Clinical Course Of Children Research Articles

Pulmonary function using impulse oscillometry system and clinical outcomes at age 4 years in children born extremely preterm with or without bronchopulmonary dysplasia

This study aimed to describe the early assessment of lung function and respiratory morbidity in children born extremely preterm with or without bronchopulmonary dysplasia (BPD). MethodsThis was a prospective study including all the children born at gestational age ≤28 weeks who received treatment in the NICU of the Centre Intercommunal de Créteil in France, from January 2006 to March 2012. Lung function, using the impulse oscillometry system, respiratory morbidity and growth were assessed at age 4 years. Lung function and clinical course of children were compared in children with and without BPD. ResultsWe included 136 extremely premature children; 26 (19 %) had BPD. Children with and without BPD did not significantly differ in resistance measurements at 5 Hz (R5) and 20 Hz (R20) and reactance (X5) measurements at age 4 years. A total of 104 (76 %) pre-term children had respiratory resistance R5 above the 95th percentile for the reference population (z-score >1.64), regardless of BPD status. The mean (SD) R5 z-score for all children was 2.1 (±0.7), whereas the mean (SD) R20 was in the normal range (z-score = 1.1 [±0.3]). After treatment with bronchodilators, all children showed no significant change in resistance. The prevalence of asthma symptoms at age 4 years was common and estimated at 30 % regardless of BPD status. ConclusionEarly assessment of lung function by the impulse oscillometry system revealed that most preschool children who were born extremely preterm had abnormal total airway resistance regardless of BPD status. The system is an essential tool for the early assessment of children born prematurely.

Children with sickle cell disease: are they protected from serious COVID-19?

COVID-19, the pandemic that hit the world in 2020, resulted in millions of deaths, with the elderly and adults succumbing to the disease more often than children. However, the presence of underlying morbidities increased the risk of death. Sickle cell disease (SCD) was previously classified as a major risk factor for severe COVID-19 disease. However, presently, there are only a limited number of studies that identify the clinical course of children with SCD and COVID-19. We conducted a retrospective observational study on children with SCD admitted due to COVID-19 at three different institutions in Saudi Arabia between March 2020 and March 2022. We studied the demographic and clinical characteristics of patients admitted to the hospital. Seventy-six patients with SCD had PCR-confirmed SARS-CoV-2 during the study period; 50.0% of our patient population were children (6-12 years old). Gender was evenly distributed, with 53.9% girls and 46.1% boys. Symptoms more commonly related to the COVID-19 infection included fever, cough, malaise, and vomiting. Chest x-ray findings revealed mild and non-specific symptoms only in approximately one-third (28) of the included children. The most common symptoms associated with SCD were vaso-occlusive crisis (47.4%) and abdominal pain (11.8%). The overall general appearance of most of the patients was reassuring. The median length of hospital stay was 4.2 ± 2.7 days. The mean white blood cell count was 11.4 ± 5.2 × 109/L, and the mean hemoglobin level was 8.3 ± 1.5 g/dl. Despite the fact that higher levels of mean D-dimer, lactate dehydrogenase, and ferritin were reported in these patients, the clinical outcome was not affected. All recruited patients received hydroxyurea as maintenance therapy. The outcome of our study was reassuring, with no significant morbidity or mortality observed among the recruited patients. Despite SCD being a chronic disease with known specific complications, there has been a claim that COVID-19 infection adds further risk. The results of this study suggest that the overall outcome of COVID-19 was favorable, with no reported mortality. Further research is needed to understand the factors that contributed to this favorable outcome. In children with SCD, it is still questionable whether hydroxyurea is one of the protective factors against severe COVID-19. Validation through large-scale research is recommended.

