bluuebunny/crossref_metadata_2025 huggingface dataset:
Extramammary Paget’s disease of the unilateral axilla: a review of seven cases in a 20‐year experience
Authors: Chiu Cheng‐Sheng , Yang Chih‐Hsun , Chen Chien‐Hsun
Published: January 2011

Background  Extramammary Paget’s disease (EMPD) is an uncommon malignancy that occurs on apocrine‐rich skin. EMPD of the axillary region is extremely rare and is not well documented.

Objectives  The aim of the study is to review our experience with axillary EMPD with regard to initial examination, treatment, and long‐term outcome.

Methods  A retrospective study of inpatient files with the diagnosis of axillary EMPD was made. The data were collected regarding patient demographics, symptoms, surgical treatments, and time to recurrence. Follow‐up information was obtained as well.

Results  Seven patients with a median age of 67.6 years were diagnosed with axillary EMPD during a 20‐year period from 1989 to 2008. The male‐female ratio was 1 : 1.3. All patients had a unilateral axillary occurrence, with three in the left and four in the right side. Patients typically presented with a chronic erythematous to brownish plaque with or without pruritus. All patients were treated with wide excision with margins up to 2–3 cm. There were no recurrences after surgery during the follow‐up period.

Conclusions  Extramammary Paget’s disease of the axillary region is a rare finding even in a large medical center. The disease process is generally a prolonged one without remarkable symptoms. The prognosis of axillary EMPD is relatively good, and long‐term follow‐up is recommended.
Control of the myiasis‐producing fly, Lucilia sericata, with Egyptian essential oils
Authors: Khater Hanem F. , Hanafy Abeer , Abdel‐Mageed Abla D. , Ramadan Mohamed Y. , El‐Madawy Reham S.
Published: January 2011

Background  Myiasis caused by Lucilia sericata (Diptera: Calliphoridae) is widely distributed throughout the world and affects both humans and animals. In addition, L. sericata larvae and adults may play a role in spreading causal agents of mycobacterial infections. Therefore, it is important to establish new and safe alternative methods of controlling this blowfly.

Methods  The insecticidal effectiveness of four commercially available essential oils [lettuce (Lactuca sativa), chamomile (Matricaria chamomilla), anise (Pimpinella anisum), rosemary (Rosmarinus officinalis)] against third larval instars of L. sericata was evaluated. The effects of sublethal concentrations of these oils on pupation rates, adult emergences, sex ratios, and morphological anomalies were also determined.

Results  The oils were highly toxic to L. sericata larvae, with median lethal concentrations (LC50) of 0.57%, 0.85%, 2.74%, and 6.77% for lettuce, chamomile, anise, and rosemary oils, respectively. Pupation rates were markedly decreased after treatment with 8% lettuce oil, and adult emergence was suppressed by 2% lettuce and chamomile oils. Morphological abnormalities were recorded after treatment with all tested oils, and lettuce was the major cause of deformation. There was a predominance of males over females (4 : 1) after treatment with lower concentrations of chamomile and rosemary; such a skew toward males would lead to a population decline.

Conclusions  The four tested oils are inexpensive and may represent new botanical insecticides for controlling blowflies.
Relevance of autoimmune thyroiditis in children and adolescents with vitiligo
Authors: Uncu Sibel , Yaylı Savaş , Bahadır Sevgi , Ökten Ayşenur , Alpay Köksal
Published: January 2011

Background  Vitiligo is the most common pigmentation‐related disorder worldwide. An autoimmune etiology is widely considered, and genetic factors may play an important role in its pathogenesis. The purpose of this study was to assess the incidence of thyroid dysfunctions and autoimmune thyroiditis in children with vitiligo and to identify related factors.

Methods  Fifty children with vitiligo and 50 control children were enrolled. Data on age, onset, duration, disease activity, presence of thyroid disorder, other autoimmune diseases, halo nevi, poliosis, and mucosal vitiligo were determined. Serum free triiodothyronine, free thyroxine, total T3, total T4, thyroid‐stimulating hormone, and antibodies to thyroperoxidase and thyroglobulin were measured. Thyroid gland efficiency was evaluated.

Results  The mean age at onset of vitiligo was 7.26 ± 4.43 years. The duration of vitiligo was 2.26 ± 2.95 years. Vulgaris‐type vitiligo was the most common form in our patients (56%), and 42% reported at least one family member with thyroid disorder, autoimmune disease, or both. Overt hypothyroidism or hyperthyroidism were not detected. We found a significant association between autoimmune thyroiditis and both sex and disease duration (P = 0.046 and P = 0.07, respectively), but no association between autoimmune thyroiditis and age, age at onset of vitiligo, halo nevi, poliosis, mucosal involvement, disease activity, or family history of vitiligo, autoimmunity, or thyroid disorders.

Conclusions  Children with vitiligo show an increased incidence of autoimmune thyroiditis. Children with vitiligo, especially girls and subjects with generalized/vulgaris‐type vitiligo, should be screened annually for thyroid function and antithyroid antibodies to assist in the early diagnosis and therapy of autoimmune thyroiditis.
The role of dermatopathology in conjunction with teledermatology in resource‐limited settings: lessons from the African Teledermatology Project
Authors: Tsang Matthew W. , Kovarik Carrie L.
Published: January 2011

Background  Access to dermatology and dermatopathology services is scarce in sub‐Saharan Africa. Teledermatology provides consultations for healthcare providers in resource‐limited settings where specialty medical services are difficult to obtain, and the African Teledermatology Project has helped to bridge the gap in dermatological care in Africa. This program also allows for biopsy specimens to be sent to the USA for processing in cases where the clinical diagnosis is difficult and definitive diagnosis has implications for patient management. This study characterizes conditions diagnosed through clinicopathological correlation in conjunction with photos and tissue submitted to the African Teledermatology Project.

