bluuebunny/crossref_metadata_2025 huggingface dataset:
A case of bacterial meningitis complicated by posttraumatic cerebrospinal fluid rhinorrhea
Authors: WADA NORIKO , TAKEUCHI YOSHIHIRO , FUJII MASAYO , FUJIWARA FUMIHIRO , ODANI ISAO , SAWADA TADASHI
Published: January 2011

This paper reports an 11 year old boy with bacterial meningitis accompanied by post‐traumatic cerebrospinal fluid (CSF) rhinorrhea. Streptococcus pneumoniae was cultured from CSF. The clinical course was very rapid before admission and his age relatively high for usual bacterial meningitis. Consequently, we examined the cause in detail. Immunological findings were within normal limits. Although routine graphic examinations, such as cranial X‐ray photography, horizontal cranial computed tomography (CT) and magnetic resonance imaging, could not demonstrate a bone defect, both coronal thin‐section cranial CT scanning and radioactive isotope counting by means of cotton packed into the nasal cavity were useful for detecting CSF rhinorrhea. In a case of atypical meningitis, the past history should be examined with caution and coronal thin‐section CT should be performed.
A case of cytomegalovirus mononucleosis associated with pleural effusion
Authors: KATO YOKO , MIYATA ICHIRO , SAKUMA SHUYA , KATAYAMA AKIRA , TOYODA SHIGERU , KOBAYASHI SHIN'ICHI , OKABE NOBUHIKO , MAEKAWA KIHEI
Published: January 2011

A 5 year old boy had a spiky fever accompanied by a mild pharyngitis, cervical lymphadenopathy and hepatosplenomegaly. Laboratory findings revealed leukocytosis with 26% atypical lymphocytes, and liver dysfunction. A chest X‐ray showed pneumonia and a considerable amount of pleural effusion. Serum antibody titers for cytomegalovirus (CMV) were elevated significantly and CMV‐DNA (polymerase chain reaction) was detected in the pleural effusion. Only 13 cases of pleural effusion associated with infectious mononucleosis have been reported previously in the literature, but there was no documentation that proved CMV infection. The case reported here suggests that the pleural effusion was caused by the infiltration of mononuclear cells to the pleura as a result of systemic inflammation, and the possible alternative of host immune response against CMV was related to recent Varicella zoster virus infection.
Balloon pulmonary valvuloplasty for double outlet right ventricle with valvular pulmonary stenosis
Authors: AKIBA TOMOHARU , SATO TETSUO , YOSHIKAWA MASARU , NAKASATO MITSURU , SUZUKI HIROSHI , SATO SATOSHI
Published: January 2011

A male neonate presented with cyanosis. Echocardiograms showed a double outlet right ventricle, an absent outlet septum, a subaortic ventricular septal defect and valvular pulmonary stenosis. Balloon pulmonary valvuloplasty was performed at 19 days of age, resulting in an immediate increase of the systemic arterial oxygen saturation from 67 to 87%. As far as could be found, this is the first report of a patient with a double outlet right ventricle without the outlet septum who was clinically observed and underwent balloon pulmonary valvuloplasty.
A case of siblings with Meckel's diverticulum diagnosed before operation
Authors: MATSUKUMA YOSHINORI , MATSUO YUSAKU , SAKAGUCHI MINAKO , YAMADA TAKASHI , HIRATA TOMOSHIGE , TANAKA NOBUO , MORITA JUN , ARAKI HISA‐AKI , SUE KOHJI , KANEKO TAKASHI , NAKANISHI KAZUO
Published: January 2011

The authors encountered a sibling case of Meckel's diverticulum in 9 and 11 year old Japanese boys. They were diagnosed using contrast examination and 99mTc‐pertechnetate scintigraphy before operation. The familial tendency is one in every 2500 families. Only 15 cases of familial occurence have been reported by five authors.
Hepatic calcification in an anicteric patient with biliary atresia after hepatic portoenterostomy
Authors: MATSUI AKIRA , NAKAMURA MICHIRU , AIHARA TOSHINORI , FURUSE MAKOTO , SASAKI NOBUHIKO , ARAKAWA YOICHI , YANAGISAWA MASAYOSHI , MAKINO SHUN‐ICHI
Published: January 2011

Hepatic calcification was found in an anicteric infant with biliary atresia after hepatic portoenterostomy. When she was 2 years of age, ultrasonography detected an echogenic structure with acoustic shadowing. Computerized tomography located it at the proximal portion of Segment 6 in the right lobe. Although both techniques failed to exhibit dilatation of the peripheral bile ducts, we believe intrahepatic cholelithiasis provides the most likely diagnosis. Although seven cases with biliary atresia were reported to have this complication, all of them were icteric when the diagnosis was confirmed. The patient should be carefully followed up since the succeeding stones may cause jaundice by obstructing the main duct.
Overview of sudden infant death syndrome in Japan
Authors: NISHIDA HIROSHI
Published: January 2011

In spite of rapid medical advancement in the care of infants and children, not only the general public but also many medical personnel have remained unaware of sudden infant death syndrome (SIDS) until very recently in Japan. In 1981, a research project team on SIDS financed by the Ministry of Health and Welfare was founded. Current incidence of SIDS is estimated to be about 0.5 per 1000 live births. The SIDS Family Association was organized in 1993 in Japan and began to publicize the social importance of SIDS and to support SIDS families by training befrienders. A series of articles on current achievements from SIDS research projects and from the SIDS Family Association are submitted to this issue. The present paper summarizes the status of SIDS in Japan by overviewing the activities of the research team and the SIDS Family Association.
Toward better home respiratory monitoring: A comparison of impedance and inductance pneumography
Authors: MIYASAKA KATSUYUKI , KONDO YOICHI , SUZUKI TAKESHI , SAKAI HIROKAZU , TAKATA MASAO
Published: January 2011

Various respiratory monitoring methods have been used as a part of home respiratory care, but none has been accepted as a universal method. Impedance pneumography is the most popular method at present and is used in the form of cardiorespiratory monitoring, but it has limitations for diagnosing obstructive airway problems and a high incidence of false alarms.

