bluuebunny/crossref_metadata_2025 huggingface dataset:
Early detection of cutaneous microcirculatory change during hemorrhage using a laser Doppler flowmetry
Authors: TAKAYANAGI TOSHIMITSU , FUKUDA MASAFUMI , TSUJI YOSHIROU , NAKAZAWA MAKOTO
Published: January 2011

The purpose of the present study was to evaluate the usefulness of cutaneous microcirculatory monitoring during hemorrhage. We observed changes in cutaneous blood volume, velocity and flow of five adult rabbits during hemorrhage by using a laser Doppler flowmetry. Mean arterial blood pressure, heart rate and blood gas values were measured.

Cutaneous blood volume, velocity and flow decreased significantly after drawing 10 mL/kg of blood, while heart rate, mean arterial blood pressure and blood gas did not change. The decrease of cutaneous blood velocity preceded that of blood volume and was associated more deeply with the reduction of blood flow.

In conclusion, cutaneous microcirculatory monitoring using laser Doppler flowmetry is a sensitive technique for detecting early changes of circulatory failure caused by hemorrhage.
Evaluation of rate‐pressure product in obese children
Authors: HO TING FEI , YIP WILLIAM CHIN LING
Published: January 2011

The purpose of this study was to evaluate the relationship between rate‐pressure product (RPP) and degree of obesity in 511 obese 7 and 12 year old Chinese children. Obesity was assessed by anthropometry and skinfold thickness. The children were defined obese by relative weight (RW) > 120%. Bodyweight (W) and height (H) were used to derive body mass index (W/H2). Brachial systolic and diastolic pressures in these children were measured by cuff sphygmomanometry. RPP was calculated by the formula: heart rate x mean arterial pressure.

More obese children (RW ≥ 150%) had greater mean RPP (7 year old: 6389 vs 5976 beats/min x mmHg, P < 0.05; 12 year old: 7024 vs 6686 beats/min x mmHg) than those with RW < 150%. Children in the upper 25 percentiles of RPP had significantly larger BMI (7 and 12 year olds) and RW (12 year olds), thicker biceps, triceps and subscapular skinfolds (12 year olds) (P < 0.01 ‐ < 0.05).

The results indicate that RPP is, to some extent, related to the degree of obesity in obese children. The differences in RPP may imply varying degrees of hemodynamic stress to the heart. Whether such differences may contribute to the long‐term development of cardiovascular morbidity in more obese individuals is uncertain.
Intra‐abdominal fat in obese children
Authors: IWATA FUJIHIKO , TANIGUCHI KAZUO , YAMAZAKI HIROKI , HARA MITSUHIKO , OKADA TOMOO , HARADA KENSUKE
Published: January 2011

We investigated the distribution of abdominal fat accumulation in obese children to know whether a clustering of coronary risk factors was demonstrated in visceral fat obesity as reported in adults. The relative indicator of intra‐abdominal fat accumulation was obtained from computed tomography scans at the umbilicus level in 36 obese subjects (24 males, 12 females). There was no visceral fat obesity in this study by reported criteria. All metabolic variables except triglyceride did not correlate significantly with intra‐abdominal fat accumulation. We conclude that visceral fat obesity is a rare status and has no close relationship to coronary risk factors in childhood.
Pulse methylprednisolone therapy in children with membranoproliferative glomerulonephritis
Authors: EMRE SEVINÇ , ŞIRIN AYDAN , ALPAY HARIKA , TANMAN FAIK , UYSAL VELI , NAYIR AHMET , BILGE ILMAY
Published: January 2011

The effects of pulse methylprednisolone (PM) therapy were studied in 15 patients (aged 3–14 years) with biopsy proven membranoproliferative glomerulonephritis (MPGN). Patients were treated with intravenous PM 30 mg/kg (max 1 g) given over 30 min every other day for a mean of 9.8 days (3–15 days). Oral prednisolone therapy was continued at a dose of 1 mg/kg/24 h for 1 month and subsequently tapered off the following month. Eight patients had hematuria and six had medically controlled hypertension. Serum C3 levels were low in 11 patients and all of the patients had proteinuria. Following PM therapy proteinuria was significantly reduced from 2602.9 ± 1852.5 mg/24 h to 1871.2 ± 2090.8 mg/24 h (P < 0.05) and at final evaluation, proteinuria was 774.33 ± 1225.67 mg/24 h which was significantly lower than pre‐ and post‐PM therapy values (P < 0.05). Serum creatinine levels were high in five patients before PM therapy and remained high in one of the patients who progressed to end‐stage renal failure. After PM therapy, high serum creatinine levels normalized in three patients and was reduced, but still above normal, in one. One patient, with initially normal serum creatinine, had elevated levels afterwards. Nine of the patients were considered responsive and six non‐responsive according to our tentatively defined criteria. Mean follow‐up period was 27.4 ± 24.1 months (6–84 months). Three patients were lost for follow‐up, and 12 were re‐evaluated. At final evaluation, all of the patients except one with end‐stage renal failure had normal creatinine levels. There was no correlation between the clinical and laboratory features at onset and the outcome of the disease in the responder or non‐responder patients. Results of our study show that PM therapy reduced proteinuria and may affect renal function positively in patients with MPGN. However, a prospective controlled study with repeat biopsies is required to draw a conclusion.
Congenital hypothyroidism with delayed rise in serum TSH missed on newborn screening
Authors: ASAMI TADASHI , OTABE NAOKO , WAKABAYASHI MARIKO , KIKUCHI TOURU , UCHIYAMA MAKOTO
Published: January 2011

