Pan-Genotypic Direct-Acting Antiviral Agents for Undetermined or Mixed-Genotype Hepatitis C Infection: A Real-World Multi-Center Effectiveness Analysis.

Although the pan-genotypic direct-acting antiviral regimen was approved for treating chronic hepatitis C infection regardless of the hepatitis C virus (HCV) genotype, real-world data on its effectiveness against mixed-genotype or genotype-undetermined HCV infection are scarce. We evaluated the real-world safety and efficacy of two pan-genotypic regimens (Glecaprevir/Pibrentasvir and Sofosbuvir/Velpatasvir) for HCV-infected patients with mixed or undetermined HCV genotypes from the five hospitals in the Changhua Christian Care System that commenced treatment between August 2018 and December 2020. This retrospective study evaluated the efficacy and safety of pan-genotypic direct-acting antiviral (DAA) treatment in adults with HCV infection. The primary endpoint was the sustained virological response (SVR) observed 12 weeks after completing the treatment. Altogether, 2446 HCV-infected patients received the pan-genotypic DAA regimen, 37 (1.5%) patients had mixed-genotype HCV infections and 110 (4.5%) patients had undetermined HCV genotypes. The mean age was 63 years and 55.8% of our participants were males. Nine (6.1%) patients had end-stage renal disease and three (2%) had co-existing hepatomas. We lost one patient to follow-up during treatment and one more patient after treatment. A total of four patients died. However, none of these losses were due to treatment-related side effects. The rates of SVR12 for mixed-genotype and genotype-undetermined infections were 97.1% and 96.2%, respectively, by per-protocol analyses, and 91.9% and 92.7% respectively, by intention-to-treat population analyses. Laboratory adverse events with grades ≥3 included anemia (2.5%), thrombocytopenia (2.5%), and jaundice (0.7%). Pan-genotypic DAAs are effective and well-tolerated for mixed-genotype or genotype-undetermined HCV infection real-world settings.

Real-World Experience of Chronic Hepatitis C-Related Compensated Liver Cirrhosis Treated with Glecaprevir/Pibrentasvir: A Multicenter Retrospective Study.

BACKGROUND: Glecaprevir/pibrentasvir is a protease inhibitor-containing pangenotypic direct-acting antiviral regimen that has been approved for the treatment of chronic hepatitis C. The present study aimed to evaluate the safety and efficacy of glecaprevir/pibrentasvir in patients with compensated cirrhosis in a real-world setting. METHODS: We evaluated the real-world safety and efficacy of glecaprevir/pibrentasvir in patients with compensated cirrhosis from five hospitals in the Changhua Christian Care System, who underwent treatment between August 2018 and October 2020. The primary endpoint was a sustained virological response observed 12 weeks after completion of the treatment. RESULTS: Ninety patients, including 70 patients who received the 12-week therapy and 20 patients who received the 8-week therapy, were enrolled. The mean age of the patients was 65 years, and 57.8% of the patients were males. Sixteen (17.8%) patients had end-stage renal disease, and 15 (16.7%) had co-existing hepatoma. The hepatitis C virus genotypes 1 (40%) and 2 (35.6%) were most common. The common side effects included anorexia (12.2%), pruritus (7.8%), abdominal discomfort (7.8%), and malaise (7.8%). Laboratory adverse grade ≥3 events included anemia (6.3%), thrombocytopenia (5.1%), and jaundice (2.2%). The overall sustained virological response rates were 94.4% and 97.7% in the intention-to-treat and per-protocol analyses, respectively. CONCLUSIONS: the glecaprevir/pibrentasvir treatment regimen was highly effective and well tolerated among patients with compensated cirrhosis in the real-world setting.

Coil embolization of pulmonary sequestration in two infants--A safe alternative management.

