1991
DOI: 10.1093/ndt/6.10.747
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End-Stage Renal Failure in Kartagener's Syndrome

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Cited by 11 publications
(8 citation statements)
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“…The association of SI totalis with nephrotic syndrome has been reported only four times so far. Osman et al [2] reported a case of amyloidosis due to bronchiectasis in a patient with SI similar to our case. This patient progressed to end stage renal failure (ESRD) and followed up under chronic hemodialysis treatment in contrast to our patient having normal renal functions.…”
Section: Open Accesssupporting
confidence: 87%
See 1 more Smart Citation
“…The association of SI totalis with nephrotic syndrome has been reported only four times so far. Osman et al [2] reported a case of amyloidosis due to bronchiectasis in a patient with SI similar to our case. This patient progressed to end stage renal failure (ESRD) and followed up under chronic hemodialysis treatment in contrast to our patient having normal renal functions.…”
Section: Open Accesssupporting
confidence: 87%
“…Primary ciliary dyskinesia (PCD) and resultant situs inversus (SI) totalis are very rare anomalies characterized by the total inversion of all abdominal and thoracic organs and chronic bronchiectasis [1]. The association of SI totalis with nephrotic syndrome has been reported only four times so far in which focal segmental glomerulosclerosis and amylodiosis were the etiologic factors [2][3][4][5]. Herein we report a case of nephrotic syndrome and amyloidosis due bronchiectasis in a patient with SI to talis.…”
Section: Introductionmentioning
confidence: 99%
“…Studies in human patients with polycystic kidney diseases have noted a high incidence of bronchiectasis, a previously uncharacterized manifestation of this disease (Driscoll et al, 2008). In addition, some patients with PCD also have retinitis pigmentosa or kidney failure (Bonneau et al, 1993;Moore et al, 2006;Osman et al, 1991). These results suggest that genes involved in BBS might act as modifiers of PCD.…”
Section: Rfx Proteins and Human Syndromesmentioning
confidence: 99%
“…Doma nski et al hypothesized that the underlying mechanism for ESRD was the disturbance of the transforming growth factor-b and Smad protein signaling because of both nephritic syndrome and SI totalis. 6 Some cases of renal amyloidosis in Kartagener's syndrome combined with SI, chronic sinusitis, and bronchiectasis have been reported; 7,8 however, the cases presented only had SI. In our report, cases 1 and 2 were siblings, which suggests that some genetic abnormalities may have influenced renal dysfunction, leading to ESRD.…”
Section: Discussionmentioning
confidence: 99%