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BRIEF COMMUNICATION
Year : 2013  |  Volume : 56  |  Issue : 3  |  Page : 272-275

Focal segmental glomerulosclerosis associated with maternally inherited diabetes and deafness: Clinical pathological analysis


State Discipline and State Key Laboratory of Kidney Disease (Chinese PLA General Hospital, 2011DAV00088), Beijing 100853, PR China

Correspondence Address:
Ri-Bao Wei
State Discipline and State Key Laboratory of Kidney Disease, (Chinese PLA General Hospital, 2011DAV00088), Beijing 100853, PR China

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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.120392

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Maternally inherited diabetes and deafness (MIDD), which is caused by an A to G substitution at position 3243 (m.3243A>G) in the transfer ribonucleic acid leucine gene, is characterized by diabetes and hearing loss. Patients with MIDD frequently have renal disease, which may precede the diagnosis of either diabetes or deafness or may be the sole manifestation of the m.3243A>G mutation. Recently, progressive renal failure was reported in adults, and a number of childhood cases of focal segmental glomerulosclerosis (FSGS) of MIDD have been reported. However, little is known about the glomerular lesions in FSGS in MIDD. In the present study, we reported two cases of FSGS associated with MIDD and studied the clinical features of the proband and her mother.


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