Congenital hyperinsulinism (CHI) is a common cause of hypoglycaemia due to unregulated insulin secretion from pancreatic β cells. Medical management includes use of oral diazoxide (a KATP channel agonist) and daily injectable octreotide (somatostatin analogue) therapy. However, diazoxide is associated with severe sideeffects such as coarse facies, hypertrichosis and psychosocial/compliance issues in adolescents. Lanreotide (a long-acting somatostatin analogue) is used in adults with neuroendocrine tumours; however, its role in patients with CHI has not been well described. A 15-year-old girl with diazoxide-responsive CHI had severe hypertrichosis secondary to diazoxide and subsequent compliance/psychosocial issues. She was commenced on 30 mg of lanreotide every 4 weeks as a deep subcutaneous injection, in an attempt to address these issues. She was able to come off diazoxide treatment 2 months after starting lanreotide. Presently, after 2.5 years of lanreotide treatment, her blood glucose control is stable with complete resolution of hypertrichosis. Clinically significant improvements in the self-reported Paediatric Quality of Life (PedsQL) questionnaire and Strengths and Difficulties Questionnaire (SDQ) were reported after 1 year on lanreotide. No side effects were found, and her liver/thyroid function and abdominal ultrasound have been normal. We report the first case on the use of lanreotide in an adolescent girl with diazoxide-responsive CHI with significant improvement of quality of life.
Multiple myeloma (MM) is a progressive plasma cell malignancy, with a range of clinical features including bone lesions, renal insufficiency, anaemia, and hypercalcaemia. Novel agents have significantly improved patient survival, however most patients will suffer multiple relapses. Although clinical challenges and economic costs of relapse are recognised, the psychological impact of relapse is not fully appreciated. Additionally, there is little information on how physicians perceive the impact of relapse on their patients' emotional state and how this might affect patient management. Through face-to-face interviews with 50 relapsed and/or refractory MM patients and 30 haematologists across ten countries, we have used real-world evidence to explore and characterise the burden of living with MM, particularly the impact of relapsed disease. This exploratory study illustrates the impact of the disease on friends and family, and the physical and emotional burden experienced by the patient resulting from both MM and its treatment. Haematologists feel poorly equipped to deal with the emotional aspects of patient relapse, lacking the time and resources to adequately deal with these issues. Focused educational and support tools/resources targeted at both physicians and patients are required to facilitate physician-patient communication to help reduce the emotional burden of living with MM.
OBJECTIVES Pediatric behavioral health admissions to children’s hospitals for disposition planning are steadily increasing. These children may exhibit violent behaviors, which can escalate to application of physical limb restraints for safety. Using quality improvement methodology, we sought to decrease physical restraint use on children admitted to our children’s hospital for behavioral health conditions from a baseline mean of 2.6% of behavioral health patient days to <1%. METHODS We included all children ≥3 years of age admitted to our hospital medicine service with a primary behavioral health diagnosis from July 1, 2016, to February 1, 2020. A multidisciplinary team, formed in July 2018, tested interventions based on key drivers targeted toward our aim. The primary outcome measure was the percent of behavioral health patient days on which physical restraints were ordered. The balancing measure was the percent of patient days with a staff injury event. Statistical process control charts were used to view and analyze data. RESULTS Our cohort included 3962 consecutive behavioral health patient encounters, encompassing a total of 9758 patient days. A 2-year baseline revealed physical restraint orders placed on 2.6% of behavioral health patient days, which was decreased to 0.9% after interventions and has been sustained over 19 months without any change in staff injuries. CONCLUSIONS Team-based quality improvement methodology was associated with a sustained reduction in physical restraint use on children admitted for behavioral health conditions to our children’s hospital. These results indicate that physical restraint use can be safely reduced in children’s hospitals.
Postural Orthostatic Tachycardia Syndrome should not be a contraindication to pregnancy. Symptom course is variable during pregnancy and the post-partum period. Continuing pre-conception medication may help symptoms, with no significant risks reported. Obstetric complications, not Postural Orthostatic Tachycardia Syndrome, should dictate mode of delivery. Postural Orthostatic Tachycardia Syndrome did not appear to affect the rate of adverse events. These results are important in determining appropriate management and care in this population.
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