Reconstruction of an eye lid or even a part of it requires a minimum of three elements; an outer layer of skin; an inner layer of mucosa; and a semi-rigid skeleton interposed between them. The upper lid is the most difficult to reconstruct as it must be movable. This report contains of a congenital upper lid coloboma of which reconstructed by Abbe-type full thickness flap of the lower eye lid. He is a 15 years old boy who has congenital middle third defect of left upper eye lid. The defect was rectangular shape, the edge rolled, and without lashes and glandular structures, with the base located at the central portion of upper lid margin. The inner margin is about 3mm apart from superior lacrimal punctum. He has no other associated congenital anomalies. His visual acuity of left eye was light perception that resulted from chronic exposure keratopathy. The authors operated a case of congenital lid colob@ma and obtained good cosmetic result.
Adolescent ; Coloboma* ; Humans ; Male ; Mucous Membrane ; Skeleton ; Skin ; Visual Acuity

The authors performed midline operation upon esotropia with abducens nerve paralysis and diplopia for 6 months following head trauma. Eye ball was straight without diplopia, but there was no improvement of lateral gaze in the paralytic eye.
Abducens Nerve ; Craniocerebral Trauma ; Diplopia ; Esotropia ; Paralysis

The Di(a+b-) phenotype is extremely rare among Caucaclans and mostly confined to mongoloids. The incidences of Di antigen among Koreans were reported to be 6.1 to 14.5%, with an average of 9.6%; it can be calculated that the frequency of Di(a+b-) is 0.25% on the average. Authors report the first case of hemolytic disease of the newborn due to anti-Dib in Korea. The Diego phenotypes of the mother, baby and father were Di(a+b-), Di(a+b+) and Di (a-b+), respectively. The mother's serum and eluate from infant's erythrocytes contained anti-DP antibody active in the antiglobulin phase. After transfusions of Di-negative red cells from the mother and mother's relative, the patient was recovered from anemia and jaundice.
Anemia ; Erythrocytes ; Fathers ; Humans ; Incidence ; Infant, Newborn* ; Jaundice ; Korea ; Mothers ; Phenotype

BACKGROUND: Hypocalcemia is the most common side effect in large-volume leukapheresis (LVL) for collection of peripheral blood stem cells (PBSCs). We evaluated the effect of intravenous calcium infusion on the hypocalcemic symptoms during LVL. METHODS: Seventy-six LVLs with hypocalcemic symptoms were participated in this study. LVLs were performed using Gambro Spectra (blood rate, 80 mL/min; whole blood to ACD-A with heparin, 24:1) until 20 L of blood was processed. Blood flow rate was slowed to 60-70% of initial in 35 LVLs (group A) and 20 mL of 3% CaCl2 was infused intravenously in 41 LVLs (group B). Serum levels of ionized calcium, total magnesium, and electrolytes were determined before and after each LVL and analyzed with students?-test. RESULTS: The observed hypocalcemic symptoms were perioral paresthesias (71%), digital numbness (17%), chest tightness (4%), chills (4%), headache (3%), and vomiting (1%). Serum ionized calcium decreased by 15.9% in group A (P<0.001) and increased by 1.3% in group B. Total magnesium in group A (11.4%, P=0.002) and potassium in both groups (9.5%, P<0.001 and 8.6%, P=0.02, respectively) were significantly reduced. In group A, hypocalcemic symptoms were not completely relieved but weakened in 63%, and blood flow rate had to be kept below 55 mL/min in 37%. In group B, the symptoms were relieved by 20 mL of 3% CaCl2 in 27% and by 40 mL in 73%. CONCLUSION: Reduction of blood flow for relief of hypocalcemia symptoms was only helpful in lightening the symptoms. Intravenous infusion of calcium was more effective and convenient to alleviate hypocalcemic symptoms and did not affect LVL procedures nor serum levels of ionized calcium.
Calcium* ; Chills ; Citric Acid ; Electrolytes ; Headache ; Heparin ; Hypesthesia ; Hypocalcemia ; Infusions, Intravenous ; Leukapheresis* ; Magnesium ; Paresthesia ; Potassium ; Stem Cells ; Thorax ; Vomiting

BACKGROUND: Various olfactory tests have already been proposed in order to clinically assess the olfactory function, for example, UPSIT, T & T olfactometer, CCCRC test, GITU, IV olfaction test. At recent, electro-olfactogram(EOG), olfactorhinometry, olfactory evoked potential, contingent negative variation was tried as the objective olfactory test. OBJECTIVES: We use the functional imaging of MRI which affords the potential for exploring regional pathophysiologic change in living brain as an olfactory function test. MATERIALS AND METHODS: Functional MRI scans of the brain were performed on 5 healthy subjects and 3 patients with olfactory dysfunction. 2 of the patients were diagnosed Parkinson's disease and the other one had basal skull fracture. Then, all subjects were performed CCCRC test. RESULT: 6 of 8 subjects showed significant region of activation in olfactory bulb and tract. Additional region of activation were also observed in amygdala and parahippocampus. Average activation ratio was 3.42+/-2.37%. CONCLUSION: These studies indicate that functional MRI have many limitations but it may be used to evaluate olfactory dysfunction and predict prognosis.
Amygdala ; Brain ; Contingent Negative Variation ; Echo-Planar Imaging* ; Evoked Potentials ; Humans ; Magnetic Resonance Imaging* ; Olfactory Bulb ; Parkinson Disease ; Prognosis ; Skull Fractures ; Smell

