bluuebunny/crossref_metadata_2025_split huggingface dataset:
A mild case of nesidioblastosis with diagnostic and therapeutic difficulty
Authors: YASUKURA KEIKO , ONO ATSUSHI , WOO MAN , MATSUZAKI SHUJI , IWASE SUIKO , KOBAYASHI YOHNOSUKE 
Published: January 2011

AbstractA female infant with nesidioblastosis who showed mild clinical symptoms is reported. In this patient, insulin levels and insulin to glucose ratios (IRI/G) were often normal. Regular milk feedings supplemented with continuous glucose infusion (0.7‐2 mg/kg per min) or oral glucose feedings (4.5 mg/kg per min) prevented hypoglycemia. As leucine‐sensitivity was diagnosed at 2 months of age, she was started on diazoxide. This was, however, ineffective, and adverse effects appeared. Subtotal pancreatectomy (95%) was therefore attempted at 5 months of age, and persistent normoglycemia as well as normal growth and development followed up to 3 years after the operation. The pancreas showed characteristic signs of nesidioblastosis. The above clinical observation suggests that a patient with nesidioblastosis whose blood glucose level is easily controllable may develop an unexpected episode of hypoglycemia in the presence of a leucine sensitivity. In such a patient, diazoxide or, when it is of no avail, surgical intervention should promptly be instituted to prevent possible neurologic sequelae induced by hypoglycemia.
A case of urethral prolapse in a young girl
Authors: OKADA AKIRA , TAKEHARA HIROO , MASAMUNE KATSUHIRO , KOMI NOBUHIKO 
Published: January 2011

AbstractUrethral prolapse is rarely reported in the pediatric literature. We report a 4 year old female with urethral prolapse and discuss the etiology and the treatments in children documented in the literature.
Dipyridamole‐provoked chest pain implies severe coronary artery disease in children
Authors: TOMITA HIDESHI , IKEDA KAZUO , NAGATA NOBUO , CHIBA SHUNZO , KUBOTA MASAHIRO , TSUDA TAKATOSHI 
Published: January 2011

AbstractThe diagnostic significance of dipyridamole‐provoked chest pain was studied in 17 children with severe coronary arterial stenotic lesions (CAL) complicated with Kawasaki disease. Although dipyridamole induced chest pain in seven patients (symptomatic group), 10 reported no pain (asymptomatic group). In the asymptomatic group, seven children had one vessel disease (1VD) of right coronary artery (RCA) and the other three had two vessel disease (2VD) involving the RCA and left anterior descending artery (LAD). Four multivessel disease patients, one three vessel disease (3VD) and three 2VD of LAD and RCA, and three 1VD of LAD, were symptomatic. In the thallium scans, all patients, except two of the asymptomatic group, showed perfusion abnormalities. In addition, the extent score of the symptomatic group was significantly worse than that of the asymptomatic group (P = 0.01). While only one in six of the asymptomatic group showed abnormal ST depression on treadmill exercise electrocardiography, all patients in the symptomatic group (P = 0.02) showed ischemic ST depression. These findings suggest that the occurrence of chest pain after medication with dipyridamole closely correlates with the severity of CAL in children.
Histopathologic and coronary angiographic assessment of effectiveness of aspirin or aspirin‐and‐gammaglobulin in Kawasaki disease
Authors: HANEDA NORIYUKI , MORI CHUZO 
Published: January 2011

