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 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

While fifty years ago 20 p. 100 of cystic fibrosis patients only reached the age of one year, more than 50 p. 100 of the patients now live more than twenty years. The clinical manifestations of cystic fibrosis are more diverse in adults than in children, so that the diagnosis might concern several specialties. In actual fact, only 3 to 7 p. 100 of cystic fibroses are diagnosed after thirteen to sixteen years, and in half the cases the symptoms had been present before the age of one year. In adults, the respiratory manifestations of cystic fibrosis are predominant, whereas the gastrointestinal manifestations tend to be blurred. Radiography of the chest shows interstitial lesions (opacities, cystic images, disorders of ventilation), principally located in the right side and the apex. The most common functional defect is an obstructive syndrome corresponding to a gradual involvement of the peripheral airways. A number of complications may develop, including recurrent Pseudomonas infection of the lung, pneumothorax, heart failure, malnutrition, liver cirrhosis, episodes of intestinal occlusion, etc. The longer life span of these patients raises the problems of diabetes with its vascular complications, infertility or pregnancy, social and professional insertion, and so forth. The prognosis of cystic fibrosis in adults depends on the date the diagnosis was made, on the therapeutic follow-up and on the creation of specialized centres. The control of Pseudomonas infections and the development of lung transplantation are the main advances to be expected.
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PMID:[Cystic fibrosis in adults]. 236 14

Thirty-two women, aged 21 to 44 years, who had undergone single (25) or multiple (7) heart valve replacement conceived 46 times and gave rise to 33 live-born infants. There were 12 abortions and one stillborn; a premature baby died 24 hr post-partum. Twenty-eight patients had mechanical prostheses and 4 had porcine xenografts, 29 patients being anticoagulated with acenocoumarol during the pregnancy. Cardiac status remained clinically satisfactory under medical treatment in all but one patient who developed heart failure. Fetal complications included cerebral hemorrhage in 1 premature infant, low birth weight in 3 newborns and 1 case of nasal hypoplasia. The incidence of abortion has decreased significantly during the latter part of our experience. It was significantly greater in patients with mitral prostheses. This review suggests that inadequate cardiac function, excessive anticoagulation and a history of primary infertility may increase the risk of abortion. Better control of anticoagulant therapy (accepting an elevation of the therapeutic limit) and improved prevention of pregnancy in high-risk patients are considered responsible for the improved results seen in recent years.
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PMID:Pregnancy after heart valve replacement. 674 26

To accurately evaluate thyroid disorders in pregnancy, the physician must understand the physiologic changes that occur both in thyroid gland size and in thyroid function tests. The effect of thyrotoxicosis on pregnancy outcome largely depends on whether metabolic control is achieved. Women who become euthyroid on treatment usually can expect satisfactory outcomes. Propylthiouracil is considered to be the drug of choice for treating thyrotoxicosis during pregnancy. Because of the significant risk of hypothyroidism and obvious goiter in the infant, the use of iodide should be reserved for severe disease, such as thyroid storm or heart failure. Thyrotoxic infants may need antithyroid treatment until TSAbs are metabolized. Since overt hypothyroidism is often associated with infertility, it is uncommon in pregnancy. Hypothyroid women who do become pregnant, however, have an increased risk of low-birth-weight or stillborn infants. These women may require a greater dosage of thyroid hormone during pregnancy. The effects of subclinical hypothyroidism are not well defined. Accordingly, the need for treatment hinges on the woman's clinical history. Infants of hypothyroid mothers usually show no evidence of thyroid dysfunction, but those who are hypothyroid should receive prompt thyroid replacement therapy. To minimize the sequelae of congenital hypothyroidism, mass screening of infants and prompt treatment of those affected is recommended. During pregnancy, thyroid nodules should be evaluated by ultrasound and fine-needle aspiration or tissue biopsy. Radioiodine scanning should be avoided during pregnancy. If thyroid cancer is diagnosed, pregnancy should not delay treatment. Because postpartum thyroid dysfunction is fairly common yet difficult to detect, physicians and patients should be aware of the symptoms and risk factors.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Thyroid disease in pregnancy. ACOG Technical Bulletin Number 181--June 1993. 790 64

