Ali Ataya

Titanium exposure and yellow nail syndrome

Yellow nail syndrome is a rare disease of unclear etiology. We describe a patient who develops yellow nail syndrome, with primary nail and sinus manifestations, shortly after amalgam dental implants. A study of the patients nail shedding showed elevated nail titanium levels. The patient had her dental implants removed and had complete resolution of her sinus symptoms with no change in her nail findings. Since the patients nail findings did not resolve we do not believe titanium exposure is a cause of her yellow nail syndrome but perhaps a possible relationship exists between titanium exposure and yellow nail syndrome that requires further studies.

A Rare Case of Raoultella planticola Pneumonia: An Emerging Pathogen

Raoultella planticola (R. planticola), considered an environmental organism, is a gram negative, motile, bacillus with phenotypic similarities to the genus Klebsiella. The organism remains a rare cause of human infection with a few cases reported in the literature. However, since its description in 1981 there have been increasing rates of infections caused by R. planticola with reports of conjunctivitis, liver abscess, cholangitis, pancreatitis, and necrotizing fasciitis. More concerning are reports of carbapenemase-producing isolates which have led to the only 2 mortalities associated with R. planticola infections. To our knowledge, we report the third case of R. planticola pneumonia in an immunocompromised patient with no known direct exposure to the reported risk factors.

Pulmonary arterial hypertension and acute respiratory distress syndrome in a patient with adult-onset stills disease:

Adult-onset Still’s disease (AOSD) is an inflammatory disorder characterized by recurrent fevers, arthralgia, leukocytosis, and a salmon-colored rash. Diagnosis is made based on the Yamaguchi criteria. Various cardiac and pulmonary manifestations have been described in association with AOSD, including acute respiratory distress syndrome (ARDS) and pulmonary arterial hypertension (PAH). We describe the first case of both PAH and ARDS in a patient with AOSD who, despite aggressive therapy, declined rapidly and ultimately died. There was concern for pulmonary veno-occlusive disease given the rate of her decompensation, but this was found not to be the case on autopsy. Treatment of AOSD with cardiopulmonary involvement requires rapid identification of AOSD followed by aggressive immunosuppression.

A Review of Targeted Pulmonary Arterial Hypertension-Specific Pharmacotherapy

Significant advances in the understanding of the pathophysiology of pulmonary arterial hypertension over the past two decades have led to the development of targeted therapies and improved patient outcomes. Currently, a broad armamentarium of pulmonary arterial hypertension-specific drugs exists to assist in the treatment of this complex disease state. In this manuscript, we provide a comprehensive review of the current Food and Drug Administration (FDA)-approved pulmonary arterial hypertension-specific therapies, and their supporting evidence for adults, targeting the nitric oxide, soluble guanylate cyclase, endothelin, and prostacyclin pathways.

Images in clinical medicine. Hemichoreoathetosis in neurosarcoidosis.

A 39-year-old black man presented with progressively worsening involuntary movements of the left arm that had been sudden in onset. A physical examination revealed chorea of the left upper arm and athetosis of the fingers of the left hand, shown in a video.

Adult Kawasaki disease

A 44-year-old male presented with 1 h history of substernal, persistent pressure-like chest pain. The pain had no radiation and started while he was watching TV. He denied associated nausea, vomiting, palpitations or shortness of breath. He has history of hypertension controlled with irbesartan. He never smoked or drank alcohol. Exam showed normal vital signs; cardiovascular examination showed regular rate and rhythm, no gallops, murmurs or pericardial rub, had symmetric and bounding distal pulses. Electrocardiogram showed normal sinus rhythm with no ST or T wave changes, and with normal intervals. Chest X-ray was normal. Laboratory studies showed elevated cardiac …

Transudative pleural effusion of malignant etiology: Rare but real

A 62-year-old female presented to the emergency room with one-month history of epigastric abdominal pain, nausea and vomiting. She endorsed progressive dyspnea over two weeks. CT of the abdomen demonstrated bilateral pleural effusions and pancreatic inflammation, so the working diagnosis was pancreatitis. A diagnostic thoracentesis was performed and the pleural fluid analysis was classified as transudate by Lights criteria. Given the atypical features in history and concern for malignancy, fluid was sent for cytological examination and immunohistochemistry which suggested a mucinous malignancy. EGD revealed poorly differentiated signet ring cell adenocarcinoma of stomach. Patient underwent placement of indwelling pleural catheters for symptomatic improvement and was discharged to hospice. The decision whether to routinely send transudative effusions for cytological evaluation remains controversial. This case demonstrates the importance of using clinical judgement to guide that decision.