AbstractCoronary angiography and right ventricular endomyocardial biopsy were performed in 36 children during convalescence (days of illness 23–86; mean = 41.5 days) following acute Kawasaki disease. Treatment of the acute stage was not randomized; it consisted of aspirin alone in 14 subjects (during the years 1980‐88), and gammaglobulin and aspirin in 22 subjects (1984‐91). The dosage of aspirin was 30 mg/kg orally during the acute febrile stage and 5–10 mg/kg orally after lysis of fever. The dosage of gammaglobulin was 200 mg/kg × 5 days in 19 patients, 200 mg/kg × 3 days in one patient, 200 mg/kg × 1 day + 400 mg/kg × 4 days in one patient and 200 mg/kg × 4 days + 400 mg/kg × 4 days in one patient. Among the 14 patients treated with aspirin alone, large coronary aneurysms were noted in four, moderate aneurysms in three and transient aneurysms in one. Among 22 patients treated with gammaglobulin, only five had aneurysms and these were transient. The histopathological (HP) score based on myocardial disarrangement, degeneration and hypertrophy; interstitial edema, large mononuclear cell infiltration and fibrosis; and endocardial abnormalities was higher in the aspirin group than in gammaglobulin group. Moderate to severe HP. changes were noted in five subjects in the aspirin group, while moderate HP changes were found in only two subjects in the gammaglobulin group. Gammaglobulin therapy not only reduced the incidence of the coronary arterial lesions but also reduced the severity of myocardial damage from moderate or severe to mild in Kawasaki disease.
Etiology of 19 infants with apparent life‐threatening events: Relationship between apnea and esophageal dysfunction
Authors: TSUKADA KAZUKO , KOSUGE NORIKO , HOSOKAWA MAYUMI , UMEZU RYOJI , MURATA MITSUNORI 
Published: January 2011

AbstractThere are various identifiable diseases or conditions that can cause apparent life‐threatening events (ALTE; e.g. gastroesophageal reflux (GER) and seizures). Nineteen infants with ALTE (mean age: 4.3 months) were brought to our hospital between June 1986 and August 1991. The causes of these ALTE were investigated. Analysis of laboratory data, radiological studies and esophageal function tests led to the diagnosis of GER in six of 19 infants; pertussis in five; upper respiratory infection in three; vagotonia‐like condition with esophageal dysfunction in two; laryngostenosis with cerebral palsy in two; choking on food or drink in two; and epilepsy in one infant. Two cases (one case of pertussis and one of vagotonia‐like condition) were associated with GER. Some of the cases demonstrate that ALTE in infants may be induced by GER or some esophageal dysmotility. Further studies of ALTE are needed to ascertain how frequently GER or esophageal dysmotility is responsible for ALTE.
Chronic ventilator‐assisted children in university hospitals in Japan
Authors: SAKAKIHARA YOICHI , YONEYAMA AKIRA , KAMOSHITA SHIGEHIKO 
Published: January 2011

AbstractThe status of chronic ventilator‐assisted children admitted to university hospitals in Japan was investigated. Of 81 university hospitals which responded to the survey, 52 hospitals had admitted 102 patients requiring chronic ventilatory support. More than 60% of the patients were suffering from neurologic disorders. Werdnig‐Hoffmann disease was the most common disease, followed by hypoxic‐ischemic encephalopathy, congenital heart disorders and various myopathies. The average duration of ventilator care was 823 days. Only nine patients were weanable from the mechanical ventilation. Although the population of chronic ventilator‐dependent patients was not large, the chronic nature of the basic disorders and the need for intensive care of the patients have raised serious social and medical issues.
Defective activity of Epstein‐Barr virus (EBV) specific cytotoxic T lymphocytes in children with chronic active EBV infection and in their parents
Authors: FUJIEDA MIKIYA , WAKIGUCHI HIROSHI , HISAKAWA HIROAKI , KUBOTA HARUO , KURASHIGE TAKANOBU 
Published: January 2011

