bluuebunny/crossref_metadata_2025 huggingface dataset:
Respiratory syncytial virus infection in lower respiratory tract and asthma attack in hospitalized children in North Hokkaido, Japan
Authors: SAIJO MASAYUKI , ISHII TAKUMA , KOKUBO MASAYO , TAKIMOTO MASATOSHI , TAKAHASHI YOUJI
Published: January 2011

Respiratory syncytial virus (RSV) infection is severe and life‐threatening in some infants. To investigate the epidemiology of RSV infection in hospitalized children in North Hokkaido, Japan, we tried to detect RSV antigen in nasopharyngeal aspirates (NPA) from those children with lower respiratory tract infection (LRTI) and asthma attack. From April 1991 to March 1992, 317 patients were hospitalized in our pediatric ward for the treatment of LRTI and asthma attack. The presence of RSV antigen in NPA taken from 283 patients (89.3%) were examined by enzyme immunoassay. RSV antigen was detected in 88 patients (31.1%). RSV LRTI were noted throughout the year, and the epidemic peak was observed in November and December. There was no significant correlation between the RSV antigen positive rate and mean temperature. RSV played an important role in LTRI in children in North Hokkaido, Japan. RSV LRTI in North Hokkaido was not rare in summer, indicating that RSV was transmitted commonly among children throughout the year.
Increased level/dose ratio of amphotericin‐B in premature infants with renal failure
Authors: HIGUCHI RYUZO , KUSUMOTO SACHIKO , BAN HIROSHI , IWAHASHI SEIJI , KOBAYASHI MASAKAZU , SUMIYAMA KEIICHIRO , KOIKE MICHIO
Published: January 2011

We introduced continuous intravenous infusion of amphotericin‐B (AMPH‐B) to extremely low birth‐weight (ELBW) infants (< 1000 g) with or without renal failure as a single agent for treating definite or probable systemic candidiasis. The species of Candida isolated from blood or tracheal aspirate or urine were C. albicans in seven infants, C. glablata in two, C. tropicalis in one and C. parapsilosis in one. The minimal inhibitory concentrations (MIC) of AMPH‐B required against these isolates were less than 0.2 μg/mL except for that against one strain of C. albicans (0.78 μg/mL). Serum AMPH‐B levels were 0.31‐0.78 (0.51 ± 0.14) μg/mL when doses of 0.2‐0.55 (0.32 ± 0.11) mg/kg per day were being administered. The serum level was higher than the MIC of each isolate in all but one infant who died of disseminated intravascular coagulation and Candida pneumonia. Another infant died of congenital heart disease. The other nine infants survived. The serum level showed no correlation with the daily dose. The ratio of the serum level to the daily dose (L/D ratio) showed a significant correlation to serum creatinine (r = 0.787) and the linear regression curve followed the equation: L/D ratio = 0.223 x serum creatinine + 1.11 (P < 0.01). Few adverse effects due to AMPH‐B were noted. Our data may give a simple reference to serum AMPH‐B levels during continuous intravenous infusion from the dose and the serum creatinine level.
Echocardiographic findings in mild and severe forms of sickle cell anemia
Authors: KILINÇ YURDANUR , ACARTÜRK ESMERAY , KÜMI METIN
Published: January 2011

M‐mode echocardiographic findings were compared between sickle cell anemic and healthy children. Patients were composed of two groups; Group 1: mild group with no crises, no blood transfusions at the ages of 5.0 to 13.0, total of 12 children; Group 2: severe group, with frequent crises with requirement of blood transfusions at the ages of 3.0 to 13.0 years, total of 18 children. Control group was composed of 12 healthy children aged 5.0 to 13.0. When M‐mode echocardiographic findings were compared, important findings were as follows: Mean left atrium dimension was increased both in the mild and severe groups (P < 0.001) compared with controls. This finding also supports the increase in the left ventricle end‐diastolic dimension in both the severe and mild groups as compared with controls (P < 0.001). The increase in end‐diastolic left ventricle dimension could be due to anemia present in the patients, but there was no difference between the two patient groups. Posterior left ventricle thickness and left ventricle mass was increased in both the mild and severe groups compared with controls (P < 0.001, P < 0.05), respectively. Left ventricular fractional shortening was more or less the same with controls. In spite of left ventricular volume load and dilatation, left ventricular contraction was good and systolic function was normal, and there was no correlation between the ECHO findings and hematological indices.
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

