bluuebunny/crossref_metadata_2025 huggingface dataset:
Evaluation of diagnostic criteria of acute renal failure in premature infants
Authors: ISHIZAKI YUKO , ISOZAKI‐FUKUDA YUKA , KOJIMA TAKATSUGU , SASAI MISA , MATSUZAKI SHEUJI , KOBAYASHI YOHNOSUKE
Published: January 2011

A prospective study was performed to investigate the validity of renal failure index (RFI) or fractional excretion of sodium (FENa) in preterm infants. The subjects were 128 newborn infants, 72 with oliguria and 56 without renal dysfunction (control). Oliguric infants were divided into two categories: acute renal failure (ARF) and prerenal failure (PRF), according to creatinine clearance (Ccr). Furthermore, all subjects were divided into four groups according to gestation, that is, 38 infants with gestational age of 25–28 weeks (group 1), 28 with 29–30 weeks (group 2), 38 with 31–36 weeks (group 3) and 24 of > 37 weeks (group 4). As a result, differentiation between ARF and PRF was valid when the RFI or FENa was used in infants of > 29 weeks gestation (groups 2, 3 and 4). Although infants of > 31 weeks gestation (groups 3 and 4) who present with an RFI > 3 or an FENa > 3% may be diagnosed as having ARF, infants in group 2 with an RFI of > 8 or an FENa of > 6% may be diagnosed as having ARF. For the infants in group 1, the application of RFI or FENa for diagnosis of ARF may be limited because of some overlap among the groups.
Urinary arginine vasopressin (AVP) measurement in children: Water deprivation test incorporating urinary AVP
Authors: SHIMURA NAOTO
Published: January 2011

The value of a water deprivation test incorporating urinary arginine vasopressin (AVP) measurement was investigated in 13 patients with polydipsia and/or polyuria (complete central diabetes insipidus [CCDI] in four; incomplete central diabetes insipidus [ICDI] in five; secondary nephrogenic diabetes insipidus [NDI] in three; compulsive water drinking [CWD] in one) and a group of 25 control subjects (C). Urine samples were collected after water deprivation during sleep and the urinary osmolalities and AVP concentrations were measured.

Analysis of the results of 104 urine samples from the 25 control subjects demonstrated a close correlation between urinary osmolality and AVP (r = 0.89, P < 0.001). After water deprivation during sleep, the respective mean maximal urinary osmolalities and AVP concentrations were: 127.4±34.4 mOsm/kgand 1.1±0.5 pg/mL in the patients with CCDI (14 samples, four children); 410.3±101.8 and 6.1±3.5 in those with ICDI (16 samples, five children); 348.7±71.2 and 100 + 45.1 in those with NDI (nine samples, three children); 541.5±143.5 and 43.6±33.2 in the patient with CWD (two samples, one child) and 898.8±186.3 and 97.4±50.4 in group C (54 samples, 18 children). Furthermore, the urinary AVP level relative to the osmolality in each patient varied depending on the AVP secretion status and renal concentrating ability. Each patient, except the one with CWD, could be discriminated from the normal subjects using this test. It seems that this test is easy to perform and useful for diagnosis and follow‐up of patients with partial/complete posterior pituitary function defects and those with renal concentration impairment.
Calcification of basal ganglia in a patient with partial trisomy 5q and partial monosomy 18q
Authors: NAKAYAMA TSUGUYO , SAKAKIHARA YOICHI , HANAOKA SHIGERU , AKAGI KUNIHIKO , KAMOSHITA SHIGEHIKO
Published: January 2011

A patient with partial trisomy for the distal segment of the long arm of chromosome 5 (q35.1 ← qter) with partial 18q monosomy is presented. The mother of the patient was phenotypically normal and was proved to be a carrier of a reciprocal translocation of the long arm of chromosomes 5 and 18 46,XX,t(5;18)(q35.1;q23). The patient shows mild mental retardation, short stature, mild obesity, dysmorphic face, eczema, minor malformations of the extremities, and bilateral intracranial calcification in the basal ganglia. Most of the clinical manifestations of the patient are compatible with the previously reported clinical features of partial trisomy of the distal segment of 5q. However, the calcification of bilateral basal ganglia has not been reported for this chromosomal anomaly.
Difficulty in removing a percutaneous central venous catheter inserted from a peripheral vein
Authors: MORIMOTO TSUYOSHI , HOSOYA RYOHTA , MATSUFUJI HIROSHI , TACHI MASAHIRO , YOKOYAMA JOHTARO , NISHIMURA KOZO
Published: January 2011

Two girls with acute lymphocytic leukemia (ALL) are reported. There were difficulties in removing their central venous catheters inserted from a peripheral vein. One girl required surgery under general anesthesia for the removal. The other patient received continuous infusion of low dose urokinase from a distal peripheral vein. The residual catheter was then removed successfully.
A long‐term suppression by α‐interferon of Philadelphia chromosome in children with chronic myelogenous leukemia
Authors: KIKAWA YOSHIHARU , FUJI YASUSHI , TAKANO TOMOKO , SHIGEMATSU YOUSUKE , SUDO MASAKATSU , OKAMOTO MASANORI , MIZUTANI SHUKI
Published: January 2011

An 8 year old girl with adult type Philadelphia (Ph1)‐positive chronic myelogenous leukemia received natural α‐interferon therapy in the chronic phase. Complete suppression of the Ph1 clone of bone marrow cells was achieved after 1 month of therapy, which was determined by disappearance of rearranged breakpoint cluster region (BCR) gene in Southern blot analysis. Complete hematological remission was also attained following 2 months of therapy. Both the suppression and the hematological remission have been sustained for 24 months with α‐interferon. in spite of the detection of the chimeric BCR/ABL mRNA in her bone marrow by polymerase chain reaction assay 12 months after therapy.
Assessment of body composition measured by bioelectrical impedance in children
Authors: IWATA KYOKO , SATOU YOSHIYUKI , WATA FUJIHIKOI , HARA MITSUHIKO , FUCHIGAMI SACHIKO , KIN HIDEO , FUCHIGAMI TATSUO , KADA TOMOOO , HARADA KENSUKE , OHKUNI MASAHIKO , RYOU SHIGEO , OHASHI SHUNJI
Published: January 2011

Bioelectorical impedance methods have been found to be a valid and reliable way of estimating per cent body fat (%BF) in adults. We applied them to healthy children and compared them with conventional anthropometry methods.

