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Biomedical subjects

S Makni

Publications and source records attributed to S Makni.

At least 19 recordsLinked to original sources
PubMed1993

[Lupus in children in Tunisia].

In this study, we have analyzed the clinical and serological features related to 16 Tunisian children in whom diagnosis of systemic lupus erythematosus was made before or at the age of 15. Renal involvement was found in 75% of cases and renal biopsies have mostly revealed severe histologic patterns. All of the patients who have been followed received corticosteroids and in some cases required additional cytotoxic drugs in order to control disease activity. Five children died in a context of a renal failure. This study of childhood lupus in Tunisia confirms that the clinical course of this disease in children is often aggressive.

Adolescent↗
PubMed1992

[Schwartz-Jampel syndrome (osteochondromuscular dystrophy)].

BACKGROUND: Schwartz-Jampel syndrome is a rare disorder inherited as an autosomal recessive trait and characterized by growth retardation, multiple skeletal abnormalities, myotonia-like muscle disorders and unusual facies. CASE REPORTS: Case n. 1: A boy, aged 3 years 4 months, was admitted for acute respiratory disease. His main abnormalities included rigid facial expression, blepharophimosis, puckered lips, short neck, pectus carinatum, acetabular dysplasia with coxa vara, platyspondyly and marked growth retardation. There was a continuous muscle fiber activity at rest, with abnormal discharges originating in the muscle component of the neuromuscular junction. Blood investigations revealed low values of IgA. The child died at 4 years. Case n. 2: The sister of case n. 1 was examined at 14 months of age. She presented milder facies abnormalities, difficulties of gait because of stiff hips, muscular hypertrophy, coxa vara and growth retardation. X-rays showed skeletal abnormalities and the electromyogram was similar to those of her brother. She had dislocation of her optic lens. CONCLUSION: These 2 sibs have the characteristic manifestations of Schwartz-Jampel syndrome. Parental consanguinity was also present. The IgA deficiency observed in case n. 1 and the lens dislocation in case n. 2 have both been occasionally reported in this syndrome.

Child, Preschool↗
PubMed1992

[Characterization and evolution of blood cryoglobulin in visceral leishmaniasis].

The evolution of immunological parameters in mediterranean visceral leishmaniasis reveal a type III mixed cryoglobulin with rhumatoid factor activity and antileishmania antibodies. This cryoglobulin follows the clinical signs of the disease and disappear under treatment. The authors insist on the transitory character of this cryoglobulin which is in relation with polyclonal stimulation of the immune system by parasitical infection.

Adult↗
PubMed1990

[Monoclonal gammopathies in Tunisia].

Protein electrophoresis and immunoelectrophoresis carried out on 4805 sera and 93 urine samples from Tunisian patients over 8 years, and a monoclonal protein was detected in 198 cases. The distribution of the monoclonal compounds to the clinical diagnosis was studied. 115 (58%) were classified as multiple myeloma (MK), 34 (17%) as alpha heavy chain disease (MCL a), 34 (17%) as monoclonal gammapathy associated to various diseases (GMOD), 11 (6%) as benign essential monoclonal gammapathies (GMBE), 4 (2%) as Waldenström's macroglobulinemia (MW). The relationship between the monoclonal immunoglobulin and the clinical diagnosis, the distribution of the monoclonal compounds according to the heavy chain class and the identification of the light chains were studied. The results obtained are in line with the principal data in literature concerning monoclonal gammapathy. However there is a particularity about monoclonal gammapathy observed in the Tunisian's population studied: Higher percentage of alpha heavy chain diseases, monoclonal IgD and monoclonal light chain. Lower percentage of monoclonal IgM in GMBE or GMOD, as in MW.

Aged↗
PubMed1990

[Intraorbital involvement in multiple myeloma].

According to data from the literature, intraorbital involvement in multiple myeloma is rare. Such involvement may result in exophthalmia, itself the first manifestation and presenting feature of myeloma. These lesions can respond remarkably to radiotherapy. The authors present a new case report.

Exophthalmos↗
PubMed1990

[Familial syndrome combining short stature, microcephaly, mental deficiency, seizures, hearing loss, and skin lesions. A new syndrome].

We report the observations of three sisters with the same autosomal recessive syndrome characterized by growth retardation, microcephaly, mental deficiency, seizures, sensorineural hearing loss, and skin lesions. The congenital nature of these symptoms was confirmed by their high prevalence among other family members. This syndrome is one of the many neurocutaneous syndromes and does not seem to fit any of the previously published descriptions.

