We report a patient who had been initially diagnosed as a myelodysplastic syndrome in 1998 presenting purpuric patches on the left arm that started to develop about a year prior. The purpuric lesions were diagnosed as leukemia cutis by skin biopsy. Her subsequent bone marrow biopsy showed progression into an atypical chronic myeloid leukemia with increased numbers of leukocytes in the peripheral blood. Leukemia cutis typically is regarded as a sign of progression of disease or a manifestation of recurrent disease in treated patients with an established diagnosis of leukemia. We suggest that the skin lesion in this patient could have been a sign of con-version into atypical chronic myeloid leukemia.
Arm ; Biopsy ; Bone Marrow ; Diagnosis ; Humans ; Leukemia* ; Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative* ; Leukocytes ; Myelodysplastic Syndromes* ; Skin

No abstract available.
Appendicitis* ; Child* ; Humans

Persistent trigeminal artery is an embryonic remnant of the anastomotic channel linking the internal carotid artery and the basilar artery. Cases of vertebrobasilar insufficiency caused by the persistent trigeminal artery with internal carotid artery stenosis has been described previously, but vertebrobasilar insufficiency entirely due to in situ stenosis of the persistent trigeminal artery has not been reported. A 71-year-old man presented with frequent dizzy episodes. The brain MRI showed no parenchymal lesions. MR angiography showed poor visualization of vertebrobasilar system. He was diagnosed as having vertebrobasilar insufficiency. Cerebral angiography revealed that there was complete occlusion at the vertebrobasilar junction, and the basilar artery was supplied by the persistent trigeminal artery which had severe stenosis at its origin. There was no stenosis of the internal carotid artery of both sides. We believe that this is the first report of vertebrobasilar insufficiency due to stenosed persistent trigeminal artery, without internal carotid artery stenosis.

No abstract available.
Facial Paralysis* ; Humans ; Malocclusion*

No abstract available.
Chorion* ; Chorionic Villi Sampling* ; Chorionic Villi* ; Female ; Pregnancy

A 49-year-old woman with anemia who developed headache and seizure after blood transfusion was diagnosed with posterior reversible encephalopathy syndrome (PRES). Magnetic resonance imaging showed typical PRES findings including lesions in bilateral parieto-occipital subcortical white matter and overlying cortex. Only a few cases of PRES after transfusion have been reported and this case is unique in that there was a latent period between infusion and development of PRES. We postulate that rapid change of hemoglobin level may disrupt cerebral autoregulation and result in delayed PRES. We suggest that neurological symptoms after blood transfusion should be appropriately investigated.

The CYP11A1 gene encodes for the cholesterol side-chain cleavage enzyme (P450scc), which initiates steroid hormone biosynthesis. Defective P450scc activity results in severe glucocorticoid and mineralocorticoid deficiencies. We describe a case of P450scc deficiency due to a novel homozygous CYP11A1 variant inherited from the mother with a possibility of uniparental disomy (UPD). The patient was a female, had no family history of endocrine disease, and showed adrenal insufficiency at 13 days of age. Hormonal analysis with an adrenocorticotropic hormone stimulation test showed both glucocorticoid and mineralocorticoid deficiencies, presumed to be a defect of the early stage of steroidogenesis. Exome sequencing reported a novel homozygous frameshift variant of CYP11A1 (c.284_285del, p.Asn95Serfs*10), which was inherited from the mother.Additionally, homozygosity in 15q22.31q26.2, which included CYP11A1, was identified using a chromosomal microarray. It was suggested that the possibility of maternal UPD was involved as the cause of a P450scc deficiency by unmasking the maternally derived affected allele. To our understanding, P450scc deficiency associated with UPD encompassing CYP11A1 had not been reported in Korea before. Genetic analysis can help diagnose rare causes of primary adrenal insufficiency, including P450scc deficiency.

