Blood that was far from red: hypertriglyceridaema thalassaemia syndrome: case report

Distinct risk factors for early and late blood transfusion following pancreaticoduodenectomy

Outcomes after kidney transplantation are largely driven by the development of de novo donor-specific antibodies (dnDSA), which may be triggered by blood transfusion. In this single-center study, we investigated the link between early blood transfusion and dnDSA development in a mainly anti-thymocyte globulin (ATG)-induced kidney-transplant cohort. We retrospectively included all recipients of a kidney transplant performed between 2004 and 2015, provided they had >3 months graft survival. DSA screening was evaluated with a Luminex assay (Immucor). Early blood transfusion (EBT) was defined as the transfusion of at least one red blood-cell unit over the first 3 months post-transplantation, with an exhaustive report of transfusion. Patients received either anti-thymocyte globulins (ATG) or basiliximab induction, plus tacrolimus and mycophenolic acid maintenance immunosuppression. A total of 1088 patients received a transplant between 2004 and 2015 in our center, of which 981 satisfied our inclusion criteria. EBT was required for 292 patients (29.7%). Most patients received ATG induction (86.1%); the others received basiliximab induction (13.4%). dnDSA-free graft survival (dnDSA-GS) at 1-year post-transplantation was similar between EBT+ (2.4%) and EBT- (3.0%) patients (chi-squared p=0.73). There was no significant association between EBT and dnDSA-GS (univariate Cox’s regression, HR=0.88, p=0.556). In multivariate Cox’s regression, adjusting for potential confounders (showing a univariate association with dnDSA development), early transfusion remained not associated with dnDSA-GS (HR 0.76, p=0.449). However, dnDSA-GS was associated with pretransplantation HLA sensitization (HR=2.25, p=0.004), hemoglobin >10 g/dL (HR=0.39, p=0.029) and the number of HLA mismatches (HR=1.26, p=0.05). Recipient’s age, tacrolimus and mycophenolic-acid exposures, and graft rank were not associated with dnDSA-GS. Early blood transfusion did not induce dnDSAs in our cohort of ATG-induced patients, but low hemoglobin level was associated with dnDSAs-GS. This suggests a protective effect of ATG induction therapy on preventing dnDSA development at an initial stage post-transplantation.

Carotid atherosclerosis (CAS), a key precipitator of cardiovascular incidents, is linked to postprandial triglyceride-rich lipoproteins (TRL), as reflected by elevated triglycerides (TG) and remnant cholesterol (RC). This study explores the oral fat tolerance test (OFTT) for its predictive value in CAS, using postprandial TRL levels as a diagnostic biomarker. A total of 107 volunteers with normal fasting lipid profiles and no CAS at baseline were included. They received an OFTT after consuming a meal containing 60% fat (1500 kcal). Serum lipid profiles were monitored at fasting and 2, 4, 6, 8, and 10 h postprandially. The participants were categorized into postprandial normotriglyceridemia and postprandial hypertriglyceridemia groups based on their postprandial TG levels. After a 6-year follow-up, fasting lipid profiles and CAS status were reassessed. The baseline fasting and postprandial lipid levels in the CAS and non-CAS groups were compared. Repeated-measures analysis of variance was used to analyze the postprandial lipid profiles across different groups. Logistic regression models were constructed to assess the effects of postprandial TG and RC levels on CAS incidence. The incidence of CAS in the postprandial hypertriglyceridemia group was 66.0%, which was significantly higher than the 13.3% observed in the postprandial normotriglyceridemia group (P < 0.001). In the CAS group, postprandial TG and RC levels peaked 4 h after a high-fat meal and did not return to fasting levels, even after 10 h. The levels of 4h-postprandial TG (TG4h), maximum postprandial TG (TGmax), 4h-postprandial RC (RC4h), and maximum postprandial RC (RCmax) were significantly higher in the CAS group than in the non-CAS group (P < 0.05). At baseline, TG4h (P < 0.001), TGmax (P = 0.006), RC4h (P < 0.001), and RCmax (P = 0.003) were statistically significant predictors of CAS, whereas fasting TG (P = 0.200) and fasting RC (P = 0.200) were not significantly associated with CAS. The standardized OFTT has predictive value for CAS, and elevated TRL levels after a high-fat meal in individuals with normal fasting lipid profiles may serve as an early marker for CAS.

