Top PDF Risk Factors of Pulmonary Hypertension in Brazilian Patients with Sickle Cell Anemia.

Risk Factors of Pulmonary Hypertension in Brazilian Patients with Sickle Cell Anemia.

Risk Factors of Pulmonary Hypertension in Brazilian Patients with Sickle Cell Anemia.

This study was a prospective cross-sectional cohort study of 125 patients with sickle cell anemia (SS) between the ages of 16 to 60 years. Enrolled patients were followed-up pro- spectively for 15 months. Demographic, clinical, hematological and routine biochemical data were obtained on all patients. Six-minute walk test and Doppler Echocardiography were performed on all patients. A tricuspid regurgitant jet velocity (TRJV) < 2.5 m/sec was considered normal, 2.5  TRJV  3.0 was considered mild-moderate and > 3.0 m/sec, severe. Patients with abnormal TRJV were significantly older and more anemic, had signifi- cantly higher lactate dehydrogenase (LDH) levels, reticulocyte count and incidence of death. The logistic multimodal model implemented for the 125 patients indicated that age was the covariate that influenced the outcome of normal or abnormal TRJV with a cutoff age of thirty-two years. The survival rate for the group of patients with creatinine (Cr) > 1.0 mg/dL was lower than the group with Cr  1 and normal TRJV. A coefficient matrix showed that the LDH values were weakly correlated with the reticulocyte count but strongly corre- lated with hemoglobin suggesting that the TRJV values were not correlated with the hemo- lytic rate but with anemia. Ten patients died during the follow-up of whom 7 had TRJV > 2.5 m/sec. Acute chest syndrome was the most common cause of death followed by sepsis. In conclusion, this study shows that patients with SS older than thirty-two years with high LDH, elevated TRJV, severe anemia and Cr > 1 have poor prognosis and may be at risk of having pulmonary hypertension and should undergo RHC.
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Rev. Bras. Hematol. Hemoter.  vol.31 número4

Rev. Bras. Hematol. Hemoter. vol.31 número4

Sickle cell disease is an inflammatory condition with a pathophysiology that involves vaso-occlusive episodes. Mutations of the methylenetetrahydrofolate reductase (MTHFR) and cystathionine beta-synthase (CBS) genes are risk factors for vascular disease. Due to the importance of identifying risk factors for vaso-occlusive events in sickle cell patients, we investigated the frequencies of the C677T and 844ins68 mutations of the MTHFR and CBS genes, respectively. Three hundred patients with Hb SS, HB SC and HbS/Beta thalassemia, from Brasília, Goiânia, Rio de Janeiro, São Jose do Rio Preto and São Paulo were evaluated. Samples of 5 mL of venous blood were collected in EDTA after informed consent was received from patients. Classical diagnostic methods were used to confirm the hemoglobin phenotypes. The hemoglobin genotypes and polymorphisms studied were evaluated by Restriction Fragment Length Polymorphism and Allele Specific amplification. The results showed that 93 patients (31.00%) were heterozygous and 13 (4.33%) homozygous for the C677T mutation and 90 were heterozygotes (30.00%) and 8 homozygous (2.66%) for the 844ins68 mutation, both with significant differences for genotype frequency between the localities. The allelic frequencies are in Hardy-Weinberg equilibrium for both polymorphisms. The frequency of mutations was significant and the presence of related vaso-occlusive events was more common in patients with Hb SS
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Interleukin 8 as a vaso-occlusive marker in Brazilian patients with sickle cell disease

Interleukin 8 as a vaso-occlusive marker in Brazilian patients with sickle cell disease

