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Hypophosphatemic nephrolithiasis/osteoporosis 1(NPHLOP1)

MedGen UID:
436776
Concept ID:
C2676786
Disease or Syndrome
Synonyms: Nephrolithiasis-Osteoporosis, Hypophosphatemic, 1; NPHLOP1
 
Gene (location): SLC34A1 (5q35.3)
 
Monarch Initiative: MONDO:0012850
OMIM®: 612286

Clinical features

From HPO
Hypercalciuria
MedGen UID:
43775
Concept ID:
C0020438
Finding
Abnormally high level of calcium in the urine.
Hyperphosphaturia
MedGen UID:
78638
Concept ID:
C0268079
Disease or Syndrome
An increased excretion of phosphates in the urine.
Kidney stone
MedGen UID:
98227
Concept ID:
C0392525
Disease or Syndrome
Kidney stones (calculi) are mineral concretions in the renal calyces and pelvis that are found free or attached to the renal papillae.
Renal phosphate wasting
MedGen UID:
335116
Concept ID:
C1845169
Finding
High urine phosphate in the presence of hypophosphatemia.
Osteopenia
MedGen UID:
18222
Concept ID:
C0029453
Disease or Syndrome
Osteopenia is a term to define bone density that is not normal but also not as low as osteoporosis. By definition from the World Health Organization osteopenia is defined by bone densitometry as a T score -1 to -2.5.
Osteoporosis
MedGen UID:
14535
Concept ID:
C0029456
Disease or Syndrome
Osteoporotic fractures are an important public health problem affecting both sexes. The pathogenesis of osteoporosis is complex and involves both environmental and genetic components. Osteoporotic bone is characterized by decreased bone strength, reflected by a decreased bone mineral density (BMD) and disrupted microarchitecture. Bone is remodeled continuously during adult life, and osteoporosis is typically the result of an imbalance in bone remodeling, with resorption exceeding formation (summary by Tofteng et al., 2007). See BMND1 (601884) for a list of bone mineral density (BMD) quantitative trait loci, some of which have been associated with susceptibility to osteoporosis. Association has been suggested between variation in the ESR1 gene (133430) and BMD.
Increased susceptibility to fractures
MedGen UID:
234655
Concept ID:
C1390474
Finding
An abnormally increased tendency to fractures of bones caused by an abnormal reduction in bone strength that is generally associated with an increased risk of fracture.
Hypophosphatemia
MedGen UID:
39327
Concept ID:
C0085682
Disease or Syndrome
The concentration of phosphate ion in the blood circulation is below the lower limit of normal.

Recent clinical studies

Etiology

Marini F, Giusti F, Marasco E, Xumerle L, Kwiatkowska KM, Garagnani P, Biver E, Ferrari S, Iolascon G, Iantomasi T, Brandi ML
Eur J Endocrinol 2023 Jan 10;188(1) doi: 10.1093/ejendo/lvad001. PMID: 36762943
Gordon RJ, Li D, Doyle D, Zaritsky J, Levine MA
J Clin Endocrinol Metab 2020 Jul 1;105(7):2392-400. doi: 10.1210/clinem/dgaa217. PMID: 32311027Free PMC Article

Diagnosis

Marini F, Giusti F, Marasco E, Xumerle L, Kwiatkowska KM, Garagnani P, Biver E, Ferrari S, Iolascon G, Iantomasi T, Brandi ML
Eur J Endocrinol 2023 Jan 10;188(1) doi: 10.1093/ejendo/lvad001. PMID: 36762943

Therapy

Marini F, Giusti F, Marasco E, Xumerle L, Kwiatkowska KM, Garagnani P, Biver E, Ferrari S, Iolascon G, Iantomasi T, Brandi ML
Eur J Endocrinol 2023 Jan 10;188(1) doi: 10.1093/ejendo/lvad001. PMID: 36762943

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