Isovaleric acidemia is an inborn error in metabolism due to a defect in isovaleryl-CoA dehydrogenase. Accumulation of serum isovaleric acid causes poor feeding, vomiting, lethargy, hypothermia, convulsion, mental retardation, etc. It is inherited as an autosomal recessive trait. Since the first reports of isovaleric acidemia by Tanaka et al in 1966, more than 60 cases have been reported. There are two clinically different presentations of isovaleric acidemia, with about half the patients presenting with an acute severe neonatal form and about half with a chronic intermittent forrn. The difference in clinical presentation may not be a consequence of differing severities of the causative mutation, but a result of the timing of application of catabolic stress or the ability to form isovalerylglycine. We described here clinical and organic acid analytical findings of brothers with chronic intermittent form of isovaleric acidemia. (J Korean Pediatr Soc 2000;43:828-831)
Humans ; Hypothermia ; Intellectual Disability ; Isovaleryl-CoA Dehydrogenase ; Lethargy ; Metabolism ; Seizures ; Siblings* ; Vomiting

No abstract available.
Peer Review*

No abstract available.
Biopsy* ; Prostate*

Cardiac myxoma is a rare but potentially treatable cause of stroke. The diagnosis is rarely made on typical triad of constitutional, embolic, and obstructive symptoms, and it is difficult because there is no specific history, physical examination, chest X-ray, or electrocardiographic findings, but with the development of echocardiography the correct diagosis is now usualy made. Neurologic manifestation is frequent(25-45%) and presented with embolic infarction, aneurysm formation, intracranial hemorrhage, and distant metastasis. We experienced five patients with cardiac myxoma who presented with these neurologic manifestations. Also we should be considered in the differential diagnosis of cerebral infarction, particularly when multiple cerebral infarction and constitutional or obstructive symptoms are conjoined in young age stroke. We describe five patients who presented with embolic infarction, cerebral metastasis and aneurysm formation.
Aneurysm ; Cerebral Infarction ; Diagnosis ; Diagnosis, Differential ; Echocardiography ; Electrocardiography ; Humans ; Infarction ; Intracranial Hemorrhages ; Myxoma* ; Neoplasm Metastasis ; Neurologic Manifestations* ; Physical Examination ; Stroke ; Thorax

No abstract available.

OBJECTIVES: This study was conducted to identify differences in job stress, satisfaction and commitment of cooking employees working in hotel kitchens with and without HACCP systems. METHODS: Culinary employees of 12 five-star hotels were surveyed and 504 valid data were used for SPSS analysis. Sub factors of working environment factors (job stress, job satisfaction, and job commitment) were examined for analysis. RESULTS: The results showed that hotels that implemented the HACCP system had significantly higher values for the five sub factors of employee job stress (job demand, relationship conflict, organizational system, lack of job autonomy, and job instability; p < 0.001). For the sub factors of job satisfaction (internal and external satisfaction) statistic showed a statistically significant value in hotels that did not implement the HACCP system (p < 0.001). Job attachment and job importance, which are sub factors of job commitment, showed no difference in relation to the implementation of HACCP system, and job responsibility showed a higher p-value in hotels that did not implement HACCP (p < 0.05). CONCLUSIONS: The results of the study indicate that culinary employees working at venues with HACCP systems have more job related stress, lower job satisfaction and partially less job commitment. Based on this outcome, venues that have already implemented or are planning to implement HACCP systems should consider the implications regarding their management of employees. Managerial policies that enhance autonomy, job stability, achievement, self-development, promotion, and compensation should also be implemented. Finally, meticulous attention and high investments into the work environment and human resources are necessary.
Compensation and Redress ; Cooking ; Hazard Analysis and Critical Control Points ; Humans ; Investments ; Job Satisfaction

