1/1345. 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 = liver (Clic here for more details about this article) |
2/1345. Inflammatory pseudotumor of the liver and pregnancy.Inflammatory pseudotumor of the liver (IPL) is a rare, nonneoplastic entity of unknown etiology. Our patient represents the first reported case of IPL that was managed conservatively during an intrauterine pregnancy.- - - - - - - - - - ranking = 5keywords = liver (Clic here for more details about this article) |
3/1345. spinal cord injury in a fetus.In her eighth month of pregnancy a woman was stabbed in the abdomen with a barbecue fork. Upon delivery one week later, the child was noted to have two scars in the thoracic region on the back. The legs were flaccid. Surgical exploration at the age of seven months revealed marked, dense scarring of spinal cord and arachnoid membrane. No similar case was found in the literature.- - - - - - - - - - ranking = 1keywords = liver (Clic here for more details about this article) |
4/1345. osteonecrosis of the femoral head associated with pregnancy.A 31-year-old pregnant woman suddenly complained of bilateral hip pain 2 weeks before delivery. She was delivered of triplets by Caesarean section. She had been treated with human menopausal gonadotropin and human chorionic gonadotropin (hMG-hCG) before pregnancy. Radiograms of the hip joint showed collapse of the femoral heads bilaterally. magnetic resonance imaging revealed a band pattern of low signal intensity for both hips on T1- and T2-weighted images. She had no history of steroid therapy or alcohol abuse. osteonecrosis of the femoral heads bilaterally associated with pregnancy was confirmed. pathology of the femoral head showed typical empty lacunae and necrosis of the trabecula.- - - - - - - - - - ranking = 2keywords = liver (Clic here for more details about this article) |
5/1345. Normal female infants born of mothers with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency.women with congenital adrenal hyperplasia due to 21-hydroxylase deficiency, especially those patients with the salt-losing form, have decreased fertility rates. pregnancy experience in this population is limited. We report the pregnancy outcomes and serial measurements of maternal serum steroid levels in four women with classic 21-hydroxylase deficiency, three of whom were female pseudohermaphrodites with the salt-losing form. These glucocorticoid-treated women gave birth to four healthy female newborns with normal female external genitalia, none of whom were affected with 21-hydroxylase deficiency. In three women, circulating androgen levels increased during gestation, but remained within the normal range for pregnancy during glucocorticoid therapy. In the fourth patient, androgen levels were strikingly elevated during gestation despite increasing the dose of oral prednisone from 5 to 15 mg/day (two divided doses). Notwithstanding the high maternal serum concentration of androgens, however, placental aromatase activity was sufficient to prevent masculinization of the external genitalia of the female fetus and quite likely the fetal brain, consistent with the idea that placental aromatization of androgens to estrogens is the principal mechanism that protects the female fetus from the masculinizing effects of maternal hyperandrogenism. These four patients highlight key issues in the management of pregnancy in women with 21-hydroxylase deficiency, particularly the use of endocrine monitoring to assess adrenal androgen suppression in the mother, especially when the fetus is female. Recommendations for the management of pregnancy and delivery in these patients are discussed.- - - - - - - - - - ranking = 1keywords = liver (Clic here for more details about this article) |
6/1345. Development of primary hypothyroidism with the appearance of blocking-type antibody to thyrotropin receptor in Graves' disease in late pregnancy.Spontaneous remission of Graves' disease with a decrease of thyroid stimulating antibody (TSAb) activity is commonly observed in pregnancy. In this article, however, a Graves' patient who developed primary hypothyroidism with an appearance of thyroid stimulation-blocking antibody (TSBAb) activity in late pregnancy is reported. A 25-year-old woman presented with clinical and biochemical hyperthyroidism with an elevation of 99mTcO4- thyroid uptake (4.7%; normal range, 0.7%-3.0%) and mildly elevated activity of thyrotropin-binding inhibitory immunoglobulin (TBII; 30.4%). She was euthyroid with normal TBII (8.0%) and TSAb (126%) before pregnancy, when the patient was taking a 5-mg daily dose of methimazole (MMI). MMI was stopped by the patient when she became pregnant. Subsequently, the patient progressed into primary hypothyroidism with a marked elevation of TBII activity (78.4%) in the third trimester of the pregnancy (at that time, TSAb activity was not detected). TSBAb measured 2 weeks later was detected at the activity of 85.0%. Replacement therapy was initiated with levothyroxine (LT4) (0.05-0.1 mg/day), which was discontinued on the 55th day postpartum because of the onset of mild thyrotoxicosis followed by short-term euthyroid state despite high TSBAb activity. Subsequently, because the patient developed primary hypothyroidism 5 months after delivery, replacement therapy with LT4 (0.1-0.125 mg/day) was readministered. Thus, it is suggested that the development of hypothyroidism with the appearance of TSBAb in Graves' patients can occur even in late pregnancy.- - - - - - - - - - ranking = 1keywords = liver (Clic here for more details about this article) |
