BMC Hematology最新文献

Background: There are a paucity of data on epistaxis as it pertains to sickle cell anaemia. Some case studies suggest epistaxis to be a significant complication in patients with sickle cell anaemia in sub-Saharan Africa; however, no robust studies have sought to establish the epidemiology or pathophysiology of this phenomenon.

Methods: We conducted a case-control study with the aim of investigating the importance of epistaxis among children presenting with sickle cell anaemia at the Mbale Regional Referral Hospital in eastern Uganda. Cases were children aged 2-15 years with an existing diagnosis of laboratory confirmed sickle cell anaemia, while controls were children without sickle cell anaemia who were frequency matched to cases on the basis of age group and gender. The frequency and severity of epistaxis was assessed using a structured questionnaire developed specifically for this study. Odds ratios controlled for age group and gender were calculated using unconditional logistic regression.

Results: A total of 150 children were included, 73 children with sickle cell anaemia and 77 children without sickle cell anaemia. The overall prevalence of epistaxis among children with sickle cell anaemia and children without sickle cell anaemia was 32.9 and 23.4% respectively. The case-control odds ratios for epistaxis, recurrent epistaxis and severe epistaxis were, 1.6 (95%CI 0.8-3.4; p = 0.2), 7.4 (1.6-34.5; 0.01), and 8.3 (1.0-69.8; 0.05) respectively.

Conclusions: Our results suggest that in eastern Uganda, children with sickle cell anaemia experience epistaxis more frequently and with greater severity than children without sickle cell anaemia. Further studies are indicated to confirm this conclusion and investigate aetiology.

Background: Anemia is a significant wide spread public health threat especially among the adolescent girls who are more vulnerable towards low level of hemoglobin particularly of low and middle income countries (LMICs). We investigated the prevalence of anemia among the adolescent girls (10-19 years) in Bangladesh and its socio-demographics distribution.

Methods: We collected data digitally in ODK platform from a sub-sample of a nationwide cross-sectional survey of 1314 adolescent girls in 2015. Capillary blood hemoglobin level was estimated using HemoCue®; anthropometric measurements through standardized procedure and details socio-demographic information were captured and analyzed. Malnutrition was defined as BMI-for-age Z-score below -2SD (BAZ < -2SD), measured in WHO-AnthroPlus. Univariate analysis followed by multiple logistic regression were performed to examine the association between socio-demographic variables and anemia, while controlling the effect of potential confounding variables.

Results: Overall, 51.6% girls were suffering from any form of anemia (non-pregnant-Hb < 12 g/dl; pregnant-Hb < 11 g/dl) while 46% were mildly (non-pregnant-Hb: 10-11.9 g/dl; pregnant-Hb: 10-10.9 g/dl) and 5.4% were moderately (Hb: 7-9.9 g/dl) anemic while only 0.2% were severely anemic. After controlling for relevant covariates in multiple logistic regression model, malnutrition (AOR: 1.42, 95% CI = 1.0-2.10, p-value = 0.083), non-pregnancy (AOR: 6.10, 95% CI = 2.70-13.78, p-value < 0.001), and households with bottom wealth quintile (AOR: 1.54, 95% CI = 1.03-2.30, p-value = 0.037) were identified as significant risk factors of anemia among adolescent girls of Bangladesh.

Conclusions: Higher number of adolescent girls are still suffering from anemia in Bangladesh and non-pregnant adolescent girls contributed the most. Immediate, long term and sustainable public health intervention would require to combat the situation.

Background: Zinc protoporphyrin (ZPP) has been used to screen and manage iron deficiency in individual children, but it has also been recommended to assess population iron status. The diagnostic utility of ZPP used in combination with haemoglobin concentration has not been evaluated in pre-school children. We aimed to a) identify factors associated with ZPP in children aged 12-36 months; b) assess the diagnostic performance and utility of ZPP, either alone or in combination with haemoglobin, to detect iron deficiency.

