Search

Background: A retrospective study was conducted in order to investigate and describe the characteristics of Immunoglobulin A vasculitis (IgAV), previously known as Henoch-Schӧnlein purpura, in the paediatric population of a community-based healthcare delivery system in the Italian region of Abruzzo., Methods: This is a population-based retrospective chart review of the diagnosis of IgAV in children ages 0 to 18, admitted to the Department of Paediatrics of Chieti and Pescara between 1 January 2000 and 31 December 2016. All children enrolled presented with clinical symptoms and laboratory findings and met the EULAR/PRINTO/PRES 2008 criteria., Results: Two-hundred-eight children met the criteria for IgAV, with the highest incidence reported among children below 7-years of age. A correlation with recent infections was found in 64% of the cohort; the onset was more frequently during the winter and fall. Purpura had a diffuse distribution in the majority of patients; joint impairment was the second most frequent symptom (43%), whereas the gastrointestinal tract was involved in 28% of patients., Conclusions: Hereby, we confirm the relative benignity of IgAV in a cohort of Italian children; with regards to renal involvement, we report a better outcome compared to other studies. However, despite the low rate of renal disease, we observed a wide use of corticosteroids, especially for the treatment of persistent purpura., (© 2021. The Author(s).)

This multicenter, open-label, phase I study assessed the safety and antitumor activity of acalabrutinib in Japanese patients with relapsed/refractory (r/r) B-cell malignancies. Parts 1 (dose confirmation) and 2 (dose expansion) of this three-part study are reported. Treatment was a single dose of 100 mg acalabrutinib (day 1), followed by a washout period and then twice daily 100 mg acalabrutinib in part 1, or twice daily 100 mg acalabrutinib in part 2. Patients from parts 1 and 2 with r/r chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL), and r/r mantle cell lymphoma (MCL) were assessed as r/r CLL/SLL and r/r MCL cohorts, respectively. Twenty-five patients received treatment (part 1, n = 6). Median age was 71.0 years. Nine (one patient from part 1) and 13 (two patients from part 1) patients were included in the r/r CLL/SLL and r/r MCL cohorts, respectively. Treatment-related adverse events (AEs) occurred in 88% of patients (grade ≥3, 36%); the most common were headache (28%) and purpura (24%), both grade 1/2. No AEs resulted in treatment discontinuation or death. Median duration of treatment was 31, 20, and 7 months for part 1, r/r CLL/SLL cohort, and r/r MCL cohort, respectively. Overall response rate (ORR) was 89% and 62% for the r/r CLL/SLL and r/r MCL cohorts, respectively. The median progression-free survival (PFS) was not reached for the r/r CLL/SLL cohort and was 7 months for the r/r MCL cohort. Acalabrutinib (100 mg twice daily) was generally safe and well-tolerated in adult Japanese patients with B-cell malignancies., (© 2021 The Authors. Cancer Science published by John Wiley & Sons Australia, Ltd on behalf of Japanese Cancer Association.)

Background: The evaluation of children with purpuric rash and fever (PRF) is controversial. Although many of them have viral infections, on occasion such patients may be infected with Neisseria meningitidis. We described all children aged 0-18 years with PRF in southern Israel during the period 2005 ̶ 2016 and compared their microbiologic, laboratory, clinical and outcome characteristics in relation to various etiologies of this syndrome., Methods: Data were summarized from electronic patient and microbiology files. Viral diagnoses were made by serology and/or PCR., Results: Sixty-nine children with PRF were admitted; 30 (43.48%), 9 (13.04%) and 30 (43.48%) had a syndrome of bacterial, viral or non-established etiology, respectively. N. meningitidis infection was diagnosed in 16/69 (23.19%) patients and in 16/30 (53.33%) patients with bacterial etiology; 14/30 (46.67%) patients suffered from a non-invasive bacterial disease (9 with Rickettsial disease). Adenovirus and Influenza B (3 and 2 cases, respectively) represented the most frequent etiologic agents among patients with viral etiology. More patients with PRF of bacterial etiology were older, of Bedouin ethnicity, looked ill on admission, had higher rates of meningitis and were treated more frequently with antibiotics compared with patients with non-bacterial PRF. Fatality rates among patients with bacterial, viral and non-established etiology were 5/30 (16.7%), 0% and 2/39 (5.1%)., Conclusions: Although PFR was uncommon, high rates of meningococcal infections were recorded in children with PRF, which was associated with high fatality rates. Rickettsial infections were frequent, emphasizing the need for a high index of suspicion for this disease in endemic geographic areas., (Copyright © 2019. Published by Elsevier B.V.)

