MIBG at doses up to 18 mCi/kg can be safely administered 6 weeks prior to a BuMel consolidative regimen for refractory neuroblastoma. Preceding MIBG did not impair engraftment following BuMel. This regimen is being further evaluated in a Children's Oncology Group (COG) trial.
Depending upon the method of measurement, hypocalcemia occurs in 15% to 88% and hypercalcemia occurs in 15% of hospitalized patients. Ionized calcium should be measured in place of total serum calcium to avoid errors related to hypoalbuminemia, which is seen commonly in acutely ill patients. Symptomatic hypocalcemia requires prompt intravenous calcium administration. Symptomatic hypercalcemia (most often caused by hyperparathyroidism or malignancy) warrants aggressive intravenous hydration to correct volume depletion and, usually, additional therapy with diuretics, bisphosphonates, calcitonin, or corticosteroids. Identification and treatment of the underlying cause of the calcium derangement must be addressed after the acute electrolyte abnormality is stabilized.
Scleroderma-associated pulmonary arterial hypertension (SSc-PAH) is associated
with worse outcome than idiopathic pulmonary arterial hypertension (IPAH),
potentially due to worse right ventricular adaptation to load as suggested by
pressure–volume loop analysis. The value of non-invasive load-adaptability
metrics has not been fully explored in SSc-PAH. This study sought to assess
whether patients with incident SSc-PAH have worse echocardiographic
load-adaptability metrics than patients with IPAH. Twenty-two patients with
incident SSc-PAH were matched 1:1 with IPAH based on pulmonary vascular
resistance. Echocardiographic load-adaptability indices were divided into:
surrogates of ventriculo-arterial coupling (e.g. right ventricular area
change/end-systolic area), indices reflecting the proportionality of load
adaptation (e.g. tricuspid regurgitation velocity-time integral normalized for
average right ventricular radius), and simple ratios (e.g. tricuspid annular
plane systolic excursion/right ventricular systolic pressure). The prognostic
value of these indices for clinical worsening (i.e. death, transplant, or
hospitalization for heart failure) at one year was explored. The two groups were
comprised of patients of similar age, with similar cardiac index, pulmonary
resistance, capacitance and NT-proBNP levels (p > 0.10).
There was no difference in baseline right ventricular dimension, function or
load-adaptability indices. At one year, eight (36.4%) SSc-PAH patients had
experienced clinical worsening (eight hospitalizations and two deaths) versus
one hospitalization in the IPAH group. Load adaptation at one year in survivors
was not worse in SSc-PAH (p > 0.33). Patients with IPAH
responded better to therapy than SSc-PAH in terms of reduction of right
ventricular areas at one year (p < 0.05). Right ventricular
load-adaptability echocardiographic indices do not appear to capture the
increased risk of negative outcomes at one year associated with SSc-PAH.
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