Pulmonary sequestration is a rare congenital anomaly with nonfunctioning lung tissue, for which the arterial blood supply is usually derived from the thoracic or abdominal aorta. Surgical resection is the conventional treatment for pulmonary sequestration. The arterial embolization of pulmonary sequestration is an alternative technique and a less invasive treatment than conventional surgical resection. Here, we report two infants with intralobar and extralobar types of pulmonary sequestration, successfully treated with coil embolization of the feeding artery without any complications.

Postoperative chylothorax: differences between patients who received median sternotomy or lateral thoracotomy for congenital heart disease.

BACKGROUND: Chylothorax after surgery for congenital heart disease is rare. We wanted to compare the different presentations of chylothorax in patients who received median sternotomy or lateral thoracotomy. PATIENTS AND METHODS: We retrospectively studied pediatric patients with congenital heart disease who received palliative or corrective surgeries and developed postoperative chylothorax between January 1992 and July 2003. Patients were divided into two groups by the type of surgery: median sternotomy and lateral thoracotomy. The average daily fluid amount (mL/kg/24 hours), latency period, duration of chylothorax, and requirement for surgery were compared. RESULTS: Seventeen patients (11 boys, 6 girls; mean age, 14.0 +/- 12.8-month-old) were enrolled. Diagnoses were tetralogy of Fallot (n = 8), right isomerism with complex heart defects (n = 3), patent ductus arteriosus (n = 2), transposition of the great artery (n = 1), ventricular septal defect (n = 1), and endocardial cushion defect (n = 2). There were 9 in the lateral thoracotomy group and 8 in the median sternotomy group. Only one patient required surgery because of the failure of conservative treatment. The lateral thoracotomy group had a significantly lower average body weight (6.9 +/- 2.9 kg vs. 11.0 +/- 3.8 kg) and longer average latency period before postoperative chylothorax (15.1 +/- 9.2 days vs. 7.2 +/- 4.7 days). CONCLUSION: The majority of pediatric patients who develop chylothorax after cardiac surgery can be successfully managed by medical treatment only. To avoid complications in pediatric patients after cardiac surgery, chylothorax should be suspected for patients with unexplainable, prolonged, and abundant pleural effusion.

Critical management in patients with severe enterovirus 71 infection.

OBJECTIVE: The aim of this study was to analyze clinical details occurring in children with severe enterovirus 71 (EV71) infection and synthesize the critical care experience for patients with severe EV71 infection. METHODS: A retrospective clinical, laboratory, and hemodynamic study was performed in a pediatric intensive care unit in a university hospital. From March 1998 to April 2000, seven consecutive pediatric patients with severe EV71 infection were retrospectively analyzed as the comparison group. From May 2000 to March 2003, eight consecutive patients with severe EV71 infection who had received the protocol therapy were enrolled as the study group. Detailed information about clinical treatment and pharmacological therapy was collected for comparison. RESULTS: The clinical presentations and laboratory findings between the comparison and the study groups were not significantly different. The amount of intravenous fluid in the first 24 h was significantly higher in the comparison group (9.2+/-5.0 vs 4.9+/-1.3 mL/kg per h). More patients in the study group received low doses of dopamine infusion, patients in the comparison group received more epinephrine, and none of them received milrinone. The acute-stage and long-term survival rates were higher in the study group (100% vs 43%, 87% vs 29%). CONCLUSION: Early cardiopulmonary support may prevent the vicious cycle of cardiopulmonary failure and improve the clinical outcome of severe EV71 infection. Milrinone may be the ideal inotropic agent for these patients. Echocardiography, a central line, and an arterial line could be an alternate method to replace direct intracardiac hemodynamic monitoring for guiding critical management.

Isolated anomalous origin of right coronary artery from the main pulmonary artery.