AbstractAntibodies of Epstein‐Barr virus (EBV), EBV‐specific cytotoxic T lymphocyte (EBVCTL) activity and the lymphocyte subset of CTL were examined in 13 Japanese children with chronic active EBV infection (CAEBV) and their parents (eight fathers and 10 mothers).Anti‐virus‐capsid antigen (VCA)‐IgG antibody titers ranged from 1: 640 to 1: 5120 in the patients with CAEBV and from 1: 40 to 1: 640 in the parents. While anti‐VCA‐IgM antibody was detected in three patients, anti‐VCA‐IgA antibody in five and anti‐early‐antigen (EA)‐IgG antibody in 11, no antibody was detected in the parents except anti‐EA antibody, which was positive in the mothers of cases 5 and 13 (1: 10 and 1: 40). Anti‐EBV‐associated nuclear antigen (EBNA) antibody was ≥ 1: 10 in six out of 13 patients with CAEBV and in 10 out of 18 parents tested. Epstein‐Barr virus activity was significantly lower (P < 0.005) both in the children with CAEBV and in their parents than in seropositive age‐matched controls. Proportions of a CTL subset (CD8+ CD11− lymphocytes) in the patients with CAEBV were significantly higher (P< 0.005) than in controls, while those in the parents were at the same level as in controls.Defective EBVCTL activity and anti‐EBNA‐antibody responses were frequently observed both in children with CAEBV and in their parents, which may suggest that the abnormal immune response to EBV may be based on a familial disorder, though no familial involvement has been reported in Japanese children with CAEBV.
Active immunization of children with leukemias and lymphomas against infection by hepatitis B virus
Authors: Rokicka‐Milewska Roma , JACKOWSKA TERESA , SOPYŁO BARBARA , KACPERSKA ELZBIETA , SEYFRIED HALINA 
Published: January 2011

AbstractEngerix B vaccine was administered to 54 children with leukemias and lymphomas aged from 2 to 15 years. In 36 cases chemotherapy was completely stopped, and 18 cases were receiving maintenance treatment. Engerix B was given at 0, 1, 2 and 6 months in a dose of 20 μg to children < 10 years, and 40 μg to older patients. The effectiveness of active immunization was demonstrated after complete therapy cessation in 88% of cases. The levels of antibodies determined 1 year after primary vaccination remained high, and in most of the vaccinated children they were > 1000 mIU/mL.In children vaccinated in the course of maintenance treatment the levels of antibodies did not give sufficient protection against infection.
The increase of yellow skin colour beyond that of serum bilirubin: A proposed indicator of risk for bilirubin encephalopathy in the newborn
Authors: KNUDSEN AAGE , EBBESEN FINN , HANSEN HELLE , BRODERSEN ROLF 
Published: January 2011

AbstractForty‐seven newborn infants with 1 min Apgar score < 7 were studied. On the third postnatal day the following measurements were made: yellow skin colour, serum bilirubin concentration, reserve albumin concentration and plasma pH. Given the bilirubin concentration and the regression curve between the yellow skin colour and the bilirubin concentration, Δ‐TcB was calculated as the difference between measured yellow skin colour and the expected yellow skin colour. There was a negative correlation between Δ‐TcB and Apgar score (P = 0.003), pH (P = 0.026) and reserve albumin concentration (P = 0.045). Fourteen of the included newborns had central nervous system symptoms in the days just following birth. A tendency towards higher Δ‐TcB was noted in this group (P = 0.08). The results suggest that further study of Δ‐TcB determination as a tool in the assessment of the icteric newborn infant is justified.
A study of prognosis in 52 cases with juvenile rheumatoid arthritis
Authors: KOBAYASHI TOSHIAKI , TANAKA SHINSUKE , MAEDA MOTOHARU , OKUBO HIROKO , MATSUYAMA TAKESHI , WATANABE NOBUO 
Published: January 2011

