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

Intracavernous (IC) injection therapy of vasoactive agents is now being widely choser as the first armamentarium for the treatment of male erectile dysfunction, nevertheless, has a troublesome problems due to the drug delivery vehicle with needle. Therefore, the less invasive route of drug administration has been needed. We investigated the efficacy and safety of intraurethral (IU) instillation of lyposomal prostaglandin E1 on the induction of penile erection. Thirty patients with male erectile dysfunction were enrolled in a prospective study. Overall, 73.3% of cases were responded with 33.3% of full erection and 40% of partial erection after IU instillation. IU self-instillation at home was more effective than at clinic on the induction of penile erection. It is somewhat effective in cases of diabetogenic, psychogenic, neurogenic and mild vasculogenic erectile dysfunction but much less effective in severe vasculogenic and cavernosal disease. Interval of onset and duration of erection were delayed and shortened in IU instillation rather than IC injection. Side effect were mild and transient urethral pain with the most frequent incidence and followed by transient hypotension in less. In conclusion, intraurethral instillation of lyposomal prostaglandin E1 can be selected as one of therapeutic modality to restore the penile erection for some limited patients with erectile dysfunction to want to select a less invasive treatment lternative.
Alprostadil* ; Erectile Dysfunction ; Humans ; Hypotension ; Incidence ; Male ; Needles ; Penile Erection* ; Prospective Studies

Coenzyme Q is concentrated in Golgi apparatus membranes and mitochondria, but not in other membranes. Although it is difficult to prove the metabolic action of coenzyme Q administered exogenously in clinical cases, the effect of this substance can be evaluated by criteria based on clinical findings. In an attempt to evaluate the effect of coenzyme Q for the treatment of 67 patients(male 26 cases, female 41 cases) of congestive heart failure, we administered Coenzyme Q1030mg daily for 4 to 8 weeks. Most of them were valvular heart disease(74.6%) and hypertension (14.9%). Clinical effects were evaluated at least 4 weeks later by the criteria using a scoring method of severity of congestive heart failure which was devised by Ishiyama, etc. In summary, a definite effect was found in 13 cases(19%) and a mild effect was observed in 46 cases(69%). During treatment there were no significant side effects, and also no significant changes in heart rate and blood pressure.
Blood Pressure ; Estrogens, Conjugated (USP)* ; Female ; Golgi Apparatus ; Heart ; Heart Failure* ; Heart Rate ; Humans ; Hypertension ; Membranes ; Mitochondria ; Research Design ; Ubiquinone

Purpose: The prevalence of microdeletion of the Y chromosome is 13% in non-obstructive azoospermic patients. Klinefelter's syndrome may be found in about 11% of azoospermic patients. The prevalence and correlation of microdeletion of the Y chromosome in Klinefelter's syndrome, which is the most common cause of chromosomal disorders in male infertility, were investigated. Materials and Methods: Hormone tests (Testosterone, LH and FSH) were performed and peripheral genomic DNA of 82 patients detected as Klinefelter's syndrome between September 2001 and December 2003. The microdeletion of the Y chromosome was examined by a PCR technique. The primers used for the PCR were Sequence-Tagged sites (STS) of the long arm of the Y chromosome (sY84, sY129, sY134, sY254 and sY255) and SRY (control). Results: The mean age, and values of testosterone, LH and FSH in the 82 Klinefelter's syndrome patients were 32.71 3.13 years, 1.84 1.31ng/ml, 14.88+/-5.38mlU/ml and 38.79 12.40mlU/ml, respectively. No patient in this study was found to have Y chromosomal microdeletion. Conclusions: As the role of the Y chromosome in the spermatogenesis of male is well known, microdeletion of the Y chromosome causes severe damage to the spermatogenesis in infertile males. A microdeletion of the Y chromosome could not be detected in patients with Klinefelter's syndrome. Therefore, multiple factors or other mechanisms that influence the defect of spermatogenesis in Klinefelter's syndrome may exist.
Arm ; Chromosome Deletion ; Chromosome Disorders ; DNA ; Humans ; Infertility, Male ; Klinefelter Syndrome* ; Male ; Polymerase Chain Reaction ; Prevalence ; Sequence Tagged Sites ; Spermatogenesis ; Testosterone ; Y Chromosome*

