Catherine Gordon

Alarmingly high prevalence of obesity in haemophilia in the state of Mississippi.

Summary.  The state of Mississippi has consistently been ranked as the state with most number of obese people in the United States with prevalence rates of >30%. Our aims in this study were to estimate the prevalence of overweight and obesity in children and adults diagnosed with haemophilia in Mississippi, and to assess whether race/ethnicity and the severity of haemophilia are important risk factors. A retrospective chart review was performed for all haemophilic patients seen at the Mississippi Hemophilia Treatment Center. Patients were classified into two major age groups: age 2–19.9 years and ≥20 years. Body mass index (BMI) was calculated from the height and weight in kg m−2 from the last clinic visit. Out of a total of 132 haemophilic patients, 61% were white and 37% were African American. Overall, 51% of the haemophilic patients were either obese or overweight. The prevalence of obesity in the  adult (≥20 years old) haemophilic patients was 36% and an additional 32% were overweight. A significantly greater proportion of patients >20 years old were overweight or obese as compared with the patients in the 2–19.9 year age range (P < 0.002). However, race/ethnicity and severity of haemophilia were not significant risk factors for overweight and obesity. There is a very high prevalence of obesity in the Mississippi haemophilic population, especially in adults. Particular attention at clinic visits should be paid to the BMI in order to identify patients that are overweight or obese to allow for early and appropriate intervention.

The use and effectiveness of complementary and alternative medicine for pain in sickle cell anemia

Pain is the clinical hallmark for sickle cell disease (SCD). The objective of this study was to survey the extent and effectiveness of complementary and alternative medicine (CAM) use for pain control among adults with SCD. Of a total of 227 African-American adults with SCD, 208 (92%) admitted to using at least one type of CAM. The three most common types of CAM were prayer (61%), relaxation technique (44%), and massage (35%). Multiple logistic regression showed that marital status was associated with use of relaxation techniques (p = 0.044), and age between 18 and 24 years and at least a high school level of education were associated with use of prayer (p = 0.008 and p = 0.004 respectively). Our study showed that CAM use is common among adult patients with SCD. Further well designed prospective studies are needed to help develop best practices that emphasize an optimized balance of conventional and evidence based CAM therapies.

Angioedema associated with dihydropyridine calcium-channel blockers in a child with Burkitt lymphoma

PURPOSE A case of severe macroglossia and angioedema in a child with Burkitt lymphoma who was treated with two dihydropyridine calcium-channel blockers is reported. SUMMARY An eight-year-old white boy arrived at the pediatric emergency department with complaints of abdominal pain and distention after an episode of mild abdominal trauma. Physical examination results were significant for diffuse abdominal tenderness and distention, with a large palpable mass in the right quadrants. Computed tomography revealed a large abdominal mass, and a biopsy confirmed a diagnosis of Burkitt lymphoma. Before initiation of chemotherapy, the child developed tumor lysis syndrome, with subsequent renal failure and cardiorespiratory compromise. Once the patient was stabilized and sedated on mechanical ventilation, tumor-directed chemotherapy was initiated, and rapid tumor regression ensued. To control episodes of hypertension, nicardipine was initiated and titrated to achieve the blood pressure goals. Three days after initiation of nicardipine therapy, the child developed facial swelling and significant, protruding macroglossia. Eight days after nicardipine initiation, a tracheotomy was required due to upper airway obstruction; at that time, the patient was converted to amlodipine administered via nasogastric tube for continued blood pressure control. The boys macroglossia persisted for another 18 days, until a multi-disciplinary drug therapy review resulted in the discontinuation of amlodipine. Within one week of the withdrawal of amlodipine, the childs macroglossia was completely resolved. CONCLUSION An eight-year-old boy with Burkitt lymphoma developed severe macroglossia and angioedema when treated with nicardipine. The reaction persisted throughout treatment with amlodipine and resolved quickly after amlodipine was withdrawn.