Adolescent↗
PubMed1989

Study of circulating immune complexes during the evolution of visceral Mediterranean leishmaniasis.

In the present study, sera of 56 children suffering from visceral Mediterranean leishmaniasis were analysed. The incidence of circulating immune complexes (CIC) by ELISA technique using the conglutinin binding assay was 39% before treatment, 28% after the first treatment, and nil at the end of the second one. The C4 levels remain low even after treatment. The anti-leishmania antibodies (AAL) account for 95% of the cases before treatment and 84% after treatment. The rheumatoid factor (FR) also remains with more or less the same frequency (74%) before and after treatment. The search for a correlation between the presence of CIC and the other immunological factors show that the average titre of AAL is higher in the group of patients that have CIC than in the group that does not. The C4 levels are significantly reduced in the CIC positive group of patients, whereas the rate of IgG is significantly increased in the same group. The study of the evolution of CIC under treatment in nine patients shows that they disappear at the end of the treatment, and that the FR reduces but does not disappear.

Animals↗
PubMed1989

[The value of using HEP/2 cells as compared to sections of rat liver in the detection of autoantibodies using indirect immunofluorescence in human pathology].

1,400 sera taken from patients suspected of having autoimmune diseases and sent to the laboratory for determination of antinuclear antibodies, are tested by comparative indirect immunofluorescence on 2 subtrata; rat liver section and HEP/2 cells. The 143 positive sera on rat liver sections are also positive on HEP-2. In the 1,010 sera which are negative on rat liver sections, 165 are positive on HEP-2, 113 give a nuclear fluorescence, 26 give a cytoplasmic fluorescence and 26 give a nuclear and cytoplasmic fluorescence. Three positive sera were also used in immunofluorescence on another cells: VERO and MRC 5, as well as dual immunodiffusion versus thymic and splenic cell extracts and 76 p. cent of these sera were found positive with these techniques. This confirms the advantage of the use of HEP/2 cells in demonstrating autoantibodies, especially when they are not detected on rat liver sections, like the anticentromer antibodies. This substratum offers the advantage of detecting not only antibodies directed against nuclear antigens, but also those directed against cytoplasmic antigens.

Antibodies, Antinuclear↗
PubMed1989

Anti-leishmanial antibodies during natural infection of Psammomys obesus and Meriones shawi (Rodentia, Gerbillinae) by Leishmania major.

Sera from 77 rodents (Psammomys obesus: 64; Meriones (M.) shawi: 10; M. libycus: 3) trapped in a focus of zoonotic cutaneous leishmaniasis (ZCL) (Leishmania (L.) major) were examined for the presence of anti-leishmanial antibodies by the indirect fluorscence antibody technique (IFAT) (L. major antigens) using homologous antiglobulins and/or anti-Rattus conjugates. Sera from 21 animals were positive with titres that ranged from 1: 20 up to 1: 640. The investigation revealed that a good correlation existed between the occurrence of antileishmanial antibodies in the sera and amastigotes in the rodents' ears. The data also suggest the suitability of the IFAT as an epidemiological tool to estimate rates of infection by L. major in Psammomys obesus and Meriones Spp. in foci of ZCL.

Animals↗
PubMed1989

[Cervicofacial hydatidosis. Apropos of 7 cases].

The authors report seven cases of hydatidosis located in neck region. They reviewed components of positive diagnosis, particularly serological one. Surgery is the treatment in all cases, and preventive treatment is the best one before apparition of cysts.

Adolescent↗
PubMed1987

Discordance between red cell and saliva Lewis phenotypes in patients with hydatid cysts.

Patients with hydatid cysts and controls of the same Tunisian area were typed for ABO, Lewis and secretor phenotypes. A high incidence of red cell Le(a-b-) phenotype (34-37%) was found among hydatid cyst patients as compared to normal controls (13-16%). However, a large proportion of the patients with Le(a-b-) red cell phenotype had discordant red cell and saliva Lewis phenotypes since they secreted Lea and/or Leb antigens in saliva. In addition, 1 patient with Le(a+b-) red cell phenotype secreted Leb antigen in saliva. The remaining patients and all the controls had concordant red cell and saliva Lewis phenotypes. The discordant results between the phenotypes obtained in serum and saliva of hydatid cyst patients are probably the consequence of a decrease in the concentration of the circulating Lewis glycosphingolipids, secondary to the disease.

ABO Blood-Group System↗