心脾综合征的多普勒超声心动图诊断

目的:探讨多普勒超声心动图对心脾综合征的诊断价值,分析其心脏伴随畸形漏诊及误诊原因,提高对心脾综合征诊断的准确率。方法:本文总结了221例心脾综合征患儿的超声检测结果,并与其心血管造影或磁共振检查结果进行对照。结果:超声心动图诊断为心脾综合征与心导管或磁共振结论符合者199例(占90.0%),诊断不符合者22例(占10.0%)。结论:多普勒超声心动图可以较准确地诊断心脾综合征,但对其心脏伴随畸形检出的准确率尚待进一步提高。     

Relationship between Visitation Policy in a Pediatric Intensive Unit and Parental Anxiety

The purpose of this study was to examine the levels of anxiety of mothers with children in pediatric intensive care units with different types of visitation policies, both structured and individualized Forty subjects were obtained from two children's hospitals A comparative research design was used Data showed that mothers who experienced individualized visitation had significantly lower anxiety scores (p < 005) than mothers who experienced structured visitation Findings also revealed that mothers viewed their child's illness as less severe when individualized visitation was permitted as opposed to structured visitation.     

Simultaneous non-parametric confidence intervals for survival probabilities from censored data

In medical studies with censored data Kaplan and Meier's product limit estimator has frequent use as the estimate of the survival function. Simultaneous confidence intervals for the survival function at various time points constitute a useful addition to the analysis. This study compares several such methods. We consider in a simulation investigation two whole curve confidence bands and four methods based on the Bonferroni inequality. The results show that three Bonferroni-type methods are essentially equivalent, all being better than the other methods when the number of time points is small (3 or 5).     

Rhinitis Symptom Utility Index (RSUI) in Chinese Subjects: A Multiattribute Patient-preference Approach

Background: The Rhinitis Symptom Utility Index (RSUI), originally developed in the United States, consists of a patient-preference weighting scheme and a 10-item questionnaire measuring the severity and frequency of rhinitis related symptoms over a 14-day period. This study aimed to determine whether the Chinese RSUI could adopt the US-based multi-attribute utility function (MAUF) in scoring rhinitis symptoms. Methods: In a Hong Kong study, 116 Chinese adults with allergic rhinitis completed the RSUI questionnaire and 36-item Short-Form Health Survey (SF-36) after they had been seen by two otorhinolaryngologists for disease-severity ratings. Respondents then completed computer-administered direct preference measures, i.e., visual analogue scale (VAS) and standard gamble (SG) assessments. The VAS and SG data were used to estimate a MAUF for the Chinese-based RSUI. Results: The derived MAUF was somewhat different than the one developed for the US RSUI. Test–retest reliability for the Chinese RSUI was satisfactory (ICC = 0.71, p<0.001). Scores differentiated among cases with mild, moderate, and severe symptoms (p<0.001); and between those who did and did not require medications to control symptoms (p = 0.031). Findings were significantly correlated with SF-36 domain scores (r = 0.19 to 0.37; p=0.041 to <0.001). When the US-based scoring function was applied to the Chinese subjects, the resulting mean RSUI score was significantly lower (p<0.001). Comparisons between directly measured VAS and SG scores between the US and Chinese samples, demonstrated significant differences (all p<0.05), with the US subjects consistently rating rhinitis symptoms as worse than Chinese subjects. Conclusions: The Chinese RSUI has good measurement properties that reflect patient preferences from the Chinese. Results suggest that there are differences in preference rating between US and Chinese subjects and that use of the US-based preference function for the RSUI would bias the measurement of rhinitis symptom outcomes in Chinese subjects.     

Characteristics of thyroidectomy in Taiwan.

