Practical guidelines of the EOTTD for pathological and genetic diagnosis of hydatidiform moles.

Hydatidiform moles are rare and thus most pathologists and geneticists have little experience with their diagnosis. It is important to promptly and correctly identify hydatidiform moles given that they are premalignant disorders associated with a risk of persistent gestational trophoblastic disease and gestational trophoblastic neoplasia. Improvement in diagnosis can be achieved with uniformization of diagnostic criteria and establishment of algorithms. To this aim, the Pathology and Genetics Working Party of the European Organisation for Treatment of Trophoblastic Diseases has developed guidelines that describe the pathological criteria and ancillary techniques that can be used in the differential diagnosis of hydatidiform moles. These guidelines are based on the best available evidence in the literature, professional experience and consensus of the experts' group involved in its development.

Symptoms compatible with long COVID in an Italian pediatric cohort of Tourette patients with and without SARS­CoV­2 infection: a short-term follow-up assessment.

BACKGROUND: Tourette Syndrome (TS) is a childhood-onset neurodevelopmental disorder with a worldwide prevalence of about 0.3-1% of the population. During the pandemic caused by SARS-CoV-2 infection, the impact on the mental health of children and adolescents was very important. The persistence of symptoms in the post-acute phase of the disease has been termed Long COVID. The neuropsychiatric symptoms seem to be the most common impairment in children and adolescents with long COVID. OBJECTIVES: Considering the impact of pandemic on mental health, in this study we analyzed the long-term effects of SARS-CoV-2 infection in children and adolescents affected by TS. METHODS: We conducted an online questionnaire covering socio-demographic and clinical data among 158 patients affected by TS or chronic tic disorders (CTD), of which 78 participants reported a positive SARS-CoV-2 infection. Data were collected to investigate tic severity and both the comorbidities, as well as lockdown-related changes to daily life activities and, in case of infection of SARS-CoV-2, possible symptoms of acute infection and long COVID. Markers of systemic inflammation including C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), ferritin, iron, electrolytes, white blood cell counts, platelet cell counts levels, markers of liver, kidney and thyroid function were analyzed. First, all patients were screened with the Schedule for affective disorders and Schizophrenia for School age children-present and lifetime (Kiddie-SADS-PL) to rule out primary psychiatric disorders considered as criteria of exclusion. Then, all patients were clinically assessed at baseline (T0), and after three months (T1) through the administration of Yale Global Tic Severity Rating Scale (YGTSS), Multidimensional Anxiety Scale for Children (MASC), Child Depression Inventory (CDI) and Child Behavior Checklist (CBCL). RESULTS: Among the cohort of TS patients that contracted SARS-CoV-2 infection, 84.6% (n = 66) experienced any acute symptoms, and long COVID symptoms occurred in 38.5% (n = 30). A worsening of clinical symptoms of tics and eventually associated comorbidities occurred in 34.6% (n = 27) of TS patients that contracted SARS-CoV-2 infection. TS patients with or without SARS-CoV-2 infection showed an increase in the severity of tics and also behavioral, depressive and anxious symptoms. Instead, this increase was more evident in patients who contracted the infection than in patients who did not contract it. CONCLUSIONS: SARS-CoV-2 infection may have a role in the increase of tics and associated comorbidities in TS patients. Despite of these preliminary results, further investigations are necessary to improve knowledge about the acute and long-term impact of SARS-CoV-2 in TS patients.

Congenital anomalies of the tubular gastrointestinal tract.

Congenital anomalies of the tubular gastrointestinal tract are an important cause of morbidity not only in infants, but also in children and adults.The gastrointestinal (GI) tract, composed of all three primitive germ layers, develops early during embryogenesis. Two major steps in its development are the formation of the gut tube (giving rise to the foregut, the midgut and the hindgut), and the formation of individual organs with specialized cell types.Formation of an intact and functioning GI tract is under strict control from various molecular pathways. Disruption of any of these crucial mechanisms involved in the cell-fate decision along the dorsoventral, anteroposterior, left-right and radial axes, can lead to numerous congenital anomalies, most of which occur and present in infancy. However, they may run undetected during childhood.Therapy is surgical, which in some cases must be performed urgently, and prognosis depends on early diagnosis and suitable treatment.A precise pathologic macroscopic or microscopic diagnosis is important, not only for the immediate treatment and management of affected individuals, but also for future counselling of the affected individual and their family. This is even more true in cases of multiple anomalies or syndromic patterns.We discuss some of the more frequent or clinically important congenital anomalies of the tubular GI, including atresia's, duplications, intestinal malrotation, Meckel's diverticulum and Hirschsprung's Disease.

Antitissue transglutaminase antibodies' normalization after starting a gluten-free diet in a large population of celiac children-a real-life experience.

