No abstract available.
Encephalomyelitis, Acute Disseminated* ; Magnetic Resonance Imaging

BACKGROUND: Hemophagocytic lymphohistiocytosis(HLH) is a rare and fatal disorder in children. Recently its clinical characteristics and etiology of uncontrolled activation of cellular immune system in genetically predirected patients have keen elucidated. The authors analyzed the clinical characteristics and response to immunosuppressive agents of HLH patients in single institute. METHODS: The authors retrospectively analyzed various clinical data including CSF and bone marrow examination at diagnosis and follow up period in the 6 patients who were diagnosed as HLH at Asan Medical Center during last 2 years. Antithymocyte globulin(ATG : 10 mg/kg/day) and methylprednisolone(methyl-PD: 5 mg/kg/day) for 5 consecutive days as induction treatment and cyclosporin A(CsA) as maintenance treatment after induction with weekly intrathecal methotrexate for 5-6 weeks were given to the recently diagnosed 3 patients. RESULTS: All the patients except one were infants. Persistent fever, hepatosplenomegaly and pancytopenia were observed in all the patients. Family history of suspicious HLH was observed in 4 patients(67%). The characteristic elevated serum triglyceride(TG) level and/or decreased fibrinogen level were noted in all. Mild to moderate CSF pleocytosls with increased lymphocytes and monocytes was also observed in all during the disease course. Variable degree of nonqr-Langerhans cell histiocytic infiltration and hemophagocytosis were observed in all the cases. Of the 3 patients treated with ATG and methyl-PD, one achieved complete remission and the others achieved partial remission within 2 weeks of treatment, but all expired after 5 months, remission with CsA. Regardless of treatment regimen, all the 6 patients expired due to CNS sequelae of HLH. CONCLUSIONS: HLH mainly developed in infants. Persistant fever, hepatosplenomegaly and pancytopenia were observed in most cases with the characteristic change of serum TG and/or fibrinogen level. HLH should be included in the differential diagnosis in patients with these features especially when the family history of suspicious HLH is present, and histologic comfirmation of HLH could be easily accomplished with bone marrow study in most cases. Remission induction of HLH could be achieved with immunosuppressive treatment but it was difficult to maintain long term remission.
Bone Marrow ; Bone Marrow Examination ; Child ; Chungcheongnam-do ; Cyclosporine ; Diagnosis ; Diagnosis, Differential ; Fever ; Fibrinogen ; Follow-Up Studies ; Humans ; Immune System ; Immunosuppressive Agents ; Infant ; Lymphocytes ; Lymphohistiocytosis, Hemophagocytic* ; Methotrexate ; Monocytes ; Pancytopenia ; Remission Induction ; Retrospective Studies

BACKGROUND: Despite the development of empiric antibiotics in treatment of neutropenic fever(NF) in pediatric cancer patients, bacterial infection is still the most important cause of death in these patients. In this study the authors analyzed clinical characteristics and tried to find out the possible risk factors of the pediatric cancer patients who succumbed to the documented bacterial infection after episodes of neutropenic fever. METHODS: 17 pediatric cancer patients expired after episodes of neutropenic fever(NF) in the pediatric department of Asan Medical Center from Mar. 1990 to Feb. 1996 were grouped by the results of bacterial culture. 7 cases (Group A) had documented bacterial culture results and 10 cases (Group B) had negative culture results. The clinical characteristics of these two groups were analyzed retrospectively. RESULTS: There were no differences in the types of cancer between the two groups. All the documented bacteria were gram negative organisms and all cultured from the blood. There were no differences between the two groups in the treatment duration, known risk factors of infection before and at the onset of fever, antibiotics administered, and interval from the onset of NF to the administration of antibiotics. The response rate of initial empiric antibiotics was lower in group A(14%) compared to Group B(40%), even though all the cultured organisms except 2 cases in Group A were sensitive to initial empirical antibiotics. Septic shock was the cause of death in 86% of Group A patients, but only in 10% of Group B patients. The other causes of death were progression of cancer, bleeding, hepatic failure, adult respiratory distress syndrome and multiple organ failure, which showed no difference between the 2 groups. The interval from the onset of fever to death was significantly shorter in Group A(6.2+/-2.3 days) compared to Group B(24.9+/-18.6 days). CONCLUSIONS: Bacterial infection is still the most frequent cause of death after NF. Most patients succumbed to the documented bacterial infection showed rapid progression of bacteremia to septic shock despite administration of sensitive antibiotics. The known risk factors of infection before the onset of NF and other treatment factors shows no differences between the two groups in this study. These results suggest that the bacteremia is obvious risk factor of poor outcome after NF episode, and antibiotics alone may be insufficient to prevent the rapid progression of septic shock in these patients.
Anti-Bacterial Agents ; Bacteremia ; Bacteria ; Bacterial Infections ; Cause of Death* ; Chungcheongnam-do ; Fever* ; Hemorrhage ; Humans ; Liver Failure ; Multiple Organ Failure ; Respiratory Distress Syndrome, Adult ; Retrospective Studies ; Risk Factors ; Shock, Septic

