Fibrolipomatosis of varying degree was observed in 24 transplanted kidneys. In 8 patients there was no history of urinary tract obstruction or multiple infections, leaving earlier rejection periods as the probable factor responsible for development of fibrolipomatosis after transplantation.
Neurofibromatosis is a congenital and hereditary disease. It may involve any organ of neuroectodermal or mesodermal origin. Manifestations of the skin, subcutaneous tissue and bones are common. Neurofibromatosis of the gastrointestinal tract may result in complications such as obstruction, bleeding or malignancy.1-9 The disease may involve the vascular system10-13 and cause coarctation of the aorta, stenotic or aneurysmal changes in arteries, and may-if renal arteries are involved-result in hypertension. 14-19The purpose of the present communication is to emphasize the importance of angiography in generalized neurofibromatosis with hypertension and for determination of the source of the gastrointestinal bleeding. We intend, however, also to show that in a patient with generalized neurofibromatosis vascular or intestinal lesions detected at angiography, and resembling those described in generalized neurofibromatosis may really be of different etiology. CASE REPORTS CASE 1. 23 year old male with generalized neurofibromatosis (caf6 au lait spots, previous right leg amputation for &dquo;foot anomaly&dquo;, bone lesions of spine and ribs). Blood pressure between 160-170 mm Hg systolic and 106--130 diastolic. An aortogram at another hospital had demonstrated a superior mesenteric artery aneurysm, and multiple renal artery aneurysms. The patient was admitted to the University of Alabama Hospital for further hypertensive work-up. Three urinary VMA determinations and an intravenous pyelogram were normal. The renal vein renin values were elevated, 725 nonnog/1 on the left and 555 nonnog/1 on the right. Bilateral selective renal angiograms demonstrated six saccular aneurysms of the right renal artery (Fig. 1). The largest of the aneurysms measured 2 cm. A single small aneurysm originated from the left main renal artery base (Fig. 2). The bilateral renal artery disease was considered not correctable by surgery and the patient was discharged with medical antihypertensive treatment. CASE 2. 41 year old female with generalized neurofibromatosis (caf6 au lait spots, cutaneous neurofibromata, bone lesions of skull, spine and left femur) was admitted to the University
In 61 patients (167 examinations) the pulsatile flow index (PFI) was used to diagnose the cause of renal transplant dysfunction. The results were correlated with histology and clinical course and outcome, angiography or quantitative radionuclide renography. Renal transplant rejection was diagnosed by PFI with a sensitivity of 85%. The specificity was 81% and the diagnostic accuracy 83%. The positive predictive value was found to be 76%, whereas the negative predictive value was 89%. In presence of acute tubular necrosis (ATN) the PFI was normal in 89% of examinations and therefore distinguishable from acute rejection.
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