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1.
Blood Adv ; 5(1): 207-215, 2021 01 12.
Artigo em Inglês | MEDLINE | ID: mdl-33570644

RESUMO

We aimed to identify predictors of outcomes and survival in patients living in 4 major metropolitan areas who had sickle cell disease (SCD) and COVID-19 to inform best approaches to prevention and care. Data were collected at baseline and during the clinical course in SCD patients diagnosed with COVID-19 in four COVID-19 epicenters. Patients were followed up posthospital discharge for up to 3 months. Of sixty-six SCD patients with COVID-19, fifty patients (75%) required hospitalization, and seven died (10.6%). Patients with preexisting kidney disease (chronic kidney disease) were more likely to be hospitalized. The most common presenting symptom was vaso-occlusive pain. Acute chest syndrome occurred in 30 (60%) of the 50 hospitalized patients and in all who died. Older age and histories of pulmonary hypertension, congestive heart failure, chronic kidney disease, and stroke were more prevalent in patients who died, as were higher creatinine, lactate dehydrogenase, and D-dimer levels. Anticoagulation use while inpatient was twice less common in patients who died. All deaths occurred in individuals not taking hydroxyurea or any other SCD-modifying therapy. Patients with SCD and COVID-19 exhibited a broad range of disease severity. We cannot definitively state that the overall mortality is higher in patients with SCD, although our case fatality rate was ∼10% compared with ∼3% in the general population, despite a median age of 34 years. Individuals with SCD aged >50 years, with preexisting cardiopulmonary, renal disease, and/or stroke not receiving hydroxyurea, who present with high serum creatinine, lactate dehydrogenase, and D-dimer levels, are at higher risk of death, irrespective of genotype or sex.


Assuntos
Anemia Falciforme/complicações , /complicações , Síndrome Torácica Aguda/sangue , Síndrome Torácica Aguda/complicações , Síndrome Torácica Aguda/mortalidade , Síndrome Torácica Aguda/terapia , Adulto , Anemia Falciforme/sangue , Anemia Falciforme/mortalidade , Anemia Falciforme/terapia , Antidrepanocíticos/uso terapêutico , /mortalidade , Progressão da Doença , Feminino , Produtos de Degradação da Fibrina e do Fibrinogênio/análise , Humanos , Hidroxiureia/uso terapêutico , Masculino , Fatores de Risco , Adulto Jovem
6.
Pediatr. aten. prim ; 22(85): e21-e25, ene.-mar. 2020. tab
Artigo em Espanhol | IBECS | ID: ibc-193440

RESUMO

La aparición de clínica neurológica en un cuadro respiratorio agudo, una vez descartadas hipoxia e hipercapnia secundarias a insuficiencia respiratoria grave, nos debe hacer pensar en una hiponatremia subyacente, formando parte de un síndrome de secreción inadecuada de hormona antidiurética (SIADH). Esta alteración hidroelectrolítica se ha considerado un factor de mal pronóstico en la evolución del proceso respiratorio, por lo que es deseable detectarla precozmente y corregirla. Presentamos el caso de un lactante que debutó con hipoactividad y pausas de apnea previas a la clínica respiratoria de una bronquiolitis


The appearance of neurological symptoms in an acute respiratory pattern, once hypoxia and hypercapnia are ruled out secondary to severe respiratory failure, should lead us to think about an underlying hyponatremia, forming part of a syndrome of inappropriate secretion of antidiuretic hormone (SIADH). This hydroelectrolytic alteration has been considered a factor of poor prognosis in the evolution of the respiratory process, so it is desirable to detect it early and correct it. We present the case of an infant who debuted with hypoactivity and apnea pauses prior to the respiratory symptoms of bronchiolitis


Assuntos
Humanos , Masculino , Lactente , Hiponatremia/etiologia , Síndrome Torácica Aguda/complicações , Bronquiolite/complicações , Síndrome de Secreção Inadequada de HAD/complicações , Coinfecção/complicações , Desequilíbrio Hidroeletrolítico/diagnóstico , Apneia/diagnóstico , Fator Natriurético Atrial/fisiologia , Índice de Gravidade de Doença , Fatores de Risco
8.
Pediatr Pulmonol ; 54(7): 993-1001, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-31012283

