The elevated markers of hypercoagulability in children with Henoch-Schönlein purpura

Twenty-eight children with HSP and 79 healthy children were entered into study. Activities of protein C, free-protein S and antithrombin, activated protein C resistance, levels of fibrinogen. D-dimer, thrombin-antithrombin complex (TAT), prothrombin fragments 1 + 2 (PF(1+2)), and von Willebrand factor antigen (vWAg) and its activity (RiCof) were investigated in acute and recovery phases of HSP and controls. Fibrinogen, D-dimer, TAT, PF(1+2), vWAg, and RiCof levels in patients with HSP during the acute phase were significantly higher than those of recovery phase and of the controls. A significant correlation was detected between severity of disease and TAT, PF(1+2), vWAg, and D-dimer levels.     

SARS-CoV-2-associated Henoch–Schönlein purpura in a 13-year-old girl

In the context of the current coronavirus disease 2019 (COVID-19) pandemic, cutaneous lesions are being described. Here, we report on a 13-year-old girl with SARS-CoV-2-associated Henoch–Schönlein purpura and Epstein–Barr virus (EBV) infection. She presented without any respiratory symptoms, only a purpuric skin rash, abdominal pain, low-grade fever, and pharyngitis. Virology tests by polymerase chain reaction (PCR) were positive for SARS-CoV-2 and EBV. The potential association of Henoch–Schönlein purpura and SARS-CoV-2 should be kept in mind in order to reduce the spread of the virus, particularly in children with few respiratory symptoms.     

Henoch-Schönlein purpura in a child following varicella

Henoch-Sch?nlein purpura (HSP) is one of the most common vasculitis of childhood. It is characterized by nonthrombocytopenic palpable purpura, arthritis, renal and gastrointestinal system (GIS) involvement. HSP is usually triggered by an antigenic stimulus including infectious agents, drugs, cold, insect bite or food. HSP is rarely triggered by Varicella zoster infection. We herein presented a case with HSP following varicella.     

Haemorrhagic bullous lesions in a 3-year-old girl with Henoch-Schölein purpura

Henoch–Schönlein purpura (HSP) is the most common vasculitis in children and is characterized by cutaneous purpura, arthritis, abdominal pain and nephritis. We report a case involving a 3‐year‐old girl with HSP who displayed rapidly evolving haemorrhagic bullae from primary purpuric lesions during systemic corticosteroid therapy. Conclusion: The bullae disappeared within 7 days of systemic corticosteroid therapy, although some scarring of the skin occurred. Also, bullae should not be considered as a poor prognostic factor of renal outcome in HSP.     

Hypertension in Henoch-Schönlein purpura with minimal urinary findings

THOUGH HYPERTENSION IS A COMMON FEATURE OF HENOCH—Schönlein purpura (HSP) acute nephritis, it is seen only rarely and transiently if renal function is normal and abnormal urinary findings are minimal. We report a 3.4 year old girl who had the typical arthritis and rash of HSP, but also had significant hypertension with only minimal urinary findings. Imaging investigations revealed one normal kidney, and one that was almost destroyed from previous reflux nephropathy; her hypertension resolved after unilateral nephrectomy. Other causes of hypertension should be excluded in children with HSP and minimal urinary findings.

     

Severe scrotal pain in boys with Henoch-Schönlein purpura: incidence and sonography

Background. Henoch-Schönlein purpura (HSP) is a systemic vasculitis with multiorgan involvement. The scrotal involvement and its sonographic appearance are less well recognized than that of the kidneys and abdomen, and the reported incidence is varied.¶Objective. To review the incidence of significant scrotal involvement in boys with HSP and its sonographic characteristics.¶Materials and methods. Thirteen boys (ages 4–11 years) out of 87 boys diagnosed with HSP, over a 15-year period had significant scrotal complaints. Seven underwent sonographic evaluation to define the extent of scrotal involvement and because testicular torsion was being considered.¶Results. Scrotal involvement producing significant pain occurred in 15 % of boys with HSP. The majority of boys had the diagnosis of HSP established before developing scrotal complaints. Sonographic findings consistently included an enlarged, rounded epididymis, thickened scrotal skin, and a hydrocele. The testes themselves were usually sonographically normal.¶Conclusions. Scrotal involvement in boys with HSP is not uncommon. The sonographic findings in the scrotum are sufficiently characteristic to allow distinction from torsion in most cases.     

Non-Hodgkin’s lymphoma presenting with uterine and renal enlargement in a young girl

Non-Hodgkins lymphoma (NHL) is the fourth most common childhood malignancy. Uterine involvement with NHL is well described in adults, rare in children and has not been described in the first 2 years of life. While renal involvement in NHL is well recognised, diffuse renal enlargement is an uncommon finding. We report a unique case of B-cell lymphoma of primitive phenotype in a 15-month-old girl with uterine and renal involvement at presentation. We describe the US and MRI features at presentation that helped in the prospective diagnosis of this condition.     

Gastrointestinal manifestations in Henoch-Schönlein purpura: a review of 261 patients

Aim: Henoch‐Schönlein purpura is an IgA‐mediated autoimmune vasculitis of children. It often presents with symptoms including purpuric rash, abdominal pain, renal involvement or arthritis. Abdominal pain is a frequent symptom in children with HSP and raises the suspicion of intussusception or perforation. We sought to evaluate abdominal pain via stool occult blood and image studies. Methods: A retrospective study of 261 patients diagnosed with Henoch‐Schönlein purpura from December 1991 to December 2001 was conducted. Image studies, including abdominal echo, abdominal CT and panendoscopy, were performed for patients who suffered from abdominal pain. Results: Of the 261 patients, 151 (58%) had abdominal pain, and 46 (17.6%) suffered either overt gastrointestinal bleeding or had positive stool occult blood. Seven patients had gross bloody stools. One acute intussusception and one bowel perforation were noted. One patient suffered from hypovolemic shock due to massive gastrointestinal bleeding. When stool occult blood was 3+ or 4+, the incidence of a positive image finding was high. Conclusion: We found that stool occult blood and image studies may be necessary regarding severe gastrointestinal involvement. Ultrasonography is an important tool when intussusception or bowel perforation is suspected. Monitoring the vital signs is important, especially in patients with massive gastrointestinal bleeding.     

