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The Democratic Republic of the Congo (DRC) is the third most affected country worldwide by sickle cell disease (SCD). However, this disease is still orphaned in the country; large-scale control actions are rare, and little is known about its management.To assess current practices in the management of SCD in Kisangani, DRC.This cross-sectional study was conducted in six health facilities in Kisangani. It involved 198 presumed sickle cell patients attending the above health facilities. The study focused on the sociodemographic and clinical data of the participants, obtained through a clinical examination and their medical records. Diagnostic confirmation of SCD was made by high-performance liquid chromatography coupled to mass spectrometry. Data were analyzed using SPSS 20.0.The diagnosis of SCD was confirmed in 194 (98.0%; 95% CI: 94.9-99.2) participants, while it was not confirmed in 4 (2.0%; 95% CI: 0.8-5.1) participants. The diagnosis was mainly made by the Emmel test (42.9%). 45.8% of participants had previously been transfused with the blood of their parents. Folic acid was taken by 48.5% of participants and the previous intake of hydroxyurea was reported in 5.1% of participants. The participants vaccinated againstStandard-care practices for SCD patients in Kisangani are insufficient. The Congolese government should regard this disease as a health priority and consider actions to improve its management.

OBJECTIVES: COVID-19 predisposes patients to thrombotic disease. The aim of this guidance document is to provide Belgian health-care workers with recommendations on anticoagulation management in COVID-19 positive patients. METHODS: These recommendations were based on current knowledge and a limited level of evidence. RESULTS: We formulated recommendations for the prophylaxis and treatment of COVID-related venous thromboembolism in ambulatory and hospitalised patients, as well as recommendations for the use of antithrombotic drugs in patients with prior indication for anticoagulation who develop COVID-19. CONCLUSIONS: These recommendations represent an easy-to-use practical guidance that can be implemented in every Belgian hospital and be used by primary care physicians and gynaecologists. Of note, they are likely to evolve with increased knowledge of the disease and availability of data from ongoing clinical trials. ispartof: ACTA CLINICA BELGICA vol:77 issue:2 pages:280-285 ispartof: location:England status: published

The aim of this study was to identify early clinical and laboratory predictive factors of a severe coronavirus disease 2019 (COVID-19).A retrospective study was conducted on adult patients hospitalized for COVID-19 in our hospital. Diagnosis was based on a positive real-time reverse transcription-polymerase chain reaction (RT-PCR) on nasopharyngeal samples. The cohort was divided into two groups, i.e. a favorable evolution (FE) group and an unfavorable evolution (UFE) group, including intensive care unit (ICU) and deceased patients.Results: A total of 198 patients were enrolled in the study, with 138 FE (70%) and 60 UFE (30%). Older age, male gender, comorbidities and dyspnea at admission constituted significantly worse prognosis factors. Among laboratory features, lymphocyte and platelet counts as well as corrected glomerular filtration rate were significantly lower in UFE patients, while neutrophil to lymphocyte ratio, inflammation biomarkers, creatinine, aspartate aminotransferase, lactate dehydrogenase (LDH), glycemia and D-dimer were significantly higher. Procalcitonin and LDH appeared as the most accurate variables according to receiver operating characteristic curves.This Belgian study revealed clinical and laboratory features able to predict high risk of ICU requirement, or even death, at admission time. These results provide a potential tool for patient's triage in a context of pandemic.

ispartof: EJHaem vol:1 issue:1 pages:338-341 ispartof: location:United States status: Published online