We report on a female patient with congenital hypothyroidism (CH) missed on a newborn screening test. She is now 10 years old with retarded development. The patient was born premature at 34 weeks of gestation with birth‐weight of 1515 g, and was judged to be normal in the screening programme of Niigata Prefecture. However, she gradually suffered from poor weight gain and retarded development with stridor at breathing. Serum thyroid stimulating hormone (TSH) levels were rechecked and showed high values with normal T3 and T4 levels. She was referred to our hospital at the age of 13 months. She was diagnosed as having CH (ectopic thyroid) with a delayed rise in blood TSH concentration, probably due to the prematurity of the hypothalamic‐pituitary‐thyroid axis. l‐thyroxine therapy brought a decline in TSH levels with partial improvement of her symptoms. Regardless of the result of newborn screening, infants with elevated serum TSH levels should be carefully examined for possible CH, even when T3, T4 and free T4 values are in the normal range.
Acute renal failure after exercise in a child with renal hypouricemia
Authors: FUJIEDA MIKIYA , YOKOYAMA WAKAKO , OISHI NAOBUMI , OKADA TAISUKE , KURASHIGE TAKANOBU , HAMADA GIBUN
Published: January 2011

We describe a 15 year old boy with renal hypouricemia who developed acute renal failure after a school athletics meeting, accompanied by appendicitis. During acute renal failure, the highest level of uric acid was 5.0 mg/dL, creatinine 7.9 mg/dL and urea nitrogen 58.6 mg/dL. After recovery, the serum uric acid fell to 0.9 mg/dL and the fractional excretion of uric acid (FEuA) exceeded the normal range. The probenecid and pyrazinamide tests showed that the patient had a total defect of uric acid reabsorption. This case suggested that strenuous exercise could be responsible for acute renal failure in patients with renal hypouricemia.
Herpes zoster in a normal child after varicella vaccination
Authors: MATSUBARA KOUSAKU , NIGAMI HIROYUKI , HARIGAYA HIDEKAZU , BABA KUNIZOU
Published: January 2011

A healthy 5 year old girl developed herpes zoster in the dermatome supplied by the ophthalmic branch of the fifth cranial nerve 40 months after varicella vaccination. She was admitted to our hospital because of high fever and painful vesicular lesions over the left side of her forehead. She was treated successfully with systemic and topical acyclovir without developing herpetic keratoconjunctivitis. Our acute and convalescent phase evaluations showed that non‐specific cellular and humoral immunity was normal. This is the fourth case of herpes zoster developing in an immunocompetent child following vaccination. Unlike the previously reported cases, our patient required hospitalization mainly to prevent ocular involvement. The issue concerning whether the universal introduction of varicella vaccination of normal children will reduce the incidence of the subsequent occurrence of herpes zoster must await further studies involving longer follow‐up periods.
Changes in apolipoproteins during the acute phase of Kawasaki disease
Authors: KIM HIDEAKI , YAMAGUCHI HIDEO , INAMO KOJI , OKADA TOMOO , HARADA KENSUKE
Published: January 2011

Abstact

To determine disturbances of lipid metabolism in patients with Kawasaki disease, we investigated changes in the serum levels of apolipoproteins and serum lipids. Results were as follows. Total serum cholesterol (TC) and high‐density lipoprotein cholesterol (HDL‐C) decreased during the early stage of Kawasaki disease. The apo A‐I and A‐II levels were low until the 2nd week of illness. Apo B decreased during the 1st week of illness, but rose slightly during the 2nd and 3rd weeks. The apo B/apo A‐I ratio and the apo B/TC ratio were high in the early stages of illness. The group of patients with coronary artery lesions showed low levels of TC and HDL‐C, and low levels of apo A‐I, A‐II and B during the early stages, compared with the group without coronary artery lesions. The apo B/TC ratio was significantly higher in the patients with coronary artery lesions during the 2nd week of illness.

Our findings suggest an association between changes in serum lipids and apolipoproteins and coronary artery involvement in Kawasaki disease. These abnormalities may indicate the presence of early coronary arteriosclerosis.
Reduced immune response to hepatitis B vaccine in children with insulin dependent diabetes
Authors: FIÇICIOǦLU CAN , MIKLA SHARARE , MIDILLI KENAN , AYDIN AHMET , ÇAM HALIT , ERǦIN SEVGI
Published: January 2011

Twenty‐four patients with moderately controlled insulin dependent diabetes with a duration of diabetes ranging from 2 to 10 years as well as 17 control subjects were vaccinated against hepatitis B virus using Gen Hevac B vaccine. The vaccine was injected 0.5 mL intramuscularly into the deltoid region on three separate occasions at intervals of 1 month. If subjects were still negative for anti‐hepatitis B surface antigen (HBs) or had inadequate antibody after the third injection, a fourth administration of vaccine was given 3 months later. The mean anti‐HBs titer was 243.3 ± 97.2 mi.u./mL in control subjects and 39.8 ± 53.2 in diabetic patients (P < 0.001). In the control group optimal protection was obtained in 100% of subjects, whereas 11 diabetic patients (45.8%) had low anti‐HBs titer (< 10 mi.u./mL). All of 11 diabetic patients showed adequate (> 10 mi.u./mL) anti‐HBs titer after the fourth dose of vaccine. In diabetic patients the most striking feature was the reduced CD4/CD8 ratio which was significantly lower (P < 0.001) than that of the control group. We conclude that diabetic children have an impaired immune response to hepatitis B vaccine. It is suggested that diabetic children should be vaccinated against hepatitis B virus with four injections instead of three.
Cartilage‐hair hypoplasia associated with IgG2 deficiency
Authors: KAWASAKI HIROHIDE , KOHDERA URARA , TANIUCHI SHOICHIRO , KOBAYASHI YOHNOSUKE
Published: January 2011

We report on a 1 year old boy with cartilage‐hair hypoplasia (CHH). He suffered from recurrent upper respiratory infections and short‐limbed dwarfism. As with most patients with CHH, he had impaired cellular immunity as determined by lymphocyte reactivity. In addition, he had a selective IgG2 deficiency. This combination of immunodeficiencies has not previously been reported for patients with CHH. His recurrent upper respiratory infections were likely to be associated with cellular immunodeficiency and IgG2 deficiency.
Overview of the Hanshin‐Awaji earthquake disaster
Authors: NAKAMURA HAJIME
Published: January 2011

A severe earthquake struck the southern part of Hyogo prefecture at 5:46 a.m., 17 January 1995. The Kobe and Hanshin district, one of the greatest industrial and commercial areas in Japan, suffered many great disasters as a consequence of this earthquake. More than 5500 deaths, disconnection of electricity, water and gas, failure of telecommunications, collapse of highways and railroads, destruction of houses, apartment buildings and official buildings and fires in high‐density housing areas brought about the biggest disasters since the 1923 Tokyo quake.

