Syndrome In Pediatric Patients Research Articles

Prevalence of dry eye syndrome in a South African diabetic paediatric population

BackgroundDiabetes mellitus (DM) can be defined as a metabolic disorder that results from either the inability of insulin to perform its normal functions or decreased insulin secretion. Dry eye disease is a complex ailment of the tear film resulting in discomfort characterised by pain, a feeling of heavy eyes, grittiness, burning, dryness, itchiness, foreign body sensation, and visual disturbances. Patients with chronic conditions, i.e. DM, are more susceptible to developing dry eye due to various complications that may arise, such as, peripheral neuropathy, inflammatory alterations and reduced lacrimal gland function. AimTo identify the prevalence of dry eye syndrome in paediatric patients diagnosed with Diabetes Mellitus (Type 1/2). Method37 children with diabetes and 40 healthy, age group matched controls were enrolled in this study. Participants underwent a complete dry eye assessment in the following order: Ocular Surface Disease Index (OSDI) and McMonnies questionnaire, meibomian gland evaluation using white light, Tear Break-up Test (TBUT), Phenol Red Thread (PRT) test and Tear film Osmolarity test. Duration of diabetes was used as the parameter. ResultsThe incidence of dry eye syndrome was found to be higher among diabetics, with 15 (40.5 %) from the diabetic population compared to 4 (10.0 %) from the control group. TBUT and PRT test values were significantly reduced in children with diabetes (p < 0.001). TFO values were higher in the diabetics. No statistically significant differences were found the questionnaire scores, of the two groups, however the diabetic group appeared to be less symptomatic. ConclusionDry eye is more common in the diabetic paediatric population. TBUT and PRT results were found to be lower in diabetics. Results showed a strong correlation between duration of diabetes and TBUT values. Healthcare practitioners, working with children diagnosed with diabetes, should raise awareness as well as encourage early dry eye screening and intervention. Clinical significanceDue to the steady increase in the number of children and adolescents with diabetes, the characteristics and prevalence of dry eye syndrome in this population has become more valuable.

Retrospective analysis of veno-occlusive disease/sinusoidal obstruction syndrome in paediatric patients undergoing hematopoietic cell transplantation -a multicentre study

BackgroundSinusoidal obstructive syndrome is a potentially fatal complication following hematopoietic cell transplantation, high-intensity chemotherapies and increasingly seen with calicheamicin based leukaemia therapies. Paediatric specific European Society for Blood and Marrow Transplantation (pEBMT) diagnostic criteria have demonstrated benefit in single centre studies compared to historic criteria. Yet, the extent to which they have been universally implemented remains unclear. MethodsWe conducted a retrospective multi-centre study to examine the potential impact of the Baltimore, modified Seattle and pEBMT criteria on the incidence, severity, and outcomes of sinusoidal obstructive syndrome among paediatric hematopoietic cell transplantation patients. FindingsThe incidence of sinusoidal obstructive syndrome in this cohort (n=488) was higher by pEBMT (21·5%) versus historic modified Seattle (15·6%) and Baltimore (7·0%) criteria (p<0·001). Application of pEBMT criteria identified 44 patients who were not previously diagnosed with sinusoidal obstructive syndrome. Overall, 70·5% of all patients diagnosed with sinusoidal obstructive syndrome ultimately developed severe/very severe disease and almost half of diagnosed patients required critical care support. Overall survival was significantly lower in patients who were diagnosed with sinusoidal obstructive syndrome versus those who were not. InterpretationTaken together, pEBMT criteria may be a sensitive method for prompter diagnosis of patients who subsequently develop severe/very severe sinusoidal obstructive syndrome. To our knowledge, this is the first multi-centre study in the United States (US) to demonstrate that pEBMT guidelines are associated with earlier detection of sinusoidal obstructive syndrome. Since early initiation of definitive treatment for sinusoidal obstructive syndrome has been associated with improved survival in paediatric patients and implementation of pEBMT criteria appears feasible in the US, universal adoption should facilitate prompter diagnosis and lead to improved outcomes of children with sinusoidal obstructive syndrome.Funding: None

Review of Opsoclonus-Myoclonus Ataxia Syndrome in Pediatric Patients.