One thousand two hundred and sixteen children participated in this study. Impedance and skinfold thickness were measured, and %BF was estimated using these values. Bodyweight and height were measured, and per cent obesity (%OB) and body mass index (BMI) were obtained.

The values of %BF by the bioelectrical impedance method (%BFi) were 8.6 ± 4.0% in the junior male group, 14.2±2.8% in the junior female group, 7.9±4.7% in the senior male group and 16.1 ±2.9% in the senior female group. The % BFi correlated strongly with skinfold thickness, %OB and BMI. Thus %BFi correlated strongly with variables from conventional methods. It was concluded that it is a reliable way of assessing lipid storage in children.
Relapses after termination of therapy of acute lymphoblastic leukemia in children
Authors: OCHOCKA MARIA , MATYSIAK MICHAL , KULUS MAREK , ARMATA JERZY , BALWIERZ WALENTYNA , BOGUSLAWSKA‐JAWORSKA JANINA , CHYBICKA ANNA , JACKOWSKA TERESA , JAKOWICKA MARIA , MICHALEWSKA DANUTA , PIETRAS WOJCIECH , RADWAŃSKA URSZULA , ROKICKA‐MILEWSKA ROMA , RYTLEWSKA MARIA , SONTA‐JAKIMCZYK DANUTA , SROCZYNSKA MARIA , SLADKOWSKA DANUTA , TACIK JUSTYNA
Published: January 2011

In the past 16 years, 2004 children with acute lymphoblastic leukemia (ALL) have been treated in the Polish Pediatric Group centers. Eight hundred and eighty‐seven (44.3%) of these patients discontinued treatment after the first remission. Acute lymphoblastic leukemia relapse occurred in 180 patients (20.3%). This group was analyzed for the method of treatment and its influence on long‐term survival, the time between cessation of treatment and relapse, the character and localization of relapse and later follow‐up. It was shown that the patients with the best chance of a second remission are those with late testicular relapse. The most frequent and prognostically poor are bone marrow (BM) relapses which warrant intensive chemotherapy with BM transplantation. Patients with ALL relapse still have the possibility of a second remission and long‐term survival.
Increased urinary excretion of human cytomegalovirus in children with malignancy: Detection by polymerase chain reaction
Authors: KAJIWARA MICHIKO , YAMAGUCHI YUKI , HIRAI KANJI , YATA JUNICHI
Published: January 2011

Human cytomegalovirus (HCMV) is one of the most important agents causing opportunistic infections in immunocompromised hosts. In this study, we examined the urinary excretion of HCMV in children with malignancy using polymerase chain reaction (PCR).

Urine samples were collected from on‐therapy, off‐therapy patients with malignancy, and healthy controls. A simple DNA extraction method using glass powder was employed, and inhibitory effect of urine on PCR was prevented. For PCR, a pair of primers from the HCMV major immediate early gene sequence was used.

Among patients who received intensive chemotherapy, 52.0% had urinary HCMV excretion after the chemotherapy course. In contrast, off‐therapy patients and healthy controls showed a lower incidence of urinary HCMV excretion (20.4 and 8.7%, respectively). The incidence of HCMV urinary excretion in the on‐therapy group was significantly higher than healthy controls (P < 0.05). In the on‐therapy group, the total white blood cell count of the virus excreters was lower than that of non‐excreters.

The incidence of HCMV excretion was high in on‐therapy patients. Most of the virus excreters were seropositive, so their viruria was thought to be caused by reactivation. Repeated monitoring of virus excretion by this rapid and simple method may be useful to detect HCMV infection early and to control it in such patients.
Congenital heart disease in the adult
Authors: LINDE LEONARD M.
Published: January 2011

With improved medical and surgical care, more patients with congenital heart disease are now surviving to adulthood and presenting with previously unobserved problems. This review discusses the course of older operated and unoperated patients as far as physical and psychosocial problems and suggests methods of dealing with these previously unencountered clinical situations.
International cooperation with Islamabad Children's Hospital, Pakistan: The JICA project
Authors: YAMASHITA FUMIO , URABE DAISAKU , ITO HARUMICHI , KONDO SHIGENOBU , HANAWA YOSHIYUKI
Published: January 2011

The experience gained during 7 years of cooperation between the Japan International Cooperation Agency (JICA) and the Islamabad Children's Hospital (JICA‐ICH project, July 1986‐June 1993) is described. Islamabad Children's Hospital achieved the goals of the project and became a centre for excellence in health care, education and research for children, fulfilling the objectives of the project. This achievement was evaluated as one of the most successful projects in medical cooperation ever performed by JICA by a third party evaluation team. The problems arising and the lessons experienced through the process are discussed. The importance of the role which should be undertaken by pediatricians in international cooperation with developing countries is emphasized.
Maternal and child health in Indonesia
Authors: OKAMOTO AKIRA
Published: January 2011

AIIKU‐HAN activity was initiated by the Imperial Gift Foundation BOSHI‐AIIKU‐KAI in Japan in 1936 and has been introduced and accepted to community‐based maternal and child health development in Indonesia through the international cooperation project conducted by Japan International Cooperation Agency (JICA) in order to encourage community health activities carried out by Indonesian women volunteers.

