bluuebunny/crossref_metadata_2025 huggingface dataset:
Bone marrow transplantation for late infantile metachromatic leukodystrophy: Pathogenic investigation for graft rejection
Authors: NAGAI TOSHIRO , KANEKO TAKASHI , SHICHIJOU HIROMI , KARATO TAKAKO , MARUYAMA AKIKO , TSUCHIYA YUTAKA
Published: January 2011

A Japanese boy aged 2 years 11 months with late infantile metachromatic leukodystrophy underwent bone marrow transplantation (BMT) from his human leukocyte antigen (HLA) identical but mixed lymphocyte culture reactive father. Chimerism and increased arylsulfatase A activities of leukocytes had been observed with retarded progression of neurological deterioration during the first 3 months post‐BMT. Graft rejection gradually occurred and donor cells were almost completely eliminated from the patient at 1 year after BMT. The process of neurodegeneration progressed clinically and neuroradiologically. Three possible reasons for the pathogenesis of graft rejection are: (i) T cell depletion of donor marrow cells as graft‐versus‐host disease (GVHD) prophylaxis; (ii) a slightly weak conditioning regimen; and (iii) a small number of marrow cells transplanted. It is stressed that as BMT is still a preliminary therapy for metachromatic leukodystrophy indications, conditioning, and GVHD prophylaxis for BMT should be considered individually.
Asymptomatic Becker muscular dystrophy: Histological changes in biopsied muscles
Authors: TACHI NOBUTADA , WATANABE YUKIE , OHYA KAZUHIRO , CHIBA SHUNZO
Published: January 2011

The histological changes observed in consecutive tests of biopsied muscles from an asymptomatic patient with Becker muscular dystrophy (BMD) are reported. The first biopsy showed active muscle necrosis and clustered regenerating fibers. The second biopsy revealed chronic myopathic changes mixed with neurogenic involvement. It is stressed that active myogenic changes observed in younger and asymptomatic BMD patients were followed by chronic myogenic or neurogenic change with age. Finally, we confirm the necessity of checking for dystrophin of abnormal size or reduced abundance by immunoblot analysis.
Estimation of myocardial damage in Kawasaki disease using antimyosin antibody
Authors: NOMURA YUICHI , YOSHINAGA MASAO , OKU SHOZO , KONO YUKIHARU , YUASA YOSHIHIRO , NODA TAKASHI
Published: January 2011

In a retrospective study, 121 children with Kawasaki disease (KD) were investigated to determine (i) the incidence of myocardial damage using the antimyosin antibody (AMA) titer; (ii) the differences in the electrocardiograms between the AMA‐positive and ‐negative patients; and (iii) the effect of treatment with intravenous gamma globulin (IVGG) on the AMA. Comparisons were made with 117 normal children (controls).

Patients with KD showed a significantly higher mean AMA titer and more patients were positive for AMA than the controls. The AMA titer in the KD group was not related to the presence of coronary artery lesions. Electrocardiograms obtained during the acute and the convalescent stage of KD revealed that patients positive for AMA had a significantly lower voltage of T wave in lead V6 at week four than at week two of illness, whereas patients negative for AMA showed no T wave change after week two. The group treated with IVGG showed a significantly lower AMA titer than that not given IVGG.

These observations suggest that myocardial damage occurs in some patients with KD which is unrelated to the presence of coronary artery lesions and that the treatment with IVGG reduces the AMA titer in patients with KD.
Stroke and meningitis in a case of SLE with anti‐phospholipid antibodies
Authors: WATANABE TOHRU , SATOH MASAHISA , ABE TOKINARI , HAYASHI MIWAKO , ODA YOSHIHIKO
Published: January 2011

Anti‐phospholipid antibodies (aPL) are concerned with many central nervous system diseases in systemic lupus erythematosus (SLE). However, no report has described the relationship between aseptic meningitis and aPL in SLE. We report a case of SLE with aPL, presenting cerebral infarction and aseptic meningitis.

A 14 year old female with SLE with aPL experienced cerebral infarction and recurrent aseptic meningitis. Combination therapy with steroids and aspirin improved the condition and prevented relapses.

The aPL are associated with cerebral infarction, even in young patients with SLE. In addition, aPL may induce aseptic meningitis in SLE.
Immunological aspects of juvenile rheumatoid arthritis
Authors: YOSHINO KAZUYA
Published: January 2011

This article reviews the evidence from recent studies on immunological abnormalities associated with pathophysiologic mechanisms operating in three clinical subtypes of juvenile rheumatoid arthritis (JRA) (polyarticular, pauciarticular and systemic). The main discussion is focused on three hallmarks of immunopathological studies. First, abnormalities in phenotype and function of lymphocytes from peripheral blood and inflamed synovium are discussed. The aberrations of lymphocytes are elucidated by T and B cells expressing phenotypic cell‐markers such as CD20, CD21, CD4, CD8 and DR in association with different subtypes and disease activity. The functional imbalance and impairment of T and B cells are mainly observed by abnormal proliferation and/or in vitro Ig production in response to mitogens and alloantigens. Second, because the appearance of rheumatoid factors (RF) in serum indicates that the pathogenesis of JRA may be based on the autoimmune mechanism, the prevalence of RF including IgM, IgA and IgG isotype, hidden IgM RF and cross‐reactive idiotype RF, and their characteristic properties are discussed. Moreover, specific auto‐antibodies (antinuclear antibodies and others) for JRA are illustrated in this paper. Third, the production of various pro‐inflammatory cytokines resulting in the release of tissue‐damaging chemical mediators is also discussed. This may play a central role in the generation of systemic inflammation and joint involvement in JRA.
Non‐steroidal anti‐inflammatory drugs and slow‐acting anti‐rheumatic drugs in juvenile rheumatoid arthritis
Authors: FUJIKAWA SATOSHI
Published: January 2011