Opsoclonus-myoclonus ataxia syndrome (OMAS), also known as Kinsbourne syndrome, is a rare disorder that presents with myoclonus, ataxia, abnormal eye movements, irritability, and sleep disruptions, often in young children. We report a case of an infant barely 6 months old, with no significant past medical history, who presented to the emergency department with tremors, jerking motions of the head and arms, and rapid eye movements. After an extensive workup, she was found to have a neuroblastoma, which was subsequently surgically removed via thoracotomy. Despite an initial improvement in symptoms post-resection, the patient's symptoms recurred. She was subsequently treated with dexamethasone, intravenous immunoglobulin (IVIG), and rituximab. After treatment, the patient was noted to have mild global developmental delays but was otherwise well. This case report highlights the rare occurrence of OMAS in an infant barely 6 months old at diagnosis. Using the PubMed database, a systematic review was conducted to highlight the clinical presentation, diagnosis, and management of OMAS.

Mimics of Guillain–Barré Syndrome in Pediatric Patients Admitted to the Neuro-Intensive Care Unit of a Tertiary Care Hospital—A Case Series

Guillain–Barré syndrome is the most common cause of acute flaccid paralysis in children, but several diseases mimic GBS. We aimed to identify and report the clinical pointers and battery of tests required to differentiate Guillain–Barré syndrome from its observed mimics in the pediatric population admitted to our neuro-critical care unit. We conducted a retrospective record analysis of all pediatric patients admitted over ten years from 2008–2018, whose initial presentation was compatible with a clinical diagnosis of GBS. Eighty-three patients were at first treated as GBS, of which seven (8.4%) were found to have an alternate diagnosis—three cases of paralytic rabies, one case each of acute disseminated encephalomyelitis, cervical myeloradiculopathy, neuromyelitis optica, and a case of community-acquired Staphylococcus aureus pneumonia associated sepsis. Neurophysiological and neuro-virological testing, central nervous system imaging, and sepsis screening helped to confirm the alternate diagnosis. Our case series provides knowledge of subtle clinical differences along with the mindful use of diagnostic testing to facilitate the accurate diagnosis of GBS mimics.

Zinner syndrome in pediatric patients: rare disease leading to challenging management.

Zinner syndrome (ZS) is the association of seminal vesicle cysts, ipsilateral ejaculatory duct obstruction, and ipsilateral renal agenesis. This condition is very rare in children and both diagnosis and treatment may be challenging. We reviewed the clinical presentation and treatment describing our experience with a series of three patients. From January 2016 to January 2021, three patients (patients 1, 2, and 3) with symptomatic ZS, aged 2, 15, and 17 years, respectively, were diagnosed and treated. All three patients were symptomatic, manifesting pelvic pain and dysuria. The diagnosis was made by physical examination, ultrasonography, and abdominopelvic MRI. Patient 1 underwent open surgery, while for patients 2 and 3, laparoscopic excision was performed. The renal agenesis regarded the left side in patients 1 and 3, and the right side in patient 2. In all cases, the cystic complex was excised. The mean operating time was 4 h and the mean hospitalization time was 5 days (range 4-6 days). The mean follow-up period was 5 years (range 2-5 years). Patients 1 and 3 showed a complete resolution of the symptoms during postoperative follow-up. In patient 2, clinical symptoms relapsed because of the persistence of a 9 mm cyst requiring a redo laparoscopic excision. Seminal vesicle cyst with ipsilateral renal agenesis, even if rare in pediatric age, should be suspected in young male patients presenting with pelvic cystic masses, pelvic pain, dysuria, and ipsilateral renal absence. Conservative management should be reversed to asymptomatic patients. Surgical treatment is mandatory in symptomatic cases and the preferred approach is minimally invasive surgery to magnify the operating field to spare anatomical structures, primarily the contralateral vas deferens. Radicality is crucial to avoid the persistence of symptoms and the need for reintervention.

