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Biomedical subjects

P K Woolf

Publications and source records attributed to P K Woolf.

32 records · Page 2Linked to original sources

Acrocallosal syndrome: additional manifestations.

The acrocallosal syndrome (ACS) is a probable autosomal recessive condition of macrocephaly, craniofacial and hand and foot abnormalities, absence of the corpus callosum, and mental retardation. This patient had characteristics of the ACS but also had a severe congenital heart defect and other visceral malformations. After comparing the ACS with and contrasting it to other disorders, we concluded that the internal organ abnormalities found in this patient probably represent further manifestations of the ACS.

Abnormalities, Multiple↗

Symptomatic cardiac dysfunction in children with human immunodeficiency virus infection.

Eight children with human immunodeficiency virus (HIV) infection had symptomatic cardiac dysfunction. The median age was 1.4 years (range 0.2 to 7.9 years). All had hepatosplenomegaly, fever, pneumonia with tachypnea, and tachycardia ascribed to infection and anemia. An S3 gallop was present in six of eight. All had normal creatine phosphokinase values. Chest x-rays did not aid in the diagnosis of cardiac dysfunction. ECG showed flattened T waves in five of eight with left ventricular hypertrophy, right ventricular hypertrophy, or both in seven of eight. Results of echocardiography showed decreased left ventricular function in all eight, despite anemia, with dilated left ventricular myopathy in six, concentric left ventricular wall thickening in two of eight, an enlarged right ventricle in two, and pericardial fluid in three. Medical therapy improved cardiac function in all. All patients subsequently died of noncardiac causes. Results of autopsies on four of eight patients showed focal myocarditis in two (with cytomegalovirus inclusions in one) and dilated cardiomyopathy in two others. We conclude: (1) Preexistent hepatosplenomegaly, fever, infection, and anemia result in physical findings that mimic findings of heart failure, thereby masking the occurrence of cardiac dysfunction; (2) an S3 gallop may indicate the presence of impaired heart function when other clinical signs are masked; (3) confirmation of cardiac compromise may be accomplished by noninvasive evaluation with echocardiography and (4) medical therapy can improve cardiac dysfunction in HIV-infected children.

Acquired Immunodeficiency Syndrome↗

Infants with apparent life threatening events. Cardiac rhythm and conduction.

The role of cardiac dysrhythmias in the pathogenesis of Sudden Infant Death Syndrome (SIDS) is uncertain, but there have been several reports of infants with Apparent Life Threatening Events (ALTE) due to significant dysrhythmias. To further characterize the cardiac rhythm and conduction of these "at-risk for SIDS" infants, we performed 24-hour continuous (Holter) electrocardiograms on 100 full term, healthy infants with ALTE. Sixty-two patients (62%) had one or more dysrhythmias on Holter monitor. Twenty-five patients (25%) had premature ventricular depolarizations (PVD), including five with couplets. Thirty (30%) had QTc greater than 2 SD above the mean, and, of these, 40 percent had PVD's. Fifteen (15%) had premature atrial depolarizations and 39 (39%) had evidence of moderate or marked sinus node irregularity. There was no prexcitation, supraventricular tachycardia, ventricular tachycardia, or atrioventricular block. Two patients with marked sinus node dysfunction were treated with propantheline and did well. All patients were monitored at home, with no deaths or clinically significant dysrhythmias on follow-up (1-32 months (mean = 18]. In summary, when Holter monitoring was performed, a high incidence of dysrhythmia was found in infants with ALTE. Most dysrhythmias were clinically insignificant. The incidence of ventricular dysrhythmias and long QTc are consistent with previously advanced theories of cardiac electrical instability in some of these patients, but no patient with ventricular dysrhythmias required therapy. The incidence of sinus node dysfunction requiring therapy was 2 percent. Although Holter monitoring of infants with ALTE only infrequently determines therapy, it may provide data linking theories of cardiac etiology of SIDS with actual clinical events.

Arrhythmia, Sinus↗

Academic promotion at a medical school. Experience at Johns Hopkins University School of Medicine.

We studied promotions at Johns Hopkins University School of Medicine to determine whether clinician-teachers are less likely to be promoted or are promoted later in life than researchers and whether those who are promoted have more articles published than those who are not promoted. Over a five-year period, 93 percent of candidates for the rank of associate professor and 79 percent of the candidates for the rank of professor were promoted. There were no significant differences between clinical and research faculty members in terms of the probability that they would be promoted or their age at promotion to either associate professor or professor. Despite these findings, the responses to a questionnaire indicated that former faculty members perceived clinician-teachers as less likely than researchers to be promoted. Those who were promoted had had about twice as many articles published in peer-reviewed journals as those who were not promoted. We recommend improved counseling of medical school faculty members and more extensive discussion of the criteria for promotion and the chances of academic success.

Age Factors↗

Variable arginine vasopressin levels in neonatal congestive heart failure.

Arginine vasopressin levels in 17 neonates with cardiac disease were compared with control levels in 10 healthy newborn infants. Infants with congestive heart failure who were free of left ventricular outflow tract obstruction had a mean level of 80 +/- 18 pg/ml, which was significantly greater than the mean control level (p less than 0.001). Infants with congestive heart failure and left ventricular outflow tract obstruction had a mean vasopressin level of 3 +/- 0.7 pg/ml, which was lower than the mean control level of 6 +/- 0.7 pg/ml (p less than 0.05). The data suggest that impaired forward flow to high pressure sinoaortic and ventricular baroreceptors is necessary for vasopressin release in congestive heart failure. In left ventricular outflow tract obstruction with heart failure these receptors may be impaired or absent, leading to decreased vasopressin release. Low plasma arginine vasopressin may adversely affect circulatory homeostasis.

