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1.
Cancer ; 128(3): 435-446, 2022 02 01.
Artículo en Inglés | MEDLINE | ID: mdl-34735022

RESUMEN

Von Hippel-Lindau disease (VHL) is a multineoplasm inherited disease manifesting with hemangioblastoma of the central nervous system and retina, adrenal pheochromocytoma, renal cell carcinoma, pancreatic neuroendocrine tumors and cysts, and neoplasms/cysts of the ear, broad ligament, and testicles. During 2018-2020, the VHL Alliance gathered several committees of experts in the various clinical manifestations of VHL to review the literature, gather the available evidence on VHL, and develop recommendations for patient management. The current report details the results of the discussion of a group of experts in the pancreatic manifestations of VHL along with their proposed recommendations for the clinical surveillance and management of patients with VHL. The recommendations subcommittee performed a comprehensive systematic review of the literature and conducted panel discussions to reach the current recommendations. The level of evidence was defined according to the Shekelle variation of the Grading of Recommendations, Assessment, Development, and Evaluation grading system. The National Comprehensive Cancer Network Categories of Evidence and Consensus defined the committee members' interpretation of the evidence and degree of consensus. The recommendations encompass the main aspects of VHL-related pancreatic manifestations and their clinical management. They are presented in a clinical orientation, including general planning of screening and surveillance for pancreatic neuroendocrine tumors, utility of biochemical biomarkers, the optimal choice for imaging modality, indirect risk stratification, indications for tissue sampling of VHL-related pancreatic neuroendocrine tumors, and interventions. These recommendations are designed to serve as the reference for all aspects of the screening, surveillance, and management of VHL-related pancreatic manifestations.


Asunto(s)
Neoplasias de las Glándulas Suprarrenales , Hemangioblastoma , Neoplasias Renales , Neoplasias Pancreáticas , Feocromocitoma , Enfermedad de von Hippel-Lindau , Neoplasias de las Glándulas Suprarrenales/complicaciones , Neoplasias de las Glándulas Suprarrenales/diagnóstico , Neoplasias de las Glándulas Suprarrenales/terapia , Femenino , Hemangioblastoma/diagnóstico , Humanos , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/etiología , Neoplasias Pancreáticas/terapia , Feocromocitoma/diagnóstico , Feocromocitoma/terapia , Proteína Supresora de Tumores del Síndrome de Von Hippel-Lindau , Enfermedad de von Hippel-Lindau/complicaciones , Enfermedad de von Hippel-Lindau/diagnóstico , Enfermedad de von Hippel-Lindau/terapia
2.
Mol Cell Endocrinol ; 520: 111091, 2021 01 15.
Artículo en Inglés | MEDLINE | ID: mdl-33248229

RESUMEN

We recently described X-linked acrogigantism (X-LAG), a condition of early childhood-onset pituitary gigantism associated with microduplications of the GPR101 receptor. The expression of GPR101 in hyperplastic pituitary regions and tumors in X-LAG patients, and GPR101's normally transient pituitary expression during fetal development, suggest a role in the regulation of growth. Nevertheless, little is still known about GPR101's physiological functions, especially during development. By using zebrafish models, we investigated the role of gpr101 during embryonic development and somatic growth. Transient ectopic gpr101 expression perturbed the embryonic body plan but did not affect growth. Loss of gpr101 led to a significant reduction in body size that was even more pronounced in the absence of maternal transcripts, as well as subfertility. These changes were accompanied by gastrulation and hypothalamic defects. In conclusion, both gpr101 loss- and gain-of-function affect, in different ways, fertility, embryonic patterning, growth and brain development.


Asunto(s)
Acromegalia/genética , Desarrollo Embrionario/genética , Enfermedades Genéticas Ligadas al Cromosoma X/genética , Gigantismo/genética , Receptores Acoplados a Proteínas G/genética , Proteínas de Pez Cebra/genética , Pez Cebra/crecimiento & desarrollo , Pez Cebra/genética , Acromegalia/complicaciones , Animales , Femenino , Fertilización/genética , Gastrulación/genética , Regulación del Desarrollo de la Expresión Génica , Gigantismo/complicaciones , Hipotálamo/patología , Mutación/genética , Óvulo/metabolismo , Receptores Acoplados a Proteínas G/metabolismo , Transducción de Señal/genética , Temperatura , Transcriptoma/genética , Regulación hacia Arriba/genética , Proteínas de Pez Cebra/metabolismo , Cigoto/metabolismo
3.
J Altern Complement Med ; 21(7): 395-400, 2015 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-26052873

