Current status of surgery for rheumatic carditis in children

Background

The incidence of rheumatic heart disease (RHD) has increased recently in the western United States. We reviewed our 18-year surgical experience with RHD in children to examine current surgical techniques and results.

Methods

From 1985 until 2003, 596 children (<21 years) with rheumatic fever were seen at Primary Children's Medical Center. Rheumatic carditis was diagnosed in 366 patients (61.4%). Twenty-six with carditis (26/366, 7.1%) required operation for rheumatic valve disease including 8 for mitral regurgitation, 7 for mitral and aortic regurgitation, 4 for aortic regurgitation, 4 for mitral regurgitation and stenosis, 2 for combined mitral stenosis and regurgitation with aortic insufficiency, and 1 for mitral and tricuspid regurgitation.

Results

Mean age at operation was 13.5 ± 4 years. Three patients required operation during the acute phase of rheumatic fever (< 6 weeks), 2 during the subacute phase (< 6 months), and 21 during the chronic phase after the episode of rheumatic fever (6.7 ± 3 years). Mitral valve repair was possible in 19 of 22 patients who required mitral operation. Aortic valve repair was possible in 4 patients whereas replacement was necessary in 9, including 2 Ross procedures. No operative deaths were recorded and 2 late deaths occurred at 4.6 and 10 years. Actuarial survival was 94% at 5 years and 78% at 10 years. Six patients required reoperation; actuarial freedom from reoperation was 78% at 5 years, 65% at 10 years, and 49% at 15 years. All survivors are in New York Heart Association class I or II.

Conclusions

Children with RHD in the United States uncommonly require valve operation. Mitral repair with a technique that allows annular growth is possible in most children with good long-term functional results. Long-term surveillance of children with RHD is necessary because of the possible need for late valve operation.     

Reproducibility of linear tumor measurements using PACS: comparison of caliper method with edge-tracing method

The aim of this study was to evaluate inter- and intra-observer reproducibility when making electronic caliper linear tumor measurements on picture archiving and communications systems (PACS) and compare them with linear measurements obtained from circumferential tracing of tumor perimeter. Three radiologists measured 64 masses from 30 patients on body CT scans in two separate settings. Long axis and perpendicular short axis were measured using electronic calipers. The edge of each tumor was traced electronically and the long and short axes were calculated by computer software. The reproducibility of a measurement was evaluated by computing and comparing the absolute value of the mean difference between initial and subsequent measurements. The mean differences ±95% confidence interval (CI) between two measurements of the long by short axis were 3.8±2.6×3.1±1.8 mm when the caliper method was used and 3.5±2.0×3.2±1.5 mm when the tumor tracing method was used. There was no statistically significant difference in individual intra-observer reproducibility of tumor axes measurements. Neither long- nor short-axis single-dimension measurements resulted in significantly greater or lesser intra-observer reproducibility. When comparing caliper and tracing measurements, the overall mean difference (3.42±1.8 vs 3.38±1.4 mm) was not statistically significant. There was close correlation between the individual measurements made by each observer whether these were made by electronic calipers and when these were calculated from electronic tracings (Pearson correlations between 0.79 and 0.949). Current PACS systems allow reproducible linear, long or short axis, tumor measurements. There is no significant difference in reproducibility of measurements whether these are made directly with electronic calipers or calculated from tumor edge tracings.     

Thyrotrophin estimation in diagnosis and treatment of childhood thyroid disorders

Serum thyrotrophin (TSH) was estimated by double-antibody radioimmunoassay in 200 children aged 2 months to 16 years with normal thyroid function. There was no apparent variation in TSH with age or sex and only 4 children had TSH levels greater than 5 muU/ml. High TSH values were obtained in 9 children with primary hypothyroidism, in 3 children with thyroiditis, and in one girl with a lingual thyroid. Moderately raised TSH was found in 3 girls with thyroiditis, 2 brothers with goitres due to enzyme defect, and a girl with an ectopic thyroid. In one girl with a defect of iodine organification and in 3 boys with thyroxine binding globulin deficiency the TSH levels were normal despite very low serum thyroxine values. Serum TSH was also estimated in 20 children during treatment for primary hypothyroidism. 3 of these children showed slightly raised TSH levels despite apparently adequate replacement therapy with L-thyroxine. One girl showed a very high TSH level 3 weeks after treatment had been temporarily withdrawn.     

Communicating hydrocephalus secondary to venous complications following intraatrial baffle operation (mustard procedure) for d-Transposition of the great arteries

Summary A 39-month-old male had macrocephaly and communicating hydrocephalus secondary to superior vena cava (SVC) obstruction which was a postoperative complication of an intraatrial baffle operation (Mustard procedure) performed at age 10 months. Computerized tomography revealed progressive hydrocephalus. Cardiac catheterization showed an SVC pressure of 24 mm Hg. IVC and systemic vein atrium pressures were 5 mm Hg. Cineangiograms showed huge azygos collaterals from SVC to IVC. The operation was revised using a Dacron baffle instead of the original pericardial baffle. Nine months postoperatively, the head circumference was 55.0 cm and there was no clinical evidence of SVC obstruction.Supported by American Heart Association grant 78-1217     

Efficacy of OKT3 retreatment for refractory cardiac allograft rejection

Although OKT3 monoclonal antibody is a useful therapy for refractory cardiac allograft rejection, the use of OKT3 for prophylaxis may be limited by the potential of sensitization and subsequent loss of efficacy on retreatment. OKT3 was required for refractory rejection in 21 of 165 recipients transplanted between March 1985 and August 1988. Twelve of these patients had previously been exposed to OKT3, and the retreatment efficacy was evaluated. The study population averaged 42.1 +/- 15.3 years of age (mean +/- SEM) and had experienced 2 +/- 1 previous episodes of rejection. The prior episodes of rejection had been treated with pulse methylprednisolone and antithymocyte globulin, and in addition 3 patients (25%) also required a course of antilymphoblast globulin. Retreatment OKT3 for refractory rejection was required 120 +/- 94 days following transplantation. CD3+ lymphocytes were eliminated from the circulation within 24-48 hr in 11 of 12 patients, all of whom showed histologic improvement within the first week. Total resolution on the initial follow-up biopsy was noted in 9 (75%) during the course of therapy. Subsequent rejection episodes occurred in 9 (82%) of the survivors at 71 +/- 64 days. One-year survival was 83% in this vigorously rejecting patient population. Serious infections occurred within 3 months of therapy in 4 (36%). The side effects of OKT3 retreatment were similar to those seen with first exposure and did not require OKT3 discontinuation. Thus OKT3 may be administered with success in most patients who have previously been exposed to it.     

筛查和诊断糖尿病的新观点

美国约有30％的糖尿病患者（约620万人）被漏诊。超过25％的新诊断糖尿病患者已经患有糖尿病视网膜病变或出现微量白蛋白尿，这意味着患者在2型糖尿病发病和确诊之间被“忽视”了7年。有时发现在糖尿病前期，即空腹血糖（FPG）略有偏高（IFG，空腹血糖受损）或口服葡萄糖耐量试验（OGTT）略有偏高，甚至已存在微血管和大血管并发症。