Case report We report the case of a young Cameroonian woman who presented with cough, hyperthermia, weight loss, pancytopenia, and hepatosplenomegaly. A positive HIV serology was discovered and a chest radiography revealed a 'miliary pattern'. Bone marrow aspiration pointed out yeast inclusions within macrophages. Given the morphological aspect, the clinical presentation and immunosuppression, histoplasmosis was retained as a working hypothesis. Antiretroviral and amphotericin B treatments were promptly initiated. Review Given the immigration wave that Europe is currently experiencing, we think it is important to share experience and knowledge, especially in non-endemic areas such as Europe, where clinicians are not used to face this disease. Histoplasmosis is due to Histoplasma capsulatum var. capsulatum, a dimorphic fungus. Infection occurs by inhaling spores contained in soils contaminated by bat or bird droppings. The clinical presentation depends on the immune status of the host and the importance of inoculum, varying from asymptomatic to disseminated forms. AIDS patients are particularly susceptible to develop a severe disease. Antigen detection, molecular biology techniques, and microscopic examination are used to make a rapid diagnosis. However, antigen detection is not available in Europe and diagnosis needs a strong clinical suspicion in non-endemic areas. Because of suggestive imagery, clinicians might focus on tuberculosis. Our case illustrates the need for clinicians to take histoplasmosis in the differential diagnosis, depending on the context and the patient's past history.

Highlights • Hydroxychloroquine (HCQ) 2400 mg over 5 days was used in Belgium for COVID-19. • Impact of HCQ on mortality among 8075 patients with COVID-19 was assessed. • Lower mortality in HCQ-treated patients as compared to supportive care. • Lower mortality was irrespective of symptom duration., Hydroxychloroquine (HCQ) has been largely used and investigated as therapy for COVID-19 across various settings at a total dose usually ranging from 2400 mg to 9600 mg. In Belgium, off-label use of low-dose HCQ (total 2400 mg over 5 days) was recommended for hospitalised patients with COVID-19. We conducted a retrospective analysis of in-hospital mortality in the Belgian national COVID-19 hospital surveillance data. Patients treated either with HCQ monotherapy and supportive care (HCQ group) were compared with patients treated with supportive care only (no-HCQ group) using a competing risks proportional hazards regression with discharge alive as competing risk, adjusted for demographic and clinical features with robust standard errors. Of 8075 patients with complete discharge data on 24 May 2020 and diagnosed before 1 May 2020, 4542 received HCQ in monotherapy and 3533 were in the no-HCQ group. Death was reported in 804/4542 (17.7%) and 957/3533 (27.1%), respectively. In the multivariable analysis, mortality was lower in the HCQ group compared with the no-HCQ group [adjusted hazard ratio (aHR) = 0.684, 95% confidence interval (CI) 0.617–0.758]. Compared with the no-HCQ group, mortality in the HCQ group was reduced both in patients diagnosed ≤5 days (n = 3975) and >5 days (n = 3487) after symptom onset [aHR = 0.701 (95% CI 0.617–0.796) and aHR = 0.647 (95% CI 0.525–0.797), respectively]. Compared with supportive care only, low-dose HCQ monotherapy was independently associated with lower mortality in hospitalised patients with COVID-19 diagnosed and treated early or later after symptom onset.

Background Calibrated Automated Thrombography (CAT) has been widely used to assess in vitro thrombin generation as an informative intermediary phenotype of coagulation. Interlaboratory exercises have documented a worrisome poor reproducibility. There are some data on the normalisation with an appropriate external reference plasma (RP). This multicentre study of the French-speaking CAT Club aimed at providing further evidence for the usefulness of such a normalisation. Materials and Methods Lyophilised aliquots of a RP along with 3 plasmas (P1 = normal; P2 = hypo-; P3 = hypercoagulable) were sent to 34 laboratories (corresponding to 38 instruments). CAT was studied using 1 and 5 pM tissue factor and other dedicated reagents. Normalisation with the local RP in use in the laboratory could also be performed. Interlaboratory CVs were calculated for each plasma before and after normalisation. Results Regarding endogenous thrombin potential, a good discrimination between the 3 plasmas was achieved in all laboratories but there was no overlap after normalisation only. CVs were generally not reduced with the use of local RP but were generally improved with normalisation using the external RP, often becoming lower than 10%. Regarding P2 however, the benefit of normalisation was poor, and there were analytical difficulties as well, some laboratories being unable to get a useable signal. Conclusions We confirm that normalisation of CAT results with a suitable external RP is useful in “real life” practice as it often permits an acceptable level of interlaboratory variability. In case of frank hypocoagulability, further improvements are required to get reliable, potentially clinically relevant results.

