Dactylitis infant

Statistical learning of physical stimulus characteristics is important for the development of cognitive systems like language and music. Rhythm patterns are a core component of both systems, and rhythm is key to language acquisition by infants. Accordingly, the physical stimulus characteristics that yield speech rhythm in "Babytalk" may also describe the hierarchical rhythmic relationships that characterize human music and song. Computational modelling of the amplitude envelope of "Babytalk" (infant-directed speech, IDS) using a demodulation approach (Spectral-Amplitude Modulation Phase Hierarchy model, S-AMPH) can describe these characteristics...

UNLABELLED: Non-polio enteroviruses are ubiquitous viruses responsible for a wide spectrum of disease in people of all ages, although infection and illness disproportionately affect infants and young children. Hand-foot-mouth disease (HFMD) is an enteroviral clinical syndrome most frequently caused by coxsackievirus-A16 and enterovirus-A71. Since 2008, a novel coxsackievirus-A6 genotype has been associated with more severe HFMD in both children and adults, presenting with a unique constellation of findings, and whose prevalence has been increasing over the last few years...

Background & objectives: Homozygous sickle cell (SS) disease in Central India runs a more severe clinical course than reports from other areas of India. The current study was undertaken to compare the disease in Central India (Nagpur) with that in Jamaica, both populations defined by newborn screening. Methods: The Nagpur cohort included infants born to sickling-positive mothers from May 2008 to 2012, examined by high-pressure liquid chromatography and DNA analysis...

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No abstract text is available yet for this article.

Salmonella osteomyelitis involving the hand is a rare, but potentially morbid, complication of sickle cell disease in children. This entity can be difficult to distinguish from the more frequent presentation of dactylitis, but accurate diagnosis is critical to direct proper treatment. We report on a 15-month-old patient with sickle cell disease who ultimately developed osteomyelitis of 1 hand after an acute vasoocclusive episode caused 4 extremity dactylitis. The case description illustrates the diagnostic and treatment challenges...

BACKGROUND: The clinical phenotypes of children with sickle cell disease (SCD) are poorly described in many sub-Saharan countries including Nigeria. OBJECTIVES: The objective was to highlight various clinical phenotypes of SCD in children and investigate the influence of sociodemographic indices on the development of SCD complications. METHODS: We carried out a cross-sectional study of 240 pediatric patients attending the sickle cell clinic and the emergency room in a teaching hospital in South-Western Nigeria over a 12-month period...

Blistering distal dactylitis is a distinct clinical entity, generally due to S. pyogenes, unfrequently reported. Characteristically, blistering distal dactylitis is described as a localized infection involving the distal phalanx of the digits, and it usually presents as a fluid-filled blister. Between October 2009 and June 2014, 69 children (median age: 60 months, extremes: 0,6-176) were enrolled. The sensitivity of GAS rapid antigen detection test was 97 % (CI 95 %: 83-100 %), the specificity was 76 % (CI 95 %: 60-89 %), the negative predictive value was 97 % (CI 95 %: 83-100 %), and the positive predictive value 76 % (CI 95 %: 60-89 %)...

BACKGROUND: Sickle cell disease (SCD) has extremely variable phenotypes, and several factors have been associated with the severity of the disease. OBJECTIVES: To analyze the chronic complications of SCD and look for predictive risk factors for increased severity and number of complications. METHODS: Retrospective study including all children followed for SCD in the Paediatric Haematology Unit of a tertiary hospital in Portugal, who completed 17 yr old between the years 2004 and 2013...

Prior analyses of the Cooperative Study of Sickle Cell Disease (CSSCD) newborn cohort identified elevated white blood cell (WBC) count, low baseline hemoglobin and dactylitis between the ages of 1 and 2 years as markers of severe disease. Reticulocytosis was also associated with severe disease. Here, we further analyzed data collected on enrolled CSSCD infants for the predictive value of those markers for stroke and death later in life. Three hundred fifty-four CSSCD subjects were identified who had absolute reticulocyte counts (ARC) measured during infancy (2 to 6 months of age)...

BACKGROUND: The epidemiology of painful episodes in infants and younger children with SCD has not been well studied, particularly for pain managed at home. PROCEDURE: SCD infants identified by newborn screening were enrolled in a longitudinal observational study of pain symptoms requiring parents to report the presence or absence of pain daily. When sickle cell related-pain events occurred, pain occurrence, location, associated symptoms and the treatment provided also were reported...