We evaluated a new investigational method, inductance pneumography, in terms of the incidence of false alarms (waveform out of range), using infants recovering from general anesthesia. They were used for this study because they go through all the stages of sleep in a short period and their relatively long period of light sleep was advantageous in evaluating motion artifact interference.

Waveforms went out of range 24.6 ± 2.9% of the time with impedance pneumography and 15.9 ± 4.5% with pulse oximeter, but 11.0 ± 3.4% with inductance pneumography. These differences are statistically significant (P < 0.01). Electrocardiography electrodes for impedance pneumography dislodged briefly in one case but the Respiband dislodged in four cases and the Flex II probe dislodged in three cases, indicating the need for better fixation of sensors.

It was found that inductance pneumography, in addition to being able to detect obstructive apnea, has a significantly lower incidence of false alarms. Further elaboration of this method is warranted for better home respiratory monitoring.
The airway occlusion test as a screening for sudden infant death syndrome (SIDS)
Authors: HASEGAWA HISAYA
Published: January 2011

The airway occlusion test was carried out as a screening test for sudden infant death syndrome (SIDS). To obtain the normal values for use as control values, the relationship of the airway occlusion test with various variables was determined in 234 infants. The result shows that the best correlation was seen between percentage prolongation and the corrected gestational age. Percentage prolongation increased with the progression of age up to about the 40th week of corrected gestational age. Subsequently, there was no remarkable change in percentage prolongation. In six out of eight cases with apparent life threatening events, percentage prolongation was reduced. These results indicate that the determination of percentage prolongation can be used for screening of high risk babies for SIDS.
Sudden cerebral hypoperfusion enhances respiratory suppression in hypoxic newborn piglets
Authors: TOGARI HAJIME , KATO INEKO , YAMAGUCHI NOBUYUKI
Published: January 2011

The relationship between sudden cerebral hypoperfusion and sudden respiratory suppression was investigated in newborn piglets. A total of 11 experiments on five newborn piglets were divided into three groups: normoxic, hyperoxic and hypoxic. The sudden cerebral hypoperfusion was created by manual compression of the Arteria carotis communis and Vena jugularis interna. Respiratory suppression was calculated by measuring the duration of apnea and the per cent reduction of respiratory amplitude. The means of apnea duration in normoxic and hyperoxic groups were 6.6 ± 0.8 and 1.2 ± 2.4 s, respectively, while two experiments on the hypoxic group showed 8.4 s and complete cessation of respiration in one of them. The per cent reductions of respiratory amplitude in the normoxic and hyperoxic groups were 23.3 ± 8.5 and 17.6 ± 11.7%, respectively. In the hypoxic group, one of two experiments showed a 22.7% reduction, which was equivalent to that of the normoxic group. We conclude that sudden cerebral hypoperfusion enhances respiratory suppression, especially when it coincides with hypoxia. The phenomenon may be one of the possible causes for a certain type of sudden infant death syndrome. Further study will be undertaken to elucidate the direct effects of the mechanism of cerebral hypoperfusion on respiratory suppression.
Risk factors for SIDS in Japan: A record‐linkage study based on vital statistics
Authors: FUJITA TOSHIHARU , KATO NORIKO
Published: January 2011

This study examines the effect of items as reported on birth certificates on sudden infant death. We linked infant death certificates with birth certificates for the infants born in 1989 to residents of the Tohoku, Tokai and Kyushu regions in Japan (n= 409 679), that is, about one‐third of the infants born in Japan that year.

The mortality rate from sudden infant death, including 88 deaths from sudden infant death syndrome (SIDS) and 17 deaths from instantaneous death, was 25.6 per 100 000 live births. Elevated risk of sudden infant death was associated with low birthweight, late birth order, illegitimacy, male gender and young maternal age. These results correspond to previous studies from Western countries, suggesting a similar pattern for SIDS in Japan.
Epidemiology of sudden infant death syndrome in Japan
Authors: WATANABE NOBORU , YOTSUKURA MASAHIRO , KADOI NOBUAKI , YASHIRO KIMIO , SAKANOUE MASAMICHI , NISHIDA HIROSHI
Published: January 2011

An epidemiological survey was carried out to examine the present situation with respect to sudden infant death syndrome (SIDS) in Kanagawa Prefecture. Questionnaires on sudden unexpected death of infants aged < 1 year in 1990‐91 were sent to the hospitals and clinics in Kanagawa Prefecture which may take care of such infants. By analysing information from 10 485 replies, 48 out of 73 reported sudden infant deaths were confirmed to be SIDS, although autopsy was not performed in 13 cases (27%). The incidence of SIDS per 1000 live births in Kanagawa Prefecture was 0.29 in 1990 and 0.31 in 1991; and if limited to autopsy cases 0.19 and 0.25, respectively. Sudden infant death syndrome cases in Japan were found to occur more frequently when infants were < 6 months old, at home and sleeping alone, but less in the winter and between midnight and early morning. There was little difference between the numbers in prone and supine sleeping positions at discovery. It was not clear whether SIDS occurred more often to babies sleeping prone than supine, because there were no controls matched with the SIDS cases. In future, continuous epidemiological surveys of SIDS in Japan should be carried out.
The role of respiratory syncytial virus in acute bronchiolitis in small children in northern Japan
Authors: SAIJO MASAYUKI , TAKAHASHI SATORU , KOKUBO MASAYO , SAINO TOMOYUKI , ISHII TAKUMA , IN‐YAKU FUMIE , TAKIMOTO MASATOSHI , TAKAHASHI YOUJI
Published: January 2011