BACKGROUND AND PURPOSE: Thyroidectomy is a common treatment for thyroid disorders in Taiwan, and constitutes a significant percentage of medical expenses. This study investigated the characteristics of thyroidectomy in Taiwan. METHODS: A total of 21 senior general surgeons from 16 medical centers and 5 regional hospitals participated. One surgeon from each hospital reviewed the hospital's medical records of thyroid operations performed in the year 2001. Medical records for 3846 thyroidectomies were retrospectively analyzed, including surgical indications and modalities, complications, pathology reports, and the use of antibiotics. RESULTS: Most of the patients were women (85%). Indications for surgery included proven malignancy (9%), suspicious malignancy (30%), evident compression symptoms (20%), hyperthyroidism (20%), and cosmetic reasons (12%). The majority of patients (78%) underwent a surgical procedure with lobectomy or bilateral thyroidectomy; 13% had unilateral partial thyroidectomy. Prophylactic antibiotics were administered in 46% of procedures, and continued postoperatively in 12%. Postoperative complications occurred in 12% of patients. Hypocalcemia was the most common complication (8%), and its incidence was significantly correlated with the frequency of total thyroidectomy (p < 0.01). Complete pathology reports for the thyroidectomized specimens were available in only 65% of the cases. The frequency of cosmetic reasons for surgery, unilateral subtotal resection, routine antibiotic administration, and incomplete pathology reports were significantly higher in regional hospitals than in medical centers. CONCLUSIONS: Reduction in the high rates of cosmetic reasons for surgery, unilateral partial thyroidectomy, incomplete pathology reports, and use of antibiotic prophylaxis are needed to improve the quality of thyroidectomy in Taiwan.     

Recurrent acalculous cholecystitis and sclerosing cholangitis in a patient with X-linked hyper-immunoglobulin M syndrome.

X-linked hyper-immunoglobulin M (IgM) syndrome (XHIGM) is a rare genetic primary immunodeficiency disease caused by mutations of the CD40 ligand (CD40L) gene with normal or elevated levels of IgM and markedly decreased serum IgG, IgA, and IgE. Liver disease may occur as a clinical manifestation in XHIGM. This complication appears to increase with age. We report an 18-year-old male patient who had recurrent episodes of acalculous cholecystitis (AC) and sclerosing cholangitis (SC). The diagnosis of XHIGM was confirmed by the finding of CD40L expression < 1% of normal and a tyrosine 169 asparaginase (t526a) mutation in exon 5 (the tumor necrosis factor domain) of the CD40L gene. The patient had direct hyperbilirubinemia (direct bilirubin 5.5 mg/dL, total bilirubin 8.7 mg/dL), cholestasis (alkaline phosphatase 1133 U/L, gamma-glutamyl transferase 1019 U/L) and elevated transaminases (aspartate aminotransferase 70 U/L, alanine aminotransferase 101 U/L). Findings on abdominal ultrasound and abdominal computed tomography were compatible with AC. After the fourth episode of cholecystitis, cholecystectomy and liver biopsy were performed. Operative cholangiography revealed poor opacification of the hepatic duct and proximal common bile duct; the upstream intrahepatic bile ducts were not visualized. The biopsy specimen showed marked fibrosis of the portal areas. Enterococcus species was cultured from the bile. Children or adolescents with recurrent AC and SC should be evaluated for an underlying immunodeficiency syndrome such as XHIGM.     

SNAP-II predicts mortality among infants with congenital diaphragmatic hernia.

OBJECTIVE: Outcomes analysis in congenital diaphragmatic hernia (CDH) requires a validated risk-adjustment tool. The purpose of this study was to use the Canadian Neonatal Network (CNN) database to validate the Score for Neonatal Acute Physiology, Version II (SNAP-II) for prediction of mortality among CDH infants admitted to a neonatal intensive care unit (NICU), and to compare this to the predictive equation recently developed by the Congenital Diaphragmatic Hernia Study Group (CDHSG). STUDY DESIGN: Infants with CDH in the CNN database were identified. Bivariate and multivariable logistic regression models were used to identify risk factors predictive of mortality. Model predictive performance and calibration were assessed using the area under the receiver operator characteristic curve and the technique of Hosmer-Lemeshow, respectively, and compared with the CDHSG predictive equation. RESULTS: There were 88 patients with CDH among 19,507 admissions to CNN hospitals. The mortality rate among CDH patients surviving to NICU admission was 17%, and 12.5% received extracorporeal membrane oxygenation therapy. Gestational age and admission SNAP-II score predicted mortality. Model predictive performance and calibration were optimized with these variables combined. The CDHSG equation was equally predictive of mortality, but was only marginally calibrated. CONCLUSIONS: SNAP-II is highly predictive of mortality among patients with CDH, and can be used to risk-adjust these patients.