The single enzyme P-450c17 hydroxylase catalyzes the 17a-hydroxylation of both pregnenolone and progesterone and the side-chain cleavage of 17a-hydroxypregnenolone and 17a-hydroxypro- gesterone to dehydroepiandrosterone and androstenedione. This enzyme is located in the endoplasmic reticulum and consists of a P-450c17 and a specific flavoprotein NADPH-cytochrome P-450 reductase. The clinical picture and hormonal pattern in 17a-hydroxylase deficiency have been consistent in both genotypic sexes with hypergonadotropic hypogonadism in whom the virtual absence of gonadal steroids results in a female phenotype with primary amenorrhea and pseudohermaphro- ditism in the male and underdeveloped secondary sex characteristics and hypermineralocorticoidism with hypertension, hypokalemia, suppressed renin-angiotensin system and extremely reduced aldo-sterone production. A 17-year-old girl visited endocrine clinic because of amenorrhea, absence of pubic and axillary hair, and hypertension. she had elevated levels of serum corticosterone, deoxycorticosterone(DOC), 18-hydroxycorticosterone(18-OHB). Stumulation with ACTH effected minimal increase in the elevated steroids and the ACTH-stimulated 18-OHB to aldosterone ratio was more than 280. These hormonal patterns appear to be homozygote in 17a-hydroxylase deficiency.
Adolescent ; Adrenocorticotropic Hormone ; Aldosterone ; Amenorrhea ; Androstenedione ; Corticosterone ; Dehydroepiandrosterone ; Endoplasmic Reticulum ; Female ; Flavoproteins ; Gonads ; Hair ; Homozygote ; Humans ; Hypertension ; Hypogonadism ; Hypokalemia ; Male ; Oxidoreductases ; Phenotype ; Pregnenolone ; Progesterone ; Renin-Angiotensin System ; Sex Characteristics ; Steroids

BACKGROUND: This study was performed to evaluate the association between food intake frequencies and long-term blood glucose control using glycated hemoglobin (HbA1c) in Korean diabetes. METHODS: This study used data collected from the 4th Korea National Health and Nutrition Examination Survey. Patients with diabetes were defined as people who responded "yes" to the question "Are you currently suffering from diabetes?" or in whom serum fasting glucose was 126 mg/dL or more. Using the food frequency questionnaire and HbA1c in surveys, we examined the association between the annual food-frequencies of each food group and long-term blood glucose control. RESULTS: After adjustment for other covariates, HbA1c decreased significantly as the frequency of the intake of fish increased in each population (P = 0.001 for all, P = 0.043 for men, P = 0.001 for women). The intake of mackerel played an especially important role in the control of diabetes. Among women, HbA1c decreased as the frequency of intake of legumes increased (P = 0.029) and increased as the frequency of intake of staple carbohydrates increased (P = 0.015). On the other hand, any intake of other food groups showed no significant relationship with HbA1c. CONCLUSION: Frequent intake of fish in all populations and legumes in woman were associated with good glycemic control in diabetics. Frequent intake of carbohydrates was associated with elevated HbA1c in women.
Blood Glucose ; Carbohydrates ; Eating ; Fabaceae ; Fasting ; Female ; Fishes ; Glucose ; Hand ; Hemoglobin A, Glycosylated ; Hemoglobins ; Humans ; Korea ; Male ; Nutrition Surveys ; Perciformes ; Stress, Psychological ; Surveys and Questionnaires