[The impact of COVID-19 pandemic on doctor-patient relationship]. / La relación médico-paciente en tiempos de COVID-19. ¿Un cambio de paradigma?

Doctor-patient relationship (DPR) is the cornerstone of clinical medicine, mobilizing powerful human resources. This article analyzes the impact of COVID-19 pandemia on DPR. Due to fear of contagion, the use of telephone or digital consultation was preferred, in which only the results of laboratory and imaging tests can be reviewed, and the patient receives a diagnostic conclusion. Patients are afraid of face-to-face consultations, and healthcare centers developed measures to reduce patient influx. Thus, a new relationship ensued, generating suspicion, mistrust and fear. The empathy and affection that the doctor must project and deliver to the patient was reduced. Depending on the duration of the pandemia, doctors and patients will eventually get used to this type of relationship and a paradigm shift will occur, in which Hippocratic medicine gives way to digital medicine.

La relación médico-paciente en tiempos de COVID-19. ¿Un cambio de paradigma? / The impact of COVID-19 pandemic on doctor-patient relationship

Doctor-patient relationship (DPR) is the cornerstone of clinical medicine, mobilizing powerful human resources. This article analyzes the impact of COVID-19 pandemia on DPR. Due to fear of contagion, the use of telephone or digital consultation was preferred, in which only the results of laboratory and imaging tests can be reviewed, and the patient receives a diagnostic conclusion. Patients are afraid of face-to-face consultations, and healthcare centers developed measures to reduce patient influx. Thus, a new relationship ensued, generating suspicion, mistrust and fear. The empathy and affection that the doctor must project and deliver to the patient was reduced. Depending on the duration of the pandemia, doctors and patients will eventually get used to this type of relationship and a paradigm shift will occur, in which Hippocratic medicine gives way to digital medicine.

Genotype-Phenotype Variations of Renal Complications in Fabry Disease Q279X Mutation

Abstract In more than 800 GLA gene mutations causing Fabry Disease (FD), renal involvement vary according to the α-GAL A mutation. The aim is to describe the genotype/phenotype variations of renal complications in two siblings with confirmed FD with the mutation p.Q279X in exon 6. We present a retrospective study of two venezuelan male siblings, ages 34 (patient 1) and 33 (patient 2), evaluated by general lab tests, renal ultrasound, renal scintigram , and renal biopsy. Fabry disease diagnose was made by α-galactosidase A activity determined in dried blood spot. Genomic DNA was sequenced by Sanger method. Patient 1 developed CKD grade 5 and high blood pressure, treated by hemodialysis during 8 years. Patient 2 showed GFR >60 ml/min, and proteinuria less than 600 mg/24H. Renal biopsy showed segmental sclerotic lesions and hypertrophic podocytes with vacuolated cytoplasm. Both patients received ERT every two weeks since 2003. Patient 1 died because dialysis complications (hyperparathyroidism, cardiomyopathy). The genotype/phenotype variation of the c.835C>T mutation (p.Gln279Ter. Q279X) in exon 6 of the GLA gene can express an important renal variation with a wide range of clinical manifestations that cannot be predicted, therefore, an early nephrological evaluation and periodic follow-up of these patients are necessary.

Long-term clinical outcomes in type 1 Gaucher disease following 10 years of imiglucerase treatment.

