Changes of peripheral blood tenascin-C level in children with Kawasaki disease and its clinical significance / 中华实用儿科临床杂志

Objective:To compare peripheral blood tenascin-C (TN-C) level in patients with Kawasaki disease (KD) on admission, after treatment and at recovery, and to assess the potential of TN-C as a novel predictor for coronary artery lesion.Methods:Retrospective study.Blood samples of 44 KD patients [including 21 patients with coronary artery lesions (CAL + group) and 23 patients without coronary artery lesions(CAL - group)], 39 anaphylactoid purpura patients and 36 non-infected and non-vasculitis controls in the Affiliated Hospital of North Sichuan Medical College during January 1, 2018 and November 1, 2018 were collected.TN-C level was measured by enzyme-linked immunosorbent assay.Normally distributed data were compared by the t test; otherwise, they were compared by the Mann- Whitney U test. Pearson product-moment correlation coefficient or Spearman rank correlation coefficient was used to analyze the correlation between TN-C and other laboratory indexes. Results:For KD patients, TN-C levels on admission [(32.0±13.8) μg/L] and after treatment [(33.5±11.4) μg/L] were significantly higher than that at recovery [(23.3±10.8) μg/L](all P<0.01), which was positively correlated with C-reactive protein ( r=0.317, P=0.038), and negatively correlated with sodium level ( r=-0.472, P=0.004). No significant difference in TN-C level was found between CAL + group and CAL - group [on admission: (31.7±15.4) μg/L vs.(32.3±12.5) μg/L; after treatment: (32.2±11.6) μg/L vs.(34.8±11.3) μg/L; at recovery: (22.6±7.3) μg/L vs.(24.0±13.4) μg/L; all P>0.05]. In addition, TN-C level in patients with KD [(32.0±13.8) μg/L] and anaphylactoid purpura [(37.2±18.2) μg/L] was significantly higher than that of control children [(24.0±8.05) μg/L] (all P<0.01). Conclusions:The study findings are able to prove the potential of peripheral blood TN-C as a predictor for coronary artery lesion in KD patients, nor as a maker of vascular injury.Nevertheless, it may be used as an indicator of immune response in the acute phase of KD.

Clinicopathologic comparisons of IgA nephropathy and IgA vasculitis nephropathy in children: a ten-year single-center experience.

BACKGROUND: To investigate the similarities and differences of renal clinical and renal pathology between IgA nephropathy (IgAN) and IgA vasculitis nephritis (IgAVN) in children. METHODS: A total of 237 children with IgAN and 190 children with IgAVN were included. The general conditions, clinical characteristics, final diagnosis, clinical and pathological classification of the children were intercepted at the time of admission, and the retrospective comparative analysis was carried out. RESULTS: The results showed that the median course of disease in IgAN group was longer than that in IgAVN group (p = 0.02). Patients with IgAN had a significantly higher duration of infection than the patients with IgAVN (p = 0.03). The white blood cell count (WBC), hemoglobin (HGB) in IgAN group were significantly lower than that in IgAVN group (p = 0.02). The serum creatinine in IgAN group was higher than that in IgAVN group (p = 0.02). Patients with IgAN and IgAVN had statistically significant differences in pathological typing between clinical types: hematuria and proteinuria, nephrotic syndrome and chronic nephritis (p = 0.004). DISCUSSION: The clinical manifestations of IgAN and IgAVN were similar, but the onset of IgAN was hidden and the clinical manifestations were relatively serious. Renal pathology was mainly glomerulosclerosis and renal tubular atrophy. IgAVN was characterized by acute onset and good renal function. Renal pathology was dominated by endothelial hyperplasia and crescent formation. These differences did not support the hypothesis that the two diseases are the same.

Immunoglobulin A Nephropathy and Immunoglobulin A Vasculitis.

