Prolonged treatment with open-label pirfenidone in Hermansky-Pudlak syndrome pulmonary fibrosis.

PURPOSE: Limited information is available regarding chronic treatment with pirfenidone, an anti-fibrotic drug. Effects of long-term open-label pirfenidone were evaluated in a small cohort with Hermansky-Pudlak syndrome (HPS), a rare autosomal recessive disorder with highly penetrant pulmonary fibrosis. RESULTS: Three patients with HPS pulmonary fibrosis treated with open-label pirfenidone and twenty-one historical controls randomized to placebo were studied at a single center. Mean duration of treatment with pirfenidone for 3 patients with HPS pulmonary fibrosis was 13.1 years. Annual changes in FVC and DLCO with pirfenidone treatment were 0.46 and - 0.93% predicted, respectively. In comparison, historical controls randomized to receive placebo experienced mean annual changes in FVC and DLCO of -4.4 and - 2.3% predicted, respectively. High-resolution computed tomography (HRCT) scans revealed improved ground glass opacities with development of minimal interstitial reticulations in 1 patient after 12.8 years of treatment with pirfenidone. Slowly progressive increase in bilateral interstitial fibrosis developed in a different patient, who received pirfenidone for 18.1 years and died at 73 years of age due to HPS pulmonary fibrosis. Another patient treated with pirfenidone for 8.4 years had attenuated ground glass opacification on HRCT scan and improved oxygenation; this patient died due to chronic complications from colitis, and not pulmonary fibrosis. Adverse effects were generally limited to mild gastrointestinal discomfort and transient elevations of alanine aminotransferase in one patient. CONCLUSIONS: Chronic treatment with pirfenidone may provide clinical benefit with few adverse effects for some patients with HPS pulmonary fibrosis. These results suggest that compassionate use of pirfenidone could be considered on a case-by-case basis for patients with HPS pulmonary fibrosis.

Hermansky-Pudlak syndrome: health care throughout life.

Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disease that displays genetic heterogeneity; there are 9 known subtypes. HPS is characterized by oculocutaneous albinism, a platelet storage pool deficiency and resultant bleeding diathesis, and lysosomal accumulation of ceroid lipofuscin. Patients with HPS, specifically those with the genotypes HPS-1, HPS-2, or HPS-4, are predisposed to interstitial lung disease. In addition, some patients with HPS develop granulomatous colitis. Optimal health care requires a thorough knowledge of the unique health risks and functional limitations associated with this syndrome.

Personal and household hygiene, environmental contamination, and health in undergraduate residence halls in New York City, 2011.

BACKGROUND: While several studies have documented the importance of hand washing in the university setting, the added role of environmental hygiene remains poorly understood. The purpose of this study was to characterize the personal and environmental hygiene habits of college students, define the determinants of hygiene in this population, and assess the relationship between reported hygiene behaviors, environmental contamination, and health status. METHODS: 501 undergraduate students completed a previously validated survey assessing baseline demographics, hygiene habits, determinants of hygiene, and health status. Sixty survey respondents had microbiological samples taken from eight standardized surfaces in their dormitory environment. Bacterial contamination was assessed using standard quantitative bacterial culture techniques. Additional culturing for coagulase-positive Staphylococcus and coliforms was performed using selective agar. RESULTS: While the vast majority of study participants (n = 461, 92%) believed that hand washing was important for infection prevention, there was a large amount of variation in reported personal hygiene practices. More women than men reported consistent hand washing before preparing food (p = .002) and after using the toilet (p = .001). Environmental hygiene showed similar variability although 73.3% (n = 367) of subjects reported dormitory cleaning at least once per month. Contamination of certain surfaces was common, with at least one third of all bookshelves, desks, refrigerator handles, toilet handles, and bathroom door handles positive for >10 CFU of bacteria per 4 cm(2) area. Coagulase-positive Staphylococcus was detected in three participants' rooms (5%) and coliforms were present in six students' rooms (10%). Surface contamination with any bacteria did not vary by frequency of cleaning or frequency of illness (p>.05). CONCLUSIONS: Our results suggest that surface contamination, while prevalent, is unrelated to reported hygiene or health in the university setting. Further research into environmental reservoirs of infectious diseases may delineate whether surface decontamination is an effective target of hygiene interventions in this population.

Determinants of personal and household hygiene among college students in New York City, 2011.

BACKGROUND: Although several studies have characterized the hygiene habits of college students, few have assessed the determinants underlying such behaviors. OBJECTIVES: Our study sought to describe students' knowledge, practices, and beliefs about hygiene and determine whether there is an association between reported behaviors and frequency of illness. METHODS: A sample of 299 undergraduate students completed a questionnaire assessing demographics, personal and household hygiene behaviors, beliefs and knowledge about hygiene, and general health status. RESULTS: Variation in reported hygiene habits was noted across several demographic factors. Women reported "always" washing their hands after using the toilet (87.1%) more than men (65.3%, P = .001). Similarly, freshmen reported such behavior (80.4%) more than sophomores (71.9%), juniors (67.7%), or seniors (50%, P = .011). Whereas 96.6% of participants thought that handwashing was either "very important" or "somewhat important" for preventing disease, smaller proportions thought it could prevent upper respiratory infections (85.1%) or gastroenteritis (48.3%), specifically. There was no significant relationship between reported behaviors and self-reported health status. CONCLUSION: The hygiene habits of college students may be motivated by perceptions of socially acceptable behavior rather than scientific knowledge. Interventions targeting the social norms of incoming and continuing students may be effective in improving hygiene determinants and ultimately hygiene practices.

Rates of asymptomatic nonurethral gonorrhea and chlamydia in a population of university men who have sex with men.

OBJECTIVES: The study determined prevalence of asymptomatic nonurethral gonorrhea and chlamydia in men who have sex with men (MSM) seen at the Columbia University Health Service for routine care. PARTICIPANTS: The study enrolled 200 participants from March 2007 to May 2010. RESULTS: Specimens were tested using culture and nucleic acid amplification testing (NAAT): 3.5% (n = 7) tested positive for pharyngeal gonorrhea by NAAT, none were positive by culture; 3% (n = 6) tested positive for rectal chlamydia by NAAT and 0.5% (n = 1) by culture. CONCLUSIONS: The incidence of pharyngeal gonorrhea and rectal chlamydia in MSM who visited the Columbia Health Service was similar to rates of asymptomatic nonurethral gonorrhea and chlamydia in studies conducted in the MSM population in non-university settings. This suggests that, following the Centers for Disease Control and Prevention guidelines, 3-site testing for MSM seen at the Columbia clinic is indicated. NAAT is more sensitive than culture for nonurethral gonorrhea and chlamydia.

Successful bilateral lung transplantation for pulmonary fibrosis associated with the Hermansky-Pudlak syndrome.

Hermansky-Pudlak syndrome (HPS) is a genetic disorder characterized by oculocutaneous albinism, a bleeding diathesis, and in a subset of patients, pulmonary fibrosis. Lung transplantation, the only curative therapy for pulmonary fibrosis, has not been previously reported as a successful treatment strategy for patients with HPS because the bleeding diathesis was thought to contraindicate major thoracic surgery. We successfully performed bilateral sequential lung transplantation in a patient with pulmonary fibrosis and HPS after transfusion of 6 units of platelets. Lung transplantation is a viable therapeutic option in patients with pulmonary fibrosis and only a mild bleeding diathesis associated with HPS.