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

No abstract available.
Alexander Disease*

No abstract available.
Leukoencephalopathies*

No abstract available.
Epilepsy* ; Vigabatrin*

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

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

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

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

Virus associated hemophagocytic syndrome, class ll histiocytoses, characterized by high fever, severe constitutional symptoms, abnormal liver function and coagulation, perigheral blood pancytopenia and histiocytic hyperplasis with prominent hemophagocytosis in bone marrow and lymph nodes has been reported and associated with active viral infection. It is non-malignant and reversible. It must be differentiated from histiocytic medullary reticulosis because of the inappopriateness of immunosuppressive of cytotozic therapy which is the therapeutic method for HMR, but is contraindicated in the treatment of VAHS. This paper describes two patients whose clinicopathology was compatible with the diagnosis of virus associated hemophagocytic syndrome.
Bone Marrow ; Diagnosis ; Fever ; Histiocytosis ; Humans ; Liver ; Lymph Nodes ; Lymphohistiocytosis, Hemophagocytic* ; Pancytopenia

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