Cyclic Neutropenia is a benign, unusual hematologic disorder characterized by regularly recurring episodes of severe neutropenia occurring approximately every 21 days. Beside neutrophils, monocytes, lymphocytes, platelets and reticulocytes all cycle with strict periodicity suggest that this disease should be viewed as cyclic hematopoiesis, not merely as cyclic neutropenia. During neutropenic periods, patients regularly experience aphthous stomatitis, fever, malaise, cervical lymphadenitis, cutaneous infections and occasional pneumonia and otitis media. The exact cause of cyclic neutropenia is unknown. But it is strongly suggested that cyclic neurtopenia is due to an abnormality in the regulation of early hematopoietic precursor cells. We have documented a case of cyclic neutropenia for a period of 2.5 years in a 11 year-old boy who had suffered from recurrent fever, stomatitis, gingival swelling, cervical lymphadenitis and skin infections at 3 weeks intervals since 5 years of age. A brief review of the related literatures is presented.
Child ; Fever ; Hematopoiesis ; Humans ; Lymphadenitis ; Lymphocytes ; Male ; Monocytes ; Neutropenia* ; Neutrophils ; Otitis Media ; Periodicity ; Pneumonia ; Reticulocytes ; Skin ; Stomatitis ; Stomatitis, Aphthous

OBJECTIVES: Recently, the outpatient group psychotherapy has been changed from traditional long-term therapy to short-term or time-limited therapy. The termination time and session numbers are given from start line in the time-limited therapy while 10 to 40 sessions are needed in the short-term therapy. And therapist and patients come to an agreement of the accomplishable therapeutic goal and work together actively. The time-limited group psychotherapy is being accepted newly as an efficient therapeutic modality because effective therapeutic outcomes within given time have been reported. The objectives of this study are as follows; firstly, we examine the therapeutic outcomes and the therapeutic factors after performing the time-limited group psychotherapy for outpatients in the psychiatric clinic; secondly, we analyze the characteristics showed in the process of study; lastly, we verify the feasibility and the validity of this group psychotherapy and then establish the basis of the effective outpatient therapy that would be settled down as an applicable treatment modality economically and efficiently in the current medical environment of Korea. METHODS: This study was performed weekly in total 12 sessions from July 1999, April 2000 to and each session took 70-80 minutes. The experimental group were composed of the highly-functioning psychotic patients among outpatients in the psychiatric clinic. The control group were composed of the similar patients through one-to-one matching of age, sex, diagnosis, and duration of illness. The participants were 8 patients each. BDI(Beck Depression Inventory), STAI(Spielbergs State-Trait Anxiety Inventory), MMPI(Minnesota Multiphasic Personality Inventory), and EDMT(Ewha Defence Mechanism Test) as the evaluation scales were performed before and after treatment. And 13-therapeutic factors scale was performed just after each session and the self-report total assessment questionnaire was performed only in experimental group at the end of this study. RESULTS: The mean score of BDI was decreased significantly in experimental group in comparison with control group. The mean score of STAI-S was not changed significantly in both group, and the mean score of STAI-T was decreased in experimental group in comparison with control group. The mean score of MMPI was decreased significantly in experimental group in comparison with control group, but the comparison of difference scores(post-treatment score - pre-treatment score) between the experimental and control group was not decreased significantly. This result may come from psychopharmacotherapy and individual supportive psychotherapy given equally to both group. The mean scores of immature defence and neurotic defence in EDMT were decreased significantly in experimental group compared with control group. Comparing the therapeutic factors to divide into the first, middle, and the last stage, existential factor was ranked first, and catharsis, identification with therapist, universality, and group cohesiveness were ranked high in all stages. Interpersonal learning was ranked first in the last stage although being ranked low in the first and middle stage. The total assessment questionnaires about group psychotherapy were reported that 100% of participants were helped, 75% of participants were changed in their lives, and 75% of participants had minds to join this kind of program again if given for them. CONCLUSIONS: This result shows that not only symptom relief but also personality change was achieved when the time-limited group psychotherapy was practiced in addition to simple psychophamarcotherapy and individual supportive psychotherapy for outpatients. The degree of satisfaction for group psychotherapy of participants were evaluated highly. Group psychotherapy has been accepted in public as a therapeutic modality that must be as equally effective as psychotherapy although group psychotherapy derives from individual psychotherapy. In our current medical environment that has been changed suddenly by the separation of medical practice and pharmaceutical dispensing and the decrease of medical fee, group psychotherapy or time-limited therapy would be settled down as low-expense and high-efficiency therapeutic modalities that were as equally effective as individual psychotherapy or long-term therapy.
Anxiety ; Catharsis ; Depression ; Diagnosis ; Fees, Medical ; Humans ; Korea ; Learning ; MMPI ; Outpatients* ; Psychotherapy ; Psychotherapy, Group* ; Surveys and Questionnaires ; Weights and Measures

