1/103. prenatal diagnosis of thyroid hormone resistance.A 29-yr-old woman with pituitary resistance to thyroid hormones (PRTH) was found to harbor a novel point mutation (T337A) on exon 9 of the thyroid hormone receptor beta (TRbeta) gene. She presented with symptoms and signs of hyperthyroidism and was successfully treated with 3,5,3'-triiodothyroacetic acid (TRIAC) until the onset of pregnancy. This therapy was then discontinued in order to prevent TRIAC, a compound that crosses the placental barrier, from exerting adverse effects on normal fetal development. However, as the patient showed a recurrence of thyrotoxic features after TRIAC withdrawal, we sought to verify, by means of genetic analysis and hormone measurements, whether the fetus was also affected by RTH, in order to rapidly reinstitute TRIAC therapy, which could potentially be beneficial to both the mother and fetus. At 17 weeks gestation, fetal dna was extracted from chorionic villi and was used as a template for PCR and restriction analysis together with direct sequencing of the TRbeta gene. The results indicated that the fetus was also heterozygous for the T337A mutation. Accordingly, TRIAC treatment at a dose of 2.1 mg/day was restarted at 20 weeks gestation. The mother rapidly became euthyroid, and the fetus grew normally up to 24 weeks gestation. At 29 weeks gestation mild growth retardation and fetal goiter were observed, prompting cordocentesis. Circulating fetal TSH was very high (287 mU/L) with a markedly reduced TSH bioactivity (B/I: 1.1 /- 0.4 vs 12.7 /- 1.2), while fetal FT4 concentrations were normal (8.7 pmol/L; normal values in age-matched fetuses: 5-22 pmol/L). Fetal FT3 levels were raised (7.1 pmol/L; normal values in age-matched fetuses: <4 pmol/L), as a consequence of 100% cross-reactivity of TRIAC in the FT3 assay method. To reduce the extremely high circulating TSH levels and fetal goiter, the dose of TRIAC was increased to 3.5 mg/day. To monitor the possible intrauterine hypothyroidism, another cordocentesis was performed at 33 weeks gestation, showing that TSH levels were reduced by 50% (from 287 to 144 mU/L). Furthermore, a simultaneous ultrasound examination revealed a clear reduction in fetal goiter. After this latter cordocentesis, acute complications occured, prompting delivery by cesarean section. The female neonate was critically ill, with multiple-organ failure and respiratory distress syndrome. In addition, a small goiter and biochemical features ofhypothyroidism were noted transiently and probably related to the prematurity of the infant. At present, the baby is clinically euthyroid, without goiter, and only exhibits biochemical features of RTH. In summary, although further fetal studies in cases of RTH are necessary to determine whether elevated TSH levels with a markedly reduced bioactivity are a common finding, our data suggest transient biochemical hypothyroidism in RTH during fetal development. Furthermore, we advocate prenatal diagnosis of RTH and adequate treatment of the disease in case of maternal hyperthyroidism, to avoid fetal thyrotrope hyperplasia, reduce fetal goiter, and maintain maternal euthyroidism during pregnancy.- - - - - - - - - - ranking = 1keywords = pituitary (Clic here for more details about this article) |
2/103. Histologically proven lymphocytic hypophysitis: spontaneous resolution and subsequent pregnancy.Of the 128 previously reported cases of lymphocytic hypophysitis, the diagnosis was histologically proven in 6 patients in whom the pituitary mass regressed spontaneously; only 1 subsequently became pregnant. Among six additional patients who became pregnant after a diagnosis of presumed lymphocytic hypophysitis, the disease was confirmed histologically in only three, two of whom underwent surgical debulking and one who had no follow-up imaging. To our knowledge, we describe the second patient with histologically proven lymphocytic hypophysitis, associated with adrenocorticotropic hormone (corticotropin) and prolactin deficiencies, in whom the pituitary mass regressed completely with physiologic hydrocortisone therapy only and in whom a spontaneous pregnancy occurred subsequently with no postpartum recurrence of the pituitary mass. This information lends credence to previous recommendations that, in the absence of visual field defects, surgical and corticosteroid therapy may be safely withheld, with periodic reassessment.- - - - - - - - - - ranking = 3keywords = pituitary (Clic here for more details about this article) |
3/103. Cushing's syndrome in pregnancy treated by ketoconazole: case report and review of the literature.We report on a 30-year-old female with a pituitary-dependent Cushing's disease, who refused transsphenoidal surgery and was treated with ketoconazole and cabergoline. After approximately 3 years of therapy, the patient herself decided, without the knowledge of her treating physician, to interrupt contraception. As the patient became pregnant she ceased the intake of all medication (between the third and seventh week), but resumed it soon after pregnancy was diagnosed because of relapsing clinical signs. pregnancy and vaginal delivery at 37 weeks gestation passed uneventfully. The newborn male infant did not demonstrate any congenital malformations and was normally sexually developed. With reference to this case, we discuss the difficulties in the medical treatment of Cushing's syndrome during pregnancy. Whereas outside pregnancy only efficacy and side-effects are taken into account, teratogenicity is an important question in these patients. Experience with different drugs is listed. This is only the second time that ketoconazole has been used during pregnancy for the treatment of Cushing's syndrome. We argue that ketoconazole may be safe as well as effective in pregnancy and, furthermore, without any consequences for the child.- - - - - - - - - - ranking = 1keywords = pituitary (Clic here for more details about this article) |