Despite all the controversial reports which reported different levels of the so-called modulating factors in sickle cell disease syn- dromes, our results demonstrate a signifi- cant change in IL-8 levels which was empha- sized by the correlation of nonsymptomatic sickle cell patients and healthy controls. On the basis of our results, we conclude that the increased IL-8 levels could be considered as a marker of VOC risk in our population and that the finding of some VOC patients with low IL-8 levels may be related to specific crisis-inducing factors in association with the individual genetic background of each patient, since some genetic inheritance has been associated with the pathogenetic course of the disease (35). Further studies should be conducted in order to determine the exact point before VOC, when serum IL-8 levels begin to increase. This requires the monitor- ing of the IL-8 levels in sickle cell patients out of crisis, showing how rapidly these levels increase just before VOC develop- ment. The ELISA for IL-8 measurement can be performed quickly and can be included in the standard follow-up protocols for these patients.
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Genetic, laboratory and clinical risk factors in the development of overt ischemic stroke in children with sickle cell disease

Genetic, laboratory and clinical risk factors in the development of overt ischemic stroke in children with sickle cell disease

Cerebrovascular disease, particularly stroke, is one of the most severe clinical complica- tions associated with sickle cell disease and is a significant cause of morbidity in both children and adults. Over the past two decades, considerable advances have been made in the understanding of its natural history and enabled early identification and treatment of children at the highest risk. Transcranial Doppler screening and regular blood transfu- sions have markedly reduced the risk of stroke in children. However, transcranial Doppler has a limited positive predictive value and the pathophysiology of cerebrovascular disease is not completely understood. In this review, we will focus on the current state of knowl- edge about risk factors associated with ischemic stroke in patients with sickle cell disease. A search of PubMed was performed to identify studies. Full texts of the included articles were reviewed and data were summarized in a table. The coinheritance of alpha-thalassemia plays a protective role against ischemic stroke. The influence of other genetic risk factors is controversial, still preliminary, and requires confirmatory studies. Recent advances have established the reticulocyte count as the most important laboratory risk factor. Clinical features associated with acute hypoxemia as well as silent infarcts seem to influence the development of strokes in children. However, transcranial Doppler remains the only avail- able clinical prognostic tool to have been validated. If our understanding of the many risk factors associated with stroke advances further, it may be possible to develop useful tools to detect patients at the highest risk early, improving the selection of children requiring intensification therapy.
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Rev. Bras. Hematol. Hemoter.  vol.25 número2

Rev. Bras. Hematol. Hemoter. vol.25 número2

Sickle cell nephropathy is now a well- characterized entity with specific manifestations, risk factors, and prognoses. Clinical renal abnormalities are common in sickle cell anemia and include hyposthenuria, incomplete renal tubular acidosis, hyperkalemia, hematuria and papillary necrosis. Hyposthenuaria is the first clinical symptom of the defective medullar tonicity and can produce a higher than usual obligatory urine output, thereby increasing the risk of dehydration. Proteinuria is a frequent finding in sickle cell disease sometimes with nephrotic syndrome. 8 Focal segmental sclerosis, 9
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Analysis of oral health conditions and risk factors for dental caries in patients with sickle cell disease

Analysis of oral health conditions and risk factors for dental caries in patients with sickle cell disease

buffers, mucin, bicarbonate and monophosphate and perform mouth autoclisis through masticatory movements [18]. Its buffering capacity is an important resistance factor in avoiding dental caries. A reduction in salivary flow, which is usually associated with a low buffer capacity, may provoke the development of oral mucosal infections [18]. Due to the importance of saliva in relation to caries prevention, routine exams should include salivary tests in order to evaluate patients for the risk of developing the disease [18]. Since demineralization and remineralization events can cause caries disease, detecting caries as early as possible is crucial for pursuing the early stages of the disease, which based on the biological model, may allow real epidemiological data regarding the presence of incipient and active / inactive lesions. Thus it is essential to research preventive oral disease issues and improve the life quality of sickle cell disease patients. Prevention is very important in reducing the need for radical treatment and the elimination of oral infection sources. Morever acute infection treatment should be mandatory since an infection may trigger the sickle cell crisis [1].
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Repercussões respiratórias da anemia falciforme

