Background and objectives: Neurologic involvement is the most threatening complication of diarrhea-associated hemolytic uremic syndrome (D؉HUS).Design, setting, participants, & measurements: We report a retrospective multicenter series of 52 patients with severe initial neurologic involvement that occurred in the course of D؉HUS.Results: Verotoxigenic Escherichia coli infection was documented in 24. All except two patients had acute renal failure that required peritoneal dialysis, hemodialysis, or both techniques. A first group of eight patients remained with normal consciousness; five of them had protracted seizures. A second group of 23 patients had stuporous coma; five of these had protracted severe seizures, and 18 had a neurologic defect including pyramidal syndrome, hemiplegia or hemiparesia, and extrapyramidal syndrome. A third group of 21 patients had severe coma. Plasma exchanges were undertaken in 25 patients, 11 of whom were treated within 24 hours after the first neurologic sign; four died, two survived with severe sequelae, and five were alive without neurologic defect. Magnetic resonance imaging (MRI) for 29 patients showed that (1) every structure of the central nervous system was susceptible to involvement; (2) no correlation seemed to exist between special profile of localization on early MRI and the final prognosis; and (3) MRI did not exhibit any focal lesions in three patients. The overall prognosis of the series was marked by the death of nine patients and severe sequelae in 13.Conclusions: Neurologic involvement is associated with a severe renal disease but does not lead systematically to death or severe disability.

A national population-based pregnancy cohort, compiled at 231 maternity units in the United Kingdom, enrolled 2359 diabetic women in a 12-month period. Type 1 diabetes was diagnosed in 1707 enrollees and type 2 diabetes in 652. Compared to women with type 1 diabetes, those with type 2 diabetes were more likely to come from a black, Asian, or other ethnic minority group, and also likelier to be from a deprived area. In addition, these women were older at the time diabetes was diagnosed and also at the time of delivery. They were less likely than women with type 1 diabetes to be primigravidas. Just over one-third of the 1606 women studied by 13 weeks' gestation had good glycemic control.Adjusting for maternal age, stillbirths were 4.7 times more frequent in diabetic women than in the general maternity population. Rates of perinatal and neonatal mortality were 3.8-fold and 2.6-fold greater, respectively, in cases of diabetes. Perinatal mortality rates were comparable in cases of type 1 and type 2 diabetes. The prevalence of major congenital anomalies in women with diabetes was more than double that expected. Much of the difference was accounted for by nervous system anomalies -particularly neural tube defects -and congenital heart disorders. Nearly two-thirds of infants with anomalies were diagnosed before birth. Congenital heart disease was diagnosed antenatally in 55% of offspring, and other anomalies in approximately 72%.Compared to the general maternity population, infants of women who have type 1 or type 2 diabetes are at increased risk of dying perinatally and of having congenital anomalies. The number of pregnant women with diabetes is expected to increase. There is evidence that good glycemic control at the time of conception and in early pregnancy can reduce adverse outcomes, but a majority of diabetic women fail to achieve such control. ABSTRACTGestational diabetes mellitus (GDM)-carbohydrate intolerance that begins or is first recognized during pregnancycarries increased risk for the mother, fetus, and newborn infant. Both impaired insulin secretion and increased resistance to insulin have been described in GDM. Short-term exogenous corticosteroids can derange glucose metabolism when given alone or with beta-adrenergic drugs, but little is known about whether long-term steroid treatment adversely affects glucose tolerance during pregnancy. This case-control study compared 25 pregnant women with idiopathic thrombocytopenic purpura (ITP) who received steroids (5-10 mg daily of prednisone) for longer than 4 weeks with 108 pregnant women who had not received steroids. Women having pregestational diabetes were excluded. The two groups were similar with regard to age and body mass index.Steroids had been given to the ITP group for nearly 10 weeks on average, and the duration of treatment correlated positively with the risk of the development of GDM which was diagnosed in 24% of steroid-treated women, and in 3% of control women. Subsequent diabetes also was more prevalent in steroid-treated women. With one excep...

We provide a detailed description of features associated with ASPM mutations. Borderline microcephaly at birth, borderline-normal intellectual efficiency, and brain malformations can occur in ASPM-related primary hereditary microcephaly.

In contrast to ultrasound, MRI allows measurements of brain (and not skull), which are available independently of the position of the fetal head. The timing of the appearance of the different sulci is also available using MRI. It is considered to be a good marker of fetal brain maturation. The biochemical modifications contemporary to myelination make it possible to evaluate this phenomenon in the fetal brain using MRI.

Diffusion tensor imaging in utero can provide a quantitative assessment of the microstructural development of fetal white matter. Anisotropic parameters of the diffusion tensor should improve with technical advances.