OBJECTIVE: We studied the effect of long-term alglucerase/imiglucerase (Ceredase®/Cerezyme®, Genzyme, a Sanofi company, Cambridge, MA, USA) treatment on hematological, visceral, and bone manifestations of Gaucher disease type 1 (GD1). METHODS: The International Collaborative Gaucher Group (ICGG) Gaucher Registry identified GD1 patients treated with alglucerase/imiglucerase who had dose and clinical data at first infusion and after 10 years of follow-up. Data for hemoglobin, platelet count, organ volumes, bone pain, and bone crisis were analyzed. Tests of the null hypothesis (no change from first infusion to 10 years) were performed using t tests for within-patient absolute change in continuous measurements and McNemar/chi-square tests for change in distributions using categorical values. An alpha level of 0.05 designated statistical significance. RESULTS: As of October 2011, 557 nonsplenectomized and 200 splenectomized patients met the inclusion criteria. The majority of GD1 patients had at least one N370S allele. Compared with nonsplenectomized patients at first infusion, splenectomized patients had lower percentages of anemia (26.0 % vs. 42.8 %) and thrombocytopenia (14.2 % vs. 76.3 %), similar percentages of moderate or severe hepatomegaly (81.2 % vs. 80.0 %), and higher percentages of bone pain (88.9 % vs. 52.4 %) and bone crises (38.3 % vs. 16.0 %). After 10 years, both groups showed significant (p < 0.05) improvements in mean hemoglobin levels, platelet count, liver, and spleen (nonsplenectomized) volumes, and bone crises. Initial dosing in both groups ranged from <15 U/kg to ≤90 U/kg every 2 weeks. After 10 years, the majority was receiving 15 to ≤45 U/kg every 2 weeks. CONCLUSION: Ten years of imiglucerase treatment results in sustainable improvements in all GD1 parameters.

[Gaucher disease in Latin America. A report from the Gaucher Disease International Registry and the Latin American Group for Gaucher Disease]. / Enfermedad de Gaucher en Latinoamérica. Un informe del Registro Internacional y del Grupo Latinoamericano para la enfermedad de Gaucher.

Gaucher disease -due to its low frequency- is considered an orphan disease. In 1991 the International Gaucher Registry was created and in 1992 the first patients from Latin America were enrolled. In 2008 the Latin American Group for Gaucher Disease was initiated. Its main objectives are to promote regional consensus, to stimulate the enrollment of patients into the International Gaucher Registry and the enhancement of knowledge on this disease, and to achieve better care and quality of life of patients in our Region. Until April 2010, 5828 patients have been enrolled all around the world, 911 (15.6%) from Latin America. This is the first comprehensive report of the disease in the Region. In our population there is a predominance of females, the most common clinical form is the type I (95%) and the age at diagnosis is before 20 years in 68% of patients. The most frequent clinical manifestations at diagnosis are splenomegaly (96%) and anemia (49%). Eighty percent of patients had radiographic findings of bone involvement. In our Region, the vast majority of patients (89%) had received enzyme replacement therapy with imiglucerase; with a long follow-up (up to 10 years) they have achieved the therapeutic goals, showing the great effectiveness of therapy. While the percentage of patients with therapy is high, discontinuations are common. The main deficiencies in our Region are: the lack of visceral volumetric evaluations and densitometries as well as molecular analysis for some patients. The main problem is the under-diagnosis of patients.

Enfermedad de Gaucher en Latinoamérica: Un informe del Registro Internacional y del Grupo Latinoamericano para la Enfermedad de Gaucher / Gaucher disease in Latin America: A report from the Gaucher Disease International Registry and the Latin Americam Group for Gaucher Disease

La enfermedad de Gaucher, por su escasa frecuencia, está incluida dentro de las llamadas enfermedades huérfanas. En 1991 se creó el Registro Internacional de Gaucher y en 1992 se incorporaron los primeros pacientes de Latinoamérica. En el año 2008 se creó el Grupo Latinoamericano para la Enfermedad de Gaucher (GLAEG) cuyos principales objetivos son fomentar la realización de consensos regionales, difundir el ingreso de pacientes al registro internacional y aumentar el conocimiento sobre la enfermedad para lograr mejorar la atención y la calidad de vida de los pacientes. Hasta abril del 2010 ingresaron 5828 pacientes de todo el mundo, 911 (15.6%) son de Latinoamérica. Este es el primer informe global de la enfermedad en la Región: hay un predominio del sexo femenino, la forma clínica más frecuente es el tipo I (95%); al diagnóstico la mayoría son <20 años (68%). Las manifestaciones clínicas más frecuentes al diagnóstico son esplenomegalia (96%) y anemia (49%), el 80% presentó hallazgos radiológicos de compromiso óseo. En nuestra Región, la gran mayoría de los pacientes (89%) ha recibido alguna vez terapia de reemplazo enzimática con imiglucerasa logrando, con un seguimiento prolongado (hasta10 años), las metas terapéuticas que muestran la gran eficacia de la terapia. Si bien el porcentaje de pacientes con terapia es alto, las suspensiones de tratamiento son frecuentes. Las principales deficiencias en nuestra Región son: la carencia de evaluaciones viscerales volumétricas, de densitometría y de estudios moleculares en algunos pacientes. El principal problema es el subdiagnóstico.

