Objective: To analyse nutritional status of children underwent total cavopulmonary connection (TCPC) also called Fontan procedure, furthermore to investigate risk factors related to malnutrition including underweight, stunting, and thinness. Methods: Clinical data of 314 children underwent Fontan procedure in Shanghai Children's Medical Center from Jan. 2012 to Dec. 2015 were reviewed. WHO Z score was used to evaluate nutritional status of the children underwent Fontan procedure at the time of long-term follow-up. According to the nutritional status, children were divided into underweight and no-underweight groups, stunting and no-stunting groups, thinness and no-thinness groups. Univariate and multivariate Logistic regression analysis were used to detect risk factors for malnutrition in the children after Fontan procedure. Results: In the follow-up survey after Fontan procedure, the proportion of underweight, stunted and thin children were 11.9%, 17.8%, and 16.9%, respectively. Multivariate Logistic regression analysis showed that preoperative malnutrition, worse cardiac function, moderate or severe atrioventricular valve regurgitation and long time use of diuretics were the risk factors for underweight in the children after Fontan procedure (P=0.008, P=0.000, P=0.025, P=0.001); preoperative malnutrition, elevated mean pulmonary artery pressure, worse cardiac function, moderate or severe atrioventricular valve regurgitation and long time use of diuretics were the risk factors for stunting in the children after Fontan procedure (P=0.001, P=0.046, P=0.000, P=0.019, P=0.004); preoperative malnutrition, elevated mean pulmonary artery pressure, worse cardiac function, moderate or severe atrioventricular valve regurgitation and long time use of diuretics were the risk factors for thinness in the children after Fontan procedure (P=0.019, P=0.018, P=0.015, P=0.026, P=0.000). Conclusion: Malnutrition is still remained during follow-up period in the children with single ventricle physiology after Fontan procedure. Preoperative malnutrition, worse cardiac function, elevated pulmonary artery pressure, long time use of diuretics and moderate or severe atrioventricular valve regurgitation may be associated with malnutrition after Fontan procedure.

Antimicrobial Cationic Peptides ; biosynthesis ; genetics ; DNA-Binding Proteins ; biosynthesis ; genetics ; Humans ; Microbial Sensitivity Tests ; Muramidase ; biosynthesis ; genetics ; Peptides, Cyclic ; biosynthesis ; genetics ; Pichia ; metabolism ; Recombinant Proteins ; biosynthesis ; genetics ; isolation & purification

Adolescent ; Adrenal Cortex Diseases ; complications ; pathology ; surgery ; Adult ; Child ; Female ; Humans ; Hydrocortisone ; blood ; urine ; Male ; Middle Aged ; Pigmentation Disorders ; complications ; pathology ; surgery

OBJECTIVETo study the association between sleep hygiene and sleep duration and quality among school-age children, and to explore the risk factors related to poor sleep hygiene.

METHODSTotally 2019 grade-five children were sampled by stratified cluster random sampling from 10 primary schools in Shanghai, during November and December 2009. Questionnaires were used to investigate children and their parents. Adolescent Sleep Hygiene Scale (ASHS) was used to inquiry sleep hygiene of children; Children Sleep Habits Questionnaire (CSHQ) was used to assess their sleep duration and quality; Family and Social Environment Questionnaire was used to collect demographic and socio-economic information. T-test, one-way ANOVA and multiple linear regression model were established to identify the risk factors for sleep hygiene.

RESULTSThe age of subjects was (10.81 ± 0.38) years old, 49.0% (989/2019) were boys. The ASHS total score was 125.43 ± 15.17, girls with better sleep hygiene than boys (127.05 ± 14.41 vs 123.74 ± 15.75, P < 0.05). The sleep duration was (9.47 ± 0.58)h/d, children slept less than 9 h/d had lower ASHS total score than those slept 9 - 10 h/d or over 10 h/d (121.69 ± 16.09 vs 126.17 ± 14.62 vs 126.50 ± 15.36, P < 0.05). Children with poor sleep quality had worse sleep hygiene than those with good sleep quality (121.00 ± 15.84 vs 128.36 ± 13.92, P < 0.05). Children with television set in bedroom had lower ASHS total score than the others (122.40 ± 15.76 vs 126.74 ± 14.66, P < 0.05). Children from single parent family had lower ASHS total score (117.90 ± 16.80 vs 125.94 ± 14.89, P < 0.05). Children whose father had irregular sleep or wake pattern had lower ASHS total score (122.65 ± 15.30 vs 125.89 ± 14.90 vs 127.79 ± 14.71, P < 0.05). The regression model confirmed that existence of television set in children's bedroom, single-parent family and father's irregular sleep pattern were the risk factors of poor sleep hygiene.

