Summary.Hydroxyurea increases fetal haemoglobin in many patients with sickle cell anaemia, but its effectiveness in thalassaemia appears to be less consistent. We describe the response to hydroxyurea in an adult male with homozygous b thalassaemia, symptomatic paraspinal extramedullary haemopoiesis, bone pain, and progressive tissue iron loading. Prior to therapy with hydroxyurea the circulating haemoglobin (Hb) concentration was 7·0 g/dl and absolute fetal haemoglobin concentration was 5·0 g/dl.Administration of sodium phenylbutyrate had induced no increase in either parameter. Subsequent therapy with hydroxyurea was associated with increases in total haemoglobin to 9·0 g/dl, and in fetal haemoglobin to 7·6 g/dl. Ineffective erythropoiesis was reduced and extramedullary haemopoiesis regressed during therapy.
Measuring RPs by this simple whole-blood cytometric technique discriminated very well between the acute ITP and non-ITP groups. This test has a strong positive predictive value and may prove very useful in the assessment of childhood acute ITP and the screening of candidates for bone marrow aspiration.
Iron deficiency and anaemia are common in pregnancy. Audit data from our tertiary obstetrics unit demonstrated 22% of maternity patients experiencing a postpartum haemorrhage received a transfusion; a third of whom were anaemic on admission intrapartum. Australian Patient Blood Management (PBM) Module 5 Obstetrics guidelines focuses on maximising red cell mass at the time of delivery and reducing the reliance on transfusion as a salvage therapy to treat blood loss. A clinical practice improvement partnership began in February 2015 and completed in April 2016; which aimed to implement systems to improve antenatal identification and management of iron deficiency, and improve postpartum anaemia management. In order to develop change strategies, reasons for poor detection and correction of iron deficiency in the antenatal period were identified following a quality improvement methodology. Education was delivered to maternity healthcare providers. Standardised algorithms and an oral iron prescription handout were developed and piloted. Follow-up audit, staff and patient feedback, and other hospital data were collected to measure outcomes. The rate of anaemia on admission intrapartum fell from 12.2% in 2013 to 3.6% in 2016 following the introduction of unselective ferritin screening and other antenatal interventions. Sixty to 70% of maternity patients screened each month had iron deficiency. The algorithms aided staff to become confident in blood test interpretation and management of iron deficiency and anaemia. Patients found the oral iron prescription handout helpful. Additionally, single unit transfusions significantly increased from 35.4% to 50% (p=0.037) over the project timeframe. This project demonstrated the potential to improve patient blood management in obstetrics, reduce anaemia and transfusions by active antenatal interventions.
Background: Small intestinal mucositis is a common side-effect following high-dose chemotherapy, causing patients to experience pain and abdominal complications often leading to extended stays in hospital. A biomarker to detect these small intestinal changes does not exist in clinical practice. This study aimed to assess the noninvasive 13 C-Sucrose breath test (SBT) to detect small intestinal damage associated with mucositis in pediatric cancer patients having chemotherapy.Patients and Methods: Small intestinal function was assessed in 15 pediatric cancer patients and 26 healthy children. Subjects were studied for small intestinal permeability (SIP; lactulose/rhamnose), digestive and absorptive capacity (SBT; sucrose), and oro-cecal transit time (OCTT; lactulose), by ingesting two sugar drinks containing the respective sugars. Combined tests were carried out at baseline, day1, day3-5 and day6-9, and in healthy individuals on two separate occasions. A total of 25 cycles of chemotherapy were assessed. Breath samples for the SBT were collected every 15 min for 3 h (expressed as % cumulative dose at 90 min (CD)), a 5 h urine collection for SIP and breath hydrogen determined every 30 min for three hours for OCTT.Results: Clinical mucositis occurred in seven of the 25 cycles of chemotherapy (28%). No significant difference was observed for SIP and OCTT. The SBT %CD at 90min was significantly lower in the mucositis group compared to the unaffected group and controls at baseline (p < 0.05). Patients who developed mucositis maintained a significantly lower %CD, for all test points (p < 0.05) compared to the unaffected patients. In patients who developed mucositis the SBT was below the reference range of the controls at all time points.Conclusion: The findings show for the first time that it is possible to noninvasively detect and monitor gut damage associated with chemotherapy-induced mucositis in pediatric cancer patients.
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