Gaucher disease -due to its low frequency- is considered an orphan disease. In 1991 the International Gaucher Registry was created and in 1992 the first patients from Latin America were enrolled. In 2008 the Latin American Group for Gaucher Disease was initiated. Its main objectives are to promote regional consensus, to stimulate the enrolment of patients into the International Gaucher Registry and the enhancement of knowledge on this disease, and to achieve better care and quality of life of patients in our Region. Until April 2010, 5828 patients have been enrolled all around the world, 911 (15.6%) from Latin America. This is the first comprehensive report of the disease in the Region. In our population there is a predominance of females, the most common clinical form is the type I (95%) and the age at diagnosis is before 20 years in 68% of patients. The most frequent clinical manifestations at diagnosis are splenomegaly (96%) and anemia (49%). Eighty percent of patients had radiographic findings of bone involvement. In our Region, the vast majority of patients (89%) had received enzyme replacement therapy with imiglucerase; with a long follow-up (up to 10 years) they have achieved the therapeutic goals, showing the great effectiveness of therapy. While the percentage of patients with therapy is high, discontinuations are common. The main deficiencies in our Region are: the lack of visceral volumetric evaluations and densitometries as well as molecular analysis for some patients. The main problem is the under-diagnosis of patients.

Enfermedad de Gaucher en Latinoamérica: Un informe del Registro Internacional y del Grupo Latinoamericano para la Enfermedad de Gaucher / Gaucher disease in Latin America: A report from the Gaucher Disease International Registry and the Latin Americam Group for Gaucher Disease

La enfermedad de Gaucher, por su escasa frecuencia, está incluida dentro de las llamadas enfermedades huérfanas. En 1991 se creó el Registro Internacional de Gaucher y en 1992 se incorporaron los primeros pacientes de Latinoamérica. En el año 2008 se creó el Grupo Latinoamericano para la Enfermedad de Gaucher (GLAEG) cuyos principales objetivos son fomentar la realización de consensos regionales, difundir el ingreso de pacientes al registro internacional y aumentar el conocimiento sobre la enfermedad para lograr mejorar la atención y la calidad de vida de los pacientes. Hasta abril del 2010 ingresaron 5828 pacientes de todo el mundo, 911 (15.6%) son de Latinoamérica. Este es el primer informe global de la enfermedad en la Región: hay un predominio del sexo femenino, la forma clínica más frecuente es el tipo I (95%); al diagnóstico la mayoría son <20 años (68%). Las manifestaciones clínicas más frecuentes al diagnóstico son esplenomegalia (96%) y anemia (49%), el 80% presentó hallazgos radiológicos de compromiso óseo. En nuestra Región, la gran mayoría de los pacientes (89%) ha recibido alguna vez terapia de reemplazo enzimática con imiglucerasa logrando, con un seguimiento prolongado (hasta10 años), las metas terapéuticas que muestran la gran eficacia de la terapia. Si bien el porcentaje de pacientes con terapia es alto, las suspensiones de tratamiento son frecuentes. Las principales deficiencias en nuestra Región son: la carencia de evaluaciones viscerales volumétricas, de densitometría y de estudios moleculares en algunos pacientes. El principal problema es el subdiagnóstico.(AU)