A chimeric influenza virus vaccine expressing fusion protein epitopes induces protection from human metapneumovirus challenge in mice.

Human metapneumovirus (HMPV) is a common virus associated with acute respiratory distress syndrome in pediatric patients. There are no HMPV vaccines or therapeutics that have been approved for prevention or treatment. In this study, we constructed a novel recombinant influenza virus carrying partial HMPV fusion protein (HMPV-F), termed rFLU-HMPV/F-NS, utilizing reverse genetics, which contained (HMPV-F) in the background of NS segments of influenza virus A/PuertoRico/8/34(PR8). The morphological characteristics of rFLU-HMPV/F-NS were consistent with the wild-type flu virus. Additionally, immunofluorescence results showed that fusion proteins in the chimeric rFLU-HMPV/F-NS could work well, and the virus could be stably passaged in SPF chicken embryos. Furthermore, intranasal immunization with rFLU-HMPV/F-NS in BALB/c mice induced robust humoral, mucosal and Th1-type dominant cellular immune responses in vivo. More importantly, we discovered that rFLU-HMPV/F-NS afforded significant protective efficacy against the wild-type HMPV and influenza virus challenge, with significantly attenuated pathological changes and reduced viral titers in the lung tissues of immunized mice. Collectively, these findings demonstrated that chimeric recombinant rFLU-HMPV/F-NS as a promising HMPV candidate vaccine has potentials for the development of HMPV vaccine.

Acute Compartment Syndrome in Pediatric Patients on Extracorporeal Membrane Oxygenation Support.

When acute compartment syndrome (ACS) occurs in pediatric patients requiring venoarterial extracorporeal membrane oxygen (VA ECMO) support, there is little data to guide surgeons on appropriate management. The purpose of this study is to characterize the presentation, diagnosis, timeline, and outcomes of patients who developed this complication. This is a single-center retrospective case series of children below 19 years old on VA ECMO support who subsequently developed extremity ACS between January 2016 and December 2022. Outcomes included fasciotomy findings, amputation, mortality, and documented function at the last follow-up. Of 343 patients on VA ECMO support, 18 (5.2%) were diagnosed with ACS a median 29 hours after starting ECMO. Initial cannulation sites included 8 femoral, 6 neck, and 4 central. Femoral artery cannulation was associated with an increased risk of ACS [odds ratio=6.0 (CI: 2.2 to 15), P <0.0001]. In the hospital, the mortality rate was 56% (10/18). Fourteen (78%) patients received fasciotomies a median of 1.2 hours after ACS diagnosis. Only 4 (29%) patients had all healthy muscles at initial fasciotomy, while 9 (64%) had poor muscular findings in at least 1 compartment. Patients with worse findings at fasciotomy had a significantly longer duration between ischemia onset and ACS diagnosis. Patients required a median of 1.5 additional procedures after fasciotomy, and only 1 (7%) developed a surgical site infection. Of the 7 surviving fasciotomy patients, 2 required amputations, 3 developed an equinus contracture, 1 developed foot drop, and 3 had no ACS-related deficits. Four patients did not receive fasciotomies: 3 were deemed too ill and later died, and 1 was diagnosed too late to benefit. The only surviving nonfasciotomy patient required bilateral amputations. Pediatric ECMO-associated ACS is not exclusive to patients with femoral artery cannulation. The majority of fasciotomy patients were diagnosed with ACS after muscle necrosis had already started. We were unable to definitively conclude whether fasciotomies provide better outcomes. There is a need for increased awareness and earlier recognition of this rare yet potentially devastating complication. Level IV-retrospective case series.

