Hemophilia is a recessive inherited disorder linked to the
X chromosome. On July 28, 2020, a case of
hemophilia A with severe left
kidney hydronephroses and acute left
kidney hemorrhage was admitted to the Affiliated
Hospital of Qinghai
University. The
patient had a
family history of
hemophilia A and activated partial
thromboplastin before
surgery. The APTT was 95.0s (
reference value 22.7-31.8s), factor Ⅷ activity (FⅧ∶C) was 3.2% (
reference value 70%-150%), and B-
ultrasonic examination revealed severe
hydronephroses in the left
kidney, and the nephrogram indicated that the left
kidney was absent of
perfusion. On the 9th day of admission, the
pain in the left lower
back was aggravated than before. The CT scan of the whole
abdomen revealed irregular patches with slightly higher density in the left
kidney. Considering
bleeding, through multidisciplinary
consultation and perioperative infusion of
human coagulation factor Ⅷ, the
patient was underwent retroperitoneal laparoscopic left
nephrectomy. There was no
bleeding during and after operation. After 20 months of follow-up, the renal function and coagulation function were normal, and there were no symptoms of
hematuria and
low back pain.