Many Japanese volunteers as well as foreign volunteers helped refugees after the earthquake. The actions of the refugees and their self‐possessed judgement, despite great inconveniences, in helping each other and saving aged and handicapped people, surprised many Japanese and foreigners alike.

The preliminary reports in this issue describe the circumstances of child health and the medical care given after the disaster by the pediatricians who played an active role in the area affected by the earthquake.
Non‐conventional portosystemic shunts in children with extrahepatic portal vein obstruction
Authors: D'CRUZ ASHLEY J. , KAMATH PATRICK S. , RAMACHANDRA C. , JALIHAL ANAND
Published: January 2011

Portal hypertension due to extrahepatic portal vein obstruction is ideally treated by the use of a selective shunt. In a four year period between July 1987 and June 1992, 50 surgical procedures were carried out in 48 children with portal hypertension‐related variceal hemorrhage. The portal, splenic, mesenteric, or coronary vein was not available for anastomosis in four children who, therefore, underwent non‐conventional shunts which are described here. There was no postoperative mortality. One patient had a repeat gastrointestinal hemorrhage, probably due to stenosis of the shunt and failure to ligate the coronary vein. No rebleeding occurred on follow‐up of at least 18 months. We conclude that in selected children requiring surgery for portal hypertension due to extrahepatic portal venous obstruction, a non‐conventional shunt may be used with beneficial results.
Standard and intermediate risk acute lymphoblastic leukemia in Poland: A report of the Polish Children's Leukemia/Lymphoma Study Group
Authors: RADWANSKA URSZULA , MICHALEWSKA DANUTA , KOLECKI PAWEL , ARMATA JERZY , BALWIERZ WALENTYNA , BOGUSLAWSKA‐JAWORSKA JANINA , CHYBICKA ALICJA , CYKLIS ROMANA , KOWALCZYK JERZY , OCHOCKA MARIA , PAWELEC KATARZYNA , ROKICKA‐MILEWSKA ROMA , SONTA‐JAKIMCZYK DANUTA , SLADKOWSKA GRAZYNA , ZELENAY ELZBIETA
Published: January 2011

A total of 527 children with acute lymphoblastic leukaemia (ALL) from the most frequent risk groups: standard risk group (SRG) and intermediate risk group (IRG) were treated between 1987 and 1991 according to an intensified treatment program (based on the BFM protocol) including the use of an intermediate dose of methotrexate in the IRG. A comparison of the treatment results in this group from 513 children treated between 1981 and 1987 indicates that the chance for a 6 year event‐free survival has increased to 73% (previously 55%).
An infantile case of cytomegalovirus induced idiopathic thrombocytopenic purpura with predominant proliferation of CD10 positive lymphoblast in bone marrow
Authors: MIZUTANI KENICHI , AZUMA EIICHI , KOMADA YOSHIHIRO , ITO MASAHIRO , SAKURAI MINORU , HIRONAKA TAKESHI , HIRAI KANJI
Published: January 2011

An infant with cytomegalovirus infection (CMV) developed idiopathic thrombocytopenic purpura (ITP) at 4 months of age. A bone marrow (BM) aspiration showed a remarkable increase of immature megakaryocytes and prominent proliferation of lymphoblasts. Flow cytometric analysis of the bone marrow cells showed that the predominant cells in the lymphocyte cluster were of B‐lineage (CD19) with CD10 (common acute lymphoblastic leukemia antigen) positive. Virus study showed a high titer of CMV antibody. Cytomegalovirus DNA was detected by the polymerase chain reaction (PCR) method in urine, peripheral cells and marrow cells. Low‐grade fever, diarrhea and petechiae were accompanied by mild liver dysfunction. Complete remission was made with intravenous high‐dose immunoglobulin (IVIg) without progression to overt acute leukemia. The percentage of CD10+/CD19+ lymphocytes in bone marrow also diminished. We postulated that the proliferation of immature lymphocytes and megakaryocytes in bone marrow was caused by maturation arrest that might result from CMV infection.
A case with dicentric translocation between chromosome 9 and 18: Confirmation by fluorescent in situ hybridization on metaphase spread
Authors: ISOYAMA KEIICHI , IMAI MITSURU , ISHIKAWA AKIRA , FUJITA KAZUHIRO , SUTO YUMIKO , SHINOHARA TAMIKO
Published: January 2011

A female child with dicentric translocation between chromosome 9 and chromosome 18 presented non‐specific minor anomalies with laryngomalacia. Chromosomal analyses were performed by the G‐banding method and a fluorescence in situ hybridization (FISH) technique with a specific probe for the centromeric region of chromosome 18 and the painting probe for the chromosomes 9 and 18. Her full karyotype was confirmed as 45, XX, tdic (9;18)(p24;p11). This is the first case of dicentric translocation between chromosomes 9 and 18. The FISH technique is an important tool in chromosome diagnostics.
A patient with WT syndrome and Castleman disease
Authors: VERGIN CANAN , ÇETINGÜL NAZAN , KAVAKLI KAAN , ÖZTOP SENAY , ÖZKINAY FERDA , ÇOKER MAHMUT , NIŞLI GÜNGÖR , SOYDAN SALIHA , BALIK EROL , AKAR ORHAN
Published: January 2011

WT syndrome, an autosomal dominant condition, combines hematological abnormalities with mild lib defects. Anemia, pancytopenia, leukemia and lymphoma can occur at varying ages from childhood to middle age. Limb defects include ulnar and radial defects, bifid or hypoplastic thumbs and cutaneous syndactyly. Castleman disease is characterized by tumorous masses of lymphoid tissue showing plasma cell or hyaline vascular type changes in histological specimens.

