A 20-year-old female had undergone definitive surgical repair for pulmonary atresia with intact ventricular septum soon after birth. She was referred to our institution with the chief complaint of clubbing fingers. A thorough examination revealed platypnea-orthodeoxia syndrome due to an interatrial right-to-left shunt through a secundum atrial septal defect. Percutaneous closure with an Amplatzer Septal Occluder resulted in resolution of the syndrome.
Dyspnea/*diagnosis/*etiology/surgery ; Female ; Heart Defects, Congenital/complications/*surgery ; Heart Septal Defects, Atrial/*complications/*diagnosis/surgery ; Humans ; Hypoxia ; Pulmonary Atresia/complications/*surgery ; *Septal Occluder Device ; Syndrome ; Treatment Outcome

PURPOSE: Outcomes in 6 neonates with pulmonary atresia and intact ventricular septum (PAIVS) undergoing radiofrequency pulmonary valvulotomy and valvuloplasty (RPVV) were reported to identify the factors favorable for RPVV as the treatment of choice. MATERIALS AND METHODS: From May 2000 to January 2008, 6 patients with PAIVS were included in this retrospective study. They were aged 1 day to 90 days old. Study modalities included review of recordings of presentations and profiles of chest radiography, electrocardiography, echocardiography, and cardiac catheterization with angiography. Hemodynamic profiles from the echocardiography and the cardiac catheterization were analyzed. RESULTS: Echocardiography showed severe tricuspid regurgitation, membranous atresia of the pulmonary valve, intact ventricular septum, patent ductus arteriosus, and hypoplastic right ventricle in 6 patients. The pulmonary valve annulus were 4.2 to 6.9 mm in diameters, and those of the tricuspid valve were 7.1 to 10.1 mm. Elevated serum level of cardiac enzymes were found in 1 patient with ventriculocoronary communication (VCC). At cardiac catheterization, the ratio of systolic pressure of the right ventricle to that of the left ventricle ranged from 1.43 to 2.33 before RPVV, and from 0.54 to 1.15 after RPVV (p=0.027). The pressure gradients ranged from 76 to 136 mmHg before RPVV, and from 15 to 39 mmHg after RPVV (p=0.028). The echocardiographic gradients ranged from 16 to 32 mmHg within 24 hours after RPVV, and from 15 to 50 mmHg at the follow-ups. CONCLUSION: RPVV can be a treatment of choice for neonates with PAIVS, if there is patent infundibulum, no right-ventricular dependent coronary circulation, and adequate tricuspid valve and pulmonary valve.
Balloon Dilatation/*methods ; Echocardiography ; Female ; Heart Catheterization/*methods ; Hemodynamics ; Humans ; Infant, Newborn ; Male ; Pulmonary Atresia/*surgery ; Pulmonary Valve/abnormalities/*surgery ; Treatment Outcome ; *Ventricular Septum

OBJECTIVETo investigate the expression of EWS-FLI1/ERG fusion transcript resulting from t(11;12)(q24;12) in paraffin-embedded tissues and its diagnostic implication for Ewing's sarcoma/peripheral primitive neuroectodermal tumors (ES/pPNET).

METHODSOne-step reverse transcriptase-polymerase chain reaction (RT-PCR) was employed to detect a characteristic EWS-FLI1/ERG fusion transcript in 25 cases of ES/pPNET and 15 cases of other small round cell tumors (including 8 cases of rhabdomyosarcoma, 4 cases of synovial sarcoma, 2 cases of neuroblastoma and 1 case of lymphoma) using formalin-fixed and paraffin-embedded tissues.

RESULTSEWS-FLI1/ERG fusion transcript was detected in 20 of the 25 ES/pPNET cases (80%). The 15 non-ES/pPNET control cases were negative for EWS-FLI1/ERG fusion transcript.

CONCLUSIONSDetection of EWS-FLI1/ERG fusion transcript is a reliable index for molecular diagnosis of ES/pPNET. One-step RT-PCR is a practical method for such analysis in routine paraffin-embedded tumor tissues.

Adolescent ; Adult ; Diagnosis, Differential ; Female ; Humans ; Male ; Middle Aged ; Neuroectodermal Tumors, Primitive, Peripheral ; diagnosis ; metabolism ; Oncogene Proteins, Fusion ; metabolism ; Paraffin Embedding ; Proto-Oncogene Protein c-fli-1 ; RNA-Binding Protein EWS ; Reverse Transcriptase Polymerase Chain Reaction ; Rhabdomyosarcoma ; diagnosis ; metabolism ; Sarcoma, Ewing ; diagnosis ; metabolism ; Sarcoma, Synovial ; diagnosis ; metabolism ; Transcription Factors ; metabolism

