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1.

Background  

Obesity is a one of the most common nutritional disorder worldwide, clearly associated with the metabolic syndrome, condition with implications for the development of many chronic diseases.  相似文献   

2.

Background  

The relation between fetal growth retardation and cardiovascular and metabolic diseases in later life has been demonstrated in many studies. However, debate exists around the potential independent role of postnatal growth acceleration. Furthermore, it is unknown whether a potential effect of growth acceleration on cardiovascular and metabolic function is confined to certain timeframes.  相似文献   

3.

Background  

Obesity and metabolic syndrome are strongly associated with type 2 diabetes mellitus and cardiovascular diseases, thus the increasing trend in their prevalence among children and adolescents from developing countries requires a further understanding of their epidemiology and determinants.  相似文献   

4.

Background  

Mucopolysaccharidoses are heritable, metabolic diseases caused by deficiency in an activity of one of specific lysosomal enzymes involved in degradation of mucoplysaccharides (glycosaminoglycans). Among many medical problems of patients with mucopolysaccharidoses, there are frequent episodes of diarrhea of unknown etiology.  相似文献   

5.

Objective  

Surgical repair of pectus excavatum (PE) in childhood is a well-established procedure. Previously used operative techniques to correct PE were largely based on the Ravitch technique. Since about 10 years, the minimally invasive repair (MIRPE) by Nuss is well established. Conservative treatment with the vacuum bell to elevate the funnel in patients with PE represents a potential alternative to surgery in selected patients.  相似文献   

6.

Background  

The portoenterostomy (PE) procedure for treating biliary atresia (BA) has been so repeatedly modified that it currently hardly resembles Kasai’s original PE (KOPE). Now PE involves an extended lateral dissection and a wide anastomosis (extended PE: EPE). We reappraised KOPE and created our-KOPE (OKOPE) by adhering strictly to its principles and techniques. We compared outcome of EPE and OKOPE.  相似文献   

7.

Background  

Prior studies in adults suggest that a wedge-shaped peripheral consolidation may be predictive of pulmonary embolism (PE). In contrast, a previous study in children provided no evidence of an association between this finding and PE, but it was limited by a small sample size and was not specifically designed to answer this question.  相似文献   

8.

Background  

This study was to investigate the advantages of thoracoscopy-assisted minimally invasive Nuss operation for the treatment of pectus excavatum (PE) in children.  相似文献   

9.

Background  

Children with progressive metabolic, neurological, or chromosomal conditions and their families anticipate an unknown lifespan, endure unstable and often painful symptoms, and cope with erratic emotional and spiritual crises as the condition progresses along an uncertain trajectory towards death. Much is known about the genetics and pathophysiology of these diseases, but very little has been documented about the trajectory of symptoms for children with these conditions or the associated experience of their families. A longitudinal study design will help to close this gap in knowledge.  相似文献   

10.

Background

Gestational diabetes and preeclampsia are leading causes of complications during pregnancy.

Aims

The aims of this study were to quantify the probability that both diseases occur together, to evaluate commonality of risk factor profiles, and to clarify the connection between gestational diabetes and preeclampsia in combination with the maternal body mass index.

Study design

We analysed data of the German Perinatal Quality Registry 2006, an annual full inventory of all hospital births in Germany.

Subjects

The Registry contains the complete national birth cohort of 668,085 newborn infants and 647,392 mothers from 896 German obstetric clinics.

Outcome measures

Each case of gestational diabetes or preeclampsia that was identified during pregnancy by a gynaecologist or in the hospital was fully registered.

Results

The prevalence of GDM was 2.32% and that of PE was 2.31%, resulting in 0.09% of all pregnant women being diagnosed with both diseases. GDM was found to be an independent risk factor for PE. Increased maternal age, nulliparity, and multiple gestation pregnancies could be identified as common risk factors for both diseases, while increased pre-pregnancy body mass index was found to be the most important predictor for both diseases.

Conclusions

As PE and GDM share similar risk factors, identification of high-risk groups by simultaneous screening methods seems to be reasonable for prevention of complications. Further studies will be needed to investigate possible pathophysiological pathways increased body mass index has on the induction of both diseases.  相似文献   

11.

Objectives  

To evaluate the cardiovascular and endocrine effects of childhood obesity as well as prevalence of metabolic syndrome associated with it.  相似文献   

12.

Background  

The Nuss procedure for repair of pectus excavatum (PE) has been accepted worldwide because of minimal invasiveness and excellent cosmetic results. We summarized our experience with the treatment of 115 patients aged 2.7–18 years.  相似文献   

13.