A 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

Urethral 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

The 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

Coronary 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

There 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

The 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

Antibodies 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

Engerix 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

Forty‐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

The 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

Lupus 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

Small 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

The 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

The 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

Although 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

An 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

Nine 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

The 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.
An outbreak of group B streptococcus infection in a neonatal nursery and subsequent trial for prophylaxis of nosocomial transmission
Authors: TAKAYANAGI TOSHIMITSU , TANAKA HIROYA , YOSHINAGA MUNEYOSHI , AOKI MIKIHIRO , TESHIMA HIDETAKA , FUKUDA MASAFUMI , TSUJI YOSHIROU , HOSHINA KIYOSHI
Published: January 2011

An outbreak of sepsis and meningitis caused by group B streptococcus occurred in three very low birthweight infants. To prevent further nosocomial transmission, immune globulin and ampicillin sodium were administered intravenously to other very low birthweight infants. After this prophylaxis, no other infants were involved in this outbreak. Immuno‐ and chemoprophylaxis may be considered as procedures to prevent nosocomial infection for very low birthweight infants.
A case of intractable diarrhea firmly suspected to have autoimmune enteropathy
Authors: YAMAMOTO HATSUMI , SUGIYAMA KENJI , NOMURA TOYOKI , TAKI MICHIO , OKAZAKI TORU
Published: January 2011

Protracted diarrhea with insulin dependent diabetes mellitus (DM) and hypothyroidism in a 9 month old Japanese girl who was firmly suspected to have autoimmune enteropathy (AIE) is reported. Her severe secretory diarrhea failed to respond to intensive antidiarrheic treatment and was gradually improved with steroid therapy. The circulating autoantibodies to enterocytes in her serum were detected by indirect immunofluorescence technique and the impaired suppressor T (Ts) cell function was proved by plaque forming assay using bead‐separated CD4 or CD8 T cells together with CD19 B cells. The anti‐enterocyte antibodies were exclusively of immunoglobulin M (IgM) class and were detected with the progress of the protracted diarrhea. Maximum antibody titer was obtained at the onset of DM and the disappearance of autoantibodies was associated with the resolution of the clinical symptoms and signs. The helper functions of adult CD4 T cells to induce Ig‐secreting cells from adult and the patient were strikingly suppressed by adult CD8 T cells. However, the CD8 T cells from the patient lost the ability to inhibit the induction of these Ig‐secreting cells when stimulated with adult CD4 T cells. Moreover, the patient's CD8 T cells stimulated rather than suppressed the induction of Ig‐secreting cells from the patient when stimulated with the patient's CD4 T cells. These results suggest that the impaired Ts cell function in this patient might play some immunological role in the pathogenesis of AIE
An extrapancreatic action of diazoxide to inhibit glucose transport activity on adipocytes
Authors: GOTO YOSHINORI , KIDA KAICHI , KAINO YUKIKAZU , ITO TAKUO , MATSUDA HIROSHI
Published: January 2011

The effect of diazoxide on 3‐O‐methylglucose (3‐O‐MG) transport was studied in isolated rat adipocytes to elucidate its extrapancreatic action. Diazoxide (0.3‐3 mmol/L) significantly inhibited 3‐O‐MG uptake into adipocytes in a basal state or an insulin‐stimulated state. The inhibitory effect was mainly due to the inhibition of insulin responsiveness for 3‐O‐MG uptake. The insulin responsiveness is determined by the capacity in the process of insulin action and in the final glucose transport activity, and diazoxide mainly inhibited the 3‐O‐MG transport activity itself. Based on these findings, this extrapancreatic action of diazoxide is considered to contribute partially to raising the blood glucose level in children receiving the drug. Diazoxide, as a glucose transport inhibitor, may be a useful tool for studying the issues related to glucose transport or insulin action.
A study of urinary metabolites in patients with dicarboxylic aciduria for differential diagnosis
Authors: SHIMIZU NOBUO , YAMAGUCHI SEIJI , ORII TADAO
Published: January 2011

