Antepartum findings in fetal protein C deficiency.

A pregnancy with fetal homozygous protein C deficiency was complicated in the third trimester by fetal ventriculomegaly, intraorbital thrombosis and placental infarcts, which could be imaged by combined use of ultrasonography and MRI.

Comparative study using oral solutions of bambuterol once daily or terbutaline three times daily in 2-5-year-old children with asthma. Bambuterol Multicentre Study Group.

The aim of this study was to compare safety and efficacy of bambuterol hydrochloride (10 mg) oral solution administered once daily in the evening with terbutaline sulphate (0.075 mg/kg body weight) oral solution administered three times daily in 2-5-year-old children with asthma. There were two treatment groups: (2/3) of the patients received bambuterol and (1/3) received terbutaline. The study was double-blind, randomized, and of a parallel group design, and it lasted for 3 months after a 2-week run-in period. The primary objective was to evaluate safety (adverse events, and changes in blood pressure, pulse rate, hematology, and clinical chemistry parameters). Plasma concentrations of terbutaline and/or bambuterol were also measured. Evaluation of efficacy (diary card data) was a secondary objective. A total of 155 patients (range, 2-6 years; 3 patients were 6 years old at randomization) were treated with the study drugs; 104 patients received bambuterol and 51 patients received terbutaline. Both treatments showed a good safety profile with respect to clinical and laboratory tests, and they were generally well tolerated. Reported adverse events were mild to moderate. There were no statistically significant differences between treatment groups in any of the efficacy variables (diary variables: peak expiratory flow (PEF), asthma symptoms, restlessness, other reported symptoms, use of inhaled bronchodilators, and nighttime awakenings). For morning PEF, the mean increase from run-in to treatment was 16.9 L/min in the terbutaline group and 23.3 L/min in the bambuterol group. For evening PEF, the mean increase was 20.2 L/min in the terbutaline group and 20.6 L/min in the bambuterol group. In conclusion, once-daily bambuterol is as safe and effective as terbutaline given three times daily. The study also confirmed that bambuterol has a 24-hr duration of action, and therefore its once daily administration, makes it a preferred bronchodilator agent. Pediatr Pulmonol. 2000:29:194-201.

Determinants of bronchial responsiveness at school age in prematurely born children.

The bronchial challenge test using isocapnic hyperventilation of cold air (IHCA) was used to evaluate bronchial responsiveness in 63 offspring of multiple pregnancies when they were 8-15 years old. At birth, 27 (43%) children had had intrauterine growth retardation (IUGR, birth weight <-2 SD, or birth weight difference between twin pairs >1.3 SD). The median birth weight was 2,050 g (range, 800-3, 150), and the median gestational age was 35 weeks (range, 28-38). None of the children had asthma or suffered from asthma-like symptoms. In the interpretation of the IHCA test, a fall of 9% or more in the forced expiratory volume in 1 sec (FEV(1)) was considered as abnormal, and these children were classified as "cold air responders." The number of responders was 16 (25%); their baseline FEV(1)/forced vital capacity ratio (FEV(1)/FVC) and forced expiratory flow between 25-75% FVC (FEF(25-75)), but not FEV(1) were significantly lower than the corresponding values in nonresponders. No differences were found in perinatal or neonatal factors between responders or nonresponders. Eight (30%) of the 27 IUGR and 8 (22%) of the 36 appropriate for gestational age (AGA) children were IHCA responders. In particular, IUGR was not correlated with maximal FEV(1) falls following the IHCA test. Respiratory infections after the neonatal period were equally common in IUGR and AGA children; but infections were associated with subsequent IHCA responsiveness. Adenoidectomy, tonsillectomy, and/or myringotomy had been performed significantly more often in the responders than in the nonresponders. At least one of the above invasive procedures had been performed in 20 (32%) of the children; this group was termed the "ENT (ear, nose, throat) surgery group." Fifty-six percent of the responders, but only 26% of the nonresponders, belonged to the ENT surgery group (P = 0.02). We conclude that intrauterine growth retardation or prematurity is not associated with abnormal cold air responsiveness in the IHCA test.

Effects of intrauterine growth retardation and prematurity on spirometric flow values and lung volumes at school age in twin pairs.

Lung volumes and pulmonary expiratory flow values were investigated in 67 children from multiple pregnancies (30 twins, one set of triplets, one set of quadruplets) at the age of 7-15 years. At birth, 30 of 67 children (44%) had intrauterine growth retardation (IUGR, birth weight <-2 SD or birth weight difference between twin-pairs >1.3 SD). The median gestational age was 35 weeks (range, 28-38 weeks), and the median birth weight was 2,050 g (800-3,150 g). Lung functions were measured with a heated pneumotachograph. Data were standardized using height-based reference equations. No differences were found in lung volumes between children with IUGR and those children who had normal birth weight. Gestational age did not correlate with either airway flow rates or lung volumes. Maximum mid-expiratory flow (FEF50) did not correlate with standardized birth weight or with gestational age. In discordant twin pairs, the IUGR twins had significantly lower FEF50 than their normal birth weight counterparts (p=0.03, Z=-2.13). In the whole study group (67 children), children with IUGR had significantly lower FEF50 than children with normal birth weight (p=0.04; CI, 0.3-19.9). We propose that IUGR has the most pronounced effect on the growth of airways, and no detectable influence on lung volumes. This study confirms the crucial effect of appropriate intrauterine growth on subsequent growth on pulmonary airways.

Familial atrial fibrillation with fetal onset.

A woman presented during two pregnancies (at 25 and 23 weeks' gestation, respectively) because the fetuses had rapid, irregular tachycardia and hydrops. After maternal drug treatment and achievement of slower fetal heart rates, the hydrops gradually resolved. Both babies were born full term with continuing atrial fibrillation. In the first, an ectopic atrial rhythm was temporarily achieved during high dose flecainide treatment but, in the younger sibling, all medications and repeated cardioversions failed even temporarily to convert the atrial fibrillation with an almost isoelectric baseline in ECG to sinus rhythm. Good rate control has been achieved with digoxin in both patients. No infective, immunological, or structural cause was found in either case, and thus an inherited aetiology is probable.