Evaluation of children applying to emergency service after motor vehicle trauma

BackgroundTraffic accidents are significant causes of death and serious injury in children. Children's clinical findings are more subtle and there is a risk of faster deterioration. On the other hand, radiation sensitivity due to imaging is also high. These challenging factors highlight the importance of approaching pediatric trauma cases. In our study, we aimed to contribute to current approaches by examining the clinical course and profiles of children involved in traffic accidents. This study aimed to analyze the profiles and clinical courses of children involved in road traffic accidents. MethodsPediatric patients brought to our hospital due injuries from traffic accidents were retrospectively investigated. ResultsDuring the two-year period analyzed in the study, 605 child patients applied due to traffic accidents, accounting for 5.4 % of pediatric trauma cases. The majority of the children were between the ages of 12–17, with most accidents occurring in the summer. In total, 56.9 % were from in-vehicle traffic accidents, 36.7 % were from extra-vehicular traffic accidents, and 6.4 % were from motorcycle accidents. The imaging rate among the patients was 89 %, and 50.6 % had more than one tomography. More than half of the cases had no pathological findings, and only 6 % required hospitalization. Most cases were treated successfully with medical care, while 21.4 % needed interventional procedures, and 10 % received no treatment. The most common injuries were superficial skin lesions (11.1 %) and extremity fractures (10.2 %), with orthopedics being the most consulted department. Fractures were most common in the tibia and were more likely in motorcycle accidents than in-car accidents. ConclusionIn-vehicle accidents were the most frequent type of accident, and children under 4 years old had a higher incidence of in-car accidents than other age groups. Males made up 60 % of the cases, with boys having higher rates of motorcycle and extra-vehicular accidents compared to girls. While most children had no injuries from the accidents, those who did generally recovered well with outpatient monitoring. On the other hand, 89 % of patients underwent imaging at a high rate.

Is there a need for revised guidelines in the treatment of perforated appendicitis in children? A study of risk factors for prolonged hospital stay and postoperative complications

PurposeTo study the need for revised guidelines in the treatment of children with acute perforated appendicitis (APA) by describing the clinical course of children surgically treated for APA in one county in Sweden, with focus on length of hospital stay (LOS) and risk of postoperative complications. The study also aimed to identify variation in practice, comparing a university children's hospital (UCH) to county hospitals (CH). MethodsTwo thousand children, <16 years, who had surgery for acute appendicitis 2014-2018 in four public hospitals (1 UCH and 3 CH), were identified. Patients with perforation of the appendix, n=383(19%) were selected for study and retrospective assessment of patient records. Uni and multivariable logistic regression analyses were done to identify risk factors for prolonged length of stay (PLOS) and complications. ResultsThe median LOS was 5.05 days (0.5-61.9), 6.79 (1.81-61.91) for the UCH (n=186) and 3.65 (0.54-35.65) for CH's (n=197)(p<0.0001). PLOS (=>5 days in hospital) was seen in 147 (79%) at UCH and 53(26.9%) at CH's(p<0.0001). Intra-abdominal abscess within 30 days was identified in 36 (9.4%) and surgical re-intervention was needed in 19 (5%) with no differences between hospitals. The need for readmission within 30 days was higher at CH n=22(11.4%) than at UCH n=4(2.2%), (p=0.0006). Multivariable analyses showed independent predictors of PLOS to be: male gender(OR 2.97 (1.68-5.23)), treatment at UCH(OR 10.24 (6.38-16.44)), CRP >135mg/l(OR per 50 units 1.42 (1.16-1.73)), prehospital delay>2.5 days(OR 1.22 (1.01-1.47)), delayed time to surgery(OR per 10 hours 1.74 (1.26-2.41)) extended surgery time(OR per 2 hours 4.59 (1.43-14.76)) and use of urinary catheter(OR 2.99 (1.42-6.29)). ConclusionGuidelines for treatment of childhood APA, focusing on minimizing antibiotics and facilitating early discharge, would optimize care of the patients but also the economical use of resources. Most children with APA have an uncomplicated course, but factors predicting PLOS have been identified. We found a pronounced variation in practice between the UCH and CH's, without increasing the risk of postoperative complications.

Tenfold Increase: Acute Pediatric Mastoiditis Before, During, and After COVID-19 Restrictions.

