CLINICAL PRESENTATION: This report describes a case of a 24-year-old, acyanotic, female patient presenting with mild effort dyspnea, a right ventricular heave, a displaced cardiac apex, a loud single S2, a holosystolic murmur on the left parasternal area, and a continuous murmur at the left posterior chest. Transthoracic and transesophageal echocardiography demonstrated dilated left atrium and ventricle, hypertrophied right ventricle and a large ventricular septal defect with overriding of the aorta. However, both studies failed to visualize the pulmonic valve. The patient underwent cardiac catheterization which revealed the presence of pulmonary atresia. A descending aortogram showed the presence of a single large major aortopulmonary collateral artery (MAPCA) arising from the descending thoracic aor ta which supplies confluent left and right pulmonary arteries. The pulmonary arterial vasculature is enlarged and pruned in appearance. The main pulmonary artery is non-atretic. Mean proximal pulmonary artery pressure was 68 mmHg, indicating severe pulmonary hypertension. Patient wasstarted on digoxin and sildenafil.
SIGNIFICANCE: This case report emphasizes the importance of a well-developed central pulmonary circulation and an adequate but not excessive pulmonary blood supply from an aortopulmonary vascular connection in the survival of patients with pulmonary atresia into adulthood.

Human ; Female ; Adult ; Young Adult ; Aorta ; Cardiac Catheterization ; Dyspnea ; Heart Atria ; Heart Septal Defects, Ventricular ; Hypertension, Pulmonary ; Pulmonary Atresia ; Pulmonary Circulation ; Pulmonary Valve ; Tetralogy Of Fallot

Hemoglobin Bart's hydrops fetalis, characterized by a deletion of all four a-globin genes is the most severe and lethal form of Thalassemia disease. Mortality rate usually ranges from 60-100% of cases. Given the poor overall prognosis, most countries resort to pregnancy termination or expectant management as the only options to offer affected pregnancies.
This paper presents a case of the successful management of a primigravid, diagnosed with hydrops fetalis at 29 4/7 weeks age of gestation. She delivered successfully to a live, preterm, baby boy who was later found out to have hydrops fetalis due to Hemoglobin Bart's disease, and currently, continues to thrive past eight months of age.
This report aims to improve the clinicians' knowledge regarding the work up and management of pregnant patients diagnosed with hydrops fetalis, and increase the clinician's awareness on the epidemiology, importance of targeted screening, and diagnosis of Alpha-Thalassemia in Filipino patients.

Human ; Female ; Adult ; Alpha-thalassemia ; Hydrops Fetalis ; Hemoglobin Bart's ; Hemoglobin, Sickle ; Anemia, Sickle Cell ; Prognosis

INTRODUCTION: Amputation is often associated with depression, isolation and anxiety, resulting in changes
in the functioning and quality of life of an individual. This study aimed to compare the quality of life of
amputees before and after prosthetic rehabilitation and to determine the level of satisfaction with the
services provided by the rehabilitation team.

METHODS: This was a longitudinal study conducted at the Philippine School of Prosthetics and Orthotics
from 2016 to 2017. Quality of life was measured at baseline and at the end of prosthetic rehabilitation
using the Short Form-36 version 2 Philippines (Tagalog) and compared. Patient satisfaction was determined
using the UERMMMCI Out-Patient Satisfaction Survey. Statistical analysis was done using paired t-test
and stratified analysis.

RESULTS: Twelve participants were included; majority were adult males and all were unemployed. There
were no statistically significant changes in the quality of life of amputees after prosthetic rehabilitation.
Stratification analysis revealed significant decreases in scores in bodily pain and general health scales for
females. On the other hand, males had statistically significant increases in vitality scores. Participants
were generally satisfied with the services they received.

CONCLUSION: While no significant changes were observed for overall quality of life, certain aspects may be
related to factors such as sex, comorbidities, and type of caregiver. Recommendations include further studies
to explore other factors affecting quality of life among Filipino amputees after prosthetic rehabilitation.

Human ; Amputees ; Prostheses And Implants

DNA mismatch repair gene mutS (2.56 kb) was PCR modified and cloned into a secretive prokaryotic expression vector pET32a (+) which carries a N-terminal His.tag + and thioredoxin sequence. MutS protein was expressed with high level after IPTG induction using the strain E. coli AD494(DE3). SDS-PAGE revealed that the expected protein with a molecular weight of 108 kD which is about 35% of the total bacterial proteins is almost soluble. The expected protein was purified directly by immobilized metal (Ni2+) chelation affinity chromatography and the purity is over 90%. MutS protein activity verified using mismatch DNA showed that the expression product can recognize and bind to base-pair mismatch specifically.
Adenosine Triphosphatases ; biosynthesis ; genetics ; isolation & purification ; Bacterial Proteins ; Base Pair Mismatch ; Chromatography, Affinity ; DNA ; metabolism ; DNA Repair ; DNA-Binding Proteins ; Escherichia coli Proteins ; biosynthesis ; genetics ; isolation & purification ; Magnesium ; pharmacology ; Molecular Weight ; MutS DNA Mismatch-Binding Protein ; Recombinant Proteins ; biosynthesis

Background and Objective: Comparable data are sparse for infl ammatory demyelinating diseases of the central nervous system (CNS) in the Asia-Pacifi c region, and we aimed to establish a registry of patients with such diseases in the region. Methods: A network of neurologists in the Asia-Pacifi c Region was established to register patients with the targeted diseases. A standardized register form and relevant instructions in English, translated into the local language when needed, were prepared before the study start and used for data collection. Results: Eight study centres from different countries/areas participated in the study. In total, 857 patients with a validated diagnosis of different infl ammatory demyelinating diseases of the CNS were registered, 591 females and 266 males with a female-to-male ratio 2.2. The mean age at onset for all patients was 35.9 (SD: 12.9) years, signifi cantly younger (p = 0.010) for females (35.1 years, SD: 12.6 years) than for males (37.6 years, SD: 13.4 years). Conclusion: Patients with different infl ammatory demyelinating diseases of the CNS were in the fi rst time registered in a multi-centre study from eight countries/areas in the Asia-Pacifi c region. A platform and basis has been established for further study in the fi eld.

