bluuebunny/crossref_metadata_2025 huggingface dataset:
Left and right ventricular volume characteristics in tetralogy of Fallot and their relationship to arterial oxygen saturation and age
Authors: AKIBA TOMOHARU , NAKASATO MITSURU , SATO SATOSHI , ISHIKAWA ATSUKO , SATO TETSUO
Published: January 2011

Left (LV) and right ventricular (RV) volume characteristics in 43 patients with tetralogy of Fallot (TOF) undergoing no prior surgical intervention, aged 3–50 months, were evaluated. The control group consisted of 45 patients with Kawasaki disease without cardiac lesions, aged 12–82 months. The TOF patients were divided into four groups: those having arterial oxygen saturation < 80% with an age at the time of study < 18 months (group 1a) or with that ≧ 18 months (group 1b), and those with arterial oxygen saturation ≧ 80% with an age < 18 months (group 2a) or with that ≧ 18 months (group 2b). The results were compared with those in control subjects. In group 1a, each of LV end‐diastolic volume (EDV), LV ejection fraction (EF), RVEDV and RVEF was reduced. In group 1b, LVEDV, LVEF and RVEF were decreased. In groups 2a and 2b, RVEF alone was depressed. From these results, the severity of hypoxemia was an important risk factor for ventricular dysfunctions. No influence of age on the volume characteristics was found. The investigations suggested that patients with TOF having an arterial oxygen saturation < 80% are probably candidates for early surgical intervention.
Enteric pathogens in severe forms of acute gastroenteritis in Ghanaian children
Authors: HORI HIROKI , AKPEDONU PATIENCE , ARMAH GEORGE , ARYEETEY MARY , YARTEY JULIANA , KAMIYA HITOSHI , SAKURAI MINORU
Published: January 2011

Diarrheal disease is the major cause of childhood morbidity in developing countries. Although malnutrition is known as a risk factor for severe gastroenteritis, the role of enteric pathogens in the clinical severity is unclear. The present study was conducted in well nourished Ghanaian preschool children during a 3 month period of the rainy season to assess the relationship between enteric pathogens and severe gastroenteritis. Two hundred and twenty‐five children with acute gastroenteritis and 64 age‐matched control children were prospectively examined for the severity of dehydration and enteric pathogens in their stools. Of the 225 children with gastroenteritis, 69.8% (157/225) had mild dehydration and 30.2% (68/225) had severe dehydration. Bacteria were similarly isolated in stool samples from children with mild and severe dehydration and controls. Rotavirus accounted for 20.6% of children with severe dehydration and was more often isolated in stools from patients with severe dehydration than those from controls. Furthermore, the mixed infections associated with rotavirus and bacteria were more often found in patients with severe dehydration than those with mild dehydration or controls. Parasites were similarly found at low incidences among the three groups.

The present study implied that rotavirus was more responsible for severe gastroenteritis than bacteria or parasites. However, factors other than enteric pathogens must be sought in a considerable number of severe cases. A large scale study throughout a year is recommended to obtain more precise information that would reflect the seasonal variation of rotavirus infections.
Giant cerebral arterial aneurysm in an infant: Report of a case and review of 42 previous cases in infants with cerebral arterial aneurysm
Authors: KASAHARA ETSUO , MURAYAMA TAKASHI , YAMANE CHIEKO
Published: January 2011

A giant cerebral aneurysm with subarachnoidal hemorrhage in a 4‐month‐old female infant is reported. The clinical course was complicated by an electrolyte imbalance and unexpected intoxication by phenobarbital. She died as a result of the third episode of rerupture of the aneurysm on the 16th day after onset. Histological findings showed that the aneurysm was of the saccular type, 35 × 20 × 22 mm in size, arising from the basilar artery. The aneurysmal wall consisted microscopically of fibrous tissue with partial absence or fragmentation of the internal elastic lamina and muscular lamina. Forty‐three cases of cerebral aneurysms during the first year of life including the present case were summarized and it was found that they could be characterized as follows: (i) a high incidence of onset in early infancy; (ii) larger than 10 mm; (iii) a large number arose from the posterior circulation of the circle of Willis; and (iv) a poor prognosis of non‐operated groups, especially in the posterior circulation.
Neurally mediated syncope complicated with paroxysmal atrial fibrillation
Authors: KATO HITOSHI , MASUTANI SATOSHI , HOSHIYAMA MASAKI , SHIBUYA KAZUHIKO , ISODA TAKAYOSHI , HISHI TOSHIO , MURAKAWA YUJI , YANAGISAWA MASAYOSHI
Published: January 2011

Recently, it has come to be accepted that the result of the tilt test is specific to neurally mediated syncope (vasovagal syncope). Only rarely is a case of paroxysmal atrial fibrillation without any organic diseases in childhood reported. A case reported here of a 14‐year‐old boy with neurally mediated syncope; which was complicated by paroxysmal atrial fibrillation, and which was diagnosed by performing the tilt test. Atrial fibrillation can be induced by the extraordinary stimulation of the vagal nerve during syncope. In a child, neurally mediated syncope complicated with paroxysmal atrial fibrillation has not been previously reported.
A comparison of patients with premature thelarche and idiopathic true precocious puberty in the initial stage of illness
Authors: ARITAKI SEKOIYA , TAKAGI AKIRA , SOMEYA HAYASHI , JUN LI
Published: January 2011

