End-Stage Renal Failure in Kartagener's Syndrome

Osman, Eman; Abboud, Omar; Sulaiman, Suad M.; Musa, A.; Beleil, Omer M.; Sharfi, Amal

doi:10.1093/ndt/6.10.747

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%

“…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%

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A rare complication of a rare disease: Amyloidosis in situs inversus

Özkök¹,

Keskinler²,

Eken³

et al. 2013

OJIM

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A 31-year-old female admitted with productive cough and progressively increasing edema in her legs. Four years ago she had the diagnosis of situs inversus (SI) totalis. She had6.5 g/day proteinuria in 24-hour urine analysis. High resolution computed tomography revealed bronchiectatic areas in the left lung. In renal biopsy, kongo and amyloid AA antibody positivity was detected in arteriolar walls and mesangial areas. The patient is currently being followed with ramipril and colchicine treatments. Primary ciliary dyskinesia (PCD) and resultant SI totalis are very rare anomalies characterized by the total inversion of all organs and chronic bronchiectasis. There are very few reports on the association of SI totalis with nephrotic syndrome. Patients with SI totalis and chronic bronchiectasis have increased risk of AA amyloidosis and possibly end stage renal disease and thus these patients should be regularly followed with proteinuria and serum creatinine levels.

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Section: Open Accesssupporting
confidence: 87%

Section: Introductionmentioning
confidence: 99%

A rare complication of a rare disease: Amyloidosis in situs inversus
Özkök¹,
Keskinler²,
Eken³
et al. 2013
OJIM
0
0
0
0
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“…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%

RFX3 governs growth and beating efficiency of motile cilia in mouse and controls the expression of genes involved in human ciliopathies
Zein
¹
,
Aït-Lounis
²
,
Morlé
³

et al. 2009
Journal of Cell Science
109
8
116
1
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“…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%

Living‐donor kidney transplantation in an end‐stage renal disease patient with situs inversus totalis: Three case reports
Ochi
¹
,
Unagami
²
,
Akanuma
³

et al. 2019
IJU Case Reports
1
0
1
0
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Introduction Only few cases of renal dysfunction in patients with situs inversus totalis have been reported. Thus, studies on kidney transplantation in patients with this condition are still limited. Case presentation We present three cases of end‐stage renal disease patients with situs inversus totalis: a 30‐year‐old man, 21‐year‐old woman, and 31‐year‐old man. Each left living‐donor kidney was transplanted in the right iliac fossa in the usual way. Because of the anatomical reversal and right external iliac vein being deep, the internal iliac vein was cut for venous anastomosis in one patient. Another one patient developed temporary congestive kidney, which was speculated to be due to poor blood flow in the renal vein. All recipients could be weaned off dialysis, with stable allograft function. Conclusion Kidney transplantation in patients with situs inversus totalis is the same as anatomical normal cases, except that attention is paid to venous anastomosis.

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End-Stage Renal Failure in Kartagener's Syndrome

Cited by 11 publications

References 0 publications

A rare complication of a rare disease: Amyloidosis in situs inversus

A rare complication of a rare disease: Amyloidosis in situs inversus

RFX3 governs growth and beating efficiency of motile cilia in mouse and controls the expression of genes involved in human ciliopathies

Living‐donor kidney transplantation in an end‐stage renal disease patient with situs inversus totalis: Three case reports

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