Hematopoyesis extramedular en paciente con esferocitosis hereditaria asintomática / Extramedullary hematopoiesis in patients with asymptomatic hereditry spherocytosis

En diversas patologías con alteraciones en la producción de los elementos formes de la sangre pueden desarrollarse focos de hematopoyesis extramedular en diferentes sitios. Los más frecuentes son: bazo, hígado, ganglios linfáticos, y más raramente otros órganos como: glándulas adrenales, hillios renales, cartílagos, ligamentos, tejido adiposo, timo, pulmón, mediastino y duramadre de cráneo y columna. Generalmente el proceso es difuso pero pueden formarse grandes tumores de tejido hematopoyético. Las condiciones patológicas de la médula ósea más frecuentemente asociadas a hematopoyesis extramedular son: esferocitosis hereditaria, talasemia, síndromes mieloproliferativos con fibrosis medular, ocupación medular por patologías neoplásicas. presentamos un paciente de 45 años con esferocitosis hereditaria con masas de tejido hematopoyético extramedular paravertebral mediastinal que respondieron favorablemente a la esplenectomía.

Hereditary spherocytosis (HS) is a relatively common inherited hemolytic disorder in northern Europe and in the US. The reported prevalence of HS in Western countries is 1:5000. We describe a patient 45 years old, with hereditary spherocytosis with masses of mediastinal paravertebral extramedullary hematopoietic tissue, with a favorable response to splenectomy. The medical lieterature refers some cases of extramedullary hematopoiesis as a clinical expression of hereditary spherocytosis, mainly as thoracic masses with usually paravertebral localization. HS should be distinguished from other spherocytic hermolytic anemias. Diagnosis is usually made uring infancy or in young adults, but it can be at any moment of their life, until the seventh decade of life. Ocasionally, the diagnosis is first made in old age. The clinical expression of HS is highly variable, ranging from asymptomatic condition to a severe life-threatening hemolytic anemia. Laboratory features include spherocytosis, osmotic fragility, manifestations of hemolytic disease, elevated unconjugated bilirubin and reticulocytosis. The principal diagnostic test, RBC osmotic fragility, measures the surface/volume Ratio of the cells. The treatment of choice of HS in patients with inherited spherocytosis is splenectomy, which corrects hemolytic anemia. According to he literature, cases of failure following splenectomy have been reported.

Selección de receptores pediátricos en trasplante de riñón / Selection of pediatric renal trasplant recipients

El trasplante renal es la terapia de elección para el manejo de la Enfermedad Renal Crónica Terminal (ERCT) en pediatría. La evaluación previa debe ser cuidadosa, para minimizar los riesgos del trasplante y de las condiciones que afecten la sobrevida del paciente y del injerto. El estudio comprende una adecuada selección del donante, y una evaluación completa del paciente, que debe considerar la etiología de la ERCT, las infecciones e inmunizaciones que ha presentado el paciente a lo largo de su vida, así como el estado serológico para ciertas infecciones relevantes como el CMV y EBV. El estudio inmunológico comprende antecedentes de eventos sensibilizantes, tipificación de grupo sanguíneo y HLA, estudio de anticuerposy pruebas cruzadas (XM), que permita predecir los riesgos de rechazo y planificar estrategias de inmunosupresión individualizadas, de acuerdo a cada situación particular. Se describen los nuevos métodos de estudio, en fase sólida, de la respuesta aloinmune.

Kidney transplantation is the therapy of choice for management of chronic renal disease (ESRD) in children. The appraisal should be careful to minimize the transplant risks and conditions that affect patient and graft survival. The study includes an adequate selection of the donor, and a thorough patient evaluation, which must consider the etiology of ESRD, infections and immunizations presented by the patient throughout their lives, and the serologic status for certain relevant infection, as CMV and EBV. The immunological assesment includes accurate donor typing, history of sensitizing events, blood group and HLA typing and HLA antibody study and crossmatch (XM), to predict the risk of rejection and plan individualized strategies of immunosuppression, according to each particular situation. Novel methods of measuring T-cell alloinmune potential are briefly discussed.

Effects of soy milk as a dietary complement during the natural aging process.

INTRODUCTION: Oxidative stress is one mechanism that could contribute to the acceleration of aging and age-related diseases. On the other hand, because of their antioxidative qualities soybean derived foods could have beneficial effects on the aging process. OBJECTIVES: The aim of our work was to study the effects of a diet supplemented with soy milk on certain biological features of aging in rats. METHODS: Male Wistar rats of 3 to 18 months of age, were assigned to one of two diets: 1) Experimental Group, commercial rat formula and soy milk; 2) Control Group, commercial rat formula and water. Every three months both lipid profile and lipid peroxidation were determined and neuronal cells of hippocampus were counted in control and experimental rats. RESULTS: The soy milk diet significantly improved the plasmatic lipid profile, decreasing serum cholesterol (total as well as LDL) and serum triglycerides, HDL-cholesterol was significatively higher in experimental animals. The LDL/HDL ratio was thus significantly lowered. The soy diet also produced decreased values of lipid peroxidation in brain, liver and kidney. These effects were significant after 6 to 9 months. The experimental animals lost fewer hippocampal neurons than the controls. Finally at 18 months of age, a greater number of surviving animals in experimental group with respect to the control one was observed. CONCLUSIONS: 1) soy intake could have beneficial effects as a complement of normal diet, but not as a replacement for animal proteins and 2) these effects are the result of a very long period (almost lifelong) of consumption of this diet.

Recovery from experimental malnutrition with soymilk: immunological and genetic aspects.

Experimental malnutrition models have been useful to study the effects of malnutrition at early ages. Substantial evidence exists that malnutrition in critical stages of development could result in chromosomal damages. The effect of nutritional rehabilitation with soymilk as a complement of a restricted diet, on plasma and muscle proteins, chromosomal integrity, and unspecific and mucosa immune responses, was studied. Adult male and female Wistar rats (5 weeks old) were assigned to different nutritional conditions: (a) 14 days on protein restricted diet (corn flour and water), followed by 14 days in which water was replaced by soymilk, as nutritional rehabilitation; (b) the same conditions above but periods of 28 days of a protein restricted diet, and 28 days of nutritional rehabilitation and (c) age-matched malnourished (protein restricted diet without nutritional rehabilitation) and normally nourished controls. After both nutritional rehabilitation periods, the weights reached were significantly higher (p < 0.001) than the malnourished control values, but lower than the normal control ones. Plasma protein concentrations were similar in all groups. Muscle proteins that were diminished during the restricted diet, reached normal control values after both rehabilitation periods. The protein restricted diet, produced numeric and structural chromosomal abnormalities. Nutritional rehabilitation was only partially able to revert these abnormalities. The phagocytic activity and gut mucosa IgA-secreting cells were significantly reduced (p < 0.001) during the restricted diet; both nutritional rehabilitation periods induced a significant increase of both, phagocytic activity and IgA secreting cells. These values were similar to controls. Our results show that the supplementation of a protein-restricted diet with soymilk improved tissue protein content, as well as unspecific and gut mucosa immune responses, even though it was not able to reinstate fully normal body weight and a normal chromosome karyotype.