A comparative study of patients with premature thelarche and patients with idiopathic true precocious puberty was conducted. The age at the first visit tended to be lower for those with precocious puberty. In comparison with normative data for children, the frequency of low birthweight and small for date (SFD) status was greater in the 55 patients with premature thelarche, but SFD was also frequent in the 18 patients with precocious puberty. The height, weight and Kaup's index were all within the normal range for these two groups. The ratios for bone age/chronologic age and bone age/height age tended to be high in both groups. In the patients with premature thelarche, the blood luteinizing hormone (LH) level showed a normal response, and the blood follicle stimulating hormone (FSH) level a hyper‐response, to stimulation with luteinizing hormone‐releasing hormone (LH‐RH). In contrast, both the blood LH and FSH levels showed a normal response to LH‐RH stimulation in most of the patients with precocious puberty, and a hyper‐response was rare among them. Although the blood estradiol (E2) level was higher in patients with precocious puberty than in those with premature thelarche, about 50% and 90% of the patients in the respective groups had normal levels. These results suggest that normal responses of blood LH and excessive responses of blood FSH to LH‐RH loading may be useful in some patients for diagnosing premature thelarche at an early stage.
Effect of oral and inhaled salbutamol in infants with bronchiolitis
Authors: CENGİZLİER REHA , SARAÇLAR YILDIZ , ADALIOǦLU GÖNÜL , TUNCER AYFER
Published: January 2011

The response of bronchiolitis to bronchodilator drugs is controversial. The present study was designed to evaluate the efficacy of oral or metered dose inhaler (MDI) salbutamol using a coffee cup as a spacer device in bronchiolitis. In the trial, 31 hospitalized patients between 6 and 24 months of age, who exhibited the first episode of acute bronchiolitis without any other predisposing illness such as cystic fibrosis, congenital heart disease etc., were randomly assigned to receive oral salbutamol (n=11, 0.1 mg/kg per dose, four times a day), or MDI salbutamol (n = 12, 200 μg per dose, every 3 h) or formed the control group without any bronchodilator therapy (n = 8). All of the patients were given supplemental oxygen as needed and adequate hydration was maintained. The patients were evaluated with clinical symptom scores. There were no differences in the beneficial or side effects of salbutamol, or the number of days in hospital between the treatment groups and the control group. It was concluded that there is no beneficial effect in using bronchodilators in infants with bronchiolitis. Supplemental oxygen and maintenance of normal hydration may be adequate.
A case retaining contact urticaria against egg white after gaining tolerance to ingestion
Authors: YAMADA KAZUE , URISU ATSUO , HAGA YOSHIKO , MATSUOKA HIROSHI , KOMADA HIDEKATSU , TORII SHINPEI
Published: January 2011

A girl, 5.7 years old, gained tolerance to egg white ingestion in spite of high immunoglobulin E (IgE) antibody titers to egg white but retained contact urticaria against egg white. She developed atopic dermatitis on her face at 2 months of age and showed high IgE antibody titers to egg white and cow's milk. Accidental ingestion of egg products initiated immediate symptoms such as wheezing, urticaria, erythema and edema of the eyelids and conjunctiva three times. These symptoms were confirmed by challenge tests using boiled egg white at 3.9 years of age. She also reacted positively to a 20 min patch test on her volar arm with raw egg white. However, there were no reactions to the oral challenge test by boiled egg and freeze‐dried egg white at 5.1 and 5.7 years of age, respectively. This non‐responsiveness was confirmed by a double‐blind, placebo‐controlled food challenge using freeze‐dried egg white. Nevertheless, she showed positive reactions to a 20 min patch test with freeze‐dried egg white. Her IgE antibody titers to the egg white components including ovomucoid, ovalbumin, ovotransferrin and lysozyme as well as egg white were high from 2.9 to 5.7 years old. Her IgE antibody titers for the ovomucoid fragments digested by pepsin, chymotrypsin and trypsin were not lower than those of positive control subjects. The binding activity of IgE antibody to ovomucoid, however, decreased from 2.9 to 5.6 years as shown by radioallergosorbent test (RAST) inhibition assays. The IgE antibody showed weaker binding activity to pepsin‐ and chymotrypsin‐digested ovomucoid that were filtered through cut‐off 10 000 filter at the age of 2.1 and 5.7 years. We speculated that the maturation of secretion of digestive enzymes was involved in the mechanisms of the acquisition of tolerance to egg white ingestion in spite of the persistence of contact urticaria.
Clinical trials of new antibiotics in children in Japan
Authors: FUJII RYOCHI
Published: January 2011

Japan is the most advanced of the industrialized nations in the development of antibiotics. Compared to the United States and countries of the European Union, there is a rich selection and availability of new and appropriate antibiotics for patients with infectious diseases in Japan that is unchallenged under a medical system where its people are all covered by a national health insurance plan. This can also be said in the area of antimicrobial treatment of children and newborns.

In Japan, the turning point (T‐point), which the author defines as the point when the average life expectancy of newborns equals that of 1 year olds, was in 1970. Keeping infant deaths from infectious diseases under control was indispensable for this achievement, to which antibiotics had greatly contributed. After the T‐point, another methodology was needed in pediatrics.

The situation in Japan, where most newly developed antibiotics are equipped with statements concerning methods of administration, dosage and safety for children or newborns, differs considerably from overseas situations. The procedures and methods of the clinical trials on children that were performed in strict compliance with good clinical practice are described. Trial studies cannot be performed easily in Japan.

