No abstract available.
Hypogonadism*

No abstract available.

No abstract available.
Humans ; Infant* ; Measles*

No abstract available.

No abstract available.
Tachycardia, Supraventricular*

Seckel syndrome is a rare, autosomal recessive disorder of severe growth retardation and distinct craniofacial, orodental, and skeletal anomalies. We report hereby the first two Korean cases of typical Seckel syndrome who had characteristic symptoms of intrauterine growth retardation, small head, large eyes, sharp facial features (beaked nose, dysplastic ears and narrow face) with underdeveloped chin, dwarfism, severe mental retardation, and other malformation. We report two cases of Seckel syndrome with a brief review of related literatures.
Chin ; Dwarfism ; Ear ; Fetal Growth Retardation ; Head ; Intellectual Disability ; Nose

The use of recombinant DNA technology to produce human growth hormone has resulted in a marked increase in availability of Growth Hormone(GH) to treat short stature due to GH deficiency and other conditions, such as Turner syndrome, familial short stature, chronic renal insufficiency and intrauterine growth retardation (IUGR). But, the GH therapy may result in the adverse events such as sodium and water retention, pseudotumor cerebri, slipped capital femoral epiphysis, growth of nevi, recurrence of tumor. We experienced a case of severe hypertension associated with GH therapy in a 14-year-old male who presented high blood pressure up to 190/100 mmHg and normalized at 2-3 weeks after discontinuation of GH. Therefore, we think that the blood pressure should be carefully monitored during GH therapy.
Adolescent ; Blood Pressure ; DNA, Recombinant ; Fetal Growth Retardation ; Growth Hormone* ; Human Growth Hormone ; Humans ; Hypertension* ; Male ; Nevus ; Noonan Syndrome ; Pseudotumor Cerebri ; Recurrence ; Renal Insufficiency, Chronic ; Slipped Capital Femoral Epiphyses ; Sodium

Nowadays, to evaluate growth hormone(GH) deficiency-suspicious short stature, we usually use more than two kinds of provocative tests using various pharmacologic agents such as clonodine, L-dopa, insulin, etc. However, the importance of physiologic natural secretion of GH was recently approved. In the past, diagnosis of GH neurosecretory dysfunction was made by studying the 24-hour spantaneous GH secretion profile. But, because it is very clinically difficult, and so we measured and evaluated the mean GH concentrations of blood samples, obtained every 30 minutes during the first 3 hours' nocturnal sleep, instead of that. At the department of Pediatrics, Hanyang University Hospital from November, 1992 to February, 1994, we selected 34 GH deficiency-suspected children on the base of their growth data and bone age, etc. After GH stimulation with clonidine(100-150 ug/m2) and L-dopa(200-250 mg/m2), we measured their peak GH values by the immunoradiometric assay(IRMA) kit. And, we measured the mean GH concentrations of blood samples, obtained every 30 minutes during the first 3 hours noctumal sleep(22:OOPM-1:OOAM). RESULTS: 1. We analyzed the relationship between the mean of 3-hours' physiologic night-time surge of GH(X) and the phamacologically stimulated peak GH level(Y), except for the two neurosecretory dysfunction suspicious children. And so, for the 32 children, the relationship was Y=1.806X+ 3.177, r= 0.794, p<0.01. However, for all the tested children(34), the relationship is Y=1.709X+ 4.06, r=0.737, p<0.01. 2. We analyzed the relationship between the peak of 3-hours' physiologic night-time surge of GH(X) and the phamacologicaUy stimulated peak GH level(Y), except for the two neurosecretory dysfunction suspieious children. And so, for the 32 children, the relationship was Y=0.683X+ 3.686, r=737, p<0.01. CONCLUSION: For all the tested children(34), the relationship between the mean 3hr physiologic GH night time surge and stimulated peak GH value is Y=1.709X+4.06, r=0.737, p<0.01. The percentage of GH neurosecretory dysfunction is 5.8%(2/34). And, except for the two childrren, the relationship is more good, Y=1.806X+3.177, r=0,794, p<0.01. On the above relationship, the 3-hour GH night time-surge test might to give us some helpful information for the diagnosis of neurosecretory dysfunction.
Child* ; Diagnosis ; Growth Hormone* ; Humans ; Insulin ; Levodopa ; Pediatrics

Some sexual differentiation disorders are associated with gonadal neoplasia and increased incidence of testicular tumors has been discribed in the patients with, XY gonadal dysgenesis. The incidence of testicular tumors in infants and children are rare, representing only 1% of all pediatric solid tumors. In general, gonadal stromal tumors are one of the most characteristic endocrine tumors of the testis, endocrine activity occurs in at least 10-20%, among them Leydig cell tumors and Sertoli cell tumors are clinically important. Although the exact pathogenesis is unknown, endocrine activity due to estrogen secretion can be manifested clinically with gynecomastia or precocious puberty. We experienced and reported a child who visited for sexual precocity and had XY gonadal dysgenesis with Sertoli cell tumor.
Child ; Disorders of Sex Development ; Estrogens ; Gonadal Dysgenesis ; Gonads ; Gynecomastia ; Humans ; Incidence ; Infant ; Leydig Cell Tumor ; Male ; Puberty, Precocious ; Sertoli Cell Tumor* ; Testicular Neoplasms ; Testis

No abstract available.
Child* ; Growth Hormone* ; Humans ; Insulin* ; Insulin-Like Growth Factor I*