No abstract available.
Child* ; Dehydration* ; Gastroenteritis* ; Humans

A 4-year-old boy has had a solitary sclerotic depressed plaque on the right anterior chest since birth. The histopathologic findings are consistent with morphea profunda: thickening, hyalinization, and homogenization of collagen bundles in the dermis and subcutaneous tissues, admixture with a prominent lymphocytic and plasma cell infiltrate, and sweat glands en-trapped between the thickened collagen bundles. We report a case of congenital solitary morphea profunda.
Child, Preschool ; Collagen ; Dermis ; Humans ; Hyalin ; Male ; Parturition ; Plasma Cells ; Scleroderma, Localized* ; Subcutaneous Tissue ; Sweat Glands ; Thorax

No abstract available.
Thanatophoric Dysplasia*

Plexiform neurofibroma is considered a pathognomic of Von Recklinghousen's disease, which involves the deep and large nerve trunk. These are large irregular nerve fascicles which result from an increase in endoneural matrix within individual nerve facicles, without an increased number of nerve fibers. We experenced a case of Von Recklinghausen's disease in a 24 year-old male who had variable cutaneous skeletal, and CNS lesions. He presented multiple neurofibromas, cafe-au-lait spots, and axillary freckles as common cutaneous lesions of NF-I and giant pigmentation, sacral hypertrichosis, and plexiform neurofibroma as unusual cutaneous lesions. Also he had a scoliosis, bowing deformity of the humerous and wedging deformity of the body of the 5th cervical spine as a skeletal manifestation and cortical calcification in the occipital area as a CNS manifestation.
Cafe-au-Lait Spots ; Congenital Abnormalities ; Humans ; Hypertrichosis ; Male ; Melanosis ; Nerve Fibers ; Neurofibroma, Plexiform* ; Neurofibromatoses ; Neurofibromatosis 1 ; Pigmentation* ; Scoliosis ; Spine ; Young Adult

BACKGROUND: Bentonite clay, which is a major component of mud pack, has been used for various purposes in cosmetics. Glycolic acid is known to be effective in the treatment of acne. Al-though those products are used widely, information on the mode of action and effects on the skin are little and controversial till now. OBJECTIVE: To investigate whether bentonite alone, or bentonite with glycolic acid in mixed formulation affect the stratum corneum leading to alteration on cutaneous barrier function and whether those products alter the lipid lamellae and desmosomes of corneocytes. MATERIALS AND METHODS: Mud pack-type ointment of bentonite, bentonite and 5% glycolic acid formulation, bentonite and 10% glycolic acid formulation were applied on the volar fore-arm of the five healthy men and flank skin of five 6-8 week old hairless mice. Transepidermal water loss and capacitance were measured. Electron microscopic examination after ruthenium tetroxide postfixation was performed on the flank skin of the mice. RESULTS: Transepidermal water loss(TEWL) increased immediately and normalized 4 to 6 hours later after removal of vapor permeable membrane in both mouse and human. Capacitance did not show any evidence of change in the water content of the stratum corneum. Electron microscopic examination revealed that lipid lamellae and desmosome of corneocytes were not de-graded, but lamellar body secretion and partially electron-lucent material was-increased in 10% glycolic acid and bentonite mixture-treated area. CONCLUSION: Barrier function of stratum corneum is not disturbed by bentonite and glycolic acid formulations at the concentration used. Barrier structures are not disrupted, but lamellar body secretion and partially electron-lucent material was increased by bentonite and glycolic acid formulations at higher concentration.
Acne Vulgaris ; Animals ; Bentonite* ; Desmosomes ; Humans ; Male ; Membranes ; Mice ; Mice, Hairless ; Mud Therapy ; Ruthenium ; Skin ; Water

