Hyperreninism

Classification according to ICD-10
E26	Hyperaldosteronism
D41.0	New formation of unsafe or unknown behavior: Kidney - Juxtaglomerular Tumor - Reninoma
ICD-10 online (WHO version 2019)

The Hyperreninismus is a disease with increased renin in the blood plasma due to an endocrine tumor usually the kidney . Clinically, arterial hypertension is in the foreground. Usually the new formation starts from the juxtaglomerular apparatus (juxtaglomerular cell tumor), e.g. B. hemangiopericytoma , nephroblastoma , much less often from other organs , e.g. B. Small cell lung cancer .

Synonyms are: Robertson-Kihara syndrome; Primary hyperreninism; Reninoma; Hyperreninemia

The name refers to the authors of second descriptions from 1967 by the internist and pathologist PW Robertson and colleagues and from 1968 by I. Kihara and colleagues. The name was proposed in 1972 by Jerome W. Conn .

Occurrence

Juxtaglomerular cell tumors can occur at any age, more often in the 2nd and 3rd decades of life, and women are more frequently affected.

root cause

Hyperreninism is caused by a hormone-active neoplasia, mostly of the juxtaglomerular apparatus: hemangiopericytoma, nephroblastoma, less often with small-cell bronchial carcinoma, ovarian leiomyosarcoma or others.

The overproduction of renin leads to arterial hypertension and secondary hyperaldosteronism .

Clinical manifestations

Clinical criteria are:

Therapy-resistant high blood pressure with headache , changes in the fundus , left ventricular hypertrophy
normal kidney function
Hypernatremia
Hypokalaemia with myasthenia , constipation , decreased muscle reflexes , polydipsia and polyuria
increased renin and aldosterone in blood plasma

diagnosis

The diagnosis can be made by separate renin determination from the kidney venous blood.

Differential diagnostics

The following are to be distinguished:

as well as imaging other kidney tumors such as:

therapy

The treatment is performed surgically .

literature

R. Kreutz, H. Zhou, U. Pfeifer, JM Gasc, D. Ganten, FJ Kessler: Primary hyperreninism: a rare cause of secondary arterial hypertension. In: DMW - German Medical Weekly. 118, 2008, p. 1110, doi: 10.1055 / s-2008-1059433 .
PP Nawroth, Reinhard Ziegler (Ed.): Clinical Endocrinology and Metabolism. , P. 515, Springer, 2001. doi: 10.1007 / 978-3-642-56784-1 ISBN 978-3-642-62970-9 eBook ISBN 978-3-642-56784-1

Individual evidence

↑ ^a ^b ^c ^d Pschyrembel online
↑ ^a ^b ^c Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
↑ PW Robertson, A. Klidjian, LK Harding, G. Walters, MR Lee, AH Robb-Smith: hypertension due to a renin-secreting renal tumor. In: The American journal of medicine. Volume 43, Number 6, December 1967, pp. 963-976, doi: 10.1016 / 0002-9343 (67) 90256-2 , PMID 6060417 .
^ I. Kihara, S. Kitamura, T. Hoshino, H. Seida, T. Watanabe: A hitherto unreported vascular tumor of the kidney: a proposal of "juxtaglomerular cell tumor". In: Acta pathologica japonica. Volume 18, Number 2, May 1968, pp. 197-206, PMID 5755102 .
↑ JW Conn, EL Cohen, WJ McDonald, WM Blough, CP Lucas, GH Mayor, WC Eveland, JJ Bookstein, J. Lapides: The Syndrome of Hypertension, Hyperreninemia and Secondary Aldosteronism Associated with Renal Juxtaglomerular Cell Tumor (primary Reninism). In: Journal of Urology. 109, 1973, p. 349, doi: 10.1016 / S0022-5347 (17) 60423-3 .
↑ ^a ^b Radiopaedia
↑ PW Anderson, L. Macaulay, YS Do, A. Sherrod, G. d'Ablaing, M. Koss, T. Shinagawa, B. Tran, FJ Montz, WA Hsueh: Extrarenal renin-secreting tumors: insights into hypertension and ovarian renin production. In: Medicine. Volume 68, Number 5, September 1989, pp. 257-268, doi: 10.1097 / 00005792-198909000-00001 , PMID 2677594 .
^ R. Pursell: Secondary hypertension due to a renin-producing teratoma. In: American Journal of Hypertension. 16, 2003, p. 592, doi: 10.1016 / S0895-7061 (03) 00865-3 .
↑ Collective canal carcinoma. In: Orphanet (Rare Disease Database).

[pschy-1] Pschyrembel online

[Leiber-2] Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .

[3] PW Robertson, A. Klidjian, LK Harding, G. Walters, MR Lee, AH Robb-Smith: hypertension due to a renin-secreting renal tumor. In: The American journal of medicine. Volume 43, Number 6, December 1967, pp. 963-976, doi: 10.1016 / 0002-9343 (67) 90256-2 , PMID 6060417 .

[4] I. Kihara, S. Kitamura, T. Hoshino, H. Seida, T. Watanabe: A hitherto unreported vascular tumor of the kidney: a proposal of "juxtaglomerular cell tumor". In: Acta pathologica japonica. Volume 18, Number 2, May 1968, pp. 197-206, PMID 5755102 .

[5] JW Conn, EL Cohen, WJ McDonald, WM Blough, CP Lucas, GH Mayor, WC Eveland, JJ Bookstein, J. Lapides: The Syndrome of Hypertension, Hyperreninemia and Secondary Aldosteronism Associated with Renal Juxtaglomerular Cell Tumor (primary Reninism). In: Journal of Urology. 109, 1973, p. 349, doi: 10.1016 / S0022-5347 (17) 60423-3 .

[radio-6] Radiopaedia

[7] PW Anderson, L. Macaulay, YS Do, A. Sherrod, G. d'Ablaing, M. Koss, T. Shinagawa, B. Tran, FJ Montz, WA Hsueh: Extrarenal renin-secreting tumors: insights into hypertension and ovarian renin production. In: Medicine. Volume 68, Number 5, September 1989, pp. 257-268, doi: 10.1097 / 00005792-198909000-00001 , PMID 2677594 .

[8] R. Pursell: Secondary hypertension due to a renin-producing teratoma. In: American Journal of Hypertension. 16, 2003, p. 592, doi: 10.1016 / S0895-7061 (03) 00865-3 .

[9] Collective canal carcinoma. In: Orphanet (Rare Disease Database).