Shigella sepsis.

It is generally believed that bacteremia rarely occurs with enteric Shigella infections. During a five-year period, 110 children were admitted to the University Hospital of the Arizona Health Sciences Center, Tucson, with Shigella enteritis. Eight (7%) had Shigella bacteremia/septicemia, and four of the eight died. An analysis of these patients and their course disclosed clinical characteristics that were of potential prognostic importance. Some of these indicators are identifiable early and hence could be useful in management. The patients at greatest risk were afebrile; 10% or more dehydrated; malnourished, with a low serum albumin level; infected with ampicillin-resistant strains; had leukopenia during the course of the illness; and had persistent watery hemoglobin-positive stools.

Coccidioides immitis osteomyelitis of the mandible in an infant.

Coccidioidomycotic osteomyelitis developed at the angle of the right side of the mandible in a 5-month-old Papago infant. The disease was successfully treated with a combination of amphotericin B, surgery, and transfer factor with complete immunological, microbiological, and radiological cure. At 4 years of age, the only residual effect is prominence of the right hemimandible with asymmetry of the jaw.

Serum complement depression during viral lower respiratory tract illness in cystic fibrosis.

Cystic fibrosis (CF) patients with viral lower respiratory tract illnesses (LRI) had depressed levels of the third and fourth components of complement, which returned to normal after recovery. There was no clinical evidence of immune complex disease. CF patients with LRI and no virus isolates, CF patients in stable status, and non-CF patients with LRI did not have complement depression. It is postulated that antigen-antibody complex activation of complement may occur in CF patients with viral LRI.

Parallel tubular arrays in severe combined immunodeficiency disease: an ultrastructural study of peripheral blood lymphocytes.

The ultrastructure of the lymphocytes from three children with severe combined immunodeficiency disease (SCID) is described. Parallel tubular arrays (PTA) were found in a large percentage of circulating lymphocytes (53%, 41%, and 13%) in three SCID patients when compared to age-matched controls. The size of these inclusions was quite variable, with some attaining a length of 1.7 micrometer. They contained a tubular substructure with a diameter of 36--44 nm. The PTA were mostly located in the centriolar and Golgi regions of the cytoplasm, and were sometimes membrane bound. A centriolar origin of the inclusion was suggested. A second inclusion, the tubuloreticular structure, was found in only 1.4% of the circulating lymphocytes from one SCID patient. The origin of the PTA and its occurrence in severe combined immunodeficiency disease are discussed.

Kwashiorkor not associated with poverty.

Four infants are described with kwashiorkor that was unassociated with poverty or infection as predisposing factors of their disease. The condition followed various dietary regimens deficient in protein of five weeks to seven months in duration. Features of this "sugar-bady" form of kwashiorkor included a low level of physician awareness of malnutrition as a diagnostic consideration, growth retardation, edema, muscle wasting, mental changes, and mild lymphocytosis. Initially, recovery was associated with rapid loss of weight and anemia and subsequently with weight gain and return to good health.

Herpes simplex virus infections in guinea pigs deficient in the fourth component of complement.

Separate groups of normal and C4-deficient guinea pigs were inoculated with herpes simplex virus by intradermal (i.d.) and intraperitoneal (i.p.) routes. Virus infection, confirmed by clinical, virological, and serological criteria, did not last longer and was not more severe in C4-deficient guinea pigs than in normal guinea pigs. Serum C component levels were measured before, during, and after herpes simplex virus infection. In normal gruinea pigs there was no evidence for C4 utilization after either i.d. or i.p. inoculation. In both normal and C4-deficient guinea pigs, C1 and C3-9 levels remained unchanged in spite of i.d. or i.p. infection. These data suggested that C4 and the classical C pathway were not important for virus clearance.

Pseudomonas cepacia pneumonia in a child with chronic granulomatous disease and selective IgA deficiency.

A 6 1/2 year-old boy with chronic granulomatous disease (CGD) and selective IgA deficiency developed a chronic progressive pneumonia which failed to respond to several conventional combinations of antimicrobial therapy. On lung biopsy, Pseudomonas cepacia was obtained in pure culture, sensitive to chloramphenicol, tetracycline, kanamycin and nalidixic acid. With specific therapy, he slowly recovered. P. cepacia has not been previously described as a cause of persistent pneumonia in immunodeficient children. The occurrence of CGD and selective IgA deficiency together is a very rare combination of immunodeficiencies.