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Extreme Blood Pressure Fluctuations in a Patient with Intact Autonom Ic Reflexes a Nd Intact Sod Iu M Conservation 1

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Extreme Blood Pressure Fluctuations in a Patient with Intact Autonom Ic Reflexes a Nd Intact Sod Iu M Conservation 1 EDITORIAL COMMITIEE Tomas Berl, Editor William Henrich Mark PaIIer Fred Silva Denver, CO Toledo, OH Minneapolis, MN Oklahoma City, OK DESCRIPTION OF THE NEPHROLOGY TRAINING PROGRAM UNIVERSITY OF FLORIDA COLLEGE OF MEDICINE The Division of Nephrology. Hypertension and Transplantation at the University of Florida has a distinguished faculty of 1 1 individuals who are involved in patient care. research. and teaching activities. Clinical fellows are exposed to an active diagnostic and treatment service drawing from 548 beds in Shands Hospital. 433 beds in the adjacent Gainesville Veterans Affairs Medical Center. and the ambulatory care facilities from both hospitals. The clinical rotations are designed to emphasize the diagnosis and management of renal disease and fluid and electrolyte disorders. Over 100 diagnostic renal biopsies are performed each year. providing extensive experience in biopsy interpretation. The dialysis program provides fellows with the opportunity to manage acute renal failure by using a variety of techniques, including hemodialysis. hemofiltration, CAVH, and CAVHD. An outpatient chronic hemodialysis population of 70 patients and an ambulatory peritoneal dialysis population of 45 patients provide practical experience in the management of ESRD requiring dialysis. The division supports an active transplant program that performs 125 kidney transplants each year. A clinical hypertension service provides in-depth clinical and research opportunities for fellows in the field of hypertension. Clinical and research training is a major focus of division activities. and three to four fellows are enrolled each year in the academic program of the division. Fellows are offered the opportunity to obtain research experience in one of several research laboratories in the division. The faculty can offer extensive research experience in renal physiology. renal pathology. cell biology and biochemistry. molecular biology. pharmacology. transplant immunobiology. hypertension. and related disciplines. Currently. there is also active collaboration in areas of mutual interest with faculty members in the departments of pharmacology. biochemistry and molecular biology, pathology, and physiology, and fellows have the opportunity to participate in these activities. A particularly strong aspect of our training program is the extensive one-on-one interaction fellows enjoy with the faculty in both the clinical and the laboratory seffing. The division also offers a special 1-yr transplant fellowship that provides the trainee with an in-depth experience in all phases of solid organ transplantation. In addilion. the extensive inpatient and outpatient exposure to the management of transplant recipients and their donors allows the trainee to become familiar with tissue typing and organ procurement. Opportunities are also provided by the faculty for the fellows to pursue either clinical or laboratory investigation. Extreme Blood Pressure Fluctuations in a Patient With Intact Autonom ic Reflexes a nd Intact Sod iu m Conservation 1 Yousri M. Barn,2 Marian C. Limacher, and Christopher S. Wilcox tion while supine or standing. In contrast, the hyper- Y.M. Barn, 0.5. Wilcox, Division of Nephrology, Hyper- tensive episodes were associated with marked tachy- tension and Transplantation, University of Florida, cardia, sweating, anxiety, abdominal pain, and very Gainesville, FL high levels of plasma norepinephrine concentration. MC. Limacher, Division of Cardiology, Universily of Extensive investigations failed to support a diagnosis Florida, Gainesville, FL of pheochromocyfoma. The testing of baroreceptor function and autonomic reflexes was normal. Blood (J. Am. Soc. Nephrol. 1995; 6:1347-1353) pressure was not salt sensitive. It was concluded that this patient has a unique clinical syndrome of ex- ABSTRACT treme fluctuation of blood pressure and sympathetic A patient who had episodes of profound hypotension nervous activity yet intact cardiovascular reflexes and alternating with severe hypertension without an obvi- normal sodium conservation. The abnormal blood ous precipitating cause is reported. The hypotensive pressure regulation most likely has a central origin. episodes were accompanied by tiredness, syncope, Key Words: Hypotension. hypertension. autonomic. function. bradycardia, and a low norepinephrine concentra- baroreflex. norepinephrine 1 Received October 3, 1994. Accepted March 13, 1995. B lood pressure (BP) changes little with posture in 2 Correspondence to Dr. Y.M. Barn, Department of Medicine, Presbyterian Hos- normal human subjects because of barorefiex pifal of Dallas, 8200 Walnut