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Chauhan M, Alpers DH, Hamilton JP, et al. If Hoofbeats are not From Horses, It Could be Zebras!! Isolated Hyper-alkaline Phosphatasemia. J Clin Exp Hepatol. 2020;11(5):619-622doi: 10.1016/j.jceh.2020.12.001.
Chauhan, M., Alpers, D. H., Hamilton, J. P., & Thuluvath, P. J. (2021). If Hoofbeats are not From Horses, It Could be Zebras!! Isolated Hyper-alkaline Phosphatasemia. Journal of clinical and experimental hepatology, 11(5), 619-622. https://doi.org/10.1016/j.jceh.2020.12.001
Chauhan, Mahak, et al. "If Hoofbeats are not From Horses, It Could be Zebras!! Isolated Hyper-alkaline Phosphatasemia." Journal of clinical and experimental hepatology vol. 11,5 (2021): 619-622. doi: https://doi.org/10.1016/j.jceh.2020.12.001
Chauhan M, Alpers DH, Hamilton JP, Thuluvath PJ. If Hoofbeats are not From Horses, It Could be Zebras!! Isolated Hyper-alkaline Phosphatasemia. J Clin Exp Hepatol. 2021 Sep-Oct;11(5):619-622. doi: 10.1016/j.jceh.2020.12.001. Epub 2020 Dec 17. PMID: 34511824; PMCID: PMC8414313.
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J Clin Exp Hepatol. 2021 Sep-Oct;11(5):619-622. doi: 10.1016/j.jceh.2020.12.001. Epub 2020 Dec 17.

If Hoofbeats are not From Horses, It Could be Zebras!! Isolated Hyper-alkaline Phosphatasemia.

Journal of clinical and experimental hepatology

Mahak Chauhan, David H Alpers, James P Hamilton, Paul J Thuluvath

Affiliations

  1. Institute of Digestive Health and Liver Diseases, Mercy Medical Center, Baltimore, MD, USA.
  2. Department of Medicine, Washington University School of Medicine, St Louis, MO, USA.
  3. Department of Medicine, The Johns Hopkins University School of Medicine, Baltimore, MD, USA.
  4. Department of Medicine, The University of Maryland School of Medicine, Baltimore, MD, USA.

PMID: 34511824 PMCID: PMC8414313 DOI: 10.1016/j.jceh.2020.12.001

Abstract

Alkaline phosphatase (AP) is a membrane bound enzyme and when it is elevated in blood, it is mostly due to either hepatobiliary or bone diseases. We report isolated intestinal hyperphosphatasemia (IAP) in two sisters. Both sisters presented with identical trends of isolated AP elevation. Both underwent extensive workup for liver diseases including cholangiograms, and none was identified. Subsequent isoenzyme electrophoresis showed that 45%-56% of the elevated AP was due to IAP. This elevation of the intestinal AP is consistent with a rare hereditary biochemical abnormality, benign familial intestinal hyperphosphatemia. This condition should be considered in the differential diagnosis of otherwise isolated serum AP levels to avoid unnecessary investigations.

© 2020 Indian National Association for Study of the Liver. Published by Elsevier B.V. All rights reserved.

Keywords: BFIH, Benign familial intestinal hyperphosphatasemia; CKDs, Chronic kidney diseases; DILI, Drug-induced liver injury; ERCP, Endoscopic retrograde cholangiopancreatography; IAP, Intestinal alkaline hyperphosphatasemia; MRCP, Magnetic resonance cholangiopancreatography; PSC, Primary sclerosing cholangitis; TNAP, Tissue non-specific AP; alkaline phosphatase isozyme; elevated alkaline phosphatase; familial benign intestinal alkaline hyperphosphatasemia; hyperphosphatasemia

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