Biomedical subjects
Publications and source records attributed to A Martini.
Eight new cases of "ear-pits-deafness syndrome" are reported here; in particular, the audiometric and tomographic findings of the ear and renal echography are discussed. Although it is difficult to give a nosologic definition of the syndrome, these cases can be classified as branchio-oto-renal dysplasia and branchio-oto dysplasia.
The synthesis of a series of 6-(4R-phenyl)-5-hydroxymethyl-4,5-dihydro-3(2H)pyridazinones is reported. The compounds were evaluated for their oral antihypertensive activity in rats and some of them [(V b), R = NH2] and [(V c), R = NHCOCH3] were found to induce a high decrease in systolic blood pressure. Moreover all the compounds displayed an antithrombotic activity comparable to, or greater than, that of ASA.
We describe a 14-month-old child with dermatomyositis in whom calcinosis was the first sign of the disease. This case shows that calcinosis, usually a late complication of dermatomyositis, may be the presenting sign of the disease even in young children and when Gottron's sign is still absent and muscular weakness not prominent.
The fermenting ability of 380 strains of Yersinia spp. on esculin, L-fucose and D-arabitol was studied. All the strains of Y. enterocolitica belonging to biotype 2 serotype 0:9 and to biotype 4 serotype 0:3 failed to utilize such carbohydrates; the strains belonging to the other bioserotypes as well as the strains of Y. enterocolitica-like showed different reactions.
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Islet cell antibodies (ICA-IgG and complement-fixing-ICA), parietal cell antibodies (PCA), intestinal epithelial cell antibodies (IECA), thyroglobulin (TgA) and thyroid microsomal antibodies (MsA), antinuclear (ANA) and reticulin antibodies (RA), were studied in 55 insulin-dependent diabetic patients (30 males and 25 females), aged 2-19 years with diabetes from a few days up to 14 years. In 58% of the diabetics one or more autoantibodies were found: ICA-IgG (31%), CF-ICA (16%), PCA (34%), TgA (9%), MsA (9%), ANA (13%), RA (2%). Autoantibodies were significantly more frequent in females (76%) than in males (43%) (P less than 0.025). ICA-IgG, CF-ICA, PCA, ANA were significantly more frequent in patients than in controls. The frequency of ICA-IgG and CF-ICA was significantly higher during the first 3 years of disease than afterwards (P less than 0.001); a similar pattern was observed for PCA, TgA, MsA. Of the 87 parents and 30 siblings screened for ICA-IgG, CF-ICA, PCA, IECA, TgA, MsA, ANA and RA, 42 (44%) had one or more autoantibodies, which were more frequent in females than in males. Seven relatives (6%) were ICA-IgG positive (four mothers, two fathers and one brother), and only one mother, ICA-IgG negative, was CF-ICA positive. Other autoantibodies were also more frequent in parents than in controls. Autoantibody-positive relatives have been asymptomatic up to now.
A 5.5-year-old boy with Schönlein-Henoch purpura presented flank pain, macroscopic haematuria and voiding of blood clots. Radiologic examination showed a stenosing ureteritis. Treatment with prednisone was accompanied by resolution of the urologic manifestations. Ureteritis is a potentially serious complication of Schönlein-Henoch purpura and its incidence is probably underestimated. Less than half of the reported patients have had symptoms suggesting a urologic complication. Microscopic examination of the urinary sediment can be helpful for the precocious diagnosis of ureteritis in the Schönlein-Henoch purpura.
Leishmania major, agent of zoonotic cutaneous leishmaniasis, has been isolated from the rodent Meriones shawi shawi in central Tunisia. As a result, the North saharan arid mediterranean area can be considered as epidemiologically homogeneous.
A new composite broth medium combining o-nitrophenyl-beta-D-galactopyranoside (ONPG) and urease and indole tests in a single tube is described. High-level agreement with individual conventional tests was recorded in comparative studies with 2,412 cultures of members of the family Enterobacteriaceae, i.e., 100% agreement with the exception of Hafnia spp. (96.3% agreement) for the ONPG test and Citrobacter, Enterobacter, and Hafnia spp. (75, 86.4, and 98.2% agreement, respectively) for the urease test. The new medium seems especially promising as a screen for Salmonella subgroup I which encompasses most pathogenic Salmonella species other than the Arizona subgroup.
