Shunsuke Kitagawa

Hereditary haemorrhagic telangiectasia (Rendu-Osler-Weber disease)

Correspondence to: Dr Uichiro Fuchizaki, Keiju Medical Center, 94 Tomioka-cho, Nanao, Ishikawa 926-8605, Japan (e-mail: fuchi@p1.coralnet.or.jp) Hereditary haemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber disease, is an autosomal dominant disorder characterised by epistaxis, cutaneous telangiectasia (figure 3), and visceral arteriovenous malformations (AVMs). The characteristic lesions in this disorder are telangiectases that consist of focal dilatations of postcapillary venules and AVMs. Recurrent epistaxis often restricts daily life, as in our patient (case presentation). AVMs can cause serious complications if located in the lungs, liver, or brain. Hereditary haemorrhagic telangiectasia (Rendu-Osler-Weber disease)

Acute glomerulonephritis after human parvovirus B19 infection

We evaluated clinical and histological characteristics of four adult patients who presented with acute glomerulonephritic syndrome with serological confirmation of recent HPB19 infection. All patients had generalized edema with urinary abnormalities. Body weight gain ranged from 3 to 10 kg. Three of the patients had contact with erythema infectiosum simultaneously with or within 10 days before development of flu-like symptoms. Two patients had an erythematous rash, and one patient had lower-extremity purpura. Joint pain was present in three of the patients. All patients had proteinuria and hematuria. Renal functions were normal except in one patient who had a serum creatinine of 3.2 mg/dL. Three of the patients had hypocomplementemia. All renal biopsy specimens were characterized by glomerular leukocyte infiltration and endothelial cell swelling. Mesangiolysis was seen in three of the patients. C3 was deposited in a coarse granular pattern along the capillary walls in all cases. Electron microscopic examination showed marked expansion of the subendothelial space of glomerular capillaries in all patients. Subendothelial electron-dense deposits were present in all patients. Immunohistochemical analysis using monoclonal anti-HPB19 antibody showed that one of the four patients had positive staining in the glomeruli. DNA extracted from renal biopsy specimens contained HPB19 DNA, as shown by polymerase chain reaction (PCR) analysis in all patients. PCR amplification of the renal DNA generated a 104-bp product, which hybridized to an HPB19-specific probe. No control group subjects contained HPB19 DNA as determined by PCR. This circumstantial evidence indicates that HPB19 infection may be one of the causes of acute glomerulonephritis in normal individuals.