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BackgroundSchool urinary screening has been performed in Japan.MethodsIkeda City and Toyono Town introduced, in 2012 and 2013, urinary protein/creatinine (Cr) ratio measurement into the urine-screening protocols designed for students aged between 4 and 15 years. For each student whose urinary protein/Cr ratio was ≥ 0.15 g/gCr (positive case), an appointment was made with a specialist at Ikeda City Hospital. The results of these screening urinalyses conducted through 2018 are summarized.Results14,606 junior high and elementary school students aged between 6 and 15 years were included. On average, they underwent 4.16 screening tests. 77 positive cases were detected, and seven students were diagnosed with high-risk chronic kidney disease (CKD). Of these, four underwent renal biopsy, and two, one, and one were diagnosed with IgA nephropathy, MPGN, and FSGS, respectively. In three students, detection of CKD would have been difficult without urinary screening. Incident rates of high-risk CKD and IgA nephropathy are estimated as 11.5 and 3.3 cases/100,000 students/year. 78.0% of positive cases without high-risk CKD showed no urinary abnormality after one year. 2301 kindergarten students aged between 4 and 6 years received an average of 1.74 screening urinalyses; none was positive or high-risk CKD. The estimated cost of detecting one high-risk CKD student whose detection would have been difficult without this screening was 3,156,711 Japanese yen.ConclusionSchool urinary screening using the urinary protein/Cr ratio can efficiently refer to a specialist. It detects a few children with high-risk CKD early with spending high cost.

Objective: Sporadic nonautoimmune hyperthyroidism caused by germline mutations of the thyrotropin receptor (TSHR) gene is a very rare disease that manifests as severe congenital hyperthyroidism. We describe a case presenting with sporadic nonautoimmune hyperthyroidism during adolescence.Methods: Laboratory data, imaging, histopathology, clinical follow-up, and DNA sequencing were evaluated.Results: The patient was born at term with a normal birth weight but showed increased growth velocity at approximately 7 months. He had no medical consultation before tachycardia was detected at a school physical examination at the age of 13 years. Thyroid function tests confirmed hyperthyroidism with negative anti-TSHR antibodies. After maintaining methimazole therapy for 12 years, he underwent total thyroidectomy, and the weight of the resected thyroid tissue was 131 g. The subsequent histopathologic findings showed an encapsulated follicular adenoma, adenomatous goiter, and hyperplastic changes of the follicular epithelium without stromal lymphoid infiltration in normal parenchyma. Sequence analysis of the TSHR gene showed two compound heterozygous germline variants (R310H and D619G) on different alleles. The TSHRR310H was a nonfunctioning variant from the maternal allele. The TSHR-D619G was previously detected as a somatic mutation in patients with toxic adenomas. The TSHR-D619G mutation, which was absent in his parents, was a de novo mutation on the paternal allele.Conclusion: A TSHR germline mutation (D619G) was identified as the cause of sporadic nonautoimmune hyperthyroidism in a Japanese patient. This patient showed no obvious complications until the age of 13 years, but long-term follow-up with methimazole treatment showed further goiter and benign nodular formation.Abbreviations: MMI methimazole; TRAb thyrotropin-receptor antibody; TSHR thyrotropin receptor

Using 415 residual blood samples subjected to (1→3) -β-D-glucan assay, we studied the correlation of measurement results between Fungitec G Test MKII "Nissui" (Nissui Pharmaceutical Co., Ltd., Tokyo) and its predecessor, Fungitec G Test MK (Seikagaku Corporation, Tokyo), which is now out of production. Their major difference is that MK II uses reagents derived from blood cells of Limulus polyphemus, the American horseshoe crab, whereas MK used those of Tachypleus tridentatus, an Asian horseshoe crab. Passing-Bablok analysis showed a linear regression with nearly one-to-one correspondence (slope=1.065, intercept=-0.287) between the two test kits over the regular range of measurements (4.0 pg/ml -500 pg/ml ). This was also true when data were subdivided and analyzed in the low to medium (≦150 pg/ml ) and in the low range (≦50 pg/ml ). There were several cases, however, that showed a wide discrepancy between the pair of measurements. This discrepancy is believed to be due in part to the difference between Limulus and Tachypleus in their reactivity to β-glucans with diverse side chains. Despite of this, the even distribution on either side of the regression line of those samples that are associated with deep fungal infection and the abrupt disappearance of such samples below 20 pg/ml attest that MK II "Nissui" is an acceptable successor of MK.

