Vascular access is emerging as a critical issue for hemodialysis patients in Puerto Rico. In more than 50% of the hemodialysis patients, tunneled hemodialysis catheters are the sole access for providing dialysis therapy. Most disturbing is the fact that a significant number of these catheters are nontunneled temporary catheters, sometimes placed in the subclavian vein. These facts have contributed significantly to the morbidity and mortality seen in chronic dialysis patients. In addition, many cases of early or late dysfunction of arteriovenous access are not detected and treated in a timely manner due to the lack of a comprehensive vascular access program for end-stage renal disease (ESRD) patients. In fact, monitoring programs to identify and detect vascular access dysfunction are virtually nonexistent in many chronic dialysis units. Even when diagnosed, it is not treated in a timely fashion. Recently literature has shown that procedure-related delays in the treatment of patients with renal disease can be minimized and nephrology care more efficiently delivered by a nephrologist trained in nephrology-related procedures. In an effort to optimize the care of our ESRD patients, we took the initiative to develop an interventional nephrology program that effectively deals with vascular access-related procedures in a timely manner. This approach has minimized delays, decreased hospitalizations and the use of temporary catheters, and improved the medical care of our chronic dialysis patients. So far we have performed more than 400 procedures in the 6 months since the initiation of the program. In this article we describe our initial experience with interventional nephrology in Puerto Rico.
Cardiac myxomas are rare tumors. They usually appear as a sporadic isolated condition in the left atrium of middle-aged women with no other coincidental pathology. Carney and others have described in young people a special complex group of cardiac myxomas associated to a distinctive complex pathology, giving identity to the “Syndrome Myxoma” or “Carney's Syndrome”. Four additional cases of this syndrome, treated from 1977 to 1999 at the Hospital Clínico de la Universidad de Chile are presented here with a comprehensive review of the literature, accumulating 100 cases. The main features of our cases include the presence of malignant non cardiac tumors, a familial trend, follow-up of 23 years and an iterative recurrence in the elder case. To date all patients are tumor free. Reviewing the literature, patients with Carney's Syndrome were younger, with a mean age of 26 years and female predominance (62%). Cardiac myxomas affected the four chambers of the heart: 64% the left atrium; 44% the right atrium; 14% the left ventricle and 12% the right ventricle. They were multiple tumors in 41% and involved more than one chamber in 31%, being synchronous or metachronous. There was a marked familial trend (52%), a high incidence of recurrence (20%), with more than one occurring in half the cases. Extra-cardiac involvement consisted of: 68% pigmented skin lesions, 40% cutaneous myxomas, 37% adrenal cortical disease, 27% myxoid mammary fibroadenoma and 34% male patients with testes tumors. A low percentage had pituitary adenoma, melanotic schwannomas and thyroid disease. The diagnosis is made when two or more of these criteria are present. In agreement with these findings the four chambers of the heart should be examined at surgery for atypical myxoma locations, right atriotomy and combined superior-transseptal approach improve exposure of the cavities, careful screening of the first degree family members should be conducted, and closed short and long term follow up controls are important. Complex myxoma appears as a multi-systemic disorder, occasionally having an ominous prognosis and malignant potentiality, and is still undergoing investigation for better understanding and identification.
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