Materials and methods  Retrospective case review of tissue specimens submitted over three years.

Results  Fifty‐five biopsy specimens met inclusion criteria and represent cases of malignancy (35%), infection (7%), suspected infection (15%), lichenoid tissue reaction (5%), dermatitis (15%), and other various conditions (18%). Three biopsy specimens were non‐diagnostic (5%). Clinicopathological concordance between submitting clinician and biopsy results occurred in 32 out of 55 cases (58%). Clinical and pathological diagnoses differed in 21 out of 55 cases (38%). Kaposi sarcoma (KS) represents the clinical diagnosis most often suspected in the evaluated biopsy specimens (42%) and was correctly recognized clinically in 13 out of 23 cases (57%).

Conclusion  Clinical images may not provide sufficient information to definitively diagnose certain infectious and malignant dermatological conditions submitted through telemedicine consultation. Microscopic examination of skin biopsy specimens is an important adjunct for accurate diagnosis of disease and determination of appropriate treatment strategies.
Clinical presentation and management of dermatological toxicities of epidermal growth factor receptor inhibitors
Authors: Balagula Yevgeniy , Garbe Claus , Myskowski Patricia L. , Hauschild Axel , Rapoport Bernardo L. , Boers‐Doets Christine B. , Lacouture Mario E.
Published: January 2011

The last decade in oncology has been highlighted by the emergence of novel, highly specific anti‐cancer agents, targeting a variety of molecular structures and able to inhibit aberrantly activated oncogenic pathways. Epidermal growth factor receptor inhibitors (EGFRIs) represent one type of such “targeted” agents. Their use made treatment more tolerable and resulted in significant reduction of systemic adverse effects. However, EGFRIs are associated with toxicities affecting the skin and adnexal structures, and mucosal surfaces that affect the majority of treated patients. Significant dermatologic toxicities have changed the role and involvement of dermatologists in their care. It is essential to be familiar with these adverse effects, potential complications, long‐term sequelae, and available effective treatment strategies in order to appropriately manage these patients. This review will describe the clinical presentation, histopathology, underlying mechanisms, and management options, emphasizing evidence‐based approaches.
The effect of tannic acid and polyethylene glycol on the absorption capacity of chicken intestine for d‐xylose and β‐carotene
Authors: Mansoori B. , Modirsanei M.
Published: January 2011

In an experiment, the possible influence of tannic acid (TA) and polyethylene glycol (PEG) on the absorption capacity of intestine for d‐xylose and β‐carotene in broiler chicken was investigated. Four groups of nine 28‐day‐old broiler cockerels received d‐xylose (500 mg) and β‐carotene (52 μg) solutions (Group 1 to 4) with TA (1 g, Group 2 to 4) and PEG (500 mg Group 3 and 1 g Group 4), orally. One blood sample prior to, and four others after the administration of test materials, were collected from wing vein on 40 min basis, for 160 min and the concentration of plasma d‐xylose was determined. The concentration of β‐carotene was also measured in plasma of blood samples taken prior to and 160 min post‐administration of the test materials. Plasma d‐xylose concentration of all groups showed quadratic correlations with time (p < 0.001, r2 = 0.84, 0.60, 0.70 and 0.74 for Group 1 to 4, respectively). Administration of TA reduced the plasma concentration of d‐xylose in Group 2. However, feeding PEG after TA raised the concentration of d‐xylose in Group 4 to the level that there was no difference in that variable between this group and Group 1. Although the plasma concentration of β‐carotene was increased in 160 min post‐ingestion of the test material, no difference was found in that variable among the experimental groups. In conclusion, TA and its interaction with PEG have impacts on the absorption capacity of intestine for d‐xylose and highly likely other simple sugars, but TA or PEG have no influence on the absorption of β‐carotene and most probably other fat soluble vitamins.
Distinct and combined effects of acute immobilization and chronic isolation stress on MAO activity and antioxidative protection in the heart of normotensive and spontaneously hypertensive rats
Authors: Djordjevic J. , Jasnic N. , Vujovic P. , Lakic I. , Djurasevic S. , Gavrilovic Lj. , Cvijic G.
Published: January 2011

The heart is an organ especially sensitive to the sympathetic overstimulation and therefore to the influence of stressors and hypertension. The aim of the present study was to investigate the effect of two distinct types of stressors, acute immobilization (2 h) and chronic isolation stress (21 days), as well as their combined effect on the activity of monoamine oxidase (MAO), superoxide dismutase, catalase (CAT) and the ascorbic acid (AA) content in the heart of normotensive and spontaneously hypertensive rats (SHR). The results obtained show that in basal conditions heart MAO and CAT activity (p < 0.05), as well as AA concentration (p < 0.01) were higher in SHR than in normotensive ones. The acute immobilization significantly decreased heart MAO activity in both examined strains (p < 0.01). On the other hand, chronic isolation, separately or in combination with immobilization, did not affect this enzyme, in the heart of either hypertensive or normotensive rats, which was associated with the reduced antioxidative protection (p < 0.01, p < 0.05).
Hydrogen resuscitation, a new cytoprotective approach
Authors: Zheng Xing‐Feng , Sun Xue‐Jun , Xia Zhao‐Fan
Published: January 2011

1. Hydrogen is a colourless, odourless, tasteless and flammable gas. Hydrogen is considered a physiologically inert gas and is often used in deep sea diving medicine. In mammals, endogenous hydrogen is produced as a result of the fermentation of non‐digestible carbohydrates by intestinal bacteria and it is absorbed into the systemic circulation.

