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Renal Biopsy-induced Hematoma and Infection in a Patient with Asymptomatic May-Hegglin Anomaly
- Source :
- Journal of Nippon Medical School = Journal of Nippon Medical School. 88(6):579-584
- Publication Year :
- 2021
- Publisher :
- The Medical Association of Nippon Medical School, 2021.
-
Abstract
- The May-Hegglin anomaly is characterized by inherited thrombocytopenia, giant platelets, and leukocyte cytoplasmic inclusion bodies. The Fechtner, Sebastian, and Epstein syndromes are associated with mutations of the MYH9-coding nonmuscle myosin heavy chain ⅡA, similar to the May-Hegglin anomaly, and are together classified as MYH9 disorders. MYH9 disorders may include symptoms of Alport syndrome, including nephritis and auditory and ocular disorders. A 6-year-old boy was diagnosed with an MYH9 disorder after incidental discovery of hematuria and proteinuria. Focal segmental glomerulosclerosis was detected on renal biopsy. However, despite no prior bleeding diatheses, he developed a large post-biopsy hematoma despite a preprocedural platelet transfusion calculated to increase the platelet count from 54,000/μL to >150,000/μL. Idiopathic thrombocytopenic purpura is a major cause of pediatric thrombocytopenia following acute infection or vaccination, and patients with MYH9 disorders may be misdiagnosed with idiopathic thrombocytopenic purpura and inappropriately treated with corticosteroids. Careful differential diagnosis is important in thrombocytopenic patients with hematuria and proteinuria for the early detection of thrombocytopenia. Patients with MYH9 disorders require close follow-up and treatment with angiotensin Ⅱ receptor blockers to prevent the onset of progressive nephritis, which may necessitate hemodialysis or renal transplantation. The need for renal biopsy in patients with MYH9 disorders should be carefully considered because there could be adverse outcomes even after platelet transfusion.
- Subjects :
- Male
medicine.medical_specialty
Biopsy
Hearing Loss, Sensorineural
Gastroenterology
Asymptomatic
03 medical and health sciences
0302 clinical medicine
Focal segmental glomerulosclerosis
Internal medicine
medicine
Humans
Child
Hematuria
Hematoma
Purpura, Thrombocytopenic, Idiopathic
medicine.diagnostic_test
Myosin Heavy Chains
business.industry
Glomerulosclerosis, Focal Segmental
General Medicine
medicine.disease
Thrombocytopenic purpura
Thrombocytopenia
Transplantation
Proteinuria
Platelet transfusion
030220 oncology & carcinogenesis
May–Hegglin anomaly
030211 gastroenterology & hepatology
Renal biopsy
medicine.symptom
business
Nephritis
Subjects
Details
- Language :
- English
- ISSN :
- 13454676
- Volume :
- 88
- Issue :
- 6
- Database :
- OpenAIRE
- Journal :
- Journal of Nippon Medical School = Journal of Nippon Medical School
- Accession number :
- edsair.doi.dedup.....32902b14efbd841ff6060e1b2112a3ee