Silver-Russell syndrome associated to Mayer-Rokitansky-Kuster-Hauser syndrome, diabetes and hirsutism.

We report an unusual association of two rare conditions, the Silver-Russell syndrome and the Mayer-Rokitansky-Küster-Hauser syndrome, with extra endocrine anomalies. A 15-year old Silver-Russell syndrome girl was referred to our Unit because of primary amenorrhea and hirsutism of adrenal origin. A thorough assessment confirmed previous diagnoses and revealed the presence of diabetes, hyperinsulinism and a complete Mayer-Rokitansky-Küster-Hauser syndrome with an ectopic localization of both ovaries. Although initial medical treatment was not unsatisfactory, future medical, psychological and social implications are expected.

Obstetric significance of anti-Jr(a) antibody.

The first case in the English-language medical literature of anti-Jr(a) antibody during pregnancy in a Spanish woman is described. The few reported cases to date about this issue are reviewed, with advice as to an obstetric guideline to follow for pregnant women with this antibody.

Effects of prandial glycemic changes on objective fetal heart rate parameters.

BACKGROUND: There is confusion in the literature about the potential effect of maternal glucose levels on the fetal heart rate (FHR) cardiotocographic interpretation. STUDY DESIGN: prospective clinical descriptive study. SUBJECTS: 21 pregnant women with diabetes mellitus, 23 women with gestational diabetes and 18 healthy non-diabetic pregnant volunteers (control group). TREATMENT: maternal capillary glucose measurement and objective FHR analysis (Oxford System 8002) pre- and 1 h post-meal. STATISTICAL ANALYSIS: descriptive statistics. Student t-tests and Pearson correlation studies. RESULTS: Maternal capillary glucose levels ranged between 2.7-10.5 mmol/l pre-meal and 4.2 14.8 mmol/l post-meal. The differences between objective FHR parameters pre- and postmeal were not significant in any of the groups of women studied. No correlation was found between prandial glycemic and FHR changes. Women with optimal and suboptimal glycemic control exhibited similar objective FHR parameters pre- and post-meal. Women with gestational diabetes showed similar prandial cardiotocographic changes irrespective of whether they were on insulin therapy or on hypoglycemic diet only. CONCLUSIONS: Objective FHR parameters are unaffected by prandial glycemic changes over a wide range of maternal glucose levels. Timing the non-stress test in relation to the meals seems irrelevant in clinical practice.

Post-cesarean section morbidity in HIV-positive women.

BACKGROUND: The present work is an audit of post-cesarean section morbidity in HIV-positive women in the tertiary teaching hospital La Fé, Valencia, Spain. STUDY DESIGN: Retrospective case-control study. SUBJECTS: Forty-five HIV-positive pregnant women and 90 appropriately matched controls, delivered by cesarean section in the same hospital and managed using a uniform protocol. MAIN OUTCOME MEASURES: The duration of stay in hospital after cesarean section, the need for postoperative antibiotics and the incidence of major and minor puerperal complications. Baseline characteristics of HIV-positive women were also analyzed in relation to the morbidity after surgery. STATISTICAL ANALYSIS: Chi-square analysis for categorical data and parametric and non-parametric tests for numerical data, where appropriate. RESULTS: Most HIV-positive women (86.7%) had a complicated recovery after surgery. A longer duration of stay in hospital (p<0.0005) and a greater incidence of major (p<0.003) and minor (p<0.00001) postoperative complications were observed in the HIV-positive group compared to the control group. HIV-positive women with > or =500 CD4 lymphocytes/mm3 had less post-cesarean section morbidity CONCLUSIONS: A greater post-cesarean section morbidity was found in HIV-positive women compared to the control women. Immunological status of HIV-positive women may be important in predicting puerperal morbidity after surgery.

Isolated human chorionic vascular reactivity: technical considerations for fresh preparations.

1. Sixty chorionic vascular rings from normal term placentas were immersed in an organ bath for isometric tension recording to study (A) the contractile response to 120 mM of potassium chloride (KCl) after adjustment and equilibration to 1-5 g of passive tension; and (B) the concentration-response curve to KCl after adjustment and equilibration to the optimal passive tension. 2. Adjustment to 4 g of passive tension elicited the maximal (P < 0.007) and the latest (P < 0.006) KCl-induced contraction among arterial rings. Venous rings showed the greatest contraction when adjusted to 3 g, but the differences were not significant except when compared to 1 g of passive tension (P < 0.03). 3. The EC50 for chorionic arteries and veins was 14.2 and 25.7 mM, respectively (P < 0.003). The maximal contraction was already obtained with 40 mM of KCl. 4. Our results suggest that (A) the optimal passive tension for fresh human chorionic arteries is 4 g; (B) chorionic venous reactivity is less influenced by the initial tension; and (C) the optimal concentration of KCl to be used as a contracting agent of these tissues is 40 mM.