Few reports on malignant transformation of adenomyosis are available, and endometrioid adenocarcinoma arising from cystic adenomyosis is further rarely reported. We report a case of a 67-year-old asymptomatic woman who was referred to our hospital for evaluation of a cystic lesion in the pelvis, which had been diagnosed as cystic degeneration of leiomyoma for 3 years. Magnetic resonance imaging revealed a cystic mass measuring 11 cm in diameter, which was contiguous with uterine myometrium. The lesion contained solid areas enhanced on gadolinium-enhanced T1 -weighted imaging. Transabdominal simple total hysterectomy with bilateral salpingo-oophorectomy was performed. Pathological examination revealed endometrioid adenocarcinoma arising from cystic adenomyosis. The patient underwent six courses of adjuvant combination chemotherapy with paclitaxel and carboplatin. No metastasis or recurrence has been demonstrated for 16 months following surgery. Our case demonstrates that cystic adenomyoma possesses the risk of malignant transformation, indicating the importance of long-term follow-up with imaging examination.

Kasarin is an antibacterial marine alkaloid that was isolated from the microorganism Hyphomycetes sp. Its structure was previously reported to be an azetinone compound 1 . However, detailed spectroscopic analysis of the natural compound and its related synthetic analogs revealed that the structure of kasarin should be revised to be a novel 5-malonyloxy-1-methoxy-pyrazin-2( 1H )-one derivative 2 .

視床下部過誤腫に伴った難治性てんかんと行動異常が定位的ラジオ波焼灼術により軽快した9歳の女児例を経験した。3歳頃から笑い発作が出現し、4歳頃から急速な身長の伸びを認め、4歳10カ月時に尿失禁を伴う意識消失発作が出現した。頭部CTでは異常を指摘されず、抗てんかん薬療法が開始されたが難治に経過。5歳10カ月時に頭部MRIにて視床下部過誤腫と診断され、ガンマナイフ治療を行うとともに抗てんかん薬の調整を行う。発作は軽減したが完全抑制されず、8歳10カ月時に定位的ラジオ波焼灼術を施行したところ、発作が消失し、情緒障害も改善した。視床下部過誤腫に対する定位的ラジオ波焼灼術は、比較的安全で有効な治療であると思われた。今後さらなる症例の検討により、その適応や治療方針が確立されることが望まれる。

Using 415 residual blood samples subjected to (1→3) -β-D-glucan assay, we studied the correlation of measurement results between Fungitec G Test MKII "Nissui" (Nissui Pharmaceutical Co., Ltd., Tokyo) and its predecessor, Fungitec G Test MK (Seikagaku Corporation, Tokyo), which is now out of production. Their major difference is that MK II uses reagents derived from blood cells of Limulus polyphemus, the American horseshoe crab, whereas MK used those of Tachypleus tridentatus, an Asian horseshoe crab. Passing-Bablok analysis showed a linear regression with nearly one-to-one correspondence (slope=1.065, intercept=-0.287) between the two test kits over the regular range of measurements (4.0 pg/ml -500 pg/ml ). This was also true when data were subdivided and analyzed in the low to medium (≦150 pg/ml ) and in the low range (≦50 pg/ml ). There were several cases, however, that showed a wide discrepancy between the pair of measurements. This discrepancy is believed to be due in part to the difference between Limulus and Tachypleus in their reactivity to β-glucans with diverse side chains. Despite of this, the even distribution on either side of the regression line of those samples that are associated with deep fungal infection and the abrupt disappearance of such samples below 20 pg/ml attest that MK II "Nissui" is an acceptable successor of MK.