Next, the reason why the clinical trials of the antibiotics in pediatrics were performed and accurately evaluated without incident over 50 years by comparatively small numbers of specialists and facilities is described historically and retrospectively. During the 30 years since modern methods were established, clinical trials of antibiotics with children and newborns have been performed only on essential agents; about one‐half and one‐third, respectively, of the 91 new antibiotics on which clinical trials with adults were conducted. The author has recently published evaluation criteria for clinical studies on antibiotics in the pediatric field. In addition, as the trial's director/administrator, the author states his concept for future clinical development of new antibiotics for children.
Drug compliance of children and infants with oral antibiotics for pediatric use
Authors: IWAI NAOICHI
Published: January 2011

Many oral antibiotics recently available for pediatric use are palatable and easier for children to take owing to the manufacturer's endeavor to improve the preparations. Quite a few drugs, however, still have some problems because they contain bitter active ingredients such as ester‐type β‐lactams and new macrolides. In the present paper, the current status of physicians' prescriptions are discussed, together with the guardians' contrivances for giving prescribed drugs to children and infants, and the drug compliance of pediatric patients. They are discussed in relation to the palatability or internal acceptability of the drugs, which is as important as the drug efficacy and safety. Also discussed are the physicians' countermeasures and guidance to cope with the drug non‐compliance of pediatric patients.
Carnitine depletion during total parenteral nutrition despite oral L‐carnitine supplementation
Authors: HIROSE SHINICHI , HIRATA MASAAKI , AZUMA NAOMI , SHIRAI ZENTARO , MITUDOME AKIHISA , ODA TEIICHI
Published: January 2011

Carnitine (CAR) plays an important role in the β‐oxidation of fatty acids. Less attention, however, has been paid to CAR compared to other nutrients even in total parenteral nutrition (TPN). To examine CAR metabolism during TPN and the effect of simultaneous oral L‐CAR supplementation on CAR levels, the blood CAR level was measured in a 3‐year‐old boy receiving long‐term TPN because of short bowel syndrome. Both the total and acyl CAR in the serum were evaluated under various nutritional conditions including oral supplementation of L‐CAR. Low CAR concentrations were observed especially when lipid containing TPN regimens were in place. Oral L‐CAR supplementation was not sufficient to restore the low CAR levels in the present index patient even when the dose was increased to 120 mg/kg in accordance with the result of the L‐CAR absorption test that revealed poor intestinal absorption of this nutrient. Moreover, a markedly low CAR level was measured during the onset of sepsis in the patient, and the blood CAR was depleted when lipid metabolism was activated by lipid loading or sepsis. To date, the late effects of CAR depletion on child growth have not been well examined. It is recommended that the blood CAR level be maintained at normal levels before any prominent manifestations of the deficiency have developed. The intravenous administration of CAR appears to be necessary to supply a sufficient amount of CAR for patients with severe malabsorption.
Urinary enzyme changes in newborns with unconjugated hyperbilirubinemia
Authors: KAVUKÇU SALIH , TÜRKMEN MEHMET , POLAT MUZAFFER , FADILOǦLU MERAL , AKHUNLAR HÜLYA , ÖZKAN HASAN , ANAL ÖZDEN
Published: January 2011

Various changes in renal function caused by unconjugated hyperbilirubinemia in newborns have been suggested in previous reports. Disclosing an injury in renal tubulus epithelium is feasible by measurement of urinary enzymes. Thus, renal function tests and urinary enzymes in 25 term newborns with unconjugated hyperbilirubinemia were evaluated before and after phototherapy. Ten healthy term newborns without hyperbilirubinemia formed the control group. Mean values of the variables obtained before and after phototherapy in the study group and in the controls were, respectively: urine osmolality (osm/kg H2O); 0.147 ± 0.009, 0.174 ± 0.011, and 0.153 ± 0.018; endogenous creatinine clearance (mL/min per 1.73 m2): 45.7 ± 2.15, 46.0 ± 1.6 and 46.7 ± 3.9; fractional excretion of sodium (%): 1.27 ± 0.30, 0.79 ± 0.19 and 1.24 ± 0.07; tubular phosphorus reabsorption (%): 85.8 ± 3.3, 87.8 ± 2.8 and 86.6 ± 1.7; urinary N‐acetyl‐β‐D glucosaminidase/creatinine (IU/mg): 0.617 ± 0.226, 0.574 ± 0.214 and 0.619 ± 0.210; fractional excretion of alkaline phosphatase (%): 0.422 ± 0.103, 1.001 ± 0.374 and 0.596 ± 0.201; fractional excretion of lactic dehydrogenase (LDH; %): 0.102 ± 0.019, 0.121 ± 0.023 and 0.119 ± 0.041; fractional excretion of AST (%): 0.433 ± 0.127, 0.530 ± 0.113 and 0.502 ± 0.074; fractional excretion of alanine aminotransferase (ALT; %) 0.856 ± 0.413, 1.619 ± 1.076 and 1.066 ± 0.366. No significant difference was found between these values before and after phototherapy in the study group, or between the values before phototherapy in hyperbilirubinemic neonates and in the control group. In conclusion, unconjugated hyperbilirubinemia up to a serum level of 18.4 mg/dL in term neonates does not seem to result in injury of normal tubulus epithelium as shown by urinary enzyme levels.
Effect of weight changes on serum transaminase activities in obese children
Authors: TAZAWA YUSAKU , NOGUCHI HIROO , NISHINOMIYA FUJIHIKO , TAKADA GORO
Published: January 2011

To examine the effect of weight changes on serum transaminase activities, glutamic oxaloacetic and pyruvic transaminases (GOT/GPT), a 3‐month observation of 110 obese outpatients treated by a mild regimen for obesity was carried out. Patients were divided into two major groups, group I (n = 73) and group II (n = 37), with or without persistent elevation of serum GOT/GPT (> 30 IU/L), and retrospectively classified into four subgroups according to weight changes: group A, weight loss > 5%; group B, weight loss of < 5%; group C, an increase of < 5%; group D, an increase > 5%. In group IA, the incidence of cases with normalization of serum GOT/GPT was 70%, and was significantly greater than those of the other three subgroups, respectively (P< 0.01). The incidences of decreased serum GOT/GPT activities were observed corresponding to the degree of weight changes not only in group I (100–27%) but also in group II (100–33%). These facts indicate that a mild regimen for obese outpatients for 3 months, significantly improves serum transaminase activities in patients not only with weight reduction but also weight gain, and that fatty liver may be present even in obese children with normal serum transaminase levels. The normalization of serum GPT activity in patients with weight gain suggests the presence of another factor contributing hypertransaminasemia in pediatric obese patients.
A nationwide surveillance study of rheumatic diseases among Japanese children
Authors: FUJIKAWA SATOSHI , OKUNI MASAHIKO
Published: January 2011

