bluuebunny/crossref_metadata_2025_split huggingface dataset:
Increased incidence of spina bifida occulta in fluorosis prone areas
Authors: GUPTA SUNIL K , GUPTA RAMESH C , SETH ASHOKA K , CHATURVEDI CHANDRA S 
Published: January 2011

AbstractSpina bifida, a congenital deformity of the posterior wall of vertebrae of the spine, is a midline defect of skin, vertebral arches and neural tube, usually in the lumbosacral region. Its incidence is reported to be 0.2 to 0.4 per 1000 live births. Various hypotheses have been put forward as etiological factors for spina bifida including consumption of potato affected by blight and hardness of drinking water but these have not been proven. Two groups of 50 randomly chosen children were established. The study group consisted of children aged 5 to 12 years, weighing 15 to 30 kg, consuming fluoride rich drinking water (4.5 and 8.5 ppm fluoride; WHO permissible limit is 1.5 ppm fluoride), and manifesting either clinical, dental and/or skeletal fluorosis. The control group consisted of age and weight‐matched children, consuming less than or equal to 1.5 ppm fluoride in drinking water and not showing any evidence of fluoride toxicity. These children were evaluated for antenatal history, general clinical examination (especially for dimples, tufts of hair, haemangioma on skin throughout the length of spine), other congenital abnormalities, evidence of fluoride toxicity, biochemical estimation for fluoride levels in blood and serum and by skiagrams of the spine to examine for the presence of spina bifida occulta. A total of 22 (44%) of the 50 children in group A, the study group, and 6 (12%) of the 50 children in group B, the control group, revealed spina bifida occulta in the lumbosacral region. The proportion of children with spina bifida occulta in these fluoride rich areas was thus 44%. Since this defect occurs during the antenatal period, these observations indicate that an association may exist between spina bifida occulta and high fluoride intake during the antenatal period.
Electron microscopic finding of eccrine sweat gland epithelial cells in a patient with Krabbe disease
Authors: OHNO ICHIRO , SHINODA KAZUKO , TSUGAWA KEIKO , TAKIZAWA NOBORU , TANIGUCHI NOBORU , KIMURA SEIJI 
Published: January 2011

AbstractA 13 month old boy was found to have severely reduced β‐galactocerebrosidase activity suggesting infantile Krabbe disease. Clinically, the patient showed a progressive neurological deterioration with white‐matter disease on radiological study. Axillary skin biopsy was performed to support the diagnosis. On electron microscopy, needle‐like inclusions, which are the typical finding seen in the cytoplasm of astrocytes and Schwann cells in the classic infantile form, were present in eccrine sweat gland epithelial cells. This method is useful for diagnosis when nerve biopsy and biochemical analysis are not readily available.
Type la glycogen storage disease with focal nodular hyperplasia in siblings
Authors: TAKAMURA MAYUMI , MUGISHIMA HIDEO , OOWADA MISATO , HARADA KENSUKE , UCHIDA TOSHIKAZU 
Published: January 2011

AbstractGlycogen storage disease type I (GSD‐I) is an inherited disorder that is due to a glucose‐6‐phosphatase (G6Pase) deficiency. There have been recent reports of hepatocellular tumors in adults with this disease. Hepatic adenoma is the most common tumor described but others, including hepatocellular carcinomas, hepatoblastomas, and focal nodular hyperplasia (FNH) have been reported.FNH of the liver is a rare benign lesion that has been reported in eight patients with GSD‐I. Three of these eight patients, in addition to the patient in our study, had been treated with portacaval shunts. When these patients were compared with patients who had not received such treatment, it appeared that the portacaval shunts may have induced the development of FNH and may have been associated with earlier complications.FNH is a benign tumor that may coexist with adjacent fibrolamillar carcinomas and/or adenomas and requires careful follow‐up.
Transient dilatation of the abdominal aorta in an infant with Kawasaki disease associated with thrombocytopenia
Authors: MIYAKE TOSHIHARU , YOKOYAMA TATUO , SHINOHARA TOHRU , SETO SHIRO , OIKI MIHO 
Published: January 2011

