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??Objective??To detect the level of fecal primary and secondary bile acids in infants with infantile cholestatic hepatopathy??ICH??and analyze its clinical value. Methods??Thirty infants with ICH were enrolled in this study??who were diagnosed with infantile cholestatic hepatopathy. Thirty infants with good health condition were enrolled as the healthy control group. The fecal samples were collected respectively in the preparatory treatment phase and treatment phase from infants with ICH and from the healthy infants. Bile acids were extracted from infants’ feces and were quantitatively analyzed by liquid chromatography-mass spectroscopy. Results??Among the fecal primary bile acids??the level of cholic acid??chenodeoxycholic and glycochenodeoxycholic acid both in the ICH preparatory treatment group and ICH treatment group was significantly lower than that in the healthy control group??P??0.016??.The level of fecal cholic acid and chenodeoxycholic acid of ICH treatment group was higher than in the ICH preparatory treatment group??P??0.016??. Among the fecal secondary bile acids??the level of lithocholic acid both in the ICH preparatory treatment group and ICH treatment group was significantly lower than that in the healthy control group??P??0.016????and the level of ursodeoxycholic acid in the ICH preparatory treatment group was lower than that in the ICH treatment group and healthy control group??P??0.016??. Conclusion??In infants with ICH, the changes of fecal primary bile acids and fecal secondary bile acids have their own characteristics at the early stage of treatment, which may be caused by the short-term treatment, the prognosis of the disease itself and the changes of intestinal function, including intestinal bacteria. Clinical attention should be paid to these changes.     

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??Abstract?? The interstitial lung disease ??ILD?? in infants has been better diagnosed with the application of high-resolution CT in recent years. Mutiple factors play important roles in the pathogenesis and development of ILD in infants?? especially the infection. Moreover?? the infants with ILD are also prone to infection which could further aggravate their ILD. So a correct understanding of the relationship between infection and ILD in infants is significantly important.     

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??Abstract??Though interstitial lung disease ??ILD?? can occur at any age in children?? disorders more common in infancy and young children have received increased attention as an important group that is disproportionally affected?? linked to lung development and lung injury?? and represents disorders not seen in adult ILD. The specific disorders causesd by alveolar growth abnormalities??AGA?? is the most common in infancy. The presentation?? evaluation?? treatment?? and clinical course in infants are discussed in infants in this paper.     

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间质性肺疾病(intertitial lung diseaseILD)是一大类在临床(氧合障碍)-影像(弥漫性病变征象)-病理(炎症和纤维化)上具有相似特征,但病因不同的异质性疾病的总称。本文主要讨论儿童ILD中特发性间质性肺炎(idiopathic interstitial pneumonia,IIP)和特发性肺含铁血黄素沉着     

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??Abstract??The literature is becoming progressively populated by reports of diffuse parenchymal lung disease/interstitial lung disease??DPLD/ ILD?? that appear distinct to infants and young children. The classification?? clinical features?? management and outcome for these disorders are briefly mentioned.     

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目的探讨细胞外基质在早产儿慢性肺疾病(CLD)支气管肺泡灌洗液(BALF)中动态改变及其在CLD形成中的作用。方法对1999-12—2004-11在华中科技大学同济医院NICU住院需机械通气的62例患儿每日行支气管肺泡灌洗,并检测灌洗液中细胞总数及分类、透明质酸(HA)、Ⅲ型胶原(PCⅢ)及羟脯氨酸(Hyp)水平。病例分为4组:肺炎组20例;肺透明膜病(HMD)组13例;CLD组15例;对照组14例(无肺部疾病者)。结果(1)CLD组BALF中HA和PCⅢ水平分别较肺炎组及HMD组明显升高,并且也分别高于对照组BALF中的水平;(2)CLD组BALF中的HA和Hyp均分别与细胞总数和中性粒细胞数(%)呈正相关,而PCⅢ与肺泡巨噬细胞总数呈正相关。另外,HA、PCⅢ及Hyp之间均呈明显的正相关。结论CLD患儿肺内HA、PCⅢ及Hyp产生增多;BALF中HA、PCⅢ及Hyp的水平从不同侧面反映了疾病活动性并具有估计预后的作用。     

