Digital Response to Physical Crises: The Role of an E-Health Platform in the 2023 Southern Turkey Earthquakes.

The catastrophic earthquakes that struck Southern Turkey in 2023 highlighted the pressing need for effective disaster management strategies. The unprecedented scale of the crisis tested the robustness of traditional healthcare responses and highlighted the potential of e-health solutions. Despite the deployment of Emergency Medical Teams, initial responders - primarily survivors of the earthquakes - faced an enormous challenge due to their lack of training in mass-casualty situations. An e-health platform was introduced to support these first responders, offering tools for drug calculations, case management guidelines, and a deep learning model for pediatric X-ray analysis. This commentary presents an analysis of the platform's use and contributes to the growing discourse on integrating digital health technologies in disaster response and management.

High Inborn Errors of Immunity Risk in Patients with Granuloma.

OBJECTIVE: Granuloma etiology includes infections, vasculitis, chemicals, malignancies, lymphoproliferative disorders, and immunological diseases. We hypothesized that patients with granuloma have an underlying primary immunodeficiency disease (PIDD). PATIENTS AND METHODS: We retrospectively enrolled 82 patients with immunological evaluation among 294 biopsy-proven granuloma patients (0- to 20-year-old). At the same time frame, we followed up with 1910 patients in the same age group. RESULTS: Out of 82 patients, male/female ratio was 45/37. Median age at symptom onset was 5 years (28 days-17.4 years), age of granuloma at diagnosis was 8.6 years (36 days-19.4 years). Common symptoms at disease onset were fever (23.2%), lymphadenopathy (19.6%), abdominal pain (12.2%), and cough (12.2%). Granuloma was frequent in lymph nodes (26.8%), skin (19.5%), lung (13.4%), and bone (11%). Common infectious agents isolated were Mycobacterium spp. (23.2%) and EBV (4.9%). We document PIDD in 76.8% (63/82) of patients. 49.4% (40/81) of immunologically evaluated granuloma patients had hypogammaglobulinemia. Granuloma rate in pediatric PIDD was 3.3%(63/1910). Patients with multiple granulomas (n = 16) had a PIDD diagnosis. Lung involvement was three times more in PIDD. Brain involvement was only seen in PIDD. Fibroadipose tissue and liver involvement were more frequent in patients without documented PIDD and patients whose evaluation was not completed. The mortality rate in PIDD with granuloma was 15.9%, whereas there was no mortality in patients without PIDD. CONCLUSION: As documented here, even in a university hospital, the immunologic evaluation ratio is about one-third. We showed high PIDD frequency in children with granuloma, and higher mortality in PIDD with granuloma. Thus, an immunologic evaluation performed meticulously by immunologists is a must for accurate diagnosis and decision of individualized therapeutic options.

Comparison of IVIG resistance predictive models in Kawasaki disease.

BACKGROUND: We aimed to compare the ten different scores (by Kobayashi, Egami, Harada, Formosa, Sano, Piram et al., Wu et al., Yang et al., Tan et al., and Kanai et al.) to assess their performance in predicting IVIG resistance in Turkish children. METHODS: Complete and incomplete KD patients diagnosed with KD at Hacettepe University between June 2007 and September 2019 were evaluated retrospectively. RESULTS: A total of 129 patients, 79 boys (61.2%), with a median age 36 (IQR 19.5-57.0) months were evaluated. Sixteen patients (12.4%) had IVIG resistance. Sensitivity was low for all the ten scores. Tan, Sano, and Egami predictive models had the highest specificity (97.3, 89.4, 86.7%, respectively). Almost all scoring systems distinguished the group of patients with low risk for IVIG resistance but could not differentiate IVIG-resistant patients. Multivariate analysis for the laboratory features showed that platelet count <300 × 109/L and GGT serum levels were independent risk factors for IVIG resistance (OR: 3.896; 95% CI: 1.054-14.404; p = 0.042 and OR: 1.008; 95% CI: 1.001-1.015; p = 0.050). CONCLUSIONS: The current scoring systems had a low sensitivity for predicting the risk for IVIG resistance in Turkish children. On the other hand, increased serum GGT levels and low platelet count were risk factors for predicting IVIG resistance. IMPACT: Intravenous immunoglobulin (IVIG) resistance may be observed in 10-20% of patients diagnosed with Kawasaki disease. Coronary artery involvement is more frequent in IVIG-resistant patients. It is important to predict the patients who might develop IVIG resistance to improve prognosis. The performance of the IVIG resistance predictive models in Kawasaki disease in our population is limited due to the low sensitivity.

Penile involvement of immunoglobulin a vasculitis/Henoch-Schönlein purpura.

BACKGROUND: Immunoglobulin A vasculitis/Henoch-Schönlein purpura (IgAV/HSP) is a leukocytoclasia vasculitis of childhood, predominantly affecting the skin, joints, gastrointestinal tract, and kidneys. The involvement of the penis is rare. OBJECTIVE: We aimed to describe this rare manifestation of IgAV/HSP and to review the previous studies, including similar cases. METHODS: Clinical data were reviewed for two children of penile involvement of IgAV/HSP in our hospital. More clinical cases were retrieved from the databases of PubMed/MEDLINE and Scopus from database inception to February 1, 2020. RESULTS: We presented two boys aged three and five years both of whom had penile lesions after presenting with the typical rash of IgAV/HSP on lower extremities. The penile lesions improved entirely in a few days without treatment in one and with corticosteroid treatment in the other. The literature review revealed 12 articles describing 20 patients with penile involvement of IgAV/HSP. The penile findings were edema, erythema, ecchymosis, purpuric rash, edema, color change, stiffness of the shaft or prepuce, and tenderness. Penile lesions appeared before the purpuric rash of IgAV/HSP in three of 22 patients. The penile involvement could make the diagnosis challenging especially if the penile lesions appear before the typical rash of the disease. The lesions improved entirely in the short term in all patients; in five without treatment in fifteen after corticosteroid or immunosuppressive drug treatment. CONCLUSIONS: It is important to raise awareness about this rare manifestation among health care providers. It is not clear whether corticosteroid treatment should be initiated for treatment since it seems as a self-limited feature. Treatment with corticosteroids could be considered in the treatment of selected cases especially with systemic involvement.

A Patient With AIRE Mutation Who Presented With Severe Diarrhea and Lung Abscess.

Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) (polyglandular endocrinopathy type 1) is a rare autosomal recessive disorder caused by mutations in the autoimmune regulator gene (AIRE). The major clinical features of APECED are hypoparathyroidism, adrenal insufficiency (Addison disease), and chronic mucocutaneous candidiasis. This disease is also associated with multiple other and uncommon autoimmune (autoimmune hepatitis, autoimmune enteropathy, atrophic gastritis with or without pernicious anemia, gonadal failure, diabetes mellitus, hypothyroidism, functional hyposplenism), ectodermal (alopecia and vitiligo), and inflammatory (intestinal lung disease, nephritis) features. Here, we report a case of a 13-year-old Turkish boy who presented wih enteropathy and lung abscess. Molecular genetic analysis demonstrated a homozygous frameshift mutation (p.Asp70fs, c.208_209insCAGG) in exon 2, in AIRE gene. APECED may present with severe, life-threatening infections due to functional hyposplenism. Multidisciplinary approach, careful follow-up, and molecular genetic studies are needed.