Renal Transplantation in the Bardet-Biedl Syndrome, A Form of Laurence-Moon-Biedl Syndrome

Nordén, Gunnela; Friman, S.; Frisenette‐Fich, Carsten; Persson, Hans; Karlberg, Ingvar

doi:10.1093/ndt/6.12.982

Cited by 10 publications

(9 citation statements)

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“…Prakash [9] reported another 30-yearold patient with BBS who had cysts in the left kidney with normal renal function. ESRD in BBS patients has been reported at age range of 4-57 years [2][3][4][5][15][16][17][18][19]. Management of BBS is supportive and includes training and rehabilitation for blind and mentally retarded patients, hearing aids for deafness, and diet and exercise for obesity.…”

Section: Discussionmentioning

confidence: 99%

“…Still, it represents a very rare indication for kidney transplantation [7,8,15]. Renal transplantation is a viable option of renal replacement therapy in these patients.…”

Section: Discussionmentioning

confidence: 99%

“…With regard to genotype-phenotype correlation, patients with a mutation in the BBS6, BBS10, or BBS12 gene (10 of 33 patients) had more severe renal disease [8]. Still, it represents a very rare indication for renal transplantation [8,15,16]. Such renal transplant patients are known to be at increased risk for the development of severe obesity, malignancies and cardiovascular complications after transplantation [17].…”

Section: Discussionmentioning

confidence: 99%

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Bardet–Biedl Syndrome: A Rare Cause of Chronic Kidney Disease

et al. 2012

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Bardet-Biedl syndrome (BBS) is a rare autosomal recessive condition characterized by retinitis pigmentosa, postaxial polydactyly, central obesity, and renal involvement. Renal failure is the commonest cause of death. We report a case of BBS with chronic kidney disease (CKD) at younger age (17 year) from India. This diagnosis should be considered in patients with renal disease and the characteristic phenotype of retinitis pigmentosa, postaxial polydactyly and central obesity. These patients should undergo regular monitoring of renal function test to early diagnosis and treatment of CKD to prevent morbidity and mortality. Renal transplantation is a viable option of renal replacement therapy in these patients. These findings are valuable for comparing phenotype of BBS patients with CKD from various national and international centers.

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Section: Discussionmentioning

confidence: 99%

“…Still, it represents a very rare indication for kidney transplantation [7,8,15]. Renal transplantation is a viable option of renal replacement therapy in these patients.…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Bardet–Biedl Syndrome: A Rare Cause of Chronic Kidney Disease

et al. 2012

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“…Among the rare reports of renal transplantation in pediatric patients with Biedl-Bardet syndrome [10,11], one patient was reported to have multiple episodes of upper respiratory tract infections, including several episodes of severe sinusitis. Decreased mobility and impaired postoperative pulmonary ventilation in BiedlBardet syndrome may predispose patients to respiratory infections.…”

Section: Discussionmentioning

confidence: 99%

“…Of patients with Biedl-Bardet syndrome, 50%-90% suffer from renal disease ultimately leading to chronic renal failure [9]. Few pediatric patients with Biedl-Bardet syndrome and renal transplants have been reported [10,11]. Some reports suggest that posttransplant management in these patients may be more difficult than in otherwise healthy pediatric renal transplant recipients [11].…”

Section: Introductionmentioning

confidence: 99%

Obstructive airway disease caused by Moraxella catarrhalis after renal transplantation

et al. 2000

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We report a case of severe acute obstructive airway disease 2 months after renal transplantation in a 16-year-old patient with Biedl-Bardet syndrome who was transplanted for end-stage renal failure secondary to cystic kidney disease. Symptoms of severe obstructive airway disease developed 2 months after transplantation under immunosuppression with prednisone, azathioprine, and tacrolimus. The patient did not develop signs of infection; progressive shortness of breath remained the only symptom for several weeks. After extensive diagnostic evaluation, bronchoalveolar lavage revealed Moraxella catarrhalis as the single infectious agent. After 3 weeks of appropriate antibiotic therapy, symptoms of obstructive airway disease were completely relieved. This atypical presentation of Moraxella infection in an immunocompromised host represents a rare complication of renal transplantation, especially in young patients. Special aspects such as frequency, diagnosis, differential diagnosis, and management of this rare complication of renal transplantation in a pediatric patient are discussed.

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Renal transplantation in Bardet–Biedl Syndrome

et al. 2016

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At a median follow-up time of 97 months, relatively few complications of renal transplantation were reported in the patients of this study. However, body mass index was significantly elevated in transplanted individuals compared to non-transplanted individuals participating in CRIBBS at the most recent follow-up. Although the frequency of obesity and other manifestations of the metabolic syndrome warrant meticulous management in this high-risk population, favorable long-term outcomes suggest that renal transplantation is a viable option for patients with BBS and ESRD.

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Renal Transplantation in the Bardet-Biedl Syndrome, A Form of Laurence-Moon-Biedl Syndrome

Cited by 10 publications

References 0 publications

Bardet–Biedl Syndrome: A Rare Cause of Chronic Kidney Disease

Bardet–Biedl Syndrome: A Rare Cause of Chronic Kidney Disease

Obstructive airway disease caused by Moraxella catarrhalis after renal transplantation

Renal transplantation in Bardet–Biedl Syndrome

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