Respiratory syncytial virus (RSV) plays an important role in acute bronchiolitis, which is life threatening in some infants. We investigated the epidemiology of RSV acute bronchiolitis in children less than 3 years old in northern Japan. From April 1991 to March 1993, 162 infants with acute bronchiolitis were hospitalized in our pediatric wards. The diagnosis of RSV acute bronchiolitis was based on the typical clinical manifestations and the presence of RSV antigen in their nasopharyngeal specimens or the rise of the RSV antibody titer. 124 out of 162 patients (76.5%) were diagnosed as having RSV acute bronchiolitis. 43.5% of patients with RSV acute bronchiolitis were 6 months old or less. The epidemic of RSV acute bronchiolitis commenced in October, peaked in December and ended in summer. RSV is quite prevalent in infants with acute bronchiolitis in northern Japan.
Importance of recall and follow‐up screening for chronic hepatitis C in children receiving blood products prior to 1990 in Japan
Authors: MATSUOKA SUGURU , TATARA KATSUYOSHI , USHIROGUCHI YURI , KUBO MASAHIRO , NII MASAKI , TAGUCHI YOSHIYUKI , KURODA YASUHIRO
Published: January 2011

The objective was to detect chronic hepatitis C virus infection in recipients of blood products using retrospective analysis by recall and enrollment of recipients. 226 patients who received blood products for open heart surgery from January 1983 to June 1992 were examined for HCV antibody by using a second generation assay and liver function test. 22 (14%) of the 161 patients who received blood products before November 1989 had detectable HCV antibody, but none of the 65 recipients receiving blood products after 1990, the year the Japanese blood bank began to screen for HCV‐antibody. Abnormal alanine aminotransferase (ALT) levels, more than 25 iu/L, during the chronic phase of HCV infection was recognized in nine of 22 (41%) seropositive patients. The liver function test and second generation HCV antibody in the serum are effective markers to screen for chronic hepatitis C in blood product recipients transfused before 1990.
Characterization of renal tubular damage in preterm infants with renal failure
Authors: KOJIMA TAKATSUGU , SASAI‐TAKEDATSU MISA , HIRATA YUKIO , KOBAYASHI YOHNOSUKE
Published: January 2011

This study attempts to clarify the characteristics of renal tubular damage in preterm infants with renal failure. Sixty‐one neonates (17 term and 44 preterm infants) were divided into three groups: 15 infants with intrinsic renal failure (IRF), five term and 10 preterm; 19 with pre‐renal renal failure (PRF), five term and 14 preterm; and 27 without renal dysfunction (control), seven term and 20 preterm. Urine was collected for an 8 h period on the 2nd or 3rd day of life to determine the following parameters: creatinine clearance (Ccr), fractional excretion of sodium (FENa), urinary N‐acetyl‐beta‐D‐glucosaminidase (NAG) index and endothelin‐1 (ET‐1) excretion. Parameters of renal tubular function and/or renal tubular damage such as FENa, NAG index and ET‐1 excretion were considered as a useful marker to differentiate IRF from PRF in preterm infants. However, these parameters were significantly elevated in preterm infants with PRF. These findings led us to make the following speculations: (i) renal tubular damage may easily occur in preterm infants; and (ii) there still remains difficulty in differentiation between IRF and PRF using Ccr instead of the fluid challenge test.
Differences of LAK‐activity and IL‐2 responsiveness between α/β and γ/δ T cells which developed after thymus transplantation
Authors: NAGASAWA MASAYUKI , MORIO TOMOHIRO , TAKAGI SHUJI , YATA JUNICHI
Published: January 2011

A patient with severe combined immunodeficiency was transplanted with T cell depleted haploidentical bone marrow from his father and was later given a thymic graft from an unrelated donor, α/β and γ/δ T cells of bone marrow donor origin appeared only after the thymus transplantation procedure. Among the peripheral blood lymphocytes (PBL), γ/δ T cells comprised 10–20% and most of them were δTCS1+. The α/β T cells were single positive cells, either CD4+ or CD8+. Expression of CD5, CD7 and CD8α,β molecules on α/β T cells was reduced. Functional studies showed that γ/δ T cells proliferated slightly in response to anti‐CD3 stimulation, and proliferated well with exogenous IL‐2 stimulation, while α/β T cells did not proliferate following mitogenic stimulation even in the presence of IL‐2. γ/δ T cells but not α/β T cells exhibited some LAK activity after culturing with IL‐2. Since α/β T cells expressed IL‐2R α and β chains after mitogenic stimulation and bound IL‐2, the deficit(s) in these cells was considered to occur after IL‐2 binding to the IL‐2R. These results indicate thymic dependency of both types of T cells and that two types of T cells differed in the acquisition of IL‐2 responsiveness during development.
Primary pulmonary choriocarcinoma in a four month old boy complicated with precocious puberty
Authors: OTSUKA TAKAYUKI , OHSHIMA YUKIO , SUNAGA YASUO , NAGASHIMA KANJI
Published: January 2011

A four month old boy with pulmonary choriocarcinoma, showing iso‐sexual precocious puberty is reported. His serum human chorionic gonadotropin (HCG) was highly elevated. A round isolated tumor in the right lung, found by chest X‐ray, magnetic resonance imaging and gallium scintigraphy, was removed surgically a month and a half after clinical manifestations were noticed. It was determined to be choriocarcinoma on the basis of histological findings and positive HCG on histochemical examination. Skull irradiation and chemotherapy (cisplatinum, vinblastin and bleomycin) had only a transient effect on reducing tumor size and normalizing the serum level of HCG. This case suggests that a more effective treatment regimen needs to be established.
A case of familial thyroxine binding globulin excess associated with growth hormone deficiency
Authors: NAKAI AKIO , SHIGEMATSU YOSUKE , KIKAWA YOSHIHARU , SUDO MASAKATSU
Published: January 2011

