Literature DB >> 33537138

X-linked hypophosphatemic osteomalacia with PHEX mutation presenting late in Pakistan.

Nawazish Zehra1, Lena Jafri1, Salman Kirmani2, Aysha Habib Khan1.   

Abstract

ABSTRACTINTRODUCTIONANDIMPORTANCE: Autosomal dominant hypophosphatemic rickets is the most common form of rare rickets, commonly manifests in children but sometimes the condition remains undiagnosed due to lack of knowledge &/or awareness of treating physicians or surgeons. CASE
PRESENTATION: We describe a case of 43 years old female with multiple fragility fractures since childhood, corrected surgically but never investigated. She had stunted growth, bowing deformities and loss of teeth. CLINICAL DISCUSSION: A detailed history and examination along with metabolic and genetic work up mounted the diagnosis of X linked hypophosphatemic osteomalacia. The pathophysiology involves the mutation or the loss of the phosphate regulating gene on PHEX, that causes reduced mineralization of bones and teeth.
CONCLUSION: Diagnostic delay in this patient resulted in increased disabilities affecting her mobility and lif estyle.
© 2021 Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd.

Entities:  

Keywords:  Autosomal dominant; Case report; Osteomalacia/rickets; PHEX mutation

Year:  2021        PMID: 33537138      PMCID: PMC7840437          DOI: 10.1016/j.amsu.2021.01.067

Source DB:  PubMed          Journal:  Ann Med Surg (Lond)        ISSN: 2049-0801


  14 in total

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2.  FGF-23 is a potent regulator of vitamin D metabolism and phosphate homeostasis.

Authors:  Takashi Shimada; Hisashi Hasegawa; Yuji Yamazaki; Takanori Muto; Rieko Hino; Yasuhiro Takeuchi; Toshiro Fujita; Kazuhiko Nakahara; Seiji Fukumoto; Takeyoshi Yamashita
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Review 7.  Hereditary hypophosphatemic rickets with hypercalciuria: pathophysiology, clinical presentation, diagnosis and therapy.

Authors:  Clemens Bergwitz; Ken-Ichi Miyamoto
Journal:  Pflugers Arch       Date:  2018-08-14       Impact factor: 3.657

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Journal:  Birth Defects Orig Artic Ser       Date:  1971-05

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Authors:  Sezer Acar; Korcan Demir; Yufei Shi
Journal:  J Clin Res Pediatr Endocrinol       Date:  2017-12-27

Review 10.  Genetic approaches to metabolic bone diseases.

Authors:  Fadil M Hannan; Paul J Newey; Michael P Whyte; Rajesh V Thakker
Journal:  Br J Clin Pharmacol       Date:  2018-11-28       Impact factor: 4.335

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