Richard E. Ringel

Pulmonary vein stenosis

Pulmonary vein stenosis (PVS) is a rare disorder. Accurate diagnosis often requires anatomical examination. We report four children with pulmonary vein stenosis. Autopsy showed bilateral lesions in two patients who were thought clinically to have unilateral disease. A diagnosis of PVS was made at autopsy in the third case. Intimal and medial fibromuscular proliferation was noted in extrapulmonary and intrapulmonary veins. Some of the fibromuscular proliferation were eccentric, resembling organized thrombi. In one case a focal organizing thrombus was found in a clinically unobstructed but anatomically narrowed veno-atrial junction. In another case injection of contrast medium into the stenotic pulmonary vein (PV) showed anastomosis between PV and bronchial vessels as well as small pulmonary arteries. Bilateral hypertensive arteriopathy was observed in unilateral and bilateral PVS. Our histological finding of intrapulmonary venous lesions in the lobes in which PVS was not detected clinically suggests that during surgical correction of unilateral PVS multiple biopsies of the opposite lung may help to evaluate possible bilateral disease. Our study also suggests that thrombosis in a stenotic pulmonary vein may further compromise the lumen and contribute to the progression of pulmonary vein obstruction. The possible pathogenesis of bilateral pulmonary hypertensive arteriopathy in unilateral PVS also is discussed.

Cardiologic Perspectives of Chest Pain in Childhood: A Referral Problem? To Whom?

Discussed are the diagnostic and therapeutic issues of cardiac disease in the pediatric age group that have the potential to result in ischemic ventricular dysfunction. The discussion then turns to the nontraumatic thoracic, gastrointestinal, and psychogenic disturbances that are likely to produce symptoms of chest pain. In this context, the question of when to refer, when to reassure, when to begin a diagnostic evaluation, and when to institute longitudinal follow-up are addressed.

Cardiovascular Reflex Abnormalities in Children and Adolescents With Diabetes Mellitus

OBJECTIVE To assess the usefulness of specific cardiovascular reflex tests in childhood and to estimate the prevalence of cardiovascular reflex abnormalities among children with IDDM. In adults, abnormal cardiovascular reflexes are a frequent complication of diabetes, associated with increased morbidity and mortality. RESEARCH DESIGN AND METHODS We measured heart-rate responses to deep breathing and standing in ambulatory children with and without IDDM between 6–19 yr of age. A subgroup of the IDDM patients was retested after 1 yr. RESULTS We found the best techniques for detecting cardiovascular reflex abnormality in children were as follows: to record heart-rate responses to deep breathing either as the change inheart rate corrected for inspiratory heart rate or as the ratio of R-R intervals during expiration and inspiration; and to use the Maximum-minimum ratio for heart-rate responses to standing. HR-DBC was lower in diabetic than nondiabetic children (28.6 ± 9.2% [n = 248] vs. 33.6 ± 6.8% [n = 60]; P < 0.0005). Similarly, E:I was lower in children with IDDM than control subjects (1.42 ± 0.19 [n = 248] vs. 1.52 ± 0.15 [n = 60]; P < 0.0005). In the IDDM group, 21% of the children had abnormal HR-DBc or E:I responses. HR-STND M/m was lower in children with IDDM than control subjects (1.28 ± 0.20 [n = 167] vs. 1.38 ± 0.22 [n = 45]; P < 0.014). Among children with IDDM, 11.4% had abnormal HR-STND M/m responses. Overall, 29% of IDDM children tested abnormal in either HR-DBC or HR-STND M/m; 3% were abnormal in both tests. We found no correlation of HbA1c levels (n = 74) or duration of diabetes with either HR-DB, expiration to inspiration (n = 248), or HR-STND M/m (n = 167). In patients who were reevaluated after 1 yr we found a high correlation of the first and repeat HR-DBC tests (r = 0.47, n = 75, P < 0.0001), E:I (r = 0.53, n = 75, P < 0.0001), and HR-STND M/m (r = .49, n = 37, P < 0.002), but no evidence of an increased number of children with cardiovascular reflex abnormality. CONCLUSIONS With easily performed HR-DB and HR-STND tests, we detected cardiovascular reflex abnormality in 29% of children with IDDM. We found no correlation of changes in HR-DB and HR-STND with HbA1c or duration of diabetes. These tests provide an objective clinical measurement to monitor autonomic neuropathy in children with diabetes.