A 13 year old boy, diagnosed as WT syndrome with ulnar and radial deviation and 5th finger clinodactyly also had neutropenia, cervical and mediastinal lymphadenopathy. Histology of the cervical lymph node showed angiofollicular hyperplasia of the hyaline‐vascular type (Castleman disease). This interesting patient is reported because Castleman disease, together with WT syndrome has not been previously described.
Hypertrophic cardiomyopathy in Noonan syndrome
Authors: NISHIKAWA TOSHIO , ISHIYAMA SHIGERU , SHIMOJO TAKASHI , TAKEDA KAZUYO , KASAJIMA TAKKSHI , MOMMA KAZUO
Published: January 2011

Noonan syndrome, a well‐known multiple congenital anomalies syndrome, is frequently accompanied by cardiovascular diseases including hypertrophic cardiomyopathy (HCM). The incidence of HCM in Noonan syndrome is approximately 20–30% and one‐third of cases reveal ventricular outflow obstruction. HCM in Noonan syndrome is occasionally associated with a congenital heart defect, whereas classic HCM seldom accompanies cardiac malformations. Asymmetric septal hypertrophy and symmetric septal hypertrophy (concentric hypertrophy) can be observed both in HCM with Noonan syndrome and in classic HCM. but apical hypertrophy has not been reported in Noonan syndrome yet, although it appears in classic HCM. Congestive heart failure is the major cause of death in patients with HCM in Noonan syndrome, but cases of sudden death have also been reported. The histopathologic findings of ventricular myocardial tissue in HCM with Noonan syndrome are similar to those in classic HCM.
Growth hormone treatment in Noonan syndrome
Authors: TANAKA TOSHIAKI
Published: January 2011

Growth responses to growth hormone (GH) treatment in Noonan syndrome are compared with those in short children with the other growth disorders. The responses in Noonan syndrome are much less than those in children with GH deficiency, a little less than those in children with non‐endocrine short stature and almost the same as those in children with Turner syndrome. As it is speculated that GH induces puberty earlier that expected in Noonan Syndrome, the efficiency of GH treatment for final height in Noonan syndrome is not promising.
Immunogenicity of the whole antigen and glycoprotein I of varicella‐zoster virus (VZV) and the VZV skin test antigen
Authors: KAWANO SHOJI , TERADA KIHEI , HIRAGA YUMIKO , MORITA TETSURO
Published: January 2011

Specific cellular immunity against the whole antigen and glycoprotein I (gp I) of varicella‐zoster virus (VZV) and the skin test antigen were determined for 13 healthy immune adults to compare the immunogenicity of these antigens using a lymphoproliferative assay. The stimulation index values (mean ± s.d.) were 16.4 ± 11.2 against the whole VZV antigen, 4.2 ± 2.9 against the VZV skin test antigen and 1.1 ±0.5 against gp I. The immunogenicity of the skin test antigen, which mainly contains gp III and gp IV, appears to be more favorable than that of gp I alone, but to be weaker than that of the whole VZV antigen.
Outbreak of acute glomerulonephritis in children: Observed association with the T1 subtype of group A streptococcal infection in Northern Kyushu, Japan
Authors: MASUYAMA TATSUO , ISHII EIICHI , MURAOKA KENJI , HONJO SATOSHI , YAMAGUCHI HIDETO , HARA TOSHIRO , SHIMAZAKI KOHJI , KOGA TATSUHIKO , MORIYA KAZUO , IDE MIKIKO , MIYAZAKI SUMIO
Published: January 2011

Group A streptococcal infection is associated with the occurrence of acute glomerulonephritis (AGN) and rheumatic fever (RF). A surveillance study in the Saga area, in northern Kyushu, Japan, showed a small variation in the reported number of group A streptococcal infections in the period 1988–94. However, of the AGN cases reported in this period, more than half were observed in 1992. In order to examine whether some change had occurred in the serotype distribution of Streptococcus pyogenes during the period, patients in the Saga area diagnosed as having group A streptococcal infection and patients with AGN or RF were analyzed. Serological T‐typing of S. pyogenes was carried out for patients with group A streptococcal infections, and the association between the occurrence of AGN or RF and the distribution of each different T subtype was analyzed. M‐typing of S. pyogenes was also carried out and the correlation between T and M types was examined. From 1988 to 1994, the annual number of patients with group A streptococcal infections in the Saga area showed a small variation, range 65–100 patients/year. Of the 42 patients with AGN and three with RF observed in this period, 27 with AGN (64%) and one with RF (33.3%) were detected in 1992. Only the T1 subtype increased in 1992; the other T subtypes showed little variation in incidence. The number of patients with the T1 subtype was significantly correlated with the occurrence of AGN by regression analysis (P < 0.01). Of the 170 subjects tested for both T and M subtypes, 44 of the 45 T1‐typed subjects had the M1 protein. Our epidemiological study suggested that the T1 subtype of streptococcal infection was associated with an outbreak of AGN in 1992 in the Saga area.
Evaluation of the peripheral circulatory status of the neonate during homeothermal adjustment by plethysmo‐time‐interval
Authors: OISHI MASAYA , NISHIDA HIROSHI , HOSHI JUN
Published: January 2011

