Bullous presentation of Henoch-Schönlein purpura in an adult

Henoch-Sch?nlein purpura is a small vessel vasculitis occurring mainly in childhood and rarely in adulthood. Typical cutaneous eruption may begin as macular or urticarial erythematous lesions progressing to a palpable purpura. In adults, the disease has a propensity to be more severe and chronic and affects mainly the ankles and feet. Bullae, vesicles and ulcers are occasionally seen in this group. The prognosis depends on renal involvement, commonly seen in adults. We report a new case of Henoch-Sch?nlein purpura in a 36-year-old man presenting with a bullous eruption followed by the appearance of abdominal pain and hematuria.     

Brucellosis mimicking Henoch-Schönlein purpura

A young male immigrant from Syria with a vasculitic-appearing leg rash, asymmetrical polyarthritis, microscopic haematuria, and raised inflammatory markers was provisionally diagnosed with Henoch-Sch?nlein purpura. Skin biopsy showed leukocytoclastic vasculitis. Low-grade fevers persisted despite non-steroidal anti-inflammatory therapy, and Brucella sp. was subsequently grown from both blood and synovial fluid aspirates. Further tests gave positive results for B. abortus, and triple antibiotic therapy produced a rapid clinical response. Cutaneous vasculitis has rarely been described in brucellosis, and this is the first report in the English medical literature of brucellosis mimicking Henoch-Sch?nlein purpura.     

Sex, smell and vasculitis: Henoch-Schönlein purpura in Kallmann's syndrome

Kallmann's syndrome (KS) is the most common cause of isolated hypogonadotropic hypogonadism. An increased prevalence of rheumatic and autoimmune diseases has been noted in patients with hypogonadism including Kallmann's syndrome. Both in vitro and in vivo studies indicate that testosterone deficiency may promote inflammatory response such as vasculitis by altering inmmunoglobulin and cytokines profiles. We report the novel occurrence of Henoch-Sch?nlein purpura in a patient with markedly low testosterone level due to Kallmann's syndrome. We discuss the potential mechanisms by which hypoandrogenism may lead to vasculitis.     

Successful treatment of severe gastrointestinal involvement in adult-onset Henoch-Schönlein purpura

Henoch-Sch?nlein purpura is a small vessel vasculitis which is uncommon in adults. The presentations of adult-onset disease are different from those seen in childhood. The commonly-recognised serious gastrointestinal complications of childhood are less well-documented in adults. We report three cases of adult-onset Henoch-Sch?nlein purpura with severe gastrointestinal involvement. All were men, aged 22, 35 and 42 years, respectively. Two of these patients had evidence of mesenteric ischaemia on computed tomography of the abdomen. All three patients were successfully treated with steroids.     

A 10-year-old girl with IgA nephropathy who 5 years later developed the characteristic features of Henoch-Schönlein purpura nephritis

We report a patient who developed Henoch-Sch?nlein purpura (HSP) 5 years after she presented with immunoglobulin A nephropathy (IgAN). A 10-year-old Japanese female was identified with proteinuria and hematuria by a school urinary screening. The first renal biopsy showed mesangial proliferative glomerulonephritis with immunofluorescent findings consistent with IgAN. She was treated with prednisolone, warfarin, and dilazep dihydrochloride, and the proteinuria and hematuria disappeared 4 months after the onset of treatment. Five years later she developed abdominal pain, gross hematuria and a classic purpuric rash of HSP after acute pharyngitis. The second renal biopsy showed diffuse mesangial proliferation with cellular crescent formation, and the patient was treated with methylprednisolone pulse therapy, prednisolone and mizoribine, resulting in a gradual decrease in urinary protein excretion. Our patient is unusual in that she developed Henoch-Sch?nlein purpura nephritis 5 years after clinical and biopsy evidence of IgAN, which suggests that IgAN and HSP are different clinical manifestations of the same disease, probably sharing a common pathogenesis.     