No abstract available.
Caroli Disease*

PURPOSE: To determine the efficacy of CarnitilR (acetylcarnitine, Hanmi, Korea) therapy in idiopathic oligoasthenospermic men. MATERIALS AND METHODS: Forty-four subfertile men with abnormal semen parameters were treated between March, 2003 and March, 2004 with 3 g of CarnitilR daily for 3 months. Changes in semen parameters were evaluated 3 months after this therapy. RESULTS: The mean age was 34.2 years and the mean follow-up duration was 3.7 months. In asthenospemic patients (n=28), semen analysis before and after CarnitilR treatment showed an increase in volume (2.64+/-1.65 ml vs. 3.10+/-1.60 ml), motility (35.1+/-17.7% vs. 45.9+/-20.4%) and viability (51.4+/-20.3% vs. 59.3+/-13.6%) respectively. In oligoasthenospermic patients (n=16), semen analysis before and after CarnitilR treatment showed an increase in sperm count (10.7+/-54.4 million/ml vs. 38.4+/-32.5 million/ml) respectively. CONCLUSIONS: These results suggested that in idiopathic oligoasthenospermic men the empirical medical therapy with acetylcarnitine may be considered as primary treatment.
Acetylcarnitine* ; Carnitine ; Follow-Up Studies ; Humans ; Infertility, Male ; Male ; Semen ; Semen Analysis ; Sperm Count

A clinical study was conducted to compare effectiveness of IV Globulin with that of steriod in childhood acute ITP. We carried out an experiment to 36 patients of acute ITP who recovered over 50.000/mm(3) of platelet count and not to recur in 6 months. Study group consist of 10 children who were treated with steriod only (Group A), 13 children who were treated with IV Globulin (0.4g/kg/d, 5 days, Group B), and 13 children combined with steroid (Group C) Study period was from January 1988 to December 1992, folllowing results were obtained. 1) There were no significant difference in sex ratio. age distribution, pretreatment hemoglobin level, platelet count and prolonged bleeding time among study groups. 2) The days which platelet count reached to 50.000/mm(3), 100,000/mm(3) were 3.7 days (Group A), 2.1 days (Group B), and 2.3 days (Group C), respectively (p<0.05). 3) Complete response rates within 4 weeks did not show significant difference (90% in Group A, 84.6% in Group B, 92.3% in Group C), the complete response rate within 1 week were significantly different among three group (44.4% in Group A, 90.9% in Group B, 83.3% in Group C)(p<0.05). In conclusion, it is more desirable for early treatment of a childhood ITP because IV Globulin is easy to normalize platelet count in a short time.
Age Distribution ; Bleeding Time ; Child ; Humans ; Platelet Count ; Sex Ratio

Electrocardiograms were observed in 168 patients with typhoid fever and were analyzed according to the duration of illness and hemoglobin level. The following results were obtained. 1) 124 patients out of 168 patients showed abnormal electrocardiographic findings. 2) The most prevalent abnormal findings were observed during third to fourth week of illness. 3) The lower the hemoglobin level, the higher frequency of electrocardiographic abnormalities were found. 4) Among the electrocardiographic disorders, sinus tachycardia was the most common finding and it was found in 60 cases(35.7%). The major abnormalities were ST-T change, low QRS voltage, prolongation of Q-T interval and prolongation of P-R interval: the over all incidence of these were 26.8%, 15.5%, 13.7% and 9.55 respectively. In addition to the major abnormalities, incomplete right bundle branch block (5.4%), premature ventricular contractions(3.0%) and abnormal Q wave(1.2%) were observed.
Bundle-Branch Block ; Electrocardiography* ; Humans ; Incidence ; Tachycardia, Sinus ; Typhoid Fever*

The authors examined 3,169 fecal specimens from inhabitants of seven localities such as City, Eups and Myons in Cheju Do on July 1970 with cellophane thick smear technique. The results were analysed and summarized as follows: The overall egg positive rate of helminths was 82.2% and it was attributed to high rates of soil-transmitted helminths as in case of other parts in Korea. The infection rates of each species were; A. lumbricoides 44.3%, T. trichiurus 65.6%, hookworm 1.5%, T. orientalis 0.8%, C. sinensis 0.2%, P. westermani 0.4%, M. yokogawai 0.9%, Taenia sp. 12.7% and H. nana 1.4%. The infection rates of soil-transmitted helminths were relatively lower than those of mainland Korea especially in case of hookworm and T. orientalis. The proportion of unfertilized ova passers among the Ascaris infected case was 22.1%. The ova of heterophyids, most probably Metagonimus yokogawai were detected in lower percentage but discovered throughout the localities surveyed and in all age groups. It is certainly presumed that Metagonimus infection is autochthonous. The most interesting results were obtained in Taenia sp. infection and the higher rates were shown in rural area than in urbanized areas. The positive rates were within 5% in childhood and adolescence but abruptly increased up to 36.4 % in adults.
parasitology-helminth-nematoda-trematoda-cestoda ; Ascaris lumbricoides ; Trichuris trichiura ; hookworm ; Trichostrongylus orientalis ; Clonorchis sinensis ; Paragonimus westermani ; Metagonimus yokogawai ; Taenia sp. ; Hymenolepis nana