Patients with congestive cardiomyopathy as cardiac transplant recipients. Indications for and results of cardiac transplantation and comparison with patients with coronary artery disease.

In recent years end-stage congestive cardiomyopathy has become an increasingly frequent clinical diagnosis in candidates for cardiac transplantation. Forty-six patients who underwent transplantation because of congestive cardiomyopathy and 59 because of coronary artery disease were studied between 1971 and 1978 at Stanford University. The overall 1 year survival rate was similar in the two groups: cardiomyopathy-transplant, 64 percent and coronary artery disease-transplant, 55 percent. The survival rate has improved substantially for both groups within the last decade: The 3 year survival rate for cardiomyopathy-transplant patients undergoing cardiac transplantation since 1974 is nearly 60 percent. In contrast, 36 similarly ill patients with cardiomyopathy not undergoing transplantation had a 1 year survival rate of 23 percent and a 3 year survival rate of 4 percent (p less than 0.001). Survival rates in the cardiomyopathy-transplant group were unaffected by age (greater or less than 40 years). Patients in this group under age 40 had a lower frequency of infection (1 per 313 patient-days versus 1 per 195 patient-days in the older group, p less than 0.05) and a significantly longer interval to second rejection episodes (p less than 0.05), a measure of rejection frequency. Cardiomyopathy-transplant patients under age 40 had fewer deaths due to rejection (17 percent) compared with older patients in this group (36 percent). Cardiac transplantation is an effective treatment for end-stage congestive cardiomyopathy.

Anomalies in an infant with Nager acrofacial dysostosis.

We report on an infant with Nager acrofacial dysostosis, laryngeal and epiglottic hypoplasia, abnormal septation of the right middle lobe of the lung, hypoplastic right first rib, and dislocation of the right hip. These findings suggest the possibility that patients with the Nager syndrome may have other developmental defects in addition to the facial and acral anomalies associated with this syndrome.

Immune complex-induced enteropathy. Effects of repeated injections of immune complexes on the small intestine of the rat.

Rats injected intravenously with immune complexes (IC) prepared in 5x antigen excess were reported previously to develop a distinctive striped pattern of serosal hyperemia of the small intestine accompanied by mucosal edema and hemorrhage. This study tested the effect of repeated injections of IC given at 3-day intervals. After the last of four, six, or nine injections, rats continued to show the same gross and histologic lesions observed in rats injected once with IC. After serial injections, however, the distal small intestine was more severely involved than the proximal intestine. In addition, villous shortening, stromal hemosiderin deposits, and gut-associated lymphoid tissue and mesenteric lymph node sinus hemorrhage and hemosiderin deposits were seen in rats given nine injections. There was a remarkable absence of stromal infiltration of the villi by inflammatory cells. Experiments conducted with 125I-labeled IC plus sufficient "cold" IC to induce intestinal lesions showed that IC were deposited at extravascular sites in the gut. The failure of such IC to provoke cellular infiltration in the gut is unexplained.

Toxicity but not arthritogenicity of Mycoplasma arthritidis for mice associates with the haplotype expressed at the major histocompatibility complex.

The use of inbred and congenic mouse strains established that toxicity and death induced by Mycoplasma arthritidis associates with the haplotype expressed at the murine major histocompatibility complex. Mice bearing H-2k and H-2d are susceptible, whereas those bearing H-2b are much more resistant. Mice susceptible to toxicity exhibited massive peritoneal adhesions and a decreased ability to clear organisms from the peripheral circulation. However, the severity of acute arthritis developing over a 3-month period was not statistically related to the haplotype expressed at the major histocompatibility complex. Lymphocyte activation in vitro by a soluble T-cell mitogen is also dependent on a similar haplotype expression.

Thymic cysts in mediastinal Hodgkin disease.

Three cases of proved thymic cysts associated with mediastinal Hodgkin disease are presented. Two illustrate regression of lymphoma with chemotherapy but persistence of thymic cysts. The third case demonstrates a thymic cyst in untreated Hodgkin disease. These cases suggest that such cysts are probably neither coincidental with nor a consequence of therapy but are probably related to initial thymic involvement by Hodgkin disease.