Case report: Thrombotic microangiopathy in pediatric multisystem inflammatory syndrome associated with COVID-19: a case series.

Introduction: This report provides insight into three distinct pediatric cases exhibiting a nexus between multisystem inflammatory syndrome in children (MIS-C) and thrombotic microangiopathy (TMA) triggered by COVID-19. The aim is to underscore the range of clinical presentations and the essentiality of early interventions. Case presentations: This report presents three cases aged 10 months, 7 years, and 3 years with persistent fever, diarrhea, nausea, and vomiting. The first case, a 10-month-old girl, demonstrated acute kidney injury (AKI) and microangiopathic hemolytic anemia (MAHA) following a COVID-19 infection. Despite initial negative SARS-CoV-2 RT-PCR results, her condition escalated rapidly, presenting increased levels of LDH (peaking at 4,200 U/L) and requiring renal replacement therapy (RRT) to manage deteriorating renal function. Interventions with eculizumab and anakinra led to marked improvements, with a stable follow-up of 13 months post-hospitalization. The second case involved a 7-year-old girl who developed symptoms of MIS-C, hemolytic uremic syndrome (HUS), and posterior reversible encephalopathy syndrome (PRES) post-exposure to COVID-19, evidenced by heightened LDH levels (3,522 U/L at peak). After a precarious period of deteriorating kidney function and exacerbated hypertension, she responded positively to treatments, inclusive of IVIG, steroid therapies, and eculizumab, with a favorable 6-month follow-up showcasing stable laboratory results. The third case discusses a 3-year-old boy, without any medical history, manifesting HUS symptoms and COVID-19 infection. He exhibited increased LDH levels (peaking at 3,946 U/L) alongside elevated creatinine, marking renal impairment. He responded well to hemodialysis, IVIG, and steroid therapy, showcasing substantial recovery by the 19th day of hospitalization, which marked his discharge with a tapering steroid regimen. Conclusion: This case series underscores that MIS-C-associated TMA is a significant complication in pediatric COVID-19. Our findings illuminate the potential for treatment success but simultaneously emphasize the need for a more comprehensive understanding of the underlying pathophysiology.

Real-world Treatment Patterns and Outcomes in Stage III Non-small Cell Lung Cancer: Middle East and Africa - KINDLE Study.

INTRODUCTION: The Middle East and Africa (MEA)-KINDLE, as part of a real-world global study, evaluated treatment patterns and survival outcomes in stage III non-small cell lung cancer (NSCLC) in the MEA. MATERIALS AND METHODS: Retrospective data were analyzed from 33 centers for patients diagnosed between January 01, 2013, and December 31, 2017. Descriptive analyses included clinical-demographics and treatment patterns; inferential statistics evaluated an association of first-line treatment with progression-free survival (PFS) and overall survival (OS). RESULTS: Of 1,046 patients enrolled, the median (range) age was 61.0 years (24.0-89.0); 83.2% were men, 80.8% were current or past smokers, 58.9% had stage IIIA disease, 47.8% had adenocarcinoma and 20.0% of tested (35/175) had epidermal growth factor receptor mutations. Of 86.0% of patients receiving an initial therapy, about 16% underwent surgical resection. Concurrent chemoradiotherapy (cCRT) (32.3%) was the most frequent treatment modality followed by chemotherapy (CT) alone (19.6%) and sequential CRT (12.1%). Median PFS and OS (months, 95% CI) were 11.8 (10.6, 12.4) and 22.9 (21.2, 26.3), respectively for the overall MEA subset. OS (months) was highest with surgery-based therapies for stage IIIA (IIIA: 37.3, IIIB: 24.1) followed by cCRT (IIIA: 28.9, IIIB: 24.4). Female gender, adenocarcinoma, and cCRT or Sx+CRT in first-line were associated with higher OS (P < .05). CONCLUSIONS: The data reveal an unmet need in stage III NSCLC with worse PFS and OS in the MEA subset than in the global cohort. Better access to newer therapies and quality care will be crucial in improving patient outcomes in the MEA.

Effects of location, size and shape of tympanic membrane perforations on hearing: analysis of 400 cases.

