A 9-year-old boy is brought to the office for routine follow-up of his chronic renal disease. He has end-stage renal disease due to focal segmental glomerulosclerosis and was started on hemodialysis 3 months ago. The patient has had failure to thrive due to the disease. His nephrologist has recommended renal transplantation, and several family members have offered to donate a kidney. The patient lives with his mother, his maternal uncle, and his 2 siblings. His father died in a car accident 4 years ago. The patient's blood group is A+. His 7-year-old brother has blood group O-. His 19-year-old sister has blood group A+; she has uncontrolled major depressive disorder and was recently admitted to a psychiatric facility after a suicide attempt. His mother is 38 years old, has asthma, has normal kidney function, and has blood group A+. His uncle is 40 years old and has diabetes mellitus, hypertension, and left ventricular hypertrophy; the uncle has normal kidney function and has blood group A-. Physical examination reveals a boy who appears smaller than his stated age and in no acute distress. Cardiopulmonary examination is normal. Who is the most appropriate potential kidney donor?

Question

A 9-year-old boy is brought to the office for routine follow-up of his chronic renal disease.  He has end-stage renal disease due to focal segmental glomerulosclerosis and was started on hemodialysis 3 months ago.  The patient has had failure to thrive due to the disease.  His nephrologist has recommended renal transplantation, and several family members have offered to donate a kidney.  The patient lives with his mother, his maternal uncle, and his 2 siblings.  His father died in a car accident 4 years ago.  The patient's blood group is A+.  His 7-year-old brother has blood group O-.  His 19-year-old sister has blood group A+; she has uncontrolled major depressive disorder and was recently admitted to a psychiatric facility after a suicide attempt.  His mother is 38 years old, has asthma, has normal kidney function, and has blood group A+.  His uncle is 40 years old and has diabetes mellitus, hypertension, and left ventricular hypertrophy; the uncle has normal kidney function and has blood group A-.  Physical examination reveals a boy who appears smaller than his stated age and in no acute distress.  Cardiopulmonary examination is normal.  Who is the most appropriate potential kidney donor?

Quizplus · Accepted Answer

Explanation: 11ec2b69_c1c2_7628_8f63_65852ec87c6f_MD0005_00 Kidney transplantation is a life-saving procedure for patients with end-stage renal disease. Compared with chronic dialysis, transplantation is associated with increased survival, improved growth, and better quality of life. Advances in immunosuppressive therapy have increased both the pool of possible kidney donors and post-transplant survival. The most important factors in matching a kidney donor to a recipient include human leukocyte antigen (HLA) matching and ABO compatibility. In addition, kidney transplants from living donors, when available, have better outcomes than those from deceased donors (Choice A). Compared with deceased kidney donation, living donated kidneys have longer graft survival. Finally, transplant outcomes are best when the transplant can be performed before the patient requires dialysis (pre-emptive transplantation). Because living donor organs are available sooner, the need for dialysis can be limited or avoided entirely. Size is generally not a consideration, as transplanted kidneys are placed in a different location from the native kidneys. This patient's mother is a living, related (eg, HLA-similar), ABO-matched donor with no major medical problems and is therefore likely to be the best match. She should have a full medical, surgical, and psychosocial assessment. A single kidney is capable of sustaining an otherwise healthy donor, and long-term survival of donors is unaffected after nephrectomy. (Choices B and D) Siblings are generally good donors, as they have a 1 in 4 chance of a full HLA match based on inheritance patterns. However, neither of this patient's siblings would be ethically permitted to donate a kidney; the Organ Procurement and Transplant Network (OPTN) considers age <18 (brother) and untreated psychiatric disease (sister) to be absolute contraindications to organ donation. (Choice E) Diabetes and hypertension with end-organ damage (eg, left ventricular hypertrophy) are absolute contraindications to kidney donation according to the OPTN. Therefore, this patient's uncle is not a candidate for kidney donation. Educational objective: The optimal kidney donor for a child with end-stage renal disease is a living, related donor with an identical blood type. Donors must be adults who are capable of making an informed decision and must be free of major medical contraindications. References: Kidney transplantation in children. (http://www.ncbi.nlm.nih.gov/pubmed/25099579)

A 9-Year-Old Boy Is Brought to the Office for Routine