Acute mastoiditis (AM) is a relatively rare complication arising from acute otitis media, a common condition among children. The COVID-19 pandemic has significantly impacted AM cases. We noted a surge in pediatric AM cases in Germany after COVID-19 restrictions were lifted in 2022. This study assesses AM incidence and the clinical course in children before, during, and after the pandemic. The study also explores complication rates and microbial changes. Participants: We included children (0-18 yr) diagnosed with AM who underwent mastoidectomy at a tertiary-care university hospital from January 2012 to June 2023.Objectives: We aimed to evaluate AM incidence during pre-COVID, COVID, and post-COVID periods; assess complications; and analyze the microbial spectrum.Data Analysis: Incidence and complication rates were compared between periods, along with the microbial spectrum. Population: 75 children were included (median age, 3.3 yr).Incidence: Significant increases in AM cases occurred in the post-COVID period compared to pre-COVID and COVID periods. No significant difference was observed between pre-COVID and COVID periods.Complications: Complication rates increased notably in the post-COVID period compared to pre-COVID and COVID periods with respect to more sensitive imaging methods being used in the post-COVID period. No significant difference was observed between pre-COVID and COVID periods.Spectrum of Pathogens: No significant differences were found in pathogen distribution between periods. Streptococcus pyogenes and Streptococcus pneumoniae were common throughout. The study highlights a substantial rise in AM cases and complications after COVID-19 restrictions were lifted in Germany. This underscores the importance of monitoring infectious diseases and their complications during health crises. Additionally, the study highlights the importance of contrast-enhanced imaging. Further research is needed to explore the mechanisms behind this trend. The study reveals a significant increase in pediatric AM cases and complications following the COVID-19 pandemic in Germany. Adequate computed tomographic or magnetic resonance imaging, including contrast enhancement, is shown to be a very important parameter beside clinical symptoms in deciding for the right therapy. Thus, surgical treatment became more important. Continuous monitoring and adaptive healthcare strategies during health crises are vital for optimal patient care. Further research is warranted to understand the reasons behind these trends and to inform future pandemic preparedness efforts.

Effect of Colchicine Treatment on Clinical Course in Children with PFAPA Syndrome.

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome (PFAPA) is the most common periodic fever condition in children. There is no consensus on treatment to prevent attacks and reduce their frequency. In this study, we aimed to evaluate the effectiveness of colchicine treatment in PFAPA syndrome. In addition, we described the demographic and clinical features of PFAPA patients. We retrospectively analyzed 58 PFAPA patients who were started on colchicine treatment between January 2017 and January 2022. Demographic data, clinical features, laboratory tests, genetic analysis of MEditerranean FeVer (MEFV) mutations, and autoinflammatory disease activity index (AIDAI) scores of all patients were evaluated. In addition, patients were divided into two groups according to MEFV variants and compared. Attack frequency, duration, and AIDAI scores decreased in all patients after colchicine treatment. Duration of follow-up was 13.53±6.65 months. The median±IQR age at diagnosis was 3.2 (2-5) years. Thirty three (56.9%) patients had heterozygous mutations of MEFV. The most common MEFV variants were M694V (63.6%). There was no significant difference between the two groups in terms of colchicine responses. Colchicine treatment is effective and safe in patients with PFAPA who have frequent attacks. No association was established between the presence of heterozygous mutations of MEFV and colchicine response.

SEVERE COURSE OF PARAGANGLIOMAS IN CHILDREN WITH SUCCINATE DEHYDROGENASE MUTATIONS – A REPORT OF TWO CASES

Objective: Paragangliomas and pheochromocytoma (PPGL) are rare causes of arterial hypertension (AH) in children. This study aimed to present the clinical course in two adolescents with PPGL tumors due to succinate dehydrogenase mutations. Design and method: We analyzed age, gender, clinical manifestations, laboratory findings, including plasma methoxycatecholamines, genetic and imaging findings, pharmacological and surgical treatment, and outcome. Results: Case 1: A 14-year-old boy presented with tinnitus, dizziness, and balance disorders. A computed tomography (CT) revealed a paraganglioma in the area of the left internal jugular vein bulb, causing ear and bone destruction. The boy was diagnosed with AH; his plasma normetanephrine was 343pg/mL (normal range: 18-138), metanephrine and 3-methoxytramine were normal. The patient was started on doxazosin, and the tumor was removed. A genetic study showed a pathogenic variant in the SDHD gene (c.33C&gt;A). PET/CT showed another tumor between the inferior vena cava and diaphragm. A successful tumor resection was performed. Currently, the boy is 17 years old, methoxycatecholamine levels are normal. The patient has AH (primary hypertension) and is treated with rampiril and amlodipine. Case 2: A 17.5-year-old girl was admitted to the hospital due to headaches and AH lasting about six months. Laboratory tests revealed a markedly elevated plasma normetanephrine - 8734.59pg/mL (normal range: 16-142) and a slightly elevated 3-methoxytramine - 25.52pg/mL (normal range &lt; 14). CT showed a large tumor (11x7x7cm) at the level of the diaphragm, modeling the heart, liver, and inferior vena cava. Whole-body PET/CT and MIBG scintigraphy did not detect other tumors. A genetic test showed a pathogenic variant in the SDHB gene (c.181dupT). The girl was treated with doxazosin. Surgical treatment included two transarterial embolizations, followed by surgical removal of the tumor. Currently, the patient is 18 years and 2 months old, has normal methoxycatecholamines, and does not require antihypertensive drugs. The same variant of the SDHB gene was found in the patient's father and older brother. Conclusions: 1.PPGL can have an unusual clinical course in children, and patients may present with a variety of symptoms 2.Pediatric patients with PPGL require genetic tests and individualized, multidisciplinary medical care.