Gaucher disease -due to its low frequency- is considered an orphan disease. In 1991 the International Gaucher Registry was created and in 1992 the first patients from Latin America were enrolled. In 2008 the Latin American Group for Gaucher Disease was initiated. Its main objectives are to promote regional consensus, to stimulate the enrolment of patients into the International Gaucher Registry and the enhancement of knowledge on this disease, and to achieve better care and quality of life of patients in our Region. Until April 2010, 5828 patients have been enrolled all around the world, 911 (15.6%) from Latin America. This is the first comprehensive report of the disease in the Region. In our population there is a predominance of females, the most common clinical form is the type I (95%) and the age at diagnosis is before 20 years in 68% of patients. The most frequent clinical manifestations at diagnosis are splenomegaly (96%) and anemia (49%). Eighty percent of patients had radiographic findings of bone involvement. In our Region, the vast majority of patients (89%) had received enzyme replacement therapy with imiglucerase; with a long follow-up (up to 10 years) they have achieved the therapeutic goals, showing the great effectiveness of therapy. While the percentage of patients with therapy is high, discontinuations are common. The main deficiencies in our Region are: the lack of visceral volumetric evaluations and densitometries as well as molecular analysis for some patients. The main problem is the under-diagnosis of patients.(AU)

Utility of rare disease registries in latin america.

There are many registries in Latin America as dialysis and kidney transplantation, breast cancer, primary immunodeficiency, acute coronary syndromes, but the focus here are the registries of lysosomal storage diseases (LSD) because is our experience. Registry of Gaucher disease, Fabry disease, Pompe disease, and mucopolysaccharidosis type I are comprehensive observational voluntary programs that aim to collect clinical and laboratory data of initiation, progression, and evolution of those diseases, with and without treatment, using questionnaires of quality of life and/or skills and functions. There are two more programs of LSD: Hunter outcome survey and Fabry outcome survey. The registries are a kind of phase IV clinical trials, postmarketing studies delineate additional information including the drug's risks, benefits, and optimal use, and in addition we have data from natural history. The demographics of the Gaucher, Fabry, MPS I, and Pompe Registries show that a total of patients, being 16%, 8%, 15%, and 7%, respectively, of this population, and 19%, 19%, 18%, and 13%, respectively, of all physicians participating in the program are from Latin America. In the Gaucher Registry, we can observe that the percentage of children in Latin America (29%) is bigger than the rest of the world (20%), what can mean more severe disease in this population. These diseases are rare, and a database of clinical data from a larger number of patients gives us the opportunity to know about the natural history of these diseases, their phenotypic variability, and the response to specific enzyme replacement therapy in our population.

End-stage renal disease in patients with Fabry disease: natural history data from the Fabry Registry.

BACKGROUND: Fabry disease, an X-linked lysosomal storage disorder caused by deficiency of alpha-galactosidase activity, is associated with progressive loss of kidney function. This study was undertaken to characterize Fabry disease among patients who reached end-stage renal disease. METHODS: Data from 2,712 patients in the Fabry Registry were analysed to identify clinical characteristics of patients who received renal replacement therapy (RRT) during the natural history period (i.e. prior to any enzyme replacement therapy). RESULTS: A total of 213 patients [186 of 1,359 males (14%) and 27 of 1,353 females (2%)] received RRT at a median age of 38 years in both males and females. Males who received RRT were diagnosed at a median age of 35 years, compared to 23 years for non-RRT males. Sixty-one males and 10 females were not diagnosed with Fabry disease until after they had received RRT. Compared to other Fabry Registry patients, a higher percentage of RRT patients also experienced either a serious cardiovascular event or a stroke. Ninety-two of 186 males who had RRT (50%) experienced a cardiac event or stroke, compared to 230 of 1,173 non-RRT males (20%). Ten of 27 RRT females (37%) had experienced a cardiac event or stroke, compared to 226 of 1,326 non-RRT females (17%). Patients who had RRT experienced cardiovascular events and strokes at earlier ages than did patients who had not received RRT, and most received RRT before having a cardiac event or stroke. CONCLUSIONS: While all Fabry patients are at risk of cardiovascular events and strokes, patients with Fabry nephropathy who develop kidney failure appear to have concurrent involvement of other major organ systems. It is important that Fabry patients are diagnosed early and that their renal function is monitored carefully.

