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

The minor fissure is an important anatomical landmark in the localization of the pulmonary diseases. For the evaluation of the normal feature of the minor fissure, we analyzed the high-resolution computed tomography (CT) scans in 51 normal patients. The purposes of this study are to evaluate the normal appearance of the minor fissure on high-resolution CT scans and to compare it with that on the coventional CT and chest radiographs. We analysed the morphologic feature of the minor fissure on the high-resolution CT scans in 51 normal patients, and compared it with that on the conventional CT scans. On the high-resolution CT scans, we particularly paid attention to the completeness and types according to Berkmen classification. And finally, we compared the types determined by the high-resolution CT scans with those by the plain radiographs. In most patients (n=47), the minor fissure was seen as a hyperattenuating line or hand on the high-resolution CT scans. In contrast, it was mostly seen as a lucent zone on the conventional CT scans (n=44). Of 47 patients having a hyperattenuating line or band on the high resolution CT scans, the minor fissure was considered to be complete in 17 patients (36%), and incomplete in 30 patients (64%), who had defect at medial portion of the minor fissure. The most common type of the minor fissure seen on the high-resolution CT scans was type I variety (n=23), followed by type IIa (n=10) and type II (n=8). We could not determine the type in six patients. The type determined by the high resolution CT scans was highly well correlated with that determined by the plain radiographs (p<0.05). In conclusion, the minor fissure was seen on CT studies as variable appearances and high-resolution CT scans were superior to the conventional CT scans in the evaluation of the minor fissure. The type of the minor fissure determined by the high-resolution CT scans were well correlated with those seen on the radiographs.
Classification ; Hand ; Humans ; Lung Diseases ; Radiography, Thoracic ; Tomography, X-Ray Computed

The minor fissure is an important anatomical landmark in the localization of the pulmonary diseases. For the evaluation of the normal feature of the minor fissure, we analyzed the high-resolution computed tomography (CT) scans in 51 normal patients. The purposes of this study are to evaluate the normal appearance of the minor fissure on high-resolution CT scans and to compare it with that on the coventional CT and chest radiographs. We analysed the morphologic feature of the minor fissure on the high-resolution CT scans in 51 normal patients, and compared it with that on the conventional CT scans. On the high-resolution CT scans, we particularly paid attention to the completeness and types according to Berkmen classification. And finally, we compared the types determined by the high-resolution CT scans with those by the plain radiographs. In most patients (n=47), the minor fissure was seen as a hyperattenuating line or hand on the high-resolution CT scans. In contrast, it was mostly seen as a lucent zone on the conventional CT scans (n=44). Of 47 patients having a hyperattenuating line or band on the high resolution CT scans, the minor fissure was considered to be complete in 17 patients (36%), and incomplete in 30 patients (64%), who had defect at medial portion of the minor fissure. The most common type of the minor fissure seen on the high-resolution CT scans was type I variety (n=23), followed by type IIa (n=10) and type II (n=8). We could not determine the type in six patients. The type determined by the high resolution CT scans was highly well correlated with that determined by the plain radiographs (p<0.05). In conclusion, the minor fissure was seen on CT studies as variable appearances and high-resolution CT scans were superior to the conventional CT scans in the evaluation of the minor fissure. The type of the minor fissure determined by the high-resolution CT scans were well correlated with those seen on the radiographs.
Classification ; Hand ; Humans ; Lung Diseases ; Radiography, Thoracic ; Tomography, X-Ray Computed