As AIIKU‐HAN activity in Japan and Dasa Wisma health activity in Indonesia have much in common, transferring concepts, methods and experiences met less obstacles when Indonesian community health volunteers learned AIIKU‐HAN activity.

Experience gained while developed countries were less developed can be transferred by shifting the time‐scale of history in developed countries.
Ghana/JICA/WHO training course in polio diagnostic procedures and vaccine potency testing
Authors: KOBAYAKAWA TAKATOSHI
Published: January 2011

The Ghana/Japan International Cooperation Agency/WHO training course in polio diagnostic procedures and vaccine potency testing was initiated at the Noguchi Memorial Institute for Medical Research in Ghana for 5 consecutive years from 1992. The purpose of the course is to create virologists and technicians competent in the laboratory methods to support the polio eradication initiative in Africa. The participants are invited mainly from anglophone countries in the region.
The joint JMA‐JICA project in Nepal
Authors: KURATSUJI TADATOSHI
Published: January 2011

Nepal is among the 20 countries with the lowest human development index, according to the criteria of the United Nations Development Program (UNDP), and has a very high under‐five mortality rate, according to the United Nations Children's Fund (UNICEF).1 In December 1992, the Japan Medical Association (JMA) and the Japan International Cooperation Agency (JICA) began jointly carrying out a maternal and child health (MCH) project. Its beneficiaries are the population of Kavre District, the District Public Health Office in Kavre, the Central Region Health Directorate, and the Ministry of Health of Nepal.

The MCH project stems from a request to Japan made by His Majesty's Government of Nepal to implement and evaluate a new health system policy in a model area, the Kavre District. The project's main goal is to improve the general health and to reduce the maternal, infant, and under‐five child mortality rates by providing basic primary health care services and means of disease prevention. It will improve the government's medical care delivery system, better educate the population and promote community action in questions related to health and hygiene.

This is the first cooperative project organized by JICA that involves the participation of a nongovernmental organization, in this case, the JMA. The JMA will construct the primary Health Care (PHC) Center, equip it with a dormitory and safe water supply system, and provide sufficient drugs for 2 years. The Japan International Cooperation Agency will dispatch MCH experts and provide medical equipment and supplies. The government of Nepal will dispatch a medical staff of 12 members, including a medical doctor, nurses, midwives, and laboratory technician, and other assistants as counterparts, and will be supported and monitored by the National Children's Hospital Medical Research Center of Japan. This joint project and new health policy will be evaluated after 2 years to decide whether the new PHC center system should be expanded.
Pediatric and MCH training in Japan: JICA training program in the National Children's Hospital
Authors: NAKANO MIWAKO
Published: January 2011

The experience of the training program for overseas doctors in the National Children's Hospital during 8 years is described. The program was supported by the Japan International Cooperation Agency (JICA) and the objectives of the training course are to provide doctors from developing countries with a better understanding of diagnosis and treatment as specialized pediatricians and pediatric surgeons and to introduce recent medical techniques and equipment for child care. From 1984 to 1993, 37 doctors from 22 developing countries of Asia, Middle and South America and Africa have attended this program. There were 22 pediatricians, 14 pediatric surgeons and one dentist. The outline of this group training program and problems with the course are discussed.
Japan‐Russia Pediatric Society
Authors: NIHEI KENJI , THUNEMATHU YUKIKO , KOBAYASHI NOBORU
Published: January 2011

In March 1990, medical interchange between Japan and the Soviet Union began with a letter from the local health bureau of Khabarovsk. We visited Khabarovsk three times and Kamchatka once, and saw many hospitals and patients. Russian doctors of pediatrics visited Japan. Medical information was exchanged and discussed. The Japan‐Russia Pediatric Society was established to perform interchange of medical information, technology and staff such as doctors, nurses and technicians between Japan and Russia, especially the Far East district of Russia. The Society meeting has been held three times: Tokyo (1991), Khabarovsk (1992) and Niigata (1993). It is necessary to continue the interchange between the two countries.
Cooperative program for Asian pediatricians
Authors: SAKAKIHARA YOICHI , NAKAMURA YASUHIDE
Published: January 2011

The Cooperative Program for Asian Pediatricians (CPAP) is a non‐government organization established in 1989 to promote mutual understanding and friendship among young pediatricians in Asian countries. Unlike other government programs and non‐government organizations, CPAP is solely facilitating mutual relationships among young inexperienced pediatricians who would otherwise have no chance to travel overseas.

It has been funded by donations from members of the alumni association of the Department of Pediatrics at the University of Tokyo and many private companies and individuals.

The Cooperative Program for Asian Pediatricians has so far invited 36 Asian pediatricians from 11 countries. By constructing a human network among Asian pediatricians, it is hoped that CPAP will contribute to making international cooperation in the Asian region easier and smoother.
Resistance to thyroid hormone and its molecular basis
Authors: REFETOFF SAMUEL
Published: January 2011

Generalized resistance to thyroid hormone (GRTH) is an inherited syndrome characterized by hyposensitivity of target tissues to thyroid hormone. The clinical presentation is variable. The syndrome is usually suspected when elevated serum thyroid hormone levels are associated with a non‐suppressed thyroid‐stimulating hormone (TSH). While goiter and thyroid test abnormalities have more often led to the suspicion of thyroid gland dysfunction, short stature, hyperactivity, learning disability and goiter in children or adolescents and recalcitrant goiter in adults, should raise the suspicion of GRTH. Hypothyroidism has been considered when growth or mental retardation was the presenting symptom and thyrotoxicosis when confronted with attention deficit, hyperactivity or tachycardia. Failure to recognize the inappropriate persistence of TSH secretion in spite of elevated thyroid hormone levels has commonly resulted in erroneous diagnosis leading to antithyroid treatment. More than 300 subjects with this syndrome have been identified. The mode of inheritance in the majority of families is autosomal dominant. Recessive transmission has been found in only one family.