AbstractWe report on an 8 month old infant with Kawasaki disease associated with giant coronary aneurysms and transient thrombocytopenia. The patient's platelet count decreased to 24000/mm3 on the 31st day of illness and fibrin degradation product was 5 μg/mL. Platelet count increased to the normal level (357000/mm3) on the 35th day of illness. On the 27th day of illness, dilatation of the distal abdominal aorta adjacent to the bifurcation of the iliac arteries was observed by B‐mode and color Doppler ultrasonography. It gradually returned to a normal size by the 45th day of illness. Aspirin administered from the 3rd to the 26th day of illness was replaced with flubioprophen because of liver dysfunction. Although we can not eliminate aspirin allergy as the cause of the transient thrombocytopenia, we think that the thrombocytopenia may have been related to the regression of the abdominal aorta.
Selective IgA deficiency with unusual features: Development of common variable immunodeficiency, Sjögren's syndrome, autoimmune hemolytic anemia and immune thrombocytopenic purpura
Authors: SANAL ÖZDEN , ERSOY FÜGEN , METÍN AYSE , TEZCAN ÍLHAN , BERKEL A. ÍZZET , YEL LEMAN 
Published: January 2011

AbstractWe report on a girl with selective IgA deficiency and persistently low complement component 4 (C4) levels compatible with heterozygous C4 deficiency. Deterioration of her serum immunoglobulin levels and transition to common variable immunodeficiency were observed within a 5 year follow‐up. She also developed Sjögren's syndrome, autoimmune hemolytic anemia and immune thrombocytopenic purpura While these abnormalities have been described before in various combinations, to our knowledge, they have not been reported in a single individual.
Hypothyroidism and hypoparathyroidism in an 11 year old boy with hemochromatosis secondary to aplastic anemia
Authors: HIMOTO YUUSUKE , KANZAKI SUSUMU , NOMURA HIROAKI , ARAKI TORU , TAKAHASHI YUKIO , SEINO YOSHIKI 
Published: January 2011

AbstractThis is the first reported case, to our knowledge, of hypoparathyroidism and hypothyroidism due to secondary hemochromatosis with onset during childhood. The patient was a boy with refractory aplastic anemia in whom primary hypothyroidism and hypoparathyroidism became apparent at the age of 10 and 11 years old, respectively. He had received a total of 100 L of transfused blood by the age of 10 years. The patient showed poor annual height gain due to primary hypothyroidism, together with hypocalcemia, cataract and intracranial calcification due to hypoparathyroidism. The early appearance of both thyroid and parathyroid dysfunction in this patient may have been due to the delay of initiation of iron‐chelating agents and liver dysfunction due to hepatitis type C.
Treatment of a stage I testicular yolk sac tumor with vascular invasion
Authors: IKEDA HITOSHI , MATSUYAMA SHIRO , SUZUKI NORIO , TAKAHASHI ATSUSHI , KUROIWA MINORU , NAGASHIMA KIKUO , HIRATO JUNKO 
Published: January 2011

AbstractThere is controversy concerning the treatment of stage I yolk sac tumor of the testis, particularly of those with histological factors that indicate a high risk of relapse. Usually orchiectomy alone is sufficient and adjuvant chemotherapy is unnecessary. Retroperitoneal lymphadenectomy is indicated for patients with persistently high alpha‐fetoprotein. Once recurred, treatment at that time is thought to be curative. However, postoperative chemotherapy may be necessary for patients with a tumor expressing histological factors that predict possible relapse. In this paper we report on a case of a 2 year old boy whose tumor invaded the testicular veins. The patient suffered from recurrent disease but was successfully treated by chemotherapeutic regimens including cisplatin and retroperitoneal lymphadenectomy. The importance of the histological factors in making a decision on the treatment strategy for stage I testicular yolk sac tumor is discussed.
Successful autologous bone marrow transplant for relapsed Ki‐1 lymphoma
Authors: TAKAMURA MAYUMI , MUGISHIMA HIDEO , NAGATA TOSHIHITO , SHICHINO HIROYUKI , SHIMADA TOSHIAKI , SUZUKI TAKASHI , CHIN MOTOAKI , HARADA KENSUKE , FUJIMOTO ZYUNICHIRO 
Published: January 2011

AbstractWe report on a 16 year old girl with relapsed Ki‐1 lymphoma and a very poor prognosis. The initial manifestation was multiple bone metastases and lymphadenopathy. The patient achieved remission with modified adriamycin, bleomycin, vincristine, daunomycine therapy. However, 14 months after the completion of therapy, relapse occurred in a new cervical lymph node on the left side. After preparation with chemotherapy and total lymphoid irradiation (TLI) the patient underwent autologous bone marrow transplantation (A‐BMT).Ki‐1 lymphoma shows clinically diverse symptoms, but hematopoietic stem cell transplantation should be performed in relapsed cases. It may be effective to give TLI followed by A‐BMT for patients such as ours who have lymph node involvement without bone marrow metastasis.
Consideration of Helicobacter pylori infection in childhood: Immune response, endoscopic and morphological findings
Authors: STUDENIKIN MITROFAN YA , NIJEVITCH ALEXANDER A , MUTALOV AIRAT G 
Published: January 2011

