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INTRODUCTION AND OBJECTIVE:Tumor staging and grading do not usually reflect the possibility of Wilms tumor (WT) relapse. Aim: To predict WT recurrence by using P53, Ki67 and cyclin A immunohistoche...

Background In 2014, Hama Governorate was exposed twice to chlorine gas, with 15 patients presenting to Kafr Zita Hospital in Northwest Syria. This study aimed to describe clinical manifestations of chlorine gas exposure to identify factors leading to facility admission and the need for ICU/intubation in conflict-affected areas with limited healthcare infrastructure. Methods We conducted a case-series study, using medical records of suspected chlorine-exposed patients presenting at Kafr Zita Hospital on either 11 April or 22 May 2014. Data on age, sex, initial clinical presentation, therapeutic management, and outcome were compared by hospital admission/non-admission and attack date. All patients provided verbal informed consent. Results Fifteen patients with signs of chlorine gas exposure had detailed medical records. The mean age was 25.7 years (range 2-59), eight were male (53%), and three (20%) were under age 16. At initial presentation, all experienced respiratory distress, due to severe airway inflammation confirmed by nonspecific pulmonary infiltrates on chest x-ray, and similar intestinal, neurological, dermatological, ophthalmological, and psychological signs and symptoms. Acute management consisted of oxygen and bronchodilators for all patients, hydrocortisone (93%), antiemetics (80%), and dexamethasone (13%). Seven (47%) made a rapid symptomatic recovery and were discharged the same day and eight (53%) were admitted for a median of two days (range 1-6 days), one of whom required intubation and later died. The only significant associations found were higher mean pulse rate (i.e. 138 versus 124; p=0.043) and body temperature (37.0 versus 36.5; p=0.019) among admitted patients compared to non-admitted. Conclusion Our results demonstrated that even in low-resource humanitarian settings the survival rate for chlorine gas exposed patients is fair. Despite the small sample, this study provides insight into the clinical presentation, management, and outcomes of weaponized chlorine gas exposure, though further research is required to understand any chronic consequences.

Egypt is located in the arid belt with semi-arid Mediterranean coast. The characteristic maritime oolitic sand dunes stretched parallel to the sea from Alexandria westward to Mersa Matruh. This habitat prevailing highly specialized floristic composition never grows southward. Recently, the oolitic sand dune habitats threatened by urbanization expansion and global climate change, induce a notable decline in floristic composition and species richness. The present study aims at assessing the flora land vegetation of the oolitic sand dunes. The floristic composition in the surveyed area revealed a total of 116 psammophyte species (91 genera belongs to 27 families), annuals comprise 47.4% and the perennials record 40.5. The chronological analysis revealed the dominance of the Mediterranean elements either mono-, bi- or pluri-regional chorotypes that recorded 64.7% of the total number of species. The relationship between the prevailing species composition and soil variables using CCA revealed that CaCO3, organic carbon positively affected and fractions of sand & clay and chloride ions negatively controlling the species distribution. The deterioration of the oolitic habitats as a result of the construction of resorts and urbanization reported a severe decline in the vegetation and flora of the area and this expansion must be stopped.

World Health Organization (WHO) is encouraging reporting of children with Multisystem Inflammatory Syndrome (MIS-C) associated with SARS-CoV-2 infection for better understanding and management of the disease.This retrospective study included the first 15 pediatrics patient with a confirmed diagnosis of MIS-C associated with SARS-CoV-2 infection in the state of Qatar. We studied and analyzed their demographic data, clinical manifestations, laboratory tests, treatment, and outcome.A total of 15 children were studied (mean age 3.5 ± 2.7year). Recent severe acute respiratory syndrome coronavirus 2 infection was identified in all of them (100%). The majority of these patients had 4 or more systems involvement. Nine of the 15 presented with Kawasaki disease - picture and all had gastrointestinal symptoms (vomiting and diarrhea). Five required Pediatrics Intensive Care Unit (PICU) admission. Lab investigations revealed high D-Dimer, hyponatremia, and hypoalbuminemia in all. Low hemoglobin (Hb) , thrombocytopenia, and sterile pyuria occurred in 86.6%, 60% and 75% of them, respectively. Treatment with combined anti-inflammatory medications (intravenous immunoglobulin, corticosteroids) was used in along with immunomodulatory agents (Anakinra) in a selected group of refractory patients. No mortality happened.Our young children who presented with MIS-C related to SARS-CoV-2 infection had significantly higher Kawasaki-disease picture compared to other reports. One third of them required PICU admission but no mortality occurred.

