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Dermatology (Basel, Switzerland) 2022Acute hemorrhagic edema is a skin-limited small-vessel leukocytoclastic vasculitis, which affects infants 4 weeks to 2 years of age and remits within 3 weeks. The...
BACKGROUND
Acute hemorrhagic edema is a skin-limited small-vessel leukocytoclastic vasculitis, which affects infants 4 weeks to 2 years of age and remits within 3 weeks. The diagnosis is made clinically in not-ill appearing children with acute onset of raised annular or nummular eruptions and edema. In this vasculitis, type, distribution, and evolution of the rash have never been systemically investigated. To address this issue, we employed the data contained in the Acute Hemorrhagic Edema Bibliographic Database, which incorporates all reports on acute hemorrhagic edema.
SUMMARY
Key features of rash were documented in 383 children. Annular eruptions in a strict sense, usually targetoid, were reported in 375 (98%) cases (many children also presented polycyclic or arciform eruptions). Nummular eruptions were also very common (n = 358; 93%). Purpuric eruptions and ecchymoses were reported in the vast majority of cases. Macules and wheals were described in a minority of cases. Edema, detected in all cases, was mostly painful, indurated and nonpitting. The following regions were affected, in decreasing order, by annular or nummular eruptions: legs, feet, face, arms, ears, trunk, and genitals. With the exception of feet, which were very often affected, the same distribution was reported for edema. The initial eruption was often a wheal or a macule that evolved into a nummular or an annular eruption. Nummular eruptions successively evolved into annular ones.
KEY MESSAGE
This study carefully characterizes type, distribution, and evolution of skin eruption in acute hemorrhagic edema. The data help physicians to rapidly and noninvasively make the clinical diagnosis of this vasculitis.
Topics: Acute Disease; Child; Child, Preschool; Diagnosis, Differential; Edema; Exanthema; Humans; Infant; Vasculitis, Leukocytoclastic, Cutaneous
PubMed: 34551420
DOI: 10.1159/000519009 -
Vox Sanguinis May 2020In adult immune thrombocytopenia (ITP), an acquired autoimmune bleeding disorder, anti-platelet autoantibody testing may be useful as a rule-in test. Childhood ITP has...
BACKGROUND
In adult immune thrombocytopenia (ITP), an acquired autoimmune bleeding disorder, anti-platelet autoantibody testing may be useful as a rule-in test. Childhood ITP has different disease characteristics, and the diagnostic and prognostic value of anti-platelet antibody testing remains uncertain.
OBJECTIVE
To systematically review the diagnostic accuracy of anti-platelet autoantibody testing in childhood ITP.
METHODS
PubMed and EMBASE were searched for studies evaluating immunoassays in childhood ITP. Study quality was assessed (QUADAS2), and evidence was synthesized descriptively.
RESULTS
In total, 40 studies (1606 patients) were identified. Nine studies reported sufficient data to determine diagnostic accuracy measures. Anti-platelet IgG antibody testing showed a moderate sensitivity (0·36-0·80 platelet-associated IgG [direct test]; 0·19-0·39 circulating IgG [indirect test]). In studies that reported control data, including patients with non-immune thrombocytopenia, specificity was very good (0·80-1·00). Glycoprotein-specific immunoassays showed comparable sensitivity (three studies) and predominantly identified IgG anti-GP IIb/IIIa antibodies, with few IgG anti-GP Ib/IX antibodies. Anti-platelet IgM antibodies were identified in a substantial proportion of children (sensitivity 0·62-0·64 for direct and indirect tests).
CONCLUSION
The diagnostic evaluation of IgG and IgM anti-platelet antibodies may be useful as a rule-in test for ITP. In children with insufficient platelets for a direct test, indirect tests may be performed instead. A negative test does not rule out the diagnosis of ITP. Future studies should evaluate the value of anti-platelet antibody tests in thrombocytopenic children with suspected ITP.
Topics: Autoantibodies; Child; Humans; Immunoassay; Platelet Glycoprotein GPIIb-IIIa Complex; Platelet Glycoprotein GPIb-IX Complex; Purpura, Thrombocytopenic, Idiopathic; Sensitivity and Specificity; Serologic Tests
PubMed: 32080872
DOI: 10.1111/vox.12894 -
Case Reports in Hematology 2020While the association of immune thrombocytopenic purpura (ITP) and inflammatory bowel disease (IBD) has been described in a few case reports, management of ITP as an...
