No abstract available.
Humans ; Infant* ; Infant, Low Birth Weight* ; Infant, Newborn

Granulomatous mycosis fungoides is an extremely rare and unusual histologic variant of mycosis fungoides. This form is clinically characterized by spontaneous resolution of ulcerated nodular lesions into poikiloderma. Histologically, a strong granulomatous component can obscure the underlying cutaneous lymphoma, which is frequently mistaken for non-neoplastic dermatitides or cutaneous sarcoidosis. We report a case of granulomatous mycosis fungoides occurring on the left cheek of 34-year-old man confirmed histologically with an aid of immunohistochemistry and clinical course (immediate response to PROMACE-CYTOBAM chemotherapy), with emphasis on differential diagnosis, along with a review of literature. This is the first documented report in the Korean literature.
Male ; Humans ; Diagnosis, Differential

The deletion 9p syndrome is a well characterized syndrome with about one hundred cases having been reported. Most patients have dysmorphic facial features, cardiac anomalies, and mental retardation. We report on a female infant with micrognathia, corneal opacity, cleft palace, cardiac anomaly, left polycystic kidney, and deletion 9p. Chromosome analysis and fluorescence in situ hybridization (FISH) showed her to have a derived chromosome 9 inherited from a maternal t(9;16) (p22;p13.2) by adjacent I segregation There are few reports of this particular chromosome rearrangement. We review deletion Sp syndrome.
Chromosomes, Human, Pair 9 ; Corneal Opacity ; Female ; Fluorescence ; Humans ; In Situ Hybridization ; Infant ; Intellectual Disability ; Polycystic Kidney Diseases

PURPOSE: Tracheostomy is used to aid airway management in perdiatric respiratory care. This study was designed to review causes and outcomes of pediatric tracheostomy. METHODS: We performed a retrospective chart review of 153 patients, less than 18 years of age, who underwent tracheostomy between January 1995 and July 2010. Age at tracheostomy, indications, durations, complications and mortality were evaluated. RESULTS: Subglottic stenosis (19%) was the most common indication for tracheostomy. The median age at tracheostomy was 1.3 years (range, 22 days to 17.8 years). Seventy-three (47.7%) tracheostomies were performed in children under 1 year of age. Respiratory diseases were significantly more prevalent in patients under 1 year of age, while neuromuscular disease were more frequently found in patients older than 1 year (P=0.013). Stoma or tracheal granuloma formation (36.6%) was the most common complication of pediatric tracheostomy. Decannulation was accomplished in 61 (39.9%) patients with median cannulation time of 141 days (range, 1 to 2,529 days). Overall mortality rate was 10.5% (n=16), but only one patient (0.7%) died from tracheostomy-related complications. CONCLUSION: Respiratory diseases, such as subglottic stenosis and neuromuscular disease, are the main cause of pediatric tracheostomy. Although complications, like stoma or tracheal granuloma formation occur, tracheostomy in children is a safe way to aid airway management.
Airway Management ; Catheterization ; Child ; Constriction, Pathologic ; Granuloma ; Humans ; Neuromuscular Diseases ; Retrospective Studies ; Tracheostomy

Lethal catatonia is a rare and fatal clinical syndrome. It has diverse etiologies, both functional and organic. We experienced a man with left hemiplegia and striking catatonia. Brain imaging showed a large right hemispheric infarction and hypoperfusion. Symptoms and signs of catatonia were markedly improved after treatment of dopa-agonist and benzodiazepine. This case suggests that right hemispheric infarction could be a possible cause of lethal catatonia, and supports earlier clinical literature relating catatonia and the parietal lobe.
Benzodiazepines ; Catatonia* ; Hemiplegia ; Infarction* ; Middle Cerebral Artery* ; Neuroimaging ; Parietal Lobe ; Strikes, Employee

BACKGROUND: Rhinocerebral mucormycosis (RCM) is an uncommon and fatal clinical syndrome resulting from an opportunistic infection caused by a fungus of the order Mucorales in immunocompromized patients. The mortality and morbidity in the patients with intracranial involvement is invariably high, and it was reported that most survivors had early diagnosis and received aggressive treatment. Therefore, we retrospectively reviewed four patients of pathologically confirmed mucormycosis to find out the clues for early diagnosis of RCM and for prediction of fatal intracranial involvement. METHODS: The clinical, radiological features and histo-pathological involvement sites in the patients with intracranial involvement were compared to those without intracranial involvement. RESULTS: All the patients had uncontrolled diabetes and were admitted with orbital involvement. On the precise physical examination, mucosal involvement was found in all patients; three of them in only nasal mucosa and one in oral palatal mucosa. Despite the vigorous antifungal therapy, two patients had intracranial involvement and expired. MRI finding of extensive sphenoid sinusitis adjacent cavernous sinus preceded the intracranial involvement of RCM, which was not found in the patients without intracranial involvement. CONCLUSIONS: Our findings suggest that early observation of oral or nasal mucosal changes might be an important clinical clue for differentiation of RCM from other causes of rhino-oculo-cerebral symptoms in uncontrolled diabetic patients, and that extensive sphenoid sinusitis might be an important radiological feature for predicting the fatal intracranial involvement of RCM.
Cavernous Sinus ; Diabetes Mellitus ; Early Diagnosis* ; Fungi ; Humans ; Magnetic Resonance Imaging ; Mortality ; Mucorales ; Mucormycosis* ; Mucous Membrane ; Nasal Mucosa ; Opportunistic Infections ; Orbit ; Physical Examination ; Retrospective Studies ; Sphenoid Sinus ; Sphenoid Sinusitis ; Survivors

