Febrile ulceronecrotic PLEVA is an unusually severe from of PLEVA, characterized by the sudden onset of diffuse ulceronecrotic eruption aasociated with high fever. A mild eruption precede the acute fulminating course, We observed a 13-year-old boy presenting the form of febrile ulceronecrotic PLEVA. The histologic features were those of PLEVA. He received the systemic corticosteroids with aupportive care. To recognize this disorder is important because of a possibly fatal outcome, So close attention and vigorous therapy are necessary.
Adolescent ; Adrenal Cortex Hormones ; Fatal Outcome ; Fever ; Humans ; Male ; Pityriasis Lichenoides* ; Pityriasis*

We performed analysis of 79 childhood patients diagnosed as vitiligo and nevus depigmentosus in Tediatric Dermatologie Department of Seoul National University Hospital from March 1987 to September 1987. The results were as follows : 1. Of the 79 patients, 31 patients(39.2%) were vitiligo 21 patients(26.6% )were nevus depigmentosus and 27 patients(34.2%)were uncertain by cliniral diagnostic ctriteri.. 2. Viiligo began rarely in infancy(6.4% )and most appeared evenly in all childhood over 1 year old. Nevus clepigmentosus was present at birth in 57.1% of patients and was discovered within the first 6 months after birth in 42.9% of patients. 3. In vitiligo, most lesions were multiple. The most common site of involvement was the face, and there was this tendency of periorficial predisposition. In nevus ciepigmentosus, most lesions were solitary. The most, common aites of involvement were the lowed limbs and trunk, but there was no tendency of periorfcial presposition. 4. We found focal type of vitiligo in,51,6% of patients, vulgaris type in 32. 3 % acrofacial type in 9.6% and segmerital type in 6.5%. We also found isolated pattern of nevus clepigrnento us in 76.2% of patients, ciermatomal p<:ttern in 19.0ki and whorlec1 pattern in 4.8% The contrast enhiancement between the hypopigmented skin and nomal surrounding skin by Wood's light, examinatior reflected the epiclermal melanin pig merit, i. e., the more marked loss of pigmentation, the more accentuatian of the contrast The before, Wood's light examination without I:3OPA staining could help to ciiagnose the atisculte type of vitiligo showing eidermal melanocytes and nevu c.Iepigmeritosus.
Child* ; Dihydroxyphenylalanine* ; Extremities ; Humans ; Melanins ; Melanocytes ; Nevus* ; Parturition ; Pigmentation ; Seoul ; Skin ; Vitiligo*

Cutaneous plasmacytosis is a rare disorder characterized by multiple red-brown plaques, mainly on the trunk, which histologically show a marked hyperplasia of mature plasma cells with no mitotic figures or cellular atypia. It is sometimes accompanied by extracutaneous symptoms like lymphadenopathy or interstitial pneumonia, but without plasma cell infiltration. Although many treatments such as corticosteroid lesional injection, cyclophosphamide, melphalan, systemic PUVA, and local tacrolimus application have been introduced for cutaneous plasmacytosis, there is no reported post-treatment data on cutaneous plasmacytosis in Korea. We report a case of a Korean male patient who was diagnosed with cutaneous plasmacytosis which was improved by PUVA therapy.
Cyclophosphamide ; Humans ; Hyperplasia ; Korea ; Lung Diseases, Interstitial ; Lymphatic Diseases ; Male ; Melphalan ; Plasma Cells ; PUVA Therapy* ; Tacrolimus

We report a case of pulmonary edema developed in a 33-year-old female who underwent two-stage bilateral carotid body tumor excision. About 1 month ago, she had undergone a left carotid body tumor excision. After the operation, her tongue was deviated to left side. Bilateral hypoglossal nerve injury was suspected. These injuries should be carefully monitored in patients who will undergo a similar procedure on both sides because a bilateral deficit of the hypoglossal nerve is poorly tolerated, resulting potentially serious pulmonary edema. In recovery room, she became pale and SpO2 was fall down. We reintubated her immediately and the pulmonary edema was treated using a supportive management. She was discharged without any signs of dyspnea or airway obstruction, but hypoglossal nerve injury remained. We discuss the possible etiology of the upper airway obstruction after the neck surgery and review the literatures associated with the pulmonary edema following upper airway obstruction.
Adult ; Airway Obstruction ; Carotid Body Tumor* ; Carotid Body* ; Dyspnea ; Female ; Humans ; Hypoglossal Nerve ; Hypoglossal Nerve Diseases ; Hypoglossal Nerve Injuries ; Neck ; Pulmonary Edema* ; Recovery Room ; Tongue

Down syndrome is the most common autosomal syndrome in man. The incidence of trisomy 21 due to translocation is about 3.5-5%. Translocations are usually centric fusions between a 21 and a D group (54.2%) or a G group (40.9%) chromosome. Since the short arm of 21 carries no phenotypically active genes, even if the short arm of 21 is lost, usually there is no imbalance with clinical significance. Rare translocations in Down syndrome may also occur between a 21 and other chromosomes (4.9%). We experienced a case of unusual (1q;21q) translocation Down syndrome inherited from a t (1q;21q) balanced carrier mother. The segmental trisomy was confirmed by FISH analysis. This is the first case of a familial Down syndrome with t (1q;21q) in Korea.
Arm ; Down Syndrome* ; Humans ; Incidence ; Korea ; Mothers* ; Trisomy