AbstractSeventy‐five children (aged 9–14 years) infected with Helicobacter pylori were studied endoscopically and morphologically for the signs of infection and immune response by ELISA technique (total IgE and specific IgG against H. pylori); a control group of 36 children (not infected with H. pylori) were studied simultaneously. Helicobacter pylori positive children examined endoscopically revealed a number of mucous membrane changes including erythema, erosions, lymphoid nodular hyperplasia and ulcers. Gastritis was confirmed by histology in 58 children; 6% were termed ‘active’, others were ‘non‐active’. When studying the concentrations of anti‐H. pylori IgG in children from the control group they were considered to be seronegative but in children infected with H. pylori a considerable increase was noted. An evaluation of the interaction between anti‐H. pylori IgG titers and age, endoscopic signs and histology was carried out. Suppositions were made about the presence of links between these characteristics. Children with H. pylori infection showed a considerable increase of total IgE titers in comparison with the control group. The role of IgG and IgE in the development of chronic gastroduodenal diseases associated with H. pylori is discussed.
Patterns of 24 h intragastric acidity in duodenal ulcers in children: The importance of monitoring and inhibiting nocturnal acidity
Authors: YAMASHIRO YUICHIRO , SHIOYA TAKEHIRO , OHTSUKA YOSHIKAZU , NAGATA SATORU , OGUCHI SATOSHI , SHIMIZU TOSHIAKI , SATO MITSUYOSHI 
Published: January 2011

AbstractThe objective of this study was to compare the gastric acidity patterns of patients with duodenal ulcers and| normal children. Eight patients with duodenal ulcer had their intragastric pH monitored for two consecutive 24 h periods using intragastric glass electrodes. The first 24 h period elucidated pH patterns in the absence of treatment and the second period evaluated the acid suppressive effect of 15 mg/kg of cimetidine when given in three divided doses. Results showed that the ulcer patients were hyperacidic, particularly at midnight. This finding was in marked contrast to the results obtained in the study of normal controls. The mean pH of normal children was above 3 around midnight. This phenomenon is known as intragastric pH inversion. The mean pH 3 time (the cumulative duration of the time for which gastric pH is maintained at ≥ pH 3) was significantly shorter in patients with ulcers. However, pH 3 time of these patients significantly increased throughout the 24 h recording period during the daytime and at night after the introduction of cimetidine. This resulted in an induction of apparent nocturnal intragastric pH inversion for the ulcer patients. This study demonstrates the usefulness of 24 h continuous intragastric pH monitoring in children. The data showed that there was a pattern of gastric hyperacidity in pediatric ulcer patients which is clearly distinct from that of normal children, particularly in the patterns occurring at midnight. Cimetidine at 15 mg/kg per day in three divided doses was effective in suppressing secretion even at night.
Detection of immunodeficiencies and persistent infections by urinary neopterin measurement
Authors: HARA TOSHIRO , MATSUO MUNEAKI , TASAKI RYUJI , MIYAZAKI SUMIO , BAMBA MASAHIRO 
Published: January 2011

AbstractWe evaluated the clinical significance of measurement of urinary neopterin levels in primary immunodeficiencies and persistent infections exclusively at afebrile or asymptomatic periods. Despite the examinations at afebrile or asymptomatic periods, urinary neopterin levels were elevated in some patients with primary immunodeficiencies and in patients with persistent infections of human immunodeficiency virus (HIV) or Epstein‐Barr virus (EBV). Therefore, urinary neopterin measurement at afebrile periods will be useful as one of the screening tests in the detection of such disorders among children with occasional episodes of infections.
Lipoprotein (a) and apolipoprotein A‐1 and B in schoolchildren whose grandparents had coronary and cerebrovascular events: A preliminary study of 12–13 year old Japanese children
Authors: OKADA TOMOO , SATO YOSHIYUKI , YAMAZAKI TAKAHIRO , IWATA FUJIHIKO , HARA MITSUHIKO , KIM HIDEAKI , KARASAWA KENSUKE , AYUSAWA MAMORU , FUCHIGAMI TATSUO , HARADA KENNSUKE , OKUNI MASAHIKO , RYO SHIGEO 
Published: January 2011

