No abstract available.
Ganglia* ; Hemolytic-Uremic Syndrome*

No abstract available.
Epilepsy* ; Vigabatrin*

No abstract available.
Alexander Disease*

No abstract available.
Leukoencephalopathies*

No abstract available.
Arnold-Chiari Malformation* ; Diagnosis* ; Prenatal Diagnosis* ; Ultrasonography*

No abstract available.
Hematology* ; Humans ; Thyroid Gland*

No abstract available.
Infant ; Infant, Newborn ; Spasms, Infantile*

No abstract available.
Attention Deficit Disorder with Hyperactivity* ; Epilepsy*

OBJECTIVES: The hospital standardized mortality ratio (HSMR) has been widely used because it allows for robust risk adjustment using administrative data and is important for improving the quality of patient care. METHODS: All inpatients discharged from hospitals with more than 700 beds (66 hospitals) in 2008 were eligible for inclusion. Using the claims data, 29 most responsible diagnosis (MRDx), accounting for 80% of all inpatient deaths among these hospitals, were identified, and inpatients with those MRDx were selected. The final study population included 703 571 inpatients including 27 718 (3.9% of all inpatients) in-hospital deaths. Using logistic regression, risk-adjusted models for predicting in-hospital mortality were created for each MRDx. The HSMR of individual hospitals was calculated for each MRDx using the model coefficients. The models included age, gender, income level, urgency of admission, diagnosis codes, disease-specific risk factors, and comorbidities. The Elixhauser comorbidity index was used to adjust for comorbidities. RESULTS: For 26 out of 29 MRDx, the c-statistics of these mortality prediction models were higher than 0.8 indicating excellent discriminative power. The HSMR greatly varied across hospitals and disease groups. The academic status of the hospital was the only factor significantly associated with the HSMR. CONCLUSIONS: We found a large variation in HSMR among hospitals; therefore, efforts to reduce these variations including continuous monitoring and regular disclosure of the HSMR are required.
*Hospital Mortality ; Hospitals/*statistics & numerical data ; Humans ; Logistic Models ; Outcome Assessment (Health Care)/standards ; Quality of Health Care/standards ; Republic of Korea

Kallmann syndrome is characterized by hypogonadotropic hypogonadism resulting from insufficient release of GnRH and associated with anosmia or hyposmia. We experienced two cases of Kallmann syndrome with abnormal brain MRI findings(olfactory bulb aplasia) & secondary sexual dysfunction.
Brain ; Gonadotropin-Releasing Hormone ; Hypogonadism ; Kallmann Syndrome* ; Magnetic Resonance Imaging ; Olfaction Disorders