bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical aspects of obesity in childhood and adolescence
Authors: Kiess W. , Galler A. , Reich A. , Müller G. , Kapellen T. , Deutscher J. , Raile K. , Kratzsch J. 
Published: March 2003

SummaryThe level of fatness of a child at which morbidity acutely and/or later in life increases is determined on an acturial basis. Direct measurements of body fat content, e.g. hydrodensitometry, bioimpedance, or DEXA, are useful tools in scientific studies. However, body mass index (BMI) is easy to calculate and is generally accepted now to be used to define obesity in children and adolescents clinically. An increased risk of death from cardiovascular disease in adults has been found in subjects whose BMI had been greater than the 75th percentile as adolescents. Childhood obesity seems to substantially increase the risk of subsequent morbidity whether or not obesity persists into adulthood. The genetic basis of childhood obesity has been elucidated to some extent through the discovery of leptin, the ob gene product, and the increasing knowledge on the role of neuropeptides such as POMC, neuropeptide Y (NPY) and the melanocyte concentrating hormone receptors (for example, MC4R). Environmental/exogenous factors largely contribute to the development of a high degree of body fatness early in life. Twin studies suggest that approximately 50% of the tendency toward obesity is inherited. There are numerous disorders including a number of endocrine disorders (Cushing's syndrome, hypothyroidism, etc.) and genetic syndromes (Prader‐Labhard–Willi syndrome, Bardet Biedl syndrome, etc.) that can present with obesity. A simple diagnostic algorithm allows for the differentiation between primary or secondary obesity. Among the most common sequelae of primary childhood obesity are hypertension, dyslipidemia, back pain and psychosocial problems. Therapeutic strategies include psychological and family therapy, lifestyle/behaviour modification and nutrition education. The role of regular exercise and exercise programmes is emphasized. Surgical procedures and drugs used in adult obesity are still not generally recommended in children and adolescents with obesity. As obesity is the most common chronic disorder in industrialized societies, its impact on individual lives as well as on health economics has to be recognized more widely. This review is aimed towards defining the clinical problem of childhood obesity on the basis of current knowledge and towards outlining future research areas in the field of energy homoeostasis and food intake in relation to child health. Finally, one should aim to increase public awareness of the ever increasing health burden and economic dimension of the childhood obesity epidemic that is present around the globe.
A review of the literature of Bardet–Biedl disease and report of three cases associated with metabolic syndrome and diagnosed after the age of fifty
Authors: Iannello S. , Bosco P. , Cavaleri A. , Camuto M. , Milazzo P. , Belfiore F. 
Published: March 2003

SummaryBardet–Biedl syndrome (BBS) is a genetic autosomal‐recessive disease (formerly grouped with Laurence–Moon–Biedl syndrome but considered today as a separate entity) characterized by abdominal obesity, mental retardation, dysphormic extremities (syndactyly, brachydactyly or polydactyly), retinal dystrophy or pigmentary retinopathy, hypogonadism or hypogenitalism (limited to male patients) and kidney structural abnormalities or functional impairment. The expression and severity of the various clinical BBS features show inter‐ and intrafamilial variability. This study focuses on three cases of familial BBS – two sisters and one brother (66, 64 and 51 years of age, respectively) – with the main cardinal findings of the disease plus a classic ‘metabolic syndrome’ (characterized by abdominal obesity, atherogenic dyslipidaemia, raised blood pressure, insulin resistance with or without glucose intolerance, and prothrombotic risk and proinflammatory states). One female patient (not affected by reproductive dysfunction) had three healthy offspring, while the other two patients were unmarried. Another severely affected brother died at 70 years of age; two other brothers are lean but affected by nephropathy, retinopathy, slight mental retardation, polydactyly, hypertension and thrombotic diseases, and had healthy offspring. BBS is a rather rare but severe syndrome that is often mis‐ or undiagnosed. Ophthalmologists, endocrinologists and nephrologists should be aware of BBS because of its adverse prognosis – early onset of blindness, associated findings of metabolic syndrome and increased vascular risk, and severe renal impairment (the most frequent cause of reduced survival and death early in life).
Prevalence, risk factors and outcome of hyperlactataemia in HIV‐infected patients*
Authors: Hocqueloux L , Alberti C , Feugeas J‐P , Lafaurie M , Lukasiewicz E , Bagnard G , Carel O , Erlich D , Molina J‐M 
Published: March 2003

