scholarly journals Decreased glycolysis induced dysfunction of NK cells in Henoch-Schonlein purpura patients

2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Wenjia Chai ◽  
Xiaolin Wang ◽  
Wei Wang ◽  
Hui Wang ◽  
Wenjun Mou ◽  
...  
Keyword(s):  
Nk Cells ◽  
Systemic Vasculitis ◽  
Metabolic Reprogramming ◽  
Healthy Children ◽  
Henoch Schonlein Purpura ◽  
Reduced Frequency ◽  
Activating Receptors ◽  
Innate Lymphocytes ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

Abstract Background Henoch-Schonlein purpura (HSP) is the most common systemic vasculitis of the childhood. However, its mechanisms and pathogenesis still need more exploration. Natural killer (NK) cells are innate lymphocytes, and there is a growing appreciation that cellular metabolism is important in determining the immune responsiveness of lymphocytes. Thus, we aimed to analyze the NK cells phenotype and explore the association between glucose metabolism and NK cells function in HSP patients. Results A total number of 64 HSP patients and 34 healthy children were included. The HSP patients were divided into two groups according to whether accompanied with nephritis or not. NK cells in HSP patients without nephritis showed a reduced frequency in peripheral blood, a down-regulated expression of activating receptors both NKp30 and NKp46, and an attenuated cytotoxic function against tumor cells. In addition, the function impairment of NK cells was shown to exacerbate in HSPN. Our data further revealed an aberrant metabolic reprogramming of NK cells in HSP patients. Upon stimulation with cytokines (IL-15, IL-12 and IL-2), NK cells from healthy controls switched to an elevated glycolysis rate to support their effector function. By contrast, the glycolysis rate of activated NK cells in HSP group was not significantly up-regulated from the resting level possibly owing to the inhibition of mTORC1. Conclusions Our study found that HSP patients were accompanied with dysfunction of NK cells. We concluded that the dysfunction of NK cells in HSP patients was induced with a decreased glycolysis rate and suggested that metabolic reprogramming of NK cells might be a player in the pathogenesis of HSP.

Steroid Effects on the Course of Abdominal Pain in Children With Henoch-Schonlein Purpura

PEDIATRICS ◽  
1987 ◽  
Vol 79 (6) ◽  
pp. 1018-1021
Author(s):  
NORMAN D. ROSENBLUM ◽  
HARLAND S. WINTER
Keyword(s):  
Abdominal Pain ◽  
Gastrointestinal Symptom ◽  
Systemic Vasculitis ◽  
Skin Lesions ◽  
Intestinal Bleeding ◽  
Controlled Trials ◽  
Henoch Schonlein Purpura ◽  
Intestinal Lesions ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

Henoch-Schonlein purpura is a systemic vasculitis of unknown cause that is characterized primarily by abdominal pain, arthritis, and purpuric skin lesions. Abdominal pain is the most common gastrointestinal symptom, but intestinal bleeding and intussusception may occur. Previous studies have supported the use of steroids in managing the abdominal pain of Henoch-Schonlein purpura.1,2 Because there are no controlled trials using steroids in this disease, their value in affecting the intestinal lesions of Henoch-Schonlein purpura remains unknown. The purpose of this retrospective study was to assess the effect of corticosteroids on the outcome of abdominal pain in children with Henoch-Schonlein purpura. PATIENTS AND METHODS

scholarly journals An evaluation of biomarkers indicating endothelial cell damage, inflammation and coagulation in children with Henoch-Schönlein purpura

2019 ◽  
Vol 44 (5) ◽  
pp. 676-682
Author(s):  
Nilgun Selcuk Duru ◽  
Kamil Sahin ◽  
Cihan Coskun ◽  
Ala Üstyol ◽  
Murat Elevli ◽  
...  
Keyword(s):  
Endothelial Cell ◽  
Cell Damage ◽  
Serum Levels ◽  
Healthy Children ◽  
Henoch Schonlein Purpura ◽  
Endothelial Cell Damage ◽  
Biochemical Tests ◽  
Acute Phase Reactants ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

