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In this work, plant-based shakes were prepared (5% oil, 6% protein, 1% lecithin, 88% water) (w/w) using two processing techniques (i) only mixing versus (ii) mixing followed by high pressure homogenisation, as well as two processing sequences (i) adding all ingredients together versus (ii) stepwise addition of ingredients. Shakes only mixed consisted of large, irregular particles (1–100 µm). Eventually, this resulted in a relatively low lipid and protein digestion extent after 2 h of gastric pre-digestion (9% and < 1%, respectively). In contrast, shakes that were subjected to high pressure homogenisation displayed small, homogeneous particles (

Introduccion. Los trastornos de la biogenesis de los peroxisomas se deben a mutaciones en los genes PEX, que codifican peroxinas requeridas para la biogenesis peroxisomica. Clinicamente se expresan como un espectro del sindrome de Zellweger, y hay una amplia variedad fenotipica. Su diagnostico se realiza bioquimicamente y la confirmacion es molecular. El objetivo de este caso ilustrativo es resaltar la importancia de la clinica y de las pruebas bioquimicas en el abordaje de una enfermedad peroxisomica. Caso clinico. Nino de 3 anos con hipotonia neonatal, retraso global del desarrollo y fallo de medro, con un patron en resonancia cerebral de leucodistrofia hipomielinizante, en quien se habia sospechado un trastorno de la biogenesis de los peroxisomas por encontrarse una variante de significado incierto en PEX5, pero su clinica, los estudios bioquimicos y el analisis critico de las pruebas moleculares hacian improbable este diagnostico. Se hace enfasis en el abordaje que deberia tenerse cuando se sospecha un trastorno del espectro del sindrome de Zellweger. Conclusion. En el caso descrito se sospecho un trastorno de la biogenesis de los peroxisomas por una secuenciacion exomica que, al analizarse criticamente junto con la clinica y los hallazgos bioquimicos, hacia muy poco probable una enfermedad peroxisomica. Cuando se tiene sospecha clinica y por neuroimagenes, el abordaje diagnostico principal debe partir del analisis bioquimico. Aunque la confirmacion es molecular, estas pruebas deben interpretarse con precaucion.

Sickle cell anemia and alpha-thalassemia have a heterogeneous distribution in Venezuela with a high frequency in the coastal area (sea level) and few cases in the mountains. Most of our population is an ethnic admixture of Europeans (Spaniards colonists), Africans (slaves), and Amerindians. The purpose of our study was to determine the origin of the beta(s) globin haplotype, age and survival dependency, and the admixture among the different African groups in our population. The alpha(3.7) globin gene deletion status was also studied and found in a very high frequency. DNA from peripheral blood of 191 non-related patients (81 with HbS homozygous and 15 patients compound heterozygous for HbS, HbC, HbD with beta-thalassemia, and 95 with sickle cell trait) were studied. The beta(s) chromosome was linked 51% to the Benin Haplotype, 29.5% with the CAR, 12.5% to the Senegal, and 2.5% to the Cameroon. We did not find any significant difference between the haplotype distribution among adults and children and among sickle cell patients and traits. Only 8.6% of the patients have homozygosity for the Benin haplotype. These results show a very high frequency of admixture in our African origin population.

beta Thalassemia (Thal) mutations were studied in DNA from 80/159 patients with hemolytic anemia and high levels of Hb A2 by the amplification refractory mutation system technique (ARMS-PCR). This method detects point mutations and insertions or deletions of just a few nucleotides in the beta globin gene by the polymerase chain reaction of allele-specific priming. In 43/80 patients with different clinical presentations of beta Thalassemia and 37/80 compound heterozygous for hemoglobinopathies and beta Thalassemia the most frequent mutation found was the -29 (of African origin), followed by the CD39 (of Mediterranean origin) and in a lower frequency also was found the -88, the IVSI-6 and the IVSI-110. We conclude that this technique is an useful approach in determining the beta thalassemia mutations in population surveys, because it allows to make a differential diagnosis between beta Thalassemia minor and individuals with high levels of Hb A2. It helps to clarify the diagnosis of patients with structural hemoglobinopathies that also presents high levels of Hb A2.

