Saleh Alazemi

Interferon-induced sarcoidosis

Interferons (IFNs) are widely used for the treatment of various medical diseases. They have marked immunomodulatory effects, and many reports have been published associating IFN therapy with the induction of autoimmune phenomena and other disorders of immune regulation such as sarcoidosis. The clinical presentation of IFN‐induced sarcoidosis (IIS) is insidious and can be confused with common constitutional side effects of these drugs. The age of onset of IIS is later than that of naturally occurring sarcoidosis. The most common organs involved are the lungs and skin. In the majority of cases, IIS follows a benign course. As we show in an illustrative case report, complete resolution after discontinuation of IFN therapy can be expected. This review summarises 65 cases of IIS reported in the literature and highlights the pathophysiology, clinical features, diagnostic modalities and therapeutic options for this increasingly recognised phenomenon.

Outcomes, Health-Care Resources Use, and Costs of Endoscopic Removal of Metallic Airway Stents

BACKGROUND The use of self-expandable metallic airway stents (SEMAS) for airway compromise may be associated with significant complications requiring their removal/replacement. The aim of this study is to describe the complications, health-care resources use (HRU), and costs associated with endoscopic removal of SEMAS. METHODS A retrospective analysis of patients who underwent endoscopic removal of SEMAS during a 10-year period (January 2000-August 2009) was performed. HRU was analyzed in terms of the number of endoscopic procedures, hospital and ICU stay, need for mechanical ventilation and airway restenting, and estimation of respective hospital costs. RESULTS Fifty-five SEMAS were removed from 46 patients with a mean age of 58.6 +/- 15.8 years. Eighty percent of the stents were placed for benign airway disorders with an average stent in situ duration of 292 days. The median number of removal and total procedures during each encounter was one and two, respectively. Patients required hospitalization and ICU admission in 78% and 39% of the encounters with a median length of stay of 3.5 and 0 days, respectively. The estimated median total cost per encounter to remove the stents was

Clinical Characteristics of Subjects With Symptoms of α1-Antitrypsin Deficiency Older Than 60 Years

10,700, ranging from

Exacerbations in subjects with alpha-1 antitrypsin deficiency receiving augmentation therapy.

3,700 to

Effects of a Disease Management Program in Individuals with Alpha-1 Antitrypsin Deficiency

69,800. The measured outcomes were statistically significantly better when in situ stent duration was <or= 30 days (P < .05). CONCLUSIONS Endoscopic removal of SEMAS is feasible; however, it is associated with significant complications, HRU, and costs. The use of SEMAS should be restricted to a well-selected patient population and should be planned by a team experienced with this type of therapeutic strategy.

Staged repair of benign tracheo-neo-esophageal fistula 12 years after esophagectomy for esophageal cancer.

BACKGROUND The clinical characteristics of elderly subjects with alpha(1)-antitrypsin deficiency (AATD)-associated COPD have not been described. METHODS The clinical, demographic, health-related quality of life (HRQoL) characteristics and 1-year exacerbation rates of 275 subjects with AATD and COPD receiving augmentation therapy aged > 59 years (mean [+/- SD] age, 66.3 +/- 5.7 years) were compared to those of 354 subjects aged 50 to 59 years (mean age, 54.3 +/- 2.8 years) and 293 subjects < 50 years (mean age, 43.9 +/- 3.8 years). RESULTS Older subjects received diagnoses later in life (mean age at diagnosis, 55.0 +/- 8.5 years) and had a longer diagnostic delay (mean age at diagnosis, 12.9 +/- 14.3 years) than subjects in the other two age groups. Although the proportion of lifetime nonsmokers was higher in the older group, the majority (64%) had significant tobacco exposure but with a longer interval of tobacco abstinence. The mean FEV(1) values (n = 641) were similar between the three age groups, suggesting a slower disease progression in the oldest group. Subjects in the older group were less symptomatic, had less concomitant asthma, and had significantly better scores in most domains of two HRQoL instruments. During follow-up, older subjects had fewer acute exacerbations. CONCLUSIONS Subjects with AATD-associated COPD who reach an older age exhibit a more indolent clinical course than younger affected individuals, possibly related in part to differences in tobacco exposure. This finding supports current guidelines that recommend screening of all patients with COPD for AATD, regardless of their age and prior smoking history.