PURPOSE: The study was performed to investigate the IgG subclasses in acute febrile phase of Kawasaki disease(KD) to find correlation between selective IgG4 deficiency and incidence of KD patients. METHODS: The patients group consisted of 64 cases in acute phase of KD hospitalized from Jan. 1996 to Jul. 1999, and control group consisted of age matched 36 cases who admitted without KD. The obtained sera were measured the level of total IgG, IgM, IgA and IgG subclasses(IgG1, IgG2, IgG3, IgG4) by using Enzyme Immuno Assay and Single Radial Immuno-Diffusion method. RESULTS: The sex ratio of male to female was 1.4:1.0, and febrile days before admission was 4.6+/-2.4 days. Total levels of IgG, IgM and IgA were normal range in both groups. The levels of IgG1, IgG2 and IgG3 were not specific changes but significantly decreased the level of IgG4 in patients group as compared with control group(P<0.05). CONCLUSION: Selective decreased the levels of serum IgG4 can be one of factors to increasing the incidence of KD.
Female ; Humans ; Immunoglobulin A ; Immunoglobulin G* ; Immunoglobulin M ; Incidence ; Male ; Mucocutaneous Lymph Node Syndrome* ; Reference Values ; Sex Ratio

Tibialis anterior muscle hernia is the most common hernia among lower extremity muscles. This condition can be diagnosed by physical examination and radiologic findings, especially by dynamic ultrasonography. There are surgical methods of treatment for muscle hernia, including direct repair, fasciotomy, fascial patch grafting using autologous fascia lata or synthetic mesh. We report a case of tibialis anterior muscle hernia treated with local periosteal rotational flap. Because there are several advantages to the local periosteal rotational flap, such as lack of donor site morbidity, lack of skin irritation, low cost, simplicity, and an easy approach, this technique could be an option for tibialis anterior muscle hernia.
Fascia Lata ; Hernia ; Humans ; Lower Extremity ; Muscle, Skeletal ; Muscles ; Physical Examination ; Skin ; Tissue Donors ; Transplants

OBJECTIVES: The aim of this study was to evaluate the subjective well-being of schizophrenic patients who switched their antipsychotics to risperidone as the maintenance treatment over 24 weeks. METHODS: Thirty-one patients were evaluated using Positive and Negative Syndrome Scale (PANSS) for psychopathology and UKU side effects rating scale for drug-induced adverse events. Also the subjects were asked to score the 10 items self-rating scale (KmSWN-10), which derived from the 19-items of Korean modified version of Subjective Well-being under the neuroleptic treatment (KmSWN) scale. The assessments were administered at baseline, 2 weeks, 12 weeks and 24 weeks after switching to risperidone. Analysis were done using repeated measures of ANOVA. Pearson correlation analysis were also done to assess the correlation between KmSWN-10 and psychopathology and drug induced side effects. RESULTS: There were significant improvements on the scores of KmSWN-10 and reductions on the PANSS and UKU side effect rating scores during the treatment of risperidone. KmSWN-10 was negatively correlated with the total, negative and general psychopathology scales of PANSS at baseline, and also negatively corrleated with total, positive, negative, general psychopathology scales and depressive factor of PANSS scores and total UKU side effects rating scores at 24 weeks of treatment. CONCLUSION: The results suggest that the maintenance treatment with risperidone positively affected on the subjective well-being of schizophrenic patients due to improvement of psychotic symptoms and decrement of side effects. KmSWN-10 may be more related with negative and general psychopathlogy scales of PANSS in this study.
Antipsychotic Agents* ; Humans ; Psychopathology ; Quality of Life ; Risperidone* ; Schizophrenia ; Weights and Measures