INTRODUCTIONHypertriglyceridemia Thalassemia Syndrome is a rare condition with few reported cases. In here, we reported an interesting case of Hypertriglyceridemia Thalassaemia Syndrome in a young infant presented to our centre. CASEA 1-year-old Malay girl presented with poor growth, progressive pallor and lethargy for the past six months. She had no significant family history of blood or lipid disorder. On examination, she is small for her age, pale and icteric. Liver and spleen were palpable 4cm below costal margin respectively. She has no lymphadenopathy or xanthomas. Laboratory investigations showed low haemoglobin level (Hb 7.2 g/dL) but normal platelet count (375 x 109/L), and white cell count (10 x 109/L). She had raised reticulocytes count (11%) and lactate dehydrogenase (LDH 532U/L). Direct coombs test was negative. Peripheral blood smear revealed hypochromic microcytic red blood cells with polychromasia and severe anis poikilocytosis. Her serum was grossly lipidemic after centrifugation. Lipid profile showed raised triglyceride level (TG 9.05 mmol/L) but other lipid parameters are in normal range (total cholesterol 2.85 mmol/L, HDL-c 0.26 mmol/L, LDL -1.25 mmol/L). Her infective status is negative and her thyroid function test is normal. Other acquired causes of hypertriglyceridemia are also excluded. She was transfused with total 40 cc/kg pack cells and her Hb raised to 12 g/dl and no more lipidemic serum sample. Hb analysis revealed HbE- Beta thalassemia. Her mother`s blood showed Hb-E trait with normal lipid profile. She defaulted follow up until 4 months later she presented to our care again with similar presentation. CONCLUSIONHypertriglyceridemia thalassemia is rare reported association and regular blood transfusion usually resolved the high TG level. Early recognition is essential to deal with complications such as acute pancreatitis or increased coronary risk.

Background: Each patient's risk-benefit profile must be carefully assessed because tamoxifen's anti-estrogenic properties may have an impact on lipid metabolism. Objectives: This research looks into how tamoxifen affects Kurdish women's lipid profiles. Methods: This cross-sectional study was conducted at the Azadi Hematology Oncology Centre in Duhok City, Kurdistan Region, Iraq, on 165 females with estrogen- and/or progesterone-positive breast cancer receiving adjuvant hormonal therapy with tamoxifen for three months or longer. Results: The mean age was 42.4 years; 83.6% were married and multiparous (77.6%), with a BMI of 29.1 kg/m². 56.1% of participants had normal lipid profiles, 32.4% had elevated levels, and 11.5% had low levels. Age showed minimal impact on lipid biomarkers, with very weak correlations. Obese patients had normal TC (68.6%), HDL-C (68.6%), LDL-C (44.8%), TC/HDL-C (73.1%), and LDL-C/HDL-C (88.1%) ratios, as well as elevated TG (65.7%) and VLDL-C (77.6%), with weak correlations. Patients treated with tamoxifen for ≥ 24 months showed higher proportions of normal and high lipid profiles, with minimal impact of treatment duration. Conclusions: Abnormal HDL-C, TG, VLDL-C, and TC/HDL-C were more frequent in obese patients, suggesting BMI had more impact on lipid profile than age or therapy duration. Tamoxifen had limited effects on lipid profile, with minimal associations with age or treatment duration.

