Hydroa Vacciniforme is a rare, light sensitive dermatitis characterized by discrete vesicles followed by varioliform scar formation, which was first described by Bazin on 1862. We have expe"ienced three cases of hydroa vacciniforme in childhood. The first case was a 6-year-old boy who had discrete vesicles, crusts and pitting scars on forehead, both cheeks, ears and dorsum of the hands in symmetrical distribution with itching sensation, which had used to recur in every summer since last three years ago. Histologic section of a primary lesion showed necrosis and severe edema in the epidermis and adjacent dermis with the dermal inflammatory cell infiltration. The second case was a 10-year-old girl who had discrete vesicles, crusts, erosive and depigmented patch and scars on face, lip, dorsum of the hand and forearm with itching sensation, which had also developed on last spring. The third case was a 7-year-old boy who had vesiclee, erosive patch, crusts and pitting scars on face, Iower lip, both dorsum of the hand and forearm with itching sensation, which had recurred in every summer since last two years ago, On urine examination, porphyrin, was negative in all three cases. Diagnosis was established by clinical characteristics and laboratory findings and histological pictures. Symptomatic treatment was done in all three cases. The literatures were briefly reviewed.
Cheek ; Child ; Cicatrix ; Dermatitis ; Dermis ; Diagnosis ; Ear ; Edema ; Epidermis ; Female ; Forearm ; Forehead ; Hand ; Humans ; Hydroa Vacciniforme* ; Lip ; Male ; Necrosis ; Pruritus ; Sensation

Immunosuppressive therapy based on the use of antilymphocyte globulin (ALG) has become standard therapy for patients with splastic anemia who are not eligible for bone marrow transplantation. In this study, T cell subsets before and after ALG therapy, hematologic responses, complications and prognostic factors were analysed. Eleven (42%) out of twenty-six patients treated with ALG showed response, but two patients showed relapse. Most of the response (9 cases) was noticed within 6 months after the initiation of ALG therapy (median: 3 months). The main complications of ALG therapy were fever (91%), thrombocytopenia (86%), neutropenia (63%), and serum sickness (56%). Four patients were died just ALG therapy because of serum sickness (2 cases), intracranial hemorrhage (1 case), and shock (1 case). Short interval from diagnosis to treatment suggested to show good response (P=0.0575), but it was not significant statistically. Lymphocyte subsets were measured in the blood of 23 patients. Helper T/suppressor T cell ratio (T4/T8 ratio) at the initiation of ALG therapy (day 0) was higher significantly in patients who were responded (P=0.0299). The patients who showed above 1.0 of T4/T8 ratio on day 0 might be speculated good response (P=0.032). More difference of T4/T8 ratio between day 14 after ALG therapy and day 0 might show good response (P=0.0673). Then the actuarial probability of survival at 3 years in patients treated with ALG was 77%. Our data suggest that ALG therapy may be used as an alternative treatment to bone marrow transplantation, and T4/T8 ration of peripheral blood at the initiation of therapy may be used as one of the prognostic factors.
Anemia ; Anemia, Aplastic* ; Antilymphocyte Serum* ; Bone Marrow Transplantation ; Child* ; Diagnosis ; Fever ; Humans ; Intracranial Hemorrhages ; Lymphocyte Subsets ; Neutropenia ; Recurrence ; Serum Sickness ; Shock ; T-Lymphocyte Subsets ; Thrombocytopenia

No abstract available.

A female newborn had the following characteristics; a congenital localized absence of skin over the lower extremities; blistering of the skin or mucous membrane, incited by trauma, which heals without scarring; and congenital absence or deformity of the nails. In respect to the characteristic manifestation, clinical course and electron microscopic features, our patient seems to fit well into Bart's syndrome. The inheritance pattern appears to be autosomal dominant but, as in this report, isolated cases have been recognized. As the review of the literatures, congenital localized absence of skin has been observed in various subsets of inherited E.B. But, we believe that the term Bart's syndrome should be used to identify patients with good prognosis as the Bart's initial description To the best of our knowedge, this is the first reported case of Bart's syndrome in the korean literature.
Blister ; Cicatrix ; Congenital Abnormalities ; Female ; Humans ; Infant, Newborn ; Inheritance Patterns ; Lower Extremity ; Mucous Membrane ; Prognosis ; Skin

