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1/106. Congenital unilateral fusion of the maxilla and mandible.

    We present an uncommon severe first branchial arch congenital malformation, in which complete unilateral bony fusion between the maxillary and mandibular processes was found in a newborn exposed to carbamazepine medication all through pregnancy. This condition interferes with oral feeding, intubation, growth and development. In a review of previously reported cases it was found that this anomaly was commonly associated with other abnormalities. The etiology of this malformation was uncertain in our case, as carbamazepine was not proven to be the definite cause.
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2/106. fertility and its complications in a patient with salt losing congenital adrenal hyperplasia.

    A report is made concerning fertility and its complications in a patient with salt losing congenital adrenal hyperplasia. fertility with a successful outcome of pregnancy has rarely been reported in women with salt losing congenital adrenal hyperplasia. Problems which have been identified in the past include non-compliance, poor endocrine follow up, secondary polycystic ovarian disease with menstrual irregularity, anovulation and problems related to sexual function. There has been only one report in the literature of a woman with salt losing congenital adrenal hyperplasia who has had two pregnancies with live births. There has been no previous report of subsequent problems with neonatal management of these children. This case highlights some of the long term hazards of management of salt losing congenital adrenal hyperplasia and reports for the first time neonatal complications possibly consequent upon prenatal maternal therapy.
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3/106. Neonatal mydriasis due to effects of atropine used for maternal Tik-20 poisoning.

    A neonate was born to a mother who had consumed an organophosphorus(OPC) compound with suicidal intent. The mother was administered atropine and this caused mydriasis in the neonate without any other pharmacological effects. There was no evidence of placental dysfunction. There are no case reports of OPC consumed in pregnancy and its effect on neonates or of effects of massive doses of atropine in the mother and its effects on the fetus or the newborn.
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4/106. Perinatal vasoconstrictive renal insufficiency associated with maternal nimesulide use.

    A full-term newborn developed oliguric renal failure at 24 hr of life, which persisted for several days. Her mother ingested therapeutic doses of nimesulide, a non-steroidal anti-inflammatory (cyclo-oxygenase-2 inhibitor) drug, during the last 2 weeks of pregnancy. She was found at delivery to have developed oligohydramnion, esophagitis, and a bleeding peptic ulcer. The infant's fractional excretion of sodium was very low (0.5%) pointing for a severe vasoconstrictive mechanism involved. Renal sonogram showed hyperechogenic medullary papillae, which resolved during convalescence. This case emphasizes the importance of renal prostagandins in the control of vascular tone and sodium homeostasis. This is the first report of an adverse effect of fetal renal circulation by maternal ingestion of nimesulide.
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keywords = pregnancy
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5/106. Cloverleaf skull and multiple congenital anomalies in a girl exposed to cocaine in utero: case report and review of the literature.

    The case of a girl with cloverleaf skull (CLS) and multiple congenital anomalies is reported. Both parents have a history of drug use. Maternal cocaine abuse during the first trimester of pregnancy was obvious, and other drugs, such as marihuana and alcohol, were also taken by the mother. Many central nervous system malformations have been reported in association with cocaine abuse, the most severe being midline defects and neural tube defects. To our knowledge this is the first case reported of CLS anomaly associated with drug exposure. We also describe other anomalies not previously reported in association with CLS.
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6/106. Multimodal cancer chemotherapy during the first and second trimester of pregnancy: a case report.

    This paper reports treatment with combined chemotherapy during pregnancy. A 39-year-old woman with breast cancer was given adjuvant chemotherapy including cyclophosphamide, methotrexate and 6-fluorouracil from the 6th to the 24th week of gestation. The possibility of teratogenic effects on the fetus was explained to the patient however she refused to terminate the pregnancy. A 30-week male infant with only a minor malformation was delivered. The authors reviewed the literature regarding chemotherapeutic agents given during the first trimester of pregnancy. Most cytotoxic drugs have teratogenic effects on experimental animal subjects. However, actual data on human fetuses are sparse because of the variety of therapeutic regimens and the rarity of administering chemotherapy during pregnancy. The long-term effects of exposure to cytotoxic drugs in utero, needs further research.
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ranking = 8
keywords = pregnancy
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7/106. Disorders of maternal calcium metabolism implicated by abnormal calcium metabolism in the neonate.