Methods: We used baseline data from 338 Kenyan children enrolled in a community-based randomised trial. To identify factors related to ZZP measured in whole blood or erythrocytes, we used bivariate and multiple linear regression analysis. To assess diagnostic performance, we excluded children with elevated plasma concentrations of C-reactive protein or α₁-acid glycoprotein, and with Plasmodium infection, and we analysed receiver operating characteristics (ROC) curves, with iron deficiency defined as plasma ferritin concentration < 12 μg/L. We also developed models to assess the diagnostic utility of ZPP and haemoglobin concentration when used to screen for iron deficiency.

Results: Whole blood ZPP and erythrocyte ZPP were independently associated with haemoglobin concentration, Plasmodium infection and plasma concentrations of soluble transferrin receptor, ferritin, and C-reactive protein. In children without inflammation or Plasmodium infection, the prevalence of true iron deficiency was 32.1%, compared to prevalence of 97.5% and 95.1% when assessed by whole blood ZPP and erythrocyte ZPP with conventional cut-off points (70 μmol/mol and 40 μmol/mol haem, respectively). Addition of whole blood ZPP or erythrocyte ZPP to haemoglobin concentration increased the area-under-the-ROC-curve (84.0%, p = 0.003, and 84.2%, p = 0.001, respectively, versus 62.7%). A diagnostic rule (0.038689 [haemoglobin concentration, g/L] + 0.00694 [whole blood ZPP, μmol/mol haem] >5.93120) correctly ruled out iron deficiency in 37.4%-53.7% of children screened, depending on the true prevalence, with both specificity and negative predictive value ≥90%.

Conclusions: In young children, whole blood ZPP and erythrocyte ZPP have added diagnostic value in detecting iron deficiency compared to haemoglobin concentration alone. A single diagnostic score based on haemoglobin concentration and whole blood ZPP can rule out iron deficiency in a substantial proportion of children screened.

Trial registration: ClinicalTrials.gov NCT02073149 (25 February 2014).

Background: Despite the public health significance of anaemia in African children, its broader and often preventable risk factors remain largely under described. This study investigated, for the first time, the prevalence of childhood anaemia and its risk factors in an urban setting in Uganda.

Methods: A total of 342 children were enrolled. Venous blood samples were collected in EDTA tubes and analyzed using Symex 500i (Symex Corp. Japan). Stool and urine samples were analyzed according to established standard methods. Anthropometric indicators were calculated according to the CDC/WHO 1978 references. Ethical approval was granted.

Results: Categorically, the prevalence of anaemia was; 37.2, 33.3 and 11.8% among children aged 1-5 years, 6-11 years and 12-14 years respectively. Overall anaemia prevalence was 34.4%. The risk of anaemia was higher among males than females [(OR = 1.3, 95% CI = 0.8, 2.1), P = .22]. Malaria was associated with a 1.5 times risk of anaemia though not statistically significant in the multivariate analysis (P = .19). Maternal parity <5 (P = .002), and stunting [(OR = 2.5, 95% CI = 1.3, 4.7), P = .004] were positively associated with anaemia. There was a positive correlation between household size and income (Pearson X²  = 22.96; P = .001), implying that large families were of higher socioeconomic status.

Conclusions: This study demonstrates that anaemia is more prevalent in the under-5 age. The risk factors are stunting and low maternal parity. Interventions that address nutritional deficiencies in both pre-school and school children are recommended. Malaria and helminthiasis control measures counter the risk of anaemia. Further studies are required to investigate the association between maternal parity and anaemia found in this study.

Background: Portal hypertension has a broad differential diagnosis. POEMS syndrome is an uncommon cause of it. POEMS syndrome is a rare disease involving multiple organs. In differential diagnosis of portal hypertension, POEMS syndrome should be considered especially when other symptoms such as numbness, organomegaly, endocrine alteration and skin changes also present, as it is highlighted by our case.

Case presentation: We report a 46-year-old Chinese male, a teacher, presenting with portal hypertension. Electromyography revealed peripheral neuropathy. Immunofixation showed monoclonal immunoglobulin A lambda protein. The diagnosis of POEMS syndrome was established. After treatment of lenalidomide combined with dexamethasone over 2 years, the patient achieved a considerable improvement.

