1/18. valproic acid and lamotrigine treatment during pregnancy. The risk of chromosomal abnormality.A baby born to an epileptic mother had dysmorphological features associated with 47,XXX karyotype. The mother had been treated with valproic acid (1800mg per day) and lamotrigine (100mg per day) throughout pregnancy. Dysmorphological features detected in baby were intrauterine growth retardation, hypertelorism, flattened nasal bridge, low set malformed auriculas, micrognathia, very small an bow-shaped mouth with thin upper lip, cleft palate, arachnodactyly, camptodactyly, secundum atrial septal defect, bilateral hammer toes and decreased creases on the soles. At 6 months old she showed motor retardation. The molecular analysis of parents revealed that extra x chromosome was inherited from the mother. In this case whether the dysmorphological features and 47,XXX karyotype were caused by lamotrigine and valproic acid treatment during pregnancy or coincidence is in question.- - - - - - - - - - ranking = 1keywords = cleft palate, cleft, palate (Clic here for more details about this article) |
2/18. angiotensin ii-receptor-antagonists: further evidence of fetotoxicity but not teratogenicity.BACKGROUND: Like angiotensin converting enzyme (ACE) inhibitors angiotensin ii (AT II)-receptor-antagonists may cause persistent or even lethal fetotoxic defects when used during the late second or third trimester. There are insufficient data on first-trimester exposure to these substances in terms of teratogenicity. The two databases of the berlin teratology Information Service (TIS) were evaluated for pregnancy outcome following exposure to AT II-receptor-antagonists. One database covers case reports on newborns with congenital abnormalities identified after birth, in which drug-effect associations can be evaluated retrospectively. The other enrolls women prospectively according to exposure to particular drugs during pregnancy, with follow-up of pregnancy outcome. CASES: Five cases (four retrospective and one prospective) involving late-pregnancy use of AT II-receptor-antagonists were recently reported to us, each of which included one or more of the following abnormalities: oligohydramnios/anhydramnios, anuria, hypoplastic skull bones, limb contractions, lung hypoplasia, and neonatal death. Among 37 prospectively enrolled first-trimester-exposed pregnancies there were 30 live births including one with a major malformation (cleft palate). One pregnancy was electively terminated after exencephaly had been diagnosed. CONCLUSIONS: AT II-receptor-antagonists may induce fetotoxic effects when used in the second and third trimesters. The available data on first-trimester use do not strongly support a teratogenic potential. AT II-receptor-antagonists should not be used by pregnant women. In case of inadvertent exposure, therapy should be changed to the known antihypertensives of choice (e.g., metoprolol, methyldopa, and hydralazine) and fetotoxic effects should be ruled out by ultrasound. Treatment with AT II-receptor-antagonists during early pregnancy is not in itself an indication for termination of a wanted pregnancy.- - - - - - - - - - ranking = 1keywords = cleft palate, cleft, palate (Clic here for more details about this article) |
3/18. Valproate embryopathy: clinical and cognitive profile in 5 siblings.Valproate embryopathy is a well recognized syndrome caused by prenatal exposure to the anticonvulsant valproic acid (Depakote). We report five half-siblings with the same mother (four different fathers) who all have valproate embryopathy. valproic acid was the sole anticonvulsant in all five pregnancies, with doses ranging from 500 to 2,000 mg per day. All children were examined by a clinical geneticist and developmental pediatrician, and had formal developmental testing. Mean birth weight at term was 2,900 g (range: 2,400-3,400 g). Common features in the five children included: flat, broad nasal bridge (5/5), hypoplastic midface (4/5), apparent hypertelorism/telecanthus (4/5), smooth philtrum (4/5), thin upper lip (5/5), long thin tapering fingers (4/5), hypoplastic 5th toenails (2/5), and irregularly placed toes (2/5). Less frequent features were micro/brachycephaly (2/5), cleft palate (1/5), duplication cyst of small intestine (1/5), and hemangioma (1/5). None had neural tube defect. Neuropsychologic testing of the three children older than 4 years of age showed cognitive ability in the low normal or borderline range (mean IQ = 83; range: 75-86), with significantly lower scores in adaptive behavior and motor skills. Study of this family offers insight into the potentially high risk of valproate embryopathy in exposed pregnancies, and affords a unique opportunity to study the variability of expression and cognitive profile of the syndrome within one family.- - - - - - - - - - ranking = 1keywords = cleft palate, cleft, palate (Clic here for more details about this article) |