Introduction Evidence supporting the use of Thromboelastography (TEG®) and rotational thromboelastometric (ROTEM®) in the trauma setting remains limited. We present the results of a practical evaluation of the potential interest of ROTEM® in the diagnosis of acute coagulopathy and the need for emergent blood product transfusion in the general trauma population of a non-trauma Belgian emergency department. Methods Extracting a convenience cohort from the initial prospective TICCS study, we performed a retrospective analysis to test the following hypothesis: ROTEM® might be helpful to discriminate trauma patients with or without acute coagulopathy. Fifty patients were included and ROTEM® results were compared to conventional coagulation tests results, blood transfusion need and outcome. Results With a negative predictive value of 97.6% and a positive predictive value of 42.9%, a strictly normal ROTEM® profile at the time of admission seems to be able to exclude the presence of acute coagulopathy. ROTEM® also seems to be accurate in identifying patients without the need for emergent blood product transfusions. Conclusion In a population of trauma patients of a Belgian general emergency department, a strictly normal coagulation profile evaluated by ROTEM® at hospital entry is associated with a normal coagulation profile evaluated by INR and fibrinogen levels and the absence of any indication of blood product transfusion. ROTEM® may be useful for preselection of trauma patients at risk for coagulopathy within the global trauma population. This, however, would need confirmation in further investigations.clinicaltrials.gov NCT02132208 Registered 6 May 2014.

The Trauma-Induced Coagulopathy Clinical Score (TICCS) was developed to be calculable on the site of injury to discriminate between trauma patients with or without the need for damage control resuscitation and thus transfusion. This early alert could then be translated to in-hospital parameters at patient arrival. Base excess (BE) and ultrasound (FAST) are known to be predictive parameters for emergent transfusion. We emphasize that adding these two parameters to the TICCS could improve the scoring system predictability.A retrospective study was conducted in the University Hospital of Liège. TICCS was calculated for every patient. BE and FAST results were recorded and points were added to the TICCS according to the TICCS.BE definition (+ 3 points if BE - 5 and + 3 points in case of a positive FAST). Emergent transfusion was defined as the use of at least one blood product in the resuscitation room. The capacity of the TICCS, the TICCS.BE and the Trauma-Associated Severe Hemorrhage (TASH) to predict emergent transfusion was assessed.A total of 328 patients were included. Among them, 14% needed emergent transfusion. The probability for emergent transfusion grows with the TICCS and the TICCS.BE values. We did not find a significant difference between the TICCS (AUC 0.73) and the TICCS.BE (AUC 0.76). The TASH proved to be more predictive (AUC 0.89). 66.6% of the patients with a TICCS ≥ 10 and 81.5% with a TICCS.BE ≥ 14 required emergent transfusion.Adding BE and FAST to the original TICCS does not significantly improve the scoring system predictability. A prehospital TICCS 10 could be used as a trigger for emergent transfusion activation. TASH could then be used at hospital arrival. Prehospital TASH calculation may be possible but should be further investigated.Diagnostic test, level III.

Identifying trauma patients that need emergent blood product transfusion is crucial. The Trauma Induced Coagulopathy Clinical Score (TICCS) is an easy-to-measure score developed to meet this medical need. We hypothesized that TICCS would assist in identifying patients that need a transfusion in a large cohort of severe trauma patients from the TraumaRegister DGUA total of 33,385 severe trauma patients were extracted from the TR-DGU for retrospective analysis. The TICCS was adapted for the registry structure. Blood transfusion was defined as the use of at least one unit of red blood cells (RBC) during acute hospital treatment.With an area under the receiving operating curve (AUC) of 0.700 (95% CI: 0.691-0.709), the TICCS appeared to be moderately discriminant for determining the need for RBC transfusion in the trauma population of the TR-DGU. A TICCS cut-off value of ≥12 yielded the best trade-off between true positives and false positives. The corresponding positive predictive value and negative predictive values were 48.4% and 89.1%, respectively.This retrospective study confirms that the TICCS is a useful and simple score for discriminating between trauma patients with and without the need for emergent blood product transfusion.

