原发性遗尿症儿童工作记忆缺陷的事件相关电位研究

目的 通过事件相关电位（ERP）探讨原发性遗尿症儿童工作记忆障碍的认知神经机制。方法 纳入原发性遗尿症（遗尿症组）与正常儿童（对照组）各14例,采用学习-再认任务测试,对两组儿童识别旧图（学习过的）及新图（未学习过的）时Fz导联ERP成分P2、N2、P3进行比较分析。结果 遗尿症组识别旧图时P2、N2波幅明显低于对照组（P < 0.05）;而两组识别旧图时P2、N2、P3潜伏期及P3波幅差异均无统计学意义。遗尿症组识别新图时P2潜伏期较对照组明显延长（P < 0.05）,N2波幅明显低于对照组（P < 0.05）;而两组识别新图时P2、P3波幅及N2、P3潜伏期差异均无统计学意义。结论 原发性遗尿症儿童比正常儿童记忆分类信息提取能力下降及反应时延长,记忆容量负荷减少、记忆巩固减弱及冲突监测能力下降,导致工作记忆障碍。     

Cognitive P300‐evoked potentials in school‐age children after surgical or transcatheter intervention for ventricular septal defect

Background: Some studies have suggested that neurological development may be adversely affected in children with severe coronary heart disease who have undergone long periods of deep hypothermic cardiopulmonary bypass (CPB). Reports of cognitive function in VSD patients in whom surgical repair required only a relatively brief period of CPB are rare. Also, CPB is unnecessary for VSD patients undergoing transcatheter closure. The aim of this study was to assess the cognitive function in patients with ventricular septal defect. Methods: A total of 29 patients treated with surgery, and 35 treated with transcatheter closure and their age‐ and sex‐matched best friends completed the cognitive P300 auditory‐evoked potentials test and the intelligence test. Results: The patients and their best friends had normal intelligence quotient; however, the patients had longer P300 peak latencies in cranial frontal lobe and cranial vertex leads (329.2 ± 24.8 and 335.1 ± 20.0 ms) than the healthy controls did (319.1 ± 20.6 and 313 ± 18.2 ms) (P < 0.05). Patients who underwent surgery had longer P300 peak latency in the cranial frontal lobe and cranial vertex leads than did those with transcatheter closure and controls. When cardiopulmonary bypass and aortic clamping were used, the duration was associated with P300 peak latency for patients (P < 0.05). Conclusion: VSD patients, especially those undergoing surgery, showed poor cognitive function, which may be associated with duration of cardiopulmonary bypass or aortic‐clamping.     

Thrombomodulin serum levels in ventilated preterm babies with respiratory distress syndrome

A soluble form of thrombomodulin (TM), an anticoagulant proteoglycan of the endothelial cell membrane, considered a marker of vascular endothelial damage, was measured in plasma of preterm infants with respiratory distress syndrome (RDS). In these patients, lung immaturity leads to endothelial leak of plasma proteins and to surfactant inhibition. In 18 babies with RDS, plasma TM concentration was significantly elevated compared with values of a matched group of babies without pulmonary disease (276.1 ng/ml vs 141.3 ng/ml) (P<0.05). Furthermore, TM levels of mechanical ventilated babies (IPPV) with severe RDS were higher than those of babies with moderate RDS and treated with nasal CPAP (340.9 ng/ml vs 174.2 ng/ml) (P<0.05). Conclusion These data show that TM can be used as marker of pulmonary endothelial damage in preterm babies treated with mechanical ventilation for RDS and suggest early intervention with exogenous surfactant to limit alveolar protein leakage and surfactant inactivation. Received: 20 February 1997 and in revised form: 7 July 1997 / Accepted: 8 July 1997     

A neurophysiological marker of impaired preparation in an 11‐year follow‐up study of attention‐deficit/hyperactivity disorder (ADHD)

