Prune belly syndrome

Orpha code: 2970OMIM code: 100100

Definition

A rare lower urinary tract obstruction (LUTO) characterized by varying degrees of an enlarged urinary bladder, dilated ureters, hydronephrosis, and poorly contractile and disorganized detrusor and ureteral smooth muscle, in association with hypoplastic or absent midline abdominal skeletal musculature, and bilaterally undescended testes in males.

Disease data
Classification

Malformation syndrome

Synonyms
Abdominal muscle deficiency syndrome
Obrinsky syndrome
Zespół Eagle'a i Barreta
Zespół niedorozwoju mięśni brzucha
Zespół triady
Eagle-Barret syndrome
Obrinsky syndrome
Triad syndrome
ORPHA code
2970
OMIM code
100100
ICD10 code
Q79.4
ICD11 code
LD2F.10

No additional description.

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