Contributions
Abstract: PB1992
Type: Publication Only
Background
Fanconi anemia (FA) is an autosomal recessive genetically transmitted hereditary disease which predisposes the patient to progressive bone marrow failure, growth retardation, leukemia, and solid tumors. In nearly 50% of the children with FA, skeletal anomalies are found. Approximately 70% of them are upper extremity anomalies. The most frequently encountered problems are observed along the thumb-side, and radial edge of the forearm. Besides, much information is not available on wrist bones (proximal, central, and distal rows) and relevant anomalies in patients with FA.
Aims
We wanted to present wrist bone anomalies which we evaluated in 78 cases together with literature information.
Methods
Seventy-five cases with the diagnosis of FA followed up by 12 Departments of Hematology-Oncology from 9 different cities were evaluated by a single radiologist based on criteria defined in Greulich Pyle Hand Wrist Atlas Radiology, and changes of hand-wrist bones of all cases aged between 15 months, and 27 years were determined.
Results
Demographic characteristics, and hand-wrist bone anomalies of all cases were determined, and tabulated.
Wrist bones belong to a group of short bones. Congenital anomalies may be embryologically classified in the “underdevelopment group.” Embryogenesis of the upper extremity develops from lateral edge of the embryo at 4. week after fertilization. At 8. week of the fertilization, formation of all extremities is completed.
Conclusion
In the radiologic, endocrinologic, and orthopedic evaluation, and interpretation of wrist radiograms, the abovementioned signs, and symptoms of this disease should be also investigated.
Session topic: 24. Hematopoiesis, stem cells and microenvironment
Keyword(s): Fanconi anemia
Abstract: PB1992
Type: Publication Only
Background
Fanconi anemia (FA) is an autosomal recessive genetically transmitted hereditary disease which predisposes the patient to progressive bone marrow failure, growth retardation, leukemia, and solid tumors. In nearly 50% of the children with FA, skeletal anomalies are found. Approximately 70% of them are upper extremity anomalies. The most frequently encountered problems are observed along the thumb-side, and radial edge of the forearm. Besides, much information is not available on wrist bones (proximal, central, and distal rows) and relevant anomalies in patients with FA.
Aims
We wanted to present wrist bone anomalies which we evaluated in 78 cases together with literature information.
Methods
Seventy-five cases with the diagnosis of FA followed up by 12 Departments of Hematology-Oncology from 9 different cities were evaluated by a single radiologist based on criteria defined in Greulich Pyle Hand Wrist Atlas Radiology, and changes of hand-wrist bones of all cases aged between 15 months, and 27 years were determined.
Results
Demographic characteristics, and hand-wrist bone anomalies of all cases were determined, and tabulated.
Wrist bones belong to a group of short bones. Congenital anomalies may be embryologically classified in the “underdevelopment group.” Embryogenesis of the upper extremity develops from lateral edge of the embryo at 4. week after fertilization. At 8. week of the fertilization, formation of all extremities is completed.
Conclusion
In the radiologic, endocrinologic, and orthopedic evaluation, and interpretation of wrist radiograms, the abovementioned signs, and symptoms of this disease should be also investigated.
Session topic: 24. Hematopoiesis, stem cells and microenvironment
Keyword(s): Fanconi anemia