Symmetric Age Association of Retinal Degeneration in Patients with CLN2-Associated Batten Disease.

Purpose: Mutations in the CLN2 gene lead to a neurodegenerative and blinding lysosomal storage disorder: late infantile neuronal ceroid lipofucinosis, also known as "CLN2 disease." The purpose of the current study was to characterize the evolution of CLN2-associated retinal manifestations using the Weill Cornell Batten Scale (WCBS) and the age association of the retinal degeneration using central subfield thickness (CST) measurements and then correlate these findings with fundus photography and OCT to determine a critical period for retinal intervention.

Design: Retrospective, single-center cohort.

An online survey on burden of illness among families with post-stem cell transplant mucopolysaccharidosis type I children in the United States.

Background: Severe mucopolysaccharidosis type I (also known as Hurler syndrome) is a rare devasting recessive genetic disease caused by the deficiency of an enzyme. Hematopoietic stem cell transplant is the standard of care in the United States, usually conducted before the child is 3 years of age, but little is known about the continued medical and educational needs of the child after transplant. A greater understanding of the burden of illness on the primary caregiver is also needed.

Choice of hemostatic agent and hospital length of stay in cardiovascular surgery.

Hemostatic agents (HAs) are efficacious in reducing blood loss during surgery, which may affect postoperative length of stay (LOS). The purpose of this study was to compare the expected and actual LOS by HA in cardiac procedures. Hospital claims data between 2003 and 2006 were extracted from a US service-level comparative database.

Current concepts in reoperative cardiac surgery.

This article summarizes techniques used in reoperative cardiac surgery, outlines a risk-stratified approach to operative planning, and reviews the literature on outcomes after reoperative valvular and coronary surgery.

An observational, retrospective analysis of retreatment with bortezomib for multiple myeloma.

Purpose: The aim of this retrospective chart review of patients with multiple myeloma (MM) was to describe patterns of retreatment with bortezomib-based therapy and responses to retreatment in a community-based setting.

Patients And Methods: Data were retrospectively extracted from the medical records of patients treated in US Oncology-affiliated community oncology clinics who received 2 separate treatments with bortezomib-based therapy. Eligible patients had > or = 60 days between treatments and > or = 4 bortezomib doses during initial treatment.

The role of gastric pH testing with small-bore feeding tubes: in the intensive care unit.

In this observational study, we sought to assess the usefulness of pH values from residuals in intensive care unit (ICU) patients with small-bore feeding tubes. A review of the literature demonstrated that most critically ill patients were excluded in previous studies on gastric pH testing, suggesting that pH testing in critical care is not warranted. Our results demonstrated that 21% of intestinal testing and 64% of gastric testing yielded potential misinformation to the bedside nurse with regards to possible tube location.

Hyperglycemia in the hospital setting: the case for improved control among non-diabetics.

Objective: To review studies on the role of hyperglycemia in acutely ill adults, regardless of diabetes diagnosis, and the impact of glucose control on health outcomes.

Data Sources: Searches on Ovid MEDLINE, Ovid Evidence-Based Medicine (EBM), and PubMed MEDLINE, limited to articles written in English, trials conducted on adult subjects, and material published between 1994 and April 2004. Search words included the major MeSH term hyperglycemia and title words glucose, hyperglycemia/hyperglycemic, or insulin therapy, with text words admission, hospitalized, in-hospital, or inpatient.

Overview of inpatient coding.

The main classification-based and nomenclature-based coding systems used in the United States, as well as the process and importance of documenting in the patient record, are discussed. Hospital pharmacists usually have limited knowledge of and exposure to coding and reimbursement in the inpatient system. Coding allows for reporting of mortality data to the World Health Organization (WHO), reporting morbidity data in the U.