IgG subclass levels of sera from 26 patients with juvenile chronic arthritis (JCA) were determined by means of mouse monoclonal antibodies. Patients were divided into three groups according to clinical activity of the disease: active disease, partial remission, and remission. One hundred and sixty four age matched, healthy children served as controls. IgG subclass concentrations were log transformed, and a robust regression method was applied to obtain expected values for the different ages. We found a significant increase of IgG3 (p less than 0.0001), IgG1 (p less than 0.002), and IgG2 (p less than 0.035) in JCA sera, while IgG4 values did not differ significantly from those of controls. When patients were divided according to clinical activity significant increases of IgG2 and IgG4 were observed in the patients in partial remission. Our data suggest that differential increase of IgG subclasses during the courses of JCA may be of relevance to the pathogenesis of the disease.
Auranofin [S-triethylphosphine gold-2,3,4,6 tetra-O-acetyl-l-thio-beta-D-glucopyranoside) SK&F 39162) has been administered at 0.1-0.25 mg/kg/day as the sole remission-inducing drug to 46 children affected with juvenile chronic arthritis (JCA). There were 22 males and 24 females; 12 children were affected with pauciarticular onset JCA, 26 with polyarticular onset JCA and 8 with systemic onset JCA. Three sets of efficacy criteria were evaluated quarterly: eight clinical, (Ritchie Index, number of affected, swollen and limited joints, number of joint with increased temperature, morning stiffness, Steinbrocker functional class, physician's disease evaluation), three hematochemical and one therapeutical. In most patients a panel of immunological parameters was routinely performed inclusive of peripheral blood lymphocyte subsets, serum immunoglobulins and C3c-C4 complement components. Patients who showed a definite improvement of at least two out of the three orders of efficacy criteria were classified as responders to auranofin. Out of the 35 patients evaluable after at least six months of treatment there were 24 (68%) responders. Nonresponders had a basal higher level of serum IgA and a basal lower level of serum C4. Both responders and nonresponders presented a reduction of the T4/T8 ratio during auranofin treatment, while only in responders did the basal high levels of IgG and C3c show a definite decrease.
Blood mononuclear cells from a total of 23 children with juvenile arthritis were stimulated in vitro to produce interleukin 1 (IL-1) and interleukin 2 (IL-2) and compared with age matched healthy controls. Peripheral blood monocytes from patients with juvenile arthritis produced a higher amount of IL-1 than controls, whereas peripheral blood lymphocytes from the same patients produced lower amount of IL-2 than controls. These findings could not be explained by concurrent therapy. The increase of IL-1 production was more marked in patients with active disease and therefore may have been secondary to the pathological process. However, the decrease of IL-2 production did not depend on disease activity, thus suggesting an immunoregulatory abnormality.
A 10-month-old female presented with anuria due to bilateral cystine urolithiasis. Although bilateral obstruction of the ureters by calculi is very rare in children, it may be the presenting feature of cystinuria even in the first year of life.
A new diagnostic two-steps scheme for the species identification of enterobacteria was employed with 703 strains. The diagnoses were compared with those obtained by employing Api 20 E and Enterotube II. On the basis of the reported results this new scheme is shown to be reliable (99.71% right diagnosis) and easy to perform.
The Authors propose a new two-steps key for the identification of the species belonging to the Enterobacteriaceae Family. This key can be employed both with standard tube methods and most commercial kits. The proposed method is a simple, easy and rapid one; moreover it considers each genus and species of Enterobacteriaceae Family, associated with human pathology, which has been defined within the month May, 1984.
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The aim of the present study is to evaluate the capacity of ABRs to detect the limit between reversible dysfunction and irreversible damage to the brain-stem after severe head injuries. ABRs were recorded in 40 severely head-injured patients, strictly selected on the basis of clinical and CT scan findings and the absence of otologic disease. The interpeak latency of waves V-I (IPL V-I) was calculated and compared to that recorded in 19 healthy control subjects. The distribution curve for IPL V-I in surviving patients was analysed. The overall mortality was 47.5%; all the patients showing an IPL V-I greater than 4.48 msec died or remained vegetative, while all but 2 of the patients with IPL V-I less than 4.48 msec survived. The IPL V-I was significantly different from normal value in surviving patients, showing a brain-stem dysfunction in reversible post-traumatic coma. The 95% confidence limits for the mean IPL V-I in the surviving population were 4.08 and 4.24 msec. Our results confirm the high prognostic accuracy of ABR (P less than 0.001) and suggest the hypothesis that 95% of survivors have an IPL V-I ranging between 3.82 and 4.50 msec.
A 9 year old girl presented with clinical manifestations of Henoch-Schönlein syndrome and macroscopic haematuria. Laboratory investigations showed selective IgA deficiency and renal biopsy showed mesangial proliferative glomerulonephritis with diffuse granular deposits of C3 on immunofluorescence. IgA deposits were absent.