No abstract available.
Axis, Cervical Vertebra*

PURPOSE:Bone mineral mass increases with age, weight and pubertal development. Several factors influence the process of bone mineralization, and evaluation of bone mineral density(BMD) in children gives important clue to the different mechanisms of bone mass accumulation. To investigate the abnormality in bone mineralization, the normal range of bone mineral content in healthy children should be understood. The pattern, time and velocity in the decrease of bone mineral contents depend on disease entities. Because the ratio of trabecular and cortical bones and turn-over rates differ in each bone, it is needed to make normal data for trabecular and cortical bones respectively in children. METHODS:In 75 children(40 boys) with normal growth and development, BMD was measured with XR26 Bone densitometry using DEXA. The BMD of trabecular bone was obtained at lumbar spine at 2-4 level, and that of cortical bone was measured at femur neck. RESULTS:The BMD of trabecular bone increased with age without sexual difference. But the BMD of cortical bone was higher in male significantly at 8-9 and 10-11 yr of age. In both male and female, the BMD of trabecular and cortical bones correlated positively with age, bone age, height, weight, and body surface area. CONCLUSION: With this data, the changes in BMD affected by several diseases could be assessed.
Body Surface Area ; Bone Density* ; Calcification, Physiologic ; Child* ; Densitometry ; Female ; Femur Neck ; Growth and Development* ; Humans ; Male ; Reference Values ; Spine

We experienced a boy manifesting sexual precocity with unilateral testicular tumor, who was finally diagnosed as CAH with 21-hydroxylase deficiency. Initial laboratory findings were compatible with peripheral precocious puberty. Ultrasonogram for testes showed heterogenous high echoic mass in the right testicle. Radical orchiectomy was performed and its pathology revealed benign Leydig cell tumor without Reinke crystal. But, testosterone was not decreased after 1 month of surgery. Second laboratory exam revealed increased DHEA-S and 17-hydroxyprogesterone. Finally, this case was recognized as CAH(simple virilizing type) with unilateral testicular adrenal rest tumor. In conclusion, CAH should always be considered during etiologic study for the male sexulal precocity even with unilateral testicular tumor.
17-alpha-Hydroxyprogesterone ; Adrenal Hyperplasia, Congenital ; Adrenal Rest Tumor ; Humans ; Leydig Cell Tumor* ; Male ; Orchiectomy ; Pathology ; Puberty, Precocious ; Steroid 21-Hydroxylase ; Testis ; Testosterone ; Ultrasonography

Central nervous system impairment is common in diabetic patients, even in the early stages of the disease, and could be associated with peripheral neuropathy. The aims of this study were to prospectively investigate central nerve conduction in young adults with type 1 diabetes using pattern-reversal visual evoked potentials (PRVEP) and to determine how those results were related to clinical risk factors and the parameters of the peripheral nerve conduction study (NCS). A total of 36 type 1 diabetic patients (15 males) 5-24 years of age (mean 14.5 ± 4.7) underwent PRVEP and NCS annually for five years. For comparison, 39 healthy age and sex matched individuals (mean 14.8 ± 5.0) were evaluated as the control group. The P100 latencies of the PRVEP were prolonged at the study entry in the patients compared with the controls (p< 0.001). Significant correlations were not found between any of the parameters of PRVEP and the glycosylated hemoglobin levels; however, the changes in the parameters of the peripheral NCS were well correlated with metabolic control. The latencies and amplitudes of the P100 were not related to the majority of the parameters of the NCS. A prolonged PRVEP latency may be a sign of optic pathway dysfunction, which begins before apparent diabetic retinopathy. Poor glycemic control proved to be an important risk factor over the 5 years in terms of its relation to the development of peripheral neural pathway abnormalities. However, once central conduction was delayed, its changes were poorly related to diabetic control and the attributes of the peripheral nerve conduction study over the 5-year follow-up.
Diabetes Mellitus

We experienced two cases of congenital adrenal hyperplasia(CAH), complicated by true precocious puberty with early maturation of the hypothalamic-pituitary-gonadal axis in a boy and a girl. The boy was diagnosed as CAH at 8 years of age, when he showed pseudoprecocity with adrenal rest tumor. The girl was diagnosed at neonate, when she showed ambiguous genitalia and salt-losing symptom. They developed precocious puberty during steroid treatment. Clinically they showed precocity, advanced bone age, and decreased predicted adult height, even though they showed partial gonadotrophin response to luteinizing hormone releasing hormone (LHRH) stimulation test. They were treated with LHRH analogue(leuprolide depot) resulting no further progression of precocity.
Adrenal Hyperplasia, Congenital* ; Adrenal Rest Tumor ; Adult ; Axis, Cervical Vertebra ; Disorders of Sex Development ; Female ; Gonadotropin-Releasing Hormone ; Humans ; Infant, Newborn ; Male ; Puberty, Precocious*