While the association of immune thrombocytopenic purpura (ITP) and inflammatory bowel disease (IBD) has been described in a few case reports, management of ITP as an extraintestinal manifestation of Crohn's disease (CD) is less studied. There are approximately a dozen cases describing the management of patients dually diagnosed with CD/ITP. Previous reports postulated that the mechanism of ITP in CD was through the presence of circulating immune complexes in the serum and antigenic mimicry due to increased mucosal permeability in active colitis, versus increased mucosal production of TH1-type proinflammatory cytokines during CD flares, which may account for remission of ITP with surgery for CD. We present a case of a 27-year-old man who presented with medically refractory CD and ITP who responded to surgical management with colectomy and splenectomy, along with a systematic review of the literature. These cases suggest that colectomy should be considered in the treatment of medically refractory ITP among patients with concomitant CD.
PubMed: 32274225
DOI: 10.1155/2020/4785759 -
Bioengineered Dec 2021The association of neutrophil-to-lymphocyte ratio (NLR) and mean platelet volume (MPV) with the severe gastrointestinal (GI) involvement in pediatric Henoch-Schonlein... (Meta-Analysis)
Meta-Analysis
The association of neutrophil-to-lymphocyte ratio (NLR) and mean platelet volume (MPV) with the severe gastrointestinal (GI) involvement in pediatric Henoch-Schonlein Purpura (HSP) has been reported in many studies. However, the conclusions from the previous studies were controversial. Therefore, for the first time, we performed a meta-analysis to systematically evaluate the relationship of NLR and MPV to the severe GI involvements. We retrieved PubMed, EMBASE, Web of Science, and Chinese National Knowledge Infrastructure (CNKI) (up to October 2020) thoroughly to acquire eligible studies. The pooled standard mean difference (SMD) with 95% confidence interval (CI) was used to describe the correlation of NLR and MPV with the severe GI involvement. A total of 1 studies comprising 2168 patients with HSP were included in this meta-analysis. Our combined analysis showed that NLR in HSP patients with the severe GI involvement was significantly higher than that in those without the severe GI involvement (SMD = 1.37; 95% CI: 0.70-2.05; p < 0.01). In addition, a lower MPV was observed in children with severe GI involvement (SMD = -0.29; 95% CI: -0.56 - -0.01, p = 0.042). Our sensitivity analysis and publication bias evaluation indicated that our combined results were reliable. Taken together, our study suggested NLR and MPV may be used as biomarkers for predicting or diagnosing the severe GI involvement in children with HSP. Nevertheless, more homogeneous studies with a larger sample size are required to validate these findings.
Topics: Child; Child, Preschool; Female; Gastrointestinal Hemorrhage; Humans; IgA Vasculitis; Intussusception; Leukocyte Count; Lymphocytes; Male; Mean Platelet Volume; Neutrophils
PubMed: 33412982
DOI: 10.1080/21655979.2020.1865607 -
BMC Complementary Medicine and Therapies Nov 2021Henoch-Schönlein purpura nephritis (HSPN) is listed as the most common secondary glomerular diseases among children. Approximately 15 to 20% of children eventually...
BACKGROUND
Henoch-Schönlein purpura nephritis (HSPN) is listed as the most common secondary glomerular diseases among children. Approximately 15 to 20% of children eventually could develop into chronic renal failure. Chinese patent herbal medicine Huaiqihuang (HQH) has been widely used in children with HSPN. This study aimed to evaluate the effectiveness and safety of HQH for HSPN in children, so as to provide evidence for clinical use.
METHODS
Randomized controlled trials (RCTs) on HQH for HSPN in children were searched in eight Chinese and English databases from their inception to December 2020. We included children with HSPN received HQH combined with conventional medicine. Cochrane "Risk of bias" tool was used to assess methodological quality, and "Grading of Recommendations Assessment, Development, and Evaluation (GRADE) approach" to summarize the certainty of evidence for main findings. Effect estimates were presented as risk ratio (RR), mean difference (MD) or standardized mean difference (SMD) with 95% confidence interval (CI) in meta-analyses using RevMan 5.3. Data not suitable for statistical pooling were synthesized qualitatively.
