Four adult hemoglobin types in one mulatto family.

The studied family showed the presence of four different types of hemoglobin. The family member who gave rise to this study (=propositus) presented Hb C and the hybrid Hb CG-phila. The propositus has three children, all of which have Hb AC; none of the family members showed any clinical symptoms. The investigation of the hemoglobin arose from the finding of target red cells in a blood test done during the pre-operatory examination for lower limb varicose vein stripping. The hybrid Hb CG-phila is due to two gene pairs, each of which with individual expression, determining the synthesis and the particular type subunits. The hybrid Hb CG-phila is formed by the combination velocity of the subunits alpha 2G-phila beta 2; therefore the proportion of the hybrid Hb CG-phila is lower than Hb G-phila and Hb C. The identification and molecular characterization of Hb G-phila showed the position alpha 2(68) Asn-->Lys beta 2 and Hb C showed alpha 2 beta 2(6) Glu-->Lys.

[Treatment of sickle cell anemia crisis with dipyrone, hydrocortisone, and fluid therapy]. / Tratamento da crise de anemia falciforme pela dipirona, hidrocortisona e hidratação.

It was observed "in vitro" that dipirone improves hemoglobin S solubility, and that corticosteroids by antiinflammatory action relieve pain in sickle cell anemia crises. In the period between 1983 and 1988, 116 patients with sickle cell anemia having painful crises were studied. All patients were hydrated with DW (5% normal Serum Sodium/NSS) 1:1, in a total volume of 2250 ml/square meters (sgm) day. Patients were distributed as follows: Group 1: 26 patients treated with an association of dipirone and hidrocortisone; Group 2: 21 patients treated with hidrocortisone only; Group 3: 22 patients treated with dipirone only; Group 4: 23 patients treated only with hidration; Group 5: 24 patients treated only with DW 50%. Treatment efficacy was evaluated on the subjective assessment (complete pain relief of patients, 24 hours after the beginning of the treatment). We have concluded that treatment with dipirone and/or hidrocortisone plus hidration was efficient for pain relief in sickle cell crises.

[Hemoglobin H: laboratory identification]. / Hemoglobina H: identificação laboratorial.

Hemoglobin H (Hb H) disease is an alpha thalassemia form characterized by low synthesis of alpha chain and high beta chain concentration; this unbalance induces the beta chain tetramers formation. Hb H is relatively frequent in Thailand and Greece. Isolated cases have been reported in Chinese, Filipinos, Malaysians. In the Near East occasional cases were observed in Greek Cypriots and Jordanian Arabs. Hb H carriers were found in Italy, Spain, Canada, Indonesia and other countries. In Brazil there are descendants of Italians, Chinese and people of negro origin who are carriers of Hb H. We identified the Hb H by electrophoresis, instability and characteristic inclusion bodies.

Funduscopic alterations in SS and SC hemoglobinopathies. Study of a Brazilian population.

Fundus changes in sickle cell disease are well studied and documented according to their clinical and angiographic aspects. Sickle cell retinopathy was studied in a Brazilian population of 63 patients (41 with SS hemoglobinopathy, and 22 with SC hemoglobinopathy). All ophthalmoscopic changes observed in our patients were more frequent in the SC group with the exception of atrophic retinal tears, found only in the SS group. Proliferative retinopathy including its complications was seen in 54.54% of the eyes of the SC group, and in only 14.64% of the eyes of the SS group. Decreased vision is consequently greater in the SC group. Despite the great frequency of funduscopic changes in both groups, only 1 patient suffered irreversible visual loss due to retinal detachment operated on without success.