4/103. Acute adrenal insufficiency during pregnancy and puerperium: case report and literature review.Acute adrenal dysfunction during pregnancy is rare. Nevertheless, adrenal insufficiency can present as an adrenal crisis, and may be life threatening. There is a wide range of clinical symptoms and signs, and the differential diagnosis is challenging. A full adrenal and pituitary evaluation, both structural and hormonal, must be performed to reach the correct diagnosis, and appropriate treatment must not be delayed. A case is presented of acute adrenal insufficiency that occurred 24 hours after a cesarean delivery. The initial symptoms included hypoglycemic seizures and coma. The workup, both hormonal and structural, revealed isolated adrenocorticotrophic hormone deficiency. This considers this case and reviews the differential diagnosis, diagnostic workup, and the treatment of adrenal dysfunction in pregnancy and the puerperium, as well as the obstetric outcome in women suffering from this disorder. TARGET AUDIENCE: Obstetricians & Gynecologists, family physicians. learning OBJECTIVES: After completion of this article, the reader will be able to understand the various presentations of hypopituitarism, the various etiologies of this condition, and the appropriate work up and management of a patient with hypopituitarism.- - - - - - - - - - ranking = 7.0851108192073keywords = hypopituitarism, pituitary (Clic here for more details about this article) |
5/103. Spontaneous recovery from pathologically confirmed lymphocytic adenohypophysitis with a dramatic reduction of hypophyseal size.A pituitary mass compressing the optic nerve was revealed by magnetic resonance imaging (MRI) in a 35-year-old woman complaining of visual disturbance in the post-partum period. Responses of plasma gonadotropin and corticotropin-cortisol levels to respective hypothalamic hormones were delayed or blunted, but the response of plasma prolactin to thyrotropin-releasing hormone was exaggerated. diabetes insipidus was not associated. biopsy revealed lymphocytic adenohypophysitis, and no hypophysectomy was performed. Only five weeks later, the pituitary mass spontaneously disappeared on MRI. The pituitary function was normalized. Anti-thyroidal and anti-pituitary antibodies were negative throughout the clinical course. Pituitary masses developing during late pregnancy or the post-partum period should be carefully observed.- - - - - - - - - - ranking = 4keywords = pituitary (Clic here for more details about this article) |
6/103. Lymphocytic adenohypophysitis mimicking a pituitary macroadenoma.A 24-year-old woman developed headache and rapidly progressive visual disturbances during the last trimester of her first pregnancy. Magnetic resonance tomography (MRI) of the brain documented an intra- and suprasellar mass lesion. For preservation of vision, transsphenoidal microsurgical decompression was performed. Immediately postoperatively, visual acuity improved and hemianopia resolved. Histological examination yielded the diagnosis of primary lymphocytic adenohypophysitis. This is a rare inflammatory pituitary disease. There are no typical clinical, laboratory, or radiological findings that allow precise preoperative diagnosis. Even though this autoimmune disorder is principally steroid-responsive, an improvement of visual disturbances under steroid therapy cannot be predicted. Therefore, surgery is justified not only to establish the diagnosis, but also to restore vision by decompression of the optic nerves and the chiasm.- - - - - - - - - - ranking = 5keywords = pituitary (Clic here for more details about this article) |
7/103. Placental growth hormone and IGF-I in a pregnant woman with Pit-1 deficiency.The respective contributions of pituitary and placental GH to circulating IGF-I in pregnant women have not been well established. We measured the serum concentrations of placental growth hormone (PGH) and IGF-I in a woman with pit-1 deficiency before, during and after pregnancy, resulting in the birth of a healthy child (not pit-1 deficient). Both PGH and IGF-I concentrations were below the assay detection limit before and after pregnancy. During pregnancy, PGH and IGF-I levels increased steadily; the concentrations of PGH and IGF-I in late pregnancy were comparable with levels previously measured in normal pregnancies. PGH and IGF-I concentrations were strongly correlated throughout pregnancy (r = 0.90; P = 0.002). PGH was undetectable in cord serum, whilst the IGF-I concentration was within the normal range. The findings of this case study corroborate the notion that PGH is the prime regulator of maternal serum IGF-I during pregnancy.- - - - - - - - - - ranking = 1keywords = pituitary (Clic here for more details about this article) |
