Pulmonary fibrosis is a progressive, fatal manifestation of Hermansky-Pudlak syndrome (HPS). Some patients with advanced HPS pulmonary fibrosis undergo lung transplantation despite their disease-associated bleeding tendency; others die while awaiting donor organs. The objective of this study is to determine the clinical management and outcomes of a cohort with advanced HPS pulmonary fibrosis wh...

Hantavirus pulmonary syndrome (HPS) is an increasing health problem in Brazil because of encroachment of sprawling urban, agricultural, and cattle-raising areas into habitats of subfamily Sigmodontinae rodents, which serve as hantavirus reservoirs. From 1993 through June 2007, a total of 884 cases of HPS were reported in Brazil (case-fatality rate 39%). To better understand this emerging diseas...

BACKGROUND Ultrasound guidelines for hypertrophic pyloric stenosis (HPS) have fixed minimum measurements and do not account for variation in patient weight or age. We sought to determine if preoperative pyloric measurements correlated with weight and age in patients with surgically proven HPS. METHODS A retrospective analysis was conducted of 189 patients with HPS treated at a single institut...

Hemophagocytic syndrome (HPS) is a rare condition characterized by overactive histiocytes, hepatosplenomegaly, fever and cytopenia, with two major types: familial, autosomal recessive genetic disease and acquired that can occur during systemic infections, immunodeficiency or malignancy. Inappropriate activation of macrophages by cytokines is the major mechanism of the disease. We report a case ...

We isolated Andes virus (formal name: Andes virus [ANDV], a species in the genus Hantavirus), from serum of an asymptomatic 10-year-old Chilean boy who died 6 days later of hantavirus pulmonary syndrome (HPS). The serum was obtained 12 days after his grandmother died from HPS and 2 days before he became febrile. No hantavirus immunoglobulin (Ig) G or IgM antibodies were detected in the serum sa...

Hemophagocytic syndrome (HPS) is a rare disorder of lymphocyte and histiocyte activation through cytokine dysfunction leading to widespread organ compromise. With widespread lymphocytic and histiocytic activation, it is known to cause considerable end-organ dysfunction quickly and with relatively few options for treatment.1 HPS has been associated with Epstein-Barr virus (EBV), human immunodefi...

Hemophagocytic syndrome (HPS) is an extremely rare condition arising from the overactivation of one's own immune system. It results in excessive inflammation and tissue destruction. Prompt initiation of treatment is warranted in either scenario in order to decrease mortality. Most cases are triggered by infectious agents, malignancy, or drugs. We describe the first case of a CLL patient present...

OBJECTIVES Recent investigations have demonstrated that the classic hypochloremic, hypokalemic, metabolic alkalosis of hypertrophic pyloric stenosis (HPS) is not a common finding.Some have suggested a trend over time, but none has investigated factors contributing to laboratory derangement, such as duration of vomiting or patient age at presentation. We sought to determine the proportion of pat...

UNLABELLED Hepatopulmonary syndrome (HPS) is a complication of portal hypertension (PH) defined by the presence of liver disease, abnormal pulmonary gas exchange and evidence of intrapulmonary vascular dilatations (IPVD) producing a right to left intrapulmonary shunt. Liver transplantation (LT) is the treatment of choice; however, severe hypoxemia may contraindicate LT. The use of transjugular ...

Human palatine gland saliva (HPS) was collected from young adult subjects (mean age 22 yr) of both sexes by gentle mechanical stimulation. The HPS contained 9-13 of the common amino acids within the range of 2-850 nmol/ml, but most samples had low or no concentrations of arginine, tryptophan and proline. Methionine and cysteine were not reliably detected. The molar ratios of most amino acids va...