The preferred drugs for the initial treatment of juvenile rheumatoid arthritis (JRA) are salicylates or other non‐steroidal anti‐inflammatory drugs (NSAID) such as tolmetin or naproxen. If the disease activity does not respond adequately to the treatment, slow‐acting anti‐rheumatic drugs (SAARD) such as oral gold agents, low‐dose D‐penicillamine, or sulfasalazine should be given in addition to NSAID. If the systemic manifestations are severe, corticosteroid therapy may be commenced. Furthermore, if the joint destruction is progressive, immunosuppressants such as methotrexate would be selected as the third‐line drugs of choice.

The safety and efficacy of SAARD and immunosuppressants for the treatment of children with JRA, however, have not yet been confirmed, as the adverse effects such as bone marrow suppression, oncogenicity and mutagenicity are sometimes intense. Consequently, the strict indications for use and new therapeutic concepts for the management of JRA based on its pathogenesis are required.
Quality of life and daily management of children with rheumatic disease
Authors: TAKEI SYUJI , HOKONOHARA MASASHI
Published: January 2011

Appropriate management of the daily life of patients with rheumatic diseases is an important part of therapy for favorable results and a high quality of life.

At home, gymnastic exercise, night splints and appropriate fun activities will help to preserve the function of affected joints of patients with juvenile rheumatoid arthritis (JRA). Improvement of home fixtures according to patient disability will provide a higher quality of life for the patients.

The patient should be helped to perform as many school activities as would normally be possible. Instructional charts for management of school life for patients with JRA and systemic lupus erythematosus is shown in this article. Cooperation with school personnel is also crucial to support the patient emotionally and physiologically.

For the best long‐term treatment and a good relationship between patient and physician, comprehensive management is essential. Not only physicians, but also psychiatrists, occupational therapists, physical therapists and social workers should be involved in the treatment of children with rheumatic diseases.
Mixed connective tissue disease in childhood
Authors: YOKOTA SHUMPEI
Published: January 2011

Since 1981, 21 children diagnosed with mixed connective tissue disease (MCTD) have been reported in detail in the literature in Japan. Overlapping clinical features and laboratory findings of these children were analyzed according to the established criteria of the Ministry of Health and Welfare, Japan. Mixed connective tissue disease in childhood typically begins with Raynaud's phenomenon preceded by several months or years with the appearance of other symptoms and signs including fever, arthralgia, myalgia, and/or progressive systemic sclerosis like skin manifestations. Serologically all the children with MCTD were positive for anti‐ribonuclear protein (RNP) antibody with speckled‐type antinuclear antibody. Hypergammaglobulinemia, positive rheumatoid factor, and normocomplementemia were characteristic. In general, prognosis is considered to be fairly good as opposed to systemic lupus erythematosus in childhood, but severe pericarditis/myocarditis or nephrotic syndrome can occur. Long‐term follow‐up study and improved laboratory detection of anti‐ribonucleoprotein antibodies will be necessary for further characterization of MCTD in childhood, and for the improvement of therapy.
Usefulness of a new perchloric acid treatment method to measure endotoxin in cerebrospinal fluid by the limulus test
Authors: SUDA HIDETOSHI , INADA KATSUYA , YOSHIDA MASAO
Published: January 2011

The modernized limulus lysate assay on cerebrospinal fluid pretreated by the new perchloric acid (PCA) treatment was evaluated in one patient with Escherichia coli meningitis and four non‐meningitis patients. The endotoxin (ET) concentration was much higher by the new PCA treatment than by the ordinary dilution method in the exacerbation phase of E. coli meningitis. The recovery rate of added ET also demonstrated the superiority of the new PCA treatment.
Cord blood erythropoietin in the presence and absence of labor in normal infants
Authors: KALMANTI M. , DIMITRIOU H. , KARASTAMATIS S. , KOUMANTAKIS E. , KARKAVITSAS N. , RELAKIS K. , ATHITAKIS N. , SPYROPOULOU A. , KALMANTIS Th
Published: January 2011

To evaluate erythropoietin (Ep) levels in normal labor and cesarean section we studied the cord serum of 111 term pregnancies, divided into three groups: (i) those born by normal vaginal delivery (n = 69); (ii) those delivered vaginally from mothers who were smokers (n = 20); and (iii) those delivered by elective cesarean section (n = 22).

The three groups did not differ in maternal age, gestational age, birthweight, infant sex and Apgar scores. No correlation was found between Ep and hematocrit in all three groups of normal terms studied. Although not statistically significant the lower mean Ep value observed in cesarean section compared with the values obtained from normal deliveries could suggest that the process of labor may be a cause of these differences.
Leukemoid reaction induced by prenatal administration of betamethasone
Authors: COHEN AMNON , BARAK MILA , HERSCHKOWITZ SILVIA , ZECCA SILVIA
Published: January 2011

A case of leukemoid reaction in a premature baby whose mother was antenatally treated by betamethasone is reported. The neonatal and maternal factors which might lead to leukocytosis or leukemoid reaction were excluded. The presented case is the last in a series of eight previously reported cases of leukemoid reaction, in which the common denominator was the antenatal administration of betamethasone.
International cooperation of Japanese pediatricians with Cairo University Pediatric Hospital
Authors: OKAMATSU TAKAO , MURAKAMI ATSUBUMI , GOMI AKIRA , KAZUMA HIROSHI
Published: January 2011

In connection with ‘Child Year’ in 1980, an agreement on the construction and donation of a pediatric hospital was concluded between the Egyptian and Japanese Governments.