AbstractThe aim of this study was to evaluate the relationship between the serum levels of lipoprotein (a) [Lp (a)] and apolipoproteins (apo A‐1 and apo B) in schoolchildren with a history of coronary and cerebrovascular events in their grandparents.We measured serum concentrations of Lp (a) and apoliproteins immunochemically in 289 schoolchildren aged 12–13 years and questioned parents about coronary and cerebrovascular events in the children's grandparents. In boys and girls, mean ± s.d. levels of apo A‐1, apo B and Lp (a) were 134 ± 20.3 and 136 ± 17.4 mg/dL, 61 ± 16 and 66 ± 15 mg/dL and 12.5 ± 15.3 and 12.5 ± 15.1 mg/dL, respectively. There were no significant sex differences in the levels of apo A‐1, apo B, and Lp (a).The Lp (a) levels (mean ± s.d., 12.5 ± 15.2 mg/dL; median 7.5 mg/dL, n = 289) were not affected by other variables. The Lp (a) distribution was strongly positively skewed and 75% of schoolchildren had very low levels. In the total 289 schoolchildren, thirty‐two grandparents who had had coronary vascular events (21 myocardial infarction, 11 angina pectoris) and twenty‐three grandparents who had had cerebrovascular events were recorded. By the boxplot statistical analysis, no difference was found in Lp (a) levels in children whose grandparents had myocardial infarction compared with those whose grandparents had no such history, or compared with those whose grandparents had suffered cerebrovascular events. Analysis also showed that the values of log Lp (a) in children whose grandparents had myocardial infarction tended to be higher than the values in children whose grandparents had no such history (P = 0.09). No significant differences in the levels of apo A‐1 and apo B and in the apo B/A‐1 ratio could be seen between children grouped according to the presence or absence of coronary and cerebrovascular events in their grandparents.These results suggest that high levels of Lp (a) in schoolchildren aged 12–13 years may partly reflect the existence of coronary vascular disease in older family members. Lp (a) may account for the strongest index of family history to disease risk in comparison with other apolipoproteins. Further study is needed to clarify the appropriate mass measurement method for Lp (a) in schoolchildren.
Multivariate autoregressive analysis of carotid artery blood flow waveform in an infant of a diabetic mother with cardiomyopathy
Authors: KOJO MASANOBU , OGAWA TERUYUKI , YAMADA KATSUHIKO , SONODA HIROTOMI , SAITO KIYOSHI 
Published: January 2011

AbstractWe analyzed the carotid artery blood flow waveform (CABFW) of an infant of a non‐insulin dependent diabetic mother with hypertrophic cardiomyopathy (IDM cardiomyopathy) through multivariate autoregressive analysis and compared the developmental change of his CABFW with that of normal newborns. The total power was lower than normal newborns on the second and third day of life when his heart dysfunction was severe, and elevated on the fifth day of life when normal‐heart function was recovered. The power of component 3 (C3), of which the damping frequency was 7–11 Hz, was slightly high on the second and third day of life and it decreased to the normal range on the fifth day of life by component analysis. In contrast, the power of C3 increased with decreasing resistance index of anterior cerebral artery (RI of ACA) which shows the cerebral vascular resistance of normal newborns.These results suggest that the carotid artery blood flow volume decreased by low cardiac output and the cerebral vascular resistance decreased to maintain the cerebral circulation, when the heart dysfunction was severe.
Analysis of the circumstances of death of 56 children suffering from cancer: Proposal for the development of terminal medicine in Japan
Authors: HONGO TERUAKI , FUJII YUJI , YAJIMA SHUHEI , MATSUSHITA TAKASHI , OGAWA NORIKO , KINJO YASUKO 
Published: January 2011

AbstractIn Japan terminal medicine for children dying from cancer has not yet been developed nor has a support system for home terminal care and bereaved families been established. We have analyzed our own experiences in these areas and researched the possibilities of establishing support systems.In the 16 years from 1978 to 1993, 56 children with cancer have been treated and have died at Hamamatsu University Hospital. We analyzed the circumstances of their deaths. We interviewed 25 sets of parents about their acceptance of their child's death.Three children (5%) died unexpectedly during treatment, 27 children (48%) died from the side‐effects of intensive treatment, and 26 children (47%) died during terminal care. More children with leukemia and lymphoma died from side‐effects than children with solid tumors (P < 0.05). Six out of the 25 families had not yet accepted the loss of their child due to regrets associated with the missed opportunity for terminal care. From our experiences with the five children who received terminal care at home, we recognize the need for a support system run by the hospital and conclude the time is ripe for initiating home‐based terminal care in Japan.
Length polymorphism of heterochromatic segment of the Y chromosome in boys with acute leukemia
Authors: ERÇAL M DERYA , BRÖNDUM‐NIELSEN KAREN 
Published: January 2011