Acute Disease↗

Life-threatening dysrhythmias in varicella myocarditis.

Varicella myocarditis is an unusual complication of a common childhood disease. Two patients with life-threatening dysrhythmias and circulatory failure are reported. One patient required permanent pacemaker implantation for acquired complete heart block, not previously described with varicella infection. Previously reported cases of varicella myocarditis are reviewed.

Arrhythmias, Cardiac↗

Elevated atrial natriuretic peptide after the Fontan procedure.

To assess the response of atrial natriuretic peptide (ANP) to rapidly changing right atrial pressures in vivo, we measured ANP levels in 15 patients undergoing the Fontan procedure and compared them with control levels in nine patients undergoing cardiac surgery for lesions not associated with atrial hypertension. There were no significant differences in preoperative ANP levels: 57 +/- 15 pg/ml for patients undergoing the Fontan procedure, 43 +/- 8 pg/ml for control patients. There was no significant change in ANP during surgery or in the postoperative period in control patients. In contrast, ANP increased significantly to 333 +/- 70 pg/ml (p less than .0025) after establishment of right atrial-pulmonary artery continuity with the Fontan procedure and was related to right atrial pressure, which increased from 5 mm Hg before to 14 mm Hg after the Fontan procedure (p less than .001). There was no significant change in left atrial pressure. During the first postoperative day, ANP levels fell to 141 +/- 34 pg/ml (p less than .01) but later increased to 290 +/- 80 pg/ml (p less than .025), a finding that may suggest depletion of a readily releasable intracellular pool of ANP before mobilization of a storage pool. There was no direct relationship between ANP levels and effusions in patients undergoing the Fontan procedure. Pharmacologically significant increases in ANP occur in patients undergoing the Fontan procedure, correlating with increased right atrial pressures, but the relationship between ANP and the fluid derangements after the procedure remains unclear.

Atrial Natriuretic Factor↗

Pressure to publish and fraud in science.

Pressure to publish is an overworked and ill-used phrase that encompasses at least two meanings: the pressure an individual scientist may feel, and the pressure exerted by the institutions of science. It is important not to confuse the two. A modest level of institutional pressure is legitimate and healthy for science. Persons who respond to these pressures by publishing extravagantly may be doing a disservice to impressionable young colleagues and to the notions of responsible authorship.

Authorship↗

A comparison of Blalock-Taussig, Waterston, and polytetrafluoroethylene shunts in children less than two weeks of age.

Results obtained with Blalock-Taussig, Waterston, and polytetrafluoroethylene (PTFE) shunts were compared in 67 cyanotic infants less than 2 weeks of age. A different shunt was preferably used at each of three institutions. The incidences of early shunt failure (3 out of 21, 14%), mortality after revision of early shunt failure (0 out of 3), and overall hospital mortality (1 out of 21, 5%) were all lowest for the PTFE shunt. Incidence of congestive heart failure secondary to excessive flow was comparable for the Blalock-Taussig and PTFE shunts, both of which were lower than the Waterston shunt. Cumulative probabilities of late shunt adequacy were calculated for hospital survivors. At 1 year, all shunts provided comparable adequate palliation (greater than 80%). Probability of late shunt failure was significantly higher (p = 0.04) for the PTFE shunt at 3.5 years. Results suggest that the PTFE shunt may be the safest and most effective shunt in neonates, but that elective shunt replacement or total repair may be warranted in the first or second year of life.

Age Factors↗

Surgical treatment of purulent pericarditis in children.

Since 1971 we have seen 15 children with the diagnosis of purulent pericarditis. The causative organism was Hemophilus influenzae in seven, Staphylococcus aureus in three, and five were due to other organisms. In one child the diagnosis was unsuspected until autopsy. The other 14 patients were all treated with intravenous antibiotics to which the organism was sensitive. One child had an immediate pericardiectomy because of tamponade. The other 13 patients had pericardiocentesis for diagnosis and initial therapy. Pericardiocentesis alone resulted in recovery of four patients and failed in nine, including all seven patients with H. influenzae. These nine had recurrent tamponade or a persistent picture of sepsis that was unresponsive to repeated pericardiocenteses and necessitated operative intervention. The procedure used was subxiphoid tube drainage in two patients. One recovered and the other required further operation. The remaining seven patients were treated with pericardiectomy. All pericardiectomy patients recovered without complications or recurrent symptoms. Survivors are asymptomatic with no evidence of pericardial constriction. We recommend immediate pericardiocentesis for diagnosis and initial therapy. Early pericardiectomy should be performed if the causative organism is H. influenzae, if tamponade occurs after initial pericardiocentesis, or if fever persists despite appropriate antibiotics.

Acute Disease↗

Pediatric radiofrequency ablation for tachyarrhythmias: the Westchester Medical Center experience.

Radiofrequency ablation, first performed in the late 1980s, has become a standard therapeutic procedure for tachyarrhythmias in children and adults. This procedure can achieve a high degree of success with minimal complications. This report describes the pediatric radiofrequency ablation experience at Westchester Medical Center, including 89 consecutive children who have undergone 104 ablations of 96 arrhythmia substrates with an overall success rate of 95%. The series is analyzed in the context of previously published series of pediatric radiofrequency ablations. Unresolved issues in pediatric ablation related to anesthesia, learning curve, necessity for hospital admission, anticoagulation protocol, and technical aspects of the procedure are discussed.

Adolescent↗