Immunological profile in a pediatric population of patients with spherocytosis. A single-center experience.

Spherocytosis is a hereditary disease caused by the deficiencies of different membrane proteins of red blood cells. Currently, splenectomy is the main therapeutic strategy available, although it is accompanied by an increased risk of sepsis. Several evidences have supported the hypothesis of spleen dysfunction in patients with spherocytosis that haven't yet undergone splenectomy. The aim of this study is to furtherly characterize this aspect, by describing the immune subpopulations in peripheral blood samples obtained from 41 pediatric patients with hereditary spherocytosis by flow cytometry, in order to evaluate changes in the composition of the immune populations compared to 16 healthy donors. Patients were divided in two groups: splenectomized and non-splenectomized. In the splenectomized population, data showed neutrophilic leukocytosis, thrombocytosis, increase in NK and reduction in CD4+ lymphocytes. However, we observed that most of the results obtained in the splenectomized group were found in the non-splenectomized patients as well (increase in neutrophils, in NK, reduction of CD19+, CD4+ lymphocytes and CD4+ and CD8+ naïve cells). The alterations of the immune system may be mainly due to the disease itself, regardless of splenectomy. Therefore, immunological criteria could be included in clinical phenotype assessment in order to better optimize the timing for splenectomy.

Diagnosis of COVID-19 in children guided by lack of fever and exposure to SARS-CoV-2.

BACKGROUND: The objective of this study is to test how certain signs and symptoms related to COVID-19 in children predict the positivity or negativity of the SARS-CoV-2 nasopharyngeal swab in children. METHODS: We review the data of children who were tested for SARS-CoV-2 for a suspected infection. We compared the clinical characteristics of the subjects who tested positive and negative, including the sensibility, positive and negative predictive value of different combination of signs and symptoms. RESULTS: Of all the suspected infected, 2596 tested negative (96.2%) and 103 tested positive (3.8%). The median age was 7.0 and 5.3 years for the positive and negative ones, respectively. The female to male ratio was ~1:1.3. Fever and respiratory symptoms were mostly reported. Most positive children had a prior exposure to SARS-CoV-2-infected subjects (59.2%). A total of 99.3% of patients without fever nor exposure to the virus proved negative to the SARS-CoV-2 test. CONCLUSIONS: Our study suggests that a child without fever or contact with infected subjects is SARS-CoV-2 negative. If this were to be confirmed, many resources would be spared, with improved care of both COVID-19 and not COVID-19-affected children. IMPACT: Key message: lack of fever and exposure to SARS-CoV-2-infected people highly predicts a negative results of the SARS-CoV-2 nasopharyngeal swab in the paediatric population. Added value to the current literature: this is the first article to prove this point. IMPACT: reduction of emergency department accesses of children with suspected SARS-CoV-2 infection; increased outpatient management of children with cough or other common respiratory symptoms of infancy; sparing of many human and material health resources.

Association Between Obesity/Overweight and Functional Gastrointestinal Disorders in Children.

OBJECTIVE: Although emerging data indicate that obese/overweight children are more likely to develop functional gastrointestinal disorders (FGIDs) than normal-weight peers, contrasting results have been reported. The present observational, case-control study aimed at estimating the prevalence of FGIDs in obese/overweight children compared to normal-weight peers. METHODS: Consecutive obese and overweight children aged 4 to 18 years attending the obesity outpatient clinic were enrolled as study cases. Normal-weight children were enrolled as comparison group. All the enrolled patients received a thorough health examination from both a pediatric endocrinologist and gastroenterologist. Moreover, they were asked to fill out the Rome III questionnaire for the diagnosis of FGIDs. Data were analyzed to compare the prevalence of FGIDs between cases and controls. RESULTS: Throughout the study period we enrolled 103 cases and 115 controls. No significant age and sex differences were found between the 2 groups. FGIDs were significantly more prevalent in obese/overweight compared to normal-weight children (47.57% vs 17.39%; P < 0.0001). Increased prevalence was observed for functional constipation (18.44% vs 7.82%; P = 0.025), functional dyspepsia (23.33% vs 6.95%; P = 0.001), and irritable bowel syndrome (10.67% vs 2.60%; P = 0.024), whereas no difference was observed for functional abdominal pain (1.94% vs 2.60%; P = 1.00). CONCLUSIONS: Our data suggest that there is a link between excess body fat and FGIDs in children. This finding may offer a model of patients in which the effects of food and nutritional substances, the gut microbial environment, and psychosocial factors are fitting well with the emerging biopsychosocial conceptual model for FGIDs.

Clitoromegaly in Childhood and Adolescence: Behind One Clinical Sign, a Clinical Sea.

The clitoris is a highly complex organ whose structure has only been clarified in recent years through the use of modern imaging techniques. Clitoromegaly is an abnormal enlargement of this organ. It may be congenital or acquired and is usually due to an excess of androgens in fetal life, infancy, or adolescence. Obvious clitoromegaly in individuals with ambiguous genitalia is easily identifiable, whereas borderline conditions can pass unnoticed. Case reports of clitoromegaly with or without clinical or biochemical hyperandrogenism are quite numerous. In these subjects, a comprehensive physical examination and an accurate personal and family history are needed to investigate the enlargement. We reviewed the literature on the conditions that may be involved in the development of clitoromegaly in childhood and adolescence.

Medical management for neurosurgical related seizures.

INTRODUCTION: Seizures or chronic epilepsy are a relatively common occurrence in a neurosurgical setting. However, seizure treatment after neurosurgery has received less attention compared with other causes and only few data are availaible in the literature on management in neurosurgical patients. Areas covered: This paper reviews the availaible data on the risk of seizures in patients undergoing neurosurgery and discusses the role of antiseizure therapy in the management of the postoperative period. Finally, some controversial issues on this topic are addressed. Expert opinion: Despite the studies so far published on this topic, there are still no guidelines for the clinical practice. International recommendations do not generally support the use of antiseizure drugs in postsurgical patients. Nevertheless, their use still remains wide in the routine practice. Initiation of a treatment should be considered when the risk for prolonged seizures or chronic epilepsy is high and the risk of toxicity is acceptable. First generation antiseizures drugs seem to be quite effective although new drugs are associated with lower adverse effects risk and better tolerability.

Pediatric status epilepticus: improved management with new drug therapies?

INTRODUCTION: Status Epilepticus (SE) is the most common neurological emergency of childhood. It requires prompt administration of appropriately selected anti-seizure medications. Areas covered: Following a distinction between estabilished and emergent drugs, we present pharmacological treatment options and their clinical utility in children, with a short mention on alternatives to drug treatment. We also propose an algorithm for the management of pediatric SE. For this review a Pubmed, Medline and Embase search was performed. Expert opinion: In early SE in children, in the prehospital setting, rectal diazepam or buccal midazolam are efficacious drugs; whereas in the hospital setting, intravenous lorazepam or diazepam are indicated. As regard estabilished stage of SE, in addition to the 'classic' compounds, such as phenytoin and phenobarbital, other drugs such as valproic acid, levetiracetam and lacosamide have been demonstrated efficacious. Treatment recommendations of refractory SE depend on retrospective case series and uncontrolled studies. We reported experiences about the use of midazolam, propofol, ketamine and lidocaine. They could be a valid option, but further prospective studies are necessary. Over the last few decades, important advances in basic mechanisms underlying refractory SE have been achieved, but few data are available regarding management of these stages.