Abstract Objective Henoch-Schönlein purpura (HSP) is characterized by generalized vasculitis. The etiopathogenesis of the disease is unknown, but inflammation and endothelial dysfunction have been held responsible. Therefore, herein we investigated serum levels of biomarkers indicating endothelial cell damage, inflammation and coagulation in children with HSP. Materials and methods Twenty six patients with HSP and 26 healthy children were included in the study. Routine biochemical tests and laboratory parameters showing inflammation, coagulation, and endothelial cell damage were examined in all subjects. Results White blood cell (WBC) number, C-reactive protein (CRP) level, erythrocyte sedimentation rate (ESR), neutrophil/lymphocyte rate (NLR), triglyceride, immunoglobulin A (IgA), and C3 were significantly higher in children with HSP than the controls. HDL and albumin levels were lower in the patients with HSP. Endocan levels were not significantly different between the HSP and control groups (p = 0.884). Serum endocan levels in patients with HSP were inversely correlated only with activated partial thromboplastin time (APTT) (r = −0.485, p = 0.012). Conclusion Coagulation abnormalities and increased acute phase reactants were present in patients with HSP while no difference was determined in endocan levels.

scholarly journals Intestinal Infarction and Portal Vein Thrombosis in a Patient with Henoch Schonlein Purpura

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Mekdess Abebe ◽  
Asha Patnaik ◽  
Frederick Miller ◽  
Heidi Roppelt ◽  
Nand K. Wadhwa ◽  
...  
Keyword(s):  
Gastrointestinal Tract ◽  
Portal Vein ◽  
Portal Vein Thrombosis ◽  
Therapeutic Intervention ◽  
Systemic Vasculitis ◽  
Henoch Schonlein Purpura ◽  
Vein Thrombosis ◽  
Schonlein Purpura ◽  
Benign Course ◽  
Henoch Schönlein Purpura

Henoch Schonlein purpura is a systemic vasculitis that commonly affects children and teenagers but also affects adults of all ages. In most instances it has a benign course. Organ involvement, particularly in adults, and notably the kidneys and gastrointestinal tract may require therapeutic intervention and may have a less favorable outcome. We report a case of a 58-year-old man who presented with purpura and who rapidly developed catastrophic intestinal vasculitis, leading to his demise.

scholarly journals Not Just A Rash!

2010 ◽  
Vol 9 (1) ◽  
pp. 27-29
Author(s):  
MA Fox ◽  
◽  
JA Fox ◽  
Al-Shamma S ◽  
Leiper K ◽  
...  
Keyword(s):  
Hidradenitis Suppurativa ◽  
Systemic Vasculitis ◽  
Gastrointestinal Haemorrhage ◽  
Henoch Schonlein Purpura ◽  
Classic Case ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

Henoch-Schonlein Purpura (HSP) is the most common systemic vasculitis in childhood and can present in adults. It is a self-limiting disease characterised by a tetrad of manifestations including the mandated typical cutaneous hallmark. We present a classic case of HSP complicated by gastrointestinal haemorrhage associated with hidradenitis suppurativa.

scholarly journals Henoch-Schönlein purpura

2016 ◽  
Vol 1 (3) ◽  
Author(s):  
Natalia Zdanowska ◽  
Agnieszka Owczarczyk-Saczonek ◽  
Waldemar Placek
Keyword(s):  
Surgical Intervention ◽  
Systemic Vasculitis ◽  
Bowel Perforation ◽  
Careful Monitoring ◽  
Henoch Schonlein Purpura ◽  
Life Threatening ◽  
Adults And Children ◽  
The Central Nervous System ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

