Sickle cell disease and infection

Nuzzo, Dayana V. P. Di; Fonseca, Silvana Fahel da

doi:10.2223/jped.1218

Cited by 19 publications

(17 citation statements)

References 21 publications

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“…Os aspectos clássicos do tratamento das DF, incluindo a terapêutica dos episódios agudos (crises de falcização) e das infecções, estão extensivamente descritos em revisões anteriores 3,5,11,22 . Merece ser ressaltada, no entanto, a importân-cia do uso profilático de penicilina e da vacinação contra pneumococos e Haemophilus influenzae tipo b, microrganismos encapsulados contra os quais as crianças com DF são muito mais susceptíveis que crianças saudáveis 11,22 .…”

Section: Tratamentounclassified

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The genetics of blood disorders: the hereditary hemoglobinopathies

Sonati¹,

Costa²

2008

J Pediatr (Rio J)

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ResumoObjetivo: Sumarizar os dados disponíveis na literatura recente sobre os aspectos fisiopatológicos, de diagnóstico e tratamento das doenças falciformes e da talassemia β, hemoglobinopatias hereditárias de maior relevância nas populações. Fontes dos dados AbstractObjective: To summarize recently published data on the pathophysiology, diagnosis and treatment of sickle cell diseases and β-Thalassemias, the most relevant hereditary hemoglobinopathies in the global population.Sources: Searches were run on the MEDLINE and SCIELO databases, limited to the period from 2003 to May 2008, using the terms hereditary hemoglobinopathies, sickle cell diseases and β-thalassemia. Two books and two chapters were also included. Summary of the findings:More than 2,000 articles were identified; those providing the most important information and broadest views were selected.Conclusions: Morbidity and mortality rates from sickle cell diseases and β-thalassemia are still very high and represent an important challenge. Increased understanding of pathophysiological aspects has lead to significant improvements in treatment and prevention of these diseases.

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Section: Tratamentounclassified

“…Merece ser ressaltada, no entanto, a importân-cia do uso profilático de penicilina e da vacinação contra pneumococos e Haemophilus influenzae tipo b, microrganismos encapsulados contra os quais as crianças com DF são muito mais susceptíveis que crianças saudáveis 11,22 . .…”

Section: Tratamentounclassified

The genetics of blood disorders: the hereditary hemoglobinopathies

Sonati¹,

Costa²

2008

J Pediatr (Rio J)

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“…In Gabon, SCD is a major public health problem, with 24% of the population carriers of hemoglobin S (HbS) and a 2% -3% level of SS (HbSS) homozygous [5] [6]. Infectious complications are responsible for high rates of morbidity and mortality in SCD patients [4] [7]- [12]. In developed countries, the initiation of effective preventive measures (newborn screening, oral penicillin prophylaxis, pneumococcal and Haemophilus type b immunization) has resulted in the dramatic reduction in these rates [7] [12] [13].…”

Section: Introductionmentioning

confidence: 99%

Fever among Children with Sickle-Cell Disease: Findings from the General Pediatric Ward of the Owendo Pediatric Hospital in Libreville, Gabon