AbstractThe prognosis in 52 patients with juvenile rheumatoid arthritis (JRA) was studied. There were 35 cases of systemic onset, 12 of polyarticular onset and 5 of pauciarticular onset. Thirteen systemic cases developed a polycyclic course with chronic polyarthritis. Many monocyclic JRA in systemic cases subsided within 1 year. There were no instances of polyarticular cases or pauciarticular cases that shifted to other type. However, there were many cases with a long active polyarticular JRA and with remission at an early stage in the pauciarticular type. The stage and class were I or II in 90% of cases with a good prognosis for the joints, but there were some serious cases. Transient carditis or iritis which developed at an early stage subsided later. The intractable systemic cases had drug‐induced complications. The cases with steroid‐induced complications tended to be chronic. One death in a systemic case was caused by hepatic failure.
Current topics in childhood lupus nephritis
Authors: TOCHIMARU HIROYUKI , YASUDA KAZUE , TAKEKOSHI YASUO , MASTUMOTO SHUZO 
Published: January 2011

AbstractLupus nephritis is a major predictor of the prognosis of systemic lupus erythematosus (SLE). The present paper discusses lupus nephritis from clinical and immunopathological points of view.Although recent advances in diagnosis and treatment improve the prognosis of children with SLE, there remain many unsolved clinical problems. One of the current topics in the treatment for SLE is intermittent intravenous cyclophosphamide therapy which is effective even for the steroid‐resistant patients with severe lupus nephritis, at least for short‐term observation.Immunopathologically, the following issues are discussed: (i) The C5b‐9 terminal complement complex plays an important role in the pathogenesis of lupus nephritis. The possible interaction of vitronectin and SP‐40,40 is also mentioned; (ii) A semi‐quantitative analysis of the charge barrier of the glomerular basement membrane reveals that the charge barrier dysfunction plays an important role in the pathogenesis of proteinuria in lupus nephritis. This study also demonstrates that the charge of immune deposits is important for the initiation of glomerular injury in lupus nephritis; (iii) It is demonstrated that the histopathological diversity of lupus nephritis is based on biological properties of nephritogenic autoantibodies in murine lupus models.
Small bowel obstruction in children: Review of 10 years experience
Authors: IKEDA HITOSHI , MATSUYAMA SHIRO , SUZUKI NORIO , TAKAHASHI ATSUSHI , KUROIWA MINORU , HATAKEYAMA SHINITSU 
Published: January 2011

AbstractSmall bowel obstruction, excluding postoperative adhesive ileus, in patients > 1 month old treated between June 1982 and May 1992 at Gunma Children's Hospital Medical Center is reviewed. There were 32 patients, 22 boys and 10 girls, whose ages ranged from 1 month to 6 years (median 9 months). Intussusception was the most frequent cause of obstruction and was seen in 17 patients (53.1%). Causative lesions were identified in five patients, and were ileal duplication cysts in four and Meckel's diverticulum in one. Incarcerated inguinal hernia and mesenteric cysts resulted in bowel obstruction in six and three patients, respectively. Other causes included mesodiverticular band, ileal volvulus without malrotation, abnormal adhesion of omentum, abnormal band, vitelline duct remnant and trapping in a mesenteric defect. As for the age distribution, there was no significant correlation between the causes of obstruction and the age of patients. Ultrasonography was useful in differential diagnosis, and this modality should therefore be used in every patient with signs of small bowel obstruction.
Contribution of pulmonary hemodynamics on manifestation of allergic asthma in patients with congenital heart disease
Authors: MATSUOKA SUGURU , TATARA KATSUNORI , USIROGUCHI YURI , KUBO MASAHIRO , AKITA HIROSHI , KURODA YASUHIRO 
Published: January 2011