Repercussões respiratórias da anemia falciforme

The technological advances made in recent decades have allowed the noninvasive measurement of peripheral oxygen saturation (SpO 2 ). Oxygen saturation is currently considered the fifth vital sign and has been universally used in inten- sive care units, operating rooms, emergency rooms, and outpatient clinics. Knowing the SpO 2 level is fundamental to clinical decision-making by pulmonologists, informing decisions such as whether to use oxygen therapy or therapy with positive airway pressure. However, the physician should always be alert to situations in which this measurement could be spurious. The article by de Souza and Viegas on sleep and pulmonary function in sickle cell anemia, in this issue of the Brazilian Journal of Pulmonology, is an example of how the interpretation of SpO 2 , in special situations, can be confusing. (4)
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PDF EN Jornal Brasileiro de Pneumologia 3 2 english

PDF EN Jornal Brasileiro de Pneumologia 3 2 english

Children and adolescents frequently present respira- tory diseases of the upper and lower airways, which can lead to decreased oxygen availability. Adenotonsillar hypertrophy frequently leads to obstructive sleep apnea- hypopnea syndrome (OSAHS), characterized by intermittent obstruction of the upper airway and nocturnal hypoxemia. Cerebrovascular accidents are more common in sickle cell anemia patients between the ages of 5 and 7, a period that coincides with greater adenotonsillar growth. These children present a higher risk of having OSAHS, due to adenoton- sillar compensatory growth after autosplenectomy caused by an increased reactive response to multiple infections with encapsulated germs and by increased hematopoietic needs resulting from hemolytic anemia. Obstructive sleep apnea in children has been associated with a higher frequency of morning vaso-occlusive crises, which become less frequent after adenotonsillectomy. (1) Since obstructive sleep apnea
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Fatores de risco para aloimunização em pacientes com anemia falciforme

Fatores de risco para aloimunização em pacientes com anemia falciforme

Objective: To determine erythrocyte phenotyping in blood donors and patients with sickle cell anemia (SS) treated at Hemocentro of Alagoas and describe the frequency and factors associated with erythrocyte alloimmunization. Methods: Cross-sectional study with 102 SS patients and 100 blood donors. he following tests were performed: erythro- cyte phenotyping, Direct and Indirect antiglobulin test, and detection of irregular anti- bodies by panel of phenotyped red blood cells. Data were compared by Mann-Whitney, qui-square or Fisher’s exact tests. Factors associated with alloimmunization were studied by univariate and multiple logistic regression analysis. Results: he most frequent anti- gens found in patients and blood donors were: c, e, M, s, JK(a). Signiicant diferences were observed between the frequency of the phenotype of patients and donors in regard to antigens s, FY(a) and JK(b). Of 79 transfused patients, 10 presented positive Indirect Coombs. hirteen alloantibodies were found, 7 of the Rh system, 2 of Kell and 4 were not identiied. Factors associated with alloimmunization were the period of time between the last transfusion and the date of the test and more than 10 red blood cell transfu- sions. Patients who received more than 10 transfusions were 16.39 (95% CI: 2.23-120.59) times more likely to be alloimmunized than patients with fewer transfusions. Conclu- sion: he prevalence of alloimmunization in SS patients was 12.7%, with 70% of antibod- ies belonging to the Rh and Kell systems. his study shows the importance of performing erythrocyte phenotyping in blood donors and receptors to decrease the risk of alloim- munization.
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Prevalence and Risk Factor of Pulmonary Hypertension in Patients with Thalassemia Major of Ilam/ West of Iran in 2014

Prevalence and Risk Factor of Pulmonary Hypertension in Patients with Thalassemia Major of Ilam/ West of Iran in 2014

Pulmonary arterial hypertension (PAH) is a progressive disease with high morbidity and mortality rates. Research has shown that PAH has a prevalence rate of 10-79% in thalassemia major patients. This cross-sectional study was carried out in 2014 to determine the prevalence and risk factors of PAH in all thalassemia major patients of over 18 years of age in Ilam, Iran. A cardiologist measured systolic pulmonary artery pressure (SPAP) by using Doppler echocardiography. SPAP of higher than 25 mm Hg was defined as PAH criterion. The obtained data was analyzed using SPSS, version 17. Of the 36 studied patients, 17 (47.2%) were male and 19 (52.8%) were female. The mean age of the patients was 26.0±5.6 years. The prevalence of PAH in the thalassemia major patients was estimated to be 47.2% and the mean SPAP was determined to be 26.2±14.6 mm Hg. Among the examined PAH risk factors, only the history of pulmonary disease (0.02) and transfusion (0.03) was found to be significant.
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Risk factors and comorbidities in Brazilian patients with orofacial clefts

