No abstract available.

A 42-year-old woman presented with ichthyosiform eruptions on her trunk and buttock which developed 20 days prior to consulatation. She had taken the cholesterol-lowering drug(lovastatin, a 3-hydroxy-3-methylglutaryl-coenzyme A (HMG CoA) reductase inhibitor) for 4 months. After stopping the drug, the skin lesions gradually improved and after two months most of the lesions had disappeared. After 3 months of follow up the skin lesions could not be seen any more. Acquired ichthyosis in our patient could be an untoward effect of HMG CoA reductase inhibitor by disturbing the skin lipid metabolism.
Adult ; Buttocks ; Female ; Follow-Up Studies ; Humans ; Hydroxymethylglutaryl CoA Reductases ; Ichthyosis* ; Lipid Metabolism ; Lovastatin ; Oxidoreductases ; Skin

Freeman-Sheldon Syndrome is one of the very rare genetic diseases which primarily affects face, hands and feet. At first, Freeman and Sheldon described this syndrome as cranio-carpo-tarsal dystrophy in 1938 and later Burian, as "whistling face" syndrome in 1963. There were 60 cases of reports in the world up to now, and only one paper with 5 cases in a family was reported in Korea. The authors report 2 cases of Freeman-Sheldon Syndrome associated with bilateral inguinal hernias and undescended tests in a family, briefly review the literature and alert orthopaedic surgeons to this condition.
Cryptorchidism ; Foot ; Hand ; Hernia, Inguinal ; Humans ; Korea ; Male ; Surgeons

No abstract available.
Decompression, Surgical* ; Exophthalmos*

BACKGROUND: Nitric Oxide (NO) has been discovered to be an important endothelium-derived relaxing factor. The exogenous inhaled NO may diffuse from the alveoli to pulmonary vascular smooth muscle and produce pulmonary vasodilation, but any NO that diffuses into blood will be inactivated before it can produce systemic effects. To examine the effects of NO on pulmonary and systemic hemodynamics, NO was inhaled by experimental dogs in an attempt to reduce the increase in pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR) induced by hypoxia in dogs. METHODS: Eight mongrel dogs were studied while inhaling 1)50% O2 (baseline), 2)12% O2 in N2 (hypoxia), 3)followed by the same hypoxic gas mixture of O2 and N2 containing 20, 40 and 80 ppm of NO, respectively. RESULTS: Breathing at FIO2 0.12 nearly doubled the pulmonary vascular resistance from 173 56dyn sec cm-5 to 407 139dyn sec cm-5 and significantly increased the mean pulmonary artery pressure from 16 3mmHg to 22 4mmHg. After adding 20~80 ppm NO to the inspired gas while maintaining the FIO2 at 0.12, the mean pulmonary artery pressure decreased (p<0.05) to the level when breathing oxygen at FIO2 0.5 while the PaO2 and PaCO2 were unchanged. The pulmonary vascular resistance decreased significantly and the right ventricular stroke work index returned to a level similar to breathing at FIO2 0.5 by addition of NO into the breathing circuit. Pulmonary hypertension resumed within 3~5 minutes of ceasing NO inhalation. In none of our studies did inhaling NO produce systemic hypotension and elevate methemoglobin levels. CONCLUSIONS: Inhalation of 20~80 ppm NO selectively induced pulmonary vasodilation and reversed hypoxic pulmonary vasoconstriction without causing systemic vasodilation and bronchodilation. Methemoglobin and NO2 were within normal limit during the study.
Animals ; Anoxia ; Dogs ; Endothelium-Dependent Relaxing Factors ; Hemodynamics* ; Hypertension, Pulmonary ; Hypotension ; Inhalation* ; Methemoglobin ; Muscle, Smooth, Vascular ; Nitric Oxide* ; Oxygen ; Pulmonary Artery ; Respiration ; Stroke ; Vascular Resistance ; Vasoconstriction* ; Vasodilation