Background: This longitudinal electrophysiological study investigated the course of multiple impaired cognitive brain functions in attention‐deficit/hyperactivity disorder (ADHD) from childhood to adulthood by comparing developmental trajectories of individuals with ADHD and typically developing controls. Methods: Subjects with ADHD (N = 11) and normal controls (N = 12) diagnosed in childhood [mean age ADHD/CTRL = 10.9 years [SD 1.72]/10.0 years (SD 1.03)] were followed up after 1.1 and 2.4 years, and as young adults [ADHD/CTRL: 21.9 years (SD 1.46)/21.1 years (SD 1.29)]. At all four times, event‐related potential (ERP) maps were recorded during a cued continuous performance test (CPT). We focused on residual deficits as adults, and on developmental trajectories (time and time × group effects) for CPT performance and attentional (Cue P300), preparatory (CNV: contingent negative variation) and inhibitory (NoGo P300) ERP components. Results: All ERP components developed without significant time × group interactions. Only the CNV remained reduced in the ADHD group, although 8/11 individuals no longer met a full ADHD diagnosis as adults. Cue P300 and NoGo P300 group differences became nonsignificant in early adulthood. The CNV parameters correlated with reaction time (RT) and RT‐SD. Perceptual sensitivity improved and the groups’ trajectories converged with development, while RT‐SD continued to be elevated in adult ADHD subjects. Conclusions: Attentional and preparatory deficits in ADHD continue into adulthood, and the attenuated CNV appears to reflect a particularly stable ADHD marker. Although some deficit reductions may have gone undetected due to small sample size, the findings challenge those developmental lag models postulating that most ADHD‐related deficits become negligible with brain maturation.     

Thyroid functions in mild and severe forms of sickle cell anemia

In the present study, the weight, height, bone age and growth indices of 24 children with homozygote sickle cell anemia were measured and the relationship of these parameters to thyroid function was evaluated and compared with 14 healthy controls in the same age group. The patients consisted of two groups with either mild (n = 12) or severe (n = 12) clinical courses. There was no difference between both patient groups or with the control group with respect to weight (P > 0.05). However, the difference between the mean height percentiles of the homozygote-severe group and the control group was found to be statistically significant (P < 0.05). The bone age remained 41.6% behind normal for age in all homozygote sickle cell anemia patients. The serum T3 and T4 levels of all patients showed no significant differences from those of the control group (P > 0.05). These results show that patients with severe clinical courses may have short stature but their thyroid hormones are within normal limits during the first decade of life.     

Tissue damage in rat ovaries subjected to torsion and detorsion: effects of <Emphasis Type="SmallCaps">l</Emphasis>-carnitine and <Emphasis Type="Italic">N</Emphasis>-acetyl cysteine

We aimed to evaluate histopathological changes, to detect HIF-1α staining intensities and to determine MDA levels in rat ovaries, which were subjected to torsion and detorsion and treated with l-carnitine or N-acetyl cysteine (NAC). Forty-eight prepubertal female Sprague–Dawley rats were divided into five groups (n = 8): 1, control; 2, ischemia; 3, reperfusion; 4, l-carnitine; and 5, NAC groups. In groups 3, 4 and 5, an ischemic period of 3 h was followed by reperfusion for 24 h. In groups 4 and 5, ischemia was performed and either l-carnitine or NAC was infused intraperitoneally 30 min before reperfusion. Ovarian tissues were examined histopathologically; tissue MDA levels and serum IL-6 levels were determined biochemically. HIF-1α was applied to all ovaries immunohistochemically. Total tissue damage scores, tissue MDA levels and HIF-1α scores, were significantly higher in group 2 (all P < 0.001) than group 4, and group 3 than group 4 (P < 0.001, P = 0.05 and P < 0.001, respectively). They were also significantly higher in group 2 (all P < 0.001) than group 5. When group 3 is compared to group 5, total tissue damage scores and tissue MDA levels were significantly higher in the former (P < 0.01 and P < 0.001, respectively). Serum IL-6 levels were significantly higher in group 2 when compared to groups 1, 4 and 5 (all P < 0.01). The degree of tissue damage of the torsioned ovaries decreased after a reperfusion period of 24 h in the torsioned ovaries. However, ovaries of both l-carnitine and NAC groups showed better recovery than the reperfusion group. This study was accepted for poster presentation in the 21st European Congress of Pathology, held in Istanbul, Turkey, on 8–13 September 2007.     

