Recombinant human erythropoietin α in the correction of anaemia in epidermolysis bullosa

Antunes, Isabel; Resende, Carlos Xavier; Granjo, Elisa; Mesquita-Guimarães, J

doi:10.1111/j.1468-3083.1999.tb01016.x

Cited by 9 publications

(14 citation statements)

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“…65,70,71 Although low endogenous erythropoietin levels have been found in some anemic EB patients, 65,71 elevated levels have been found in others. 65,70 Despite anemia driving the increased secretion of erythropoietin, animal studies have shown reduced erythropoietin levels associated with protein depletion.…”

Section: Anemia Key Points D Severe Multifactorial Anemia Is a Complimentioning

confidence: 91%

“…72 Because many anemic EB patients also have significant nutritional compromise and low serum albumin levels, 64 these may contribute to the low observed erythropoietin levels in some patients. 65,71 However, even in patients with elevated endogenous erythropoietin levels, a combination of intravenous ferric gluconate or intramuscular iron dextran, given with weekly subcutaneous darbepoetin alfa, improved energy levels and hemoglobin measurements in four patients with RDEB. 70 Drawbacks to treatment with erythropoietin include the need for intravenous or subcutaneous administration and the high cost.…”

Section: Anemia Key Points D Severe Multifactorial Anemia Is a Complimentioning

confidence: 98%

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Extracutaneous manifestations and complications of inherited epidermolysis bullosa

Fine

Mellerio

2009

Journal of the American Academy of Dermatology

241

178

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Section: Anemia Key Points D Severe Multifactorial Anemia Is a Complimentioning

confidence: 91%

Section: Anemia Key Points D Severe Multifactorial Anemia Is a Complimentioning

confidence: 98%

Extracutaneous manifestations and complications of inherited epidermolysis bullosa

Fine

Mellerio

2009

Journal of the American Academy of Dermatology

241

178

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“…Patients with RDEB often present with severe anemia, becoming dependent on multiple blood transfusions (Antunes et al, 1999, Fine and Hintner, 2009). …”

Section: Introductionmentioning

confidence: 99%

Prevalence of anemia in patients with epidermolysis bullosa registered in Australia

Hwang

Daniel

Fergie

et al. 2015

International Journal of Women's Dermatology

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BackgroundAnemia is a common complication of epidermolysis bullosa (EB). To date, no extensive data on the prevalence of anemia in EB patients have been well characterized worldwide.ObjectiveTo determine and to characterize the prevalence of anemia in the Australian EB population by conducting a retrospective cross-sectional study.MethodsAll (n = 368) EB patients registered in the Australasian Epidermolysis Bullosa Registry (AEBR) from 2006 to 2012 were reviewed for pathological evidence of anemia. Patients with EB without anemia and those without hematological parameters were excluded from the study. Patients’ particulars were separated into pediatric (< 18 years old) and adult (≥ 18 years old) male and female subgroups.ResultsOne-hundred sixty-nine out of 368 EB patients had eligible blood results to be analyzed, as milder forms of EB did not routinely have laboratory testing; 27.8% (n = 47/169) of EB patients were anemic at any time point in their lifetime. All generalized severe junctional EB (JEB-GS) cases (100%, n = 4/4); 68.0% (n = 17/25) of recessive dystrophic EB (RDEB); and 37.5% (n = 6/16) of generalized intermediate JEB (JEB-I) patients were anemic.LimitationsAs EB is an orphan disease, the limited sample size may have affected the significance of the study result.ConclusionThe high prevalence of anemia seen in RDEB and JEB generalized severe (JEB-GS) patients in our cohort is similar to those reported in case series.

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“…Proteinuria, hypertension and renal failure are prognostic factors clinically relevant to the evolution of IgAN, whereas mesangial hypercellularity, glomerulosclerosis, interstitial fibrosis, and elevated numbers of crescents are the histological ones [10]. As shown by an Italian study [12], it is reasonable to reserve immunosuppressive therapy for patients with severe clinical and histological risk factors, also considering the risk of poorly controllable infections.…”

Section: Discussionmentioning

confidence: 97%

“…EB-induced anaemia could be corrected by recombinant human erythropoietin, as preparation for renal biopsy rather than support therapy for clinically significant anaemia [10]. Finally, renal biopsy revealed IgA nephropathy (IgAN), so that the treatment was conditioned by the presence of negative clinical prognostic factors and the histological characteristics of the renal biopsy [11].…”

Section: Discussionmentioning

confidence: 99%

End-stage renal disease secondary to IgA nephropathy in recessive dystrophic epidermolysis bullosa: a case report

et al. 2008

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Epidermolysis bullosa (EB) consists of a group of dominant or recessive autosomal diseases characterised by skin and mucosa fragility. The lesions leave erosions and scars that, in turn, can cause stenosis of tracheal, oesophageal, and genitourinary tract mucosae. The significantly increased survival of EB patients has determined the onset of complications never observed before, including genitourinary disorders such as hydroureteronephrosis, recurrent urinary tract infections, renal amyloidosis, IgA nephropathy and post-infectious glomerulonephritis. A 6-year-old boy diagnosed with recessive dystrophic EB Hallopeau-Siemens type (RDEB-HS) was referred to our clinic because of microhaematuria that evolved into intra-infectious macrohaematuria. Renal biopsy revealed an increase in both extracellular matrix and mesangial cells, with a focal segmental glomerulosclerosis with severe chronic tubulointerstitial damage. Immunofluorescence showed IgA mesangium deposits. Five years later, he was started on haemodialysis, because of worsening renal function. This is a rare case of a child with EB who was successfully treated with haemodialysis. The pertinent literature has been reviewed.

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Recombinant human erythropoietin α in the correction of anaemia in epidermolysis bullosa

Cited by 9 publications

References 1 publication

Extracutaneous manifestations and complications of inherited epidermolysis bullosa

Extracutaneous manifestations and complications of inherited epidermolysis bullosa

Prevalence of anemia in patients with epidermolysis bullosa registered in Australia

End-stage renal disease secondary to IgA nephropathy in recessive dystrophic epidermolysis bullosa: a case report

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