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

Coronary spasm may be induced by a variety of physiologic and pharmacologic stimuli but specific receptor blockade has not been consistently shown to prevent the attacks. Most patients with variant angina respond well to treatment with calcium antagonists and nitrates. A small proportion of patients are refractory to this therapy. We report a case of the patient with a 9-year-history of variant angina who has been refractory to high doses of calcium antagonists and nitrates. The repeated addition of clonidine was consistently effective in abolishing both symptoms and objective evidence of myocardial ischemia in this particular patient.
Calcium ; Clonidine* ; Humans ; Myocardial Ischemia ; Nitrates ; Spasm

For the study on the effect of retinoic acid on the formation of palatal rugae and the cleft palate, retinoic acid was administered orally 150mg/kg of body weight by gastric tube at GD 10.5 to Sprague-Dawley rats. The pregnant rats were sacrificed on GD 17.5 under ether anesthesia, and laparatomized. After removal of uterus, the number of pregnant sacs and fetuses were counted. The fetuses weighed, the MEE (medial edge epithelium) thickness measured and the mitotic figures counted after routine processing and H·E stain. All the palates were photographed, and the number of rugae & the rugal pattern analysed. TEM photographs of MEE cells were observed after routine processing. The results were as follows ; 1. Rat fetus body weight after retinoic acid treatment increased significantly compared with the control group. 2. Mitotic figures in the retinoic acid treated group increased significantly compared with control group. 3. In the retinoic acid treated group, 79.3% of fetuses had cleft palates. Among fetuses with cleft palates, complete cleft palates were 10.6%, incomplete cleft palate 89.4%. Incomplete clefts were of two types ; median type (cleft palate at the intermolar region) and soft palate type (cleft posterior to the 8th rugae). Median type was 64.6% and the soft palate type 35.4%. 4. 2.3% of the fetuses had the numerical anomaly of the palatal rugae in the control group, but that of retinoic acid treated group 87.7%. 5. 17.4% of palatal rugae of the control group was disrupted, but 100% of the retinoic acid treated group disrupted. 6. Rugal papillae were observed in the 15.1% of fetuses of the control group and 63.1% of fetuses of the retinoic acid treated group. 7. Longitudinal rugae were observed in 19% of fetuses of the retinoic acid treated group, but not in the control group. 8. In TEM photographs, cytoplasmic processes, intercellular space, and desmosomes decreased. Swelling of mitochondria & ER were also found in the retinoic acid treated groups. According to the above results, it appears that there is close relationship between palatal rugae and cleft palates, and that excess retinoic acid induces disruption of pattern and numerical variations of rat fetus palate rugae. Also retinoic acid has an inhibitory effect on the proliferation of medial edge epithelial cells of palatal shelves. The cleft palates may be induced by the above mentioned retinoic acid effects. But, the exact mechanisms of retinoic acid on cleft palate formation is not thoroughly known and should be further studied.
Anesthesia ; Animals ; Body Weight ; Cleft Palate* ; Cytoplasm ; Desmosomes ; Epithelial Cells ; Ether ; Extracellular Space ; Fetus ; Mitochondria ; Palate ; Palate, Soft ; Rats ; Rats, Sprague-Dawley ; Tretinoin* ; Uterus

OBJECTIVES: The aim of this study was to evaluate the differences in patients with positional dependent sleep apnea according to their non-supine apnea-hypopnea index (AHI, > or =5 vs. <5). METHODS: 92 patients with positional sleep apnea were evaluated. The patients were divided into two groups : group I was non-supine AHI having > or =5 ; group II was non-supine AHI having less than 5. Statistical analysis was performed to find the difference between two groups. RESULTS: In 92 patients, the number of group I patients was 11 (12%) and the number of group II patients was 81 (88%). In the severe AHI group, percentage of group I was dominated (70%) and showing a significant difference compared with the mild and moderate AHI groups (p<.05). In the severe body mass index (BMI) group, percentage of group I was dominated (54.5%) and showing a significant difference compared with of the mild and moderate BMI groups (p<.05). The percentage of group I was significantly higher than group II (p<.05) in the AHI, supine AHI, non-supine AHI and snore time. CONCLUSIONS: In patients with positional sleep apnea, severe OSA and high BMI are more common in patients with non-supine AHI> or =5 than non-supine AHI<5.
Body Mass Index ; Humans ; Sleep Apnea Syndromes ; Sleep Apnea, Obstructive

No abstract available.
Hand* ; Regional Blood Flow*

Lymphoma of the prostate is exceedingly rare. Much more common than primary lymphoma of the prostate id secondary involvement of malignant lymphomas originating at other sites. A variety of treatments have bee n used, including prostatectomy, radiotherapy or combinations of chemotherapy and radiotherapy. Generally, the prognosis is very poor, and there is currently no consensus regarding treatment. We report on a patient with T-cell lymphoma who presented with symptoms of bladder neck obstruction. The patient has been asymptomatic and under complete remission after completion of doxorubicin-based chemotherapy for 2 cycles.
Bees ; Consensus ; Drug Therapy ; Drug Therapy, Combination* ; Humans ; Lymphoma ; Lymphoma, T-Cell* ; Prognosis ; Prostate* ; Prostatectomy ; Radiotherapy ; T-Lymphocytes* ; Urinary Bladder Neck Obstruction

No abstract available.
Gonadal Dysgenesis, Mixed*

Bladder neck suspension to replace the bladder neck and proximal urethra into a high fixed retropubic position is paramount in the surgical treatment of genuine stress incontinence. Thirteen patients with grade II stress incontinence and one patient with grade III one underwent the Raz bladder neck suspension. All patients had no previous anti-incontinence operation. radiation therapy and pelvic trauma. Thirteen patients(93%) were cured and one patient(7%) improved with mean Follow-up period of 26 months. One failure case was due to insufficient suspension toward high retropubic position. The complications were not significant except temporary urinary retention of 2 to 9 days. The Raz bladder neck suspension providing good mobilization of paraurethral tissues is considerable way to the treatment of all types of genuine stress incontinence except those secondary to urethral intrinsic damage.
Follow-Up Studies ; Humans ; Neck ; Urethra ; Urinary Bladder ; Urinary Incontinence* ; Urinary Retention

The aims of this study are to gain adequate treatment and prevention of injury. We have undertaken a study of 115 (185 cases) unselected Hanyang University players (foot ball, base ball, basket ball and Volley ball) from Jan. 1975 to Dec. 1977. The results were as follows: 1. The first grade showes high frequency in injury. 2. The injuries of extremities are more of ten than head and trunk. 3. The order of frequency of injury is as follows; foot ball, base ball, basket ball and volley ball. 4. Contusion is most frequent in foot ball but sprain in oters. 5. The highest incidence of players were forward in foot ball, pitcher in base ball, center in basket ball and st riker in volley ball. 6. Many injuries are developed during jumping and running. 7. Hard training and overwork showes high frequency in cause of injury. 8. The six (5.6%) patients can not return to playing.
Athletic Injuries ; Clinical Study ; Contusions ; Extremities ; Foot ; Head ; Humans ; Incidence ; Running ; Sports ; Sprains and Strains