ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1996.tb14269.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

This review is based on longitudinal studies on our seven patients with congenital generalized lipodystrophy, our patient with acquired generalized lipodystrophy, and published papers on these subjects. An inability to store energy in adipose tissue is of pathogenetic importance. In congenital lipodystrophy, insulin resistance is present from birth, resulting in hyperinsulinaemia, dyslipidaemia, and insulin‐resistant diabetes with an anabolic syndrome worsened by a voracious appetite. Clinically, we observed increased height velocity in pre‐school age children, and organomegaly with hypertrophic cardiomyopathy, which seems to be lethal in early adulthood: three of our patients died at the ages of 24,32 and 37 years. The oldest alive, 39 years, suffers from stenocardia. Regarding treatment, it is most important to reduce energy consumption. The congenital form is recessively inherited. The aetiology may be related to insulin receptor or postreceptor mechanisms. Acquired generalized lipodystrophy seems to be an autoimmune disorder with secondary destruction of the adipose organ; the anabolic syndrome with insulin‐resistant diabetes is secondary. Our patient died when 24 years old from pneu

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1996.tb14262.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

The structural basis for the functions of the brain is the wiring of nerve cells. This wiring is achieved by 100 billion neurons via some 100,000 billion synaptic connections. Formation of this network is a formidable task which requires intricate, but also reliable mechanisms at work. It is generally recognized that the precise connections between nerve cells are accomplished by two different sorts of mechanism. First, various molecular guidance cues lead outgrowing axons from specific areas to particular target regions which are rather broadly defined. The second stage includes an exact, point‐to‐point matching between each axon and the specific target neurons. This is achieved by an intricate interaction between the involved nerve cells. In the following, the attention will primarily regard the second type of events, but some aspects on the initial outgrowth of axons and axon guidance mechanisms will also be given. A large part of the description will focus on the neuromuscular junction, since it has been extensively used as a model for the study of synapse developm

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1996.tb14270.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1996.tb13881.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1996.tb14283.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

The present report describes psychobiological studies of behavior around the time of birth. An adaptive, ecological perspective is presented in which stimulation of the fetus and newborn is purported to instigate adaptive postpartum behavior. Studies describing the perinatal sensory environment are reviewed, with a consideration of emergent sensory function of the fetus. It is asserted that afferent input associated with parturition perturbs the fetus and neonate, producing a general arousal state that facilitates breathing, suckling, and early learning. The view developed herein is that perinatal sensory input induces and canalizes the newborn's behavior, thereby regulating adaptive postpartum function. Deviations in afferent input may alter ontogenetic trajectories and compromise developmental outcome by reducing availability of conditions necessary for adequate postpartum adaptation.

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1996.tb14271.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Under the umbrella of coeliac disease (CD), or gluten‐sensitive enteropathy, the concepts of silent, latent and potential CD have recently been introduced. While silent CD is marked by severe damage to the jejunal mucosa in the absence of clinical symptoms, both latent and potential CD are characterized by a jejunal mucosa that would be reported as normal by most clinical pathologists in an individual on a gluten‐containing diet. As opposed to potential coeliac patients, latent subjects sometime in their life have had a flat jejunal biopsy which recovered on a gluten‐free diet. Latent coeliac patients are often symptomatic; neither high titres of gliadin antibodies nor mucosal changes (including raised intraepithelial lymphocyte counts) are obligate features of latent CD, although the presence of elevated endomysial antibodies is probably the best predictor of progression towards villous atrophy. The term potential CD has been proposed for those subjects who do not have, and have never had, a jejunal biopsy consistent with overt CD, and yet have immunological abnormalities similar to those found in coeliac patients. Good markers of potential CD include the presence of serum endomysial antibodies, a high count of intraepithelial lymphocytes and subtle pathological alterations such as increased density of intraepithelial lymphocytes expressing γδ T cell receptor, signs of activated mucosal cell‐mediated immunity, coeliac‐like intestinal antibody pattern, and positive rectal glute

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1996.tb14240.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1996.tb14285.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Several recent studies, in particular the Italian SIGEP study, have demonstrated that mass screening for coeliac disease using gliadin and endomysial antibody testing is now a realistic possibility. If mass screening is to receive serious consideration then it must be shown to be (i) effective, (ii) acceptable, and (iii) worthwhile (cost‐effective). At present there is insufficient evidence as to the health benefits from treating screen‐detected disease and as to the size of the risks associated with having undetected coeliac disease. It is also unclear whether truly asymptomatic screen‐detected coeliacs will accept long‐term dietary restriction. To answer these questions randomized trials of screening are needed. On current evidence, mass screening cannot be justified. Efficient case‐finding may prove as effective and more a

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1996.tb14241.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Whether fetal memory exists has attracted interest for many thousands of years. The following review draws on recent experimental evidence to consider two questions: does the fetus have a memory? And, if so, what function(s) does it serve? Evidence from fetal learning paradigms of classical conditioning, habituation and exposure learning reveal that the fetus does have a memory. By comparison little attention has been paid to the possible function of memory. Possible functions discussed are: practice, recognition of and attachment to the mother, promotion of breastfeeding, and language acquisition. It is concluded that the fetus does possess a memory but that more attention to the functions of fetal memory will guide future studies of fetal memory abilities.

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1996.tb14272.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY