PURPOSE:Precocious puberty is the development of secondary sexual characteristics before the age of 8 years in girls and 9.5 years in boys. It is usually associated with premature, rapid skeletal maturation and closure of the epiphyseal plates, resulting in short stature compared with genetic height potential and can produce significant psychological distress for patients. We examined effects of treatment with long-acting gonadotropin-releasing hormone(GnRH) agonist on somatic and skeletal growth in patients with central precocious puberty(CPP). MATERIALS & METHODS:Two male and seven female patients were diagnosed as having central precocious puberty(CPP) on the basis of onset age of secondary sexual characteristics, bone age, results of GnRH stimulation test and levels of sex hormones. They were treated with Triptorelin or Leuprorelin acetate(80-100ug/kg, IM every 4 weeks) for 1 year. The patients have been analyzed in terms of changes in auxological parameters including height velocity(HV), HV SDS CA, height SDS CA, height SDS BA and predicted adult height(PAH) SDS before and 1 year after treatment with GnRH agonist. RESULTS:The growth velocity a year after treatment was decreased to 4.1+/-0.9 from 7.5+/-1.2cm/year(P<0.01) and the height velocity standard deviation score(SDS) for chronologic age decreased to -1.6+/-0.4 from 2.8+/-0.8(P<0.01). The height SDS for chronologic age was increased to 2.0+/-0.7 from 3.8+/-1.0 a year after treatment (P<0.01). However, no significant difference were observed in height SDS for bone age(-1.9+/-0.2 from -2.1+/-0.3)(p>0.05) and predicted adult height SDS(-2.2+/-0.5 from -2.3+/-0.4)(p>0.05) one year after treatment. CONCLUSION: We observed a remarkable growth deceleration a year after treatment with GnRH agonist in CPP patients. However, the results of this study shows no benefit of GnRH agonist treatment in improving predicted adult height. It is still not clear whether GnRH agonist treatment will eventually help the patients with CPP achieve a final adult height within the range of their genetic target height or not. Further extensive long-term study using strict selection criteria for GnRH agonist treatment is required to address this issue.
Adolescent ; Adult ; Age of Onset ; Deceleration ; Female ; Gonadal Steroid Hormones ; Gonadotropin-Releasing Hormone* ; Growth Plate ; Humans ; Leuprolide ; Male ; Patient Selection ; Puberty ; Puberty, Precocious* ; Triptorelin Pamoate

PURPOSE: Most children who have been treated for craniopharyngioma eventually develop multiple pituitary hormone deficiencies as well as growth hormone deficiency(GHD). However, some of them may grow normally or even have accelerated growth velocity despite GHD postoperatively. This study was undertaken to evaluate several factors influencing change in growth velocity after surgery for craniopharyngioma in patients with GHD. METHODS: Fifteen patients operated on for craniopharyngioma had a pharmacological assessment of hypothalamic-pituitary function and at least two standard GH provocation tests. All patients had multiple pituitary hormone deficiencies including GHD after surgery. Patients were classified in two groups according to their growth rate during the first postoperative year. Group 1 consisted of 6 children with normal growth velocity or more than 2 standard deviation score(SDS) above the normal mean, and group 2 consisted of 9 children with decreased growth velocity more than 2 SDS below the normal mean. RESULTS: Height velocity was 8.3+/-.2 cm/year in group 1 and 2.8+/-.3 cm/year in group 2 during the first year. During the second year, height velocity was 4.4+/-.3 cm/year and 3.3+/-.4 cm/year, respectively. Body mass index(BMI) change between before and after surgery was 0.83+/-.4 kg/m2 in group 1 and 0.03+/-.3 kg/m2 in group 2 but there was no difference between both groups. However, BMI changes was correlated positively with height SDS change for 1 year following surgery in 15 patients(P<0.05, r=0.601). Prolactin levels before surgery were not significant difference between group 1 and group 2. However, there was a significant positive correlation between prolactin levels before surgery and height SDS change(P<0.01, r=0.671). Postoperative IGF-1 levels were low in all patients except one, who showed decreased growth rate. CONCLUSION: In this study, there were no significant differences in height velocity, BMI, prolactin, and IGF-1 levels between normal growth group and growth failure group after surgery. Further studies are needed to find out any other growth promoting factors related to growth without growth hormone.
Child* ; Craniopharyngioma* ; Growth Hormone* ; Humans ; Insulin ; Insulin-Like Growth Factor I ; Obesity ; Prolactin