Unexpected finding or persistence of low human chorionic gonadotropin (hCG) levels is not a rare situation. It requires a clinico-biological approach in order to avoid misunderstandings that could lead to inappropriate diagnostic or therapeutic attitudes. Beyond pregnancy, persistent low levels of hCG may be associated with various benign and malignant conditions, i.e. quiescent gestational trophoblastic disease (QTD), raised pituitary hCG or false positive elevation caused by circulating heterophile antibodies. We report the cases of two non-pregnant patients with low serum hCG. In the first case, hCG levels raised during several years following a spontaneous abortion. The likelihood of heterophilic antibodies interference was ruled out and extensive clinical investigation excluded the presence of a tumour. The diagnosis was QTD. In the second case, elevated hCG came to light as an incidental finding in a women with chronic renal failure and led the clinicians to question the laboratory. The cause was probably an increase in pituitary hCG consecutive to terminal renal failure. These cases illustrate the importance of understanding the biology of the hCG and the causes of its persistent low elevation, which are reviewed in this article. It is essential to demonstrate clinically the presence of a tumour in order to avoid unnecessary and ineffective chemotherapy and/or hysterectomy.

In Belgium, prevention of anti-D immunization is currently based on systematic postnatal prophylaxis associated with targeted antenatal injection in high-risk situations of foeto-maternal haemorrhage.The failures of prevention are mainly due to the non-respect of established guidelines for RhlG prophylaxis, and to spontaneous undetected foetal-maternal haemorrhages without any obvious cause during the third trimester of pregnancy. In order to reduce the rate of residual post-pregnancy anti-D immunization, several countries decided to associate the classical prophylaxis to a routine antenatal anti-D prophylaxis (RAADP) during the 28th or 29th week of gestation. Since a few years, the foetal RHD genotyping in maternal plasma enables us to limit the antenatal prophylaxis only to those D- women carrying a D+ foetus. This paper deals with: the advantages of an antenatal prevention in the light of non-invasive foetal RHD genotyping, the rules rendering prevention protocols efficient whatever the algorithm applied, and the recommended immuno-haematology follow-up of women who received RhlG.

Dear Sir,The toxicity of sustained-release diltiazem formulation is well documented, with a delayed onset but with a sustained vasoplegia. The variable time course of toxicity relates to the unpredictable changes in the concentration of diltiazem. The treatment of diltiazem poisoning is mainly suppo

Objectives Combined oral contraceptives (COCs) decrease testosterone (T) levels. This study investigated restoration of T and other androgen concentrations during COC use by 'co-administration' of dehydroepiandrosterone (DHEA). Study design In this randomized, double-blind, placebo-controlled study in 99 new COC starters (18-35 years old with body mass index range 18-34 kg/m2), a COC containing 30 mcg ethinylestradiol (EE) and 3 mg drospirenone (DRSP) was used for 3 cycles, followed by 6 cycles of the same COC combined with either 50 mg/day DHEA or placebo. Total T, albumin, sex hormone-binding globulin (SHBG), DHEA-sulfate (DHEA-S), Δ4-androstenedione (AD), 3α-androstanediol glucuronide (ADG) and estradiol (E2) were measured, whereas free T and the free T index (FTI) were calculated. Assessments took place at baseline (no COC use), after the run-in period (COC use alone) and during the treatment period (DHEA or placebo). Results During COC use alone, androgen levels decreased, especially total T by 62% and free T by 86%, and SHBG increased by 243%. Total T increased with DHEA compared to placebo (change from end of run-in period to end of treatment period - 1.3±1.2 nmol/L vs. 0.0±0.4 nmol/L; p