Under this agreement, the Cairo University Pediatric Hospital (CUPH) was opened in March 1983, as a four‐storey building with 240 beds, and including four operating rooms, an intensive care unit (ICU) and neonatal ICU.

The technical cooperation and supply of medical equipment in all areas of CUPH is ongoing during this project. The subject matter of technical cooperation is as follows.

(1) Dispatch of Japanese experts to Egypt for short and long terms.

(2) Acceptance of Egyptian trainees to Japan.

(3) Supply of medical equipment and materials.

Through the first 5 years of this cooperation, the management of ICU, operating theaters, medical and surgical wards has improved markedly with great efforts from the Egyptian and Japanese staff.

Based on this successful cooperation, the Pediatric Cardiovascular Center was established in 1988 with a two‐storey extension in the hospital including two cardiac operation theaters, ICU and other special facilities.

Now, CUPH has become the center of pediatric medical care in not only Egypt but also the surrounding Arabic countries, supported by the friendly and close relationship between Egypt and Japan.
Infectious diseases in African children
Authors: HORI HIROKI , WATANABE MASAHIRO , SAKURAI MINORU
Published: January 2011

Present status and problems of infectious diseases in African children are detailed. The Department of Paediatrics, Mie University School of Medicine has 10 years' experience of international medical cooperation with African countries. At present, the department is participating in two projects in Ghana and Zambia. The activities have been carried out in the field of priority infectious diseases in African children. Major infectious diseases in Africa are malaria, diarrhoeal diseases, acute respiratory infections and some specific parasitic diseases. Human immunodeficiency virus infection has also become a threat to the health and survival of children in Africa. To reduce morbidity and mortality due to these diseases, primary health care activity may be an effective and economical measure. Japan is expected to make further technological and economical contributions to the control of the infectious diseases in developing countries. Japanese paediatricians should be aware of the condition of child health in developing countries and consider what can be done to help.
JICWELS' MCH training program in the Aiiku Institute: Asian MCH workshop
Authors: HIRAYAMA MUNEHIRO , OYAMA OSAMU , ASANO MARIKO
Published: January 2011

Taking a form of Official Development Aid (ODA), the Japan International Cooperation of Welfare Services (JICWELS) and Imperial Gift Foundation, Boshi‐Aiiku‐Kai (Aiiku Association for Maternal and Child Health and Welfare) have extended a study program on maternal and child health (MCH) since 1989 on the commission of the Ministry of Health and Welfare. ‘Community participation’ is the key to the first international study program focused solely on MCH.

The purpose of the program is to help to imporve the planning and administration in the field of MCH. Through this, the information and experience attained in Japanese MCH activities are introduced especially by participation in community‐level activities of ‘Aiiku‐Han’ in which local citizens play a major role.

The operation system of the Asian MCH Workshop, contents of the workshop, evaluation and future prospects are discussed.
Cooperative studies on diarrheal diseases
Authors: TAKEDA TAE , BHATTACHARYA SUJIT K , NAIR G BALAKRISH
Published: January 2011

Enteric infection still causes the highest global morbidity and mortality in children under 5 years old.

While there have been impressive developments in the early treatment of diarrhea, adequate diagnostic techniques are not yet available. An international collaboration was conducted with the National Institute of Cholera and Enteric Diseases, Calcutta on the rapid diagnosis and prevention of diarrheal diseases. Forty to fifty per cent of the 780‐bed Infectious Diseases Hospital is occupied by cases of diarrhea. Vibrio cholerae OI continues to occupy a prominent position, and the prevalence in children up to 2 years is 30.9%. Accordingly, immuno‐enzymatic detection of cholera toxin in stool was achieved. Bead enzyme‐linked immunosorbent assay (ELISA) could successfully detect 40 pg/mL toxin within 4 h, but the detection rate of culture, bead ELISA and polymerase chain reaction (PCR) method was 72.2, 71.1 and 95.9%, respectively, indicating that PCR provides the most sensitive and specific assay for diagnosis of cholera directly from the stool of patients.

Heat‐stable enterotoxin (STa) is another virulence factor of gastroenteritis. Various monoclonal antibodies were established against it, and developed a competitive ELISA for the detection of STa producing strains. A DNA probe was also prepared, and genus vibrio was monitored. V. mimicus were found to be the reservoirs of STa among clinical vibrios. Various monoclonal antibodies against STa were also useful for the epitope mapping, providing information on the topology of the toxin‐host interaction.
Developmental change in activity of red cell porphobilinogen deaminase and its electrophoretic variant in the Japanese population
Authors: NAMBA HIROSHI , NARAHARA KOUJI , TSUJI KAZUSHIRO , YOKOYAMA YUJI , MURAKAMI MASAE , MATSUBARA TSUNENORI , SEINO YOSHIKI
Published: January 2011

The activity of porphobilinogen deaminase (PBGD), an enzyme whose partial deficiency is associated with acute intermittent porphyria (AIP), changes during development. Little is known about the postnatal change of PBGD activity and the prevalence of its electrophoretic variant in the Japanese population.

The activity of PBGD was measured fluorometrically in 194 infants aged 0–12 months, while isoelectric focusing of PBGD was performed in 400 healthy Japanese adults aged 20–45 years and 30 children with various hematological disorders aged 1–15 years.

The PBGD level was 1.9 times higher in the neonates than in the adults, decreased abruptly during the first month of life, and reached the adult level at the age of 9 months. None of the 400 healthy Japanese adults and the 30 children with hematological disorders showed any electrophoretic variant.

