A homozygous mucosa-associated lymphoid tissue 1 (MALT1) mutation in a family with combined immunodeficiency.

Background: Combined immunodeficiency (CID) is characterized by severe recurrent infections with normal numbers of T and B lymphocytes but with deficient cellular and humoral immunity. Most cases are sporadic, but autosomal recessive inheritance has been described. In most cases, the cause of CID remains unknown.

Anticipatory guidance and early child development: pediatrician advice, parent behaviors, and unmet needs as reported by parents from different backgrounds.

Anticipatory guidance is acknowledged as an important aspect of pediatric practice, and research shows that parents expect and value the information and advice they receive from their pediatric health care providers. This study surveyed 373 parents (88% mothers) of infants in waiting rooms of diverse pediatric practices in Memphis,Tennessee, about their recall of anticipatory guidance received, unmet needs for anticipatory guidance, preferences for sources of information, and activities to promote healthy development. Most parents recalled specific guidance received (diet, communication, and discipline being the most common), and unmet needs (discipline and development being among the most common).

Prevalence and costs of acute and chronic potentially avoidable pediatric hospitalizations in Tennessee.

Background: Potentially avoidable pediatric hospitalizations (PAPH) can now be identified using an analytical tool developed by the federal Agency for Healthcare Research and Quality (AHRQ). We apply this new tool to Tennessee inpatient discharge records for 2005 to determine the prevalence of PAPH and analyze the variation patterns of PAPH across racial, gender, and insurance status lines.

Methods: Retrospective analysis of administrative data based on the UB-92 claims forms submitted by all short-term acute-care hospitals in Tennessee for 2005.

Variations in costs for the care of low-birth-weight infants among academic hospitals.

Objectives: To determine the relative role that academic hospitals (AHs) play in providing neonatal care for low-birth-weight infants within a single state and to determine if there are variations in inpatient costs for neonatal services among AHs.

Design: Retrospective analysis of hospital costs for low-birth-weight infants.

Setting: Cases were identified using 2003-2005 data from the Tennessee Hospital Discharge Data System.

Is environmental tobacco smoke exposure a risk factor for acute gastroenteritis in young children?

Because passive smoke exposure has not been previously linked to diarrhea diseases in children, it was hypothesized that very young children exposed to environmental tobacco smoke (ETS) exposure at home would also be more likely to develop infectious gastroenteritis (GE) than their unexposed counterparts. During 1-year period, 260 children 36 months and younger were prospectively followed up in a private pediatric practice in a southern community in the United States. Multiple logistic regression analysis showed that ETS was strongly predictive of acute GE in the univariate analysis (P = .

Imaging characteristics of metastatic lesions to the cerebellopontine angle.

Objective: To describe characteristic features of metastatic lesion to the cerebellopontine angle (CPA) and internal auditory canal (IAC).

Study Design: Retrospective review.

Setting: Tertiary care medical center.

Potentially avoidable pediatric hospitalizations as defined by the Agency for Healthcare Research and Quality: what do they tell us about disparities in child health?

This study was conducted to determine if the Agency for Healthcare Research and Quality's newly established pediatric quality monitors, which measure potentially avoidable hospitalizations, are useful in detecting disparities in health care delivery. Data for all hospital discharges in Tennessee in 2002 were evaluated for the 5 pediatric discharge monitors identified by the Agency for Healthcare Research and Quality. These diagnoses were asthma, short-term complications of diabetes, gastroenteritis, urinary tract infection, and perforated appendix.

Do first years really last a lifetime?

Multiple early childhood intervention programs have been introduced to provide at-risk children with a better start in life. This is due to the recognition that early childhood experiences, both positive and negative, can affect the physical, mental, behavioral, and economic well-being of the child. A few of these programs have evaluated long-term outcomes and have demonstrated that the effects of interventions may still be apparent years after the intervention.

Black/white differential use of health services by young children in a rural Mississippi community.

Background And Objectives: Use of health services in rural communities has not been well studied. We explored how black and white children used health services in a rural Mississippi community.

Methods: Data were prospectively collected for 396 children attending a private practice to determine if race was associated with the use of health services in this community.

Environmental tobacco smoke exposure: prevalence and mechanisms of causation of infections in children.

Background And Objectives: Environmental tobacco smoke (ETS) exposure is probably one of the most important public health hazards in our community. Our aim with this article is to (1) review the prevalence of ETS exposure in the United States and how this prevalence is often measured in practice and (2) summarize current thinking concerning the mechanism by which this exposure may cause infections in young children.

Methods: We conducted a Medline search to obtain data published mainly in peer-reviewed journals.

Breast-feeding initiation: predictors, attitudes, and practices among blacks and whites in rural Mississippi.

Objectives: We attempted to determine the incidence, predictors, attitudes, and practices of breast-feeding initiation among women in a low-income rural Mississippi community.

Methods: We interviewed 420 new mothers regarding breast-feeding.

Results: Ninety-six women (24%) initiated breast-feeding.

Follow-up of pediatric patients with recurrent infection and mild serologic immune abnormalities.

Background: Children with recurrent infections significant enough to warrant referral to an immunologist frequently have mild abnormalities of the humoral immune system. Parents of these children are generally reassured that their child will outgrow the clinical problems that prompted their referral.