The plethysmo‐time‐interval (PTI) is the time interval between the beginning of the QRS complex on an ECG and the upstroke of the pulse wave on a plethysmogram, as measured by pulse oximetry. In the present study, we investigated homeothermal acclimatization to the extra‐uterine environment in human neonates using the Coretemp thermometer and a pulse oximeter. Temperature was measured at three sites: the central deep temperature (CDT) on the upper sternum, the peripheral deep temperature (PDT) on the flat part of the left sole of the foot and the surface temperature (ST) at the side of the abdomen. After delivery, CDT and ST were higher than PDT. The difference between CDT and PDT was large at first, but gradually decreased. PDT, initially in the range of 32.4 ± 0.28°C, reached a stable value (34.4 ± 0.41 °C) at 2.5 h after delivery. PTI was prolonged in parallel with PDT. The difference between CDT and PDT probably reflected the contraction of skin vessels, particularly the arterioles, which occurs as a body defense mechanism against heat loss. As PTI was prolonged in parallel with PDT, we demonstrated objectively that this catch‐up phenomenon of PDT after delivery was affected by the increase in skin blood flow as a result of dilatation of peripheral arterioles. It was concluded that PTI can be used to evaluate the peripheral circulatory status of the neonate, even during homeothermal adjustment after birth, by applying a new principle of pulse oximetry that is widely used in neonatal intensive care units.
Change of immunological parameters in the clinical course of a myasthenia gravis patient with chronic graft‐versus‐host disease
Authors: HAYASHI MASATOSHI , MATSUDA OSAMU , ISHIDA YASUSHI , KIDA KAICHI
Published: January 2011

The change in immunological parameters was studied during the clinical course of a myasthenia gravis (MG) patient with chronic graft‐versus‐host disease (GVHD), which developed after bone marrow transplantation from an HLA‐identical donor. Anti‐acetylcholine receptor antibody gradually decreased in the patient treated with immunosuppressive agents, but was not detected in the donor. Lymphocyte numbers were low just before the onset of MG, increased abruptly within several days and then gradually decreased with treatment. The percentage of CD3+ and CD19+ lymphocytes was higher at onset than before, the percentage of CD4+ cells was higher at onset and gradually decreased with treatment, while CD8+ cells showed the lowest level just before onset and gradually increased during the clinical course. CD4+/CD45RA− cells also showed the highest levels at onset and a gradual decrease with treatment. Cellular, as well as humoral immune responses, might be associated with the pathogenesis of MG with chronic GVHD.
Successful percutaneous closure of a coronary artery fistula with a detachable balloon
Authors: OZASA HIROSHI , KAMIYA ICHIRO , HANEDA NORIYUKI
Published: January 2011

Precutaneous transcatheter closure technique of a coronary artery fistula with a detachable balloon was performed for a 14 year old male student. Complete closure of the fistula without any complications was confirmed by angiography after the procedure. When the patient underwent a second angiography 6 months after the closure, it was confirmed that the position of the balloon had not changed, that the interruption of the flow of the fistula had been maintained and that the diameter of the left coronary artery and the fistula were reduced. Percutaneous closure technique using a detachable balloon may become the primary treatment for a coronary artery fistula in place of surgical ligation.
Serum and cerebrospinal fluid zinc levels in children with febrile convulsions
Authors: GÜNDÜZ ZÜBEYDE , YAVUZ İSMET , KOPARAL MEHMET , KUMANDAŞ SEFER , SARAYMEN RECEP
Published: January 2011

The mechanisms underlying febrile convulsions (FC), which have multiple etiological factors, are not yet clear. The aim of the present study was to determine whether there were any changes in serum and cerebrospinal fluid (CSF) zinc (Zn) levels in children with febrile convulsion during seizures. A total of 102 children were included in the study, with four groups formed as follows: group A, 40 children with FC (aged 9 months to 5 years); group B, 20 children having fever without convulsion (aged 6 months to 5 years); group C, 20 children with afebrile convulsion (aged 6 months to 6 years) and group D, 22 healthy children (aged 5 months to 6 years). Serum and CSF zinc levels for groups A, B and C and serum Zn levels only for group D were measured. The serum Zn levels of 17 children in group A were again measured during healthy periods. Serum Zn levels of groups A, B, C and D had a mean of 0.70 ± 0.10 mg/dL, 1.07 ± 0.08 mg/dL, 1.26 ± 0.32 mg/dL and 1.17 ± 0.21 mg/dL, respectively, and the values of group A were lower than those of the other three groups (P < 0.001). In group B, serum Zn levels were also lower than those of groups C and D (P < 0.05). The CSF Zn levels of groups A, B and C were found to have a mean of 0.07 ± 0.02 mg/L, 0.12 ± 0.02 mg/L and 0.14 ± 0.04 mg/L, respectively. In group A, the CSF Zn levels were lower than those of groups B and C (P < 0.001), and in group B they were lower than those of group C (P < 0.05). For the 17 patients in group A, serum Zn levels during healthy periods (0.87 ±0.10 mg/dL) were found to be higher than the values shortly after seizures, but lower than those of groups B, C and D (P < 0.001). We could not observe any relationship between zinc levels of the serum and CSF and the degree and duration of the fever. These findings suggest that serum and CSF Zn levels decreased during infectious diseases, and that this decrease was more significant in patients with FC.
Long‐term follow up of abdominal aortic aneurysm complicating Kawasaki disease: Comparison of the effectiveness of different imaging methods
Authors: FUYAMA YUUICHI , HAMADA ROUSEI , UEHARA RITEI , YANO ICHIRO , FUJIWARA MASAKO , MATOBA MASAKO , KAWAMURA KENICHI , NONAKA ZENJI , MAEKAWA KIHEI
Published: January 2011

A 2 month old boy with Kawasaki disease developed the rare complication of abdominal aortic aneurysm (AAA). He was followed up over 7 years by ultrasonography (2D‐ECHO) with and without Doppler flow evaluation, angiography and computed tomography. Calcification was noted 33 months after the onset of the disease but the aneurysm did not decrease in size.