Características ultraestructurales del músculo esquelético en pacientes con proteinuria glomerular / Ultrastructure of skeletal muscle in patients with glomerular proteinuria

La proteinuria produce un desbalance importante del metabolismo proteico y lipídico, que favorece la desproteinización del organismo, con grandes repercusiones sistémicas que llegan a afectar hasta el músculo esquelético. En los pacientes con falla renal, usualmente disminuye su masa muscular y se observa debilidad. Los pacientes urémicos terminales, manifiestan atrofia de las fibras musculares tipo II, además de daño miopático primario, causado probablemente por un déficit de la microcirculación. En un afán de dilucidar las posibles alteraciones ultraestructurales precoces presentes en el músculo esquelético de pacientes con glomerulopatías primarias que cursan con proteinuria, se estudiaron 4 pacientes (2 hombres y 2 mujeres) con edades entre 42 y 66 años, pertenecientes al servicio de nefrología del hospital militar ®Dr. Calos Arvelo¼ de Caracas-Venezuela. En todos ellos se realizaron mediciones de enzimas musculares (CK, LDH, AST y ALT), electromiografía de los miembros inferiores y biopsia del músculo cuadriceps femoral. En ninguno de los pacientes se observaron modificaciones en los niveles séricos de las enzimas y a excepción de uno de ellos (caso 4), los registros eletromiográficos fueron normales. A nivel ultraestructural se observó: hinchamiento de los componentes del sistema sarcotubular con presencia de tríadas prominentes, signos de atrofia muscular, núcleos hipercromáticos, glucógeno abundante y glucogenosomas. Algunos capilares intramusculares con la luz parcial o totalmente ocluida, citoplasma endotelial electrón denso, presencia de prolongaciones del citoplasma endotelial hacia la luz del capilar, vesículas pinocíticas y cavéolas. Dichos cambios nos permiten concluir el compromiso estructural del músculo esquelético en pacientes con proteinuria, el cual se hace más evidente a medida que avanza hacia la falla renal y que dicho compromiso tiene una base microvascular que condiciona cambios del metabolismo muscular.

The proteinuria produces an important unbalance of the protein and lipid metabolism that it favors thedesproteinitation of the organism, with big systemic repercussions that end up affecting the skeletal muscle. In the patients with renal failure it usually diminishes their muscular mass and weakness is observed. The terminal uremic patient, manifests atrophy of the fibers muscular type II, besides miopatic primary damage, caused probably for a deficit of the microcirculación. In a desire of elucidating the early ultraestructural alterations present in the skeletal muscle of patient with primary glomerulopaties that curse with proteinuria, four patients were studied (2 men and 2 women) with ages between 42 and 66 years, belonging to the service of nephrology of the military hospital ®Dr. Calos Arvelo¼ of Caracas-Venezuela. In all they were carried out mensurations of muscular enzymes (CK, LDH, AST and ALT), electromiografie of the inferior members and biopsy of the muscle femoral cuadriceps. In none of the patients modifications were observed in the seric levels of the enzymes and to exception of one of them (case 4); the eletromiografic registrations are normal. At ultraestructural level it was observed: swelling of the components of the system sarcotubular with presence of prominent triads, signs of muscular atrophy, hyperchromatic nuclei, abundant glucogen and glucogenosome. Some capillary ones intramuscular with the partial or completely occluded light, electron dense cytoplasm, presence of continuations of the endothelial cytoplasm toward the light of the capillary one, pinocitic vesicles and caveole. This changes allow us to conclude the structural commitment of the skeletal muscle in patient with proteinuria, which becomes more evident as it advances toward the renal failure and that said commitment has a microvascular origin that conditions changes of the muscular metabolism.