Dicarboxylic aciduria (DCA‐uria) is a relatively common finding in the screening of organic acidemias by gas chromatography/mass spectrometry (GC/MS). A considerable number of patients with DCA‐uria are involved in disturbances of mitochondrial and peroxisomal fatty acid β‐oxidation. The differential diagnosis of DCA‐uria was investigated using a combination of organic acid analysis by GC/MS, carnitine determination, acylcarnitines by fast atom bombardment/mass spectrometry (FAB/MS) and acylglycines by stable‐isotope dilution analysis. The relative distribution of urinary metabolites was examined in 46 patients with DCA‐uria of different origins, including physiological ketosis of childhood, disorders of propionic acid metabolism, glutaric aciduria type II, Zellweger syndrome and patients who were clinically diagnosed as having Reye syndrome.

Zellweger syndrome seemed to be distinguishable from other disorders by the high sebacic acid/adipic acid ratio of DCA‐uria and increased excretion of 4‐hydroxyphenyllactic acid and 2‐hydroxysebacic acid. The mild form of glutaric aciduria type II was often missed by current organic acid analysis alone, but was readily diagnosed by acylcarnitine and acylglycine determination.

The ratio of free/total carnitine was low in most of the DCA‐uria patients except for two of five cases of Zellweger syndrome and one of three cases of Reye syndrome. The acylcarnitine analysis by FAB/MS showed adipyl‐, suberyl‐, sebacyl‐ or dodecanedioylcarnitine as major peaks in most of these patients, although these were not specific. Disease‐specific peaks were detectable only in congenital organic acidemias such as glutaric aciduria type II, methylmalonic acidemia and propionic acidemia.
Effect of specific immunotherapy in Dermatophagoides pteronyssinus allergic children
Authors: KÖKER ORKIDE , GUNEŞER SEVAL , ALTINTAS DERYA , KOZANOGLU MUSTAFA
Published: January 2011

The effect of immunotherapy on 21 Dermatophagoides pteronyssinus (Dpt)‐sensitive patients (15 children with extrinsic asthma and six children with extrinsic asthma plus allergic rhinitis) aged 8.9±0.71 years was studied. Their subjective and objective respiratory symptom score was recorded before beginning and after the first year of immunotherapy. The respiratory subjective symptoms, such as cough day/night, running nose, sputum production, sneezing, shortness of breath at night and asthma attacks, decreased significantly from pretreatment level (P < 0.05). The objective symptoms, such as wheezing, dyspnea with prolonged expiration, sibilant rales and retractions score, gradually decreased during treatment (P < 0.01). Total eosinophil count decreased in 76.2% of patients after immunotherapy (P < 0.01). While total immunoglobulin E (IgE) decreased in 81% of patients and specific IgE for Dpt decreased in 90.5% of patients, specific IgG for Dpt increased in 76% of patient (P < 0.001). Our results suggested that rising IgG and falling IgE values do not always coexist in the patients who benefit from immunotherapy.
Circadian rhythm of the autonomic nervous system in long QT syndrome
Authors: KATO HITOSHI , YANAGAWA YUKISHIGE
Published: January 2011