Henoch-Schönlein purpura nephritis and immunoglobulin A nephropathy are common glomerulopathies in the pediatric population that deserve special attention. In some cases the primary care provider can follow the patient but others need more intensive management. Delaying this treatment can lead to worse morbidity. This article provides information on the pathogenesis, outcomes, and follow-up strategies that will aid in the diagnosis and referral of patients at risk for kidney disease.

The relationship between red cell distribution width and the risk of Henoch-Schönlein purpura nephritis.

INTRODUCTION: Red blood cell distribution width (RDW) is elevated in various inflammatory diseases, but its clinical significance in Henoch-Schönlein purpura nephritis (HSPN) in unknown. The aim of this study was to determine the value of RDW as a risk factor or marker for HSPN in children. METHODS: This was a case-control study of 105 Henoch-Schönlein purpura (HSP) patients, 120 HSPN patients and 192 healthy controls. The relationship between RDW-coefficient of variation (RDW-CV) and the clinical characteristics of HSPN patients was determined by a multiple logistic regression analysis (MVLRA). Receiver operating characteristic (ROC) curves were applied to compare the diagnostic potential of the RDW-CV, a panel of routine markers and combinations of these indices. RESULTS: The RDW-CV values were significantly higher in the HSPN group than the HSP group and controls (P < 0.001). Significant correlations were found between RDW-CV and ESR (P = 0.001). A combination of RDW-CV and ESR in a ROC curve showed 80% sensitivity and 84.9% specificity in the HSP patients, and 85.8% sensitivity and 93.8% specificity in the HSPN patients. The MVLRA revealed that RDW-CV (OR 1.69, 95% CI 1.16-2.48, P = 0.007) was an independent predictor of HSPN. CONCLUSIONS: The RDW levels were highest in the HPSN group, suggesting that RDW, especially the combination of RDW and ESR, may have value when assessing the risk of HSPN.

Cutaneous Manifestations of Small-Vessel Leukocytoclastic Vasculitides in Childhood.

In childhood, cutaneous small-vessel vasculitides include Henoch-Schönlein syndrome, a systemic vasculitis, and Finkelstein-Seidlmayer syndrome, a skin-limited vasculitis. Both Henoch-Schönlein and Finkelstein-Seidlmayer syndromes are seen more frequently in white or Asian compared with black children and occur especially in winter and spring with a male-to-female ratio of approximately 2:1. In everyday clinical practice, both conditions are diagnosed on clinical grounds without histological confirmation. The characteristic cutaneous hallmarks of Henoch-Schönlein syndrome include a purpuric rash in all and a subcutaneous edema in approximately every second case, which are often preceded by non-specific red or pink macular elements that mimic a non-itching urticarial rash. Recent data point out that Henoch-Schönlein children often present further cutaneous findings such as Köbnerization, Rumpel-Leede capillary fragility phenomenon, and blistering eruptions. Children with Finkelstein-Seidlmayer syndrome are usually ≤24 months of age and not ill-appearing. They present with (a) large, round, red to purpuric plaques (often with a targetoid appearance) predominantly over the cheeks, ears, and extremities and (b) often tender non-pitting edema of the distal extremities, ears, and face (without pruritus). Both in Henoch-Schönlein syndrome and Finkelstein-Seidlmayer syndrome, there is often scrotal involvement. The cutaneous findings remit without sequelae within 2 months in Henoch-Schönlein and 3 weeks in Finkelstein-Seidlmayer syndrome.

Histopathological and immunological changes during the acute and recovery phase in Henoch-Schönlein purpura rabbit model.