A 7 years 3 months old Japanese boy with familial thyroxine binding globulin (TBG) excess associated with growth hormone (GH) deficiency is reported. The patients height was 106.4 cm (‐ 2.86 s.d.) and his bone age was 5 years and 3 months. He had no goiter and his developmental milestones were normal. The serum thyroid stimulating hormone (TSH) was 2.8 μU/mL, triiodothyronine (T3) 3.1 ng/mL, thyroxine (T4) 23.4 μg/dL and free T4 1.8 ng/dL. The serum TBG level was beyond 80.0 μg/mL, with normal TSH response to the thyrotropin‐releasing hormone (TRH) test. Familial study revealed that his grandmother, mother, uncle, younger sister and younger brother had high TBG and T3 levels, thus an X‐linked co‐dominant transmission was suggested. The peak GH responses to insulin and clonidine hydrochloride were 5.8 and 8.2 ng/mL, respectively. The mean nocturnal GH concentration was 2.5 ng/mL. His growth velocity increased from 4.8 to 8.4 cm/year and his serum TBG levels decreased gradually after human growth hormone (hGH) treatment.
A case of Kabuki make‐up syndrome with central diabetes insipidus and growth hormone neurosecretory dysfunction
Authors: TAWA RITSUKO , KAINO YUKIKAZU , ITO TAKUO , GOTO YOSHINORI , KIDA KAICHI , MATSUDA HIROSHI
Published: January 2011

A case of a 6 year old boy with Kabuki make‐up syndrome with central diabetes insipidus and growth hormone neurosecretory dysfunction is reported. Magnetic resonance imaging revealed abnormal findings of the pituitary gland and stalk. Good catch‐up growth was obtained by treatment with growth hormone. These findings suggest that hypothalamic‐pituitary dysfunction might be involved in Kabuki make‐up syndrome.
A case of Fanconi syndrome whose origin was suspected to be cystinosis
Authors: EHARA AKIRA , TAKASE AIKO , TAKETAZU GEN‐YA , ISHIZAKI SHOUICHI , ISHIKAWA AKASHI , FUKUSHIMA NAOKI , WAGATSUMA YOSHINORI
Published: January 2011

We report a case of 6 year old girl with Fanconi syndrome the origin of which was suspected to be cystinosis. Pathological findings of a renal biopsy showed needle‐like materials in the epithelial cells but the cystine content of the white blood cells was normal. Although excessive urinary loss of growth hormone was detected, the height and weight of the patient was normal. Urinary loss of growth hormone did not cause growth retardation in this case.
Acute thrombocytopenic purpura associated with primary Epstein‐Barr virus infection
Authors: KANEGANE HIROKAZU , MIYAWAKI TOSHIO , IWAI KAZUYUKI , TSUJI TAKAO , TANIGUCHI NOBORU
Published: January 2011

Acute idiopathic thrombocytopenic purpura (ITP) often appears to be related to the sensitization by some viral infections. However, the causative viral agents are not identified in most cases. Although the primary infection with Epstein‐Barr virus (EBV) occurs during early childhood in Japan, the majority of cases are usually asymptomatic. A minority are associated with acute infectious mononucleosis (IM), which is characterized by fever, tonsillitis, lymphadenopathy, splenomegaly and liver dysfunction. In this report, three cases are described of children with EBV‐induced ITP who clinically had atypical findings of IM. Their primary EBV infections were confirmed by serological test and, in addition, were verified by the enhanced expression of activation antigens (HLA‐DR and CD45RO) on T cells as well as the inverted ratio of CD4+ to CD8+ subsets. These observations imply that ITP can occur as one of the host responses during primary EBV infections, irrespective of clinical manifestations. Evaluation of lymphocyte subpopulations may be useful for the assessment of primary EBV infection in ITP.
An early‐onset case of multiple sclerosis with thalamic lesions on MRI
Authors: ASAI KOUICHI , INAGAKI MASUMI , MAEGAKI YOSHIHIRO , YAMAMOTO TOSHIYUKI , SUZAKI ICHIRO , OHTA SHIGERU
Published: January 2011

We present here an early‐onset case of multiple sclerosis (MS) with thalamic lesions. The patient first experienced an episode of ataxic gait at 2 years 3 months of age, with spontaneous remission within 1 month. At 5 years 4 months, she was admitted because of cerebellar ataxia, oculomotor restriction and feeding difficulty. Magnetic resonance imaging (MRI) showed multiple well‐defined lesions in the white matter of the cerebellum, mid‐brain, periventricle and right frontal lobe. Cerebrospinal fluid (CSF) showed a mild elevation of both immunoglobulin G (IgG) and myelin basic protein (MBP). Serum anti‐myelin antibody was also positive, although leukocytosis and elevation of C‐reactive protein were not found. Methylprednisolone pulse therapy relieved symptoms within 2 weeks and the abnormal MRI and CSF findings gradually improved. At 6 years 6 months of age, she incurred a third episode of cerebellar ataxia and disturbance of consciousness. Magnetic resonance imaging revealed recurrence and extension of the previous lesions as well as new lesions in the thalamus and internal capsule. CSF IgG and MBP level showed a higher elevation than in the second episode. The combination of the cerebellar, brain‐stem, cerebral and thalamic lesions with remission and exacerbation, supported by MRI and CSF findings, allowed the diagnosis of clinically definite MS to be made. This is one of the youngest cases of MS yet described, with the first attack occurring at 27 months of age. In addition, this case is unique for the involvement of the gray matter in the thalamus.
Lymphoma syndrome leukemia revealed interesting finding on brain CT and ultrasound scan of abdomen at the initial presentation
Authors: IYORI HIDEAKI , ISHITOYA NAOKO , HASEGAWA NOZOMI , HIROTSU TAKUO , AKATSUKA JUN‐ICHI
Published: January 2011