PURPOSE: The aim of this study was to determine the potential usefulness of uterine artery embolization (UAE) for the management of uterine leiomyoma. MATERIALS AND METHODS: Sixty nine patients (mean age; 40.3 years, age range; 31-52 years) who underwent UAE for symptomatic fibroids (with menorrhagia, dysmenorrhea and bulk-related symptoms) from January 2000 to December 2000 were retrospectively analyzed. The mean follow-up period was 3.5 months (range: 1-8 months). The fibroids ranged in size from 2.0 cm to 13.2 cm with a mean size of 5.8 cm. We performed embolization using polyvinyl alcohol particles (250-710microgram). The improvement of the clinical symptoms was analyzed. Reduction of the uterine and predominant fibroid volumes was assessed using MRI. RESULTS: Symptom improvement for the menorrhagia (87.5%), dysmenorrhea (83.3%) and the bulk-related symptoms (79.2%) was reported. Complications included ovarian failure in four patients (5.8% of the total patients, mean age: 43.3 yrs) and infection in three patients (4.3% of the total patients) who underwent conservative management with intravenous antibiotics and analgesics. The volume reduction rate of the uterus and the predominant fibroids after uterine artery embolization were 36.3% and 56.6%, respectively. CONCLUSION: UAE is a promising new treatment for symptomatic fibroids and may be a valuable alternative to hysterectomy.
Analgesics ; Anti-Bacterial Agents ; Dysmenorrhea ; Female ; Follow-Up Studies ; Humans ; Hysterectomy ; Leiomyoma* ; Magnetic Resonance Imaging ; Menorrhagia ; Polyvinyl Alcohol ; Retrospective Studies ; Uterine Artery Embolization* ; Uterine Artery* ; Uterus

PURPOSE: To evaluate the significance of arthrocentesis on transient synovitis of hip in children with a moderate degree of limping and large joint effusion. MATERIALS AND METHODS: 119 cases of transient synovitis of the hip were evaluated retrospectively between January 1999 and December 2003, with respect to age (mean age: 5.6 years), sex (M: F=89: 30), location (Right: Left=68: 51), chief complaints (limping: 72; hip pain: 39; knee pain: 37), physical examination (limited internal rotation: 98), sonography of capsule neck distance (mean 5.9 mm), duration of hospitalization and morbidity. Among them, 47 cases with greater than 8 mm in capsule neck distance on sonography were compared, based on the duration of hospitalization, morbidity, and the change of capsule bulging between 18 cases in the arthrocentesis group and 29 cases in the non-arthrocentesis group. RESULTS: The mean duration of hospitalization and morbidity were 1.7 days, 4.7 days in the arthrocentesis group and 2.1 days, 6.6 days in the non-arthrocentesis group, the distance of the capsule bulging decreased from 9.2 mm to 4.5 mm in the arthrocentesis group and from 9.0 mm to 7.6 mm in the non-arthrocentesis group. The duration of hospitalization, morbidity, clinical course and decrease in capsule bulging were more effective, statistically in the arthrocentesis group than in the non-arthrocentesis group. CONCLUSION: Arthrocentesis for transient synovitis can be effective method of treatment in cases of moderate degrees of limping and large joint effusions, in spite of the disadvantage of the invasiveness of the procedure.
Child* ; Hip* ; Hospitalization ; Humans ; Joints ; Knee ; Neck ; Physical Examination ; Retrospective Studies ; Synovitis*