Outcome of overt stroke in sickle cell anaemia, a single institution's experience

Stroke is a traumatic complication in sickle cell anaemia (SCA) that is associated with significant morbidity and a risk of recurrent overt stroke of 2·2–6·4 events per 100 patient‐years. A retrospective study was performed on all paediatric SCA patients diagnosed with a history of overt stroke between 1997 and 2010. A total of 31 children with SCA had new onset overt stroke. The mean age of the active patients (n = 27) was 17·9 years (range 6·8–27·6 years) with a total period of observation of 305 patient‐years. Twenty‐two of 27 (81%) were receiving long term red blood cell transfusions and 16 (59%) were taking the anti‐platelet agent, aspirin, since diagnosis of the stroke. Two of 27 (7%) patients had a second overt stroke with an overall risk of recurrent stroke of 0·66/100 patient‐years (one stroke was ischaemic and the other haemorrhagic). In patients taking aspirin with 180 patient‐years of follow up, the recurrence rate of haemorrhagic stroke was 0·58/100 patient‐years. We have an excellent outcome for overt stroke in paediatric SCA patients with a low rate of recurrent stroke. Further studies are needed to determine the risk‐benefit ratio of aspirin therapy in the prevention of recurrent stroke in paediatric SCA.

Complementary and Alternative Medicine Use in Pediatric Hematology/Oncology Patients at the University of Mississippi Medical Center

OBJECTIVE To examine the prevalence and modalities of complementary and alternative medicine (CAM) use in children with cancer and sickle cell disease; the reasons for use of CAM; and the use of CAM before, during, and after treatment in children with cancer. METHODS This single-center, observational study administered caregivers a written questionnaire regarding the use of CAM therapies. RESULTS A total of 101 caregivers completed questionnaires. Including prayer, total CAM use in oncology and sickle cell disease was 64% and 63%, respectively. Non-prayer CAM use was 30% in oncology and 23% in sickle cell disease. Of respondents who reported using any CAM, the three most commonly used types were prayer (62.3% oncology; 60.0% sickle cell disease), vitamins/minerals (14.8% oncology; 10.0% sickle cell disease), and massage (9.8% oncology; 7.5% sickle cell disease). The primary reasons for using CAM were to provide hope, to improve quality of life, and to lessen adverse effects. In oncology patients, CAM use tended to increase during treatment compared with before and after treatment. CONCLUSIONS The reported prevalence of non-prayer CAM use was lower (23%-30%) in this sample than has been reported in national samples or other geographic regions of the United States. Nonetheless, participants reported many positive reasons for using CAM, including to gain hope, improve quality of life, and control pain. Thus, CAM use appears to be an important aspect of medical care for many pediatric hematology/oncology families and should be a consideration when providers are discussing treatment and quality of care with families.

A rare case of peripheral neuropathy from relapse of acute lymphoblastic leukemia to the brachial plexus.

Acute lymphoblastic leukemia (ALL) is the most common cancer in children. ALL frequently involves the central nervous system and testicles, but has also been reported to metastasize to the liver and lung. We report a case of a 4-year-old African-American male with a previous history of ALL and hematopoietic stem cell transplant who presented with decreased arm movement and abdominal pain with relapse of primary disease into the brachial plexus and pancreas.

Transcriptome Analysis of Minimal Residual Disease in Subtypes of Pediatric B Cell Acute Lymphoblastic Leukemia

Acute lymphoblastic leukemia (ALL) is the most common childhood cancer and the leading cause of cancer-related death in children and adolescents. Minimal residual disease (MRD) is a strong, independent prognostic factor. The objective of this study was to identify molecular signatures distinguishing patients with positive MRD from those with negative MRD in different subtypes of ALL, and to identify molecular networks and biological pathways deregulated in response to positive MRD at day 46. We compared gene expression levels between patients with positive MRD and negative MRD in each subtype to identify differentially expressed genes. Hierarchical clustering was applied to determine their functional relationships. We identified subtype-specific gene signatures distinguishing patients with positive MRD from those with negative MRD. We identified the genes involved in cell cycle, apoptosis, transport, and DNA repair. We also identified molecular networks and biological pathways dysregulated in response to positive MRD, including Granzyme B, B-cell receptor, and PI3K signaling pathways.

Synovial sarcoma leading to a paraesophageal abscess in a child

BACKGROUND Synovial sarcoma (SS) is an uncommon malignant neoplasm arising from primitive pluripotential mesenchyme primarily affecting the soft tissues of the extremities. Rarely other locations are involved, including the mediastinum. CASE REPORT After treatment for mediastinal SS by surgical resection, radiation therapy, and chemotherapy, an 11-year-old boy developed an esophageal stricture and fistula, the latter resulting in a paraesophageal abscess. Management of the esophageal stricture and fistula required a multi-disciplinary approach. We report our experience with the management of this difficult complication, as well as a brief review of the literature on SS. CONCLUSIONS Rare conditions, particularly those with unusual complications, present therapeutic challenges requiring a multi-disciplinary team approach. Reporting experiences with difficult cases can benefit providers faced with similar problems in the future.