To estimate the number of children with rheumatic diseases, a questionnaire was distributed to the pediatrics department of 1290 hospitals in Japan in June 1994. From this survey, 1606 cases with juvenile rheumatoid arthritis (JRA), 906 cases with systemic lupus erythematosus (SLE), 320 cases with dermatomyositis/polymyositis (DM/PM), 28 cases with scleroderma (PSS), 70 cases with Sjögren's syndrome (Sjs), 93 cases with mixed connective tissue disease (MCTD), 25 cases with aortitis syndrome, 20 cases with polyarteritis (PN) and 51 cases with Behçet disease were reported. The crude annual incidence rates per 100 000 among the childhood population were estimated as JRA, 0.83; SLE, 0.47; DM/PM, 0.16; PSS, 0.01; Sjs, 0.04; MCTD, 0.05; aortitis syndrome, 0.01; PN, 0.01; and Behçet disease, 0.03. The present study reveals that there are more children with rheumatic diseases than are estimated from the reported cases in the literature and the number of children who are receiving Assistance Medical Costs Insurance covered by the Japanese government.
Clinical analysis of 570 cases with juvenile rheumatoid arthritis: Results of a nationwide retrospective survey in Japan
Authors: FUJIKAWA SATOSHI , OKUNI MASAHIKO
Published: January 2011

The purpose of the present study was to investigate the incidence of juvenile rheumatoid arthritis (JRA) among Japanese children and to evaluate the clinical features of this disease. A questionnaire was sent to the department of pediatrics of 1290 hospitals in Japan, in 1994, asking for the number of rheumatic patients during the past 10 years. Subsequently, a second questionnaire was sent asking for the type of onset, clinical features, treatment, and other details. The results of 570 cases were obtained. Of these, 310 cases (54%) were the systemic onset type, 140 cases were the polyarticular onset type (25%), and 120 cases (21%) were the pauciarticular onset type. Hence, in the present series of children, the proportion of the pauciarticular type was less than the other two types of JRA. In the laboratory findings of the systemic onset type, hyperferritinemia and thrombocytosis were noted, in addition to leukocytosis, positive C‐reactive protein (CRP) and accentuated erythrocyte sedimentation rate (ESR). The rheumatoid factor was positive in 50% of patients with the polyarticular onset type. Chronic uveitis was recognized in 13 cases (10.8%) of the pauciarticular onset type. In four girls, uveitis started before the onset of arthritis. Nonsteroidal anti‐inflammatory drugs were used in almost one‐third of cases, and methotrexate (MTX) was used in 12.8% of cases. The quality of life of children with JRA was disturbed in almost 20% of cases. Therefore, for the early and definitive diagnosis of the systemic type of JRA, diagnostic procedures including thrombocyte counts and serum ferritin level, should be performed. In order to obtain good results and to avoid side effects, a protocol for the use of disease modifying anti‐rheumatic drugs and immunosuppressants, especially for the use of MTX, must be established.
Clinical features of Japanese children and adolescents with systemic lupus erythematosus: Results of 1980–1994 survey
Authors: TAKEI SYUJI , MAENO NOBUAKI , SHIGEMORI MASAHIKO , IMANAKA HIROYUKI , MORI HIROSUMI , NEROME YASUHITO , KANEKURA SYOKO , TAKEZAKI TOMOKO , HOKONOHARA MASASHI , MIYATA KOICHIRO , FUJIKAWA SATOSHI
Published: January 2011

Marked advances have been made in the past decade in the management of adults with systemic lupus erythematosus (SLE). Therefore, a nationwide retrospective survey was conducted between 1980 and 1994 to investigate the clinical manifestations of SLE in Japanese children and adolescents. Questionnaires were sent to 340 hospitals. Of 405 patients reported by 176 hospitals, 373 patients, diagnosed by the criteria established by the Pediatric Study Group of the Japanese Ministry of Health and Welfare in 1985, were enrolled in the study. Forty‐nine of the 354 patients (13.8%) had relatives with a connective tissue disease within the third degree of consanguinity. The frequent manifestations in 373 patients were the presence of antinuclear antibody (98.9%), immunologic disorders (93.0%), hypocomplementemia (87.1%), malar rash (79.6%) and fever (74.0%). Lupus nephritis was present in 148 of the 309 patients (47.9%) at their first visit to a clinic, and 261 of the 373 patients (70.0%) developed renal involvement during the observation period. Of 370 patients, 92 patients (24.9%) exhibited central nervous system lupus. Of 368 patients, 192 patients (52.2%) were treated by methylprednisolone pulse therapy and 148 patients (40.2%) received immunosuppressants in combination with steroid therapy at some stage during the observation period. Survival rate at 5 years from onset was 95.9%. Management of infection, coagulopathies, and central nervous system involvement is essential to improve the prognosis of SLE in Japanese children and adolescents.
Characteristics of juvenile dermatomyositis in Japan
Authors: KOBAYASHI SHINICHI , HIGUCHI KAORU , TAMAKI HISASHI , WADA YASUYUKI , WADA NORIYUKI , KUBO MASAKATSU , KOIKE YUICHI , NAGATA MASATO , TATSUZAWA OSAMU , FUJIKAWA SATOSHI
Published: January 2011