Analysis of Laboratory Parameters before the Occurrence of Hepatic Sinusoidal Obstruction Syndrome in Pediatric Patients after Hematopoietic Stem Cell Transplantation

Introduction: Hepatic sinusoidal obstruction syndrome (SOS), also known as veno-occlusive disease of the liver, is a serious complication following hematopoietic stem cell transplantation (HSCT). Early diagnosis and treatment with defibrotide can improve patient outcome. The pathogenesis is not fully understood, but it is obvious that some laboratory parameters are already changed before the onset of SOS. The aim of our retrospective study was to detect laboratory parameters following HSCT that can predict the occurrence of SOS. Methods: We analyzed 182 children with a median age of 7.2 years who underwent allogeneic or autologous HSCT for the first time and without defibrotide prophylaxis. Our study population consisted of 126 patients who received allogeneic HSCT (69.2%) and 56 patients who underwent autologous HSCT (30.8%). The stem cell sources were peripheral blood stem cells (50.5%) or bone marrow (49.5%) and the most frequent underlying diseases were solid tumors (34.1%), genetic diseases (19.2%), acute lymphoblastic leukemia (18.1%), and acute myeloid leukemia (13.7%). The diagnosis of SOS was based on the pediatric criteria of European Society for Blood and Marrow Transplantation. We investigated the following 15 laboratory parameters after HSCT before the onset of SOS: international normalized ratio (INR), activated partial thromboplastin time (aPTT), reptilase time, factor VIII, factor XIII, von Willebrand factor (vWF), von Willebrand factor activity (vWF activity), fibrinogen, antithrombin III, protein C, protein S, D-dimer, alanine aminotransferase (ALT), bilirubin, and ferritin. The last laboratory values within one week before the onset of SOS were analyzed in the SOS patient group. In the non-SOS patient group, we investigated the parameters for four weeks after transplantation and calculated the median. We selected cut-off values for the continuous parameters based on receiver operating characteristic curves analyses and existing reference values. We performed Mann-Whitney U test, univariate and multivariate analyses to find significant ( P &amp;lt; .05) associations between the values of laboratory parameters and the occurrence of SOS. The probability of developing SOS was calculated using the significant independent variables. Results: The overall incidence of SOS was 14.8% at a median time of 13 days after HSCT. SOS developed in 24 of 126 allogeneic (19.1%) and in 3 of 56 autologous (5.4%) HSCT patients. Only 3 out of 27 patients (11.1%) who suffered from very severe SOS died within 100 days after HSCT. In the univariate analysis, we found significant associations between the onset of SOS and the following parameters: INR ≥ 1.3, aPTT ≥ 40 sec, reptilase time ≥ 18.3 sec, factor VIII ≤ 80%, antithrombin III ≤ 75%, protein C ≤ 48%, D-dimer ≥ 315 µg/L, bilirubin ≥ 9 µmol/L, and ferritin ≥ 3100 µg/L. In multivariate analysis, we confirmed INR ≥ 1.3 [odds ratio (OR) = 8.104, P = .006], aPTT ≥ 40 sec (OR = 10.174, P = .001), protein C ≤ 48% (OR = 5.215, P = .014), and ferritin ≥ 3100 µg/L (OR = 7.472, P = .004) as independent risk factors after HSCT before SOS. If three of the four significant cut-off values were present, the probability of developing SOS was more than 70%. The probability of SOS was 96%, if all four laboratory parameters were changed according to the cut-off values. The values of factor XIII, vWF, vWF activity, protein S, fibrinogen, and ALT were not relevant for the occurrence of SOS. Conclusions: In summary, the laboratory parameters INR, aPTT, protein C, and ferritin were very useful to predict the occurrence of SOS. These four parameters can enable earlier diagnosis and treatment leading to an improved outcome. In addition, this is the first report on a significant association between SOS and high values of INR and aPTT after HSCT before SOS.