ObjectiveWe describe the prevalence, risk factors and outcome of hyperlactataemia (HL) in a cohort of 140 HIV‐infected patients.Patients and methodsPatients were enrolled consecutively within a 3‐month period (July to September 1999) and followed until 31 October 2000. One hundred and forty HIV‐infected patients had venous plasma lactate levels measured. HL was defined at baseline by two consecutive lactate levels > 2.1 mmol/L (upper limit of normal). We compared baseline demographic characteristics, immuno‐virological parameters, antiretroviral therapy and outcome between patients with HL (cases) or without HL (controls). We described the clinical features of patients with HL.ResultsAmong 129 patients included in the analysis, HL was found in 11 patients (8.5%), all of whom were receiving nucleoside reverse transcriptase inhibitors (NRTIs). Cases were more likely than controls to receive didanosine or stavudine (82% vs. 19%, P= 2.7 × 10−6 and 82% vs. 48%, P= 0.03, respectively). Only 4/11 cases (36%) had symptoms consistent with HL. After a median follow‐up of 15 months, lactate level returned to normal in all three patients who discontinued NRTIs, but in only 2/8 patients who did not (P = 0.06). Only one case experienced lactic acidosis and died during follow‐up. Mortality rate was similar in cases and controls.ConclusionHL is associated with NRTI use, in particular didanosine and stavudine, and discontinuation of NRTIs seems to be associated with rapid resolution of HL. Lactic acidosis remains rare and the long‐term outcome of patients with HL does not seem to be poorer than controls.
Familial mastocytosis associated with neurosensory deafness
Authors: Trevisan Giusto , Pauluzzi Paolo , Gatti Alessandro , Semeraro Argeo 
Published: March 2003

AbstractMastocytosis is a disease characterized by excessive accumulation of mast cells in different tissues and symptoms caused by the release of mast cell mediators. The skin is frequently directly involved in mastocytosis. The disease is rarely seen in other members of the subjects’ family; only 49 cases of familial mastocytosis have been reported. Familial mastocytosis associated with hearing loss may represent a newly described inherited entity. We describe a brother and sister exhibiting skin mastocytosis and neurosensory deafness, associated with a history of hearing loss in their father’s family. The appearance of the mast cell disease in two siblings, who presented with similar clinical features represents a familial form of mastocytosis; the association with an inherited form of deafness may constitute a new syndrome. Our patients show several features similar to some previously reported cases but different insofar that additional congenital defects and mental retardation are absent.
Effects of lithium carbonate (Li2CO3) on in‐vitro‐cultured normal human skin explants
Authors: Wolf Ronni , D’Avino Mariarosaria , De Angelis Francesca , Ruocco Eleonora , Lombardi Maria Luisa 
Published: March 2003

AbstractThe early morphological changes induced by lithium carbonate, a well‐known psoriasis‐provoking drug, were studied on cultured skin.Normal human skin from patients undergoing mastectomy was cultured in the presence of 3 m m, 6 m
m and 10 m
m of Li2CO3 for 4 days. The morphological changes were then evaluated by three observers in a blind manner and their reports were matched and collated.The cultured skin in the presence of Li2CO3 showed cell crowding of keratinocytes in the lower part of the epidermis, indicating epidermal hyperplasia. Another striking finding was intercellular oedema and vacuolar alteration with formation of small cavities in the upper dermis. There was no evidence of parakeratosis or any other histological characteristic of psoriasis, except hyperproliferation of the epidermis.Based on our knowledge of mechanisms of lithium action, we proposed two competitive explanations for its action on the epidermis: i) that lithium acts directly on dividing cells of the epidermis; and ii) that it acts indirectly by altering epidermal barrier function.Although we lack definite proof, we suggest that the observed morphological changes, in particular the non‐specific stimulus to epidermal proliferation, are the primary events which initiate the process that will ultimately lead to the development of psoriatis in a predisposed patient.
The role of HLA antigens in the development of paracoccidioidomycosis
Authors: Fernanda Reis Gavazzoni Dias Maria , Carlos Pereira Antônio , Pereira Angela , Alves Monica S R 
Published: March 2003

