Symptomatic hypogammaglobulinaemia in children younger than 2 years of age was studied to rule out a primary immunodeficiency. Thirty-four patients were referred to the Immunology Service to study the hypogammaglobulinaemia-associated clinical picture. Food allergy was documented in 10 patients by personal and familial history, presence of specific immunoglobulin E (IgE) and elevated total serum IgE levels. Coeliac disease and human immunodeficiency virus infection were also ruled out. Protein loss through stools was assessed by clearance of alpha1-antitrypsin (AAT). Serum immunoglobulin levels were determined by nephelometry and functional antibodies were studied by enzyme-linked immunosorbent assay. The cellular immune response was assessed by in vitro lymphocyte proliferation in response to mitogens and cell subsets were analysed by flow cytometry. In five patients of the 10 patients we suspected a protein loss through the mucosa. Four of these five patients showed an increased AAT and the other showed an extensive cutaneous lesion. Immunological studies revealed normal antibody function, in vitro lymphoproliferative responses and cell numbers in four of the 5 patients. One patient showed abnormally low numbers of CD4(+) T cells as well as a defective proliferative response to mitogens. After diagnosis of cow milk allergy, milk was replaced with infant milk formula containing hydrolysed proteins. Recovery of immunoglobulin values and clinical resolution were achieved. Hypogammaglobulinaemia during early childhood in some children may be secondary to cow milk allergy, and immunoglobulins and cells may leak through the inflamed mucosa. Resolution of symptoms as well as normalization of immunoglobulin values may be easily achieved by avoidance of the offending allergen.
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http://dx.doi.org/10.1111/j.1365-2567.2007.02628.x | DOI Listing |
Genet Med
January 2025
Division of Human Genetics, Children's Hospital of Philadelphia; Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
Purpose: Noonan syndrome and related disorders (NS) are multisystemic conditions affecting approximately 1:1000 individuals. Previous natural history studies were conducted prior to widespread comprehensive genetic testing. This study provides updated longitudinal natural history data in participants with molecularly confirmed NS.
View Article and Find Full Text PDFAdv Clin Exp Med
January 2025
Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland.
Background: We still know little about the effective pharmacological treatment of heart failure (HF) associated with the Fontan circulation. One of the new options may be sodium glucose cotransporter-2 inhibitors (SGLT2i), which have been proven effective in classic forms of left ventricular HF.
Objectives: To evaluate the effect and safety of SGLT2i inclusion in adults with Fontan circulation.
Dev Sci
March 2025
Department of Pediatrics and Adolescent Medicine, Comprehensive Center for Pediatrics, Medical University of Vienna, Vienna, Austria.
Newborns are able to neurally discriminate between speech and nonspeech right after birth. To date it remains unknown whether this early speech discrimination and the underlying neural language network is associated with later language development. Preterm-born children are an interesting cohort to investigate this relationship, as previous studies have shown that preterm-born neonates exhibit alterations of speech processing and have a greater risk of later language deficits.
View Article and Find Full Text PDFHypertension
January 2025
The George Institute for Global Health, Faculty of Medicine, University of New South Wales, Newtown, Australia (L.C., S.Y., N.E., M.W., T.L., Y.G., C.S.A., K.H., X.C., R.P.).
Background: The association between systolic blood pressure and all-cause mortality differs between frail and nonfrail individuals, highlighting uncertainties about the effectiveness of antihypertensive treatments in frail populations.
Methods: Using data from the SHEP trial (Systolic Hypertension in the Elderly Program), a baseline frailty index (FI), including 55 variables, was constructed. Fine-Gray subdistribution hazard models and Cox proportional hazards regression models were used to explore the association between baseline FI and the risks of stroke, cardiovascular disease, and all-cause death, as well as to examine whether the impact of antihypertensive treatment on these outcomes was modified by baseline FI.
Circ Cardiovasc Qual Outcomes
January 2025
Department of Preventive Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL. (N.A.C., X.H., L.C.P., H.N., N.S.S., A.M.P., P.G., D.M.L.-J., K.N.K., S.S.K.).
Background: Suboptimal cardiovascular health (CVH) in pregnancy is associated with adverse maternal and offspring outcomes. To guide public health efforts to reduce disparities in maternal CVH, we determined the contribution of individual- and neighborhood-level factors to racial and ethnic differences in early pregnancy CVH.
Methods: We included nulliparous individuals with singleton pregnancies who self-identified as Hispanic, non-Hispanic Black (NHB), or non-Hispanic White (NHW) and participated in the nuMoM2b cohort study (Nulliparous Pregnancy Outcomes Study: Monitoring Mothers-to-Be).
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