A single-center study of clinical features of pediatric Sjögren’s syndrome

ObjectiveSjögren’s syndrome (SS) is a rare disease with unclear diagnostic criteria among the children and adolescents. The purpose of this study is to describe the clinical features of pediatric Sjögren’s syndrome and validate with Japanese diagnostic guidelines criteria of 2018.MethodsWe conducted a retrospective analysis of the clinical data of a cohort of 54 patients with pediatric Sjögren’s syndrome admitted to our hospital over a total of 10 years from September 2013 to September 2022.ResultsThe ratio of females to males was 49:5 among the 54 children (34 cases of primary SS and 20 cases of secondary SS), the average age of onset of symptoms for the first time was 9.9 years, and the average age at diagnosis was 10.2 years. In terms of subjective symptoms, 7 cases (13.0%) presented with dry mouth and 5 cases (9.3%) reported dry eyes. The positive rates were 9.3% for Schirmer I test, 70.4% for salivary gland function test, and 55.6% for salivary gland ultrasonography. The positive rates were 94.4% for Anti-Ro/SSA antibodies, 66.7% for Anti-La/SSB antibodies, 88.9% for ANA, 59.3% for RF, and the elevation rate of IgG was 63.0%. Among the EULAR Sjögren’s syndrome disease activity index (ESSDAI) domains, the biological, constitutional, glandular, cutaneous, and lymphadenopathy domains were most involved. Treatment consisted of glucocorticoids in 88.9% of the patients in our study and hydroxychloroquine in 92.6%. As per the Japanese version of the clinical practice guidance for Sjögren’s Syndrome in pediatric patients (2018), 5 cases were identified as Definite SS, 35 cases as Probable SS, and 14 cases as Possible SS. With respect to primary and secondary SS, there was essentially no significant difference between the groups in any of the above aspects.ConclusionsPatients with pediatric SS presented with a wide spectrum of clinical features, a low prevalence of reported symptoms of dry mouth and dry eyes, and various clinical manifestations with multi-system involvement. These are similar to other pediatric study cohorts in terms of epidemiology, auxiliary investigation results, disease activity scores, and treatment. The coincidence between our study and the Japanese version of the clinical practice guidance for Sjögren’s Syndrome in pediatric patients (2018) is good for the diagnosis of pediatric SS.

Cognitive disengagement syndrome in pediatric patients with long COVID: associations with mood, anxiety, and functional impairment

ABSTRACT Children with long COVID often report symptoms that overlap with cognitive disengagement syndrome (CDS, previously sluggish cognitive tempo (SCT)), a set of behaviors distinct from attention-deficit/hyperactivity disorder (ADHD) including excessive daydreaming, mental fogginess, and slowed behavior and thinking. Those with long COVID also frequently report low mood and anxiety, which are linked to CDS. The relationships between cognitive difficulties, mood, and functional impairment have yet to be explored in pediatric long COVID. Specifically, it is unclear how much cognitive difficulties (CDS, inattention) contribute to functional impairment, when accounting for mood/anxiety symptoms in this population. Retrospective parent-reported data was collected from 34 patients with long COVID (22 females, Mage = 14.06 years, SD = 2.85, range 7–19) referred for neuropsychological consultation through a multidisciplinary Post-COVID-19 clinic. Compared to community and clinically referred samples, on average, long COVID patients showed elevated CDS symptoms, including Sluggish/sleepy (e.g., fatigue) and Low Initiation subscales (e.g., difficulty performing goal directed behaviors). Low Initiation, mood, anxiety, and inattention were associated with functional impairment. In multiple hierarchical regression models, after controlling for mood and anxiety, Low Initiation and inattention were no longer predictive of functional impairment. Instead, anxiety remained the sole predictor of functional impairment. Our results demonstrate that children with long COVID have high levels of CDS symptoms. The association between cognitive difficulties and functional impairment dissipated with the inclusion of mood and anxiety, suggesting behavioral health interventions targeting anxiety may help improve daily functioning and quality of life in pediatric long COVID patients.