Stauffer's Syndrome Variant with Cholestatic Jaundice A Case Report

BACKGROUND The frequency, characteristics and impact of acute exacerbations in patients with alpha-1 antitrypsin deficiency (AATD) and COPD who are on intravenous alpha-1 antitrypsin augmentation therapy have not been described. METHODS 922 subjects with AATD and COPD on augmentation therapy (mean age 54.5 years) were followed with monthly telephone surveys to record exacerbation characteristics, as well as healthcare resource utilization and health-related quality of life (HRQoL). Exacerbations were defined by symptom-based and healthcare resource utilization (HRU) criteria. RESULTS During the 1-year follow-up, 91.5% of participants experienced at least one exacerbation (mean 2.4 exacerbations per subject, median 2, and mean duration 17 days per episode, regardless of the definition used). Most exacerbations were categorized as severe by symptoms and moderate by HRU criteria. Subjects who had 3 or more exacerbations (48.6%) were younger, had higher medication use and had higher tobacco consumption compared with subjects with less exacerbations. Subjects with frequent exacerbations had the worst baseline HRQoL scores, as well as more physician visits, emergency room visits, and hospitalizations. Although most subjects received augmentation therapy on a weekly basis, other infusion schedules were more commonly observed in subjects with fewer exacerbations. CONCLUSION COPD exacerbations occur frequently and are associated with significant disease burden in subjects with AATD receiving augmentation therapy.

Mediastinal Migration of Self-Expanding Bronchial Stents in the Management of Malignant Bronchoesophageal Fistula

Disease management programs improve outcomes in subjects with chronic obstructive pulmonary disease (COPD), but their effect in subjects with alpha-1 antitrypsin deficiency (AATD) has not been evaluated. To assess the impact of a disease management program, applicable to subjects with AATD-associated COPD throughout the United States, on exacerbations, healthcare resource utilization and health-related quality of life (HRQoL). The Alpha-1 Disease Management and Prevention Program (ADMAPP) consisted of comprehensive written educational patient-directed material for self-study and treatment plans. Program reinforcement was performed through monthly phone calls by specialized coordinators. Outcomes were collected prospectively for 12 months before, and 12 months after enrollment into the program. Exacerbations and healthcare resource utilization were recorded monthly. HRQoL was measured with the St Georges Respiratory Questionnaire (SGRQ) every 6 months and the Short Form-36 (SF-36) every 12 months. A total of 878 subjects completed the 2-year study. During the intervention year, there was a significant increase in the use of long-acting bronchodilators, better compliance with oxygen therapy, and more use of steroid courses during exacerbations. Total exacerbation rates, unscheduled physician visits and emergency room visits significantly decreased. There was also a statistically significant slowing in the deterioration of the SGRQs activity domain, while total SGRQ scores remained stable during the study. Significant improvements were observed in some of the SF-36 domains, particularly in the general health domain. The ADMAPP improved health outcomes in subjects with AATD-associated COPD.

An Elderly Woman With Chronic Dyspnea and Endobronchial Lesion

Benign tracheo-neo-esophageal fistula is a rare complication after esophagectomy. We report a 60-year-old man who presented 12 years after a McKeown esophagectomy with a fistula between the tracheal carina and the gastric conduit. In view of his severe sepsis and profound malnutrition, he underwent placement of a silicon Y-stent with a successful three-stage surgical repair consisting of duodenal exclusion with drainage gastrostomy. Six weeks later, the patient had closure of the fistula through a right thoracotomy. He finally underwent Roux-en-Y gastro-jejunostomy through a left thoraco-abdominal approach to restore the gastrointestinal continuity. Eighteen months postoperatively, he reports no dysphagia and has regained his premorbid weight.

Pneumothorax after reexpansion pulmonary edema: experience with 2 patients.

Cholestasis is a common feature of several malignant diseases, including pancreatic, hepatic, gallbladder, and ampullary carcinomas. It is usually secondary to main bile duct obstruction or widespread hepatic metastasis, but it can also be a paraneoplastic syndrome of other underlying malignancies. Stauffer’s syndrome is a rare paraneoplastic manifestation of renal cell carcinoma (RCC) that is characterized by elevated alkaline phosphatase, erythrocyte sedimentation rate, α-2-globulin, and γ-glutamyl transferase, thrombocytosis, prolongation of prothrombin time, and hepatosplenomegaly, in the absence of hepatic metastasis and jaundice. A rare variant of this syndrome with jaundice has recently been described in 3 cases in the literature. We report a patient who presented with abdominal pain and cholestatic jaundice in whom RCC was incidentally found during initial workup. Jaundice and liver dysfunction resolved completely after surgical resection of the tumor. This case illustrates the protean manifestations of RCC, and the importance of considering Stauffer’s syndrome and its variant in the differential diagnosis of anicteric and icteric cholestasis, which may allow early recognition and treatment of an underlying malignancy.