@article {10513, title = {Does the Application of Heat Gel Pack After Eutectic Mixture of Local Anesthetic Cream Improve Venipuncture or Intravenous Cannulation Success Rate in Children? A Randomized Control Trial.}, journal = {Pediatr Emerg Care}, volume = {34}, year = {2018}, month = {2018 Feb}, pages = {e24-e27}, abstract = {

OBJECTIVE: Needle-related procedures are the most common sources of pain for children in the hospital setting. The most used topical anesthetic, eutectic mixture of local anesthetic (EMLA) cream, may cause transient vasoconstriction. It has been postulated that this vasoconstriction may decrease vein visualization. The application of heat gel pack after removal of EMLA cream in the site of venipuncture counteracts the vasoconstriction, improving vein visualization. We assessed using a prospective randomized controlled trial whether the application of heat gel pack increases the needle procedure success rate. The primary study outcome was procedural success rate at the first attempt.

METHODS: The study enrolled 400 children, 200 of whom applied heat gel pack after removing EMLA (treatment group) and 200 did not (control group). Procedural success rate at the first attempt, vein perception before procedure, procedural pain, and adverse events were recorded in both groups.

RESULTS: Eighty-eight percent of the procedures were successful at the first attempt in the treatment group and 89\% in the control group (P = 0.876). Vein perception was not significantly different in the 2 groups (P = 0.081). Pain score after the procedure was similar in the 2 groups.

CONCLUSIONS: This study shows that the application of heat gel pack after removal of EMLA cream does not improve venipuncture or intravenous cannulation success rate.

}, keywords = {Anesthetics, Local, Child, Child, Preschool, Female, Hot Temperature, Humans, Lidocaine, Lidocaine, Prilocaine Drug Combination, Male, Pain, Pain Management, Phlebotomy, Prilocaine, Prospective Studies}, issn = {1535-1815}, doi = {10.1097/PEC.0000000000001248}, author = {Schreiber, Silvana and Cozzi, Giorgio and Patti, Giuseppa and Taddio, Andrea and Montico, Marcella and Pierobon, Chiara and Barbi, Egidio} } @article {8483, title = {Describing Kawasaki shock syndrome: results from a retrospective study and literature review.}, journal = {Clin Rheumatol}, volume = {36}, year = {2017}, month = {2017 Jan}, pages = {223-228}, abstract = {

Kawasaki shock syndrome (KSS) is a rare manifestation of Kawasaki disease (KD) characterized by systolic hypotension or clinical signs of poor perfusion. The objectives of the study are to describe the main clinical presentation, echocardiographic, and laboratory findings, as well as the treatment options and clinical outcomes of KSS patients when compared with KD patients. This is a retrospective study. All children referred to two pediatric rheumatology units from January 1, 2012, to December 31, 2014, were enrolled. Patients were divided into patients with or without KSS. We compared the two groups according to the following variables: sex, age, type of KD (classic, with less frequent manifestations, or incomplete), clinical manifestations, cardiac involvement, laboratory findings, therapy administered, response to treatment, and outcome. Eighty-four patients with KD were enrolled. Of these, five (6~\%) met the criteria for KSS. Patients with KSS had higher values of C-reactive protein (p = 0.005), lower hemoglobin levels (p = 0.003); more frequent hyponatremia (p = 0.004), hypoalbuminemia (p = 0.004), and coagulopathy (p = 0.003); and increase in cardiac troponins (p = 0.000). Among the KSS patients, three had a coronary artery involvement, but none developed a permanent aneurysm. Intravenous immunoglobulin resistance was more frequent in the KSS group, although not significantly so (3/5, 60~\% vs. 23/79, 30~\%, P = NS). None of the five cases was fatal, and all recovered without sequelae. KSS patients are more likely to have higher rates of cardiac involvement. However, most cardiovascular abnormalities resolved promptly with therapy.

}, keywords = {C-Reactive Protein, Child, Child, Preschool, Echocardiography, Female, Heart Failure, Hemoglobins, Humans, Immunoglobulins, Intravenous, Male, Mucocutaneous Lymph Node Syndrome, Retrospective Studies, Shock, Syndrome}, issn = {1434-9949}, doi = {10.1007/s10067-016-3316-8}, author = {Taddio, Andrea and Rossi, Eleonora Dei and Monasta, Lorenzo and Pastore, Serena and Tommasini, Alberto and Lepore, Loredana and Bronzetti, Gabriele and Marrani, Edoardo and Mottolese, Biancamaria D{\textquoteright}Agata and Simonini, Gabriele and Cimaz, Rolando and Ventura, Alessandro} } @article {8030, title = {Delayed reactivation of chronic infantile neurologic, cutaneous, articular syndrome (CINCA) in a patient with somatic mosaicism of CIAS1/NLRP3 gene after withdrawal of anti-IL-1 beta therapy.}, journal = {Clin Exp Rheumatol}, volume = {33}, year = {2015}, month = {2015 Sep-Oct}, pages = {766}, keywords = {Antibodies, Monoclonal, Carrier Proteins, Cryopyrin-Associated Periodic Syndromes, Drug Administration Schedule, Female, Genetic Predisposition to Disease, Humans, Immunosuppressive Agents, Interleukin 1 Receptor Antagonist Protein, Interleukin-1beta, Mosaicism, Phenotype, Recurrence, Remission Induction, Time Factors, Treatment Outcome, Young Adult}, issn = {0392-856X}, author = {Paloni, Giulia and Pastore, Serena and Tommasini, Alberto and Lepore, Loredana and Taddio, Andrea} } @article {7780, title = {Different presentations of mevalonate kinase deficiency: a case series.}, journal = {Clin Exp Rheumatol}, volume = {33}, year = {2015}, month = {2015 May-Jun}, pages = {437-42}, abstract = {

