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Histidine transport defect

MedGen UID:
82825
Concept ID:
C0268642
Disease or Syndrome
Synonyms: Histidinuria due to a renal tubular defect; Histidinuria renal tubular defect; Renal histidinuria
SNOMED CT: Histidine transport defect (78311009); Hyperhistidinuria (78311009); Histidinuria (78311009); Renal histidinuria (78311009)
 
Monarch Initiative: MONDO:0009346
OMIM®: 235830
Orphanet: ORPHA2158

Definition

A rare disorder of histidine metabolism characterized by histidinuria without histidinemia due to impaired intestinal and renal tubular absorption of histidine. Developmental delay, intellectual disability, seizures, and mild dysmorphic features have been reported in association. There have been no further descriptions in the literature since 1992. [from ORDO]

Clinical features

From HPO
Impaired histidine renal tubular absorption
MedGen UID:
870207
Concept ID:
C4024642
Pathologic Function
Histidinuria
MedGen UID:
1731918
Concept ID:
C5399766
Finding
An increased concentration of histidine in the urine.
Short middle phalanx of finger
MedGen UID:
337690
Concept ID:
C1846950
Finding
Short (hypoplastic) middle phalanx of finger, affecting one or more fingers.
Rounded middle phalanx of finger
MedGen UID:
870672
Concept ID:
C4025126
Anatomical Abnormality
An abnormally round shape of the middle phalanx of the finger.
Sensorineural hearing loss disorder
MedGen UID:
9164
Concept ID:
C0018784
Disease or Syndrome
A type of hearing impairment in one or both ears related to an abnormal functionality of the cochlear nerve.
Macrotia
MedGen UID:
488785
Concept ID:
C0152421
Congenital Abnormality
Median longitudinal ear length greater than two standard deviations above the mean and median ear width greater than two standard deviations above the mean (objective); or, apparent increase in length and width of the pinna (subjective).
Intellectual disability
MedGen UID:
811461
Concept ID:
C3714756
Mental or Behavioral Dysfunction
Intellectual disability, previously referred to as mental retardation, is characterized by subnormal intellectual functioning that occurs during the developmental period. It is defined by an IQ score below 70.
Generalized myoclonic seizure
MedGen UID:
892704
Concept ID:
C4021759
Disease or Syndrome
A generalized myoclonic seizure is a type of generalized motor seizure characterized by bilateral, sudden, brief (<100 ms) involuntary single or multiple contraction of muscles or muscle groups of variable topography (axial, proximal limb, distal). Myoclonus is less regularly repetitive and less sustained than is clonus.
Smooth philtrum
MedGen UID:
222980
Concept ID:
C1142533
Finding
Flat skin surface, with no ridge formation in the central region of the upper lip between the nasal base and upper vermilion border.
Wide nasal bridge
MedGen UID:
341441
Concept ID:
C1849367
Finding
Increased breadth of the nasal bridge (and with it, the nasal root).
Long philtrum
MedGen UID:
351278
Concept ID:
C1865014
Finding
Distance between nasal base and midline upper lip vermilion border more than 2 SD above the mean. Alternatively, an apparently increased distance between nasal base and midline upper lip vermilion border.
Thin upper lip vermilion
MedGen UID:
355352
Concept ID:
C1865017
Finding
Height of the vermilion of the upper lip in the midline more than 2 SD below the mean. Alternatively, an apparently reduced height of the vermilion of the upper lip in the frontal view (subjective).
Hypoplastic toenails
MedGen UID:
332409
Concept ID:
C1837279
Finding
Underdevelopment of the toenail.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHistidine transport defect

Professional guidelines

PubMed

Rose EC, di San Filippo CA, Ndukwe Erlingsson UC, Ardon O, Pasquali M, Longo N
Hum Mutat 2012 Jan;33(1):118-23. Epub 2011 Oct 11 doi: 10.1002/humu.21607. PMID: 21922592Free PMC Article
Kodama H, Fujisawa C, Bhadhprasit W
Curr Drug Metab 2012 Mar;13(3):237-50. doi: 10.2174/138920012799320455. PMID: 21838703Free PMC Article

Supplemental Content

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