Case of an elderly man with associated Henoch-Schönlein purpura during treatment of acute pancreatitis

A 70-year-old man with acute pancreatitis (acute exacerbation of chronic pancreatitis) was admitted to our department. Despite temporary improvement, the pancreatitis worsened on the 21st hospital day, forming a pancreatic pseudocyst, with infection in the cyst. After treatment with various antibiotics, a blood test on the 71st hospital day indicated improved inflammatory response despite continuing abdominal cramps. From the 75th hospital day, the patient developed purpura and arthralgia of the lower limbs, with melena and hematuria. Henoch-Schonlein purpura was diagnosed definitively by skin biopsy. Such a complication of acute pancreatitis with Henoch-Sch?nlein purpura is rare. This case also suggests that microvasculitis around the pancreas resulting from Henoch-Schonlein purpura might have prolonged the pancreatitis.     

The lymphocyte subset and blood interleukin-2 level in children with Henoch-Sch?nlein purpura before and after cimetidine treatment]

The objective of this study was to evaluate the effect of cimetidine treatment on cellular immunity in children with Henoch-Sch?nlein purpura. T cell subset was detected by double marker flow cytometry and the level of IL-2 in peripheral blood was measured by MTT method, before and after cimetidine treatment. The results showed that CD3+, CD4+ T cell subset, CD4+/CD8+ ratio, and blood IL-2 level increased after the treatment (P < 0.05). There was no significant change in CD8+ T subset after the treatment. These findings indicate that cimetidine can promote T lymphocyte proliferation and differentiation, especially TH1 lymphocyte activity.     

Distinct characteristics and new prognostic scoring system for Chinese patients with Waldenström macroglobulinemia

Background Waldenstr（o）m macroglobulinemia （WM） is an uncommon lymphoid malignancy.The characteristics and prognosis of WM have never been systematically studied in the East.Methods We analyzed the clinical characteristics and the prognostic factors of 90 Chinese WM patients,and compared them with the Western reports.Results The median age was 62 years old with a male-to-female ratio of 3.74.The most common symptoms at diagnosis were fatigue （77.8％） and bleeding （20％）,while only 6 patients （6.7％） were asymptomatic.In the univariate analysis,age ＞62 years,thrombocytopenia,leucopenia,cytopenias ≥2,and high risk on the international prognostic scoring system for WM were the adverse risk factors,but only age ＞62 years and ≥2 cytopenias were the independent prognostic factors in the multivariate analysis.Using age ＜62 years and ≥2 cytopenias,three significantly different prognostic groups could been distinguished,with 5-year overall survival of 71.6％,48.6％,and 17.0％ （P ＜0.001）.Conclusion Distinct characteristics exist in WM in China compared to the West and we describe a new simple prognostic model for newly diagnosed WM patients.     

Study on acid-base disorders in patients with adult respiratory distress syndrome

Arterial blood gases and electrolytes were determined in 159 cases of adult respirato-ry distress syndrome(ARDS).It was found that disordered acid-base balance was a commonfinding in various kinds of ARDS.In mild ARDS,respiratory alkalosis and the combination ofrespiratory alkalosis plus metabolic alkalosis or metabolic acidosis were usually encounted,whilein moderate and severe cases of ARDS,triple acid-base disorders,respiratory acidosis,and thecombination of respiratory acidosis plus metabolic acidosis were commonly seen.Severe alkalosiswas one of the factors to result in death.     

Effects of cyclosporine A on serum and urinary soluble interleukin-2 receptor in patients with lupus nephritis.

OBJECTIVE To evaluate the association of the level of urine and serum soluble interleukin-2 receptor (sIL-2R) with disease activity and response to cyclosporine A (CsA) therapy in patients with lupus nephritis (LN).

METHODS Sixteen hospitalized patients with LN were studied. At admission, fifteen patients had type IV-LN and one had type V-LN. All patients received CsA 6 mg/kg per day for 6-8 weeks, then tapered off gradually to 2 mg/kg per day. The levels of urinary and serum sIL-2R were determined by enzyme-linked immunosorbent assay (ELISA). Serum antinuclear antibody (ANA), anti-dsDNA antibody (A-ds-DNA), complement C3 and C4, total IgG, creatinine, urinary red blood cells and protein excretion, and lymphocyte subpopulations in the peripheral blood were also measured before and after CsA treatment.

