The effect of the COVID-19 pandemic on newborn hyperphenylalaninemia screening program

Phenylketonuria is an inherited metabolic disease that can be treated if detected early and can easily be screened with phenylalanine as a biomarker. Phenylketonuria is included in the National Neonatal Screening Program. We aimed to investigate the possible effects of the pandemic on phenylketonuria screening in our country during coronavirus disease 2019 (COVID-19) era. We retrospectively evaluated patients who were referred to our center due to elevated blood phenylalanine levels detected by national screening program retrospective- and prospectively within one-year as of 11 March 2020, the date when first COVID-19 case was detected in our country. Multivariate analysis showed birth region and phenylalanine levels to be independently associated with the time period until the first admission to hospital, but none of the parameters were significantly associated with the pandemic. There was no evidence that the COVID-19 pandemic affected the screening of newborn phenylketonuria, however since the data was limited to our center and to draw generalized conclusions larger scaled multicentric studies are needed.

Interdisciplinary Approach Is Essential for Sufficient Diagnosis and Therapy in Igg4-Related Disease Involving Multiple Organs

Introduction: IgG4-related disease (IgG4-RD) is a systemic, chronic inflammatory syndrome, with enlargement of involved organs, elevated serum levels of IgG4, dense lymphoplasmocytic infiltrates, rich in IgG4-positive plasma cells, and fibrosis in involved organs. The most frequently involved organs are the pancreato-hepatobiliary tract, salivary and lacrimal glands, the retroperitoneum, kidneys, lungs, and aorta. Often multiple organ systems are involved. As an initial treatment, glucocorticoids are recommended. In patients with relapse along with glucocorticoid dose reduction, various immunosuppressive agents have been reported. Aims & Methods: We reviewed 98 patients (2019-now) who were treated in the special outpatient unit for IgG4-RD at the University Hospital of Essen and identified 10 patients with IgG4-RD involving multiple organ systems. Result(s): The first patient is a 65-year-old male diagnosed with an IgG4- RD involving parotitis, lymphadenitis, sialadenitis with orbitopathy and elevated IgG4 serum level (7400 mg/l). Clinically response to therapy with steroids was documented. The second patient is a 63-year-old man with an IgG4-positive pancreatitis, sialadentis, lymphadenopathy, and elevated IgG4 serum level (3960 mg/l). Immunosuppression with tacrolimus leaded to clinical benefit. As the third patient, we report a 48-year-old man with IgG4-related inflammatory condition in pancreas and kidneys with high IgG4 serum levels. The patient was successfully treated with azathioprine and prednisolone. As the fourth patient, we demonstrate a 34-year-old man with IgG4-related autoimmune hepatitis, lymphadenitis, and pancreatitis. After treatment with tacrolimus in combination with rituximab, a significant decrease of IgG4-level was detected. The fifth patient, a 65-year-old man, was diagnosed with IgG4-related fibro- inflammatory pseudotumors in the liver, esophagitis, and lymphadenopathy combined with high serum levels of IgG4 (12000 mg/l). Clinically response to therapy with steroids and azathioprine was reported. As the sixth patient we demonstrate a 29-year-old male with IgG4-related lymphadenopathy, recurrent myocarditis, and pancreatitis. The patient has symptom-free episodes under low-dose prednisolone. We also found an IgG4-RD with multiple organ involvement in our seventh patient. A 54-year-old man with IgG4-related cholangitis, pancreatitis, prostatitis, and very high serum level of IgG4 (26700 mg/l) were treated with steroids and azathioprine. As our eight case, we present a 23-year-old man with congenital hepatic fibrosis, after living-donor liver transplantation, who developed an IgG4- related disease with high IgG4 serum levels (45300 mg/l) after infection with SARS-CoV-2. Pathologically enlarged lymph nodes were detected. In a biopsy of retroperitoneal lymph nodes, IgG4-positive plasma cells were detectable. Intestinal biopsies have shown numerous positive plasma cells in the IgG4-staining (40 IgG4 positive plasma cells/HPF). Treatment with rituximab is planned. The ninth patient is a 56-year-old woman with lymphadenitis and cholangitis, who clinically responded to a treatment with budesonide. As the last patient we present a 59-year-old man with an IgG4-related aortitis, cardiac fibrosis, cholangitis, hepatitis, exocrine pancreatic insufficiency and Hashimoto's thyroiditis responding very sufficient to rituximab. Conclusion(s): An interdisciplinary approach is essential for a sufficient diagnosis and therapy in IgG4-RD involving multiple organs. This collective is extremely heterogeneous, and treatment is often based on individual concepts.