Thirty cases of peripheral nerve injury were selected and analysed by means of electromyographic studies at Severance Hospital, Yonsei University, from January 1972 to August 1972. 1. The sex ratio was 17:13 (male: female). The peak incidence occurred in the twenty to thirty year age group. Involved side: right side 18 cases: left side 12 cases. 2. Nerve involved: peroneal nerve (15 cases), tibial nerve (2 cases), median nerve (7 cases), ulnar nerve (4 cases) and radial nerve (2 case). 3. Mode of nerve injuries 1) Peroneal nerve: Nerve compression, variable (10 cases) Tibia & fibular fracture complication (4 cases) Stab wound, fibular neck region (1 case) 2) Tibial nerve: Injection neuritis, buttock (2 cases) 3) Median nerve: Cut glass laceration, wrist region (3 cases) Carpal tunnel syndrome (3 cases) Undetermined mode (1 case) 4) Ulnar nerve: Cut glass laceration, wrst region (2 cases) Forearm bones fracture complication (1 case) Supracondylar fracture (cubitus valgus) (1 case) 5) Radial nerve: Cut glass laceration, wrist region (1 case) Crushing injury, elbow region (1 case) 4. Among the 15 cases of peroneal nerve injury, there were 10 cases of partial denervation and 5 cases of complete denervation. 5. The mode of injury in 10 cases of partial denervation was nerve compression from the following causes: lithotomy posture during forceps delivery (1 case), external rotation of legs during recovery state after surgery (3 cases), abnormal posture during coma state after CO intoxication (2 cases), tight long leg cast (3 cases), and direct trauma while descending stairs (1 case). 6. In all 10 cases of partial denervation of the peroneal nerve, decreased motor nerve conduction velocities, diminished amplitude and prolonged latencies were observed. 7. In partial denervation of the peroneal nerve, the earliest that reinnervation was observed was within 3 weeks in 3 out of 10 cases. 8. Spontaneous fibrillation was observed 3weeks after injury in all cases except one in which it was observed only 7 days after peroneal nerve injury. 9, The earliest appearance of positive sharp waves among all peroneal nerve injuries was observed 2 weeks after injury. 10. The initial appearance of nascent potentials (polyphasic potentials) in peroneal nerve injuries was observed 3 weeks after injury (1 case), 5 weeks after injury (1 case), and 5 months after injury (1 case). They are all partial denervation cases due to nerve compression. 11. Ten cases of partial denervation of the peroneal nerve were treated with electrical stimulation and drop foot board with good recovery. Five cases of complete denervation of the peroneal nerve were treated with neurorrhaphy (1 case) and short leg bracing (4 cases). 12. The initial appearance, of polyphasic potentials was observed 5 months after injury in one case out of 3 cases of median nerve injury. 13. Three cases of median nerve injury were classified as carpal tunnel syndrome and occurred in women only. Observations included absence of sensory nerve action potentials below the lesion, delayed distal latency at wrist and normal motor nerve conduction velocity. They were treated by division of the deep transverse carpal ligament with good result. 14. The initial appearance of nascent potentials was observed 7 months after injury in one case among 3 cases of ulnar nerve injury. 15. In two cases each of radial and tibial nerve injury, no reinnervation pattern was observed until the six month follow-up study after injury. At that time no muscle contraction could be seen or palpated clinically. 16. We observed normal motor unit potentials in the muscles in the follow-up studies even though muscle contraction could not be seen or palpated clinically. 17. Electromyographic examination at selected intervals made accurate diagnosis and prognosis possible and aided in evaluating the course of nerve regeneration, which permictted the choice of appropriate treatment.
Action Potentials ; Braces ; Buttocks ; Carpal Tunnel Syndrome ; Coma ; Denervation ; Diagnosis ; Elbow ; Electric Stimulation ; Extremities ; Female ; Follow-Up Studies ; Foot ; Forearm ; Glass ; Humans ; Incidence ; Lacerations ; Leg ; Ligaments ; Median Nerve ; Muscle Contraction ; Muscles ; Neck ; Nerve Regeneration ; Neural Conduction ; Neuritis ; Peripheral Nerve Injuries ; Peripheral Nerves ; Peroneal Nerve ; Posture ; Prognosis ; Radial Nerve ; Sex Ratio ; Surgical Instruments ; Tibia ; Tibial Nerve ; Ulnar Nerve ; Wounds, Stab ; Wrist