INTRODUCTION: Few data are available regarding the trend of IgA anti-transglutaminase antibodies (TGA-IgA) in children with celiac disease (CD) on a gluten-free diet (GFD). Our aim is to examine the normalization time of CD serology in a large pediatric population, and its predictors. MATERIAL AND METHODS: We retrospectively evaluated the normalization time of TGA-IgA and its predictive factors (age, sex, ethnicity, symptoms, associated diabetes/thyroiditis, Marsh stage, TGA-IgA and endomysial antibody levels at diagnosis, diet adherence), in 1024 children diagnosed from 2000 to 2019 in three pediatric Italian centers, on a GFD. RESULTS: TGA-IgA remission was reached in 67,3%, 80,7%, 89,8% and 94,9% after 12, 18, 24 and 36 months from starting a GFD, respectively (median time = 9 months). TGA-IgA >10´upper limit of normal at diagnosis (HR = 0.56), age 7-12 years old (HR = 0.83), poor compliance to diet (HR = 0.69), female sex (HR = 0.82), non-Caucasian ethnicity (HR = 0.75), and comorbidities (HR = 0.72) were independent factors significantly associated with longer time to normalization. CONCLUSIONS: Our population is the largest in the literature, with the majority of patients normalizing CD serology within 24 months from starting a GFD. We suggest a special attention to patients with comorbidities, language barriers or age 7-12 years for a proper management and follow-up.

Healthcare Professionals and In-Flight Medical Emergencies: Resources, Responsibilities, Goals, and Legalities as a Good Samaritan.

Common in-flight emergencies include syncope, respiratory symptoms, nausea/vomiting, cardiac symptoms, and seizures. Flight conditions, such as changes in air pressure and humidity, can exacerbate existing chronic medical conditions. In 2017, US airlines carried 849.3 million passengers. Undoubtedly, there were many requests for in-flight medical assistance. Whenever a medical event occurs, it is standard procedure that an announcement be made by a flight attendant, requesting medical personnel to identify themselves. The 1998 Aviation Medical Assistance Act provides liability protection for a healthcare professional (HCP) acting as a good Samaritan. Nevertheless, HCPs may initially experience trepidation providing care in an aircraft. They may be unaware that a first aid kit, a emergency medical kit, and an automatic external defibrillator are on every plane. Flight crews have been trained in cardiopulmonary resuscitation, and a support system, including a ground-based consultation service, is available to provide radio assistance from an on-call physician. When multiple HCPs volunteer, the most experienced should assume leadership of care. After evaluating the ill passenger, the HCP communicates the assessment to the crew and, when necessary, to the ground-based physician. The goal of in-flight care is to medically stabilize the ill passenger and facilitate the individual's arrival at the scheduled destination for continued medical care. When unable to stabilize the passenger's condition, the decision to divert the plane rests with the flight's captain. Our article helps HCPs to best understand their resources, structured support, liability, and role during an in-flight medical event. With this knowledge of resources, a good Samaritan can confidently attend to an ill airline passenger in flight.

Responses of blood mononucleated cells and clinical outcome of non-celiac gluten sensitive pediatric patients to various cereal sources: a pilot study.

Non-celiac gluten sensitivity (NCGS) is a clinical entity recently documented by the scientific community in pediatric patients. Nevertheless, its triggering mechanisms remain largely unsettled. We studied 11 children with NCGS who were diagnosed based on a clear-cut relationship between wheat consumption and development of symptoms, after excluding celiac disease (CD) and wheat allergy, matched with 18 children with active CD. Sixteen pediatric patients were also enrolled as controls. Cultured peripheral blood mononucleated cells (PBMCs) obtained from NCGS, CD and control patients were cultured in the presence of wheat proteins extracted from ancient and modern cultivars. Results demonstrated that wheat proteins induced an overactivation of the proinflammatory chemokine CXCL10 in PBMC from NCGS pediatric patients and that this overexpression also depended on the wheat cultivar from which proteins were extracted. Proteins from modern wheat cultivar activated CXCL10 to a greater extent than those extracted from ancient wheat genotypes.

Intradural extramedullary Ewing's sarcoma. Recurrence with acute clinical presentation and literature review.

The intradural extramedullary space is an extremely unusual site for the onset of Ewing's sarcoma. We describe a case of recurrence of intradural extramedullary Ewing's sarcoma and review the literature available on this topic.

The incidence of gestational trophoblastic disease in Italy: a multicenter survey.

OBJECTIVE: To estimate the incidence of gestational trophoblastic disease (GTD) in Italy and identify problems in data retrieval. STUDY DESIGN: The number of all GTD cases recorded from January 2005 to December 2009 was retrieved from pathology department databases of 39 hospitals located in different regions of Italy. The number of total pregnancies registered in each hospital was requested. Regional and National Institute for Statistics Registries on demographics and pregnancies data were used to reach the best estimate of the population at risk for GTD. RESULTS: The raw number of all GTD cases recorded was 1,297 (951 partial moles, 329 complete moles, 17 choriocarcinomas) over 5 years. The 15-49-year-old female resident population living in the hospitals' referral areas was of 2,570,313 women (18.3% of the total). CONCLUSION: The rate of complete hydatidiform mole was 0.5/1,000 pregnancies and 0.8/1,000 deliveries. The rate of choriocarcinoma was 0.013/1,000 pregnancies and 0.030/1,000 deliveries. The rate of GTD was 2.3/10,000 women aged 15-49 years. A frequent cause of incompleteness or lack of GTD cases in data retrieval was due to encoding. Problems in identification of the population at risk originated often from incomplete or incomparable data on number of pregnancies among different Institutions.