CONCLUSIONSleep hygiene was closely associated with sleep duration and quality among school-age children. Children with television set in bedroom, male, from single parent family and whose father had irregular sleep or wake pattern had worse sleep quality.

Child ; Female ; Habits ; Humans ; Male ; Quality of Life ; Sleep ; Sleep Wake Disorders ; Students ; Surveys and Questionnaires

OBJECTIVETo study the pathology, diagnostic and therapeutic method of primary pigmented nodular adrenocortical disease (PPNAD).

METHODSThe data of 5 cases of PPNAD were analyzed retrospectively. Among the 5 cases, 2 were male and 3 were female. The range of age was from 12 to 53 years. All the 5 cases had symptoms of Cushing syndrome. The diagnose depended on the results of endocrine exams, ultrasound, CT, MR and pathological reports. All patients received operation of unilateral adrenalectomy. The therapeutic effects were determined by post-operative results, which concluded clinical symptoms and endocrine exams.

RESULTSThe follow-up time was from 4 months to 3 years. All patients' symptoms of Cushing syndrome were relieved in 6 months after operation. The endocrine exam was normal in one case and obvious improved in the other four cases. Up to now, one patient drop out of the follow-up, the other 4 cases had no evidence of recurrence.

CONCLUSIONPPNAD is a rare type of Cushing syndrome. Diagnose depends on endocrine exams and pathological results. Operation is the effective method for the disease.

Adolescent ; Adrenalectomy ; Adult ; Child ; Cushing Syndrome ; diagnosis ; pathology ; surgery ; Female ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Pituitary-Adrenal Function Tests ; Retrospective Studies ; Tomography, X-Ray Computed

Objective:To explore the feasibility and safety of robotic-assisted living donor left lateral segmentectomy (LDLLS) in a large pediatric liver transplant program.Methods:Retrospective analysis was performed for clinical data of 45 LDLLS donors and recipients from June 2021 to September 2022.Traditional open donor liver resection (n=30) and robotic-assisted segmentectomy (n=15) were performed.Two groups were compared with regards to operative duration, intraoperative hemorrhage, postoperative healing and postoperative complications.SPSS 21.0 was utilized for statistical analysis.Independent sample T, paired sample T, Wilcoxon rank sum and Chi-square tests were performed for examining the inter-group differences.Results:Operative duration of robot-assisted surgery group was substantially longer than that of traditional open surgery group ( P<0.001). Intraoperative blood loss was less in robot-assisted surgery group was less than that in traditional open surgery group[(106.0±39.8) vs.(251.0±144.8) ml, P=0.001]. Postoperative hospital stay of robot-assisted surgery group was shorter than that of traditional open surgery group[6.0(6.0, 6.0) vs.7.0(6.0, 9.0), P<0.05]. Two cases of postoperative biliary leakage were observed in donor of traditional open surgery group.Among 2 cases of abdominal infection, one was due to biliary leakage from liver section and secondary surgery was then performed.One case of incisional infection and another case of thrombosis occurred in donor of traditional open surgery group.In robot-assisted surgery group, only one donor had amylase elevation.In traditional open surgery group, there were one case of local thrombosis in middle hepatic vein and one case of bile duct stricture.No long-term complications occurred in robot-assisted surgery group during a follow-up period of over 6 months.Finally recipient data analysis indicated that no significant inter-group differences existed in operative duration, intraoperative blood loss, postoperative hospital stay or postoperative abdominal infection ( P=0.634, P=0.180, P=0.86 and P=0.153). Conclusions:Robotic-assisted LDLLS proves to be be a safe and reliable option for living donor segmentectomy.It is superior to conventional LDLLS in terms of shorter hospital stay, less intraoperative blood loss and fewer postoperative complications.