Risk factors and comorbidities in Brazilian patients with orofacial clefts

Abstract: Considering that environmental risk factors substantially contribute to the etiology of orofacial clefts and that knowledge about the characteristics and comorbidities associated with oral clefts is fundamental to promoting better quality of life, this study aimed to describe the risk factors, main characteristics, and comorbidities of a group of patients with cleft lip and/or cleft palate (CL/P) from Rio Grande do Norte (RN), Brazil. Data were obtained from 173 patients with CL/P using a form from the Brazilian database on Orofacial Clefts. Most patients were male with cleft lip and palate and had a normal size and weight at birth; presented few neonatal intercurrent events; and had anemia and respiratory and cardiovascular diseases as main associated comorbidities. They also required timely surgical rehabilitation and multidisciplinary care to stimulate their neuropsychomotor development. In addition, a high frequency of familial recurrence and of parental consanguinity was evidenced in the studied population, especially for the cleft lip and cleft palate type. Other relevant findings were the considerable maternal exposure to alcohol, infections, smoking, and hypertension, as well as low supplementation with vitamins and minerals and deliberate consumption of analgesics, antibiotics, and antihypertensives during pregnancy. Characterization of the CL/P patient profile is essential for the planning of health services and integration among the health professionals involved in the diagnosis and treatment of these malformations. Our results reinforce the need for additional research to confirm the association between environmental factors and the development of orofacial clefts.
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Braz. oral res.  vol.26 número1

Braz. oral res. vol.26 número1

Abstract: The aim of the present study was to investigate caries preva- lence and socioeconomic factors in children with sickle cell anemia. This study was conducted in 160 children with sickle cell anemia aged 3 to 12 years attending the Center for Hematology in Recife, Brazil. Data col- lection included interviews with guardians concerning social factors and oral examinations to determine the caries prevalence. Statistical analyses were performed using the Kruskal-Wallis and Pearson’s chi-square tests at a 5% signiicance level. The caries prevalence was 55.0%. The dmft index was 2.12, and the DMFT index was 1.50. Income signiicantly in- luenced dmft; the mean dmft was 4.57 in children whose family income was less than the Brazilian minimum wage (BMW), whereas in children with a family income three times the BMW or higher, the mean dmft was 2.27. No statistically positive association was found between the educa- tional level of parents and guardians and the caries indices. A statisti- cally signiicant association was found between dental caries prevalence and family income.
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Wellington dos Santos Silva1 , Maria de Nazaré Klautau-Guimarães

Wellington dos Santos Silva1 , Maria de Nazaré Klautau-Guimarães

Two studies have examined HbSS individuals from Salvador: in one, 17 out of 80 patients had the CAR/CAR genotype (21.3%) (Gonçalves et al., 2003), while in the other, eight out of 40 patients (20.0%) had this genotype (Lyra et al., 2005). In contrast, the frequency of this geno- type did not exceed 12.0% among individuals in Recon- cavo Baiano. In a more recent study involving a larger sample of patients with sickle cell anemia in the city of Sal- vador, 14.4% had the CAR/CAR genotype (Adorno et al., 2008). This phenomenon can be explained by internal mi- grations of Afro-descendants from other Brazilian regions to the State capital. Cardoso and Guerreiro (2006) observed the same phenomenon when they studied haplotype distri- bution in HbSS individuals from Belém city, in northern Brazil; these authors also found a higher frequency for the CAR haplotype than reported in historical records.
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Hepatitis C virus infection in a Brazilian population with sickle-cell anemia

