A Case of Laryngotracheal Reconstruction With Radial Forearm Free Flap in Patient With Thyroid Squamous Cell Carcinoma / 대한이비인후과학회지

Primary squamous cell carcinomas arising from the thyroid is an extremely rare malignancy, which clinically can invade the larynx and trachea. In such an event, thyroidectomy with en bloc resection and reconstruction is the treatment of choice. However, laryngotracheal reconstruction remains a challenge and no ideal reconstruction has yet been established. Herein, we report a case of a thyroid squamous cell carcinoma invading the laryngotrachea. The tumor was completely resected surgically, including the laryngotrachea wall, which was reconstructed with a radial forearm free flap. The patient was decannulated one year after surgery and no evidence of disease was detected two years after surgery.

A Case of IgG4-Related Disease With Sinonasal Involvement Presenting With Decreased Visual Acuity

IgG4-related disease (IgG4-RD) is a systemic inflammatory disease characterized by IgG4-positive plasma cell and T lymphocyte infiltration of multiple organs. It commonly involves the pancreas, lacrimal glands, and salivary glands, and it has been rarely reported in the sinonasal cavity. We herein report the case of a 47-year-old male patient whose chief complaint was decreased visual acuity. A tumefactive mass was found on imaging studies, originating from the sinonasal cavity and invading the orbit, kidney, and meninges. The mass was resected through endoscopic sinus surgery and was pathologically confirmed to be IgG4-RD. The patient was treated with steroid therapy and showed clinical improvement.

The Results of Nation-Wide Registry of Age-related Macular Degeneration in Korea

PURPOSE: To evaluate the incidence and clinical features of age-related macular degeneration (AMD) in Korea. METHODS: Web-based (www.armd-nova.or.kr) registration was conducted for AMD patients aged 50 or more who were newly diagnosed by retinal specialists in Korea from August 20, 2005 to August 20, 2006. Patient data including ophthalmologic examination, fundus photography, fluorescein angiogram and/or indocyanin green angiogram (ICG), past medical history, behavioral habit, combined systemic diseases were up-loaded. RESULTS: Among finally enrolled 1,141 newly diagnosed AMD patients, 690 patients (60.5%) were male and 451 patients (39.5%) were female. The average age of AMD patients was 69.7+/-8.0. Early AMD was observed in 190 patients and 951 patients had late AMD. Classic choroidal neovascular membrane (CNVM) was observed in 18.6% of exudative AMD patients and 63.4 % had occult CNVM. Subfoveal CNVM was observed in 80.4% of the patients with CNVM. Among the 580 exudative AMD eyes that performed indocyanin green angiography (ICG), 184 eyes (31.7%) had polypoidal choroidal vasculopathy (PCV) and 36 eyes (6.2%) showed retinal angiomatous proliferation (RAP). Age, male gender, smoking, diabetes and hypertension significantly increased the risk of the AMD among Koreans. CONCLUSIONS: Because of the low rate of participation by retinal specialists, definite incidence of AMD was not obtainable. However, the estimated 1-year AMD incidence in the Pusan area of Korea is at least 0.4%. In contrast to Western people, 31.7% of exudative AMD cases were revealed to be PCV and 6.2% were revealed to be RAP. This discrepancy between ethnic groups should be considered in the diagnosis and treatment modality selection of Korean AMD patients.

Postischemic Treatment with Aminoguanidine Inhibits Peroxynitrite Production in the Rat Hippocampus Following Transient Forebrain Ischemia

Transient forebrain ischemia results in the delayed neuronal death in the CA1 area of the hippocampus. The present study was performed to determine effects of aminoguanidine, a selective iNOS inhibitor, on the generation of peroxynitrite and delayed neuronal death occurring in the hippocampus following transient forebrain ischemia. Transient forebrain ischemia was produced in the conscious rats by four-vessel occlusion for 10 min. Treatment with aminoguanidine (100 mg/kg or 200 mg/kg, i.p.) or saline (0.4 ml/100 g, i.p.) was started 30 min following ischemia-reperfusion and the animals were then injected twice daily until 12 h before sacrifice. Immunohistochemical method was used to detect 3-nitrotyrosine, a marker of peroxynitrite production. Posttreatment of aminoguanidine (200 mg/kg) significantly attenuated the neuronal death in the hippocampal CA1 area 3 days, but not 7 days, after ischemia-reperfusion. 3-Nitrotyrosine immunoreactivity was enhanced in the hippocampal CA1 area 3 days after reperfusion, which was prevented by the treatment of aminoguanidine (100 mg/kg and 200 mg/kg). Our findings showed that (1) the generation of peroxynitrite in the hippocampal CA1 area 3 days after ischemia-reperfusion was dependent on the iNOS activity; (2) the postischemic delayed neuronal death was attenuated in the early phase through the prevention of peroxynitrite generation by an iNOS inhibitor.