Henoch-Schönlein purpura (HSP) is a systemic vasculitis mediated by autologous immune complex. Animal models of HSP are scarce. Here, we describe the characteristics of HSP rabbit model in the acute and recovery phase. First, we constructed the HSP rabbit models, and then assessed immunologic indicators of models by enzyme-linked immunosorbent assay and immunoturbidimetry. Histomorphological characteristics were analyzed by haematoxylin-eosin, immunofluorescence and special staining. In the acute stage (24 h) after antigen challenge, the model group rabbits featured skin ecchymosis and abnormal laboratory examination results. Three weeks following the allergic reaction, purple spots improved markedly, and edema and blood seeping decreased, but obvious inflammation was present in the skin, kidneys, joints, gastrointestinal, lung and liver. Serological results of CD4, CD/CD8, IL-2, IL-4, and TNF-α, IgA, IgG, TropI, Alb and T were still abnormal. IgA and C3 expressed in skin and kidney and eosinophils expressed in skin and lungs were increased. The rabbit model can mimic human HSP lesions in symptoms, pathology, and immunology and may provide valuable insight into the pathogenesis of HSP and serve as a tool for future therapeutic development targeting HSP.

Anaphylactoid purpura triggered by cellulitis as a favorable prognosis: case report and literature review.

BACKGROUND: An anaphylactoid purpura affects small capillaries in the skin and other organs. Although two cases of anaphylactoid purpura exacerbated by cellulitis have been reported in Japanese literatures, its prognosis remains still unclear. Because cellulitis exacerbates various cutaneous inflammations, it has been speculated that cellulitis might also exacerbate cutaneous inflammation, such as vasculitis. FINDINGS: In this article, we report that 78-year-old woman exhibited anaphylactoid purpura, following cellulitis. We also reviewed the literature concerning about this subject. CONCLUSIONS: This type of anaphylactoid purpura is thought to have a favorable prognosis dependent on the treatment for cellulitis.

Evolution of IgA nephropathy into anaphylactoid purpura in six cases--further evidence that IgA nephropathy and Henoch-Schonlein purpura nephritis share common pathogenesis.

BACKGROUND: As the morphological and immunohistochemical manifestations of immunoglobulin A (IgA) nephropathy and Henoch-Schonlein purpura nephritis (HSPN) are very similar, they are considered to share a common pathogenesis. Although HSPN usually develops after the appearance of anaphylactoid purpura, we have encountered patients whose renal symptoms preceded purpura. METHODS: We reviewed the clinical courses of patients who were first diagnosed with IgA nephropathy, but developed purpura later, at the National Center for Child Health and Development in Tokyo, Japan. RESULTS: Of the 53 patients who were diagnosed with primary IgA nephropathy at our institute during the study period (March 2002 to July 2015), six (11 %) developed anaphylactoid purpura after the diagnosis of primary IgA nephropathy and therefore met the inclusion criteria. Duration between the onset of nephritis and subsequent appearance of purpura ranged from 5 months to 14 years. One patient reached end-stage renal failure due to IgA nephropathy and developed purpura after renal transplantation. All renal biopsies performed before the appearance of purpura showed mesangial proliferation with predominant IgA deposits. Urinary findings deteriorated in three patients after the appearance of purpura, including one patient who developed rapidly progressive glomerulonephritis. Renal biopsy findings worsened in two patients. At the last observation, two patients showed mild renal insufficiency. CONCLUSIONS: Our clinical experience and previous reports support the argument that IgA nephropathy and HSPN are different manifestations of a single disease. Hence, it is acceptable to consider that they are variants of a single disease.

Pharmaceutical Intervention and Care for Anaphylactoid Purpura Patient by Clinical Pharmacist / 中国药师

Objective:To improve the effectiveness and safety of drugs and the compliance of patients with anaphylactoid purpura through the participation of clinical pharmacists in the practice of pharmaceutical treatment .Methods:In the treatment of one patient with anaphylactoid purpura , clinical pharmacists took part in the whole process and provided the individualized regimen , adverse reac-tion monitoring , relative indices monitoring and drug education after the discharge .Results:Through the participation of clinical phar-macists in the medication development , the rational use of drugs was strengthened and the treatment process was monitored .As a re-sult, the infection of the patient obtained effective control .Conclusion:The participation of clinical pharmacist in the treatment of pa-tients with anaphylactoid purpura reflects the patient-oriented pharmacy service concept , which improves the efficiency and safety of treatment.