The anomalous origin of the right coronary artery (ARCA) from the main pulmonary artery (MPA) is a rare congenital cardiac malformation and usually associated with other cardiac anomalies. Most patients with isolated ARCA from MPA remain asymptomatic, but they may develop myocardial ischemia and even sudden death. We reported an asymptomatic 7-year-old boy referred for evaluation of a heart murmur. Isolation of ARCA from MPA was diagnosed by echocardiography and then confirmed by cardiac catheterization and angiography. The right coronary artery was re-implanted into the ascending aorta. A preoperative thallium-201 myocardial perfusion showed a myocardial ischemia pattern in the anterolateral septal area after a dipyridamole stress test; the ischemia was completely resolved after surgery.

Prediction of outcome in infants with congenital diaphragmatic hernia or severe diaphragmatic eventration.

Congenital diaphragmatic hernia (CDH) and severe congenital diaphragmatic eventration (SDE) still have high mortality. Our aims were to identify clinical prognostic factors for CDH and SDE, and to determine whether the size or area of the proximal bilateral pulmonary arteries (PA) correlate with the clinical outcome. We retrospectively analyzed medical charts of 26 patients--20 with CDH and 6 with SDE, but no obvious other associated anomalies--admitted over a 12-year period. We compared prenatal history, clinical manifestations, blood gas, and echocardiography before surgery in the survivors and the non-survivors. Ten patients (8 CDH, 2 SDE) died 2 to 16 days after birth, including 2 patients without surgery due to progressive hypoxemia. The survivors had significantly higher 1- and 5-min Apgar scores, higher the worst preductal arterial blood gas pH levels, lower oxygen indices, and lower PaCO2 (P < 0.05). The McGoon index of PA size measured by echocardiography was higher in survivors, but not statistically significant. Nakada PA index results, however, were statistically significant (93.07 +/- 32.02 vs. 121.07 +/- 27.08, P < 0.05) In conclusion, Apgar scores, preductal PaCO2, oxygen index, and pH level can predict prognosis in infants with CDH and SDE. The Nakada PA index, however, might be a useful prognostic marker for patients with CDH and SDE.

Successful management of a neonate with Pierre-Robin syndrome and severe upper airway obstruction by long term placement of a laryngeal mask airway.

The severity of airway obstruction varies in infants with Pierre-Robin syndrome (PRS). Some have severe upper airway obstruction that results in respiratory failure and even death. We report a case of neonate with isolated PRS who had a severe airway obstruction and respiratory failure after birth. She had complications of bilateral pneumothorax, subcutaneous emphysema, and hypoxaemia due to difficult tracheal intubation. Respiratory failure recurred immediately after extubation; she was resuscitated by inserting a laryngeal mask airway. The laryngeal mask airway was left inserted for 6 days. It was successful in this patient and eliminated the need for invasive surgical procedures. In conclusion, the relatively long term use of a laryngeal mask airway, which has not been reported before, could be an alternative therapy for patients with PRS with airway obstruction.

Treatment of complicated parapneumonic pleural effusion with intrapleural streptokinase in children.

OBJECTIVE: To evaluate the role of intrapleural streptokinase in the management of complicated parapneumonic effusions in children. DESIGN: Prospective comparative study. SETTING: Cheng Kung University Hospital, a tertiary medical center in Tainan, Taiwan. PATIENTS AND METHODS: We enrolled as our prospective study group 20 consecutive children with complicated parapneumonic effusions who received intrapleural streptokinase treatment between August 2000 and July 2002. We also retrospectively analyzed a comparison group of 22 consecutive children with complicated parapneumonic effusions who received chest tube drainage without streptokinase treatment from January 1992 to July 2000. We then compared the clinical manifestations and outcome of these two patient groups. The patient population (21 boys and 21 girls) ranged in age from 9 to 130 months (mean age, 41.5 +/- 26.3 months [mean +/- SD]). The characters of pleural effusion showed no difference between the two groups. Nineteen patients had positive findings for Streptococcus pneumoniae, 2 patients had positive findings for Staphylococcus aureus, 2 patients had positive findings for Pseudomonas aeruginosa, and 19 patients had undetermined pathogens. All patients were treated with appropriate antibiotics. RESULTS: More pleural fluid was drained from the streptokinase group than from the comparison group during streptokinase treatment (816 +/- 481 mL vs 279 +/- 238 mL, p < 0.01). The duration of fever after chest tube insertion was also significantly lower in the study group (5.3 +/- 3.1 days vs 7.9 +/- 4.6 days, p < 0.05). Only two patients in the streptokinase group required surgical intervention compared with nine patients in the comparison group (p < 0.05). No major side effects were noticed after streptokinase instillation. CONCLUSION: Intrapleural fibrinolytic treatment with streptokinase is safe and effective, and it can obviate the need for surgery in most cases. The combination treatment should be attempted early on, when complicated parapneumonic effusion is first diagnosed.