AbstractThe purpose of this study was to determine whether pulmonary hemodynamic abnormalities relate to manifestations of allergic asthma.In 448 patients with congenital heart disease the relationships between asthma and age or pulmonary arterial blood (PA) flow were studied. Asthma (allergic and non‐allergic) was more common in 39 (19%) of 201 patients with high PA flow, compared with the incidence in those with normal PA flow (6/117, 5%; P < 0.001) and reduced PA flow (1/130, 1%; P < 0.05). In the high PA flow group, the frequency of asthma declined significantly (P < 0.01) with age, from 25–26% in the 6 month‐5 year patient group to 5% in the 6–12 year old patients.The frequency of asthma, including allergic type, was significantly (P < 0.01) greater in patients with pulmonary hypertension (15/24, 63%) than in those without (10/77, 13%) at the age of 6 months to 1 year.Asthma in the high PA flow group was associated with other allergic diseases in 30 (77%) of 39 patients, including food allergy in nine (23%), atopic dermatitis in 14 (36%), allergic rhinitis in seven (18%) and abnormally high total IgE levels in 14 (36%).These findings suggest that high pulmonary flow or pulmonary hypertension enhances the manifestation of allergic disease, particularly asthma.
Echocardiographic evaluation of vagolytic effects of atropine sulfate during pediatric halothane anesthesia
Authors: HORIGOME HITOSHI , TSUJI MARIKO , YAMASHITA MASAO , HIRANO TAKEKI 
Published: January 2011

AbstractThe aims of this study were to define the antagonistic effects of atropine sulfate to halothane‐induced cardiovascular depression in children, and to clarify whether or not a larger dose of atropine is more effective in attenuating the cardiovascular depression.Thirty‐four children aged 1–12 years who had undergone minor surgery, free from cardiac or pulmonary disease, were assigned at random to two groups. M‐mode echocardiographic evaluation of left ventricular function in each patient was performed at three points (before induction, point A; after induction, point B; and following administration of atropine, point C). Results were compared between points A and B, B and C and C and A, and between the two study groups with different doses of atropine (0.01 mg/kg vs 0.02 mg/kg).Heart rate (HR), mean blood pressure (MBP) and left ventricular shortening fraction (LVSF) decreased, and left ventricular end‐diastolic dimension (LVEDD) were increased significantly by halothane induction. Although HR and MBP recovered following atropine, LVSF and LVEDD remained unchanged. There were no differences found between the values after vagolysis in both study groups, except for HR and mean velocity of circumferential fiber shortening (mVcf). Heart rate increased above that of pre‐induction, even following the smaller dose of atropine.The myocardial depression cannot be necessarily attenuated by vagolysis regardless of the dosage of atropine. The smaller dose (i.e. 0.01 mg/kg) seems to be sufficient only to antagonize the bradycardia and hypotension during halothane anesthesia in children.
Medium‐term results of percutaneous transluminal balloon aortic valvuloplasty with Inoue balloon catheter for congenital aortic stenosis
Authors: OZASA HIROSHI , ECHIGO SHIGEYUKI , MORI CHUZO , KAMIYA TETSURO , FUSE SHIGETO , KUROE KENJI , KIMURA KOJI , TAKAMIYA MAKOTO , INOUE KANJI 
Published: January 2011

AbstractAlthough percutaneous transluminal balloon aortic valvuloplasty (PTAV) has been performed for congenital aortic stenosis in infants and children for several years, its efficacy and the associated aortic regurgitation (AR) have not been widely discussed. Percutaneous transluminal balloon aortic valvuloplasty using an Inoue balloon catheter was performed for congenital aortic stenosis in 12 patients (4–16 years old) in this study. The systolic aortic valve pressure gradient ranged from 42 to 111 mmHg before PTAV and became < 50 mmHg immediately after PTAV in 10 cases (83%). Eight of these 10 patients had no increase in the gradient during subsequent observation for a period of 9–40 months. Aortic regurgitation increased immediately after PTAV in nine cases (75%). It increased from grade 1 to grade 2 in eight cases and from grade 1 to grade 3 in one patient; no significant enlargement of the left ventricular end‐diastolic diameter and no significant change in the left ventricular end‐diastolic pressure (LVEDP) or the cardiac index was observed during follow‐up in these patients. There was a correlation between the diameter of the balloon and efficacy; an appropriate diameter was considered to be about 90% of the aortic annular diameter. Changes in the hemodynamic parameter after PTAV with an Inoue balloon were small in most patients and this procedure is considered to be a treatment that should be attempted prior to surgery for congenital aortic stenosis.
Histopathologic evidence for cardiomyopathy in a child with mitral valve prolapse
Authors: NAKADA TOSHIMASA , YONESAKA SUSUMU 
Published: January 2011

