Myelodysplastic Syndrome and Minimal Change Nephrotic Syndrome treated with Steroid Challenge / 대한내과학회지

Glomerulonephritis associated with malignancy is deemed to be paraneoplastic glomerulonephritis. Myelodysplastic syndrome (MDS) is a group of heterogeneous clonal hematopoietic stem cell disorders characterized by impaired hematopoietic cell differentiation and cytopenia. The pathophysiology of MDS is thought to be immune-mediated in part. A few reports have documented various forms of glomerulonephritis in patients with MDS and suggested that immune dysregulation is important in the development of paraneoplastic glomerulonephritis. Here, we report a patient with MDS and refractory anemia with excess blast-2 accompanied by minimal change nephrotic syndrome. The patient was treated with prednisolone, and the nephrotic-range proteinuria and pancytopenia improved markedly.

A First Case of Osteomesopyknosis in Korea

Osteomesopyknosis is a rare sclerosing bone disorder of autosomal dominant inheritance. We report a first case of osteomesopyknosis in Korea. A 16-year old girl complained of diffuse back pain for 1 year. We performed physical examination, biochemical investigations and imaging studies. A radiograph of spine revealed rugger-jersey vertebra and sandwich vertebra. Bone specific alkaline phosphatase, osteocalcin and C-terminal telopeptides of type I collagen were normal. Only an axial skeleton involvement was shown on the whole body bone scan. This patient was diagnosed to have osteomesopyknosis. Osteomesopyknosis is characterized by normal level of bone turnover marker and an axial bone involvement. Osteomesopyknosis can be occurred in Korea and needs to be considered when patients, especially young patients, suffer from back pain and have only axial osteosclerosis.