Questionnaires were sent to 1290 hospitals in Japan asking for data on patients with juvenile dermatomyositis (JDM) diagnosed between June 1984 and May 1994. Of the 204 patients identified by these questionnaires, 102 met the criteria for JDM. JDM is categorized into three subtypes: Banker‐type JDM, Brunsting‐type and fulminant‐type; patients with the latter exhibit markedly elevated serum levels of creatinine phosphokinase (> 10 000 U/mL) and appear to be at risk of renal failure. Cutaneous manifestations were present in 98% of patients and preceded the appearance of other symptoms. This tendency is one of the reasons for the difficulty in some cases in diagnosing the onset of JDM. Better criteria for early treatment of JDM are needed. The results of the present study suggest that itching and calcinosis are factors that indicate a poor prognosis in patients with JDM. Muscle enzyme levels do not always reflect disease activity, suggesting that methods other than measurement of muscle enzymes, such as measurement of the levels of neoprerin and von Willebrand factor antigen, as well as magnetic resonance imaging should be used to be evaluate disease severity. Patients with Brunsting‐type JDM who exhibit dysphagia and antinuclear antibody positivity and patients with Banker‐type JDM should be treated aggressively. Pulse therapy should be selected as the initial therapy in patients with fulminant‐type JDM.
Non‐furosemide‐related renal calcifications in premature infants with bronchopulmonary dysplasia
Authors: TOFFOLO ANTONELLA , TREVISANUTO DANIELE , MENEGHETTI STEFANO , TALENTI ENRICO , ZACCHELLO GRAZIELLA , ZANARDO VINCENZO
Published: January 2011

Renal calcification is a known complication of long‐term furosemide therapy in infants with bronchopulmonary dysplasia (BPD). In a prospective study the clinical course and long‐term renal sequelae of renal calcifications of 19 consecutive premature neonates (birthweight < 1250 g) with bronchopulmonary dysplasia who did not receive furosemide were examined. Infants were divided into two different groups on the basis of ultrasound evidence of renal calcifications (RC group) or absence of renal calcifications (NRC group). Serial examinations, performed at the age of 1, 2, 3, 6, 9 and 12 months, showed that 12 infants at the mean age of 68.5 ± 12.8 days of life had renal calcifications (63%), and 3 of them had nephrolithiasis; 8 had bilateral renal calcifications. Among the 9 survivors, 2 had chronic renal calcifications at the age of 9 months; however, all normalized at the age of 12 months. Twelve infants received hydrochlorothiazide and spironolactone (63%), 17 had prolonged courses of xanthines and dexamethasone (89.5%), while furosemide was not part of the routine pharmacological administration. Statistical analysis showed that birthweight, gestational age, Apgar score and length of parenteral nutrition were comparable in the RC and NRC group infants. Mean serum creatinine, creatinine clearance, fractional sodium excretion and urinary calcium excretion values during the 12‐month study period were comparable in the RC and NRC groups. Mechanical ventilation and hospital stay length were instead associated with renal calcification occurrence. The strongest indicator of renal calcification risk for this high‐risk population is the severity of the unresolved acute lung disease, where different facets of respiratory management, other than the addition of furosemide, represent sufficient stimuli and renal injury to potentiate stone formation.
Interleukin 8 and granulocyte elastase in the tracheobronchial aspirate of infants without respiratory distress syndrome or intrauterine infection and development of chronic lung disease
Authors: TAKASAKI JIRO , OGAWA YUNOSUKE
Published: January 2011

To elucidate the mechanism of the development of chronic lung disease (CLD) in infants without respiratory distress syndrome or intra‐uterine infection, we serially measured the concentrations of interleukin 8 (IL‐8) and granulocyte elastase α1 proteinase inhibitor complex (E‐α1 PI) and elastase activity in the tracheobronchial aspirate of very low birth weight infants without respiratory distress syndrome or intra‐uterine infection until day 28. IL‐8 concentration and elastase activity between day 21 and 28 in infants who developed CLD later were significantly higher compared with those in infants who did not develop CLD. E‐α1 PI concentration between day 25 and 28 in infants who developed CLD later was significantly higher compared with those in infants who did not develop CLD. The area under the curve of the IL‐8 and E‐α1 PI concentrations and elastase activity between day 1 and day 28 in infants with CLD was significantly higher than those in infants without CLD. These data suggest that the lung tissue injury caused by the enzymes from neutrophils accumulated and activated by IL‐8 also play an important role in the development of this type of CLD.
Platelet activation in congenital heart diseases
Authors: OLGUN NUR , UYSAL KAMER MUTAFOǦLU , IRKEN GüLERSU , üNAL NURETTIN , üNDAR BüLENT , AKKOÇ NURULLAH , AKÇORAL ADNAN , SARIALIOǦLU FAIK , ÇEVIK NAMIK
Published: January 2011

The research presented here investigated platelet activation in cyanotic and acyanotic congenital heart diseases (CHD). Children with cyanotic CHD are prone to both thrombosis and hemorrhage. However, patients with acyanotic CHD may also have a mild bleeding disorder. The platelet activation in CHD was investigated in support of a hypothesis that platelet activation may play a role in the hemostatic abnormalities reported in these patients. Platelet activation was determined by using flow cytometry with anti‐CD62 monoclonal antibody (mAb), which has been shown to be a specific marker of platelet activation. Thirteen children with cyanotic CHD, 33 children with acyanotic CHD and 17 healthy children serving as controls were studied. Platelet activation was significantly higher in the cyanotic group and also in the acyanotic group compared with the healthy children (P = 0.0000 and P = 0.019, respectively). In the cyanotic group, platelet activation showed a direct correlation with arterial O2 saturation (SaO2) (P = 0.014). There was no correlation between platelet activation and erythrocyte related parameters in either group. Platelet activation occurs in CHD, particularly in patients with cyanotic CHD (even in patients with no evidence of clinical thrombosis) and it may play a role in the pathogenesis of thrombotic disorders seen in these patients.
Extrahepatic portal venous obstruction in childhood: Etiology, clinical and laboratory findings and prognosis of 34 patients
Authors: GüRAKAN FIGEN , KOĈAK NURTEN , YüCE AYSEL , ÖZEN HASAN
Published: January 2011

Extrahepatic obstruction of the portal vein is a well known cause of portal hypertension in childhood, that causes severe morbidity. We evaluated 34 children (24 boys, 10 girls, age 4.5 months to 12 years, mean 5.5 ± 3.8 years) with this diagnosis, to define the clinical picture, laboratory changes, diagnostic tools and therapeutic modalities.

