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F Mampaso

Publications and source records attributed to F Mampaso.

97 records · Page 6Linked to original sources

Familial C1q deficiency associated with renal and cutaneous disease.

A familial C1q deficiency of complement in three siblings has been established. The patients were two brothers and a sister (12, 11 and 9 years old) with clinical and pathological features of Rothmund-Thomson syndrome (Poikiloderma congenital) and mesangial proliferative glomerulonephritis with diffuse IgM deposits. Abnormality has been defined as a total lack of CH50 haemolytic activity, undetectable C1q, failure to correct the defect with functionally pure C2 to C9 complement components, normal values for C2, C3, C4 and C5 and restoration of CH50 haemolytic activity when purified human C1q was added to the assay.

Adult↗

Mesangial deposits of IgM in patients with the nephrotic syndrome.

Kidney biopsies from fourteen patients with the nephrotic syndrome were studied by light, immunofluorescence and electron microscopy. Morphologically, all cases showed moderate diffuse mesangial cell proliferation. In all cases, immunofluorescent microscopy demonstrated diffuse and generalized deposits of IgM as the sole or predominant immunoglobulin. Electron microscopic examination showed electron dense deposits localized in the mesangium in seven cases. One nephrotic patient experienced spontaneous remission and eight others were steroid responsive. Only one of the five steroid resistant patients treated with chlorambucil showed clinical remission. Five steroid responsive patients relapsed. At present, four patients are healthy, having not relapsed for the past two years. Although deposits of IgM may be related to the renal pathology in cases of the nephrotic syndrome, immunofluorescence findings do not seem to be sufficiently consistent or characteristic to justify a possible subclassification of the idiopathic nephrotic syndrome.

Adolescent↗

A possible common pathogenesis of the mesangial IgA glomerulonephritis in patients with Berger's disease and Schönlein-Henoch syndrome.

High serum levels of polymeric IgA, partially as immune complexes, have been found in patients with Berger's disease and Schönlein-Henoch syndrome. Polymeric IgA was also found in the renal mesangium in both entities as judged by its affinity for the human secretory component. These data reinforce the clinical and morphological suspicion that both entities may represent variations of the spectrum of the same disease.

Fluorescent Antibody Technique↗

Tubulointerstitial nephritis and asymptomatic uveitis.

We describe a case of a 10 year-old boy who had fever, weakness, anorexia, weight loss and general malaise. No other remarkable symptoms were present. He had been treated with Aspirin and Ibuprofen. Deterioration of renal function, glucosuria, proteinuria, anemia and increased erythrocyte sedimentation rate were detected. After 7 days observation with no treatment, renal function worsened, glucosuria increased and fever persisted. A renal biopsy was performed and acute tubulointerstitial nephritis was diagnosed. The most common aetiologies of this entity were excluded. An ophthalmologic study revealed bilateral anterior uveitis, therefore the patient was diagnosed as having tubulointerstitial nephritis with uveitis. The child improved on corticosteroid therapy, but uveitis relapsed when treatment was stopped.

Biopsy, Needle↗

Steroid-dependent nephrotic syndrome with minimal glomerular changes and mesangial IgA deposits.

We report 3 children with steroid-dependent nephrotic syndrome, without hematuria. The renal biopsies showed minimal-change disease with mesangial IgA deposits. Their clinical presentation and therapeutic responses were similar to our cases of biopsied nephrotic syndrome with minimal glomerular abnormalities. We believe that our cases have minimal-change disease and not Berger's disease with nephrotic syndrome.

Child↗