Periosteal changes secondary to prostaglandin administration

underestimated, as illustrated by Dr. OSullivans report and our experience. Mild symptoms and objective evidence for psychomotor impairment have been reported at COHb concentrations <10%? Smokers commonly have COHb concentrations in the 5% to 10% range, and may be especially vulnerable to undetected indoor sources of carbon monoxide. Finally, carbon monoxide exposure is potentially lethal and, once suspected, can be easily verified by measuring COHb in a venous blood sample. Jose Velasquez Rojas, M.D. Anthony L. Mansell, M.D. Department o f Pediatrics Pediatric Pulmonary Division College o f Physicians & Surgeons o f Columbia University 630 W. 168th St. New York, N Y 10032 REFERENCES 1. Ayres SM, Muller HS, et al: Systemic and myocardial hemodynamic responses to relatively small concentrations of carboxyhemoglobin. Arch Environ Health 18:699, 1969. 2. Morandi M, Eisenbud M: Carbon monoxide exposure in New York City: A historical overview. Bull NY Acad Med 56:817, 1980. 3. U.S. Environmental Protection Agency: A study of indoor air quality. Research Triangle Park, NC, 1974, No. 650/474-042. 4. U.S. Environmental Protection Agency: Indoor air pollution in the residential environment, vol. 1. Data collection: Analysis and interpretation. Research Triangle Park, NC, 1978, HUD-US, EDA 600/7-78-229a. 5. National Academy of Sciences: Carbon monoxide. Washington, DC, 1977, The Academy.

Red blood cell Na+,K+-ATPase in men with newly diagnosed or previously treated essential hypertension.

Alterations of cellular function of Na+,K+-adenosine triphosphatase (ATPase; Na+-K+ pump) have been implicated in the pathophysiology of essential hypertension. Therefore, this aspect of red blood cell (RBC) Na metabolism was studied in black men with newly diagnosed, untreated essential hypertension (NEH) and a normotensive control group. RBC Na content, Na+-K+ pump number (ouabain binding sites), and pump activity were measured. No statistically significant differences were found between the two groups for any of these three parameters. However, a group of previously treated essential hypertensive subjects (PEH) who had been withdrawn from therapy in the preceding 6 weeks were also studied. This group differed significantly from the NEH subjects in regard to all RBC Na+-K+ pump parameters. Their RBC Na content (10.27 +/- 3.23 vs 7.77 +/- 2.52 mmol Na/LRBC; p = 0.006) was higher, and their Na+-K+ pump activity (166 +/- 50 vs 221 +/- 87 nmol inorganic phosphate/mg membrane protein/hr; p = 0.03) and Na+-K+ pump number (213 +/- 40 vs 284 +/- 85 binding sites/RBC; p = 0.001) were lower compared with those in NEH subjects. Although the PEH subjects were older and somewhat less hypertensive than their NEH counterparts, these factors were not found to influence the Na+-K+ pump parameters. These results indicate that chronic diuretic therapy of patients with essential hypertension is associated with a reduced number of RBC Na+-K+ pumps. Since RBCs are not considered target cells for diuretics, the effects of these drugs on RBC electrolyte metabolism may occur at the time of erythropoiesis by the production of RBCs with fewer Na+-K+ pumps.(ABSTRACT TRUNCATED AT 250 WORDS)

Endogenous Inhibition of Red Blood Cell Na, K-ATPase in Essential and Pregnancy-Induced Hypertension

