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INTRODUCTION: Biliary atresia is a major cause of extrahepatic obstructive jaundice in neonates. Early Kasai operation is the gold standard of treatment. In this study, we evaluated the effectiveness of stool color card screening by using claims data from the National Health Insurance Research Database. METHODS: This was a retrospective cohort study. Data from medical charts of all inpatients who were diagnosed with biliary atresia from 1996 to 2008 were collected from Taiwan's National Health Insurance Research Database. Patients who received a Kasai operation or liver transplant were identified by the Operation code. The patients' gender, age at admission, and type of operation were collected and analyzed. RESULTS: From 1996 to 2008, the overall incidence of biliary atresia was 1.48 per 10 000 live births. The median age at first admission for patients with suspected biliary atresia decreased after the implementation of stool color card screening (47 vs 43 days). The proportion of very late referral decreased from 9.5% to 4.9%. The median age of Kasai operation advanced from 51 to 48 days. The proportions of Kasai operation within 60 days of age were 68.9% before and 73.6% after screening program. CONCLUSION: Stool color card screening seemed to increase parents' and physicians' awareness of biliary atresia. It also was associated with a decline in the proportion of late referral. Thus, screening might be especially effective in areas with high a proportion of late referral. Improvements in the speed of workup and the operation room should be the focus of education and training in the future.
Superior mesenteric artery syndrome (SMAS) is an uncommon disease involving upper gastrointestinal obstruction. It has been postulated that a decrease in the angle between the superior mesenteric artery and the aorta results in occlusion of the third portion of the duodenum, especially in patients who lose body weight rapidly. We present a case of SMAS in a well-nourished, risk-free boy who was admitted owing to abdominal pain and vomiting after eating a large meal and suffered subsequent gastric perforation several hours later. Diagnostic evaluation revealed compression over the third portion of the duodenum by the SMA.
Primary extragonadal choriocarcinoma (PEGCC) in male is rare. It is highly malignant, typically presents with distant metastasis at the time of diagnosis, and responds poorly to treatment. Because of its associated high levels of PD-L1, the PD-1/PD-L1 pathway is a likely therapeutic target. Herein, we report our experience of treating pediatric PEGCC in six boys at a tertiary hospital.
Background The major burden of diseases in childhood has shifted from infectious diseases to chronic health conditions in recent decades. Although the rates of infectious diseases have decreased, the incidence of chronic diseases stemming from infectious agents continues to grow. Enterovirus is a major infectious disease of childhood and has been linked to numerous chronic diseases. We analyzed population-based data from Taiwan’s National Health Insurance Research Database (NHIRD) to investigate the correlations between enterovirus infection and major chronic health conditions in children. Methods Children diagnosed with enterovirus (EV) infection during 1999-2003 were identified from the Longitudinal Health Insurance Database 2000 (LHID 2000), a subdataset of Taiwan’s National Health Insurance Research Database (NHIRD). A total of 14,168 patients were selected after excluding patients with existing chronic diseases and missing data. Another 14,168 patients matched by age and sex were selected as the control group. Five primary outcomes, including attention deficit and hyperactivity disorder (ADHD), epilepsy, asthma, allergic rhinitis, and atopic dermatitis, were recorded. Results The risks of ADHD, asthma, allergic rhinitis, and epilepsy were significantly increased in the EV group compared with the control group. The risk of atopic dermatitis was significantly increased in the crude model. However, there were no significant differences in the adjusted model. The risks of ADHD, asthma, allergic rhinitis, and epilepsy were also significantly increased in patients with severe EV infection compared with patients with non-severe EV infection. [Is this what you mean? If you are just comparing patients with and without EV infection, you have already mentioned this finding in the first sentence of the results.] Conclusion Chronic diseases, such as ADHD, epilepsy, and allergic diseases were shown to be associated with enterovirus infection during childhood. EV infection during early childhood might have long-term public health implications and thus prevention strategies should be implemented.
Background: Thymic lymphoepithelioma-like carcinoma (LELC) is an extremely rare neoplasm in children. The causative role of Epstein-Barr virus (EBV) in the pediatric thymic LELC is unclear with limited treatment guidelines. Materials and methods: At our hospital, a 17-year old boy with thymic LELC was studied based on plasma levels of EBV DNA measured multiple times using real time quantitative polymerase chain reaction (RT-QPCR). For comparison, other patients <18 years old with thymic LELC reported in the English literature were also reviewed. Results: There were only 17 cases of pediatric thymic LELC in the literature. Including the present case, we analyzed a total of 18 such patients. The male to female ratio was 14:4, and the average age was 12.6 years old (range: 4 to 17). Of these 18 patients, the associations with EBV were not determined in 6 due to incomplete data, and the remaining 12 were all EBV-related. The mortality rate of pediatric thymic LELC was 68.8%, with an average survival time of 12.6 months after diagnosis. The plasma level of EBV RT-QPCR of the patient was 77048 copies/ml before chemotherapy. As his expression of PD-L1/PD-1 was 70%, pembrolizumab (2 mg/kg for one day) was also given. After 6 cycles of the above treatments, his CT scan showed a reduction in tumor size of >50% at the mediastinum. Conclusion: Despite limited data and our single case, we are inclined to conclude that children with thymic LELC likely associate with EBV pathogenicity. Immunotherapy may be useful in improving the prognosis.
Refractory/relapsed acute myeloid leukemia is an advanced hematological malignancy with a poor prognosis in pediatric. The therapeutic options are limited and has been slow development of therapies for this age group. A combination of Venetoclax and Azacitidine therapy in newly diagnosed elderly patients with acute myeloid leukemia and refractory/relapsed disease has achieved impressive results. Whether similar results can be attained in pediatric patients is unknown. We present a case of adolescent girl with acute myeloid leukemia secondary to myelodysplastic syndrome, who was deemed unfit to undergo conventional chemotherapy and, was treated instead with the Venetoclax and Azacitidine.
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