Congestive heart failure, hypertension, and hyperreninemia in bilateral Wilms' tumor: Successful medical management

Stine, Kimo C.; Goertz, Kenneth K.; Poisner, Alan M.; Lowman, James T.

doi:10.1002/mpo.2950140115

Cited by 23 publications

(23 citation statements)

References 10 publications

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“…The incidence of hypertension has been reported as high as 60% in some series [2]. Its development has commonly been ascribed either to catecholamine production or to hyperreninemia and can lead to dilated cardiomyopathy, and consequently congestive heart failure [3][4][5].…”

Section: Discussionmentioning

confidence: 98%

Wilms tumor and cardiomyopathy

Trebo

Mann

Dworzak

et al. 2003

Med. Pediatr. Oncol.

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It is well known that renal tumors may be associated with hypertension due to hyperreninemia and/or raised catecholamines, which in extreme cases may lead to congestive heart failure. We, however, encountered two Wilms tumor patients with dilated cardiomyopathy, who displayed no evidence of hyperreninemia or raised catecholamines, and in one not even hypertension.We conducted a retrospective case analysis on both children with special emphasis on cardiovascular physiology and activity of the renin/catecholamine system. Heart function was assessed by clinical examination and trans-thoracic echocardiography (TTE) immediately at admission, and then at regular intervals during and after therapy. In detail, the following parameters were determined: left ventricular enddiastolic diameter (LVEDd), and left ventricular shortening fraction (SF). The functional status of the neuro-endocrine system was evaluated by measurement of serum levels of renin, and of urinary levels of vanillylmandelic acid (VMA), homovanillic acid (HVA), 5-hydroxyindoleacetic acid (5-HIAA), epinephrine, norepinephrine, and dopamine. Further details follow.The first patient was a 2.5-years-old Caucasian girl, who presented with a right-sided Wilms tumor stage IV; her blood pressure was 100 mmHg systolic. A routine pre-treatment TTE examination revealed a dilated cardiomyopathy with an increased LVEDd (¼ 42 mm), decreased SF (¼ 22%), and a hypokinetic septum without evidence of anomalous intracardiac anatomy; ECG was normal and showed no signs of left ventricular hypertrophy. The serum level of renin and the urinary levels of VMA, HVA, 5-HIAA, epinephrine, norepinephrine, and dopamine were not elevated. After chemotherapy, following the SIOP 93-01/GPOH protocol (modified by exclusion of doxorubicin, and by inclusion of etoposide and carboplatin), and complete tumor excision plus radiation therapy (total dose of 20 Gy), the patient is in complete remission. The histologic examination of the specimen revealed a classic triphasic Wilms tumor stage pT2b. In the early postoperative period, the LV function started to return gradually to normal with a LVEDd of 38 mm and a SF of 35% at 3 years after presentation. The second child was an 8-months-old Caucasian girl, who presented with a right-sided Wilms tumor and arterial hypertension of up to 154 mmHg systolic. Standard ECG showed abnormalities of repolarization, but no signs of hypertrophy. TTE revealed a normal intracardiac anatomy, and normal LVEDd (¼ 29.7 mm), but a significantly reduced SF of 22.41%. The urinary levels of VMA, HVA, and dopamine were not elevated. Complete resection of the abdominal mass could be performed after reduction of blood pressure by captopril. Histology of the operative specimen showed a partially differentiated cystic Wilms tumor, stage pT1. Therefore, further chemotherapy was needless. Since operation the patient is in complete remission. At 12 days after operation, the LV function started to return gradually to normal with a SF of 34.6% at 2 months after presentation.

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Section: Discussionmentioning

confidence: 98%

Wilms tumor and cardiomyopathy

Trebo

Mann

Dworzak

et al. 2003

Med. Pediatr. Oncol.

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“…Symptomatic hypertension with hyperreninemia is rare in children [2] and usually is related to a renin-pro ducing tumor [3] or renal artery stenosis. In this patient, the hyperreninemia was thought to be due to focal inter stitial nephritis of the right kidney.…”

Section: Discussionmentioning

confidence: 99%

Hyperreninemia, Hypertension, and Congestive Heart Failure in Focal Interstitial Nephritis

Matsui

Ono²,

Nakagawa³

1996

Nephron

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Hyperreninemia probably due to focal interstitial nephritis of the right kidney caused an acute onset of hypertension and cardiac dysfunction including valvular insufficiency in a 5-year-old boy. This congestive heart failure in association with valvular insufficiency and hypertension was dramatically improved following right nephrectomy. Abruptly activated renin-angiotensin II may lead to congestive heart failure and valvular dysfunction without myocardial hypertrophy in a young heart.

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“…Although there had been several reports of increased renin activity in serum or tissues of patients with renal tumors using bioassays for analysis [27,28], none had followed the clinical course and biochemical evaluation of the patients and utilized agents interfering with angiotensin to modulate the course of the disease. We reported on a patient with bilateral Wilms tumor (nephroblastoma) who exhibited congestive heart failure, hypertension and elevated serum renin using a more contemporary radioimmunoassay and an international standard [29]. We found the patient's clinical course and tumor size in response to surgery and chemotherapy were paralleled by serum renin concentrations (PRC) and his hypertension was ameliorated by saralasin, a peptide angiotensin receptor antagonist (this was before non-peptide ARBs were available).…”

Section: Renin In Human Tumors 41 Enzymatic Analysismentioning

confidence: 99%

From Angiotensin to Renin to Prorenin and from the Adrenal to the Kidney to the Placenta and the Lungs: An Historical Journey

Poisner¹

2020

Selected Chapters From the Renin-Angiotensin System

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In 1966 I carried out a study on the role of calcium on angiotensin's stimulant effects on the adrenal medulla. Since then I have been studying the reninangiotensin system (RAS) for over a half-century in a wide variety of biological preparations, while awareness of its complexity has exploded. My journey has involved studies on genes, proteins, organelles, cells, tissues, glands, organs and whole animals. This chapter reviews what my colleagues and I have learned from these different levels of organization and is not meant to be an update on all features of the RAS. My studies have included experiments on: perfused cat adrenal glands; genetic and second messenger control of catecholamine synthesis and secretion from cultured bovine chromaffin cells and from rats in vivo; renin storage and release in the rat kidney and secretory granules; properties of isolated renin, prorenin and renin-like proteins; hormonal and second messenger control of prorenin secretion from human utero-placental tissues; renin/prorenin in a variety of tumors; and the effect of RAS drugs in a rodent model of pulmonary fat embolism. This most recent study has direct clinical application. I conclude with what I have learned about biomedical research and lessons for the future.

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Congestive heart failure, hypertension, and hyperreninemia in bilateral Wilms' tumor: Successful medical management

Cited by 23 publications

References 10 publications

Wilms tumor and cardiomyopathy

Wilms tumor and cardiomyopathy

Hyperreninemia, Hypertension, and Congestive Heart Failure in Focal Interstitial Nephritis

From Angiotensin to Renin to Prorenin and from the Adrenal to the Kidney to the Placenta and the Lungs: An Historical Journey

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