Research on imbalances of peripheral blood Th17 cells and Treg cells in Anaphy-lactoid purpura children / 中国免疫学杂志

Objective:To evaluate the roles of imbalance between peripheral blood T helper17(Th17) cells and CD4+CD25+regulatory T(Treg) cells in the pathogenesis of Anaphylactoid purpura(AP).Methods: Peripheral blood samples were obtained from fifty-two patients with AP and thirty age-and sex-matched healthy controls.The percentage of Th17 cells and Treg cells in peripheral blood were detected with Flow cytometry.Furthermore,the serum level of IL-6,IL-17,IL-22 and IL-23 and transforming growth factor (TGF)-βand IL-10 were detected with the use of enzyme linked immunosorbent assay(ELISA).Results: In comparison with the controls,the AP children showed a higher percentage of Th17 cells and an increased serum level of IL-6,IL-17,IL-22 and IL-23(P<0.05),but the percentage of Treg cells and the serum level of TGF-βand IL-10 were significantly lower in AP group(P<0.05).Con-clusion:AP children experience a change in the percentage of peripheral blood T helper17 cells and CD4+CD25+regulatory T cells in peripheral blood.The imbalance between the Th17 cells and Treg cells in peripheral blood may contribute to the development of Ana-phylactoid purpura.

A Case Which Presented Morbidity Considered to Be Anaphylactoid Purpura after Thoracic Endovascular Aortic Repair / 日本心臓血管外科学会雑誌

We herein report a 79-year-old man who developed anaphylactoid purpura after thoracic endovascular aortic repair, which he underwent for a distal aortic arch aneurysm of saccular type. On the third postoperative day he had purpura over his lower legs and abdomen accompanied by intermittent fever. His serum C-reactive protein concentration reached a maximum of 12 mg/dl, and remained at around 4 mg/dl thereafter. A dermatologist diagnosed anaphylactoid purpura ; this gradually improved with topical steroid and the nature and dosage of the oral medication. We suspected the presence of malignancy ; however, appropriate investigations failed to identify a cause for the purpura. During 6 months of outpatient follow up he has been free of recurrence. Anaphylactoid purpura occurs most frequently in childhood, often after an upper respiratory tract infection, whereas this condition is rare in adults. Triggers for anaphylactoid purpura include surgery, infection, certain medications, chronic lung, liver, or renal failure, and malignancy. We believe that the stress of undergoing thoracic endovascular aortic repair was the trigger in this case. Anaphylactoid purpura may be complicated by arthritis, gastrointestinal involvement and renal manifestations. There were no such complications in this case.

A case of anaphylactoid purpura nephritis accompanied by pulmonary hemorrhage and review of the literature.

Cases of Henoch-Schönlein purpura and purpura nephritis accompanied by pulmonary hemorrhage are rare. Mild cases are easily ignored due to a lack of evident bleeding, and severe cases may be fatal. We have only treated one patient with Henoch-Schönlein nephritis (HSPN), a female child. The clinical manifestations were not evident, however, the imaging manifestations were clear. Finally, the patient was definitively diagnosed with HSPN accompanied by pulmonary hemorrhage. Following treatment with antiinflammatory and steroidal agents, tripterygium glycosides and traditional Chinese medicine, the patient recovered. In the present study, we report the diagnosis and treatment of this disease, with a review of the literature.

Henoch-Schonlein purpura with intestinal perforation and cerebral hemorrhage: a case report.

Henoch-Schonlein purpura (HSP) with intestinal perforation and cerebral hemorrhage is a very rare clinical condition. There has been no report of HSP complicated with both intestinal perforation and cerebral hemorrhage until October 2012. Here we describe a case of HSP with intestinal perforation and cerebral hemorrhage in a 5-year-old girl. Plain abdominal radiograph in the erect position showed heavy gas in the right subphrenic space with an elevated diaphragm. Partial resection of the small intestine was performed, and pathological analysis suggested chronic suppurative inflammation in all layers of the ileal wall and mesentery. Seventeen days after surgery, cerebral hemorrhage developed and the patient died.

