ESPN 50th Annual Meeting

ESPN 2017


 
A very uncommon cause of acute kidney injury in infancy
DORVAL GUILLAUME 1 CHOMTON MARYLINE 2 KRID SAOUSSEN 1 RABANT MARION 3 DUONG JEAN-PAUL 3 ECKART PHILLIPE 4 MORTAMET GUILLAUME 5 DELACOURT CHRISTOPHE 6 DE SAINT-BLANQUAT LAURE 5 KRUG PAULINE 1 SALOMON RéMI 1 NEVEN BéNéDICTE 2 BOYER OLIVIA 1

1- DEPARTMENT OF PEDIATRIC NEPHROLOGY - ASSISTANCE PUBLIQUE HôPITAUX DE PARIS - NECKER ENFANTS-MALADES HOSPITAL - PARIS - FRANCE
2- DEPARTMENT OF PEDIATRIC IMMUNOLOGY AND HEMATOLOGY - ASSISTANCE PUBLIQUE HôPITAUX DE PARIS - NECKER ENFANTS-MALADES HOSPITAL - PARIS - FRANCE
3- DEPARTMENT OF PATHOLOGY - ASSISTANCE PUBLIQUE HôPITAUX DE PARIS - NECKER ENFANTS-MALADES HOSPITAL - PARIS - FRANCE
4- DEPARTMENT OF PEDIATRICS - UNIVERSITY HOSPITAL OF CAEN - FRANCE
5- PEDIATRICS INTENSIVE CARE UNIT - ASSISTANCE PUBLIQUE HôPITAUX DE PARIS - NECKER ENFANTS-MALADES HOSPITAL - PARIS - FRANCE
6- DEPARTMENT OF PEDIATRIC PNEUMOLOGY - ASSISTANCE PUBLIQUE HôPITAUX DE PARIS - NECKER ENFANTS-MALADES HOSPITAL - PARIS - FRANCE
 
Introduction:

Severe Combined Immuno-Deficiency (SCID) is rarely associated with non-infectious/non-toxic acute kidney injury (AKI).

Material and methods:

We report herein a unique cause of AKI related to lymphoproliferation, revealing a SCID.

Results:

A previously healthy four month-old boy was admitted for AKI (creatinine 363µM), hypertension, and edema, in the setting of a rhinovirus-positive bronchiolitis. He exhibited hyperechoic enlarged kidneys, nephrotic syndrome, anemia (8.8g/dL), thrombocytopenia (87000/mm3), elevated LDH, 1.7% schistocytes, and reticulocytes 135000/mm3. He was started on peritoneal dialysis. An atypical HUS was suspected and eculizumab was given. Despite rapid hematologic parameter normalization, renal function did not improved.  Kidney biopsy, performed after the fifth dose of eculizumab, revealed acute tubulointerstitial nephritis with lymphocyte and macrophage infiltration, inflammatory cells within the glomerular capillaries, with no evidence of thrombotic microangiopathy. Eculizumab was withdrawn, and three methylprednisolone pulses followed by oral prednisone were given without any improvement. Due to persistent lymphopenia and agammaglobulinemia, immune-deficiency was suspected and confirmed by immunophenotyping showing low T-cells (900 CD3/μl-93% γδ-T-cells), B-cells (70/μl) and NK-cells (10/μl) compatible with the diagnosis of SCID. Surprisingly, γδ-T cells were from maternal origin. Immunoscope showed oligoclonal profile of T-cells. Immunologic analyzes revealed an IL-2R common-γ chain defect causing X-linked SCID, and the diagnosis of materno-foetal graft-versus-host disease (MF-GVHD) caused by intrauterine materno-fetal transfusion was almost indisputable. He underwent successful geno-identical stem-cell transplantation at 7 months of age, followed by cadaveric kidney transplantation at the age of 3.4 years.

Conclusions:

Placenta barrier allows bidirectional passage of nucleated cells during pregnancy. While healthy infants reject these cells, profound immunodeficiency related to SCID allows persistence of maternal T-cells in newborns and peripheral expansion possibly driven by microorganisms. Maternal T-cell engraftment may be responsible for MF-GVHD mainly of skin, liver or digestive tract.  We report herein a highly singular case of isolated nephritis related to presumed MF-GVHD.