Virilizing adrenal adenoma and primary amenorrhea in a girl with adrenal hyperplasia.

We report a 14-year-old girl with primary amenorrhea and virilization. The chromosomal analysis showed a normal 46,XX female karyotype and the hormonal assays disclosed high serum levels of testosterone (T): 450 ng/dL (normal 5-90), dehidroepiandrosterone-sulfate (DHEA-S): 690 microg/dL (normal 30-450) and 17-hydroxiprogesterone (17-OHP) >20 ng/mL (normal <2). A pelvic ultrasound disclosed a small uterus and bilateral enlargement of the ovaries, a computed axial tomographic scan of the abdomen identified a large right mass in the adrenal gland and a norcholesterol-I 131 adrenal gammagraphy revealed a functional adrenal tumor. The histological analysis of the surgical removed tumor revealed and adrenal adenoma. After surgery, a steep decline to normal serum levels of T and DHEA-S was observed, remaining an elevated level of 17-OHP: 5.4 ng/mL. During the first three months of follow up, the hirsutism declined sharply and spontaneous mammary development occurred, remaining elevated the 17-OHP serum level: 4.8 ng/mL. Prednisone 5 mg/day, was initiated decreasing the 17-OHP to normal level: 1.4 ng/mL, appearing the menarche followed by cyclical menses. One year after surgery, prednisone was withdrawn during one week, and an ACTH test and HLA typing were done, disclosing a 17-OHP response of an heterozygote for adrenal hyperplasia, and identifying B65 a subtype of B14, and DR1, that are frequently associated to adrenal hyperplasia. Previous reports have informed silent adrenal tumors associated to adrenal hyperplasia, but this is the first report of a functional adrenal tumor associated to adrenal hyperplasia.

[Maternal and fetal morbidity in a group of women with gestational diabetes]. / Morbilidad materna y fetal en un grupo de mujeres con diabetes gestacional.

Normal pregnant women in the second and third trimester were screened to detect gestational diabetes. Using the protocol proposed by the World Health Organization, we identified 33 women whose two hr glucose levels was > 200 mg/dl. Only sixteen women had less than 34 weeks of pregnancy when were seen for the first time at the diabetes clinic, the other seventeen women had more than 34 weeks when they presented to the diabetes clinic. The first group, was called the treated group and the second group was the non-treated group. The main clinical characteristics of these patients, treated vs non-treated, were (X +/- SD): age (years) 33.2 +/- 5.2 (20-40) vs 30.2 +/- 6.5 (20-39), p < 0.05; weeks of pregnancy at diagnosis: 27.9 +/- 4.1 (19-33) vs 36.1 +/- 2.3 (34-40), p < 0.05; weight (Kg): 79.9 +/- 13.1 (61.8-108) vs 87.4 +/- 16.8 (60.8-118), p = NS; length of pregnancy (weeks) 38 +/- 1.3 (36-40) vs 38.4 +/- 1.4 (35-40), p = NS; newborns weight (g): 3,654 +/- 650 (2,475-5,100) vs 3,221 +/- 529 (2,650-4,650), p = NS. There was an intrauterine death of a macrosomic fetus in the non-treated group. There were three macrosomic newborns in the treated group and one in the non-treated group, p = NS. Also, there was a premature newborn of 1,975 g, whose pregnancy was interrupted for acute fetal distress. Delivery by cesarean section occurred in 29 women (87.8%), and it was mainly related to the diabetes diagnosis. The prevalence of macrosomia in the treated group supports the idea that treatment has to be established at least at 24 weeks of pregnancy, to reduce this rate. It is concluded that gestational diabetes is associated to an increase in maternal and fetal morbidity, requiring strict supervision to detect and treat fetal distress and a tight glucose control to decrease the macrosomia rate.

[Diabetes and pregnancy in Mexico]. / Diabetes y embarazo en México.

OBJECTIVE: To review the Mexican studies published in the last 15 years on diabetes mellitus (DM) and pregnancy. MATERIAL AND METHODS: We found five descriptive studies on DM and pregnancy and six on the detection of gestational diabetes (GDM) in normal pregnant women using the oral glucose tolerance test (OGTT). RESULTS: The first group of studies comprised 689 patients: 331 with pregestational DM type 2 and 32 type 1; the other 326 patients (47%) had GDM established by clinical symptoms and hyperglycemia, or by fasting hyperglycemia (> 140 mg/dL) in at least two ocassions. The distribution of types 1 and 2 was similar in the five studies. Maternal complications were toxemia in 18%, polyhydramnios is 10% and urinary infection in 6%. Perinatal mortality was 7%, congenital malformations in 6%, macrosomia in 25% and prematurity in 8%. In the puerperium 71 women with GDM were reevaluated: 48 (68%) were type 2, one (1%) type 1, three (4%) glucose intolerance (GI) and 19 (27%) were normal to an OGTT test. The six studies on GMD in normal pregnants were done using the OGTT. Three studies were done in the city of Monterrey, state of Nuevo Leon and show a prevalence of 4.3 to 6%, mean 5.3%. The other three studies show a prevalence of 10.5 to 11%. In the puerperium, 26 women were reevaluated: only three were type 2 (12%), five GI (19%) and 18 (69%) had a normal response to the OGTT. CONCLUSIONS: Type 2 DM is the more frequent type of diabetes associated to pregnancy in Mexican groups; the systematic screening for GDM in normal pregnant women yields 4 to 11% of positivity.

Gestational diabetes mellitus and glucose intolerance in a Mexican population.

OBJECTIVE: To determine the prevalence of gestational diabetes mellitus (GDM), glucose intolerance (GI) and macrosomia in a Latin Mexican population, using the one-step procedure proposed by the World Health Organization. METHOD: Consecutive pregnant women (n = 667) who attended the antenatal clinic and delivered at the hospital were recruited after 24 weeks of gestation, mean 34.2 weeks, range 24-40 weeks. After a minimum of 3 days with a 150-g carbohydrate diet, a glucose load of 75 g was administered and 2 h after, a blood sample for glucose was taken, plasma was separated and immediately processed for glucose, using a glucose-oxidase method. RESULTS: Twenty women were diagnosed with GDM (3%), 87 had GI (13%) and 560 had a normal glucose response (84%). Macrosomia occurred in two (10%) of GDM, in nine (10.3%) of GI, and in 29 (5.4%) gravidas with a normal response, respectively. Only one of the macrosomic infants whose mother had GI, had neonatal hypoglycemia and hyperbilirubinemia. CONCLUSION: The overall prevalence of glucose abnormalities during pregnancy was 16%. GI in this group was associated to macrosomia at the same rate as GDM.

[Adrenoleukodystrophy. Diagnosis of a typical case using magnetic resonance]. / Adrenoleucodistrofia. Diagnóstico de un caso típico por resonancia magnética.

A case of a two and a half year old boy with unexplained repeated episodes of hyponatremic dehydration and hyperkalemia, as well as noticeable psychomotor retardation and hyperpigmentation is presented. His neurologic development was considered normal until he was two, when he progressively began to regress. Magnetic resonance imaging of the brain showed white matter demyelination and cortical atrophy as well as primary adrenal insufficiency when stimulated with ACTH. Both magnetic resonance imaging and the ACTH stimulation test are useful for the diagnosis of adrenoleukodystrophy. Adrenoleukodystrophy; hyponatremic dehydration; cutaneous hyperpigmentation; white matter degenerative disease; magnetic resonance imaging.