布地奈德雾化治疗对哮喘小鼠糖皮质激素受体及核因子-κB表达的影响

目的观察布地奈德雾化吸入对哮喘小鼠糖皮质激素受体(GR)和核因子-κB(NF-κB)表达的影响。方法 24只健康6~8周龄雄性BALB/c小鼠随机分为生理盐水对照组、哮喘模型组(哮喘组)和布地奈德治疗组(BUD组),每组8只。通过腹腔注射卵清蛋白和氢氧化铝混悬液致敏以及卵清蛋白溶液雾化吸入激发哮喘。末次激发24 h后处死小鼠,取支气管肺泡灌洗液(BALF)进行嗜酸性粒细胞计数,取肺组织行病理学检查及免疫组化检测GR和NF-κB表达水平。结果哮喘组BALF中嗜酸性粒细胞计数较对照组显著增加(P0.05);BUD组与哮喘组相比嗜酸性粒细胞数量显著减少,但仍较对照组增高(P0.05)。哮喘组小鼠肺组织可见嗜酸性粒细胞、淋巴细胞等炎症细胞浸润,BUD组炎症细胞浸润较哮喘组明显减轻。哮喘组GR阳性细胞百分比与对照组比较明显减少(P0.05),BUD组GR阳性细胞百分比较哮喘组明显增加,但仍低于对照组(P0.05);哮喘组NF-κB阳性细胞百分比较对照组明显增加(P0.05),BUD组NF-κB阳性细胞百分比较哮喘组明显减少,但仍高于对照组(P0.05)。结论 BUD治疗哮喘小鼠的作用机制可能与上调GR水平及抑制NF-κB的活性有关。     

Psychosocial aspects and psychiatric disorders in children with thalassemia major

β-thalassemia major (TM), a chronic, genetically determined hematological disorder, has received little investigation on the psychological aspects of the disease and the psychosocial adjustment of patients with this anemia. In the present study, the aim was to assess the mental capacity, self-image, hopelessness and anxiety displayed by children who suffered from TM, and to investigate the existence of psychiatric disorders in these children. Twenty-five children (16 boys and 9 girls) with TM, 12.0–19.6 years old, from the Hematology Unit of the Department of Pediatrics at the SSK Tepecik Teaching Hospital, were included in the study. Fifteen healthy cases matched for age, sex and socio-economic status were used as controls. The Wechsler Intelligence Scale for Children (or Wechsler Adult Intelligence Scale), Offer Self-Image Questionnaire, Beck Hopelessness Scale, Trait Anxiety Inventory, Symptom Check List (revised) and the Family Assessment Device were performed on all patients. Then, the patients were evaluated for a psychiatric disorder by a psychiatrist (according to the diagnostic criteria of the Diagnostic and Statistical Manual IV of the American Psychiatric Association). The results for the patients and control cases were compared statistically using Mann-Whitney and Kruskal-Wallis tests. Self-image was found to be significantly lower in patients with TM than in control cases (P < 0.01). Hopelessness and Trait-Anxiety scores were determined to be significantly higher in patients with TM than in control cases (P < 0.01 and P < 0.05, respectively). Eighty percent of the patients with TM have had at least one psychiatric disorder. As a result, the study showed that most of the patients with TM had severe psychosocial problems. Relying on these data, it was concluded that medical therapy of these patients should be supported with psychological aid and psychiatric treatment.     

