Microcytosis, defined as a mean corpuscular volume (MCV) level of less than 82 f L, is a result of impaired hemoglobin (Hb) synthesis in thalassemias and in disorders of iron metabolism. It also occurs in some structural hemoglobinopathies with Hb E [b26(B8)Glu!Lys] and Hb C [b6(A3)Glu!Lys]. In Hb C, the replacement Glu!Lys results in an increased positive charge that causes the adherence of this abnormal Hb to the red cell membrane, leading to the loss of Kþ and water, and increasing the mean corpuscular Hb concentration (MCHC) and cell density. In homozygous Hb C disease and in Hb SC disease there is a reduction of erythrocyte diameter, intracellular crystallization of Hb and formation of microspherocytes. In Hb C trait, a low MCV value is frequently observed and has sometimes been suggested be the result of a co-existent aþ-thalassemia (thal), which reaches high frequencies in endemic malaria areas where Hb C is also common. However, there are little available data on the hematological aspects of the association Hb C trait and a-thal. In the Brazilian Black population, Hb C and aþ-thal (the a rightward deletion) are found in frequencies around 1 and 23%, respectively. To evaluate the presence and the effects of this interaction on MCV levels, the a deletion was investigated in 136 adult Hb C trait individuals (Hb AC), referred to the UNICAMP University Hospital, in Campinas, State of São Paulo, in the

CONTEXT:There are today only a limited number of studies defining growth parameters and nutritional status for HIV children. OBJECTIVE:To study the nutritional status of infants infected with the human immunodeficiency virus. TYPE OF STUDY:Longitudinal study. SETTING:Department of Pediatrics, Faculty of Medical Sciences, UNICAMP, Campinas, Brazil. PARTICIPANTS:One hundred and twenty-four children born to HIV infected mothers were evaluated from birth until the age of two years. They were subdivided into two groups: 71 infected children and 53 non-infected children. MAIN MEASUREMENTS:Growth was evaluated in both groups by comparing Z-scores for weight/age (w/a), length/age (H/a) and weight/length (w/H) (using the NCHS curves as reference). RESULTS:The Z-score analyses showed that there was a significant difference between the two groups for all the variables studied, except for the H/a value at 3 months of age and the W/H value at 21 months of age, which showed P > 0.05. CONCLUSIONS:The growth of infected infants was observed to be severely affected in comparison with that of seroreversed infants in the same age groups. Although clinical manifestations may take time to appear, the onset of growth changes begin soon after birth.

Seven unrelated patients with hemoglobin (Hb) H disease and 27 individuals with alpha-chain structural alterations were studied to identify the alpha-globin gene mutations present in the population of Southeast Brazil. The -alpha3.7, --MED and -(alpha)20.5 deletions were investigated by PCR, whereas non-deletional alpha-thalassemia (alphaHphalpha, alphaNcoIalpha, alphaalphaNcoI, alphaIcalpha and alphaTSaudialpha) was screened with restriction enzymes and by nested PCR. Structural alterations were identified by direct DNA sequencing. Of the seven patients with Hb H disease, all of Italian descent, two had the -(alpha)20.5/-alpha3.7 genotype, one had the --MED/-alpha3.7 genotype, one had the --MED/alphaHphalpha genotype and three showed interaction of the -alpha3.7 deletion with an unusual, unidentified form of non-deletional alpha-thalassemia [-alpha3.7/(alphaalpha)T]. Among the 27 patients with structural alterations, 15 (of Italian descent) had Hb Hasharon (alpha47Asp-->His) associated with the -alpha3.7 deletion, 4 (of Italian descent) were heterozygous for Hb J-Rovigo (alpha53Ala-->Asp), 4 (3 Blacks and 1 Caucasian) were heterozygous for Hb Stanleyville-II (alpha78Asn-->Lys) associated with the alpha+-thalassemia, 1 (Black) was heterozygous for Hb G-Pest (alpha74Asp-->Asn), 1 (Caucasian) was heterozygous for Hb Kurosaki (alpha7Lys-->Glu), 1 (Caucasian) was heterozygous for Hb Westmead (alpha122His-->Gln), and 1 (Caucasian) was the carrier of a novel silent variant (Hb Campinas, alpha26Ala-->Val). Most of the mutations found reflected the Mediterranean and African origins of the population. Hbs G-Pest and Kurosaki, very rare, and Hb Westmead, common in southern China, were initially described in individuals of ethnic origin differing from those of the carriers reported in the present study and are the first cases to be reported in the Brazilian population.

(2000). Hb Campinas [α26(B7)Ala→Val]: A Novel, Electrophoretically Silent, Variant. Hemoglobin: Vol. 24, No. 2, pp. 143-148.