Efficacy and safety of topical corticosteroid treatment under occlusion for severe alopecia areata in children: asingle-centre retrospective analysis.

Alopecia areata (AA) has a poor clinical course in children. There are no reliable therapeutic options for children with severe AA, including alopecia totalis (AT) and alopecia universalis (AU). We evaluated the efficacy and adverse effects of a potent topical corticosteroid (TCS) under occlusion in paediatric patients with severe AA. We reviewed records of 23 patients under the age of 10 years with AT or AU treated with a potent TCS (0.05% clobetasol propionate or 0.3% diflucortolone valerate) for 8 h under occlusion with a plastic film. We used the Severity of Alopecia Tool (SALT) to measure clinical improvement. The primary endpoint was a SALT score of ≤ 20 at 6 months. We analysed the change in cortisol levels to identify the long-term safety of TCS therapy on the hypothalamus-pituitary-adrenal axis. Nineteen of the 23 patients (83%) reached SALT ≤ 20 at 6 months. Six patients relapsed over the 6-month follow-up period. Four patients were suspected of having adrenal insufficiency. However, the cortisol levels of the patients recovered to normal within 1 month of lowering the TCS potency or changing to nonsteroidal treatments. Limitations include the retrospective design and small sample size. This study shows that a potent TCS occlusion may be a safe treatment option in paediatric patients with severe AA. Further long-term studies are required to evaluate the safety and recurrence of TCS occlusion therapy for paediatric AA.

Autoimmune gastritis. Features of diagnosis and clinical course in children

Introduction. Autoimmune atrophic gastritis (AAG) is an immune-mediated disease characterized by inflammation and progressive atrophy of the mucous membrane of the fundus and body of the stomach, while the antrum remains intact for the autoimmune process. The pathogenesis of AAG is determined by the development of atrophic, hyperplastic, and metaplastic changes in the mucous membrane of the stomach body, which is a predictor of the development of neuroendocrine tumors type I. However, among pediatricians and gastroenterologists, there is still no proper alertness regarding the diagnosis of AAG. Clinical examples of complex diagnosis of AAG in children using clinical laboratory, endoscopic, and pathohistological methods are presented. To make a diagnosis, attention should be paid to concomitant autoimmune diseases, characteristic endoscopic and pathomorphological signs, as well as clinical and laboratory changes. The analysis of these changes increases the likelihood of a correct diagnosis of AAG, which is an important aspect of cancer prevention.

Impulse oscillometry assessment of respiratory function in pediatric patients with a history of COVID-19.

While coronavirus disease 2019 (COVID-19) is generally considered to exhibit a less severe clinical course in children than in adults, studies have demonstrated that respiratory symptoms can endure for more than 3 months following infection in at least one-third of pediatric cases. The present study evaluates the respiratory functions of children aged 3-15 years within 3-6 months of their recovery from COVID-19 using impulse oscillometry (IOS) and compares them with the values of healthy children. Included in this prospective cross-sectional study were 63 patients (patient group) aged 3-15 years who contracted COVID-19 between December 2021 and May 2022, as well as 57 healthy children as a control group, matched for age and sex. The demographic, clinical, and laboratory data of the patients were recorded, and respiratory function was assessed based on airway resistance (zR5, zR20, R5-20) and reactance (zX5, zX20, reactance area[AX], resonant frequency [Fres]) using an IOS device. There were no significant differences in the age, weight, height, and body weight z score values of the two groups (p > .05). While the zR5 and R5-20 levels of the patient group were higher (p = .008 and p < .001, respectively) than those of the controls, the zR20, AX, and Fres values did not differ significantly between the groups (p > .05). The parameters indicating the reactance, including zX5 and zX20, were significantly lower in the patient group than in the control group (p = .028 and p < .001, respectively). Total and peripheral airway resistances were found to be elevated in children who had recovered from COVID-19 in the preceding 3-6 months.

