Articles: disease.
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Observational Study
Analysis of recurrence after resection of well-differentiated non-functioning pancreatic neuroendocrine tumors.
Although pancreatic neuroendocrine tumors (PNETs) are generally considered to have a favorable overall prognosis after resection, disease recurrence has been observed. Few studies have specifically addressed recurrence after resection of PNETs, especially for non-functioning PNETs (NF-PNETs). The aim of our study is to analyze the recurrence of resected well-differentiated NF-PNETs. ⋯ Multivariate analysis identified that Ki67 index ≥5% (hazard ratio [HR], 4.69; 95% confidence interval [CI], 1.36-16.75, P = .015), positive lymph nodes (HR, 6.75; 95% CI, 1.73-24.43, P = .006) were independently associated with recurrence. The 5-year disease-free survival rate was 53% (95% CI, 14.20-91.81%) for patients with Ki-67 ≥5% or (and) positive lymph nodes, while 95% (95% CI, 82.26-100%) for the patients without these 2 factors. Ki67 index and lymph node status are independently associated with recurrence after resection of well-differentiated NF-PNETs in this study.
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X-linked hyper-IgM syndrome is a type of primary combined immunodeficiency disorder caused by mutations in CD40 ligand. Opportunistic infections caused by P jirovecii, cytomegalovirus (CMV), or fungi are frequently the first presenting symptom of the patients with X-linked hyper-IgM syndrome. ⋯ Opportunistic infections should be suspected in infants presenting with severe interstitial pneumonia. Primary immune deficiency diseases should also be considered in infants diagnosed with opportunistic infections.
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Case Reports
Successful sirolimus therapy of an aplastic anemia patient with chronic kidney disease: A case report.
It is very difficult to treat patients with aplastic anemia accompanied by chronic kidney disease. The nephrotoxicity of cyclosporine limits its use in these patients. Most of these patients also lack suitable sibling donors. Sirolimus, as a new type of immunosuppressive agent, has good therapeutic effect, lower toxicity, especially lower nephrotoxicity, thus attracting the attention of hematologists. ⋯ We consider that sirolimus is a safe, effective, and well-tolerated oral drug that can be used as a treatment for aplastic anemia patients with chronic kidney disease.
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Observational Study
Clinical manifestations and genetic analysis of 4 children with chronic granulomatous disease.
Pediatricians are unfamiliar with chronic granulomatous disease (CGD) because of its rarity and paucity of available data, potentially leading to misdiagnosis, late treatments, and mortality. The main purpose of this study was to summarize the clinical manifestations and auxiliary examination findings of four children with CGD confirmed by genetic testing. This was a case series study of children hospitalized at the Pediatric Respiratory Department of Shandong Provincial Hospital. ⋯ Gene sequencing could provide molecular evidence for diagnosis. The treatments of CGD include the prevention and treatment of infections and complications. Immunologic reconstitution treatment is currently the only curative treatment for CGD.
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In China, heat-clearing and detoxifying Chinese medicines combined with conventional therapy are commonly applied to treat the mild hand, foot, and mouth disease (HFMD). However, there is lack of solid evidence on the efficacy and safety of such therapies. ⋯ In conclusion, the heat-clearing and detoxifying Chinese medicines on top of conventional therapy can effectively reduce the progressive rate of mild HFMD and improve healing of skin and oral mucosal lesions. More studies are needed for the time to fever resolution.