Heart disease presenting in the first week of life: implications of advances in diagnosis and management since 1973

The spectra of congenital heart diseases presenting in the first week of life - diagnosis, treatment, and prognosis - were analyzed in a recent series and compared with a series 9 years earlier. Eighty percent of all infants aged 0 to 7 days referred to the pediatric cardiology service in these series had actual cardiovascular defects; 42% had critical cardiovascular disease. Use of echocardiography in the neonate with suspected congenital heart disease increased from 0 to 82% in the 9-year period, whereas, frequency of cardiac catheterization decreased. Treatment with prostaglandin E(1) and catecholamine-derived pressor agents increased. Surgical mortality in critical neonatal heart disease fell from 58 to 18%, whereas, early cardiac deaths fell from 49 to 24%. These findings illustrate a favorable trend in the care of the neonate with congenital heart disease based on less hazardous diagnostic methods, more effective medical stabilization, and advances in surgical technique.     

Laser photocoagulation for stage 3+ retinopathy of prematurity

Twenty-two infants with "threshold" stage 3+ retinopathy of prematurity (ROP) were entered into a prospective, randomized clinical trial to compare the efficacy of transscleral cryotherapy versus laser photocoagulation delivered by the indirect ophthalmoscope. Eighteen infants have been followed for at least 3 months. Fifteen of 16 eyes randomized to laser and 9 of 12 eyes randomized to cryotherapy showed regression. The results suggest that laser therapy is as effective as cryotherapy in the treatment of ROP (P = 0.285).     

Bilateral knife cuts to the perforant path disrupt spatial learning in the Morris water maze.

Both the hippocampus and the entorhinal cortex are known to be crucial for spatial learning, but the contribution of the pathway linking the two structures, the perforant path (PP), has never been tested in a spatial learning paradigm. The present study examined the role of the PP in spatial learning using the Morris water maze. Seven days after bilateral transection of the PP with a fine-bladed knife, rats were habituated to the pool, then trained to swim from varying start locations to a platform submerged in a fixed location. After 28 training trials over 5 days, probe trials (without any platform present) were given to assess spatial memory for the location. Compared to sham-operated controls, lesioned rats showed slower learning and poorer asymptotic performance in terms of both swim path distance and escape latency, and less preference for the correct quadrant during probe trials. When the platform location was "reversed" to the opposite quadrant, the lesioned rats again showed poorer learning, poorer asymptotic performance, and reduced preference for the correct quadrant on the probe trial. When tested with a visible platform whose position varied from trial to trial, lesioned rats performed as well as controls. These results are congruent with previous analyses of the contributions of the entorhinal cortex and hippocampus to spatial learning and suggest that for spatial learning, the PP is a critical functional link between these two structures.     

Screening for complement deficiency in bacterial meningitis

Seventy-seven children with bacterial meningitis were screened for complement deficiency. Both the classical and the alternate pathways were normal in 75 patients. Transiently reduced total haemolytic activity of the classical pathway was documented in a boy with meningococcal meningitis. Total haemolytic activity of both the classical and the alternate pathways were reduced in another patient with pneumococcal meningitis: individual complement components determination indicated predominant activation of the alternate pathway.     

The epidemiology of preterm labour—why have advances not equated to reduced incidence?

The major burden of preterm birth is in the developing world, where most of the increasing death and morbidity is secondary to infectious diseases such as malaria, HIV, tuberculosis, bacterial vaginosis and intestinal parasites. In some developing countries, the growth of medical care has outstripped the growth of preventive public health, with an associated increase in iatrogenic preterm births. In developed countries, more than one-third of preterm births are medically indicated because of conditions such as fulminating pre-eclampsia or severe intrauterine growth restriction. Neither of these conditions is currently preventable. One in five preterm births is associated with multiple pregnancy, and these have been greatly increased by assisted reproduction techniques. The use of tocolytics has proved disappointing perhaps because inflammation rather than spontaneous uterine activity is increasingly recognised as the final common pathway. Inappropriate antibiotics used late in pregnancy are ineffective and may have adverse effects. Currently, the most promising interventions are public health related and include reducing the transmission of communicable diseases, improvements in the management of diabetes and reduction in harmful behaviours such as smoking and drug abuse.     

Clinical-electrocardiographic correlates of newly acquired left bundle branch block: the Framingham Study

To determine whether any associated electrocardiographic findings in persons with newly acquired complete left bundle branch block (LBBB) correlate with the prevalence of associated clinically apparent cardiovascular abnormalities, electrocardiograms (ECGs) from all 55 members of the Framingham Study cohort in whom LBBB developed during 18 years of routine prospective biennial examinations were reviewed. A QRS axis left of or equal to 0 degrees, left atrial conduction delay and an inverted T wave in lead V6 on the first ECG with LBBB, and an abnormal ECG in the Framingham examination preceding the appearance of LBBB each correlated with the prevalence of systemic hypertension, cardiomegaly, coronary heart disease and congestive heart failure. However, neither the PR interval nor the duration of the QRS complex on the first ECG with LBBB correlated with the prevalence of any of the associated cardiovascular abnormalities. The 8 patients with neither left atrial conduction delay nor a QRS axis left of or equal to 0 degrees on the first Framingham ECG with LBBB nor an abnormal ECG on the examination preceding the appearance of LBBB were 6 times more likely to remain free of all of the clinical cardiovascular abnormalities than the 47 patients with 1 or more of these 3 electrocardiographic findings (p less than 0.001).     

Heterogeneity of B cell involvement in acute nonlymphocytic leukemia

In order to study the pattern of B cell involvement in acute nonlymphocytic leukemia (ANLL), multiple B lymphoid cell lines were established by Epstein-Barr virus transformation of peripheral blood mononuclear cells from two patients with the disease who were heterozygous for the X chromosome-linked glucose-6-phosphate dehydrogenase (G6PD). In one patient, the progenitor cells involved by the leukemia exhibited multipotent differentiative expression, whereas in the other patient the cells showed differentiative expression restricted to the granulocytic pathway. In the patient whose abnormal clone showed multipotent expression, the ratio of B-A G6PD in B lymphoid cell lines was skewed in the direction of type B (the enzyme characteristic of the leukemia clone) and significantly different from the 1:1 ratio expected. It is, therefore, likely that the neoplastic event occurred in a stem cell common to the lymphoid series as well as to the myeloid series. In contrast, evidence for B cell involvement was not detected in the patient whose ANLL progenitor cells exhibited restricted differentiative expression. These findings underscore the heterogeneity of ANLL. Clinically and morphologically similar malignancies in these two patients originated in progenitors with different patterns of stem cell differentiative expression. This difference may reflect differences in cause and pathogenesis.