Atypical teratoid rhabdoid tumour (ATRT) is a malignant tumour of the central nervous system with a dismal prognosis. There is no consensus on optimal treatment and different multimodal strategies are currently being used in an attempt to improve outcomes. To evaluate the impact of high-dose chemotherapy followed by autologous stem-cell rescue (HD48 SCR), radiotherapy (RT) at first line, intrathecal chemotherapy (IT) and extent of surgical resection upon recurrence-free survival (RFS) and overall survival (OS). An online database search identified prospective and retrospective studies focused on the treatment of children and adolescents with newly diagnosed ATRT. Clinical, therapeutic and outcome data were extracted and an individual pooled data analysis was conducted. Out of 389 publications, 12 manuscripts were included in our review. Data from 332 patients were analysed. Median age at diagnosis was 37 months (range 1-231). HD-SCR, RT and IT had been administered to 28.6% (58/203), 49.6% (118/238) and 21% (65/310) of the patients, respectively. Gross total resection (GTR) had been achieved in 46.5% (152/327) of the cases. In the multivariate analysis, hazard ratios (95% Confidence Interval) for HD-SCR were: RFS-HR = 0.570 (0.357-0.910) p = 0.019, and OS-HR = 0.388 (0.214-0.704) p = 0.002; and for RT: RFS-HR = 0.551 (0.351-0.866) p = 0.01, and OS-HR = 0.393 (0.216-0.712) p = 0.002. IT and GTR were not significantly associated with improved RFS or OS in the multivariate analysis. In our pooled data review, HD-SCR and RT at first line were associated with improved outcomes in children and adolescents with newly diagnosed ATRT.
We evaluated the clinical presentation and prognosis of three children with acute glomerulonephritis (AGN) associated with pneumonia. The patient database of Niño Jesús Children's Hospital was analyzed retrospectively (1996-2007) for patients diagnosed at discharge with both pneumonia and AGN. Those with recent pharyngeal or cutaneous infection were excluded. Three patients (1.67, 4.25, and 5 years old, respectively) were selected. All of them had lobar pneumonia, developing both macrohematuria and proteinuria within 24 h after admission. Decreased glomerular filtration rate was found in one patient. Two children developed arterial hypertension and one a slight pleural effusion. Two children had low C3 levels, and one had both low C3 and C4 levels. A Streptococcus pneumoniae serotype 17F was isolated from the blood culture of one patient. On follow-up, all patients had normal blood pressure and renal function with microhematuria persisting long term. Our patients with pneumonia-associated AGN had a good prognosis for both pulmonary and renal involvement.
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