A Successful Treatment Approach in 2 Patients With Type 1 Plasminogen Deficiency: Intratracheal Application of Fresh Frozen Plasma and Tissue Plasminogen Activator.

BACKGROUND: Respiratory system involvement is common in congenital plasminogen deficiency. Although many treatment approaches have been tried, there is still no definitive treatment for respiratory system involvement. OBSERVATIONS: We report 2 congenital plasminogen deficiency cases, who presented with severe respiratory symptoms, for whom a novel treatment modality was tried. After intravenous administration of FFP (fresh frozen plasma), tissue plasminogen activator and FFP were administered intratracheally, and respiratory system findings improved. CONCLUSIONS: Intratracheal administration of tissue plasminogen activator and FFP is an available treatment modality for patients with lung involvement. Fibrin plaques should be carefully removed and new lesion formation should be prevented.

Transepidermal water loss in allergic diseases.

Background: In recent years, the epithelial barrier hypothesis has been emphasized in the formation of allergic diseases. Transepidermal water loss (TEWL) occurs through diffusion and evaporation from the skin to the external environment. There are few studies on TEWL in allergic diseases. Objective: This study evaluated the relationship between patients with atopic diseases and healthy controls and hygiene habits in TEWL. Methods: The study was conducted on patients who were followed up for atopic disease (asthma, allergic rhinitis, immunoglobulin E mediated food allergy, and atopic dermatitis) and healthy children. TEWL measurement was in a room that was stable in terms of humidity and temperature by using a widely validated open room system. During the measurement, the participants reported their frequency of taking a shower and cleaning product use. Results: In the study group, TEWL was measured in 182 patients, and the median (min-max) TEWL was 21.3 g/hm² (7.8-101.3 g/hm²) in the disease group and 9.6 g/hm2 (3.9-30.3 g/hm²) in the control group (p < 0.001). The number of weekly baths was higher in the disease group (p < 0.001). The cutoff for atopic diseases was 13.2 g/hm² (sensitivity, 83.2%; specificity, 84.3%; p < 0.001). Conclusion: High TEWL in atopic diseases supports the epithelial barrier hypothesis associated with disease development. Further studies are necessary to determine the threshold between healthy controls and the patients in the disease group. The TEWL measurement can be an effective method to determine the risk groups. Moreover, further studies related to factors on TEWL and treatment methods to reduce this loss are necessary, too.

Does the severity of atopic dermatitis change with allergic sensitization? Is it real or a myth?

OBJECTIVE: Atopic dermatitis (AD) is a chronic inflammatory skin disease that can occur at any age. This study aimed to evaluate the impact of food allergy on disease severity as well as clinical/laboratory findings in children with AD. METHODS: Clinical and laboratory data of AD patients evaluated for food allergy between January 2021 and December 2022 were examined retrospectively. RESULTS: Of the 52 patients evaluated, 32 (61.5%) were males, with a median age of 6 months (2-118 months). Among them, 26 (50%) had food allergies (FA) and five (9.6%) had inhalant allergen sensitivity. No significant difference in AD severity was observed between patients with and without FA. However, the FA group showed higher serum lactate dehydrogenase (LDH) levels (343.3 ± 81.5 U/L vs 297.7 ± 77.4 U/L; P = 0.011) and lower red cell distribution width (RDW). Inhalant allergen sensitivity was associated with higher AD severity. CONCLUSION: While guidelines recommend investigating food allergies in moderate to severe AD, this study found no significant difference in the relationship between AD severity and the presence of FA. However, inhalant allergen sensitivity was linked to increased AD severity. Therefore, a comprehensive patient history should include an evaluation of food allergies in children with AD, regardless of disease severity. Elimination and provocation tests related to suspected food items should be performed, and allergenic foods should be removed from the diet if they are found to contribute to the allergy.

Is There a Clinical Significance of Very Low Serum Immunoglobulin E Level?