Koko¹,

Gahouma²,

Ategbo³

et al. 2014

OJPed

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Sickle-cell disease (SCD) represents a substantial public health problem in Gabon. Fever is one of the principal reasons for the hospitalization of children afflicted by major sickle-cell disorder, since it can be a clinical reflection of severe infections that have the potential to become life threatening. Objectives: Identification of the main causes of fever in children with SCD in our clinical setting, with the aim of optimizing treatments. Patients and Methods: This is a retrospective study of all the medical files for children with SCD that were admitted to our ward, over a two year period, due to fever (>38.5˚C) lasting more than 24 hours. Only those files that contained at least the following five fundamental medical examinations were retained for further evaluation: Complete Blood Count (CBC), blood smear, blood culture, urine culture and chest X-ray. Out of a total of 118 admissions (103 patients), 87 (73.7%) were due to the incidence of fever. The medical files of 11 patients were deemed to be unusable. Seventy-six episodes of fever were observed among 69 children, of which 42 were male and 27 female (sex ratio of 1.5). Among these, seven (10%) were admitted twice. Results: The age groups that were most affected included 12 -18 year-olds (30 cases: 43.5%) and 6 -12 year-olds (26 cases: 37.7%). The most common accompanying symptoms were bone and joint pain (43.4%), asthenia (22.4%), cough (19.7%), vomiting (17%) and headache (15.8%). The specific cause of the fever could not be pinpointed in 29 cases (38.1%). Aside from these cases, the main causes of fever were malaria (30.3%) and bronchopulmonary infections (22.4%). The white blood cell count was >20,000/mm 3 in 47% of respiratory infections, 43.5% of the cases involving malaria and 55.2% of cases of fever with unknown cause. Hemoglobin levels * Corresponding author. J. Koko et al. 263were <5g/dl for 52.2% of the cases involving malaria and 22.6% for those of unknown origin. For four patients, all less than 10 years of age, the disease was fatal. Conclusion: For the majority of fever episodes, the underlying cause could not be determined. Nonetheless, malaria was identified as one of the principal identifiable causes of fever among children with SDC in Libreville. Treatment for malaria upon admission, and the promotion of preventative measures, therefore seems to be appropriate for our clinical setting. In light of the large number of unresolved cases, systematic prescription of broad-spectrum antibiotics may also be called for.

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“…5,14 The clinical complications of SCA include chronic hemolytic anemia, of moderate or severe intensity, painful and intermittent episodes of vasoocclusion, permanent risk of infections as a result of autosplenectomy, acute chest syndrome, cerebral vascular accidents (CVA), priapism, retinopathy and cumulative damage to multiple organs. 5,[22][23][24][25][26][27][28][29][30] Pulmonary hypertension has also been recognized as a serious complication particularly in adults. [31][32][33][34][35][36] Inflammation, endothelial activation, abnormalities of the erythrocyte membrane, leukocyte adhesion, platelet aggregation and activation, coagulation activation and abnormal bioavailability of several …”

Section: Hereditary Hemoglobinopathiesmentioning

confidence: 99%

“…3,5,11,22 It is worth emphasizing, however, the importance of the prophylactic use of penicillin and vaccination against pneumococcus and Haemophilus influenzae type b, both of which are encapsulated microorganisms to which children with SCD are much more susceptible than healthy children. 11,22 The introduction of this treatment dramatically reduced the infant morbidity and mortality of these patients. We will now deal with the drugs used to treat SCD and with new prospects for treatment.…”

Section: Treatmentmentioning

confidence: 99%

Genética das doenças hematológicas: as hemoglobinopatias hereditárias

Sonati¹,

Costa²

2008

J. Pediatr. (Rio J.)

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Objective: To summarize recently published data on the pathophysiology, diagnosis and treatment of sickle cell diseases and β-Thalassemias, the most relevant hereditary hemoglobinopathies in the global population.Sources: Searches were run on the MEDLINE and SCIELO databases, limited to the period from 2003 to May 2008, using the terms hereditary hemoglobinopathies, sickle cell diseases and β-thalassemia. Two books and two chapters were also included. Summary of the findings:More than 2,000 articles were identified; those providing the most important information and broadest views were selected.Conclusions: Morbidity and mortality rates from sickle cell diseases and β-thalassemia are still very high and represent an important challenge. Increased understanding of pathophysiological aspects has lead to significant improvements in treatment and prevention of these diseases. J Pediatr (Rio J). 2008;84(4 Suppl):S40-51:Hereditary hemoglobinopathies, sickle cell diseases, β-thalassemia.

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Sickle cell disease and infection

Abstract: This review hopes to provide the pediatric community with information concerning the association between sickle cell disease and infections, so as to minimize the occurrence of complications.

Cited by 19 publications

References 21 publications

The genetics of blood disorders: the hereditary hemoglobinopathies

The genetics of blood disorders: the hereditary hemoglobinopathies

Fever among Children with Sickle-Cell Disease: Findings from the General Pediatric Ward of the Owendo Pediatric Hospital in Libreville, Gabon

Genética das doenças hematológicas: as hemoglobinopatias hereditárias

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