PURPOSE: The purpose of this study was to evaluate clinical manifestation, etiology and prognosis of hypocalcemic infants who were admitted with seizure. METHODS: We reviewed medical records of 32 infants admitted at the Asan Medical Center with hypocalcemic seizure retrospectively. We classified patients into vitamin D deficiency group(n=7, 21.9%), transient hypoparathyroidism group(n=4, 12.5%), relative hypoparathyroidism with hyperphosphatemia group(n=16, 50%), and others(n=5, 15.6%) according to the laboratory results. RESULTS: Of the 32 patients, 29 patients were improved. There were no differences in gestational age and birth weight among the three groups. In the vitamin D deficiency group, age of onset was later than those of the transient hypoparathyroidism group and relative hypoparathyroidism with hyperphosphatemia group(51.6+/-2.7 vs 8.3+/-.5, 8.2+/-.6 days). In the age when all laboratory results were normalized, transient hypoparathyroidism group was younger than those of vitamin D deficiency group and relative hypoparathyroidism group(33.2+/-4.6 vs 93.6+/-8.5, 77.1+/-2.4 days). In the total treatment period, relative hypoparathyroidism with hyperphosphatemia group was longer than those of vitamin D deficiency group and transient hypoparathyroidism group(68.9+/-3.5 vs 42.0+/-5.0, 25.0+/-4.3 days). Others included two 22q11.2 deletion syndrome patients, a congenital hypoparathyroidism, a pseudohypoparathyroidism, and an early neonatal hypocalcemia. CONCLUSION: Transient hypoparathyroidism and hyperphosphatemia were major causes of neonatal hypocalcemia. And high calcitonin and peripheral organ resistance to parathyroid hormone act on hypocalcemia. In infants after one month, vitamin D deficiency was also an important cause of hypocalcemia. Most of the patients were improved within 1-2 months after proper management, but relative hypoparathyroidism with hyperphosphatemia group needed longer treatment. So, it is necessary to perform a systematic study for several complex causes that explain above fact.
Birth Weight ; Calcitonin ; Chungcheongnam-do ; DiGeorge Syndrome ; Gestational Age ; Humans ; Hyperphosphatemia ; Hypocalcemia ; Hypoparathyroidism ; Infant* ; Medical Records ; Parathyroid Hormone ; Prognosis ; Pseudohypoparathyroidism ; Retrospective Studies ; Seizures ; Vitamin D Deficiency

Genetic and clinical observations were recorded on 16 patients with xeroderma pigmentosum, 14 of them having visited the Department of Dermatology, Chonnam National University Hospital, Kwangju, Korea from 1968 through 1982, and 3 of them having visited the Department of Dermatology, Chosun University Hospital located in the same city during same period(one patient was duplicated). Xeroderma pigmentosum was found to occur with a frequency higher than one in 183,000 births in Chonnam province, Korea. It waa recognized that xeroderma pigmentosum is a disease of autosomal recessive inheritance. The sex ratio of xeroderma pigmentosum was male to female l. 7 to 1. The age of onset was between 5 months and 10 years, and the age when the patients visited the hospital for the first time was 6 months and 25 years. The skin malignancies were observed in 9 patients, among which squamous cell carcinomas occurred in 6, basal cell carcinomas in 2, and actinic keratosis in l. The associated ocular abnormalities were photophobia in 4 patients, chalazion in 1, blepharoconjunctivitis in 1, pterygium in 1 and squamous cell carcinomas in 1. Only cerebral galsy as a neurological abnormality was observed in 1 patient.
Age of Onset ; Carcinoma, Basal Cell ; Carcinoma, Squamous Cell ; Chalazion ; Dermatology ; Female ; Gwangju ; Humans ; Ichthyosis* ; Jeollanam-do ; Keratosis, Actinic ; Korea ; Male ; Parturition ; Photophobia ; Pterygium ; Sex Ratio ; Skin ; Wills ; Xeroderma Pigmentosum*

PURPOSE: This study was undertaken to identify the frequencies and the risk factors of microvascular complications in subjects with type 1 diabetes mellitus METHODS: The frequencies and their relation to risk factors of microvascular complications were analyzed in 29 type 1 diabetes mellitus subjects with duration of disease more than 5 years. Microvascular disease was defined as the presence of either retinopathy, microalbuminuria or neuropathy. RESULTS: The overall prevalence rate of microvascular disease was 8/29(27.6%). Retinopathy has developed in 3 patients(10.3%), microalbuminuria in 7 patients(24.0 %) and neuropathy in 5 patients(17.2%). The mean HbA1C was significantly higher in the patients with microvascular complications(11.6+/-.2% in microvascular complication group vs 9.3+/-.6% in control group). CONCLUSION: In childhood onset type 1 diabetes mellitus, poor glycemic control is an important risk factor for microvascular complications.
Diabetes Mellitus, Type 1* ; Humans ; Prevalence ; Risk Factors*