Aspectos ultraestructurales del riñón remanente en ratas uninefrectomizadas sometidas a dietas hipoproteicas suplementadas con aminoácidos esenciales y no esenciales / Ultrastructural aspects of remaining kidney of unilaterally nephrectomized rats receiving hypoproteic diets enriched with amino acids

Es bien conocido el efecto benéfico de la restricción en la ingesta proteica de la dieta para la preservación de la función renal, cuando la masa renal se encuentra reducida, en un intento de aumentar la sobrevida de los pacientes. Sin embargo, la literatura al respecto es extremadamente contradictoria. En este trabajo estudiamos el efecto de la alimentación con dietas hipoprotéicas ricas en aminoácidos esenciales (lisina y arginina) y no esenciales (asparagina, prolina y ácido glutámico) sobre la ultraestructura del riñón remanente de ratas uninefrectomizadas. Después de 9 semanas de realizada la intervención quirúrgica, bajo Nesdonal, durante las cuales las ratas uninefrectomizadas fueron alimentadas con dietas especialmente diseñadas para el experimento, se retiraron los riñones remanentes y se procesaron para ser observados al Microscopio Electrónico de transmisión, siguiendo el procedimiento convencional. Como conclusión del estudio microscópico, para todas las dietas estudiadas se observó una vacuolización prominente de las células tubulares proximales así como hinchamiento tanto de las mitocondrias como pérdida de material en áreas tubulares. Se apreciaron túbulos con lúmina dilatada, mitocondrias dispuestas irregularmente y con un engrosamiento de la membrana basal, característica inespecífica pero constante de las enfermedades renales en diabéticos y glomérulos alterados. Por consiguiente, la adición de ciertos aminoácidos a la dieta no parece proteger eficientemente contra el deterioro histológico en riñones fisiológicamente comprometidos.

Dietary restriction of proteins has been used in patients with kidney failure in an attempt to preserve renal functionand prolong their lives. However, literature shows contradictory results. We studies the effects of diets enriched with essential (lysine, arginine) or non-essential (asparagine, proline or glutamic acid) amino acids on the ultrastructure of remaining kidney of uninephrectomized rats. The kidney was removed under barbiturate anesthesia and after that the animals received specially designed diets during nine weeks. Then, the remaining kidney was removed and conventionally processed to be studies by transmission electronic microscope. For all the diets used, we observed a prominent vacuolisation of proximal tubular cells, as well as mithocondrial edema and tissue loss at the tubular level. The preparations showed tubules with dilated lumen, irregularly placed mithocondria and basal membrane thickening, a non-specific but constant finding in diabetic kidneys and in some glomerulopathies. In conclusion, amino acid-enriched diets are not effective to protect functionally compromised kidneys against progressive anatomical changes.

[Effect of hypoproteic diets enriched with essential and non-essential amino acids on the uninephrectomized rat ]. / Efecto de dietas hipoproteicas ricas en aminoacidos esenciales y no esenciales sobre el modelo experimental de rata uninefrectomizada.

