Essential thrombocythemia in childhood is a rare clonal myeloproliferative disorder in the multipotent stem cell origin and is associated with an increased risk of thrombohemorrhagic complications. The one of diagnostic criteria is a platelet count of more than 600,000/mm3. We diagnosed this disease in 8 year old boy incidentally and treated with hydroxyurea. We report a case of essential thrombocythemia to summarize the current trends in the diagnosis and management with a brief review of related literatures.
Child ; Diagnosis ; Humans ; Hydroxyurea ; Male ; Multipotent Stem Cells ; Myeloproliferative Disorders ; Platelet Count ; Thrombocythemia, Essential*

Acrodermatitis enteropathica, an autosomal recessive disease, usually presents with severe acral and circumorificial dermatitis, diarrhea, alopecia, intercurrent bacterial infection during early infancy, and is eventually fatal if left untreated. We report a case of acrodermatitis enteropathica in a 2-month-old male infant who presented with chronic diarrhea not responsive to conventional therapy and developed disseminated intravascular coagulation (DIC). He showed the characteristic eczematoid skin lesions, chronic diarrhea, failure to thrive, and low serum zinc concenturation. Zn2+ was administered with dramatic improvement of skin lesions, DIC and diarrhea. He rapidly catched up normal growth and development on continuing zinc supplementation.
Acrodermatitis* ; Alopecia ; Bacterial Infections ; Dacarbazine ; Dermatitis ; Diarrhea* ; Disseminated Intravascular Coagulation* ; Failure to Thrive ; Growth and Development ; Humans ; Infant ; Male ; Skin ; Zinc

This study aimed to analyze changing trends in child injury deaths from 2006 to 2016 and to provide basic data for initiatives to help prevent child injury deaths through improvements in social systems and education. Specific causes of death were analyzed using micro-data of the death statistics of Korea from 2006 to 2016, which were made available by Statistics Korea. Types and place of death were classified according to the KCD-7 (Korean Standard Classification of Diseases and Causes of Death). The data were compared to those of other Organization for Economic Cooperation and Development countries. Changing trends were presented. The number of child deaths by injury was 270 in 2016. The death rate was 8.1 per 100,000 population in 2006, while it was 3.9 in 2016. The death rate of boys was 1.7 times greater than that of girls. Unintentional injury deaths comprised 72.6% of all child injury deaths in 2016, while intentional injury deaths comprised 27.4%. The first leading cause of unintentional injury deaths in infants (less than 1-year-old) was suffocation, while that of children aged 1 to 14 years was transport accidents. The second leading cause of death in infants was transport accidents, that of children aged 1 to 4 was falling, and that of children aged 5 to 14 was drowning. Pedestrian accidents comprised 43.7% of the transport accidents from 2014 to 2016. To prevent child injury deaths by both unintentional and intentional causes, nation-wide policy measures and more specific interventions according to cause are required.
Accidental Falls ; Asphyxia ; Cause of Death ; Child ; Classification ; Drowning ; Education ; Female ; Humans ; Infant ; Korea ; Mortality ; Organisation for Economic Co-Operation and Development ; Republic of Korea ; Self-Injurious Behavior

Diencephalic syndrome is a rare cause of failure to thrive in infancy and early childhood. The syndrome is characterized by profound emaciation with normal appetite, loss of cutaneous adipose tissue, hyperactivity, euphoria, and nystagmus. It commonly occurs in association with chiasmatic and hypothalamic gliomas. It has also been described in association with other histologic types. There is the marked increase of serum growth hormone, which may exhibit an inappropriate, even paradoxical response in stimulation test. A male infant of 12 months of age, showed markedly elevated growth hormone but he had failure to thrive findings. Evenly enhanced round mass was seen at suprasella area in brain CT. Its histological findings was "Desmoplastic infantile ganglioglioma", very rare histologic type. Here we report a case of diencephalic syndrome presented by failure to thrive in association with hypothalamic tumors.
Adipose Tissue ; Appetite ; Brain ; Emaciation ; Euphoria ; Failure to Thrive ; Glioma ; Growth Hormone ; Humans ; Hypothalamic Neoplasms ; Infant ; Male

