OBJECTIVES: To create a follow-up protocol for pregnant patients with Marfan syndrome. PATIENTS AND METHODS: We retrospectively reviewed the charts of patients who delivered in the Jeanne de Flandre University Hospital between June 1996 and June 1999. Four pregnant patients with Marfan syndrome were identified. RESULTS: Three of these patients had Bentall procedure. One of them had vaginal delivery and the two others underwent cesarean section. One of these two patients developed aortic valve thrombus at 14 weeks of amenorrhea. The fourth patient did not have surgery and had two vaginal deliveries. DISCUSSION: According to our results and after reviewing literature pregnant patients with Marfan syndrome were divided into two groups. The 1st group was comprised of patients who underwent Bentall procedure. The 2nd one was comprised of patients who did not undergo any surgical procedure. The possibility of vaginal delivery for patients who underwent Bentall procedure (one case) and the interest of Propanolol and anticoagulant treatment are emphasized. CONCLUSION: The multivariant approach of pregnant patients with Marfan syndrome is stressed out with special reference to the potential complications of this syndrome such as aortic dissection and to the problems related to the anticoagulant treatment.

Adult↗

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Anti-TJa alloimmunization (anti-PP1Pk): two consecutive pregnancies of an anti-TJa-carrying patient.

Anti-TJa alloimmunization is an extremely rare condition which can cause miscarriages during the first and second terms of pregnancy. At 20-year-old female patient, an anti-TJa carrier, was given plasma transfusions in the course of her first two pregnancies. The cordocentesis, which was the main follow-up test during the first pregnancy, could not be carried out during the second pregnancy due to the presence of a huge subchorionic hematoma. Both pregnancies were characterized by fetal growth retardation, which was particularly considerable in one. Due to chronic fetal distress, both infants were delivered prematurely by cesarean section. The immunohematological data concerning anti-TJa alloimmunization and the different palliative treatments available to date are reviewed.

Adult↗

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Prenatal diagnosis of thrombocytopenia-absent radius syndrome.

Thrombocytopenia-absent radius (TAR) syndrome is an autosomal-recessive disorder characterized by a thrombocytopenia and a bilateral radial aplasia with normal thumbs. Only TAR syndrome, out of diseases which may present with radial aplasia, typically has normal thumbs. The prenatal diagnosis is rarely made. We report two observations of TAR syndrome diagnosed in utero in the sibling. The malposition of fetal hands detectable as soon as 11 weeks of gestation requires careful search for longitudinal limb defect of the forearm, especially radial ray defect. The radial aplasia is associated with numerous causes (chromosomal, teratogenic, genetic, multifactorial). The determination of fetal hematologic values revealing a thrombocytopenia allows the prenatal diagnosis of the index case of TAR syndrome.

Cordocentesis↗

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[Painful hypertonia of the calves. The role of the level of arousal apropos of 25 new cases].

Role of the level of alertness. Based upon 25 new cases. The author, after referring to the leg muscle bed syndrome associated with exercise, reports the frequency of painful spasticity of the calves, occurring initially at rest and in particular at night in the form of a restless legs syndrome, then secondarily during the day with varying degrees of persistence at rest and during exercise. This problem frequently has a lateral dominance and appears to be induced by self-maintained functional muscular spasticity of the gastrocnemii and soleus, explained by a study. It is frequently attributed to a venous etiology. This study based upon 25 cases enabled definition of the diagnosis and showed the probable links between EMG hyperactivity and a high level of alertness indicative of a predisposing situation.

Acepromazine↗

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