It has long been speculated that this defect is likely to be caused by an abnormal thyroid hormone receptor (TR), but this hypothesis could not be directly tested until the isolation of two TR genes, TRα and TRβ. Mutations in the TRβ gene have been identified in 42 families with GRTH. All are located in the T3‐binding domain straddling the putative dimerization region and exhibit various degrees of hormone‐binding impairment. This finding, and the fact that heterozygous subjects with complete TR deletion are not affected while those with point mutations are, indicates that interactions of a mutant TR with normal TR and with other factors are responsible for the dominant inheritance of GRTH and its heterogeneity. Elucidation of the etiology of GRTH has not only added a new means for the early diagnosis of the syndrome but provided new insights in the understanding of the mechanism of hormone action.
Actions of peroxovanadate or tungstate on glucose transport by isolated rat adipocytes
Authors: GOTO YOSHINORI , KIDA KAICHI , KAINO YUKIKAZU , ITO TAKUO , MATSUDA HIROSHI
Published: January 2011

The effects of peroxovanadate or tungstate on 3‐O‐methylglucose uptake were characterized using isolated rat adipocytes to elucidate the mechanism(s) of their actions. The stimulatory effect of peroxovanadate was observed from 1 μmol/L and reached the maximum at about 100 μmol/L. The concentration showing the half‐maximal effect was approximately 16 μmol/L. The maximal response of peroxovanadate was 1.19 times higher than that of insulin significantly (P < 0.01). On the other hand, the stimulatory effect of tungstate was seen only at the higher concentrations of 10–30 mmol/L Judging from the experiments using different tungsten compounds, tungstic acid (WO42‐) appeared responsible for the effect. The effects of 20 mmol/L tungstate and 20 nmol/L insulin were not additive. The stimulatory effects of 1 mmol/L peroxovanadate, 20 mmol/L tungstate or 20 nmol/L insulin were not seen in the adipocytes deprived of ATP by exposure to 2 mmol/L KCN. The adipocytes which had been stimulated with insulin and further exposed to 2 mmol/L KCN were used to test whether or not peroxovanadate works directly on the function of glucose transporters. In such cells on which GLUT4‐rich transporters were rendered immobile, the effect of peroxovanadate was not observed. These results indicate that the effects of peroxovanadate or tungstate are ATP or energy dependent and may be exerted through the mechanism analogous to that of insulin action, and suggest that peroxovanadate does not directly activate the function of GLUT4.
Long‐term effects of bone marrow transplantation for inborn errors of metabolism: A study of four patients with lysosomal storage diseases
Authors: IMAIZUMI MASUE , GUSHI KAZUO , KUROBANE IKUO , INOUE SHIGEO , SUZUKI JUN , KOIZUMI YOSHITSUGU , SUZUKI HOSHIROU , SATO ATSUSHI , GOTOH YOU‐ICHI , HAGINOYA KAZUHIRO , KIKUCHI MASAHIRO , AIKAWA JUN‐ICHIROU , NARISAWA KUNIAKI , OHUNUMA AKIRA , OHMURA KIYOSHI , SHINTANI HARUO , TANAKA AKEMI , TADA KEIYA
Published: January 2011

Long‐term effects of bone marrow transplantation (BMT) were evaluated in patients with I‐cell disease, metachromatic leukodystrophy (MLD), Maroteaux‐Lamy syndrome or Hunter syndrome (mild form). Donors were human leukocyte antigen (HLA)‐matched siblings, and the follow‐up periods were 24–71 months after BMT. The enzyme activities were increased in leukocytes, plasma or liver tissues compared with pre‐BMT levels. A patient with I‐cell disease acquired development of 4–8 month old infants and showed no further progression in cardiac dysfunctions. A patient with MLD showed a decelerated disease progression and an improved peripheral neuropathy, but progressive brain atrophy was not prevented. Patients with Maroteaux‐Lamy syndrome or Hunter syndrome showed improvements in hepatomegaly, joint contractures, short stature and tight skin, and this greatly increased their quality of life. These results indicated that the long‐term therapeutic effects achieved by BMT were subject to multiple factors including biochemical improvements, a reversibility of affected tissues, or advanced states of disease and central nervous system impairments in inborn errors of metabolism.
Changes in hypothalamic‐pituitary function following bone marrow transplantation in children
Authors: KUBOTA CHIDORI , SHINOHARA OSAMU , HINOHARA TOMOYUKI , HATTORI KINYA , YABE HIROMASA , YABE MIHARU , KATO SHUNICHI , TAKAKURA IWAO
Published: January 2011

Patients who undergo bone marrow transplantation (BMT) frequently experience impaired pituitary function, but precise assessment using repeated provocative tests has not been described. We studied 32 children (16 boys) who had BMT after receiving preparative irradiation. Assessment of pituitary function was performed by infusing insulin, luteinizing hormone‐releasing hormone (LHRH), and thyrotropin‐releasing hormone (TRH) on several occasions at various intervals during the follow‐up period.