AbstractBackground Paracoccidioidomycosis is a systemic granulomatous disease that involves primarily the lungs and may disseminate to other organs and systems. It is caused by Paracoccidioides brasiliensis, a fungus that exhibits reversible thermal dimorphism and whose natural habitat is presently unknown. There are two main clinical forms: the acute (subacute) juvenile form and the chronic adult form. The former runs a more rapid course and is more severe than the latter. This mycosis is found throughout Latin America. Brazil accounts for 80% of reported cases. Presumably P. brasiliensis thrives in humid and hot places, especially near forests or farms. The infection is endemic in certain areas, especially in Brazil, Colombia and Venezuela, where nearly 100% of the population show cutaneous paracoccidioidina positive skin tests, indicating previous contact with the fungus, although a small percentage show clinical manifestations of the disease.Methods We compared the expression of HLA class I antigens in a healthy group (control) and in a group of patients with paracoccidioidomycosis (chronic adult form) using the Terasaki lymphocytotoxicity test modified by Amos for HLA antigen analysis.Aims To discover indications of whether or not individual susceptibility to P. brasiliensis might depend on some specific immunological defect.Results There is no evidence of association between a specific HLA antigen and paracoccidioidomycosis in the subjects studied. Further investigations are recommended.
Acute Crohn’s colitis with lobular panniculitis – metastatic Crohn’s?
Authors: Yosipovitch Gil , Hodak Emmilia , Feinmesser Maora , David Michael 
Published: March 2003

AbstractOne of the common lesions in Crohn’s colitis is erythema nodosum, a septal panniculitis that may appear before diagnosis or in conjunction with flare up. We report a case of Crohn’s colitis where the presenting sign was neutrophilic lobular panniculitis with few granulomas. The possibility that this case represents a forme fruste of metastatic Crohn’s is suggested. As skin biopsies are not usually performed by gastroenterologists in erythema nodosum‐like skin lesions in Crohn’s patients it may well be that the incidence of lobular panniculitis is higher than reported in the literature.
Circle hairs: a clinical curiosity
Authors: Contreras‐Ruiz J , Duran‐McKinster C , Tamayo‐Sanchez L , Orozco‐Covarrubias L , Ruiz‐Maldonado R 
Published: March 2003

AbstractA case of circle hairs is reported and the literature on the subject reviewed. Differential diagnoses are discussed as well as some theories on the nature of circle hairs.
Adjuvant botulinum toxin A in dyshidrotic hand eczema: a controlled prospective pilot study with left–right comparison
Authors: Wollina U , Karamfilov T 
Published: March 2003