2D‐ECHO was adequate for evaluating the size of an aneurysm, but did not show thrombus formation or calcification. Doppler flow studies did not show abnormally high blood flow velocities either at the inflow or outlet of aneurysms, which are indicative of stenosis. On the other hand an abdominal aortogram provided information regarding the luminal shape and abnormalities in flow pattern suggestive of thrombus formation. Fluoroscopy cannot demonstrate calcification in the early stages. Computed tomography (CT) was the imaging method of choice for the evaluation of obstructive or calcific changes; an organizing thrombus was clearly demonstrated and early detection of calcification was possible.
Dyskeratosis congenita: Unusual onset with isolated neutropenia at an early age
Authors: YEL LEMAN , TEZCAN ILHAN , SANAL ÖZDEN , ERSOY FÜGEN , BERKEL A IZZET
Published: January 2011

A 3.5 year old male patient with dyskeratosis congenita (DC) presented at the age of 13 months with isolated neutropenia preceding characteristic skin findings. The average absolute neutrophil count of 500/mm3 persisted without the presence of anemia or thrombocytopenia during the follow up. Neutropenia responded to granulocyte‐colony stimulating factor (G‐CSF) at a dose of 10 μg/kg per day. Immunologic findings were normal as was the chromosomal stability and sister chromatid exchange.
Granulocyte transfusion: Stimulation of low dose granulocyte colony‐stimulating factor in donors for leukapheresis
Authors: NOMURA AKIHIKO , OHGA SHOUICHI , MATSUZAKI AKINOBU , KAI TAKESHI , INABA SHOICHI , SUDA MASAHIRO , UEDA KOHJI
Published: January 2011

In order to assess the clinical utility of granulocyte transfusions (GT), the stimulating effects of donor granulopoiesis for GT therapy were examined using either low dose recombinant human granulocyte colony‐stimulating factor (rhG‐CSF) or dexamethasone (DEX). The increment of leukocytes, polymorphonuclear cells (PMN) and monocytes in the subjects stimulated with rhG‐CSF (0.7 μg/kg SC) surpassed each increment in those with DEX alone (1 mg PO). The lymphocyte counts after DEX stimulation decreased in contrast to those after G‐CSF stimulation. This dose of G‐CSF did not enhance the priming effects on the superoxide release from PMN. The serum levels of lysozyme, but not of lactate dehydrogenase, in G‐CSF stimulated donors were higher than those in DEX‐treated donors. The serum macrophage/monocyte‐colony stimulating factor (M‐CSF) levels in DEX stimulation were lower than in either G‐CSF stimulation or no stimulation. The net yield of the PMN in GT on G‐CSF stimulation was practically larger than that on DEX stimulation. One of the two patients who received GT collected by DEX stimulation died of aspergillosis. Two of the five patients who received PMN mobilized by G‐CSF died of fungal infections or necrotizing fasciitis, although two of the remaining patients overcame severe bacterial infections. These results suggest that low dose G‐CSF effectively and safely mobilizes a sufficient quantity of PMN from GT‐donors without excessive superoxide generation from the transfused cells. This low dose G‐CSF stimulation may be substituted for conventional DEX stimulation for GT.
Flow cytometric evaluation of the DNA profile and cell cycle of zinc supplemented human Chang liver cells
Authors: PARAMANANTHAM RAMANUJAM , BAY BOON‐HUAT , SIT KWOK‐HUNG
Published: January 2011

Zinc, an essential trace element, is important for normal cell growth. Growing children, especially at puberty, require increased zinc (2.8 mg/day for males and 2.65 mg/day for females). The DNA profile and cell cycle of human Chang liver cells grown in 0–900 μmol/L zinc chloride supplemented serum‐free media for 24 h were analyzed using a Coulter flow cytometer. There was no significant difference in the G1, S and G2/M phases between zinc treated cells and control cultures except at 90 and 900 μmol/L zinc chloride. At these two higher dosages, fragmentation of genomic DNA into sub‐2N DNA (sub‐G1 DNA), generally considered a hallmark of programmed cell death (PCD), was noted. Results of the present study seem to suggest that growth regulation by zinc during growth spurts such as at puberty, could also be influenced by other factors besides its direct effect on DNA synthesis. In addition, high dosages of zinc could be cytotoxic.
Cerebral energy metabolism in insulin induced hypoglycemia in newborn piglets: in vivo31P‐nuclear magnetic resonance spectroscopy
Authors: IMAI TADASHI , KONDO MASATOSHI , ISOBE KENICHI , ITOH SUSUMU , ONISHI SHOJU
Published: January 2011

The effect of insulin induced hypoglycemia on cerebral energy metabolism was examined in four newborn piglets. Cerebral energy metabolism was assessed using in vivo 31P‐nuclear magnetic resonance spectroscopy. It was demonstrated that the normal level of phosphocreatine/inorganic phosphate (PCr/Pi), an indicator of phosphorylation potential, was maintained at a blood glucose level of 40 mg/dL or above, whereas when blood glucose was reduced to less than 40 mg/dL, PCr/Pi rapidly decreased in parallel with this. Below the critical blood glucose level of 40 mg/dL, a positive correlation (y = 0.02x + 0.632; r = 0.668; P < 0.001) existed between blood glucose and PCr/Pi. In the present investigation, a reduction of blood glucose level to 20 mg/dL or lower resulted in a PCr/Pi of less than 1, indicating a state of cerebral energy failure. The intracellular pH (pHi) was 7.08 ± 0.05 at the onset and 7.15 ± 0.07 in the hypoglycemic state, indicating no significant difference between the two groups. The present study has clarified that cerebral energy failure occurs when the blood glucose level is about 20 mg/dL or lower. The critical point of blood glucose exists to maintain brain energy metabolism.
A case of prolonged human parvovirus B19 DNA‐emia associated with polyclonal B cell activation
Authors: NOBUTOKI TATSUROU , HORI HIROKI , HIGASHIGAWA MASAMUNE , AZUMA EICHI , SAKURAI MINORU , YOSHIZUMI TAMOTSU , NUNOUE TADASU
Published: January 2011