Objective: This is a retrospective study with the objective being to evaluate changes in immune measurements and clinical status of children with recurrent infections followed in an immunology clinic.

Immunologic considerations in the child with recurrent or persistent sinusitis.

The diagnosis and management of chronic sinusitis in children represents a difficult challenge for the clinician. Part of the problem stems from the fact that normal children have many upper respiratory infections and it is sometimes difficult to determine whether the upper respiratory symptoms a child experiences with these infections are consistent with a normal number of "routine" childhood infections or whether there is a more significant problem. The issue is complicated by the fact that sinus infections in children may not have the same clinical manifestations as similar infections in adults.

Repeated invasive pneumococcal infections in young children without apparent underlying immunodeficiency.

During a 30-month interval at LeBonheur Children's Medical Center, 394 patients had a blood or cerebrospinal fluid culture positive for Streptococcus pneumoniae. Sixteen of these episodes (4%) were repeated infections; 6 of these 16 patients had sickle cell disease. Six of the remaining 10 patients had immunologic evaluations of varying completeness; no immunodeficiency was identified by these tests or on follow-up.

Antibody response to unconjugated Haemophilus influenzae b and pneumococcal polysaccharide vaccines in children with recurrent infections.

Background: Increasingly, antibody testing is being used to evaluate the status of humoral immunity in patients with recurrent infection and suspected immunodeficiency. In the past, we had been impressed that immunization with unconjugated Haemophilus influenzae b (uHib) vaccine provided useful information about the ability to produce antibody to polysaccharides and that the use of pneumococcal polysaccharide (PPS) vaccine frequently produced results that were difficult to interpret.

Objective: The study was carried out to compare antibody responsiveness to vaccination with uHib with the response seen after PPS vaccination.

Practice parameters for the diagnosis and management of immunodeficiency. The Clinical and Laboratory Immunology Committee of the American Academy of Allergy, Asthma, and Immunology (CLIC-AAAAI).

In this brief review, only the most useful immunologic tests available for defining host defects that lead to susceptibility to infection have been emphasized. It should be pointed out that those evaluations and tests ordered by the physician will rule out the vast majority of the currently recognized defects. Finally, it is important that any patients identified as abnormal by these screening tests be characterized as fully as possible in centers specializing in these diseases before therapy is initiated, since what may appear to be a simple diagnosis on the surface may be an indicator of more complex underlying problems.

Immunization status of hospitalized preschool children: risk factors associated with inadequate immunization.

Objectives: The purposes of this study were to determine the accuracy of the immunization histories of hospitalized preschool children, assess the sociodemographic factors associated with delayed immunizations, and interview parents or guardians concerning their views on ways of improving immunization delivery.

Methods: The immunization status of 215 preschool children admitted to a pediatric hospital was determined by interviewing parents or guardians regarding their children's immunization histories. The patient's immunization records were subsequently reviewed for confirmation.

Cell-mediated immune status of children with recurrent infection.

Objective: To evaluate the cell-mediated immune status of children with recurrent respiratory tract infections.

Design: We evaluated the cell-mediated immune status of 76 patients referred because of recurrent infection. Patients were divided into those with serologic abnormalities and those without such findings.

Elevated in vitro IL-4 production in a patient with elevated serum IgE.

Background: We have cared for an unusual patient with serum IgE concentrations of up to 150,000 IU/mL who did not have the hyper-IgE syndrome.

Objective: Our objective in this study was to compare the regulation of in vitro IgE synthesis in this patient with results reported for in vitro IgE production by patients with the hyper-IgE syndrome.

Methods: We utilized an enzyme-linked immunosorbent assay to measure in vitro IgE production under varying conditions.

Use of intravenous immunoglobulin (IVIG) therapy in adults.

Although intravenous immunoglobulin (IVIG) has primarily been recognized as useful in patients with antibody deficiencies, it continues to be used for an ever-expanding list of conditions. In addition to antibody replacement, IVIG may act as an immune modulator in certain autoimmune and inflammatory conditions. Its use should be carefully weighed against the high cost of the product.

Hyper IgM syndrome associated with defective CD40-mediated B cell activation.

Recent studies show that most patients with X-linked hyper IgM syndrome have defects in the gene for CD40 ligand. We evaluated 17 unrelated males suspected of having X-linked hyper IgM syndrome. Activated T cells from 13 of the 17 patients failed to bind a soluble CD40 construct.

Oral dehydroepiandrosterone in physiologic doses modulates immune function in postmenopausal women.

Objective: This study tests the hypothesis that dehydroepiandrosterone or its metabolic products are immunomodulatory in postmenopausal women with relative adrenal androgen deficiency.

Study Design: A prospective, randomized, double-blind, crossover study of 11 subjects with 3-week treatment arms separated by a 2-week washout period was performed. Immunologic evaluation at the beginning and end of the treatment arms consisted of flow cytometry to delineate T-cell populations, in vitro T-cell mitogenic response and cytokine production, and natural killer cell cytotoxicity.

Clinical significance of IgG subclasses.

IgG subclass deficiency has been noted in association with a variety of clinical conditions. It's relative importance as a contributing factor to these conditions is unclear. Despite reservations about the importance of partial IgG subclass deficiency, evidence is beginning to emerge that suggests that in select patients with recurrent infection and subclass deficiency, gamma globulin therapy may be beneficial.