2. Recent evidence indicates that hydrogen is a potent anti‐oxidative, anti‐apoptotic and anti‐inflammatory agent and so may have potential medical application. The present review evaluates the concept of ‘hydrogen resuscitation’, based on knowledge that hydrogen treatment effectively protects cells, tissues and organs against oxidative injury and helps them recover from dysfunction.

3. Hydrogen therapy can be delivered by inhalation, the administration of hydrogen‐enriched fluid or by approaches that affect endogenous hydrogen production.

4. Studies have shown that hydrogen resuscitation has cytoprotective effects in different cell types and disease models, including ischaemia–reperfusion injury, inflammation, toxicity, trauma and metabolic disease. The underlying mechanism may be the selective elimination of hydroxyl radicals, although other mechanisms may also be involved (e.g. hydrogen functioning as a gaseous signalling molecule).

5. Hydrogen resuscitation may have several potential advantages over current pharmacological therapies for oxidative injuries. However, more work is needed to identify the precise mechanism underlying the actions of hydrogen and to validate its therapeutic potential in the clinical setting.
Renal inflammation, autoimmunity and salt‐sensitive hypertension
Authors: Rodríguez‐Iturbe Bernardo , Franco Martha , Tapia Edilia , Quiroz Yasmir , Johnson Richard J
Published: January 2011

1. The present article reviews the role of immune‐competent cells infiltrating the kidney and their association with oxidative stress and renal angiotensin activity in the development of salt‐sensitive hypertension.

2. We discuss changes in the pressure–natriuresis relationship resulting from renal inflammation and its improvement resulting from immunosuppressive treatment.

3. The potential role of T‐cell‐driven reactivity in sustaining the renal inflammation is examined in the light of accumulating evidence of autoimmune mechanisms in experimental and clinical hypertension.
Improving nutritional status of children with cystic fibrosis at Red Cross War Memorial Children's Hospital
Authors: van der Spuy Dorothy A , Cader Shihaam , van der Spuy Gian D , Westwood Anthony T
Published: January 2011

Aim:  To determine the nutritional status of children attending a cystic fibrosis clinic in a tertiary hospital in South Africa and compare it to previously reported 10‐year rates.

Methods:  Weights and heights were measured of 69 (37 male and 32 female) children aged between 1 year and 18 years. Expected weight‐for‐age, expected height‐for‐age, expected weight‐for‐height and body mass index (BMI) were compared with international standards for underweight, stunting, wasting and BMI goal.

Results:  The nutritional status of the patients has improved over the last 10 years, most significantly for wasting, which decreased from 58.3% in 1996 to 15.9% in 2006 (95% confidence interval (CI), 1.315–14.09, P < 0.05). Fifty‐two percent of the children were underweight in 2006, compared with 66.7% in 1996 (95% CI, 0.044–13.96, P < 0.05). Stunting was found in 31.9% of the current sample. Females over 15 years had expected weight‐for‐age 25.9% lower than those between 10 years and 15 years, while no difference was found between the male age groups. Female height‐for‐age was 7.06 percentage points greater than males between 10 years and 15 years (95% CI, 2.16–11.96, P < 0.01). Males between 10 years and 15 years had significantly lower BMIs than the corresponding female group. Coloured patients had significantly lower BMIs than white patients in all age groups.

Conclusions:  These children demonstrated continuing improvement in nutritional status, although deficits remain. The normalisation of mean weight‐for‐age and weight‐for‐height with far fewer wasted patients is encouraging. Interventions are needed in some areas to ensure that all children show progress.
Maternal reasons for non‐immunisation and partial immunisation in northern Nigeria
Authors: Babalola Stella
Published: January 2011

Aim:  To compare maternal reasons for non‐immunisation and for partial immunisation in northern Nigeria, and determine the link between specific reasons and future intentions to immunise.

Methods:  Responses to open‐ended questions collected through a 2007 questionnaire survey were individually coded for key words using the regexm command in Stata (StataCorp, College Station, TX, USA). Simple percentages are used to analyse the differences in reasons for non‐immunisation and partial immunisation. Logistic regression serves to assess the relationship between specific reasons for non‐immunisation and future intentions to immunise.

Results:  The reasons for non‐immunisation generally differ from those advanced for partial immunisation. In general, reasons for non‐immunisation have to do with ideational and normative factors. In contrast, supply‐side factors are the reasons most often advanced for partial immunisation, although lack of knowledge also plays a strong role. Some reasons for non‐immunisation are more compatible with future intention to immunise than others.

Conclusions:  Efforts to promote the uptake of immunisation need to address both demand‐ and supply‐side factors. Increasing knowledge about immunisation, changing negative attitudes about immunisation, debunking myths and rumours about immunisation, and addressing religious, ethnic and political bases for resistance to immunisation are necessary to encourage parents to initiate child immunisation. To promote timely completion of immunisation schedule, programmes will need to improve vaccine supply, strengthen provider's capacity for quality service and increase community knowledge about immunisation.
Two color flow cytometric analysis of lymphocytes in HTLV‐I‐carrier children
Authors: SATO TAKAMI , YANAGISAWA MASAYOSHI , MUCHI HIROMU
Published: January 2011

Phenotypic changes in lymphocytes from healthy children with antibody for human T lymphotrophic virus type I (HTLV‐I) were studied using two color flow cytometry. The subjects were high school students within a small district of Kyushu, Japan. In the carrier group, a subset of lymphocytes which expressed CD8+CD57+ was significantly lower in percentage (P < 0.01) and in number (P < 0.05). There was no significant difference between carriers and non‐carriers in the percentage and number of CD4+ Leu8+cells or CD4+ HLA‐DR+ cells. The IgG levels were slightly higher in carriers than in non‐carriers (P < 0.05). These findings suggest that HTLV‐I‐infected cells may affect the immune system in healthy carrier children.
The in vivo and in vitro postantibiotic effect of aminoglycosides using a clinically isolated micro‐organism
Authors: ARAI SHOJIRO , KAWAMURA REIKO , KAMIYA HITOSHI , ISHII TAKAHIRO , SAKURAI MINORU
Published: January 2011