PURPOSE: Initial drug resistance of children with tuberculosis is a useful performance indicator of tuberculosis control programme in the concerned country because it represented infection transmitted from adult patients with either primary or acquired drug resistance. But there have been a few studies available. The present study was made to know the incidence of drug-resistant tuberculosis in hopes of selecting effective treatment regimens for the treatment of childhood tuberculosis. METHODS: Tuberculosis patients were selected from those whose cultures had been submitted to drug susceptibility testing in the Korean National Tuberculosis Association from Jan. 1987 to Jun. 1995. We have investigated the drug resistance and reviewed retrospectively the medical records of children. A total of 48 strains were tested for drug susceptibility. Of these cases medical records were available in one half and 22 patients did not have a history of previous antituberculosis therapy. RESULTS: 1) Of the 48 strains, 18 (37.5%) were resistant to 1 or more antituberculosis drugs. The 14 (29.2%) and 8 (16.7%) strains were resistant to isoniazid and rifampin, respectively. The 7 (14.6%) strains were resistant to both isoniazid and rifampin. 2) Of the 22 strains isolated from patients without previous therapy, 4 (18.2%) were resistant to 1 or more antituberculosis drugs. The 2 (9.1%) strains were resistant to isoniazid. There were no resistant strains to rifampin and to both isoniazid and rifampin. CONCLUSIONS: Primary resistance rate for isoniazid was 9.1%. These limited data suggested that four-drug regimens is indicated in childhood tuberculosis patients.
Adult ; Child* ; Drug Resistance ; Hope ; Humans ; Incidence ; Isoniazid ; Medical Records ; Retrospective Studies ; Rifampin ; Tuberculosis ; Tuberculosis, Multidrug-Resistant*

Dural arteriovenous malformations are rare, accounting for 10-15% of all intracranial arteriovenous malformation and are thought to be acquired lesion caused by dural sinus thrombosis. The authors report a rare case of extrasinusal dural arteriovenous fistula in the right middle fossa adjacent to the superior orbital fissure. During the preceding 17 years, this 31-year-old female had complained of proptosis and ocular pain in the right eye. Computed tomography and magnetic resonance imaging showed a retrobular intraconal mass in the right orbit; Angiography revealed an extrasinusal dural arteriovenous fistula in the right middle fossa adjacent to the superior orbital fissure, which drains into the superior ophthalmic and inferior temporal vein. After embolization of the fistula by injection of N-butylcyanoacrylate(NBCA), we intended to remove the retrobulbar intraconal mass seen on CT and MRI by means of a unilateral partial orbitotomy on the supraorbital rim and orbital roof, and to this end, explored the right orbit. We were unable, however, to locate any mass lesion in the orbital cavity, and postoperative CT and MRI showed no such lesion. We were of the opinion that the mass shown on preoperative CT and MRI was a dilated intraorbital vein structure which disappeared after embolization, operative ligation and cutting of the dural arterivenous fistula. Finally, proptosis and ocular pain disappeared, and the patient recovered with any no neurologic deficit.
Adult ; Angiography ; Arteriovenous Malformations* ; Central Nervous System Vascular Malformations ; Exophthalmos ; Female ; Fistula ; Humans ; Intracranial Arteriovenous Malformations ; Ligation ; Magnetic Resonance Imaging ; Neurologic Manifestations ; Orbit ; Sinus Thrombosis, Intracranial ; Veins

Rotor syndrome is a rare benign familial disorder characterized by chronic, fluctuating, nonhemolytic and predominantly direct bilirubinemia with normal liver tissue. We have recently experienced two cases of Rotor syndrome in a brother and sister. They revealed icteric sclerae with mild hepatomegaly in physical examination. Laboratory findings showed increased serum bilirubin with direct bilirubin predominance. The urinary excretion of total coproporphyrin was slightly elevated. The 99mTc-DISIDA scan showed a markedly decreased hepatic uptake and poor visualization of gallbladder and biliary tree which could be compatible to the Rotor syndrome. We report two cases with a review of the literature.
Biliary Tract ; Bilirubin ; Gallbladder ; Hepatomegaly ; Humans ; Hyperbilirubinemia ; Hyperbilirubinemia, Hereditary* ; Liver ; Physical Examination ; Sclera ; Siblings* ; Technetium Tc 99m Disofenin