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1/4. Biliary lithiasis in early pregnancy and abnormal development of facial and distal limb bones (Binder syndrome): a possible role for vitamin k deficiency.

    BACKGROUND: Binder syndrome is a maxillonasal dysostosis characterized by midface and nasal hypoplasia, sometimes associated with short terminal phalanges of fingers and toes and transient radiological features of chondrodysplasia punctata. warfarin- or phenytoin-induced vitamin k deficiency during early pregnancy is a well-established etiology for this syndrome, which occurs nevertheless sporadically in most cases. CASE(S): We describe here the first case, to our knowledge, of Binder syndrome in a child whose mother presented with biliary lithiasis in early pregnancy. The mother proved to have a decrease in clotting factors II, VII, and X, and in prothrombin time, at 11 weeks of gestation, which was highly suggestive of vitamin k deficiency. CONCLUSIONS: The biliary lithiasis-induced vitamin k deficiency in early pregnancy is likely to have resulted in Binder syndrome. This observation should prompt physicians to carefully check for vitamin k deficiency in pregnant women presenting with biliary lithiasis, in order to prevent Binder syndrome in the fetus by providing intravenous vitamin K supplementation as soon as possible. Finally, reassuring genetic counseling regarding the genetic risk for future pregnancies is to be provided to the parents.
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2/4. The neurodevelopmental consequences of prenatal alcohol exposure.

    During pregnancy, ingestion of alcohol, a known teratogen, can cause harm to the fetus. Prenatal alcohol exposure is one of the leading causes of birth defects, developmental disorders, and mental retardation in children. The fetal central nervous system is particularly vulnerable to alcohol; this vulnerability contributes to many of the long-term disabilities and disorders seen in individuals with prenatal alcohol exposure. Diagnoses associated with prenatal alcohol exposure include fetal alcohol syndrome (FAS), partial fetal alcohol syndrome, fetal alcohol effects, alcohol-related neurodevelopmental disorder, and alcohol-related birth defects. Once diagnosed, early intervention improves the long-term outcome of affected children. Without documentation of maternal alcohol use, a diagnosis, and consequently treatment, is often difficult to attain. It is imperative that nurses, physicians, and other healthcare providers become comfortable with obtaining a history of, and providing anticipatory guidance and counseling about, alcohol use.
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3/4. Disorders of the gastrointestinal tract in children: consultation-liaison experience.

    Disorders of the gastrointestinal tract are common in children. Fortunately, many are short-lived, related to infection, food intolerance, or specific etiology. Those that persist or recur require greater attention on the part of the physician and can require psychiatric consultation. The frequency of consultation will depend in large part on the psychosocial sophistication and philosophy of care of the referring physician. When consulted, the child psychiatrist can complement the medical care by examination in greater detail of the psychosocial environment of the child, the family, and by psychiatric evaluation of the child. Formulation of these factors may then point the way to more helpful management of the child and treatment. The most serious problems, such as regional ileitis and ulcerative colitis, require not only collaboration of pediatricians and child psychiatrist, but surgeons as well if patients are to receive optimum care.
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4/4. Congenital conduction defects in children born to asymptomatic mothers with anti-SSA/SSB antibodies: report of two cases.

    It is very difficult to identify pregnant asymptomatic mothers carrying anti-SSA/SSB antibodies. We report two cases of neonatal lupus erythematosus, born to asymptomatic mothers with anti-SSA/Ro antibody, who developed isolated complete congenital cardiac heart block and transient second degree conduction defect associated with cardiac abnormalities, respectively. The first died suddenly of acute myocarditis at the age of 20 months, while the second underwent surgery at the age of 10 years for a ventricular septal defect, after two episodes of second degree atrioventricular block in infancy. We believe that in both cases the diagnosis could have been made in utero after correct heart beat analysis. We propose careful monitoring of fetal heart beat in all pregnant mothers. The occurrence of heart beat modification should prompt clinicians to test the mother for antibody positivity. This approach may permit early diagnosis and in utero treatment in order to spare the child from cardiac conduction defects. We provide the evidence of these cases and propose a flow chart for all physicians dealing with pregnancy.
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