RESULTS In LN patients, both urinary (534 +/- 101 U/ml) and serum SIL-2R levels (326 +/- 148 U/ml) were higher than those in normal controls. These findings were associated with higher levels of peripheral blood CD4 + and CD8 + lymphocytes (29.3 +/- 4.24 and 28.6 +/- 9.12%), higher titer of serum anti-ds-DNA, lower levels of serum complement C2 and C4 (0.98 +/- 0.23 and 0.24 +/- 0.12 g/L), as well as more proteinuria (Upro 2.99 +/- 0.76 g/24 hrs) and hematuria (URBC 83.9 +/- 95.2 10(4)/ml). These abnormalities were gradually ameliorated by CSA therapy. The changes in the levels of both serum (116 +/- 58.6 U/ml) and urine (136 +/- 43.2 U/ml) SIL-2R induced by CsA (at 8 weeks) were correlated with the changes in the levels of CD4 + and CD8 + cells (23.2 +/- 3.30 and 26.7 +/- 3.54%), degrees of immune abnormalities (serum C3 and C4 1.28 +/- 0.14 and 0.42 +/- 0.06 g/L), and renal injuries (Upro 1.07 +/- 0.46 g/24 hrs, URBC 5.82 +/- 3.15 10(4)/ml).

CONCLUSIONS These results suggest that serum and urinary sIL-2R are sensitive markers for the disease activity in patients with LN. CsA, a powerful immunosuppressive agent, significantly improves both immunologic disorders and renal functional impairments, the mechanism of which on patients with LN appears to inhibit the lymphocyte activation in the peripheral blood and renal tissues as indicated by the decrease in sIL-2R levels.

     

Complications of influenza in 272 adult and pediatric patients in a German university hospital during the seasonal epidemic 2017–2018

Wiener Medizinische Wochenschrift - The influenza season 2017–2018 of the northern hemisphere was the highest since 2001 and was caused predominantly by influenza&nbsp;B virus. We...     

Association of defective HLA-Ⅰ expression with antigen processing machinery and their association with clinicopathological characteristics in Kazak patients with esophageal cancer

Background It has been confirmed that defective expression of human leukocyte antigen class Ⅰ (HLA-Ⅰ) molecules can contribute to the immune evasion of cancer cells in some types of cancer. The aim of this study was to examine the expression of HLA class Ⅰ antigen and the antigen-processing machinery (APM) components in esophageal squamous cell carcinoma (ESCC) and their role in high risk human papillomavirus (HPV) infection, and to analyze their association with histopathological characteristics in the Kazak ethnic group.Methods A total of 50 formalin-fixed, paraffin-embedded ESCC lesions were collected from the First Affiliated Hospital of Xinjiang Medical University, China. The expression levels of HLA-Ⅰ antigen and APM components were determined by immunohistochemistry; the HPV DNA were detected using polymerase chain reaction (PCR).Results A high frequency of down-regulation or loss of expression of HLA and APM components were found in esophageal cancer in Kazak people. HLA-Ⅰ, TAP1, CNX, LMP7, Erp57, Tapasin and ERAP1 were down-regulated in 68%,44%, 48%, 40%, 52%, 32% and 20% of ESCC lesions then, respectively. The loss of expression of HLA-Ⅰ antigen was significantly correlated with part of the APM components and positively correlated with high risk HPV16 infection. TAP1,CNX, LMP7, Erp57 and Tapasin loss were significantly associated with tumor grading, lymph node metastasis and depth of invasion (P＜0.05).Conclusion Our results suggest that APM component defects are a mechanism underlying HLA-Ⅰ antigen down-regulation in ESCC lesions, and indicate that the loss expression of HLA-Ⅰ and APM components will become an important marker of ESCC and analysis of HLA-Ⅰ and APM component expression can provide useful prognostic information for patients with ESCC from the Kazak ethnic group.     