Purpose  

Test the diagnostic reliability of the score for neonatal acute physiology-perinatal extension-II (SNAPPE-II) and the metabolic derangement acuity score (MDAS) as predictors of surgery in patients with necrotizing enterocolitis (NEC).  相似文献   

14.

Objective  

To assess whether apoB/apoA1 ratio is associated or not with metabolic syndrome in obese children.  相似文献   

15.

Introduction  

Children after craniopharyngioma surgery often develop rapid weight gain and hyperphagia. We investigate the metabolic syndrome features, risk factors, and the insulin dynamics in these patients.  相似文献   

16.

Objectives  

To study the effects of early parenteral amino acid administration on body weight, fluid compartments and metabolic parameters during the first week of life in sick premature infants.  相似文献   

17.

Aim

The decrease in childhood mortality, the growing clinical complexity and the greater technification of intensive care units have changed the circumstances of death of paediatric patients. The aim of this study is to describe the context of death and end-of-life decision-making.

Methods

Single-centre, retrospective, observational study of deaths in inpatients or home hospitalised children under 18 years old between 2011 and 2021. Demographic data, pathological history and circumstances of death were obtained from the medical record. The whole study period was divided into two halves for the analysis of the temporal trends.

Results

A total of 358 patients died, 63.2% under the age of 1 year old; 86.9% had underlying life-limiting illnesses and 73.2% died in the intensive care unit, with no differences between the two time periods. Death at home was significantly higher in the second study period (3.8% vs. 9%). A total of 20.1% died during advanced cardiopulmonary resuscitation. Life-sustaining treatment was withheld or withdrawn in 53.6%, with no differences between the time courses. Life-sustaining treatment was withheld mainly in patients with neurological, metabolic and oncological conditions, and less frequently in patients with cardiovascular or respiratory diseases or who were previously healthy. Most patients coded as palliative care (PC) or followed up by PC teams had an advance care plan (ACP) recorded, while in the others it was infrequent. PC coding, following by PC teams and ACP recording increased in the last years of the study.

Conclusions

Death of children in our setting usually occurs in relation to complex underlying pathology and after the decision of withdrawing or withholding life-sustaining treatment. In this context, PC and ACP acquire greater importance. In our study, PC involvement resulted in better documentation of ACP and PC coding.  相似文献   

18.

Introduction

Haematopoietic stem cell transplantation (HSCT) involves implanting cellular elements capable of generating a new and healthy haematopoietic system. Reduced intensity conditioning (RIC) consists of an immunosuppressive treatment to facilitate a progressive implant with lower morbidity. This type of conditioning can also lead to myelosuppression, which is potentially reversible over time.Reduced intensity conditioning enables HSCT to be performed on patients with genetic diseases for whom added comorbidity is undesirable due to the high doses of chemotherapy that accompanies conventional myeloablative regimens.

Patients and methods

An analysis was performed on the outcomes of 68 paediatric patients with genetic diseases who underwent HSCT with RIC between 2005 and 2013 in the of Paediatric Haematopoietic Stem Cell Transplantation Units that are part of the Spanish Working Group for Bone Marrow Transplantation in Children.A multicentre study was conducted including 68 patients, of whom 43 had Primary Immunodeficiency, 21 with congenital haematological diseases, and 4 with metabolic diseases.

Results

Fifty (73.5%) of the 68 patients were still alive. The Overall Survival (OS) at nine years was 0.74. Twenty-three (33.8%) had some event during the course of the HSCT, with an event-free survival rate of 0.66. The OS in patients with haematological diseases was 0.81, being 0.7 in primary immunodeficiencies, and 0.4 in metabolic diseases. No significant difference was observed between the 3 groups of diseases. As regards the source of haematopoietic progenitors, there was an OS rate of 0.74 in patients transplanted with peripheral blood, 0.70 with bone marrow, and 0.70 and with cord blood, with no statistically significant differences.

Conclusions

Favourable results have been obtained in HSCT with reduced intensity conditioning in genetic diseases. It should be noted that the risks and benefits of the RIC in patients with metabolic diseases need to be assessed on an individual basis.  相似文献   

19.

Background  

There are still few reports on thoracoscopic lobectomy in neonates. The rate of prenatally diagnosed congenital cystic lung diseases is increasing, and such diseases appear to be a good indication for thoracoscopic lobectomy.  相似文献   

20.

Objectives  

To describe pneumococcal diseases in pediatric intensive care unit (PICU) and non-PICU patients.  相似文献   

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