The circadian changes in the activity of the autonomic nervous system in a group showing long QT duration in electrocardiogram (ECG) were studied in order to differentiate symptomatic congenital long QT syndrome from asymptomatic. The asymptomatic group presented only long QT duration (QTc > 0.46). Seven girls and two boys, including two subjects experiencing syncope of non‐neurological origins, were examined by using heart rate (HR) power spectrum analysis. In three subjects, the peak of the high frequency band, indicating the parasympathetic activity, disappeared during night‐time (sleep), which means the possibility of a high risk of sudden cardiac death. In two of three subjects, moreover, the averaged sympathetic activity during daytime was significantly increased compared to that during night‐time, in addition to the abrupt increase of sympathetic activity in the morning. These two subjects, in which one boy had a family history of Romano‐Ward syndrome, were the same people experiencing the syncope attack. We succeeded in objectively elucidating that congenital long QT syndrome is adrenergic‐dependent, and suggest that HR power spectrum analysis may be of value in distinguishing symptomatic congenital long QT syndrome from asymptomatic showing only long QT duration.
Erythromycin improves gastrointestinal motility in extremely low birthweight infants
Authors: KUBOTA MASAYA , NAKAMURA TOSHIHIKO , MOTOKURA TAKANORI , MORI SUMITO , NISHIDA AKIRA
Published: January 2011

Erythromycin (EM) was administered to five extremely low birthweight infants (ELBWI) with delayed enteral feeding to evaluate the clinical effect on severely impaired gastrointestinal motility. Five patients studied responded well to EM administration without any adverse effects during the course. Four patients were given 15–30 mg/kg per day EM intravenously as a loading and thereafter 3–5 mg/kg per day as a maintenance dose. One patient responded well without loading. The infants could be fed enterally 4, 5, 6, 4 and 2 days after the initiation of EM administration, respectively. Erythromycin administration is a safe and useful way to facilitate gastrointestinal motility in ELBWI who require prolonged ventilator support with an increased risk for nutrient deprivation.
Transient erythroblastopenia due to human parvovirus B19 infection: A case report of a boy suffering from purpura
Authors: MIYATA HIROSHI , YAGI KAZUROU , TAKEMURA TUKASA , MAKI SUNAO
Published: January 2011

Transient erythroblastopenia of childhood (TEC) was observed in a boy infected by human parvovirus B19 (B19) before the manifestation of the typical clinical picture of erythema infectiosum (EI). He did not have any apparent symptoms of anemia, and spontaneous resolution of erythroblastopenia was observed before the manifestation of the symptoms of EI. The difference between primary B19 infection and classical TEC is discussed.
Effect of surfactant lavage in a rabbit model of meconium aspiration syndrome
Authors: OHAMA YOICHI , ITAKURA YUKINO , KOYAMA NORIHISA , EGUCHI HIDESHI , OGAWA YUNOSUKE
Published: January 2011

Meconium aspiration syndrome (MAS) is a frequent cause of respiratory distress in neonates. Recent reports have suggested that surfactant dysfunction contributes to the pathophysiology of MAS and surfactant therapy improves oxygenation of infants with MAS. We evaluated the effect of bronchial lavage with surfactant solution in a rabbit model of meconium aspiration. All animals were given 5 mL/kg of a 20% slurry of human meconium into the endotracheal tube and mechanically ventilated. The animals were then divided into saline lavage (n = 5) or surfactant lavage (n = 5). Lavage was performed an hour after meconium instillation. After the lavage the total amount of meconium recovered was measured. Blood gas was monitored during the experiment. The amount of meconium recovered by saline lavage was 14%, and by surfactant lavage was 57%. The surfactant group had a significant improvement in gas exchange, whereas the saline group had no improvement. It was concluded that the lavage with surfactant solution effectively washed out meconium and improved gas exchange in rabbit model of MAS.
Ventricle works as a converting organ of atrial blood flow: Physiological significance of mean ventricular pressure
Authors: SENZAKI HIDEAKI , HISHI TOSHIO , UEDA JUNKO , SAKAKIHARA YOICHI , KAMOSHITA SHIGEHIKO
Published: January 2011

The roles and characteristics of the ventricle were examined using mean ventricular pressure (MVP) in ventricular‐vascular assocation.

One hundred and two patients with congenital heart diseases who had undergone cardiac catheterization were studied. They were divided into five groups: Group 1, atrial septal defect without pulmonary hypertension (PH); Group 2, ventricular septal defect (VSD) without PH; Group 3, VSD with PH; Group 4, pulmonary valvular stenosis; and Group 5 as a control group. Then, we examined the relationships between mean pulmonary artery pressure (MPAP) and mean right ventricular pressure (MRVP), and also between mean systemic arterial pressure (MSAP) and mean left ventricular pressure (MLVP) among the five groups. Furthermore, we defined new indicators to express the ease of blood flow through each ventricle. They were referred to as a conductance of the right ventricle (CDR) and a conductance of the left ventricle (CDL), respectively. Then they were compared among the five groups.