This report describes the pathologic features of 17 cases of Castleman's disease, examined at the Department of Pathology, Seoul National University Hospital during a period from 1973 to 1989. The lesions in 12 cases were hyaline-vascular type and the remainders plasma cell type. The pathologic features favoring the plasma cell type over the hyaline vascular type included a sufficient number to large-sized follicles. However, a histologic overlapping between two types was present. In the hyaline vascular type the age of the patients ranged from 7 to 76 years and they appeared to be no particular sex predominence. The majority of the lesions occurred in the neck and within the chest. Almost all cases presented with a solitary mass except three cases. Neither conventional symptoms nor systemic manifestations were associated. The plasma cell type was characterized by presentation of constitutional symptoms, involvement of intra abdominal and inguinal lymphnodes, in association with unusual clinicopathologic features including IgA nephropathy, diabetes mellitus, systemic progressive sclerosis, peripheral neuropathy, and anemia. Immunohistochemical study was performed in three cases of the plasma cell type. Two cases revealed poly-clonal plasma cell infiltration. In a patient with IgA nephropathy, however, serum IgA was increase and a strong immunoreactivity to IgA heavy chain was found. Another case, associated with systemic progressive sclerosis and neuropathy, revealed monoclonal plasma cell infiltration (IgG and lambda light chain). The above results support a possibility that in some of the plasma cell type an altered immune mechanism is involved in its pathogenesis.

No abstract available.
Skin*

BACKGROUND: Many reports have shown that hormone replacement therapy(HRT) in postmenopausal women decreases lipoprotein(a)[Lp(a)]. However these had small numbers of subjects, short duration of therapy, or comparisons of only a few regimens. The influences of progesterone on Lp(a) and lipids, administered with estrogen, are controversial. METHODS: Five hundred and fifty-one postmenopausal women were divided into 4 groups : group A ; 0.625mg conjugated equine estrogen(CEE)(m=140), group B ; 0.625mg CEE plus 5mg medroxyprogesterone acetate(MPA)(m=97), group C ; 0.625mg CEE plus 10mg MPA(n=109), and group D ; 2mg estradiol valerate(E2) plus 0.5mg norgestrel(N)(n=134) and group E ; control(n=71). Lp(a) and lipids levels were measured before and 12 months after HRT. RESULTS: Estrogen replacement therapy(ERT) for 12 months lowered Lp(a) level by 37.1%. The addition of progesterone attenuated the Lp(a)-lowering effect of estrogen and decreased by 27.7%, 29.6%, and 30.3% in groups B(p<0.05), C(p<0.05), and D(p<0.0001) respectively. High density lipoprotein cholesterol(HDL-C) was increased markedly in group A(16.5%), increased moderately in groups B(10.8%) and C(11.3%), and not changed in group D. Low density lipoprotein cholesterol was decreased by 10.9%, 13.7%, 11.3%, and 17.6% in groups A, B, C, and D respectively. CONCLUSIONS: Reduction of Lp(a) with estrogen replacement therapy may be one of mechanisms for cardioprotective effect in postmenopausal women. The combined therapy of estrogen and progesterone may reveal different effects on heart due to adverse actions of progesterone on Lp(a) and HDL-C. The variations in the androgenic potency of progesterone may explaine inconsistent results on HDL-C in previous studies.
Cholesterol, LDL ; Estradiol ; Estrogen Replacement Therapy ; Estrogens ; Female ; Heart ; Hormone Replacement Therapy* ; Humans ; Lipoprotein(a)* ; Lipoproteins ; Medroxyprogesterone ; Progesterone*

No abstract available.
Intussusception*

Congenital smooth musele hamartomas appear at birth as hypertrichotic patches or plaques with or without hyperpigmentation. Histologic characteristic is hyperplasia of dermal smooth muscle bundles. We report a case of congenital smooth muscle harnartoma in 5-year-old female, who showed localized excessive-hairy, skin-colored patch on the lateral surface of left. elbow, which were found at birth. Biopsy specimen showed hyperpigmentation of the basal layers of epidermis, melanophages in upper dermis, and hyperplasia of smooth muscle bundles in lower dermis.
Biopsy ; Child, Preschool ; Dermis ; Elbow ; Epidermis ; Female ; Hamartoma* ; Humans ; Hyperpigmentation ; Hyperplasia ; Hypertrichosis ; Muscle, Smooth* ; Parturition