[Management of beta-thalassemias in a developing country. Experience of a pediatric service in Oran (Algeria)].

The management of beta-thalassemia in a developing country faces a host of organizational, logistic, and funding problems. Experience acquired against this background of multiple deficiencies is reported here. Only 60% of children with documented beta-thalassemia were monitored more or less regularly.

Genetic factors involved in human G gamma and A gamma globin gene expression.

G gamma to A gamma globin ratios, haplotypes at the beta globin gene cluster and the C----T substitution at -158 5' to the G gamma globin gene were studied in three Algerian families that include SS or S-beta(0) thal patients. G gamma to A gamma ratios were found similar, within a family, in subjects displaying the same combination of chromosomes 11, the ratio observed for a given combination depending on the chromosome haplotypes. Our data can be explained by the existence of several alleles of a genetic factor closely linked to the beta globin gene cluster and involved in the determination of G gamma to A gamma globin ratio.

[Pediatric cancerology in Algeria. Current status and perspectives].

Pediatric oncohematology represents 40% of our daily activity. Its management is analyzed and conditions of its development discussed. In underdeveloped countries, financial, human and management health resources are severely limited.

[Familial type of hypophyseal nanism. Apropos of 7 families in western Algeria].

Eighteen pituitary dwarfs belonging to 7 different West Algerian families were studied. Eleven patients from 4 families presented isolated growth hormone deficiency, 7 patients from 3 families had multiple pituitary hormone deficiencies. Serum GH levels before and after standard pharmacological stimulations were below 2 ng/ml in all cases.

[Medical transfers in pediatric oncology. Reflections apropos of economic, technical and ethical problems (Oran 1977-1983)].

In an underdeveloped country such as Algeria [20 million inhabitants, 2,140 US Dollars per capita gross national product (GNP)], satisfying a high level of health demand is difficult both in primary care and hospital. Limited facilities (hospital beds 2.3%; physicians 1:2,500; public health expenditure: 2% of GNP) combined with demographic pressure (population under 15 years of age: 47%) further compromise medical benefits.

[Burkitt's lymphoma-Epstein-Barr virus association in western Algeria. Clinical, cytological and serological aspects apropos of 34 pediatric cases].

Burkitt's lymphoma (BL) and naso-pharyngeal carcinoma (NPC) are tumors generally believed to be associated with Epstein-Barr virus (EBV). Algerian population could be considered at high risk for BL-EBV association. In a series of 34 BL patients observed by us between 1982 and 1984 characteristics were: 70% of children were under 5 years age, abdominal location in 90% of cases (n = 30), 13% only isolated, massive tumor burden (70% stage III after Murphy, 30% stage IV after Murphy and Duque-Hammershaimb), EBV serology titers above or equal to 640 in 60% of cases (anti-EBNA and anti-VCA) and 50% (anti-EA) respectively, presence of EBNA antigen in tumor cells found in 63% of cases, no complete correlation between serology positivity and EBNA presence.

[Association of severe intestinal villous atrophy and abdominal non-Hodgkin lymphoma. Apropos of 3 cases].

Malignant lymphoma is a recognized late complication of adult celiac disease. In children, however, the association of lymphoma with celiac disease was so far identified only in one case of an Algerian girl in 1981. We have observed during the last 5 years 78 patients with non-Hodgkin lymphoma, 56% located in the abdomen.

[Renal involvement in the Laurence-Moon-Bardet-Biedl syndrome. Apropos of 3 cases].

Three cases of Laurence-Moon-Bardet-Biedl (LMBB) syndrome with renal involvement characterized by tubulo-interstitial lesions are reported. Caliceal deformations were noted on urography. From 55 additional cases found in the literature the main characteristics of the renal disease in LMBB are defined : impairment of renal concentration is present in 36 % of the cases, and urographic abnormalities, mainly dysplasic features and multiple cystic formations on the calices, in 90 %; tubulo interstitial lesions are the most frequent histological finding.

[Acute lymphoblastic leukemia in children. Evaluation of risk factors and results of treatment in a pediatric milieu].

Rapid progress in acute lymphoblastic leukemia treatment in childhood has allowed five years survival rates at least for 50% vs 30% of standard risk (60-70%) and high risk (30-40%) patients. Analyzing our material from University Hospital Pediatric Department (Oran, Algeria) was leading to such conclusions as: First: excess of higher risk acute lymphoblastic leukemias; Second: excess of bad prognosis enzymatic/cytologic markers; Third: post therapeutic 5 years survival probability compares with lowest figures found in literature. Bearing in mind higher risk clinical material and poorer resources of hospital facilities, medical manpower and nursing in such an underdeveloped country as Algeria, therapeutic results seemed reasonably promising.

Composition, cross-linking and thermal stability of bone and skin collagens in patients with osteogenesis imperfecta.

The composition, cross-linking, and thermal stability of the collagens were determined in bone and skin biopsies from 4 patients with moderate to severe forms of osteogenesis imperfecta (OI). The major modification observed with respect to control subjects was an overhydroxylation of lysine in type I bone collagen (hydroxylysine content doubled in three patients and increased by 50% in the last patient). This overhydroxylation is confirmed by a similar increase in the dihydroxylated cross-link of bone collagen: the dehydrodihydroxylysinonorleucine.

[A molecular abnormality of the erythrocyte membrane in beta-thalassemia].

We present a new abnormality of the red cell membrane observed in the course of a study on beta-thalassemia. In a child suffering from a beta 0-thalassemia major, the neutral membrane bound phosphatase, instead of having Michaelis-Menten kinetics, displayed a biphasic kinetics consistent with inhibition by substrate excess. This abnormality is discussed along with other erythrocyte membrane alterations that are known to occur in beta-thalassemia.