Brain melanosis is a common manifestation in children with large cutaneous melanotic nevi, most commonly found in the anterior temporal lobes (amygdala), brainstem, cerebellum and cerebral cortex. Hindbrain melanosis is associated with hypoplasia of the affected structures. Early imaging is optimal to provide the greatest sensitivity for diagnosis and to guide proper management.
Background: Secondary hyperparathyroidism (SHPT) is associated with high incidences of cardiovascular disease, bone fracture, and mortality. This study was conducted to demonstrate the effectiveness of cinacalcet treatment on chronic kidney disease-mineral bone disorder (CKD-MBD) markers in chronic hemodialysis patients with severe SHPT. Methods: In phase 1, 30 adult HD patients were randomized to cinacalcet or control groups for 12 weeks to explore the achievement of >30% reduction of iPTH. In phase 2, 45 patients were participated to further explore the effect of cinacalcet on CKD-MBD parameters for 24-week follow up and 12 additional weeks after cinacalcet discontinuation. Results: In phase 1, the baseline serum iPTH levels were not different [1374 (955, 1639) pg/mL in the control group vs. 1191 (1005, 1884) pg/mL in the cinacalcet group], the percentage of patients achieving iPTH target were significantly higher in the treatment group [80% vs. 13%, p = .001]. In phase 2, the significant reductions of iPTH, FGF-23, tartrate-resistant acid phosphatase 5b, and slightly decreased size of parathyroid gland and stabilized vascular calcification were observed at 24-week follow up and markedly rebounded after discontinuation of cinacalcet. Conclusions: The effectiveness of cinacalcet were still obviously demonstrated even in chronic HD patients with severe SHPT. In addition, the improvements of bone markers and FGF-23, and stabilization of vascular calcification were observed. Therefore, cinacalcet can provide salutary effects on CKD-MBD in severe SHPT and might be an initially effective PTH-lowering therapy prior to surgical parathyroidectomy as well as an alternative treatment in the patients unsuitable for surgery. Clinical trial registration: ClinicalTrials.gov: NCT02056730. Date of registration: February 4, 2014.
Background Hypertrophic pachymeningitis (HP) is a rare chronic inflammatory disorder characterized by marked fibrous thickening of the cerebral and/or spinal dura mater. This condition is caused by infection, inflammation, autoimmune disorder, neoplasms, or idiopathic. Magnetic resonance imaging (MRI) may play an important role in differentiating idiopathic HP from secondary HP, may avoid unnecessarily invasive dural biopsy, and prompt specific treatment. Objective To determine the specific MRI findings for differentiation between idiopathic HP and secondary HP. Method A total of 34 patients underwent MRI of the brain and cervical spine from January 2003 to December 2015. In all, 23 patients were diagnosed idiopathic HP and 11 patients were secondary HP. Demographic data and imaging findings reveal the following: configuration, thickness, signal intensity on T1-weighted image (T1WI), T2-weighted image (T2WI), and enhancement pattern of the lesions. The data were analyzed by T-test and Fisher’s exact test. Result Secondary HP were significantly located at anterior and middle cranial fossa (P = 0.033). There is no significant difference of lesions in configurations, T1 and T2 signal intensity and patterns of enhancement. There was significant and exclusive difference in T2 hypointense/dark intensity and homogeneous enhancement in idiopathic HP (75%, P = 0.044). Conclusions MRI may play a complimentarily important role in distinguishing idiopathic HP from secondary HP. Idiopathic HP is probably preferred diagnosis in the lesions with T2-rim pattern and T2 hypointense/dark intensity with homogeneous enhancement.
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