The current paper reports an 8 year old girl with arthralgia and polyclonal B cell activation induced by human parvovirus B19 infection (HPV B19). The infection was diagnosed by the presence of the virus genome in sera. The patient presented with transient arthritis in the wrist, ankle joint and neck and elevation of immunoglobulin IgM antibodies to HPV B19 and rubella, antibodies to Mycoplasma and antistreptolysin O but without the typical clinical features of erythema infectiosum. The polyclonal B cell activation was paralleled by the presence of the virus genome of HPV B19 in sera. In some children with arthralgia, it is important to examine the genomes of viruses that may cause arthritis as well as the antibody titers to the viruses.
Intractable wheezing in infants with nasopharyngeal reflux
Authors: KUROKI HARUO , ISHIKAWA NOBUYASU , KUROSAKI TOMOMICHI , NIIMI HIROO
Published: January 2011

Two infants with intractable wheezing and moist cough were referred to Chiba Municipal Kaihin Hospital. Their symptoms were persistent even after the usual treatment for respiratory disease. No definite etiological agents were detected. They usually gagged while feeding and barium swallow tests revealed nasopharyngeal reflux and cricopharyngeal incoordination. One of the patients had remarkably high titers of IgE and IgE RAST of cow's milk before she received treatment with thickened formula. She also had peripheral eosinophilia and nasal eosinophilia. These findings were thought to be caused by nasopharyngeal reflux. Four months after therapy commenced, those titers and symptoms were greatly reduced. The clinical and roentgenographic findings in these infants, and their response to therapy, strongly support a causal relationship between nasopharyngeal reflux and wheezing. Therefore, nasopharyngeal reflux should be considered when a baby has intractable wheezing, even when there is no developmental problem.
Efficacy of salmeterol in the treatment of childhood asthma
Authors: TOMAÇ NAZAN , TUNCER AYFER , SARAÇLAR YILDIZ , ADALIOǦLU GÖNÜL
Published: January 2011

The aim of the clinical study reported here was to investigate the efficacy of salmeterol, a new long‐acting selective β2‐agonist, in patients with bronchial asthma. Twenty‐four children with moderate asthma were enrolled in the longitudinal study, consisting of a run‐in period of 2 weeks followed by a treatment period of 4 weeks. Maintenance treatment consisted of inhaled corticosteroid and disodium cromoglycate, or both, at the same daily dose throughout the study. During the run‐in period, the patients continued to inhale salbutamol when needed and some of them (37%) also received theophylline. They were subsequently treated with salmeterol 50 μg twice daily and prn salbutamol for 4 weeks. Efficacy was evaluated using symptom scores and pulmonary function tests including forced vital capacity parameters, pulmonary volumes, airway resistance and specific airway conductance that were measured sensitively by a whole body plethysmograph. As a result, salmeterol produced significant improvement in morning and evening peak expiratory flow rates, vital capacity, airway resistance, conductance and asthma symptoms versus salbutamol and theophylline. Adverse reactions were judged to be mild and few.
Exercise‐induced ventricular tachycardia without demonstrable heart disease in childhood
Authors: NAGASHIMA MASAMI , BABA REIZO , GOTO MASAHIKO , NISHABATA KENJI , NAGANO YOSHIKO
Published: January 2011

Exercise‐induced ventricular tachycardia (VT) without demonstrable heart disease was studied in pediatric patients. The study population consisted of 17 patients aged 5–14 years (average 9.1 years), who demonstrated reproducible VT during or immediately after exercise testing using a treadmill. The main reasons for the exercise testing were episodes of exercise‐related syncope in two patients, exercise‐related palpitation in seven and evaluation of sporadic ventricular premature contraction (VPC) in eight. Of the eight patients in the asymptomatic group, two developed sustained VT and the other six had non‐sustained VT. Of the nine patients in the symptomatic group, six developed sustained VT. Verapamil produced a good response in seven of 14 patients, and propranolol in six. None of the patients died during the follow‐up period, an average of 59.6 months. In four patients, both VT and VPC disappeared, not only on exercise testing, but also on Holter electro‐cardiograms, so the anti‐arrhythmic agents were discontinued. One patient had syncope and convulsion caused by rapid bidirectional VT in the follow‐up period. It was concluded that the prognosis of exercise‐induced VT without demonstrable heart disease in children is relatively benign, but careful follow‐up is required.
Transcatheter closure of patent ductus arteriosus using controlled‐release coils
Authors: ÇELIKER ALPAY , BİLGİÇ ARMAN , ALEHAN DURSUN , CEVİZ NACİ , LENK MUSTAFA
Published: January 2011

Controlled‐release coils have become available recently for the closure of patent ductus arteriosus (PDA). Transcatheter closure of patent arterial ducts was attempted in 13 patients, ranging in age from 5 months to 15 years, mean 4.1 years. Implantation of controlled‐release PDA coils was attempted via the femoral artery through 5 Fr catheters in all cases except one, in whom both the femoral arterial and venous routes were used. The procedure was successful in 10 of the 13 patients. In these, the pulmonary artery systolic pressure ranged between 25 and 42 mmHg and the duct diameter varied from 1.5 to 6 mm at its narrowest point. Six of the patients received a single coil. Two coils were inserted in three patients and three coils in one patient. In three patients the ducts were too large for safe release of the coils, despite attempted implantation of up to three coils simultaneously. These coils were easily withdrawn into the catheter Immediately at the end of the procedure, the duct was completely occluded in nine of the 10 patients, and in one patient there was a small residual flow. The procedure time varied between 35 min and 2.5 h, mean 81 min and the fluoroscopy time varied from 5 to 78 min, mean 25 min. None of the patients experienced hemorrhage, diminished lower extremity pulse, hemolysis or infection. In one patient, a 5 mm coil embolized into the right pulmonary artery soon after release. It was retrieved with a snare, then 8 mm and a 5 mm coil were implanted satisfactorily in the arterial duct. At follow‐up by color Doppler echocardiography, the duct was completely occluded in all patients.