OBJECTIVE: To compare the haematological and clinical features of homozygous sickle cell (SS) disease in Bantu and Benin haplotypes in a cross-sectional study of 115 Ugandan patients attending the Sickle Cell Clinic at Mulago Hospital, Kampala, Uganda, with 311 patients in the Jamaican Cohort Study METHODS: This involved comparison of clinical features and haematology with special reference to genetic determinants of severity including fetal haemoglobin levels, beta-globin haplotype and alpha thalassaemia status...

This study was aimed to explore the mutations of ribosomal protein (RP) genes in patients with Diamond Blackfan anemia (DBA). Twenty-one cases of DBA admitted in our hospital from Dec 2008 to Aug 2012 were screened by PCR for mutations in the nine known genes associated with DBA: RPS19, RPS24, RPS17, RPL5, RPL11, RPS7, RPL35a, RPS10 and RPS26. The results found that 8 patients (38.1%) with DBA had mutations in the genes coding for ribosomal protein, in which RPS19 mutation was identified in 3 patients, RPS24, RPS7, RPL5, RPL11 and RPL35A mutations were identified respectively in 1 of the patient...

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The Pediatric Hydroxyurea Phase 3 Clinical Trial (BABY HUG) was a phase 3 multicenter, randomized, double-blind, placebo-controlled clinical trial of hydroxyurea in infants (beginning at 9-18 months of age) with sickle cell anemia. An important secondary objective of this study was to compare clinical events between the hydroxyurea and placebo groups. One hundred and ninety-three subjects were randomized to hydroxyurea (20 mg/kg/d) or placebo; there were 374 patient-years of on-study observation. Hydroxyurea was associated with statistically significantly lower rates of initial and recurrent episodes of pain, dactylitis, acute chest syndrome, and hospitalization; even infants who were asymptomatic at enrollment had less dactylitis as well as fewer hospitalizations and transfusions if treated with hydroxyurea...

OBJECTIVES: To analyse demographic and clinical variables in patients with disease onset before and after 40, 45 and 50 years in a large series of Brazilian SpA patients. METHODS: A common protocol of investigation was prospectively applied to 1424 SpA patients in 29 centres distributed through the main geographical regions in Brazil. The mean age at disease onset was 28.56 ± 12.34 years, with 259 patients (18.2%) referring disease onset after 40 years, 151 (10...

The BABYHUG study is the first randomized controlled prospective trial of hydroxycarbamide in infants with sickle cell anemia to look at its effect on organ function. Almost 200 patients were recruited, irrespective of disease severity, over a 3-year period and followed for 2 years. The primary end points of splenic function and glomerular filtration rate were not reached. The trial did show a benefit in frequency of pain, dactylitis, acute chest syndrome, hospital admissions and transfusion requirements. Adverse effects were low...

PURPOSE OF REVIEW: The International League of Associations for Rheumatology criteria parse out juvenile idiopathic arthritis (JIA) into seven groups, with the aim of creating homogeneous subgroups suitable for clinical and research evaluation. However, prior studies have shown that psoriatic JIA (psJIA) may be a heterogeneous entity. RECENT FINDINGS: PsJIA is composed of two subgroups, differentiated by age at onset. Older children with psJIA have features of spondyloarthritis, including relative male preponderance, increased risk of axial involvement, and enthesitis...

BACKGROUND: Sickle-cell anaemia is associated with substantial morbidity from acute complications and organ dysfunction beginning in the first year of life. Hydroxycarbamide substantially reduces episodes of pain and acute chest syndrome, admissions to hospital, and transfusions in adults with sickle-cell anaemia. We assessed the effect of hydroxycarbamide therapy on organ dysfunction and clinical complications, and examined laboratory findings and toxic effects. METHODS: This randomised trial was undertaken in 13 centres in the USA between October, 2003, and September, 2009...

AIM: To describe the severity of sickle cell disease (SCD) in newborns in Belgium and evaluate the impact of neonatal screening (NS) on clinical outcome. METHODS: Universal NS of umbilical cord blood for hemoglobinopathy was progressively deployed in Brussels and Liège starting in 1994. No particular population was targeted. Samples were analyzed initially using the isoelectric focusing technique and since 2008 the capillary electrophoresis technique. If a hemoglobin variant was suspected, further analysis was carried out using high performance liquid chromatography...