We reported a case of abscess in which Serratia marcescens was isolated as the causative organism. We measured the postantibiotic effect (PAE) of dibekacin (DKB) and gentamicin (GM) against S. marcescens and studied the relationship between the clinical effect and the PAE. The minimal inhibitory concentration (MIC) of DKB against S. marcescens was 6.25 μg/mL and the serum concentration 30 min after infusion of 100 mg DKB was 5.99 μg/mL. The abscess was cured by the administration of DKB every 12 h. The PAE in vivo was 2.5, 2.9 and 3.3 h when DKB was administered at 50 mg/kg, 100 mg/kg and 200 mg/kg, respectively. This PAE is one of the reasons that infection can be effectively treated with intermittent administration, even if the serum concentration is below the MIC.
Role of vagotony in sinus node dysfunction in children with symptomatic congenital long QT syndrome
Authors: MATSUOKA SUGURU , AKITA HIROSHI , TAKAHASHI YOSHIO , NISHIOKA ATSUKO , KURODA YASUHIRO
Published: January 2011

The present study examined chronotropic dysfunction and the role of vagotony in congenital long QT syndrome, sinus node function and the effects of parasympathetic blockade. Six patients with congenital long QT syndrome were studied. The four males and two females, aged 1–15 years, had episodes of syncope and malignant ventricular arrhythmias. Congenital long QT syndrome was defined as a corrected QT interval greater than 0.45 s, T wave alternans and the age at diagnosis.

The sinus heart rate measured from a 24 h electrocardiograph was abnormally low (< 50 min) in three patients (1, 4 and 5 years old) and did not increase sufficiently with the administration of atropine in five of the six patients with congenital long QT syndrome. From intracardiac electrophysiological studies, the corrected sinus node recovery time was prolonged in three patients and the total sinoatrial conduction time was prolonged in two patients. In most patients who had an abnormally long sinoatrial conduction time and corrected sinus node recovery time, these values returned to normal following atropine administration. In one patient, the corrected sinus node recovery time was prolonged paradoxically by atropine. Sinus node dysfunction in congenital long QT syndrome was affected by vagotony associated with a right sympathetic nerve system abnormality.
Peripheral expansion of ωδT cell receptor‐positive cells in a patient with Crohn's disease
Authors: HARA TOSHIRO , MATSUMOTO TADASHI , MIZUNO YUMI , NISHIZAKI MARI , UEDA KOHJI , MOTOOKA MAKOTO , KIMURA NOBUHIRO , OSHIMI KAZUO , OHGA SHOICHI , YOSHIKAI YASUNOBU
Published: January 2011

A13 year old boy with Crohn's disease had a prominent expansion (over 40%) of ωδT cell receptor‐positive cells (ωδT cells) in the peripheral blood. The ωδT cells were gradually decreased from 48 to 22% in 18 months. Anti‐ωδT cell receptor‐triggered cytotoxicity was evident at onset and after 1 year. The significance of peripheral expansion of ωδT cells in a patient with Crohn's disease is discussed.
Dilated cardiomyopathy in a patient receiving continuous ambulatory peritoneal dialysis
Authors: OHMOTO SHUKO , MIHARA SEIKO , FUJINO MITSUYOSHI , MORITA TETSURO , MORITA KOICHI , KOUNO KOJI
Published: January 2011

We describe a unique case of a 10 year old girl with dilated cardiomyopathy who has been receiving continuous ambulatory peritoneal dialysis. She responded to treatment with a β1‐agonist. We note that electrocardiography and echocardiography are effective for early recognition of the onset of dilated cardiomyopathy and that a thallium scan should be performed to assess the severity of dilated cardiomyopathy in patients with chronic renal insufficiency.
Transient scoliosis in a child with juvenile rheumatoid arthritis
Authors: IWATA FUJIHIKO , HANAWA YOSHIO , TAKASHIMA HIROTADA , JOE TAKETOSHI
Published: January 2011

An 8 year old girl was admitted to hospital complaining of arthralgia in a few large joints. According to the clinical course and serological tests, a diagnosis of a polyarticular type of juvenile rheumatoid arthritis was made. About 6 weeks after the onset, scoliosis was observed. The curvature regressed spontaneously over the following 6 months.

Generally, scoliosis associated with juvenile rheumatoid arthritis has been noted a few years after the onset. A transient scoliosis in the early phase of this disease is rare.
An autopsied case of Williams syndrome complicated by moyamoya disease
Authors: KAWAI MIE , NISHIKAWA TOSHIO , TANAKA MASATO , ANDO AKIKO , KASAJIMA TAKESHI , HIGA TAKASHI , TANIKAWA TATSUYA , KAGAWA MIZUO , MOMMA KAZUO
Published: January 2011

An 18 year old girl with typical clinical features of Williams syndrome suddenly died of intracerebral hemorrhage due to moyamoya disease. Autopsy revealed vascular abnormalities, such as supravalvular aortic stenosis (SAS) and an abnormal complicated cerebrovascular network in the cerebral arteries. The arterial wall of the SAS lesion consisted of thickened medial tissue showing elastic disorganization with prominence of the smooth muscle cells. The narrowed vessels of the circle of Willis showed intimal thickening with an extremely wavy internal elastic lamina and marked thinning of the media. To our knowledge, this is the first report of moyamoya disease associated with Williams syndrome.
Polymerase chain reaction for diagnosis of infectious diseases
Authors: NARITA MITSUO
Published: January 2011