Transcatheter closure of patent arterial ducts by controlled‐release PDA coils is effective and safe. Even when more than one coil is inserted, it is still cheaper than transcatheter umbrella closure. This method is therefore of great value, particularly in less affluent countries.
Reversal of fluorosis in children
Authors: GUPTA SUNIL K , GUPTA RAMESH C , SETH ASHOKA K , GUPTA ALKA
Published: January 2011

Large populations consume fluoride‐contaminated water, especially in developing countries. The toxic effects of fluorosis take three forms: clinical, skeletal and dental. Research thus far indicates that the manifestations of fluorosis are irreversible. However, it has been observed that the ingestion of calcium, vitamin C or vitamin D, individually, is effective in protection from fluoride toxicity to a certain extent. Therefore, a double blind control trial was conducted to examine the effect of a combination of calcium, vitamin D3 and ascorbic acid supplementation in fluorosis‐affected children. In the present study, 25 children were selected from an area consuming water containing 4.5 p.p.m. of fluoride, All the children were in the age group 6–12 years and weighed 18–30 kg. They were graded for clinical, radiological and dental fluorosis and relevant biochemical parameters. Grade I skeletal fluorosis and all grades of the manifestation of dental and clinical fluorosis were observed. The children were given ascorbic acid, calcium and vitamin D3 well below the toxic dosages in a double blind manner using lactose as a placebo. Follow up revealed a significant improvement in dental, clinical and skeletal fluorosis and relevant biochemical parameters in these children. Thus, the study indicated that fluorosis can be reversed, at least in children, by a therapeutic regimen that is fairly cheap, simple and easily available and without any side effects.
Effect of clonidine on the height of a child with glycogen storage disease type VI: A 13 year follow‐up study
Authors: ASAMI TADASHI , KIKUCHI TOURU , ASAMI KEIKO , UCHIYAMA MAKOTO
Published: January 2011

A 9‐month‐old male was found to have hepatomegaly when he was treated by his doctor for bronchitis. At the age of 2 years and 3 months, glycogen storage disease (GSD) of type VI (GSD VI) was diagnosed in this patient. Despite the recommended diet therapy, his growth was not good, changing under or along the line of −2.0 SD. At the age of 6 years, oral clonidine therapy (0.15 mg/day, 0.2 mg/m2 body surface per day) was started. Six to 10 months after the initiation of clonidine therapy, his height began to increase more than the values for −2.0 SD and once reached the value for −1.0 SD at the age of 10 years. His growth rate and bone age increased. Clonidine therapy was continued regularly for 7 years until the age of 13 years, 11 months. At that time his development was normal and his height reached 150.8 cm (–1.34 SD). However, cessation of the treatment at the patient's free will resulted in a reduction of the growth rate at age 15 years 6 months. These observations suggest the effect of clonidine therapy on height. Side effects were not noted during the clonidine therapy. Other clinical and laboratory findings of GSD VI also completely improved during treatment. In conclusion, administration of clonidine could be another treatment modality in children with GSD, not only of type VI but also I and III.
Motor nerve conduction studies on children with spinal muscular atrophy
Authors: MIYANOMAE YURI , TAKEUCHI YOSHIHIRO , NISHIMURA AKIRA , KAWASE SYOUJI , HIRAI KIYOSHI , OCHI MASAHARU , SAWADA TADASHI
Published: January 2011

Median and posterior tibial motor nerve conduction studies were performed on 10 children with spinal muscular atrophy (SMA). Three patients with SMA type I, in whom rapid deterioration occurred, showed reduced motor nerve conduction velocity and a remarkably low M‐wave amplitude in both nerves. In type II and III patients, the motor nerve conduction velocity was normal in the median nerve, although the M‐wave amplitude was small in the tibial nerve. In four patients, a reduction of the M‐wave amplitude was observed as clinical symptoms advanced. These findings may suggest that motor conduction studies in spinal muscular atrophy provide complementary information for understanding the pathogenesis and are also useful to clarify the heterogeneity of this disease.
Change of renal function during vancomycin therapy in extremely low birthweight infants
Authors: SAKATA HIROSHI , MARUYAMA SHIZUO , ISHIOKA TORU , SHIRAI MASARU , TAKETAZU MIO , TAKETAZU GENYA
Published: January 2011

Twenty extremely low birthweight infants were treated with vancomycin (VCM). Their gestational age was 26.3 ± 1.4 weeks (range 24.0–28.7 weeks) and their birthweight was 829 ± 133 g (range 562–990 g). At the time of initial administration of VCM, postnatal age was 29.5 ± 15.8 days (range 5–54 days). Vancomycin was administered in a dose between 9.3 and 11.0 mg/kg every 12 h for a period of 6.3 ± 2.4 days (range 4–13 days). Serum and urinary specimens were obtained before and after (within 24 h following the last dose) VCM therapy. Serum creatinine and sodium, and urinary N‐acetyl‐β‐D‐glucosaminidase (NAG) activity, creatinine, sodium, and β‐2‐microglobulin (BMG) concentrations were measured. Fractional excretion of sodium (FENa) and NAG index (NAG: creatinine ratio) were calculated. There were no significant differences between the before and after VCM treatment in serum creatinine, urinary BMG concentration, FENa and NAG index. No infant showed any symptoms of renal insufficiency. However, the NAG index and FENa increased after treatment in 1 of 20 infants. Vancomycin is effective and safe in the treatment of serious infections in extremely low birthweight infants.