Iron deficiency anemia (IDA) is the most common cause of anemia in both developed and developing countries. Leukopenia is an infrequent side effect of iron therapy reported in the literature as sporadic cases.To assess the prevalence of leukopenia, neutropenia and/or lymphocytopenia and its possible clinical impact if any, after intravenous iron therapy in adult patients with IDA.This is a retrospective study conducted in Hamad Medical Corporation, Doha (Qatar). The clinical and biochemical data of 1.567 females (mean age: 29.5 years) with IDA who attended the Hematology Clinic and were treated with intravenous (i.v.) iron therapy were collected and analysed. Complete and differential blood counts and iron profile were studied before and after i.v. iron therapy. In addition, cases who developed infections during the time of leukopenia were noted and checked for possible complications.30 cases (1.91%) developed leukopenia,15 cases (0.95%) developed neutropenia and 12 cases (0.76%) developed lymphocytopenia. All had normal white blood cell counts before treatment. Two patients (6.66%) had infection. One had upper respiratory tract infection and the other had urinary tract infection, the latter was treated with antibiotics. There was no reported other infection during or after i.v. iron therapy.Leukopenia in form of neutropenia or lymphocytopenia may occur as a side effect of i.v. iron therapy, however, its clinical significance appears to be limited.

Letter to Editor.

Liquid biopsy is a valuable precision oncology tool that is increasingly used as a non-invasive approach to identify biomarkers, detect resistance mutations, monitor disease burden, and identify early recurrence. The Tempus xF liquid biopsy assay is a 105-gene, hybrid-capture, next-generation sequencing (NGS) assay that detects single-nucleotide variants, insertions/deletions, copy number variants, and chromosomal rearrangements. Here, we present extensive validation studies of the xF assay using reference standards, cell lines, and patient samples that establish high sensitivity, specificity, and accuracy in variant detection. The Tempus xF assay is highly concordant with orthogonal methods, including ddPCR, tumor tissue-based NGS assays, and another commercial plasma-based NGS assay. Using matched samples, we developed a dynamic filtering method to account for germline mutations and clonal hematopoiesis, while significantly decreasing the number of false-positive variants reported. Additionally, we calculated accurate circulating tumor fraction estimates (ctFEs) using the Off-Target Tumor Estimation Routine (OTTER) algorithm for targeted-panel sequencing. In a cohort of 1,000 randomly selected cancer patients who underwent xF testing, we found that ctFEs correlated with disease burden and clinical outcomes. These results highlight the potential of serial testing to monitor treatment efficacy and disease course, providing strong support for incorporating liquid biopsy in the management of patients with advanced disease.

Sickle cell disease (SCD) is traditionally associated with growth failure and delayed puberty. Wasting and stunting are still prevalent in children and adolescents with SCD, especially in developing countries. In addition, sperm abnormalities are frequent in males with SCD, with high rates of low sperm density, low sperm counts, poor motility, and increased abnormal morphology. Severe anaemia, vaso-occlusive attacks with ischemic injury to different organs including the pituitary gland and testis, and nutritional factors are incriminated in the pathogenesis of defective growth, puberty, and spermatogenesis. There is great phenotypic variability among patients with SCD. The variability in the clinical severity of SCD can partly be explained by genetic modifiers, including HbF level and co-inheritance of α-thalassaemia. In the past, severe disease led to early mortality. Advancements in treatment have allowed patients with SCD to have a longer and better quality of life. For most patients, the mainstays of treatment are preventive and supportive. For those with severe SCD, three major therapeutic options are currently available: erythrocyte transfusion or exchange, hydroxyurea and hematopoietic stem cell transplantation. In this mini review the authors tried to recognize, delineate, and update knowledge on abnormalities due to SCD from those created by the use of different treatment modalities. (www.actabiomedica.it)

Keloid scars result from excess collagen deposition in the dermis or subcutaneous tissue in response to surgery or tissue trauma. The penis is a rare site for keloid formation, even in predisposed individuals. Only few cases of penile keloids have been reported so far. In this report, we present penile keloids complicating neonatal circumcision in twin brothers. Risk of recurrence in previous reports and measures to guard against its occurrence are also discussed.

