Browsing by Author "Jimenez, Macarena"

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    Characterization of Oral Health Status in Chilean Patients with X-Linked Hypophosphatemia
    (2021) Marin, Alejandro; Morales, Pilar; Jimenez, Macarena; Borja, Eugenia; Ivanovic-Zuvic, Danisa; Collins, Michael T.; Florenzano, Pablo
    X-Linked Hypophosphatemia (XLH) is the most common cause of inherited hypophosphatemic rickets. Dental involvement, including spontaneous abscesses and/or fistulae, is an important part of the disease and has not been completely defined, especially in cohorts from developing countries. To describe oral health status in a cohort of Chilean patients with XLH and explore its correlation with biochemical presentation and treatment, we conducted a cross-sectional observational study of patients with PHEX mutation-confirmed XLH. All patients had an oral clinical exam, radiographic evaluation; clinical and biochemical data were obtained to determine their association with oral features. Twenty-six patients were included, 77% adults and 23% children. Most adults (89%) had past or current dental pulp pathology (abscesses and/or fistulae). Pulpal chamber enlargement and radiolucent apical lesions were common radiological features (94 and 74%, respectively). In children, abscess and/or fistulae were also common (33%). Caries index, which was determined by dmft/DMFT, was higher than the Chilean national average. Early and long-term therapy with phosphate and activated vitamin D was associated with lower carious index and attachment loss. XLH patients frequently present with high pulpal involvement and carious index. Conventional therapy was associated with lower carious index and attachment loss. These data highlight the importance of early and periodical dental care in order to prevent dental damage and assure a good quality of oral health for XLH patients.
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    Nephropathic Cystinosis: A Distinct Form of CKD-Mineral and Bone Disorder that Provides Novel Insights into the Regulation of FGF23
    (2020) Florenzano, Pablo; Jimenez, Macarena; Ferreira, Carlos R.; Nesterova, Galina; Roberts, Mary Scott; Tella, Sri Harsha; Fernandez de Castro, Luis; Gafni, Rachel I.; Wolf, Myles; Juppner, Harald; Gales, Barbara; Wesseling-Perry, Katherine; Markovich, Daniela; Gahl, William A.; Salusky, Isidro B.; Collins, Michael T.
    Background The rare lysosomal storage disease nephropathic cystinosis presents with renal Fanconi syndrome that evolves in time to CKD. Although biochemical abnormalities in common causes of CKD-mineral and bone disorder have been defined, it is unknown if persistent phosphate wasting in nephropathic cystinosis is associated with a biochemical mineral pattern distinct from that typically observed in CKD-mineral and bone disorder.
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    Steatotic livers. Can we use them in OLTX? Outcome data from a prospective baseline liver biopsy study
    (MEXICAN ASSOC HEPATOLOGY, 2012) Gabriel, Mauricio; Moisan, Fabrizio; Vidal, Marcela; Duarte, Ignacio; Jimenez, Macarena; Izquierdo, Guillermo; Dominguez, Pilar; Mendez, Javier; Soza, Alejandro; Benitez, Carlos; Perez, Rosa; Arrese, Marco; Guerra, Juan; Jarufe, Nicolas; Martinez, Jorge
    Introduction. Steatotic livers have been associated with greater risk of allograft dysfunction in liver transplantation. Our aim was to determinate the prevalence of steatosis in grafts from deceased donors in Chile and to assess the utility of a protocol-bench biopsy as an outcome predictor of steatotic grafts in our transplant program. Material and methods. We prospectively performed protocol-bench graft biopsies from March 2004 to January 2009. Biopsies were analyzed and classified by two independent pathologists. Steatosis severity was graded as normal from absent to < 6%; grade 1: 6-33%; grade 2: > 33-66% and grade 3: > 66%. Results. We analyzed 58 liver grafts from deceased donors. Twenty-nine grafts (50%) were steatotic; 9 of them (16%) with grade 3. Donor age (p < 0.001) and BMI over 25 kg/m(2) (p = 0.012) were significantly associated with the presence of steatosis. There were two primary non-functions (PNF); both in a grade 3 steatotic graft. The 3-year overall survival was lower among recipients with macrovesicular steatotic graft (57%) than recipients with microvesicular (85%) or non-steatotic grafts (95%) (p = 0.026). Conclusion. Macrovesicular steatosis was associated with a poor outcome in this series. A protocol bench-biopsy would be useful to identify these grafts.