PURPOSE: High serum immunoglobulin (Ig) E levels are associated with allergies, parasitic infections, and some immune deficiencies; however, the potential effects and clinical implications of low IgE levels on the human immune system are not well-known. This study aims to determine the disorders accompanying very low IgE levels in children and adults. METHODS: The patients whose IgE levels were determined between January 2015 and September 2020 were analyzed, and the patients with an IgE level < 2 IU/mL were included in this study. Demographic data, immunoglobulin levels, autoantibody results, and the diagnoses of the patients were noted from the electronic recording system of the hospital. RESULT: The IgE levels were measured in 34,809 patients (21,875 children, 12,934 adults), and 130 patients had IgE levels < 2 IU/mL. Fifty-seven patients were children (0.26%); 73 were adults (0.56%). There was a malignant disease in 34 (9 of them children) (26%), autoimmune diseases in 20 (3 of them children) (15.4%), and immunodeficiency in 17 (14 of them children) (13.1%) of the patients. The most common reasons were other diseases, immunodeficiency and malignancy in children, and malignancy, autoimmune disorders, and other diseases in the adults, in rank order. The IgE level did not show any correlation with the levels of other immunoglobulins. CONCLUSION: Although rare, a low IgE level has been shown to accompany malignancies, autoimmune disorders, and immune deficiencies. Patients with very low IgE levels should be carefully monitored for systemic disorders.

Is There an Optimal Training Interval to Improve the Correct Use of Adrenaline Auto-Injectors?

BACKGROUND: Accurate use of adrenaline auto-injectors (AAIs) for anaphylaxis is critical to decrease mortality and morbidity. OBJECTIVE: In this study, we aimed to assess user knowledge of AAIs and evaluate the factors that affect their correct use. METHODS: The study involved caregivers of pediatric patients diagnosed with anaphylaxis who were trained with trainer injectors up to 24 months ago. The demographics of the caregivers, anaphylaxis history of the patients, usage of AAIs in the case of anaphylaxis, and the reasons for not using AAIs in anaphylaxis were evaluated. Users were asked to demonstrate the use of Penepin® with a trainer injector. RESULTS: Fifty-nine caregivers were enrolled in the study. Forty-seven (79.7%) users stated that they always carry AAIs with them. Forty-one (69.5%) of the users demonstrated all steps of the use of AAIs. The time from the last AAI training was the most significant parameter affecting the ability to use AAIs correctly (OR 0.678, 95% CI 0.546-0.841, p < 0.0001). AAI training every 6 months results in the proper usage of AAIs, with 96% probability. Thirty (50.8%) caregivers stated that anaphylactic reactions occurred in their children after the last AAI training. Of these, 16 (53.3%) users stated that they did not use an AAI for the anaphylactic reaction. The most common reason (50%) was not carrying an AAI on their person. CONCLUSION: Training users at least every 6 months is associated with the proper application of AAIs. Although regular training increases the frequency of AAI use in anaphylaxis, awareness of carrying AAIs is the most important factor for usage of AAI in anaphylaxis.

Evaluation of risk factors for recurrence of cutaneous adverse reactions due to anti-seizure medications in children: A retrospective study.

Background: Cutaneous adverse reactions (CARs) are one of the most important reasons for anti-seizure medication (ASM) discontinuation in epilepsy. However, such discontinuations can cause an increase in seizures. This study investigates the risk factors for ASM-related rash recurrence in children. Methods: This retrospective case-control study consisted of the patient group with a single rash due to ASMs (group 1), the patient group with rash recurrence (group 2), and the control group. While the demographic and clinical features of group 1 and the control group were compared in terms of a single rash, group 1 and group 2 were compared for rash recurrence. Results: Group 1, group 2, and control group consisted of 112, 33, and 166 patients, respectively. Female gender was a risk factor for a single rash (P < 0.001) but not for recurrence (P = 0.439). Presence of atopic disease [odds ratio (OR): 9.5, 95% confidence interval (CI): 3.8-23.1, P < 0.001], family history of drug allergy (OR: 26.3, 95% CI: 9.6-72.1, P < 0.001), and polytherapy (OR: 23.5, 95% CI: 8.7-62.9, P < 0.001) were risk factors for rash recurrence. Aromatic nature of both the ASMs associated with the first rash (OR: 14.4, 95% CI: 3.2-63.2, P < 0.001) and rash recurrence (OR: 11.3, 95% CI: 4.6-27.5, P < 0.001) were determined as risk factors separately. Conclusion: Careful use of aromatic drugs may prevent recurrence of ASM-related CAR in children, particularly in cases of personal history of allergic disease and family history of drug allergy.