Background: Hormonal treatment of hypogonadism in thalassaemia major (TM) is a complex issue due to the co-existence of other contributing factors such as severity of iron overload, associated chronic liver disease and other endocrine complications. Objectives: Data about adverse events (AEs) of testosterone replacement therapy (TRT) in hypogonadal males with TM is scarce. We report the adverse events registered during TRT in 95 young patients with TM. Results: These AEs included gynecomastia, documented in 41/95 (43.1%) TM patients of mild to moderate severity (90%). Persistent pain in the injection site and local reactions to testosterone (T) skin patch occurred in a third of patients. Priapism was reported in 2 patients on treatment with intramuscular T enanthate. In both patients, substitution with T gel was successful, and no recurrence during the following 24 months was observed. Conclusions: Clinicians should exercise caution when considering TRT for hypogonadal men with TM. (www.actabiomedica.it)

Juvenile fibromyalgia syndrome (JFMS) is a chronic condition characterized by symptoms of chronic diffuse musculoskeletal pain and multiple painful tender points on palpation. It is often accompanied by fatigue, disorders of sleep, chronic headaches, irritable bowel syndrome, and subjective soft tissue swelling. The complexity of the presenting clinical picture in JPFS has not been sufficiently defined in the literature. Similarities to adult fibromyalgia syndrome in JFMS are often difficult to compare, because many of the symptoms are “medically unexplained” and often overlap frequently with other medical conditions. However, a valid diagnosis of JFMS often decreases parents’ anxiety, reduces unnecessary further investigations, and provides a rational framework for a management plan. The diagnostic criteria proposed by Yunus and Masi in 1985 to define JFMS were never validated or critically analyzed. In most cases, the clinical diagnosis is based on the history, the physical examination that demonstrates general tenderness (muscle, joints, tendons), the absence of other pathological conditions that could explain pain and fatigue, and the normal basic laboratory tests. Research and clinical observations defined that JFMS may have a chronic course that impacts the functional status and the psychosocial development of children and adolescents. This paper briefly reviews the existing knowledge on JFMS focusing on the diagnosis, clinical and the epidemiological characteristics in children and adolescents for better understanding of this disorder. (www.actabiomedica.it)

IntroductionIron deficiency anemia (IDA) is the most common cause of anemia in both developed and developing countries. Leukopenia is an infrequent side effect of iron therapy reported in the literature as sporadic cases. We conducted a study to clarify this issue and to check its consequences in a big cohort of patients with IDA.ObjectiveTo assess the relationship between iron therapy (intravenous) and leukopenia, neutropenia or lymphocytopenia, and its clinical impact, if any, on patients.Materials and Methodsthis is a retrospective study conducted in Hamad Medical Corporation, Doha/Qatar. the clinical and biochemical data of 1567 females with IDA who attended the hematology clinic and were treated with intravenous iron therapy were collected and analysed. Complete and differential blood counts and iron profile were studied before and after iron treatment. In addition, cases who developed infections during the time of leukopenia were noted and checked for possible complications.ResultsAfter iron therapy, out of 1567 case of iron deficiency anemia, 30 cases (1.914%) developed leukopenia,15 cases (0.957%) developed neutropenia and 12 cases (0.765%) developed lymphocytopenia. All had normal WBC counts before treatment. 2 patients (6.66%) had infection, 1 had upper respiratory tract infection and 1 urinary tract infection, the latter was treated with antibiotics. There were no reported infections during or after iron therapy.ConclusionsLeukopenia in form of neutropenia or lymphocytopenia may occur as a side effect of IV iron therapy, however, its clinical significance appeared to be limited.