Maternal middle cerebral artery velocimetry in normal pregnancy and postpartum.

OBJECTIVE: To study changes throughout gestation and the puerperium of middle cerebral artery flow velocity waveforms obtained by transcranial Doppler ultrasound. DESIGN: Prospective, observational study. SETTING: John Radcliffe Maternity Hospital, Oxford, UK. PARTICIPANTS AND METHODS: Middle cerebral artery velocities were measured by transcranial Doppler ultrasound in 25 nonpregnant women, 22 women longitudinally followed throughout pregnancy and 21 women serially studied from delivery until the late puerperium. MAIN OUTCOME MEASURES: Transcranial Doppler changes in pregnancy and the puerperium. RESULTS: The transcranial Doppler ultrasound measurements showed good reproducibility. Signals from one middle cerebral artery could not be obtained in 4.6% of the examinations, but otherwise readings were similar on both sides. Women in the second half of pregnancy had lower middle cerebral artery mean velocities than nonpregnant women. Velocities decreased with advancing gestation but increased in the immediate puerperium to levels comparable to those found in nonpregnant women. CONCLUSIONS: Transcranial Doppler ultrasound is a noninvasive technique suitable for studying maternal cerebral haemodynamics in pregnancy and postpartum. Middle cerebral artery mean velocity decreased with advancing gestation and increased to nonpregnant values in the immediate puerperium. These physiological changes need to be considered when comparing data with measurements taken in abnormal pregnancy states.

The effect of nifedipine and methyldopa on maternal cerebral circulation.

OBJECTIVE: To study how the treatment of severe gestational hypertension affects maternal middle cerebral artery velocimetry. DESIGN: Prospective, clinical, descriptive study. SETTING: John Radcliffe Maternity Hospital, Oxford, England. PARTICIPANTS: Pregnant and puerperal women who required acute or chronic antihypertensive treatment with nifedipine (n = 46) or methyldopa (n = 26), respectively. METHODS: Transcranial Doppler ultrasound examinations of maternal middle cerebral arteries were performed before and 45 min after nifedipine; and before and 48 hours after the onset of methyldopa therapy. Blood pressure and heart rate were also recorded. MAIN OUTCOME MEASURES: Clinical and transcranial Doppler changes induced by the antihypertensive medication. RESULTS: Blood pressure and middle cerebral artery velocities decreased significantly following both short- and long-acting antihypertensive therapy. Nifedipine-induced changes were more pronounced and uniform than those found after methyldopa (16.7% and 6.4% decrease in middle cerebral artery mean velocity, respectively). The middle cerebral artery mean velocity decrease was independent of changes in the blood pressure or heart rate. CONCLUSIONS: Maternal cerebral haemodynamics are influenced by antihypertensive treatment. The reduction of middle cerebral artery flow velocities following administration of nifedipine and methyldopa may suggest that cerebral vasodilatation is occurring, which is consistent with the concept that cerebral vasospasm is present in women with pre-eclampsia. The cerebral vasodilatation could result from a direct effect of the medication on the arteries in question.

Early prenatal diagnosis of an ominous aneurysm of the vein of Galen by color Doppler ultrasound.

A case of an aneurysm of the vein of Galen detected by color Doppler ultrasound (CDU) at 25 weeks of pregnancy is presented. The initial finding was a small defect of the posterior cerebral vein. Sonographic follow-up demonstrated an enlargement of the cerebral lesion as well as severe hemodynamic consequences. Fetal demise finally occurred at 28 weeks. Necropsy findings confirmed the diagnosis. This is the first report describing the value of CDU in the early diagnosis of such aneurysms.

Dexamethasone and fetal heart rate variation.