Clear cell carcinoma of the ovary is a clinicopathologic entity with distinct morphologic characteristics and known to be associated with poor prognosis irrespective of stage and prognostic benefits of chemotherapy have not been evidently demonstrated. This represents 2-3.6% of all ovarian malignancies. From October, 1991 to January, 1998, nine patients with primary clear cell carcinoma of the ovary treated in the deptartment of Gynecology and Obstetrics, University of Ulsan, Asan Medical Center were identified, and clinical and histologic review(including architectural pattern, mitotic activity, nuclear grading) was performed retrospectively. The age of the patients ranged from 30 to 67 year(mean+S.D.; 46.0+11.8) and the mean parity was 1.2 (+1.3). Five(55.6%) of nine patients had elevated level of CA 125. The size of the tumors varied from 3 cm to 20 cm with the mean maximal diameter of 12.1 (+6.3) cm. The FIGO stages of the patients were IC(6 cases, 66.7%), IIC (2 cases, 22.2%) and IV(1 case, 11.1%). All patients except one were treated by total abdominal hysterectomy and bilateral salpingoophorectomy with or without omentectomy and pelvic lymph node dissection. All except two were received postoperative chemotherapy with regimens such as CEC (cyclophosphamide + epirubicin + carboplatin), TC(taxol + carboplatin), CC(cyclophosphamide + carboplatin) or CAP (cyclophosphamide + adriamycin + cisplatin). The mean follow-up duration was 14.6 (+ 8.6) months and 8 patients were free of disease and one patient with stage IV disease died of disease during postoperative chemotherapy(4 months after surgery).
Chungcheongnam-do ; Doxorubicin ; Drug Therapy ; Epirubicin ; Female ; Follow-Up Studies ; Gynecology ; Humans ; Hysterectomy ; Lymph Node Excision ; Obstetrics ; Ovary* ; Parity ; Prognosis ; Retrospective Studies ; Ulsan

Alloimmune neonatal neutropenia (ANN) is due to maternal IgG neutrophil-specific antibodies that cross the placenta to sensitize fetal neutrophils and cause neutropenia in the neonate. Infection appears within a few days of life. Neutropenia worsens during the very first days of life and persists until 20-50 days in spite of the disappearance of detectable antibody in the infant's serum. We report a case of ANN in female neonate who was admitted on the 5th day of life due to poor oral intake. The diagnosis was made by indirect immunofluorescence with fluorescein-tagged antihuman globulin reagent. The patient was treated successfully with systematic intravenous antibiotics and highdose intravenous immunoglobulin (IVIG).
Anti-Bacterial Agents ; Antibodies ; Diagnosis ; Female ; Fluorescent Antibody Technique, Indirect ; Humans ; Immunoglobulin G ; Immunoglobulins ; Immunoglobulins, Intravenous ; Infant, Newborn ; Neutropenia* ; Neutrophils ; Placenta

OBJECTIVES : The aim of this study was to identify the characteristic symptoms for diagnosis of Hwabyung (HB), a culture-related anger syndrome in Korea; to construct a rating scale for HB and test its validity and reliability; and propose diagnostic criteria for HB. METHODS : Subjects were male and female Korean patients, who were diagnosed following Structured Clinical Interview (SCID) as having depressive disorders, anxiety disorders and somatoform disorders and who reported as having self-labeled HB. A HB Scale was constructed with 22 of the most common symptoms of HB as identified by previous studies. The HB scale's inter-rated reliability was tested with 60 subjects. Its validity was tested by comparison between a HB only group(n=47) and depressive disorder only group (n=44). Logistic regression analysis was conducted to make a predictive model of HB. Based on these results diagnostic criteria for HB was proposed. RESULTS : Inter-rated reliability in each of all the items and the total score of the HB Scale were statistically significant. The HB scale differentiated HB from depressive disorder with statistical significance. In logistic regression analysis, the ability of the HB model to predict symptoms of heat sensation, ukwool/boon (feeling of unfairness), and subjective anger was high with sensitivity of 80.0%, specificity of 88.4%, accuracy of 84.1% and area under ROC of 0.92. Based on these results and information from previous research, diagnostic criteria of HB were formulated. CONCLUSION : The HB Scale was found to be reliable and valid. Consequently, diagnostic criteria of HB were proposed, to include subject anger, "kwool/boon" (Feeling of unfairness), expressed anger, heat sensation, hostility, "haan", pushing-up in the chest, epigastric mass, respiratory stuffiness, palpitation, dry mouth, sighing, racing thoughts, and lamentation.
Anger ; Anxiety Disorders ; Continental Population Groups ; Depressive Disorder ; Female ; Hemoglobin, Sickle ; Hostility ; Hot Temperature ; Humans ; Logistic Models ; Male ; Mouth ; Sensation ; Sensitivity and Specificity ; Somatoform Disorders ; Thorax