P846 Impact of 5-ASA discontinuation in children with ulcerative colitis under biological therapy

Abstract Background Mesalamine (5-ASA) is recommended as a first-line medication for inducing and maintaining remission in mild to moderate ulcerative colitis (UC), but indications regarding its use in children with moderate to severe disease treated with biologics are lacking. We aimed to evaluate whether discontinuing 5-ASA might impact the outcomes of children with UC treated with anti-TNFα. Methods Retrospective, single-center, case-control study of children with UC receiving anti-TNFα therapy between January 2018 and January 2023 and with a minimum follow of 6 months. Children who discontinued 5-ASA within six months from the biological therapy initiation were compared to those who continued mesalamine as maintenance therapy. Every 6 months, during a 2-year follow-up, major outcomes defined as disease flares, IBD-related hospitalization, surgery, need for step-up treatment were recorded. Results Ninety-eight children were included in the final analysis, 51 (52%) maintained 5-ASA and 47 (48%) discontinued 5-ASA. The 2 groups were comparable for all the baseline clinical and demographic characteristics. At 6 months, the cumulative incidence of major outcomes, acute severe colitis and need for step up treatment was significantly higher in children who discontinued 5-ASA (p &amp;lt; 0.05). Throughout the follow-up period, children who discontinued 5-ASA were at significant higher risk of hospitalization (Log Rank p=0.02), need for step up treatment (Log Rank p=0.02) and acute severe colitis (Log Rank p=0.003). Conclusion Our data suggest that 5-ASA discontinuation might have a negative impact on the clinical course of children with UC treated with anti-TNFα in terms of major outcomes. However, conclusive evidence regarding this matter requires prospective randomized trials.

Analysis of the Clinical Course of Children With Initial Negative Polysomnography.

To determine the clinical course of children with initial negative polysomnography (PSG) tests. A retrospective chart review was performed on pediatric patients seen by an otolaryngologist who underwent a PSG between October 2012 and March 2019 for obstructive sleep apnea at a single, academic, tertiary-care center. Data including demographics, follow-up PSG tests, and surgeries were collected. A total of 2018 pediatric patients underwent PSG during the timeframe. About 535/2018 (26.5%) patients were negative for obstructive sleep apnea by PSG and had no prior adenotonsillectomy. About 408/535 (76.3%) did not obtain follow-up testing or surgeries; 69/535 (12.9%) underwent subsequent adenotonsillectomy for worsening symptoms without repeat PSG; and 58/535 (10.8%) obtained 1 or multiple follow-up PSG tests. Of the 58 who obtained repeat PSG, 25 (43.1%) were subsequently positive, with 17 of those 25 (29.3% of 58) undergoing adenotonsillectomy. Taken together, 94/535 (17.6%) of patients with initial negative PSG had worsening sleep disordered breathing. A significant minority of children who initially tested negative for pediatric obstructive sleep apnea met criteria for diagnosis on follow up PSG. Additionally, other children with initial negative PSG underwent adenotonsillectomy for worsening symptoms in lieu of repeat testing. Patients should be educated that snoring in children could persist or worsen over time, even in the setting of a initial negative PSG.

Acute Hematogenous Osteomyelitis of the Pelvis in Children.

Pelvic involvement has been reported in 3%-14% of acute hematogenous osteomyelitis (AHO) cases in children. One guideline suggests need for a longer antibiotic course in pelvic AHO, however, recent data are lacking. We describe the clinical course of children with pelvic AHO and compare it to nonpelvic AHO. A retrospective review of patients with a diagnosis of AHO admitted to Texas Children's Hospital from January 2012 to December 2020 was conducted. Patients 6 months-<19 years old and with ≤14 days of symptoms at admission were eligible. Patients with sickle cell disease or immunocompromised were excluded. Wilcoxon rank-sum test assessed for differences between continuous variables and Fisher exact for categorical variables using STATA 17. We compared 104 cases of pelvic AHO to 314 cases of nonpelvic AHO. Patients had similar microbiology, length of stay and length of antibiotic therapy. Patients with pelvic AHO had pyomyositis identified by magnetic resonance imaging more often (28.8 vs. 9.4%, P < 0.001) and bone abscess less often (22.1 vs. 46.5%, P < 0.001). Rates of chronic complications were comparable between patients with pelvic AHO and nonpelvic AHO (8.4% vs. 15.1%, P = 0.1). Nineteen patients (18.3%) with pelvic AHO received ≤30 antibiotic days without complications, but they had less need for intensive care or bone abscesses than patients treated longer. Pelvic AHO in children may be more frequent than previously reported but is not associated with more complications. Four weeks of therapy may be sufficient in selected patients. Prospective studies to compare outcomes with different lengths of therapy are needed.