Detection of Early Cardiac Dysfunction in Patients with β-Thalassemia Major and Thalassemia Trait by Tissue Doppler Echocardiography

Cardiac complications are the leading cause of death in β-thalassemia major (TM) patients. The aim of this study was to investigate the impact of iron overload on ventricular functions using conventional and tissue Doppler imaging (TDI) in patients with TM and compare them with children with thalassemia trait (TT) and healthy controls. This prospective study includes 3 groups: group 1: 29 patients with β-TM; group 2: 28 patients with TT; group 3: 29 healthy controls. Peak late relaxation velocity determined by conventional echocardiography for the right ventricle was significantly higher and the E/A ratio for the right ventricle and left ventricle were significantly lower in TM patients than the other groups (P < .05). Peak late relaxation velocity determined by TDI for the left ventricle, interventricular septum, and right ventricle were significantly higher in TM patients than the TT subjects and controls (P < .001). The E/A ratio determined by TDI for the left ventricle, interventricular septum, and right ventricle were significantly lower in group 1 than the other 2 groups (P < .001). There was a negative correlation between the ferritin level and E/A ratio for the left ventricle, interventricular septum, and right ventricle using TDI (P < .05). Conventional echocardiographic techniques have failed to distinguish ventricular functions of asymptomatic patients with TM from the subjects with TT and from normal controls when global functions were examined. The present study indicates that TDI should be used for screening of TM and TT subjects’ cardiac functions.     

Severe hypoglycaemia,metabolic control and diabetes management in children with type 1 diabetes in the decade after the Diabetes Control and Complications Trial – a large-scale multicentre study

Hypoglycaemia is frequently the limiting factor in achieving optimal glycaemic control. Therefore, insulin therapy, the incidence of hypoglycaemia, and glycaemic control were investigated in 6309 unselected children with type 1 diabetes in a large-scale multicentre study. Using standardised computer-based documentation, the incidence of severe hypoglycaemia, HbA1 _c levels, insulin regimen, diabetes duration, and the number of patients attending a treatment centre were investigated for the age groups 0-<5 years ( n =782), 5-<7 years ( n =1053), and 7-<9 years ( n =4474). The average HbA1 _c level was 7.6% (no significant difference between age groups). Young children had more severe hypoglycaemic events (31.2/100 patient years) as compared to older children (19.7; 21.7/100 patient years, P <0.05) independent of the treatment regimen. Our data suggest that diabetes centres treating less than 50 patients per year have a higher incidence of hypoglycaemia in 0-<5-year-old children (43.0/100 patient years) as compared to larger centres (24.1/100 patient years; P <0.0001). Significant predictors of hypoglycaemia were younger age ( P <0.0001), longer diabetes duration ( P <0.0001), higher insulin dose/kg per day ( P <0.0001), injection regimen ( P <0.0005), and centre experience ( P <0.05). Conclusion:Despite modern treatment, young children have an elevated risk for developing severe hypoglycaemia compared to older children, especially when treated at smaller diabetes centres. The therapeutic goal of carefully regulating metabolic control without developing hypoglycaemia has still not been achieved. Further advances in diabetic treatment may result from giving more attention to hypoglycaemia in young children.On behalf of the German Initiative on Quality Control in Paediatric Diabetology.     

Comparison of synchronized and conventional intermittent mandatory ventilation in neonates

Between October 1993 and April 1995, a total of 77 neonates requiring mechanical ventilation were enrolled in this study and were randomly divided into two groups. Group A consisted of 31 premature infants (mean birthweight 1.36 ± 0.29 kg) with respiratory distress syndrome (RDS) and seven neonates (mean birthweight 3.2 ± 0.5 kg) with meconium aspiration syndrome (MAS). Group B consisted of 31 premature infants (mean birthweight 1.31 ± 0.3 kg) with RDS and eight neonates (mean birthweight 3.3 ± 0.5 kg) with MAS. Infants in group A received synchronized intermittent mandatory ventilation (SIMV) and infants in group B received conventional intermittent mandatory ventilation (CIMV) therapy. In premature infants with RDS, our data showed: (i) the duration of ventilation was significantly shorter (P < 0.05) in the synchronized group (156 ± 122 h) compared to the conventional group (242 ± 175 h); (ii) significantly fewer (P <0.05) patients required reintubation in the synchronized group than in the conventional group (three vs 11 patients); (iii) incidence of severe intraventricular hemorrhage (grades 3 and 4) was significantly lower (P < 0.05) in the synchronized group compared to the conventional group (one vs seven patients); (iv) incidence of bronchopulmonary dysplasia was significantly lower (P < 0.05) in the synchronized group than in the control group (one vs seven patients). In neonates with MAS, our data showed no significant difference (P > 0.05) on duration of ventilation, incidence of reintubation, incidence of pneumothorax or mortality rate between synchronized and control groups.     

