PMID- 36381012
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20221117
IS  - 2472-1972 (Electronic)
IS  - 2472-1972 (Linking)
VI  - 6
IP  - 12
DP  - 2022 Oct 26
TI  - Romosozumab-aqqg in the Treatment of Osteoporosis in a Patient With 
      Hypophosphatasia.
PG  - bvac159
LID - 10.1210/jendso/bvac159 [doi]
LID - bvac159
AB  - Hypophosphatasia is a rare, inherited condition that causes osteomalacia and 
      recurrent fractures. Therapeutic options for osteoporosis in patients with 
      hypophosphatasia are limited because of concerns for a greater likelihood of 
      atypical femoral fractures with antiresorptive agents. We report here the case of 
      a patient with hypophosphatasia and osteoporosis who was treated with 
      romosozumab-aqqg (Romo). An 81-year-old woman presented for management of 
      osteoporosis with multiple fractures. She experienced a decline in bone mineral 
      density over 20 years despite sequential osteoporosis treatment with oral 
      bisphosphonates, hormone replacement therapy, teriparatide, and denosumab. 
      Hypophosphatasia was suspected because of low serum alkaline phosphatase levels 
      and was confirmed by genetic testing. After diagnosing hypophosphatasia, bone 
      mineral density continued to decline and a trial of Romo was begun. After 1 year 
      of Romo therapy, bone mineral density improved by 21%, and 10% at the lumbar 
      spine and total hip, respectively. These changes were substantially greater than 
      what she had experienced with prior teriparatide therapy. Blood alkaline 
      phosphatase remained low on Romo. To our knowledge, this is the first report of a 
      patient with hypophosphatasia and osteoporosis treated with Romo. In our patient, 
      Romo did not significantly impact serum alkaline phosphatase, but improved bone 
      mineral density significantly. In conclusion, Romo is a potential treatment 
      option for osteoporosis in patients with hypophosphatasia for whom limited 
      alternatives exist.
CI  - (c) The Author(s) 2022. Published by Oxford University Press on behalf of the 
      Endocrine Society.
FAU - Khanjee, Naveed
AU  - Khanjee N
AUID- ORCID: 0000-0001-8617-9483
AD  - Department of Internal Medicine, Division of Endocrinology, University of Texas 
      Southwestern Medical Center, Dallas, TX 75390-8885, USA.
FAU - Maalouf, Naim M
AU  - Maalouf NM
AUID- ORCID: 0000-0002-0997-283X
AD  - Department of Internal Medicine, Division of Endocrinology, University of Texas 
      Southwestern Medical Center, Dallas, TX 75390-8885, USA.
AD  - The Charles and Jane Pak Center for Mineral Metabolism and Clinical Research, 
      University of Texas Southwestern Medical Center, Dallas, TX 75390-8885, USA.
LA  - eng
PT  - Case Reports
DEP - 20221027
PL  - United States
TA  - J Endocr Soc
JT  - Journal of the Endocrine Society
JID - 101697997
PMC - PMC9646966
OTO - NOTNLM
OT  - hypophosphatasia
OT  - osteoporosis
OT  - romosozumab-aqqg
EDAT- 2022/11/17 06:00
MHDA- 2022/11/17 06:01
PMCR- 2022/10/27
CRDT- 2022/11/16 02:47
PHST- 2022/07/08 00:00 [received]
PHST- 2022/11/16 02:47 [entrez]
PHST- 2022/11/17 06:00 [pubmed]
PHST- 2022/11/17 06:01 [medline]
PHST- 2022/10/27 00:00 [pmc-release]
AID - bvac159 [pii]
AID - 10.1210/jendso/bvac159 [doi]
PST - epublish
SO  - J Endocr Soc. 2022 Oct 27;6(12):bvac159. doi: 10.1210/jendso/bvac159. eCollection 
      2022 Oct 26.