COVID-19 frequency and clinical course in children with asthma.

The epidemic of severe acute respiratory syndrome coronavirus 2 infection, known as the coronavirus disease 2019 (COVID-19), has caused a global health concern. Since its emergence, numerous studies have focused on various clinical manifestations and outcomes in different populations. However, studies are ongoing as the consequences and impact of COVID-19 in children with chronic diseases such as asthma are controversial. To fill this research gap by retrospectively evaluating the course, laboratory, and clinical findings of COVID-19 among 414 asthmatic children followed up from the pediatric allergy outpatient clinic and known to have had COVID-19. The data of 5510 patients over the age of 5 diagnosed with asthma in our hospital's data were retrospectively scanned with specific parameters using protocol numbers from the hospital filing system. The data included retrospective evaluation of pulmonary function test results before and after COVID-19, routine hematological and biochemical parameters, sensitization states (total IgE, specific IgE, and skin prick test results), and radiological (computed tomography) findings. To inquire about the course and symptoms of COVID-19, asthma patients or their parents were then called and evaluated with a questionnaire. As a result of retrospectively scanning the data of 5510 asthma patients over the age of 5, it was determined that 414 (7.5%) patients had COVID-19. The mean age of 414 patients was 17.18 ± 4.08 (min: 6; max: 28) years. Two hundred and three of our 414 patients are male, and 211 are female. When their vaccination status was questioned, 21.5% were vaccinated. When the symptoms of our 290 patients were questioned, it was stated that 59.0% had fever symptoms. The rate of using regular prophylactic asthma medications was 19%. The rate of using salbutamol in asthma was found to be 22%. The rate of patients using methylprednisolone was 1%. Emergency service admission was 17.2%, and hospitalization was found to be 4.8%. Leukopenia (< 4000) was found in 14.1% of patients, and 8.08% of our patients had neutropenia (< 1500). Lymphopenia (< 1500) was detected in 44.4% of patients, and lymphocytosis (> 4000) was found in 5.05% of patients. In 65% of our patients, the C-reactive protein value was elevated. A high aspartate aminotransferase and alanine aminotransferase value was detected in 3.2% and 5.4% of patients were found, respectively. 31% of patients had an elevated lactate dehydrogenase value. Typical radiological findings for COVID-19 were detected in 3/309 of patients. According to our study, there is a correlation between the severity of COVID-19 and asthma symptoms and the course of the disease. However, it is worth noting that the retrospective nature of the study and the differences in sample size, age, and demographic characteristics between the two groups do not allow for an optimal comparison. Therefore, further investigation is needed to explore the relationship between COVID-19 and asthma, and it can be suggested that COVID-19 may trigger asthma attacks and asthma may impact the course of COVID-19.

Impact of Early Diagnostic and Therapeutic Interventions and Clinical Course in Children and Adolescents with Multiple Endocrine Neoplasia Types 1 and 2.

Multiple endocrine neoplasia types 1 (MEN1) and 2 (MEN2) are inherited endocrine tumor syndromes caused by mutations in the MEN1 or RET genes. This study aimed to investigate clinical outcomes and molecular characteristics among children with MEN. This study included eight patients from seven unrelated families. Data on clinical course, biochemical findings, and radiologic studies were collected by retrospective chart review. All diagnoses were genetically confirmed by Sanger sequencing of MEN1 in three MEN1 patients and RET in four patients with MEN2A and one patient with MEN2B. Three patients with MEN1 from two families presented with hypoglycemia at a mean age of 11±2.6 years. Four patients with MEN2A were genetically diagnosed at a mean of 3.0±2.2 years of age by family screening; one of them was prenatally diagnosed by chorionic villus sampling. Three patients with MEN2A underwent prophylactic thyroidectomy from 5 to 6 years of age, whereas one patient refused surgery. The patient with MEN2B presented with a tongue neuroma and medullary thyroid carcinoma at 6 years of age. Subsequently, he underwent a subtotal colectomy because of bowel perforation and submucosal ganglioneuromatosis at 18 years of age. This study described the relatively long clinical course of pediatric MEN with a mean follow-up duration of 7.5±3.8 years. Insulinoma was the first manifestation in children with MEN1. Early diagnosis by family screening during the asymptomatic period enabled early intervention. The patient with MEN2B exhibited the most aggressive clinical course.