This paper reports on a patient with lymphoma syndrome leukemia (LSL) who showed interesting findings on brain computed tomography (CT) and ultrasound scans of the abdomen at the initial presentation. The patient was a 5 year old girl. When she was admitted to our hospital, there were many lymph nodes palpable. The abdomen was distended and the liver and spleen were palpable below the umbilicus. Hematologic examinations revealed a leukocyte count of 275 800/μL with 98% lymphoblasts. Chest X‐ray film revealed a mediastinal mass. The diagnosis of LSL was made. Her brain CT scan showed a low density area in the right thalamic region without contrast enhancement; infarction was suspected. Furthermore, her abdominal ultrasound scan showed hepatosplenomegaly, kidney swelling with increasing echogenicity and hydronephrosis and stones in the renal pelvis and bladder. These findings are unprecedentedly rare in cases of childhood acute lymphoblastic leukemia (ALL), much less in LSL.
Acute heart failure and acute renal failure in Kawasaki disease
Authors: SENZAKI HIDEAKI , SUDA MATSUKO , NOMA SEIJI , KAWAGUCHI HARUO , SAKAKIHARA YOICHI , HISHI TOSHIO
Published: January 2011

Acute renal failure and acute heart failure are rare in Kawasaki disease. We experienced two patients with Kawasaki disease who presented acute renal failure and acute heart failure. These two patients gave us an important insight into the understanding of water balance and fluid therapy in Kawasaki disease. One patient showed acute prerenal failure due to fluid exudation from the intravascular to the extravascular space, and subsequent acute heart failure. The other patient showed acute heart failure caused by fluid infusion for the treatment of dehydration. It is suggested that acute renal failure could be caused by a fluid shift from the intravascular to the extravascular space in Kawasaki disease. It is also demonstrated that the reserve of cardiac function could be decreased in patients with Kawasaki disease due to myocarditis even with normal echocardiography and chest X‐rays.
A case of non‐tuberculous mycobacterial lymphadenitis diagnosed with new skin tests and PCR
Authors: NISHI JUN‐ICHIRO , KANAME TADASHI , YOSHINAGA MASAO , NAGAI TAKU , MURAKAMI NAOKI , KATAOKA NAOKI , MIYATA KOICHIRO
Published: January 2011

Non‐tuberculous mycobacterial (NTM) cervical lymphadenitis has been rarely reported in Japan. We report on a case of a 15 month old child with NTM cervical lymphadenitis who was diagnosed with new skin tests containing NTM antigens and a polymerase chain reaction (PCR) method. These two diagnostic methods were highly useful in the present case, therefore we anticipate they will become available to other Japanese physicians to expedite the diagnosis of NTM lymphadenitis.
Spinal muscular atrophy in trizygotic triplets
Authors: KOMATSU HIROSHI , TAKEUCHI YOSHIHIRO , MIYANOMAE YURI , YOSHIOKA HIROSHI , DANIELS RACHAEL , DAVIES KAY E. , SAWADA TADASHI
Published: January 2011

The clinical, electrophysiological, pathological and genetic findings in trizygotic triplets with spina muscular atrophy (SMA) are reported. The first child was clinically affected shortly after birth and the third one first showed symptoms at 1 month of age. Electromyography and a muscle biopsy provided evidence of lower motor neuron disease. The second child remains clinically normal, but electromyography showed fibrillation potentials and regular spontaneous motor unit activity at rest. Genetic linkage analysis revealed that the two siblings with typical type 1 SMA had the same chromosome 5q haplotype, and that the second child had a different haplotype. It is considered that in this family there is a link to SMA 5q and there is little possibility that the second child is affected. These data emphasize the need to adhere to strict clinical criteria for the diagnosis of chromosome 5q SMA.
Development of mucosal immune function in man: potential for Gl disease states
Authors: MACDONALD THOMAS T
Published: January 2011

The human mucosal immune system is structurally mature and has all the necessary cellular components to generate an immune response at birth. However, in the absence of dietary antigens and bacterial flora, there are no secondary follicles in the Peyer's patches and virtually no immunoglobulin A plasma cells in the lamina propria. Reactive follicle centers develop after birth but it takes 2 years for mucosal IgA plasma cell density to reach adult levels. T cells are present in the epithelium and lamina propria at birth, albeit at a lower frequency than later in life and there are major differences in phenotype between T cells in fetal intestine and postnatal intestine.

There is no information on the impact of the massive antigenic challenge at birth on the mucosal immune system. Well‐documented deficiencies in the ability of the blood T cells of the neonate to produce interleukin‐4 and interferon‐gamma may also occur in the intestine.

It is still an open question whether it is better to try to prevent immunological sensitization of the newborn by avoiding potential allergens (i.e. cow's milk), or whether early exposure (as happens when premature infants are given formula feeds) might tolerize the infant. Hydrolysed cow's milk formulae are probably less antigenic than whole cow's milk and have been widely used in the treatment of cow's milk allergy. Some thought is now being given as to whether the prophylactic use of hydrolysates can reduce cow's milk allergy in ‘at‐risk’ infants.
The regulation of intestinal hypersensitivity reactions to ovalbumin by ω‐3 fatty acid enriched diet: Studies of IEL and LPL in mucosal damage
Authors: YAMASHIRO Y. , OHTSUKA Y. , YABUTA K.
Published: January 2011