AbstractAn intriguing relationship between idiopathic mitral valve prolapse (MVP) and cardiomyopathy has been reported in adult cases of MVP. This paper reports a girl with idiopathic MVP, who presented with progressive cardiomegaly and cardiomyopathic findings on cardiac biopsy; right ventricular endomyocardial biopsy at the age of 15 years showed myocardial hypertrophy, disarray, myocardial degeneration, interstitial fibrosis and endocardial thickening. To our knowledge, this is the first case of idiopathic MVP in childhood that has been shown to have positive biopsy findings for cardiomyopathy.
Treatment with flecainide for symptomatic and refractory tachyarrhythmias in children
Authors: ISMAIL ZULKIFLI , ALWI MAZENI , LIM MK , MURTAZAM HZA AHMAD , JAMALUDDIN AN 
Published: January 2011

AbstractNine children, aged 2.5 months to 16 years, presenting with tachyarrhythmias were treated with intravenous (i.v.) flecainide, a type 1C antiarrhythmic drug. There were four boys and five girls; seven were supraventricular and two ventricular tachycardias and three had structural cardiac abnormalities. The i.v. dose required to terminate the arrhythmias ranged from 1.0 to 2.4 mg/kg (mean 1.55 mg/kg) although a mean of 1.94 mg/kg per dose was required to maintain sustained sinus rhythm after a single i.v. dose. Eight of the patients — six supraventricular and two ventricular tachyarrhythmias, required maintenance oral flecainide. Oral dosages of 6.7–9.5 mg/kg per day (mean of 7.97 mg/kg per day in three divided doses) were required to effectively prevent the tachyarrhythmias. Intravenous and oral flecainide are safe and effective in terminating supraventricular and ventricular tachyarrhythmias. No evidence of proarrhythmia was found in the patients during follow up of between 5 and 9 months. The present limitation of performing radiofrequency ablation on infants and small children justifies the important place of medical therapy for re‐entrant supraventricular tachyarrhythmias.
The peritoneal equilibration test variables in pediatric CAPD patients
Authors: FUKUDA MASAMICHI , KAWAMURA KEN , OKAWA TOSHIYA , KAWAHARA KAZUHIKO , KAMIYAMA YASUNORI , HONDA MASATAKA 
Published: January 2011

AbstractThe peritoneal equilibration test (PET) is generally accepted as a valuable tool in individualizing continuous ambulatory peritoneal dialysis (CAPD) treatment schedules in adults. However, the classification of peritoneal permeability as described by Twardowski is not available for children due to differences in peritoneal transport abilities. In this paper, we report the results of the PET for 20 patients aged from 1.5 to 24 years (mean 10.3 years), who started CAPD therapy at age 0–15 years, using Twardowski's standardized technique. D/P creatinine (dialysate to plasma ratio of creatinine at 4 h), D/D0 glucose (the ratio of dialysate glucose at 4 h dwell time to dialysate glucose at 0 dwell time), and the residual volume (RV) in the abdominal cavity were calculated. The mean and standard deviation values for D/P creatinine and D/D0 glucose were of a higher permeability level than those reported in Twardowski's data. Furthermore, a significant correlation of creatinine and glucose with fluid drainage ability of the peritoneum were observed. A large amount of RV (425±125 mL/m2) was detected in the patients' peritoneal cavity and this had a significant correlation with dialysate leak occurrences. The authors suggest that the RV would be an indicator for leaks and especially latent leaks. The cause of a large RV is unknown, but there is a probability that drainage volume and transport rates of metabolites decrease because the concentration of the instilled dialysate is immediately diluted by a large RV within the peritoneum.