Tratamiento anestésico para cesárea en una paciente con epidermolisis bullosa distrófica recesiva

García, I.; Manrique, Susana; Muñoz, C.; López-Gil, M.V.; Munar, F.; Montferrer, N.

doi:10.1016/s0034-9356(09)70460-8

Cited by 6 publications

(2 citation statements)

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“…As mentioned earlier there have been many reports on surgical procedures in patients with EB [14, 15, 16–19]. Moreover, a report of successful kidney transplant in patient with the same type of EB appeared in March of 2014.…”

Section: Discussionmentioning

confidence: 99%

“…Data on patients with EB attending surgery is promising. Lifesaving operations like appendectomy or even cardio surgical procedures are performed without any further complications [14, 15, 18, 19]. We expect however, that our patient is at higher risk of adhesions and fibrosis in the abdomen even though the operation itself does not involve opening the peritoneal cavity.…”

Section: Discussionmentioning

confidence: 99%

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End-stage kidney disease in patient with epidermolysis bullosa - what are the treatment options? – case report

2017

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BackgroundEpidermolysis bullosa is a group of diseases caused by mutations in genes for proteins responsible for cells’ anchorage at the dermo-epidermal junction. Their common feature are dysfunctional or even absent connections between cells. The typical clinical sign is the formation of blisters, with possible excessive scarring, in response to minimal skin irritation. End stage renal disease may be one of the comorbidities in patients with epidermolysis bullosa. The implementation of renal replacement therapy may be very difficult in this population. This is mainly due to problems in obtaining the proper dialysis access. The choice of appropriate method may be crucial for patient’s survival.Case presentationWe present a case of 29-year-old woman with Epidermolysis bullosa caused by laminin 5 gene mutation. The patient suffered from additional conditions: blindness, complete bilateral sensorineural deafness and oesophageal stenosis. When end stage renal disease was diagnosed, the problem of renal replacement therapy had to be faced. There have been few reports concerning ESRD in this specific group of patients in the available literature. In most of them the prognosis was very poor. Nevertheless, we were very determined to overcome all the difficulties. Special procedures and cooperation with the patient’s family allowed us to consider kidney transplantation as a treatment option.ConclusionThere should be no limitations in renal replacement therapy in patients with epidermolysis bullosa. Haemodialysis, peritoneal dialysis and kidney transplantation are all possible treatment options. Nevertheless, either method requires special procedures.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%