8/103. Intra-uterine treatment of a hypothyroid fetus.A woman in her 24th week of gestation was referred for treatment of hypothyroidism, after she underwent radioablation of the thyroid during the 13th week of gestation. Because of the high risk of hypothyroidism in the fetus, prenatal administration of intramuscular T-4 to the fetus was begun at 32 weeks. The last dose of T-4 was given 2 weeks before delivery; cord blood levels of T-4 and T-3 were undetectable and the TSH concentration was markedly elevated. The case illustrates several important physiological concepts regarding thyroid hormone and TSH metabolism in the fetal-placental unit, including the minimal placental permeability to iodothyronines and TSH, independent function (including feedback control) of the fetal hypothalamic-pituitary-thyroid axis, and the TSH response at parturition. In addition we suggest that administration of T-4 to the hypothyroid fetus in utero is an acceptable modality of treatment and may help to minimize irreversible mental retardation in known high risk infants. However, further studies are necessary to assess the effectiveness and safety of this approach.- - - - - - - - - - ranking = 1keywords = pituitary (Clic here for more details about this article) |
9/103. Recurrent ACTH-independent Cushing's syndrome in multiple pregnancies and its treatment with metyrapone.A 17-year-old primigravid woman presented with Cushing's syndrome. Typical clinical symptoms and signs developed at the beginning of pregnancy. By week 17 of gestation, plasma cortisol diurnal rhythm was absent and there was a paradoxical increase in plasma cortisol after a 1-mg dexamethasone overnight suppression test. Basal urinary free cortisol was 10 times above the upper limit (in pregnancy) and ACTH levels were suppressed. The diagnosis of ACTH--independent Cushing's syndrome was established. MRI scans revealed normal adrenal and pituitary glands. To control hypercortisolism, the patient was treated with metyrapone. At 34 weeks of gestation, the patient developed preeclampsia and underwent caesarean section. A female infant weighing 1070 g was delivered. No apparent metyrapone-induced teratogenic effects were observed. Cushing's syndrome in the patient resolved within three weeks of delivery. No corticosteroid replacement therapy either for child or mother was needed. Eight months after delivery the patient became pregnant again and rapidly developed Cushing's syndrome with typical clinical symptoms and signs and laboratory results (urinary free cortisol 6464 nmol/24 h). This second pregnancy was unwanted and terminated by artificial abortion that was followed by rapid resolution of hypercortisolism. A third pregnancy, 12 months after delivery was also accompanied by the rapid development of hypercortisolism which recovered after artificial termination. The mechanisms by which pregnancy-induced Cushing's syndrome occurred in this patient are unclear. Aberrant responsiveness or hyperresponsiveness of adrenocortical cells to a non-ACTH and non-CRH substance produced in excess in pregnancy should be considered. metyrapone suppression of hypercortisolism currently represents the best treatment for these rare cases.- - - - - - - - - - ranking = 1keywords = pituitary (Clic here for more details about this article) |
10/103. Short-term hyperthyroidism followed by transient pituitary hypothyroidism in a very low birth weight infant born to a mother with uncontrolled Graves' disease.Transient hypothyroxinemia in infants born to mothers with poorly controlled Graves' disease was first reported in 1988. We report that short-term hyperthyroidism followed by hypothyroidism with low basal thyroid-stimulating hormone (TSH) levels developed in a very low birth weight infant born at 27 weeks of gestation to a noncompliant mother with thyrotoxicosis attributable to Graves' disease. We performed serial thyrotropin-releasing hormone (TRH) tests in this infant and demonstrated that TSH unresponsiveness to TRH disappeared at 6.5 months of age. The maternal thyroid function was free triiodothyronine (FT(3)), 21.1 pg/mL; free thyroxine (FT(4)), 8.1 ng/dL; TSH, <0.03 microU/mL; thyroid-stimulating hormone receptor antibody, 52% (normal: <15%); thyroid-stimulating antibody, 294% (normal: <180%); and thyroid-stimulation blocking antibody, 9% (normal: <25%) on the day of delivery. A nonstress test revealed fetal tachycardia >200 beats per minute, and a male infant weighing 1152 g was born by emergency cesarean section. Thyroid-stimulating hormone receptor antibody was 16% and thyroid-stimulating antibody was 370% in the cord blood. We administered 10 mg/kg per day of oral propylthiouracil from day 1. tachycardia along with elevated FT(4) and FT(3) levels in the infant decreased from 200/minute to 170/minute, 4.7 ng/dL to 2.9 ng/dL, 7.0 pg/mL to 4.8 pg/mL, respectively, in the first 33 hours. At 5 days, FT(4) and FT(3) were 1.1 ng/dL and 2.9 pg/mL, respectively, and we stopped propylthiouracil administration. Although FT(4) decreased to 0.4 ng/dL, TSH was quite low and did not respond to intravenous TRH by 14 days of age. We began daily levothyroxine 5-micro/kg supplementation. The responsiveness of TSH to TRH did not become significant until 4 months old and normalized at 6.5 months old. At this time, levothyroxine was stopped. We conclude that placental transfer of thyroid hormones may cause hyperthyroidism in the fetal and early neonatal periods and lead to transient pituitary hypothyroidism in an infant born to a mother with uncontrolled Graves' disease.- - - - - - - - - - ranking = 5keywords = pituitary (Clic here for more details about this article) |
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