The polymerase chain reaction (PCR) has been utilized and demonstrated to be useful for detecting minute amounts of a wide variety of infectious agents. In such studies, one must keep in mind that the most appropriate conditions for amplification vary with organisms of interest. In this study, PCR was used as a rapid and sensitive method for detecting infectious agents for which three assay systems were devised comprising the method for the amplification of human T cell leukemia virus type I, Mycobacterium tuberculosis and Mycoplasma pneumoniae. The possibility of vertical transmission of human T cell leukemia virus type I through cord blood was demonstrated using cord blood mononuclear cells from carrier mothers of this virus. In the study of Mycobacterium tuberculosis, PCR was shown to be efficient, particularly in detecting this organism in extrapulmonary cases. Evidence of direct invasion into the central nervous system by Mycoplasma pneumoniae and the concomitant occurrence of mycoplasmaemia in the mycoplasmal central nervous system involvement was obtained using PCR. These results validated the potential of PCR in the clinical research of infectious diseases.
Granulocyte elastase α1‐proteinase inhibitor complex measurement in very low birthweight infants with severe intraventricular hemorrhage
Authors: TAKASAKI JIRO , KAMIYA KENJI , OGAWA YUNOSUKE , TANAKA TAIHEI , KOYAMA NORIHISA
Published: January 2011

We investigated the relationship between the levels of granulocyte elastase α1‐proteinase inhibitor complex (E‐α1‐PI) in plasma and severe intraventricular hemorrhage (IVH) in very low birthweight infants. The concentrations of E‐α1‐PI and the ratio of the concentrations of E‐α1‐PI to polymorphonuclear leucocyte counts within 24 h of birth in infants with severe IVH were significantly higher compared with those in infants without severe IVH. E‐α1‐PI seems to be a useful indicator of IVH.
Increased type III/I collagen and α1(I)/α2(l) chain in a bronchopulmonary dysplastic lung
Authors: TOGARI HAJIME , HASHIMOTO YOKO , WADA YOSHIRO , HAYAKAWA TARO
Published: January 2011

The type ratio of collagen III/I and molecule ratio of α1(I)/α2(I) were analysed in lungs from five infants with bronchopulmonary dysplasia (BPD) and seven control infants. All five BPD lungs showed typical characteristics, with pulmonary fibrosis and irregular cystic formation at postmortem examination. A pepsin digestion technique followed by interrupted gel electrophoresis was performed for the determination and molecular analysis of collagens III and I. The ratio of collagen types III and I were significantly increased in BPD lung (P < 0.005). The ratio of α1(I) and α2(I) were also significantly increased in BPD lung (P < 0.05). It is likely that collagen III is predominantly increased and that the α1(I) trimer is formed in the active stage of BPD lungs.
Recombinant human granulocyte colony‐stimulating factor therapy for cyclic neutropenia associated with common variable immunodeficiency
Authors: TSUDA MASAHIKO , URAKAMI TATSUHIKO , WATANABE SHUICHIRO , SHIMIZU HISASHI , INANA ICHIRO , KIKKAWA YUMIO , KITAGAWA TERUO
Published: January 2011

A 14 year old boy with common variable immunodeficiency (CVID) had regularly recurring episodes of severe infections independently of the serum γ‐globulin level. Serial blood counts revealed that this patient also had cyclic neutropenia. Recently, recombinant human granulocyte colony‐stimulating factor (rhG‐CSF) was reported to be an effective treatment for this disease. We tried rhG‐CSF therapy for this patient and a prompt increase in the neutrophil count was noted. However, the cyclic alterations and duration of the nadir of the neutrophil count were not altered, which suggested that rhG‐CSF has a variable efficacy in at least some patients with cyclic neutropenia.
Evaluation of proximal tubular function in preterm infants by urinary α1‐microglobulin
Authors: TSUKAHARA HIROKAZU , HIRAOKA MASAHIRO , KURIYAMA MASANORI , HARUKI SHINICHI , NAKAMURA KATSUJI , SUEHIRO FUMIHIKO , SUDO MASAKATSU
Published: January 2011

α1‐Microglobulin is a low molecular weight protein that is relatively stable in urine of low pH. There have been few reports on urinary α1‐microglobulin (U‐A1M) excretion in preterm infants. This study was designed to establish the ranges for U‐A1M in clinically stable preterm infants and to investigate changes observed in sick preterm infants. We measured U‐A1M and urinary β2‐microglobulin (U‐B2M) levels at 1, 4, 7, 14, 28 and 90 days after birth in stable preterm infants (Group 1) and sick preterm infants who were depressed at birth and required immediate resuscitation (Group 2). In Group 1 infants, both parameters were high during the first 28 days and appeared to decline thereafter. U‐A1M in Group 2 infants was only significantly increased compared with Group 1 on day 1, as was U‐B2M. On each day of the study, U‐A1M had significant positive correlations with U‐B2M for all the infants studied. The changes of the two parameters observed in Group 1 probably reflect postnatal evolution of proximal tubular function in stable preterm infants. A comparison of groups 1 and 2 shows a high prevalence of acute tubular injury at birth in sick infants and also suggests that U‐A1M as well as U‐B2M may be a sensitive index for detecting acute tubular damage and for following its course in preterm infants.
A longitudinal study of blood pressure, cholesterol and left ventricular muscle volume in children: The Shimane Heart Study
Authors: ABE KATSUTOSHI , NISHIO TOSHIKAZU , MORI CHUZO , HANEDA NORIYUKI , WATANABE KOJI
Published: January 2011

