OBJECTIVE: To explore the value of galactose-deficient IgA1 (Gd-IgA1) in the early diagnosis of Henoch-Schönlein purpura nephritis (HSPN) in children. METHODS: A total of 67 hospitalized children who were definitely diagnosed with HSPN between January and April 2018 and 58 hospitalized children with Henoch-Schönlein purpura (HSP) were enrolled in the study. Twenty children undergoing routine physical examinations served as controls. The levels of serum and urine Gd-IgA1 were determined using ELISA. The receiver operating characteristic curve was used to analyze the value of serum Gd-IgA1 and urine Gd-IgA1/urine creatinine ratio in the diagnosis of HSPN. RESULTS: The level of serum Gd-IgA1 and urine Gd-IgA1/urine creatinine ratio in children with HSP or HSPN were significantly higher than those in healthy control group (P<0.01), with a significantly greater increase observed in children with HSPN (P<0.01). Serum Gd-IgA1 ≥1 485.57 U/mL and/or urine Gd-IgA1/urine creatinine ratio ≥105.74 were of favorable value in the diagnosis of HSPN. During the six-month follow-up of the 49 children with HSP, the incidence of HSPN was 47% (23/49), which included a 100% incidence in children with serum Gd-IgA1 ≥1 485.57 U/mL and a 73% incidence in children with urine Gd-IgA1/urine creatinine ratio ≥105.74. CONCLUSIONS Serum and urine Gd-IgA1 is of favorable clinical value in the early diagnosis of HSPN.
Child ; Early Diagnosis ; Galactose ; Glomerulonephritis, IGA ; Humans ; Immunoglobulin A ; Purpura, Schoenlein-Henoch

Henoch-Schönlein purpura (HSP) is systemic vasculitis disease with various clinical manifestations. Gastrointestinal symptoms in patients with HSP are usually common, with an incidence rate of 62-90%. Most of these gastrointestinal symptoms occur after typical skin purpura, which is a very important clinical evidence for making a diagnosis of HSP. It is difficult to diagnose HSP without skin rash. About 25% of patients may experience gastrointestinal symptoms as their first symptoms. Herein, we report a case of ileo-colic intussusception associated with HSP in a 5-years-old girl presented with diffuse abdominal distension. Our patient did present any symptoms of HSP, such as purpura, arthralgia or arthritis, before surgery.
Arthralgia ; Arthritis ; Diagnosis ; Exanthema ; Female ; Humans ; Ileus ; Incidence ; Intussusception* ; Purpura* ; Purpura, Schoenlein-Henoch ; Skin ; Systemic Vasculitis

Acute Disease ; Humans ; Male ; Middle Aged ; Pancreatitis, Chronic ; diagnosis ; drug therapy ; Purpura, Schoenlein-Henoch ; diagnosis ; drug therapy ; Steroids ; therapeutic use ; Tomography, X-Ray Computed

OBJECTIVETo investigate the changes and clinical significance of biomarker fecal bile acids (BA) in children with Henoch-Schönlein purpura (HSP).

METHODSNineteen children with HSP and twenty-seven healthy children were enrolled in this study. The stool samples were obtained at the acute and remission phases. Fecal BA levels were measured by high performance liquid chromatography mass spectrometry (HPLC-MS).

RESULTSThe fecal cholic acid level in the HSP remission group was significantly higher than in the HSP acute group and the healthy control group (P<0.016). The fecal chenodeoxycholic acid level in the HSP remission group was significantly higher than in the healthy control group (P<0.016). The levels of fecal secondary colonic bile acids, deoxycholic acid and lithocholic acid, in the HSP acute and remission groups were significantly lower than in the healthy control group(P<0.05, P<0.016 respectively). No significant differences were found in the levels of fecal urosodeoxycholic acid among the three groups (P>0.05).

CONCLUSIONSFecal secondary colonic bile acids, deoxycholic acid and lithocholic acid, are in decrease in children with HSP at the acute stage, which may be involved in the pathogenesis and treatment outcomes of HSP.

Bile Acids and Salts ; analysis ; Biomarkers ; analysis ; Child ; Feces ; chemistry ; Female ; Humans ; Male ; Purpura, Schoenlein-Henoch ; diagnosis ; therapy

OBJECTIVETo screen biomarkers which can be used as an auxiliary method in the diagnosis of Henoch-Schönlein purpura (HSP) and to evaluate their diagnostic values by receiver operating characteristic (ROC) curve analysis.

METHODSA total of 127 children diagnosed with HSP between April 2012 and March 2014 were included in the HSP group and an equal number of healthy children were included in the control group. Twelve parameters, i.e., serum amyloid protein A (SAA), interleukin-6 (IL-6), immunoglobulins (IgA, IgG, IgM, and IgE), C-reactive protein (CRP), white blood cell (WBC) count, complements C3 and C4, anti-streptolysin O, and ferritin, were analyzed. The values of the screened biomarkers for diagnosis of HSP were assessed by ROC curve analysis.

RESULTSThe HSP group had significantly higher levels of SAA, IL-6, CRP, WBC, IgA, and IgM than the control group (P<0.05). The areas under the ROC curve of SAA, IL-6, WBC, IgA, and IgM for the diagnosis of HSP were higher than 0.7 (P<0.05). The optimal cut-off values of SAA, IgA, IgM, WBC, and IL-6 for the diagnosis of HSP were 3.035 μg/mL, 1579.5 mg/L, 922.5 mg/L, 8.850 × 10⁹/L, and 7.035 pg/mL, respectively; the corresponding sensitivities of the optimal cut-off values for the diagnosis of HSP were 95.1%, 75.6%, 72.3%, 78.0%, and 63.4%, respectively, and the corresponding specificities were 90.2%, 85.4%, 82.4%, 70.7%, and 80.5%, respectively.

