Asuman Demirbuğa

Purpose: The COVID-19 pandemic has had a significant impact on solid organ transplantation (SOT), creating challenges in clinical management and contributing to substantial morbidity and mortality. By presenting our experience with immunosuppressive therapy management during COVID-19 infection in liver and kidney transplant recipients followed at our center, we want to raise awareness among transplant physicians about the effects of COVID-19 and discuss their management.Methods: The files of patients who were followed up with a diagnosis of COVID-19 at the Istanbul Faculty of Medicine Pediatric Pandemic Service and outpatient clinic between January 2020 and January 2023 were retrospectively evaluated. Demographic characteristics, transplantation characteristics, immunosuppression treatments, COVID-19 severity, and laboratory parameters were evaluated.Results: Thirty children diagnosed with COVID-19, with a history of liver and kidney transplantation, were included in the study. Twenty-one of these patients (n = 21, 70%) were liver transplant recipients and 9 (n = 9, 30%) were kidney transplant recipients. A total of 53.3% of the patients were male (n = 16) and the mean age was 10.1±4,85 years. Mild-to-moderate disease was observed in 93.3% patients. The rate of comorbidities, such as hypertension, obesity, and chronic lung disease, was 16.7%, and the hospitalization rate was significantly higher in these patients (p = 0.014). Of the nine patients whose immunosuppressive treatment was discontinued, three were kidney transplant recipients and six were liver transplant recipients; there was no statistically significant difference between them in terms of treatment discontinuation (p = 1,000). Patients in whom immunosuppressive treatment was discontinued had a significantly higher rate of severe disease (p = 0.049). Myocarditis was detected in one kidney transplant patient. No mortality was observed in any patient. The COVID-19 vaccination rate among patients was 13% (n = 4/30).Conclusions: Pediatric liver and kidney transplant recipients generally experienced mild COVID-19 courses without mortality. Comorbidities were associated with hospitalization. Severe disease was linked to immunosuppressive discontinuation, particularly mycophenolate mofetil. Our findings highlight the importance of individualized immunosuppression management during COVID-19 in pediatric transplant recipients.

Background/Objectives: Patients with rheumatic diseases have an increased burden of infection owing to biological disease-modifying antirheumatic drug (bDMARD) therapy. Therefore, vaccination is crucial for the prevention of infection in these patients. In this case–control study, we aimed to evaluate vaccine response to hepatitis B, pneumococcus, and measles using antibody titers in patients undergoing biological therapy. Methods: This study included 16 patients aged 5–18 years of age who received bDMARD treatment and 20 healthy controls. Serum samples of the patients were collected at baseline and subsequently on the 3rd and 6th months after bDMARD therapy, and IgG antibodies against pneumococcal capsular polysaccharide antigen (PCP), measles, and hepatitis B were measured. Results: There were no statistically significant differences in mean anti-HBsAg, anti-PCP, and anti-measles antibody titers between the study and control groups. The percentages of patients with anti-HbsAg, anti-PCP, and anti-measles protective antibodies were 68.8% (n = 11/16), 100% (n = 16/16), and 56.25% (n = 9/16), respectively. There were no statistically significant differences in the mean antibody titers at baseline and 3rd month. Only the anti-measles IgG titer level decreased below 200 (mIU/mL) in one patient in the 3rd month and in two patients in the 6th month. Conclusions: Patients with low or declining hepatitis B and measles antibody titers before or during bDMARD treatment may require close monitoring to ensure adequate protection against vaccine-preventable diseases. Regular screening and follow-up are essential in this patient population.

Aim. This study aimed to describe barriers and facilitators of the adherence of children with human immunodeficiency virus (HIV) to antiretroviral therapy (ART) from the perspectives of their caregivers. Methods. In-depth interviews were held with the caregivers of 15 children. The collected data were analyzed using thematic analysis procedures. The Consolidated Criteria for Reporting Qualitative Research (COREQ) were followed in the study. Results. The perspectives of caregivers were categorized under four themes and subthemes. Barriers and facilitators of the adherence of children with HIV to ART were categorized into 4 main themes: (1) medication-related, (2) child-related, (3) caregiver-related, and (4) health system-related. The results indicated that understanding the factors that influence pediatric ART adherence is critical to the development of adequate strategies. In addition, disclosure of HIV status to the child is also an important factor affecting drug administration in the social environment. Conclusions. To ensure adherence to lifelong ART, targeted caregiver support through continuous supervision, clear guidance on drug preparation, and strategies for effective administration should be integrated into context-specific interventions that address the combined influence of factors related to the child, medication, healthcare, and of the sociocultural environment.

