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The authors present the unique case of an 8-month-old baby diagnosed with severe left ventricular failure of unknown etiology. Due to a lack of organ availability for this age, a mechanical assist device and assessment for cardiac transplantation were not offered. Subsequent comprehensive echocardiographic dyssynchrony assessment and the presence of left bundle branch block were suggestive of response to cardiac resynchronization therapy. Dual-chamber epicardial pacing was initiated, resulting in prompt marked clinical and echocardiographic improvement, which continued until complete normalization of cardiac function. The pacing system was safely turned off 6 months after its implantation. In conclusion, cardiac resynchronization therapy should be considered as a treatment option even in infancy, regardless of the etiology of disease and/or patient age.

To compare national neonatal extracorporeal membrane oxygenation data and deaths from primary respiratory disorders of term neonates between the United Kingdom and the United States from 1999 to 2005.

Severe bleeding is a recognized complication during mechanical cardiopulmonary support with extracorporeal membrane oxygenation. We present the use of recombinant activated factor VII (rFVIIa) for severe, refractory bleeding during extracorporeal membrane oxygenation support after open-heart surgery for congenital heart disease.

OBJECTIVE: To assess the effects of treatment with prasterone (dehydroepiandrosterone) on bone mineral density (BMD) in female patients with mild to moderate systemic lupus erythematosus (SLE) receiving chronic treatment with glucocorticoids. METHODS: Fifty-five female patients with SLE who had received prednisone (or glucocorticoid equivalent) ≤ 10 mg/day for ≥ 6 months were treated for 1 year with either prasterone 200 mg/day (n = 24) or placebo (n = 31) in this randomized, double blind trial. Prasterone or placebo was added to each patient's one or more concomitant standard SLE medications, including glucocorticoids, nonsteroidal antiinflammatory drugs, antimalarials, methotrexate, azathioprine, and other immunosuppressives, which were to be maintained at fixed doses for the duration of the study. RESULTS: BMD was significantly improved in patients who received prasterone compared to placebo. At the lumbar spine, there was a mean (SEM) gain in BMD of 1.7 ± 0.8% in the prasterone group compared to a mean loss in BMD of –1.1 ± 0.5% in the placebo group (p = 0.003 between groups). For the total hip, mean gain was 2.0 ± 0.9% in the prasterone group vs a mean loss of –0.3 ± 0.4% in the placebo group (p = 0.013 between groups). In the prasterone treatment group, the mean gains from baseline at both lumbar spine and hip were statistically significant. CONCLUSION: Prasterone treatment prevented BMD loss and significantly increased BMD at both the lumbar spine and total hip in female patients with SLE receiving exogenous glucocorticoids.

OBJECTIVE: To assess the timing of onset of clinical benefit following the initial infusion of infliximab and to obtain additional safety experience of infliximab when given in an office setting to patients with rheumatoid arthritis (RA). In addition, the safety of reducing the infusion time from 2 hours to 1 hour was evaluated. METHODS: Patients (n = 553) with active RA despite receiving methotrexate (MTX) were treated with infliximab 3 mg/kg given over 2 h at baseline (Week 0), and Weeks 2, 6, and 14 in this multicenter open-label trial. Patients continued to receive a stable dose of MTX (> or = 7.5 mg/wk). At selected sites, patients tolerating the first 4 infusions were eligible to receive 2 additional infusions at twice the usual infusion rate (given over 1 h). Patients returned for efficacy assessments at 48 h following the initial infusion and several times throughout study participation. RESULTS: By 48 h following the first infusion, significant (p < 0.001) improvements were observed in duration of morning stiffness (34% mean improvement), physician's global disease assessment scores (30%), patient's global disease assessment scores (25%), and patient's pain assessment scores (30%). By Week 16, 52 to 63% mean improvements in these efficacy variables were observed (p < 0.001), the significant improvement was maintained through the end of study participation in the subset of patients who received the additional 1 h infliximab infusions. Through 16 weeks, 10% (54/553) of patients reported an adverse event associated with at least 1 of the 4 infusion procedures; the majority were mild and transient in nature. In the subset of 197 patients who received 2 additional infusions over 1 h, no increase in the frequency or severity of infusion-related adverse events was observed compared to the 2 h infusion. CONCLUSION: Infliximab administered to patients with RA in an outpatient setting resulted in significant clinical improvement within 48 h that was sustained with additional infusions. Approximately 10% of patients experienced an infusion reaction, highlighting the need for direct supervision over patient treatment. Patients who tolerated infliximab infusions given over 2 h also tolerated a 1 h infusion.

PE, a common and serious pulmonary problem, most often results as a complication of DVT in the lower extremities. There are no specific symptoms or signs that allow a clinical diagnosis of PE. The best approach to the diagnosis is controversial and is at least partially dependent on the diagnostic capabilities and expertise available at a given hospital. Pulmonary arteriography is the definitive diagnostic test and should be considered in patients with results of less-than-high probability V-Q scans, those at high risk of bleeding complications with anticoagulation therapy, and those considered candidates for thrombolytic therapy.