Prevalence of Insulin Resistance and Metabolic Syndrome in Pediatric Patients with Type 1 Diabetes Mellitus and Its Association with Macrovascular Complications

Background Insulin resistance (IR) is an independent risk factor for the development of micro and macrovascular diseases in both type 2 diabetes (T2DM) and type 1 diabetes (T1DM) patients. Objective To assess the frequency of insulin resistance and metabolic syndrome among pediatric patients with T1DM and highlight the factors associated with both. Subjects and Methods Analytical cross-sectional study, including eighty (80) patients with T1DM divided into two equal groups according to body mass index(BMI) (Group1= 40 patients with normal BMI, Group 2= 40 obese or overweight patients). Both groups were subjected to: Detailed history, through clinical examination with special emphasis on assessment of Insulin Resistance and Metabolic Syndrome. Carotid intima media thickness (CMIT) was assessed as a marker of macrovascular complication. Results In group 2, (93%) of participants showed manifestations of metabolic syndrome Also, the same group showed significantly higher HbA1c (P &amp;lt; 0.05) and waist/ hip ratio (P &amp;lt; 0.05). Estimated glucose disposal rate formula (eGDR) (5.49 ± 3.53) were significantly lower in group 2 compared with group 1 (10.51 ± 2.20) (P &amp;lt; 0.05).The best cut-off value of eGDR for prediction of IR among patients with T1DM was &amp;lt; 7.15 mg/kg/min. CIMT was significantly higher in group 2 (0.93 ± 0.13) compared to group 1 (0.66 ± 0.09) (P &amp;lt; 0.05). Furthermore, there was a strong negative correlation between eGDR and CIMT. Conclusion Overweight and obese youth with T1DM had significantly higher prevalence of components of metabolic syndrome including insulin resistance. A specific novel finding of this study is the relationship between eGDR and macrovascular complications.

Editorial Comment on Frequency of tethered cord syndrome in pediatric patients with refractory daytime urinary incontinence.

Editorial Comment on Frequency of tethered cord syndrome in pediatric patients with refractory daytime urinary incontinence.

Histopathological Subtypes of Primary Nephrotic Syndrome in Paediatric Patients at the University Teaching Hospital in Lusaka, Zambia

Background: Minimal change disease (MCD) has been shown to be a common histological presentation in children presenting with nephrotic syndrome (NS) in developed countries. In Africa, information on clinical and histological characteristics of paediatric NS is scarce. This study assessed the characteristics and histological subtypes of NS.&#x0D; Methods: This was a prospective study that consecutively indexed children aged 2-16 years with the diagnosis of NS who were referred to the largest tertiary teaching hospital, Lusaka, Zambia. Thirteen children presenting with NS were biopsied under ultrasound guidance after ethical approval. The primary outcome of the study was to describe the histological characteristics of paediatric NS.&#x0D; Results: Thirteen children of African descent with a median age of onset of NS of 9.25 years (2.0-15.0) were enrolled in the study. The histopathologic lesions were as follows; four had MCD, four had focal segmental glomerulosclerosis (FSGS), one had immune complex mediated glomerulonephritis (ICMN) and four were inconclusive. Haematuria was present in eight out of 13 patients (61.5%) and hypertension was present in five of 13 patients (38.0%). Three children did not have either haematuria or hypertension. Ten of the 13 participants had primary steroid resistance. &#x0D; Conclusion: This study has demonstrated the need to perform a pre-treatment renal biopsy in paediatric patients presenting with NS in view of the atypical presentation, variable histopathologic findings, and unpredictable response to steroid therapy.

Incidence of Air Leak Syndrome in Pediatric Patients With SARS-COV-2 Pneumonia and Respiratory Failure: A Single-Center Retrospective Study.