Gastrointestinal bleeding was the commonest mode of presentation (64.7%), with the second being splenomegaly. The cause of the obstruction could be determined in 38.2% (13/34) of the subjects. At the beginning of the study the main diagnostic procedure was splenoportography although in more recent years pulsed duplex Doppler ultrasonography has been used. The follow up period was a median of 5 years (range 1–11 years). The mean number of bleeding episodes was 4.7 ± 5.9 (range 1–26), while nine patients never bled. There was no mortality. Ten patients underwent surgery, while sclerotherapy was performed on 10. Twenty‐one patients received beta‐blocker drugs. No difference was found among these therapeutic modalities.

It is well established that the major risk for children with extrahepatic portal vein obstruction is gastrointestinal bleeding which is tolerated quite well. Surgery should be indicated only in children where bleeding cannot be controlled by medical means including sclerotherapy.
Genetic analysis of isolated persistent hypermethioninemia with dominant inheritance
Authors: NAGAO MASAYOSHI , OYANAGI KAZUHIKO
Published: January 2011

We describe a type of mild hypermethioninemia due to a point mutation in the MATA1 gene, which was inherited dominantly in a family. Three patients coming from the same family pedigree were detected by the presence of isolated hypermethioninemia on a mass‐screening program. The measurement of methionine adenosyltransferase (MAT) activity in a patient's liver revealed a partial deficiency of hepatic MAT with a reduction in the Km for methionine. Single strand conformation polymorphism (SSCP) analysis and direct sequencing of the patients' genomic DNA revealed a G to A mutation at nucleotide 791 that converts Arg‐264 to His (R264H) in one allele of MATA1 gene. The other allele was normal in all the patients examined. Gene tracking in the family revealed that the hypermethioninemia is associated with heterozygosity for the R264H mutation in the MATA1 gene.
Transient erythrophagocytosis in Diamond‐Blackfan anemia
Authors: SEKINE ISAO , MASUDA TOMOO , KAWAMURA YOUICHI , DOBASHI HIROSHI , KOGAWA KAZUHIRO , YOSHIOKA SHIGETAKE
Published: January 2011

We report on a 4‐month‐old Japanese infant girl with Diamond‐Blackfan anemia (DBA) as shown by congenital macrocytic pure red cell hypoplasia with marked reduction of erythroid precursors in bone marrow, reticulocytopenia, increased fetal hemoglobin, and elevated adenosine deaminase activity in peripheral blood. She responded poorly to conventional doses of corticosteroids, however, with high‐dose corticosteroids she responded with reticulocytosis and an elevation of hemoglobin level above 12 g/dL. Erythrophagocytosis was noted during the tapering period of prednisone when her hemoglobin level declined to 7.6 g/dL and reticulocyte level to 0.4%. At that time, the erythrophagocytosis was noted in about 60% of marrow histiocytes. These findings were not observed prior to or during the high dose prednisone therapy. We speculate that one of the causes of pure red cell aplasia and reticulocytopenia in DBA is mediated by erythrophagocytosis.
A case of X‐linked α‐thalassemia/mental retardation syndrome: Analysis of hemoglobin by an automated glycated hemoglobin analyzer
Authors: KUNO TATEO , IDEGUCHI HIROSHI , YOSHIDA NOBUYUKI , MASUYAMA TATSUO , OHTA MITSUHIRO , NISHIMURA SHINJI , TASAKI HAKARU , MIYAZAKI SUMIO , HARA HIROMICHI , MATSUMOTO KAZUHIRO
Published: January 2011

A 5‐year‐old male patient with X‐linked α‐thalassemia/mental retardation syndrome is reported. He showed multiple minor anomalies including characteristic facial abnormalities, α‐thalassemia, severe mental retardation, and hypogonadism. Analysis of his hemoglobin by high performance liquid chromatography using an automated glycated hemoglobin analyzer revealed an abnormal peak. Identification of an abnormal peak by an automated glycated hemoglobin analyzer will aid in the diagnosis of patients with X‐linked α‐thalassemia/mental retardation syndrome.
Imipenem for treatment of relapsing Salmonella meningitis in a newborn infant
Authors: KOĈ ESIN , TURKYILMAZ CANAN , ATALAY YILDIZ , SEN EMEL
Published: January 2011

Salmonella meningitis is a rare clinical entity that occurs mainly during early infancy. Treatment of Salmonella infections may be complicated by the bacteria's growing resistance to clinically important antimicrobial agents, especially third‐generation cephalosporins. A report is presented of a newborn infant with Salmonella meningitis who relapsed after 4 weeks of cefotaxime treatment and was cured completely with imipenem cilastatin therapy.
Familial atrial septal defect with prolonged atrioventricular conduction
Authors: GüNAL NAZLIHAN , GüL SEHER , KAHRAMANYOL ÖZ
Published: January 2011

A family is described in which a mother, her two children and another two relatives all had atrial septal defect of the ostium secundum type. The index case and her mother also had prolonged atrioventricular conduction. It is considered that the defect is the familial type and inherited as a mendelian dominant trait.
Linkage analysis and identification of deletion in Alagille syndrome gene
Authors: YUAN ZENG‐RONG , KOHSAKA TAKAO , KOBAYASHI NOBORU
Published: January 2011