Prevalence of obesity and its subsequent cardiometabolic complications is on exponential rise. Hyperinsulinemia develops in obese individuals long before other metabolic derangements of obesity take place and may be a common pathophysiological factor tying together various components of cardiometabolic dysfunction. Present study was aimed at evaluating the role of insulin as a sensitive and independent cardiovascular risk marker in apparently healthy overweight and obese individuals with normal lipid profile. This was an opd based case Control study including 100 overweight and obese individuals with normal lipid profile & 100 age and sex matched normal weight healthy controls. Participants were evaluated based on detailed history, clinical examination and laboratory investigations. Blood samples were collected after overnight fast. Serum insulin was estimated by chemiluminescence method, glucose and lipid profile (CHOLESTEROL, HDL, TG, LDL) by chemical assays on a fully automated analyser system. RESULTS were analyzed by unpaired t-test, p-value was determined & Correlation coefficient was calculated amongst various parameters. Significant difference was noted in mean values of BMI (29.69±1.28 VS 23.47±1.09), waist / hip ratio (0.91±0.07 VS 0.79±0.05) and serum insulin (10.54±2.5 VS 5.94±1.53) (p<0.01) in cases as compared to controls respectively. Glucose levels were high in cases (89.58±8.0 mg/dl) as compared to controls (88.8±7.56 mg/dl) but the difference was statistically insignificant (p=0.11). Hyperinsulinemia was observed in 41 cases & 4 controls. Serum insulin highly correlated with Waist/ hip ratio (R=0.53) than BMI (R=0.26). Study suggests Insulin; a simple, sensitive & independent cardiovascular risk predictor in obesity even with normal lipid profile with a potential to reveal hidden burden of metabolic dysfunction and offers a hope that, cardiovascular event can be well prevented with appropriate interventions.

Objective: To compare the outcome of early blood transfusion i.e. within 6hrs. (hours) of admission VS late blood transfusion (after 6 hrs.) in Puerperal septic shock patients with anemia. Study design: A prospective quasi-experimental controlled trial in ICU(intensive care unit) of Lady Willingdon Hospital, a tertiary care hospital of Obstetrics and Gynecology from Aug2013 to July 2016. Population: Patients admitted with septic shock and anemia during puerperium in ICU. Methods: 74 patients were included in the study by purposive sampling technique. Exclusion criteria were an irreversible shock, chronic lung, and heart disease or those requiring surgical intervention. Surviving sepsis campaign guidelines were followed for the management of these patients. Effect of blood transfusion on the recovery of these subjects was studied. The study group consisted of 43 patients in which blood was transfused within 6hrs of admission. Control group had 31 patients in whom blood could not be transfused within this period. The demographic features and outcome of both groups were recorded and entered on spss20 statistical package. Results: The time of recovery from hypotension, tachycardia, pulmonary edema, ventilator and cardiac support was significantly shorter in the study group compared to the control. A lesser number of blood transfusions was required to treat anemia in the study group. Maternal mortality was less in the study group (23.25%) as compared to control group (48.39%) with an odds ratio of decrease in maternal mortality of 0.323(.119-.877). Conclusion: Early blood transfusion shortens the time of recovery and decreases maternal mortality and morbidity in Puerperal septic shock patients with anemia.

BackgroundThere is no existing adequate blood transfusion needs determination tool that Emergency Medical Services (EMS) personnel can use for prehospital blood transfusion initiation. In this study, a simple and pragmatic prehospital blood transfusion needs scoring system was derived and validated.MethodsLocal trauma registry data were reviewed retrospectively from 2004 through 2013. Patients were randomly assigned to derivation and validation cohorts. Multivariate logistic regression was used to identify the independent approachable risks associated with early blood transfusion needs in the derivation cohort in which a scoring system was derived. Sensitivity, specificity, and area under the receiver operational characteristic (AUC) were calculated and compared using both the derivation and validation data.ResultsA total of 24,303 patients were included with 12,151 patients in the derivation and 12,152 patients in the validation cohorts. Age, penetrating injury, heart rate, systolic blood pressure, and Glasgow coma scale (GCS) were risks predictive of early blood transfusion needs. An early blood transfusion needs score was derived. A score > 5 indicated risk of early blood transfusion need with a sensitivity of 83% and a specificity of 80%. A sensitivity of 82% and a specificity of 80% were also found in the validation study and their AUC showed no statistically significant difference (AUC of the derivation = 0.87 versus AUC of the validation = 0.86, P > 0.05).ConclusionsAn early blood transfusion scoring system was derived and internally validated to predict severe trauma patients requiring blood transfusion during prehospital or initial emergency department resuscitation.

