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Common variable immunodeficiency and IgG subclass deficiency in central Alabama hemochromatosis probands homozygous for HFE C282Y.

Eight hemochromatosis probands with HFE C282Y homozygosity had frequent, severe, or unusual infections and common variable immunodeficiency (CVID) or immunoglobulin (Ig) G subclass deficiency (IgGSD). Thus, we performed serum Ig isotyping and other characterization of 43 additional unselected probands, 5 human leukocyte antigen (HLA)-identical siblings, and 240 consecutive CVID or IgGSD index patients. C282Y allele frequencies were estimated in 58 CVID or IgGSD index patients without hemochromatosis phenotypes and in 341 controls. HLA-A and -B haplotypes and frequencies were determined in all 51 probands, 186 CVID or IgGSD index patients without hemochromatosis phenotypes, and 751 controls. Thirteen unselected probands (30%) had CVID or IgGSD. Among all 21 hemochromatosis probands with CVID (n = 4) or IgGSD (n = 17), Ig subclass deficiency patterns were IgG(1) (n = 5), IgG(1) and IgG(3) (n = 6), IgG(3) (n = 9), and IgG(1), IgG(3), and IgG(4) (n = 1). IgG(2) or IgA deficiency was not detected; one proband had IgM deficiency. Mean values of total IgG, IgG(1), and IgG(3) were significantly lower in probands with CVID or IgGSD. Mean values of age, transferrin saturation, and ferritin at diagnosis and phlebotomy units required to induce iron depletion were similar in probands with or without CVID or IgGSD; phlebotomy had no apparent effect on IgG levels. C282Y frequencies were similar in CVID or IgGSD index cases without hemochromatosis phenotypes and in controls. There was concordance of Ig and hemochromatosis phenotypes in probands and respective HLA-identical siblings. Eight of 240 CVID or IgGSD index patients had hemochromatosis phenotypes and C282Y homozygosity (3 vs 0.7% and 0.2% controls; P < 0.0001, respectively). The frequency of A*03-B*07 was greater in CVID and IgGSD index cases without hemochromatosis phenotypes than in controls (0.0968 vs 0.0546, respectively; P = 0.0032). HLA-A*03-B*07 was the predominant haplotype in probands grouped by presence or absence of CVID or IgGSD. Some probands in each group were A*03-B*07 homozygotes; group A*03-B*07 frequencies were similar. We conclude that serum IgG abnormalities characteristic of CVID or IgGSD are common in hemochromatosis probands, and that the prevalence of hemochromatosis is increased in CVID and IgGSD index cases. These observations could be explained by the increased frequencies of HLA-A*03-B*07 in C282Y homozygotes and in CVID and IgGSD, and by the common occurrence of putative CVID or IgGSD allele(s) on haplotypes bearing C282Y.

Adult↗

Cerebrospinal fluid shunt infections in infants and children in Mobile, Alabama.

Cerebrospinal fluid shunt systems are used to treat hydrocephalus in infants and children; unfortunately, some shunt systems become infected. We sought to define the epidemiology of shunt infections and shunt survival prior to infection at our institution. We identified 268 shunt procedures performed from January 1990 to June 1996 in 145 patients. There were 29 episodes of shunt infection for an incidence of 10.8% per procedure and 13.1% per patient. Staphylococcus epidermidis was the most common isolate recovered. The probability of shunt infection was highest during the first 8 weeks after a shunt procedure and subsequent infection was less likely after 28 weeks.

Adolescent↗

High rate of varicella complications among Mexican-born adults in Alabama.

OBJECTIVE: Our study examines risk factors for severe varicella in an outbreak among Mexican-born adults, and it compares susceptibility to infection and reliability of self-reported varicella history for these individuals with that for adults born in the United States in the outbreak locale, which may guide vaccination strategies. METHODS: We interviewed case patients and non-case persons in the affected apartment complex and workplace, assessed disease history and susceptibility by testing for varicella-zoster virus immunoglobulin G antibodies, and reviewed the clinical data of case patients. RESULTS: Five of 18 case patients had serious complications for which they sought medical care; 1 was hospitalized for pneumonia, and 1 was hospitalized for Guillain-Barré syndrome. Only intense exposure (e.g., sharing a bed) was marginally associated with severe disease (P=.08). In the workplace, varicella susceptibility was higher among Mexican-born workers (20%) than among workers born in the United States (3%) (adjusted prevalence odds ratio, 5.4; 95% confidence interval, 2.3-14.8). Mexican-born persons had the highest positive predictive value of self-reported disease (100%) in predicting immunity, and those born in the United States had the lowest negative predictive value of self-reported history (10%) in predicting susceptibility. CONCLUSIONS: Varicella is a more serious disease among adults than among children, and Mexican-born adults living in the United States might have a higher risk of acquiring varicella than US-born adults. Varicella outbreaks involving adults should be prioritized for control efforts. Outbreaks can be prevented by vaccinating susceptible adults.

Adolescent↗

Incidence and risk factors for sexually transmitted infections among women in an Alabama HIV clinic.

The objectives of this study were to assess the prevalence and incidence of curable sexually transmitted infections (STI), i.e., gonorrhea, chlamydial infection, or trichomoniasis, in a cohort of HIV-infected women. The study population was derived from women seeking primary care at an outpatient university HIV clinic who were participants in a women's natural history study. Enrollees (n = 225) were predominantly African-American, heterosexually infected, with mean age 35 years. Mean entry CD4+ T cell count was 405 cells/mm3. Over 6% were STI positive at initial screening. Subsequently, the combined curable STI incidence was 4.82/1000 woman-months over a median of 33 months of observation. Of 36 incident STIs, trichomoniasis was most common (n = 32). Predictors for acquisition of a curable STI included absenteeism at scheduled clinic appointments, RR = 1.99 (1.28, 3.08), and a higher CD4+ T cell count, RR = 1.15 (1.0028, 1.3115) for 100 cells. Interventions to prevent curable STI in HIV-infected women are warranted in primary care settings.

Absenteeism↗

Efficacy of pneumococcal polysaccharide vaccine in preventing acute otitis media in infants in Huntsville, Alabama.

In a study of the efficacy of pneumococcal capsular polysaccharide vaccines in preventing otitis media due to Streptococcus pneumoniae, 179 infants were randomly assigned to receive either a "target" vaccine containing serotypes 1, 3, 6, 7, 14, 18, 19, and 23 (which account for most cases of pneumococcal otitis media) or a control vaccine containing serotypes 2, 4, 5, 8, 9, 12, and 25 (which account for few cases). Vaccine (0.5 ml) was administered subcutaneously; a dose contained 25 micrograms of each serotype. The incidence of otitis media due to "target" serotypes was determined in both groups. There were not significant differences between vaccines and controls in either the incidence or etiology of episodes of otitis media before immunization. During a two-year follow-up, no significant difference was observed regarding the incidence of episodes due to "target" serotypes in infants who were immunized after 12 months of age. However, of 66 control infants immunized before 12 months of age, 22 experienced 33 pneumococcal episodes due to a "target" serotype, whereas only 13 of 67 vaccines accounted for 22 such episodes. Among infants who had two or fewer episodes before immunization, four vaccines and 13 controls continued to have episodes of otitis media and became "otitis prone," i.e., six or more episodes (chi 2 = 4.84; P = 0.05). Low levels of serum antibody were not protective, and in one case a level of 609 ng of antibody nitrogen/ml of middle ear effusion was not protective. Thus, although the vaccine may be associated with a lower incidence of otitis media, the results of this study do not show a statistically significant efficacy of the vaccine.

Acute Disease↗