Disodium cromoglycate(DSCG) has been widely used in the treatment of bronchial asthma and has become the prophylactic drug of choice in patients with mild-to-moderate asthma, because it has anti-allergic, anti-inflammatory and mast cell stabilizing properties. DSCG has been considered one of the safest medications among drugs for asthma treatment. The most common side effects are irritation of throat and dry mouth. Bronchospasm and chest tightness have been reported, but they were usually experienced by patients using spinhaler powdered capsules rather than metered dose inhalers (MDI). In this report, we presented a case of recurrent DSCG-induced bronchoconstriction with brief review of the literature. He had had an aspirin-induced asthma and had been well controlled with steroid inhalers and DSCG-MDI. After aspirin-bronchopro vocation test, he complained of chest discomfort and tightness immediately after inhaling DSCG-MDI. On serial monitoring of peak expiratory flow rate (PEER), there was a significant decline of PEER after two puffs inhalation of DSCG-MDI. Thus we performed DSCG-inhalation broncho provocation test with the nebulizer solution, which was pure DSCG without any addition of ingradients or propellants. This produced an early asthmatic reaction with more than 40% decline of FEV,. He was diagnosed as DSCG-induced bronchoconstriction and his clinical conditions were improved after avoidance of DSCG-MDI.
Asthma* ; Asthma, Aspirin-Induced ; Bronchial Spasm ; Bronchoconstriction* ; Capsules ; Humans ; Inhalation ; Mast Cells ; Metered Dose Inhalers ; Mouth ; Nebulizers and Vaporizers ; Occupations ; Peak Expiratory Flow Rate ; Pharynx ; Thorax

BACKGROUND: Induction of anesthesia with propofol commonly associated with reduction in systemic arterial pressure, especially in elderly and high risk patients. This reduction is influenced by the dose and rate of propofol injection. The aim of this study was to examine the effect of different injection rate of propofol on vital signs, dose requirement and induction time during induction period. METHODS: Unpremedicated one hundred and twenty ASA physical status I and II patients aged 20~60 years scheduled for elective surgery were randomly allocated into one of four (150, 300, 600, 1200 ml/hr) groups according to speed of injection of propofol during induction period. Loss of verbal contact was taken as the end-point of induction. Vital signs, SpO2, dose requirement of propofol and induction time were checked. RESULTS: As the injection rate of propofol became slower, there were significant reduction in induction dose and increase in induction time (p<0.05). For example, induction dose and time were 1.82 mg/kg, 223 +/- 58 sec in 150 ml/hr group and 3.14 mg/kg, 50 +/- 11 sec in 1200 ml/hr group, respectively. Also, decrease in systolic and diastolic pressure were less marked at lower injection rates. CONCLUSIONS: Slower injection of propofol produces less vital sign changes and dose requirement for the induction of anesthesia.
Aged ; Anesthesia* ; Arterial Pressure ; Blood Pressure ; Humans ; Propofol* ; Vital Signs*

PURPOSE: This study was to investigate the effects of problem solving group counseling on the index of obesity and health habits for obese children. METHOD: Forty seven obese children participated in the study(Exp.=22, Cont.=25). Children were recruited from the forth and fifth grade withhigher than 20% of the obesity degree. The problem solving counseling lasted for 10 weeks. In order to evaluate the effects of counseling, physical characteristics and health habits were measured three times; pretest, posttest, and at 10 weeks follow-up. The obtained data was analyzed by Chi-squared-test, t-test, and repeated measures ANOVA, using the SPSS WIN 10.0program. RESULT: Problem solving group counseling was effective on the physical characteristics(BMI, obesity degree, body fat ratio, waist measurement) and health habits over time. Children in the experimental group controlled their body weight better and reported lower scores in the index of obesity than children in the control group at 10 weeks follow-up. CONCLUSION: This counseling program helped obese children modify their health habits so that they could decrease their scores in the obesity index. It can be concluded that problem-solving counseling enhanced problem-solving abilities of obese children, which could help modify their ordinary health habits.
Child ; *Counseling ; Female ; *Health Behavior ; Humans ; Male ; Obesity/*nursing ; *Weight Loss