Three hundred and forty‐four healthy schoolchildren living in Izumo City, Shimane Prefecture, Japan, were assessed at 3 year intervals from 6 to 15 years, starting in 1978 (Cohort 1) or 1981 (Cohort 2). Tracking indices (Ti) were calculated as follows: Ti = (2x + y ‐ z)/N/0.89, where x, y and z refer to the total number in the same, adjacent and remote trisections, respectively, and N = x + y + z. If Ti > 1, there is positive tracking. For systolic blood pressure (SBP) measurements (except at 6 years old) and serum cholesterol levels, all Ti were greater than 1.0 regardless of the time when tracking was commenced. Tracking indices were slightly greater in the serum cholesterol levels than in the SBP measurements. Left ventricular muscle volume indices (LVMVI) were calculated by echocardiographic measurements. In girls, the LVMVI was larger only in the above‐median SBP group at the age of 12 years (P < 0.01), but the LVMVI of the higher SBP (above median) group was larger than in the lower (below median) group at every age in boys; the difference was statistically significant (P < 0.05) at 12 and 15 years of age. Left ventricular mass enlargement may occur in the prehypertensive stage in humans as well as in rats.
Endoscopic ultrasonography in a child with chronic pancreatitis
Authors: KATO SEIICHI , FUJITA NAOTAKA , SHIBUYA HIDENORI , NAKAGAWA HIROSHI
Published: January 2011

In a case with chronic pancreatitis and pseudocysts related to muscular cytochrome—c oxidase deficiency, endoscopic ultrasonography (EUS) was performed to morphologically examine the pancreas for features that may have been missed by abdominal computed tomography and pancreatography. Conventional abdominal sonography had failed to show valuable diagnostic information of chronic pancreatitis in this case. EUS demonstrated several new findings. Parenchymal atrophy was remarkable, especially in the pancreatic tail, and a dilated pancreatic main or branch duct was clearly found in the region. Moreover, pancreatic stones in the main duct were also distinctly proved. EUS may become a useful new diagnostic method in pediatric gastroenterology, especially when sufficient findings are not obtained by conventional imaging techniques. However, an ultrasonographic endoscope with a shorter rigid portion and smaller caliber should be developed for pediatric patients.
Bile duct atresia following extended right hepatectomy because of a tumor
Authors: TAKEHARA HIROO , KOMI NOBUHIKO , OKADA AKIRA , MASAMUNE KATSUHIRO , ASAI AKIKO
Published: January 2011

A 19 month old male infant with a mesenchymal hamartoma of the liver underwent an extended right hepatectomy. Serum bilirubin gradually rose until 3 months after the surgery, and obstructive jaundice and acholic stools were manifested at 6 months. Percutaneous transhepatic cholangiodrainage was performed. Cholangiography showed dilation of the intrahepatic bile duct of the residual lateral segment and complete obstruction of the extrahepatic bile duct. A second operation for reconstruction of the biliary tract was performed 10 months after the first surgery. No aspect of an extrahepatic biliary tract was found. Histological inspection of a surgical specimen of remnant tissue revealed only cicatricial connective tissue without any biliary structures. The clinical course has been uneventful for 18 months since the second surgery. The cause of bile duct atresia in this case is strongly suggested to be an ischemic change due to devascularization of the extrahepatic biliary tract following hepatic resection because of a tumor. To prevent this kind of complication, hepaticoenterostomy should be performed close to the cut surface of the liver.
A case of biliary stricture caused by cavernous transformation of the portal vein
Authors: TAKEHARA HIROO , KOMI NOBUHIKO , OKADA AKIRA , MASAMUNE KATSUHIRO , ASAI AKIKO
Published: January 2011

A case is reported of extrahepatic obstruction of the portal vein with the unusual complication of obstructive jaundice that resulted from compression of the common bile duct caused by cavernous transformation of the varicose network. It is surgically impossible to repair choledochal stenosis resulting from enlarged varices in the vicinity of the common bile duct. In this case, internal drainage from the biliary duct to the duodenum using a silastic tube and a reservoir implanted in the subcutaneous space successfully eliminated the jaundice.
Child traffic accident injuries in Japan
Authors: SAITO RYOKO
Published: January 2011

This study analyzes the characteristics of traffic accidents involving children. It discusses why children with certain attributes and in specific situations are exposed to a higher traffic accident risk, in light of their ability to deal with traffic and their body characteristics. Finally, based on these results, it offers some conclusions and recommendations on ways of improving child safety in road traffic.
Role of a children's hospital safety centre in injury control
Authors: HANNAH LYNDA , NOLAN TERRY
Published: January 2011

Over the past 13 years, child safety centres have been established at several Australian Children's hospitals. In a short time, they have developed an important role in community injury control programmes. These centres provide an efficient conduit for transferring up‐to‐date injury surveillance and prevention information to families, industry, health and education professionals, and government. The activities of the Child Safety Centre at the Royal Children's Hospital in Melbourne are described, together with ways in which a degree of self‐funding can be achieved.
Influence of long‐term carbamazepine treatment on thyroid function
Authors: YÜKSEL ADNAN , YALÇIN ERDINÇ , CENANİ ASIM
Published: January 2011