Objectives: Onset of primary SS is usually between 40 and 60 years of age, with severe systemic complications in 15% of cases. We sought to determine whether early-onset disease is related to a specific phenotype and if it is predictive of a poor outcome., Methods: Biological and clinical data from 393 patients recruited in the ASSESS cohort, a French multicentre prospective cohort, were compared according to age at diagnosis., Results: Fifty-five patients had early-onset disease, defined as age ⩽35 years at diagnosis, and presented a significantly higher frequency of salivary gland enlargement (47.2% vs 33.3%, P = 0.045), adenopathy (25.5% vs 11.8%, P = 0.006), purpura (23.6% vs 9.2%, P = 0.002) and renal involvement (16.4% vs 4.4%, P = 0.003). They had a higher frequency of hypergammaglobulinaemia (60.8% vs 26.6%, P < 0.001), RF positivity (41.5% vs 20.2%, P < 0.001), low C3 level (18.9% vs 9.1%, P = 0.032), low C4 level (54.7% vs 40.2%, P = 0.048) and autoantibodies [84.6% with anti-SSA vs 54.4% (P < 0.001) and 57.7% with anti-SSB vs 29.7% (P < 0.001)]. The change in ESSDAI scores between baseline and the 5-year follow-up was significantly different (P = 0.005) with a trend for worsening in the early-onset group (0.72, P = 0.27) and a significant improvement in the later onset group (-1.27, P < 0.0001)., Conclusion: Early-onset primary SS is associated with a specific phenotype defined by clinical and biological features known to be predictive factors of severe systemic disease. Interestingly, we showed a different evolution of the ESSDAI score depending on the age at disease onset, patients with early-onset disease tending to worsen over time., (© The Author(s) 2018. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Introduction: Febrile purpura (FP) is suggestive of meningococcal disease, requiring almost always further investigations and a treatment based on broad spectrum antibiotics. This study aimed to determine the incidence of meningococcal infections as well as their associated clinical signs in children with febrile purpura hospitalized in the emergency department., Methods: We conducted a descriptive, retrospective study in the pediatric emergency department at the Children's Hospital of Casablanca over a period of 3 years. The hospitalized children with FP who had undergone bloodculture, whether or not associated with lumbar puncture, were included in the study. Statistical analysis was performed using SPSS v.16 software., Results: We enrolled 96 children, 49 boys and 47 girls. The average age was 53.3 ± 40.5 months. Mean body temperature was 38.9°C. Meningococcal infection was diagnosed in 35/96 children. The diagnosis of meningococcemia was retained in 22 children, associated with meningitis in four patients. Symptoms and physical signs significantly associated with meningococcal infection included lethargy (p = 0.04), convulsions (p = 0.01) and purpura occurring outside the skin area drained by the superior vena cava (p = 0.01)., Conclusion: FP occurring outside the skin area drained by the superior vena cava or associated with convulsions is srongly related to meningococcal infection, whose incidence seems to be high among Moroccan children.

Objective: To determine the nationwide incidence of non-traumatic subconjunctival hemorrhage (NTSCH) in Taiwan from 2000-2011 and to analyze the risk factors for NTSCH using a case-control analysis., Methods: This is a population-based cohort administrative database study. Randomly selected 1,000,000 residents from the Taiwan National Health Insurance Research Database in 2000 and followed for 12 years to determine the population incidence of NTSCH. Individuals with the first diagnosis of subconjunctival hemorrhage were identified by the corresponding International Classification of Diseases code (ICD) 372.72. Traumatic subconjunctival hemorrhages (ICD-9 codes 921, 871, 850-854 and 959.01) were excluded. The association of NTSCH with various demographic factors, comorbidities and use of medications was studied by a population based case-control analysis using data of 2008-2011., Results: A total of 67,720 patients with a first-time diagnosis of NTSCH were identified during the 12 years period. The mean annual incidence was 65 per 10,000 individuals (crude incidence) and 60 per 10,000 individuals (age- and sex-standardized incidence). The incidence rate of NTSCH was higher in women than in men, [men-women ratio: 0.80 (95% confidence interval: 0.78-0.81)]. The age-specific incidence decreased from childhood to the group of teenagers, after which it increased to a maximal value of 136.2 per 10,000 people in the age group of 60-69 years. Case-control analysis showed that comorbidities of hypertension, purpura and thrombocytopenia, and the use of aspirin were significantly associated with the risk of NTSCH., Conclusions: This study indicates that NTSCH is a common eye disease that occurs once in 167 individuals in a general East Asian population per year. It occurs more often in women than in men and the age-specific incidence peaked in the age group of 60-69 years. Hypertension, purpura and thrombocytopenia, and the use of aspirin are the major risk factors for NTSCH.