BACKGROUND: The in vivo skin prick test (SPT) or in vitro detection of allergen specific IgE in serum is commonly used for the diagnosis of allergic disease. In this study, we evaluated the usefulness of a new multiple allergen simultaneous test (MAST) immunoblot assay, Polycheck Allergy (Biocheck GmbH, Germany). METHODS: A total of 100 patients with clinical findings of allergic diseases were tested by SPT and three different MAST assays: Polycheck Allergy (Biocheck GmbH, Germany), MAST CLA allergy system (Hitachi Chemical Diagnostics, USA) and Allergy Screen (R-biopharm, Germany). The results of MAST assays were compared with those of SPT. RESULTS: Concordance rates of MAST assays with SPT were 79-100% for Polycheck Allergy, 88.9-100% for MAST CLA and 72.7-98.3% for Allergy Screen. In ROC curve analysis, significant differences were observed in four of 25 allergens analysed: Alternaria, Birch, Hazelnut and D. farinae. For Alternaria and Birch, Polycheck Allergy (P<0.001) and Allergy Screen (P=0.0075) showed significantly larger AUC (area under the curve) than MAST CLA. For Hazelnut, Polycheck Allergy (P=0.0021), and for D. farinae, MAST CLA (P=0.015) showed significantly larger AUCs than the other two tests. The ROC analysis for overall 16 food allergens showed better results in Polycheck Allergy (P<0.001), and that for overall 21 inhalants did not show significant differences among three MAST assays (P>0.05). CONCLUSIONS: Since Polycheck Allergy showed similar or superior result to the others, it can be used for the detection of allergen specific IgE antibodies.
Adolescent ; Adult ; Allergens/*immunology ; Area Under Curve ; Child ; Child, Preschool ; Female ; Humans ; Hypersensitivity, Immediate/*diagnosis ; Immunoblotting/*methods ; Immunoglobulin E/*blood ; Male ; Middle Aged ; ROC Curve ; Reagent Kits, Diagnostic ; Sensitivity and Specificity ; Skin Tests/methods

We report a two-generation Korean family in which 2 siblings have congenital cataract and phenotype i. This report is a first case in Korean people and shows the evidence suggesting the linkage of Ii blood group with a recessive form of congenital cataracts.
Cataract* ; Humans ; Phenotype* ; Siblings*

Angioedema is a well-demarcated localized edema involving the deeper layers of the skin, including the subcutaneous tissue. Angioedema occurs with Cl esterase inhibitor (Cl INH) deficiency that may be inborn as an autosomal dominant characteristic or may be acquired. Acquired angioedema (AAE) is a rare disorder characterized by adult onset and lack of evidence of inheritance of the disease. Two types of AAE are known today: type I in which there are lowering of functional Cl INH, an underlying disease such as a B-cell disease, and no detectable autoantibodies to Cl INH, type II with anti Cl INH autoantibodies in the circulation without detectable underlying disease and with depressed functional Cl INH levels. We experienced a case of angioedema in a 29-year old man. He had no family history of angioedema and laboratory data showed depressed Cl-INH levels. We diagnosed the case as acquired type of angioedema. Even though we could not measure anti-Cl INH auto-antibodies, we identified the case as type II because there was no evidence of underlying disease.
Adult ; Angioedema* ; Angioedemas, Hereditary* ; Autoantibodies ; B-Lymphocytes ; Complement C1 Inhibitor Protein* ; Complement C1s* ; Edema ; Humans ; Skin ; Subcutaneous Tissue ; Wills

Episodic angioedema with eosinophilia is characterized by recurrent angioedema, urticaria, fever, weight gain and hypereosinophilia. Although this syndrome shows similarities to idiopathic hypereosinophilic syndrome, it differs in that it has good prognosis and has no organ involvement. The pathogenesis is unclear, but IL-5 is thought to be major cytokine of pathogenesis. It has benign course with spontaneous remission or low-dose prednisolone treatment. Here, we describe a patient whose clinical and laboratory features are similar to those observed in episodic angioedema with eosinophilia.
Angioedema* ; Eosinophilia* ; Fever ; Humans ; Hypereosinophilic Syndrome ; Interleukin-5 ; Prednisolone ; Prognosis ; Remission, Spontaneous ; Urticaria ; Weight Gain

Angioedema is a non-pitting edema that occurs in the skin and mucus membranes. It is known that major etiologies include hereditary deficiency of C1 esterase inhibitor, temperature extreme, trauma, food sensitivity, and medications such as penicillin, aspirin, NSAIDS and ACE inhibitors. ACE inhibitors are blocking the conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, and increasing local levels of bradykinin, a potent vasodilator. This increased bradykinin has been theorized to cause angioedema and cough in patients on ACE inhibitors. However, there has been very few causes of angioedema induced by angiotensin II receptor blocker. This is the first report of a patient presenting angioedema induced by losartan -angiotensin II receptor blocker- in this century.
Angioedema* ; Angiotensin I ; Angiotensin II ; Angiotensin-Converting Enzyme Inhibitors ; Anti-Inflammatory Agents, Non-Steroidal ; Aspirin ; Bradykinin ; Cough ; Edema ; Hereditary Angioedema Types I and II ; Humans ; Losartan* ; Membranes ; Mucus ; Penicillins ; Receptors, Angiotensin ; Skin