Effect of metabolic syndrome on prognosis and clinical characteristics of revascularization in patients with coronary artery disease

Background People with metabolic syndrome are at higher risk for developing coronary artery disease (CAD). The effect of the metabolic syndrome on outcomes in patients with preexisting CAD has not been well studied. This study was conducted to assess the prevalence, characteristics, in hospital and long term prognosis of CAD with metabolic syndrome and to determine the factors influencing the prognosis of the disease. Methods The DESIRE registry contains data of 3696 patients with CAD between 2001 and 2004. Mean long term followup was (829±373) days. Diagnosis of metabolic syndrome was based on modified International Diabetes Federation (IDF) Worldwide Definition of the Metabolic Syndrome，using body mass index (BMI) instead of waist circumference. Results Of 2596 patients with complete records of height, weight, and so on, 1280 (49.3%) were identified with metabolic syndrome. The patients with metabolic syndrome had higher level of body mass index, systolic blood pressure, diastolic blood pressure, fasting glucose and disordered blood lipid (all P&lt;0.0001), with higher creatinine [(10.5±4.3) mg/L vs (9.9±2.9) mg/L, P&lt;0.0001] and the number of white blood cells [(7.49±2.86)×10(9)/L vs (7.19±2.62) ×10(9)/L, P=0.008) compared with those without metabolic syndrome. The patients with metabolic syndrome showed severer coronary angiographic alterations (left main artery and/or ≥2-vessel) (73.6% vs 69.6%, P=0.031). There were no significant differences of major adverse cardiac and cerebral events (MACCE) or mortality in hospital between the two groups. During followup, the ratio of MACCE in CAD with metabolic syndrome patients increased significantly (11.8% vs 10.0%, P=0.044). Fasting blood glucose (≥1000 mg/L) and triglyceride (TG, ≥1500 mg/L) were responsible for most of the increased risk associated with the metabolic syndrome (adjusted OR 1.465, 95% CI 1.037－1.874, P=0.032; OR 1.378, 95% CI 1.014－1.768, P=0.044). Conclusions The prevalence of metabolic syndrome was very high in CAD patients. The metabolic syndrome confers a higher risk of long term MACCE in patients with CAD, and dysglycaemia and hypertriglycaemia appear to be responsible for most of the associated risk.     

Neurophysiological characteristics in infants and young children with auditory neuropathy

Objective: To analyze neurophysiological characteristics in infants and young children with auditory neuropathy (AN) and explore their clinical significance. Methods: Audiological measurements(acoustic immittance, EOAEs, ABR, CM, MLR and ERPs) and peripheral neurological tests were conducted and evaluated in 13 infants and young children with AN. Six of them received highresolution temporal bone CT scans and/or cerebral MRI examinations. Results: All of the children showed type ““A““ tympanograms with abseatation of stapedial reflexes. EOAEs were normal in 12 of 13 subjects. In one child who had a history of anoxia during the birth, the EOAEs were not elicited. Click-evoked ABRs were absent in 12 of 13 subjects when maximum output of the instrument was reached. The CM potentials were presented bilaterally in all individuals, which were independent of the EOAEs and ABR. Of eight cases tested, all had clear MLR and six showed normal ERPs(P300 and MMN). Peripheral neurological tests and radiological findings were within the normal ranges. Conclusion: The diagnosis of AN in infants and young children should focus on analyzing their neurophysiological characteristics,especially on CM,MLR and ERPs. Combined use of EOAEs, ABR and CM was recommended for hearing screening on newborns with high risk factors.     

Serum resistin and adiponectin concentrations in patients with overweight and obesity

Objective： To explore the serum levels of resistin and adiponectin in patients with overweight and obesity. Methods： Fifty-eight cases with normal weight and 24 patients with overweight and obesity have taken fasting blood samples for measurements of plasma glucose, plasma lipids, insulin, C-peptide, thyroid hormones, C-response protein, interleukin-6, TNF-α, leptin, adiponectin and resistin. Results： The concentrations of resistin in cases with overweight and obesity were significant higher than those in the normal weight cases （16. 01 ± 8. 60 vs 11. 63 ± 9. 05 ng/ml, P = 0. 047）. Pearson relation analysis showed that serum resistin concentrations were positively correlated with age （r= 0. 476, P = 0.019）, but negatively correlated with C-peptide （r=-0.45, P=0. 024）, and adiponectin concentrations were positively correlated with HDL-c （r=0. 463, P=0. 023） and systolic blood pressure （r=0. 409, P=0. 047） in overweight and obesity cases. Conclusion： The concentrations of resistin in cases with over-weight and obesity are higher, and there is no correlation between resistin and blood glucose, blood lipids and insulin, while the serum adiponectin concentrations positively correlated with HDL-c and systolic blood pressure.