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Proteinuria, low molecular weight, with hypercalciuria and nephrocalcinosis

MedGen UID:
333426
Concept ID:
C1839874
Disease or Syndrome
Synonyms: Low Molecular Weight Proteinuria with Hypercalciuria and Nephrocalcinosis; Proteinuria, Low Molecular Weight, with Hypercalciuria and Nephrocalcinosis
 
Gene (location): CLCN5 (Xp11.23)
 
Monarch Initiative: MONDO:0010644
OMIM®: 308990

Definition

Low molecular weight proteinuria with hypercalciuria and nephrocalcinosis is a form of X-linked hypercalciuric nephrocalcinosis, a group of disorders characterized by proximal renal tubular reabsorptive failure, hypercalciuria, nephrocalcinosis, and renal insufficiency. These disorders have also been referred to as the 'Dent disease complex' (Scheinman, 1998; Gambaro et al., 2004). For a general discussion of Dent disease, see 300009. [from OMIM]

Clinical features

From HPO
Focal segmental glomerulosclerosis
MedGen UID:
4904
Concept ID:
C0017668
Disease or Syndrome
Segmental accumulation of scar tissue in individual (but not all) glomeruli.
Glycosuria
MedGen UID:
42267
Concept ID:
C0017979
Finding
An increased concentration of glucose in the urine.
Hypercalciuria
MedGen UID:
43775
Concept ID:
C0020438
Finding
Abnormally high level of calcium in the urine.
Nephrocalcinosis
MedGen UID:
10222
Concept ID:
C0027709
Disease or Syndrome
Nephrocalcinosis is the deposition of calcium salts in renal parenchyma.
Hyposthenuria
MedGen UID:
68565
Concept ID:
C0232831
Finding
An abnormally low urinary specific gravity, i.e., reduced concentration of solutes in the urine.
Aminoaciduria
MedGen UID:
116067
Concept ID:
C0238621
Disease or Syndrome
An increased concentration of an amino acid in the urine.
Microscopic hematuria
MedGen UID:
65997
Concept ID:
C0239937
Finding
Microscopic hematuria detected by dipstick or microscopic examination of the urine.
Renal insufficiency
MedGen UID:
332529
Concept ID:
C1565489
Disease or Syndrome
A reduction in the level of performance of the kidneys in areas of function comprising the concentration of urine, removal of wastes, the maintenance of electrolyte balance, homeostasis of blood pressure, and calcium metabolism.
Proximal tubulopathy
MedGen UID:
326534
Concept ID:
C1839603
Disease or Syndrome
Dysfunction of the proximal tubule, which is the portion of the duct system of the nephron of the kidney which leads from Bowman's capsule to the loop of Henle.
Low-molecular-weight proteinuria
MedGen UID:
333360
Concept ID:
C1839606
Finding
Excretion in urine of proteins of a size smaller than albumin (molecular weight 69 kD).
Renal tubular atrophy
MedGen UID:
388054
Concept ID:
C1858395
Finding
The presence of renal tubules with thick redundant basement membranes, or a reduction of greater than 50% in tubular diameter compared to surrounding non-atrophic tubules.
Beta 2-microglobulinuria
MedGen UID:
1373797
Concept ID:
C4476798
Finding
Increased level of beta 2-microglobulins in the urine.
Short stature
MedGen UID:
87607
Concept ID:
C0349588
Finding
A height below that which is expected according to age and gender norms. Although there is no universally accepted definition of short stature, many refer to "short stature" as height more than 2 standard deviations below the mean for age and gender (or below the 3rd percentile for age and gender dependent norms).
Hypophosphatemia
MedGen UID:
39327
Concept ID:
C0085682
Disease or Syndrome
An abnormally decreased phosphate concentration in the blood.

Recent clinical studies

Etiology

Ye Q, Shen Q, Rao J, Zhang A, Zheng B, Liu X, Shen Y, Chen Z, Wu Y, Hou L, Jian S, Wei M, Ma M, Sun S, Li Q, Dang X, Wang Y, Xu H, Mao J; for Chinese Children Genetic Kidney Disease Database (CCGKDD), “Internet Plus” Nephrology Alliance of National Center for Children's Care
Clin Genet 2020 Mar;97(3):407-417. Epub 2020 Jan 13 doi: 10.1111/cge.13663. PMID: 31674016

Diagnosis

Makino S, Empitu MA, Naito T, Ishii M, Wakabayashi H, Lee C, Aizawa M, Asanuma K
CEN Case Rep 2020 Nov;9(4):380-384. Epub 2020 Jun 12 doi: 10.1007/s13730-020-00491-9. PMID: 32533415Free PMC Article
Ye Q, Shen Q, Rao J, Zhang A, Zheng B, Liu X, Shen Y, Chen Z, Wu Y, Hou L, Jian S, Wei M, Ma M, Sun S, Li Q, Dang X, Wang Y, Xu H, Mao J; for Chinese Children Genetic Kidney Disease Database (CCGKDD), “Internet Plus” Nephrology Alliance of National Center for Children's Care
Clin Genet 2020 Mar;97(3):407-417. Epub 2020 Jan 13 doi: 10.1111/cge.13663. PMID: 31674016

Therapy

Makino S, Empitu MA, Naito T, Ishii M, Wakabayashi H, Lee C, Aizawa M, Asanuma K
CEN Case Rep 2020 Nov;9(4):380-384. Epub 2020 Jun 12 doi: 10.1007/s13730-020-00491-9. PMID: 32533415Free PMC Article

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