Biomaterial Database

A case of prune belly syndrome. 2000

Z V Peshev, and M B Krusteva, and V H Danev

Departement of General and Clinical Pathology, Higher Medical Institute, Plovdiv, Bulgaria.

The prune belly syndrome was first described in 1839 by Frolich. Till now about 300 cases have been reported in literature. The complete form of the syndrome is presented by a classic triad that is seen only in boys and is incompatible with life. The etiology of the syndrome is uncertain. Genetic factors, intrauterine infections, adverse mechanical factors have been implicated. The prenatal diagnosis relies on the ultrasonographic findings of oligohydramnion, renal anomalies, and non-immune fetal hydropsy. The present case concerns a newborn of male sex suffering from the most severe and rare form of the syndrome. The child died 5 minutes after his birth. The pathological examination found the patient devoid of striated muscles anterior abdominal wall, urethral atresia, hyperthrophy of the urinary bladder, bilateral hydroureter and hydronephrosis and coinciding cryptorchidism. The case indicates the PBS as one of the possible reasons for non-immune fetal hydropsy.

UI	MeSH Term	Description	Entries
D006984	Hypertrophy	General increase in bulk of a part or organ due to CELL ENLARGEMENT and accumulation of FLUIDS AND SECRETIONS, not due to tumor formation, nor to an increase in the number of cells (HYPERPLASIA).	Hypertrophies
D007231	Infant, Newborn	An infant during the first 28 days after birth.	Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D011535	Prune Belly Syndrome	A syndrome characterized by abdominal wall musculature deficiency, cryptorchism, and urinary tract abnormalities. The syndrome derives its name from its characteristic distended abdomen with wrinkled skin.	Abdominal Muscle Deficiency Syndrome,Congenital Absence of the Abdominal Muscles,Eagle-Barrett Syndrome,Obrinsky Syndrome,Prune-Belly Syndrome,Eagle Barrett Syndrome,Prune Belly Syndromes,Prune-Belly Syndromes,Syndrome, Eagle-Barrett,Syndrome, Obrinsky,Syndrome, Prune Belly,Syndrome, Prune-Belly,Syndromes, Prune Belly,Syndromes, Prune-Belly
D001743	Urinary Bladder	A musculomembranous sac along the URINARY TRACT. URINE flows from the KIDNEYS into the bladder via the ureters (URETER), and is held there until URINATION.	Bladder,Bladder Detrusor Muscle,Detrusor Urinae,Bladder Detrusor Muscles,Bladder, Urinary,Detrusor Muscle, Bladder,Detrusor Muscles, Bladder
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D006869	Hydronephrosis	Abnormal enlargement or swelling of a KIDNEY due to dilation of the KIDNEY CALICES and the KIDNEY PELVIS. It is often associated with obstruction of the URETER or chronic kidney diseases that prevents normal drainage of urine into the URINARY BLADDER.	Hydronephroses
D000009	Abdominal Muscles	Muscles forming the ABDOMINAL WALL including RECTUS ABDOMINIS; ABDOMINAL OBLIQUE MUSCLES, transversus abdominis, pyramidalis muscles and quadratus abdominis.	Cremaster Muscle,Pyramidalis Muscle,Quadratus Abdominis,Transverse Abdominal,Transversus Abdominis,Abdominal Muscle,Abdominal, Transverse,Abdominals, Transverse,Abdomini, Quadratus,Abdominis, Quadratus,Cremaster Muscles,Muscle, Abdominal,Muscle, Cremaster,Muscle, Pyramidalis,Muscles, Abdominal,Muscles, Cremaster,Muscles, Pyramidalis,Pyramidalis Muscles,Quadratus Abdomini,Transverse Abdominals
D014515	Ureteral Diseases	Pathological processes involving the URETERS.	Disease, Ureteral,Diseases, Ureteral,Ureteral Disease
D014521	Urethra	A tube that transports URINE from the URINARY BLADDER to the outside of the body in both the sexes. It also has a reproductive function in the male by providing a passage for SPERM.	External Urethral Sphincter,External Urinary Sphincter,Internal Urethral Sphincter,Internal Urinary Sphincter,Internal Vesical Sphincter,Urethral Sphincters,External Urethral Sphincters,External Urinary Sphincters,Internal Urethral Sphincters,Internal Urinary Sphincters,Internal Vesical Sphincters,Sphincter, External Urethral,Sphincter, External Urinary,Sphincter, Internal Urethral,Sphincter, Internal Urinary,Sphincter, Internal Vesical,Sphincter, Urethral,Urethral Sphincter,Urethral Sphincter, External,Urethras,Urinary Sphincter, External,Urinary Sphincter, Internal,Vesical Sphincter, Internal
D018482	Muscle, Skeletal	A subtype of striated muscle, attached by TENDONS to the SKELETON. Skeletal muscles are innervated and their movement can be consciously controlled. They are also called voluntary muscles.	Anterior Tibial Muscle,Gastrocnemius Muscle,Muscle, Voluntary,Plantaris Muscle,Skeletal Muscle,Soleus Muscle,Muscle, Anterior Tibial,Muscle, Gastrocnemius,Muscle, Plantaris,Muscle, Soleus,Muscles, Skeletal,Muscles, Voluntary,Skeletal Muscles,Tibial Muscle, Anterior,Voluntary Muscle,Voluntary Muscles