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Fig. 2 | Orphanet Journal of Rare Diseases

Fig. 2

From: Genetic spectrum and clinical characteristics of 3β-hydroxy-Δ5-C27-steroid oxidoreductase (HSD3B7) deficiency in China

Fig. 2

Evolution of renal lesions. In patient P2: before the commencement of chenodeoxycholic acid (CDCA) administration, diminished corticomedullary differentiation and multiple small cystic were revealed with high signal on T2WI-FS (P2-A) and low signal on T1WI-Flash (P2-B) in bilateral renal medulla by MRI. This presented as bilateral renal sponge-like degeneration with point-like deposition of calcium salts seen by renal ultrasound (P2-C and P2-2D). After CDCA treatment for a period of about 33 months (2012.2–2014.11), MRI showed disappearance of the renal lesions in this patient (P2-E and P2-F). In patient P21: calcium deposition in both kidneys was noticed by ultrasound (P21-A and P21-B) at first referral, and normal kidneys were shown by CT scan 18mo after liver transplantation (P21-C)

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Orphanet Journal of Rare Diseases

ISSN: 1750-1172