No abstract available.
Chlorambucil* ; Nephrotic Syndrome*

No abstract available.
Child* ; Humans

No abstract available.
Aneurysm*

PURPOSE: To compare the prevalance and the amount of astigmatism between the epiblepharon patients who had been operated epiblepharon and normal school children. METHODS: The noncycloplegic refraction using autorefractor was performed in 120 epiblepharon patients from Jan. 1997 to June 2000. Astigmatism in excess of 0.5 D was studied. We compared the findings of epiblephron patients and 443 normal school children in Seoul. RESULTS: The prevalence of astigmatism was 72.9% in epiblephron group and 41.6% in normal children group, and the difference was statistically significant (P< 0.001). The amount of astigmatism was 1.52 D and 0.73 D in each group, and the difference was statistically significant (P< 0.001).The distribution of astigmatism showed 1.0 D or less in 22.5%, from above 1.0 D to 2.0 D or less in 26.3%, from above 2.0 D to 3.0 D or less in 10.8%, from above 3.0 D to 4.0 D or less in 9.6% and above 4.0 D in 3.8% in epiblepharon group and in normal children group 26.2%, 11.2%, 2.6%, 1.0%, and 0.7% respectively. The difference between both groups was statistically significant (P< 0.001). With-the-rule astigmaism was found in 77.9% and against-the-rule was 22.1% in epiblephron group and 72.8% and 27.2% respectively in normal children group. CONCLUSIONS: The prevalence and the amount of astigmatism were higher in epiblepharon group than in normal children group. It might be caused by the increase of orbicularis muscle tone and the effect of skin fold overriding lid margin in epiblepharon patients.
Astigmatism* ; Child* ; Humans ; Prevalence ; Seoul ; Skin

We report a case of pneumonia in 36 year-old male patient who presented acute respiratory failure and associated radiologic findings of bilateral ground-glass opacity with focal cystic changes, showing rapidly aggravating course and was diagnosed as concomitant Pneumocystis carinii and Cytomegalovirus pneumonia accompanied by acquired immunodeficiency syndrome through antemortem open lung biopsy.
Acquired Immunodeficiency Syndrome ; Biopsy ; Cytomegalovirus ; Humans ; Lung ; Male ; Pneumocystis carinii ; Pneumonia ; Respiratory Insufficiency*

Transient tachypnea of the newborn(TTN) is a benign self-limited disease characterized by early onset and rapid recovery of tachypnea although it may occasionally have a more prolonged and protracted course. A retrospective clinical study was mad on 29 neonates with TTN admitted to NICU of Pohang St. Mary's Hospital from January 1992 to June 1993. The results were as follows: 1) TTN(36.2%) was the most common cause of respiratory distress in the neonate followed by idiopathic respiratory distress syndrome(23.8%), pneumonia(18.8%), meconium aspiration syndrome(6.2%), perinatal asphyxia(6.2%), polycythemia(3.8%), anemia(1.2%), persistent fetal circulation(1.2%), paroxysmal supraventricular tachycardia(1.2%) and tracheoesophageal fistula(1.2%) 2) TTN was more frequent in the male term infants but can occur in premature(41.4%) and low birth weight infants(34.5%). 3) The associated perinatal conditions were oxytocin-induction(8 cases, 27.6%), Cesarean delivery(10 cases, 34.5%) and asphyxia(7 cases, 24.2%) 4) Arterial blood gas analysis showed respiratory acidosis in 3 cases and metabolic acidosis in 3 cases but none of TTN showed hypoxia unresponsive to oxygen 5) Chest X-ray showed hyperaeration in 10 cases(34.5%), increased pulmonary vascularity in 8 cases(27.6%), hyperaeration and increased pulmonary vascularity in 6 cases(20.6%) and cardiomegaly in 12 cases(53%). 6) Tachypnea usually appeared within 6hours and abated by 48-72hours but sustained more than 73hours in 7 cases(24.1%) 7) In majority of cases, maximal respiratory rates were below 100 rates/min and administered oxygen concentrations were 20-40%, and their mean values were 81 rates/min, 37.6% respectively. 8) TTN with more prolonged course(> or=48hours) was associated with low birth weight infants, prematurity and higher respiratory rates(> or=100 breaths a minute)(p<0.05).
Acidosis ; Acidosis, Respiratory ; Anoxia ; Blood Gas Analysis ; Cardiomegaly ; Gyeongsangbuk-do ; Humans ; Infant ; Infant, Low Birth Weight ; Infant, Newborn ; Male ; Meconium Aspiration Syndrome ; Oxygen ; Respiratory Rate ; Retrospective Studies ; Tachypnea ; Thorax ; Transient Tachypnea of the Newborn*

