A new type of acquired C1 inhibitor deficiency associated with systemic lupus erythematosus.

Acquired C1 inhibitor (C1-INH) deficiency with consequent angioedema is a rare condition that may indicate an underlying lymphoproliferative disorder. The defect is caused by increased catabolism, which is often associated with the presence of serum autoantibodies to C1-INH. The present report describes 3 patients with systemic lupus erythematosus who developed typical symptoms of acquired angioedema, characterized by recurrent swelling of subcutaneous and mucous tissues. The 3 patients demonstrated a major classical pathway-mediated complement consumption, with very low levels of C3 antigen and decreased levels of C1-INH antigen. Neither antibodies to C1-INH nor associated lymphoproliferative disease was found. No patient had clinical and biologic signs of lupus activity at the time the angioedema occurred. All patients were treated with steroids and exhibited a good response, without relapse of angioedema and with normalization of plasma levels of C1-INH. In lupus patients who present with an angioedema syndrome, acquired or hereditary angioedema must be sought by examining parameters of the classical pathway and levels of C1-INH. Our observations suggest the existence of a new form of acquired C1-INH deficiency associated with a major classical pathway-mediated complement consumption and systemic autoimmunity.

Levels of L-T3 in maternal and foetal compartments following experimental modifications of the maternal thyroid state in rats.

In pregnant female rats, concentrations of tri-iodo-L-thyronine in maternal serum, amniotic fluid and placental tissue after 15, 18 and 20 days of gestation were measured by homologous radioimmune-analysis. The three experimental groups of pregnant rats were: 1) euthyroid (or control), 2) hypothyroid, provoked by iodine-deficient diet for two months before conception and during gestation, 3) hyperthyroid, provoked by subcutaneous injections of L-thyroxine during gestation. Maternal serum L-T3 was measured in order to check the thyroid state. Significant decreases in L-T3 concentrations were found at all stages of gestation in the amniotic fluid of hypothyroid group. The hormonal concentrations in the placental tissues were correlated with the different treatments (decreased in hypothyroid state and increased in hyperthyroid state). This could suggest that the transfer of maternal iodothyronines to the foetus influences its foetal thyroid development.

[Acute erythroblastopenia disclosing homozygous beta-thalassemia. Role of parvovirus infection]. / Erythroblastopénie aiguë révélatrice d'une bêta-thalassémie homozygote. Rôle d'une infection à parvovirus.

An acute, transient aplastic crisis in a 15-month old boy revealed the presence of homozygous beta-thalassaemia. The crisis was very likely due to a parvovirus infection, in view of the presence of specific IgM at the onset and of seroconversion to total antibodies. Later, requirements for transfusions were in favour of an intermediate type thalassaemia. The responsibility of the parvovirus is discussed in the light of recent data concerning the inhibitory action of this virus on bone marrow erythropoiesis.

[Acquired toxoplasmic chorioretinitis with a late onset]. / Choriorétinite toxoplasmique acquise à révélation tardive.

Maternal toxoplasma infection was responsible for congenital toxoplasmosis with ocular and cerebral manifestations in the infant. The acquired character of the toxoplasmosis in the 5th month of pregnancy was confirmed by serologic data and isolation of the parasite from the placenta. Five years later, an evolutive chorioretinitis was found in the mother. The toxoplasma origin of the ocular injury was proven by the study of aqueous humor. This case report is compared with the rare similar cases in the literature. The late discovery of ocular impairment is discussed.