BACKGROUND: Tourniquets are used to provide a bloodless surgical field for extremities. Hypotension due to vasodilation and bleeding after tourniquet deflation is a common event. Hemodynamic stability is modulated by the autonomic nervous system (ANS). Heart rate variability (HRV) is a sensitive method for detecting individuals who may be at risk of hemodynamic instability during general anesthesia. The purpose of this study was to investigate ANS function to predict hypotension after tourniquet deflation. METHODS: Eighty-six patients who underwent total knee replacement arthroplasty (TKRA) were studied. HRV, systolic blood pressure variability (SBPV) and baroreflex sensitivity (BRS) were analyzed. We assigned two groups depending on the lowest systolic blood pressure (SBP) or mean BP (MBP) after tourniquet release (Group H; SBP < 80 mmHg or MBP < 60 mmHg, Group S; SBP > 80 mmHg and MBP > 60 mmHg). RESULTS: Fifteen patients developed severe hypotension and ten patients were treated with ephedrine. Of the parameters of HRV, SBPV, and BRS, only BRSSEQ was significant being low in Group H. BRS and high-frequency SBPV were correlated with the degree of MBP change after tourniquet deflation. CONCLUSIONS: Preoperative low BRS is associated with hypotension after tourniquet deflation, suggesting the importance of baroreflex regulation for intraoperative hemodynamic stability.
Anesthesia, General ; Arthroplasty ; Arthroplasty, Replacement, Knee ; Autonomic Nervous System ; Baroreflex ; Blood Pressure ; Ephedrine ; Extremities ; Heart Rate ; Hemodynamics ; Hemorrhage ; Humans ; Hypotension ; Tourniquets ; Vasodilation

PURPOSE: To evaluate the changes and remission of disease activity with changes in inflamed synivial volumes of rheumatoid joints after therapy. MATERIALS AND METHODS: Seven rheumatoid arthritis patients who had been treated with antiinflammatory drugs were followed up. Using NIH imaging and a segmentation technique, pre- and post-enhanced images were measured in subtracted images. Intra- and interobserver variation were evaluated by two radiologists(A and B), using two independent measurements. For comparison, the cases were assigned to one of two groups : remission and non-remission. Changes in ESR and total joint counts(TJC) after therapy were compared with inflamed synovial volumes. RESULTS: Intraobserver variations were 3.2% and 2.7% in A and B, respectively, interobserver variation between A and B was 7.1%. Changes in inflamed synovial volumes correlated well with those in ESR (r=0.88, p<0.009) and TJC (r=0.78, p<0.037) after therapy. Changes between the remission and non-remission group were insignificant, however. CONCLUSION: Changes in inflamed synovial volumes reflect those in the activity of rheumatoid arthritis between pre- and post- treatment. This technique may be used as a tool for predicting therapeutic response in rheumatoid arthritis cases. Changes in inflamed synovial volumes are of limited value, however, in predicting the remission of rheumatoid arthritis after therapy.
Arthritis, Rheumatoid ; Humans ; Joints ; Observer Variation ; Synovial Membrane* ; Synovitis* ; Wrist Joint* ; Wrist*

PURPOSE: To evaluate the changes and remission of disease activity with changes in inflamed synivial volumes of rheumatoid joints after therapy. MATERIALS AND METHODS: Seven rheumatoid arthritis patients who had been treated with antiinflammatory drugs were followed up. Using NIH imaging and a segmentation technique, pre- and post-enhanced images were measured in subtracted images. Intra- and interobserver variation were evaluated by two radiologists(A and B), using two independent measurements. For comparison, the cases were assigned to one of two groups : remission and non-remission. Changes in ESR and total joint counts(TJC) after therapy were compared with inflamed synovial volumes. RESULTS: Intraobserver variations were 3.2% and 2.7% in A and B, respectively, interobserver variation between A and B was 7.1%. Changes in inflamed synovial volumes correlated well with those in ESR (r=0.88, p<0.009) and TJC (r=0.78, p<0.037) after therapy. Changes between the remission and non-remission group were insignificant, however. CONCLUSION: Changes in inflamed synovial volumes reflect those in the activity of rheumatoid arthritis between pre- and post- treatment. This technique may be used as a tool for predicting therapeutic response in rheumatoid arthritis cases. Changes in inflamed synovial volumes are of limited value, however, in predicting the remission of rheumatoid arthritis after therapy.
Arthritis, Rheumatoid ; Humans ; Joints ; Observer Variation ; Synovial Membrane* ; Synovitis* ; Wrist Joint* ; Wrist*