In order to clarify the mechanisms of food‐sensitive enteropathy, a food hypersensitive model was generated by feeding ovalbumin to female BALB/c mice after intraperitoneal injection of cyclophosphamide and morphological and immunological changes in the gut mucosa were investigated. Villus atrophy, crypt hyperplasia and increased numbers of intra‐epithelial lymphocytes (IEL) were confirmed in this model, as seen in food‐sensitive enteropathy in humans. Subpopulations of IEL and lamina propria lymphocytes were enumerated by immunohistochemical observation. CD8‐positive cells were increased both in epithelium and lamina propria, whereas CD4‐positive cells were decreased in lamina propria. We document here that orally administered food antigen actually induces food‐sensitive enteropathy and mucosal damage is generated by lymphocytes that infiltrate the intestinal mucosa. We also investigated the effect of feeding an ω‐3 fatty acid‐enriched diet in this model and found that it was efficient in attenuating mucosal damage.
Sorrento studies of diet and fecal flora in the newborn
Authors: WHARTON BRIAN A , BALMER SUSAN E , SCOTT PAUL H
Published: January 2011

The fecal flora of a breast‐fed baby is very different from that of a bottle‐fed baby. This paper reviews five previous studies, performed at this hospital concerning the effect of various dietary components (whey proteins, casein, lactoferrin, iron, nucleotides) on the fecal flora.

The babies received either breast milk or one of the test formulas from birth. Fecal samples were examined by quantitative microbiological methods at 4 and 14 days and at various intervals thereafter.

By 14 days differences in the fecal flora were established. Among breast‐fed babies bifidobacteria, lactobacilli and staphylococci were predominant organisms, whereas in the formula‐fed babies the predominant organisms were enterococci, coliforms, and Bacteroides. A whey‐based formula without bovine lactoferrin, iron or nucleotides gave a flora a little closer to but still remote from the breast‐fed one.

Despite extensive modification of cow's milk in the manufacture of a modern infant formula, the fecal flora of bottle‐fed babies remains substantially different from that of breast‐fed babies.
Biophysical properties of protein‐free, totally synthetic pulmonary surfactants, ALEC and Exosurf, in comparison with surfactant TA
Authors: TAKAHASHI AKIO , NEMOTO TERUKO , FUJIWARA TETSURO
Published: January 2011

An artificial pulmonary surfactant prepared from chloroform‐methanol extract of bovine pulmonary surfactant (surfactant TA) has been shown to be effective in both the prevention and the treatment of respiratory distress syndrome in premature babies. Recently, two types of protein‐free totally synthetic surfactants, artificial lung expanding compound (ALEC) and Exosurf, have been evaluated in clinical trials of surfactant therapy. Artificial lung expanding compound was used initially as a dry powder, but is now prepared as a crystalline suspension in saline at 4°C. In this study we compared the biophysical properties of three different forms of ALEC (dry powder, crystalline suspension at 4°C and 37°C), Exosurf and surfactant TA (Surfacten) using a modified Wilhelmy surface balance and a pulsating bubble surfactometer. Surface activity of a crystalline suspension of ALEC in cold saline was no better than the dry powder of ALEC. Surfactant activity of ALEC was improved by addition of hydrophobic surfactant protein B and C (SP‐B, SP‐C) which are important constituents of surfactant TA. Surface properties of ALEC in any form and Exosurf were not superior to those of surfactant TA. These results suggest that a surfactant which contains SP‐B and SP‐C does not necessarily have to be dry or crystalline for an effective exogenous surfactant.
Urinary prolactin excretion in children with renal disease
Authors: DE FELICE CLAUDIO , TANAKA TOSHIAKI , HAYAKAWA K. , ITOH KATZUMI , WATANABE TAKAYUKI , KATSUMATA NORIYUKI , HIBI ITSURO
Published: January 2011

The contribution of the kidney to the metabolism of prolactin has not yet been established. In the present study, urinary prolactin concentrations in 30 children with renal disease were measured by a newly devised, highly sensitive, time‐resolved immunofluorometric assay. Median prolactin concentrations in the urine of children with renal disease, were 1.86 pmol/L−1 of urine (range: 0.17–546.31 pmol/L−1. By stepwise regression analysis, change in urinary prolactin levels as a function of the urinary β 2‐microglobulin concentrations was detected. These results indicate that prolactin urinary excretion in children with renal disease is dependent on the renal proximal tubular function.
T cell subsets In peripheral blood and cerebrospinal fluid from children with aseptic meningitis
Authors: TABATA NOBUTADA , OITANI KAICHI , MORITA KUMIKO , SHOGO YUKIO , SAKURAI NAKAO , ZHANG SHAO‐LI , ZHOU YAN‐WEN , KOMADA YOSHIHIRO
Published: January 2011

T cell subsets in peripheral blood (PB) and cerebrospinal fluid (CSF) obtained from patients with aseptic meningitis were studied using quantitative two‐color fluorescence analysis with a flow cytometer. The percentage of HLA‐DR+/CD3+ lymphocytes (activated T cells) in CSF was significantly increased in the recovery phase when compared to the acute phase, while no significant change in the activated T cells in PB was observed. More interestingly, CD4+ T lymphocytes in CSF were increased in the acute phase and subsequently decreased in the recovery phase. Instead, CD8+ T lymphocytes gradually accumulated into the CSF in the recovery phase, resulting in a successive decrease in the CD4/CD8 ratio. On the other hand, the CD4/CD8 ratio in PB remained normal during the course of aseptic meningitis. The present results suggest that T lymphocytes (CD4+ subset in the acute phase and CD8+ in the recovery phase) could be infiltrated and further activated at the site of inflammation, possibly in the subarachnoid space in the patients with aseptic meningitis.
Inhibitory effects of diazoxide or polymyxin B on glucose transport by isolated rat erythrocytes or adipocytes
Authors: GOTO YOSHINORI , KIDA KAICHI , KAINO YUKIKAZU , ITO TAKUO , MATSUDA HIROSHI
Published: January 2011