Alpha1-antitrypsin Deficiency—Increased Knowledge and Diagnostic Testing after Viewing Short Instructional Video

ABSTRACT Many individuals with Alpha-1 Antitrypsin Deficiency (AATD) are unaware of their diagnosis. In the absence of an AATD diagnosis, irreversible damage continues, and incorrect care is provided. Research demonstrates low levels of knowledge about AATD among health care providers. To address this ongoing issue, a short educational video was developed for health care providers with the goal of increasing knowledge and testing for AATD. A five-question test on the video material was developed. Invitations to participate in the study were sent via email to providers at both public teaching hospitals and private practices across the country. Respondents completed three parts online: pre-test, video, and post-test. To confirm retention of knowledge gained, providers who completed all three were invited to take the same test 3–6 months later. There were 683 providers who responded, and 213 completed all three portions; 105 of those providers completed the 3–6-months of follow-up testing. The average pre-test score of the 213 providers was 54.6% (std. dev. = 26.2%). The average post-test score immediately following the video viewing was 74.7% (std. dev. = 27.7%). The average follow-up test score 3–6 months later was 63.2% (std. dev. = 22.0%). During the follow-up period, 11 providers reported testing for AATD for the first time. This short educational video demonstrated both immediate and sustained improvement in knowledge and an increase in testing for AATD. Short digital videos may provide an effective platform for the ongoing effort to identify individuals with AATD.

Pulmonary arterial hypertension and associated conditions

Pulmonary hypertension (PH) refers to an increase in pulmonary arterial pressure, defined as mean pulmonary artery pressure (mPAP) Z25 mmHg at rest, that if left untreated may progress to right ventricle dysfunction and failure. Worldwide, pulmonary hypertension associated with schistosomiasis is considered the most common form of the disease. In the United States, postcapillary pulmonary hypertension due to left heart disease is the most prevalent form of PH. Pulmonary arterial hypertension (PAH) is a form of pre-capillary pulmonary hypertension that is defined based on cardiopulmonary hemodynamics as a mPAP Z 25 mmHg, pulmonary artery occlusion pressure (PAOP) r 15 mmHg, and pulmonary vascular resistance (PVR) Z 3 Wood units (WU) at rest. According to the latest classification by the World Symposium in Pulmonary Hypertension (WSPH), this group of pre-capillary pulmonary hypertension, termed Group 1 PAH, consists of idiopathic pulmonary arterial hypertension (IPAH) and heritable PAH (HPAH) that occur in the absence of any identifiable cause, and PAH associated with other conditions such as the use of anorexigens and other drugs, schistosomiasis infection, connective tissue diseases, congenital heart disease, HIV, or chronic liver disease. IPAH and HPAH are rare, with an estimated incidence of 1–2 cases per million in the general population according to the REVEAL registry. Historically, the first report of PAH dates back to 1891 by Ernst von Romberg who described findings of “pulmonary vascular stenosis” on autopsy. However, it was not until the 1970s with the recognition of fenfluramine/phentermine (also known as fen–phen)-associated PAH, that prompted the first WSPH meeting to address, describe, and classify PH. Since then, great strides have been made in the advancement of research and management of this disease.

Vasovagal response secondary to permanent contraception device in pulmonary arterial hypertension.

Adequate contraception is an essential component of managing pulmonary hypertension in women of childbearing age. Intrauterine devices are a popular contraceptive choice for many women but are associated with a risk of vagal response upon placement in certain patients with pulmonary hypertension, which may not be well tolerated. More recently, newer permanent contraception devices have emerged in the market, such as the Essure. We describe the first case, to our knowledge, of vagal-associated response due to an Essure device placement.