Patients with atypical or incomplete Kawasaki disease are at same risk for development of coronary artery complications as typical Kawasaki disease. In this communication we report six patients with unusual presentation of Kawasaki disease complicated by coronary artery aneurysms, in whom correct diagnosis were not made in time for proper treatment. One of these patients died from massive myocardial ischemia due to giant aneurysms along the entire coronary artery system. These patients had either less than enough number of diagnostic criteria at initial presentation or diagnostic signs which occurred over an extended period of time, resulting in difficulty in diagnosis during the acute phase. As a result, none of these patients received intravenous gamma globulin treatment. Thus strict adherence to currently accepted criteria for diagnosis of Kawasaki disease may lead to failure to recognize atypical form of this illness with potential sequelae of myocardial ischemia or sudden death. We would like to emphasize from this experience that clinicians must be aware of the wide variations in clinical presentation of Kawasaki disease and take an aggressive approach in making correct diagnosis by obtaining early cardiac evaluation in order to initiate prompt treatment with intravenous gamma globulin.
Aneurysm ; Coronary Aneurysm* ; Coronary Vessels ; Death, Sudden ; Diagnosis ; gamma-Globulins ; Humans ; Mucocutaneous Lymph Node Syndrome* ; Myocardial Ischemia

No abstract available.

Rett syndrome is progressive neurodegenerative disorder in female patients, characterized by autistic behavior, mental retardation, loss of purposeful hand skills, stereotypic hand movement, breathing dysfunction, severely impaired language, ataxia, and seizure. The diagnosis of Rett syndrome is based on its characteristic clinical manifestation and course. The electroencephalographic (EEG) findings of Rett syndrome are nonspecific, but a progressive deterioration in the EEG, characterized by a slowing of background activity and spike sharp wave discharges, may be observed. We experienced one case of Rett syndrome in a 5 year old girl having mental retardation, loss of purposeful hand skills, stereotypic hand movements (clapping, washing, hand-to-mouth), breathing dysfunction (hyperventilation/apnea). Her EEG findings on Video-EEG monitoring are excessive slowing waves during awake state and frequent spike discharges from left or centrotemporal area during sleeping. We report a case of Rett syndrome with brief review of related literatures.
Ataxia ; Child, Preschool ; Diagnosis ; Electroencephalography ; Female ; Hand ; Humans ; Intellectual Disability ; Neurodegenerative Diseases ; Respiration ; Rett Syndrome* ; Seizures

No abstract available.
Gaucher Disease*

No abstract available.
Hypoglycemia*

We evaluated the distribution of normal conjuctival flora and the change of antibiotic sensitivity of cultured bacteria. Specimens were obtained from inferior conjunctiva cul de sac with sterile cotton-tipped applicator from healthy 216 human beings. Antibiotic sensitivity test was done by disk diffusion method. The results indicated that overall positive culture rate was 47.7% and major organisms were Coagulase-negative Staphylococcus(55.7%), Staphylococcus aureus(16%), Gram negative bacteria(7.5%), Streptococcus(4.7%). Antibiotic sensitivity test of cultured Gram positive bacteria demonstrated less than 60% rate of susceptability in gentamicin, tetracyclin, erythromycin and 80.5% rate of susceptability in ciprofloxacin. Gram negative bacteria demonstrated less than 60% rate of susceptability in cephalothin, ampicillin. From the above results, we concluded that antibiotic resistance of normal conjunctival flora was increased from the past and the principle of antiviotic eye drop use should be emphasized.
Ampicillin ; Bacteria ; Cephalothin ; Ciprofloxacin ; Conjunctiva ; Diffusion ; Drug Resistance, Microbial ; Erythromycin ; Gentamicins ; Gram-Negative Bacteria ; Gram-Positive Bacteria ; Humans ; Staphylococcus