Hill Lane, Dallas, TX 75231. mechanisms that adjust the sympathetic and para- 1046.6673/0605-1347103.00/0 sympathetic nervous discharge. The changes in heart Journal of the American Society of Nephrology Copyright © 1995 by the American Society of Nephrology rate that occur on standing up or lying down are due Journal of the American Society of Nephrology 1347 Extreme Blood Pressure Fluctuations for the most part to this barorefiex mechanism. Severe fundi was negative for evidence of diabetic or hyper- fluctuations in BP in the absence of changes in blood tensive retinopathy. Otherwise, the examination was volume usually imply interruption in this baroreflex unremarkable including a normal neurologic exami- mechanism. For example. patients with autonomic nation. failure experience severe orthostatic hypotension, sometimes accompanied by supine hypertension. The Laboratory Investigations plasma catecholamine levels are normal or sup- Initial laboratory data revealed normal values for pressed. Excessive release of catecholamines can oc- serum creatinine, BUN, electrolytes, urinalysis, creat- cur with baroreflex failure or with pheochromocytoma mine clearance, and liver function tests. There was no and is associated with episodic or sustained hyperten- evidence of hypoglycemia during repeated tests, and sion. On other occasions, pheochromocytoma can most blood sugar levels ranged between 80 and 200 cause hypertension alternating with hypotension and mg/dL. tachycardia. In this study, we describe a patient with episodes of hypotension alternating with hypertension Cardiac Evaluation without evidence of pheochromocytoma who has ex- She had a normal electrocardiogram, a normally treme fluctuation in plasma catecholamine levels, in- functioning pacemaker, and an echocardiogram that tact autonomic reflexes, and normal salt conserva- revealed normal cardiac valves, and normal cardiac tion. output while lying and after head-up tilt of6O degrees. CASE REPORT Neither head-up tilt nor isoproterenol infusion in- duced syncope. Clinical History Our patient is a 67-yr-old female retired book- Evaluation for Pheochromocyfoma keeper. For 1 yr. she has had intermittent episodes Plasma catecholamines were measured on several that last several days at a time of orthostatic dizziness occasions, particularly during the spontaneous epi- with syncope and falls, accompanied by weakness and sodes of hypertension and hypotension. On none of lethargy. At these times, her systolic BP is typically 55 these occasions was she taking any medication. The to 90 mm Hg with a heart rate of 60 to 70 beats/mm. plasma norepinephrine levels were remarkably van- These symptoms alternate with episodes of head- able and, as shown in Figure 1 , correlated closely with aches, sweating. palpitations. anxiety, nausea, vomit- the level of systolic BP. The normal range of plasma Ing, and abdominal pain. At these times, her systolic norepinephrine is 1 10 to 700 pg/mL. When her sys- BP is typically 1 60 to 220 mm Hg with a heart rate of tolic BP was low, the plasma norepinephrine concen- 100 to 160 beats/mm. During the hypotensive or tration was frequently below the limit of normal, hypertensive episodes, there is little orthostatic fall in whereas when it was very high, it was frequenily well BP and standing is accompanied by an appropriate above the upper limit of normal. A clonidine suppres- increase in heart rate. She has required frequent sion test was performed on two occasions to further hospital admissions, averaging once a month, pre- investigate the diagnosis of pheochromocytoma (Fig- dominantly for hypotenslon. On one occasion, she had ure 2). On one occasion (during a hypotensive epi- a syncopal episode, fell, and fractured her left lateral sode), plasma norepinephrine was below normal and malleolus. She denies flushing. diarrhea, visual symp- toms, fever, chest pain. or shortness of breath. She 200 was referred to our Institution for a further work-up. In 1983, she was diagnosed to have sick sinus syn- 180 drome and a demand pacemaker was inserted. Car- . diac catheterization at that time was normal. In 1986, 160 she developed diabetes mellitus that was well con- -a.--- . trolled with insulin therapy. One year before presen- 120 tation, she developed Staphylococcus aureus endocar- . ___ ditis, which was successfully treated with antibiotics. Q. 100 #{149} 00 . She is a nonsmoker, and she denies alcohol or illicit Cl) o #{149} r= +0.69 drug abuse. Her only regular medication at presenta- . n=16 p<0.01 tion was insulin (25 U of NPH in the morning and 1 2 U 60 in the evening, and 12 U of regular in the morning). 40 Physical Examination 20 0 200 400 600 800 1000 1200 1400 1600 1800 2000 2200 2400 2600 Physical examination during the first clinic visit Plasma Norepinephrlne (pg #{149}mr1) when she was quite asymptomatic revealed a normal affect, a lying BP of 156/66, a lying heart rate of 78
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