Galactose-1-phosphate uridyl transferase deficiency is not associated with Müllerian aplasia in Dutch patients.

STUDY OBJECTIVE: To study whether a deficiency in galactose-1-phosphate uridyl transferase (GALT) activity of mothers was an explanation for the occurrence of Müllerian aplasia of their daughters. DESIGN: A case control study. SETTING: The patients were selected from the outpatient clinic of the University Medical Center Nijmegen, and compared with the general population in The Netherlands. PARTICIPANTS: Patients (n=9) diagnosed with the syndrome of Müllerian aplasia and their mothers were included. INTERVENTIONS: A questionnaire for medical and family history was taken, and a venous blood sample and urine were collected. MAIN OUTCOME MEASURES: GALT activity (in blood), galactose and galactilol (in urine) were measured. Measured values were analyzed by Student's paired t-test. RESULTS: All patients and their mothers had normal GALT activities> or =20 micromol/h/g Hb. The mean value did not differ from the mean of the normal Dutch population, which was 31.6 (SD=5.0) mumol/h/g Hb. CONCLUSION: GALT deficiency is not an explanation for Müllerian aplasia, at least in the Dutch population.

The B-Lynch technique for postpartum haemorrhage: an option for every gynaecologist.

Postpartum haemorrhage may be a life threatening complication. Seven cases are described in which the B-Lynch surgical technique (a brace like suture over the uterus) was successful in obtaining haemostasis. In four cases, the B-Lynch technique was the first line of treatment. In three cases, the B-Lynch was used after, or in combination with artery or other vessel ligation.

Role of magnetic resonance imaging in the diagnosis of incarceration of the gravid uterus.

BACKGROUND: Incarcerated uterus is a rare condition with potentially serious complications that is often difficult to diagnose. This series shows the value of magnetic resonance imaging (MRI). CASES: Five pregnant women with incarcerated uterus are presented and the MRI findings compared with MRI in normal pregnancy. CONCLUSION: The use of MRI can be helpful in diagnosing this rare condition.

Maturation of the human fetal brain as observed by 1H MR spectroscopy.

Proton MRS was used to monitor cerebral metabolite tissue levels in 35 normal fetuses during development in the gestational age range of 30-41 weeks. First, MRI in three orthogonal orientations was performed. A volume of interest (VOI) (15-43 cc) of fetal brain tissue was then selected for (1)H MRS. For localization, two pulse sequences (stimulated echo acquisition mode (STEAM) at TE = 20 ms, and point-resolved spectroscopy (PRESS) at TE = 135 ms) were applied. The MR spectra of the brain showed signals for inositol (Ino), choline (Cho), creatine (Cr), and N-acetyl (NA) compounds. From 30 to 41 weeks the absolute tissue level of NA, and the ratios of NA/Cr and NA/Cho increased, whereas the ratio of Cho/Cr decreased. These changes reflect maturation of the brain. Considering the diagnostic value of proton MRS in pediatric neurology, this new approach may also be useful for characterizing pathological conditions in the fetal brain.

1H-NMR spectroscopy of cerebrospinal fluid of fetal sheep during hypoxia-induced acidemia and recovery.

The purpose of the study was to investigate the sequence of processes occurring during and after hypoxia-induced acidemia. We used proton nuclear magnetic resonance spectroscopy, which provides an overview of metabolites in cerebrospinal fluid (CSF), reflecting neuronal metabolism and damage. The pathophysiological condition of acute fetal asphyxia was mimicked by reducing maternal uterine blood flow in 14 unanesthetized pregnant ewes. CSF metabolites were measured during hypoxia-induced acidemia, and during the following recovery period, including the periods at 24 and 48 h after the hypoxic insult. Maximum values of the following CSF metabolites were reached during severe hypoxia (pH

Pre- and postjunctional alpha(2)-adrenergic receptors in fetal and adult ovine cerebral arteries.

In ovine cerebral arteries, adrenergic-mediated vasoconstrictor responses differ significantly with developmental age. We tested the hypothesis that, in part, these differences are a consequence of altered alpha(2)-adrenergic receptor (alpha(2)-AR) density and/or affinity. In fetal (approximately 140 days) and adult sheep, we measured alpha(2)-AR density and affinity with the antagonist [(3)H]idazoxan in main branch cerebral arteries and other vessels. We also quantified contractile responses in middle cerebral artery (MCA) to norepinephrine (NE) or phenylephrine in the presence of the alpha(2)-AR antagonists yohimbine and idazoxan and contractile responses to the alpha(2)-AR agonists clonidine and UK-14304. In fetal and adult cerebral artery homogenates, alpha(2)-AR density was 201 +/- 18 and 52 +/- 6 fmol/mg protein, respectively (P < 0.01); however, antagonist affinity values did not differ. In fetal, but not adult, MCA, 10(-7) M yohimbine significantly decreased the pD(2) for NE-induced tension in the presence of 3 x 10(-5) M cocaine, 10(-5) M deoxycorticosterone, and 10(-6) M tetrodotoxin. In fetal, but not adult, MCA, UK-14304 induced a significant decrease in pD(2) for the phenylephrine dose-response relation. In addition, stimulation-evoked fractional NE release was significantly greater in fetal than in adult cerebral arteries. In the presence of 10(-6) M idazoxan to block alpha(2)-AR-mediated inhibition of prejunctional NE release, the fractional NE release was significantly increased in both age groups. We conclude that in fetal and adult ovine cerebral arteries, alpha(2)-AR appear to be chiefly prejunctional. Nonetheless, the fetal cerebral arteries appear to have a significant component of postjunctional alpha(2)-AR.