Hyperalimentation solutions, with low protein content but rich in amino acids, have been more frequently used as a dietary treatment for renal terminal patients, with the purpose to increase their survival. However, the literature in this respect is contradictory. Some authors justify the use of amino acids due to the fact that they seem to regenerate damaged tubular cells (glycine, for example). Other authors, on the contrary, do not agree with this position, since some amino acids, like L-Serine and Lysine, are nephrotoxic. In 1977, it was demostrated that Lysine and Arginine inhibited protein tubular reabsorption, inducing proteinuria, while Glycine, Alanine, Asparagine and Glutamic Acid did not. In order to clarify this issue, we carried out a controlled animal study using uninephrectomized rats fed during nine weeks, with different hypoproteinic diets (4% protein content), enriched individually with five different amino acids. The hypoproteinic diets were enriched with Lysine and Arginine (essential amino acids) and Proline, Glutamic Acid and Asparagine (non essential amino acids). Assays for serum biochemical markers and renal function were carried-out pre-nephrectomy, two weeks after nephrectomy (post-nephrectomy control) and nine weeks post-diet for all the animals, no matter the diet to which they were subjected, the serum biochemistry results showed that all the hypoproteinic diets, enriched with amino acids, affected the renal function. The nephrotoxicity of the tested amino acids, followed this decreasing order: Glutamic Acid > Proline > Lysine > Asparagine > Arginine. hypoproteinic diets enriched with Lysine, Asparagine and Arginine, produced glomerular hyperfiltration, without proteinuria. In summary, our results point towards the idea that, contrarily to what has been described in the literature by some authors: enrichment of hypoproteinic diets with certain amino acids does not seem to protect against progression of renal disease in physiologically compromised kidneys.

Efecto de dietas hipoproteicas ricas en aminoacidos esenciales y no esenciales sobre el modelo experimental de rata uninefrectomizada / Effect of hypoproteic diets enriched with essential and non-essential amino acids on the uninephrectomized rat

RESUMEN: La hiperalimentación con soluciones ricas en aminoácidos ha sido usada para el tratamiento de pacientes renales terminales, con el fin de aumentar la sobrevida de los mismos. Sin embargo, la literatura al respecto es contradictoria. Algunos autores hablan de aminoácidos regeneradores de las células tubulares (por ejemplo, la glicina). Otros autores, por el contrario, afirman que ciertos aminoácidos como la L-serina y la lisina son nefrotóxicos. En 1977, se demostró que los aminoácidos Lisina y Arginina inhibían la reabsorción tubular de proteínas (induciendo proteinuria), mientras que otros como la Glicina, la Alanina, el Ácido Aspártico y el Ácido Glutámico no lo hacían. En un intento de clarificar los datos de la literatura, realizamos un estudio animal controlado utilizando ratas uninefrectomizadas, las cuales fueron alimentadas durante nueve semanas con una serie de dietas diferentes, todas hipoproteicas (4% de proteínas) y, enriquecidas individualmente con cinco diferentes aminoácidos. Los aminoácidos empleados para enriquecer las dietas hipoproteicas fueron, en cada caso individual, Lisina, Arginina (ambos son aminoácidos esenciales), Prolina, Asparagina y Ácido Glutámico (los tres son aminoácidos no-esenciales). Se realizaron mediciones de valores bioquímicos plasmáticos y de funcionalismo renal (depuración de creatinina), tanto en condiciones controles (pre-nefrectomía), dos semanas después de la uninefrectomía (control post-nefrectomía) y, nueve semanas después de haber comenzado la dieta. En todos los animales, sin importar la dieta a la cual estuvieron sometidos, los resultados bioquímicos mostraron que la dieta hipoproteica enriquecida con aminoácidos, afecta la función renal. Sin embargo, nuestros resultados sugieren que el daño renal es diferente con cada aminoácido en particular y que la nefrotoxicidad de los aminoácidos presenta la siguiente secuencia: Ácido Glutámico > Prolina > Lisina > Asparagina> Arginina. También observamos que las dietas hipoproteicas enriquecidas con Lisina, Asparagina y Arginina, producen hiperfiltración glomerular, que no cursa con proteinuria. En conclusión, los resultados bioquímicos encontrados indican que, contrariamente a lo descrito por algunos autores, el enriquecer una dieta hipoproteica con ciertos aminoácidos, no parece proteger eficientemente contra la progresión de la patología renal en riñones fisiológicamente comprometidos.