OBJECTIVES: This study was conducted to identify differences in job stress, satisfaction and commitment of cooking employees working in hotel kitchens with and without HACCP systems. METHODS: Culinary employees of 12 five-star hotels were surveyed and 504 valid data were used for SPSS analysis. Sub factors of working environment factors (job stress, job satisfaction, and job commitment) were examined for analysis. RESULTS: The results showed that hotels that implemented the HACCP system had significantly higher values for the five sub factors of employee job stress (job demand, relationship conflict, organizational system, lack of job autonomy, and job instability; p < 0.001). For the sub factors of job satisfaction (internal and external satisfaction) statistic showed a statistically significant value in hotels that did not implement the HACCP system (p < 0.001). Job attachment and job importance, which are sub factors of job commitment, showed no difference in relation to the implementation of HACCP system, and job responsibility showed a higher p-value in hotels that did not implement HACCP (p < 0.05). CONCLUSIONS: The results of the study indicate that culinary employees working at venues with HACCP systems have more job related stress, lower job satisfaction and partially less job commitment. Based on this outcome, venues that have already implemented or are planning to implement HACCP systems should consider the implications regarding their management of employees. Managerial policies that enhance autonomy, job stability, achievement, self-development, promotion, and compensation should also be implemented. Finally, meticulous attention and high investments into the work environment and human resources are necessary.
Compensation and Redress ; Cooking ; Hazard Analysis and Critical Control Points ; Humans ; Investments ; Job Satisfaction

The current paper reports on a case of subdural empyema secondary to frontal sinusitis in an otherwise healthy child. Sinusitis is a common and benign condition in most pediatric cases. Because of the widespread use of antibiotics, intracranial extension of pediatric sinusitis is rarely seen today; however, complications (e.g., cavernous sinus thrombosis, orbital infection, meningitis, and subdural empyema) are potentially life threatening. A 15-year-old right-handed male presented with a 3-day history of fever, headache, and left-sided palsy. Computed tomography revealed right-sided subdural empyema with right frontal sinusitis and maxillary sinusitis. A postoperative inpatient neurological consultation was requested 2 months post-surgery due to motor function deficits. The results suggested that early and accurate diagnosis of subdural empyema leads to prompt treatment and a favorable outcome for the patient.
Adolescent ; Anti-Bacterial Agents ; Brain Abscess ; Cavernous Sinus Thrombosis ; Central Nervous System ; Child* ; Diagnosis ; Empyema, Subdural* ; Fever ; Frontal Sinus ; Frontal Sinusitis ; Headache ; Humans ; Inpatients ; Male ; Maxillary Sinus ; Maxillary Sinusitis ; Meningitis ; Orbit ; Paralysis ; Sinusitis*

Pseudoxanthoma elasticum is a rare, hereditable disease of connective tissue, characterzed by abberrant calcification of the elastic tissue of the skin, eyes, vasculature. We report a 61years old female patient with pseudoxanthoma elasticum with typical peau d'orange skin lesion around the neck, angioid streaks in the retina and multifocal cerebral infarction.
Angioid Streaks ; Cerebral Infarction* ; Connective Tissue ; Elastic Tissue ; Female ; Humans ; Neck ; Pseudoxanthoma Elasticum* ; Retina ; Skin

Pure red cell aplasia in uncommon disorder characterized by finding of anemia, absence of nucleated red blood cell in the marrow, absence of reticulocytes in the peripheral blood and normal peripheral platelet and leukocytes counts. We experienced one case of pure red cell aplasia associated with hemolytic anemia characterized by hemoglobinuria, reticulocytopenia, and erythroid hypoplasia of the bone marrow. The cause of the illness was not definitely identified, but we concluded that this patient had simultaneous occurrence of PRCA and hemolytic anemia following administration of diphenylhydantoin after craniotomy rather than virus or bacteria induced. The simultaneous occurrence of PRCA and hemolytic anemia in uncommon and the mechanism for diphenylhydantoin induced PRCA and hemolytic anemia is unclear.
Anemia ; Anemia, Hemolytic ; Bacteria ; Blood Platelets ; Bone Marrow ; Craniotomy ; Erythrocytes ; Hemoglobinuria ; Humans ; Leukocytes ; Phenytoin ; Red-Cell Aplasia, Pure* ; Reticulocytes