1/151. Cerebral malformation associated with metabolic disorder. A report of 2 cases.A clinical and neuropathological study is presented of two cases each of which showed neuronal heterotopia. Microgyria was also present in one case. One patient was suffering from a degenerative disorder affecting the white matter. The other was a case of Menkes' disease. It is suggested that the antenatal damage may have been caused by an imbalance of the maternal metabolism, the predisposing factor being the mother's carrier state for a metabolic defect. This is the first report of teratogenesis in a case of Menkes' disease. It is also noted that in this case there is interference with the postnatal as well as the antenatal development of the brain.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
2/151. Anaesthetic management of a woman who became paraplegic at 22 weeks' gestation after a spontaneous spinal cord haemorrhage secondary to a presumed arteriovenous malformation.A 19-yr-old woman developed a paraplegia with a T10 sensory level at 22 weeks' gestation. The spinal injury was caused by spontaneous bleed of a presumed arteriovenous malformation in the spinal cord. She presented for Caesarean section at term because of the breech position of her fetus. The successful use of a combined spinal epidural-regional anaesthetic is described and the risks of general and regional anaesthesia are discussed.- - - - - - - - - - ranking = 1.25keywords = malformation (Clic here for more details about this article) |
3/151. Treating allergic rhinitis in pregnancy. safety considerations.Allergic rhinitis affects approximately one-third of women of childbearing age. As a result, symptoms ranging from sneezing and itching to severe nasal obstruction may require pharmacotherapy. However, product labels state that medications for allergic rhinitis should be avoided during pregnancy due to lack of fetal safety data, even though the majority of the agents have human data which refute these notions. We present a systematic and critical review of the medical literature on the use of pharmacotherapy for the management of allergic rhinitis during pregnancy. Electronic databases and other literature sources were searched to identify observational controlled studies focusing on the rate of fetal malformations in pregnant women exposed to agents used to treat allergic rhinitis and related diseases compared with controls. immunotherapy and intranasal sodium cromoglycate (cromolyn) and beclo-methasone would be considered as first-line therapy, both because of their lack of association with congenital abnormalities and their superior efficacy to other agents. First-generation (e.g. chlorpheniramine) and second-generation (e.g. cetirizine) antihistamines have not been incriminated as human teratogens. However, first-generation antihistamines are favoured over their second generation counterparts based on their longevity, leading to more conclusive evidence of safety. There are no controlled trials with loratadine and fexofenadine in human pregnancy. Oral, intranasal and ophthalmic decongestants (e.g. pseudoephedrine, phenylephrine and oxymetazoline, respectively) should be considered as second-line therapy, although further studies are needed to clarify their fetal safety. No human reproductive studies have been reported with the ophthalmic antihistamines ketorolac and levocabastine, although preliminary data reported suggest no association between pheniramine and congenital malformations. There are no documented epidemiological studies with intranasal corticosteroids (e.g. budesonide, fluticasone propionate, mometasone) during pregnancy; however, inhaled corticosteroids (e.g. beclomethasone) have not been incriminated as teratogens and are commonly used by pregnant women who have asthma. In summary, women with allergic rhinitis during pregnancy can be treated with a number of pharmacological agents without concern of untoward effects on their unborn child. Although the choice of agents in part should be based on evidence of fetal safety, issue of efficacy needs to be addressed in order to optimally manage this condition.- - - - - - - - - - ranking = 0.5keywords = malformation (Clic here for more details about this article) |
4/151. Atrioventricular septal defect with separate right and left atrioventricular valvar orifices in a patient with foetal hydantoin syndrome.The teratogenic properties of phenytoin, including cardiac malformations, have been previously documented. We report one patient with foetal hydantoin syndrome and atrioventricular septal defect with common atrioventricular junction but separate right and left atrioventricular valves, an association that has not been described, to the best of our knowledge.- - - - - - - - - - ranking = 0.25keywords = malformation (Clic here for more details about this article) |
5/151. 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 = 0.25keywords = malformation (Clic here for more details about this article) |
6/151. Limb anomalies among offspring of women with a septate uterus: a report of three cases.A retrospective analysis of 467 births among 255 women with uterine malformations revealed that three (0.64%) newborns had limb reduction defects. Two women had a subseptate uterus and one a complete septate uterus with a longitudinal vaginal septum. One newborn had a bilateral split hand and split foot, one had absence of left hand and wrist. One infant born without left hand, wrist and one antebrachial bone associated with omphalocele and diaphragmatic hernia died during the neonatal period. An association between severe limb reduction defects and septate uterus is possible, although the mechanism is unclear. The findings indicate a need to evaluate the uterine cavity if a newborn has this invalid defect, and a detailed ultrasound examination of fetal limbs is warranted in a case of a pregnant septate uterus.- - - - - - - - - - ranking = 0.25keywords = malformation (Clic here for more details about this article) |