RESUMO

BACKGROUND: Airway involvement in patients with sickle cell disease (SCD) involves recurrent episodes of acute chest syndrome (ACS), co-existent asthma, lower airway obstruction (LAO), or airway hyper-responsiveness/ bronchodilator response (AHR/BDR). With increased recognition that sickle cell (SC) airway inflammation may be distinct from asthma, our aim was to study regional and individual practices among pediatric pulmonologists and elucidate the patient characteristics that determine the diagnosis of asthma or SC airway inflammation. METHODS: A cross-sectional web-based survey including 6 case scenarios on diagnosis and management of pulmonary manifestations of pediatric SC airway disease was conducted. The case scenarios, combined different risk factors for airway inflammation: history of recurrent ACS, atopy, family history of asthma, LAO, or AHR/BDR, with possible responses including - diagnosis of asthma, SC airway inflammation, both or neither. RESULTS: Of the 130 responses, 83 were complete. "Asthma" was diagnosed when LAO (OR, 7.96 [4.28, 14.79]; p < 0.001), family history of asthma (OR 18.88 [5.87, 60.7]; p < 0.001), and atopy (OR 3.19 [1.74, 5.8]; p < 0.001) were present. "SC airway inflammation" was diagnosed when ACS (OR 3.95 [2.08, 7.51]; p < 0.001), and restrictive pattern on PFT (OR 3.75 [2.3, 6.09]; p < 0.001) were present in the scenarios. Regardless of the diagnosis, there was a high likelihood of initiating or stepping up inhaled corticosteroid as compared to prescribing montelukast. CONCLUSION: There is variability in the diagnosis and management of SC airway inflammation among pediatric pulmonologists. This study highlights the need for consensus guidelines to improve management of SC airway inflammation.


Assuntos
Síndrome Torácica Aguda/terapia , Obstrução das Vias Respiratórias/terapia , Anemia Falciforme/terapia , Asma/terapia , Síndrome Torácica Aguda/complicações , Obstrução das Vias Respiratórias/complicações , Anemia Falciforme/complicações , Asma/complicações , Estudos Transversais , Humanos , Médicos , Inquéritos e Questionários
9.
PLoS One ; 13(3): e0192710, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29494636

RESUMO

Globally, the majority of persons born with sickle cell disease do not have access to hydroxyurea or more expensive interventions. The objectives were to estimate the survival in homozygous sickle cell disease, unbiased by symptomatic selection and to ascertain the causes of death in a pre-hydroxyurea population. The utility of early life biomarkers and genetically determined phenotypes to predict survival was assessed. A cohort study based on neonatal diagnosis was undertaken at the Sickle Cell Unit, a specialist clinic delivering care to persons with sickle cell disease in Jamaica. Screening of 100,000 deliveries detected 315 babies with homozygous sickle cell disease of whom 311 have been followed from birth for periods up to 43 years. Pneumococcal prophylaxis and teaching mothers splenic palpation were important, inexpensive interventions. Anticipatory guidance, routine care and out-patient acute care were provided. Each participant was classified as alive, dead, or defaulted (usually emigration). Causes of death were ascertained from clinical records and/or post-mortem reports. Survival was assessed using the Kaplan-Meier function. Sex-adjusted Cox semi-parametric proportional hazards and Weibull modelling were used to assess the effects on survival of biomarkers. Survival to 40 years was 55.5% (95% CI 48.7% to 61.7%). Acute Chest Syndrome (n = 31) and septicemia (n = 14) were significant causes of death at all ages. Acute splenic sequestration (n = 12) was the most common cause of early deaths. Survival was significantly shorter in those with lower hemoglobin at 1 year, high total nucleated count at 1 year, and a history of dactylitis ever. In these hydroxyurea naïve patients, survival into midlife was common. Causes of death were often age specific and some may be preventable. Early life biomarkers predictive of decreased survival in SS disease identify a patient group likely to benefit from close clinical supervision and potentially high risk therapies.