OBJECTIVE: To determine the effect of maternal administration of dexamethasone on fetal heart rate and its variation. DESIGN: Retrospective analysis of computerised data derived from cases studied over three years. SETTING: High risk pregnancy unit, John Radcliffe Hospital, Oxford. SUBJECTS: Twenty-eight pregnant women, at 27 to 32 weeks of gestation, to whom dexamethasone was given to accelerate pulmonary maturation in the expectation of preterm delivery. METHODS: Dexamethasone (two doses of 12 mg intramuscularly, 12 h apart) was given on 51 occasions at weekly intervals (one to four occasions per patient). Complete data were available for cardiotocograph analysis from computerised measurement of fetal heart rate variables for two days before and four days after dexamethasone and, in 19 women, measurements of umbilical arterial flow velocity waveforms before and after dexamethasone. RESULTS: In 10 pregnancies without fetal distress there was a highly significant (P < 0.01) transient rise in short term fetal heart rate variation after dexamethasone administration, from means (SE) 6.4 (0.28) to 9.8 (0.4) ms. In 18 pregnancies with subsequent delivery for fetal distress (abnormal fetal heart rate pattern) and high umbilical arterial resistance index [mean 0.93 (0.06 SE)], the rise in short term fetal heart rate variation was less (P < 0.01), from mean (SE) 5.4 (0.26) to 6.1 (0.48) ms. In a further case of discordant twin pregnancy, the larger twin continued to respond to dexamethasone administrations with a rise in fetal heart rate variation for five weeks; the smaller twin, with maintained tachycardia and reduced umbilical arterial end-diastolic flow velocity, failed to respond after the first two weeks. CONCLUSION: The results show that maternal dexamethasone administration normally causes a rise in fetal heart rate variation for up to a day. This rise is reduced in pre-eclampsia or intrauterine growth retardation, associated with a reduction in umbilical flow, perhaps because of a consequential lower concentration of steroid in the fetus. The results contrast with those for betamethasone which has been reported to reduce fetal heart rate variation.

Aetiological factors involved in the low response to gonadotrophins in infertile women with normal basal serum follicle stimulating hormone levels.

This study was designed to investigate possible aetiological factors involved in the low response to gonadotrophins in women with normal basal serum follicle stimulating hormone (FSH) concentrations, stimulated for assisted reproduction. Nine of these patients with normal basal serum FSH and 22 normal controls (five of whom had had a normal response to previous gonadotrophin stimulation) were prospectively subjected to: (i) transvaginal pulsed colour Doppler ultrasound evaluation of the vessels surrounding the dominant follicle for blood flow impedance analysis, (ii) the clonidine test to explore the ability of the pituitary to release growth hormone, and (iii) detection of anti-granulosa cell auto-antibodies in blood using an enzyme-linked immunosorbent assay (ELISA). The pulsatility and resistance indices (PI, RI) were significantly (P < 0.01) higher in the women with low responses as compared to the controls on days -1 and 0 (day 0 = ovulation). Seven out of the nine low responders were out of the range calculated for normal values after evaluation of the controls. A significant (P < 0.05) decrease in the secretion of growth hormone 60-90 min after clonidine ingestion was observed in the low responders as compared to five controls with previous normal response to ovarian stimulation. Six out of the nine low responders showed a negative clonidine test. No increase in anti-granulosa cell auto-antibodies was observed in the low responders as compared to the controls, including normal responders. In conclusion, an abnormal follicular blood flow impedance in the natural cycle may be related to low responses to gonadotrophins in patients with normal serum FSH concentrations.(ABSTRACT TRUNCATED AT 250 WORDS)

Neonatal outcome after pregnancy complicated by abnormal velocity waveforms in the umbilical artery.

The neonatal outcome of 61 infants born after pregnancies complicated by absent or reversed end diastolic flow velocities (AREDFV) in the fetal umbilical artery was compared with that of 61 controls matched for gestational age born after high risk pregnancies with documented forward end diastolic flow velocities (EDFV). The AREDFV group was significantly more growth retarded, had lower platelet counts at birth, and were more likely to become significantly thrombocytopenic in the first week after birth. Owing to concerns about the possible increased risk of necrotising enterocolitis in newborn infants after AREDFV, this group was started on enteral feeds later and was more likely to receive parenteral nutrition than the EDFV group. Seven infants with AREDFV and one control infant developed necrotising enterocolitis.

Fetal cerebral Doppler in the recognition of fetal compromise.

