The Turkish Journal of Pediatrics 2019 , Vol 61 , Num 1
BK virus associated nephropathy and severe pneumonia in a kidney transplanted adolescent with Schimke immuneosseous- dysplasia
Ali Düzova 1 ,Bora Gülhan 1 ,Rezan Topaloğlu 1 ,Fatih Özaltın 1-2 ,Ali Bülent Cengiz 3 ,Ayşe Filiz Yetimakman 4 ,Deniz Doğru 5 ,Şafak Güçer 6 ,Nesrin Beşbaş 1
1 Department of Pediatrics Division of Pediatric Nephrology, Hacettepe University Faculty of Medicine, Ankara, Turkey
2 Nephrogenetics Laboratory, Hacettepe University Faculty of Medicine, Ankara, Turkey
3 Division of Pediatric Infectious Diseases, Hacettepe University Faculty of Medicine, Ankara, Turkey
4 Division of Pediatric Intensive Care, Hacettepe University Faculty of Medicine, Ankara, Turkey
5 Division of Pediatric Pulmonary Medicine, Hacettepe University Faculty of Medicine, Ankara, Turkey
6 Department of Pediatric and Perinatal Pathology Research, Hacettepe University Faculty of Medicine, Ankara, Turkey
DOI : 10.24953/turkjped.2019.01.018 Düzova A, Gülhan B, Topaloğlu R, Özaltın F, Cengiz AB, Yetimakman AF, Doğru D, Güçer Ş, Beşbaş N. BK virus associated nephropathy and severe pneumonia in a kidney transplanted adolescent with Schimke immune-osseous-dysplasia. Turk J Pediatr 2019; 61: 111-116.

Patients with juvenile onset Schimke immune-osseous-dysplasia (SIOD) have less severe symptoms and can survive in the second and third decade of life. We present an 18 year-old adolescent with juvenile onset SIOD who was diagnosed after renal transplantation and developed BK virus associated nephropathy (BKVAN) and severe pneumonia during follow-up. The patient developed nephrotic syndrome, unresponsive to immunosuppressives, at the age of 8 years. He had a history of meningitis, short stature, microcephaly, prominent ears, and bilateral cryptorchidism. A renal transplantation was performed at the age of 15 years. During follow-up, he suffered from leucopenia, urinary tract infections, herpes labialis, and candida esophagitis. Sanger sequencing of SMARCAL1 revealed a missense mutation on exon 11 (R586W). A renal biopsy performed after a sharp increase in serum creatinine (without significant viremia) revealed BKVAN which responded to sirolimus monotherapy and cidofovir. Three months later, he suffered from productive cough and dyspnea with diffuse ground glass pulmonary infiltrates. His clinical situation deteriorated and non-invasive mechanical ventilation was started. Cidofovir (2 mg/kg) was re-started weekly for a possible BKV pneumonia with intravenous immunoglobulin. After 5 doses of cidofovir and intense antibiotic regime, his dyspnea resolved with stable graft functions. In our case; BKVAN, which developed without significant viremia, and possibly associated pneumonia were treated successfully with cidofovir and sirolimus monotherapy. Keywords : BK virus associated nephropathy, kidney transplant, Schimke immuneosseous- dysplasia, pneumonia, sirolimus

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