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

A case of dysplasia eplphyseaalis hemlmelica (Fairbank, 1956) in a 8 years old boy, showlng typical clinical and roentgenographical appearance, is reported together wlth a brief review of literature in this paper. This condition is a rare developmental disorder of childhood in which there is a asymmetrical cartilaginous overgrowth of one or more than one epiphysis, or of a tarsal or carpal bone. Thls disorder is usually Iimited to elther the medial or lateral half of a single extremity. The disease is characterised radiographically by delay in the appearance of the epiphyseal nucleus, which is mottied and of irregular density. The most common deformities were genu valgum or varum and valgus or equinus deformity of the ankle. These deformities tended to increased wlth growth but the size of the lesion increased in proportion to the growth of the normal bone. Concomitant involvement of the knee and ankle is the rule, and in those cases the affected limb Is usually longer than Its mate. The lesion Is often microscopically indistingulshable from an osteochondroma.
Ankle ; Carpal Bones ; Congenital Abnormalities ; Epiphyses ; Equinus Deformity ; Extremities ; Genu Valgum ; Humans ; Knee ; Male ; Osteochondroma

OBJECTIVE: This study was done to evaluate the risk factors and prevalence of symptom-giving pelvic girdle relaxation in pregnant women. METHODS: Three hundreds thirty-two postpartum women were asked to fill out a questionnaire within one week after their parturition. The diagnostic criteria of this lesion were adopted from the Norwegian Medical Association's and Larsen's criteria. The questionnaire were included age, parity, BMI(kg/cm2), weight gain during the pregnancy, history of symptom giving pelvic girdle relaxation in previous pregnancy, level of exercise and vocational status during and before the pregnancy and baby's birth weight. The answers were evaluated to determine the risk factors. The correlation was tested by student t-test and logistic regression. RESULTS: The prevalence of symptom-giving pelvic girdle relaxation during pregnancy was 18.9%. The history of symptom-giving pelvic girdle relaxation in previous pregnancy, multiparity and the absence of regular exercise before and during pregnancy showed correlation with symptom-giving pelvic girdle relaxation in pregnancy (p<0.05). CONCLUSION: It is suggested that women who experienced symptom-giving pelvic girdle relaxation in previous pregnancy should be checked for this lesion before pregnancy. Regular exercise is required to lower the incidence and lessen the severity of this lesion in pregnancy.
Birth Weight ; Female ; Humans ; Incidence ; Logistic Models ; Parity ; Parturition ; Postpartum Period ; Pregnancy ; Pregnant Women* ; Prevalence* ; Surveys and Questionnaire ; Relaxation* ; Risk Factors* ; Weight Gain

To develop a survival prediction model and to use it as a therapeutic guideline a series of 66 cases with ganglio-thalamic hemorrhage accompanying intraventricular hemorrhage were analyzed retrospectively. They were classified into the benign and fatal groups according to their final outcomes. Fourteen indices obtained from the initial CT scans were final outcomes. Fourteen indices obtained from the initial CT scans were subjected to multivariate discriminant analysis. The resultant discriminant function(Z) included the amount of hematoma in the parenchyme(AH), bicaudate cerebroventricular index(BCCI) maximum fourth ventricular width(FVW), and third cerebroventricular ratio(TCR) in an order of decreasing discriminating power and was as follows: Z = -3.2639 + 0.3508 X 10(-1) X AH + 6.8816 X BCCI + 0.1139 X FVW-5.7794 X TCR. This function predicted survivability with accuracy of 84.9% when it was applied to the original subjects. The conclusion is that AH, BCCI, FVW, and TCR are the potent predictors of the survival of patients with ganglio-thalamic hemorrhage accompanying intraventricular hemorrhage.
Hematoma ; Hemorrhage* ; Humans ; Retrospective Studies ; Tomography, X-Ray Computed*

Cutaneous metastases from internal carcinomas are relatively rare, especially from pancreatic carcinoma. Pancreatic carcinomas are usually adenocarcinomas which arise in the head of the gland, and are known to rapidly metastasize to the lymphatic system by permeation and embolization. We report a case of cutaneous metastasis from pancreatic adenocarcinoma. in 74-year-old male patient who have two pea sized, slight erythematous nodules on the lower abdomen and posterior side of the neck.
Abdomen ; Adenocarcinoma* ; Aged ; Head ; Humans ; Lymphatic System ; Male ; Neck ; Neoplasm Metastasis* ; Peas

