'신이식'에 해당되는 글 3건

  1. 2013.07.08 현미경적 혈뇨가 있는 신이식 공여자
2013. 7. 8. 10:45

현미경적 혈뇨가 있는 신이식 공여자


신이식을 준비하다보면 간혹 공여자에게서 현미경적인 혈뇨가 있는 경우 고민이 많이 됩니다.

물론 공여자의 신장 자체에 문제가 있는 경우는 대개 신이식 공여자로서 적응증이 되지 않기 때문에 이식을 못하는데,

Thin Basement Membrane disease 같은, 양성 경과를 보이는 사구체 질환에서는 어떻게 결정을 할 것인가가 문제가 됩니다.


사실 결론적으로 Thin basement membrane disease는 아니었지만, 비슷한 환자를 경험하면서

'만약 이 사람이 Thin basement membrane disease 라면 어떻게 할 것인가?'로 Discussion 하며 정리했던 자료가 있어 포스팅합니다.


# 2013 – ERBP Guideline on Kidney

 

Haematuria

We recommend considering persistent haematuria of glomerular origin as a contraindication to living donation, because it may indicate renal disease in the donor. (1B)

However, we acknowledge thin basement membrane disease might be an exception. (Ungraded statement)

공여자의 사구체 기원의 지속적 혈뇨는 금기로 권고한다.(1B)

하지만 Thin basement membrane disease는 예외일 수 있다.

 

# 2011 – BTS-Living donor KTP1

 

Glomerular pathology precludes donation, with the possible exception of thin basement membrane disease. (B1)

 

사구체 질환은 기증에 배제되어야 하지만, thin basement membrane disease 예외일 있다.(B1)

 

Thin basement membrane nephropathy

Thin basement membrane nephropathy (TBMN) is an autosomal dominant disorder often associated with mutations in either the COL4A3 or COL4A4 genes (encoding the 3 and 4 chains of type 4 collagen). Individuals in whom both alleles of either gene are abnormal may have autosomal recessive Alport syndrome, and TBMN can be regarded as the carrier state for this condition. TBMN is present in 10-50% of patients biopsied for PANVH (6,8,12,13) and although often considered a benign diagnosis may carry some risk of progression. Both proteinuria (10-20% of patients) and renal impairment (5%) have been described (16-18), often associated with additional pathological abnormalities including FSGS (18) or IgA nephropathy (19,20) (both of which would preclude donation).

TBMN 양성진단으로 생각되지만 진행의 위험 역시 존재한다. 단백뇨는 10-20%, 신손상은 5%에서 보고된 있고, FSGS IgAN 등과 연관된 경우도 있었다.

Many individuals with TBMN but otherwise normal investigations have undoubtedly donated kidneys, either knowingly (8) or unknowingly (9), and although adverse outcomes have not been reported these donors must be made aware of uncertainty over long-term safety.

많은 TBMN 경우에서 알고 하였거나 모르고 하였거나, 신이식을 하여서 나쁜 결과가 보고된 바는 없었으나 이러한 공여자는 장기 안전성의 불확실함에 대해서 충분히 인지를 하여야한다.

Referral to a clinical geneticist for molecular testing may be warranted, especially when donating to a family member with unexplained kidney failure or where there is a family history of sensori-neural deafness or haematuria (see also section 5.17 Familial Renal Disease). Referral to a geneticist is mandatory in potential donors of Cypriot origin, where associations of TBMN and FSGS leading to significant rates of renal failure have been noted (21).

공여자의 가족력에 원인 불명의 부전, 감각신경성 난청이나 혈뇨의 병력이 있을 경우에는 임상 유전학자에게 의뢰를 하여야한다.(키프로스 출신의 공여자에게서는 필수)

TBMN must be distinguished from the carrier state of X-linked Alport syndrome (XLAS), which is associated with a 5-20% risk of progressive renal impairment (22) and generally considered to prohibit donation.

TBMN 환자는 X-linked Alport syndrome carrier status 감별해야하며, 이런 경우 5-20% 진행성 신손상이 유발될 있어 공여를 해선 안된다. (아래는 이에 관련된 연구의 결과)

 A recent study describing six XLAS carriers who donated kidneys to their affected children supports this view (23). A decline in kidney function of between 25 and 60% was observed in four of the six donors over 2–14 years of follow-up, although in no case was creatinine clearance <40 ml/min. Four of the six developed microalbuminuria or proteinuria, and four developed hypertension. Some have argued that, if no other donor can be found, women with XLAS who are over the age of 45, have normal kidney function, no proteinuria and no hearing deficiency (both risk factors for progression to end-stage kidney disease) might be considered as donors after appropriate counselling (24). Involvement of a clinical geneticist would be mandatory in the screening of such a potential donor.

Trackback 0 Comment 0