PURPOSE: This study was undertaken to determine whether the clinical presentation of patients with central precocius puberty(CPP) varies according to the etiology, whether this permits the differentiation between idiopathic and organic forms and to examine whether LH determination in a single timed blood sample after GnRH administration is sufficient to diagnose CPP. METHODS: This study included 33 girls with signs of breast development, of whom 23 were diagnosed as definite central precocious puberty. Sixteen patients had idiopathic CPP; the remaining 7 patients had organic CPP. Ten patients were classified as non-CPP. Potential clinical and laboratory predictors of CNS abnormalities were assessed and GnRH stimulation test was done. RESULTS: The age of onset in patients with organic CPP was 4.11+/-2.08 years, whereas the age in patients with idiopathic CPP was 7.25+/-1.34 years. This parameter is the only one showing statistical significance. We compared sensitivities and specificities at 0, 15, 30, 60, 90 and 120 min which yielded sensitivities of 8.7%, 87.0%, 91.3%, 87.0%, 73.9%, 60.9%. CONCLUSION: It is impossible to exclude a central nervous system lesion in patient with central precocious puberty without performing central nervous system imaging. However, this study indicates earlier the onset of disease, higher the possibility of presence of CNS lesion. According to the mean GnRH stimulated LH levels and sensitivity at each times, a single blood sample obtained for LH determined after GnRH administration at 30 min can be used to diagnose the central precocious puberty.
Age of Onset ; Breast ; Central Nervous System* ; Female ; Gonadotropin-Releasing Hormone* ; Humans ; Luteinizing Hormone ; Puberty, Precocious*

No abstract available.
Child* ; Drug Therapy* ; Graves Disease* ; Humans ; Receptors, Thyrotropin* ; Thyroid Function Tests* ; Thyroid Gland*

No abstract available.
Knee* ; Ligaments*

The tracheostomy is increasingly being performed. In most cases, the tracheostomy sites are left to heal by secondary intention, so it leaves a depressed and wide scar that is cosmetically disfigured. Another problem os that the scar is also attached directly to the trachea itself and will move vertically with the trachea during the act of swallowing. Even though the tracheostomy scar is cosmetically acceptable, the mobility and retraction of the scar is a continual nuisance to the patient. We performed a retrospective study on 9 patients who had undergone revision of the depressed thracheostomy scar by the Renner Method from June, 1997 to February, 1999. The method includes transverse fusiform incision of the original scar and excision of the depressed portion of the scar to the level of the trachea itself. To prevent attachment of the skin and trachea, a bilateral subcutaneous flap and muscle flap were simply advanced to the midline and overlapped. Then the rest of scar that was not depressed was deepithelized and flipped to augment the soft tissue volume in the central depressed area. Satisfactory results were achieved in all patients without hematoma, infection, hypertrophic scar, and keloid formation. We believe this simple Renner method is one of the best ways of performing posttracheostomy scar revision.
Cicatrix* ; Cicatrix, Hypertrophic ; Deglutition ; Hematoma ; Humans ; Intention ; Keloid ; Retrospective Studies ; Skin ; Trachea ; Tracheostomy

Leiomyosarcoma arising in the skin is rare tumor, and diagnosis usually is made microscopically. After local excision, these lesions recur in large proportion of pat ients. The authors herein report a 53-year-old male with leiomyosarcoma appeared in the skin of the right forearm and presenting as a dark reddish colored, 5*6cm in diameter, superficial ulcerated single firm nodule with intermittent pain. Histopathological examination showed poorly circumscribed tumor consisting of interlacing bundles of spindle shaped smooth muscle cells in the middle and lower parts of the dermis. The nuclei were hyperchromatic, large, vacuolated, and irregular in shape. Electron microscopic findings revealed cytoplasmic organelles such as rough endoplasmic reticulum and mitochondria of malignant smooth muscle cells in the paranuclear area, Characteristic subsarcoelmmal caveolae and dense plaque were noted and myofilaments were distributed in the peripheral cytoplasm. The tumor did not recur in 10 months' follow-up.
Caveolae ; Cytoplasm ; Dermis ; Diagnosis ; Endoplasmic Reticulum, Rough ; Follow-Up Studies ; Forearm ; Humans ; Leiomyosarcoma* ; Male ; Middle Aged ; Mitochondria ; Myocytes, Smooth Muscle ; Myofibrils ; Organelles ; Skin* ; Ulcer