To examine the durability of continent cutaneous catheterizable urinary channels (CCCC) in children and assess whether channel complications continue to arise with extended follow-up. Previous studies demonstrated that complications of CCCC cluster in the early years following surgery.The database of a tertiary center was queried for patients≤21 years who underwent CCCC. Patients with6 years of follow-up were excluded. Patients were invited for follow-up to assess continence. Clinic visits and hospital admissions were reviewed for channel complications requiring reoperation. Complications were analyzed against patient and channel characteristics and time since initial surgery.Between 1993 and 2012, a total of 120 patients underwent CCCC at a median age of 6.8(0.4-21) years and a median follow-up of 11.4(6.6-27) years. CCCC were created using the appendix, Monti channels and tapered ileal segments in 74(61.7%), 33(27.5%) and 13(10.8%), respectively. Continence relied on the extra-mural serous lined principle in 85.8% and the stoma was anastomosed to the umbilicus in 90%. Dryness with catheterization intervals of 3 hours or longer was eventually achieved in 90.8% with similar rates among different channel types (P=.149). 26(21.7%) required 42 interventions to treat channel complications with 32.5% occurring5 years following initial surgery irrespective of the channel type (P=.978). On multivariate analysis, ileal channels had 3.372 higher odds of needing reoperation compared to appendicovesicostomy (95%CI=1.240-9.166; P = .037).A high reoperation rate is anticipated throughout the lifetime of CCCC. Appendicovesicostomy has a low complication risk relative to ileal channels.

Purpose. To assess the visual, ocular, extraocular, and multifocal electroretinography (mfERG) outcomes of computer vision syndrome (CVS) among medical students. Methods. This study was designed as a cross-sectional case-control study that included 733 medical students. All students completed a specially designed and validated CVS questionnaire survey (CVS-F3). Students from the control (No-CVS) and CVS groups underwent comprehensive ophthalmic examinations including the mfERG examinations. Our main outcome measures included uncorrected and corrected distance visual acuity (UDVA and CDVA, resp.) measurements, subjective and cycloplegic refractions, slit-lamp examination, intraocular pressure measurement, pupillary reflexes tests, ocular movements’ tests, dry eye disease tests, and fundus and mfERG examinations. Results. The CVS-F3 identified that 87.9% of students had complaints that might be related to CVS. We documented a 76% prevalence rate in students undergoing an ophthalmologic exam. The most common ocular and extraocular complaints included visual blur and headache (40.9% and 46.8%, resp.). Statistical logistic and linear regression analyses showed that refractive errors, prolonged screen-hours, close eye-screen distance, improper gaze angle, poor screen-resolution, and screen-glare were risk factors for developing CVS and influencing its severity. In the mfERG subgroup, 42.5% demonstrated reduced amplitudes of mfERG rings and quadrants, indicating reduced foveal responses. Conclusion. Surveys cannot yield an accurate CVS prevalence. However, they help to identify subjects at risk who should be comprehensively assessed to confirm or exclude CVS diagnosis. Smartphone misuse primarily caused CVS among users. Our mfERG findings might be a sign of potential CVS visual sequelae; however, future studies are warranted. Clinicians need to understand these sequelae to appropriately identify and treat CVS.

Introduction: There is a high prevalence of childhood and adolescent overweight and obesity in Qatar (28 % of females and 24 % of males) with an increasing incidence of type 2 DM in children. We describe the anthropometric and biochemical profile of 100 randomly selected children and prepubertal non-diabetic adolescents with attending the Pediatric clinic of HGH from 2017: 2019. …