Objectives Combined oral contraceptives (COCs) decrease androgen levels, including testosterone (T), which may be associated with sexual dysfunction and mood complaints in some women. We have shown that 'co-administration' of dehydroepiandrosterone (DHEA) to a drospirenone (DRSP)-containing COC restored total T levels to baseline and free T levels by 47%. Here we describe the effects on sexual function, mood and quality of life of such an intervention. Study design This was a randomized, double-blind, placebo-controlled study in 99 healthy COC starters. A COC containing 30 mcg ethinylestradiol (EE) and 3 mg DRSP was used for three cycles, followed by six cycles of the same COC combined with 50 mg/day DHEA or placebo. Subjects completed the Moos Menstrual Distress Questionnaire (MDQ), the McCoy Female Sexuality Questionnaire and the short form of the Quality of Life Enjoyment and Satisfaction Questionnaire (Q-LES-Q). Safety and tolerability, including effects on skin, were evaluated. Results The addition of DHEA induced small but significant improvements compared to placebo in the MDQ score for autonomic reactions during the menstrual (- 2.0 vs. 0.71; p=0.05) and the premenstrual phase (- 3.1 vs. 2.9; p=0.01) and for behavior during the intermenstrual phase (- 1.4 vs. 3.6; p=0.02). A significant difference was found in the MDQ score for arousal during the premenstrual phase in favor of placebo (- 5.0 vs. 1.0; p=0.01). There were no statistically significant differences between groups for the MSFQ and Q-LES-Q scores. DHEA 'co-administration' resulted in an acceptable safety profile. DHEA negated the beneficial effect of the COC on acne according to the subjects' self-assessment. Conclusions 'Co-administration' with DHEA did not result in consistent improvements in sexual function, mood and quality of life indicators in women taking EE/DRSP. Retrospectively, the 50 mg dose of DHEA may be too low for this COC. Implications A well-balanced judgment of the clinical consequences of normalizing androgens during COC use may require complete normalization of free T.

Introduction Identifying patients who need damage control resuscitation (DCR) early after trauma is pivotal for adequate management of their critical condition. Several trauma-scoring systems have been developed to identify such patients, but most of them are not simple enough to be used in prehospital settings in the early post-traumatic phase. The Trauma Induced Coagulopathy Clinical Score (TICCS) is an easy-to-measure and strictly clinical trauma score developed to meet this medical need. Methods TICCS is a 3-item clinical score (range: 0 to 18) based on the assessment of general severity, blood pressure and extent of body injury and calculated by paramedics on-site for patients with severe trauma. This non-interventional prospective study was designed to assess the ability of TICCS to discern patients who need DCR. These patients were patients with early acute coagulopathy of trauma (EACT), haemorrhagic shock, massive transfusion and surgical or endovascular haemostasis during hospitalization. Diagnosis of EACT was assessed by both thromboelastometry and conventional coagulation tests. Results During an 18-month period, 89 severe trauma patients admitted to the general emergency unit at our hospital were enrolled in the study, but 7 were excluded for protocol violations. Of the 82 remaining patients, 8 needed DCR and 74 did not. With receiver operating characteristic curve analysis, TICCS proved to be a powerful discriminant test (area under the curve = 0.98; 95% CI: 0.92 to 1.0). A cutoff of 10 on the TICCS scale provided the best balance between sensitivity (100%; 95% CI: 53.9 to 100) and specificity (95.9%; 95% CI: 88.2 to 99.2). The positive predictive value was 72.7%, and the negative predictive value was 100.0%. Conclusion TICCS can be easily and rapidly measured by paramedics at the trauma site. In this study of blunt trauma patients, TICCS was able to discriminate between patients with and without need for DCR. TICCS on-site evaluation should allow initiation of optimal care immediately upon hospital admission of patients with severe trauma in need of DCR. However, a larger multicentre prospective study is needed for in-depth validation of TICCS. Trial registration Clinicaltrials.gov ID: NCT02132208 (registered 6 May 2014)

