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Bariatric Surgery for a Patient With Myosin Heavy Chain 9-Related Disorders (MYH9RD): A Case Report
Sa-Hong Kim1, Kyoyoung Park1, Chungyoon Kim1
1Department of Surgery, Seoul National University Hospital, Seoul, Korea.
Insights
Myosin heavy chain 9-related disorders (MYH9RD) patients with morbid obesity can safely undergo bariatric surgery. Careful preoperative assessment and management enabled successful laparoscopic sleeve gastrectomy and significant health improvements.
Area of Science:
- Genetics and Medicine
- Nephrology
- Bariatric Surgery
Background:
- Myosin heavy chain 9-related disorders (MYH9RD) are rare genetic conditions characterized by macrothrombocytopenia and renal issues like focal segmental glomerulosclerosis (FSGS).
- Morbid obesity presents significant surgical risks, particularly in patients with pre-existing bleeding tendencies and renal impairment.
Observation:
- A 28-year-old male with MYH9RD and a BMI >47 kg/m² underwent laparoscopic sleeve gastrectomy.
- Preoperative management included platelet transfusions to address macrothrombocytopenia and bleeding risks.
- The patient had pre-existing renal impairment due to FSGS.
Findings:
- The surgery proceeded without complications, demonstrating the feasibility of bariatric procedures in MYH9RD patients.
- Significant weight loss (147.6 kg to 90.15 kg in 1 year) was achieved.
- Postoperative improvements were noted in hypertension, liver enzymes, HbA1c, triglycerides, and LDL levels.
Implications:
- This case underscores the importance of meticulous preoperative evaluation and risk mitigation strategies for MYH9RD patients considering bariatric surgery.
- Comprehensive management can lead to successful surgical outcomes and substantial health benefits in this complex patient population.
- While not directly linked, MYH9RD management should consider the potential benefits of weight loss in obese individuals.
Abstract:
Myosin heavy chain 9-related disorders (MYH9RD) are rare autosomal dominant genetic conditions caused by MYH9 mutations, leading to macrothrombocytopenia, renal complications such as focal segmental glomerulosclerosis (FSGS), and other systemic manifestations. We report a case of 28-year-old male with MYH9RD and body mass index exceeding 47 kg/m2, who successfully underwent laparoscopic sleeve gastrectomy. Despite challenges from bleeding tendency caused by macrothrombocytopenia and renal impairment caused by FSGS, thorough preoperative evaluation and management, including platelet transfusion, enabled surgery to proceed without complications. The patient achieved significant weight loss, from 147.6 kg preoperatively to 90.15 kg at 1 year postoperatively, with improvements in hypertension and metabolic parameters, including aspartate aminotransferase/alanine aminotransferase, hemoglobin A1c, triglycerides, and low-density lipoprotein levels. While MYH9RD is not directly associated with morbid obesity, this case highlights that comprehensive preoperative evaluation and risk management can lead to successful outcomes in bariatric surgery for MYH9RD patients.
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