RESULTS
In total seven RCTs were identified. Compared with conventional medicine alone, HQH plus conventional medicine showed the better effect in improving clinical cure rate (RR 1.58; 95%CI 1.17 to 2.14; n = 6) and total effective rate (RR 1.34; 1.16 to 1.54; n = 6); reducing urine sediment erythrocyte count (MD -9.23; - 10.76 to - 7.69; n = 3) and urine β2 micro-globulin level (MD -0.09; - 0.12 to - 0.06; n = 2). No serious adverse event was recorded in all included trials.
CONCLUSIONS
Limited evidence showed HQH combined with conventional medicine had a beneficial effect for children with HSPN, and the side effects were mild. HQH may be a promising complementary therapy. However, long term follow-up, high quality and multicenter RCTs are required to confirm the findings.
Topics: Child; Drugs, Chinese Herbal; Herbal Medicine; Humans; IgA Vasculitis; Nephritis; Randomized Controlled Trials as Topic
PubMed: 34743723
DOI: 10.1186/s12906-021-03415-x -
Neuropsychopharmacology Reports Sep 2019Altered trafficking of α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptors has been reported in postmortem studies and suggested the involvement of...
BACKGROUND AND OBJECTIVES
Altered trafficking of α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptors has been reported in postmortem studies and suggested the involvement of AMPA receptors in the pathophysiology underpinning addictive disorders. However, these findings seemed mixed.
METHODS
A systematic literature search was conducted, using PubMed and Embase (last search, August 2018), to identify human postmortem studies that examined the expression of proteins and mRNA of AMPA receptor subunits in patients with addictive disorders in comparison with healthy controls.
RESULTS
Twelve (18 studies) out of 954 articles were identified to be relevant. Eight studies included alcohol use disorders, and four studies included heroin/cocaine abusers. The most frequently investigated regions were the hippocampus (three studies), amygdala (three studies), and putamen (three studies). In summary, two out of the three studies showed an increase in the expression of AMPA receptors in the hippocampus, while the other study found no change. Two studies to examine the amygdala demonstrated either a decreased or no change in receptor expression or binding. Concerning putamen, two studies showed no significant change whereas an overexpression of receptors was observed in the other.
CONCLUSIONS AND SCIENTIFIC SIGNIFICANCE
The hippocampus and amygdala may be pertinent to addictive disorders through their functions on learning and memory, whereas findings in other regions were inconsistent across the studies. Human postmortem studies are prone to degenerative changes after death. Moreover, only qualitative assessment was conducted because of the limited, heterogenous data. These limitations emphasize the need to investigate AMPA receptors in the living human brains.
Topics: Adult; Aged; Amygdala; Autopsy; Female; Hippocampus; Humans; Male; Middle Aged; Protein Binding; Protein Subunits; Putamen; RNA, Messenger; Receptors, AMPA; Substance-Related Disorders
PubMed: 31070872
DOI: 10.1002/npr2.12058 -
Medicine Jan 2021The purpose of this study is to compare the clinical efficacy of laparoscopic splenectomy (LS) and open splenectomy (OS) in the treatment of Idiopathic thrombocytopenic... (Comparative Study)
Comparative Study Meta-Analysis
BACKGROUND
The purpose of this study is to compare the clinical efficacy of laparoscopic splenectomy (LS) and open splenectomy (OS) in the treatment of Idiopathic thrombocytopenic purpura.
METHODS
We systematically searched PubMed, Web of science, EMBASE, Clinicaltrials.gov, and Cochrane Central Register for studies (study published from July 1992-January 2020). This study analyzed the clinical effect of LS and OS on idiopathic thrombocytopenic purpur.
RESULTS
This study showed that compared with OS, the LS's Overall response (OR: 0.60, 95% confidence interval (CI): 0.23-1.59, P = .30), Complication (OR: 0.59, 95% CI: 0.18-1.94, P = .38), Accessory spleen(OR: 1.70, 95% CI: 0.98-2.98, P = .06), Wound infections (OR: 0.65, 95% CI: 0.26-1.59, P = .34), Pancreatic fistula (OR: 0.73, 95% CI: 0.16-3.30, P = .68), was no significant, the Operative time (weighted mean difference (WMD): 49.33, 95% CI: 36.29-62.37, P < .00001)was longer, and the Estimated blood loss (WMD: -172.59, 95% CI: -319.96 to -25.22, P = .02), Postoperative length of stay (WMD: -4.68, 95% CI: -7.75 to -1.62, P = .003)was less.