奥马珠单抗治疗血清总IgE>1 500 IU/mL的中重度过敏性哮喘儿童的前瞻性研究

目的 评估奥马珠单抗治疗血清总免疫球蛋白E (immunoglobulin E,IgE)>1 500 IU/mL的中重度过敏性哮喘儿童的临床疗效。方法 前瞻性选择2020年12月—2022年5月在安徽省儿童医院呼吸内科诊治的中重度过敏性哮喘儿童95例为研究对象。根据血清总IgE水平及是否使用奥马珠单抗治疗分为对照组(总IgE>1 500 IU/mL,未使用奥马珠单抗治疗)、正常治疗组(总IgE水平在30～1 500 IU/mL之间,使用奥马珠单抗治疗)及超高IgE治疗组(总IgE>1 500 IU/mL,使用奥马珠单抗治疗)。分析3组患儿的临床特征和治疗前后儿童哮喘控制测试(Childhood Asthma Control Test,C-ACT)评分、急性发作患儿比例、IgE水平、肺功能指标、呼出气一氧化氮(fractional exhaled nitric oxide,FeNO)浓度的差异。结果 治疗第8周时,正常治疗组、超高IgE治疗组C-ACT评分、第1秒用力呼气量(forced expiratory volume in first second,FEV     

The effects of mesenteric ischemia on ileal colonization, intestinal integrity, and bacterial translocation in newborn piglets

The effects of mesenteric ischemia on ileal colonization, intestinal integrity, and bacterial translocation (BT) in newborn piglets were investigated in 36-2-day-old Pietrain piglets. Group I, controls were not operated upon; group II underwent a sham laparotomy; and group III underwent ligation of the mesenteric vessels in the distal ileum. After 3 days, the kidneys, spleens, livers, and ileal segments were harvested for microbial and histologic analyses. Two piglets in the ischemic group died; microscopic examination showed severe histologic lesions of the ischemic area. Escherichia coli counts were increased in the ischemic segment compared to the upper loop (P < 0.05). Ischemia favoured staphylococcal colonization, whereas in the sham group a drastic reduction of these organisms was observed (P < 0.005). BT to the kidneys, spleen, and liver occurred normally in the control group. Ischemia significantly increased the total microflora in the spleen and liver (P < 0.05) and furthered dissemination of Clostridium perfringens in the kidneys (P < 0.05); 50% of ischemic animals had proteolytic clostridia in this organ (P < 0.05). Moreover, the incidence of E. coli in the kidneys, spleen, and liver was significantly higher in the sham and ischemic groups than in the controls (P < 0.05). Ileal ischemia thus induced significant histologic lesions, and surgery rather than gut microflora controls translocation. Accepted: 16 November 2000     

Abnormalities of the tracheal cartilage in the rat fetus with tracheo-oesophageal fistula or tracheal agenesis

 Many infants with oesophageal atresia and tracheo-oesophageal fistula (OA/TOF) have associated tracheomalacia (TM), which is one of the reasons for respiratory complications after surgical correction of the atresia. OA/TOF was induced in the offspring of pregnant rats by intraperitoneal injection of adriamycin. Fetuses were harvested by caesarean section. The trachea, oesophagus, lungs, and stomach were removed en bloc and stained for cartilage using Alcian blue. The tracheas were examined, photographed, and relevant parameters pertaining to the tracheal cartilage were measured. Exposure to adriamycin resulted in a range of anatomical defects including OA/TOF (47%) and tracheal agenesis (TA) (41%). Adriamycin-treated fetuses were smaller (P < 0.01), yet had longer tracheas (P < 0.001) than control fetuses. The OA/TOF fetuses had more tracheal cartilage rings than controls (P < 0.01), whereas TA fetuses had fewer (P < 0.001). Both OA/TOF and TA fetuses had more malformed tracheal cartilage rings than controls (P < 0.001 and P < 0.05, respectively). Cartilage in the proximal part of the trachea was most frequently and severely affected (P < 0.05). These observations clarify the structural abnormalities of tracheal cartilage that occur in rat fetuses with OA/TOF or TA induced by adriamycin, and may explain the functional disturbances of TM seen in OA/TOF. Accepted: 4 November 1999     