Serum free thyroxine (FT4) and thyrotropin (TSH) levels tended to be low during the early period following BMT. Serum FT4 concentrations reverted to the low‐normal range 1 year after transplant, and eight of 29 patients had subnormal and delayed TSH response to TRH consecutively. No children showed overt hypothyroidism. Basal and peak serum gonadotropin levels in response to LHRH were elevated in the patients who had received transplant around the time of puberty. Leydig cell function assessed by human chorionic gonadotropin test was normal. Three girls experienced menarche, and one male patient fathered a normal boy 7 years after BMT. Pituitary‐adrenal function and prolactin secretion were not affected.

A high incidence of transient hypothyroidism which did not require replacement therapy and gonadal failure among pubertal children were observed. Shielding of gonads should be attempted, if possible, at the time of preparative irradiation to prevent resultant hypogonadism.
Cytotoxicity against Varicella zoster virus infected targets in children with acute leukemia
Authors: IHARA TOSHIAKI , OITANI KAICHI , TORIGOE SADAYOSHI , KITAMURA KENJI , ITO MASAHIRO , KAMIYA HITOSHI , SAKURAI MINORU
Published: January 2011

To eliminate the role of natural killer (NK), antibody‐dependent cell‐mediated cytotoxicity (ADCC), and polymorphonuclear leukocyte (PMN)‐mediated cytotoxicity in Varicella zoster virus (VZV) infections, peripheral blood mononuclear cell (PBMC)‐mediated NK and ADCC, and phorbol myristate acetate‐stimulated PMN‐mediated cytotoxicity against VZV‐infected targets were studied in children with leukemia.

Natural killer and PMN‐mediated cytotoxic activity was depressed for 6 months after complete remission and ADCC activity was depressed for 1 year after complete remission. The magnitude of three cytotoxic mechanisms in leukemic children gradually increased while they continued in complete remission.

These results suggested that decreased cytotoxic activities of PBMC and PMN might contribute to serious VZV infections and susceptibility to herpes zoster in leukemic children.
Gestational age assessment in Japanese low birthweight infants
Authors: KOGA YASUTSUGU , FUJIEDA KENJI , MATSUMOTO YOSHINORI , FUJIMOTO SEIICHIRO , HATTORI TSUKASA , HAGISAWA MASAHIRO , TAHARA YASUO , NAGASHIMA TETSURO , MIZUMOTO MASAHIKO
Published: January 2011

We evaluated the validity of the Ballard scoring system for assessing gestational age in Japanese low birthweight infants. Infants included in this study were 116 neonates who were admitted to seven hospitals in Hokkaido. Gestational ages of all infants were prenatally established by ultrasonographic measurement of the crown‐rump length performed between 8 and 12 weeks of gestation (clinical age). Of these, 74 infants were appropriate for gestational age (AGA) and the remaining were small for gestational age (SGA). Mean birthweight was 1703 g and 34% (39/116) were <1500 g. The correlation coefficient for the total population was 0.93(P <0.001). Although mean Ballard age tended to overestimate clinical age, mean differences between Ballard age and clinical age did not exceed 0.6 weeks. Agreement within 2 weeks among all infants was 86.2%. Percentages of agreement within 2 weeks did not differ to a statistically significant degree between AGA and SGA. These results indicate that the Ballard method is of value in assessing gestational age in Japanese low birthweight infants.
Biacromical and biiliac length and chest circumference measurements of male Turkish adolescents according to biological maturation
Authors: BÜYÜKGEBIZ A. , EROGLU Y. , KARAMAN Ö , KINIK E.
Published: January 2011

Adolescence is a period which is characterized by rapid physical growth and development of secondary sex characteristics. Male adolescence begins at 11–12 years of age and lasts ∼5 years. During this interval, there is a growth spurt period which is observed any time between 11 and 16 years of age, lasting 2–3 years. Some authors claim that a male may have nearly 20–25% of his adult height and 50% of his adult weight during adolescence.1–3 When evaluating the maturational aspects of adolescence, it is essential to consider bone age (BA) as well as chronological age (CA). Bone age is the mean CA at which the skeletal maturation is normally attained. It is influenced by genetic, endocrinological and nutritional factors.4–6 An adolescent may not demonstrate the expected maturational steps if his BA is retarded. This report examines the relationship between CA, biological maturation and some anthropometrical measurements.
Acute childhood leukemia in a patient with hemophilia: First report in Japan
Authors: KAWAKAMI KIYOSHI , TAKEZAKI TOSHIRO , NAKAZONO SHIN‐ICHI , KITAHARA TAKUMA , IKARIMOTO NAOAKI , TANAKA YUJI , MIYATA KOICHIRO , YOSHIOKA AKIRA
Published: January 2011

This study reports an unusual case of acute leukemia which was diagnosed as hemophilia A on initial admission for leukemia.

A 3 year old boy was admitted to Kagoshima University Hospital with anemia. He was diagnosed as acute lymphoblastic leukemia. At the same time he was revealed to have severe hemophilia A− without any previous episodes of severe bleeding tendency or family history of this disease. The laboratory investigation showed his mother to be a carrier of hemophilia A.