Air leak syndrome (ALS) is defined as the extrusion of air from an aerated compartment into an unaerated compartment with associated symptoms of respiratory distress. This syndrome can occur as a consequence of trauma, iatrogenic causes, or spontaneously. Retrospective investigations conducted in the adult population have demonstrated an elevated risk of spontaneous ALS development in patients with coronavirus disease 2019 (COVID-19) pneumonia, along with its correlation with mortality. However, no studies have yet explored this phenomenon within the pediatric population. In light of this knowledge gap, we conducted a retrospective chart review comprising 128 pediatric patients ranging in age from one month to 18 years. The primary objective was to assess the incidence of ALS in two distinct groups: patients diagnosed with COVID-19 pneumonia and those with non-COVID-19 viral pneumonia. The groups were compared using Fisher's exact test for sex, the presence of ALS, the requirement of extracorporeal membrane oxygenation (ECMO), and death. The modified Wald method was used to calculate the 95% confidence interval for the mortality rate in patients with COVID-19 pneumonia in the presence of ALS. Our findings revealed a higher prevalence of ALS in patients with COVID-19 pneumonia compared to the non-COVID-19 viral pneumonia group, with a statistically significant P-value of 0.02 and an odds ratio (OR) of 6.72. In terms of mortality rates, there was a statistically significant difference between the two groups (P = 0.025, OR = 1.083). In addition, in patients with ALS in the presence of COVID-19 pneumonia, the mortality rate was 37.5%. However, the requirement of ECMO was not statistically significant (P = 0.16, OR = 1.04). These results suggest that patients with COVID-19 pneumonia have an increased mortality rate and a heightened risk of developing ALS compared to individuals with other viral pneumonias. Furthermore, the presence of ALS was associated with a high mortality rate in COVID-19 pneumonia patients. However, it is crucial to note that obtaining a larger patient sample and involving multiple institutions would be necessary to obtain more consistent and robust data.

Emapalumab for the treatment of refractory cytokine release syndrome in pediatric patients

Emapalumab for the treatment of refractory cytokine release syndrome in pediatric patients

Pediatric Hodgkin's lymphoma presenting with nephrotic syndrome: a case report

Hodgkin's lymphoma (HL) is a common type of lymphoma in children and adolescents, and it typically presents with lymphadenopathy, fever, and weight loss. Nephrotic syndrome is a rare but reported complication of HL, and it is associated with a poor prognosis. We report a case of a 12-year-old boy who presented with nephrotic syndrome as the initial manifestation of HL. The diagnosis was confirmed by a biopsy of the lymph node, which showed the characteristic Reed-Sternberg cells. The patient was treated with chemotherapy and he achieved complete remission of both HL and nephrotic syndrome. This report aims to raise awareness of the potential association between HL and nephrotic syndrome in pediatric patients, which can aid in early detection and prompt management.

Frequency of tethered cord syndrome in pediatric patients with refractory daytime incontinence.

Frequency of tethered cord syndrome in pediatric patients with refractory daytime incontinence.

An Overview of Short-Bowel Syndrome in Pediatric Patients: Focus on Clinical Management and Prevention of Complications.

Short-bowel syndrome (SBS) in pediatric age is defined as a malabsorptive state, resulting from congenital malformations, significant small intestine surgical resection or disease-associated loss of absorption. SBS is the leading cause of intestinal failure in children and the underlying cause in 50% of patients on home parental nutrition. It is a life-altering and life-threatening disease due to the inability of the residual intestinal function to maintain nutritional homeostasis of protein, fluid, electrolyte or micronutrient without parenteral or enteral supplementation. The use of parenteral nutrition (PN) has improved medical care in SBS, decreasing mortality and improving the overall prognosis. However, the long-term use of PN is associated with the incidence of many complications, including liver disease and catheter-associated malfunction and bloodstream infections (CRBSIs). This manuscript is a narrative review of the current available evidence on the management of SBS in the pediatric population, focusing on prognostic factors and outcome. The literature review showed that in recent years, the standardization of management has demonstrated to improve the quality of life in these complex patients. Moreover, the development of knowledge in clinical practice has led to a reduction in mortality and morbidity. Diagnostic and therapeutic decisions should be made by a multidisciplinary team that includes neonatologists, pediatric surgeons, gastroenterologists, pediatricians, nutritionists and nurses. A significant improvement in prognosis can occur through the careful monitoring of nutritional status, avoiding dependence on PN and favoring an early introduction of enteral nutrition, and through the prevention, diagnosis and aggressive treatment of CRSBIs and SIBO. Multicenter initiatives, such as research consortium or data registries, are mandatory in order to personalize the management of these patients, improve their quality of life and reduce the cost of care.