<p>Henoch-Schönlein purpura (HSP) is an acute, systemic vasculitis which usually occurs in young adults and children. The skin involvement may lead to the manifestation of symptoms associated with vasculitis in intestines, kidneys, and the central nervous system. The incidence of bowel perforation in course of HSP is very seldom and it occurs about 10 days after the appearance of the first symptoms. We present a 23-year-old male patient with jejunal intussusception in the course of HSP. The patient was operated urgently with resection of part of the small intestine. Adults rarely suffer from the occurrence of abdominal pain and fever, but sometimes they require careful monitoring and surgical intervention because misdiagnosis can be life threatening.</p><p class="Standard"> </p>

scholarly journals Tubulointerstitial Infiltration of M2 Macrophages in Henoch-Schönlein Purpura Nephritis Indicates the Presence of Glomerular Crescents and Bad Clinical Parameters

2019 ◽  
Vol 2019 ◽  
pp. 1-10 ◽  
Author(s):  
Jisup Kim ◽  
Sung-Eun Choi ◽  
Keum Hwa Lee ◽  
Hyeon Joo Jeong ◽  
Jae Il Shin ◽  
...  
Keyword(s):  
Systemic Vasculitis ◽  
Renal Involvement ◽  
International Study ◽  
Clinical Parameters ◽  
M2 Macrophages ◽  
Henoch Schonlein Purpura ◽  
Severe Manifestation ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura ◽  
Purpura Nephritis

Henoch-Schönlein purpura (HSP) is the most common systemic vasculitis in children, and renal involvement (HSP nephritis, HSPN) is a severe manifestation. HSPN is histologically classified by the International Study of Kidney Disease in Children (ISKDC) based on mesangial hypercellularity and the extent of glomerular crescents. Macrophages, categorized as M1 or M2, frequently infiltrate in various glomerular and tubulointerstitial diseases and infiltration of specific subtypes is associated with disease progression. Therefore, to identify whether infiltration of M1 or M2 macrophages has clinical significance, we quantified the subtypes of macrophages in 49 HSPN specimens and correlated the counts with histologic features and clinical parameters. Higher tubulointerstitial M2 counts were associated with chronic renal failure (CRF), ISKDC classes III-IV, and crescents (P<0.001, 0.002, 0.001). Glomerular M2 counts were significantly related to ISKDC classes III-IV and crescents (area under curve, AUC 0.804, 0.833). Tubulointerstitial M2 counts were associated with CRF, ISKDC classes III-IV, and crescents (AUC 0.872, 0.778, 0.830). Tubulointerstitial M2 counts also revealed higher AUC than tubulointerstitial M1 counts for CRF (P=0.036) and ISKDC classes III-IV (P=0.047). Glomerular M2 counts revealed higher AUC than glomerular M1 counts for ISKDC classes III–IV (P=0.024). Tubulointerstitial M2 counts were the most powerful parameter for CRF (AUC 0.872) and revealed even higher AUC than ISKDC classification (AUC 0.716) with borderline significance (P=0.086) for CRF. In summary, tubulointerstitial M2 counts were a superior parameter to tubulointerstitial M1 counts and even to ISKDC classification indicating the presence of CRF.

scholarly journals Successful Outcome of a Corticodependent Henoch-Schönlein Purpura Adult with Rituximab

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Taylor Pindi Sala ◽  
Jean-Marie Michot ◽  
Renaud Snanoudj ◽  
Marion Dollat ◽  
Emmanuel Estève ◽  
...  
Keyword(s):  
Systemic Vasculitis ◽  
Transplant Rejection ◽  
Immunoglobulin A ◽  
Corticosteroid Treatment ◽  
Successful Outcome ◽  
Henoch Schonlein Purpura ◽  
Small Vessels ◽  
History Of ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

Henoch-Schönlein purpura (HSP) is a systemic vasculitis involving small vessels with deposition of immunoglobulin A (IgA) complexes, usually affecting children. Compared with children, HSP in adults is more severe and frequently associated with cancer. We report the case of a 49-year-old woman with medical history of kidney transplantation for segmental glomerular hyalinosis. Eight years after the transplantation, while taking combined immunosuppressive therapy with tacrolimus and azathioprine indicated for the prevention against transplant rejection, she developed a Henoch-Schönlein purpura. Vasculitis involves skin and sciatic peroneal nerve and she received systemic corticosteroid treatment. Because of four relapses and corticosteroid dependence, the patient was treated with rituximab (two intravenous infusions of 1000 mg given two weeks apart). Successful outcome was observed along two years of follow-up. This new case of successful use of rituximab in HSP promotes more investigations of this treatment in clinical trials.