Estudio Farmacocinético de dos formulaciones de amoxicilina (500 mg TID en cápsulas vs 750 mg BID en tabletas dispersibles) en voluntarios humanos sanos / Study pharmacokinetics of two amoxycillin formulations (500 mg TDI in capsules vs 750 mg BID in tablets dispersible in healthy human volunters

Se evaluó en voluntarios sanos, el comportamiento de la formulación de 750 mg de amoxicilina B.I.D., en tabletas dispersibles contra la formulación estándar de 500 mg en cápsulas T.D.I., mediante un estudio de dos vías, entrecruzado con nueve voluntarios sanos. Ambas formulaciones fueron administradas durante un lapso de 6 días consecutivos, con un período de lavado de cuatro semanas antes de cambiar de formulación. Los resultados obtenidos en el día 1 y en el día 6 del régimen muestran que la concentración máxima alcanzada por la formulación de 750 mg b.i.d., fue significativamente mayor (p<0.001). No hubo diferencias significativas entre ambas formulaciones en cuanto al tiempo en que se trató en alcanzar la concentración plasmática máxima promedio. El estudio del área bajo la curva de la concentración plasmática de amoxicilina en 24 horas (AUC 0-24h)demostró que ambas formulaciones son bioequivalentes. Considerando un valor de MIC= 1µ/ml (aceptado por el Comité Nacional de Standard para Laboratorios Clínicos (NCSCL) para cepas de patógenos con resistencia intermedia presentes en otitis medias agudas y en infecciones del tracto respiratorio superior) y calculando el tiempo durante el cual las concentraciones plasmáticas se encuentran por debajo de ese MIC, la diferencia entre ambas formulaciones no parece ser clínicamente importante, esto se ha comprobado en la práctica porque ambas formulaciones son igualmente eficaces para el tratamiento de varias patologías infecciosas

Comportamiento hemodinámico intradialitico de los pacientes con insuficiencia renal crónica terminal / Behavior hemodynamics intradialytic of the patients with kidney failure chronic

De la Unidad de Hemodialisis del Departamento de Nefrología del Hospital Central de las FFAA. "Dr. Carlos Arvelo" se tomaron 25 pacientes con insuficiencia renal crónica terminal, clasificándose en dos grupos; conocidos hipertensos antes de la hemodialisis (grupo B), con un rango de edad de 18-64 años, a los cuales se les realizó toma de tensión arterial y frecuencia cardíaca horaria con dinamap durante las sesiones de hemodiálisis dos o tres veces por semana con un promedio de cuatro horas y treinta minutos en un periodo de 3 meses del año 1994. De estos 25 pacientes, 20 (grupo A:7) presentaron cifras tensionales elevadas (TA sistólica: 144-208 mmHg-TA diastólica: 90-132 mmHg) y frecuencia cardíaca en rango normal independientemente de peso seco. Concluimos que la hipertensión arterial en el 60 por ciento de los pacientes es volumen dependiente; ameritando el 40 por ciento, tratamiento antihipertensivo

Hipertensión arterial: causas poco frecuentes presentación de cinco casos clínicos / Arterial hypertension: non frequent causes. Report of 5 cases

En el lapso de dos años (1987-1989) fueron diagnosticado en el Hospital Militar "Dr. Carlos Arvelo" de Caracas (347 casos de Hipertension Arterial, de los cuales 108 pertenecieron al Servicio de Nefrología; de ellos resultaron 52 casos de Hipertension Arterial Esencial y 56 casos de Hipertensión Arterial Secundaria. Traemos a consideración cinco casos de Hipertensión Arterial Secundaria: un (1) caso de Glomerulonefritis rápidamente progresiva; un (1) caso de Hipertensión Arterial multicausal; un (1) caso de Hiperaldosteronismo Primario; un (1) caso de Hipertensión Renovascular por Fibrodisplasia Renal bilateral y un (1) caso de Hipertensión Renovascular por Arteriosclerosis