In Albright's hereditary osteodystrophy (AHO) including the syndromes of pseudohypoparathyroidism (PHP) and pseudopseudohypoparathyroidism (PPHP), multiple areas of intracutaneous ossification are often encountered. The characteristic features are short stature, round face, short neck, obesity, cutaneous ossifications, and various skeletal anomalies including short metacarpal and metatarsal bones, curve of radius, and brachydactyly. The patient was a 10-month-old male infant. He presented slightly depressed erythematous hard plaques on the left upper chest and left thigh. We had taken biopsies from both skin lesions, confirming cutaneous ossification or bone formation. He also had the characteristic features of AHO. He had a history of admission due to patent ductus arteriosus and atrial septal defect. The laboratory results showed slightly decreased calcium, increased phosphorus and PTH levels. The patient received no specific corrective measures because his calcium and phosphorus levels were not far from normal values until newly developed similar skin lesions appeared.
Biopsy ; Brachydactyly ; Calcium ; Ductus Arteriosus, Patent ; Heart Septal Defects, Atrial ; Humans ; Infant ; Male ; Metatarsal Bones ; Neck ; Obesity ; Osteogenesis ; Phosphorus ; Pseudohypoparathyroidism* ; Pseudopseudohypoparathyroidism ; Radius ; Reference Values ; Skin ; Thigh ; Thorax

A 34-year-old woman presented with several intensely pruritic erythematous serpiginous thread-like skin lesions which began as a small papule on the epigastrium 4 months prior to her visit and was migrating to the left chest area. The laboratory examination showed eosinophilia and the total serum IgE level was slightly increased. Histopathologic examination revealed a pustular burrow in the epidermis with a larva-like cystic lesion apart from the burrow in the epidermis. The patient was treated with topical 10 % albendazole cream 3 times daily for 1 week without recurrence for 22 months up to now. We thought this was a rare case which was confirmed by a skin biopsy showing larva in the epidermis.
Adult ; Albendazole ; Biopsy ; Eosinophilia ; Epidermis ; Female ; Humans ; Immunoglobulin E ; Larva ; Larva Migrans* ; Recurrence ; Skin ; Thorax

There have been several reports of more than one type of porokeratosis occurring in the same family or the same individual. We hope to support the view of different phenotypic expressions of a common genetic aberration by describing an additional case of porokeratosis of Mibelli on the perianal area and DSAP on the face, forearms occurring in a 45-year-old man.
Actins* ; Forearm ; Hope ; Humans ; Middle Aged ; Porokeratosis*

No abstract available.
Drug Therapy* ; Lung*

No abstract available.
Uterine Prolapse*

No abstract available.
Ichthyosiform Erythroderma, Congenital*

No abstract available.
Female ; Ovary* ; Teratoma*

No abstract available.
Female ; Ovary* ; Teratoma*

Anaphylaxis is a life - threatening syndrome elicited in a hypersensitive individual on subsequent exposure to a sensitizing antigen with, reactions ranging widely in severity from mild pruritus and criteria to shock and death. Anaphylaxis occurs in the United States at rates estimated to be as high as one in every 3000 inpatients and may account for more than 500 deaths annually. Preparations of kelp Laminaria digitata are used as tents in gynecologic procedures to dilate the cervix, and the kelp consists principally of a glucagon carbohydrate called laminarin. Laminarin is thought to function as the active ingredient in dilatation by changing osmotic pressure or uterine prostaglandin metabolism. The tent is inserted into the cervix several hours before the procedure. As the cervix desiccates and dilates, the tent develops an hourglass shape. Recently, we experienced one patient with anaphylaxis to laminaria diagnosed by skin test and showing good response to treatment.
Anaphylaxis* ; Cervix Uteri ; Dilatation ; Female ; Glucagon ; Humans ; Inpatients ; Kelp ; Laminaria* ; Metabolism ; Osmotic Pressure ; Pruritus ; Shock ; Skin Tests ; United States

PURPOSE:Idiopathic ketotic hypoglycemia (IKH) is an important cause of hypoglycemia in children. The purpose of this study was to determine the prevalence of ketotic hypoglycemia and to describe the clinical manifestation. METHODS:We conducted a retrospective chart review of children presenting hypoglycemia to the emergency department (ED) of Ewha Womans University Hospital between January 1995 and June 2004. Information recorded for subject included age, gender, weight, time of presentation, presenting symptoms, and laboratory data. RESULTS:Eighty two children were identified for hypoglycemia during the study period. IKH was the ultimate diagnosis for 66 patients (80.5%). Other diagnoses included hyperinsulinism (2.4%), drug adverse event (1.2%), sepsis (1.2%), and other disorder. The mean age for presentation of IKH was 37.9+/-18.6 months. Thirty eight boys and twenty eight girls were identified. The median time of presentation was 9:48 a.m. Of the 66 subjects, body weight of 46.9% patients was below 25th percentile for age. The average blood glucose was 41.4+/-14.2 mg/dL. Symptoms at presentation included lethargy (24.2%), mental change (16.7%) and vomiting (16.7%). 49 patients (74.2%) were described as having a concomitant illness. CONCLUSION: IKH was responsible for 80.4% of cases of hypoglycemia in pediatric ED and it had usually concomitant intercurrent illness which resulting in poor oral intake. Awareness of IKH, and its presenting characteristics, will be helpful in the ED when taking care of hypoglycemic children.
Blood Glucose ; Body Weight ; Child ; Diagnosis ; Emergency Service, Hospital ; Female ; Humans ; Hyperinsulinism ; Hypoglycemia* ; Lethargy ; Prevalence* ; Retrospective Studies ; Sepsis ; Vomiting