The Impact of Digital Transformation in Teachers’ Professional Development During The COVID-19 Pandemic

This article presents a study conducted to reveal the positive and negative aspects of professional development programs conducted to teachers in distance education during the novice COVID-19 pandemic and investigates whether the programs contributed to the digital transformation competencies the teachers acquired through these programs. The case study was carried out with a total of 30 teachers, who took part in the study voluntarily. Qualitative and quantitative data collection methods were used in order to ensure the validity, reliability, and consistency of the research. Once the teacher-oriented professional development program was completed, the teachers were subjected to an achievement test and a self-assessment questionnaire. A focus group interview was conducted to collect various views of 18 teachers regarding the program. This study also reveals that teacher-oriented professional development programs can be applied efficiently through online education and have a crucial role in strengthening and enhancing the technical competencies of the teachers involved in distance education. © 2022, ComSIS Consortium. All rights reserved.

Stereotactic Body Radiotherapy for Primary and Metastatic Lung Cancer, Cyberknife-M6 Experience

Introduction: It was aimed to evaluate the efficacy, local control and survival in patients with inoperable primary or metastatic lung cancer who underwent stereotactic body radiotherapy (SBRT) using the Cyberknife-M6 (CK-M6) with lung optimized treatment (LOT) module. Methods: Ethics committee (no: 2018-7/6) and scientific research project (OUAP (T) 2019/1) approval were obtained. 23 lesions of 21 patients were treated between April 2019 to December 2020 at our department. The patients were immobilized in the supine position by wearing a Synchrony vest, with the hands at their sides. A planning 4D-CT was obtained in a free breathing modality. The gross target volumes was created both on the full-inhale and full-exhale phases and internal target volume (ITV) was created. By taking an image of patients on the treatment device, tracking modality was selected according to the visibility of the target. Zero-View tracking was applied in 10 patients, 1-View in 10 patients, 2-View in 1 patients. 3 to 5 mm margin added for planning target volume (PTV) according to tracking method. Median ITV and PTV was 9,38 (2-52,34) and 20,27 (9,25-82,7) cc, respectively. An InCise2 multileaf collimator optimized by the Monte Carlo algoritm was used in all patients. A pair of the orthogonal kV X-ray imaging systems were used for simultaneous target tracking. Median prescribed dose was 48 Gy in 4 fractions (30-54 Gy in 3-6 fractions) administered consecutively or every other day. Prescription isodose covering 95% of PTV was 82,5% (77,4-99,3). Median conformity and homogeneity index was 1,17 (1,02-1,77) and 1,22 (1,09-1,29), respectively. Median BED10 was 100 Gy (53,62-151,2) and median beam on time was 26 minutes (12-42). Results: Patients were evaluated on January 2022. The median follow-up was 21 months (2-33). The median age was 68 (53-80) and 40% of the cases were adenocarcinoma. Two patients diagnosed with radiologically. Median lesion size was 13 mm (9-27). SBRT was applied to 13 primary tumors, 3 lung metastases and 7 lymph nodes. At initial evaluation, complete, partial and stable response was found 30%, 65% and 5%, respectively. During the follow-up, 3 patients locally recurred at a median of 11 months (9-14). The median and one-year local recurrence free survival was 22 months, and 89%. Acute and late grade 1-2 pulmonary complications was seen in 10 patients in a median of 7 months (2-13). While the cause of death in 6 cases was existent cardiac morbidity, covid19 pneumonia, lung infection (2) and progression (2), it was unknown in 1 patient. The median and one-year survival was 23 months and 95%. Conclusions: LOT module of the CK-M6 Xsight lung tracking system allows for the application of fiducial-free motion management strategies. The advantage of our study is that the most appropriate tracking modality can be selected prospectively before treatment. In our study, excellent local control with a median survival of 23 months for primary and metastatic lung cancer. With a median treatment time 26 minutes, noninvasive CK-M6 based SBRT was efficient, safe and comfortable treatment in lung cancer. Keywords: lung cancer, Cyberknife-M6, stereotactic body radiotherapy

Anxiety and Related Factors in Parents About Coronavirus Disease-2019 for Children

Aim: The aim of study was to determine the anxiety and related factors in parents about new Coronavirus disease-2019 (COVID-19). Materials and Methods: The sample of the descriptive study consisted of 494 parents with children aged 0-18. The data were collected during April-May 2020 with tools that were prepared via Google Docs, an online study. The link to the questionnaires was shared via social media. The volunteers were agreed to participate in the survey. Results: In the study, 94.5% of the parents participating are mothers, 69.5% are between the ages of 31-40 and 59.6% have one child. 31.8% of the parents stated that they worked during the COVID-19 pandemic, 90.3% applied social isolation or quarantine. The most frequent feeling that 83.6% of the parents was concern/anxiety, 69.0% used kitchen activities such as cooking and baking cakes as coping mechanisms, 68.4% used games, and painting activities with children. Conclusion: In this study, it was determined that parents’ anxiety levels were mild. It is recommended that social and health initiatives be created to prevent and alleviate the psychosocial effects of the pandemic, and to develop programs that will reduce parents’ anxiety.