RESUMO
Weekly monitoring of absolute neutrophil count (ANC) under deferiprone therapy in thalassemia patients is recommended to avoid agranulocytosis adverse event. Actually, this recommendation may not be applicable in clinical setting. Our study aimed to establish incidence of neutropenia under deferiprone (DFP) monotherapy when it was monitored bimonthly due to socioeconomic conditions effecting local and refugee thalassemic patients including Syrian origin (SYR; n = 26) and Turkish origin (TR; n = 26) groups. Patients on DFP were followed up for 12 months. Fifteen neutropenic episodes were seen in 5 patients. All 5 patients (4 from SYR group and 1 from TR group) had splenomegaly and hypersplenism, and neutropenia ceased in 4 patients after splenectomy despite continuation of deferiprone. In the TR group, the frequency of patients who have neutropenia (absolute neutrophil count [ANC] <1500/mm(3)) was 3.8% (n = 1) in the 1st month, no patients in TR group had neutropenia until 10th month when again there was 1 patient with mild neutropenia. In SYR group, the frequency of patients who have neutropenia was 3.8% (n = 1), 7.7% (n = 2), and 11.5% (n = 3) in the 1st, 2nd, and 3rd months, respectively, and was found to be 3.8% (n = 1) between 6 and 12 months. Whether or not DFP therapy should be interrupted in case of mild neutropenia and the frequency of monitoring ANC in real-life conditions should be documented with further studies. Other causes of neutropenia in DFP-treated patients should also be kept in mind.
Assuntos
Transfusão de Sangue , Neutropenia , Piridonas , Talassemia , Adolescente , Anemia Aplástica/sangue , Anemia Aplástica/epidemiologia , Anemia Aplástica/etiologia , Anemia Hemolítica/sangue , Anemia Hemolítica/epidemiologia , Anemia Hemolítica/etiologia , Doenças da Medula Óssea/sangue , Doenças da Medula Óssea/epidemiologia , Doenças da Medula Óssea/etiologia , Transtornos da Insuficiência da Medula Óssea , Criança , Pré-Escolar , Deferiprona , Feminino , Hemoglobinúria Paroxística/sangue , Hemoglobinúria Paroxística/epidemiologia , Hemoglobinúria Paroxística/etiologia , Humanos , Masculino , Neutropenia/sangue , Neutropenia/epidemiologia , Neutropenia/etiologia , Piridonas/administração & dosagem , Piridonas/efeitos adversos , Estudos Retrospectivos , Fatores Socioeconômicos , Síria/epidemiologia , Talassemia/sangue , Talassemia/epidemiologia , Talassemia/terapia , Turquia/epidemiologiaRESUMO
One of the most important causes of mortality in thalassemic patients is infectious disease. Thalassemic patients develop severe invasive infection caused by microorganisms that are rare in healthy individuals. We describe the case of a 13-year-old splenectomized boy who presented with septic shock and who died 36 h after admission, despite broad-spectrum antibiotics and aggressive supportive care. Serratia marcescens was isolated from cultures of blood and tracheal aspirate. It is known that rare microorganisms will cause severe community-acquired infection in splenectomized patients with thalassemia major.
Assuntos
Sepse/microbiologia , Infecções por Serratia/complicações , Serratia marcescens , Talassemia beta/complicações , Adolescente , Evolução Fatal , Humanos , MasculinoRESUMO
The aim of the study was to describe the characteristics of patients who applied to the Emergency Department (ED) due to submersion injury; to recognize the risk factors, complications, causes of death, and the educational needs of families and caregivers about unsafe environments for submersion; and to develop preventive strategies. All patients were analyzed retrospectively according to demographic features, clinical and laboratory findings, association between clinical variables and submersion injuries, and patient outcomes. Fifty-five patients with submersion injury were analyzed. The mean age of patients was 10.9 ± 4.7 years. The most common Szpilman clinical scores were Grade 1 (24 patients, 43.8%), Grade 2 (15 patients, 27.3%), and Grade 5 (10 patients, 18.2%). The common location of the submersion injuries included the sea (74.5%), pool (18.4%), bathtub (7.3%), river (3.6%), and lake (3.6%). A limited swimming ability or exhaustion and suffocation (49.1%) due to unknown reasons were the most common causes of submersion injury among all patients. Most complications were due to aspiration pneumonia and hypoxic ischemic encephalopathy (HIE). Thirty-nine patients (70.9%) were followed in the ED, while 16 patients (29.1%) were admitted to the pediatric intensive care unit (PICU); 11 patients (20.0%) died. All of the risk factors of drowning should be taken into account when designing preventive measures and family education. In addition, all pediatricians should be trained periodically about the complications of submersion and the treatment strategies, particularly in coastal cities and areas where drownings occur frequently.