We have studied the effects of carbamazepine on thyroid function in sixteen recently diagnosed epileptic children and thirteen epileptic children receiving long‐term carbamazepine therapy and compared these findings with the thyroid function of thirteen healthy control subjects. Thyrotropin (TSH), tri‐iodothyronine (T3), thyroxine (T4), free tri‐iodothyorinine (FT3) and free thyroxine (FT4) serum levels were determined in both recently diagnosed but as yet untreated epileptic children and normal controls. These hormone levels were determined again after 2 months of treatment and 12 months of treatment in epileptic children. No statistically significant difference was found in the endocrine parameters of untreated epileptic children and the normal control group. After both 2 months and 12 months of carbamazepine therapy, serum levels of T4, FT4 and FT3 were found to be low, but the serum T3 concentration was unaffected. Baseline TSH levels were not changed during carbamazepine therapy either. Serum TSH levels increased rapidly after thyrotropin‐releasing hormone stimulation in both the before and 12 months after carbamazepine treatment groups, but the response was higher in the 12 months treatment group. The findings of the present study suggest that accelerated hormone metabolism is responsible for hormonal changes found in patients treated with carbamazepine. Carbamazepine also had effects on the function of the hypothalamo‐pituitary axis.
A new and rare form of Williams' syndrome
Authors: AKÇORAL ADNAN , ÖZAKSOY DINÇ , ORAN BÜLENT , NARIN NAZMI , ÇEVIK NECLA T.
Published: January 2011

A 7 year old girl and her mother have typical phenotypic features of the Williams' (elfin facies) syndrome, and both have only mild mitral regurgitation with mitral valve prolapse (MVP). Mitral valve prolapse has been reported in only three cases but pure mitral insufficiency has not been reported alone. In this paper two cases of a new rare form of Williams' syndrome with MVP are presented and this rare disorder is discussed.
Chronic hypomagnesemia and hypokalemia due to renal wasting in siblings
Authors: IWATA FUJIHIKO , HANAWA YOSHIO , TAKASHIMA HIROTADA
Published: January 2011

A 13 year old girl with carpopedal spasm is presented. Investigation values showed hypomagnesemia and hypokalemia. Her younger brother and sister demonstrated the same biochemical abnormalities without any symptoms. Their urinary excretion of magnesium and potassium were inappropriately high compared with their serum levels. Treatment with oral magnesium sulfate failed to correct the abnormalities, but serum levels of magnesium and potassium were just below the lower limits. This familial disease may represent congenital renal wasting of magnesium and potassium.
Levels of serum ferritin and total body iron among infants with different feeding regimens
Authors: HOKAMA TOMIKO
Published: January 2011

The levels of serum ferritin and total body iron of healthy infants were analyzed to evaluate iron status among infants with different feeding regimens. The results showed that the levels of serum ferritin and total body iron were lower in breast‐fed infants than in partially breast‐fed or formula‐fed infants at late infancy and 18 months of age.
Discriminant analysis of clinical tests in histologic diagnosis of renal disease
Authors: MASUTANI ATSUSHI , INOUE MAKOTO , MORI CHUZO
Published: January 2011

We have used discriminant analysis of the results of clinical examination to study whether or not it is possible to presume the histological findings of the kidney within the limits of the screening tests (urinalysis and blood tests) presently conducted at the outpatient clinic.

A retrospective analysis was made of the data for 42 children who were found in school urine tests to have urinary abnormalities. Two discriminant functional equations were made and the overall rates of correct discrimination were ≥90%. Proteinuria, IgA and complement titer were selected. These results were considered useful in determining the indication for renal biopsy.
Prediction of engraftment after peripheral blood stem cell transplantation by CD34‐positive cells in grafts
Authors: URASHIMA MITSUYOSHI , UCHIYAMA HIROSHI , HOSHI YASUTAKA , DEGUCHI YASUSHI , KAMIJOU MAKOTO , KATOU YOUKO , FUJISAWA KOUJI , AKATSUKA JUN‐ICHI , MAEKAWA KIHEI
Published: January 2011

total of 91 peripheral blood stem cell collections were performed in 26 children with various malignant tumors and peripheral blood stem cell transplantations (PBSCT) were performed in 15 of the children. There was a positive correlation between logarithm of total CD34+ cells/kg and logarithm of colony‐forming unit‐granulocyte macrophage (CFU‐GM)/kg (r = 0.86). The time elapsed until the white blood cells (WBC) exceeded 1000/μL was related to both CFU‐GM (r = 0.67) and CD34+ cell count (r = 0.60). The number of days elapsed until platelet count exceeded 5 times 104/μL was not related to the logarithm of CFU‐GM count/105 per kg transfused (r = 0.47), but was related to the logarithm of CD34+ cell counts/106 per kg transfused (r = 0.73). The number of days elapsed until the reticulocytes exceeded 5 times 104/μL was not related to the logarithm of CFU‐GM count/105 per kg (r = 0.52), but was related to the logarithm of CD34+ cell counts/106 per kg transfused (r = 0.91). Although CD34+ cell counts correlated with the number of CFU‐GM, bone marrow regeneration rates in three lineages were predicted more accurately by the number of CD34+ cells transfused than by the number of CFU‐GM. These results suggest that measurement of the CD34+ cell count may be useful in predicting bone marrow regeneration rate after PBSCT.
Partial trisomy for short arm of chromosome 5
Authors: YASUTOMO KOJI , SUZUE TAKESHI , NISHIOKA ATSUKO , KOZAN HIROKO , SEKIGUCHI TAKANORI , OHARA KATSUAKI , OKAMOTO TAKASHI , IWAI TSUYAKO , ENDO SHOICHI
Published: January 2011

We present two unrelated cases of partial trisomy for the short arm of chromosome 5, the first such cases reported in Japan. The features are characterized by hypertelorism, low set ears, arachnodactyly, laryngostenosis, hypotonia and some cerebral malformation. The characteristic facial expression and arachnodactyly are the key features used to diagnose this disorder.