Hepatitis C virus infection in a Brazilian population with sickle-cell anemia

The prevalence of HCV RNA was 82.9% (34/41 patients) among anti-HCV-positive individuals. Among the five ELISA-positive individuals who were RIBA negative (2/5) or indeterminate (3/5), only one RIBA-inde- terminate individual was positive for HCV RNA. Mean age was higher among HCV RNA-positive patients than among HCV RNA-negative patients (P = 0.57). The preva- lence of HCV RNA was higher among fe- males (88.9%) than among males (78.3%), although the difference was not significant (P = 0.32). The prevalence of HCV RNA was higher (100%) among individuals from the interior regions of the state than among individuals residing in the metropolitan area of Recife (80.6%) (P = 0.37). There was no significant difference in the prevalence of HCV RNA (P = 0.82) between individuals who had started to receive transfusions be- fore 1992 (82.5%) and those who had started to receive them after 1992 (100%). The pre- valence of HCV RNA was higher among the individuals who had received more than ten units of blood components (84.6%) than among those who had received less than ten units (80%), but the difference was not sta- tistically significant (P = 0.50) (Table 1).
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Risk factors for pulmonary hypertension in patients receiving maintenance peritoneal dialysis

Risk factors for pulmonary hypertension in patients receiving maintenance peritoneal dialysis

End-stage renal disease (ESRD) affects 10–16% of adults worldwide. It is characterized by a significantly reduced estimated glomerular filtration rate and increased urinary albumin excretion (1). ESRD is clinically defined as kidney failure requiring dialysis or transplantation, and is associated with high healthcare costs and mortality. ESRD costs nearly US$23 billion each year in health care costs in the US, and the mortality rates are eight times higher in 20- to 64-year-old ESRD patients treated by dialysis than those individuals of similar age (2). The United States Renal Data System annual data report showed that the mortality of chronic kidney disease (CKD) patients in 2008 was 1.7 times higher than that of non-CKD patients after adjusting for age, gender, race, prior hospitalizations, and comorbidity (3). In- center hemodialysis and home peritoneal dialysis (PD) are the two most common dialysis therapies. Costs associated with PD are almost US$20,000 lower than those of hemo- dialysis, but PD and hemodialysis have similar health outcomes (4). Maintenance peritoneal dialysis (MPD) is a common renal replacement therapy for ESRD patients (3).
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Risk factors for alloimmunization by patients with sickle cell disease

Risk factors for alloimmunization by patients with sickle cell disease

A significant association between Hb phenotype and alloimmunization was ob- served even after multivariate adjustment in patients older than14 years. Patients with Hb SC presented a risk of alloimmunization al- most three times as high as that for patients with Hb SS, an association hitherto unre- ported. In the Cooperative Study of Sickle Cell Disease (3) such association was not found, and a rather lower rate of alloimmu- nization was observed in patients with Hb SC phenotype. This was attributed to a lower number of transfusions received by that group, as also observed by others (20,21). In the present study, the association between Hb SC phenotype and alloimmunization was observed only for patients older than 14 years. For those below this cut-off point alloimmunization frequency was higher for SS children, although not statistically signifi- cant. Patients older than 14 years with Hb SC disease received significantly fewer blood transfusions than those with the SS type. The number of transfusions for male and female patients was closely similar. Two non-excluding hypotheses for explaining the double positive gradient shown in Table 4 for patients above 14 years of age are possible: 1) older female patients and especially those with Hb SC disease developed alloimmuniza- tion at a higher frequency because previous pregnancy and not blood transfusion was an induction factor. Menarche occurs signifi- cantly earlier in Hb SC than SS patients (27). Additionally, sexual activity, which is lower among SCD females compared with con- trols (28) may be higher for Hb SC than for
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Renal dysfunction in patients with sickle cell anemia or sickle cell trait