CONCLUSIONS
The therapeutic effect of LS was the same as that of OS in Overall response Complication Accessory spleen, while The operative time was longer, the Estimated blood loss was less, and the postoperative length of stay was shorter.
Topics: Adult; Blood Loss, Surgical; Female; Humans; Laparoscopy; Length of Stay; Male; Middle Aged; Operative Time; Purpura, Thrombocytopenic, Idiopathic; Splenectomy; Treatment Outcome
PubMed: 33530246
DOI: 10.1097/MD.0000000000024436 -
The Cochrane Database of Systematic... Jan 2021Portal hypertension commonly accompanies advanced liver disease and often gives rise to life-threatening complications, including bleeding (haemorrhage) from oesophageal...
Band ligation versus sham or no intervention for primary prophylaxis of oesophageal variceal bleeding in children and adolescents with chronic liver disease or portal vein thrombosis.
BACKGROUND
Portal hypertension commonly accompanies advanced liver disease and often gives rise to life-threatening complications, including bleeding (haemorrhage) from oesophageal and gastrointestinal varices. Variceal bleeding commonly occurs in children and adolescents with chronic liver disease or portal vein thrombosis. Prevention is, therefore, important. Randomised clinical trials have shown that non-selective beta-blockers and endoscopic variceal band ligation decrease the incidence of variceal bleeding in adults. In children and adolescents, band ligation, beta-blockers, and sclerotherapy have been proposed as primary prophylaxis alternatives for oesophageal variceal bleeding. However, it is unknown whether these interventions are of benefit or harm when used for primary prophylaxis in children and adolescents.
OBJECTIVES
To assess the benefits and harms of band ligation compared with sham or no intervention for primary prophylaxis of oesophageal variceal bleeding in children and adolescents with chronic liver disease or portal vein thrombosis.
SEARCH METHODS
We searched the Cochrane Hepato-Biliary Group Controlled Trials Register, CENTRAL, PubMed, Embase, and two other databases (April 2020). We scrutinised the reference lists of the retrieved publications, and we also handsearched abstract books of the two main paediatric gastroenterology and hepatology conferences from January 2008 to December 2019. We also searched clinicaltrials.gov, the United States Food and Drug Administration (FDA), the European Medicines Agency (EMA), and the World Health Organization (WHO) for ongoing clinical trials. We imposed no language or document type restrictions on our search.
SELECTION CRITERIA
We aimed to include randomised clinical trials irrespective of blinding, language, or publication status, to assess the benefits and harms of band ligation versus sham or no intervention for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis. If the search for randomised clinical trials retrieved quasi-randomised and other observational studies, then we read them through to extract information on harm.
DATA COLLECTION AND ANALYSIS
We used standard Cochrane methodology to perform this systematic review. We used GRADE to assess the certainty of evidence for each outcome. Our primary outcomes were all-cause mortality, serious adverse events and liver-related morbidity, and quality of life. Our secondary outcomes were oesophageal variceal bleeding and adverse events not considered serious. We used the intention-to-treat principle. We analysed data using Review Manager 5.
