Most of the patients with SVT episodes require treatment with more than 1 dose of adenosine. Doses higher than the usually described in the guidelines are necessary to revert SVT. Most patients can be discharged home from the emergency department, without the need of hospital admission.
Severe combined immunodeficiency is an inherited disease with profoundly defective T cells, B cells, and natural killer cells. X-linked severe combined immunodeficiency is the most common form. In this report, we describe a 4-month-old male infant who was admitted to our hospital with progressive breathlessness and abdominal mass. He was diagnosed with hepatoblastoma and presented a pneumocystis jirovecii pneumonia at the beginning of chemotherapy. Definitive diagnosis of X-linked severe combined immunodeficiency was established by DNA analysis of the interleukin 2 receptor gamma chain gene. This case is the first report which describes an X-linked severe combined immunodeficiency patient with hepatoblastoma.
IntroductionFaecal calprotectin (FC) has been proposed as an inflamatory marker both in children and adults, with high levels registered in full-term newborn and in preterm newborn (PTNB). Nevertheless, there is scarce data about kinetics in the first month and its relation with severe illness such as systemic infections or bowel injury.AimsTo determine normal levels of FC in PTNB and relate it with feeding-type, gestational age (GA), birth-weight and delivery method. Also checking if its figures increase days before the occurrence of symptoms in bowel inflamatory alterations and systemic infections.Materials and methodsObservational clinic-analytic longitudinal prospective study through randomised selection of PTNB≤35 weeks GA, from whom faecal samples were taken in days 4, 8, 15, 30, 60 and 90 of life to determine FC (µg/g of faeces, CALPREST® from Eurospital). As significant illness we considered clinical or analytical sepsis or bowel injury/stress. All the variables of interest in the patient’s clinical history were collected. Breast-feeding was considered predominant if>80% of the feeding was reached through this method.Results369 samples were taken from 114 PTNB. Mean GA was 30.2 (±2.38) weeks, with mean weight of 1376.9 (±429.16) grams. 72 children did not suffer any clinical event, giving a total of 76 patological events in 42 newborns (36.8% from the PTNB). No statistically significant differences were found in PTNB between beeing breast-fed or with formula, nor was difference found in those of different GA, except for those with birth-weight≥1500 g or<1500 g at day 8 of life (respectively 321.05±193.01 vs 169.67±134.22).Mean FC in healthy PTNB is significantly lower than in those who suffer illness, being statistically significant between days 4 and 30, days 4 and 15 and days 8 and 15.ConclutionSA high mean of calprotectin was observed in all cases ( higher in the first 4 days, statiscally significant, slight posterior decrease and stabilisation through the first months). During intercurrent diseases (systemic infections and intestinal distress), CF increases before the onset of symptoms or elevation of acute phase reactants and it remains 8–10 days elevated after the improvement. It is possible to use it as a predictor of digestive pathology in PTRB.
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