肾小球疾病156例的临床与病理

了解各种小儿肾脏疾病的临床表现和病理类型二者间的关系。方法对156例肾活检患儿的临床和病理的关系进行了分析。结果系膜增生性肾炎占68％,其临床表现为多种形式的肾脏疾病;IgA肾病22例,占14．1％;毛细血管内增生性肾炎占4．5％;新月体肾炎6例（3．8％）;其他型别的病理类型均较少见。结论系膜增生性肾炎是小儿肾脏疾病最常见的病理类型,临床主要见于紫癜性肾炎（34．9％）,其次见于肾病综合征（28．3％）,各种类型的肾小球肾炎（10．4％）。     

Evaluation of gastrointestinal pathology and treatment in children with suspected biliary dyskinesia

Aim  

Decreased gallbladder ejection fraction (GBEF) was reported in patients who had abdominal pain and gastrointestinal (GI) diseases. The study aims were to review pathology of GI tract in children with acalculous biliary-type abdominal pain and to evaluate the pain improvement after a 2-week trial of proton pump inhibitor (PPI) and laparoscopic cholecystectomy (LC).     

Laparoscopic ligation of splenic vessels for the treatment of hereditary spherocytosis in children

Total splenectomy is the most effective surgical treatment for hereditary spherocytosis (HS). Nevertheless, post-splenectomy sepsis and hypoimmunity can pose a great risk to children. Some alternative treatments have been proposed to avoid the post-splenectomy complications. In this study, we propose such a procedure (laparoscopic ligation of splenic vessels, L-LSV) for the treatment of HS in children and investigate its effectiveness and feasibility. A total of 17 children with HS who underwent the L-LSV at our hospital between May 2015 and Apr 2018 were enrolled in the current study. All patients were followed-up for 3–38 months (mean 19.8 months). The volume of spleen was preoperatively and postoperatively measured using the AW VolumeShare5. The size of functional spleen and the condition of splenic infarction were evaluated using ultrasound and computed tomography (CT) after surgery. The routine blood, biochemistry and coagulation tests were carried out after surgery. The L-LSV was successfully performed in all patients. The average operative time was 115 min (range 60–180 min). No patients underwent blood transfusion during surgery, and the length of hospital stay varied 5–9 days after surgery (mean 7 days). Postoperatively, the red blood cells, platelet and hemoglobin were significantly increased (P < 0.05). The postoperative volume of functional spleen was significantly smaller than preoperative volume of spleen (307.393 ± 177.634 cm3 vs. 581.242 ± 270.260 cm3, P = 0.000). The recent volume of functional spleen was significantly bigger than the postoperative 1 month volume of functional spleen in ten children who were followed-up for more than 1 year (P = 0.004). The index of splenic infarction (the proportion of the postoperative 1-month volume of splenic infarction in the preoperative volume of spleen) was 0.31–0.99 (mean 0.53). There were no patients undergoing the blood transfusion after surgery. The L-LSV is an effective treatment for HS in children; however, future studies should re-evaluate the long-term prognosis.     

儿童遗传性肾小管碱中毒诊疗进展

儿童遗传性肾小管碱中毒为临床罕见的肾小管疾病,是肾小管上皮细胞离子通道基因变异导致蛋白质功能异常引起的临床综合征。Bartter综合征、Gitelman综合征和Liddle综合征属于其中较常见的几种类型,均是以肾小管碱中毒为临床特征的遗传性失钾性疾病。文章综述上述三者的发病机制、临床表现及诊断和治疗的最新进展。     

Rod ERGs in children with hereditary retinal degeneration

Rod ERGs were obtained over a broad range of stimulus intensities from young patients with retinitis pigmentosa and progressive cone-rod degeneration. Intensity-response functions suggest that children with retinitis pigmentosa have reductions in the number of functioning rods and that surviving rods generating the ERG have reduced sensitivity. Children with cone-rod degeneration also have reduced numbers of rods, but the remaining rods have normal sensitivity. These findings suggest that the underlying mechanisms of rod degeneration differ in these two forms of disease and demonstrate that insights into the pathogenesis of hereditary retinal degeneration can be obtained by testing children. The ability to distinguish between retinitis pigmentosa and cone-rod degeneration in children is also important because of differences in the rates of progression between the two classes of progressive retinal degeneration.     

Amoebiasis in children: pathology,diagnosis, and treatment

Experience with 56 children with amoebiasis referred for surgical management is presented. Twenty-four patients had predominantly colitic disease and 32 hepatic; 3 of the 56 had both organs affected. Based on this experience an attempt is made to define therapeutic options in children. The relative roles of colonic resection and peroperative prograde colonic irrigation in the treatment of colitis and the indications for aspiration of hepatic abscesses are discussed. The pathology is outlined.