Prospective study of the occurrence of monoclonal gammapathies following bone marrow transplantation in young children.

We have prospectively studied the occurrence of monoclonal serum immunoglobulins in 38 recipients of BMT. Patients were young children with primary immunodeficiencies (n = 31), other inherited diseases (n = 4), leukemia (n = 2), or aplastic anemia (n = 1). Twenty-nine received an HLA-nonidentical marrow and nine an HLA-identical marrow.

[Developmental profile of HIV infection in children].

The natural history of HIV-1 infection in children has not yet been determined precisely. Contrary to what is observed in adults, the disease seems to follow a bimodal course. One-quarter to one-third of infected children develop, before the end of their first year of life, a deep immunodeficiency which is responsible for severe infectious and neurological symptoms; survival does not exceed 4 to 5 years.

Blood histamine levels in HIV-1-infected infants and children.

Blood histamine levels (BHL; ng histamine base/ml blood, mean +/- SEM) were determined in 118 infants born to HIV-1-infected mothers and in children infected after blood transfusion. In asymptomatic infected infants, BHL were not significantly different from HIV-1-negative infants born to HIV-infected mothers (54.1 +/- 5.

[The accelerated phase of Chediak-Higashi syndrome].

We have retrospectively analysed the clinical and biological features as well as the outcome of 18 accelerated phases having occurred in 11 patients with the Chediak-Higashi syndrome. This complication is very frequent and is characterized by a multi-visceral lymphohistiocytic infiltration with hemophagocytosis leading to pancytopenia, a bleeding disorder secondary to low fibrinogen level, hypertriglyceridemia and hemodilution. The accelerated phase of the Chediak-Higashi syndrome is identical to the manifestations of familial erythrophagocytic lymphohistiocytosis and of the viral-associated hemophagocytic syndrome.

[Prognostic factors in HIV seropositive children by materno-fetal transmission].

Ninety-four cases with HIV seropositive children who were found in the Hospital Necker Enfants Malades in France from April 1983 till September 1988 due to materno-fetal transmission were surveyed for immunological studies as well as the relationship between clinical symptoms and the prognosis. Lymphoadenopathy and/or hepatosplenomegaly were found in 98% of the total cases. Opportunistic infections, severe neurological problems and LIP were found in 28%, 16% and 15%, respectively.

[Severe respiratory syncytial virus infections. Study of 87 infants hospitalized in an epidemic].

During a winter epidemic, 87 infants were admitted to Necker-Enfants-Malades hospital with a severe respiratory syncitial virus (RSV) infection. These infants fell into two groups: 37 infants without any medical history and 50 showing an underlying pathology (immune deficiencies, heart disease, CNS disorders, digestive malformations, allergic manifestations). Of the 37 infants with no medical history, most were below the age of 6 months and the RSV infection was manifested clinically by bronchiolitis or bronchitis.

Mother-child transmission of HIV-1 and infant survival in Brazzaville, Congo.

The aim of this study was to compare the probability of survival of infants born to anti-HIV-1-positive and anti-HIV-1-negative mothers. One thousand, eight hundred and thirty-three pregnant women, recruited sequentially in two mother-child clinics in Brazzaville, were screened for anti-HIV-1 (by enzyme-linked immunosorbent assay with confirmation by Western blot). Each seropositive mother (71 out of 1833, 3.

Bronchoalveolar lavage in HIV infected patients with interstitial pneumonitis.

The value of taking microbiological and cytological specimens by flexible bronchoscopy and bronchoalveolar lavage under local anaesthesia was assessed on 43 occasions in 35 HIV infected children, aged 3 months to 16 years, with interstitial pneumonitis. In acute interstitial pneumonitis (n = 22, 26 specimens from bronchoalveolar lavages) the microbiological yield was 73%, Pneumocystis carinii being the commonest infective agent (n = 14). P carinii pneumonia was found only in children with deficient antigen induced lymphocyte proliferative responses who had not been treated with long term prophylactic co-trimoxazole.

Human immunodeficiency virus-associated thrombocytopenia in infants.

Severe thrombocytopenia was the initial manifestation of human immunodeficiency virus infections in 5 infants under 15 months of age. In only 2/5 cases maternal seropositivity was previously known while in the other 3 it was discovered after onset of the infants' thrombocytopenia. One infant died, due to intracranial hemorrhage, and the other 4 developed to disease stage IV.

Successful HLA nonidentical bone marrow transplantation in three patients with the leukocyte adhesion deficiency.

Three consecutive patients with the severe phenotype of leukocyte adhesion deficiency characterized by a defective expression of LFA-1, Mac-1 (CR3), and p150.95 on leukocytes have received HLA partially incompatible bone marrow transplantation (BMT). The degree of HLA incompatibility between related donors and recipients was 2 HLA antigens in one and one full haplotype in the two others.

A prospective study of infants born to women seropositive for human immunodeficiency virus type 1. HIV Infection in Newborns French Collaborative Study Group.

Assessment of the risks of transmission of infection with human immunodeficiency virus type 1 (HIV-1) from mother to newborn is difficult, partly because of the persistence for up to a year of maternal antibodies transmitted passively to the infant. To determine the frequency of perinatal transmission of HIV infection, we studied from birth 308 infants born to seropositive women, 62 percent of whom were intravenous drug abusers. Of 117 infants evaluated 18 months after birth, 32 (27 percent) were seropositive for HIV or had died of the acquired immunodeficiency syndrome (AIDS) (n = 6); of the 32, only 2 remained asymptomatic.

[Materno-fetal infection by the human immunodeficiency virus (HIV)].

Transmission of the HIV from mother to fetus is now virtually the only means by which children can be contaminated. Thirty to 40% of infants born to HIV-seropositive mothers are infected. Nearly half of infected neonates develop AIDS within the first two or three years of life.

Hypoglycaemia induced by antibodies to insulin receptor following a bone marrow transplantation in an immunodeficient child.

Severe hypoglycaemia developed seven months after a bone marrow transplantation in a child with severe combined immunodeficiency. His serum exerted potent insulin-like activity: (a) it stimulated insulin receptor autophosphorylation and kinase activity in cell-free systems, this effect being additive to insulin; (b) it increased glucose transport in isolated soleus muscle. These insulin-like effects were due to immunoglobulins against the insulin receptor.

Detection of HIV1 DNA in infants and children by means of the polymerase chain reaction.

The polymerase chain reaction (PCR) assay was used to investigate the possibility of HIV1 DNA detection in uncultured peripheral blood mononuclear cells from newborn infants and children of HIV-infected mothers. HIV1 DNA sequences were detected in mononuclear cells of six of fourteen symptom-free newborn infants of seropositive mothers. Only one of these infants had detectable HIV antigenaemia.

Zidovudine therapy in children with acquired immunodeficiency syndrome.

Eight children with acquired immunodeficiency syndrome (AIDS), aged four months to 12 years, were treated with zidovudine (azidothymidine) 100 mg/m2 intravenously every six hours for 14 days, followed by oral zidovudine at the same dose for a total of six months. Of the eight, six were infected at birth and two were contaminated by blood transfusion at ages eight and nine years, respectively; seven of the eight showed specific neurologic impairment consisting of encephalopathy (six children) and myelopathy (one child). In two children, a dramatic improvement of clinical status occurred, including neurologic progress in one; in three, the improvement was dissociate or transient and in the other three, no modification was observed.