{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T02:43:38Z","timestamp":1760237018064,"version":"build-2065373602"},"reference-count":36,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2020,2,20]],"date-time":"2020-02-20T00:00:00Z","timestamp":1582156800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicines"],"abstract":"<jats:p>Background: Fibromuscular dysplasia (FMD) is one of the important etiologies of renovascular hypertension in children. It is usually resistant to multiple antihypertensive agents and can cause extreme elevation in blood pressures, which can lead to end organ damage if not promptly diagnosed and treated. Treatment options include medical management with antihypertensive agents, balloon or stent angioplasties, surgical revascularization, and nephrectomy. The aim of the study was to review the efficacy of antihypertensive therapy only in the management of FMD in a very young child. Methods: This is a retrospective chart study with review of literature. Results: Here, we report a 22-month-old toddler who presented with severe resistant hypertension and cardiomyopathy who was found to have focal FMD of the right renal artery. She also presented with proteinuria, hyponatremia that was probably secondary to pressure natriuresis, hypokalemia, hyperaldosteronism, and elevated plasma renin activity. The stabilization of blood pressures was done medically with the usage of antihypertensive medications only, without the need for angioplasty or surgical revascularization. Conclusions: We demonstrate that surgical intervention may not always be necessary in the treatment of all cases of FMD, especially in a small child where such intervention may be technically challenging and lead to potential complications. Hence, medical management alone may be sufficient, at least for the short-term, in small children with controlled hypertension and normal renal function, with surgical intervention reserved for FMD with medication-refractory hypertension and\/or compromised renal function.<\/jats:p>","DOI":"10.3390\/medicines7020009","type":"journal-article","created":{"date-parts":[[2020,2,21]],"date-time":"2020-02-21T08:59:47Z","timestamp":1582275587000},"page":"9","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Efficacy of Antihypertensive Therapy in a Child with Unilateral Focal Fibromuscular Dysplasia of the Renal Artery: A Case Study and Review of Literature"],"prefix":"10.3390","volume":"7","author":[{"given":"Ratna","family":"Acharya","sequence":"first","affiliation":[{"name":"Department of Pediatrics, University of Florida, Gainesville, FL 32610, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Savannah","family":"Ellenwood","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Florida, Gainesville, FL 32610, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8441-0220","authenticated-orcid":false,"given":"Kiran","family":"Upadhyay","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Nephrology, University of Florida, Gainesville, FL 32610, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2020,2,20]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1327","DOI":"10.1007\/s00467-007-0520-4","article-title":"Reno-vascular hypertension in childhood: A nationwide survey","volume":"22","author":"Bayazit","year":"2007","journal-title":"Pediatr. Nephrol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1453","DOI":"10.1016\/S0140-6736(08)60626-1","article-title":"Renovascular hypertension in children","volume":"371","author":"Tullus","year":"2008","journal-title":"Lancet"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"394","DOI":"10.1007\/s00270-009-9623-6","article-title":"Cutting balloon angioplasty of bilateral renal artery stenosis due to Takayasu arteritis in a 5-year-old child with midterm follow-up","volume":"33","author":"Gumus","year":"2010","journal-title":"Cardiovasc. Intervent. Radiol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"3313","DOI":"10.1096\/fj.14-251207","article-title":"Clinical and biochemical profiles suggest fibromuscular dysplasia is a systemic disease with altered TGF-\u03b2 expression and connective tissue features","volume":"28","author":"Ganesh","year":"2014","journal-title":"FASEB J."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1097\/HJH.0000000000002019","article-title":"Working Group \u2018Hypertension and the Kidney\u2019 of the European Society of Hypertension (ESH) and the Society for Vascular Medicine (SVM). First international consensus on the diagnosis and management of fibromuscular dysplasia","volume":"37","author":"Persu","year":"2019","journal-title":"J. Hypertens."