The inhibitory effects of diazoxide or polymyxin B on 3‐O‐methylglucose uptake were studied in isolated rat erythrocytes or adipocytes to elucidate the mechanisms of the actions of these agents. One to three mmol/L diazoxide significantly inhibited 3‐O‐methylglucose uptake into erythrocytes by 11–33% without altering the equilibrium space, while 0.3 mmol/L diazoxide did not. The inhibitory effect was exerted in a dose‐dependent manner in this concentration range. To test whether polymyxin B affects the process of insulin action or the glucose transport activity recruited by insulin, adipocytes prestimulated with insulin and exposed to 2 mmol/L potassium cyanide (KCN) were employed since the cells, on which glucose transporters recruited by insulin were located quiescently, were useful to estimate the effect of an agent on glucose transport activity per se. Polymyxin B (100 μg/mL) inhibited the insulin‐stimulated uptake activity in this transport system by 22.5% while it inhibited the insulin‐stimulated uptake activity in intact adipocytes which were not exposed to KCN by 32.2%. These results suggest that diazoxide inhibits the function of the erythrocyte glucose transporter, GLUT1*** (classified by Bell et al.), and indicate that the inhibition of the glucose transport activity recruited by insulin is the major effect of polymyxin B (100 μg/mL) and the inhibition of the process of insulin action is rather small.
Red blood cell sorbitol in diabetic children
Authors: ISHIDA YUJI , OKAMOTO RYOZO , MINO MAKOTO , KONISHI KAZUTAKA
Published: January 2011

When red blood cells (RBC) were incubated with various concentrations of glucose, the RBC sorbitol level increased in a concentration‐dependent manner. The elevated RBC sorbitol level was not reduced by further incubation in a glucose‐free medium.

In both diabetic and non‐diabetic children, an increase of RBC sorbitol levels occurred in the oral glucose tolerance test and the return to baseline was delayed in diabetics compared with non‐diabetics.

In the majority of diabetics (87%), RBC sorbitol levels exceeded the upper limit of the normal range, which was arbitrarily determined as the mean + 2 s.d., in healthy non‐diabetic children and adults.

A good correlation was observed between RBC sorbitol levels and plasma glucose levels (r = 0.644). In both diabetics and non‐diabetics, no correlation was observed between RBC sorbitol levels and age, and in diabetics the RBC sorbitol level was not related to the duration of disease.

A good correlation was observed between RBC sorbitol levels, and hemoglobin Alc (HbAlc) or fructosamine levels in diabetic children.
Importance of mechanical damage to urinary red blood cells by the glomerular basement membrane
Authors: ISHIHARA YUKIHIRO , MORITA MIYAKO , MATSUYAMA TAKESHI , IKEDA MASAHIRO , KAWAHARA KUZUHIKO , KAWAMURA KEN , KAMIYAMA YASUNORI , HONDA MASATAKA , HASEGAWA OSAMU , ITO HIROSHI
Published: January 2011

The reasons for morphological changes of urinary red blood cells (RBC) in patients with glomerulonephritis are still controversial. In order to evaluate the importance of mechanical damage by the glomerular basement membrane (GBM), we examined urinary RBC taken from the patients with two different diseases which have characteristic GBM changes. Urinary RBC taken from 20 patients with Alport syndrome and nine with thin GBM disease were examined using a scanning electron microscope. Nineteen out of the 20 patients (95.0%) with Alport syndrome showed ‘glomerular type’, while five of the nine patients (55.6%) with thin GBM disease showed ‘glomerular type’. These results suggest that more complicated GBM abnormalities cause more severe RBC distortion. Therefore, we conclude that mechanical damage by the GBM may be the major factor in dysmorphism of urinary RBC.
Effect of corticosteroids in hereditary spherocytosis
Authors: DURU FERIDE , GÜRGEY AYTEMIZ
Published: January 2011

Prednisolone was administered to three children with hereditary spherocytosis. At the time corticosteroid therapy was started, two of the patients were suffering from severe hemolytic anemia and the 3rd patient had partially recovered from hemolytic anemia after splenectomy. All of the patients prednisolone produced a remarkable increase in the hemoglobin levels with a concomitant increase in reticulocyte counts.

We conclude that corticosteroids might stimulate erythropoiesis and may be an alternative therapy to red cell transfusion in pre‐operative states for patients with hereditary spherocytosis.
Agnogenic myeloid metaplasia in childhood: A report of two cases and efficiency of intravenous high dose methylprednisolone treatment
Authors: ÇETINGÜL NAZAN , YENER ERDAL , ÖZTOP SENAY , NISLI GÜNGÖR , SOYDAN SALIHA
Published: January 2011

Myelofibrosis with myeloid metaplasia, or agnogenic myeloid metaplasia (AMM) is a chronic myeloproliferative disorder characterized by fibrosis of the bone marrow accompanied by aniso‐ and poikilocytosis, leukoerythroblastosis and hepatosplenomegaly with extramedullary hematopoiesis. Agnogenic myeloid metaplasia is very rare in children. In this report, two cases of AMM in whom the onset of the illness were at 3 and 12 months of age, are presented. Both had severe anemia, hepatosplenomegaly and bone marrow fibrosis. Lymph node biopsy of the first patient and liver biopsy of the second revealed extramedullary hematopoiesis. They were treated with an intravenous high dose of methylprednisolone (daily 30 mg/kg for 3 days, 20 mg/kg for 4 days, 10 mg/kg for 1 week, 5 mg/kg for 1 week). A complete improvement of hematological and clinical findings was observed.
Early onset of cecal perforation in neonatal, recto‐sigmoid type Hirschsprung's disease
Authors: YAMAMOTO TOSHIHARU , HAYASHI YOSHIHIRO , SUZUKI HIDEAKI , TAHARA TAKAHIRO , FUJISAWA KOHJI , ETO YOSHIKATSU , YAMAZAKI YOJI , MAEKAWA KIHEI
Published: January 2011