Çocuklarda insan immün yetmezlik virüsü [human immunodeficiency virus (HIV)] enfeksiyonunun en yaygın bulaş yolu perinatal bulaştır. Anneden bebeğe gebelik, doğum ve emzirme sırasında HIV enfeksiyonu bulaşabilir. Bu çalışmada HIV ile enfekte annelerden doğan bebek ve annelere ait özelliklerin, perinatal HIV geçişini önlemek için uygulanan yöntem ve sonuçlarının değerlendirilmesi amaçlanmıştır. Üçüncü basamak bir hastane olan merkezimizde Ocak 2007-Aralık 2024 tarihleri arasında izlenen HIV ile enfekte anneden doğan bebeklerin verileri retrospektif olarak incelenmiştir. Bebeklerin demografik ve perinatal bilgileri, anne sütü alma durumu, uygulanan antiretroviral (ARV) profilaksi, konjenital anomali varlığı, ebeveynlerinin HIV enfeksiyonu, annelerin HIV enfeksiyonuna ve gebelik seyrine ait özellikleri kaydedilmiştir. Çalışmadaki HIV ile enfekte anneden doğan 120 bebeğin %54.2 (n= 65)'si erkek olarak belirlenmiştir. Bu olguların %62.5 (n= 75)'i diğer merkezlerden yönlendirilmiştir. On dokuz (%15.8) olgunun preterm ve 11 olgunun (%9.2) gestasyonel yaşa göre küçük doğduğu belirlenmiştir. Olguların 91 (%75.8)'inin sezaryen ile doğduğu tespit edilmiştir. İki olguda (%1.7) konjenital kardiyak anomali saptanmıştır. Annelerin 70 (%58.3)'i gebelikten önce, 40 (%33.3)'ı gebelik sırasında ve 10 (%8.3)'u doğumda tanı almıştır. Annelerin çoğunluğunun (%59.2) 20-30 yaş aralığında ve %26.7'sinin yabancı uyruklu olduğu belirlenmiştir. Annelerin 70 (%58.3)'ine gebelik öncesinde, 30 (%25)'una gebelik sırasında ARV tedavi başlanmıştır. Annelerin %73.3 (n= 88)'ünün son trimesterde HIV-1 RNA değeri < 20 kopya/mL ve %82.5 (n= 99)'inin CD4 sayısı ≥ 200 hücre/mm3 olarak değerlendirilmiştir. Olguların 82 (%68.3)'si düşük riskli anne bebeği, 38 (%31.7)'i yüksek riskli anne bebeği olarak belirlenmiştir. Yüksek riskli olmasına rağmen 15 olgu ilaç temininde yaşanan problemler nedeniyle sadece zidovudin profilaksisi almıştır. Dış merkezlerden yönlendirilen beş bebeğin geç başvuruları nedeniyle (3, 5, 2, 3, 29. gün) uygun profilaksileri almaları sağlanamamıştır. Doksan dört olguya (%78.3) 4-6 haftadan itibaren kotrimoksazol profilaksisi uygulanmıştır. Olgulardan dördünün (%3.1) izleminde HIV-RNA değerleri pozitif saptanmıştır. Bu dört olguda; annelerinin gebelik takipleri düzenli yapılamamış, doğum sonrası süreçte ARV profilaksileri yüksek riskli anne bebeği olmalarına rağmen uygun olarak başlanamamıştır. Etkili önlemlerle perinatal HIV bulaşmasını azaltmak ve ortadan kaldırmak mümkündür. Bu çalışma, sevk edilen bebeklerin yaşadığı sorunların tespitiyle bu konuda daha fazla eğitim yapılması ve yenidoğana uygulanacak profilaktik ilaçların yenidoğan formülasyonuna uygun temin edilmesinin önemini gözler önüne sermektedir.

Spinal muscular atrophy (SMA) is a disease characterized by progressive nerve cell loss in the spinal cord and brainstem. It can be fatal if left untreated, particularly in infantile forms. Nusinersen became the first disease-modifying agent to be approved in 2016 for the treatment of all types of 5q-associated SMA, across all age groups. Trials have shown that the drug has an acceptable safety profile and has significantly improved clinical care for patients; however, data on long-term risks and benefits are limited. Respiratory problems following use of the drug are an important potential complication and the mechanisms of action are not fully understood. This study reports on a patient with type 1 SMA who developed chronic respiratory failure after the first dose of nusinersen, a condition not previously reported. Although nusinersen significantly improves patients' quality of life, its long-term or rare side effects are not well understood. Patients should be closely monitored for respiratory side effects, especially after the first dose, and families should be thoroughly informed about potential respiratory complications. How to cite this article: Orhan Ö, Talay MN, Büyükşahin HN, Demirbuğa A, Özbek MN, Özgün N. A case of chronic respiratory failure after the first dose of nusinersen treatment. J Med Dent Invest. 2024; 5: e240341.