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??Abstract??Interstitial lung disease ??ILD?? is a group of widely heterogeneous etiology disease. Infant and early childhood special type of ILD belongs to a more complex etiology category. Connective tissue disease ??CTD?? is a group of autoimmune disease involves multi-systems and organs in the whole body?? lung is the organ most commonly involved. Connective tissue disease-associated interstitial lung disease ??CTD-ILD?? and ILD have similar clinical symptoms?? with their own characteristics?? but different treatment options and prognosis. Deeply understanding the infant CTD-ILD and ILD?? learning their clinical analysis ideas?? will become very important in carrying out extensive clinical research for prognosis and therapy in the future.     

Chronic kidney disease in children following lung and heart–lung transplantation

Abstract:  CKD is a major co-morbidity in pediatric lung transplant recipients. We report the prevalence of renal impairment post-lung transplant at a single center, using a modified, age-adjusted eGFR for the best approximation of true GFR, and investigated associations and possible predictors of decline in renal function post-transplant. Renal function was assessed by eGFR pre-transplant, three and 12 months post-transplant, and at last follow-up. Decline in renal function was analyzed as percentage fall in eGFR in two phases (0–3 and 3–12). Furthermore, we investigated impact of gender, age, pre-transplant diagnosis and renal function, transplant type, early post-transplant dialysis, and tacrolimus trough levels on decline in eGFR using multivariate analysis. Over a five-yr period, 30 transplants were performed. Mean eGFR pretransplant was 117 mL/min/1.73 m² (s.d. 35) with mean decline in eGFR during the first three months post-transplant of 33% (s.d. 31, p < 0.001). Thereafter, mean decline in eGFR was 8% (s.d. 18, p = 0.02). None of the factors assessed were significantly associated with decline in eGFR post-transplant. In conclusion, many children have decline in renal function following lung transplantation, particularly early post-transplant. Unlike in adults, we were unable to detect any predictors of renal impairment in pediatric lung transplant recipients.     

Chronic lung disease in very low birthweight infants: A prospective population-based study

A prospective population-based study of chronic lung disease among all very low birthweight infants (birthweight 500-1499 g) born in New Zealand in 1986 is reported. Of 413 of these infants admitted to neonatal units, 355 (86%) survived to 28 days. An additional 50 infants were recorded as liveborn but died in the labour ward or other place of birth. Both observed survival and survival adjusted for birthweight, gestation and gender were significantly (P less than 0.05) better in larger centres. Oxygen requirement was assessed at 28 days of age, 36 weeks equivalent gestation and 84 days of age, when 38.6, 23.1 and 13.8% of infants, respectively, were being treated with oxygen. To examine the joint effects of predictor variables on oxygen requirement at each age, the data were analysed using multiple logistic regression methods. At 28 days, lower birthweight, shorter gestation, respiratory distress syndrome (all P less than 0.0001), and gender and hospital principally caring for the infant (both P less than 0.05) were significantly associated with treatment with oxygen. In comparison with other studies, New Zealand appears to have a relatively high rate of chronic lung disease. We speculate that a contributing factor may be the small size of some regional neonatal units.     

儿童间质性肺疾病的分类和诊断程序

儿童间质性肺疾病是一组庞大而异质性的肺疾病,以弥漫性渗出和气体交换障碍为特征.儿童间质性肺疾病的分类主要为:婴儿特有的间质性肺疾病;原发于肺部的间质性肺疾病,包括特发性间质性肺炎;伴肺部浸润的系统性疾病;已知原因的疾病.儿童间质性肺疾病诊断应先根据病史、临床表现以及影像学检查,确定是否为间质性肺疾病;进一步通过病原学、血清学检查等寻找病因,如病因仍不明确则需通过侵袭性检查获得诊断和病理分型.     