Obesity is associated with the development of some of the most prevalent diseases of modern society. The greatest risk is for diabetes mellitus where a body mass index above 35 kg/m2 increases the risk by 93-fold in women and by 42-fold in men. The risk of coronary heart disease is increased 86% by a 20% rise in weight in males, whereas in obese women the risk is increased 3.6-fold. Elevation of blood pressure, hyperlipidaemia and altered haemostatic factors are implicated in this high risk from coronary heart disease. Gallbladder disease is increased 2.7-fold with an enhanced cancer risk especially for colorectal cancer in males and cancer of the endometrium and biliary passages in females. Endocrine changes are associated with metabolic diseases and infertility, and respiratory problems result in sleep apnoea, hypoventilation, arrhythmias and eventual cardiac failure. Obesity is not a social stigma but an actual disease with a major genetic component to its aetiology and a financial cost estimated at $69 billion for the USA alone.
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PMID:Obesity as a disease. 924 38

Eating disorders are serious illnesses affecting 1-2% of young women. Patients may present to any doctor, sometimes atypically (e.g. unexplained weight loss, food allergy, infertility, diarrhoea), delaying diagnosis and leading to needless investigation. The cardinal signs are weight loss, amenorrhoea, bingeing with vomiting and other compensatory behaviours, and disturbances in body image with an exaggeration of the importance of slimness. When other causes have been excluded, useful investigations are serum potassium, bone mineral density scanning and pelvic ultrasound. In emaciated patients multiple systems may fail with pancytopaenia, neuromyopathy and heart failure. Clinical assessment of muscle power is used to monitor physical risk. Treatment may involve individual, group or family sessions, using cognitive-behavioural, psychodynamic and family approaches. More severe or intractable illness is treated with day care, with in-patient care in a medical or specialist psychiatric unit reserved for the most severely ill patients. Antidepressants have a place in the treatment of bulimia nervosa unresponsive to psychological approaches, and when severe depressive symptoms develop. The children of people with eating disorders may have an increased risk of difficulties. Support for the patient and family, and effective liaison between professionals, are essential in the treatment of severe eating disorders.
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PMID:Review article: recognition and treatment of eating disorders in primary and secondary care. 1075 15

Research on different types of stem cells is of major interest because of its apparent very promising therapeutic prospects, such as for Parkinson's and Alzheimer's disease, spinal cord injuries, stroke, diabetes, cardiac failure, liver failure, cartilage injuries, severe blood diseases, cancer etc. Stem cells can be derived from different sources: adult tissue, foetal tissues, and from in vitro fertilised embryos. Depending on their origin they have varying capacity to multiply and differentiate to other cell types. It is at present not possible to predict which types of cells will be best suitable for various therapeutic situations. Embryonic stem cells have been shown capable of differentiating into all the different tissues and cell types of the body, but they cannot form a new individual. Because of the ethics question involved, The European Group on Ethics on Science and New Technologies for the European Commission and Parliament (EGE), and the Ethics Committee of the Nordic Council of Ministers have prepared reports and given guidelines for research on stem cells. According to the guidelines, every country should regulate the research. Only embryos, which cannot be used in infertility treatment, and have been donated for research, can be used. Creation of embryos solely for research purposes, including somatic cell nuclear transfer, is not regarded as acceptable for the time being. Both partners of the donating couple have to sign an informed consent document. Ongoing research in Sweden is well in line with these European and Nordic recommendations.
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PMID:[Ethical aspects of stem cell research. Legislation and guidelines in Europe]. 1157 92

Stem cells are undifferentiated cells, with the ability to self renew and to differentiate into specialised cells. Embryonic stem cells (ES) have proven to be truly pluripotent, since they are able to give derivatives of the three primitive embryonic layers. Human ES have a normal karyotype, maintain high telomerase activity, and exhibit remarkable long-term proliferative potential, providing the possibility for unlimited expansion in culture. Though human ES cell-based transplantation therapy holds great promises to successfully treat a variety of diseases (e.g., Parkinson's disease, diabetes, and heart failure) many barriers remain in the way of successful clinical trials. Less spectacular, the applications in the field of reproductive biology are also outstanding: stem cell biology will lead us to a better understanding of the cellular and molecular mechanisms of events such as infertility, failure of implantation, genomic imprinting and meiosis. The obstetrician and gynaecologist could act an important part in the production and study of embryonic stem cells. However, these data have to be integrated in the ethical and juridical background of embryonic stem cell research in France.
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PMID:[Embryonic stem cells: a position for the obstetrician and gynaecologist. Part one]. 1550 Nov 64