Risk factors of renal involvement and significant proteinuria in Henoch-Schönlein purpura

Risk factors of renal involvement and significant proteinuria in patients with Henoch-Sch?nlein purpura (HSP) were retrospectively evaluated by univariate and multivariate analyses. The analysis was performed in 134 patients with HSP. Renal involvement was found in 65 patients (49%) and 97% of the renal involvement was found within 3 months of disease onset. Moderate or severe proteinuria was recognised in 25 patients. A univariate analysis revealed that an age of more than 4 years at the onset, severe abdominal pain with gastrointestinal bleeding, persistent purpura over a month, coagulation factor XIII activity < 80%, and treatment with factor XIII concentrate were associated with developing renal involvement. A multivariate analysis showed that severe abdominal symptoms, an age of more than 4 years, and persistent purpura increased the risk of renal involvement. Risk factors of moderate or severe proteinuria were also examined. The risk factors in a univariate analysis were severe abdominal symptoms, persistent purpura, decreased factor XIII activity, treatment with steroids, and treatment with factor XIII concentrate. Of those, persistent purpura, treatment with factor XIII concentrate, and factor XIII activity < 80% were associated with significant proteinuria in a multivariate analysis. Among the patients with severe abdominal symptoms, factor XIII activity was significantly decreased in patients with significant proteinuria compared to other patients without significant proteinuria. CONCLUSION: Long-term prognosis of Henoch-Sch?nlein purpura is dependent on the severity of renal involvement. In those patients who have the risk factors of renal involvement, especially significant proteinuria, close attention should be paid to a urinalysis for at least 3 months from the onset of the disease.     

Prevalence of MEFV gene mutations and their clinical correlations in Turkish children with Henoch-Schönlein purpura

Aim: To investigate the frequency of MEFV mutations and their associations with the clinical and laboratory findings in children with Henoch–Schönlein purpura (HSP). Methods: One hundred and seven children with HSP were investigated for 12 common MEFV mutations. Results: Forty‐seven patients (43.9%) were found to have one of the MEFV mutations. Eight patients (7.5%) were homozygous for one mutation, 33 (30.8%) were heterozygous for one and six (5.6%) were compound heterozygous for two mutations. There were no age and sex differences between patients with or without mutations. Scrotal involvement was statistically more frequent in patients with mutations. Leucocyte counts, erythrocyte sedimentation rates, serum C‐reactive protein (CRP) concentrations, number of patients with increased CRP levels and number of patients with increased immunoglobulin A concentrations were found to be higher in patients with MEFV mutations. p.M694V was the most frequent mutation and was found to have effects on clinical and laboratory findings in children with HSP. Fifteen patients were started on colchicine with the diagnosis of familial Mediterranean fever (FMF). Conclusion: MEFV mutations are more frequent in HSP than in the general population, and mutation carriers may have more severe clinical findings with higher inflammatory response, suggesting a dysregulation of the inflammatory response because of defective gene encoding the protein pyrine. Investigation of these mutations may be beneficial to follow‐up the susceptible patients more closely leading to early diagnosis and treatment of FMF.     

A case of Henoch-Schönlein purpura and rheumatic carditis with complete atrioventricular block

Henoch-Schönlein purpura is the most common systemic vasculitis during childhood. Many antigenic stimuli and infectious agents have been proposed as a trigger of the disease. Group A beta-hemolytic streptococcus is the most common proposed infectious agent as a trigger for Henoch-Schönlein purpura. We report a 9-year-old boy who has Henoch-Schönlein purpura and acute rheumatic fever with complete atrioventricular block.     

Idiopathic testicular infarction in a boy initially suspected to have acute epididymo-orchitis associated with mycoplasma infection and Henoch-Schönlein purpura

Idiopathic testicular infarction without torsion of spermatic cord is a rare condition. We present a 12-year-old boy originally suspected of acute epididymo-orchitis, but subsequently an orchiectomy was necessary owing to unpredicted testicular infarction not associated with torsion. Elevation of immunoglobulin M against mycoplasma, reduction in serum factor XIII and the presence of sufficient blood flow detected by color Doppler ultrasonography upon initial manifestation suggested that the boy was affected by epididymo-orchitis associated with Henoch–Schönlein purpura or mycoplasma infection. However, progressive testicular enlargement was observed and subsequently testicular blood flow became barely detectable. Our case indicates that the presence of sufficient blood flow upon initial diagnosis in the affected testis does not necessarily exclude infarction and continuous monitoring of blood flow may be required to avoid radical orchiectomy.     

Clinical, laboratorial, and therapeutic aspects of 46 children with Henoch-Schönlein purpura

Forty-six children with Henoch-Sch?nlein purpura were studied.Their clinical manifestations included palpable purpura in 100%, subcutaneous edema in 30%, arthritis/arthralgia in 78%, gastrointestinal involvement in 56% and renal abnormalities in 30%. Serum IgA was elevated in 12%, ASLO titers in 27% and microscopic hematuria and proteinuria were detected in 30% of the children, including two with nephrotic syndrome. Treatment with non-steroidal antiinflammatory drugs or corticosteroid was indicated in 43% of the children. Recurrence of the disease was observed in 22% and the prognosis seems to be related to severity of renal involvement.