Idiopathic paraproteinemia. III. Increased frequency of paraproteinemia in thymectomized aging C57BL/KaLwRij and CBA/BrARij mice

The influence of thymectomy on the appearance of idiopathic paraproteinemia (IP) during aging was investigated in mice of the C57BL/KaLwRij and the CBA/BrARij strains, which under normal conditions develop IP in high and low frequency, respectively. Compared with sham-thymectomized mice, C57BL mice thymectomized at a young adult age showed a markedly increased frequency and an earlier onset of IP during aging; this was even more pronounced in neonatally thymectomized mice. A similar effect of thymectomy was also observed in mice of the CBA strain. Restriction in the heterogeneity of the serum immunoglobulins and the appearance of transient homogeneous Ig components was another frequent finding and this often preceded the appearance of IP in mice of both strains. Thymectomy did not substantially influence either the incidence of paraproteinemias due to a B cell malignancy or the isotype distribution among the paraproteins produced. The results are compatible with the hypothesis that IP develops in three stages as a consequence of an age-related immunnodeficiency that primarily affects the T immune system.     

Urinary schistosomiasis: a report of four cases and a review.

Urinary schistosomiasis is a common cause of hematuria in tropical regions, where it most often affects teenage boys. Children presenting with hematuria in the developed world are usually considered to have bacterial cystitis or a structural lesion and are investigated and treated accordingly. The authors report on a family recently returned to Canada from Nigeria in which all four children had urinary schistosomiasis. Nocturnal enuresis was the presenting symptom in the index case; this patient also had hematuria and dysuria. Nocturia occurred in a second child, and the other two children were asymptomatic. All four were cured by a single dose of praziquantel, a new schistosomicide. The life cycle of the causative organism, the clinical manifestations of schistosomiasis, the host response and treatment with praziquantel are reviewed.     

Gaucher disease diagnosed after bone marrow trephine biopsy - a report of two cases

The hematologist is at the forefront of specialists to whom patients with Gaucher disease present because of cytopenia and hepatosplenomegaly. Usually, patients with such symptoms have undergone trephine biopsy. We present the cases of two patients in whom Gaucher disease was suspected because of the discovery of Gaucher cells in trephine biopsy, and subsequently confirmed via enzymatic and molecular investigations.     

Acute pulmonary adiaspiromycosis. Report of three cases and a review of 16 other cases collected from the literature

We describe three cases of adiaspiromycosis with acute clinical manifestations and diffuse lung lesions. Sixteen previously reported similar cases are also reviewed. The best designation for this syndrome is acute pulmonary adiaspiromycosis.     

Folate-responsive neuropathy: report of 10 cases.

Ten patients with severe neurological disease that was clinically indistinguishable from subacute combined degeneration of the spinal cord were found to have normal serum vitamin B12 levels. All were folate deficient. Specific folate treatment led to significant reversal of the neuropathy. These findings indicate the need to review orthodox concepts of the role of folic acid in maintaining the integrity of the nervous system.     

Yellow-nail syndrome: report of three cases.

The yellow nail syndrome, a combination of yellow discolouration of and dystrophic changes in the nails, pleural effusions and lymphedema, is thought to be relatively rare; to date 44 cases have been reported. Of a further three patients with this syndrome, one had all three features, one had the yellow nails alone and the other had pleural effusions and lymphedema without classic nail changes. Each had recurrent lower respiratory tract infections; and of all 47, chronic pulmonary infections occurred in approximately one quarter and were frequently associated with chronic sinus infections. The underlying abnormality is presumed to be a congenital defect of the lymphatics, but so far this has not been demonstrated to be the cause of the nail changes, the pathogenesis of which remains obscure.     

Coronary stenting using a 6 F. diagnostic catheter: a report of two cases

Standard guiding catheters for coronary interventions may not selectively engage the coronary ostium despite a successful cannulation with a diagnostic catheter of the same curve. This discrepancy is explained by a slight difference in shape between the two catheters (shorter tip of the guiding, absence of tip tapering, etc.). The authors report on the use of 6 F. coronary diagnostic catheters as guiding catheters for coronary angioplasty and stent implantation in two patients with critical stenoses: in the right coronary artery distal to a right internal mammary artery anastomosis in one and in the ostial right coronary artery in the other. In both cases, the use of diagnostic catheters was the key to success despite the vessel orifice being unfavourable for the use of different types of guiding catheters.