Dysmetabolic nephropathy in children with hereditary connective tissue dysplasia
E. A. Yurieva,
V. V. Dlin,
E. S. Vozdvizhenskaya, 
V. S. Sukhorukov,
A. N. Semyachkina,
M. N. Kharabadze
V. S. Sukhorukov,
A. N. Semyachkina,
M. N. Kharabadze https://doi.org/10.21508/1027-4065-2020-65-1-71-76
Abstract
Nephropathy is a common associated pathology with hereditary connective tissue dysplasia
Purpose. To determine clinical and laboratory signs of renal pathology in the conditions of persisting hypoxic syndrome and anatomic abnormalities of the urinary system with hereditary connective tissue dysplasia syndromes in children
Characteristics of children and research methods. We examined 36 children with Ehlers–Danlos syndrome and 10 children with Marfan syndrome to reveal signs of metabolic disorders in the blood and urine.
Results. All children revealed abnormalities of the urinary system. In addition, children with Ehlers–Danlos syndrome demonstrated an increase in certain signs of dysmetabolic nephropathy when growing older: an increased content of parathyroid hormone in the blood, which inactivation and elimination is normally provided by the kidneys. Hypermicroproteinuria with a high content of microelements in proteins, increased excretion of medium molecules, lipid hydroperoxides, glycosaminoglycans, a decrease in antioxidant defense and crystal formation inhibitors are the characteristic signs of dysmetabolic nephropathy.
Conclusion. Children with hereditary connective tissue dysplasia syndromes have a risk of developing nephropathy with signs characteristic of dysmetabolic nephropathy, requiring dynamic monitoring by a nephrologist.
About the Authors
E. A. Yurieva
Veltischev Research and Clinical Institute for Pediatrics of the Pirogov; Russian National Research Medical University
Russian Federation
Moscow
Russian Federation
Moscow
V. V. Dlin
Veltischev Research and Clinical Institute for Pediatrics of the Pirogov; Russian National Research Medical University
Russian Federation
Moscow
Russian Federation
Moscow
E. S. Vozdvizhenskaya
Veltischev Research and Clinical Institute for Pediatrics of the Pirogov; Russian National Research Medical University
Russian Federation
Moscow
Russian Federation
Moscow
V. S. Sukhorukov
Research Centre of Neurology
Russian Federation
Moscow
Russian Federation
Moscow
A. N. Semyachkina
Veltischev Research and Clinical Institute for Pediatrics of the Pirogov; Russian National Research Medical University
Russian Federation
Moscow
Russian Federation
Moscow
M. N. Kharabadze
Veltischev Research and Clinical Institute for Pediatrics of the Pirogov; Russian National Research Medical University
Russian Federation
Moscow
Russian Federation
Moscow
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Yurieva E.A., Dlin V.V., Vozdvizhenskaya E.S., Sukhorukov V.S., Semyachkina A.N., Kharabadze M.N. Dysmetabolic nephropathy in children with hereditary connective tissue dysplasia. Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics). 2020;65(1):71-76. (In Russ.) https://doi.org/10.21508/1027-4065-2020-65-1-71-76
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