Primary Hyperoxaluria Type 1: Improved Outcome With Timely Liver Transplantation: A Single-Center Report of 36 Children

Shapiro, Rivka; Weismann, Irit; Mandel, Hana; Eisenstein, Bela; Ben‐Ari, Ziv; Bar-Nathan, N; Zehavi, I; Dinari, Gabriel; Mor, Eytan

doi:10.1097/00007890-200108150-00012

Cited by 82 publications

(57 citation statements)

References 13 publications

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“…By 15 years of age 50% of patients will have developed ESRD. [1,3,7] As the glomerular filtration rate declines, oxalate is deposited systemically into bone and soft tissues including the retina, blood vessels, nerves and heart. [6] If the condition is diagnosed early, and before significant renal failure is established, isolated liver transplantation (iLT) is an acceptable option, with excellent survival.…”

Section: Discussionmentioning

confidence: 99%

“…[6] If the condition is diagnosed early, and before significant renal failure is established, isolated liver transplantation (iLT) is an acceptable option, with excellent survival. [7] However, this only addresses the issue of oxalate formation and does not resolve the problem of an already high burden of oxalate, which would force the kidneys to eliminate the remaining stores. This can contribute to progressive CKD requiring a renal transplant at a later stage.…”

Section: Discussionmentioning

confidence: 99%

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Combined paediatric liver-kidney transplantation: Analysis of our experience and literature review

et al. 2013

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Background. Renal insufficiency is increasingly common in end-stage liver disease and allocation of livers to this category of patient has escalated. The frequency of combined liver-kidney transplantation (CLKT) has consequently increased. Indications for CLKT in children differ from those for adults and typically include rare congenital conditions; subsequently limited numbers of this procedure have been performed in paediatric patients worldwide. Scant literature exists on the subject. Methods. Subsequent to institutional approval, a retrospective chart analysis of all paediatric CLKTs performed at the Transplant Unit, Wits Donald Gordon Medical Centre, University of the Witwatersrand, Johannesburg, South Africa between January 2005 and July 2013 was conducted. Results. Defining children as younger than 18 years of age, 43 patients had received a liver transplant since 2005, of whom 8 received a CLKT. Indications included autosomal recessive polycystic kidney disease (n=3), primary hyperoxaluria type 1 (n=4) and heterozygous factor H deficiency with atypical haemolytic uraemic syndrome (n=1). Graft combinations included whole liver and one kidney (n=5), whole liver and two kidneys (n=1) and left lateral liver segment and one kidney (n=2), all from deceased donors. Patient age ranged from 4 to 17 years (median 9) and included 4 females and 4 males. Weight ranged from 13 to 42 kg (median 22.5). We describe one in-hospital mortality. The remaining 7 patients were long-term survivors with a survival range from 6 to 65 months. Conclusions. Although rarely indicated in children, CLKT is an effective treatment option, appropriately utilising a scarce resource and significantly improving quality of life in the recipient.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Combined paediatric liver-kidney transplantation: Analysis of our experience and literature review

et al. 2013

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“…18 Opinions differ regarding primary hyperoxaluria type 1 (PH1). Some centers offer pre-emptive liver transplantation 38,39 to correct the metabolic defect before the development of end-stage renal disease, others early combined liver-kidney transplantation or single kidney transplantation. Considering United Network for Organ Sharing 40 and European PH1 Transplantation Study Group 41 data, isolated kidney transplantation in these patients resulted in a strikingly high rate of early graft loss because of oxalate recurrence.…”

Section: Discussionmentioning

confidence: 99%

Results of combined and sequential liver-kidney transplantation

Becker¹,

Nyibata²,

Lueck³

et al. 2003

Liver Transplantation

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“…O momento ideal para a realização do transplante renal ou hepático é antes do desenvolvimento da oxalose sistêmica avança-da 19 . Como a enzima aminotransferase alanina:glioxilato é encontrada em quantidade significativa somente no fígado, e está localizada dentro dos peroxissomas hepáticos, o transplante hepático há 10 anos tem sido empregado como meio terapêutico mais eficiente nessa patologia, bem como transplantes combinados de fígado e rim, com resultados favoráveis 3,20,21 . Um total de 98 transplantes combinados (rim e fígado) em 93 pacientes foram relatados na Europa, com taxa de sobrevida em 10 anos de 65% 22, 23 .…”