These results suggest that there is no need to consider any polymorphism in the gene dose study of PBGD and that the biochemical screening of AIP is applicable to since the late infancy.
Dose‐related protective efficacy of immunoglobulins in experimentally induced group B streptococcal infection
Authors: IGUCHI KOSEI , INOUE SUSUMU
Published: January 2011

This study investigates the dose‐dependent effect of administration of commercially available intravenous immunoglobulins (IVIG) on the survival of newborn rats experimentally infected with group B streptococci (GBS). The opsonic activity of various concentrations of IVIG in vitro was determined by examination of opsonophagocytosis of GBS by peritoneal macrophages and bacterial killing by blood neutrophils.

The best survival was observed in newborn rats who received immunoglobulins at doses of 250, 500 and 1000 mg/kg of bodyweight. The survival of animals that received either 125 mg/kg or 2.5 g/kg of immunoglobulins was no better than that of animals who received no immunoglobulins. The maximal phagocytosis and bacterial killing were observed at in vitro immunoglobulin concentrations ranging from 32 to 250 mg/dL (these in vitro concentrations may correspond roughly to in vivo administration doses ranging from 150 to 1200 mg/kg). These doses are comparable to the doses of IVIG currently administered to neonates. However use of very high doses may be harmful to babies since the high concentration of non‐specific immunoglobulins inhibited in vitro phagocytosis and bacterial killing by phagocytes.
Serum soluble interleukin‐2 receptors and tumor necrosis factor‐α in hematological malignancies of childhood
Authors: ÇETINGÜL NAZAN , YENER ERDAL , ÖZTOP SENAY , NIŞLI GÜNGÖR
Published: January 2011

The levels of soluble interleukin‐2 receptors (sIL‐2R) and tumor necrosis factor (TNF) were determined in the serum of 61 children with hematological malignancy, including 20 patients with leukemia and 16 with lymphoma in active state of malignancy, and 20 patients with leukemia and 5 with lymphoma in complete remission. In addition, serum samples from 15 healthy children were used as controls. It was found that the mean serum levels of sIL‐2R and TNF were significantly increased in active malignancy (207.0±17.1 pmol/L for sIL‐2R; 209.7±35.0 pg/mL for TNF) compared to the remission status and normal controls (P<0.001). No such difference was observed between leukemia and lymphoma groups.

It was concluded that serum sIL‐2R and TNF are of potential value in the diagnosis and follow up of patients with neoplastic diseases.
Impact of serum antibodies to p40tax gene product in the intrafamilial transmission of human T cell leukemia virus type I
Authors: UMEMOTO MASAKAZU , TAKE HIROMICHI , SAWADA TAKASHI
Published: January 2011

We examined the sera of family members of human T cell leukemia virus type I (HTLV‐I) seropositive pregnant women who had visited Kagoshima City Hospital since 1986, and studied the routes of transmssion of HTLV‐I. A new enzyme linked immunosorbent assay (ELISA) for detecting the antibody to an HTLV‐I tax gene product, p40tax, has recently been developed. By this ELISA method, the positive rate of anti‐p40tax among HTLV‐I seropositive subjects, including 96 pregnant women (index subjects), 26 mothers, 13 husbands and 13 children was investigated. The percentage positive for anti‐p40tax among pregnant women, mothers, husbands, and children was 41.6, 50, 53.8 and 53.8%, respectively. This means that the positive rate of anti‐p40tax remains almost constant with increasing age. The rate of mother‐to‐child transmission of HTLV‐I was significantly higher in p40tax seropositive (29.6%) than in seronegative mothers (8.1%). The positive rate of anti‐p40tax in transmission from husband to wife (29%) and through blood transfusion (17%) was lower than the overall prevalence (46%). Thus, these data suggest that p40tax antibodies are associated with the frequency of HTLV‐I transmission and with the differences in the transmission routes.
Molecular weight analysis of cow's milk antigens in neonate sera
Authors: BANDO YUKI , IGARASHI MUNEO , SHIMANUKI KAORU , OHTANI HIDEKI
Published: January 2011

Some food antigens (Ag) can be detected in serum immunoglobulin G (IgG) antibodies (Ab) following ingestion. These Ag may thus be essential for inducing the production of IgE or IgG antibodies, which may in turn lead to the onset of atopic diseases such as atopic dermatitis, allergic rhinitis and asthma. The molecular weights (MW) of these Ag have yet to be determined. To select samples for MW analysis of circulating food Ag, we measured the concentrations of cow's milk (CM) Ag in the sera of 88 neonates by radio‐immunoassay. They were found present in 20 samples of the sera. Twelve of the samples were analyzed for Ag MW by immunoblotting. Several bands appeared for one of the samples. Their MW were < 13 000 and no native CM proteins could be detected.
Response to sodium benzoate treatment in non‐ketotic hyperglycinaemia
Authors: WALTHER FRANK , RADKE MICHAEL , KRÜGER GABRIELE , HOBUSCH DIRK , UHLEMANN MARLIES , TITTELBACH‐HELMRICH WERNER , STOLPE HANS JOACHIM
Published: January 2011

