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Initial testing revealed moderate proteinuria on qualitative testing, although she was incidentally noted to have severe hypertension (240\/200\u00a0mmHg). Physical examination of the carotid and femoral areas revealed significant systolic vascular murmurs. Labs showed elevated serum creatinine, hypokalemia, metabolic alkalosis, elevated renin and aldosterone and hypercalciuria. Echocardiography identified ventricular hypertrophy. Computed tomography (CT) of the abdomen and magnetic resonance angiography of the head showed multiple tortuous or interrupted arteries and multiple calcifications in the renal sinus area. B-mode ultrasonography suggested thickening of the carotid and femoral artery walls, with numerous spotted calcifications. Genetic testing revealed that <jats:italic>ABCC6<\/jats:italic> had a complex heterozygous mutation (exon 24: c.3340C\u2009&gt;\u2009T and intron 30: c.4404-1G\u2009&gt;\u2009A). Our panel of experts reviewed the evaluation of this patient with hypertension, proteinuria, hypercalciuria, and vascular abnormalities as well as the diagnosis and appropriate management of a rare disease.<\/jats:p>","DOI":"10.1007\/s00467-023-06230-3","type":"journal-article","created":{"date-parts":[[2024,1,2]],"date-time":"2024-01-02T06:03:11Z","timestamp":1704175391000},"page":"1301-1313","update-policy":"http:\/\/dx.doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["A previously healthy 3-year-old female with hypertension, proteinuria, and hypercalciuria"],"prefix":"10.1007","volume":"39","author":[{"given":"Tao","family":"Liu","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wenhong","family":"Wang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zhufeng","family":"Liu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Guanghua","family":"Pei","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chunxiang","family":"Wang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ying","family":"Jiang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chuyue","family":"Pang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2024,1,2]]},"reference":[{"key":"6230_CR1","doi-asserted-by":"publisher","first-page":"581","DOI":"10.1007\/s00467-018-4181-2","volume":"35","author":"A Jain","year":"2020","unstructured":"Jain A, Baracco R, Kapur G (2020) Pheochromocytoma and paraganglioma-an update on diagnosis, evaluation, and management. 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