Although there are many cases of hemophilia which have developed malignant tumors, most of them were caused by association with human immunodeficiency virus (HIV) infection. Only five cases with coexistence of leukemia and hemophilia without HIV infection have been reported and the present case is the first one in Japan. At this stage, hemophiliacs are not necessarily regarded to be a population at risk for the development of leukemia. Furthermore, no particular subtype of leukemia was characterized among these patients in the literature.
Hemothorax due to hemorrhagic disease of the newborn
Authors: KAUR PYAR , TAN KAH KEE
Published: January 2011

A three day old male, term infant with hemothorax due to hemorrhagic disease of the newborn was treated successfully with vitamin K and thoracocentesis. Exclusive breast feeding and absence of vitamin K prophylaxis were important diagnostic clues, although hemothorax as a sole manifestation of hemorrhagic disease of the newborn is rare. This case highlighted the good prognosis of an uncommon complication when prompt diagnosis and appropriate treatment are instituted. The importance of vitamin K prophylaxis to all newborns is emphasized.
Helicobacter pylori colonization in children with gastritis and peptic ulcer. I. The colonization rate and effects of colonization on mucin content and mucosal inflammation in the antrum
Authors: YAMASHIRO Y. , OGUCHI S. , OTSUKA Y. , NAGATA S. , SHIOYA T. , SHIMIZU T.
Published: January 2011

The incidence of Helicobacter pylori infection and effects of H. pylori colonization on mucin content and mucosal inflammation of the antral mucosa were studied quantitatively in 55 Japanese children with suspected gastritis and peptic ulcers (aged 6–16 years, mean 12.3 years). H. pylori was detected, using Warthin‐Starry stain, in nine of the 22 cases (41%) with antral histological gastritis, but in none of the 33 histologically normal cases. Five out of seven duodenal ulcer cases showed histological gastritis, and all five cases were H. pylori positive. Severity of gastritis, evaluated by means of gastritis score, was significantly higher in H. pylori positive gastritis cases than in H. pylori negative gastritis cases (5.4 ± 1.0 vs 3.1 ± 0.3, P< 0.001). A PAS‐AB index, a proportion of the periodic acid Schiff‐alcian blue (PAS‐AB) positive mucin area to the total epithelial area, was significantly lower in H. pylori positive cases than in H. pylori negative cases, irrespective of the existence of histological gastritis (23.5±7.6% vs 40.4±5.5%, 43.5±4.2%, P< 0.001). The decreased mucin content of gastric mucosa is likely to lead to weakening of an important defensive factor of gastric mucosa. These findings suggest that H. pylori plays an important role in gastritis and peptic ulcers in children, especially in cases with duodenal ulcer.
Helicobacter pylori colonization in children with gastritis and peptic ulcer. II. Ultrastructural change of the gastric mucosa
Authors: YAMASHIRO Y. , OGUCHI S. , OTSUKA Y. , NAGATA S. , SHIOYA T. , SHIMIZU T.
Published: January 2011

To evaluate the role of Helicobacter pylori in the pathogenesis of gastritis and peptic ulcer in children, ultrastructural changes of the gastric mucosa with H. pylori colonization were studied in two pediatric patients with duodenal ulcers.

The study demonstrated that H. pylori: (i) colonized the mucous layer covering normal gastric epithelium; (ii) lay atop short, irregular microvilli and at the intercellular junctions of damaged cells; and (iii) adhered firmly by specific junction zone (firm connection) and loosely by filamentous appendages to the epithelial surfaces of cells which lacked evidence of a protective mucous layer. The ultrastructural changes, which become progressively worse from patterns (i) to (iii) mentioned above, suggest that these patterns represent distinct and successive stages of the infection. These findings, therefore, lend support to the notion that H. pylori infection is quite common and may relate to the pathogenesis of gastritis and peptic ulcer in children as well as in adults.
Clinical variability of congenital myopathy with type I fiber atrophy: A long‐term observation of three cases
Authors: SHISHIKURA KEIKO , OSAWA MAKIKO , SUZUKI HARUKO , HIRAYAMA YOSHITO , ARAI YUMI , SUMIDA SAWAKO , TAKAHASHI RIEKO , FUKUYAMA YUKIO
Published: January 2011

The variable clinical courses of three cases of congenital fiber type disproportion (CFTD) over a period of 10 years are presented. All showed improvement in early childhood, but subsequently, varying degrees of deterioration were noted: specifically, marked deterioration in case 2 and decreased muscle strength in case 3. Maximal motor function levels were attained differently among the cases. Histological findings included type 1 fiber hypotrophy and increased internal nuclei in common in all cases. Fine structural changes, such as patchy areas of myofibrillar degeneration, were noted in cases 1 and 2 (second biopsy), and cytoplasmic bodies were seen in case 2 (second biopsy). Myotubes were noted in case 3. The degree of cyto‐architectural changes did not correlate with clinical severity. The heterogeneity of CFTD is also discussed.
Care for bereaved families
Authors: FUKUI STEPHANIE
Published: January 2011

Because of support groups and studies on death, a new type of care for bereaved parents has been crafted over the past several decades. Support groups and medical professionals are key to this type of care. For medical professionals this job is difficult at times since individual needs may differ. But by following some basic guidelines, medical professionals can help the parents start the recovery process for they are the ones in contact with the parents at the time of death. The guidelines include giving the families complete medical information, reassurance and a chance to talk. Also, importantly, this care includes treating the baby and family with dignity and acknowledging the magnitude of the loss. In Japan most bereaved parents still go completely unassisted at the time of their baby's death. Formation of support groups and a greater awareness by medical professionals on how to care for these families will solve this problem.
Increased granulocyte‐colony stimulating factor (G‐CSF) levels in neonates with perinatal complications
Authors: IKENO KAZUHIDE
Published: January 2011

