Humans ; Hypertensive Encephalopathy ; etiology ; Purpura, Schoenlein-Henoch ; complications ; diagnosis

Diagnosis, Differential ; Humans ; Intestinal Diseases ; etiology ; Intussusception ; etiology ; Pancreatitis ; complications ; diagnosis ; Purpura, Schoenlein-Henoch ; complications ; diagnosis

Common complications of Henoch-Schonlein purpura (HSP) that lead to surgical intervention include intussusception, perforation, necrosis, and massive gastrointestinal bleeding. Acute appendicitis is rarely seen as a complication of HSP. A sevenyear-old boy was admitted for arthralgia, abdominal pain, hematochezia, melena, and purpuric rash on the lower extremities. On admission day abdominal ultrasonography was normal, but on day 5, he became pyrexial and developed right iliac fossa pain and tenderness with guarding. Ultrasonography showed distended appendix surrounded by hyperechoic inflamed fat. On exploration an acutely inflamed, necrotic appendix was removed and grossly there was an appendiceal perforation in the appendiceal tip. Microscopically some of the small blood vessels in the submucosa showed fibrinoid necrosis with neutrophilic infiltrations. The authors report the case of a child who developed acute perforative appendicitis requiring appendectomy while on treatment for HSP.
Appendicitis/*diagnosis/*etiology ; Child ; Diagnosis, Differential ; 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

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

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

OBJECTIVEHenoch-Schonlein purpura nephritis (HSPN) and IgA nephropathy are very similar in immunopathological changes, and therefore some nephrologists considered that they are substantially one disease entity caused by IgA immune abnormalities, and IgA nephropathy is, in fact, a kind of HSPN without rashes. The present study aimed to characterize their relationship through clinico-pathological comparison between IgA nephropathy and HSPN.

METHODSThirty-one children with IgA nephropathy aged from 3 to 15 years and 120 children with HSPN aged between 4 to 15 years were enrolled in this study. Their clinical manifestations, blood biochemical test, serum immunology and follow-up data were collected and analyzed. Renal pathological findings in light microscopy, immunofluorescence and electron microscopy were analyzed and also compared between 31 children with IgA nephropathy and 32 children with HSPN.

RESULTSThe age of onset was over 12 years in 25.8% children with IgA nephropathy, but only in 10% with HSPN, and the difference was significant (P < 0.05). The clinical patterns of IgA nephropathy and HSPN were similar, but extra-renal manifestations were seen more often in HSPN, all of them had skin purpura, 59% had gastrointestinal symptoms and 47% suffered from arthralgia. While the abdominal pain occurred only in 3.2% children with IgA nephropathy. The renal pathological investigation showed global sclerosis in 35.5% of IgA nephropathy and 3.1% of HSPN, mesangial sclerosis in 41.9% of IgA nephropathy and 6.3% of HSPN, but endothelial proliferation in 65.6% of HSPN and 29% of IgA nephropathy. The differences were extremely significant (P < 0.01). Thin basement membrane nephropathy were only found in 6.5% children with IgA nephropathy, but in none with HSPN. The electronic dense deposits in HSPN were sparse, loose and widely spread in glomerular mesangium, subendothelial area and even intra basement membrane. While the deposits were dense, lumpy and mostly limited in mesangium and paramesangium in IgA nephropathy. IgG was found in glomerular immune deposits in 71.9% of HSPN, but only 19.4% of IgA nephropathy. No IgG deposit was observed in 81.6% of IgA nephropathy, among them most showed IgA and IgM and/or C(3) deposit. Predominant IgG deposits were found in 12.5% of HSPN with relatively weak IgA deposit, moreover 6.3% of HSPN showed linear IgG deposits in glomerular capillary wall, which couldn't be found in IgA nephropathy. The follow-up data of average 20 months showed complete remission in 72.5% of HSPN and 19.4% in IgA nephropathy after 34 months follow-up. Moreover, 64.5% of IgA nephropathy had consistent hematuria and proteinuria, 16.1% had active nephritides, the difference was significant (P < 0.05).

CONCLUSIONSignificant clinico-pathological differences were found between HSPN and IgA nephropathy, which does not support the one disease entity hypothesis. HSPN and IgA nephropathy are probably two diseases with similar immune abnormalities.

Adolescent ; Child ; Child, Preschool ; Diagnosis, Differential ; Female ; Glomerulonephritis, IGA ; diagnosis ; Humans ; Immunoglobulin A ; blood ; Immunoglobulin G ; blood ; Immunoglobulin M ; blood ; Immunologic Tests ; Male ; Nephritis ; diagnosis ; etiology ; Prognosis ; Purpura, Schoenlein-Henoch ; complications

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

Henoch-Schonlein purpura (HSP) is a vasculitis involving small vessels of skin, joints, gastrointestinal (GI) tract, and kidneys. The patients typically show palpable purpura with one or more characteristic manifestations including abdominal pain, hematuria or arthritis. HSP shows gastrointestinal symptoms in 50~85% of patients, and in 14~40% of patients GI symptoms precede purpuric rash which makes the diagnosis of HSP difficult. We present a case of Henoch-Schonlein purpura with GI bleeding, septic shock by ileal microperforation, small bowel obstruction as a result of ileal stricture and psoas muscle abscess.
Abdominal Pain ; Anti-Inflammatory Agents/therapeutic use ; Colonoscopy ; Gastrointestinal Diseases/*diagnosis/etiology/pathology ; Gastrointestinal Hemorrhage/diagnosis ; Humans ; Male ; Middle Aged ; Prednisolone/therapeutic use ; Psoas Abscess/etiology/*radiography ; Purpura, Schoenlein-Henoch/*complications/*diagnosis/pathology ; Tomography, X-Ray Computed