Renal dysfunction in patients with sickle cell anemia or sickle cell trait

Gross or microscopic hematuria has been detected, usually involving the left kidney, and at times severe enough to endanger the patient’s life or be confused with renal ma- lignancy (6). In this study, the percentage of patients with microscopic hematuria was rela- tively high in both Hb SS and Hb AS groups, being slightly more frequent in the latter (26 and 30%, respectively). Hematuria was basi- cally of nonglomerular origin, since only in one patient did we find dysmorphic urinary erythrocytes. It has been assumed that hema- turia is produced by increased sickling of the erythrocytes in the medulla of the kidney. This causes sludging of the blood in the inner medulla with resulting ischemia and extravasation. At least three factors known to cause sickling could be operative in the medulla of the kidney: increased acidity, hypertonicity and anoxia.
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Insulin Sensitivity in Young Patients with Hypertension Devoid of Conventional Risk Factors

Insulin Sensitivity in Young Patients with Hypertension Devoid of Conventional Risk Factors

There is impaired insulin signaling in essential hypertension [19, 20]. For example, untreated patients with essential hyper- tension have higher fasting and postprandial insulin levels than age- and sex-matched normotensive persons regardless of body mass; a direct correlation between plasma insulin lev- els and blood pressure exists [21] as confirmed by our results. Normally, there is a close relationship between insulin-medi- ated glucose disposal during euglycemic hyperinsulinemic clamp and incremental blood flow in response to insulin. This normal response is lost in insulin-resistant/obese persons, suggesting resistance to the action of insulin to induce vascu- lar nitric oxide production [22]. Accumulating data suggest that insulin sensitivity in skeletal muscle, fat, and vascular tis- sue is impaired in persons predisposed to develop hyperten- sion [22–24]. Another mechanism are impaired vascular re- laxation effects of insulin/IGF-1, mediated in part, by endo- thelial cell production of vasodilatatory factors [25, 26]. Abdominal obesity is associated with microvascular endothe- lial dysfunction through indirect mechanisms, such as insulin resistance and the association with risk factors (including dia- betes mellitus, hypertension, and dyslipidemia), and directly, among others, by the production of adipokines and pro-in- flammatory cytokines which in turn induce oxidative stress leading to reduced NO availability. Hyperinsulinemia in both HT and OB subjects has an adverse effect on lipid metabolism characterised by increased accumulation of abdominal fat and dyslipidemia, resulting in a vicious circle of events further promoting metabolic imbalance and potentiating proathero- genic effect of high blood pressure [27].
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Risk factors related to hypertension among patients in a cohort living with HIVAIDS

Risk factors related to hypertension among patients in a cohort living with HIVAIDS

individuals with HIV/AIDS may be associated with exces- sive weight gain during treatment with HAART and should be avoided. However, while uninfected individuals often have the desire and strive to lose weight, among the infected patients, particularly those who already had symptoms of AIDS, the loss of weight enhances the fantasy of appearing ill and this is a “taboo” among the infected. Thus, it is not only lipodystrophy that merits attention. A recent Brazilian study showed that the majority of adults living with HIV/ AIDS have a diet that needs improvement, and obesity was associated with a poorer quality of diet. 26 Nutritional inter-
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Ultrasound Detection of Salmonella Septic Arthritis in a Rheumatoid Arthritis Patient on Anti-TNF Treatment

Ultrasound Detection of Salmonella Septic Arthritis in a Rheumatoid Arthritis Patient on Anti-TNF Treatment

Three cases of Salmonella septicemia have been reported with anti-TNF therapy. 42 Fu et al reported a case of dissemi- nated Salmonella typhimurium infection in a psoriatic arthri- tis patient with infliximab treatment. 43 A case of disseminated Salmonella paratyphi infection in a RA patient treated with infliximab was reported. 44 Netea et al hypothesized that TNF neutralization with monoclonal antibodies may result in a decreased production of interferon (IFN)-γ, subsequently leading to a defective cellular immune response and decreased expression of Toll-like receptor 4. They concluded that as recognition of microorganisms by TLR-4 and activa- tion of phagocytes by IFN-γ are crucial mechanisms for the defence against intracellular pathogens, their inhibition by anti-TNF leads to severe complication with Salmonella infections. 45
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