A high‐resolution chromosome banding technique showed that the karyotype of the first patient was 46, XX, inv dup(5) (p13.1 ← p15.3) de novo and that of the second patient was 46, XX, dir dup(5) (p13.3 ← p15.2) de novo. The similar symptoms in the two cases, despite the difference in karyotypes, were caused by duplication of 5p including segment 5p13. This would be a key site for this disorder.
Varicella‐zoster virus replication site in internal organs of an otherwise healthy child with varicella and sudden death
Authors: ASANO YOSHIZO , YOSHIKAWA TETSUSHI , URISU ATSUO , YAZAKI TAKEHIKO , MIZOGUCHI YOSHIKAZU , KURATA TAKESHI
Published: January 2011

Pathological findings of an otherwise healthy 17 month old boy who was exposed to Varicella‐zoster virus (VZV) in his family and unexpectedly died 3 days after onset of varicella are reported. They showed a disseminated VZV infection with involvement of skin, lung, liver, spleen, gastrointestinal tract and other organs where VZV antigen was detected by the enzyme‐immunoassay with monoclonal antibodies to VZV. Since the subject was the full‐term product of an uncomplicated pregnancy, who grew and developed normally, and had no symptoms or laboratory findings suggestive of immunodeficiency until his death, these findings suggest that many organs are involved as major internal sites of viral replication before or during infection of skin with VZV in the immunocompetent host.
Moyamoya disease associated with bilateral renal artery stenosis
Authors: NAKANO TAKASHI , AZUMA EIICHI , IDO MASARU , ITOH MASAHIRO , SAKURAI MINORU , SUGA SHIGERU , KAWAGUCHI HIROSHI
Published: January 2011

Recent research has suggested that an association exists between moyamoya disease and fibromuscular dysplasia which involves systemic vessels, including renal arteries. We report a 3 year old girl with moyamoya disease associated with bilateral renal artery stenosis. This case may support the common etiology of these two clinical conditions. To our knowledge this is the youngest case of moyamoya disease associated with bilateral renal artery stenosis.
Megacystis and megacolon in an infant with Ehlers‐Danlos syndrome
Authors: SATO TADASHI , ITO HIROYUKI , MIYAZAKI SUMIO , KOMINE SHINICHIRO , HAYASHIDA YUTAKA
Published: January 2011

We report a 14 month old male infant with Ehlers‐Danlos syndrome who became ‘anuric’ due to an acutely dilated urinary bladder. Although the patient was also found to have megacolon, no diverticulum was seen in his gastrointestinal tract or urinary bladder. In order to decompress the urinary bladder and colonic wall, we put an indwelling urinary catheter in place for 2 months, and carried out daily glycerin enema for 3 months. All urological and gastrointestinal symptoms subsided with this intensive medical treatment. The diagnosis of megacystis and megacolon was made very early in life for this patient. This may indicate that the striking extension of gastrointestinal and bladder wall may lead to the development of diverticula of gastrointestinal and urinary tracts in later life.
Screening for growth hormone deficiency using urinary growth hormone measurement
Authors: KUBOTA AKIRA , OGAWA HARUO , IGARASHI YOSHIO , MIZUNO YOSHIHITO , TANIGUCHI KAZUTOSHI
Published: January 2011

The diagnostic approach in growth hormone deficiency (GHD) is complicated. Two or more provocative tests are essential for definitive diagnosis of GHD. However, such testing cannot be carried out routinely on all subjects with short stature because of the need for hospitalization and blood sampling. A simple screening method for GHD would be of great value. Human growth hormone (hGH) levels were measured in the early morning urine of 192 children aged 7–15 years with height 2.0 s.d. below the mean for their ages. Sixty‐eight subjects were selected because they showed a urinary hGH level < 10 ng/g creatinine. They were further examined in terms of bone age and plasma insulin‐like growth factor (IGF‐I) levels. In 30 subjects, the ratio of bone age: chronological age was < 0.8 and/or plasma IGF‐I level was < 0.7 U/mL.

Finally 24 of these subjects were examined with provocative tests and other endocrinological tests. Eleven subjects proved to have poor growth hormone secretion and one subject was diagnosed as having Turner syndrome.

In conclusion, 11 patients with GHD were diagnosed from 192 children with short stature using urinary hGH measurement as the first screening method. These findings suggests that urinary hGH measurement could be a useful and simple method for detecting GHD.
Virological and serological studies on poliomyelitis in Karachi, Pakistan. I. Outbreaks in 1990‐91
Authors: ISOMURA SHIN , MUBINA AGBOATWALLA , DURE‐SAMIN AKRAM , ISIHARA YUICHI , SAKAE KENJI , YAMASHITA TERUO , NISHIO OSAMU , AHMED AKHTAR
Published: January 2011

Between October 1989 and September 1991, 124 cases of poliomyelitis visited the Department of Paediatrics, Civil Hospital Karachi, Pakistan. The majority of them were between 6 months and 2 years of age and the epidemics occurred during the hot seasons. The dominant serotype was poliovirus type 1 during the epidemic season in 1990 and type 2 in 1991. All the polioviruses isolated from the patients were wild‐type. Virological studies also disclosed that enteroviruses other than polioviruses were prevalent among healthy children as well as among diarrheal and polio patients.

A serological survey to elucidate the serological efficacy of oral polio vaccine (OPV) showed that: (i) in 112 unimmunized children, after disappearance of transplacental maternal antibody during early infancy, antibody prevalence increased gradually and > 80% of the children were seropositive against all three types of polioviruses at 5 years of age; (ii) in 201 children immunized with full doses of OPV in their infancy, the decrease in antibody titer during infancy was less and seroprevalence rose sharply afterwards: at 2 years of age, > 80% of them were seropositive against all three types of the virus. The rapid increase of seroprevalence might be the effect of OPV administration. However, the prevalence was lower than that in developed countries.