Alagille syndrome (AGS) is a genetic disease and the responsible gene has already been mapped at 20p12. To more accurately detect the region of the AGS gene on the linkage map of chromosome 20p, 14 yeast artificial chromosome (YAC) clones were screened to construct a YAC contig in the candidate region and 13 locus markers and 2 sequence‐tagged sites (STS) were ordered. Combining all of the analyses, a 1.3 Mb critical region from D20S507 to D20S61 for the AGS gene was identified. As the human Jagged 1 gene (JAGI) lies just in this region and is responsible for the AGS disease, the genomic DNA in an AGS family without a visible deletion were analyzed by single‐strand conformational polymorphism (SSCP) and direct DNA sequencing, and a 2‐bp (CT) deletion mutation at exon 26 of the JAGI was identified.
Role of provocation poliomyelitis in vaccine‐associated poliomyelitis
Authors: EHRENGUT WOLFGANG
Published: January 2011

The impact of different vaccine administrations in the Federal Republic of Germany, in relation to vaccine‐associated paralytic poliomyelitis (VAPP) was studied. (This only means that on the basis of the temporal relation of vaccination and onset, the vaccine cannot be ruled out as possible cause.) Oral polio vaccine (OPV) was given between 1963 and 1977 only and recommended simultaneously with inactivated vaccines (DT/DPT) between 1980 and 1985. In the first period 10 VAPP cases occurred among 9.96 million estimated vaccinees aged under 2 years. In the second period 6 cases of provocation poliomyelitis (PRP) were found among 2.9 million estimated OPV/DT/DPT vaccinees of the same age group. Statistically the VAPP/PRP frequencies in the two periods studied did not achieve significance.
High incidence of breakthrough varicella observed in healthy Japanese children immunized with live attenuated varicella vaccine (Oka strain)
Authors: TAKAYAMA NAOHIDE , MINAMITANI MIKIO , TAKAYAMA MICHIKO
Published: January 2011

In order to know the rate of occurrence of varicella among vaccinees (breakthrough varicella: BV), questionnaire postcards were sent to 593 healthy children who had received varicella vaccine (Oka strain) from March 1987 to December 1989. The questionnaire survey was repeated once a year until January 1996. The annual attack rate from the 1st to 3rd questionnaire was approximately 12%; however, from the 5th to 8th one it was 1–4%. To February 1996, the cumulative attack rate was 157/459 (34.2%). This rate was comparable to that among vaccinees who had confirmed seroconversion; namely, 51/132 (38.6%). These rates are much higher than those reported by other authors. All BV cases were clinically mild; even subjects who had received the vaccine 7 years prior to the disease showed mild symptoms. The high incidence may be partly explained by the regional epidemiology of varicella. The decrease in annual incidence with time after vaccination may be due to the following reasons: some vaccinees remained free from BV owing to reinforcement of their immunity from subclinical infection of varicella‐zoster virus (VZV) and others from diminution of opportunity for exposure to VZV with increasing age. Varicella vaccine seems to be effective in modifying the symptoms of varicella, but not potent enough in protecting from VZV infection.
Early cardiac failure in a child with Becker muscular dystrophy is due to an abnormally low amount of dystrophin transcript lacking exon 13
Authors: ISHIGAKI CHIEKO , PATRIA SURYONO YUDHA , NISHIO HISAHIDE , YOSHIOKA AKIRA , MATSUO MASAFUMI
Published: January 2011

Two Japanese brothers with Becker muscular dystrophy were shown by polymerase chain reaction (PCR) and cDNA sequence analysis to produce a dystrophin gene transcript lacking a single exon; that is, number 13. Despite having the same deletion mutation, the brothers showed clearly different clinical phenotypes: the younger brother developed cardiac failure at the age of nine, while the elder brother was asymptomatic. As alternative splicing was not responsible for this clinical difference, the amount of dystrophin transcript was examined by using reverse transcription semi‐nested and parallel PCR. The results showed that the amount of the dystrophin transcript in the younger brother was 20% of that of the elder brother. This finding suggested that lesser amount of dystrophin transcript in the younger brother was responsible for the early onset of cardiac failure. This would represent a novel molecular mechanism for dystrophinopathy.
Early detection of prune belly syndrome in utero by ultrasonography
Authors: KOBATA RITSUYO , TSUKAHARA HIROKAZU , TAKEUCHI MOTOHIRO , HORI CHIKAHIDE , HIRAOKA MASAHIRO , UCHIDA TOSHIYUKI , KOTSUJI FUMIKAZU , ITO YASUHIKO , OKADA KENICHIRO , SUDO MASAKATSU
Published: January 2011

A report is presented of a male infant with prune belly syndrome (PBS) in whom bladder enlargement was detected by ultrasound (US) as early as 13 weeks of gestation. Subsequent fetal US identified progressive urinary tract dilatation, ascites and oligohydramnios. At 22 weeks, the fetal bladder was drained under US guidance. A gradual resolution of oligohydramnios was detected on US performed after 26 weeks of gestation. Delivery by cesarean section was performed at 34 weeks of gestation. The newborn had typical features of PBS with a vesico‐cutaneous fistula but did not show respiratory distress. Imaging studies showed hypoplastic left kidney, slightly dysplastic but functioning right kidney, megaureter, megacystis, vesicoureteral reflux and dilated prostatic urethra. The early detection of genitourinary system abnormalities and serial US suggest that a distal urethral obstruction may be the underlying mechanism of PBS. Spontaneous (or therapeutic) intra‐uterine decompression of the bladder might ameliorate obstructive nephropathy and oligohydramnios, allowing adequate lung development.
Trends in neonatal exchange transfusions at Yodogawa Christian Hospital
Authors: FUNATO MASAHISA , TAMAI HIROSHI , SHIMADA SEIICHI
Published: January 2011

