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Métodos Terapéuticos y Terapias MTCI
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1.
J Clin Lipidol ; 16(6): 797-812, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36243606

RESUMEN

The Abetalipoproteinemia and Related Disorders Foundation was established in 2019 to provide guidance and support for the life-long management of inherited hypocholesterolemia disorders. Our mission is "to improve the lives of individuals and families affected by abetalipoproteinemia and related disorders". This review explains the molecular mechanisms behind the monogenic hypobetalipoproteinemia disorders and details their specific pathophysiology, clinical presentation and management throughout the lifespan. In this review, we focus on abetalipoproteinemia, homozygous hypobetalipoproteinemia and chylomicron retention disease; rare genetic conditions that manifest early in life and cause severe complications without appropriate treatment. Absent to low plasma lipid levels, in particular cholesterol and triglyceride, along with malabsorption of fat and fat-soluble vitamins are characteristic features of these diseases. We summarize the genetic basis of these disorders, provide guidance in their diagnosis and suggest treatment regimens including high dose fat-soluble vitamins as therapeutics. A section on preconception counseling and other special considerations pertaining to pregnancy is included. This information may be useful for patients, caregivers, physicians and insurance agencies involved in the management and support of affected individuals.


Asunto(s)
Abetalipoproteinemia , Hipobetalipoproteinemias , Trastornos del Metabolismo de los Lípidos , Humanos , Abetalipoproteinemia/diagnóstico , Abetalipoproteinemia/genética , Abetalipoproteinemia/terapia , Hipobetalipoproteinemias/diagnóstico , Hipobetalipoproteinemias/genética , Hipobetalipoproteinemias/terapia , Homocigoto , Vitaminas
2.
Sci Rep ; 11(1): 17691, 2021 09 06.
Artículo en Inglés | MEDLINE | ID: mdl-34489483

RESUMEN

Leptin is an adipocyte-derived hormone that regulates appetite and energy expenditure via the hypothalamus. Since the majority of obese subjects are leptin resistant, leptin sensitizers, rather than leptin itself, are expected to be anti-obesity drugs. Endoplasmic reticulum (ER) stress in the hypothalamus plays a key role in the pathogenesis of leptin resistance. ATP-deficient cells are vulnerable to ER stress and ATP treatment protects cells against ER stress. Thus, we investigated the therapeutic effects of oral 1,3-butanediol (BD) administration, which increases plasma ß-hydroxybutyrate and hypothalamic ATP concentrations, in diet induced obese (DIO) mice with leptin resistance. BD treatment effectively decreased food intake and body weight in DIO mice. In contrast, BD treatment had no effect in leptin deficient ob/ob mice. Co-administration experiment demonstrated that BD treatment sensitizes leptin action in both DIO and ob/ob mice. We also demonstrated that BD treatment attenuates ER stress and leptin resistance at the hypothalamus level. This is the first report to confirm the leptin sensitizing effect of BD treatment in leptin resistant DIO mice. The present study provides collateral evidence suggesting that the effect of BD treatment is mediated by the elevation of hypothalamic ATP concentration. Ketone bodies and hypothalamic ATP are the potential target for the treatment of obesity and its complications.


Asunto(s)
Peso Corporal/efectos de los fármacos , Butileno Glicoles/farmacología , Estrés del Retículo Endoplásmico/efectos de los fármacos , Hipotálamo/efectos de los fármacos , Leptina/farmacología , Obesidad/tratamiento farmacológico , Ácido 3-Hidroxibutírico/metabolismo , Adenosina Trifosfato/metabolismo , Animales , Butileno Glicoles/uso terapéutico , Metabolismo Energético/efectos de los fármacos , Hipotálamo/metabolismo , Masculino , Ratones , Ratones Obesos , Obesidad/metabolismo
3.
J Atheroscler Thromb ; 28(10): 1009-1019, 2021 Oct 01.
Artículo en Inglés | MEDLINE | ID: mdl-33994405