Natural products of several plants, according to the geographic location, are used by Peruvian people in the popular treatment of diarrhea, with good success. When cholerae cases appeared in Peru, we were interested to know the "in vitro" effect against Vibrio cholerae 01, of these useful plants to treat diarrhea. The following plants were tested: Cichorium intybus, Althaea officinalis, Psorela glandulosa, Geranium maculatum, Punica granatum, Malus sativa, Cydonia oblonga, Chenopodium ambrosoides, Krameria triandria, Tea chinensis, Daucus carota, Persea gratissima, Psidium guayaba and Lippia dulcis. Decoction or infusion of the plants were used in the "in vitro" experiments. The following plants showed no "in vitro" effect against V. cholerae: Cichorium intybus, Althaea officinalis, Psorela glandulosa, Geranium maculatum, Chenopodium ambrosoides, Krameria triandria, Psidium guayaba, Lippia dulcis and Daucus carota. Decoction of Malus sativa and Cydenia oblonga showed bactericidal effect for their acidity and stone avocado (Persea gratissima) a late bactericidal effect. Tea infusión and the decoction of Punica granatum peel, showed the best bactericidal effect and we suggest to use them as to stop cholera spreading.

A mild anemia (hemoglobin 9 g/dl) was found in a patient from Seville (Spain) with marked morphological abnormalities in the peripheral blood smear. The red cell osmotic fragility showed a mild resistance curve with a mean cell fragility (MCF) of 0.375% NaCl (normal = 0.450). Chemical Chemical and thermal instability test and search for inclusion bodies gave positive results. Hemoglobin electrophoresis at pH 8.9 revealed absence of Hb A, a major component of fast mobility (94%), and increased Hb F and Hb A2 levels (1.5% and 4.6%, respectively). The fast fraction, isolated and purified by means of cellulose acetate electrophoresis, precipitated in acid acetone and treated with urea 8 M and mercaptoethanol, revealed an anomalous beta chain. Trypsin-digested globin peptides were separated by high-voltage electrophoresis at pH 6.4 and ascendant chromatography. With differential staining, an extra peptide was detected in an unusual site, more anodic than alpha Tp4 but in lower position. Peptide map of the fast beta chain, stained with ninhydrin, and also for Tyr, confirmed the position of the new peptide and the absence of the usual beta Tp13. The new peptide, separated by high-voltage electrophoresis at pH 3.5, revealed absence of Val and the presence of an additional Glu residue, which should appear only in position beta 126. The diagnosis of Hb Hofu (alpha 2 beta 2 126 Val----Glu; H4) was reached, thus interpreting its increase and the absence of Hb A, as an association with beta o-thalassemia, producing a mild hemolytic anemia. Evidence was obtained that Hb Hofu is a mild unstable hemoglobin variant.

In a survey on listeriosis in Peru a study was made of 653 stool samples; material from 8 cases of perinatal death, in which a macroscopic diagnosis of listeriosis had been made at autopsy; 1 sample of vaginal secretion from a woman with abortion due to listeriosis; and 1 case of abscess which turned out to be an hematometra. The following results have been obtained: 1. From the faeces 7 isolates of Listeria (1.07%) were obtained: 3 belonged to L. monocytogenes serovar 4ab, 3 to L. innocua (the former "4g"--nownow serovar 6b) and 1 to L. monocytogenes serovar 4a. 2. The 8 strains of autopsy materials belonged to L. monocytogenes: 6 serovar 4b and 2 serovar 4d. 3. From the vaginal secretion L. monocytogenes serovar 4b was cultured. 4. The strain from the hematometra was identified as L. monocytogenes serovar 4b. The serovars 4a, 4ab and 4d are at this time the only ones identified in Peru as cause of human listeriosis. The serovars of serogroup 1/2 frequent in Western and primarily in Central Europe, were so far never cultivated.

A case of Listeria infection is reported of a 10 year old girl with symptoms of the first menstruation but without the discharge of blood because of the absence of the vaginal duct. The blood retained in the uterus obtained by puncture through the rectum contained Listeria monocytogenes 4b. The infection was possibly acquired from a household pet.

Two hundred forty-one strains of Salmonella typhii isolated in Lima, Peru, from October 1981 through February 1983 were tested for susceptibility to antimicrobial agents. Seventy-two strains (29.9%) were resistant to chloramphenicol and other antibiotics, including ampicillin, sulfonamides, and trimethoprim. The minimal inhibitory concentrations of 17 antimicrobial agents were determined for all of these chloramphenicol-resistant strains. Sch 25393, new beta-lactams, new quinolones, and the formulation clavulanic acid-amoxicillin were effective against all the strains. Four different resistance patterns distributed among eight phage types were observed. The 72 resistant S. typhi could transfer the resistance marker into Escherichia coli C1, and all the plasmids belonged to the incompatibility group H1.