    Normal fetal and neonatal calcium homeostasis is dependent upon an adequate supply of calcium from maternal sources. Both maternal hypercalcemia and hypocalcemia can cause metabolic bone disease or disorders of calcium homeostasis in neonates. Maternal hypercalcemia can suppress fetal parathyroid function and cause neonatal hypocalcemia. Conversely, maternal hypocalcemia can stimulate fetal parathyroid tissue causing bone demineralization. We report two asymptomatic women, one with previously unrecognized hypoparathyroidism and the other with unrecognized familial benign hypercalcemia, who were diagnosed when their newborn infants presented with abnormalities of calcium metabolism. J.B. was born at 34 weeks' gestation with transient hyperbilirubinemia and thrombocytopenia. At 1 month of age he had severe bone demineralization, cortical irregularities, widening and cupping of the metaphyses, and lucent bands in the scapulae. The total serum calcium and phosphorus were normal with an ionized calcium of 5.4 mg/dL (4.6-5.4). His alkaline phosphatase, parathyroid hormone, and 1,25-dihydroxyvitamin D levels were all increased. P.B., mother of J.B., had no symptoms of hypocalcemia either prior to, or during this pregnancy. She had severe hypocalcemia and hyperphosphatemia, laboratory values typical of hypoparathyroidism. J.N. presented at 6 weeks of age with new onset of seizures and tetany secondary to severe hypocalcemia. The serum phosphorus, creatinine, alkaline phosphatase, and parathyroid hormone levels were normal. At 15 weeks of age his calcium was slightly elevated with a low fractional excretion of calcium. P.N., mother of J.N., had no symptoms of hypercalcemia either prior to, or during this pregnancy. Her serum calcium was 12.7 mg/dL and urine calcium was 66.5 mg/24 hr, with a low fractional excretion of calcium ranging from 0.0064 to 0.0073. P.N. has a brother who previously had parathyroid surgery. Both J.N. and P.N. meet the diagnostic criteria for familial benign hypercalcemia. These cases illustrate the important relationships between maternal serum calcium levels and neonatal calcium homeostasis. They emphasize the need to assess maternal calcium levels when infants are born with abnormal serum calcium levels or metabolic bone disease.
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ranking = 2
keywords = pregnancy
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8/106. Angio-oedema in a neonate with IgG antibodies to parvovirus B19 following intrauterine parvovirus B19 infection.

    We report a neonate with angio-oedema following fetal hydrops caused by maternal parvovirus B19 infection. Levels of complement components, including total haemolytic complement activity and C1 inhibitor concentration, were within normal ranges in cord blood. Neonatal angio-oedema might be included in the clinical spectrum of parvovirus B19 infection in pregnancy.
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ranking = 1
keywords = pregnancy
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9/106. Perinatal psychiatry: infant mental health interventions during pregnancy.

    The authors focus on infant mental health interventions during pregnancy in response to stressors, behaviors, and difficulties experienced by the mother-to-be (as well as by the father-to-be and surrounding family or support system) that are likely to have a negative impact on the growth, development, behavior, and psychological environment of the baby. After summarizing normal tensions and psychological tasks, the authors focus on difficulties during pregnancy: "pathology of destiny," excessive anxiety, domestic violence, fear of becoming a mother, denial of pregnancy, somatic complaints, inadequate weight gain and eating disorders, and depression. The effects of these difficulties on the baby, as well as intervention techniques (including a psychosocial support group), are highlighted.
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ranking = 7
keywords = pregnancy
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10/106. brain injuries in early foetal life: consequences for brain development.

    learning disability and cerebral palsy are often related to factors present before birth. We report three patients (two with schizencephaly, one with unilateral cerebellar agenesis) in whom the timing of an insult to the foetus was known. In the first case, the mother had a trauma at 16 weeks of pregnancy and schizencephaly was discovered in the male infant associated with a left hemiplegia. In the second child, amniocentesis performed at 16 weeks into pregnancy may have been responsible for the same cortical anomaly. In the third case, sequential foetal echographies clearly demonstrated that an apparent unilateral cerebellar agenesis was related to an haemorrhagic event secondary to cerebellar trauma that occurred at 19 weeks of pregnancy. It is suggested that these brain malformations are related to an ischemic mechanism or a traumatic event in foetal life.
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ranking = 3
keywords = pregnancy
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