Standards of care for Kasabach-Merritt phenomenon in China.

Kasabach-Merritt phenomenon (KMP) is a rare disease that is characterized by severe thrombocytopenia and consumptive coagulation dysfunction caused by kaposiform hemangioendothelioma or tufted hemangioma. This condition primarily occurs in infants and young children, usually with acute onset and rapid progression. This review article introduced standardized recommendations for the pathogenesis, clinical manifestation, diagnostic methods and treatment process of KMP in China, which can be used as a reference for clinical practice.

Altered microRNA expression profiles in a rat model of spina bifida.

MicroRNAs (miRNAs) are dynamically regulated during neurodevelopment, yet few reports have examined their role in spina bifida. In this study, we used an established fetal rat model of spina bifida induced by intragastrically administering olive oil-containing all-trans retinoic acid to dams on day 10 of pregnancy. Dams that received intragastric administration of all-trans retinoic acid-free olive oil served as controls.

Preliminary investigation of methylation status of microRNA-124a in spinal cords of rat fetuses with congenital spina bifida.

Objective: We investigated the expression of microRNA-124a and its methylation status in the spinal cords of rats with congenital spina bifida versus rats with normal fetuses.

Methods: Real-time quantitative reverse transcription-polymerase chain reaction was used to compare the expression of microRNA-124a in the spinal cords of 42 rats with all-trans retinoic acid induced congenital spina bifida and 42 rats with normal fetuses. The DNA methylation status in the promoter region of miRNA-124a was detected using methylation specific-PCR.

Postoperative steroids after Kasai portoenterostomy for biliary atresia: a meta-analysis.

Aim: The aim of this systematic review and meta-analysis was to determine if adjunct steroids affect jaundice-free, cholangitis, and survival rates after Kasai portoenterostomy.

Methods: The literature was searched using the following terms: biliary atresia, portoenterostomy, steroids, glucocorticoids, dexamethasone, prednisolone, and hydrocortisone. The primary outcome was the jaundice-free rate.

[Expression and promoter methylation of SIX2 gene in peripheral blood of pediatric patients with nephroblastoma].

Objective: To explore the transcriptional expression and promoter methylation status of SIX2 gene in peripheral blood of pediatric children with nephroblastoma and discuss their clinicopathological correlations.

Methods: Approved by the hospital ethics committee, peripheral blood samples were collected from 45 children with Wilms' tumor(case group) at the Department of Pediatric Surgery, First Affiliated Hospital, Zhengzhou University from October 2008 to January 2012. And another 15 pediatric cases gender-and-age matched, were selected as the control group (excluding cancer and other malignant diseases).

[Relationship between mRNA expression and promoter methylation status of p73 gene in peripheral blood among children with Wilms' tumor].

Objective: To investigate the mRNA expression and promoter methylation status of p73 gene in the peripheral blood of children with Wilms' tumor (WT), and their relationship.

Methods: Forty-five children with WT were selected as the case group, and 15 sex- and age- matched children (without malignancies) who visited the hospital for physical examination or other reasons were selected as the control group. Peripheral blood was collected from both groups.

Surgical management of intrahepatic vessels in children with stage III/IV hepatoblastoma.

Background: Hepatoblastoma (HB) is a rare childhood tumor. We investigated the effect of intraoperative management of the intrahepatic major vessels in children with HB.

Methods: Between April 2005 and August 2012, surgical resection was performed on 50 children with hepatoblastoma.

[Serologic characteristics and population distribution of subtypes B2 and AB2 of ABO blood group].

This study was aimed to investigate the serologic characteristics, genetic background and population distribution of B2 and AB2 subtype in Chinese ABO blood group. The classic blood group serological technology was used to detect ABO blood group of the propositus and their family members, the anti-B1 serum prepared by yourself was used to investigate the distribution of B1/B2 and AB1/AB2 subtype of the blood donor. The results indicated that the antigen of propositus was AB2 subtype and that of his child was B2 subtype.

Family and segregation studies: 411 Chinese children with primary nocturnal enuresis.

Background: The aim of the present paper was to determine the incidence of primary nocturnal enuresis (PNE) among relatives of Chinese children with PNE, the inheritance pattern, and to identify the characteristics of PNE with positive family history.

Methods: From July 2003 to June 2004, an epidemiological survey on PNE children was carried out by self-administered questionnaires to parents of 5-18-year-old Chinese students in Henan Province, central China. A detailed family history was recorded in order to determine the presence of familial PNE as defined as any close relative with PNE beyond the age of 5 years.

Detection and significance of serum protein marker of Hirschsprung disease.

Objective: The objective of this study was to identify a specific fingerprint chromatogram model of serum proteins for early screening and diagnosis of Hirschsprung disease.

Methods: To detect the protein mass spectrograms of 78 serum specimens (42 specimens of Hirschsprung disease, 16 specimens of adhesive ileus including appendicitis and Meckel diverticulum after operation and inflammatory bowel disease, and 20 specimens of normal control subjects), we used surface-enhanced laser desorption/ionization time of flight mass spectrometry technology, combined with bioinformatics methods (support vector machine) to develop and compare protein mass spectrograms from serum samples.

Results: We identified 3 protein markers, the mass-to-charge ratio of which is positioned at 3221.

Development of nocturnal urinary control in Chinese children younger than 8 years old.

Objectives: To estimate the development of nocturnal urinary control (NUC) with age in Chinese children younger than 8 years of age using cross-sectional and retrospective surveys.

Methods: We used a cross-sectional survey of 4754 children (1 to 8 years old), a retrospective investigation of 2745 children younger than 9 years old, and an anonymous questionnaire of 8222 children 9 to 18 years old . The children without NUC were subdivided into infant (1 to 3 years old), preschool age (4 to 6 years old), and primary school (7 to 8 years old) groups.

[Clinical study of multiple zonal aganglionosis in long segment Hirschsprung's disease].

Objective: To discuss pathogeny and diagnosis and management of multiple zonal aganglionosis in Hirschsprung's disease.

Methods: Records of 3 children, aging 5 days, 29 days and 18 months, 3 boys, with multiple zonal aganglionosis in long segment Hirschsprung's disease between 1987-2005 were reviewed retrospectively. Total colectomy and Soave's operations were performed.