Periodic transfusions and effective chelation have ensured that thalassemics survive in to adulthood but their life is punctuated by peculiar problems in adulthood. Three cases of scurvy are being reported presenting uniquely as purpura, right hip joint effusion and right knee joint effusion with haemorrhage in prepatellar and retropatellar bursae, respectively over an 18 month period (2009-2010). The first two cases did give a history of gum bleed. None had any coagulation disturbance or transfusion-transmitted infections or connective tissue disorder. All the three cases responded dramatically to vitamin C supplementation. It is imperative to keep in mind that recurrent blood transfusions are associated with a state of subclinical vitamin C deficiency and overt scurvy may manifest as cumulative number of transfusions increase, as in adult thalassemics.

Background: The gastrointestinal manifestations are the adverse events mainly studied to the application of the vaccine against rotavirus., Material and Methods: In order subsequent to consider the risk of associated events taken care of the vaccination against rotavirus in infants, we realized a retrospective study of 2 years, evaluating 7,691,757 distributed doses of oral vaccine, in 2008 and 2009., Results: The risk considered of an event associated after the application of the oral vaccine against rotavirus is of 2.9 events by 1,000,000 distributed doses., Conclusions: The taken care of associated events were little common subsequent to the vaccination against rotavirus.

We assessed the dermatological manifestations associated with chronic hepatitis C virus (HCV) infection and their association with liver status. Of 155 patients with chronic HCV infection in Cairo, Egypt, 71 (45.8%) had dermatological manifestations: pruritus without evident skin lesions (21.3%), pigmented purpuric eruption (5.2%), aphthous ulcer and lichen planus (3.9% each), leukocytoclastic vasculitis (2.6%), psoriasis (1.9%), tinea versicolor (1.3%) and other conditions (5.8%). Shrunken liver, splenomegaly and ascites were significantly associated with the presence of skin lesions (relative risk 8.0, 2.7 and 1.8 respectively), and shrunken liver was significantly associated with pruritus (relative risk 2.1). Sex was not associated with any of the skin lesions.

Objective: To determine the clinical profile, etiologic factors, disease associations, and the laboratory profile of adult patients with cutaneous leukocytoclastic vasculitis (LV) presenting to the Dermatology Department at Farwaniya Hospital, Kuwait., Subjects and Methods: The medical records of 57 adult patients (>18 years) with biopsy-proven cutaneous LV from January 2004 to June 2006 at Farwaniya Hospital were reviewed., Results: Of the 57 patients studied, 21 (36.8%) were classified as having primary cutaneous small vessel vasculitis. The most common type of skin lesions found was palpable purpura seen in 29 (50.9%) patients. No possible cause for the cutaneous vasculitis could be identified in 21 patients. Extracutaneous involvement was seen in 32 (56.1%) patients of which 29 had joint involvement. The most frequent laboratory abnormality was an elevated erythrocyte sedimentation rate (ESR). Altered laboratory evaluation in the form of leukocytosis, raised antistreptolysin O titers and urine abnormalities were found in association with systemic vasculitis. IgA deposits were present in 33 patients. Risk factors for chronicity of cutaneous LV were female sex, advanced age, unidentified etiology, absence of fever and presence of joint involvement., Conclusion: The commonest type of LV in Kuwait is primary cutaneous small vessel vasculitis. The most frequent laboratory abnormality associated with LV was increased ESR., ((c) 2007 S. Karger AG, Basel.)

Aims: To determine the prevalence of petechial spots in well babies., Methods: A total of 116 babies under the age of 12 months were fully examined at child health surveillance clinics. The number and site of petechiae were recorded together with details of possible causes., Results: A total of 27.6% of babies had one or more petechiae, 8.6% had two or more petechiae, and 2.6% had more than two. None of these babies subsequently developed sepsis., Conclusions: Many well infants examined in the community are likely to have petechial spots. In this setting one or two petechiae are common and their presence should not be taken as pathological without other clinical signs. Recognition of this fact may also be helpful when examining otherwise well infants with petechiae in a secondary care setting.