Objective. Neutrophil gelatinase-associated lipocalin (NGAL) measured by a research ELISA is described as an early marker of acute kidney injury (AKI). The aim of this study is to define the usefulness of plasma NGAL (pNGAL) and urine NGAL (uNGAL) measured with platform analysers to detect AKI 3 hours after cardiac surgery in fifty adult patients. Methods and Main Results. pNGAL and uNGAL were measured before and 3 hours after cardiac surgery. AKI, defined following the acute kidney injury network definition, was observed in 17 patients. pNGAL was >149 ng/mL in 8 patients with AKI, two of them died in the follow-up. We also observed elevated pNGAL in 8 patients without AKI. Only one uNGAL was >132 ng/mL among the 15 AKI patients. Sensitivity of pNGAL for prediction of AKI is 47% and specificity is 75.7%. The positive likelihood ratio (LR+) is 1.9 and negative likelihood ratio (LR−) is 0.7. uNGAL performance is slightly improved when reported to urinary creatinine. Following this study, a ratio >62 ng/mg assure a sensitivity of 66.6% and a specificity of 78.5%. LR+ is 3 and a LR− is, 0.42. Conclusions. Three hours after cardiac surgery, pNGAL predicts AKI with a low sensitivity and specificity.

We report the successful management of a craniosynostosis repair in a child with severe Type I von Willebrand disease diagnosed during the preoperative assessment and treated by coagulation factor VIII and ristocetin cofactor. Collaboration among the anesthesiologist, the neurosurgeon, the clinical pathologist, and the pediatric hematologist is important for successful management.

Background: Neonatal screening programs for sickle cell disease are now widespread in North American and European countries. Most programs apply isoelectric focusing or HPLC to detect hemoglobin variants. Because tandem mass spectrometry (MS/MS) is being used for screening of inherited metabolic disorders and allows protein identification, it was worth testing for hemoglobinopathy screening.Methods: We minimized sample preparation and analysis times by avoiding prior purification, derivatization, or separation. We developed a tryptic digestion methodology to screen for the main clinically important variants (Hb S, Hb C, and Hb E) and β-thalassemia. To ensure proper discrimination between homozygote and heterozygote variants, we selected 4 transitions with good signal intensities for each specific peptide and calculated variant/Hb A ratios for each. Method validation included intra- and interseries variability, carryover, and limit of detection. We also performed a comparative study with isoelectric focusing results on 2082 specimens.Results: Intraassay imprecision values (CVs) varied between 2.5% and 30.7%. Interassay CVs were between 6.3% and 23.6%. Carryover was Conclusions: MS/MS is a reliable method for hemoglobinopathy neonatal screening.

Background: The objective was to evaluate the diagnostic value of RHD fetal genotyping from the plasma of D- mothers as soon as 10 weeks' gestation in a routine clinical practice in Belgium. Study design and methods: A prospective study was conducted between November 2002 and December 2006. DNA extraction was performed in an automated closed tube system. Fetal RHD/SRY genotypes were detected in the plasma of 563 pregnant mothers by real-time polymerase chain reaction (PCR) targeting multiple exons 4, 5, and 10 of the RHD gene and targeting an SRY gene sequence. These were compared to the D phenotypes determined in the 581 babies they delivered. Results: By combining amplification of three exons, the concordance rate of fetal RHD genotypes in maternal plasma and newborn D phenotypes at delivery was 100 percent (99.8% including one unusual false-positive). The presence of nonfunctional RHD genes and the absence of a universal fetal marker, irrespective of fetal sex, did not influence the accuracy of fetal RhD status prediction. The RHD genotyping from 18 twin pregnancies was also assessed. Five weak D women were excluded from the RHD fetal genotyping prediction. Three discrepant results (0.5%) between predicted fetal genotype and cord blood phenotype were not confirmed by the baby phenotypes from venipuncture blood. Conclusion: Prenatal prediction of fetal RHD by targeting multiple exons from the maternal plasma with realtime PCR is highly sensitive and accurate. Over 4 years, this experience has highly modified our management of D- pregnant women. Abbreviations: Ct = cycle threshold; HDFN = hemolytic disease in fetuses and newborns.