FOCAL GLOMERULAR SCLEROSIS AND NEPHROTIC SYNDROME IN SPONDYLOEPIPHYSEAL DYSPLASIA

被引:9
作者
BOGDANOVIC, R
KOMAR, P
CVORIC, A
NIKOLIC, V
SINOTIC, M
ZDRAVKOVIC, D
OGNJANOVIC, M
ABINUN, M
机构
[1] INST MOTHER & CHILD HLTH, BELGRADE, YUGOSLAVIA
[2] INST PATHOL, BELGRADE, YUGOSLAVIA
关键词
FOCAL GLOMERULAR SCLEROSIS; NEPHROTIC SYNDROME; SPONDYLOEPIPHYSEAL DYSPLASIA;
D O I
10.1159/000187804
中图分类号
R5 [内科学]; R69 [泌尿科学(泌尿生殖系疾病)];
学科分类号
1002 ; 100201 ;
摘要
The association of a spondyloepiphyseal dysplasia and disproportionate short stature with focal glomerular sclerosis is reported in two girls. Renal disease manifested by proteinuria at the age of 2.5 and 11 years, leading to treatment-resistant nephrotic syndrome over 15 and 45 months, respectively. One patient went into end-stage renal failure shortly after nephrotic syndrome developed, the other died from sepsis. The association of spondyloepiphyseal dysplasia and focal glomerular sclerosis with nephrotic syndrome may represent a distinct disease entity.
引用
收藏
页码:219 / 224
页数:6
相关论文
共 19 条
[1]  
Bach C, 1967, Arch Fr Pediatr, V24, P23
[2]  
BURKE JR, 1983, EUR J PEDIATR, V140, P217
[3]   NEW VARIETY OF SPONDYLOEPIPHYSEAL DYSPLASIA CHARACTERIZED BY PUNCTATE CORNEAL-DYSTROPHY AND ABNORMAL DERMAL COLLAGEN FIBRILS [J].
BYERS, PH ;
HOLBROOK, KA ;
HALL, JG ;
BORNSTEIN, P ;
CHANDLER, JW .
HUMAN GENETICS, 1978, 40 (02) :157-169
[4]  
CHURG J, 1982, RENAL DISEASE CLASSI, P37
[5]  
EHRICH JHH, 1988, MONATSSCHR KINDERH, V136, P348
[6]   ASSOCIATION OF SPONDYLOEPIPHYSEAL DYSPLASIA WITH NEPHROTIC SYNDROME [J].
EHRICH, JHH ;
OFFNER, G ;
SCHIRG, E ;
HOYER, PF ;
HELMCHEN, U ;
BRODEHL, J .
PEDIATRIC NEPHROLOGY, 1990, 4 (02) :117-121
[7]   THORACIC ASPHYXIANT DYSTROPHY AND RENAL DISEASE [J].
HERDMAN, RC ;
LANGER, LO .
AMERICAN JOURNAL OF DISEASES OF CHILDREN, 1968, 116 (02) :192-&
[8]   SPONDYLOEPIPHYSEAL DYSPLASIA TARDA - A NEW AUTOSOMAL RECESSIVE VARIANT WITH MENTAL-RETARDATION [J].
KOHN, G ;
ELRAYYES, ER ;
MAKADMAH, I ;
ROSLER, A ;
GRUNEBAUM, M .
JOURNAL OF MEDICAL GENETICS, 1987, 24 (06) :366-369
[9]  
KOMAR P, 1988, PEDIATR RADIOL, V18, P277
[10]  
Lachman R S, 1975, Birth Defects Orig Artic Ser, V11, P231