A review was conducted to determine the trends in exchange transfusion (ET) of newborn infants at the Yodogawa Christian Hospital during the past 18 years. At that hospital in 1957, the first ET was performed on a term infant with severe hemolytic jaundice caused by rhesus factor (Rh) incompatibility. By 1989, ET had been performed in more than 1400 newborn infants. These cases of newborns who had had ET were retrospectively reviewed, with a focus on every 3 year period from 1974 to 1992. The total number of infants requiring ET noticeably decreased from 68 cases (14.0% of total admissions) in 1974 to 19 cases (6.1% of total admissions) in 1992. (X2, P < 0.001) There were three major significant changes in ET during those years. The first was a change in the subjects for ET. The incidence of ET for term infants showed a marked decrease, while the incidence of ET for preterm infants, especially for very low birthweight (VLBW) infants (< 1500 g), noticeably increased. The second was a change in indications for ET. There was a marked decrease in the need for ET as a result of hyperbilirubinemia, while the incidence of ET because of other etiologies, such as septicemia and/or disseminated intravascular coagulopathy, noticeably increased. The third was a change in the technical methods of ET. Now at the Yodogawa Christian Hospital, 100% of the infants are given ET with an automated peripheral two‐site method, instead of the Diamond method. Although ET might still be a useful treatment for severe hyperbilirubinemia and other acute problems, the total number of ET noticeably decreased in accord with a decrease in the number of severe hyperbilirubinemia in term newborns. On the other hand, the incidence of ET in preterm infants increased relatively, accompanied by an increase in the survival of VLBW infants. The automated two‐site method is the preferred technique for ET at the Yodogawa Christian Hospital, rather than the Diamond method. Further changes in ET might occur in accord with new alternative measures in future.
Prevalence of asthma, rhinitis and eczema among schoolchildren in Kelantan, Malaysia
Authors: QUAH BAN SENG , RAZIF ABDUL , RAZAK ABDUL , HASHIM MOHAMAD , HASSAN MOHD
Published: January 2011

The prevalence and severity of asthma, rhinitis and eczema in Kelantanese schoolchildren were determined as part of an international study of the epidemiology of asthma and allergic diseases. The international study of asthma and allergies in childhood (ISAAC) written questionnaire was administered to 7055 schoolchildren from February 1995 to August 1995. The respondents were parents or guardians of 5‐ to 7‐year‐old children (n = 3939), and schoolchildren aged 12–14 years (n = 3116). The ISAAC video questionnaire (AVQ3.0) was shown to children aged 12–14 years after the written questionnaire. The overall prevalences of ‘ever wheezed’ and ‘wheezing in last 12 months' were 9.4 and 6.0% respectively. The prevalence of ‘ever diagnosed with asthma’ was 9.4%. Both ‘ever wheezed’ and ‘wheezing in the last 12 months’ were significantly higher in 12‐ to 14‐year‐old children than in 5‐ to 7‐year‐old children, with P values of 0.0006 and 0.014 respectively. No gender differences in the prevalences were observed. For the complete study group, 4.7% of children had sleep disturbed by wheezing but only 1.1% had a severe attack limiting speech in the preceding 12 months. Sleep disturbance was more common in the 12‐ to 14‐year‐old children than in 5‐ to 7‐year‐old children (P = 0.006). There was no difference between the age groups for severe attacks limiting speech. The overall prevalence of rhinitis and eczema symptoms were 27 and 12%, respectively. The prevalence of rhinitis in the 12–14 year age group (38.2%) was significantly higher (P < 0.0001) than in the 5–7 year age group (18.2%). The prevalence of eczema in the 5–7 year age group (13.7%) was significantly higher (P = < 0.0001) than in the 12–14 year age group (9.9%). These prevalence data are comparable with previous reports in Malaysian children, but are considerably lower than those reported for most developed countries
Serum zinc, copper, and magnesium levels in obese children
Authors: YAKINCI GENGİZ , PAÇ AYŞENUR , KüÇüKBAY F ZEHRA , TAYFUN MELTEM , GüL ABDüLKADİR
Published: January 2011

Serum zinc, copper and magnesium levels were measured using atomic absorption spectrophotometry in 41 obese and 41 healthy (control) children (between 7 and 11 years of age) who were living in Malatya (Turkey). Serum zinc levels of obese children (mean value 102.40 ± 2.78 μg/dL) were found to be significantly higher than those of the control group (mean value 80.49 ± 2.98 μg/dL; P < 0.01). Serum copper concentrations were also found to be significantly higher in obese children (mean value 132.34 ± 1.79 μg/dL) than the healthy controls (mean value 107.58 ± 1.62 μg/dL; P < 0.01). On the other hand, serum magnesium levels were found to be significantly lower in obese children (mean value 1.78 ± 0.03 mg/dL) than in the healthy children (mean value 2.14 ± 0.04 mg/dL; P < 0.01).
The effects of long‐term anticonvulsive treatment on serum lipid profile
Authors: YALÇIN ERDINÇ , HASSANZADEH AHMAD , MAWLUD KHAYRYA
Published: January 2011

Serum lipids were determined in 10 untreated patients with recently diagnosed epilepsy, 21 patients treated with carbamazepine (CBZ), 10 patients treated with valproate (VPA) and in 15 healthy children. In relation to the controls, patients receiving CBZ showed increased serum high‐density lipoprotein cholesterol (HDLc), apolipoprotein A (Apo‐A) and apolipoprotein B (Apo‐B). Patients receiving VPA showed increased Apo‐B levels. There were no significant differences in total cholesterol (TC), triglycerides (TG), low‐density lipoprotein cholesterol (LDLc) or very low‐density lipoprotein cholesterol (VLDLc) between all groups. The changes in lipid metabolism may be associated with the induction of liver enzymes during anti‐epileptic drug metabolism. The CBZ‐induced change in serum lipid levels was considered to be a possible factor against atherosclerosis and coronary heart disease in epileptic patients.