Therapy with benzoic acid in a case of classic neonatal non‐ketotic hyperglycinaemia (NKH) was successful in stopping seizures but not in promoting mental development. Serum glycine levels were normalizable even by administering low doses of 53 mg sodium benzoate/kg body mass (BM) per day. Despite giving a higher dosage (240 mg/kg BM per day) normalization of glycine concentration in cerebrospinal fluid (CSF) was not achieved. However, seizures ceased. Restriction of protein intake (≤2 g/kg BM per day) seemed to be profitable. CSF glycine concentrations below 100 μmol/L may be sufficient to prevent seizures in older infants who have adapted to neuronal glycine exposure. No toxicity of sodium benzoate treatment was detected when administering doses of up to 470 mg/kg BM per day but side effects such as itching and hyperactivity were obvious.
Height and weight measurements of male Turkish adolescents according to biological maturation
Authors: BÜYÜKGEBIZ A. , EROGLU Y. , KARAMAN O. , KINIK E.
Published: January 2011

Eight hundred and seventy‐nine Turkish male adolescents aged between 11 and 19 years were investigated with respect to their physical development. Bone ages were considered as well as chronological ages in determining biacromial (BAC), biiliac lengths (BIC) and chest circumference (CC). Different bone and chronological ages were obtained at the same chronological age (CA) and bone age (BA) columns. The importance of bone ages in evaluating an adolescent's physical development was shown to be as important as the chronological ages. In this study, the mean BAC, BIC and CC values between the same CA but different BA groups showed statistically significant differences (P<0.001). When the measurements of two consecutive age groups who had the same CA and BA and who were found to be statistically significant (P<0.05) were compared, the maximum increments in BAC and BIC values which were between 13 and 14 years of age (2 and 1.4 cm, respectively) were similar to the maximum increment in height and weight of Turkish male adolescents found between 13 and 14 years of BA. The maximum increment in CC values which were between 14 and 15 years and 15 and 16 years (4.1 cm each) was 1 year after the maximum increment in height and weight for BA of Turkish male adolescents (13–14 years) and was similar to maximum weight increment for CA which was between 15 and 16 years.
Premature thelarche in Kabuki make‐up syndrome
Authors: TUTAR H ERCAN , ÖCAL GÖNÜL , İNCE ERDAL , CİN ŞÜKRÜ
Published: January 2011

A 2 year old girl who has Kabuki make‐up syndrome with isolated premature thelarche is presented. She has unique ocular abnormalities which have not been reported previously. In the literature at least 85 patients, most of them from Japan, have been reported. This is the first reported non‐Japanese Asian case.
A case of pseudo‐Bartter's syndrome due to intestinal malrotation
Authors: KOSHIDA RIE , SAKAZUME SHINOBU , MARUYAMA HIROAKI , OKUDA NORIHIKO , OHAMA KAZUNORI , ASANO SHUJI
Published: January 2011

Intestinal malrotation presenting beyond the neonatal period is associated with a multiplicity of symptoms, which are often non‐specific and, consequently, are associated with delays in diagnosis. Pseudo‐Bartter's syndrome, which mimics the manifestations of Bartter's syndrome, can be caused by a severe chloride deficiency secondary to vomiting, diarrhea, perspiration, diuretic abuse and so on.

We describe a 6 year old boy who had been admitted to hospital three times during the preceding year. The patient lapsed into a critical condition with profound hypochloremia and hypokalemic metabolic alkalosis induced by extremely massive vomiting. The attacks of vomiting were spasmodic and self‐limited. During the episodes of vomiting he fulfilled the criteria of pseudo‐Bartter's syndrome, including hyperreninemia, hyperaldosteronism and normal blood pressure, but in the intervals between attacks he was completely asymptomatic. At the third admission, examination supported an overall clinical picture of bowel obstruction, which was confirmed by radiographic examination. Laparotomy revealed a midgut volvulus with intestinal malrotation. After surgery he made a good recovery and was symptom‐free.

In this patient, the high degree of hypochloremia and hypovolemia activated the renin‐angiotensin‐aldosterone system, then aldosterone promoted intensive reabsorption of sodium and excretion of potassium into the urine. Consequently the diagnosis of pseudo‐Bartter's syndrome was established on the basis of an extreme decrease in urinary chloride and an increase in urinary potassium concentration.

It is relatively rare for vomiting due to intestinal malrotation to induce pseudo‐Bartter's syndrome. The importance of considering this rare diagnosis in such cases is discussed.
The effect of bunazosin on monocrotaline‐induced pulmonary hypertension in rats
Authors: INOUE MAKOTO , WATANABE KOJI , MORI CHUZO , TANAKA OSAMU
Published: January 2011

In order to estimate the long‐term hemodynamic and histologic effects of bunazosin hydrochloride on primary pulmonary hypertension (PPH), for which effective internal therapy has not yet been proposed, we produced subacute pulmonary hypertension in 6 week old Sprague‐Dawley rats by injecting one dose of monocrotaline. Bunazosin (10 mg/kg) was administered intraperitoneally every day and bodyweight and hemodynamic parameters were measured. Right ventricle (RV) and left ventricle with septum (LV + S) were weighed separately. Medial thickness of the small pulmonary artery was calculated.

In the bunazosin group, the increase of right ventricular systolic pressure (RVSP) as pulmonary arterial systolic pressure and the ratio of RVSP to pulmonary flow were limited, without causing systemic hypotension, compared to the control group. Weight ratio of RV/(LV + S) had a tendency to be limited in the bunazosin group. As for medial thickness, there was no significant difference between the two groups.

It may be concluded that bunazosin hydrochloride should be considered for clinical trials in treatment of PPH in humans.
Specific IgE antibodies and serum eosinophil cationic protein in children with atopic dermatitis alone
Authors: URISU ATSUO , KONDO YASUTO , WADA EIKO , HORIBA FUMIYA , TSURUTA MITSUTOSI , YASAKI TAKEHIKO
Published: January 2011

Both eosinophils and specific immunoglobulin E (IgE) to foods and mites have been considered involved in the pathogenesis of atopic dermatitis (AD). The relationship between eosinophils and specific IgE, however, remains to be elucidated.