OBJECTIVE: This study aimed to evaluate the effect of size, location and shape of tympanic membrane perforations on hearing levels of a large study group treated in a tertiary referral centre. METHOD: Medical data of 458 patients with tympanic membrane perforations were evaluated. RESULTS: A total of 336 patients had normal middle-ear findings during the surgical procedures. There was a significant difference in terms of mean pure tone average and air-bone gap values between posterior-inferior and anterior-inferior perforations (p = 0.005 and p = 0.044, respectively). The mean air-bone gap value of kidney-shaped perforations was significantly higher. Posterior-superior and posterior perforations were significant indicators for ossicular chain defects (p < 0.001; odds ratio, 14.2 and p = 0.004; odds ratio, 3.4, respectively). CONCLUSION: Perforations located in the posterior-inferior quadrant caused the greatest hearing loss. The difference between posterior-inferior and anterior-superior or inferior perforations was statistically significant. Posterior perforations had a significant relationship with ossicular chain pathologies. Kidney-shaped perforations caused higher pure tone average and air-bone gap values than annular, elliptical or pinpoint perforations.

Gender Dsyphoria and Psychiatric Disorders in Children and Adolescents with Congenital Adrenal Hyperplasia.

Context: Gender identity, psychosexual function, psychiatric adjustment and quality of life have been investigated in congenital adrenal hyperplasia(CAH) patients. Objective: We aimed to investigate gender identity problems and the psychiatric disorders and associated factors in children and adolescents with CAH patients. Subjects and methods: Forty-five children and adolescents with CAH were included in the study. Psychiatric comorbidity was assessed using the Schedule for Affective Disorders and Schizophrenia for School Age Children - Present and Lifetime Version. Gender identity problems were investigated using the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition criteria. Results: The mean age of the sample was 11.02 years (SD: 3.25, range: 6-18). 51.1% of the patients had at least one lifetime comorbid psychiatric disorder. The most common diagnoses were anxiety disorders, attention deficit hyperactivity disorder(ADHD), tic disorders and enuresis nocturna. Tic disorders and ADHD were higher in males but they were not statistically significant. Two female patients were diagnosed with gender dysphoria and 18.5% of females showed variably masculinized behaviors. The girls with gender identity problems expressed lower satisfaction with their sex than other girls and boys. Conclusions: Children and adolescents with CAH had many psychiatric disorders, especially neurodevelopmental disorders. ADHD and tic disorders should be kept in mind during assessment especially in male patients. Gender dysphoria and masculine behaviors seem to be common in female patients with CAH so they should be carefully investigated.

Characteristics of Turkish children with Type 2 diabetes at onset: a multicentre, cross-sectional study.

AIMS: To describe the baseline clinical and laboratory findings and treatment modalities of 367 children and adolescents diagnosed with Type 2 diabetes in various paediatric endocrinology centres in Turkey. METHODS: A standard questionnaire regarding clinical and laboratory characteristics at onset was uploaded to an online national database system. Data for 367 children (aged 6-18 years) newly diagnosed with Type 2 diabetes at 37 different paediatric endocrinology centres were analysed. RESULTS: After exclusion of the children with a BMI Z-score < 1 SD, those with genetic syndromes associated with Type 2 diabetes, and those whose C-peptide and/or insulin levels were not available, 227 cases were included in the study. Mean age was 13.8 ± 2.2 (range 6.5-17.8) years, with female preponderance (68%). Family history of Type 2 diabetes was positive in 86% of the children. The mean BMI was 31.3 ± 6.5 kg/m2 (range 18.7-61) and BMI Z-score was 2.4 ± 0.8 (range 1-5). More than half (57%) of the children were identified by an opportunistic diabetes screening due to existing risk markers without typical symptoms of diabetes. Only 13% (n = 29) were treated solely by lifestyle modification, while 40.5% (n = 92) were treated with metformin, 13% (n = 30) were treated with insulin, and 33.5% (n = 76) were treated with a combination of insulin and metformin initially. Mean HbA1C levels of the insulin and combination of insulin and metformin groups were 98 (11.1%) and 102 mmol/mol (11.5%), respectively, and also were significantly higher than the lifestyle modification only and metformin groups mean HbA1C levels (70(8.6%) and 67 mmol/mol (8.3%), respectively). CONCLUSIONS: An opportunistic screening of children who are at high risk of Type 2 diabetes is essential, as our data showed that > 50% of the children were asymptomatic at diagnosis. The other important result of our study was the high rate of exclusion from the initial registration (38%), suggesting that accurate diagnosis of Type 2 diabetes in youth is still problematic, even for paediatric endocrinologists.