The values of MPAP/MRVP and MSAP/MLVP were kept constant to be about 1.3 and 1.7, respectively. Furthermore, CDR was different betweeen each group according to the property of the pulmonary vascular bed, whereas CDL took almost the same value among the five groups.

The ventricle works as a converter of atrial blood flow so that it can achieve efficient blood transport.
A flow cytometric method for measurement of hydrogen peroxide generation by pediatric polymorphonuclear leukocytes stimulated by Staphylococcus aureus and Escherichia coli
Authors: IHARA TOSHIAKI , YAMAWAKI KATSUHIRO , FUJIWARA TAKASHI , KITAMURA KENJI , SAKURAI MINORU , KAMIYA HITOSHI
Published: January 2011

The generation of hydrogen peroxide (H2O2), one of the reactive oxygen species, by polymorphonuclear leukocytes (PMN) stimulated by Staphylococcus aureus and Escherichia coli was studied in infants by cytofluorography. After heparhized whole blood was incubated with bacteria for 60 min, generated H2O2 was measured. The positive rate of H2O2 generation of PMN and mean fluorescent intensity of positive PMN stimulated by S. aureus and E. coli were significantly reduced in infants aged < 1 year and H2O2 generation increased with advancing age. In 10–15 year old children, the level of generated H2O2 reached adult levels. When sera from 1 year old children were added to separated PMN from healthy adults, H2O2 generation was reduced. In contrast, H2O2 generation by PMN from 1 year old children was increased by the addition of adult sera.

These results suggest that the ability to generate H2O2 in response to S. aureus and E. coli is lower in infants and that such reduced activity may be related to the susceptibility of such infants to S. aureus and E. coli infections.
Water balance, electrolytes and acid‐base balance in extremely premature infants
Authors: TAKAHASHI NAOTO , HOSHI JUN , NISHIDA HIROSHI
Published: January 2011

There are few reported studies of water balance and electrolyte abnormalities in extremely low birth‐weight (ELBW) infants weighing < 1000 g nursed in high humidity. We retrospectively analyzed water balance, electrolyte and acid‐base balance in 100 ELBW infants, of whom 72 were appropriate for gestational age (AGA) and 28 were small for gestational age (SGA). They were cared for in closed incubators at almost full ambient humidity. Fluid intake was restricted to 50–60 mL/kg on the first day of life and was adjusted to maintain normal serum Na concentration.

Weight loss in AGA and SGA infants was 21.6 and 16.7%, respectively, and was associated with large urine volume rather than insensible water loss. The incidence of hypernatremia (> 150 mEq/L) and hyponatremia (< 130 mEq/L) was 8.0 and 33.3%, respectively. The incidence of hyperkalemia in AGA infants was 37.0%, and 14.8% in the SGA group. However, hyperkalemia requiring treatment was rare. The incidence of late metabolic acidosis in AGA and SGA infants was 84.6 and 37.5%, respectively (P< 0.01). The difference in water balance and electrolyte abnormalities in AGA and SGA infants needs to be taken into account in managing ELBW infants.
Influence of smoke exposure on serum IgE levels of atopic patients
Authors: ATICI AYTUĜ , GÜNEŞER SEVAL , ALPARSLAN NAZAN , ANTMEN BÜLENT , YILMAZ MUSTAFA , ÖNENLI NESLIHAN
Published: January 2011

