No abstract available.
Appendicitis* ; Child* ; Humans

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

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.

The word "Delayed" in the legend of Figure 2 should have been written as "Controls."

Letterer-Siwe disease occurred in a 3 year 7 month old male child showing severe emaciation, hepatosplenomegaly, otitis media, hemorrhagic and impetiginous crusted patches on the body, and seborrheic dermatitis like skin rashes in the scalp. Also showed are severe anemia, thrombocytopenia, bronchopneumonia and multiple bony defects in skull. The child expired in a month after admission.
Anemia ; Bronchopneumonia ; Child ; Dermatitis, Seborrheic ; Emaciation ; Exanthema ; Histiocytosis, Langerhans-Cell* ; Humans ; Infant ; Male ; Otitis Media ; Scalp ; Skull ; Thrombocytopenia

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*