Cardiac tumors in infants and children.

Cardiac tumors in infants and children are extremely rare. Their clinical manifestations vary widely from asymptomatic presentations to life-threatening cardiac events. Improvements in diagnostic techniques, such as those offered by echocardiography, have made early detection of cardiac masses possible, with or without the presence of clinical symptoms. Fifteen pediatric cases of cardiac tumor were diagnosed at our institution between July 1989 and July 2002 (male-female ratio, 10:5; age range, one day to nine years). Eleven of the cases involved primary cardiac tumors [rhabdomyoma (n = 10) and fibroma (n = 1)]. Ninety percent of the rhabdomyomas (9/10) were associated with tuberous sclerosis. Four of the fifteen cases were secondary metastatic tumors [hepatoblastoma (n = 2), hepatoma (n = 1) and rhabdomyosarcoma (n = 1)]. Clinical manifestations of the cardiac tumors included shortness of breath (n = 5), seizure (n = 4), cardiac murmur (n = 6), and cyanosis (n = 3). Surgery was performed for three of 11 patients with primary cardiac tumor (27%) due to severe obstruction of flow (n = 2) and other cardiac defects (n = 1). The primary cardiac tumor spontaneously regressed in five of the tuberous sclerosis patients. All four of the patients with secondary cardiac tumors continued to receive chemotherapy, and only one of them subsequently experienced regression. Based on our experiences, we conclude that: 1) rhabdomyoma is the most common primary cardiac tumor in children; 2) most pediatric tumors are associated with tuberous sclerosis; 3) clinical presentation is determined by the tumor size and number of tumors, and whether expansion of the malignancy has resulted in cardiac blood-flow obstruction; 4) there is a strong possibility of regression of the primary cardiac tumor, with surgery recommended only when cardiac symptoms are severe; and, 5) unless there is a significant obstruction of blood flow, chemotherapy is still the treatment of choice for secondary cardiac tumors.

Congenital long QT syndrome with functionally impaired atrioventricular conduction: successful treatment by mexiletine and propranolol.

Congenital long QT syndrome (LQTS) with atrioventricular block is a rare and malignant arrhythmia, which usually responds poorly to traditional beta-blocker therapy. We describe a male neonate with LQTS with ventricular tachycardia and 2:1 atrioventricular block. This patient's sister had similar presentation and died suddenly at the age of 8 months despite beta-blocker and pacemaker therapy. Our patient responded to a combination of sodium channel blocker (mexiletine) and beta-blocker (propranolol) therapy. He was asymptomatic during a 2-year follow-up period. This case suggests that propranolol combined with mexiletine might be useful in the treatment of patients with LQTS with atrioventricular block.

Diverticulum of the right ventricle associated with pulmonary stenosis, rhabdomyoma and Wolff-Parkinson-White syndrome.

Congenital cardiac diverticulum is a rare anomaly that may present as an isolated lesion or in association with other malformations. Diverticulum of the left ventricle is more common than that of the right ventricle. We report a case of cardiac diverticulum over the right ventricular outflow tract and associated pulmonary stenosis, right atrial rhabdomyoma, and Wolff-Parkinson-White syndrome in a 9-month-old boy. The delta wave disappeared after removal of the atrial rhabdomyoma.