of nitric oxide in the human stomach, there has been no report on the effect of dietary nitrate on gastric motor function. The aim of this study is to investigate the effect of dietary nitrate on gastric emptying, one of the major parameters for the gastric motor function. Fifteen healthy volunteers underwent a placebo-controlled (310 mg sodium nitrate or placebo), double-blind, crossover trial. Since a sufficient amount of gastric acid is essential for dietary nitrate-derived nitric oxide generation in the stomach, the same protocol was repeated after 1-week treatment with a proton pump inhibitor, rabeprazole. Gastric emptying was evaluated by 13C-octanoate breath test. The sodium nitrate ingestion did not affect gastric emptying either prior to or during rabeprazole treatment, although rabeprazole treatment itself significantly delayed gastric emptying, being independent of the dietary nitrate load. Confirmation of the delayed gastric emptying with rabeprazole indicates the sensitivity of the breath test employed in the present study. In conclusion, despite the potential nitrogen source of exogenous nitric oxide, the ingestion of 310 mg sodium nitrate, which is equivalent to the average daily intake of Japanese adults, does not affect gastric emptying in healthy volunteers.View full abstractDownload PDF (332K)
Letter to the Editor
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Masakuni Suzuki, Ikuo Tachibana, Takahiro Noda, Hideaki Imaizumi2009 Volume 218 Issue 1 Pages 81
Published: 2009
Released on J-STAGE: April 25, 2009
JOURNAL FREE ACCESSDownload PDF (153K)
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