Lipid-lowering drugs preserve vision and reduce the risk of hard exudates in clinically-significant macular edema(CSME) in diabetics with an abnormal lipid profile. But their role in reducing CSME in diabetics with a normal lipid profile is not yet known. To evaluate the role of atorvastatin in CSME in diabetics with a normal lipid profile. A prospective, randomized clinical trial was carried out. Thirty CSME patients with a normal lipid profile were randomly divided into Group A and B. Atorvastatin had been started in Group A four weeks prior to laser treatment. The main outcome measures were any improvement or deterioration in visual acuity and macular edema and hard exudates at six months follow-up. Both the groups were compared using unpaired t test for quantitative parameters and chi-square test for qualitative parameters. A p value of less than 0.05 was taken as significant. Visual acuity, macular edema and hard exudates resolution was not significantly different in the two groups (P = 0.14, 0.62, 0.39 respectively). Atorvastatin does not affect treatment outcome in CSME with a normal lipid profile over a short term follow-up.

BackgroundDyslipidemia is associated with cardiovascular morbidities and mortality. Currently, fasting lipid profile determination is used to monitor treatment response. Recently, postprandial lipemia is of increasing interest because of its atherogenic and thrombogenic potential and also was found to be more predictive for cardiovascular diseases.ObjectivesTo demonstrate postprandial lipemia among patients with cardiovascular diseases despite low fat diet, normal fasting lipid profile and even statin regimen.Patients and MethodsPatients aged 40-80 years old with cardiovascular diseases (i.e. coronary artery disease and cerebrovascular disease) more than 6 months, on statin treatment for more than 6 months and normal fasting lipid profile (according to NCEP ATP III guidelines) were included.Study exclusion criteria were pregnancy, acute cardiovascular events < 6 months, hepatic or renal failure. Finally, twelve patients were included.ResultsThe triglyceride level showed a significant rise from fasting to 2 hours after breakfast with a mean difference of 23.86 mg/dL (P =0.012). The level peaked at 4 hours after breakfast with a mean difference (MD) of 72.02 mg/dL (P =0.002). Subsequent triglyceride levels plateaued and were significantly higher than the baseline (P <0.05) until the 12th hour of observation. VLDL levels showed a similar pattern. Levels increased significantly from fasting to 2h after breakfast (mean difference: 4.49 mg/dL, P = 0.007), then plateaued and further increased 4 hours after breakfast (MD: 14.01 mg/dL, P = 0.002). VLDL levels were significantly higher than fasting (P < 0.05) and did not return to baseline until the 12th hour of observation. In contrast, the levels of total cholesterol, HDL and LDL decreased postprandially.ConclusionsTriglyceride and VLDL peaking and plateauing were observed in patients with cardiovascular diseases despite low fat diet, normal fasting lipid profile and statin regimen. These findings may raise more attentions in monitoring and management of dyslipidemia in patients with cardiovascular and cerebrovascular events.

