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.
Child ; Health Promotion ; Humans ; Immunization* ; Child Health

BACKGROUND: Serious pulmonary complications after lung surgery increase morbidity and mortality in perioperative period. Ketamine hydrochloride produces strong analgesic effect in spite of the psychomimetic effects. Intravenous anesthesia with ketamine was performed in lung surgery of patients with decreased pulmonary function and compared with inhalation anesthesia with enflurane. METHODS: Sixty patients, scheduled for elective lung surgery, were randomly assigned to two groups. Patients received either enflurane (Group 1, n=30) or ketamine (Group 2, n=30) as main anesthetic drug. Blood pressure and heart rate were compared in preinduction, postinduction, postintubation, postincision, intraoperative period (30 minutes, 60 minutes) and recovery room between groups, and in each group. Arterial blood gas analysis was compared in preoperative period, intraoperative period and recovery room between groups. Postoperative psychological complications evaluated in group 2. RESULTS: Blood pressure and heart rate were significantly different in postinduction, postintubation and recovery room between groups. PaO2 in group 2 was higher than in group 1 during intraoperative period and recovery room. Postoperative psychological complications occured in 4 patients (13%) in group 2. CONCLUSIONS: Ketamine affords advantage over enflurane anesthesia in terms of PaO2 during intraoperative period and recovery room in lung surgery of patients with decreased pulmonary function.
Anesthesia ; Anesthesia, Inhalation* ; Anesthesia, Intravenous* ; Blood Gas Analysis ; Blood Pressure ; Enflurane* ; Heart Rate ; Humans ; Inhalation* ; Intraoperative Period ; Ketamine* ; Lung* ; Mortality ; Perioperative Period ; Preoperative Period ; Recovery Room

No abstract available.

No Abstract Available.
Diagnosis* ; Enzyme-Linked Immunosorbent Assay* ; Flagellin* ; Leptospirosis*

BACKGROUND: To evaluate the clinical outcomes and associated repair integrity in patients treated with arthroscopic repair for a transtendinous rotator cuff tear followed by resection of the remnant rotator cuff tendon. METHODS: Between July 2007 and July 2011, we retrospectively reviewed patients who were treated for transtendinous full-thickness tears in the tendinous portion of the rotator cuff by arthroscopic repair. Clinical outcomes were evaluated using the American Shoulder and Elbow Surgeons (ASES) score, the Shoulder Rating Scale of the University of California at Los Angeles (UCLA), the Constant-Murley score, a visual analogue scale (VAS) pain score, and range of motion (ROM). The repair integrity was determined by magnetic resonance imaging or ultrasonography. RESULTS: There were 19 shoulders with transtendinous full-thickness tears in the tendinous portion of the rotator cuff. The ASES, UCLA, Constant-Murley, and VAS pain scores showed significant improvements in function and symptoms (all p<0.001). The active ROM for forward flexion and abduction was also significantly improved (p=0.002 and p<0.001, respectively). The postoperative radiological examination showed cuff integrity without a re-tear in 68.4% of patients. However, the UCLA, ASES, and Constant-Murley scores were not significantly different between healed and re-torn group (p=0.530, p=0.885, and p=0.262, respectively). CONCLUSIONS: Although repair of transtendinous rotator cuff tears followed by resection of the remnant rotator cuff tendon in the footprint has a relatively high re-tear rate, no significant difference was observed in the short-term clinical results between the re-tear and healed groups.
Arthroscopy ; California ; Elbow ; Follow-Up Studies* ; Humans ; Magnetic Resonance Imaging ; Range of Motion, Articular ; Retrospective Studies ; Rotator Cuff* ; Shoulder ; Surgeons ; Tears* ; Tendons ; Ultrasonography

Hyperplasia of the gingivae may occur as a secondary reaction in the treatment of epilepsy with diphenylhydantoin sodium. This study is intended to investigate the incidence and dharacteristics of gingival hyperplasia in 124 epileptic patients taking diphenylhydantoin longstandingly. The results are as follows: 1. The incidence of diphenylhydantoin induced gingival hyperplasia is approximately 69.4%. 2. The individual tolerance or sensitivity may determine the incidence and severity of gingival hyperplasia and are not directly related with the duration of drug use and its dosage. 3. Gingival hyperplasia is more frequent in female patients and younger patients than male and older. 4. There is no direct relationship betwwen occurance of gingival hyperplasia and meticulous oral hygiene. 5. The mechanism involved in a causation of diphenylhydantoin induced gingival hyperplasia has not been elucidated. 6. We recommand that diphenylhydantoin may be prescribed as the drug of the first choice for the control of epileptic seizure in the adolescent female patients and children especially receiving certain dental treatments.
Adolescent ; Child ; Epilepsy ; Female ; Gingiva ; Gingival Hyperplasia* ; Humans ; Hyperplasia ; Incidence ; Male ; Oral Hygiene ; Phenytoin ; Sodium

No abstract available.
Humans ; Pityriasis* ; Tinea Versicolor* ; Treatment Outcome*

Reviewing the results of the blood cultures performed at Yeungnam University Hospital during 4-year-period through January, 1, 1984 to December 31, 1987, the following results were obtained. 1) Out of 8083 blood specimens cultured microorganisms grew in 582 specimens with positivity rate of 7.20%. Polymicrobial bacteremia was found in 16 patients. 2) Among 582 positive specimens, Gram-positive cocci grew in 189 specimens, and Gram-negative bacilli, in 393 specimens. Clinically significant microorganisms consisted of 82 Staphylococcus aureus, and 20 Streptococcus species in Gram-positive cocci group, 80 Salmonella typhi, 72 Escherichia coli, 72 Salmonella paratyphi A in Enterobacteriaceae, and 46 Pseudomonas cepacia, and 16 Pseudomonas aeruginosa in glucose non-fermenting microorganisms. 3) Increasing incidence of Serratia, Acinetobacter and Pseudomonas species as major nosocomial infection source is noteworthy. They showed increased tendency from 6.3% of 1984 to 17.7% of 1987 of total positive blood cultures. 4) High isolation rate of Pseudomonas species and Aeromonas hydrophila was noted in summer, while Salmonella typhi showed high prevalence from May to September and in January. 5) In susceptibility tests of isolated organisms, staphylococcus aureus was sensitive to basic antimicrobial agents except for ampicillin. The glucose non-fermenting microorganisms showed high resistance to basic antimicrobial agents in 32.2%. In conclusion, considering the relatively higher incidence of growth of Staphylococcus epidermidis than ideal level indicates that sampling technique should be improved. Secondly, all the hospital staffs in cooperation with Hospital Infection Committee are desirable to pay efforts to decrease the nosocomial infection.
Acinetobacter ; Aeromonas hydrophila ; Ampicillin ; Anti-Infective Agents ; Bacteremia ; Burkholderia cepacia ; Cross Infection ; Enterobacteriaceae ; Escherichia coli ; Glucose ; Gram-Positive Cocci ; Humans ; Incidence ; Prevalence ; Pseudomonas ; Pseudomonas aeruginosa ; Salmonella paratyphi A ; Salmonella typhi ; Serratia ; Staphylococcus aureus ; Staphylococcus epidermidis ; Streptococcus