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

In recent decades there has been marked progress in the imaging and laboratory evaluation of dizzy patients. However, detailed history taking and comprehensive bedside neurotological evaluation remain crucial for a diagnosis of dizziness. Bedside neurotological evaluation should include examinations for ocular alignment, spontaneous and gaze-evoked nystagmus, the vestibulo-ocular reflex, saccades, smooth pursuit, and balance. In patients with acute spontaneous vertigo, negative head impulse test, direction-changing nystagmus, and skew deviation mostly indicate central vestibular disorders. In contrast, patients with unilateral peripheral deafferentation invariably have a positive head impulse test and mixed horizontal-torsional nystagmus beating away from the lesion side. Since suppression by visual fixation is the rule in peripheral nystagmus and is frequent even in central nystagmus, removal of visual fixation using Frenzel glasses is required for the proper evaluation of central as well as peripheral nystagmus. Head-shaking, cranial vibration, hyperventilation, pressure to the external auditory canal, and loud sounds may disclose underlying vestibular dysfunction by inducing nystagmus or modulating the spontaneous nystagmus. In patients with positional vertigo, the diagnosis can be made by determining patterns of the nystagmus induced during various positional maneuvers that include straight head hanging, the Dix-Hallpike maneuver, supine head roll, and head turning and bending while sitting. Abnormal smooth pursuit and saccades, and severe imbalance also indicate central pathologies. Physicians should be familiar with bedside neurotological examinations and be aware of the clinical implications of the findings when evaluating dizzy patients.
Dizziness ; Ear Canal ; Eyeglasses ; Glass ; Head ; Humans ; Hyperventilation ; Ocular Motility Disorders ; Pursuit, Smooth ; Reflex, Vestibulo-Ocular ; Saccades ; Vertigo ; Vibration

PURPOSE: The study was performed to identify the reality of urinary incontinence (UI) and to evaluate the risk factors developing UI for the patients with Parkinson's disease. MATERIALS AND METHODS: This was the cross-sectional study with interviews using structured questionnaires. The subjects who were 72 patients(male 20, female 52) and their mean age is 70.07+/-20.06(range 54~86) with Parkinson's disease visited the Neurology clinic, from September to November 2005, at one university hospital located in Jeju island. RESULTS: Subjects with restricted mobility were 45, and 13 were in first stage of Hoehn & Yahr stage, 31 were in second stage, 28 were in third stage. Fifty three(73.6%) subjects had experienced UI. Mixed UI was in 32 (44.4%), stress UI 12(16.7%), and urge UI 9(12.5%), respectively. As for risk factors developing UI, female had 1.62 times(OR=1.62, 95% CI=0.47~5.66) more than male, age of 80~86 had 3.20 times(OR=3.20, 95% CI=0.65~15.69) more than age of 54~69, subjects with restricted mobility had 2.75 times(OR=2.75, 95% CI=0.80~9.43) more than subjects without restricted mobility. Group without regular exercise had 2.9 times more than group with regular exercise(OR=2.90, 95% CI=0.92~9.22). Cognitive impairment group had 1.98 times more than normal mental status group(OR=1.98, 95% CI=0.39~9.97). Second stage had 4.91 times(OR=4.91, 95% CI=0.55~43.53) and third stage had 5.68 times(OR=5.68, 95% CI=00.64~50.73) more than first stage in Hoehn and Yahr stage. CONCLUSION: There is a high prevalence(73.6%) of UI in patients with Parkinson's diseases. Mixed UI was the most common type of incontinence. Risk factors developing UI were higher in female, older group, restricted mobility group, group without regular exercise, cognitive impairment group andhigher Hoehn and Yahr stage.
Cross-Sectional Studies ; Female ; Humans ; Male ; Neurology ; Parkinson Disease* ; Surveys and Questionnaires ; Risk Factors ; Urinary Incontinence*

OBJECTIVES: Abdominal obesity increases mortality and morbidity from cardiovascular disease and there is a possibility that smoking effects obesity. However, previous studies concerning the effects of smoking on obesity are inconsistent. The objective of this study was to examine whether smoking is positively related to abdominal obesity in men with type 2 diabetes. METHODS: Subjects consisted of 2197 type 2 diabetic patients who visited Huh's Diabetes Center from 2003 to 2009. Indices of abdominal obesity were defined as visceral fat thickness (VFT) measured by ultrasonography and waist circumference (WC). Overall obesity was defined as body mass index (BMI). RESULTS: Statistically significant differences in WC and VFT by smoking status were identified. However, there was no statistical difference in BMI according to smoking status. Means of WC and VFT were not significantly higher in heavy smokers and lower in mild smokers. Compared to nonsmokers, the BMI confounder adjusted odds ratio and 95% confidence interval for VFT in ex-smokers and current-smokers were 1.70 (1.21 to 2.39) and 1.86 (1.27 to 2.73), respectively. CONCLUSIONS: Smoking status was positively associated with abdominal obesity in type 2 diabetic patients.
Abdominal Fat/*metabolism ; Adult ; Aged ; Aged, 80 and over ; Body Mass Index ; Diabetes Mellitus, Type 2/*complications ; Humans ; Male ; Middle Aged ; Obesity/*etiology ; Smoking/*adverse effects ; Waist Circumference

Most thymomas are found in the superior and anterior mediastinum and malignant thymomas are exceedingly rare in the first 20 years of life. A child of malignant thymoma with invasion into the thoracic vertebra and spinal canal is reported. The tumor was originated from posterior mediastinum and involved posteriorly the D8, D9 vertebra resulting in paraparesis was improved. Computed tomography is mandatory in detecting the metastasis and evaluation of treatment.
Child ; Humans ; Mediastinum ; Neoplasm Metastasis ; Paraparesis ; Spinal Canal* ; Spine ; Thymoma*

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

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