The effect of environmental tobacco smoke on serum total immunogobulin E (IgE) of respiratory allergic patients was evaluated in this study. A total of 558 patients with respiratory allergies (311 boys and 248 girls) aged between 1 and 17 years (mean 6.92 ± 3.81 years) were included. None of the subjects were active smokers. IgE levels were significantly higher in children with smoking parents compared with non‐smokers (P < 0.05). Children with smoking mothers were found to have higher levels of IgE than children with smoking fathers. The highest level of IgE was found in children when both parents were smokers (P < 0.005).
A case of maple syrup urine disease misdiagnosed as Tetanus neonatorum on admission
Authors: KÜLTÜRSAY N. , KÜTÜKÇÜLER N. , BÜYÜKGEBIZ B. , KLEIJER WJ
Published: January 2011

A case of Maple Syrup Urine Disease (MSUD) is presented with clinical signs and symptoms on admission resembling neonatal tetanus. Diagnosis had to be differentiated between MSUD and other metabolic disorders and neonatal infections (especially neonatal tetanus because of severe opisthotonos) and generalized seizures of the patient. Early diagnosis of the MSUD patient is very important for effective therapy and better long‐term prognosis as well as genetic counselling and prenatal diagnosis for future pregnancies.
Successful anti‐D immunoglobulin therapy in refractory chronic idiopathic thrombocytopenic purpura showing reduction in thrombocytes even after splenectomy
Authors: SHIBUYA ATSUSHI , DANYA NAOHIRO , SHINOZAWA TAKASHI
Published: January 2011

An 8 year old female child was treated with steroid hormones, anabolic steroid hormones and high dose γ‐globulin therapy for 2 years since being diagnosed with idiopathic thrombocytopenic purpura at the age of 6 years. Treatment produced only transient efficacy, and thrombocytopenia persisted. A scintigram taken 2 years after the onset of the disease using [l25 I]‐labelled heated auto red cells revealed accumulation of radioactivities in the spleen, and therefore splenectomy was performed. However, thrombocytopenia (10 times 109/L) developed again 3 weeks after the operation, for which she was treated again with high dose γ‐globulin with only transient recovery. Then, anti‐D immunoglobulin was injected intramuscularly at 250 μg per dose for a total of four doses and the platelet count was restored to the normal range. Since then her platelet count has been maintained higher than 200 times 109/L for these 11 months. The scintigram taken after splenectomy showed an accumulation of radioactivities in the liver. After administration of anti‐D immunoglobulin, transient subclinical hemolysis appeared. The mechanism whereby anti‐D immunoglobulin exerts the efficacy described here may be considered to be by blockage of Fc receptors of macrophages, as is the case for high dose γ‐globulin therapy. However, since the recovery in the platelet count persisted, it appears that changes in the immune system other than the above described mechanism have contributed to the recovery.
Developmental brain‐stem pathology in sudden infant death syndrome
Authors: TAKASHIMA SACHIO , MITO TAKASHI , YAMANOUCHI HIDEO
Published: January 2011

The brain‐stems of control and sudden infant death syndrome (SIDS) infants were examined developmentally with Golgi and immunohistochemical methods. The development of dendritic spines changed dramatically from the prenatal to postnatal period in the ventrolateral medulla as well as in the reticular formation and vagal nuclei in controls, but persisted in SIDS infants. These observations suggest a delay in maturation of the meduallary respiratory neurons and transneuronal connection between the central chemoreceptor and neural respiratory center in SIDS. In addition, substance P (SP)‐positive nerve fibers were increased in the pons of SIDS infants. An increased activity in the afferent SP neurons in SIDS may be due to chronic hypoxia as in brain‐stem gliosis, and may be involved in cardiorespiratory regulation.
The real status of sudden infant death (SIDS) in forensic medicine
Authors: AKIKO SAWAGUCHI , MASATO FUNAYAMA , TOSHIKO SAWAGUCHI
Published: January 2011

To comprehend the real status of sudden infant death syndrome (SIDS) in forensic medicine, a statistical study was conducted on 156 cases subjected to medico‐legal autopsies at university medical schools throughout Japan from 1987 to 1991 and diagnosed as SIDS. Furthermore, 138 cases that had been subjected to autopsies at the Tokyo Medical Examiner's Office and given the diagnosis of SIDS were evaluated for comparison. It was noted that the incidence of SIDS diagnosed at each facility has increased.