PURPOSE: Survival outcome of locally advanced pancreatic cancer has been poor and little is known about prognostic factors of the disease, especially in locally advanced cases treated with concurrent chemoradiation. This study was to analyze overall survival and prognostic factors of patients treated with concurrent chemoradiotherapy (CCRT) in locally advanced pancreatic cancer. MATERIALS AND METHODS: Medical records of 34 patients diagnosed with unresectable pancreatic cancer and treated with definitive CCRT, from December 2003 to December 2012, were reviewed. Median prescribed radiation dose was 50.4 Gy (range, 41.4 to 55.8 Gy), once daily, five times per week, 1.8 to 3 Gy per fraction. RESULTS: With a mean follow-up of 10 months (range, 0 to 49 months), median overall survival was 9 months. The 1- and 2-year survival rates were 40% and 10%, respectively. Median and mean time to progression were 5 and 7 months, respectively. Prognostic parameters related to overall survival were post-CCRT CA19-9 (p = 0.02), the Eastern Cooperative Oncology Group (ECOG) status (p < 0.01), and radiation dose (p = 0.04) according to univariate analysis. In multivariate analysis, post-CCRT CA19-9 value below 180 U/mL and ECOG status 0 or 1 were statistically significant independent prognostic factors associated with improved overall survival (p < 0.01 and p = 0.02, respectively). CONCLUSION: Overall treatment results in locally advanced pancreatic cancer are relatively poor and few improvements have been accomplished in the past decades. Post-treatment CA19-9 below 180 U/mL and ECOG performance status 0 and 1 were significantly associated with an improved overall survival.
Chemoradiotherapy* ; Follow-Up Studies ; Humans ; Medical Records ; Multivariate Analysis ; Pancreatic Neoplasms* ; Survival Rate

Recently, Harms and Melcher modified Goel's approach, the C1 lateral mass and C2 pedicle screw fixation, and the new technique is currently in favor among neurosurgeons. Comparing to the advantages of Harms construct, the disadvantages were not extensively investigated. We experienced a patient with severe occipital pain developed after the C1 lateral mass screw placement for the traumatic atlantoaxial instability. We reviewed literatures about Harms construct with focus on the occipital neuralgia as a postoperative complication and suggest here technical tips to avoid the troublesome pain.
Humans ; Neuralgia ; Postoperative Complications

Bronchiolitis obliterans organizing pneumonia (BOOP) is a pulmonary disorder that exhibits various radiographic findings. It often shows bilateral, patched, or ground glass interstitial infiltrate, but a solitary nodular pattern rarely appears. We report a case of an 8-year-old boy suffering from BOOP that showed a single nodular pattern of the lung in the chest radiography. We conclude that when there is a solitary nodule discovered in the lungs of children or adolescence, the differential diagnosis must include BOOP.
Adolescent ; Bronchiolitis ; Bronchiolitis Obliterans ; Child ; Cryptogenic Organizing Pneumonia ; Diagnosis, Differential ; Glass ; Humans ; Lung ; Solitary Pulmonary Nodule ; Stress, Psychological ; Thorax

Blood Glucose ; Diabetes, Gestational ; Diagnosis ; Female ; Fetal Development ; Gestational Age ; Glucose ; Glucose Tolerance Test ; Humans ; Information Services ; Odds Ratio ; Pregnancy ; Pregnant Women

BACKGROUND AND OBJECTIVES: Congenital long QT syndrome (LQTS) is characterized by the prolongation of the QT interval, frequent episodes of syncope and Torsades de Pointes (TdP). The clinical features and electrocardiographic findings in Korean patients with LQTS have not been reported. SUBJECTS AND METHODS: We retrospectively analyzed the clinical characteristics, ECG features and response to treatments in 11 patients (6 men, 5 women) with congenital LQTS. RESULTS: The mean age at the time of the first episode was 19.4+/-22.6 years old (range: 170 years). Clinical presentations were syncope, seizure or sudden cardiac death (SCD). Predisposing factors included exercise, sudden startle or sleep. Only three patients showed familial histories of syncope or SCD. The average QTc interval was 0.58+/-0.05 second (range: 0.47-0.61 seconds). T wave morphologies were classified as normal-appearing, broad-based, low amplitude/bifid or late onset. For its management, bblockers were used in 7 patients. In 2 patients, whose clinical events were related with to an increased vagal tone or were aggravated by bblocker therapy, mexiletine was prescribed. When bradycardia or AV block was documented, pacemakers were implanted. For 2 patients at high risk of sudden cardiac death, cardioverter-defibrillators were implanted. During a mean follow up period of 23.5+/-20.2 months (range: 364 months), symptoms (cardiac arrest) recurred in 1 patient. CONCLUSION: Congenital LQTS is a heterogeneous disease, showing diverse clinical manifestations, ECG features, and response to pharmacological management. Further research on the genotype-phenotype relationship will refine the management, enabling gene-specific treatment of this life-threatening disease.
Arrhythmias, Cardiac ; Atrioventricular Block ; Bradycardia ; Causality ; Death, Sudden ; Death, Sudden, Cardiac ; Electrocardiography* ; Follow-Up Studies ; Humans ; Long QT Syndrome* ; Male ; Mexiletine ; Retrospective Studies ; Seizures ; Syncope ; Torsades de Pointes