Inhibition of the Na<Superscript>+</Superscript>/H<Superscript>+</Superscript> Exchanger Protects the Immature Rabbit Myocardium From Ischemia and Reperfusion Injury

Background This study investigated the cardioprotective effects of pharmacologic pretreatment with HOE642, a selective Na⁺/H⁺ exchanger (NHE) isoform-1 inhibitor, in immature rabbit hearts, as compared with ischemic preconditioning (IPC). Methods For this study, 36 isolated immature New Zealand white rabbit hearts were equilibrated on the Langendorff apparatus. They were randomly divided into three groups: control group, IPC group, and HOE642 group. The hearts in each group were subjected to 60 min of ischemia plus 60 min of reperfusion (I/R). In the IPC group, the hearts were preconditioned by 5 min of ischemia followed by 10 min of reperfusion before I/R. In the HOE642 group, the hearts were pretreated with HOE642 (5 μmol/l) for 15 min before I/R. Left ventricular performance (LVDP, +dp/dt_max, −dp/dt_max), coronory artery flow (CF), myocardial water content, adenosine triphosphate (ATP), cardiac-specific enzymes (creatine kinase [CK], CK fraction MB [CK-MB], and lacate dehydrogenase [LDH]), and intracellular calcium content were measured. Myocardial ultrastructure was observed under transmission electron microscopy. Results The recovery rates for left ventricular performance and CF in both the HOE642 and the IPC groups increased compared with those for the control subjects (p < 0.05). Moreover, the recovery rates for LVDP, +dp/dt_max, −dp/dt_max, and CF in the HOE642 group were markedly higher than in the IPC group at most time points of reperfusion (p < 0.05). Compared with the control group, CK, CK-MB, and LDH in the HOE642 group were decreased significantly (p < 0.05), whereas only LDH was reduced in the IPC group (p < 0.05). Water content was significantly reduced and ATP reserve was significantly increased in both the IPC and HOE642 groups (p < 0.05). However, compared with the IPC group, water content in the HOE642 group was significantly lower (81.26% ± 1.26% vs 83.58% ± 1.27%; p < 0.05) and ATP was significantly higher (21.46 ± 2.40 vs 17.66 ± 1.50 μg/g; p < 0.05). The HOE642 pretreatment exerted a better effect of reducing calcium overload than IPC (265.8 ± 41.1 vs 408.5 ± 56.8 mg/kg dry weight; p < 0.05). The blinded ultrastructural assessment under transmission electron microscopy showed that HOE642 brought about more myocyte salvage than IPC. Conclusion This study demonstrated that HOE642 pretreatment is superior to IPC against ischemia and reperfusion injury in isolated immature rabbit myocardium.     

Resveratrol attenuates inflammation and stricture formation in experimental caustic esophageal burns

The purpose of medical treatment in the caustic esophageal burns (CEB) is to decrease inflammatory reaction and to prevent stricture formation. Resveratrol has anti-inflammatory and antifibrotic properties. The aim of this study is to investigate potential therapeutic effects of resveratrol in experimental CEB. We divided 42 male Wistar albino rats into five groups: a control group, caustic groups 4 and 28 (esophageal burns were created), and resveratrol groups 4 and 28 (esophageal burns were created and resveratrol was administered). We used 25% NaOH to form CEB following the method of Gehanno and Guedon as modified by Liu and Richardson. Animals were killed on the 4th and 28th days for biochemical and histopathological examinations. We found that the mean malondialdehyde and nitric oxide assays of the caustic groups were significantly higher than that of the resveratrol groups (P < 0.05). On the other hand, glutathione assay of the resveratrol groups was significantly higher than that of the caustic groups (P < 0.05). Histologically, edema, inflammation and necrosis were found to be significantly lower in the resveratrol 4 group compared with the caustic 4 group (P < 0.05). Submucosal and muscular collagen accumulation were found significantly lower in the resveratrol 28 group compared with the caustic 28 group (P < 0.05). We conclude that resveratrol decreased both the inflammatory reaction and the stricture formation in experimental CEB.     