Section: Discussionunclassified

Primary hyperoxaluria with endstage renal failure in an infant

Macedo¹,

Yoshida²,

Viero³

et al. 2002

J Pediatr (Rio J)

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ResumoObjetivo: descrever caso clínico de um lactente com insuficiên-cia renal crônica terminal, causada por hiperoxalúria primária.Método: após revisão da literatura, verifica-se a raridade da doença; na França, a prevalência é de 1,05/milhão, com taxa de incidência de 0,12/milhão/ano. Pesquisa abordando centros especializados mundiais detectou, em 1999, 78 casos em lactentes; destes, em 14% o quadro inicial foi de uremia. A gravidade e a raridade da doença sugerem o relato deste caso.Resultados: criança de sexo feminino, com quadro de vômitos e baixo ganho de peso desde os primeiros meses de vida, desenvolveu insuficiência renal terminal aos 6 meses de idade, sendo mantida em tratamento dialítico desde então. Aos 8 meses, foi encaminhada para esclarecimento diagnóstico, apresentando déficit pôndero-estatural grave e os seguintes exames laboratoriais: uréia= 69 mg/dl, creatinina=2,2 mg/dl e clearance de creatinina= 12,5 ml/min/1.73m 2 SC. O exame de urina foi normal, a ultra-sonografia renal revelou tamanho normal e hiperecogenicidade de ambos os rins. A dosagem de oxalato urinário foi de 9,2mg/kg/dia ou 0,55 mmol/1.73m 2 SC, e a relação oxalato:creatinina, de 0,42. A biópsia renal diagnosticou presença de grande quantidade de depósitos de cristais de oxalato de cálcio no parênquima renal. A radiografia de ossos longos evidenciou sinais sugestivos de osteopatia oxalótica, e a fundoscopia indireta, sinais de retinopatia por oxalato. A criança foi mantida em diálise peritoneal ambulatorial contínua, tendo sido iniciado tratamento com piridoxina.Conclusões: a hiperoxalúria primária deve ser considerada como um dos diagnósticos diferenciais de insuficiência renal crôni-ca em lactentes, especialmente na ausência de história sugestiva de outras patologias.J Pediatr (Rio J) 2002; 78 (2): 171-5: lactente, insuficiência renal crônica terminal, hiperoxalúria primária, oxalose sistêmica. AbstractObjective: to report a case of an infant with endstage renal failure caused by primary hyperoxaluria.Methods: the review of the literature showed the rarity of the disease. In France, the prevalence is about 1.05/million and the incidence rate is 0.12/million/year. A survey, performed in international specialized centers in 1999, documented 78 cases in infants; in 14% of them the initial onset symptom was uremia. The rarity and severity of the disease justify the description of this case.Results: a girl presenting vomiting and failure to thrive within the first months of life developed endstage renal failure at 6 months of age. She was being treated with dialysis. At 8 months of age, she was referred for investigation. She was undernourished and the laboratory examinations showed urea (69mg/dl), creatinine (2.2 mg/ dl) and creatinine clearance (12.5 ml/min/1.73m 2 SA). The routine urinalysis was normal, the renal ultrasound showed increased echogenicity in both kidneys; the dosage of urinary oxalate was 9.2mg/kg/day or 0.55mmol/1.73m 2 SA; the urinary oxalate/creatinine ratio was 0.42. Renal biopsy showed calcium oxalate crystals ...

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Primary Hyperoxaluria Type 1: Improved Outcome With Timely Liver Transplantation: A Single-Center Report of 36 Children

Cited by 82 publications

References 13 publications

Combined paediatric liver-kidney transplantation: Analysis of our experience and literature review

Combined paediatric liver-kidney transplantation: Analysis of our experience and literature review

Results of combined and sequential liver-kidney transplantation

Primary hyperoxaluria with endstage renal failure in an infant

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