Assuntos
Anemia Falciforme/epidemiologia , Síndrome Torácica Aguda/complicações , Síndrome Torácica Aguda/epidemiologia , Adolescente , Adulto , Anemia Falciforme/sangue , Anemia Falciforme/complicações , Anemia Falciforme/genética , Causas de Morte , Criança , Pré-Escolar , Estudos de Coortes , Seguimentos , Homozigoto , Humanos , Lactente , Jamaica/epidemiologia , Sepse/complicações , Sepse/epidemiologia , Análise de Sobrevida , Adulto Jovem
11.
Cytokine ; 97: 104-107, 2017 09.
Artigo em Inglês | MEDLINE | ID: mdl-28609750

RESUMO

Platelet activation and platelet-derived cytokines contribute to the vascular inflammation and increased thrombotic activity known to occur in patients with sickle cell anemia (SCA). CD40 ligand (CD40L), a platelet-associated pro-inflammatory molecule that promotes endothelial cell activation, is elevated in the circulation of SCA patients. We sought to evaluate the association of CD40L and inflammation with sickle-related clinical complications and laboratory variables in SCA patients. Soluble CD40L, thrombospondin (TSP)-1 and tumor necrosis factor (TNF)-α were determined in the platelet-poor plasma of healthy individuals and steady-state SCA patients by ELISA. Lifetime clinical complications were verified by detailed review of patients' medical records. We found that plasma CD40L was associated with acute chest syndrome (ACS), and that SCA patients with a lifetime history of ACS (ACS+) presented significantly higher plasma CD40L and TSP-1 than patients who had never experienced ACS (ACS-). In the ACS+ group, both platelet-derived proteins (CD40L and TSP-1) correlated with mean corpuscular volume, mean corpuscular hemoglobin and reticulocyte hemoglobin, while in the ACS- group, CD40L correlated with low red blood cell counts, hemoglobin, hematocrit and lactate dehydrogenase, and TSP-1 correlated with reticulocyte percentage and white blood cell count. As expected, CD40L and TSP-1 correlated with platelet counts in both groups. These data highlight the possible role of platelet activation in ACS and suggest that plasma sCD40L, together with TSP-1, may represent a potential marker of susceptibility to ACS in SCA.


Assuntos
Síndrome Torácica Aguda/sangue , Síndrome Torácica Aguda/complicações , Anemia Falciforme/sangue , Ligante de CD40/sangue , Síndrome Torácica Aguda/fisiopatologia , Adolescente , Adulto , Anemia Falciforme/complicações , Biomarcadores/sangue , Feminino , Humanos , Inflamação , Masculino , Registros Médicos , Pessoa de Meia-Idade , Ativação Plaquetária , Contagem de Plaquetas , Trombospondina 1/sangue , Fator de Necrose Tumoral alfa/sangue , Adulto Jovem
12.
Rev. cuba. obstet. ginecol ; 42(2)abr.-jun. 2016. ilus, graf
Artigo em Espanhol | CUMED | ID: cum-64319

RESUMO

La anemia de células falciformes o drepanocitosis, es una de las hemoglobinopatías estructurales más comunes en el mundo. La clínica se resume en oclusión vascular e isquemia tisular, anemia hemolítica y la susceptibilidad a infecciones. La procreación en mujeres con hemoglobinopatías deviene un grave problema de salud, que exige una atención diferenciada y multidisciplinaria. Para esta afección no existe tratamiento especifico definitivo, el arsenal medico existente solo puede manejar los efectos y no la causa. La siguiente revisión tiene como objetivo ofrecer a los profesionales algunos aspectos relacionados con la fisiopatología, una discusión del problema clínico, diagnóstico y opciones terapéuticas de la enfermedad, lo que permite contribuir en la reducción de la morbilidad y mortalidad materna y perinatal. Se concluye que un alto índice de perspicacia y buen diagnóstico es menester para obtener resultados óptimos en las embarazadas afectadas por enfermedad de células falciformes(AU)