Measurement of serum total bile acid levels in long-term postoperative follow-up of patients with congenital bile-duct dilatation

 Serum total bile acid levels (STBA), a sensitive indicator of cholestasis, were measured during the long-term postoperative period in patients with congenital bile-duct dilatation (CBDD) (choledochal cyst) and the factors contributing to elevation of STBA were analyzed in 44 patients. Their ages at operation ranged from 1 month to 16 years. A STBA level over 12 nmol/ml on two or more measurements during outpatient follow-up was considered abnormal. Patients were classified into three groups: group 1, STBA, 12–50 nmol/ml; group 2, STBA ≥ 50 nmol/ml; group N, STBA normal. In 19 patients (43.2%) STBA was normal. Of the 25 patients (56.8%) with elevated STBA, groups 1 and 2 comprised 12 (27.3%) and 13 patients (29.5%), respectively. The mean age in group N was 5.1 ± 4.2 years, which was higher than in groups 1 (2.6 ± 2.3) and 2 (2.3 ± 2.5 years) (P < 0.05). Preoperative STBA and total bilirubin were higher in group 2 (79.2 ± 75.1 nmol/ml resp. 5.2 ± 4.2 mg/dl) than in groups N (20.1 ± 32.6, 1.3 ± 1.4) and 1 (22.8 ± 37.2, 1.4 ± 1.0) (P < 0.05). Preoperative alkaline phosphatase and gamma-glutamyl transpeptidase were higher in group 2 (1,006 ± 872 IU/l, 452 ± 326 IU/l) than in group N (573 ± 371, 205 ± 238) (P < 0.05). Histologic findings on liver biopsy showed fibrosis in 38.5% of group 2 patients, which was significantly higher than in groups N (15.8%) and 1 (16.7%) (P < 0.05). Cholestasis was detected in 41.7% of group 1 and 61.5% of group 2 patients, compared to 10.5% of group N patients (P < 0.05). Postoperative elevation of STBA may thus persist in more than one-half of patients with CBDD, and is likely to occur in patients of young age who have severe cholestasis or liver fibrosis preoperatively. Further investigations may be required in regard to the development of postoperative complications. Accepted: 7 February 2001     

Expression and significance of TGF-β1/Smad signaling pathway in children with IgA nephropathy

Background  IgA nephropathy (IgAN) exhibits an indolent but slowly progressive course, and about 30% of children with IgAN are found to deteriorate to end-stage renal failure characterized by overaccumulation of extracellular matrix, diffuse glomerular sclerosis, and tubulointerstitial fibrosis. The TGF-β/Smad signaling pathway plays an important role in glomerulosclerosis and tubulointerstitial fibrosis. The present study aimed to elucidate the significance of expressions of TGF-β1, phosphorylated Smad3 (p-Smad3), Smad7 and fibronectin (FN) in the renal tissue of children with IgAN. Methods  Forty-six children with IgAN were divided into 3 groups according to their clinical features: isolated hematuria group (IH group, 8 patients), hematuria and proteinuria group (HP group, 24), and nephritic syndrome group (NS group, 14). Patients were also divided into three groups according to their pathologic grade: grade I+II (22 patients), grade III (12) and grade IV (12) groups. Five normal renal specimens were used as the control group. The expression of TGF-β1, p-Smad3, Smad7 and FN in renal biopsy specimens was detected by two-step PowerVision™. The degrees of renal tubular injury and interstitial fibrosis were scored according to the Katafuchi semi-quantitative criteria. Results  The expression of TGF-β1, p-Smad3, Smad7 and FN in children with IgAN was significantly higher than that in the control group (in glomeruli: P<0.05, P<0.01, P<0.05 and P<0.01, respectively; in tubulointerstitium: P<0.05, P<0.05, P<0.01 and P<0.05, respectively) and the highest expression levels were found in the NS and grade IV groups (P<0.05, P<0.01). The expression levels of the four proteins were not only positvely correlated with each other, but also with the grade of renal tubular injury and renal interstitial fibrosis (P<0.05). Conclusion  The TGF-β1/Smad signaling pathway plays an important role in the progress of glomerular sclerosis, renal tubular injury and interstitial fibrosis in children with IgAN.     