Summary Introduction and objectives We aim to assess the long-term oncological outcomes of children with bladder/prostate rhabdomyosarcoma (B/P RMS) treated with multiagent chemotherapy as a monotherapy. We hypothesize that a highly select patient subset can be treated with multiagent chemotherapy as a monotherapy and spared the morbidity of local treatment with similar oncological outcomes. Methods Patients (≤21-year-old) treated for non-metastatic non-alveolar B/P RMS at a tertiary center and followed for>one year, were retrospectively reviewed. After pathological confirmation, patients received 12 weeks of induction VAC chemotherapy (IC) followed by second-look biopsies. Between 1996 and 2006 (group A), patients with>50% tumor size reduction and negative second-look biopsies following IC were spared local treatment and followed-up closely. Between 2007 and 2020 (group B), local treatment was routinely given at 12 weeks according to the COG protocols, irrespective of IC response. For all patients, consolidation chemotherapy was administered for additional 12–18 months. Results Between 1996 and 2020, 27 patients (10 stage II, 17 stage III) with a median age of 3(1–21) years were included. Median follow-up was 87.5(15.3–247.1) months. Among 15 patients in group A, 3 were ineligible for the monotherapy protocol and received local treatment. The remaining 12 patients [9 complete (CR) and 3 incomplete response (IR) to IC] were treated exclusively with chemotherapy, of whom 9 were alive free of relapse at last follow-up. Two patients with IR to IC had disease relapse: one had pulmonary relapse at 8.2 months and one had local relapse at 35 months. The 5-year OS and EFS of group A were 86.7% and 80%, respectively. Analyzing survival according to IC response, CR to IC was achieved in 10 patients (9 group A and one group B) and was associated with significantly better OS and EFS than IR(p = 0.026 and 0.004, respectively) (Summary figure). All patients with CR to IC were alive free of relapse at last follow-up. Discussion Treatment of RMS is traditionally multimodal. Local treatment of B/P RMS is associated with significant patient morbidity. In this study, CR to IC predicted better OS and EFS. Patients who achieved CR (radiological and pathological) to IC remained alive free of relapse irrespective of local treatment. Conclusions Our results suggest that patients with non-metastatic non-alveolar B/P RMS who achieve CR to IC can be treated with combination chemotherapy as a monotherapy and spared the morbidity of local treatment with durable survival outcomes. Prospective validation in a larger patient cohort is needed to support our hypothesis. Download : Download high-res image (321KB) Download : Download full-size image Summary figure .

The purpose of this study was to perform a meta-analysis comparing mobile-bearing with fixed-bearing total knee arthroplasty (TKA) in terms of all-cause revision rates, aspetic loosening, knee functional scores, range of motion and radiographic lucent lines and osteolysis.PubMed, Cochrane Library, Google Scholar and Web of Science were searched up to January 2020. Randomized controlled trials that compared primary mobile-bearing with fixed-bearing TKA, reporting at least one of the outcomes of interest, at a minimum follow-up of 12 months were included. All outcomes of interest were pooled at short-term ( 5 years), mid-term (5 to 9 years) and long-term ( = 10 years) follow-up intervals.A total of 70 eligible articles were included in the qualitative and statistical analyses. There was no difference between mobile-bearing or fixed-bearing TKA at short-term, mid-term and long-term follow-ups in all outcome measures including all-cause revision rate, aseptic loosening, oxford knee score, knee society score, Hospital for Special Surgery score, maximum knee flexion, radiographic lucent lines and radiographic osteolysis.The current level of evidence demonstrated that both mobile-bearing and fixed-bearing designs achieved excellent outcomes, yet it does not prove the theoretical advantages of the mobile-bearing insert over its fixed-bearing counterpart. The use of either design could therefore be supported based on the outcomes assessed in this study.Level II, Therapeutic.