Sickle cell anemia or sickle cell disease is one of the most common structural hemoglobinopathies in the world. The clinic is summarized in vascular occlusion and tissue ischemia, hemolytic anemia and vulnerability to infections. Procreation in women with hemoglobinopathies becomes a serious health problem that requires a differentiated and multidisciplinary care. There is no definitive specific treatment for this condition, the existing medical resources can only address the effects and not the cause. The following review aims to offer professionals some aspects related to the pathophysiology, a discussion of the clinical problem, diagnosis and treatment options, which can contribute in reducing morbidity and maternal and perinatal mortality. It is concluded that high level of insight and good diagnosis are necessary for optimum results in pregnant women affected by sickle cell disease(AU)


Assuntos
Humanos , Anemia Falciforme/sangue , Anemia Falciforme/diagnóstico , Anemia Falciforme/complicações , Síndrome Torácica Aguda/complicações , Pré-Eclâmpsia , Convulsões/complicações , Tromboflebite/complicações , Sepse/complicações
13.
Pediatr Nephrol ; 31(8): 1363-8, 2016 08.
Artigo em Inglês | MEDLINE | ID: mdl-27011218

RESUMO

BACKGROUND: Patients with sickle cell disease are at risk for developing chronic kidney disease (CKD). Acute kidney injury (AKI) has been linked to progression to CKD, but limited data exist to determine its role in acute complications of sickle cell disease. We hypothesized that AKI occurs in pediatric patients admitted for acute chest syndrome (ACS) and prolongs hospitalization. METHODS: We conducted a 6-year retrospective review of pediatric patients with ACS admitted to a single medical institution. RESULTS: Of the 149 pediatric patients admitted for ACS during the 6-year study period, 12 (8 %) developed AKI. Comparison of patients with and without AKI revealed a significant association between AKI and a larger drop in hemoglobin value from baseline (2.7 vs. 1.4 g/dL; p = 0.003), a lower hemoglobin value at admission (6.4 vs. 7.5 g/dL; p = 0.03), and an increased white blood cell count at admission (33.1 vs. 19.8 × 10(9)/L; p < 0.0001), respectively. AKI (p < 0.0001) together with need for advanced respiratory support (biphasic positive airway pressure or mechanical ventilation) (p < 0.0001) and need for exchange transfusion (p < 0.0001) were associated with prolonged hospitalization. CONCLUSIONS: Clinicians should monitor pediatric patients hospitalized for ACS for the development of AKI as a potentially modifiable risk factor for prolonged hospitalization.


Assuntos
Síndrome Torácica Aguda/complicações , Lesão Renal Aguda/epidemiologia , Lesão Renal Aguda/etiologia , Anemia Falciforme/complicações , Criança , Feminino , Humanos , Tempo de Internação , Masculino , Prevalência , Estudos Retrospectivos
14.
Hematology ; 21(4): 248-256, 2016 May.
Artigo em Inglês | MEDLINE | ID: mdl-26868490

RESUMO

OBJECTIVES: Tanzania has the third highest birth rate of sickle cell anaemia (SCA) in Africa, but few studies describe severity of complications or available treatments, especially in Northwest Tanzania around Lake Victoria where the sickle gene is most prevalent. This is a report of the spectrum of clinical disease and range of interventions available at Bugando Medical Centre (Bugando) in Northwest Tanzania in Africa. METHODS: A cross-sectional study was carried out in Bugando between 1 August 2012 and 30 September 2012. Children (<15 years old) with SCA attending Bugando were sequentially enrolled. A trained research assistant completed a Swahili questionnaire with the parent or guardian of each participant concerning demographic information, clinical features of disease, and treatments received. RESULTS: Among the 124 participants enrolled, the median age was 6 years (interquartile range [IQR] 4-8.5), and only 13 (10.5%) were < 3 years old. Almost all participants (97.6%) had a prior history of a vaso-occlusive episode, 83 (66.9%) had prior acute chest syndrome, and 21 (16.9%) had prior stroke. In the preceding 12 months, 120 (96.8%) had been hospitalized, and a vaso-occlusive episode was the most common reason for hospitalization (35.5%). Prescriptions for folic acid (92.7%) and malaria prophylaxis (84.7%) were common, but only one had received a pneumococcal vaccine, and none had received hydroxyurea or prophylactic penicillin. CONCLUSION: Children with SCA receiving care in Tanzania are diagnosed late, hospitalized frequently, and have severe complications. Opportunities exist to improve care through wider access to screening and diagnosis as well as better coordination of comprehensive care.