Évolution du résultat des explorations fonctionnelles respiratoires chez les enfants atteints de dysplasie bronchopulmonaire

Background.

Reports of short- and medium-term evolution of Lung Function Tests (LFT) in infants with bronchopulmonary dysplasia (BPD) are still scarce.

Population and methods.

The results of the first (before 3 months of corrected age) and the second (between 3 and 9 months of corrected age) LFT in 22 premature infants with BPD (gestational age; 31 ± 2.5 weeks; birth weight: 1570 ± 440 g; duration of mechanical ventilation: 46 ± 24 days; total duration of oxygen therapy: 88 ± 47 days) were compared to those obtained in 27 normal infants for the first LFT and 10 normal infants for the second LFT, similar to the patients for birth weight and corporeal index (CI).

Results.

In the first LFT, major abnormalities were an increased thoracic gaz volume (TGV) (165 ± 42 vs 122 ± 24 mL; P < 0.001) and TGV CI ratio (1.25 ± 0.31 vs 0.89 ± 0.17 mL/kg/m²; P < 0.0001) a decreased pulmonary compliance (2.49 ± 1.46 vs 11.60 ± 4.50 mL/cmH₂O; P < 0.0001) and specific pulmonary compliance (0.015 ± 0.10 vs 0.100 ± 0.042 mL/cmH₂O/mL de TGV; P < 0.0001), an increased total pulmonary resistance (20.4 ± 12.1 vs 10.5 ± 5.3 cmH₂O/L/s; P < 0.001). In the second LFT, an increased TGV (235 ± 62 vs 166 ± 28 mL; P < 0.01) and TGV CI ratio (1.64 ± 0,65 vs 0.98 ± 0.11 mL/kg/m²; P < 0.05), a decreased pulmonary compliance (2.68 ± 2.0 vs 15.2 ± 5.7 mL/cmH₂O; P < 0.0001) and specific pulmonary compliance (0.013 ± 0.010 vs 0.106 ± 0.050 mL/cmH₂O/mL de TGV; P < 0.0001), an increased total pulmonary resistance (17.1 ± 9.6 vs 8.6 ± 4.9 cmH₂O/L/s; P < 0.05) were noted when compared with the control group results. Major abnormalities of the blood gases were hypoxemia (63 ± 10 vs 85 ± 20 mmHg; P < 0.05), hypercapnia (38.5 vs 31 ± 4 mmHg; P < 0.0001) during the first LFT. Hypoxemia (77 ± 14 vs 90 ± 14 mmHg) and hypercapnia (37 ± 4 vs 29 ± 5 mmHg) continued in the second LFT. Thoracic distention and total pulmonary resistances in infants with BPD did not improve but their pulmonary compliance (P < 0.0001) and PaO₂ (P < 0.01) between the first and second LFT did it. Infants who had been ventilated for a hyaline membrane disease (HMD) were more hypoxic on the second LFT (P < 0.05) than those who had been ventilated for other causes. Statistically significant relationships were found between thoracic distention and duration of positive inspiratory pressure (P < 0.05; r = 0.43), duration of positive expiratory pressure (P < 0.05; r = 0.45), total oxygen therapy duration; between total pulmonary resistance and duration of mechanical ventilation with high frequency (P < 0.05; r = 0.52); between hypoxemia and duration of oxygen therapy with FiO₂ ≥ 60% (P < 0.05; r = 0.54).

Conclusions.

This study shows prolonged clinical and functional abnormalities of the respiratory functions requiring longer follow-up.