Few reports on malignant transformation of adenomyosis are available, and endometrioid adenocarcinoma arising from cystic adenomyosis is further rarely reported. We report a case of a 67-year-old asymptomatic woman who was referred to our hospital for evaluation of a cystic lesion in the pelvis, which had been diagnosed as cystic degeneration of leiomyoma for 3 years. Magnetic resonance imaging revealed a cystic mass measuring 11 cm in diameter, which was contiguous with uterine myometrium. The lesion contained solid areas enhanced on gadolinium-enhanced T1 -weighted imaging. Transabdominal simple total hysterectomy with bilateral salpingo-oophorectomy was performed. Pathological examination revealed endometrioid adenocarcinoma arising from cystic adenomyosis. The patient underwent six courses of adjuvant combination chemotherapy with paclitaxel and carboplatin. No metastasis or recurrence has been demonstrated for 16 months following surgery. Our case demonstrates that cystic adenomyoma possesses the risk of malignant transformation, indicating the importance of long-term follow-up with imaging examination.

Ovarian clear cell adenocarcinoma (OCCA) is known to have a worse prognosis than ovarian serous adenocarcinoma due to its poor response to conventional platinum-based chemotherapy. Idiopathic thrombocytopenic purpura (ITP), which usually reveals severe thrombocytopenia, is a common autoimmune disorder. However, to date very few cases of ovarian cancer associated with ITP have been reported in the literature. We report a case of a 57-year-old woman who developed OCCA 14 years after the diagnosis of ITP. The patient presented with abdominal distention and mild tenderness. We performed the operation with high-dose immunoglobulin therapy preoperatively, and diagnosed OCCA. Postoperatively, six cycles of cytotoxic chemotherapy with irinotecan hydrochloride plus cisplatin were performed every 4-5 weeks without thrombocytopenia. We conclude that combination chemotherapy with irinotecan hydrochloride plus cisplatin is useful for a case of OCCA associated with ITP.

We report a case of stage IV ovarian clear cell adenocarcinoma (OCCA) in a 72-year-old woman who was treated postoperatively with etoposide combined with cisplatin (EP). The patient exhibited bulky intrapelvic and para-aortic lymph nodes with metastases to the cervical lymph nodes. The primary lesion was resected and, postoperatively, she received one course of combination chemotherapy consisting of cyclophosphamide, 500 mg/m2; doxorubicin, 50 mg/m2; and cisplatin, 70mg/m2 (CAP), followed by six courses of combination chemotherapy consisting of etoposide, 80mg/m2 days 1 through 5 and cisplatin, 70 mg/m2 on day 5 every 4 weeks. After five courses of EP, the lymph node metastases had virtually disappeared. The patient is now disease-free 21 months after the initial surgery. These findings suggest that EP may be useful in treating OCCA.

This is a retrospective study of fifteen cases of primary peritoneal carcinoma who were treated between 2001 and 2010 in our hospital. The median age at the time of diagnosis was 63 years(range, 40 to 79 years). Three patients had clinical stage II disease, eleven patients had stage III disease, and one patient was unstaged. The serum CA125 values at pretreatment were elevated in all patients, with a median value of 4,144. 8 U/mL(range, 102. 8 to 23, 611. 0 U/mL). Optimal debulking was possible in 9 of the 15 patients. All patients were treated with paclitaxel and carboplatin chemotherapy during the preoperative and/or postoperative period. All patients at stage II disease were alive without evidence of disease at the time of evaluation(2 patients5 years, 1 patient3 years). Four patients with stage III disease had died from the disease less than 3 years after the first treatment. The results of our study showed poor survival for the group with stage III disease, but good survival for the group with stage II disease.

We measured the maximal velocity of the blood flow in the main portal trunk by duplex Doppler ultrasound in children suffering from a variety of liver diseases. The maximal velocity of the main portal vein in children with chronic active hepatitis and liver cirrhosis was decreased significantly as compared to those in control children (p < 0.01). The maximal velocity of the main portal trunk and K indocyanine green (ICG) had no significant correlation (r = 0.25, n = 27). The patient with the lowest portal velocity had esophageal and gastric varices with red color sign. We conclude that the measurement of the maximal velocity of the main portal trunk with duplex Doppler ultrasound is useful in evaluating portal hypertension in children.