scholarly journals Gastrointestinal Involvement in Henoch Schonlein Purpura : A Case Report

2020 ◽  
Vol 8 (2) ◽  
Author(s):  
Siti Kamariah CM ◽  
Rohaizan Y
Keyword(s):  
Case Report ◽  
Bowel Wall ◽  
Systemic Vasculitis ◽  
Bowel Ischemia ◽  
Gastrointestinal Involvement ◽  
Henoch Schonlein Purpura ◽  
Small Vessels ◽  
Abdominal Symptoms ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

Henoch-Schonlein purpura or anaphylactoid purpura is a systemic vasculitis of unknown cause that affects small vessels and mainly involves the skin, joints, gastrointestinal tract and kidneys. Gastrointestinal involvement occurs in more than half of patients and is thought to be related to edema and intramural haemorrhage. Radiologically the gastrointestinal findings are mainly those of bowel ischemia with “thumbprinting” and bowel wall oedema. Although this disease is usually treated conservatively, aggressive intervention is occasionally performed because of acute abdominal symptoms due to complications, such as perforation, intussusception and obstruction. This report illustrates a case of Henoch-Schonlein purpura with acute abdominal symptoms due to intussusception.

Life-threatening complications of Henoch-Schönlein purpura: diffuse alveolar haemorrhage, venous thrombosis and bowel ischaemia

2020 ◽  
Vol 13 (9) ◽  
pp. e235905
Author(s):  
Kezreen Kaur Dhaliwal ◽  
Nur Atikah Lile ◽  
Chou Luan Tan ◽  
Chong Hong Lim
Keyword(s):  
Venous Thrombosis ◽  
Systemic Vasculitis ◽  
Alveolar Haemorrhage ◽  
Diffuse Alveolar Haemorrhage ◽  
Henoch Schonlein Purpura ◽  
Bowel Ischaemia ◽  
Life Threatening ◽  
Schonlein Purpura ◽  
Purpuric Rash ◽  
Henoch Schönlein Purpura

Henoch-Schönlein purpura (HSP) is a common systemic vasculitis occurring in children. Making a diagnosis of HSP is often straightforward, managing its complications can be difficult. Diffuse alveolar haemorrhage (DAH), bowel ischaemia and venous thrombosis are rare complications of this disorder. We present a case of a 15-year-old teenage girl presenting with typical purpuric rash of HSP, developed DAH, bowel ischaemia and venous thrombosis. She was successfully treated with pulse methylprednisolone, intravenous Ig and intravenous cyclophosphamide.

scholarly journals Successful Treatment of Hemorrhagic Bullous Henoch-Schönlein Purpura with Oral Corticosteroid: A Case Report

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Celebi Kocaoglu ◽  
Ramazan Ozturk ◽  
Yasar Unlu ◽  
Fatma Tuncez Akyurek ◽  
Sukru Arslan
Keyword(s):  
Case Report ◽  
Gastrointestinal Tract ◽  
Female Patient ◽  
Oral Corticosteroid ◽  
Systemic Vasculitis ◽  
Immunoglobulin A ◽  
Henoch Schonlein Purpura ◽  
Hemorrhagic Bullae ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

Henoch-Schönlein purpura (HSP) is a vasculitis of small-sized blood vessels, resulting from immunoglobulin-A-mediated inflammation. It is the most common acute systemic vasculitis in childhood and mainly affects skin, gastrointestinal tract, joints, and kidneys. The characteristic rash of HSP consists of palpable purpuric lesions 2 to 10 mm in diameter concentrating in the buttocks and lower extremities. The occurrence of hemorrhagic bullae in children with HSP is rarely encountered. This report describes a 4.5-year-old female patient with HSP associated with hemorrhagic bullous lesions.

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