SARS-CoV-2 variants are associated with different clinical courses in children with MIS-C

BackgroundRecent infection with SARS‑CoV‑2 in children has been associated with multisystem inflammatory syndrome in children (MIS-C). SARS‑CoV‑2 has undergone different mutations. Few publications exist about specific variants and their correlation with the severity of MIS-C.MethodsThis was a single-center, retrospective study including all patients admitted with MIS-C at Rady Children’s Hospital-San Diego between May 2020 and March 2022. Local epidemiologic data, including viral genomic information, were obtained from public records. Demographics, clinical presentation, laboratory values, and outcomes were obtained from electronic medical records.ResultsThe analysis included 104 pediatric patients. Four MIS-C waves were identified. Circulating variants in San Diego during the first wave included clades 20A to C. During the second wave, there were variants from clades 20A to C, 20G, 21C (Epsilon), 20I (Alpha), and 20J (Gamma). The third wave had Delta strains (clades 21A, 21I, and 21J), and the fourth had Omicron variants (clades 21K, 21L, and 22C). MIS-C presented with similar symptoms and laboratory findings across all waves. More patients were admitted to the pediatric intensive care unit (PICU) (74%) and required inotropic support (63%) during the second wave. None of the patients required mechanical circulatory support, and only two required invasive ventilatory support. There was no mortality.ConclusionsThe various strains of SARS-CoV-2 triggered MIS-C with differing severities, with the second wave having a more severe clinical course. Whether the differences in disease severity across variants were due to changes in the virus or other factors remains unknown.

Comparative analysis of the results of multispiral computed tomography using color mapping and magnetic resonance imaging in the diagnosis of acute hematogenous osteomyelitis in children

BACKGROUND: Although acute hematogenous osteomyelitis is considered a fairly well-studied disease, several articles emphasize that the frequency of diagnostic errors remains quite high. The clinical presentation of acute hematogenous osteomyelitis largely depends on its reactivity and localization. The latter has features of the clinical course in children of different age groups. Osteomyelitis can be difficult to detect because of the variability and nonspecificity of symptoms and physical and laboratory parameters. Rapid diagnosis is crucial for successful disease outcomes because untimely treatment increases the number of complications. Therefore, visualization should be aimed at early diagnosis and, ultimately, successful treatment.&#x0D; AIM: This study aimed to evaluate the informative value of magnetic resonance imaging and multispiral computed tomography (MSCT) in the diagnosis of the intramedullary phase of acute hematogenous osteomyelitis as its earliest stage.&#x0D; MATERIALS AND METHODS: Thirty patients suspected with acute hematogenous osteomyelitis underwent magnetic resonance imaging and MSCT using color mapping techniques and X-ray density assessment. At the final stage of the diagnostic algorithm, osteotonometry was performed. The contents of the bone marrow canal were taken for microbiological and bacteriological studies.&#x0D; RESULTS: In the intramedullary phase of acute hematogenous osteomyelitis, magnetic resonance imaging and MSCT revealed signs of bone marrow edema in 96% of the cases. The sensitivity of magnetic resonance imaging was 96%, the same as that of MSCT; however, the specificity was significantly lower than that of MSCT using the color mapping method and X-ray density assessment, which was 67% and 83%, respectively (p 0.05).&#x0D; DISCUSSION: In recent years, the role of computed tomography in the diagnosis of acute hematogenous osteomyelitis has received considerable recognition in pediatric surgical practice, and MSCT with color mapping and X-ray density assessment in the diagnosis of acute hematogenous osteomyelitis in children has been used relatively recently. Simultaneously, many researchers have reported the high informativeness of MSCT in the diagnosis of acute hematogenous osteomyelitis.&#x0D; CONCLUSIONS: The intramedullary phase of acute hematogenous osteomyelitis according to magnetic resonance imaging and MSCT indicates bone marrow edema as its earliest stage. According to the data of the present study, MSCT using color mapping and X-ray density assessment has high specificity and can be used with MRI as the main method for diagnosing the intramedullary phase of acute hematogenous osteomyelitis.

Reporting the clinical spectrum of children with IgAV in a retrospective 24-year cohort: Influences of age and sex on clinical presentation.