7/151. Variations in maternal serum zinc during pregnancy and correlation to congenital malformations, dysmaturity, and abnormal parturition.serum zinc concentrations in 234 gravidae showed a gradual fall during the first and second trimesters. From the 25th week of gestation until delivery there is a levelling out of mean zinc values. No correlations between serum zinc, serum HCS, or urinary excretion of oestriol were found. Women with mature infants born by normal delivery showed significantly higher serum zinc during pregnancy than women with abnormal deliveries and/or abnormally developed infants (p less than 0.001). Eight infants showed congenital malformations. Five of the 8 mothers showed the lowest serum zinc concentrations recorded during respective week of pregnancy. A diabetic woman gave birth to an immature infant with multiple skeletal malformations. She showed the lowest serum zinc in the 21st week, and at the same time a very low alkaline phosphatase activity. Her serum proteins and serum HCS were normal. Women with dysmature infants showed significantly lower zinc values during pregnancy (p less than 0.02) than women with mature infants born by normal delivery. Data from studies on zinc metabolism show that there is a requirement of at least 375 mg of zinc during pregnancy in order to meet the demands of normal weight gain. Teenagers, women with multiple pregnancies, women with impaired intestinal absorption due to disease or drugs and in particular women with a low-protein, high-phytate diet seem to risk developing zinc deficiency during pregnancy.- - - - - - - - - - ranking = 1.5keywords = malformation (Clic here for more details about this article) |
8/151. zinc deficiency in malabsorption states: a cause of infertility?Thirteen patients with malabsorption, 7 women and 5 men, were investigated extensively. All showed low serum zinc concentrations irrespective of the duration of illness and degree of malabsorption. Eleven of the 13 had active coeliac disease. It was suspected that the low serum zinc concentrations reflected a state of zinc deficiency, and this theory was borne out by the fact that no inflammatory reaction, no clear-cut albumin deficiency, and no oestrogen or corticosteroid influence could be demonstrated. All 7 women suffered from infertility, in most of them of long standing. Two showed secondary infertility after pregnancy and abnormal labour resulting in infants with congenital malformations (one case of bilateral congenital dislocation of the hip and one of multiple cardiac anomalies). I have reported similar complications in pregnancies in which the serum zinc was low. One of the infertile women conceived after the institution of gluten-free diet and zinc therapy, but later aborted spontaneously. Investigations on zinc metabolism and intestinal absorption might well prove valuable in otherwise unexplained infertility and could open up a new therapeutic approach.- - - - - - - - - - ranking = 0.25keywords = malformation (Clic here for more details about this article) |
9/151. Vogt-Koyanagi-Harada syndrome in a pregnant patient treated with high-dose systemic corticosteroids.PURPOSE: Vogt-Koyanagi-Harada (VKH) syndrome is usually treated with high-dose systemic corticosteroids. However, in a pregnant patient with VKH syndrome, the effects of high-dose corticosteroids on the fetus are controversial. We discuss treating VKH syndrome during pregnancy, systemic corticosteroids, and abnormalities in the delivered infant. methods: Case report and literature review. RESULTS: A 26-year-old Japanese woman in the 16th week of gestation was diagnosed with VKH syndrome and treated with high-dose systemic prednisolone after the 18th week of gestation. Although the patient's ocular findings gradually improved, a low-birth-weight infant was delivered with epibulbar dermoid, lipodermoids, and preauricular appendages. Although low birth weight might result from systemic prednisolone administration, the anomalies of the infant may have been inherited rather than caused by VKH syndrome and high-dose systemic corticosteroid administration. CONCLUSION: In this case and previously reported cases, VKH syndrome and systemic corticosteroids administered during pregnancy may not precipitate abortion and congenital malformation.- - - - - - - - - - ranking = 0.25keywords = malformation (Clic here for more details about this article) |
10/151. arthrogryposis multiplex congenita and bilateral mid-brain infarction following maternal overdose of co-proxamol.We report a case of arthrogryposis multiplex congenita secondary to fetal hypokinesia in a 41-week gestation infant following antenatal central nervous system injury. The mother's pregnancy was complicated by an episode of attempted self harm, with an overdose of co-proxamol at 22 weeks of gestational age, and by the use of cocaine in combination with excess alcohol intake. Magnetic resonance imaging showed bilateral mid-brain cysts and marked atrophy of the basal ganglia and thalami.- - - - - - - - - - ranking = 0.031005053609263keywords = nervous system (Clic here for more details about this article) |
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