Ureteral Structure and Ultrastructure. Part II. Congenital Ureteropelvic Junction Obstruction and Primary Obstructive Megaureter

Hanna, Moneer K.; Jeffs, Robert D.; Sturgess, Jennifer M.; Barkin, Martin

doi:10.1016/s0022-5347(17)58987-9

Cited by 162 publications

(61 citation statements)

References 18 publications

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“…PUJ obstruction may be inherited as an autosomal dominant gene of variable penetrance [3]. Congenital PUJ obstruction is usually caused by intrinsic stenosis of the proximal ureter as a result of an interruption in the development of the circular musculature of the PUJ or an alteration of collagen fibers and composition between and around the muscle cells [4]. Other causes of intrinsic PUJ obstruction include valvular mucosal folds, persistent fetal convolutions and upper ureteral polyps.…”

Section: Discussionmentioning

confidence: 99%

Profile and Outcome of Pelviureteric Junction Obstruction

Senguttuvan

Jigy²

2014

TOUNJ

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Abstract:Obstructive uropathy represents one of the largest fractions of identifiable causes of renal failure in pediatric populations. This is one of the largest series of PUJ obstructions reported in children. PUJ obstruction is a relatively common congenital anamolyes in neonates and children and a cause of ESRD in developing countries. This series expands our knowledge about the clinical presentation and course of this disease. Pelviureteric junction (PUJ) obstruction is a partial or total blockage of the flow of urine that occurs where the ureter enters the kidney and is the most common cause of significant dilatation of the collecting system in the fetal kidney. We retrospectively analyzed children diagnosed as PUJ obstruction between the periods of 2008-2010 who presented to our institute. Forty four children were diagnosed to have PUJ obstruction. The median age at presentation was 11 months, with range of 3 months to 11 years. Male children constituted 34 (77.3%) cases. Unilateral PUJ with left sided involvement was more common (52.3%). Antenatal diagnosis was made in 18 (40.9%) cases. The common clinical presentations were fever, urinary tract infection (UTI) and abdominal pain. Hypertension was present in five (11.3%) patients and renal failure at presentation was seen in eight (18.2%) patients. Associated urogenital anomalies were present in twelve (27.3%) patients, with medullary cystic kidney disease (MCKD) being the most commonassociated anomaly. Prophylactic antibiotics were not prescribed to patients. Urinary tract infection was present in fourteen (31.9%) patients with E. coli being the most common organism (75%). Conservative management with regular follow up was done for 32 patients and the remaining twelve patients underwent pyeloplastyin view of gross hydronephrosis, and four (33.3%)of these patients later underwent nephrectomy for a nonfunctioning kidney. Antenatal screening is mandatory to make an early diagnosis and for further management and close follow upof PUJ obstruction. Randomized control trials and longer follow up studies are needed for evidence to suggest the optimal management.

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Section: Discussionmentioning

confidence: 99%

Profile and Outcome of Pelviureteric Junction Obstruction

Senguttuvan

Jigy²

2014

TOUNJ

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“…This disease is characterized by abnormal collagen and smooth muscle components at the ureteropelvic junction that contribute to mechanical or functional obstruction of urinary flow (27,28). Interestingly, the structural abnormalities in ADAMTS-1 -/-mice are responsible for the same dysfunctions described in cases of congenital ureteropelvic junction obstruction and primary obstructive megaureter in humans (29,30). Thus, the ADAMTS-1-null mouse may represent a useful new disease model for clarification of the pathogenesis of congenital ureteropelvic junction obstruction.…”

Section: Figurementioning

confidence: 99%

ADAMTS-1: a metalloproteinase-disintegrin essential for normal growth, fertility, and organ morphology and function

Shindo¹,

Kurihara²,

Kuno³

et al. 2000

J. Clin. Invest.

291

188

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“…The pre-and postCurrently there is a paucity of information on the histopathology and clinical relevance of obstructing operative radiographs and/or ultrasonograms of all children were reviewed. Obstruction was diagnosed accord-PUJ complexes [1][2][3][4][5]. In an eÂort to improve understanding of upper urinary tract obstruction in childhood, we ing to conventional criteria (ultrasonography and diuretic renography).…”

Section: Introductionmentioning

confidence: 99%

The relationship of smooth muscle damage to age, severity of pre‐operative hydronephrosis and post‐operative outcome in obstructive uropathies

Pintér¹,

Horváth

Hrabovszky³

1997

British Journal of Urology

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Objective To determine the relationship, in patients with pelvi‐ureteric junction (PUJ) obstruction, of histopathological abnormalities to their age at surgery, the severity of pre‐operative hydronephrosis and the post‐operative outcome. Materials and methods Surgical specimens from 60 consecutive PUJ complexes excised at pyeloplasty were evaluated histologically. Results There was a close relationship of smooth muscle abnormality and fibrosis with the severity of pre‐operative hydronephrosis and post‐operative outcome, with no significant correlation between histopathological abnormality and patient age. Conclusion This study appears to support the practice of the initial non‐operative management of congenital PUJ obstruction, potentially reducing the number of early and/or unnecessary interventions. Further investigations and research are necessary to direct appropriate management.

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Ureteral Structure and Ultrastructure. Part II. Congenital Ureteropelvic Junction Obstruction and Primary Obstructive Megaureter

Cited by 162 publications

References 18 publications

Profile and Outcome of Pelviureteric Junction Obstruction

Profile and Outcome of Pelviureteric Junction Obstruction

ADAMTS-1: a metalloproteinase-disintegrin essential for normal growth, fertility, and organ morphology and function

The relationship of smooth muscle damage to age, severity of pre‐operative hydronephrosis and post‐operative outcome in obstructive uropathies

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