PURPOSE: We reviewed the cases of 33 patients from our clinic and 142 patients from the literature with congenital bronchopulmonary vascular malformations (BPVM), systematically analyzed the bronchopulmonary airways, pulmonary arterial supplies, and pulmonary venous drainages, and classified these patients by pulmonary malinosculation (PM). MATERIALS AND METHODS: From January 1990 to January 2007, a total of 33 patients (17 men or boys and 16 women or girls), aged 1 day to 24 years (median, 2.5 months), with congenital BPVM were included in this study. Profiles of clinical manifestations, chest radiographs, echocardiographs, esophagographs, computer tomography (CT), magnetic resonance imaging (MRI), magnetic resonance angiography (MRA), cardiac catheterizations with angiography, contrast bronchographs, bronchoscopies, chromosomal studies, surgeries, and autopsies of these patients were analyzed to confirm the diagnosis of congenital BPVM. A total of 142 cases from the literature were also reviewed and classified similarly. RESULTS: The malformations of our 33 patients can be classified as type A isolated bronchial PM in 13 patients, type B isolated arterial PM in three, type C isolated venous PM in two, type D mixed bronchoarterial PM in five, type F mixed arteriovenous PM in one, and type G mixed bronchoarteriovenous PM in nine. CONCLUSION: Dysmorphogeneses of the primitive foregut system and the primitive aortic arch system may lead to haphazard malinosculations of the airways, arteries, and veins of the lung. A systematic classification of patients with congenital BPVM is clinically feasible by assessing the three basic bronchovascular systems of the lung independently.
Adolescent ; Adult ; Aorta, Thoracic/*abnormalities ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Infant, Newborn ; Intestines/*abnormalities/*blood supply ; Lung/*abnormalities/*blood supply ; Male ; Vascular Malformations/*classification

INTRODUCTION: Nursing home (NH) residents with out-of-hospital cardiac arrests (OHCA) have unique resuscitation priorities. This study aimed to describe OHCA characteristics in NH residents and identify independent predictors of survival. MATERIALS AND METHODS: OHCA cases between 2010-16 in the Pan-Asian Resuscitation Outcomes Study were retrospectively analysed. Patients aged <18 years old and non-emergency cases were excluded. Primary outcome was survival at discharge or 30 days. Good neurological outcome was defined as a cerebral performance score between 1-2. RESULTS: A total of 12,112 cases were included. Of these, 449 (3.7%) were NH residents who were older (median age 79 years, range 69-87 years) and more likely to have a history of stroke, heart and respiratory diseases. Fewer NH OHCA had presumed cardiac aetiology (62% vs 70%, <0.01) and initial shockable rhythm (8.9% vs 18%, <0.01), but had higher incidence of bystander cardiopulmonary resuscitation (74% vs 43%, <0.01) and defibrillator use (8.5% vs 2.8%, <0.01). Non-NH (2.8%) residents had better neurological outcomes than NH (0.9%) residents ( <0.05). Factors associated with survival for cardiac aetiology included age <65 years old, witnessed arrest, bystander defibrillator use and initial shockable rhythm; for non-cardiac aetiology, these included witnessed arrest (adjusted odds ratio [AOR] 3.8, <0.001) and initial shockable rhythm (AOR 5.7, <0.001). CONCLUSION Neurological outcomes were poorer in NH survivors of OHCA. These findings should inform health policies on termination of resuscitation, advance care directives and do-not-resuscitate orders in this population.

Objective To understand the current situation of soil-transmitted nematode infections and influencing factors of pupils in rural areas of Yi Ethnicity in Butuo County, Sichuan Province, so as to provide the evidence for the prevention and control of soil-transmitted nematode infections. Methods In 2016, the pupils of 2 villages in Butuo County were selected by the multi-stage random sampling method, and the relevant information was obtained by questionnaires, including social demographic information, diet and hygiene habits and acknowledge of parasitic disease prevention. The soil-transmitted nematode infections were examined by Kato-Katz technique. The results were statistically analyzed by the single factor analysis and multivariate logistic regression methods. Results A total of 160 pupils were investigated in this survey. The soil-transmitted nematodes included Ascaris lumbricoides, Trichuris trichiura and hookworm. The overall infection rate was 89.38%, and the multi-infection rate was 54.55%. The infection rates of A. lumbricoides and T. trichiura were higher. The infection densities were mild (75.68%) or moderate (22.97%). The logistic regression analysis showed that the protective factor of soil-transmitted nematode infections was the knowledge about correct diet and health habits notified by teachers (OR = 0.67), but the risk factor was drinking unboiled water (OR = 19.26). Conclusions The infection rate of nematodes is still high in the pupils in rural areas of Yi Ethnicity in Butuo County, Sichuan Province. Therefore, we should strengthen the prevention and control of soil-transmitted nematode infections, especially A. lumbricoides and T. trichiura infections, and mostly develop the teachers’function in health education.