Plasma concentrations of three major vitamin D3 metabolites, 25-hydroxyvitamin D3 (25-OHD3), 24R, 25-dihydroxyvitamin D3 (24,25-(OH)2D3), and 1 alpha, 25-dihydroxyvitamin D3 (1,25-(OH)2D3), were determined serially in patients with gynecological malignancies treated by chemotherapy which included cisplatin. While 25-OHD3 and 24,25-(OH)2D3 levels did not change regularly, 1,25-(OH)2D3 decreased significantly (50%) after only one or two courses of the treatment in all cases. In concert with declining 1,25-(OH)2D3, the PTH levels increased two or three times. The results were explained by the impairment by cisplatin of mitochondrial function in proximal tubules in the kidney.

Ultrasonographic examination on a fetus at 24 weeks of gestation revealed an intrathoracic cystic mass with fetal hydrops and polyhydramnios. Pathological findings obtained at autopsy which was conducted on the fetus at 28 weeks indicated a typical congenital cystic adenomatoid malformation (CCAM) of the lung. The differential diagnosis and possible intrauterine treatment for lung CCAM are discussed.

Massive intestinal protein loss was demonstrated and the site of loss determined by abdominal scintigraphy with 99mTc-labeled human serum albumin in a 9-year-old girl following the Fontan operation for pulmonary atresia with intact ventricular septum. Significant activity accumulating in the lower small intestine and moving with its contents into the colon were shown. Her condition may have resulted from intestinal lymphangiectasia, caused by increased central venous pressure. Abdominal scintigraphy with 99mTc-human serum albumin is useful in the diagnosis of protein-losing enteropathy.

We assessed the efficacy of a government-sponsored mass protection program in Osaka, Japan, for perinatal HBV infection in infants born to HBeAg positive HBV carrier mothers. We also evaluated the impact of optional follow-up procedures in such infants, including an evaluation of anti-HBs response and a booster dose of HBV vaccine for poor responders. The results demonstrated that this mass protection program protected 94.4% of the infants from perinatal HBV infection in the Osaka area. However, the proportion of infants with an unprotective level of anti-HBs was higher in the standard group than in the follow-up group both at 1.0 and 1.5 years of age, which was also the case for HBV events. Furthermore, the present study showed that a booster dose of vaccine in poor responders was very effective in promoting an anti-HBs response. In conclusion, we recommend that a follow-up blood test to confirm a response of anti-HBs to HBV vaccine should be performed at 4-8 weeks after the third injection of HBV vaccine in infants born to HBeAg positive HBV carrier mothers. We also recommend that a booster injection of HBV vaccine should be immediately given to poor responding infants who otherwise are at a considerable risk of developing HBV infection in late infancy.

Kasarin is an antibacterial marine alkaloid that was isolated from the microorganism Hyphomycetes sp. Its structure was previously reported to be an azetinone compound 1 . However, detailed spectroscopic analysis of the natural compound and its related synthetic analogs revealed that the structure of kasarin should be revised to be a novel 5-malonyloxy-1-methoxy-pyrazin-2( 1H )-one derivative 2 .

A 20-month-old boy was investigated for persistent liver dysfunction. Liver histologic findings showed chronic hepatitis. The presence of human herpesvirus-6 DNA in liver tissue was demonstrated both by in situ hybridization and by polymerase chain reaction. Human herpesvirus-6 may be a causative agent of chronic hepatitis in this case.

The purpose of the current study was to evaluate the difference in prognostic factors between stage IB and II uterine cervical carcinoma patients treated with postoperative radiation therapy.Between May 1988 and May 1998, a total of 94 patients including 47 patients with stage IB and 47 patients with stage II uterine cervical carcinoma were treated with postoperative radiation therapy at Tokyo Metropolitan Komagome Hospital. All patients were treated with 10 MV X-rays using an anterior-posterior parallel opposed field which covered the whole pelvis. Fractionation was 2 Gy per day, five fractions per week, to a total dose of 50 Gy.The 5-year overall survival rates of stage IB and II were 89.4% and 79.3%, respectively. In multivariate analysis for all patients, lymph node status, histology, and surgical margin status were recognized as prognostic factors. Limited to stage IB patients, lymph node status was the only independent prognostic factor. However, for stage II patients, tumor histology was also an independent prognostic factor.The prognostic factors of stage IB and II were different. Tumor histology was only associated with stage II patients. These findings suggested that tumor histology might have played a different role as tumor stage progressed. However, further studies with large numbers of patients will be required to confirm these results.

