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

No abstract available.
Acute Kidney Injury*

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

No abstract available.
Thoracic Nerves*

No abstract available.
Evoked Potentials, Somatosensory* ; Spinal Stenosis* ; Spine*

No abstract available.
H-Reflex*

No abstract available.
Spinal Stenosis*

Basal Cell Nevus Syndrome ; Family Characteristics ; Female ; Follow-Up Studies ; Genetic Counseling ; Humans ; Mandible ; Mass Screening ; Mothers ; Odontogenic Cysts ; Recurrence ; Siblings ; Skin ; Young Adult

Rhabdomyosarcoma are reportedly the most common soft tissue sarcoma occuring in childhood, but the biliary tree is a rare site of origin for this tumor. Recently we experienced a case of embryonal rhabdomyosarcoma of the biliary tree in a 30-month-old child. UItrasonography showed hypoechoic mass filling the dilated left. intrahepatic & extrahepatic bile ducts, and CT showed hypodense mass with heterogeneous enhancement after contrast infusion. Intraoperative cholangiography showed filling defects within the dilated left. intrahepatic & extrahepatic bile ducts. Postoperative MRI showed residual mass within the left. intrahepatic duct which was hypointense on T1WI and hyperintense on T2WI.
Bile Ducts, Extrahepatic ; Biliary Tract* ; Child ; Child, Preschool ; Cholangiography ; Humans ; Magnetic Resonance Imaging ; Rhabdomyosarcoma ; Rhabdomyosarcoma, Embryonal* ; Sarcoma

Rhabdomyosarcoma are reportedly the most common soft tissue sarcoma occuring in childhood, but the biliary tree is a rare site of origin for this tumor. Recently we experienced a case of embryonal rhabdomyosarcoma of the biliary tree in a 30-month-old child. UItrasonography showed hypoechoic mass filling the dilated left. intrahepatic & extrahepatic bile ducts, and CT showed hypodense mass with heterogeneous enhancement after contrast infusion. Intraoperative cholangiography showed filling defects within the dilated left. intrahepatic & extrahepatic bile ducts. Postoperative MRI showed residual mass within the left. intrahepatic duct which was hypointense on T1WI and hyperintense on T2WI.
Bile Ducts, Extrahepatic ; Biliary Tract* ; Child ; Child, Preschool ; Cholangiography ; Humans ; Magnetic Resonance Imaging ; Rhabdomyosarcoma ; Rhabdomyosarcoma, Embryonal* ; Sarcoma