Platelet dysfunction in myeloproliferative syndromes

The most common abnormality of the platelets in 43 patients with a variety of myeloproliferative syndromes was impaired or absent aggregation when stimulated with collagen and adrenaline. Eight unselected cases studied in more detail showed a normal prostaglandin synthesis pathway as evidenced by normal aggregation with arachidonic acid and the production of normal amounts of malonyldialdehyde. Mixing experiments with aspirin-tested platelets showed correction of the abnormal adrenaline and collagen responses and confirmed that the nature of the defect was different from that induced by aspirin. Stimulation of “myeloproliferative” platelets with thrombin after blocking the prostaglandin pathway with aspirin resulted in reduced aggregation, indicating either a deficiency of the storage pool of adenine nucleotides in the platelets or an abnormality of a membrane receptor for thrombin.     

Long-term evaluation of children with nephrotic syndrome and focal segmental glomerular sclerosis

We studied the long-term outcome of a group of children with the nephrotic syndrome who showed the histological lesion of focal segmental glomerular sclerosis (FSGS) during the course of their illness. Of 25 such children studied, a complete follow-up ranging from 3 to 19 years was available in 24. Two distinct groups could be identified. Patients in the first group were characterized by steroid resistance (SR) from the onset, whereas those in the second group were initially steroid sensitive (SS), and had the histological lesion of minimal change which, over time, evolved into FSGS. SR patients had a mean age of 7.7 +/- 3.7 years compared to SS patients who were 3.5 +/- 2.5 years old (p less than 0.01). There were more females (11 of 14) in the SR group than in the SS group (3 of 10; p less than 0.02). The incidence of hematuria was higher in the SR patients (9 of 14) than SS patients (2 of 10; p less than 0.05). SR patients also exhibited a greater degree of growth retardation at the end of the follow-up period (9 of 13 compared to 1 of 8 SS patients; p less than 0.02). SR patients reached end-stage renal failure earlier (2.3 +/- 1.3 years) than SS patients (10 +/- 5.8 years; p less than 0.01) after the initial biopsy. Of the 13 kidney transplanted into 9 SR patients, recurrence of FSGS was noted in two allografts. Of the 4 kidneys transplanted into 2 SS patients, recurrence was seen in 1. The overall recurrence rate of FSGS in allografts was 17.6%. Our study suggests that the two varieties of FSGS occurring in nephrotic patients may be distinct nosologic entities rather than a single disease with varied manifestations.     

Inadvertent duodenal stenting: a case report.

A Gianturco type metallic stent placed in the common bile duct migrated into the duodenum. The device remains in situ, and has caused no ill-effects to date. This raises the possibility that such stents may be deployed in the gastrointestinal tract.     

The Tenth Congress of the International Pediatric Nephrology Association

The Tenth Congress of the International Pediatric Nephrology AssociationSantiago-Chile 27 August/1 September, 1995