ABSTRACT
Abstract The tubular hypotrophy of the renal arteries constitutes a rare clinical entity, that is associated with the appearance of renovascular hypertension. Its diagnostic approach is complex and requires the availability of angiography to determine the features of the renal vessels. The following case exemplifies the diagnostic process of a patient with this special clinical situation.
Resumen La hipotrofia de aspecto tubular de las arterias renales constituye una rara entidad clínica asociada a la aparición de hipertensión renovascular. Su enfoque diagnóstico es complejo y exige la disponibilidad de angiografía para determinar las características propias del contorno de la vasculature renal. El caso clínico considerado en este artículo ejemplifica el proceso diagnóstico de un paciente con esta particular situación.
Subject(s)
Humans , Male , Female , Adult , Renal Artery , Anemia, Hypoplastic, Congenital , Hypertension, Renovascular , Colombia , Acute Kidney InjuryABSTRACT
Total serum protein, albumin, total globulin levels, albumin/globulin ratio as well as the various globulin fractions were determined in 96 subjects, 39 are sickle cell (SS) disease subjects (steady state), and 30 are heterozygous sickle cell (AS) trait and 27 normal control subjects. The mean standard deviation of total protein was significantly higher (P< 0.05) in sickle cell disease when compared with heterozygous AS and normal controls. There was also significant difference (P<0.05) between AS and AA. The albumin level in sickle cell disease was significantly higher than in the other two groups. There was hyperglobuneamia observed in SS individuals with a mean ±SD of 32.6±10.0g/L when compared with AS and AA subjects with a mean ±SD of 30.8± 5.9g/L and 26.7±6.2g/L respectively. There also exist a significant difference between AS and AA (P <0.005). The Albumin/Globulin ratio is significantly lower (P <0.05) in SS than the other two groups but there was no significant difference (P>0.05) observed between AS and AA controls. The globulin fractions were observed to be higher in AS individuals except the gamma globulin which is higher in SS subjects. The hyperproteineamia as a result of the hyperglobulineamia is due to the globulin fraction present in the serum of SS individuals. The AS individuals are protected from various infectious disease conditions because of the high acute phase reactants and ß- globulin present in their serum as these has been observed to exert some immunioregulatory role
Subject(s)
Anemia, Hypoplastic, Congenital , Blood Proteins , Homozygote , Serum Albumin , Sickle Cell TraitABSTRACT
La anemia Hipoplásica Eritroide Congénita o Síndrome de Blackfan-Diamond es una enfermedad poco frecuente, se caracteriza por baja talla, anemia normocítica normocrómica severa con reticulopenia e hipoplasia eritroide. Puede presentar defectos vasculares, esqueléticos y dismorfias faciales que se evidencian antes del año de edad. El Síndrome de Ehlers-Danlos tipo IV es un trastorno hereditario del tejido conectivo con manifestaciones clínicas muy variadas y formas de moderada a severa, con hiperlaxitud de la piel e hipermovilidad articular y fragilidad del sistema vascular afectando múltiples órganos y sistemas con elevado índice de mortalidad. Reportamos un caso de un paciente de 15 años de edad que debutó a los 3 meses de nacido con una anemia Hipoplásica Eritroide Congénita y un Síndrome de Ehlers-Danlos, ha recibido tratamiento con glóbulos y prednisona y tiene una evolución satisfactoria a pesar de presentar estas dos entidades clínicas
Congenital erythroblastic or Hypoplastic anemia or Syndrome of Blackfan-Diamond is a little frequent disease, which is characterized by low stature, erithroblastic severe monocytic anemia with reticulopenia and hypoplasia. It can present vascular and skeletal defects, and dimorphism face that developed before the year of age. The Syndrome of Ehlers-Danlos type IV is a hereditary disorder of the connective tissue with very varied clinical manifestations and forms from moderate to severe, with increased elasticity of the skin and articular hyper-mobility and fragility of the vascular system, multiple organs and systems being affected with high mortality rate. We present the case of a 15 year old patient who made his debut at three mo of age, born with congenital Hypoplastic anemia and syndrome of Ehlers-Danlos. He has received treatment with globules and prednisone and had a satisfactory evolution in spite of presenting these two clinical entities.
Subject(s)
Humans , Male , Adolescent , Anemia, Hypoplastic, Congenital , Ehlers-Danlos SyndromeABSTRACT
Authours have observed a case of congenital hypoplastic anemia in 21 months old Korean male patient who was diagnosed through aspirations of bone marrow. His hematologic improvement was followed by administrations of prednisolone. A brief review of related literature was made.