BACKGROUND: Studies using a transcranial Doppler (TCD) to establish cerebral vasoreactivity (CVR) have mostly focused on the anterior circulation. The purpose of this study is not only to evaluate the feasibility of the power motion mode Doppler (PMD) with a probes fixating device, but also to simultaneously measure the CVR between the middle cerebral artery (MCA) and the basilar artery (BA) during hypercapnea simulated by the rebreathing technique. METHODS: Twenty eight healthy volunteers were enrolled. Baseline hemodynamic values (heart rate, blood pressure) were measured while volunteers sat in a comfortable position for 5 minutes. The TCD was performed in two steps. First, velocities and spectra of the MCAs were simultaneously monitored. Then, the velocities and spectra of the MCA and BA were simultaneously monitored by a headset that included an anterior-posterior probes fixating device. The equation for CVR was ([maximum mean velocity baseline mean velocity] x 100/baseline mean velocity). RESULTS: Baseline mean velocities were revealed as follows: (64.0+/-13.7, 65.0+/-11.9 cm/s in right and left MCA; p>0.05; 67.3+/-12.2, -45.3+/-7.6 cm/s in dominant MCA and BA). CVR did not differ between the dominant MCA and the BA (46.1+/-12.1, 46.0+/-15.1%; p>0.05), nor between the right and left MCAs (46.9+/-15.2, 46.4+/-14.8%; p>0.05). There was a positive linear correlation between the CVR of the dominant MCA and that of the BA (r=0.856; p<0.001). CONCLUSIONS: PMD with a probes fixating device for accurate insonation is a useful tool for evaluating the relative CVR between the MCA and BA. Our study suggests that CVR values of the BA are similar to those of the MCA.
Basilar Artery ; Healthy Volunteers ; Hemodynamics ; Middle Cerebral Artery* ; Volunteers

An ectopic pregnancy in the abdominal organs is very rare. Primary intestinal pregnancy is considered the rarest form of extrauterine pregnancy, and only a few well-documented cases have been reported. Herein, a case of an abdominal pregnancy in a 25-year-old woman, with massive lower gastrointestinal bleeding, is reported. The source of bleeding could not be identified, despite gastroscopy, ultrasonography and angiography investigations. A diagnostic laparotomy disclosed an abdominal pregnancy, causing an erosion of the jejunal wall at the site of the pregnancy, with massive lower gastrointestinal bleeding. An abdominal pregnancy is seldom included in the differential diagnosis of lower gastrointestinal bleeding. The possibility of intestinal erosion in an abdominal pregnancy should be borne in mind in cases of lower gastrointestinal bleeding.
Adult ; Angiography ; Diagnosis, Differential ; Female ; Gastroscopy ; Hemorrhage* ; Humans ; Laparotomy ; Pregnancy ; Pregnancy, Abdominal* ; Pregnancy, Ectopic ; Ultrasonography

Polymyositis(PM) is one of idiopathic inflammatory myopathy, characterized by proximal muscle weakness, myalgia and muscle enzyme elevation. Currently the main pathogenesis is well documented, the cell-mediated immunity. We experienced a case of polymyositis associated with hepatitis, developed after hepatitis B virus(HBV) infection. This virus-induced autoimmunity seems to result from the cross-reactivity between muscle protein and B-viral antigen, so called antigenic mimicry. This relation of PM and HBV is more significant in Korea because of the epidemicity of HBV infection.
Autoimmunity ; Hepatitis B virus ; Hepatitis B* ; Hepatitis* ; Immunity, Cellular ; Korea ; Molecular Mimicry ; Muscle Proteins ; Muscle Weakness ; Myalgia ; Myositis ; Polymyositis*

Since the initial description of allergic fungal sinusitis by Millar et al. in 1981, reported cases have been increasing. However, one case of allergic fungal sinusitis has been reported in Korea. The diagnosis can be established by demonstrating type I hypersensitivity reaction, nasal polyposis, characteristic CT scan, eosinophilic mucus without evidence of fungal invasion into sinus tissue, and a positive fungal stain or culture of sinus contents. The authors present a case which met the diagnostic criteria of allergic fungal sinusitis. This patient had nasal polyp and showed elevated level of total IgE, peripheral eosinophilia, positive MAST and strongly positive immediate skin reactivity to fungal antigens. On histologic examination, typical allergic mucin containing sheets of eosinophils, Charcot-Leyden crystals was found. Fungal hyphae were also found in the mucin content.
Antigens, Fungal ; Diagnosis ; Eosinophilia ; Eosinophils ; Humans ; Hypersensitivity, Immediate ; Hyphae ; Immunoglobulin E ; Korea ; Mucins ; Mucus ; Nasal Polyps ; Sinusitis* ; Skin ; Tomography, X-Ray Computed