Unilateral nevoid telangiectasia(UNT) is a rare disorder and is characterized by superficial telangiectatic lesions distributed along the dermatomes unilaterally and usually on the upper part of the body. It has been said to be related to increased estrogen receptors in the involved skin. The lesions may be congenital or acquired.Acquired cases are typically associated with physiologic conditions such as pregnancy, puberty, hormonal therapy or cirrhosis, but the condition is also described in alcoholism without cirrhosis, carcinoma metastatic to the liver, and in hepatitis C. We present two cases of UNT in a 26-year-old man with heavy alcoholism and in a puberta1 16-year-old girl.
Adolescent ; Adult ; Alcoholism ; Female ; Fibrosis ; Hepatitis C ; Humans ; Liver ; Pregnancy ; Puberty ; Receptors, Estrogen ; Skin ; Telangiectasis*

PURPOSE:Noonan syndrome(NS) is characterized by short stature, congenital heart disease, and typical facies. Recombinant human growth hormone(GH) has been reported to improve growth rate in a similar fashion to that seen in Turner syndrome. We investigated the clinical characteristics and growth reponses to GH therapy in children with NS. METHODS:The cases of sixty seven patients with NS were reviewed retrospectively. Ten of the 65 patients were assessed height, weight and pubertal stage every 3 months during GH therapy. RESULTS:Webbed neck(70%), delayed development(59.7%), low set posterior hairline(56.7%), eye abnormalities(56.7%) and mental retardation(55.2%) were the leading clinical characteristics. Short stature below the 3rd percentile was presented in 73.8 %. Growth patterns in NS children were variable and the evaluation of their growth must be individualized. The increments of height SDS were significant in children with GH therapy(height SDS:from -2.8+/-.6 to -2.3+/-.9, growth velocity:from 4.4+/-.8 cm to 9.2+/-.9 cm during first year, and 6.1+/-.1 cm during second year) (P<0.05). CONCLUSION: This study characterized the clinical profiles in Korean children with NS, which should be further extended with more children with NS. Additionally, the significant increase in final adult height after GH therapy in children with NS should be observed.
Adult ; Child* ; Facies ; Heart Defects, Congenital ; Humans ; Noonan Syndrome* ; Retrospective Studies ; Turner Syndrome

PURPOSE:Noonan syndrome(NS) is characterized by short stature, congenital heart disease, and typical facies. Recombinant human growth hormone(GH) has been reported to improve growth rate in a similar fashion to that seen in Turner syndrome. We investigated the clinical characteristics and growth reponses to GH therapy in children with NS. METHODS:The cases of sixty seven patients with NS were reviewed retrospectively. Ten of the 65 patients were assessed height, weight and pubertal stage every 3 months during GH therapy. RESULTS:Webbed neck(70%), delayed development(59.7%), low set posterior hairline(56.7%), eye abnormalities(56.7%) and mental retardation(55.2%) were the leading clinical characteristics. Short stature below the 3rd percentile was presented in 73.8 %. Growth patterns in NS children were variable and the evaluation of their growth must be individualized. The increments of height SDS were significant in children with GH therapy(height SDS:from -2.8+/-.6 to -2.3+/-.9, growth velocity:from 4.4+/-.8 cm to 9.2+/-.9 cm during first year, and 6.1+/-.1 cm during second year) (P<0.05). CONCLUSION: This study characterized the clinical profiles in Korean children with NS, which should be further extended with more children with NS. Additionally, the significant increase in final adult height after GH therapy in children with NS should be observed.
Adult ; Child* ; Facies ; Heart Defects, Congenital ; Humans ; Noonan Syndrome* ; Retrospective Studies ; Turner Syndrome

No abstract available.
Bone Plates* ; Spine*

We report a rare case of glomangiomyoma in a 25-year-old man who had an asymptomatic, 1.5 × 1.5cm sized, round, skin-colored, soft mass on the right cheek. Histopathologically, the lesion was consistent with glomangiomyoma which showed irregularly dilated vessels lined by endothelial cells and several outer layers of glomus cells, and smooth muscles distributed within the tumor. The tumor cells were characterized immunohistochemically by the presence of smooth muscle actin, muscle specific actin, and vimentin. To our knowledge, this is the first case report of glomangiomyoma on the facial location in the Korean literature.
Actins ; Adult ; Cheek ; Endothelial Cells ; Glomus Tumor ; Humans ; Muscle, Smooth ; Vimentin

No abstract available.
Heart Diseases* ; Heart*

We report a case of multiple eccrine hidrocystoma (MEH) in a 57-year-old woman who had asymptomatic, discrete, skin-colored, shiny, firm papulovesicles on the face. These lesions enlarged during summer or when the patient did housework, and decreased in sue during winter or rest. Histologic examination showed a dilated, unilocular, cystic, invaginated structure within the middle dermis. The wall of the cyst generally consisted of two layers of Rat or cuboida1 epithelial cells. Decapitation secretion and myoepithelial cells were not observed. The lesions improved markedly with 1% topical atropine sulfate.
Animals ; Atropine* ; Decapitation ; Dermis ; Epithelial Cells ; Female ; Hidrocystoma* ; Housekeeping ; Humans ; Middle Aged ; Rats

Multidirectional shoulder instability is often difficult to diagnose and treat and can be cause of significant disability. Nonoperative rehabilitations and life tyle modifications are the primary treatments. Hiwever, the inferior capsular shift procedure, performed either from an anterior or posterior approach, as described by Neer and Foster, is recommended for symptomatic multidirectional instability that is unresponsive to nonoperative therapy. Twenty-seven shoulders in twenty-seven patients with inferior and multidirectional instability were managed with Neer s inferior capsular shift, through anterior or posterior approach depending on the direction in which the shoulder is most unstable. All of the patients were followed up for an average of 3 years (range one to seven years). The postoperative range of motion of the shoulders was well maintained except 1 patient. Three patients had recurrence of symptomatic and disabling multidirectional instability, but twenty-four (89%) of the shoulders continued to function well with no instability, no pain, no recurrence and no remarkable limitation of motion.
Humans ; Range of Motion, Articular ; Recurrence ; Shoulder*

Entrapment neuropathy of the suprascapular nerve is rare and frequently overlooked in the differential diagnosis of shoulder pain. There have been few published reports on a ganglion compressing the suprascapular nerve. We experienced a case of entrapment neuropathy of the suprascapular nerve by a ganglion that compressed the inferior branch of suprascapular nerve at the spinoglenoid notch.
Diagnosis, Differential ; Ganglion Cysts ; Shoulder Pain

No abstract available.