OBJECTIVES: We aimed to raise awareness among paediatricians and physicians about this often misunderstood condition.

METHODS: We discussed the clinical profiles associated with late or wrong diagnosis of mevalonate kinase deficency (MKD) in a single centre case series.

RESULTS: We analysed the most common challenges and pitfalls that a clinician might face during the diagnostic process. Five main clinical profiles were characterised.

CONCLUSIONS: We propose a new perspective on MKD, suggesting that the presentation of this disease can vary from patient to patient.

}, keywords = {Age Factors, Bacterial Infections, Child, Child, Preschool, Diagnosis, Differential, Diagnostic Errors, Female, Genetic Predisposition to Disease, Humans, Infant, Inflammatory Bowel Diseases, Male, Mevalonate Kinase Deficiency, Phenotype, Predictive Value of Tests, Recurrence, Risk Factors, Sepsis, Vasculitis, Young Adult}, issn = {0392-856X}, author = {De Pieri, Carlo and Taddio, Andrea and Insalaco, Antonella and Barbi, Egidio and Lepore, Loredana and Ventura, Alessandro and Tommasini, Alberto} } @article {1861, title = {Dealing with abdominal pain in children affected by systemic lupus erythematosus.}, journal = {Semin Arthritis Rheum}, volume = {41}, year = {2012}, month = {2012 Feb}, pages = {e3-4}, keywords = {Abdominal Pain, Female, Humans, Lupus Erythematosus, Systemic, Male}, issn = {1532-866X}, doi = {10.1016/j.semarthrit.2011.05.002}, author = {Taddio, Andrea and Ros{\'e}, Carlos and Lepore, Loredana and Ventura, Alessandro} } @article {1680, title = {Defective FOXP3 expression in patients with acute Kawasaki disease and restoration by intravenous immunoglobulin therapy.}, journal = {Clin Exp Rheumatol}, volume = {28}, year = {2010}, month = {2010 Jan-Feb}, pages = {93-7}, abstract = {

OBJECTIVES: The aims of this study were: 1) to investigate forkhead box P3 (FOXP3) expression in patients with Kawasaki disease (KD), exploring possible differences during the acute phase and after defervescence; 2) to evaluate a possible association of the FOXP3 single nucleotide polymorphism (SNP) 543 (SNP ID: rs2232367) with KD.

METHODS: FOXP3 expression was evaluated on 8 children with KD and 15 healthy children by flow cytometry and Real-Time polymerase chain reaction (RT-PCR). FOXP3 SNP 543 was genotyped by denaturing high-performance liquid chromatography (DHPLC) and sequencing on DNA samples from 58 additional children with KD and 114 healthy donors.

RESULTS: The frequencies of CD4+CD25 +FOXP3+ regulatory T cells were significantly (p=0.0002) lower during the acute phase of KD than in sex- and age-matched healthy donors (median \% + SD: 4.8+/-1.3 vs. 7.7+/-1.7) and a similar tendency was revealed for FOXP3 mRNA transcripts (p<0.0001). FOXP3 expression increased significantly, at both protein and mRNA levels, after intravenous immunoglobulin (IVIG) therapy treatment and achieving complete remission of disease (at least 48 hrs; median 9.5 days, range 2-30). Of the 58 patients screened, only one female subject (1.7\%) carried the presence of 543 SNP in heterozygosis (C>T; for a total of 1 allele out of 88), with no difference between KD patients and controls (0.0\%, 0/203 alleles).

CONCLUSIONS: Our data reinforce the notion of an impaired immunoregulation in KD, suggesting also a role of IVIG treatment in modifying the Treg compartment. FOXP3 SNP 543 does not seem to be involved in susceptibility to KD in Italian children.

}, keywords = {Acute Disease, Child, Child, Preschool, Female, Flow Cytometry, Forkhead Transcription Factors, Genotype, Humans, Immunoglobulins, Intravenous, Infant, Italy, Male, Mucocutaneous Lymph Node Syndrome, Polymorphism, Single Nucleotide, Reverse Transcriptase Polymerase Chain Reaction, RNA, Messenger, T-Lymphocytes, Regulatory}, issn = {0392-856X}, author = {Olivito, Biagio and Taddio, Andrea and Simonini, Gabriele and Massai, Cristina and Ciullini, Sara and Gambineri, Eleonora and de Martino, Maurizio and Azzari, Chiara and Cimaz, Rolando} }