Digoxin-like inhibitors of Na,K-ATPase have been implicated in the pathophysiology of essential (EH) and pregnancy-induced hypertension (PIH). A technique that enhances dissociation of digoxin from red blood cells (RBC) was used to displace endogenous digoxin-like substances from RBCs. RBC membranes were preincubated in Na and ATP (Release) or Na,K,Mg and ATP (Retention) prior to measuring ATPase activity. Groups studied were: 39 men with EH and 34 controls plus 10 women with PIH and 17 normotensive controls. All displayed similar increases in Na,K-ATPase activity (24.0 +/- 7.9%) following Release. Plasma digoxin immunoreactivity (DI) was measured in pregnant women, m = 0.25 +/- 0.07 ng/ml. No DI was detected in nonpregnant women, but RBCs from these women demonstrated the same increase in Na,K-ATPase activity after Release. The 24% increase in activity achieved by Na and ATP preincubation can be reversed by adding K and Mg to the Release suspension. However, after RBC-bound digoxin is displaced by Release preincubation, addition of K and Mg cannot promote renewed binding and pump inhibition. Thus, the observed endogenous inhibition is not due to displacement of a digoxin-like substance but probably is related to alteration of the enzyme-membrane interaction. Furthermore, even though pregnant women demonstrate DI, an inhibitory substance with digoxin-like binding could not be recognized using this technique.

Red cell cotransport activity and sodium content in black men. Relationship to essential hypertension.

Furosemide-sensitive sodium and potassium cotransport and intracellular sodium content ([Na]i) were measured in erythrocytes (red blood cells, RBCs) from a population of 90 adult black men with and without essential hypertension (EH). The mean values for sodium cotransport activity, expressed as furosemide-sensitive Na efflux (mmol/liter RBC/hr), were not significantly different among the EH patients and two control groups, normotensive subjects with a positive history (N+) and those with a negative family history (N-) for hypertensive disease (EH: 154 +/- 123, n = 53; N+: 167 +/- 93, n = 12; and N-: 207 +/- 142, n = 20; all values are means +/- SD). The mean [Na]i 9.66 +/- 3.02 mmol/liter RBC (n = 56) for the EH group was greater than the mean value for the N- control group (7.96 +/- 1.97, n = 20; p less than 0.05). The N+ group also displayed a higher mean [Na]i (10.38 +/- 3.18, n = 12; N+ vs N- p less than 0.01). Although there was substantial overlapping of [Na]i values between the groups and no clear dividing line, the distribution curve of the [Na]i values in EH was skewed toward higher concentrations than in N-. Nevertheless, we must conclude that erythrocyte cotransport and [Na]i are not clinically useful in the identification of EH in black men.

Intrapericardial triamcinolone hexacetonide in the treatment of intractable uremic pericarditis in a child

Uremic pericarditis in children on chronic hemodialysis represents a difficult management problem, necessitating vigorous medical therapy and often surgical drainage of the pericardial effusion. Standard therapeutic approaches have met with limited success. The successful use of intrapericardial triamcinolone in a 10-year anephric boy on chronic dialysis is reported and accompanied by a description of the technique applied and literature review.

Ductus Arteriosus in Premature Infants Beyond the Second Week of Life

Persistent patency of the ductus arteriosus (PDA), common in premature infants, is associated with severe respiratory distress. The likelihood and significance of finding PDA in premature infants beyond the second week of life is unknown. We retrospectively analyzed all echocardiograms obtained between 1987 and 1992 on infants <35 weeks’ gestational age. Of 446 echocardiograms 77 were obtained from infants ≥14 days. Of the 77 infants, 17 (22%) were found to have PDA (group 1) and the remainder did not (group 2). Forty-eight infants had been diagnosed as having PDA prior to 14 days of age. Of these infants, 16 were from group 1. Thus only 1/17 (6%) infants diagnosed as having PDA after 2 weeks did not have a history of PDA. The presence of PDA after 2 weeks did not relate to duration of oxygen therapy, ventilator therapy, or hospital stay. Furthermore, late closure of PDA in a subgroup of 11 infants did not appear to affect these parameters. It was concluded that premature infants beyond the second week of life are unlikely to have PDA if PDA had not been diagnosed during the first 14 days. Closure of PDA beyond the second week may not improve the infant’s respiratory status.

Pneumopericardium as a complication of balloon atrial septostomy.

SummaryAn infant with transposition of the great arteries and a coexistent pneumothorax, developed a pneumopericardium during balloon atrial septostomy.