Assuntos
Síndrome Torácica Aguda , Ácido Fólico/administração & dosagem , Hospitalização , Acidente Vascular Cerebral , Doenças Vasculares , Síndrome Torácica Aguda/complicações , Síndrome Torácica Aguda/epidemiologia , Síndrome Torácica Aguda/terapia , Criança , Pré-Escolar , Feminino , Humanos , Malária/complicações , Malária/epidemiologia , Malária/prevenção & controle , Masculino , Vacinas Pneumocócicas/administração & dosagem , Acidente Vascular Cerebral/epidemiologia , Acidente Vascular Cerebral/etiologia , Acidente Vascular Cerebral/terapia , Tanzânia/epidemiologia , Doenças Vasculares/epidemiologia , Doenças Vasculares/etiologia , Doenças Vasculares/terapia
15.
Transfusion ; 55(10): 2331-8, 2015 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-26126756

RESUMO

BACKGROUND: Sickle cell disease (SCD) patients hospitalized with vasoocclusive pain crisis tend to have prolonged length of stay (LOS) and high 30-day readmission rates. We investigated the associations of demographic characteristics, comorbidities, and blood transfusion during hospitalization with these outcomes. STUDY DESIGN AND METHODS: Repeated regression analysis was used to analyze 39,324 admissions of 4348 adults with sickle cell crisis from 2007 to 2012 in the Truven Health MarketScan Medicaid Databases. RESULTS: The mean (95% range) LOS was 5.9 (1.0-19.0) days and the 30-day readmission rate was 39.6% (95% confidence interval [CI], 39.1%-40.0%). Older age; chronic cardiopulmonary, renal, or liver disease; and sepsis were associated with both longer LOS and greater 30-day readmission rate. Female sex, iron overload, acute chest syndrome, acute renal failure, and stroke were additional predictors of longer LOS. Simple red blood cell transfusion was administered in 31.8% of the admissions, and these patients tended to have more severe disease (chronic cardiopulmonary or kidney disease, acute chest syndrome, acute kidney or liver failure, sepsis). Nevertheless, transfusion was associated with a reduced estimated odds ratio (OR) of inpatient mortality of 0.75 (95% CI, 0.57-0.99) and a decreased OR of 30-day readmission of 0.78 (95% CI, 0.73-0.83). CONCLUSION: Our findings point to blood transfusion as a potential means to reduce the 30-day readmission rate among Medicaid patients hospitalized with sickle cell crisis. There is a need for a prospective study to examine the potential benefit and safety of simple blood transfusion for this purpose.


Assuntos
Síndrome Torácica Aguda , Transfusão de Eritrócitos , Tempo de Internação , Readmissão do Paciente , Síndrome Torácica Aguda/complicações , Síndrome Torácica Aguda/epidemiologia , Síndrome Torácica Aguda/terapia , Lesão Renal Aguda/epidemiologia , Lesão Renal Aguda/etiologia , Lesão Renal Aguda/patologia , Adulto , Feminino , Cardiopatias/complicações , Cardiopatias/epidemiologia , Cardiopatias/terapia , Humanos , Sobrecarga de Ferro/epidemiologia , Sobrecarga de Ferro/etiologia , Sobrecarga de Ferro/terapia , Hepatopatias/complicações , Hepatopatias/epidemiologia , Hepatopatias/terapia , Masculino , Dor/epidemiologia , Dor/etiologia , Fatores de Risco , Fatores Sexuais , Acidente Vascular Cerebral/epidemiologia , Acidente Vascular Cerebral/etiologia , Acidente Vascular Cerebral/terapia
17.
Pediatr Blood Cancer ; 62(4): 643-7, 2015 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-25641631