AbstractThe extent of length polymorphisms of the heterochromatic and euchromatic segment of the human Y chromosome were investigated in 15 boys with acute leukemia and were compared with 15 normal controls. A greater value of the Yh/F index in relation to controls was established (P < 0.05). The length of the euchromatic segment was also shorter in the patients than the controls (P < 0.05).
Atypical hemolytic uremic syndrome
Authors: KAVUKÇU SALIH , İRKEN GÜLERSU , OLGUN NUR , GÜÇLÜ ALEV 
Published: January 2011

AbstractThe pathogenesis of atypical uremic syndrome (HUS), which is rarely encountered in childhood, is poorly understood and its mortality and morbidity rates are high. A wide variety of therapeutic approaches has been attempted and the literature contains numerous conflicting reports about the results of these approaches. In a case diagnosed as recurrent atypical HUS, pulse methyl prednisolone, fresh frozen plasma infusions and plasma exchange transfusion were used at different stages of the disease with satisfactory response.
Cardiac hydatid cyst in a child: Diagnostic value of echocardiography and magnetic resonance imaging
Authors: ALEHAN DURSUN , ÇELİKER ALPAY , AYDINGÖZ ÜSTÜN 
Published: January 2011

AbstractCardiac hydatid cyst is extremely rare in children. We report a case of a cardiac echinococcal cyst in an 11 year old boy, diagnosed by two‐dimensional echocardiography and magnetic resonance imaging (MRI). The cyst was located in the posterior wall of the left ventricle and was excised surgically. Echocardiographic and MRI findings are discussed.
Long‐term sequential changes of antibody to p40tax in HTLV‐I carrier mothers and children
Authors: KUSUHARA KOICHI , UEDA KOHJI , TOKUGAWA KEN , MIYAZAKI CHIAKI , OKADA KENJI , SAWADA TAKASHI 
Published: January 2011

AbstractAntibody to p40tax (anti‐p40tax) in serum specimens obtained sequentially from a human T cell lymphotropic virus type I carrier population of mothers and children were assayed. The prevalences of anti‐p40tax at the initial sampling were 88% (7/8) in children and 55% (16/29) in mothers. Two of the seven positive children lost their anti‐p40tax during the investigation period, resulting in a final prevalence of 63% (5/8) in children. However, anti‐p40tax status was constant in all the 22 mothers with multiple serum samples (15 remained positive and seven remained negative). A decline in the absorbance value of EIA for anti‐p40tax was observed in seven of the 15 anti‐p40tax positive mothers. This decline may result in the disappearance of anti‐p40tax in some of them.
Elastase and cell matrix interactions in the pathobiology of vascular disease
Authors: RABINOVITCH MARLENE 
Published: January 2011

AbstractUltrastructural observations in lung tissue implicated an endogenous vascular elastase (EVE), in the pathobiology of pulmonary vascular disease. In experimental rats, increased activity of a 20 kDa serine proteinase related to adipsin precedes the development of sustained pulmonary hypertension and vascular abnormalities. A further increase in activity is related to malignant progression of the disease. A cause and effect relationship was suggested by studies in which elastase inhibitors successfully prevented or retarded progression of pulmonary hypertension. In vitro studies have shown that both serum and endothelial factors induce EVE via tyrosine kinase intracellular signalling. Induction of EVE can release basic fibroblast growth factor from the extracellular matrix in an active form stimulating smooth muscle cell proliferation. Elastase activity was also observed in the process of smooth muscle cell migration and neointimal formation in coronary arteries following experimental cardiac transplantation. An immune/inflammatory response is observed with increased production of cytokines, tumor necrosis factor‐α and interleukin (IL)‐1β, reciprocally up‐regulating production of fibronectin, a glycoprotein which mediated smooth muscle cell migration. The action of IL‐1β in inducing fibronectin is facilitated by the production of elastin peptides generated by increased activity of an elastase in the coronary arteries. Our studies suggest that ligation of the elastin binding protein by elastin peptides unmasks IL‐1 receptors. Fibronectin also stimulates transendothelial migration of lymphocytes which perpetuates the inflammatory response leading to neointimal formation in this model. Masking integrins on T cells with a decoy synthetic CS‐1 (fibronectin) peptide largely prevented transendothelial migration and coronary neointimal formation following cardiac transplant.