7/1345. Successful pregnancy and delivery in a case of systemic lupus erythematosus treated with immunoadsorption therapy and cyclosporin A.A 32-year-old woman diagnosed as systemic lupus erythematosus (SLE) became pregnant. During pregnancy she was treated with a daily dosage of prednisolone 15 mg. However, because the exanthema became worse, she was hospitalized on January 14, 1997 in order to receive immunoadsorption therapy. Before delivery we implemented the immunoadsorption therapy twice and cyclosporin A (CsA) was administered simultaneously. She gave birth in her 37th week. The baby weighed 2260 g at the time of delivery and had no deformities. The mother also had no side effects. The success of pregnancy and childbirth in our case, without any side effects, shows the possibility that the combination of CsA and immunoadsorption therapy may be considered safe to control a pregnancy complicated by SLE.- - - - - - - - - - ranking = 6keywords = liver (Clic here for more details about this article) |
8/1345. Absence of teratogenicity of oral ganciclovir used during early pregnancy in a liver transplant recipient.BACKGROUND: ganciclovir (GCV) is effective for prevention of cytomegalovirus (CMV) disease. In animals it may cause some teratogenicity. There is little information on the effect of GCV on a human fetus. methods: The chart of a liver transplant recipient who received oral GCV during the first trimester was reviewed as was the published literature. RESULTS: There was no evidence of teratogenicity in the baby or in a case reported elsewhere. CONCLUSIONS: GCV has been used in a few female transplant recipients without untoward effects. The still uncertain risk of short term and long term teratogenicity, however, must be weighed against the risk of CMV disease in the recipient and the development of congenital CMV in the baby.- - - - - - - - - - ranking = 5keywords = liver (Clic here for more details about this article) |
9/1345. Enterovesical fistula complicating pregnancy. A case report.BACKGROUND: Enterovesical fistula is a rare cause of recurrent urinary tract infections. This condition is unusual in young people as common etiologies include diverticular disease and cancer. When an enterovesical fistula occurs in women of childbearing age, Crohn's disease is a likely cause. To our knowledge, enterovesical fistula complicating pregnancy has not been reported before. CASE: A pregnant woman with recurrent urinary tract infections was evaluated. cystoscopy was suggestive of an enterovesical fistula, which was confirmed by charcoaluria following oral charcoal administration. The prenatal course was complicated by two episodes of hemorrhagic cystitis despite antibiotic prophylaxis. The patient had an uncomplicated term spontaneous vaginal delivery. An upper gastrointestinal series performed postpartum was suggestive of Crohn's disease and confirmed an enterovesical fistula. Surgical repair was successfully performed three months following delivery, revealing Crohn's disease. CONCLUSION: Enterovesical fistula may be an unusual cause of recurrent urinary tract infections in pregnancy. In this case, enterovesical fistula was the presenting symptom of Crohn's disease.- - - - - - - - - - ranking = 2keywords = liver (Clic here for more details about this article) |
10/1345. prenatal diagnosis of dyssegmental dysplasia. A case report.BACKGROUND: Since the first use of sonography, most fetal dwarfism has been detectable prenatally. The correct differentiation of the subtype of dwarfism is difficult at times. Dyssegmental dysplasia is probably an exception to these subtypes because the vertebral disorganization and occipital encephalocele at times permits prenatal diagnosis. CASE: A 34-year-old woman, gravida 3, para 1, elective abortion 1 for dwarfism, was referred at 27 weeks' gestation for cystic hygroma. Further sonographic findings included: cystic hygroma with massive ascites, micromelia, occipital encephalocele, spinal disorganization and hydramnios. The fetus and both parents appeared to have a normal karyotype. Later the pregnancy was terminated with vaginal delivery. The fetus had micromelia, camptomelia, cystic hygroma, a flat face, short neck, short trunk, narrow thorax with protuberant abdomen, scoliosis and clubfeet. CONCLUSION: Sonography is effective in prenatal diagnosis of dyssegmental dysplasia. With sonography, diagnosis of dyssegmental dysplasia becomes possible as early as the first trimester.- - - - - - - - - - ranking = 1keywords = liver (Clic here for more details about this article) |
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