VATER "association" is a common condition, with the diagnosis typically based on a characteristic constellation of congenital anomalies. Reported long-term follow-up information on VATER association is limited, thus making it difficult to prognosticate the future of infants and children with this condition. Further, there are few data on how often the initial diagnosis of VATER association is correct. Some information has been published on growth deficiency and mental retardation, but these data are minimal [Bull et al., 1985; Mapstone et al., 1986; Weaver et al., 1986] and for the most part look at children under the age of 10 years. We have undertaken a long-term follow-up of individuals with VATER association originally reported by Weaver et al. [1986] or diagnosed with VATER association by his associates and him after 1986. Out of the 50 patients, we were able to contact 20 individuals or families. Two of the 20 individuals had died: 1 at 3 days with cardiac failure due to a truncus arteriosus, and 1 at 4 years of unspecified cause. Two were unwilling to participate. Of the rest, we interviewed and examined seven persons, and interviewed another nine by telephone. Of the 16, 5 had some degree of cognitive impairment. These individuals were more likely to have congenital anomalies outside of the typical scope of VATER association, such as prune belly sequence or findings of CHARGE association. Of the nine individuals with a history of imperforate anus, five had partial or complete incontinence as adults leading to difficulties in maintaining employment. Height was at the 5th centile or less in 6 of 16 patients. Three of four patients who were trying to have children, had infertility. In two women, the infertility was thought to be related to congenital anomalies of the genitourinary system and multiple pelvic operations. We also present the long-term medical and neurologic problems in these individuals.
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PMID:Adults with VATER association: long-term prognosis. 1615 41

Beta-thalassemia major is a severe, transfusion-dependent anemia that also causes infertility due to iron deposition to endocrine organs after overtransfusion. Very few pregnancies have been reported among such patients after modern therapies. In this study, 32 women with thalassemia who were admitted to Ali Asghar Children's Hospital and Thalassemia Clinic conceived spontaneously following prolonged intensive treatment with hypertransfusion and iron chelation. The aim of this study was to estimate the fertility (spontaneous ovulation or induced ovulation) and pregnancy complications for mothers and newborns. These complications included cardiac failure, endocrine and hepatic parameters monitored throughout pregnancy and postpartum, viral infections, term and preterm deliveries, and complications of pregnancy. All case notes were examined and data were analyzed with SPSS software. Twelve babies were delivered by elective cesarean section and the remainder were delivered vaginally. The mean birthweight was 2678 g. All babies were normal; 45 cases were mature and 5 were preterm; 12 cases were aborted spontaneously. Twenty-seven mothers had no cardiac problems, but 5 had cardiac failure. Pregnancy can be safe for mothers and babies in women started early on intensive treatment.
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PMID:Pregnancy in patients treated for beta thalassemia major in two centers (Ali Asghar Children's Hospital and Thalassemia Clinic): outcome for mothers and newborn infants. 1632 10

Information on pregnancy and delivery in women with biventricular repair for isolated noncomplex pulmonary atresia with a ventricular septal defect (PAVSD) is limited. Using a nationwide congenital heart disease registry (CONgenital CORvitia [CONCOR]), 9 women with biventricular repair for PAVSD (aged 21 to 38 years) were identified. Ten pregnancies were observed in 5 different women with PAVSD, including 3 spontaneous miscarriages. Clinically significant (non)cardiac complications were documented in 3 of 7 completed pregnancies. These complications were: (1) atrioventricular reentry tachycardia with symptomatic right-sided heart failure; (2) eclampsia with hemolysis, elevated liver enzymes, and low platelets syndrome further complicated by abruptio placentae leading to premature delivery of a small-for-gestational-age child; and (3) premature delivery due to cervical insufficiency with antepartum demise of an immature child. Furthermore, none of the women reported infertility. Moreover, none of the women reported irregularities of their natural menstrual cycle (age at menarche 13 years; cycle duration 28 days), with the exception of delayed menarche (>16 years) in 2 patients. In conclusion, successful pregnancy in patients with biventricular repair of PAVSD is possible, although often complicated by serious clinically significant events. Infertility and menstrual cycle disorders do not appear to be more prevalent, except for a high incidence of primary amenorrhea.
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PMID:Pregnancy after biventricular repair for pulmonary atresia with ventricular septal defect. 1682 5


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