“Confidence intervals” based upon inhalation of placebo have been proposed as criteria for defining a significant response to an inhaled bronchodilator. The published intervals were derived from a clinically heterogeneous population. We calculated the difference (delta) between spirometric data before and after placebo in 109 consecutive patients referred for methacholine bronchoprovocation challenge testing. The mean delta, expressed both as a percent change and as actual volume change for both the FVC and FEV1, was not significantly different in patients with bronchial hyperresponsiveness, as compared to subjects with a negative methacholine challenge test; however, the variance of measurements in hyperresponsive subjects was significantly greater than that of the normal population. In addition, as the category of responsiveness increased from mild to moderate to severe hyperresponsiveness, so did the variance within these groups. A negative correlation between the measured PC20FEV1and the volume and percent change was noted. We conclude that patients with hyperresponsive airways may display increased spirometric variation before and after placebo. This general approach for establishing normal limits for defining a significant response appears to be valid, but the actual values used may vary, depending on the composition of the population tested and the goals of the study. Also, the use of the term, “confidence intervals,” in this context is inappropriate; and we propose, instead, the use of percentiles and the simpler terms, upper 90th or 95th percentiles.

• To determine the daily variability of spirometry, we performed spirometry on five consecutive days on 15 normal subjects and on 24 patients with chronic bronchitis. The patients with chronic bronchitis were divided into the following two groups: 13 with chronic airflow obstruction (forced expiratory volume in 1 s [FEV1] 80%, FEV,/forced vital capacity [FVC] ratio &lt; 75%) and 11 without airflow obstruction (FEV1 and FVC > 80%). We attempted to control most of the factors known to affect spirometric variability. Our results indicate that the day-to-day FEV, has to change by 17% and the FVC by 15% in patients with airflow obstruction before the change should be considered significant. In patients with nonobstructive chronic bronchitis or in normal subjects, a greater than 5% change should occur in FEV, and FVC before considering the change to be significant.(Arch Intern Med 1982;142:1287-1291)

Eleven hypoxic (arterial oxygen pressure [PaO2] ≤ 61 mm Hg), polycythemic (hematocrit reading ≥ 54 percent) patients were studied to determine the effect of the carboxyhemoglobin level on their response to therapy with supplemental oxygen. Five nonsmokers with a mean carboxyhemoglobin level of 2.8 percent showed an exexcellent response to therapy with supplemental oxygen, with a decrease in hematocrit reading from 57 percent to 48 percent (P < 0.0025) as the PaO2increased from 53 mm Hg to 69 mm Hg (P < 0.01) and the arterial oxygen saturation (SaO2) increased from 86 percent to 94 percent (P < 0.0025). Six smokers with a mean carboxyhemoglobin level of 13 percent showed an incomplete response to therapy with supplemental oxygen, with a decrease in hematocrit reading from 62 percent to 57 percent (P < 0.0025) as the PaO2increased from 53 mm Hg to 69 mm Hg (P < 0.0025) and the SaO2increased from 82 percent to 88 percent (P < 0.01). In spite of similar values for the PaO2, the smokers had higher hematocrit readings before and during therapy with oxygen (P < 0.01), presumably due to superimposed desaturation by carboxyhemoglobin. We concluded that polycythemia in hypoxic smokers is due to additive effects of hypoxia and a high carboxyhemoglobin level. The former is responsve to therapy with oxygen, while the latter is not To achieve a “complete” response to therapy with supplemental oxygen, hypoxic polycythemic smokers should quit smoking.

Acute myocarditis of viral, bacterial, or allergic origin presents many of the features of myocardial infarction. The majority of the cases die suddenly and etiologic factors may be difficult to elicit. Myocarditis is suggested by low-grade fever, more gradual onset, the absence of severe chest pain and dyspnea out of proportion to the clinical state. The electrocardiogram usually reads nonspecific “ST-T wave” change rather than reciprocal alteration. The SGO-T levels may be elevated earlier than in myocardial infarction.

A review of the adverse effects of isoniazid (INH) was undertaken and it was found that these effects could be divided into: (1) toxic, (2) idiosyncratic and (3) hypersensitivity reactions. Major toxic reactions are peripheral neuropathies at conventional dosage and seizures from overdosage. Pyridoxine is of benefit in these disorders. Other presumed toxic reactions are autonomic neuropathy, encephalopathy, psychosis, optic atrophy, and pyridoxine responsive anemia. Idiosyncratic reactions are INH-induced lupus erythematosus, rheumatic-like syndromes and various hematologic disorders. These respond promptly to withdrawal of the drug. Hypersensitivity reactions to INH include hepatitis, dermatitis, fever, angiitis and hemolytic anemia. These reactions promptly subside with withdrawal of the drug although in mild reactions this may not be necessary. Corticosteroid therapy or desensitization can be used if INH needs to be read-ministered.

Anticoagulation in infants and children on a ventricular assist device presents particular challenges. Unfractionated heparin has poor bioavailability; it can be difficult to achieve a stable anticoagulant effect; and, in the long-term, there is a risk of osteopenia. Long-term warfarin can be difficult to manage in infants on formula milk with vitamin K supplementation. We review our recent experience with subcutaneous low molecular weight heparin. Two patients received a left ventricular assist device (Excor, Berlin Heart AG) as a bridge to transplantation. Initial anticoagulation consisted of unfractionated heparin infusion beginning 6 hours after implantation to maintain an activated partial thromboplastin time of 70 seconds, checked every 4 to 6 hours. Platelet count (aim >80,000/l) and thromboelastography were assessed daily. Antithrombin required substitution to maintain levels >70 IU/dl. To optimize anticoagulation, both infants were switched to subcutaneous low molecular weight heparin twice daily aiming for an anti-Xa activity between 0.5 and 1.0 IU/ml. Aspirin was added on day 4, checking platelet aggregation every 2 to 4 days, aiming at arachidonic acid stimulated aggregation 10% to 30% of baseline, collagen 100% of baseline. Dipyridamole was added once stability was reached if platelets count exceeded 150,000/l. There were no clinical thromboembolic or bleeding events. Both patients had successful transplantation.