This study was performed to investigate the frequency of human herpesvirus 6 (HHV-6) infection of the liver in children with a variety of liver diseases and to evaluate the role of HHV-6 infection in pediatric patients with prolonged non-B non-C hepatitis. Detection of the HHV-6 genomes in liver, in peripheral blood mononuclear cells (PBMC), and in plasma was performed by PCR or by in situ hybridization. Liver biopsy materials from 48 patients, in whom HHV-6 infection was serologically confirmed, were available for PCR analysis. Sequences of the HHV-6B genome were detectable in the livers of 36 of 48 patients (75%). The presence of the genome was not associated with serum transaminase activities. The genome was detectable in PBMC of 22 of 31 (71%) patients tested. In these 31 patients HHV-6 was detected in both the livers and PBMC of 20, was detected in PBMC but not in the livers of 2, was detected in the livers but not in PBMC of 3, and was detected in neither of samples of 6. In situ hybridization of the livers of six patients showed the presence of the HHV-6B genome in the nuclei of hepatocytes. The anti-HHV-6 immunoglobulin M antibody was detectable in 2 of 9 of the non-B non-C hepatitis patients, whereas none of the 22 patients with etiology-defined liver diseases tested positive. Cell-free viral DNA was not detectable in either group of patients. Our results showed that HHV-6B is frequently present in the livers of children with a variety of liver diseases but do not support the assumption that HHV-6B infection of the liver is associated with prolonged non-B non-C hepatitis.

We studied the effect of Sho-saiko-to (Xiao-Chai-Hu-Tang) on HBeAg clearance rate (SN rate) in fourteen children with chronic hepatitis B virus (HBV) infection and with sustained liver disease. Seven of fourteen patients (50.0%) became HBeAg negative in the average observation period of 0.47 years(0.2-0.9 years). Four of those seven patients developed anti-HBe. The annual SN rate in the She-saiko-to treated group was apparently higher than the natural annual SN rate (22.7%) of 22 untreated patients retrospectively reviewed from the onset of hepatitis. Sho-saiko-to seemed to promote clearance of HBeAg in children with chronic HBV infection and with sustained liver disease. Sho-saiko-to may be an useful drug for such patients.

We have examined a quantitative relationship between platelet aggregation and release reaction using 20 subjects including healthy women and gynecological patients. We have determined the released amount of ATP on luminescence curve which was observed during platelet aggregation using a lumi-aggregometer. The peak of this curve was measured and the released amount of ATP was calculated. Because the luminescence curve declined with time course, the amount of ATP was corrected with a comparison to standard luminescence curve which was obtained by an addition of 10μM of authentic ATP. A slope in luminescence curve was also calculated with correction against time course, and a lag time before the onset of luminescence was measured. The above three values were compared against the intensity of platelet aggregation. The stimulants were as follows; 1, 3 and 10μM ADP, 0.1, 1 and 10μg/ml of adrenaline and 1, 3 and 10μg/ml of collagen.Significant positive correlations were observed between the intensities of 5 minutes aggregation and the amounts of released ATP induced by the above reagents except 10μg/ml of collagen. Furthermore, closer correlations were obtained between the intensities of 5 minutes aggregation and logarithmic amounts of released ATP. Close correlations were also observed when the intensities of 5 minutes aggregation was compared against slopes in ATP release curves induced by ADP and collagen except in high concentrations, and lag times in ATP release induced by adrenaline except in low concentration. ADP and adrenaline in low concentrations did not induce platelet release reaction in quite a number of cases. According to the results, 3μM ADP, 1μg/ml of adrenaline and 1 and 3μg/ml of collagen were deemed suitable as stimulants to observe the relationship between aggregation and release reaction of platelets.