Characteristics of Patients With Alpha-1 Antitrypsin Deficiency From Rural Appalachia: A Retrospective Single-Center Study.

Alpha-1 antitrypsin (AAT) deficiency, an autosomal co-dominant inherited condition, significantly impacts lung and liver functions, with mutations in the SERPINA1 gene, notably the Z allele, playing a pivotal role in disease susceptibility. This retrospective descriptive study from a rural Eastern Kentucky pulmonary clinic aimed to characterize patients with AAT deficiency, focusing on demographic, clinical, and laboratory parameters extracted from electronic health records (EHR) of Appalachian Regional Healthcare (ARH). Among 100 patient encounters, 56 were analyzed, revealing notable sex-based differences in smoking rates and co-existing conditions, with males showing higher rates of black lung and chronic obstructive pulmonary disease. In comparison, females exhibited higher rates of asthma, COVID-19, pneumothorax, and obstructive sleep apnea. The study emphasizes the importance of understanding genotype-phenotype correlations and demographic factors in assessing AAT deficiency, advocating for further research to refine management strategies and elucidate causal relationships.

Unexpected Barium Aspiration.

A barium esophagram is a diagnostic test used for the evaluation of dysphagia. However, this test has the potential risk for aspiration of the barium contrast. Barium aspiration typically localizes to the right lower lobe or left lingular lobe. We present a case of barium aspiration localized to the right middle lobe that persisted on chest X-ray. A 62-year-old male with a past medical history of hypertension, long-term back pain, gastritis, and anxiety presented with the complaints of hoarseness of voice, dysphagia, and weight loss for several months. During the esophagram, the patient aspirated the barium contrast. Chest X-ray confirmed the aspiration in the right middle lobe with a tree in bud appearance suggesting involvement of bronchioles. Three months later, a repeat chest X-ray revealed residual contrast. Pulmonary complications are directly related to the amount of aspirated barium and can include hypoxia, respiratory failure, secondary aspiration pneumonia, shock, and acute respiration distress syndrome. The prognosis of a barium aspiration is dependent on the amount of barium aspirated.

Chronic Pulmonary Aspergillosis Infection in Coal Workers Pneumoconiosis With Progressive Massive Fibrosis.

Pneumoconiosis is associated with coal dust particles depositing within the lung causing nodules coalesce to form progressive massive fibrosis (PMF). Cavitary lesions can develop in these PMF areas for concerns of tuberculosis and aspergillosis. We present a 59-year-old patient who had coal workers pneumoconiosis and PMF presenting with chronic dyspnea and hemoptysis with an upper cavitary lesion noted on chest imaging. He notes dyspnea with walking very short distances with associated productive cough. He admits to occasional wheezing, paroxysmal dyspnea, hemoptysis, and orthopnea but denies chest pain. He is an everyday smoker. His physical examination was only remarkable for bronchial breath sounds. On review of his prior imaging, he had a right upper lobe infiltrate as far back as 2012. As the years progressed, a new cavitary lesion developed in the PMF area which progressively got larger with a thick wall and no eccentric region noted inside the cavity. Tuberculosis test was negative. He underwent a transbronchial biopsy with methenamine silver stain which showed acute angle branching and septation suggestive of Aspergillus species. He was diagnosed with pulmonary aspergillosis and treated with voriconazole for 1 year. With pneumoconiosis and evidence confirming aspergillosis, the presence of a new lung infiltration with progression into a cavitary lesion leads to a diagnosis of chronic cavitary pulmonary aspergillosis (CCPA). With follow-up imaging showing extensive lung fibrosis, he had chronic fibrosing pulmonary aspergillosis (CFPA), a late-stage manifestation of CCPA.

From SARS to Avian Influenza Preparedness in Hong Kong.