Local control and vertebral compression fractures following stereotactic body radiotherapy for spine metastases.

PURPOSE: We aimed to retrospectively assess the incidence of vertebral compression fractures (VCF), examine clinicopathologic factors potentially associated with VCF, and evaluate treatment response in patients who received stereotactic body radiotherapy (SBRT) for spine metastases (spMets). METHODS AND MATERIALS: We identified 78 patients with 125 spMets at baseline and subsequent assessments. Patients received SBRT doses of 16 or 18 Gy. Patients with pre-existing VCF and co-existing local progression were excluded. Spine instability neoplastic score (SINS) was used for spMets categorization. Response to SBRT and VCF were assessed according to the Positron Emission tomography Response Criteria In Solid Tumors (PERCIST) and Genant scores, respectively. Kaplan-Meier analyses were used to assess local control of disease and vertebral compression fracture-free survival (FFS). RESULTS: We treated 103 cases with single spMets and 11 cases involving double spMets with SBRT. Progressive disease was reported in 3.2% and 8.2% of the cases in the first and last PET/CT reports, respectively. The distribution of treatment response in the remaining patients was: complete response in 30.6% of patients, partial response in 47.1% of patients, and stable disease in 22.3% of patients in the first PET/CT; complete response in 62.3% of patients, partial response in 16.7% of patients, and stable disease in 21% of patients at the last monitoring. Local failures were observed in 15 (12%) of cases. Median SINS was 5 (range: 1-13); majority of patients in our cohort (70.4%) were categorized as stable according to SINS, five (4%) patients had Grade 3 VCF at a median time of 16 months after SBRT (range: 2-22 months), and 60% of VCF occurred after an interval of at least 12 months after SBRT. No bisphosphonate usage was significantly associated with VCF (r = -0.204; p = 0.022). Median FFS was 21 months. Univariate analyses indicated that female gender (p < 0.001), bisphosphonate use (p = 0.005), >6 months of bisphosphonates use (p = 0.002), and the lowest vertebral body collapse score (p = 0.023) were associated with higher FFS. Female gender (p = 0.007), >6 months of bisphosphonates usage (p = 0.018), and the lowest vertebral body collapse score (p = 0.044) retained independent significance. CONCLUSIONS: This study demonstrated that spine SBRT with doses of 16-18 Gy promises good local control of disease with acceptable VCF rates. Lowest vertebral body collapse score, female gender, and >6 months of bisphosphonate use were significantly associated with longer FFS.

Comparison of nasal cytology and symptom scores in patients with seasonal allergic rhinitis, before and after treatment.

OBJECTIVE: To evaluate symptom scores and nasal smear cytology findings in seasonal allergic rhinitis patients, before and after treatment. METHODS: Twenty-nine consecutive adult patients with seasonal allergic rhinitis were evaluated prospectively. They received mometasone furoate nasal spray and cetirizine for 21 days. Nasal and ocular symptom scores were recorded before and after treatment. Nasal cytology was also assessed as a means of determining treatment. RESULTS: The combined use of an intranasal corticosteroid and an oral antihistamine caused a significant improvement in nasal and ocular symptom scores. Cytological evaluation revealed significant reduction in nasal eosinophil, neutrophil and goblet cell counts after three weeks' treatment. CONCLUSION: Symptom scoring systems are widely used for the evaluation of drug efficacy in allergic rhinitis treatment. When investigating the disease and evaluating treatment efficacy, objective as well as subjective methods are needed. Nasal cytological assessment is a simple, objective method which provides valuable information about the nasal mucosa.

Late effects of childhood ALL treatment on body mass index and serum leptin levels.