Conclusion: This case highlights the manifestation of portal hypertension in POEMS syndrome. Lenalidomide with or without dexamethasone is effective for portal hypertension due to POEMS syndrome, though esophageal and gastric varices seems not reversible so easily.

Background: Circumcision in hemophiliacs is a delicate surgery because of bleeding risks that could be avoided by adequate substitution of coagulation factor. This practice is very challenging in countries where anti hemophilic treatment is inaccessible. The study aimed to evaluate a circumcision protocol in hemophilia A using low quantities of factor concentrates.

Methods: This prospective study included 26 hemophiliacs A who underwent circumcision in 2014. Medical treatment protocol using low quantity of factor concentrates was drafted by physicians of the Hemophilia Treatment Center and the surgical protocol by experienced surgeons. Assessment criteria were: number of hospitalization days, number of exposure days to factor concentrates, delay to healing and occurrence of bleeding events.

Results: Mean age was 9.6 years (1-30). Hemophiliacs patients were classified as severe (n = 8), moderate (n = 9) and mild form (n = 9). Mean number of exposure days to factor VIII concentrates was 6.9 days (5-12) in children and 10.75 days (7-16) in adults (p = 0.0049); mean number of hospitalization days was 3.68 days (2-10) in children and 13.5 days (13-15) in adults (p = 0.0000); delay to healing was 26.47 days (20-35) in children and 25.25 days (22-30) in adults (p = 0.697); five haemophiliacs (19.2%) presented bleeding events after the circumcision. The mean amount of FIII concentrates used per patient was 1743 IU (810-2340).

Conclusion: The study shows treatment protocol using low quantity of factor concentrates is efficient in hemophilia patients who underwent circumcision.

Background: Africa is the most affected continent with 200,000 new born affected by sickle cell anemia annually with of 5% of under five deaths. Nigeria has the largest sickle cell gene pool in the world with about 2% of all babies born to Nigerian parents. This study therefore sets out to assess the prevention practices influencing the frequency of occurrence of vaso-occlusive crisis among patients in Ogun State.

Methods: This study is a descriptive cross-sectional study conducted in Abeokuta South Local Government Area Ogun State. A consecutive non randomized sampling of all the sickle cell patients that attend the selected facilities was recruited into the study. Data were collected with the use of questionnaires which were interviewer administered. A total of 415 patients were recruited into the study. Statistical analyses were conducted using SPSS for Windows version 20.0.

Result: Two- third [64.8%] of study participants have crisis twice or more in a month. The frequency of crisis was statistically significantly associated with the age of the child [p = 0.006], use of anti-malaria prophylaxis [p = 0.006], analgesics [p = 0.0001], taking of plenty fluid [p = 0.001] and soothing herbs [p = 0.0001]. Lifestyle factors such as giving balance diet [p = 0.217], restriction from strenuous activities [p = 0.08], and attending Clinic appointments regularly [p = 0.126] were not statistically associated with reduction in the frequency of crisis. Logistic regression analysis shows that predictors of frequent crisis were individuals who were using prophylaxis antimalarial drugs [OR = 0.12, CI = 0.05-0.33] and analgesics [OR = 0.15, C.I = 0.06-0.34].

Conclusion: The study reveals that majority of the participants have high frequency of crisis in a month. Drug prophylaxis rather than lifestyle factors may be more important in the prevention of vaso-occlusive crisis among sickle cell patients.

Background: Sickle Cell Disease (SCD) is associated with chronic multisystem complications that significantly influence the quality of life (QOL) of patients early in their life. Although sub-Saharan Africa bears 75% of the global burden of SCD, there is a paucity of data on these complications and their effects on the QOL. We aimed to record these chronic complications, to estimate the QOL, and to identify the corresponding risk factors in patients with SCD receiving care in three hospitals in Cameroon.

Methods: In this cross-sectional study, a questionnaire was used to collect data from consecutive consenting patients. Information recorded included data on the yearly frequency of painful crisis, the types of SCD, and the occurrence of chronic complications. A 36-Item Short Form (SF-36) standard questionnaire that examines the level of physical and mental well-being, was administered to all eligible participants. Data were analyzed with STATA® software.

