Adys Mendizabal1, Anh-Thu Ngo Vu2, Dylan Thibault1,3, Pedro Gonzalez-Alegre1,4, Allison Willis1,3,5,6. 1. Department of Neurology University of Pennsylvania School of Medicine Philadelphia Pennsylvania. 2. Department of Neurology Emory University School of Medicine Atlanta Georgia. 3. Department of Biostatistics and Epidemiology University of Pennsylvania School of Medicine Philadelphia Pennsylvania. 4. The Raymond G. Perelman Center for Cellular and Molecular Therapeutics Children's Hospital of Philadelphia Philadelphia Pennsylvania. 5. Leonard Davis Institute of Health Economics University of Pennsylvania Philadelphia Pennsylvania. 6. Center for Clinical Epidemiology and Biostatistics University of Pennsylvania Philadelphia Pennsylvania.
Abstract
BACKGROUND: Juvenile Huntington's disease (JHD) is a childhood-onset neurodegenerative disorder. Although it is caused by the same pathologic expansion of CGA repeats as adult-onset Huntington's disease, JHD has distinct clinical features. Most clinical research in HD focuses in the adult-onset disease; therefore, little is known about acute care outcomes for patients with JHD. METHODS: The Kids' Inpatient Database (KID) was used to examine hospitalizations of children with JHD and to determine the diagnoses and procedures associated with inpatient care for JHD. Regression models were built to examine acute care outcomes, including death, length of stay, and disposition at discharge in patients with JHD compared with patients in the general KID data. RESULTS: The proportion of JHD cases among hospitalized children was 1.23 per 100,000 KID inpatient stays. Seizures/convulsions (58.5%) and psychiatric conditions (26.1%) were the most common primary or secondary diagnoses among JHD patient hospitalizations. The most common procedure was percutaneous endoscopic gastrostomy tube placement (8.6%). Compared with hospitalizations of the general population, hospitalizations of patients with JHD had a lower odds of discharge to home (adjusted odds ratio [AOR], 0.23; 95% confidence interval [CI], 0.14-0.37) and an increased likelihood of death (AOR, 8.03; 95% CI, 2.98-21.60) or discharge to a short-term care facility (AOR, 4.44; 95% CI, 2.59-7.61). A diagnosis of JHD was associated with increased length of stay (7.04 vs. 3.75 days; P < 0.01). CONCLUSIONS: Children with JHD have unique acute care patterns. Future studies are needed to determine the extent to which coordinated care may impact inpatient and disposition needs.
BACKGROUND: Juvenile Huntington's disease (JHD) is a childhood-onset neurodegenerative disorder. Although it is caused by the same pathologic expansion of CGA repeats as adult-onset Huntington's disease, JHD has distinct clinical features. Most clinical research in HD focuses in the adult-onset disease; therefore, little is known about acute care outcomes for patients with JHD. METHODS: The Kids' Inpatient Database (KID) was used to examine hospitalizations of children with JHD and to determine the diagnoses and procedures associated with inpatient care for JHD. Regression models were built to examine acute care outcomes, including death, length of stay, and disposition at discharge in patients with JHD compared with patients in the general KID data. RESULTS: The proportion of JHD cases among hospitalized children was 1.23 per 100,000 KID inpatient stays. Seizures/convulsions (58.5%) and psychiatric conditions (26.1%) were the most common primary or secondary diagnoses among JHD patient hospitalizations. The most common procedure was percutaneous endoscopic gastrostomy tube placement (8.6%). Compared with hospitalizations of the general population, hospitalizations of patients with JHD had a lower odds of discharge to home (adjusted odds ratio [AOR], 0.23; 95% confidence interval [CI], 0.14-0.37) and an increased likelihood of death (AOR, 8.03; 95% CI, 2.98-21.60) or discharge to a short-term care facility (AOR, 4.44; 95% CI, 2.59-7.61). A diagnosis of JHD was associated with increased length of stay (7.04 vs. 3.75 days; P < 0.01). CONCLUSIONS: Children with JHD have unique acute care patterns. Future studies are needed to determine the extent to which coordinated care may impact inpatient and disposition needs.
Authors: Simon C Warby; Henk Visscher; Jennifer A Collins; Crystal N Doty; Catherine Carter; Stefanie L Butland; Anna R Hayden; Ichiro Kanazawa; Colin J Ross; Michael R Hayden Journal: Eur J Hum Genet Date: 2011-01-19 Impact factor: 4.246
Authors: Walter F Stewart; G Craig Wood; Aubrey Manack; Sepideh F Varon; Dawn C Buse; Richard B Lipton Journal: J Occup Environ Med Date: 2010-01 Impact factor: 2.162