PURPOSE: We wanted to report on the efficacy and safety of the percutaneous biliary stone removal technique using hydraulic pressure after balloon sphincteroplasty through the PTBD tract for patients with bile duct stones. MATERIALS AND METHODS: The subjects of this study were 85 patients (46 men and 39 women) with bile duct stones who came to hospital over a period of the previous 4 years. All subjects had undergone attempts for with the biliary tree through PTBD. First, an 8-9 F sheath was inserted into the biliary tree through the PTBD route by using a balloon catheter prior to sphincteroplasty, and 50cc of hydraulic pressure with contrast-mixed saline solution was then injected via the sheath. Follow-up cholangiogram was performed 1-3 days later to evaluate the results of stone removal. For residual stones, we attempted second, third, and fourth trials to completely remove the stones. The size and number of stones were analyzed. The results were analyzed, together with the complications, after classifying the cases as "success", "partial removal" or "failure" according to the number of remaining stones. RESULTS: Out of 85 patients, 71 (83%) cases had successful results, and 43 (51%) cases resulted in success with the first attempt. The second, third and fourth trials were conducted on 16, 10 and 2 cases, respectively. Out of 14 failure cases, 10 patients had too many intrahepatic duct stones. The complications were abdominal pain (n=21), fever (n=9), and pancreatitis (n=2), and portal vein thrombosis, biloma and sepsis were also found in 1 case each. CONCLUSION: We report that this percutaneus biliary stone removal technique using hydraulic pressure after balloon sphincteroplasty through the PTBD is safe and effective, and particularly, it achieves good results as the primary therapy for treating only choledocholiths.
Abdominal Pain ; Bile Ducts ; Biliary Tract ; Calculi ; Catheters ; Fever ; Follow-Up Studies ; Humans ; Male ; Pancreatitis ; Sepsis ; Sodium Chloride ; Venous Thrombosis

No abstract available.
Myocutaneous Flap* ; Pressure Ulcer*

Bronchobiliary fistulae are rare disorders, with inflammatory diseases of the liver, trauma, previous surgery and biliary obstruction being frequent causative factors. Endoscopic or transhepatic biliary drainage has been used successfully to avoid surgical treatment. We describe a case of a bronchobiliary fistula in a 78-year-old man with biliary obstruction caused by impacted calculi. Without surgical or endoscopic intervention, fistulae were treated by percutaneous transhepatic biliary drainage and removal of calculi, in conjunction with balloon sphincteroplasty.
Aged ; Biliary Fistula ; Calculi ; Cholelithiasis* ; Drainage ; Fistula* ; Humans ; Liver

Gastrointestinal submucosal tumors originate from submucosal histologic structures such as muscles, lymph nodes, nerves, fibers and vessels. Most patients are asymptomatic. Lesions that are large or ulcerated may cause abdominal pain or upper gastrointestinal bleeding, and those that grow intraluminally sometimes become pedunculated and occasionally prolapse to cause intussusception. Adenocarcinoma is the most common primary gastrointestinal tumor, accounting for approximately 90-9 5 % of such lesions, while submucosal tumors account for approximately 2 -6% of all gastrointestinal tumors. Because their overlying mucosa appears normal, submucosal tumors age after difficult to visualize endo-scopically, and for this reason, barium studies or CT scans are helpful for diagnosis. In this paper, variable CT and barium study findings of the different types of gastrointestinal submucosal tumor are demonstrated, and a brief discussion of the respective disease entities is included.
Abdominal Pain ; Adenocarcinoma ; Barium ; Diagnosis ; Gastrointestinal Tract* ; Hemorrhage ; Humans ; Intussusception ; Lymph Nodes ; Mucous Membrane ; Muscles ; Prolapse ; Tomography, X-Ray Computed ; Ulcer

Since Kappis described percutaneous celiac plexus block in 1914, variations and refinements of his technic have been proposed. Recently, computed tomography (CT) guided nerve block has improved results of the block and made it safer, particularly when neurolytic drugs are injected for the relief of intractable abdominal pain caused by malignancy or chronic pancreatitis. We report a case of CT-guided alcohol block of the celiac plexus with the anterior approach in a patient with a common bile duct carcinoma.
Abdominal Pain ; Celiac Plexus* ; Common Bile Duct ; Humans ; Nerve Block ; Pancreatitis, Chronic

Since Kappis described percutaneous celiac plexus block in 1914, variations and refinements of his technic have been proposed. Recently, computed tomography (CT) guided nerve block has improved results of the block and made it safer, particularly when neurolytic drugs are injected for the relief of intractable abdominal pain caused by malignancy or chronic pancreatitis. We report a case of CT-guided alcohol block of the celiac plexus with the anterior approach in a patient with a common bile duct carcinoma.
Abdominal Pain ; Celiac Plexus* ; Common Bile Duct ; Humans ; Nerve Block ; Pancreatitis, Chronic