Striving for perfection,accepting the reality: A reflection on adherence to airway clearance and inhalation therapy for paediatric patients with chronic suppurative lung disease

Non-adherence to prescribed treatment is considered the foremost cause of treatment failure in chronic medical conditions. Airway clearance techniques (ACT) play a key role in the management of chronic suppurative lung disease yet, along with inhaled therapies such as nebulised antibiotics, adherence to these is often lower than to other treatments. In this review we discuss methods of monitoring adherence to these therapies and potential barriers and outline suggestions for improving adherence in the paediatric population.     

Chronic lung disease in very low birthweight infants: A 5-year review

To determine the incidence, clinical spectrum and outcome of very low birthweight (VLBW) infants with chronic lung disease (CLD), and evaluate associated factors.

Methodology:

Retrospective review of 265 VLBW infants managed in the NICU from January 1988 to December 1992.

Results:

The overall neonatal survival rate for VLBW infants was 83%. Sixty-five (25%) infants had CLD, of whom 42% had severe CLD. Mortality in infants with CLD was 11%. In contrast with infants without CLD, CLD infants had significantly higher risk of adverse neurodevelopment with cerebral palsy documented in 13.5% and functional disability recorded in 34.6% at 2 years corrected age. Factors associated with the development of CLD included; decreasing birthweight (OR 0.98, Cl 0.97–0.99), septicaemia (OR 4.96, Cl 1.57–15.65), necrotizing enterocolitis (OR 119.07, Cl 4.98–2845.04), hyaline membrane disease (OR 5.34, Cl 1.83–15.55), patent ductus arteriosus (OR 4.46, CI 1.75–11.36) and increasing fraction of inspired oxygen concentration in the first week of life (OR 1.09, Cl 1.04–1.14).

Conclusions:

Chronic lung disease occurs frequently in VLBW infants and is associated with a high incidence of adverse neurodevelopment. Further studies to clarify the role of non-respiratory factors such as patent ductus arteriosus (PDA) and sepsis in the pathogenesis of CLD may reduce the incidence or prevent the development of CLD in these preterm infants.     

Chronic lung disease of prematurity and respiratory outcome at eight years of age

AIM: The study aimed to determine the respiratory outcome of children who had chronic lung disease of prematurity (CLD) compared with a preterm control group of children at school age. METHODS: Fifty-two preterm infants with CLD born between 26 and 33 weeks gestation were assessed regarding respiratory illness with 47 having lung function testing. Information regarding respiratory illness was obtained from 52 children in the birthweight-matched control group of whom 45 had lung function testing. The results were compared between the CLD and control groups. RESULTS: There was no difference in respiratory symptomatology between CLD groups and control preterm infants. On lung function testing, a significantly lower mean forced expiratory flow at 25-75% of vital capacity was identified compared with the preterm controls (P=0.024). This significant difference did not persist after bronchodilator therapy. There was no evidence of increased air trapping or bronchial hyper-reactivity in the CLD children compared with the controls. CONCLUSION: Lung function in CLD children is largely normal in comparison with preterm controls, apart from some evidence of reversible small airway obstruction. Respiratory symptomatology is not increased in chronic disease children in comparison with control preterm children.     

儿童慢性肾脏病管理

2006年国际肾脏病学会(international society of ne-phrology,ISN)和国际肾脏基金联合会(international federa-tion of kidney foundation,IFKF)联合倡议,将每年3月份的第2个星期四定为世界肾脏日,目的是唤起全球各界人士对慢性肾脏病(chronic kidney disease,CKD)的高度关注。目前,儿童CKD的概念也已经得到较广泛的重视。根     

Acute suppurative thyroiditis in young children

We report two children with acute suppurative thyroiditis (AST). They presented with typical features of AST, which include fever, painful goiter and biochemical euthyroidism. An anatomical defect predisposed to thyroid infection, pyriform sinus fistula, was identified in one patient. Both patients responded well to surgical pus drainage and antibiotic treatment. Anatomical defects must be sought in all children with AST to perform specific surgical treatment and prevent recurrent infection.