Adherence to treatment in children with growth hormone deficiency, small for gestational age and Turner syndrome in Mexico: results of the Easypod™ connect observational study (ECOS).

BACKGROUND: Assessing adherence to growth hormone (GH) is challenging. The Easypod™ connect device delivers pre-set doses of recombinant human GH (r-hGH) and stores a digital record of adherence that can be shared with healthcare provider. We assessed adherence to r-hGH delivered with Easypod™ according to the approved pediatric indications for r-hGH: growth hormone deficiency (GHD), born small for gestational age (SGA) who failed to show catch-up growth and Turner syndrome (TS). METHODS: ECOS (NCT01555528) was a multicenter (24 countries), 5-year, longitudinal, observational study, which aimed to evaluate country-specific adherence to r-hGH therapy prescribed via the Easypod™ electronic injection device. The primary endpoint was yearly adherence. Secondary endpoints were height velocity, height velocity standard deviation scores (SDS), height, height SDS and IGF-1 concentrations. Clinical and auxological data were obtained from medical records and adherence from Easypod™ logs. RESULTS: This study included 147 Easypod™-naïve Mexican children assessed during 3 years (mean age: 9.96 ± 3.41 years, 56.8% boys, mean height SDS at baseline: - 2.17 ± 0.97): 118 with GHD, 24 SGA and 5 with TS. A total of 105 (71.4%) patients were GH naïve. Overall median adherence was > 90% over the first year of treatment and > 80% at 3 years. Adherence was not different by r-hGH indication or between GH-naïve or experienced patients. At 1-year follow-up, mean change in height SDS was 0.57 ± 0.34, whereas mean height velocity SDS was 2.85 ± 2.51. In all, 84.7% patients had normal IGF-1 concentrations at 1-year follow-up. Adherence was associated with change in height SDS (r = 0.239, p = 0.005) and height velocity SDS (r = 0.194, p = 0.027). CONCLUSION: Adherence rates with the Easypod™ device are high and maintained over time in GHD, SGA and TS Easypod™-naïve Mexican patients. High adherence is associated with better outcomes. Easypod™ assists physicians in monitoring adherence to r-hGH.

Iron stores and coagulation parameters in patients with hypoxemic polycythemia secondary to chronic obstructive pulmonary disease: the effect of phlebotomies.

UNLABELLED: This study was designed to determine the effects of phlebotomy on iron body contents and coagulation tests of COPD patients with polycythemia secondary to hypoxemia. Seventeen patients with COPD and hematocrits higher than 54 percent (mean Hct: 57 +/- 0.49 percent), who had not received anti-inflammatory or antiplatelet aggregation agents recently. Their mean forced expiratory volume at 1 second (FEV1) was 0.92 +/- 0.11 L. INTERVENTION: Blood work was collected to evaluate the following: serum iron and ferritin levels, total iron binding capacity, transferrin saturation index, fibrinogen plasma levels, activated partial thromboplastin time, platelet count, platelet aggregation measurements, and thromboelastography coagulation parameters. The blood samples were obtained before and about 7 days after the hematocrit correction by 300-400 ml phlebotomies done every other day. The mean number of phlebotomies done for each patient was 4.4. Postphlebotomy iron serum levels decreased from 90.1 +/- 14.8 to 59.7 +/- 9.9 mg/dl and the ferritin serum levels from 133.8 +/- 37.9 to 70.8 +/- 32.7 ng/ml (p < 0.05). Regarding the coagulation studies, there were significant increases in the platelet count, from 227,300 +/- 13,900 to 312,500 +/- 30,200 per mm3, and in the maximum clot amplitude (a) obtained by thromboelastography (from 53.6 +/- 1.4 percent to 60.4 +/- 1.1 percent). The coagulation time (k) of the thromboelastography also decreased significantly, from 7.5 +/- 0.7mm prephlebotomy to 4.5 +/- 0.3mm postphlebotomy. Although the coagulation changes were small amount, the observed significant decrease in iron contents may have clinical implications.

Retrospective prevalence and incidence of drug-induced agranulocytosis in the city of São Paulo-Brasil.

Retrospective study carried out in São Paulo, Brazil, from September 1981 through March 1990. During this period the study investigated 19,389 hematological patients, aiming to assess the prevalence and estimate the incidence of drug-induced agranulocytosis. To assess the Hematology Center's catch population, where the study took place, we adopted the incidence of hemophilia A, chronic myeloid leukemia and acute leukemia, described by the international literature as reference for our catch population estimate. Our findings revealed a prevalence of 1 case of agranulocytosis per 3,878 subjects treated at the São Paulo Hematology Center which translates into an incidence of 0.44 to 0.82 cases of agranulocytosis per million inhabitants, per year.

Tratamento da anemia ferropriva do adulto com o complexo hidroxido de ferro+++ polimaltosado usado por via oral. / Treatment of hypochromic anemia in the adult with polymaltosed iron hydroxide+++ complex used by oral route

Os autores estudaram o uso do complexo hidroxido de ferro (Fe+++) polimaltosado em pacientes com anemia ferropriva atraves um esquema do tipo doseefeito. O objetivo foi o de avaliar a eficacia e tolerabilidade do preparado terapeutico. Os pacientes foram divididos em tres grupos, cada um com 7 portadores de anemia ferropriva grave.Os grupos diferiam apenas pelo emprego da dose diaria do complexo hidroxido de ferro (Fe+++) polimaltosado na forma de xarope, contendo cada ml o equivalente em ferro elementar de 10 mg. Os pacientes do grupo I receberam 100 mg/dia, do grupo II 200mg/ dia e do grupo III 300mg/dia. Tendo em vista a nitida resposta terapeutica do tipo dose-efeito e a ausencia de efeitos colaterais nos tres grupos estudados, os autores concluem pela eficacia e boa tolerabilidade do preparado

Hemostasis in schistosomiasis mansoni.

Hemostasis was studied in 18 patients with the hepato-intestinal form of Schistosomiasis mansoni and in 23 with the hepato-splenic form. Both groups are compared referring to alterations found. The relations between plasmatic coagulation factors and hepatocytic function tests are studied. In 6 patients the explorations were repeated after administration of hycanthone. In the patients with the hepato-splenic form, the presence of chronic consumptive coagulophathy was found. The coagulopathy disappeared either by treatment with heparine or by splenectomy. This observation points out the importance of splenomegaly in the development of chronic consumptive coagulopathy, and may be an important factor in indicating splenectomy in a patient with portal hypertension due to Schistosomiasis mansoni.