RESUMEN

INTRODUCTION: The use of complementary and alternative medicine (CAM) has been on the rise in recent years in the general population, as well as among patients with chronic diseases such as diabetes mellitus. The aim of this study was to add information regarding the use of CAM in patients with type 2 diabetes mellitus (DM2) in Israel and explore possible interactions between CAM and prescription medication (PM). METHODS: This is a cross-sectional study based on questionnaires. The study included type 2 diabetic patients who were hospitalized in an internal medicine department at Assaf Harofeh Medical Center, Zerifin, Israel, between December 2013 and December 2014. Possible interactions between CAM and PM were evaluated by a clinical pharmacist and a clinical pharmacologist. RESULTS: Out of 111 diabetic patients, 23.4% used CAM. There was no significant difference between the consumers and nonconsumers in terms of age, education, income, smoking, or alcohol habits. Only 11 of the 26 CAM consumers informed their physician regarding the use. We found possible drug-herb interactions in 19 of the 26 CAM consumers. A major interaction was found between omega-3 and antiaggregants and was encountered in 7 (26.9%) of the CAM consumers. Other minor and major interactions were found with vitamin E, ginkgo-biloba, co-enzyme Q10, green tea, fenugreek seeds, pyridoxine, and dandelion. CONCLUSIONS: Since CAM consumption is on the rise, it is desirable to improve our knowledge concerning their potential effects and adverse effects, especially in conjunction with PM. Given the complexity of pharmaceutics in patients with chronic diseases, among them patients with DM, the use of supplementary medicine cannot be ignored.


Asunto(s)
Terapias Complementarias/métodos , Diabetes Mellitus Tipo 2/terapia , Anciano , Anciano de 80 o más Años , Terapias Complementarias/efectos adversos , Estudios Transversales , Diabetes Mellitus Tipo 2/epidemiología , Femenino , Interacciones de Hierba-Droga , Humanos , Hipoglucemiantes/uso terapéutico , Israel/epidemiología , Masculino , Persona de Mediana Edad , Encuestas y Cuestionarios
4.
Diving Hyperb Med ; 43(4): 218-21, 2013 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-24510327

RESUMEN

INTRODUCTION: A decrease in blood glucose levels (BGL) during hyperbaric oxygen treatment (HBOT) is a well-recognised phenomenon, but studies of this are limited and inconclusive. This study evaluated the effect of HBOT on BGL in patients with diabetes mellitus (DM), traumatic brain injury (TBI) or stroke and healthy volunteers in a prospective, open, controlled trial. METHODS: Thirty-nine participants were enrolled and evaluated twice: once during HBOT (90 minutes at 203 kPa), and once during a control session on normobaric air. Sessions were held up to two weeks apart and participants were instructed to eat the same diet. BGL was measured before, during and at the completion of each session. RESULTS: For the whole study group, there was a small but statistically significant decrease in BGL in both the HBOT (7.27 ± 3.66 mmol⁻¹ before to 6.71 ± 3.88 mmol ⁻¹ after, P = 0.037) and control (air) sessions (7.43 ± 3.49 mmol L⁻¹ before to 6.71 ± 3.77 mmol L⁻¹ after, P = 0.004). This fall did not differ between the two conditions (P = 0.59). Examining the three groups separately, BGL fell in all three subgroups, but this fall was only statistically significant for the air session in the diabetic group. There were no statistically significant differences in the BGL reduction when HBOT was compared to normobaric air in any of the three subgroups. CONCLUSIONS: BGL may decrease during HBOT and accordingly it should be monitored before entering the chamber. However, this decrease in BGL should probably not be attributed to the hyperbaric environment per se.


Asunto(s)
Glucemia/análisis , Lesiones Encefálicas/sangre , Diabetes Mellitus/sangre , Oxigenoterapia Hiperbárica , Accidente Cerebrovascular/sangre , Anciano , Glucemia/metabolismo , Lesiones Encefálicas/terapia , Estudios de Casos y Controles , Estudios Cruzados , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Accidente Cerebrovascular/terapia
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