To better characterize infants presenting with diminished immunoglobulin levels and intact antibody formation, we present 49 such infants, correlating presenting characteristics with history and time to immunoglobulin normalization. Term infants with the following characteristics were included: 1) one or more immunoglobulin classes > 2SD below mean, 2) protective antibody titer to tetanus and diphtheria, 3) intact cellular immunity, 4) no features of other syndromes. The children were 69.4% male and had recurrent otitis media (77.6%), wheezing (61.2%), and atopy (26.5%). Diminished IgA (95.9%) was most common, but 65.3% had multiple isotypes diminished. During follow-up, 25/49 (51%) normalized immunoglobulins, of whom 80% were male; only 48% normalized in infancy. Female immunoglobulin normalization was significantly delayed (p< .001). No deaths or serious infections occurred. This phenotype is predominantly seen in male infants with otitis media and wheezing. Female infants have significantly delayed immunoglobulin normalization. Transient hypogammaglobulinemia of infancy can be diagnosed only retrospectively.
Both severe combined immunodeficiency (SCID) and cystic fibrosis (CF) may present in infancy with a history of respiratory infections and failure to thrive. Elevated sweat chloride levels on multiple sweat tests is diagnostic of CF; transient elevation of sweat chloride has been reported in patients with hypogammaglobulinemia and antibody deficiency without CF. This article presents a case report of a 5-month-old boy with recurrent respiratory infections, failure to thrive, and two borderline elevated sweat test levels. Laboratory evaluation including testing for CF as well as immune deficiency was performed in this patient. Two borderline abnormal sweat chloride tests together with isolation of Pseudomonas from the airway caused clinicians initially to suspect CF; however, mutation in gene coding for the gamma-chain of the IL-2 receptor and a negative CF genetic mutation analysis ultimately led to the final diagnosis of SCID. It is essential to make the diagnosis of SCID as early as possible because infants with SCID who do not undergo reconstitution of their immune system universally die in infancy because of infection. Early diagnosis and intervention can lead to an excellent prognosis in a previously fatal disease.
Background
By identifying a patient's level of health literacy (HL) one can help to ensure that any health information is tailored to the correct level and by doing so empower the patient to take responsibility for his/her own health and consequently help improve medicines adherence and healthcare outcomes. The efficacy of oral anticoagulation treatment depends on maximising the length of time within the therapeutic range (TTR).
Purpose
To assess the HL of patients attending a pharmacist-led warfarin anticoagulation clinic using the Rapid Estimate of Adult Literacy in Medicine (REALM) screening tool. To obtain data on the TTR of each patient who has completed the REALM. To analyse the results of the above and establish whether there is a link between adequate HL and anticoagulation control as measured by TTR.
Materials and methods
Patients were asked to participate and included if they met the following criteria: aged over 18 years, not visually impaired, no hearing impairment, English as a first language and on warfarin for at least 3 months. Patients were asked a series of questions linked to HL and the REALM screening tool was administered. The level of statistical probability used to determine significance was set at p <0.05.
Results
129 patients completed the study with a mean age of 72 years (SD 9.5). The most common indication for warfarin was atrial fibrillation (74.4%). Adequate HL was usual among the patients who completed the study (82.2%). A statistically significant positive correlation was found between TTR and level of education and between REALM and level of education. A statistically significant correlation was also found between REALM and how often the patient read a book.
Conclusions
This study showed an association between a likely predictor of HL (namely level of education) and TTR. Pharmacists are well positioned to identify at-risk patients and tailor education to their needs.
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