We have investigated cord blood granulocyte‐colony stimulating factor (G‐CSF) levels in neonates with or without neonatal complications to examine some changes in the G‐CSF levels in the neonatal period. The G‐CSF levels were measured in 613 neonates by enzyme immunoassay. The results showed that G‐CSF levels were distributed in a broad range from the level under the cutting point (31 pg/mL) to over the measurable range (2000 pg/mL). Normal neonates without perinatal complications were 322. In normal neonates, the G‐CSF level correlated with the gestational age (r = 0.255, P < 0.01) and cord blood leukocyte count (r = 0.210, P < 0.01). The G‐CSF values were under 100 pg/mL in 95% of normal neonates with a median of 35.0 pg/mL. We divided the neonates into two groups: a lower (< 100 pg/mL) and a higher (≥ 100 pg/mL), based on the G‐CSF level. The percentage of neonates with higher G‐CSF levels (≥ 100 pg/mL) was greater in neonates with perinatal complications than in normal neonates (< 100 pg/mL; P < 0.01). Compared with normal neonates, the percentages of the higher group were greater in neonates with infections (P < 0.01), fetal distress (P < 0.01), premature rupture of membranes (P < 0.05), neonatal asphyxia (P < 0.01) and meconium staining of amniotic fluid (P < 0.01). Neonates with higher G‐CSF levels had larger numbers of peripheral leukocytes (P < 0.05) than did those with the lower G‐CSF levels. Counts of leukocytes were parallel with those of neutrophils. In conclusion, cord G‐CSF levels in neonates can be increased in response to, not only infections, but also to such stress states as fetal distress, premature rupture of membranes, neonatal asphyxia and meconium staining of the amniotic fluid, which may result in increased numbers of neutrophils.
A girl having congenital chloride diarrhea treated with spironolactone for seven years
Authors: ASANO TAKESHI , MARUYAMA KAZUO , JINBO OSAMU , TSUMURA HARUO , FURUYA FUJIO , MORITA TOSHISADA , HIRAYAMA TSUNEO
Published: January 2011

We report on a girl having congenital chloride diarrhea (CCD) who has been followed for 7 years and 6 months sequentially. Dilated intestinal loops, marked enlargement of the abdominal circumference of the fetus and hydramnios were noted by ultrasound examination at 31 weeks of gestation. After delivery by cesarean section for hydramnios, she excreted profuse watery yellow green stools with marked abdominal distension. At 4 months of age, hypochloremia, hyponatremia and a high concentration of chloride in the stool were identified. She was diagnosed as having CCD. Because it was difficult to administer a large volume of potassium chloride (KCl), and sodium chloride (NaCl), we decided to administer spironolactone. After administration of spironolactone, we could generate correct serum electrolytes using less amounts of KCl. At 7 years and 6 months of age, her body size was within normal limits and her intellectual, mental and physical development had been normal. In spite of normal serum electrolytes, blood pH and the presence of chloriduria, secondary hyperaldosteronism was noted. We consider that spironolactone may be useful to decrease the amount of KCl administration in the neonatal period, but frequent measurements of renin, angiotensin and aldosterone would be necessary for adequate control in CCD cases.
A Ro/SS‐A auto‐antibody positive mother's infant revealed congenital complete atrioventricular block, followed by insulin dependent diabetes mellitus and multiple organ failure
Authors: FUKAZAWA RYUJI , SEKI TAKASHI , KAMISAGO MITSUHIRO , WATANABE MASANORI , OGAWA SHUNICHI , YUGE KUNIO , HIRAYAMA TSUNEO
Published: January 2011

We experienced a congenital complete atrioventricular block infant who was born from a Ro/SS‐A antibody positive mother. Ro/SS‐A antibody was also found in this baby which was presumed to be mediated by the maternal placenta. Temporary cardiac pacing was required at birth and pacemaker implantation was performed at 9 months. At 11 months of age, the baby fell into shock and experienced multiple organ failure because of diabetes mellitus‐induced coma. The association between congenital complete heart block and the Ro/SS‐A antibody is well known. However, the accompaniment of insulin‐dependent diabetes mellitus has not been reported previously. As the Ro/SS‐A antigen appears in the cytoplasm of many tissues, the possibility of an association between Ro/SS‐A antibody and diabetes mellitus is difficult to deny. We report this rare case to draw attention to the possibility that babies who are born from an Ro/SS‐A antibody positive mother may develop diabetes mellitus as well as congenital complete heart block.
Possible hereditary Y‐chromosome instability
Authors: ZENIYA ETSUKO , IINUMA KAZUSO , KATO MIZUKI
Published: January 2011

A 9 year old boy, born to a phenotypically normal, non‐consanguineous couple was referred for clinical examination due to radio‐ulnar synostosis. We made cytogenetic analyses to investigate the genotype‐phenotype correlation. Chromosomal studies on the boy and his father revealed a very small Y chromosome in both cases, probably due to loss of the heterochromatic long arm segment. Repeated cytogenetic analysis of the boy was made, using QFQ, CBG, DA/DAPI and fluorescence in situ hybridization (FISH) methods with DYZ1 and DYZ3 probes. The results showed a likely mosaicism of cell lines with either a small Y or a normal Y found in the boy. The small Y appeared to be composed of double centromeric regions, without the heterochromatic segment of the long arm of the Y chromosome. The father refused re‐analysis. These findings indicate an inherent instability of the Y chromosome resulting in a familial small Y. The radio‐ulnar synostosis may be associated with excessive Y short arms.
Human parvovirus B19‐induced aplastic crisis in iron deficiency anemia
Authors: KUDOH TOORU , YOTO YUKO , SUZUKI NOBUHIRO , ODA TAKANORI , KATOH SHIZUE , CHIBA SHUNZO , MATSUNAGA YASUKO
Published: January 2011

Human parvovirus B19 (HPVB19) infects and replicates in erythroid progenitor cells. Its specific cytotoxic effect on these cells results in aplastic crises in patients with congenital hemolytic anemias. Aplastic crisis due to HPVB19 infection in a healthy girl revealed occult iron deficiency anemia. The condition is characterized by a high serum iron level in the aplastic phase and rapid recovery after administration of iron. Temporary HPVB19‐induced red blood cell aplasia could occur in patients with other anemias, particularly those with non‐inherited form of hemolysis.
Effects of human neonatal serum on DNA synthesis in suckling and adult rat hepatocytes in primary culture
Authors: KOHNO YUMI , FUKUNAGA MAKI , SHIRAKI KAZUO , MURA TETSUO
Published: January 2011