Background: Fresh intrapulmonary and oronasal haemorrhages in cases of sudden infant death syndrome (SIDS) might be markers for accidental or intentional smothering inappropriately diagnosed as SIDS., Aim: To compare the incidence, epidemiological association, and inter-relation of nasal haemorrhage, intrapulmonary haemorrhage, and intrathoracic petechiae in infant deaths certified as SIDS., Methods: In SIDS cases from a large nationwide case-control study, a wide range of variables were compared in cases with and without reported nasal haemorrhage and, in a subgroup of cases, in those with and without pathologically significant intrapulmonary haemorrhage., Results: Nasal haemorrhage was reported in 60 of 385 cases (15%) whose parents were interviewed. Pathologically significant intra-alveolar pulmonary haemorrhage was found in 47% of 115 cases studied, but was severe in only 7%. Infants with nasal haemorrhage had more haemorrhage into alveoli and air passages than age matched cases without nasal haemorrhage. In multivariate analysis, nasal haemorrhage was associated with younger infant age, bed sharing, and the infant being placed non-prone to sleep. Intrapulmonary haemorrhage was associated with the same three factors in univariate analysis, but in multivariate analysis only younger infant age remained statistically significant. There was no significant association between nasal or intra-alveolar haemorrhages and intrathoracic petechiae., Conclusions: Nasal and intrapulmonary haemorrhages have common associations not shared with intrathoracic petechiae. Smothering is a possible common factor, although is unlikely to be the cause in most cases presenting as SIDS.

Brazilian purpuric fever (BPF) is a life-threatening pediatric infection that is preceded by conjunctivitis and caused by Haemophilus influenzae biogroup aegyptius. BPF was first recognized during 1984 in the state of Sao Paulo. BPF was not reported in Brazil outside of Sao Paulo and the neighboring state of Parana until December 1989, when children with BPF were identified in the state of Mato Grosso. By April 1990, 10 children with confirmed BPF were identified from six widely separated cities in Mato Grosso. The overall attack rate for the combined population was 6/100,000 children less than 10 years of age. Six (60%) of the 10 children with BPF died. The recognition of BPF in Mato Grosso suggests that the H. influenzae biogroup aegyptius strain responsible for BPF has a wider geographic distribution than previously appreciated or may be capable of spreading.

A case control model was used in the study of an outbreak of Brazilian purpuric fever BPF which occurred in Serrana, S. Paulo State, Brazil, in 1986. Three hypotheses were raised: 1--purulent conjunctivitis is associated with BPF; 2--a cluster effect occurs in BPF; 3--respiratory symptoms may be a variation of the clinical picture of the disease. Numerical values were attributed to different findings, as follows: fever = 5; diarrhea and/or vomiting = 1; haemorrhagic findings = 3; thrombocytopenia and/or leukopenia = 3; Haemophilus aegyptius positive hemoculture and/or Haemophilus aegyptius positive cerebrospinal fluid culture and/or H. a. oropharynx culture = 7; Waterhouse Friedrichsen syndrome = 7. Those cases for which the sum total of points reached or exceeded 13 were considered as confirmed and those obtaining between 8 and 12 were considered as suspect. Children with a score below 5 were taken as control cases. Cases and controls were matched according to sex, age and socioeconomic level. The total groups studied included 14 confirmed cases, 38 suspect cases and 78 controls. It was concluded that purpuric fever is strongly associated with previous and/or present purulent conjunctivities; a cluster effect seems to occur; respiratory symptoms such as coughing and/or coryza were not associated with BPF.

Brazilian purpuric fever (BPF) is a life-threatening pediatric infection that is preceded by conjunctivitis and caused by a specific strain of Haemophilus influenzae biogroup aegyptius (BPF clone). BPF was recognized during 1984 in the state of Sao Paulo, Brazil, when 10 children in a town of 20,000 persons died of an acute febrile illness associated with purpura and vascular collapse. Until December 1989, no cases of BPF had been reported outside of Sao Paulo and the neighboring state of Parana. This report summarizes the recognition and investigation of BPF in the state of Mato Grosso.