Branchio-oto-renal syndrome: A case report and review of literature

Bisanna, Jagannath; Gangaraj, Smitha Soubhagya

doi:10.4103/0971-7749.94500

Cited by 2 publications

(8 citation statements)

References 6 publications

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“…Mean weight of the patients was 11.69±2.16 kg and mean age was 3.60±1.59 years. Median follow-up was 9.5 (6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17) months. The most common cause of ESRD was nephrotic syndrome (26.9 %).…”

Section: Discussionmentioning

confidence: 99%

“…As medicine advances, oral health care professionals should have a holistic approach to the management of patients with complex medical problems especially to diseases of the renal system as it is pivotal in maintaining a stable internal environment and homeostasis. Mario Negri Institute For Pharmacological Research, 6 Arkana Laboratories, 7 The Hospital For Sick Children, 8 Columbia University, 9 Division Of Nephrology And Dialysis, Bambino Gesù Pediatric Hospital, 10 Apellis Pharmaceuticals Introduction: Complement 3 glomerulopathy (C3G) and immune complex membranoproliferative glomerulonephritis (IC-MPGN) are rare diseases characterized by excessive deposition of C3 breakdown products in renal glomeruli leading to proteinuria and progressive renal disease. Pegcetacoplan is a targeted C3 investigational therapy for diseases related to complement overactivation.…”

Section: Discussionmentioning

confidence: 99%

“…Hydronephrosis resolved spontaneously in 19, improved in 8 and remained stable in 1. Surgical correction required in a total of 17(37.8%) infants with a median age of 5.8 months (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12), 4 infants at first presentation and 13 while on conservative protocol. In these 13 infants, pyeloplasty was performed at a median age of 6.7 months (1-12) before a decrease in renal function on second renogram or parenchimal thinning on subsequent ultrasounds occured.…”

Section: Discussionmentioning

confidence: 99%

“…Rajiv Sinha 1 , Kausik Mandal 2 , Subal Pradhan 3 , Sushmita Banerjee 4 , Afsana Jahan 6 , Ramprasad V 7 , Amitava Pahari 5 1 Institute Of Child Health, 2 Sgpg, India, 3 Svppgi, Cuttack, India, 4 Cmri, Kolkata, India, 5 Apollo Hospital, Kolkata, India, 6 Pratiksha Hospital, Gauhati, India, 7 Medgenome, India Introduction: Currently data are scarce on tubulopathy genotype among Indian population. We hereby present Next Generation Sequencing (NGS) generated phenotype-genotype data among children with suspected tubular disorder from Eastern India.…”

Section: Pi-3 Next Generation Sequencing (Ngs) Data Among Children From Eastern India With Suspected Inherited Tubular Disorder: East Zonmentioning

confidence: 99%

“…Results: Four confirmed and two suspicious SARS-COvid-19 cases (4 males, 2 females) were reported. The mean age of patients was 4.2 (range, [1][2][3][4][5][6][7][8][9][10][11][12] years. Four cases with newly diagnosis of nephrotic syndrome who were on 2mg/kg /day prednisolon developed rapid rises of blood pressure.…”

Section: Ep-120 Hypertensive Crisis Asmentioning

confidence: 99%

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Abstracts of the 53rd ESPN Annual Meeting, Amsterdam, The Netherlands, September 2021

Louis¹,

Degraeuwe²,

Gasthuys³

et al. 2021

Pediatr Nephrol

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Introduction: Although several studies in children with steroid-resistant nephrotic syndrome (SRNS) have shown that mutations in genes encoding proteins in the podocyte skeleton may be responsible for the etiology in only one-third of cases, the genetic features related with renal prognosis and responde to immunosuppressive are not fully recognized. The aim of this study was to investigate the genetic alterations associate

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Pi-3 Next Generation Sequencing (Ngs) Data Among Children From Eastern India With Suspected Inherited Tubular Disorder: East Zonmentioning

confidence: 99%

Section: Ep-120 Hypertensive Crisis Asmentioning

confidence: 99%

See 3 more Smart Citations

Abstracts of the 53rd ESPN Annual Meeting, Amsterdam, The Netherlands, September 2021

Louis¹,

Degraeuwe²,

Gasthuys³

et al. 2021

Pediatr Nephrol

View full text Add to dashboard Cite

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Anatomical and audiological considerations in branchiootorenal syndrome: A systematic review

Biggs

Crundwell

Metcalfe

et al. 2022

Laryngoscope Investig Oto

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Objective Establish anatomical considerations, audiological outcomes, and optimal management in patients with branchiootic/branchiootorenal syndrome (BO/BOR). Methods Databases reviewed: Medline, Pubmed, Embase, Web of Science, Cochrane Collection, and http://clinicaltrials.gov. Clinical or radiological studies of patients with BOR syndrome describing either the audiological profile or anatomical changes were included. Articles in which BOR syndrome was associated with other syndromes, and those that were focused only on general and genetic aspects of BOR syndrome were excluded. Articles were assessed using Oxford Centre for Evidence‐Based Medicine (OCEBM) grading system and the Brazzelli risk of bias tool for nonrandomized studies. Results Searches identified 379 articles. Of these, 64 studies met the inclusion criteria, reporting outcomes in 482 patients from at least 95 families. In 308 patients, hearing loss was categorized as sensorineural (29%), conductive (20%), and mixed (51%). Hearing outcomes were variable in terms of onset, pattern, and severity; ranging from mild to profound deafness. One hundred sixty‐nine patients presented with inner ear anomalies, 145 had middle, and 151 had external ear abnormalities. In 44 studies, 58 ear operations were described. Mixed outcomes were reported in patients managed with hearing aids or middle ear surgery; however, successful cochlear implantation was described in all five cases. Conclusion The anatomical and audiological profiles of patients with BO/BOR are variable. A range of surgical procedures were described, however lacked objective outcome measures. Given the range of anatomical variants, management decisions should be made on an individual basis including full audiological and radiological assessment. Level of evidence NA.

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Branchio-oto-renal syndrome: A case report and review of literature

Cited by 2 publications

References 6 publications

Abstracts of the 53rd ESPN Annual Meeting, Amsterdam, The Netherlands, September 2021

Abstracts of the 53rd ESPN Annual Meeting, Amsterdam, The Netherlands, September 2021

Anatomical and audiological considerations in branchiootorenal syndrome: A systematic review

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