AbstractObjective Dyshidrotic hand eczema is a therapeutic challenge. A prospective pilot study was performed with left–right comparison in order to investigate whether chemical de‐innervation of sudoriferic nerves would be superior to standard therapy with topical corticosteroids.Background Botulinum toxin A (BTXA) is a potent inhibitor of acetylcholine release, that induces eccrine sweat production and release. Inhibition of sweating by other measures such as tap water iontophoresis has been shown to be beneficial in dyshidrotic hand eczema.Methods Eight adult patients suffering from dyshidrotic hand eczema (atopic type) were included in a prospective, side‐by‐side controlled clinical pilot study using topical corticosteroids on both hands in combination with intracutaneous injections of 100 units of BTXA (Botox®) on the more severely affected hand on day 1. The dyshidrotic hand eczema was classified using the DASI (Dyshidrotic Eczema Area and Severity Index) before treatment (0), after 1 week, 4 weeks and 8 weeks.Results Six patients completed the study, two dropped out because of social and personal reasons. The mean DASI score changed from 28 to17 with topical therapy alone and from 36 to 3 with adjuvant BTXA (P < 0.01). Itching and vesiculation were inhibited earlier when using the combination of corticosteroids and BTXA. There was one relapse in the corticosteroid group. Relapses have not been seen in the BTXA group.Conclusions Interruption of sweating by BTXA improves the outcome and reduces relapses in patients with dyshidrotic hand eczema. BTXA is antipruritic as well suggesting that it does not only interact with acetylcholine release but substance P.
Acute generalized exanthematous pustulosis induced by ingestion of bamifylline
Authors: Galvão C , Criado Rfj , Criado Pr , Valente Nys , De Mello Jf , Fernandes Mfm 
Published: March 2003

ABSTRACTWe report a case of acute generalized exanthematous pustulosis (AGEP) in a 64‐year‐old woman, associated with the use of bamifylline. To the best of our knowledge there have been no previous reports of AGEP induced by the ingestion of bamifylline in the medical literature. We, therefore, add this drug to the list of causes for AGEP.
Phakomatosis pigmentovascularis and Lisch nodules. Relationship between Von Recklinghausen and phakomatosis pigmentovascularis?
Authors: Ruiz Villaverde R , Viera Ramirez A , Linares Solano J , Naranjo Sintes R , Gutierrez Salmerón MT 
Published: March 2003

ABSTRACTIn 1947, Ota described a malformative syndrome associating a vascular component (nevus flammeus) with melanocytic or epidermic nevi, which he named phakomatosis pigmentovascularis (PPV). We will discuss the case of a 10‐year‐old boy presenting giant nevus flammeus, nevus spillus, asymmetry in the development of both lower limbs, characteristic of PPV, together with interventricular communication and Lisch nodules, representative of Von Recklinghausen's disease. We believe that this case can be classified as PPV type IIb, associated with Lisch nodules and rarely described in Caucasian individuals.
The role of oxidants and antioxidants in psoriasis
Authors: Yildirim M , Inaloz HS , Baysal V , Delibas N 
Published: March 2003

ABSTRACTBackground Psoriasis vulgaris is a chronic inflammatory skin disease characterized by well‐demarcated erythema and scaly plaques. The pathogenesis of psoriasis still remains unclear. An increased reactive oxygen species (ROS) and insufficient antioxidant activity have been determined in psoriatic lesions.Aim of the study To evaluate and compare superoxide dismutase (SOD) and glutathione peroxidase (GP) activity in erythrocytes, catalase (CAT) activity and malondialdehyde (MDA) levels in serum of subjects with psoriasis and controls as well as MDA levels in skin biopsies from both groups.Study population Twenty‐two psoriatic patients (12 women and ten men) and 22 (12 women and ten men) healthy controls were involved in this study.Findings Statistically significant decreased levels of erythrocyte SOD and GP activities were noted in psoriatic subjects. Furthermore, a statistically significant increased serum CAT activity was found in the psoriasis group. No statistically significant difference was found in the serum MDA levels in the two groups, however, statistically significant increased tissue levels of MDA were noted in the psoriasis group.Conclusions Our results support the hypothesis of an imbalance in the oxidant–antioxidant system in psoriasis.
Forensic Risk Assessment in Intellectual Disabilities: The Evidence Base and Current Practice in One English Region
Authors: Turner Stephen 
Published: March 2003