RESUMO

BACKGROUND: Vitamin D is increasingly recognized for its roles in non-skeletal disorders. Patients with sickle cell disease (SCD) have a high prevalence of vitamin D deficiency but data are limited with respect to possible associations between low vitamin D and acute vaso-occlusive complications. We examined whether vitamin D deficiency is associated with acute pain and acute chest syndrome (ACS) in children with SCD. PROCEDURE: A cross-sectional study was conducted in 95 children with SCD who had serum 25-hydroxyvitamin D (25-OHD) measured during comprehensive care examinations. History of acute pain and ACS within two years of obtaining 25-OHD was collected. Associations between 25-OHD levels and acute vaso-occlusive events were analyzed by logistic regression. Odds ratios and 95% confidence intervals were calculated for the risk of pain and ACS associated with vitamin D deficiency (25-OHD <20 ng/ml). RESULTS: Subjects were 3-20 years old (median 10.6); 48 males, 47 females; 46 African, 49 Hispanic; 72 SS, 20 SC, 1 S/ß(0) Thalassemia, and 2 S/ß(+) Thalassemia. Median 25-OHD was 16 ng/ml. Fifty-six (59%) were vitamin D-deficient. Thirty-one (33%) and 29 (31%) had at least one episode of pain and ACS, respectively. Serum 25-OHD was significantly associated with pain (P = 0.0121) but not with ACS (P = 0.628). Of those with pain, 73% (23/31) were vitamin D-deficient while 26% (8/31) had 25-OHD ≥20 ng/ml (P = 0.04, OR = 2.7, 95%CI = 1.05-6.94). CONCLUSIONS: Our findings emphasize the high prevalence of vitamin D deficiency and its potential association with acute pain in SCD. Correcting low vitamin D may offer a simple, low-cost intervention to help reduce acute vaso-occlusive complications.


Assuntos
Síndrome Torácica Aguda , Doenças Vasculares , Deficiência de Vitamina D , Vitamina D/análogos & derivados , Síndrome Torácica Aguda/sangue , Síndrome Torácica Aguda/complicações , Síndrome Torácica Aguda/epidemiologia , Doença Aguda , Adolescente , Adulto , Criança , Pré-Escolar , Estudos Transversais , Feminino , Humanos , Masculino , Dor/sangue , Dor/epidemiologia , Dor/etiologia , Prevalência , Doenças Vasculares/sangue , Doenças Vasculares/epidemiologia , Doenças Vasculares/etiologia , Vitamina D/sangue , Deficiência de Vitamina D/sangue , Deficiência de Vitamina D/epidemiologia , Deficiência de Vitamina D/etiologia
18.
Am J Hematol ; 90(5): 381-5, 2015 05.
Artigo em Inglês | MEDLINE | ID: mdl-25616042

RESUMO

Intravenous immunoglobulin (IVIG) decreases neutrophil adhesion to endothelium and red blood cell-neutrophil interactions in sickle cell mice undergoing vaso-occlusion. In this Phase I clinical trial of sickle cell anemia (SCA) patients admitted with pain crisis, we evaluated the status of adhesion molecules on neutrophils in control and IVIG-treated subjects pre- and post-infusion up to 800 mg/kg, the same dose used in murine studies. Mac-1 function significantly decreased from baseline in the low-dose IVIG (200-400 mg/kg) cohorts. IVIG-related adverse events may have occurred in the high-dose (600-800 mg/kg) cohorts. There were no significant increases in neutrophil and leukocyte counts, suggesting that IVIG may more selectively inhibit Mac-1 function as opposed to neutrophil adhesion. This study provides the first in-human validation of pre-clinical murine studies that IVIG can decrease Mac-1 function.


Assuntos
Síndrome Torácica Aguda/tratamento farmacológico , Anemia Falciforme/tratamento farmacológico , Imunoglobulinas Intravenosas/uso terapêutico , Antígeno de Macrófago 1/sangue , Neutrófilos/efeitos dos fármacos , Dor/tratamento farmacológico , Síndrome Torácica Aguda/sangue , Síndrome Torácica Aguda/complicações , Síndrome Torácica Aguda/fisiopatologia , Adolescente , Adulto , Anemia Falciforme/sangue , Anemia Falciforme/complicações , Anemia Falciforme/fisiopatologia , Adesão Celular/efeitos dos fármacos , Criança , Esquema de Medicação , Feminino , Humanos , Masculino , Ativação de Neutrófilo/efeitos dos fármacos , Neutrófilos/metabolismo , Neutrófilos/patologia , Dor/sangue , Dor/complicações , Dor/fisiopatologia
19.
Am J Hematol ; 90(5): 371-5, 2015 May.
Artigo em Inglês | MEDLINE | ID: mdl-25619382