OBJECTIVE: To establish reference ranges for the human fetal middle cerebral artery pulsatility index (MCA PI) for the local obstetric population, and to compare computerised antenatal fetal heart rate (FHR) analysis with the MCA PI as indicators of fetal compromise. DESIGN: Prospective data collection for selected patients. SETTING: High risk pregnancy unit of a teaching hospital. SUBJECTS: Group 1 consisted of 18 healthy women with uncomplicated singleton pregnancies. Group 2 consisted of 27 women admitted to the high risk pregnancy unit over a 9 month period with intrauterine growth retardation and other related problems; all these women were delivered by prelabour caesarean section. INTERVENTION: Serial Duplex sonography to determine fetal MCA PI in Groups 1 and 2. Serial FHR analysis using computerised numerical techniques in Group 2 only. MAIN OUTCOME MEASURES: Serial MCA PI values from 24 to 39 completed weeks of gestation in Group 1. Comparison of serial MCA PI values with FHR analysis in relation to fetal outcome in Group 2. RESULTS: In Group 1 the MCA PI diminished significantly as gestation advanced from 1.73 (SD 0.25) at 24 weeks to 1.38 (SD 0.26) at 39 weeks (P < 0.01). In Group 2 eleven babies were hypoxaemic at delivery: all had low MCA PI values while only nine had an abnormal FHR prior to delivery. CONCLUSION: In normal pregnancy, there is a fall in the fetal MCA PI with advancing gestation which probably reflects a decreasing vascular resistance to fetal cerebral blood flow. Hypoxaemia at delivery appeared to be better recognised by the fetal MCA flow velocity waveform than the FHR analysis. This increased sensitivity, however, was achieved at the expense of a reduced specificity. Larger studies are needed to confirm the findings of this preliminary investigation.

Noninvasive assessment of the maternal cerebral circulation by transcranial Doppler ultrasound during angiotensin II infusion.

OBJECTIVE: To examine the relationship between experimentally induced increments in blood pressure and maternal middle cerebral artery flow velocity patterns measured by transcranial Doppler ultrasound (TCD). DESIGN: Prospective experimental study. SETTING: John Radcliffe Maternity Hospital, Oxford. INTERVENTION: Middle cerebral artery flow velocity waveforms were measured using 2 MHz pulsed Doppler ultrasound via the temporal cranial approach at 28 weeks gestation before, during and 10 min following an angiotensin II infusion. SUBJECTS: 101 normotensive primiparous women at 28 weeks gestation. RESULTS: A significant rise in blood pressure and fall in heart rate were demonstrated between pre-infusion and maximum angiotensin II infusion (maximum blood pressure) recordings. Simultaneous changes were observed in all flow velocity indices, shown as a decrease in systolic velocity and pulsatility index, and an increase in diastolic and mean velocity (P < 0.0001). These values all returned to baseline levels 10 min post infusion (P < 0.0001). Statistical analysis suggested that the change in flow velocity is related to the rise in blood pressure rather than the direct effect of angiotensin II on the cerebral circulation. CONCLUSION: Transcranial Doppler can detect changes in the cerebral circulation associated with alterations in blood pressure during pregnancy. The technique needs to be evaluated further in hypertensive disease.

Spontaneous resolution of pre-eclampsia-related thrombocytopenia.

OBJECTIVE: To observe the spontaneous resolution of pre-eclampsia-related thrombocytopenia. DESIGN: A retrospective study. SETTING: High Risk Pregnancy Unit, John Radcliffe Maternity Hospital, Oxford. SUBJECTS: Thirty women with pre-eclampsia complicated by the HELLP/ELLP syndrome who did not receive any specific treatment for their thrombocytopenia. INTERVENTION: Serial platelet counts throughout labour and the puerperium until the platelet counts returned to levels above 100 x 10(9)/l. MAIN OUTCOME MEASURES: Time taken from delivery and platelet nadir for platelet counts to return to levels above 100 x 10(9)/l. The rate of recovery from the platelet nadir was measured by the slope of the serial platelet counts plotted against time. RESULTS: The mean time until platelet count exceeded 100 x 10(9)/l was 67 h (SD 25) after delivery and 44 h (SD 17) from the platelet nadir. All women had counts above 100 x 10(9)/l by 111 h after delivery, and by 88 h after the platelet nadir. Although the time to recovery appeared to depend on the degree of thrombocytopenia, the rate of resolution did not. CONCLUSION: These data can be used as a guide by clinicians as to the expected time course for postpartum resolution of pre-eclampsia-related thrombocytopenia.