4/18. Combined chemotherapy and teratogenicity.BACKGROUND: The concomitant occurrence of breast cancer and pregnancy is relatively uncommon. We report the case of a patient with syndactyly, cleft hands, and absence of distal finger phalanges associated with maternal exposure to chemotherapeutic agents during the first trimester of pregnancy. These associations have not been previously described. CASE: The patient was born by normal delivery after 38 weeks of pregnancy. His mother became pregnant while receiving chemotherapy (cyclophosphamide, 5-fluorouracil, and adriamycin) for breast cancer, and the fetus was exposed to these drugs from conception to the 16th week of pregnancy. At birth, anomalies were observed, including a high-arched palate, microcephaly, a flat nasal bridge, bilateral syndactyly in the first and second fingers with a hand cleft between the second and third fingers and hypoplasia of the fifth fingers, and dystrophic nail of the fourth finger of the left hand. The patient's growth and development were deficient. CONCLUSIONS: The malformations associated with in utero exposure to these chemotherapeutic agents are highly variable, but growth deficiency and anomalies of the craniofacial region and limbs are the most common. The pattern of malformations in children who were congenitally exposed to chemotherapeutic agents appears to be directly related to the age at and duration of exposure, rather than to the specific drug itself. Effective contraception is essential for the safe use of a potential teratogen in nonpregnant women of reproductive age.- - - - - - - - - - ranking = 0.27513971893713keywords = cleft, palate (Clic here for more details about this article) |
5/18. Congenital malformations. cleft palate, congenital heart disease, absent tibiae, and polydactyly.A girl had cleft palate, micrognathia, Wormian bones, congenital heart disease, dislocated hips, absent tibiae, bowed fibulae, preaxial polydactyly of the feet, and abnormal dermal patterns at birth. She was born after a pregnancy complicated by exposure to multiple medications. This combination of malformations may represent a distinct entity unrelated to the medication or may be a complication of the intrauterine drug exposure.- - - - - - - - - - ranking = 1.3531588176687keywords = cleft palate, cleft, palate (Clic here for more details about this article) |
6/18. Teratogenic effects of first-trimester cyclophosphamide therapy.Intravenous cyclophosphamide was administered for severe exacerbation of systemic lupus erythematosus to a patient not known to be in the first trimester of pregnancy. The patient received no other medication except prednisone. Her neonate was born with multiple anomalies, including absent thumbs, cleft palate, low-set ears, and multiple eye abnormalities. These anomalies probably reflect teratogenic effects of cyclophosphamide, and indicate that judgment is required before its use in the first trimester. Furthermore, this case illustrates the need for effective contraception and repetitive pregnancy testing when potentially teratogenic agents are administered to presumably nonpregnant women in the reproductive age group.- - - - - - - - - - ranking = 1keywords = cleft palate, cleft, palate (Clic here for more details about this article) |
7/18. The fetal trimethadione syndrome: report of an additional family and further delineation of this syndrome.We describe a family in which seven pregnancies resulted in four infants who died and in three abortions. During these pregnancies the mother took trimethadione (Tridione), as well as other anticonvulsants. Two normal children were born after treatment with all medications were stopped. There have now been 53 reported pregnancies in which the fetuses were exposed to trimethadione or paramethadione; 48 (87%) resulted in fetal loss or a child born with congenital malformations. The most common defects include malformed ears, cleft palate, cardiac defects, urogenital malformations, and skeletal abnormalities. Delayed mental and physical development were also seen. These findings constitute a clinical entity termed the fetal trimethadione syndrome. The malformation rate is believed to be due to the teratogenic effects of trimethadione. physicians need to be aware of the danger of trimethadione and related drugs during pregnancy and should withhold these medications during this period.- - - - - - - - - - ranking = 1keywords = cleft palate, cleft, palate (Clic here for more details about this article) |
8/18. Acentric craniofacial cleft in a newborn female prenatally exposed to a high dose of diazepam.A newborn female with craniofacial clefts, including cleft lip and palate, was studied. The mother had ingested 580 mg of diazepam in a single dose at about the 43rd day of gestation. The synchronism of the drug intake and the embryological development of the affected structures suggests an etiopathogenic relationship.- - - - - - - - - - ranking = 0.64883974797704keywords = cleft, palate (Clic here for more details about this article) |
9/18. Functional palatal incompetence in the fetal anticonvulsant syndrome.We report two children with mild mental retardation and appreciable articulation difficulties whose mothers took phenobarbitone and phenytoin throughout their pregnancies. The speech difficulties in both children were due to malfunction of an anatomically normal palate.- - - - - - - - - - ranking = 0.088289704417175keywords = palate (Clic here for more details about this article) |
10/18. The isotretinoin teratogen syndrome.Two infants with prominent frontal bossing, hydrocephalus, microphthalmia, and small, malformed, low-set, undifferentiated ears were born to mothers who had taken isotretinoin in the first trimester of pregnancy. A Dandy-Walker malformation, microcephaly, hypertelorism, small ear canals, cleft palate, small mouth, and congenital heart disease were also observed. isotretinoin is a potent teratogen in man; maternal ingestion early in pregnancy leads to a distinct clinical pattern of anomalies.- - - - - - - - - - ranking = 1keywords = cleft palate, cleft, palate (Clic here for more details about this article) |
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