Cochlear hair cells convert sound into electrical signals that are relayed via the spiral ganglion neurons to the central auditory pathway. Hair cells are vulnerable to damage caused by excessive noise, aging, and ototoxic agents. Non-mammals can regenerate lost hair cells by mitotic regeneration and direct transdifferentiation of surrounding supporting cells. However, in mature mammals, damaged hair cells are not replaced, resulting in permanent hearing loss. Recent studies have uncovered mechanisms by which sensory organs in non-mammals and the neonatal mammalian cochlea regenerate hair cells, and outlined possible mechanisms why this ability declines rapidly with age in mammals. Here, we review similarities and differences between avian, zebrafish, and mammalian hair cell regeneration. Moreover, we discuss advances and limitations of hair cell regeneration in the mature cochlea and their potential applications to human hearing loss.

PURPOSE: The purpose of this study was to understand patient treatment patterns, outcomes, and healthcare resource use in cases of metastatic and/or locally recurrent, unresectable gastric cancer (MGC) in South Korea. MATERIALS AND METHODS: Thirty physicians reviewed charts of eligible patients to collect de-identified data. Patients must have received platinum/fluoropyrimidine first-line therapy followed by second-line therapy or best supportive care, had no other primary cancer, and not participated in a clinical trial following MGC diagnosis. Data were summarized using descriptive statistics. Kaplan-Meier analysis was used to describe survival. RESULTS: Of 198 patients, 73.7% were male, 78.3% were diagnosed with MGC after age 55 (mean, 61.3 years), and 47.0% were current or former smokers. The majority of tumorswere located in the antrum/pylorus (51.5%). Metastatic sites most often occurred in the peritoneum (53.5%), lymph nodes (47.5%), and liver (38.9%). At diagnosis, the mean Charlson comorbidity indexwas 0.4 (standard deviation, 0.6). The most common comorbidities were chronic gastritis (22.7%) and cardiovascular disease (18.7%). Most patients (80.3%) received second-line treatment. Single-agent fluoropyrimidine was reported for 22.0% of patients, while 19.5% were treated with irinotecan and a fluoropyrimidine or platinum agent. The most common physician-reported symptoms during second-line treatment were nausea/vomiting (44.7%) and pain (11.3%), with antiemetics (44.7%), analgesics (36.5%), and nutritional support (11.3%) most often used as supportive care. Two-thirds of inpatient hospitalizations were for chemotherapy infusion. Outpatient hospitalization (31.6%) and visits to the oncologist (58.8%) were common among second-line patients. CONCLUSION: Most patients received second-line treatment, although regimens varied. Understanding MGC patient characteristics and treatment patterns in South Korea will help address unmet needs.
Analgesics ; Antiemetics ; Cardiovascular Diseases ; Comorbidity ; Delivery of Health Care ; Diagnosis ; Drug Therapy ; Gastritis ; Hospitalization ; Humans ; Inpatients ; Kaplan-Meier Estimate ; Korea* ; Liver ; Lymph Nodes ; Male ; Nutritional Support ; Observational Study ; Outpatients ; Peritoneum ; Platinum ; Republic of Korea ; Stomach Neoplasms*

OBJECTIVETo examine the prevalence and characteristics of aortic arch calcification (AAC) in residents aged 50 or over in Guangzhou, China.

METHODSFace-to-face interview, physical examination and laboratory tests were conducted on 10 413 Chinese adults aged 50 or over. Posterior-anterior plain chest X-ray radiographs were obtained from 10 305 subjects using a Toshiba KSO-15R machine. The radiographs were reviewed together by two radiologists while 300 radiographs were independently gone through by two radiologists to assess the agreement with Kappa coefficient method.

RESULTSThe rate of agreement on Diagnosis for the two radiologists was 85.0% and Kappa coefficient was 0.68, with P < 0.001 which showed a moderate agreement between the two radiologists. Among the 10 305 subjects, there were 3064 men and 7349 women, with their mean age (+/- standard deviation) as 64.0 +/- 6.0 and 66.2 +/- 5.8, respectively. Most of them had educational level of middle school or below, and most of their occupations were factory or agricultural workers. The prevalence of AAC was 40.6%. Women showed significantly higher prevalence rate than men (41.4% versus 38.6%, P < 0.001) and the prevalence of AAC increased significantly with age. Subjects with primary educational level or below had the highest prevalence of AAC. There was no significant association found between occupation and AAC prevalence. 98.7% of the subjects with AAC occurred in aortic arch. Most AAC had a length of 10 mm or longer and a width from 1-4 mm, which indicated the severity of AAC among the subjects.

CONCLUSIONThe prevalence of AAC among Guangzhou Biobank Cohort was about 40.6%, higher than those reported in foreign studies, while most of the lesions were quite serious.

Aged ; Aorta, Thoracic ; pathology ; Calcinosis ; epidemiology ; Cardiomyopathies ; epidemiology ; China ; epidemiology ; Female ; Humans ; Male ; Middle Aged ; Prevalence

No abstract available.
Adrenal Glands* ; Eosinophils* ; Leiomyosarcoma*