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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
1
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pubmed:dateCreated |
1997-5-15
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pubmed:abstractText |
We report five patients with Laurence-Moon-Bardet-Biedl syndrome (LMBBS) who had renal involvement. Intravenous pyelography showed bilateral or unilateral calyceal clubbing and blunting in all patients. In addition, one patient had a parapelvic cyst in the left kidney and another had bilateral lobulated renal outlines of the fetal type. One patient had a urinary concentrating defect and two patients showed increased fractional sodium excretion. Estimated tubular phosphate reabsorption values were in normal limits in all of five patients. No patient had a urine acidification defect, proteinuria, glycosuria, or hyperaminoaciduria. One patient died from end-stage renal failure. The remaining four patients had normal serum creatinine values and estimated creatinine clearances. 99mTechnetium-diethylenetriamine pentaacetate renal scanning showed prolonged and delayed concentration and delayed excretion in three of the four patients who survived. A focal scar was determined on the left kidney of one of four patients by 99mtechnetium-dimercaptosuccinic acid renal scanning. All LMBBS cases with or without renal symptoms should be routinely evaluated for renal abnormalities. Renal scanning is a valuable method, especially for determining the renal involvement in the early stage of disease.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:status |
MEDLINE
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pubmed:month |
Feb
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pubmed:issn |
0931-041X
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:volume |
11
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
31-5
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pubmed:dateRevised |
2004-11-17
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pubmed:meshHeading |
pubmed-meshheading:9035169-Adolescent,
pubmed-meshheading:9035169-Child,
pubmed-meshheading:9035169-Fatal Outcome,
pubmed-meshheading:9035169-Female,
pubmed-meshheading:9035169-Follow-Up Studies,
pubmed-meshheading:9035169-Humans,
pubmed-meshheading:9035169-Kidney,
pubmed-meshheading:9035169-Kidney Function Tests,
pubmed-meshheading:9035169-Laurence-Moon Syndrome,
pubmed-meshheading:9035169-Male,
pubmed-meshheading:9035169-Tomography, X-Ray Computed,
pubmed-meshheading:9035169-Urography
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pubmed:year |
1997
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pubmed:articleTitle |
Renal involvement in the Laurence-Moon-Bardet-Biedl syndrome: report of five cases.
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pubmed:affiliation |
Osmangazi University, Faculty of Medicine, Department of Pediatrics, Eski?ehir, Turkey.
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pubmed:publicationType |
Journal Article,
Case Reports
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