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"652","DOI":"10.1161\/HYPERTENSIONAHA.117.09539","article-title":"High prevalence of multiple arterial bed lesions in patients with fibromuscular dysplasia: The ARCADIA registry (assessment of renal and cervical artery dysplasia)","volume":"70","author":"Plouin","year":"2017","journal-title":"Hypertension"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"3182","DOI":"10.1161\/CIRCULATIONAHA.112.091223","article-title":"The United States Registry for fibromuscular dysplasia: Results in the first 447 patients","volume":"125","author":"Olin","year":"2012","journal-title":"Circulation"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1186\/1750-1172-2-28","article-title":"Fibromuscular dysplasia","volume":"7","author":"Plouin","year":"2007","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"641","DOI":"10.1007\/s00467-015-3234-z","article-title":"Differences between the pediatric and adult presentation of fibromuscular dysplasia: Results from the US Registry","volume":"31","author":"Green","year":"2016","journal-title":"Pediatr. Nephrol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"S45","DOI":"10.3949\/ccjm.83.s2.06","article-title":"Fibromuscular dysplasia: Advances in understanding and management","volume":"83","author":"Brinza","year":"2016","journal-title":"Cleve Clin. J. Med."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"1593","DOI":"10.1007\/s00467-018-3983-6","article-title":"Imaging studies in pediatric fibromuscular dysplasia (FMD): A single-center experience","volume":"33","author":"Louis","year":"2018","journal-title":"Pediatr. Nephrol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"3062","DOI":"10.1161\/CIRCULATIONAHA.112.117499","article-title":"Association between 2 angiographic subtypes of renal artery fibromuscular dysplasia and clinical characteristics","volume":"126","author":"Savard","year":"2012","journal-title":"Circulation"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1001\/archsurg.1975.01360110107018","article-title":"Arterial fibrodysplasia. Histopathologic character and current etiologic concepts","volume":"110","author":"Stanley","year":"1975","journal-title":"Arch. Surg."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"176","DOI":"10.1016\/j.jacc.2016.04.044","article-title":"Dissection and aneurysm in patients with fibromuscular dysplasia: Findings from the US Registry for FMD","volume":"68","author":"Gornik","year":"2016","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1080\/08860220601166305","article-title":"Imaging modalities for renal artery stenosis in suspected renovascular hypertension: Prospective intra-individual comparison of color Doppler US, CT angiography, GD-enhanced MR angiography, and digital substraction angiography","volume":"29","author":"Rountas","year":"2007","journal-title":"Ren. Fail."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1097\/HJH.0b013e328365b29c","article-title":"Ultrasound doppler renal resistive index: A useful tool for the management of the hypertensive patient","volume":"3","author":"Viazzi","year":"2014","journal-title":"J. Hypertens."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1016\/j.jus.2009.09.006","article-title":"Doppler ultrasound and renal artery stenosis: An overview","volume":"12","author":"Granata","year":"2009","journal-title":"J. Ultrasound."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"1981","DOI":"10.1001\/archinte.162.17.1981","article-title":"Utility of captopril renal scans for detecting renal artery stenosis","volume":"162","author":"Huot","year":"2002","journal-title":"Arch. Intern. Med."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1097\/00006231-199407000-00005","article-title":"Captopril-enhanced 99Tcm-MAG3 renal scintigraphy in subjects with suspected renovascular hypertension","volume":"15","author":"Kahn","year":"1994","journal-title":"Nucl. Med. Commun."},{"key":"ref_20","first-page":"CD002944","article-title":"Balloon angioplasty versus medical therapy for hypertensive patients with renal artery obstruction","volume":"3","author":"Nordmann","year":"2003","journal-title":"Cochrane Database Syst. Rev."},{"key":"ref_21","first-page":"CD002944","article-title":"Balloon angioplasty, with and without stenting, versus medical therapy for hypertensive patients with renal artery stenosis","volume":"12","author":"Jenks","year":"2014","journal-title":"Cochrane Database Syst. Rev."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1016\/S0002-9343(02)01396-7","article-title":"Balloon angioplasty or medical therapy for hypertensive patients with atherosclerotic renal artery stenosis? A meta-analysis of randomized controlled trials","volume":"114","author":"Nordmann","year":"2003","journal-title":"Am. J. Med."