Background The rapid spread of COVID-19 renewed the focus on how health systems across the globe are financed, especially during public health emergencies. Development assistance is an important source of health financing in many low-income countries, yet little is known about how much of this funding was disbursed for COVID-19. We aimed to put development assistance for health for COVID-19 in the context of broader trends in global health financing, and to estimate total health spending from 1995 to 2050 and development assistance for COVID-19 in 2020. Methods We estimated domestic health spending and development assistance for health to generate total health-sector spending estimates for 204 countries and territories. We leveraged data from the WHO Global Health Expenditure Database to produce estimates of domestic health spending. To generate estimates for development assistance for health, we relied on project-level disbursement data from the major international development agencies' online databases and annual financial statements and reports for information on income sources. To adjust our estimates for 2020 to include disbursements related to COVID-19, we extracted project data on commitments and disbursements from a broader set of databases (because not all of the data sources used to estimate the historical series extend to 2020), including the UN Office of Humanitarian Assistance Financial Tracking Service and the International Aid Transparency Initiative. We reported all the historic and future spending estimates in inflation-adjusted 2020 US$, 2020 US$ per capita, purchasing-power parity-adjusted US$ per capita, and as a proportion of gross domestic product. We used various models to generate future health spending to 2050. Findings In 2019, health spending globally reached $8. 8 trillion (95% uncertainty interval [UI] 8.7-8.8) or $1132 (1119-1143) per person. Spending on health varied within and across income groups and geographical regions. Of this total, $40.4 billion (0.5%, 95% UI 0.5-0.5) was development assistance for health provided to low-income and middle-income countries, which made up 24.6% (UI 24.0-25.1) of total spending in low-income countries. We estimate that $54.8 billion in development assistance for health was disbursed in 2020. Of this, $13.7 billion was targeted toward the COVID-19 health response. $12.3 billion was newly committed and $1.4 billion was repurposed from existing health projects. $3.1 billion (22.4%) of the funds focused on country-level coordination and $2.4 billion (17.9%) was for supply chain and logistics. Only $714.4 million (7.7%) of COVID-19 development assistance for health went to Latin America, despite this region reporting 34.3% of total recorded COVID-19 deaths in low-income or middle-income countries in 2020. Spending on health is expected to rise to $1519 (1448-1591) per person in 2050, although spending across countries is expected to remain varied. Interpretation Global health spending is expected to continue to grow, but remain unequally distributed between countries. We estimate that development organisations substantially increased the amount of development assistance for health provided in 2020. Continued efforts are needed to raise sufficient resources to mitigate the pandemic for the most vulnerable, and to help curtail the pandemic for all. Copyright (C) 2021 The Author(s). Published by Elsevier Ltd.

Background: Glucose dysregulation (GD), including prediabetes and diabetes mellitus (DM), is a common complication of transfusion-dependent beta-thalassemia (TDT) patients. The prevalence increases with age and magnitude of iron overload, affecting a significant proportion of patients. According to the international guidelines, the development of GD is frequently asymptomatic. Therefore, an early diagnosis requires an annual oral glucose tolerance test (OGTT) in all TDT patients aged ten years or older. Purpose: This retrospective study aims to evaluate the prevalence of GD in a homogenous population of prepubertal TDT patients and to enhance understanding of the pathogenesis and progression of glucose homeostasis in this group of patients. Methods: A selected group of 28 TDT patients was followed for at least 10.3 years (range: 10.3 - 28.10 years) from prepubertal age (mean 11.0 +/- standard deviation 1.1 years) to adulthood (28.7 +/- 3.7 years). Glucose tolerance and insulin response to OGTT were assessed, and indices of beta-cell function, insulin sensitivity, and insulin secretion were calculated. Results: At baseline, 18 TDT patients had normal glucose tolerance (NGT) and 10 had isolated impaired fasting glycemia (IFG), according to the American Diabetes Association (ADA) criteria. Compared to 18 healthy prepubertal controls (mean +/- SD age: 10.9 +/- 1.1 years), the fasting plasma glucose (FPG), basal insulin level, and Homeostatic Model Assessment for Insulin Resistance (HOMA-IR) index were significantly higher in the group of TDT patients (p= 0.001, 0.01 and 0.012, respectively). At the last observation, 7/18 patients (38.8%) with NGT and 9/10 (90%) with IFG at baseline deteriorated; 3 female patients developed type 2 DM (1 from the NGT group and 2 from the IFG group). Compared to adult controls, TDT patients with NGT had a reduced oral disposition index (DI) (p= 0.006) but no significant difference in HOMA-IR and Matsuda index. Conversely, all insulin indices (HOMA-IR, nil, and DI) but one [insulinogenic index (IGI)] were statistically different in TDT patients with GD compared to controls. Conclusion: This study underlines the concept that the spectrum of glucose tolerance in TDT patients represents a continuum of glucose homeostasis disturbances and that prepubertal patients with IFG are at higher risk of developing a further deterioration of glucose metabolism with time. Moreover, it appears that one-third of adult TDT patients with normal fasting glucose may develop GD in the second-third decade of life. Thus, early intervention could help to prevent an expected further decline of glucose tolerance.