No Abstract Available.
Rosacea*

No abstract available.
Child* ; Humans

The antiphospholipid antibody syndrome is an acquired multisystemic disorder characterized by persistent elevated antiphospholipid antibodies and/or hypercoagulation in veins or arteries, or both. The clinical manifestations of the antiphospholipid antibody syodrome are recurrent thrombosis, fetal loss, thrcenbocytopenia, and various cutaneous lesions. Skin lesions are the first sign of this syndrome in 41% of patients and systemic thrombosis develops in 40% of them. Livedo reticularis is the most common cutaneous finding of the antiphosphotipid antibody syndrome. Although vasculitis has not been frequently noted in antiphospholipid antibody syndrome, some vasculitis such as polyarteritis nodosa, giant cell arteritis, and other nonspecific vasculitides have been found in association with antiphospholipid antibody syndrome. We present a male patient with typical manifestations of leukocytoclastic vasculitis with deep vein thrombosis and positive antiphospholipid antibodies. It suggests that a case of antiphospholipid antibody syndorme was accompanied with cutaneous leukocytoclastic vasculitis.
Antibodies, Antiphospholipid* ; Antiphospholipid Syndrome ; Arteries ; Giant Cell Arteritis ; Humans ; Livedo Reticularis ; Male ; Polyarteritis Nodosa ; Skin ; Thrombosis ; Vasculitis* ; Vasculitis, Leukocytoclastic, Cutaneous ; Veins ; Venous Thrombosis

The times of passage of the first stool and urine in the newborn infant are important because delayed micturition may indicate urinary tract pathology and failure to pass meconium may be an early sign of intestinal obsturuction. Times of first void and first stool in 180 consecutive low birth weight, premature infant born at Seoul National University Hospital during 44 months from January 1976 to August 1979 were recorded. Available data were gathered with regard to gestional age.The aim was to determine the relation of the first void and first stool to gestational age, mode of delivery, initial feeding and Apgar score. All infants voided by 34 hours with a tendency for preterm woid early. There was a delay in the passage of the meconium by the low birth weight, premature infant as compared with full term infant.Smaller gestational age group tended to pass the meconium late in all groups and early feeding group tended to pass the first meconium and the first void early. But there was no differences of the time of the first meconium and first urination between Apgar score or mode of delivery.If meconium passage did not occur within 24 hours after birth, close observations were needed.But Failure of low birth weight infants to pass meconium by 36 hours shoul call for examination to determine the cause of delay of meconium passage. Similarly, close observation of all premature who have not voided for 24 hours is indicated.
Apgar Score ; Gestational Age ; Humans ; Infant ; Infant, Low Birth Weight* ; Infant, Newborn ; Infant, Premature* ; Meconium ; Parturition ; Pathology ; Seoul ; Urinary Tract ; Urination

PURPOSE: Considering that renal biopsy is not routinely indicated in nephrotic syndrome in children and the risk of the procedure, we studied that it is possible to predict steroid responsiveness in nephrotic syndrome and the difference in responsivenss is related with the histopathologic type using urinary beta2-microglobulin and N-Acetyl-beta-D-Glucosaminidase activity as a safe and noninvasive method. METHODS: We measured serum creatinine, albumin, cholesterol and urinary creatinine, protein, B2M, NAG at the time of admission and after 2 weeks of treatment in 37 cases of primary nephrotic syndrome in children who were admitted to the Pediatric department of Chung-Ang Medical Center between March 1, 1994 and June 30, 1996. RESULTS: The results were as follows : 1) When urinary B2M value of less than 1000microgram/g creatinine was used as the cut-off value, the test identified steroid responsiveness with a sensitivity of 100%, a specificity of 80%. When urinary NAG activity of less than 100U/hr/mg creatinine was used as the cut-off value, the test identified steroid responsiveness with a sensitivity of 100%, a specificity of 81.8%. 2) Pretreatment urinary B2M levels in Good-response and Poor-response group were 559.8+/-718.5microgram/g creatinine, 3599.1+/-4956.7microgram/g creatinine, respectively. There was statistical significance between the two groups (P<0.05). 3) Pretreatment urinary NAG activity in Good-response and Poor-response group were 42.6+/-23.6U/hr/mg creatinine, 79.6+/-80.1U/hr/mg creatinine, respectively. There was statistical significance between the two groups (P<0.05). CONCLUSIONS: In conclusion, measurement of urinary B2M level and NAG activity for early detection of renal tubular damage is helpful in prediction of steroid responsiveness of primary nephrotic syndrome. If the urinary B2M level and NAG activity are elevated, the patient is suspected to steroid resistance. Therefore, prolonged steroid therapy should be avoided in such patients because of their greater chance of not responding. This approach would reduce the many serious side effects of prolonged treatment in patients who are unlikely to benefit.
Acetylglucosaminidase* ; Biopsy ; Child ; Cholesterol ; Creatinine ; Humans ; Nephrotic Syndrome* ; Sensitivity and Specificity