Clinical Features of Hemolytic Uremic Syndrome in Children

PURPOSE: Hemolytic uremic syndrome(HUS) is the most common cause of renal failure in childhood in western countries, but it has been a relatively rare disease in Korea. To analyze the clinical and epidemiologic characteristics of HUS in Korea, we reporte our experience, METHODS: We reviewed retrospectively the medical records of 9 patients who were diagnosed the hemolytic uremic syndrorne at Pusan National University during the period from January 1984 to December 1998. RESULTS: The age at presentation ranged from 3 years to 12 years old(mean: 5.9 years). Six of the children were typical and three patients were atypical. Six cases had grossly bloody stools and seven had abdominal pain. Oliguria developed in four cases and gross hematuria in eight cases. One case had seizure and letargy. Verotoxin producing E. coli(VTEC) were found in the stool specimens of two pa- tients. One was the serotype 0157:H7 and the other was the serotype 026:H-, Olll:H21. Verotoxin 1 (VT1) genes were identified by PCR in both of the cases. All patients required conservative treatment. Two patients were treated with peritoneal dialysis, one patient with hemodialysis and one patient with plasmapheresis. Of 9 case, 6 recovered without residual sequelae, but two patients died, and one patient progressed to end stage renal failure. CONCLUSION: Two thirds of our cases were typical type of HUS, which is now the predominant type among Korean childhood HUS sufferers. From this study, we suggest that patients diagnosed with typical HUS should be studied for the verotoxin of E. coli isolated from stool.

Correlation between the Grade of PVL on Brain MRI and Denver Development Screening Test

PURPOSE: The periventricular leukomalacia(PVL) tends to increase the risk of developing motor neurologic sequelae, delayed cognitive development, visual impairment, and epilepsy. Although several developmental screening test methods are being used, one of the oldest and best known developmental screening test was restandardized and revised as Denver Development Screening Test II(Denver II). The objective of this study is to analyse the correlation between the degree of PVL on MRI and the results of Denver II. METHODS: Among the children brought into Pusan National University Hospital between January 1996 and August 1999 with developmental delay, all of the 36 children with PVL on MRI were selected for the study. Denver II was checked in all these patients for screening of developmental delay with the review of medical records. Depending on the grade of PVL, total sample was classified into three groups, and it was based on abnormally increased signal intensity in periventricualr white matter or a reduced amount of periventricular white matter or both, and compensatory focal ventricular enlargement. We analysed the relationship of the grade of PVL and the results of Denver II. RESULTS: The 36 total patients were composed of 22 boys and 14 girls, with the age distribution between 11 to 72 months and the mean of 34.4 months. Delayed occurrence of gross motor sector were 5 cases(44.4%) in group 1, 16 cases(80.0%) in group 2, 7 cases(100.0%) in group 3. Incidence of delay was significantly higher in the high grade PVL group. Delayed rate of other sectors(fine motor-adaptive, personal-social) were higher in the high grade PVL group. But it was not statistically significant. Delayed rate of language sector has no correlation with grade of PVL on MRI. CONCLUSION: Incidence of dealy of gross motor sector was significantly higher in the high grade PVL group. However, language sector has no correlation with grade of PVL on MRI.

