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1/8. Managing passively acquired autoimmune neonatal neutropenia: a case study.

    pregnant women with autoimmune neutropenia (AIN) and circulating neutrophil-specific autoantibodies can deliver neutropenic neonates at risk of sepsis. We report the case of a woman who had two such pregnancies. The woman had been on prophylactic granulocyte colony-stimulating factor (G-CSF) treatment, but this was ceased prior to conception in both pregnancies. In the first pregnancy, there was no monitoring or interventions, and the neonate was neutropenic and required intensive care treatment. In the second pregnancy, the maternal neutrophil autoantibody level was monitored, and G-CSF treatment was introduced in the third trimester. The second infant had no neutropenia at delivery and an excellent apgar score. We discuss the management strategy in the second pregnancy that included monitoring of serial titres of the maternal autoantibody and the introduction of G-CSF in the third trimester, which may have contributed to a more favourable clinical outcome. This may assist other clinicians faced with similar dilemmas in the future.
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2/8. Severe folate deficiency in pregnancy with normal red cell folate level.

    We report here a case of megaloblastic anaemia in late pregnancy, which leads us to question whether folate supplements should be recommended in the UK routinely throughout pregnancy and not just in the preconception period and first trimester.
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3/8. Klippel-Trenaunay-type syndrome in pregnancy.

    INTRODUCTION: Klippel-Trenaunay-Type Syndrome (KTTS) is a rare congenital anomaly with variable expression and an unknown etiology characterized by capillary and venous malformations and hypertrophy of bone and soft tissue. pregnancy has been rarely reported in patients with KTTS and since 1989 there have been only 13 case reports of pregnancy in women with KTTS reported in the literature. Concurrent pregnancy is associated with adverse perinatal outcomes. To the best of our knowledge this is the second reported, and largest, series of cases. STUDY DESIGN: After a thorough review of the literature, the medical records of four obstetrical patients with KTTS were reviewed. RESULTS: The obstetrical course of women with KTTS varies. Complications include bleeding, DIC, thromboembolic events, and pain. CONCLUSIONS: The maternal and fetal risks associated with pregnancy in women with KTTS are proportional to the severity of disease, which can be exacerbated by pregnancy. Thoughtful preconceptional counseling, along with methodical and systematic intrapartum and postpartum care are keys to reducing mortality and morbidity.
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4/8. Rapid development of nephrotic syndrome, hypertension, and hemolytic anemia early in pregnancy in patients with IDDM.

    In recent years, the prognosis for a successful pregnancy has greatly improved for women with insulin-dependent diabetes mellitus (IDDM) who are under good glycemic control and free of complications such as vascular disease and nephropathy. We report the rapid development of severe nephrotic syndrome, malignant hypertension, and microangiopathic hemolytic anemia during the first trimester of pregnancy in a 29-yr-old woman with IDDM of 18 yr duration. Our patient had no pregestational history of retinopathy or hypertension and only minimal proteinuria. Significant improvement in blood glucose levels had been achieved over the 6 mo before conception. kidney biopsy performed before the termination of pregnancy at 10 wk gestation revealed diabetic nephropathy. No other etiology for her renal disease could be found. An arteriole was noted to have entrapped red blood cell fragments and platelet thrombi, revealing the probable source of her hemolytic process. By 8 wk postpartum, her nephrotic syndrome and hemolysis had completely resolved. At 3 mo postgestation, the patient's hypertension was still present but less severe. Her serum creatinine has continued to decrease toward normal. This is the first report of a woman with IDDM in White's classification C who developed a toxemia-like syndrome during the first trimester of pregnancy, attributable to the underlying diabetic state.
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5/8. potassium homeostasis in pregnancy.

    We selectively reviewed potassium (K) metabolism during human gestation, focusing on the influence of progesterone on renal K excretion. Approximately 300 mEq of K is gained during pregnancy. Two-thirds of it are in the products of conception, but little is known about renal K handling during gestation. We have suggested that progesterone may play a role in preventing the kaliuresis that normally occurs when aldosterone levels are elevated and substantial quantities of sodium are presented to distal nephron sites. In addition, we hypothesize that subtle K secretory problems, such as those known to occur in sickle cell disease, may be aggravated during gestation, probably due to elevated circulating levels of progesterone.
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6/8. Prognostic aspects of aplastic anemia in pregnancy. Experience on six cases and review of the literature.

    Our resent experience on six cases of aplastic anemia complicated with pregnancy is described. In addition, 43 similar cases were collected from the literature and reviewed to analyze some prognostic aspects of this relatively rare but potentially serious complication. Clinical and hematological data were treated to extract some clinically meaningful factors in relation to the success and failure of pregnancy. Among initial hematological parameters, no significant difference was found between successful and unsuccessful cases with an exception of hemoglobin concentration. The patients diagnosed as aplastic anemia prior to conception demonstrated an better outcome of pregnancy as well as survival rate of mother when compared with those diagnosed during pregnancy. mortality has apparently improved after the late 1950's. Success rate of pregnancy before 1958 was 21%, while it was 67% and 71% in the era of 1959-1969 and after 1970, respectively. However, hemorrhage and infection remained to be two major causes of maternal death in both eras. Based on these observations, the currently recommendable attitude to this complication is discussed.
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7/8. Maternal hyperphenylalaninaemia as a cause of microcephaly and mental retardation.

    We attempted to evaluate the role of maternal hyperphenylalaninaemia (HPA) as an isolated cause of mental retardation and microcephaly in children. This transversal study observed the plasma phenylalanine from mothers of 161 children with mental retardation and/or microcephaly of unknown origin. In this sample, we found two women with previously undiagnosed HPA, a frequency (2/161) higher than expected for our general population (1:12 500) (p < 0.001). We concluded that the plasma phenylalanine levels should be determined during preconceptional evaluation of every woman of reproductive age that already has had a child affected either by mental retardation or microcephaly of unknown cause. It is particularly significant where women currently having their pregnancies have not been screened for phenylketonuria as newborns, as happens in most developing countries.
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8/8. Term delivery in a woman with severe congenital neutropenia, treated with growth colony stimulating factor.

    The patient was diagnosed in childhood as having severe congenital neutropenia and had recurrent admissions with severe infections. In 1987, prior to getting married, she was sterilized. She continued to require i.v. antibiotics when she contracted a severe infection. On one occasion, she was treated with growth colony stimulating factor (G-CSF). Her increased neutrophil count was sustained following this treatment. In June 1993, she wished to start a family and underwent in-vitro fertilization (IVF) treatment. G-CSF was given prior to oocyte retrieval. She conceived on her first cycle and an ultrasound scan revealed a singleton pregnancy. Throughout the course of the pregnancy, her white cell count was monitored closely and remained at <1.0x10(9)/l. The pregnancy progressed uneventfully and at 37 weeks gestation she was admitted for G-CSF injections. At 38 weeks she was delivered of a boy weighing 3350 g, by elective Caesarean section. His white cell count was normal. This is the first case of G-CSF being used before conception and during pregnancy in a patient with congenital neutropenia. It shows that advances in cytokine therapy and close interdisciplinary liaison can lead to a successful outcome and help patients, who would otherwise remain childless, to achieve a family.
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