Immunoglobulin A vasculitis (IgAV) is one of the most common types of vasculitis in children. The aims of this study were to investigate the clinical characteristics of the disease, and the effects of age and sex on the clinical course in children with IgAV. This was a retrospective study including pediatric patients diagnosed with IgAV who attended follow-ups at the pediatric rheumatology department of a tertiary healthcare institution between January 1997 and December 2020. The patients were grouped and compared according to sex and age at diagnosis (<7 years vs. ≥7 years). The study included 709 children with IgAV, 392 (55.3%) of whom were male. The mean age at diagnosis was 7.9 ± 3.2 years. The most common disease onset season was autumn (31.2%). Upper respiratory infections (27.8%) were the most common predisposing factors. Gastrointestinal system (GIS), joint, and renal involvement were observed in 52.8%, 47.5%, and 17.5% of patients, respectively. Renal involvement, GIS involvement, and disease relapse were significantly more common among those diagnosed after 7 years of age compared to those diagnosed before the age of 7 (p < 0.001, p = 0.033, and p < 0.001, respectively). Scrotal involvement and subcutaneous edema were more common among those diagnosed at younger than 7 years compared to those aged ≥7 years at diagnosis (p < 0.001 and p = 0.016, respectively). GIS involvement was more frequently seen in males compared to females (p = 0.046). It was demonstrated that being ≥7 years of age at diagnosis or being a male were associated with higher likelihood of renal and GIS involvement in children with IgAV.

Prognosis of children and young adults with newly diagnosed rhabdomyosarcoma metastatic to bone marrow treated on Children's Oncology Group studies.

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. Metastatic disease occurs in 16% of all RMS cases and has a poor prognosis. There are limited studies examining the outcomes specific to patients with RMS metastatic to bone marrow despite an incidence of 6% at diagnosis. Our study aims to document the outcomes, prognostic factors, and clinical courses of children presenting with RMS metastatic to bone marrow treated on Children's Oncology Group (COG) cooperative trials. We performed a retrospective analysis of the patients diagnosed with RMS metastatic to bone marrow between 1997 and 2013 enrolled on one of four COG RMS clinical trials of D9802, D9803, ARST0431, and ARST08P1. We identified 179 cases with RMS metastatic to bone marrow. Patients had a median age of 14.8years, 58% were male, predominantly alveolar histology (76%), extremity was the most common primary site (32%), and 87% had metastatic disease to additional sites; 83% (n=149) received radiation as a treatment modality. The 3- and 5-year event-free survival was 9.4% and 8.2%, respectively. The 3- and 5-year overall survival was 26.1% and 12.6%, respectively. Age ≥10years, alveolar histology, FOXO1 fusion presence, unfavorable primary location, higher Oberlin score, and lack of radiation were identified as poor prognostic/predictive characteristics. This study represents the largest analysis of RMS metastatic to bone marrow, defining the poor prognostic outcome for these patients. These patients may be eligible for therapy deintensification or early pursuit of novel treatments/approaches that are desperately needed.

Comparing the Clinical Courses of Children With Human Rhinovirus/Enterovirus to Children With Other Respiratory Viruses in the Outpatient Setting.

While infections caused by rhinoviruses and enteroviruses are common among children, the entirety of their clinical impact remains elusive. We compared the clinical outcomes of children with rhinovirus/enterovirus infections to other common respiratory viruses in outpatient settings. We conducted a retrospective analysis of nasopharyngeal samples singly positive for human rhinovirus/enterovirus (HRV/ENT), influenza A/B (FLU) or respiratory syncytial virus (RSV) from patients ≤17 years submitted for clinical testing via multiplex polymerase chain reaction between 2016 and 2019. We evaluated the following outpatient outcomes: days of respiratory symptoms before testing; visits for respiratory symptoms; receipt of a breathing treatment; receipt of antibiotics and hospital admission. Statistical analyses were conducted controlling for age and comorbid conditions. There were 1355 positive samples included in this analysis (HRV/ENT: n = 743, FLU: n = 303 and RSV: n = 309). Compared to HRV/ENT, children with FLU had 28% fewer days of respiratory symptoms (β: -0.32; 95% confidence interval: -0.46 to -0.18; P < 0.001), fewer visits for respiratory symptoms, and significantly decreased odds of receiving a breathing treatment or antibiotics, and admission to the hospital. Children with RSV had a similar number of days of respiratory symptoms, outpatient visits and odds of hospital admission, but significantly increased odds of receiving a breathing treatment and antibiotics compared to those with HRV/ENT. Clinicians should have a high level of vigilance when managing children with positive respiratory viral testing for HRV/ENT given the potential for clinical outcomes similar to and, in some instances, worse than known highly pathogenic viruses.