Introduction: Leukemoid reactions resulting from infections other than leukemia can present with serious clinical conditions. Pertussis infection, particularly before vaccination, can lead to severe pneumonia and may trigger a leukemoid reaction. This case is presented to highlight the relationship between severe pneumonia and leukemoid reaction in pertussis. Case Presentation: A 41-day-old baby girl, who had not yet been vaccinated against pertussis, was hospitalized in the pediatric intensive care unit with a diagnosis of severe pneumonia due to pertussis infection and required mechanical ventilation. A pronounced leukemoid reaction (144,000 cells/μL) was observed. Despite hydration and supportive treatment, a partial exchange was performed due to the elevated white blood cell count. Although there was partial improvement in the white blood cell count, the patient passed away on the 11th day of follow-up. Conclusions: There is a need for further case reports to determine the appropriate timing for initiating partial exchange treatment and the number of cycles required.

Objective Although coronavirus disease 2019 (COVID-19) is mainly a respiratory system disease, many hematological abnormalities have been reported. Due to the application of primary immunization in infancy, our study aimed to examine the relationship between lymphopenia frequency and duration of lymphopenia in infants with COVID-19. Methods The files of infants hospitalized with the diagnosis of COVID-19 in the Pediatric Pandemic Service of Istanbul Medical Faculty between January 2020 and October 2022 were evaluated retrospectively. Demographic characteristics, leukocyte, lymphocyte count, comorbidity, hospitalization, and lymphopenia recovery time were recorded. Results In this study, 93 infants with COVID-19 were included. Lymphopenia was detected in 62 of these patients (n = 62/93, 66.7%). The 47.3% of the patients were female (n = 44) and the mean age was 6 ± 3.42 months. Comorbidities were detected in the 33% of the patients. Lymphopenia resolved in an average of 11 days. While the mean hospitalization period of patients with lymphopenia was 3.6 ± 2.9 (minimum: 1 and maximum: 15) days, the mean hospitalization period of patients without lymphopenia was 2.5 days. Leukopenia (p: 0.014) and lymphopenia (p: 0.005) were more common in infants with chronic disease. Similarly, the duration of hospitalization and recovery from lymphopenia were statistically significantly longer (p: 0.016). A statistically significant correlation was found between the duration of lymphopenia recovery and the duration of hospitalization (p: 0.001). Conclusion Although we found lymphopenia as a common finding in infancy in our study, it was not observed frequently enough to require the postponement of the vaccination program due to its short duration. Vaccination should not be delayed due to lymphopenia in infants with COVID-19 to avoid a missed opportunity for vaccination.

Background: In the first half of 2022, the increase in cases of severe acute hepatitis in children with no epidemiological link and unknown cause has aroused concern worldwide. In this study, we aimed to determine the frequency of adenovirus in children with hepatitis of unknown origin in Turkey. Methods: In this study, which was conducted with the participation of 16 centers from Turkey, between July 2022 and January 2023, 36 pediatric patients under the age of 18 who met the definition of acute hepatitis were included. Results: Twenty (55,6%) of patients were female and 16 (44,4%) were male, with a mean age of 41.55 ± 39.22 (3-192) months. The most common symptoms were fever (66.7%), weakness (66.7%), loss of appetite (66.7%), fatigue (61.1%), vomiting (61.1%), and diarrhea (47.2%). Six (16 %) children had acute hepatic failure. Eight of the 15 children (46%) had respiratory viral infections (rhinovirus/enterovirus, n = 4; rhinovirus + parainfluenza, n = 1; parainfluenza, n = 1, SARS-CoV-2 n = 2). Adenovirus was detected in 16 (44%) patients. Adenovirus and coinfections were detected in 7 patients. History of diarrhea and fatigue in the last month was significantly higher in the adenovirus group (P = 0.023 and 0.018). One child who had both adenovirus and rhinovirus infection required liver transplantation; all others recovered with supportive care. Conclusion: There were no deaths in our series; however, the number of cases, etiology, and severity of the clinical course may have differed between countries owing to differences in case definitions, laboratory facilities, or regional genetic differences.