Results: Of 175 participants included, 93 (53.1%) were female and 111 (aged ≥14 years) were eligible for QOL assessment. The median (interquartile range, IQR) age at diagnosis was 4.0 (2.0-8.0) years and the median (IQR) number of yearly painful crisis was 3.0 (1.0-7.0). The most frequent chronic complications reported were: nocturnal enuresis, chronic leg ulcers, osteomyelitis and priapism (30.9%, 24.6%, 19.4%, and 18.3% respectively). The prevalence of stroke and avascular necrosis of the hip were 8.0% and 13.1% respectively. The median (IQR) physical and mental scores were 47.3 (43.9-58.5) and 41.0 (38.8-44.6) respectively. Age and chronic complications such as stroke and avascular necrosis were independently associated with poor QOL.

Conclusions: In this population of patients living with SCD, chronic complications are frequent and their QOL is consequently poor. Our results highlight the need for national guidelines for SCD control, which should include new-born screening programs and strategies to prevent chronic complications.

Background: Familial and acquired thrombophilia are often etiologic for idiopathic hip and jaw osteonecrosis (ON), and testosterone therapy (TT) can interact with thrombophilia, worsening ON.

Case presentation: Case 1: A 62-year-old Caucasian male (previous deep venous thrombosis), on warfarin 1 year for atrial fibrillation (AF), had non-specific right hip-abdominal pain for 2 years. CT scan revealed bilateral femoral head ON without collapse. Coagulation studies revealed Factor V Leiden (FVL) heterozygosity, 4G/4G plasminogen activator inhibitor (PAI) homozygosity, high anti-cardiolipin (ACLA) IgM antibodies, and endothelial nitric oxide (NO) synthase (eNOS) T786C homozygosity (reduced conversion of L-arginine to NO, required for bone health). Apixaban 5 mg twice daily was substituted for warfarin; and L-arginine 9 g/day was started to increase NO. On Apixaban for 8 months, he became asymptomatic. Case 2: A 32-year-old hypogonadal Caucasian male had 10 years of unexplained tooth loss, progressing to primary jaw ON with cavitation 8 months after starting TT gel 50 mg/day. Coagulation studies revealed FVL heterozygosity, PAI 4G/4G homozygosity, and the lupus anticoagulant. TT was discontinued. Jaw pain was sharply reduced within 2 months.

Conclusions: Idiopathic ON, often caused by thrombophilia-hypofibrinolysis, is worsened by TT, and its progression may be slowed or stopped by discontinuation of TT and, thereafter, anticoagulation. Recognition of thrombophilia-hypofibrinolysis before joint collapse facilitates anticoagulation which may stop ON, preserving joints.

Background: Sickle cell anemia patients suffer from oxidative stress due to chronic inflammation and self-oxidation of sickle hemoglobin (Hb S). Chronic oxidative stress contributes to endothelial dysfunction, inflammation and multiple organ damage in sickle cell disease (SCD). Thus, antioxidant medication may favorably influence the disease. Gum Arabic (GA), edible, dried, gummy exudates from Acacia Senegal tree, has been claimed to act as an anti-oxidant and cytoprotective agent, protecting against experimental hepatic, renal and cardiac toxicities in rats. We hypothesized that regular intake of GA increases anti-oxidant capacity and reduce oxidative stress.

Methods: Forty-seven patients (5-42 years) carrying hemoglobin SS were recruited. Patients received 30 g/day GA for 12 weeks. Total anti-oxidant capacity (TAC), malondialdehyde (MDA) and hydrogen peroxide (H₂O₂) levels were measured by spectrophotometric methods before and after GA intake. Complete blood count was measured by sysmex.

Results: Gum Arabic significantly increased TAC level P < 0.001and decreased the oxidative markers MDA (P < 0.05) and H₂O₂ (P < 0.005).

Conclusions: GA has potent anti- oxidative properties in sickle cell anemia. The anti-oxidant effect of GA may thus favorably influence the clinical condition of this and further diseases characterized by oxidative stress.

Trial registration: ClinicalTrials.gov Identifier: NCT02467257. Registered 3rd June 2015. Retrospective registration.