TNFalpha Expression in the Paraffin-embedded Tissues of Leprosy / 대한피부과학회지

BACKGROUND: Tumor necrosis factor alpha (TNFalpha ) plays various roles in the pathogenesis of leprosy. Expression of TNFalpha according to the clinical type of leprosy has been studied in the patient serum, stimulated PBMC, and the skin lesions, but these results were controversial. Also, all studies of the skin lesions have been limited because of a small number of frozen tissues. OBJECTIVE: The purposes of this study were to estimate the availability of paraffin-embedded skin tissue of leprosy for the detection of TNFalpha mRNA and protein, to analysis the TNFalpha expression according to the clinical types of leprosy and lepra reaction, and to demonstrate various TNFalpha -positive cells in the skin lesions. METHODS:In the paraffin-embedded tissues of 17 new cases of leprosy, TNFalpha mRNA expression was detected by in situ hybridization with digoxigenin labelled oligonucleotide probe cocktail and TNFalpha protein expression by immunohistochemical stain. In addition, serum TNFalpha level was estimated by ELISA for the evaluation of the relationship between before and after lepra reaction. RESULTS: 1. The density of TNFalpha mRNA-positive cells ranged from 8 to 26 percent (mean 17 percent) and that of TNFalpha protein-positive cells ranged from 3 to 8 percent (mean 7 percent). They were the highest in borderline lepromatous leprosy lesions. The density of TNFalpha -positive cells was significantly higher by in situ hybridization than by immunohistochemical stain. 2. The TNFalpha -positive cells in paraffin-embedded skin lesion were CD8 positive lymphocytes, CD68 positive macrophages, endothelial cells, histiocytes, Schwann cells, and keratinocytes. The number of these cells before leprosy treatment was not significantly different from that after leprosy treatment. 3. The density of CD8-positive cells was significantly higher in lesions without lepra reaction than in lesions with lepra reaction, and that of CD68-positive cells was significantly higher in lepromatous leprosy lesions than in borderline lepromatous leprosy lesions. 4. Serum TNFalpha level was higher during lepra reaction than before lepra reaction and the high level continued even after resolution of lepra reaction. CONCLUSION: It suggested that the paraffin-embedded tissue in leprosy is an effective and available material for detecting expression of TNFalpha mRNA and protein. Also, in situ hybridization was more sensitive than immunohistochemical stain. The number of TNFalpha -positive cells was the highest in borderline lepromatous leprosy lesions. With further refinement, this may be an easier application of study for TNFalpha expression in leprosy.

Correlation between the Grade of Brain MRI and Clinical Features of Periventricular Leukomalacia

PURPOSE: Periventricular leukomalacia(PVL) is associated with various neurologic sequelae such as cerebral palsy and cortical blindness. The aim of this study was to analyse the correlation between the degree of PVL on MRI and clinical features or its severity. METHODS:Thirty-eight children with PVL on MRI among children brought to Pusan National University Hospital between January 1996 and August 1999 with development delay, cerebral palsy or epilepsy were included into the study group. We reviewed medical records of the patients including the gestational age, birth weight and neurologic sequelae. The grade for PVL was divided into 1 to 3, and it was based on abnormally increased signal intensity in periventricular white matter or a reduced amount of periventricular white matter or both, and compensatory focal ventricular enlargement. We analysed the relationship of the grade of PVL and various clinical findings. RESULTS: The age ranged from 11 months to 13 years old with a mean of 42 months. History of cesarean delivery and ventilator care were significantly frequent for the severe grade of PVL. Birth weight was significantly lower according to grade of PVL. Gestational age was lower according to the grade of PVL, but was not significant statistically. Incidence of cerebral palsy was significantly higher on the severe grade of PVL. Incidence of epilepsy and mental retardation were 34.2Yo and 23.9M, not associated with the grade of PVL. CONCLUSION: Incidence of cerebral palsy was associated with the severe grade of PVL, so we think MRI can be helpful in predicting neurodevelopmental outcome. (J Korean Pediatr Soc 2000;43 798-805)

A Case of Type II Ehlers-Danlos Syndrome

Ehlers-Danlos syndrome represents a group of inherited connective tissue disorders characterized by varying degrees of joint laxity, skin fragility and hyperextensibility, and a bleeding tendency. The essential defect is a quantitative deficiency of collagen. At present, 10 clinical forms have been recognized on the basis of extent and severity of the principal features, in combination with other abnormalities and the likely mode of inheritance. Type II Ehlers-Danlos syndrome is characterized by mild skin and joint manifestations, the latter limited to hands and feet, and inherited as an autosomal dominant pattern. We experienced a case of type II Ehlers-Danlos syndrome in 9-year-old girl with easy bruisability, skin hyperelasticity, thin, atrophic and a shiny scar, the so-called cigarette-paper scar on anterior side of lower extremities, and joint hypermobility limited to hand and feet. The diagnosis was made by characteristic clinical features and skin biopsy findings. A brief review of related literature was made.

A Case of Neonatal Myocardial Infarction with Structurally Normal Coronary Arteries

Myocardial infarction in the newborn is usually seen in association with congenital heart disease, for example anomalous origin of the left coronary artery, tricuspid atresia and left ventricular outflow obstruction. However, it is very rare in infants with structurally normal coronary artery. In infants with structurally normal hearts and coronary arteries, the most common causes of myocardial infarction are perinatal asphyxia and thromboembolic occlusion. We experienced a case of neonatal myocardial infarction with structurally normal coronary arteries complicated sepsis and hypoglycemia. The diagnosis of myocardial infarction was made by EKG and increase of plasma myocardiac enzyme. We report this case with related literature.