Predictive Value of Complete Blood Count, Venous Blood Gas Measurements, and Glucose/Potassium Ratio for Delayed Neuropsychiatric Syndrome in Children with Acute Carbon Monoxide Poisoning Due to Coal-Burning Stove.

Delayed neuropsychiatric syndrome may occur after carbon monoxide poisoning has completely healed. The literature on indicators to predict delayed neuropsychiatric syndrome in pediatric patients is limited. The aim of the study is to investigate the effectiveness of complete blood count parameters, neutrophil/lymphocyte ratio, platelet/lymphocyte ratio, systemic immune inflammation index, glucose/potassium ratio, venous blood gas parameters, and carboxyhemoglobin in predicting delayed neuropsychiatric syndrome in children with carbon monoxide poisoning due to coal-burning stove. The patients admitted to the pediatric emergency department with acute carbon monoxide poisoning between 2014 and 2019 were analyzed. The patients were divided into 2 groups as delayed neuropsychiatric syndrome (+) and delayed neuropsychiatric syndrome (-). Neutrophil/lymphocyte ratio, platelet/lymphocyte ratio, systemic immune inflammation index (platelet count×neutrophil count/lymphocyte count), and glucose/ potassium ratio were calculated. Of the 137 patients, 46 were diagnosed with delayed neuropsychiatric syndrome within 1 year of carbon monoxide poisoning. A control group was formed from 137 age- and sexmatched children. Glasgow Coma Scale < 15 was found in 11% of patients with delayed neuropsychiatric syndrome (-) and 8.7% of patients with delayed neuropsychiatric syndrome (+) (P = .773). Blood glucose, potassium, glucose/potassium ratio, platelet/lymphocyte ratio, white blood cell, neutrophil count, lymphocyte count, neutrophil/lymphocyte ratio, systemic immune inflammation index, venous partial pressure of carbon dioxide, carboxyhemoglobin, and methemoglobinemia levels were significantly different between control, delayed neuropsychiatric syndrome (+), and delayed neuropsychiatric syndrome (-) groups (P < .05). The most effective predictors for delayed neuropsychiatric syndrome were systemic immune inflammation index (area under the curve = 0.852; cut-off value > 1120; sensitivity = 89.1%; specificity = 75.8%), neutrophil (area under the curve = 0.841; cut-off value > 8000/mm3; sensitivity = 78.2%; specificity = 79.1%), and neutrophil/lymphocyte ratio (area under the curve = 0.828; cut-off value > 4; sensitivity = 78.2%; specificity = 75.5%). About one-third of children with carbon monoxide poisoning due to coal-burning stove develop delayed neuropsychiatric syndrome. Systemic immune inflammation index, neutrophil count, and neutrophil/lymphocyte ratio obtained immediately after the poisoning in the pediatric emergency department may be effective predictors for delayed neuropsychiatric syndrome.

An interesting manifestation of COVID-19 in children: case report and review on multisystem inflammatory syndrome in pediatric patients

Multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 or PIMS-TS (pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 infection) has been presenting itself in children in connection to the SARS-CoV-2 infection. Along with a brief review of global data on MIS-C, we present two case reports of critically ill pediatric patients (aged 16 and 14 years), presenting with features of MIS-C from May 2020 to March 2021, to a tertiary-care hospital in Telangana, India. The patients with MIS-C were healthy prior to testing positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. Clinical presentations were similar, with fever, abdominal pain, gastrointestinal complaints, or maculopapular rash. The diagnostic parameters revealed elevated levels of C-reactive protein, D-dimer, and NtProBNP. Other infectious aetiologies were ruled out. Both patients required ionotropic support, and intravenous immunoglobulin (IVIG), and were also given empiric antibiotics. Both were hemodynamically stable upon discharge. As knowledge of the various COVID-19 manifestations in children increases, reporting is essential to better-educating healthcare professionals in clarifying and streamlining the variety of symptoms connected to MIS-C and assessing the associated risk factors that predispose the pediatric population to develop severe disease.