Objective To investigate clinical effect and indications of neuroendoscopic surgery for treatment of intracranial diseases. Methods A total of 386 cases treated with neuroendoscopic surgeries were analyzedd retrospectively in regard to the surgical approaches, treatment outcomes, advantages and disadvantages of neuroendoscopy. Results In the 386 cases, clinical cure was achieved in 340 cases and symptomatic improvement in 40 cases; 5 cases failed to respond to the treatment, and death occurred in 1 case during the follow-up for 6-12 months. Postoperative radiography demonstrated intracranial space-occupying lesions in 285 cases, for which total resection was achieved in 259 cases and subtotal resection in 26 cases with neuroendoscopic surgery, and relapse occurred in 7 cases. Hydrocephalus occurred in 101 cases. Cerebral ventricle recovery was found in 82 cases, and in 18 cases the ventricular expansion was obviously reduced; ventricular expansion remained unchanged in 1 case after the treatment. Conclusions With careful evaluation of the surgical indications, neuroendoscopic surgeries can achieve satisfactory effects for treatment of intracranial diseases.

Postradiation nasopharyngeal necrosis is an important late effect of radiotherapy that affects prognosis in patients with nasopharyngeal carcinoma. In the present study, we reviewed the clinical and imaging features of 67 patients with pathologically diagnosed postradiation nasopharyngeal necrosis who were treated at Sun Yat-sen University Cancer Center between June 2006 and January 2010. Their clinical manifestations, endoscopic findings, and imaging features were analyzed. Early nasopharyngeal necrosis was limited to a local site in the nasopharyngeal region, and the tissue defect was not obvious, whereas deep parapharyngeal ulcer or signs of osteoradionecrosis in the basilar region was observed in serious cases. Those with osteoradionecrosis and/or exposed carotid artery had a high mortality. In conclusion, Postradiation nasopharyngeal necrosis has characteristic magnetic resonance imaging appearances, which associate well with clinical findings, but pathologic examination is essential to make the diagnosis.
Adult ; Aged ; Carcinoma ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Nasopharyngeal Neoplasms ; radiotherapy ; Nasopharynx ; pathology ; radiation effects ; Necrosis ; Osteoradionecrosis ; diagnosis ; etiology ; Radiation Injuries ; diagnosis ; etiology ; Radiotherapy, Intensity-Modulated ; adverse effects

This study was purposed to investigate whether phenylhexyl isothiocyanate (PHI) can reduce p16 gene methylation level or not. The myeloma U226 cell line was cultured with PHI of 0, 5, 10 micromol/L for 72 hours, then DNA was extracted. Hydrosulfite was used to treat the genome DNA of healthy adult, PCR amplification was carried out by using this DNA as template. The gene chip detecting methylation changes of 3 CpG in promoter region of p16 gene was constructed by designing a pair of probes which contain one methylated and one unmethylated probes. This pair of probes was used to detect 3 consecutive sites of CpG island in p16 gene. The standard curve was constructed by using gene chip after the methylated and unmethylated DNA were mixed at different ratio. Then treated samples of U266 cells were dotted on gene chip, obtained results were compared with standard curve to get the quantitative results. The results indicated that the probes on chip had excellent reproducible ability and precision, the methylation level of p16 gene in U266 cells treated with 0, 5 and 10 micromol/L of PHI was determined as 78.2%, 61.7% and 54.8%, respectively. It is concluded that the PHI can reduce the methylation level of p16 gene in U266 cells.
Cell Line, Tumor ; CpG Islands ; DNA Methylation ; Down-Regulation ; Genes, p16 ; Humans ; Isothiocyanates ; pharmacology ; Oligonucleotide Array Sequence Analysis

The aim of this study was to explore the effect of gambogic acid (GA) on MDS SKM-1 cell proliferation, apoptosis and their possible mechanism. Cell proliferation was determined by MTT method. The apoptosis percentage and cell cycle regulation of SKM-1 cells were analyzed by flow cytometry. Morphological features were observed by light microscopy. The mRNA expression of bcl-2 and bax were detected by RT-PCR. The results showed that GA could inhibit the proliferation of SKM-1 cells in a dose- and time-dependent manner (IC50 was 0.37 µg/ml at 48 h), increase the apoptotic percentage of SKM-1 cells, and arrest cell cycle at the G0/G1. The expression of bax mRNA was up-regulated while that of bcl-2 mRNA was down-regulated in SKM-1 cells treated with GA for 48 h. It is concluded that GA can induce apoptosis, which may be related to its effect of arresting cells at phase of G0/G1 and down-regulating bcl-2/bax ratio.
Apoptosis ; drug effects ; Cell Cycle Checkpoints ; Cell Line, Tumor ; Cell Proliferation ; drug effects ; Humans ; Myelodysplastic Syndromes ; metabolism ; pathology ; Proto-Oncogene Proteins c-bcl-2 ; metabolism ; Xanthones ; pharmacology ; bcl-2-Associated X Protein ; metabolism