No abstract available.
Child* ; Ear* ; Humans

PURPOSE:Effects of IDDM on bone mineral metabolism are still in controversy. Some reported that bone mineral density in IDDM had inverse relationship with HbA1c, some reported that spine BMD was normal while femur BMD was decreased. Others reported that increased urinary calcium excretion in IDDM induced early trabecular bone mineral loss. We studied the correlation of BMD with diabetic control and body measurements. METHODS:In sixteen IDDM patients, using dual energy X-ray absorptiometry, BMD was measured in lumbar spine as trabecular bone and femur neck as cortical bone. Z-score of BMD was obtained by comparing age and sex matched control data. Correlations between BMD and diabetic control parameters (HbA1c, duration of IDDM) and body measurements were calculated. RESULTS:The body measurements were in normal range in all IDDM patients, the duration of IDDM was 38.4+/-24.0months, HbA1c was in good control state (7.69+/-1.53%), and urinary Ca/creatinine ratio was not increased. The Z-score of BMD was not decreased statistically (lumbar spine: -0.255, femur neck: -0.404), and the Z-score had no correlationship with body measurements and diabetic control parameters. CONCLUSIONS:In well controlled childhood IDDM, BMD was not decreased significantly.
Absorptiometry, Photon ; Bone Density* ; Calcium ; Diabetes Mellitus, Type 1* ; Femur ; Femur Neck ; Humans ; Metabolism ; Reference Values ; Spine

A 14-year old boy presented with myoclonic seizure with rightward deviation of eyeballs. Three years ago, he was diagnosed as diabetes necessitating insulin injection. At that time, his blood glucose was 448mg/dl, HbA1c 27.8%, serum C-peptide rose from 0.4 to 1.1ng/ml after glucagon, and 24 hour urine C-peptide was 6.7microg/day. Eye examination was normal. His maternal grandmother died of diabetes at 50 years old, and his mother's sister and his elder sister had NIDDM with oral hypoglycemics. But, he didn't control hyperglycemia himself since that time. On physical exam, his grasping power was decreased in right hand, and cataract was detected at the posterior pole of lenses in both eyes requiring surgery. EEG showed partial seizure disorder in left frontoparietal area, and MRI revealed cerebral infarction in left frontoparietal cortex. Sensory-motor polyneuropathy was noted in nerve conduction velocity. His neurologic symptom was improved gradually with insulin therapy, but nerve conduction velocity and MRI abnormalities did not improved after 6 months of follow-up. Although long-term diabetic complication is common in poorly controlled diabetes, very early manifested eye and nervous system complications like this case is extremely uncommon.
Adolescent ; Blood Glucose ; C-Peptide ; Cataract ; Cerebral Infarction ; Diabetes Complications ; Diabetes Mellitus, Type 2 ; Electroencephalography ; Epilepsies, Partial ; Follow-Up Studies ; Glucagon ; Hand ; Hand Strength ; Humans ; Hyperglycemia ; Hypoglycemic Agents ; Insulin ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Nervous System ; Neural Conduction ; Neurologic Manifestations ; Polyneuropathies ; Seizures ; Siblings

No abstract available.
Congenital Hypothyroidism*

PURPOSE:The natural course of Hashimoto' thyroiditis (HT) is so dynamic that the disease progresses to overt hypothyroid or spontaneous recovery. The authors reviewed the clinical course of this disease and analysed the possible predicting factors regarding remission. METHODS:Thirty nine patients with HT (38 girls and 1 boy) were studied retrospectively. Of these patients, 30 were followed for more than 2 years. The possible remission factors were analyzed at initial diagnosis and during follow-up period. RESULTS:The mean age at the diagnosis was 11.8+/-.1 years. Initial thyroid function was euthyroid in 38.5%, compensated hypothyroid in 35.9%, overt hypothyroid in 23.1%, and hyperthyroid in 2.6% of patients. Antithyroglobulin antibody (ATA) was positive in 94.7%, and antimicrosomal antibody (AMA) was positive in 74.4%. The overall remission rate was 53.3% during the follow-up period (51+/-7 months). Initial goiter size, thyroid function status, and autoantibody titer had no relation to the remission rate statistically. Follow-up autoantibody titers in remission group were marginally lower than those in nonremission group (P<0.1), and follow-up AMA titer was significantly higher than initial titers in nonremission group (P<0.05). CONCLUSION: We could not find any predictable remission factors from the initial clinical and autoantibody status. But, during follow-up period, patients with lower autoantibody titers showed slight higher remission, and those with increasing AMA titer showed less remission. Above results suggest that we should monitor antithyroid antibody titer as well as thyroid function regularly.
Adolescent* ; Child* ; Diagnosis ; Female ; Follow-Up Studies ; Goiter ; Humans ; Retrospective Studies ; Thyroid Gland* ; Thyroiditis*