The first human H5N1 case was diagnosed in Hong Kong in 1997. Since then, experience in effective preparedness strategies that target novel influenza viruses has expanded. Here, we report on avian influenza preparedness in public hospitals in Hong Kong to illustrate policies and practices associated with control of emerging infectious diseases. The Hong Kong government's risk-based preparedness plan for influenza pandemics includes 3 response levels for command, control, and coordination frameworks for territory-wide responses. The tiered levels of alert, serious, and emergency response enable early detection based on epidemiological exposure followed by initiation of a care bundle. Information technology, laboratory preparedness, clinical and public health management, and infection control preparedness provide a comprehensive and generalizable preparedness plan for emerging infectious diseases.

Computerized three-dimensional study of a rotavirus model of biliary atresia: comparison with human biliary atresia.

Biliary atresia is a panbiliary disease causing obstructive jaundice in neonates and infants. The clinical spectrum can be broadly categorized into the fetal and perinatal types. A consistent animal model that accurately mimics the whole clinical spectrum of biliary atresia is not yet available. However, rotavirus infection of neonatal mice has been shown to produce atresia in the biliary system. This study investigates the three-dimensional computerized morphology of the murine neonatal model comparing with age-matched control mice. Newborn Balb/c mice were injected intraperitoneally with rhesus rotavirus within 24-48 h after birth. Control mice received 0.9% NaCl. Pups with symptoms of cholestasis were sacrificed from the 5th to the 15th postinjection day, as were age-matched controls. Their hepatobiliary tissues were prepared for three-dimensional computerized image reconstruction. Rotavirus infection caused obliteration of the intrahepatic bile ducts and single to multiple atresias in the extrahepatic bile duct. At 15 days postinjection, intrahepatic ductal proliferation appeared, and the three-dimensional appearances of the intrahepatic biliary structures were similar to the human disease. Cystic duct and gallbladder dilatation was frequently seen in this model, and this feature distinguishes it from the human disease in which the gallbladder is almost always atretic. This rotavirus murine model demonstrates many of the features of human perinatal biliary atresia, and can be used as an investigative tool to further study the pathogenesis of biliary atresia.

Treatment of impending tumor lysis with single-dose rasburicase.

OBJECTIVE: To report the experience of using rasburicase as a single-dose treatment for childhood leukemia presenting with hyperuricemia. CASE SUMMARIES: Three children with acute lymphoblastic leukemia presenting with hyperuricemia received rasburicase as a single intravenous dose just prior to the start of chemotherapy. This was followed by rapid reduction of serum uric acid levels within 24 hours, which remained low throughout induction therapy while allopurinol and hydration therapy without urinary alkalinization ensued. Subclinical tumor lysis was evidenced by the appearance of hyperphosphatemia and hypocalcemia in all cases and hyperkalemia in 1 patient. These abnormalities were transient, and each patient's renal function gradually improved from pretreatment baseline without requiring dialysis. DISCUSSION: Our experience suggests that hyperuricemia in children at risk for tumor lysis can be managed with a briefer regimen of rasburicase than the recommended 5- to 7-day course. CONCLUSIONS: A shorter course of rasburicase treatment, including single-dose injection, is feasible and will improve the cost-effectiveness profile of the otherwise expensive compound.

Nurses' knowledge of and compliance with universal precautions in an acute care hospital.

A cross-sectional survey was conducted to investigate the nurses' knowledge of and compliance with Universal Precautions (UP) in an acute hospital in Hong Kong. A total of 450 nurses were randomly selected from a population of acute care nurses and 306 were successfully recruited in the study. The study revealed that the nurses' knowledge of UP was inadequate. In addition, UP was not only insufficiently and inappropriately applied, but also selectively practiced. Nearly all respondents knew that used needles should be disposed of in a sharps' box after injections. However, nurses had difficulty in distinguishing between deep body fluids and other general body secretions that are not considered infectious in UP. A high compliance was reported regarding hand-washing, disposal of needles and glove usage. However, the use of other protective wear such as masks and goggles was uncommon. The results also showed no significant relationships between the nurses' knowledge and compliance with UP. It is recommended that UP educational programmes need to consider attitudes in conjunction with empirical knowledge. Nurse managers and occupational health nurses should take a leadership role to ensure safe practices are used in the care of patients.