Blood eosinophil counts, serum eosinophil cationic protein (ECP) and IgE to egg white, cow's milk, soybean, rice and Dermatophagoides pteronyssinus (Dp) were measured in subjects with AD alone or bronchial asthma (BA) alone. Subjects with positive IgE titers (Pharmacia radioallergosorbent test (RAST) units > 0.7) of one or more items were defined as RAST‐positive.

Immunoglobulin E titers to egg white, cow's milk and soybean of subjects with AD were high in early childhood and declined with aging, whereas the titers of subjects with BA were negative or low. Immunoglobulin E titers to Dp were elevated after 1 year of age in both disease groups. Eosinophil cationic protein (ECP) levels and blood eosinophil counts in the AD and BA groups were significantly higher than those of non‐atopic controls. No difference in ECP levels or blood eosinophil counts were observed between RAST‐positive and negative groups.

It is concluded that IgE to foods such as egg white, cow's milk and soybean might have a role in the pathogenesis of AD of young children, while IgE to mites might be involved in older children. Eosinophils may also participate in AD. However, different mechanisms may be responsible for the rise in specific IgE and high ECP levels and blood eosinophil counts.
A study of the iron requirement in infants, using changes in total body iron determined by hemoglobin, serum ferritin and bodyweight
Authors: HOKAMA TOMIKO
Published: January 2011

The amount of iron absorption in healthy breast‐fed infants was estimated in order to assess the iron requirement. This was carried out by monitoring changes in total body iron (TBI). Hemoglobin and serum ferritin were measure to calculate hemoglobin iron, body storage iron and TB1 at the ages of 4–5 months and 10–11 months. The amount of iron absorption was ∼ 0.4 mg/day, calculated as the sum of TBI increment and physiological iron loss. From this study, the dietary iron requirement for the weaning period was estimated as 4 mg/day.
Factors affecting the efficiency of peripheral blood stem cell collection in children treated with chemotherapy and G‐CSF
Authors: URASHIMA MITSUYOSHI , KATO YOKO , HOSHI YASUTAKA , DEGUCHI YASUSHI , KAMIJO MAKOTO , UCHIYAMA HIROSHI , FUJISAWA KOHJI , AKATSUKA JUN‐ICHI , MAEKAWA KIHEI
Published: January 2011

This retrospective study attempts to clarify the optimal timing for peripheral blood stem cell (PBSC) collection after conventional chemotherapy followed by granulocyte‐colony stimulating factor (G‐CSF) administration. Leukapheresis was performed 32 times in nine children with various cancers during bone marrow recovery phase following transient pancytopenia after chemotherapy. (On two occasions, leukapheresis was excluded because many leukemic blasts were included.) When the number of white blood cells (WBC) exceeded 1.8 times 1010/L after administration of G‐CSF (200 μg/m2, continuous infusion), many more CD34+ cells were contained in the collected peripheral mononuclear cells (P > 0.02) and a sufficient number of PBSC for transplantation (≥ 10 times 108 CD34+ cells/kg) was obtained after one run in 15 of 17 leukapheresis sessions. In contrast, sufficient PBSC were obtained only in one of 13 runs of leukapheresis when the number of WBC was < 1.8 times 1010/L. The number of WBC on the day when PBSC were collected correlated with collected nuclear cell number (r = 0.60), but not with the CD34+ cell ratio. The ratio was higher only when both platelets and reticulocytes increased in parallel with WBC. We conclude that sufficient PBSC collection is possible after conventional chemotherapy using G‐CSF, when hematopoietic recovery is parallel, without the use of high‐dose chemotherapy.
Efficacy of synchronized IMV on weaning neonates from the ventilator
Authors: MIZUNO KATSUMI , TAKEUCHI TOSHIO , ITABASHI KAZUO , OKUYAMA KAZUO
Published: January 2011

This study evaluated the efficacy of a new patient‐triggered ventilator that triggered the patient's inspiratory effort by detecting the change in airflow by means of a ‘hot wire’ anemometer. This ventilator was used in both the conventional and the synchronzied intermittent mandatory ventilation (SIMV) modes in seven neonates. Values for blood gas, spontaneous breathing rate, tidal volume of spontaneous breaths and minute volume were compared in all seven neonates. The resistive work of spontaneous breathing in five neonates, obtained with synchronized intermittent mandatory ventilation was compared with the values obtained using conventional mechanical ventilation on the previous day of weaning from the ventilator. At each the inflation time studied (0.4, 0.3, 0.24 s), all mechanical breath occurred synchronously with infants' inspiratory efforts. The median trigger delay was 80 ms. Oxygenation was improved on the new system compared with the conventional system. Tidal volume of spontaneous breathing and minute volume were increased with SIMV compared with conventional mechanical ventilation, although the resistive work of spontaneous breathing was decreased with SIMV. The tidal volume of spontaneous breaths was more constant with SIMV versus conventional mechanical ventilation. Thus, the airway flow‐triggered SIMV may lessen inspiratory muscle fatigue during weaning process.