RESUMEN

Abetalipoproteinemia (ABL) is a rare autosomal recessive disorder caused by biallelic pathogenic mutations in the MTTP gene. Deficiency of microsomal triglyceride transfer protein (MTTP) abrogates the assembly of apolipoprotein (apo) B-containing lipoprotein in the intestine and liver, resulting in malabsorption of fat and fat-soluble vitamins and severe hypolipidemia. Patients with ABL typically manifest steatorrhea, vomiting, and failure to thrive in infancy. The deficiency of fat-soluble vitamins progressively develops into a variety of symptoms later in life, including hematological (acanthocytosis, anemia, bleeding tendency, etc.), neuromuscular (spinocerebellar ataxia, peripheral neuropathy, myopathy, etc.), and ophthalmological symptoms (e.g., retinitis pigmentosa). If left untreated, the disease can be debilitating and even lethal by the third decade of life due to the development of severe complications, such as blindness, neuromyopathy, and respiratory failure. High dose vitamin supplementation is the mainstay for treatment and may prevent, delay, or alleviate the complications and improve the prognosis, enabling some patients to live to the eighth decade of life. However, it cannot fully prevent or restore impaired function. Novel therapeutic modalities that improve quality of life and prognosis are awaited. The aim of this review is to 1) summarize the pathogenesis, clinical signs and symptoms, diagnosis, and management of ABL, and 2) propose diagnostic criteria that define eligibility to receive financial support from the Japanese government for patients with ABL as a rare and intractable disease. In addition, our diagnostic criteria and the entry criterion of low-density lipoprotein cholesterol (LDL-C) <15 mg/dL and apoB <15 mg/dL can be useful in universal or opportunistic screening for the disease. Registry research on ABL is currently ongoing to better understand the disease burden and unmet needs of this life-threatening disease with few therapeutic options.


Asunto(s)
Abetalipoproteinemia/diagnóstico , Abetalipoproteinemia/terapia , Abetalipoproteinemia/sangre , Abetalipoproteinemia/patología , Apolipoproteínas B/sangre , LDL-Colesterol/sangre , Costo de Enfermedad , Manejo de la Enfermedad , Humanos , Pronóstico
4.
J Infect Chemother ; 25(9): 657-668, 2019 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-31196772

RESUMEN

The nationwide surveillance on antimicrobial susceptibility of bacterial respiratory pathogens from the patients in Japan was conducted by Japanese Society of Chemotherapy, the Japanese Association for Infectious Diseases, and the Japanese Society for Clinical Microbiology in 2014. The isolates were collected from clinical specimens obtained from well-diagnosed adult patients with respiratory tract infections during the period between January 2014 and April 2015 by three societies. Antimicrobial susceptibility testing was conducted at the central reference laboratory according to the method recommended by Clinical Laboratory Standards Institute. Susceptibility testing was evaluated in 1534 strains (335 Staphylococcus aureus, 264 Streptococcus pneumoniae, 29 Streptococcus pyogenes, 281 Haemophilus influenzae, 164 Moraxella catarrhalis, 207 Klebsiella pneumoniae, and 254 Pseudomonas aeruginosa). Ratio of methicillin-resistant S. aureus was 43.6%, and those of penicillin-susceptible S. pneumoniae was 100%. Among H. influenzae, 8.2% of them were found to be ß-lactamase-producing ampicillin-resistant strains, and 49.1% to be ß-lactamase-non-producing ampicillin-resistant strains. Extended spectrum ß-lactamase-producing K. pneumoniae and multi-drug resistant P. aeruginosa with metallo ß-lactamase were 9.2% and 0.4%, respectively.


Asunto(s)
Antibacterianos/uso terapéutico , Farmacorresistencia Bacteriana , Monitoreo Epidemiológico , Infecciones del Sistema Respiratorio/prevención & control , Programas de Optimización del Uso de los Antimicrobianos , Haemophilus influenzae/efectos de los fármacos , Humanos , Japón/epidemiología , Klebsiella pneumoniae/efectos de los fármacos , Staphylococcus aureus Resistente a Meticilina/efectos de los fármacos , Pruebas de Sensibilidad Microbiana , Moraxella catarrhalis/efectos de los fármacos , Pseudomonas aeruginosa/efectos de los fármacos , Infecciones del Sistema Respiratorio/microbiología , Streptococcus pneumoniae/efectos de los fármacos , Streptococcus pyogenes/efectos de los fármacos
5.
Masui ; 51(9): 992-5, 2002 Sep.
Artículo en Japonés | MEDLINE | ID: mdl-12382388

RESUMEN

We reported five patients had developed sudden bradycardia and hypotension under spinal anesthesia during transurethral resection of the prostate. The symptoms occurred not only just after the induction of spinal anesthesia, but also at the end of operation. It seems that these symptoms are caused from water intoxication, myocardial ischemia or vagal reflex. This emphasizes the importance of rigorous vigilance on patients until the end of operation.


Asunto(s)
Anestesia Raquidea/efectos adversos , Bradicardia/etiología , Hipotensión/etiología , Cuidados Intraoperatorios , Complicaciones Intraoperatorias/etiología , Resección Transuretral de la Próstata , Anciano , Bradicardia/diagnóstico , Bradicardia/terapia , Humanos , Hipotensión/diagnóstico , Hipotensión/terapia , Complicaciones Intraoperatorias/diagnóstico , Complicaciones Intraoperatorias/terapia , Masculino , Monitoreo Intraoperatorio , Isquemia Miocárdica/complicaciones , Reflejo , Nervio Vago/fisiopatología , Intoxicación por Agua/complicaciones
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