AbstractThe growing interest in forensic risk assessment in intellectual disability services reflects the perception that deinstitutionalization has exposed more people to a greater risk of offending. However, ‘risk’ and the related idea of ‘dangerousness’ are problematic concepts because of connotations of dichotomous definition, stability and predictability. Assessment instruments in mainstream forensic psychiatry often combine actuarial and clinical data, and increasingly stress the dynamic nature of risk as well as the importance of situational and accidental triggers. Despite this increasing sophistication of research in mainstream forensic psychiatry, the ability to predict future offending behaviour remains very limited. Furthermore, actuarial predictors developed in studies of psychiatric or prison populations may not be valid for individuals with intellectual disabilities. Offending behaviour among people with intellectual disabilities is also hard to circumscribe because it often does not invoke full legal process or even reporting to the police. In order to discover how such problems were reflected in practice, a survey of providers in the North‐west Region of England was undertaken. Seventy out of 106 providers identified as possibly relevant to this inquiry responded to a short postal questionnaire. Twenty‐nine (42%) respondents – mainly in the statutory sector – reported operating a risk assessment policy relating to offending. The number of risk assessments completed in the previous year varied from none to ‘several hundred’. Providers reported three main kinds of problems: (1) resources or service configuration; (2) interagency or interdisciplinary cooperation or coordination; and (3) issues relating to the effectiveness, design and content of assessment.
Mortality and Life Expectancy in Dutch Residential Centres for Individuals with Intellectual Disability, 1991–1995
Authors: Maaskant Marian A. , Gevers Jan P. M. , Wierda Henk 
Published: March 2003

Background Several studies showed that the individuals with intellectual disability have a shorter life expectancy than their intellectually average peers. To gain insight in the present life expectancy of people with intellectual disability, a study with recent data was performed.Methods We used data of the National Case Register (LRZ) with data of almost all residents of residential centres in the Netherlands for the period 1991–1995 (N = 29 290). Approximately 40% of all people with intellectual disability in the Netherlands reside in such centres.Results Results showed that 5‐year‐old people with intellectual disability in Dutch residential centres have a life expectancy of 41 years. The life expectancy of their peers with Down's syndrome in the centres is 46. At the age of 30 years, the respective figures are 36 and 26 years. No statistically significant differences in mortality were found between women and men and between levels of intellectual disability.Conclusions Due to prolonged longevity, the client population in residential centres will continue to age and thus the numbers of older individuals will increase. This ageing process has implications for the care for elderly individuals, because they need other care than before due to physical, psychological and social changes and spiritual challenges.
The physical and psychomotor development of children conceived by IVF and exposed to high‐frequency vaginal ultrasonography (6.5 MHz) in the first trimester of pregnancy
Authors: Gershoni‐Baruch R. , Scher A. , Itskovitz J. , Thaler I. , Brandes J. M. 
Published: March 2003

AbstractWe examined the physical and psychomotor development of 33 infants conceived by in vitro fertilization (IVF) who were exposed to high‐frequency transvaginal ultrasonography (HFTVS) (6.5 MHz) in the first trimester of pregnancy. The study group was composed of 21 singletons and six sets of twins. Thirty‐three non‐IVF controls were matched for birth weight, gestational age, sex, birth order, order in multiple delivery, mode of delivery, age at examination and parental age and education. Forty‐five IVF infants who were not exposed to HFTVS and their matched controls also served as comparison groups. The Bayley scales were used to assess the psychosocial development of the infants studied.Increases in preterm delivery, low birth weight and Cesarean section rates were within the acceptable range for IVF babies. Mean birth weight, gestational age and birth weight percentiles of exposed IVF infants were lower than the expected mean for a normal population, but did not differ from that of non‐exposed IVF infants. Mean percentiles of weight and length at examination were also low but not different from those of non‐exposed IVF and matched control groups. However, percentiles of head circumference were normal at birth and at examination both in study and control groups. Overall, Bayley scores of exposed IVF infants were within the normal range and did not differ significantly from those of their matched controls and non‐exposed peers. These scores were significantly correlated to birth weight, percentiles of birth weight and head circumference at birth and at examination, and to the mother's education. Twins had lower physical and mental indices than singletons, but did not differ from their controls. Copyright © 1991 International Society of Ultrasound in Obstetrics and Gynecology