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

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

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

Nineteen comprehensive hospital information system (HIS) data are collected. Cleaning up the database, then analysis the patients' information. Using the basic description method and association rules to analysis the data. Analysis the data come from HIS database, we found that in 2 110 patients, main five complications are Henoch Schonlein purpura nephritis, upper respiratory tract infections, renal insufficiency, virus infection and glomerulonephritis (chronic), treatment of chemical drugs with sugar cortical hormone and anti infection drugs, such as intravenous input cephalosporin drugs. Traditional Chinese medicine with blood-activating and stasis-resolving medicine, heat clearing and detoxifying medicine. Combination scheme with blood-activating and stasis-resolving medicine joint glucocorticoid, followed by blood-activating and stasis-resolving medicine combined antiallergic drugs, heat-clearing and detoxifying medicine combined antiallergic drugs. Promoting blood circulation and removing blood stasis is an important part of traditional Chinese medicine treatment in patients with Henoch Schonlein purpura.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Anti-Allergic Agents ; therapeutic use ; Child ; Child, Preschool ; Female ; Glucocorticoids ; therapeutic use ; Hospitals ; Humans ; Infant ; Infant, Newborn ; Male ; Medicine, Chinese Traditional ; methods ; Middle Aged ; Purpura, Schoenlein-Henoch ; complications ; diagnosis ; drug therapy ; Young Adult

Acute Kidney Injury ; blood ; etiology ; therapy ; Anti-Retroviral Agents ; administration & dosage ; CD4 Lymphocyte Count ; Disease Progression ; Embolization, Therapeutic ; methods ; Fatal Outcome ; Gastrointestinal Hemorrhage ; diagnostic imaging ; etiology ; physiopathology ; therapy ; Glucocorticoids ; administration & dosage ; HIV Infections ; complications ; diagnosis ; immunology ; HIV-1 ; drug effects ; isolation & purification ; Humans ; Male ; Middle Aged ; Purpura, Schoenlein-Henoch ; complications ; diagnosis ; physiopathology ; Radiography ; Renal Dialysis ; methods

Henoch-Schonlein purpura (HSP) is common in childhood and often self-limiting. There have been limited studies on elderly-onset HSP nephritis (HSPN). A 76-yr-old man was transferred to our hospital with a 1-month history of oliguria, abdominal pain, edema and palpable purpura in the legs. Three months ago, he was admitted to another hospital with jaundice, and consequently diagnosed with early common bile duct cancer. The patient underwent a Whipple's operation. Antibiotics were administrated because of leakage in the suture from the surgery. However, he showed progressive renal failure with edema and purpura in the legs. Laboratory investigations showed serum creatinine 6.4 mg/dL, 24-hr urine protein 8,141 mg/day, myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA) 1:40 and C3 below 64.89 mg/dL. Renal biopsy showed crescentic glomerulonephritis, as well as mesangial and extracapillary Ig A deposition. We started steroid therapy and hemodialysis, but he progressed to end-stage renal failure and he has been under maintenance hemodialysis. We describe elderly onset HSPN with MPO-ANCA can be crescentic glomerulonephritis rapidly progressed to end stage renal failure.
Aged ; Antibodies, Antineutrophil Cytoplasmic/*analysis ; Common Bile Duct Neoplasms/complications/surgery ; Complement C3/analysis ; Creatinine/blood ; Edema/drug therapy ; Enzyme-Linked Immunosorbent Assay ; Glomerulonephritis/pathology ; Humans ; Male ; Purpura, Schoenlein-Henoch/*diagnosis/drug therapy ; Renal Dialysis ; Renal Insufficiency/etiology/pathology ; Steroids/therapeutic use

Child ; Child, Preschool ; Endoscopy, Gastrointestinal ; methods ; Female ; Gastrointestinal Diseases ; diagnosis ; Humans ; Male ; Purpura, Schoenlein-Henoch ; complications ; diagnosis

Child ; Child, Preschool ; Female ; Humans ; Intussusception ; etiology ; Male ; Purpura, Schoenlein-Henoch ; complications ; diagnosis ; therapy

Humans ; Nervous System Diseases ; diagnosis ; etiology ; therapy ; Purpura, Schoenlein-Henoch ; complications

OBJECTIVETo investigate the clinical significance of serum Cyst-C and urinary microalbumin in early renal impairment in children with Henoch-Schonlein purpura (HSP).

METHODSForty-eight children with HSP and who had normal serum creatinine level and 31 healthy children were enrolled. Contents of serum Cyst-C and urinary microalbumin were measured using ELISA and immunoturbidimetry, respectively. Urinary routine examination was performed in children with HSP. The contents of serum Cyst-C and urinary microalbumin were re-examined one month after treatment (recovery phase).

RESULTSThe contents of serum Cyst-C (2.24+/- 0.81 mg/L) and urinary microalbumin (20.04+/- 10.32 mg/L) in the HSP group at the acute phase were significantly higher than those in the control (0.85+/- 0.20 and 2.30+/- 1.38 mg/L respectively; P< 0.01). Serum Cyst-C (1.70+/- 0.30 mg/L) and urinary microalbumin contents (13.20+/- 8.16 mg/L) were significantly reduced at the recovery phase compared with those at the acute phase in the HSP group (P< 0.01). The proportion of urinary routine abnormality (33.3%) was significantly lower than that of urinary microalbumin (68.8%) and serum Cyst-C abnormalities (72.9%) in the HSP group (P< 0.01).

CONCLUSIONSSerum Cyst-C and urinary microalbumin may serve as indexes in the assessment of early renal impairment in children with HSP.

Adolescent ; Albuminuria ; etiology ; Child ; Child, Preschool ; Creatine ; blood ; Cystatin C ; blood ; Female ; Glomerular Filtration Rate ; Humans ; Kidney Diseases ; diagnosis ; etiology ; Male ; Purpura, Schoenlein-Henoch ; blood ; complications ; urine