A 7 day old female baby suffered from perinatal asphyxia and trauma at birth. Subsequently, she had a violeceous subcutaneous plaque on back. The clinical setting, the skin manifestation and the histologic findings of the lesion were diagnostic of subcutaneous fat necrosis of the newborn. Histologic findings revealed fat necrosis with infiltration of inflammatory cells, foreign body giant cells and several fat cells contain needle shaped cleft in radial arrangement. Four weeks latter, the skin lesion was healed spontaneously.
Adipocytes ; Asphyxia ; Fat Necrosis ; Female ; Giant Cells, Foreign-Body ; Humans ; Infant, Newborn* ; Necrosis* ; Needles ; Parturition ; Skin ; Skin Manifestations ; Subcutaneous Fat*

No abstract available.
Child* ; Humans ; Neurilemmoma* ; Neurofibromatosis 1

Congenital coronary arteriovenous fistula is a rare condition which is an abnormal communication of the coronary artery with the right ventricle, right atrium, left atrium or left ventricle. Coronary artery aneurysm is an uncommon disease which is defined as coronary dilatation which exceeds the diameter of normal adjacent segments or the diameter of the patient's largest coronary vessel by 1.5 times. In young ages, symptoms are unusual, but significant symptoms and complications such as congestive heart failure, subacute bacterial endocarditis, coronary steal syndrome, aneurysm formation, rupture, and pulmonary hypertension may appear among the older age group. We report a case of giant aneurysm of a congenital coronary arteriovenous fistula between left co-ronary artery and right ventricular outflow tract with significant left to right shunt confirmed in a 84-year old female with a brief review of literature.
Aged, 80 and over ; Aneurysm* ; Arteries ; Arteriovenous Fistula* ; Coronary Aneurysm ; Coronary Vessels* ; Dilatation ; Endocarditis, Subacute Bacterial ; Female ; Heart Atria ; Heart Failure ; Heart Ventricles ; Humans ; Hypertension, Pulmonary ; Rupture

The aim of the present study was to investigate the mRNA expression of several cytokines which were not reported previously from interface tissues around loose cemented acetabulum to obtain better understanding of the biological mechanisms connected with aseptic loosening and osteolysis of THA. We investigated mRNA expression for several cytokines (interleukin-1 alpha [IL-l~a], IL-lp, IL-2, IL-2 receptor[2R], IL-4, IL-5, IL-8, IL-10, transforming growth factor-beta [TGF-p], and interferon- gamma [IFN-y]) by reverse transcription-polymerase chain reaction (RT-PCR) and release of metalloproteinase (MMP)-2 and MMP-9 from the cement-bone interface tissues around five loose polyethylene acetabular components. We did not include TNF-a and IL-6 because the biologic effect of the former is so similar to that of IL-1, and the latter fails to stimulate prostaglandin E, or collagenase production by fibroblsts or synovial cells. Expression of mRNA for IL-1p was detected in four, IL-2R and IL-8 in three, IL-10 and TGF-p in two of five interface tissues .No expression of mRNA for IL-la, IL-2, IL-4, IL-5, and IFN-p was detected. Zymographic analysis for gelatinase/type IV collagenase revealed gelatinolytic bands corresponding to metalloproteinase(MMP)-2 and MMP-9 in cemenl-bone interface tissues. Activated cells phagocytose particles in cement-bone interface tissues expressed more cytokines mRNA than previously known to be related to periprosthetic bone resorption, and secreted metalloproteinases associated with extracellular matrix degradation and fibrosis.
Acetabulum ; Arthroplasty, Replacement, Hip* ; Bone Resorption ; Collagenases ; Cytokines* ; Extracellular Matrix ; Fibrosis ; Interleukin-1 ; Interleukin-10 ; Interleukin-2 ; Interleukin-4 ; Interleukin-5 ; Interleukin-6 ; Interleukin-8 ; Metalloproteases* ; Osteolysis ; Polyethylene ; RNA, Messenger*

Primary hypothyroidism can result in reactive enlargement of the pituitary gland which is indistinguishable from primary pituitary lesions in clinical presentation and on magnetic resonance imaging. A 17-year-old girl came to the hospital due to short stature, general weakness and galac-torrhea. The magnetic resonance imaging (MRI) study showed pitutary enlargement. The hormone study showed hyperprolactinemia, decreased basal growth hormone level and primary hypothyroi-dism. By thyroid replacement therapy only, mass was successfully regressed on follow up MRI after 4 months, and growth acceleration could be achieved.
Acceleration ; Adolescent ; Female ; Follow-Up Studies ; Growth Hormone ; Humans ; Hyperplasia* ; Hyperprolactinemia ; Hypothyroidism* ; Magnetic Resonance Imaging ; Pituitary Gland ; Thyroid Gland

No abstract available.
Lipoma*