MAIN RESULTS
One conference abstract, describing a feasibility multi-centre randomised clinical trial, fulfilled our review inclusion criteria. We judged the trial at overall high risk of bias. This trial was conducted in three hospital centres in the United Kingdom. The aim of the trial was to determine the feasibility and safety of further larger randomised clinical trials of prophylactic band ligation versus no active treatment in children with portal hypertension and large oesophageal varices. Twelve children received prophylactic band ligation and 10 children received no active treatment. There was no information on the age of the children included, or about the diagnosis of any child included. All children were followed up for at least six months. Mortality was 8% (1/12) in the band ligation group versus 0% (0/10) in the no active intervention group (risk ratio (RR) 2.54, 95% confidence interval (CI) 0.11 to 56.25; very low certainty of evidence). The abstract did not report when the death occurred, but we assume it happened between the six-month follow-up and one year. No child (0%) in the band ligation group developed adverse events (RR 0.28, 95% CI 0.01 to 6.25; very low certainty of evidence) but one child out of 10 (10%) in the no active intervention group developed idiopathic thrombocytopaenic purpura. One child out of 12 (8%) in the band ligation group underwent liver transplantation versus none in the no active intervention group (0%) (RR 2.54, 95% CI 0.11 to 56.25; very low certainty of evidence). The trial reported no other serious adverse events or liver-related morbidity. Quality of life was not reported. Oesophageal variceal bleeding occurred in 8% (1/12) of the children in the band ligation group versus 30% (3/10) of the children in the no active intervention group (RR 0.28, 95% CI 0.03 to 2.27; very low certainty of evidence). No adverse events considered non-serious were reported. Two children were lost to follow-up by one-year. Ten children in total completed the trial at two-year follow-up. There was no information on funding. We found two observational studies on endoscopic variceal ligation when searching for randomised trials. One found no harm, and the other reported E nterobacter cloacae septicaemia in one child and mild, transient, upper oesophageal sphincter stenosis in another. We did not assess these studies for risk of bias. We did not find any ongoing randomised clinical trials of interest to our review.
AUTHORS' CONCLUSIONS
The evidence, obtained from only one feasibility randomised clinical trial at high risk of bias, is very scanty. It is very uncertain about whether prophylactic band ligation versus sham or no (active) intervention may affect mortality, serious adverse events and liver-related morbidity, or oesophageal variceal bleeding in children and adolescents with portal hypertension and large oesophageal varices. We have no data on quality of life. No adverse events considered non-serious were reported. The results presented in the trial need to be interpreted with caution. In addition, the highly limited data cover only part of our research question; namely, children with portal hypertension and large oesophageal varices. Data on children with portal vein thrombosis are lacking. Larger randomised clinical trials assessing the benefits and harms of band ligation compared with sham treatment for primary prophylaxis of oesophageal variceal bleeding in children and adolescents with chronic liver disease or portal vein thrombosis are needed. The trials should include important clinical outcomes such as death, quality of life, failure to control bleeding, and adverse events.
Topics: Adolescent; Child; Chronic Disease; Esophageal and Gastric Varices; Feasibility Studies; Gastrointestinal Hemorrhage; Humans; Ligation; Liver Diseases; Portal Vein; Primary Prevention; Venous Thrombosis
PubMed: 33522602
DOI: 10.1002/14651858.CD011561.pub2 -
Pediatric Dermatology Jan 2020Acute hemorrhagic edema of young children is a benign skin-limited vasculitis mainly affecting children 2 to 24 months of age, which is often considered the infantile...
BACKGROUND
Acute hemorrhagic edema of young children is a benign skin-limited vasculitis mainly affecting children 2 to 24 months of age, which is often considered the infantile variant of immunoglobulin A vasculitis (Henoch-Schönlein purpura). In most cases, the diagnosis is made on a clinical basis without a skin biopsy.
METHODS
A systematic review of the literature was performed to examine the reported prevalence of vascular immune deposits in skin biopsies of patients with acute hemorrhagic edema of young children.
RESULTS
Testing for vascular immune deposits was performed in 75 cases (64 boys and 11 girls aged from 3.5 to 72, median 11 months) published between 1970 and 2018. Vessel wall deposition of complement C3 was seen in 40 cases. Immunoglobulin M (N = 24), immunoglobulin A (N = 21), immunoglobulin G (N = 13), and immunoglobulin E (N = 3) were less frequently detected. Gender, age, clinical features, and disease duration were not statistically different in cases with and without vessel wall deposition of immunoglobulin A.
CONCLUSION
Immune deposits in skin vessels, most frequently complement C3, are common in subjects with acute hemorrhagic edema of young children, providing furhter evidence that acute hemorrhagic edema, immunoglobulin A vasculitis, and pauci-immune vasculitides are different entities.
Topics: Acute Disease; Child; Child, Preschool; Humans; IgA Vasculitis; Immunoglobulins; Skin; Vasculitis, Leukocytoclastic, Cutaneous
PubMed: 31755135
DOI: 10.1111/pde.14041