A Case of Gastric Submucosal Tumors in Children Diagnosed by Endoscopic Ultrasonography / 대한소화기내시경학회지

Benign neoplasms of the stomach are uncommon in children. They include adenomatous and hyperplastic polpys, leiomyomas, lipomas, and other lesions. Gastric leiomyoma in childhood represent only 1% of all gastrointestinal neoplasms reported, and slightly more than half of these are gastric. The tumor arises in the muscle layer of the stomach and is usually intragastric and submucosal. Barium contrast studies are useful in localizing leiomyoma with significant intramural or submucosal growth. An endoscopy can permit histologic confirmation if the tumor has eroded through the mucosa. Differentiation however, of malignant lesions from benign submucosal leiomyomas may be difficult. Using endoscopic ultrasonund imaging, highly accurate gastrointestinal tumor characterization, especially submucosal masses, and differentiation of malignant from benign lesion are now relatively possible without surgery. We experienced a case of a gastric submucosal tumor regarded as a leiomyoma, which was diagnosed using endoscopic ultrasonography, and report the findings with a review of related literatures.

A Case of Congenital Vitamin K Dependent Coaguation Factor Defeciency / 대한소아혈액종양학회지

Congenital combined deficiency of vitamin K dependent coagulation factors is a rare coagulation disorder. We experienced a 20-month old boy who was found to have a congenital vitamin K dependent coagulation factor defeciency. He presented with continuous bleeding on lacerated hard palate and had a history of numerous hemorrhagic episodes with multiple bruises after birth. Laboratory finding showed prolonged prothrombin time and partial thromboplastin time. Blood coagulation work-up showed marked decreased activities of the coagulation factors II, VII, IX, X and the natural anticoagulants proteins C and S. Assay of coagulation factors in the parents and sibling were with the normal range. There's no evidence of malabsorption, liver disease or ingestion of a coumarin compound. Response to intravenous administration of vitamin K1 was not significant but transfusion of fresh frozen plasma corrected prothrombin time and partial thromboplastin time. We reported a case of congenital combined deficiency of vitamin K dependent coagulation factors.

A case of nodular amyloidosis / 대한피부과학회지

Localized primary cuteneous nodular amyloidosis is the rarest form of the cutaneous amyloidosis, which appears as single or multiple nodules on the extremities, trunk, genitalia or face. A 44-Year-old woman had asymptomatic, translucent brown to pink papules and nodules on the toes for 5 years. Histopathologic exarnination showed deposition to pale, eosinophilic, amorphous material throughout the dermis, Under the electron microscope, the material consisted of straight, nonbranching, nonanastoimosing filaments. We thus made the diagnosis of nodular amyoidosis.

A case of multiple trichoepithelioma / 대한피부과학회지

Multiple trichoepithelioma is an uncommon skin disease with autosomal dominant transmission. A 21-year-old female ahd asymptomatic, flesh-colored, firm papules and nodules on the face, chest and back. Histopathologic examination showed multiple horn cysts and tumor islands composed of basaloid cells.

Generalized Pseudoxanthoma Elasticum

We report a patient with a cutis laxa-like, generalized PXE without systemic involvement. A 28-year-old woman had loose, pendulous skin of the neck, axillae, thighs, trunk and body folds which resulted in a prematurely aged appearance. She had no family history of related diseases. Histological examination showed considerable accumulations of swollen and irregularly clumped fibers in the middle and lower dermis and von Kossa s stain revealed calcium deposits along the altered elastic fibers.

Generalized Pseudoxanthoma Elasticum

We report a patient with a cutis laxa-like, generalized PXE without systemic involvement. A 28-year-old woman had loose, pendulous skin of the neck, axillae, thighs, trunk and body folds which resulted in a prematurely aged appearance. She had no family history of related diseases. Histological examination showed considerable accumulations of swollen and irregularly clumped fibers in the middle and lower dermis and von Kossa s stain revealed calcium deposits along the altered elastic fibers.

Allergic contact dermatitis to hydroquinone in bleaching cream / 대한피부과학회지

Bleaching creams containing hydroquinone are modestly effective in treating some cutane ous pigmentary disorders. Hydroquinone creams frequently cause mild irritant reactions and postinflammatory hyperpigmentation, but rarely allergic reactions. A case of allergic contact dermatitis due to hydroquinone in a 21-year-old woman was confirmed by the patch test.