Hirschsprung's disease has been considered to cause intestinal perforation in rare cases. Even if a perforation occurs, the majority of cases are associated with the long‐segment or total colonic type. Our case developed the perforation in the neonatal period in spite of being of the recto‐sigmoidal type, and it affected the cecum. We do not have a good explanation for this condition. However, the pathological examination of the specimens of the perforated cecum revealed some necrosis (ulceration, subcutaneous hemorrhage, congestion and severe edema) which was considered to be caused by ischemia, secondary to a localized vascular accident in the wall of the distended intestine.
Growth and development of 280 hypothyroids patients at diagnosis
Authors: ÖZER GÜLER , YÜKSEL BİLGİN , KOZANOǦLU MUSTAFA , SERBEST MURAT , TURGUT ÇİǦDEM
Published: January 2011

The growth and development of 280 hypothyroidic patients who were diagnosed from 1979 to 1991, has been evaluated by standardized anthropometric criteria. According to the chronologic age during the diagnosis, the patients were divided into five groups of 0–6, 7–12, 13–24, 25–60 and 61–144 months. In these groups, the rates of the patients' weights and heights which were found below the 5th percentile, were as follows; for height 44%, 69%, 71%, 85%, 80% and for weight 38%, 68%, 70%, 55%, 60% respectively. Height age, weight age, head circumference age, and bone age were found to be significantly lower than chronologic age in all the groups, with the exception of the head circumference age in the 0–6 months group. The bone age was found to be significantly retarded compared to the height age in all the groups.
Influence of parental obesity on the physical constitution of preschool children in Japan
Authors: HASHIMOTO NAOSHI , KAWASAKI TAKUYA , KIKUCHI TORU , TAKAHASHI HIDEO , UCHIYAMA MAKOTO
Published: January 2011

The objective of this study was to assess the influence of parental obesity on the physical constitution of preschool children. A total of 3187 children aged between 1–6 years and their parents were studied. A child whose per cent obesity (%OB; per cent overweight for age, height and sex) was greater than 15%, and a parent whose body mass index (BMI; kg/m2) was greater than the 95th percentile were defined to be obese (27.40 and 25.97 for a father and a mother, respectively). We found that the incidence of obesity in children with obese fathers (11.5%) was significantly higher than in those with non‐obese fathers (6.2%), and a similar difference was obtained between children with obese mothers (14.5%) and with non‐obese mothers (6.2%), respectively. The incidence of obesity in children was 6.0% if both parents were non‐obese; this incidence rose to 22.7% if one parent was obese, and to 30.8% if both were obese. The %OB of children was more markedly correlated with the mothers' BMI (r = 0.219) than the fathers' BMI (r = 0.165). The %OB of children correlated significantly with fathers' BMI, but only from the age of 3–6 years, whereas mothers' BMI correlated from the age of 1–6 years.

We concluded that parental obesity was of significance in determining the development of juvenile obesity even in the preschool period.
Ex vivo expansion of umbilical cord blood hematopoietic progenitor cells by combinations of cytokines
Authors: URASHIMA MITSUYOSHI , HOSHI YASUTAKA , AKIYAMA MASAHARU , KAMIJO MAKOTO , ATO YOKO K , AKATSUKA JUN‐ICHI , MAEKAWA KIHEI , MATSUZAKI MASAHARU , OHNISHI JUN , KAIHARA CHIHARU
Published: January 2011

Ex vivo expansion of hematopoietic progenitor cells in the umbilical cord blood mononuclear cells (CB‐MNC) was investigated in liquid culture system with various combinations of cytokines (stem cell factor [SCF], interleukin [IL]‐3, IL‐6, granulocyte‐colony stimulating factor [G‐CSF], erythropoietin [EPO], and interferon [INF]‐γ). Non‐lineage‐committed hematopoietic progenitor cells and lineage committed hematopoietic progenitor cells were represented as CD34+CD38− and CD34+CD38+ subpopulations, respectively. Although absolute CD34+CD38− cell numbers decreased even in the presence of multicytokines, the combinations of SCF plus IL‐6 and SCF plus IL‐3 plus IL‐6 plus INF‐γ were significantly effective in maintaining CD34+CD38− cells than the other combinations (P < 0.05). After 4 weeks of culture, CD34+CD38− cells disappeared in all combinations of cytokines. Absolute CD34+CD38+ cell numbers increased in the presence of cytokines. Maximal expansion of CD34+CD38+ cells were observed in the combinations of SCF plus IL‐3 plus IL‐6 plus EPO (19.8 ± 3.3 ‐fold) and SCF plus IL‐3 plus IL‐6 plus G‐CSF (18.3 ± 2.6). The combination of SCF plus IL‐3 plus IL‐6 was also effective to expand CD34+CD38+ cells (15.8 ± 3.9). However, the expansion was transient and they decreased to zero within 3 weeks. In the combinations of SCF plus IL‐6 and SCF plus IL‐3 plus IL‐6 plus INF‐γ, maximal expansion was inferior to the others but CD34+CD38+ cells were maintained more than 4 weeks. These results suggested that the indication of CBT can be expanded into older children by ex vivo augmentation of CB hematopoietic progenitor cells using multi‐cytokines.
Inhaled nitric oxide and extracorporeal membrane oxygenation in persistent pulmonary hypertension of the newborn
Authors: TAKATA MASAO , MIYASAKA KATSUYUKI , SAKAI HIROKAZU , FUJIWARA HIROYUKI , ITO YUSHI , KAWANO TOSHIO
Published: January 2011

Persistent pulmonary hypertension of the newborn (PPHN) may occasionally require an invasive treatment with extracorporeal membrane oxygenation (ECMO). Inhaled nitric oxide (NO) has recently been introduced as a selective pulmonary vasodilator for treatment of PPHN. We describe a case of PPHN in which neither inhaled NO nor ECMO was effective in reversing pulmonary hypertension. The clinical course of the patient suggested a potential role of NO inhalation in predicting the outcome of ECMO treatment for PPHN.