The effect of human neonatal serum on DNA synthesis in suckling and adult rat hepatocytes in primary culture was investigated to characterize growth regulating factors of the liver in neonates and to confirm whether the stimulatory factor is human hepatocyte growth factor (hHGF). Neonatal serum stimulated DNA synthesis of both adult and suckling rat hepatocytes. The stimulatory effect was dose‐dependent up to 20% in volume. The molecular weight of the stimulatory substance in neonatal serum was between 12 500 and 25 000, as estimated by gel filtration. Its activity was stable after heating at 56°C for 20 min, but was lost after heating at 90°C for 30 s, and easily passed through S‐ or heparin‐Sepharose columns. The concentration of hHGF quantified by ELISA was too low to stimulate DNA synthesis in vitro. Biological and biochemical properties of the growth stimulatory activity in neonatal serum differed from that of hHGF. The presence of other growth factors in human neonatal serum for suckling and adult hepatocytes was suggested.
Tuberculous meningitis in children
Authors: ALTUNBAŞAK ŞAKIR , ALHAN EMRE , BAYTOK VILDAN , AKSARAY NECMI , YÜKSEL BILGIN , ÖNENLI NESLIHAN
Published: January 2011

Between May 1988 and November 1992 the data from 52 patients with tuberculous meningitis (TBM) were noted down for their symptoms and signs, BCG vaccines, PPD tests; clinical, laboratory, radiologic and microbiologic findings. These data were discussed by means of literature knowledge. Cranial computed tomography (CT) demonstrated hydrocephalus (HC) in 98% of the patients. There was a statistically significant difference among the clinical stages on admission in respect to prognosis (P < 0.05). In addition, there was also a significant relationship between prognosis and HC (P < 0.05). However, we did not find any significant relationship between parenchymal involvement, basilar meningitis and prognosis (P > 0.05).
A new protocol for the perinatal management of patients with congenital diaphragmatic hernia with severe hypoplastic lungs and its clinical application
Authors: SENZAKI HIDEAKI , TAMURA MASANORI , SAKAKIHARA YOICHI , HISHI TOSHIO
Published: January 2011

It is prerequisite for the pre‐operative management of patients with congenital diaphragmatic hernia (CDH) to avoid the factors that increase pulmonary vascular resistance, because such patients easily fall into the state of persistent fetal circulation (PFC). In this paper, a new protocol is proposed for the perinatal management of CDH patients to prevent the PFC which is facilitated by the enlargement of hernia and the deviation of mediastinum caused by spontaneous breathing just after birth. We also describe the usefulness of this protocol based on our experience in which we successfully treated a patient with CDH with severe hypoplastic lungs that were diagnosed antenatally by ultrasonographic examination. In treating the CDH patient with severe hypoplastic lungs, it is effective for the respiratory control and the prevention of PFC to administer morphine and pancuronium to the neonate through the umbilical vein before the resection of the umbilical cord. In the case of Cesarean section, in addition to the direct administration of morphine to the patient, administration of morphine to the patient's mother just before the delivery is more effective to prevent PFC, which can be easily induced by the initial resuscitation at birth.
A comparison of left ventricular volumes and regurgitant fraction by Doppler echocardiography and angiography in children
Authors: TAVLI VEDIDE , AL‐KHATIB YASSER , ISABEL‐JONES JOSEPHINE , TAVLI TALAT , WONG AH LIN , WILLIAMS ROBERTA
Published: January 2011

Regurgitant fraction calculations by echocardiography were compared to angiocardiography in 11 patients with mitral regurgitation. Although there was a very good correlation when compared with angiocardiography (r = 0.99, SEE: 6.26, P< 0.001), two‐dimensional echocardiography underestimated left ventricular end‐diastolic volumes (P < 0.05). The mean regurgitant fraction was 36.2 ± 13.5% by echocardiography and 43.6 ± 15.7% (P< 0.05) by angiography. Most of the variability in measuring the regurgitant fraction is attributed to the left ventricular volume measurements. In conclusion, regurgitant fraction calculations of mitral regurgitation by echocardiography compares favorably to angiography. Since the severity of mitral regurgitation is an important determinant of prognosis, quantification of the regurgitant fraction may be useful in the long term follow‐up of those patients.
Interchange trisomy 9 due to maternal t(6; 9) translocation
Authors: NINOMIYA SHINSUKE , NARAHARA KOUJI , YOKOYAMA YUJI , TSUJI KAZUSHIRO , ITO SATOKO , AKAHORI WAITCHIRO , AKAHORI TAIITCHIRO , SEINO YOSHIKI
Published: January 2011

The occurrence of interchange trisomy due to a 3:1 malsegregation has been documented in only a few cases with trisomy 21. We describe the first case of interchange trisomy 9 due to a maternal t(6; 9) translocation. The patient, a boy neonate who died immediately after birth, had intra‐uterine growth retardation, specific craniofacial features including microcephaly with a high forehead, low‐set ears, upslanting short palpebral fissures, microphthalmia, bulbous nose and micrognathia, cryptorchidism, cystic kidney and various skeletal anomalies. His phenotype was consistent with that of the trisomy 9 syndrome. Cytogenetic analysis showed his karyotype of 47,XY,‐6, + der(6), + der(9)t(6; 9)(q27;q21.1)mat. The present report indicates that a very rare interchange mode of a 3:1 segregation can give rise to a live birth with full trisomy 9 in female carriers with reciprocal translocations involving the proximal long arm of chromosome 9.