Introduction Anemia is commonly observed in early post-transplant period. Blood transfusion is the most frequent using procedure in correction of anemia. However early post transplant blood transfusions may increase immunological risks, antibody formation and rejection. There is little information on the impact of post-transplant transfusions in children. We aimed to evaluate effects of early post-transplant blood transfusion on graft outcome in pediatric renal transplant recipients Materials and Methods We retrospectively evaluated the data of 97 renal transplant patients. Demographic data of patients, etiology of renal failure, donor types, rejections, infections, and graft outcomes were recorded. We have documented all blood transfusions given within the first one month after transplantation. All transfused blood components were leuko-depleted. Data of patients with transfused and non-transfused were compared with respect of renal outcome. Results A total of 97 patients were included study (F/M:44/52). Mean transplant age of the patients was 14.43±4.76 years and the mean duration of follow-up was 48±12.76 months. Twenty two patients (22.6 %) had received a blood transfusion within the first month after transplantation. Transfused patients had significantly younger than non-transfused patients (9.1±3.2, 12.7±4.5 respectively, p<0.05). The median time for first blood transfusion was 2 days (0-30 days), the median number of transfusions was 1 (range:1-7) after renal transplantation. The indications of blood transfusions were acute hemorrhage, infections, and decreased Hemoglobin levels. The proportion of donor types, gender, HLA mismatch and immunosuppressive treatments were similar in both groups. Also, frequency of acute and chronic rejection, graft loss, PRA formation, first and third year GFR and graft survival were not significantly different (p>0.05) Conclusion Post transplant blood transfusions are common in children especially in young patients. Our study showed that early transfusions have no negative association with increased acute and chronic rejection, PRA production and graft loss. Further studies are needed for better evaluate the long-term risk of post-transplant blood transfusions in kidney recipients.

The Association of Early Transfusion with Acute Lung Injury in Patients with Severe Injury: Holena DN, Netzer G, Localio R. J Trauma Acute Care Surg 2012;73:825–31.

Beta-thalassemia major, a severe hereditary hemolytic anemia, is typically associated with a normal serum lipid profile. However, rare cases suggest an idiopathic association with hypertriglyceridemia, termed Hypertriglyceridemia–Thalassemia Syndrome. We report a 6-month-old male infant with beta-thalassemia major and severe hypertriglyceridemia (triglyceride level: 1994 mg/dL). Following blood transfusions and dietary modifications, triglyceride levels decreased significantly to 619 mg/dL, suggesting a self-limiting mechanism. This case underscores the importance of screening for hypertriglyceridemia in thalassemia patients to mitigate associated risks, such as atherosclerosis and thromboembolism.

Association between high-density lipoprotein cholesterol and bone mineral density and content in postmenopausal women

BackgroundSecondary hypertension accounts for 5% of all cases of hypertension. Renal artery stenosis is one of the common causes of secondary hypertension. Atherosclerosis and fibromuscular dysplasia are the commonest types of stenosis associated with renal vascular hypertension, with the former accounting for 70–80% of all cases and the latter accounting for 10% of the incidence. The greatest incidence atherosclerosis is in men over the age of 40 years, mostly affecting the proximal part of the renal arteries, whereas fibromuscular dysplasia affects women ranging in age from 30 to 50 years. Currently, possible treatments are medical treatment using blood pressure-lowering drugs, balloon angioplasty with or without stent insertion, and surgery to reconstruct the artery.Case presentationWe report a case of a 46-year-old Asian woman with stenosis of two branches of renal artery bifurcation treated by percutaneous balloon dilatation and stenting of both branches after referral to our department for a renal angiogram following 8 months of uncontrolled hypertension despite receiving medications. Initially, the patient presented with severe headache and fatigue. She was a known nonsmoker, was not diabetic, and had no history of diabetes in her family. She had no history of atherosclerosis. Apart from high blood pressure, the result of her physical examination was unremarkable. Laboratory investigations revealed normal serum cholesterol, lipid profile, and serum creatinine. She had been attending a hypertension clinic and receiving antihypertensive drugs for the past 8 months on a regular basis under close observation. Despite this treatment and care, her blood pressure remained high at 175/110 mmHg, which the attending doctor concluded to be uncontrolled blood pressure. Initial imaging indicated left renal artery stenosis, and the patient was referred to our department.ConclusionsFor patients with uncontrolled hypertension despite receiving medications, renal Doppler ultrasound should be included in the diagnostic workup for secondary hypertension. Once renal artery stenosis is suspected, renal angiography is highly recommended because the technique is able to accurately diagnose stenosis in the branch arteries, unlike computed tomographic angiography and magnetic resonance angiography. Percutaneous transluminal renal angioplasty is the treatment of choice for renal artery stenosis in patients with renovascular hypertension or renal dysfunction.