In Vivo Assessment of Neurodegeneration in Type C Niemann-Pick Disease by IDEAL-IQ

OBJECTIVE: To noninvasively assess the neurodegenerative changes in the brain of patients with Niemann-Pick type C (NPC) disease by measuring the lesion tissue with the iterative decomposition of water and fat with echo asymmetry and least square estimation-iron quantification (IDEAL-IQ). MATERIALS AND METHODS: Routine brain MRI, IDEAL-IQ and 1H-proton magnetic resonance spectroscopy (1H-MRS, served as control) were performed on 12 patients with type C Niemann-Pick disease (4 males and 8 females; age range, 15–61 years; mean age, 36 years) and 20 healthy subjects (10 males and 10 females; age range, 20–65 years; mean age, 38 years). The regions with lesion and the normal appearing regions (NARs) of patients were measured and analyzed based on the fat/water signal intensity on IDEAL-IQ and the lipid peak on 1H-MRS. RESULTS: Niemann-Pick type C patients showed a higher fat/water signal intensity ratio with IDEAL-IQ on T2 hyperintensity lesions and NARs (3.7–4.9%, p < 0.05 and 1.8–3.0%, p < 0.05, respectively), as compared to healthy controls (HCs) (1.2–2.3%). After treatment, the fat/water signal intensity ratio decreased (2.2–3.4%), but remained higher than in the HCs (p < 0.05). The results of the 1H-MRS measurements showed increased lipid peaks in the same lesion regions, and the micro-lipid storage disorder of NARs in NPC patients was detectable by IDEAL-IQ instead of 1H-MRS. CONCLUSION: The findings of this study suggested that IDEAL-IQ may be useful as a noninvasive and objective method in the evaluation of patients with NPC; additionally, IDEAL-IQ can be used to quantitatively measure the brain parenchymal adipose content and monitor patient follow-up after treatment of NPC.

STUDY ON APOPTOSIS IN DEVELOPING RENAL CORPUSCLES OF MOUSE / 解剖学报

Objective To study apoptosis and morphological characteristics in the developing renal corpuscles of mouse kidney. Methods Light,transmission electron microscope and TUNEL technique were used to observe apoptosis in the developing renal corpuscles of different embryonic and postnatal mice. Results Apoptotic cells could be found when renal corpuscles occurred at embryonic day 14(E14).It peaked around E18 and decreased thereafter.Electron microscope revealed that apoptotic cells had morphological characteristics such as margination of condensed nuclear chromatin,shrinking cytoplasm.Apoptosis of endothelial cells and podocytes were more frequent.Two results of apoptotic cells were observed,1. being ingested by neighboring cells; 2. apoptotic cells were dropping into glomerular capillary lumens or Bowman capsules.Conclusion Apoptotic cells were found in all the time during the development of mouse renal corpuscles.It might play an important role in the development of renal corpuscles.