CONCLUSIONSSAA, IgA, IgM, WBC, and IL-6 are valuable biomarkers for clinical diagnosis of HSP and among them SAA seems to be the best one.

Adolescent ; Biomarkers ; blood ; C-Reactive Protein ; analysis ; Child ; Child, Preschool ; Female ; Humans ; Male ; Purpura, Schoenlein-Henoch ; blood ; diagnosis ; ROC Curve ; Serum Amyloid A Protein ; analysis

No abstract available.
Anti-Bacterial Agents/therapeutic use ; Biopsy ; Echocardiography, Doppler, Color ; Echocardiography, Transesophageal ; Endocarditis, Bacterial/complications/diagnosis/drug therapy/*microbiology ; Fluorescent Antibody Technique ; Heart Septal Defects, Ventricular/*complications/diagnosis/surgery ; Humans ; Male ; Middle Aged ; Predictive Value of Tests ; Pulmonary Valve Stenosis/*complications/diagnosis ; Purpura, Schoenlein-Henoch/diagnosis/drug therapy/*etiology ; Risk Factors

Henoch-Schonlein purpura can result from exposure to an antigen after infection with several types of organisms. However, Henoch-Schonlein purpura caused by a primary Epstein-Barr virus infection has been rarely reported. Here, we report the case of a 32-month-old female patient who presented with Henoch-Schonlein purpura. Based on abnormal liver function test results and positive results for Epstein-Barr virus infection markers, a diagnosis of Epstein-Barr virus hepatitis manifesting as Henoch-Schonlein purpura was made. Treatment with methylprednisolone and hydration improved the symptoms, and a switch to oral steroids was effective in completely alleviating the purpura. No recurrence was noted and no liver function abnormalities were detected during the follow up period.
Arthritis ; Child, Preschool ; Diagnosis ; Female ; Follow-Up Studies ; Hepatitis* ; Herpesvirus 4, Human* ; Humans ; Liver ; Liver Function Tests ; Methylprednisolone ; Purpura ; Purpura, Schoenlein-Henoch* ; Recurrence ; Steroids

Henoch-Schonlein purpura can result from exposure to an antigen after infection with several types of organisms. However, Henoch-Schonlein purpura caused by a primary Epstein-Barr virus infection has been rarely reported. Here, we report the case of a 32-month-old female patient who presented with Henoch-Schonlein purpura. Based on abnormal liver function test results and positive results for Epstein-Barr virus infection markers, a diagnosis of Epstein-Barr virus hepatitis manifesting as Henoch-Schonlein purpura was made. Treatment with methylprednisolone and hydration improved the symptoms, and a switch to oral steroids was effective in completely alleviating the purpura. No recurrence was noted and no liver function abnormalities were detected during the follow up period.
Arthritis ; Child, Preschool ; Diagnosis ; Female ; Follow-Up Studies ; Hepatitis* ; Herpesvirus 4, Human* ; Humans ; Liver ; Liver Function Tests ; Methylprednisolone ; Purpura ; Purpura, Schoenlein-Henoch* ; Recurrence ; Steroids

No abstract available.
Biopsy ; DNA Mutational Analysis ; *Diagnostic Errors ; Enzyme Replacement Therapy ; Fabry Disease/complications/*diagnosis/enzymology/genetics ; Genetic Predisposition to Disease ; Glomerulonephritis, IGA/diagnosis/etiology ; Humans ; Male ; Middle Aged ; Mutation ; Phenotype ; Predictive Value of Tests ; Purpura, Schoenlein-Henoch/*diagnosis ; alpha-Galactosidase/genetics/therapeutic use

We aimed to investigate differences in clinical manifestations and outcomes between adult and child patients with Henoch-Schonlein purpura (HSP), and to analyze the factors associated with poor prognosis for HSP nephritis. This retrospective 10-yr study enrolled 160 patients with HSP who visited Severance Hospital. Purpura was mostly detected in lower extremities, but purpura in upper extremities was more frequently observed in adults than children (41.7% vs 19.3%). Children had a greater frequency of arthralgia (55.4% vs 27.1%), while adults had a greater frequency of diarrhea (20% vs 1.6%). Anemia, elevated C-reactive protein, and level of IgA were more frequently observed in adults (25% vs 7.1%, 65.6% vs 38.4%, 26.3% vs 3.5%). Renal involvement in adults was more severe than in children (79.2% vs 30.4%). Chronic renal failure showed a significant difference in outcomes of HSP between adults (10.4%) and children (1.8%) after a follow up period of an average of 27 months. Furthermore, renal insufficiency at diagnosis was significantly related to the progression to chronic renal failure. Our results showed several differences in the clinical features of HSP between adults and children. Adults with HSP had a higher frequency of renal insufficiency and worse renal outcomes than children. Renal insufficiency at diagnosis might be of predictive value for the progression to chronic renal failure in HSP patients.
Adult ; Aged ; Aged, 80 and over ; Arthralgia/epidemiology/etiology ; C-Reactive Protein/analysis ; Child ; Child, Preschool ; Diarrhea/epidemiology/etiology ; Extremities/pathology ; Female ; Follow-Up Studies ; Humans ; Immunoglobulin A/blood ; Immunosuppressive Agents/therapeutic use ; Infant ; Middle Aged ; Odds Ratio ; Prognosis ; Purpura, Schoenlein-Henoch/complications/*diagnosis/drug therapy/*pathology ; Renal Insufficiency/epidemiology/etiology ; Retrospective Studies ; Young Adult