BACKGROUND: It is well known that survivors of acute lymphoblastic leukemia (ALL) show a tendency to become overweight. Cranial irradiation (CRT), is considered to be the primary risk factor for development of obesity. AIMS: The aim of our study was to evaluate body mass index (BMI) and serum leptin levels in survivors of childhood ALL. METHODS: Subjects (Group I) consisted of 93 survivors of childhood ALL (53 males > or = 9 years old, 40 females > or = 8 years old) diagnosed between January 1975 and December 2002 in the Hematology-Oncology Division in Cerrahpasa Medical Faculty, Istanbul University after a follow up 10.21 +/- 4.90 (mean +/- SD) years. Fifty healthy individuals of similar chronological age were taken as controls (29 males, 21 females). Seventy-four subjects had received radiotherapy (Group IA) and 19 had not (Group I B). RESULTS: In Group I, BMI was significantly higher than in Group II (21.65 +/- 4.02 vs 20.31 +/- 3.49, p = 0.04). However, BMI was significantly higher only in Group I A (21.83 +/- 4.27) than in Group II (p = 0.032). Leptin levels were significantly higher in Group I A females than in Group II females. There was a significant correlation between BMI-SDS and serum leptin levels in group IA females. CONCLUSIONS: Leukemia treatment leads to obesity. Higher leptin levels in girls may suggest that sex may be a differentiating factor for this late effect.

Effectiveness of temozolomide treatment used at the same time with radiotherapy and adjuvant temozolomide; concomitant therapy of glioblastoma multiforme: multivariate analysis and other prognostic factors.

AIM: Prognostic factors which affect treatment results of glioblastoma multiforme (GBM; WHO Grade IV) patients has been investigated in many researches. For these patients determination of prognostic factors helps generating multimodal therapy protocols. For this purpose, in the Baskent University Medical School, Neurosurgery Clinic, Adana Medical Research Center, specific characteristics of GBM patients who have surgery retrospectively investigated and factors which affect prognosis has been determined. METHODS: Between January 2005 and January 2009, 59 patients (25 female, 34 male) who have GBM have been evaluated retrospectively. Mean follow-up period was 27.4 (+/-17.3) months. Headache (66.1%) was the most seen symptom. Time of diagnosis was 1-2 months for most of the patients (54.2%). Fifty-nine patients had 67 operations totally. Preoperative Karnofski Performance Scale (KPS) was >or=70 for 43 operations, or=70, for 26 operations KPSor=70 (P=0.0000) , postoperative KPS 2 was >or=70 (P=0.0000), type of tumor resection (P=0.00002), multiple operations (P=0.001), adjuvant RT (P=0.0000) and ConcT with adjuvant TMZ (P=0.0000) were all positive prognostic factors which extend the survival. After multivariate analysis, post operative KPS was >or=70 (P=0.003; OR:0.89; % 95 CI:0.83-0.96), type of resection (P=0.055; OR:0.37; % 95 CI:0.13-0.12) and multiple operations (P=0.042; OR:2.65; % 95 CI:1.03-6.82) were independent prognostic factors. CONCLUSION: When independent prognostic factors were examined ,median survival found out 7.8 months longer fort he patients whose postoperative KPS were >or=70, 5.7 months longer for the patients who had radical resection, 6.6 months longer for the patients who had multiple operations. Although patients who had ConcT with adjuvant TMZ had 1.7 months longer survival compared to patients who had only adjuvant RT, it was not determined as an independent prognostic factor.

[Sensitivity and specificity of fine needle aspiration biopsy in parotid masses]. / Parotis kitlelerinde ince igne aspirasyon biyopsisinin duyarlilik ve özgüllügü

OBJECTIVES: We aimed to determine the sensitivity and specificity of fine needle aspiration biopsy (FNAB) in patients with parotid masses. PATIENTS AND METHODS: The study included 29 patients (15 males, 14 females; mean age 52 years; range 20 to 83 years) who underwent FNAB and parotidectomy for parotid masses. After a detailed history taking, otorhinolaryngologic and systemic examinations were performed. Neck ultrasonography and, when needed, neck computed tomography were used. Superficial parotidectomy was performed in 20 patients and total parotidectomy in nine patients. RESULTS: The results of FNAB were reported as benign in 21 patients (72.4%), malignant in six patients (20.7%), and suspicious in two patients (6.9%). Postoperative histopathologic diagnoses were reported as benign in 17 patients (58.6%) and malignant in 12 patients (41.4%). The sensitivity and specificity rates for FNAB were 54.6% and 100%, respectively. The most common histopathological diagnosis was pleomorphic adenoma (n=7, 24.1%), followed by mucoepidermoid carcinoma (n=4, 13.8%), and Warthin's tumor (n=3, 10.3%). CONCLUSION: Preoperative FNAB for parotid masses plays an important role in planning surgery.