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Neurodevelopmental delay and focal seizures as presenting symptoms of human immunodeficiency virus I infection (1989)

Habibi, P. ; Strobel, S. ; Smith, I. ; [et al.]

Springer

European journal of pediatrics 148 (1989), S. 315-317

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ISSN: 1432-1076

Keywords: HIV infection ; Immune deficiency ; Neuro-developmental delay ; CSF folate levels

Source: Springer Online Journal Archives 1860-2000

Topics: Medicine

Notes: Abstract Three children presenting with neurological symptoms were subsequently diagnosed as being infected with the human immunodeficiency virus I (HIV). All children showed normal development for about 12–18 months of age but later developed psychomotor and developmental regression. One child presented with generalised hypotonia, another with focal seizures, and the third with spastic quadriplegia. Two of the children showed areas of abnormal brain density on computed tomography and in one case there was calcification of the basal ganglia. In two of the children cerebrospinal fluid contained reduced amounts of total folate and elevated concentrations of neopterin. The possibility of a link between the deranged folate metabolism and the neurological symptoms in HIV infections is discussed.

Type of Medium: Electronic Resource

URL: http://dx.doi.org/10.1007/BF00444122

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Successful bone marrow transplantation and treatment of BCG infection in two patients with severe combined immunodeficiency (1991)

Heyderman, R. S. ; Morgan, G. ; Levinsky, R. J. ; [et al.]

Springer

European journal of pediatrics 150 (1991), S. 477-480

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ISSN: 1432-1076

Keywords: BCG infection ; Severe combined immunodeficiency ; Omenn syndrome ; Bone marrow transplantation ; Hypertrophic non-obstructive cardiomyopathy

Source: Springer Online Journal Archives 1860-2000

Topics: Medicine

Notes: Abstract We report successful bone marrow transplantation (BMT) in two patients with severe combined immunodeficiency (SCID), who had developed BCG infection following neonatal vaccination. Patient 1 had Omenn Syndrome, associated with hypertrophic nonobstructive cardiomyopathy. Patient 2 had SCID due to adenosine deaminase deficiency. This communication demonstrates that with appropriate anti-mycobacterial cover, immunological reconstitution together with full recovery from BCG infection can be achieved by BMT. As demonstrated by persistant negative Mantoux tests, specific cell-mediated immunity to BCG was not acquired following BMT. We suggest that these children may continue to be at risk from mycobacterial infection.

Type of Medium: Electronic Resource

URL: http://dx.doi.org/10.1007/BF01958426

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Fatal graft versus host disease after platelet transfusions in a child with purine nucleoside phosphorylase deficiency (1989)

Strobel, S. ; Morgan, G. ; Simmonds, A. H. ; [et al.]

Springer

European journal of pediatrics 148 (1989), S. 312-314

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ISSN: 1432-1076

Keywords: Transfusion ; Severe combined immunodeficiency ; PNP deficiency ; Graft versus host disease

Source: Springer Online Journal Archives 1860-2000

Topics: Medicine

Notes: Abstract Fatal graft versus host disease (GVHD) developed in a child with purine nucleoside phosphorylase (PNP) deficiency following an unirradiated platelet transfusion. IV treatment with an anti-T-cell monoclonal antibody (CD7) led to a transient improvement of his GVHD (Grade IV) but did not prevent the fatal outcome. This report emphasizes the need for blood products to be irradiated when cell mediated immunodeficiency is suspected, even in patients with residual immunocompetence.

Type of Medium: Electronic Resource

URL: http://dx.doi.org/10.1007/BF00444121

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