Clinical Observation on the Treatment of 34 Cases of Infantile Anaphylactoid Purpura with Self-made Huoxue Huayu Decoctoin / 国际中医中药杂志

Objective To observe the effect of treating infantile anaphylactoid purpura with self-made Huoxue Huayu decoction. Methods 68 cases with infantile anaphylactoid purpura ,were randomly recruited into a control group and a treatment group, with 34 cases in each group. The control group was taken Cetirizine, Prednisone, Rutin and Vitamin C orally. The treatment group was treated with self-made Huoxue Huayu decoction on the basis of treatment in the control group. Results The total effective rate of the control group and the treatment group was 82.35% and 94.12% respectively. As compared with the effective rate in the control group, the treatment group showed significant improvement. Conclusion It was effective to treat infantile anaphylaetoid purpura with Huoxue Huayu decoction.

Clinical observation of about heavy abdomen type of anaphylactoid purpura treated hy methylprednisolone associate with heparin / 中国小儿急救医学

Objective To observe the curative effect about heavy abdomen type of anaphylactoid pur-pura treated by Methylprednisolone associate with Heparin. Methods Randomly divided eighty three children with heavy abdomen type of anaphylactoid purpura who treated in department of pediatrics of the first hospital affiliated to Kunming medical college from January, 2006 to december, 2007 into group A (treated by Hydro-cortisone) and group B (treated by Methylprednisolone) and group C(treated by Methylprednisolone associate with Heparin). Group A treated by mainline Hydrocortisone dose of 5～8 mg/kg body weight once a day,mixed into 100ml 5% glucose injection, after 7～14 days later, instead of take orally Predisone tablet and de-grees dose by gradually. Group B treated by mainline Methylprednisolone dose of 2～4 mg/kg body weight once a day, mixed into 100ml 5% glucose injection, after 7～14 days later, instead of take orally Predisone tablet, and group C treated by Methylprednisolone by oneself (dose like group B) associate with mainline Hep-arin (dose of 0.25～0.75 mg/kg body weight once a day, mixed into 100ml 5% glucose injection) 7～10 days. Observe clinic symptomatic like as tetter, bellyache, entron hemorrhage when treatment of every group. Contrast reiteration rate of anaphylaetoid purpura, and the period of urine test get hack normal which cases with kidney tamper. Contrast these medication' s side effect such as resort of natrium and water, elec-trolyte turbulence, hemorrhage and so on. Results Group C (treated by Methylprednisolone associate with Heparin) is better then group A (treated by Hydrocortisone) and group B (treated by Methylprednisolone by oneself) in eliminate tetrer and release bellyache. The less period of group C need to treatment entron hemor-rhage and urine test get hack normal. And thereiteration rate is less of group C than group A and group B.Conclusion Methylprednisolone in association with Heparin to treat heavy abdomen type of anaphylactoid purpura is better than hydrocortisone and Methylprednisolone by oneself in controling symptom, reduing reiter-ation rate and more less side effect. So methylprednisolone in association with Heparin to treat of heavy ab-domen type of anaphylactoid purpura is a ideal project.

Anaphylactoid purpura associated with cutaneous necrosis and gangrene and its treatment with dextran infusions

A case of a 4-year old Filipino female with anaphylactoid purpura complicated by cutaneous necrosis and gangrene was herein reported. Therapy with 6% Dextran infusions was dramatic and rewarding as evidenced by cessation of further progression of the ischemic lesions and the eventual drying up of almost all cutaneous manifestations. Mention was made of the extreme rarity of the present case as attested by only a few cases so far reported in the literature. (Summary)