Transient tachypnea of the newborn(TTN) is a benign self-limited disease characterized by early onset and rapid recovery of tachypnea although it may occasionally have a more prolonged and protracted course. A retrospective clinical study was mad on 29 neonates with TTN admitted to NICU of Pohang St. Mary's Hospital from January 1992 to June 1993. The results were as follows: 1) TTN(36.2%) was the most common cause of respiratory distress in the neonate followed by idiopathic respiratory distress syndrome(23.8%), pneumonia(18.8%), meconium aspiration syndrome(6.2%), perinatal asphyxia(6.2%), polycythemia(3.8%), anemia(1.2%), persistent fetal circulation(1.2%), paroxysmal supraventricular tachycardia(1.2%) and tracheoesophageal fistula(1.2%) 2) TTN was more frequent in the male term infants but can occur in premature(41.4%) and low birth weight infants(34.5%). 3) The associated perinatal conditions were oxytocin-induction(8 cases, 27.6%), Cesarean delivery(10 cases, 34.5%) and asphyxia(7 cases, 24.2%) 4) Arterial blood gas analysis showed respiratory acidosis in 3 cases and metabolic acidosis in 3 cases but none of TTN showed hypoxia unresponsive to oxygen 5) Chest X-ray showed hyperaeration in 10 cases(34.5%), increased pulmonary vascularity in 8 cases(27.6%), hyperaeration and increased pulmonary vascularity in 6 cases(20.6%) and cardiomegaly in 12 cases(53%). 6) Tachypnea usually appeared within 6hours and abated by 48-72hours but sustained more than 73hours in 7 cases(24.1%) 7) In majority of cases, maximal respiratory rates were below 100 rates/min and administered oxygen concentrations were 20-40%, and their mean values were 81 rates/min, 37.6% respectively. 8) TTN with more prolonged course(> or=48hours) was associated with low birth weight infants, prematurity and higher respiratory rates(> or=100 breaths a minute)(p<0.05).
Acidosis ; Acidosis, Respiratory ; Anoxia ; Blood Gas Analysis ; Cardiomegaly ; Gyeongsangbuk-do ; Humans ; Infant ; Infant, Low Birth Weight ; Infant, Newborn ; Male ; Meconium Aspiration Syndrome ; Oxygen ; Respiratory Rate ; Retrospective Studies ; Tachypnea ; Thorax ; Transient Tachypnea of the Newborn*

Wilson's disease typically presents symptoms associated with liver damage or neuropsychiatric disturbances, while endocrinologic abnormalities are rare. We report an unprecedented case of hypopituitarism in a patient with Wilson's disease. A 40-year-old woman presented with depression, general weakness and anorexia. Laboratory tests and imaging studies were compatible with liver cirrhosis due to Wilson's disease. Basal hormone levels and pituitary function tests indicated secondary hypothyroidism and adrenal insufficiency due to hypopituitarism. Brain MRI showed T2 hyperintense signals in both basal ganglia and midbrain but the pituitary imaging was normal. She is currently receiving chelation therapy along with thyroid hormone and steroid replacement. There may be a relationship between Wilson's disease and hypopituitarism. Copper deposition or secondary neuronal damage in the pituitary may be a possible explanation for this theory.
Adrenal Insufficiency/diagnosis/etiology ; Adult ; Brain/diagnostic imaging ; Depression/etiology ; Female ; Hepatolenticular Degeneration/*complications ; Humans ; Hypopituitarism/complications/*diagnosis/drug therapy ; Hypothyroidism/diagnosis/etiology ; Liver Cirrhosis/complications/diagnostic imaging ; Magnetic Resonance Imaging ; Steroids/therapeutic use ; Thyrotropin-Releasing Hormone/therapeutic use

Wilson's disease typically presents symptoms associated with liver damage or neuropsychiatric disturbances, while endocrinologic abnormalities are rare. We report an unprecedented case of hypopituitarism in a patient with Wilson's disease. A 40-year-old woman presented with depression, general weakness and anorexia. Laboratory tests and imaging studies were compatible with liver cirrhosis due to Wilson's disease. Basal hormone levels and pituitary function tests indicated secondary hypothyroidism and adrenal insufficiency due to hypopituitarism. Brain MRI showed T2 hyperintense signals in both basal ganglia and midbrain but the pituitary imaging was normal. She is currently receiving chelation therapy along with thyroid hormone and steroid replacement. There may be a relationship between Wilson's disease and hypopituitarism. Copper deposition or secondary neuronal damage in the pituitary may be a possible explanation for this theory.
Adrenal Insufficiency/diagnosis/etiology ; Adult ; Brain/diagnostic imaging ; Depression/etiology ; Female ; Hepatolenticular Degeneration/*complications ; Humans ; Hypopituitarism/complications/*diagnosis/drug therapy ; Hypothyroidism/diagnosis/etiology ; Liver Cirrhosis/complications/diagnostic imaging ; Magnetic Resonance Imaging ; Steroids/therapeutic use ; Thyrotropin-Releasing Hormone/therapeutic use

Familial adenomatous polyposis (FAP) is an autosomal dominant disease characterized by formation of multiple colorectal adenomas with nearly 100 percent potential for malignant transformation. FAP is a rare condition with an incidence of 1 in 10,000 live births. Germline mutations in the adenomatous polyposis coli gene (APC) located on chromosome 5q21 have been founded in many patients with FAP. Patients with FAP can have extracolonic manifestations of their disease. These include tumors of the upper gastrointestinal tract (hamartomatous polyps, adenomas, carcinomas), small intestine adenomas or cacinoma, bile duct adenomas, papillary thyroid carcinoma, osteomas of the mandible, skull, and long bones, a variety of soft tissue lesions, including fibromas, lipomas, and desmoid tumors, congenital hypertrophy of the retinal pigment epithelium (CHRPE) and hepatoblastoma. Hepatocellular carcinoma combined with FAP is a very rare condition. Just 8 cases of Hepatocellular carcinoma with a history of FAP have been reported in the literature. We now present a report of a case of Hepatocellular carcinoma with FAP (Gardner's syndrome) in a 19 year-old girl.
Adenoma ; Adenoma, Bile Duct ; Adenomatous Polyposis Coli* ; Carcinoma, Hepatocellular* ; Female ; Fibroma ; Fibromatosis, Aggressive ; Germ-Line Mutation ; Hepatoblastoma ; Humans ; Hypertrophy ; Incidence ; Intestine, Small ; Lipoma ; Live Birth ; Mandible ; Osteoma ; Polyps ; Retinal Pigment Epithelium ; Skull ; Thyroid Neoplasms ; Upper Gastrointestinal Tract ; Young Adult