RESUMO

Previous studies have shown that the highest incidence of acute chest syndrome (ACS) in sickle cell disease occurs in children <4 years old, and a history of ACS at this age is a risk factor for future ACS episodes. However, the interval associated with the highest risk of subsequent ACS or severe pain is not known. Through this mixed retrospective-prospective observational study, the Sleep and Asthma Cohort, we sought to determine the interval after an initial ACS episode during which the majority of children <4 years old are rehospitalized for ACS or severe pain. The cumulative prevalence of rehospitalization for ACS or severe pain within 6 months, 1 years, and 2 years was calculated for children with an initial ACS episode <4 years old and compared to children with an initial ACS episode ≥4 years old. A total of 44.8% and 55.2% of participants had an initial ACS episode <4 years and ≥4 years old (Range: 4-17.7 years), respectively. At 1 year following the initial ACS episode, children <4 years old had a significantly higher cumulative prevalence of rehospitalizations for ACS or pain as compared to children ≥4 years of age, 62.5 and 39.1%, respectively (P = 0.009). After initial ACS episodes, the majority of children <4 years old will be rehospitalized for ACS or severe pain within one year, suggesting the need for a therapeutic intervention for this high-risk group.


Assuntos
Síndrome Torácica Aguda/diagnóstico , Anemia Falciforme/diagnóstico , Asma/diagnóstico , Dor/diagnóstico , Síndrome Torácica Aguda/complicações , Síndrome Torácica Aguda/fisiopatologia , Adolescente , Anemia Falciforme/complicações , Anemia Falciforme/fisiopatologia , Asma/complicações , Asma/fisiopatologia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Dor/complicações , Dor/fisiopatologia , Readmissão do Paciente/estatística & dados numéricos , Estudos Prospectivos , Estudos Retrospectivos , Fatores de Risco , Sono
20.
Ann Fr Anesth Reanim ; 33(12): 700-3, 2014 Dec.
Artigo em Francês | MEDLINE | ID: mdl-25458459

RESUMO

We report the case of an 8-year-old sickle cell anemia child admitted for acute respiratory failure complicating acute chest syndrome. Because of threatening respiratory failure, tracheal intubation was performed immediately after ICU admission. The patient met the criteria for ARDS with a PaO2/FiO2 ratio of 94mmHg. An exchange transfusion was performed immediately after admission. HbS fraction failed from 69 % to 30 %. Fluid resuscitation with crystalloids and continuous norepinephrine infusion was needed because of arterial hypotension. Due to persistent severe hypoxemia with PaO2/FiO2 ratio below 100, the patient was placed in prone positioning 16hours after admission, for a total duration of 14hours. A second 12-hour session of prone positioning was performed 41h after admission and PaO2/FiO2 ratio reached 300mmHg after. Treatment also included transfusion of two red-cell pack on day 1 and 2 after admission in order to maintain hemoglobin level above 8g/dL, and a daily folic acid supplementation. The control of hyperthermia was achieved by a systematic parenteral administration of paracetamol. Cefotaxime and erythromycine were continued until day 7 despite the negative results of all bacteriological samples. The outcome was favorable from day 3 and the patient met the criteria for extubation on day 5. A first attempt of extubation was performed on day 5, but re-intubation was required because of laryngeal edema. Steroids were given for 48h and the patient was successfully extubated on day 7. She was discharged from the ICU on day 8, and from the hospital on day 12. We discuss the various treatments available for the management of acute chest syndrome and their actual relevance in acute respiratory distress syndrome in the absence of strong evidence-based guidelines in pediatric ARDS.


Assuntos
Síndrome Torácica Aguda/complicações , Síndrome Torácica Aguda/terapia , Transfusão Total , Decúbito Ventral , /terapia , Anemia Falciforme/complicações , Anemia Falciforme/terapia , Criança , Feminino , Hidratação , Hemoglobinas/análise , Humanos , Intubação Intratraqueal , Respiração Artificial
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