Chronic allogeneic disease. I. Development of glomerulonephritis.

Glomerulonephritis, often accompanied by the nephrotic syndrome, developed in CAF(1) mice following the administration of spleen cells from normal BALB/c mice. The renal lesion was membranous glomerulonephritis. When studied with fluorescein-conjugated antisera to either mouse gamma globulin or beta(1C)-globulin, the glomeruli contained beaded and irregular deposits of these immunoproteins. The ultrastructure of the lesion was characterized by thickening of the glomerular basement membranes and the presence of electron-dense subepithelial deposits. Acid eluates of the diseased kidneys contained gamma globulin that failed to bind to sections of normal kidneys. These findings conform to the type of nephritis provoked by immune complexes. They indicate that this type of immune injury can be based on the reaction of intolerant immunocytes to normal antigens.

Biogeochemical cycle of arsenic and calculating the enrichment factor by using Li element.

In this study, the biogeochemical cycle of arsenic in the Bosporus and the Golden Horn, which have a two-layer stratified structure, was investigated and the dominant feature in this cycle was observed to be the anthropogenic (domestic + industrial) activities. On the contrary, in the rural areas which are far from human activities, such as Igneada, the seawater-atmosphere interchange can be observed evidently in the periods covering the primary production.

A commissioning rod assembly for the Jamaican SLOWPOKE-2 nuclear reactor.

Within the framework of the Material Management and Minimization Conversion Program of the U.S. Department of Energy's National Nuclear Security Administration, the Argonne National Laboratory approved the manufacturing of a Jamaica's SLOWPOKE-2 nuclear reactor (JM-1) mock-up, reactor removal tools, and commissioning rods by Polytechnique Montreal. This mock-up reactor was then used to practice dry runs of the JM-1 high enriched uranium core removal activities and fresh low enriched uranium core loading operations of the JM-1 reactor conversion. One of the most crucial elements in the commissioning of a new reactor core is the commissioning rod assembly. Hence, this paper presents the design, the fabrication, the calibration, and the dry runs performed at Polytechnique Montreal with commissioning rod assembly. The work was then resumed at Jamaica's International Centre for Environmental and Nuclear Sciences.

Hemoglobin Istanbul: substitution of glutamine for histidine in a proximal histidine (F8(92) ).

A presumably spontaneous mutation has resulted in the formation of Hemoglobin (Hb) Istanbul in which glutamine is substituted for histidine in the proximal position of the beta-chain (F8(92)). The anemia and other physiological effects that occur in the presence of Hb Istanbul were much ameliorated by splenectomy. Hb Istanbul is a relatively unstable molecule which produces a rather moderate case of "unstable hemoglobin hemolytic anemia."In the determination of structure, a method of preferential cleavage of an aspartyl-proline bond at residues 99-100 of the beta-chain was used.

Acute glomerulonephritis. Immunofluorescent and electron-microscopic observations in sporadic cases.

The authors studied by immunofluorescent and electron microscopy renal biopsy specimens from 29 patients with various glomerular diseases. Poststreptococcal glomerulonephritis was characterized by the presence of complement (beta1C) in the mesangium and/or on the basement membrane in all cases. Immunoglobulin G (IgG) was also present in less than half of the cases in the same distribution. Electron microscopy, carried out in six cases, revealed no uniform ultrastructural change: minimal subepi helial deposits were observed in three cases, intramembranous deposits were seen in two cases, and the basement membrane was normal in one case. Glomerular abnormalities during the courses of some systemic diseases were similar. Mild renal involvement was characterized by only beta1C deposition. This finding raises the question whether a mechanism other than or in addition to that involving immune complexes is operative in the pathogenesis of acute glomerulonephritis. There is circumstantial experimental evidence that an alternate pathway of complement activation and deposition may be operative in acute glomerulonephritis.

Polycythemia vera associated with Ushers's symdrome.

A 50-year-old man with Usher's syndrome, progressive retinitis pigmentosa, and congenital sensoneural deafness developed polycythemia vera. Usher's syndrome was associated with a variety of congenital herediatry disorders but there was no evidence to support more than a chance association between Usher's syndrome and polycythemia vera.

Low dose cyclosporin A versus pulsed cyclophosphamide in Behçet's syndrome: a single masked trial.

A single masked trial of cyclosporin A 5 mg/kg/day versus monthly 1 g intravenous boluses of cyclophosphamide was conducted among 23 patients with Behçet's syndrome and active, potentially reversible uveitis. The trial was unmasked after a mean of 12 (SD 2) months for the cyclosporin A group (n = 12) and a mean of 10 (SD 3) months for the cyclophosphamide group (n = 11). During the initial 6 months the visual acuity significantly improved (p < 0.001) in the cyclosporin A group whereas this was not observed in the cyclophosphamide group. The subsequent follow-up of patients up to 24 months suggested that the initial improvement in visual acuity with cyclosporin A was not sustained. More extensive and especially long-term studies of cyclosporin A in the uveitis of Behçet's syndrome are warranted.

Short term cyclosporin A treatment of Behçet's disease.