Postoperative endophthalmitis has been greatly improved over the past decade by the use of improved medical therapy and the use of vitrectomy. However, the appropriate use of the different treatment modalities and the appropriate time of vitrectomy in the treatment of postoperative endophthalmitis remains controversial. We have experienced consecutive endophthalmitis following 2 cases of cataract surgery, 1 case of glaucoma filtering surgery with cataract operation and 1 case of secondary posterior chamber intraocular lens(PCL) implantation. We report the presumed cause of endophthalmitis and its therapeutic results. 1. Four of five patients who underwent cataract and combined glaucoma surgery were detected Pseudomonas aeruginosa and its infective cause presumed to be the I and A Kit of phacoemulsifier(Cavltron Kelman 8000). 2. More severe 3 of 4 patients who developed postoperative endohthalmitis were treated with vitrectomy and mild 1 patient was treated medically. 3. At the time of follow-up(mean, 11 months), the final corrected average visual acuity of 20/30 was achieved.
Cataract ; Endophthalmitis* ; Filtering Surgery ; Glaucoma ; Humans ; Pseudomonas aeruginosa ; Visual Acuity ; Vitrectomy

OBJECTIVE: For the treatment of carotid cavernous fistula(CCF), transarterial detachable balloon occlusion(DBO) is the method of choice. When it has failed to occlude the fistula, various embolization methods are used to treat the fistula. Transvenous embolization through the superior ophthalmic vein(SOV) is another method of treatment. The venous approach through the SOV after surgical dissection and exposure of this vein has been recommended by some delete, but(here) delete delete(an) alternative treatment method by percutaneous puncture of the SOV without surgical dissection(is described). METHODS: A 19-year-old woman admitted to our hospital two months after accident, presented with proptosis, chemosis, occulomotor and abducens nerve palsies, and bruit of the right eye. The authors tried DBO via transarterial route in initial treatment and the fistula was occluded with subsequent disapearance of bruit. However, 2 weeks later, she complained of recurence of bruit. Transarterial approach was attempted again, but the fistula hole was too small for this approach. The venous approach via SOV by percutaneous puncture was then tried. Puncture was made at the medial one third of the superior orbital rim and the fistula was embolized with Guglielmi detachable coils (GDCs). RESULTS: The fistula was completely occluded and no early and late complications noted. The patient's clinical symptoms were improved within a few days. CONCLUSION: Treatment of CCF by percutaneous puncture of the SOV is an alternative and effective method when other approaches are not feasible.
Abducens Nerve Diseases ; Exophthalmos ; Female ; Fistula* ; Humans ; Orbit ; Punctures* ; Veins* ; Young Adult

Complications of mitomycin C soaking method during pterygium surgery and its effect on recurrence rate were compared with postoperative use of topical mitomycin C. Sixty three patients(seventy three eyes) with primary pterygium received surgery with the bared sclera method. Thirty nine eyes randomly chosen were treated with cotton applicator soaked with 0.2mg/ml mitomycin C for 5 minutes after the excision of pterygium(soaking group). Thirty four eyes underwent the same surgical procedure and received topical 0.2mg/ml mitomycin C postoperatively, twice a day for five days(eye drop group). During the mean follow-up period of 13 months, recurrence rate was 15.4%(6 eyes) in soaking group and 20.6%(7 eyes) in eye drop group respectively. This difference was not statistically significant(p>0.5). There were no specific complications related to mitomycin C treatment in both groups during the follow-up periods. The soaking method of 0.2mg/ml mitomycin C was found as effective and safe as postoperative use of topical 0.2mg/ml mitomycin C for prevention of the recurrence of primary pterygium.
Follow-Up Studies ; Mitomycin* ; Pterygium* ; Recurrence ; Sclera

Inflammatory pseudotumor, originally described in the lung, is a relatively rare tumor-like lesion that occurs in various organs and tissues. It is usually well demarcated from the surrounding tissue, however it can be unfortunately resected as a malignant tumor. A few inflammtory pseudotumor in the kidney have been reported in English literature, but there have been no reports in Korea. We report a case with inflammatory pseudotumor of the kidney. A 48 year old woman had an intermittent flank pain on the right side. An ultrasonographic study suggested a renal cell carcinoma and a nephrectomy was done. Grossly, there were two separate masses with a well demarcated yellowish appearance, measuring 2.3 cm and 1.3 cm in diameter, respectively. Histologically, they were composed of smooth muscle actin positive spindle cells and a large number of foamy histiocytes, lymphocytes, and plasma cells in the fibrotic backgound.
Actins ; Carcinoma, Renal Cell ; Female ; Flank Pain ; Granuloma, Plasma Cell* ; Histiocytes ; Humans ; Kidney* ; Korea ; Lung ; Lymphocytes ; Middle Aged ; Muscle, Smooth ; Nephrectomy ; Plasma Cells