We conclude that the SIMV is useful in weaning neonates from the ventilator.

oxygenation

resistive work of spontaneous breathing

synchronized intermittent mandatory ventilation

wearing process.
A new oral margarine loading test in children
Authors: SHIMIZU TOSHIAKI , YAMASHIRO YUICHIRO , YABUTA KEIJIRO
Published: January 2011

Margarine (2g/kg) was orally administered to 39 children with normal fat digestion and absorption (control group) and 17 children with abnormal fat digestion and absorption (malabsorbed group). The serum triglyceride (TG) level was serially determined by capillary blood sampling. In the control group, the TG level was significantly elevated after 2, 3 and 4 h as compared with the level before margarine ingestion (basal TG). The maximum TG elevation rate [MTER ‐ (maximum TG‐basal TG)/basal TG x 100] was > 50% in all but three children. In contrast, a significant increase in TG was not observed in the malabsorbed group after margarine ingestion, and the MTER was < 50% in all but two children. The MTER was significantly lower in children with a high microscopic score of fecal fat than that in children with a low score. This oral margarine loading test is a simple and semi‐quantitative evaluation of fat digestion and absorption, and is considered to be useful for the assessment of the presence or severity of fat maldigestion and malabsorption in children.
Differential alpha‐smooth muscle actin expression in a gastric epithelioid leiomyosarcoma in a 14 year old girl
Authors: HOSOI HAJIME , SUGIMOTO TOHRU , FUSHIKI SHINJI , MINE HIROSHI , KADONO YOHSUKE , SAWADA TADASHI
Published: January 2011

Leiomyosarcoma in childhood is very rare. This report describes the immunohistochemical study of a gastric epithelioid leiomyosarcoma in a 14 year old girl. Microscopically, the tumor showed two distinct histological patterns within the same tumor: small epithelioid cells forming a lobular pattern and spindle‐shaped cells forming interlacing fascicles, but no distinct border divided the two types of cells. Immunohistochemically, vimentin was positive in all tumor cells; a‐smooth muscle actin, however, was negative in small epithelioid cells and positive in spindle‐shaped cells. The data revealed that the tumor had components showing two stages of morphological and biochemical differentiation. This case indicates that leiomyosarcomas might lose smooth muscle actin during malignant transformation; or, alternatively, they might arise from a primitive type of smooth muscle cell and some of them could spontaneously differentiate into mature smooth muscle cells, expressing this actin.
Renal impairment in very low birthweight infants following antenatal indomethacin administration
Authors: NISHIKUBO TOSHIYA , TAKAHASHI YUKIHIRO , NAKAGAWA YAEKO , KAWAGUCHI CHIHARU , NAKAJIMA MITSURU , ICHIJO MOTOHIKO , YOSHIOKA AKIRA
Published: January 2011

Three cases of neonatal renal insufficiency in very low birthweight (VLBW) infants following repeated antenatal administration of indomethacin to prevent premature labor are reported. Three pregnant women received indomethacin (total doses of 150–850 mg) for 3–14 days from admission until delivery. The gestational ages and birthweights of the infants ranged from 24 to 28 weeks and 612 to 1432 g, respectively. Oliguria, early onset of hyperkalemia and prolonged renal dysfunction occurred after birth. Renal failure did not improve in one infant.

Despite the efficacy of indomethacin for tocolysis in premature labor, VLBW infants born after repeated maternal administration near the time of delivery may have developed impairment of the premature kidney.
A case report of placental hemangioma resulting in severe fetal distress
Authors: ITO MASAZUMI , KUMAMOTO TADASI , YAMAMOTO HATSUMI , NOMURA TOYOKI , MATSUMOTO TAKAFUMI , TAKI MITIO , TOYODA NAGAYASU
Published: January 2011

A 30 year old female was admitted twice during her pregnancy for genital bleeding. An ultrasonography at 19 weeks gestation showed several nodules on the chorionic plate. Cardiotocography during labor at 37 weeks gestation indicated a non‐reactive pattern. A 2230 g infant girl was delivered soon after rupture of membranes with asphyxia and hydrops fetalis. The placenta weighed 600 g with a maximal thickness of 6 cm. Nodules occupied about one‐third of the placenta and were diagnosed as placental hemangiomas. Postnatal examination revealed that the infant had disseminated intravascular coagulation and cardiac failure. This baby recovered after treatment in the intensive care unit, and was discharged home on the 52nd day of life.
Severe aplastic anemia in a patient with primary immunodeficiency
Authors: OHGA SHOUICHI , NAGASHIMA TAKAKO , NISHIZAKI MARI , HIRABARU CHIKAKO , INOUE TOSHIROU , ISE KEIKO , HARA TOSHIRO , UEDA KOHJI
Published: January 2011

This paper reports the case of a 6 year old boy with primary immunodeficiency disease, whose marrow cells showed dyshematopoietic features, that was subsequently transformed to severe aplastic anemia. He was first diagnosed as having congenital immunodeficiency comprising deficiency of immunoglobulin A (IgA), IgG2 and IgG4, depressed mitogen responses, lymphopenia with inverted CD4/CD8 ratio and an increased proportion of T‐cell receptor γ/δ‐bearing cells. Cytogenetic study of the peripheral blood showed a normal karyotype of 46, XY, but that of the marked hypoplastic marrow demonstrated one cell with monosomy 7 and another with trisomy 8 in the 20 cells examined.
Haemophilus influenzae type D infection and JgG2 deficiency in a patient with chronic mucocutaneous candidiasis
Authors: SAKANO TAKASHI , HAMASAKI TAKASHI , MORI MIKIO , TAKAHASHI HIROKO
Published: January 2011

A 14 year old boy with chronic mucocutaneous candidiasis and persistent pulmonary infection caused by Haemophylus influenzae and Streptococcus pneumoniae is reported. Initial bacterial culture studies showed H. influenzae type B and S. pneumoniae as causative agents. H. influenzae type D was constantly isolated from the patient's sputum. Abnormally low levels of serum immunoglobulin G2 (IgG2) found in the patient may have contributed to the pulmonary infection and H. influenzae type D may be an important causative agent in immunodeficient patients.
Cough variant asthma: A case study with antigen, histamine and methacholine inhalation challenge tests
Authors: MORIKAWA A. , MITSUHASHI M. , TABATA H. , SEKI Y. , MOCHIZUKI H. , TOKUYAMA K. , KUROUME T.
Published: January 2011