Eleven separate three-month courses of cyclosporin A, an oral solution 10 mg/kg/day, were administered to eight patients with Behçet's disease with sight-threatening posterior uveitis. It was found to be effective in arresting the inflammatory activity in the eye as well as the mucocutaneous lesions of Behçet's disease. Improvement in visual acuity was observed within one week of starting therapy. Severe exacerbations in the ocular and mucocutaneous lesions occurred on withdrawal of the drug. At this dosage side effects included hirsutism in all women, and a slight rise of serum bilirubins in two patients and of blood urea in one patient. The latter two conditions responded rapidly to dose adjustment.

Male patients with Behçet's syndrome have stronger pathergy reactions.

The effects of patient's sex and age at onset on the pathergy reaction (cutaneous hypersensitivity to a needle prick) and its correlation with disease activity in Behcet's syndrome was investigated by two independent observers in a blind protocol. Among 92 male patients the pathergy reaction was more strongly positive (p less than 0.025) than among 67 female patients of similar age and disease duration. The age at onset did not affect the severity of the pathergy reaction, although, the early onset females (age at onset 24 years or less) had the lowest prevalence of pathergy positivity (52%), compared to early onset males and late onset (age at onset 25 years or more) males and females (73-75%). As previously reported the disease was more severe among males and among those with early onset of either gender. On the other hand, no correlation between the strength of the pathergy reaction and clinical severity could be discerned.

A case of Whipple's disease complicated by fatal hepatitis.

Whipple's disease was diagnosed in a 42-year-old woman by the histological examination of a mesenteric lymph node taken at laparotomy, and the electron microscopic examination of a peroral intestinal biopsy specimen. She responded well to tetracycline therapy, but succumbed to fatal hepatitis ten weeks after the diagnosis of Whipple's disease

Symposium on membrane markers and antigens of human lymphocytes and leukemic cells.

B lymphocytes, mediators of humoral immunity, carry easily detectable SIg, form rosettes with RBC coated with IgM and complement (EAC rosetting) and bind aggregated Ig (Fc receptor activity). Human T lymphocytes, mediators of cellular immunity, lack a specific antigen found on mice T lymphocytes (theta antigens). They form spontaneous (non-immune) rosettes with SRCB (E rosetting) and they are identified by labelling or cytotoxicity tests wtih heterologous antisera. Apart from B and T lymphocytes, a third population of lymphoid cells carrying no detectable surface markers have been identified by various methods (Null cells).

Lack of correlation between skin reactivity and T lymphocytes and monocytes in Hodgkin's disease.

28 untreated patients with Hodgkin's disease have been studied in respect to skin reactivity to purified protein derivative and dinitrochlorobenzene and blood monocytes and T lymphocytes. Cutaneous anergy as defined by unresponsiveness to both purified protein derivative and dinitrochlorobenzene was found in 43% of patients and in 7% of the control group. Although there was a significant reduction in E-rosetting lymphocytes in Hodgkin's cases, we found no differences in the mean numbers of E-rosetting lymphocytes and monocytes between normergic and anergic patients. We, therefore, conclude that skin reactivity is not directly correlated with the total numbers of E-rosetting lymphocytes or monocytes in Hodgkin's disease.

The high prevalence of HLA-B5 in Behçet's disease.

Sixteen of the nineteen (84%) patients with Behçet's disease in Turkey had HLA-B5; the prevalence of HLA-B5 among 150 controls was 27%.

Heterogenous glycogen storage disease in one family.

Three brothers, aged 17, 14 and 4 ye presented. Deficiency of glucose-6-phosphatase was associated with deficiency of acid maltase in one and debranching enzyme in the other. Enzyme analyses could not be performed in the youngest sibling.

Renal amyloidosis: immunofluorescence and electron microscopy studies.

Renal biopsy specimens of 15 patients with renal amyloidosis were studied by immunofluorescence microscopy. The amyloidosis was associated with chronic pulmonary disease in five, rheumatoid arthritis in one, chronic lymphocytic leukemia in one, and familial Mediterranean fever in five patients. In three patients no associated condition could be determined although the pattern of organ involvement resembled that of secondary amyloidosis. IgG and complement (C3) were demonstrated in the glomerular capillary walls and in the mesangium in all patients. The pattern of the deposits was neither granular nor linear. Ig and C3 appeared as large confluent masses or broad ribbon-like segments. In the six patients studied by electron microscopy the fibrillary formation of amyloid was seen in the mesangium and the glomerular capillary walls corresponding to the Ig deposits. No immunofluorescence or ultrastructural differences were observed among the patients with secondary, inherited and leukemia-associated amyloidosis included in this study.

The combined use of HLA-B5 and the pathergy test as diagnostic markers of Behçet's disease in Turkey.

Forty-one of 49 (84%) patients with Behçet's disease in Turkey carried HLA-B5 (positive B5). Forty of the same 49 (82%) patients had a nonspecific skin hyperreactivity to needle prick (positive pathergy). There were no patients in whom both of these tests were negative.

The usefulness of the nonspecific skin hyperreactivity (the pathergy test) in Behçet's disease in Turkey.

49 of 58 (84%) patients with Behçet's disease in Turkey had a positive pathergy test; the same test proved positive in 3 of the 90 (3%) of healthy and diseased contols.

Olecranon nodules in a case of Behçet's disease.

A 33-year-old male with definite Behçet's disease had rheumatoid-like nodules at his elbows. This finding, we believe, represents another manifestation of vasculitis in Behçet's disease.