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      Pediatric endocrine hypertension

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          Abstract

          Endocrine causes of hypertension are rare in children and screening for endocrine hypertension in children should be carried out only after ruling out renal and renovascular causes. Excess levels and/or action of mineralocorticoids associated with low renin levels lead to childhood hypertension and this can be caused by various conditions which are discussed in detail in the article. Childhood pheochromocytomas are being increasingly diagnosed because of the improved application of genetic testing for familial syndromes associated with pheochromocytomas. Adolescents with polycystic ovarian syndrome (PCOS) can also have hypertension associated with their obese phenotype.

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          Human hypertension caused by mutations in WNK kinases.

          F. Wilson, S Disse-Nicodème, K A Choate … (2001)
          Hypertension is a major public health problem of largely unknown cause. Here, we identify two genes causing pseudohypoaldosteronism type II, a Mendelian trait featuring hypertension, increased renal salt reabsorption, and impaired K+ and H+ excretion. Both genes encode members of the WNK family of serine-threonine kinases. Disease-causing mutations in WNK1 are large intronic deletions that increase WNK1 expression. The mutations in WNK4 are missense, which cluster in a short, highly conserved segment of the encoded protein. Both proteins localize to the distal nephron, a kidney segment involved in salt, K+, and pH homeostasis. WNK1 is cytoplasmic, whereas WNK4 localizes to tight junctions. The WNK kinases and their associated signaling pathway(s) may offer new targets for the development of antihypertensive drugs.
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            Recent advances in genetics, diagnosis, localization, and treatment of pheochromocytoma.

            K Pacak, Sten Walther, Bernard Goldstein … (2001)
            Pheochromocytoma is a rare but important tumor of chromaffin cells that is frequently considered in the evaluation of hypertension, arrhythmias, or panic disorder and in the follow-up of patients with particular genetic diseases. This report provides an update about the genetics, neurochemical diagnosis, localization by imaging, and surgical management of pheochromocytoma. Specific mutations of the RET proto-oncogene cause familial predisposition to pheochromocytoma in multiple endocrine neoplasia type II, and mutations in the von Hippel-Lindau tumor suppressor gene cause familial disposition to pheochromocytoma in von Hippel-Lindau disease. Recent findings demonstrating extraordinarily high sensitivity of plasma levels of metanephrines for detecting pheochromocytoma have led to an algorithm for clinical diagnostic steps. Nuclear imaging approaches, such as(123) I-metaiodobenzylguanidine scintigraphy and 6-[(18) F]fluorodopamine positron emission tomography, enhance both diagnosis and localization of the tumor, as described in an algorithm for patients with positive biochemical test results. Since pheochromocytoma is often benign, surgical resection by laparoscopic adrenalectomy can be curative. Areas requiring further work include determining appropriate follow-up of patients with familial pheochromocytoma, elucidating the bases for phenotypic differences, improving both specificity and sensitivity of biochemical tests, optimizing cost-effectiveness of diagnostic imaging, and testing the risk for tumor recurrence after partial adrenalectomy.
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              Hypertension in pheochromocytoma: characteristics and treatment.

              Karel Pacak, M Zuber, V Kantorovich (2011)
              Pheochromocytoma is a tumor of the chromaffin cells in the adrenal medulla and sympathetic paraganglia, which synthesizes and secretes catecholamines. Norepinephrine, epinephrine, and dopamine all act on their target receptors, which causes a physiologic change in the body. High circulating levels of catecholamines can lead to severe hypertension and can have devastating effects on multiple body systems (eg, cardiovascular, cerebrovascular), and can lead to death if untreated. Although surgical treatment represents the only modality of ultimate cure, pharmacologic preoperative treatment remains the mainstay of successful outcome. Published by Elsevier Inc.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Indian J Endocrinol Metab
                Journal ID (publisher-id): IJEM
                Title: Indian Journal of Endocrinology and Metabolism
                Publisher: Medknow Publications & Media Pvt Ltd (India )
                ISSN (Print): 2230-8210
                ISSN (Electronic): 2230-9500
                Publication date (Print): October 2011
                Volume: 15
                Issue: Suppl4
                Pages: S361-S366
                Affiliations
                [1] Department of Endocrinology and Diabetes, Amrita Institute of Medical Sciences, Cochin, Kerala, India
                Author notes
                Corresponding Author: Dr. Nisha Bhavani, Department of Endocrinology and Diabetes, Amrita Institute of Medical Sciences, Cochin – 682 041, Kerala, India. E-mail: nishab@ 123456aims.amrita.edu
                Article
                Publisher ID: IJEM-15-361
                DOI: 10.4103/2230-8210.86980
                PMC ID: 3230094
                PubMed ID: 22145140
                SO-VID: 9f2c050e-d0a1-48a1-b279-65c2e7be1a57
                Copyright © Copyright: © Indian Journal of Endocrinology and Metabolism
                License:

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Categories
                Subject: Review Article

                ScienceOpen disciplines: Endocrinology & Diabetes
                Keywords: pediatric endocrine hypertension,essential hypertension,aldosterone,adrenal
                Data availability:
                ScienceOpen disciplines: Endocrinology & Diabetes
                Keywords: pediatric endocrine hypertension, essential hypertension, aldosterone, adrenal

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