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"525","DOI":"10.1161\/HYPERTENSIONAHA.110.152918","article-title":"Efficacy of revascularization for renal artery stenosis caused by fibromuscular dysplasia: A systematic review and meta-analysis","volume":"56","author":"Trinquart","year":"2010","journal-title":"Hypertension"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"421","DOI":"10.1016\/j.jvs.2011.09.006","article-title":"Short- and long-term outcomes of percutaneous transluminal angioplasty\/stenting of renal fibromuscular dysplasia over a ten-year period","volume":"55","author":"Mousa","year":"2012","journal-title":"J. Vasc. Surg."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"1067","DOI":"10.1007\/s00467-013-2730-2","article-title":"Renal artery stenosis in children: Therapeutic percutaneous balloon and stent angioplasty","volume":"29","author":"Colyer","year":"2014","journal-title":"Pediatr. Nephrol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"285","DOI":"10.5152\/dir.2014.13208","article-title":"Angioplasty for pediatric renovascular hypertension: A 13-year experience","volume":"20","author":"Zhu","year":"2014","journal-title":"Diagn. Interv. Radiol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"663","DOI":"10.1007\/s00467-006-0010-0","article-title":"Treatment of severe renal artery stenosis by percutaneous transluminal renal angioplasty and stent implantation: Review of the pediatric experience: Apropos of two cases","volume":"21","author":"Gellermann","year":"2006","journal-title":"Pediatr. Nephrol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"268","DOI":"10.1542\/peds.2005-2642","article-title":"Angioplasty for renovascular hypertension in children: 20-year experience","volume":"118","author":"Shroff","year":"2006","journal-title":"Pediatrics"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"806","DOI":"10.1007\/s004679900260","article-title":"Renovascular disease and hypertension in children with neurofibromatosis","volume":"14","author":"Fossali","year":"2000","journal-title":"Pediatr. Nephrol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1007\/BF00862057","article-title":"Renal artery stenosis in infants: Long\u2013term medical treatment before surgery","volume":"10","author":"Najarian","year":"1996","journal-title":"Pediatr. Nephrol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"276","DOI":"10.1007\/s00467-003-1069-5","article-title":"Renal artery stenosis and nephrotic syndrome: A rare combination in an infant","volume":"18","author":"Lang","year":"2003","journal-title":"Pediatr. Nephrol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"1037","DOI":"10.1007\/s00467-012-2123-y","article-title":"Hyponatremic hypertensive syndrome in pediatric patients: Is it really so rare?","volume":"27","author":"Kovalski","year":"2012","journal-title":"Pediatr. Nephrol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"374","DOI":"10.1159\/000182521","article-title":"Reversible nephrotic range proteinuria with renal artery stenosis: A clinical example of renin-associated proteinuria","volume":"30","author":"Eiser","year":"1982","journal-title":"Nephron"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"418","DOI":"10.1067\/mpd.2001.111330","article-title":"Moyamoya disease complicated with renal artery stenosis and nephrotic syndrome: Reversal of nephrotic syndrome after nephrectomy","volume":"138","author":"Kuwayama","year":"2001","journal-title":"J. Pediatr."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"406","DOI":"10.1016\/0002-9149(87)90945-3","article-title":"Proteinuria in renovascular hypertension and the effects of renal angioplasty","volume":"59","author":"Zimbler","year":"1987","journal-title":"Am. J. Cardiol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1007\/s11906-002-0040-3","article-title":"Mechanisms of pressure natriuresis","volume":"4","author":"Granger","year":"2002","journal-title":"Curr. Hypertens Rep."}],"container-title":["Medicines"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2305-6320\/7\/2\/9\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T08:59:17Z","timestamp":1760173157000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2305-6320\/7\/2\/9"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,2,20]]},"references-count":36,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2020,2]]}},"alternative-id":["medicines7020009"],"URL":"https:\/\/doi.org\/10.3390\/medicines7020009","relation":{},"ISSN":["2305-6320"],"issn-type":[{"type":"electronic","value":"2305-6320"}],"subject":[],"published":{"date-parts":[[2020,2,20]]}}}