This paper presents a 7 year old girl with cough variant asthma, in which house dust challenge test provoked fine crackles on auscultation without any changes of lung function test. Pretreatment with dl‐chlorpheniramine maleate for 7 days inhibited the appearance of fine crackles. On histamine inhalation test, fine crackles and rhonchi appeared on auscultation without bronchoconstriction (% fall of one‐second forced expiratory volume < 10%). These data suggest that the cause of cough variant asthma is mainly an immunoglobulin E‐mediated allergic reaction in the mucus secreting system.
Rapid detection of phenylketonuria mutations by non‐radioactive single‐strand conformation polymorphism analysis
Authors: YAO YEQI , MATSUBARA YOICHI , NARISAWA KUNIAKI
Published: January 2011

A non‐radioactive single‐strand conformation polymorphism (SSCP) method was used to detect various phenylketonuria (PKU) mutations in Japanese and Chinese patients. Arginine413‐to‐proline (R413P) mutation in exon 12 of the phenylalanine hydroxylase gene was identified in a Japanese patient by this method. The segregation of the R413P mutation in the proband's family was clearly demonstrated and the carrier status of each family member was determined. Analysis of DNA fragments containing exon 7 originated from Chinese patients revealed two mutations, arginine243‐to‐glutamine (R243Q) and arginine261‐to‐glutamine (R261Q), and a polymorphism, valine245‐to‐valine (V245V). Although R261Q has been identified previously among Caucasian subjects, this report is the first to describe this mutation among Orientals. Since the non‐radioactive SSCP method employs pre‐cast acrylamide gels and pre‐made gel buffer strips combined with semi‐automated temperature‐controlled electrophoresis, it can be performed without much expertise in molecular biological techniques. The ability of this method to detect various mutations as demonstrated in this study and its ease of use make it feasible to detect PKU mutations in a routine DNA diagnostic laboratory.
Prevalence of Chlamydia pneumoniae in acute lower respiratory infection in the pediatric population in Japan
Authors: OUCHI KAZUNOBU , NAKAZAWA TERUKO , KARITA MIKIO , KANEHARA YOJI
Published: January 2011

Chlamydia pneumoniae has been established as an important etiologic agent of acute respiratory tract infection in humans, especially in adults. However, there is little information available on C. pneumoniae infection in the pediatric age group. The microimmunofluorescence test and Chlamydia pneumoniae‐specific polymerase chain reaction (PCR) method were applied to reveal the role of C. pneumoniae as an etiologic agent of acute lower respiratory tract infection in children in Japan. Among 136 patients aged between 2 months and 15 years with acute lower respiratory tract infection, five patients with pneumonia were found to have recent C. pneumoniae infection by the microimmunofluorescence test. C. pneumoniae‐specific PCR products were detected from specimens of four patients among these five. Neither C. trachomatis nor C. psittaci infection was found in this population. These results suggest that C. pneumoniae is an important causative agent of acute childhood pneumonia and may be the most prevalent pathogen among the genus Chlamydia that causes acute lower respiratory disease in this age group in Japan.
Investigation of melena and hematochezia as the chief complaint of gastrointestinal bleeding in pediatric surgical patients
Authors: UNO TAKEJI , HARADA YUKIO , KIMURA TAIZO , MOCHIDA YOSHIAKI , GOTO KEIGO , MIYATANI KAORU , KUBO SEIICHI , LI XIAO RANG
Published: January 2011

The causes of melena or hematochezia in 48 pediatric patients were examined. Malrotation with volvulus was an important cause of hemorrhage during the newborn period, and intussusception was very typical in patients aged from 1 month to 1 year. Polyps of the rectum and colon were the most common causes of melena or hematochezia in patients older than 1 year. No cause of melena or hematochezia could be identified in 11 children. Ten patients have remained in good health with no further episodes of melena or hematochezia. Localized multiple polyps of the rectum with focal carcinoma were detected in only one patient. In general, although no further investigation is required after detection of the cause of bleeding and its successful treatment, it should be kept in mind that gastrointestinal malignancy can occur in children.
Two cases of insulin‐dependent diabetes mellitus under insulin treatment with slow height velocity: Relationship of growth hormone‐binding protein, metabolic control and growth
Authors: MURATA ATSUSHI , YASUDA TOSHIYUKI , NIIMI HIROO
Published: January 2011

Inadequate blood sugar control in children with insulin‐dependent diabetes mellitus (IDDM) sometimes results in low insulin‐like growth factor‐I (IGF‐I) and sluggish height growth. High affinity growth hormone‐binding protein (GHBP), which is identical to the extracellular domain of growth hormone (GH) receptor, is present in the human sera. We have determined GHBP activity in two cases of poorly controlled IDDM with low height velocity in relation to metabolic control in order to determine the mechanism of resistance to GH in this condition, as indicated by low levels of GH‐dependent growth factor IGF‐I in the face of high serum GH levels. GHBP activity was within the normal range in two cases of IDDM with slow height velocity, low IGF‐I and high hemoglobin‐A1. In both cases, improved blood sugar control normalized IGF‐I to result in accelerated height velocity without a major change in GHBP levels. These results may indicate either normal peripheral GH receptor or normal free portion of serum GH, and may suggest that the major defect in slow growth in poorly controlled diabetes is due to the post GH receptor.