Journal of Comprehensive Pediatrics
Volume:15 Issue: 2, May 2024

 It has been shown that hyperlipidemia occurs in 71% of patients following liver transplantation. Multiple risk factors, including obesity, diabetes mellitus, and diet, as well as the immunosuppressive medications used, influence the lipid profiles that are seen in these individuals, contributing to the multifactorial etiology of lipid problems.

 The aim of the present study is to compare the lipid profile in liver transplant recipients from living-related (LR) and deceased donors (DDs).

 This is a retrospective cross-sectional study performed at Shiraz University of Medical Science between 2005 and 2018. Patients under 18 years old who received liver transplants were included in the study and divided into 2 groups who received from LR and DDs, and lipid profiles were compared between the 2 groups.

 A total of 397 patients were included in the study; in the first group, 234 received a liver from a DD, and in the second group, 161 from an LR donor. The mean body mass index (BMI) was 17.51 ± 5.49 in the first group and 16.25 ± 3.29 in the second group. The most common underlying diseases were biliary atresia (22%) and autoimmune hepatitis (15%). The mean triglyceride (TG) and high-density lipoprotein (HDL) levels were 133 and 46 mg/dL in the first group and 118 and 54 mg/dL in the second group, while the differences were statistically significant. As age increased, there was a significant difference in the mean values of fasting blood sugar (FBS) and HDL, with FBS increasing and HDL decreasing. There was no significant difference in the use of immunosuppressant drugs between the 2 groups.

 Patients who received a liver from an LR donor have a significantly lower TG, higher HDL, and a lower cardiovascular risk.

 Migraine and functional constipation are prevalent chronic conditions among children, with many children suffering from migraines also experiencing functional constipation.

 This study aims to investigate the impact of constipation treatment on headaches in children with migraines.

 This clinical trial involved 32 children aged 4 to 15 years, all diagnosed with both migraine and functional constipation. They were randomly divided into two groups: an intervention group and a control group, each comprising an equal number of participants. In the intervention group, both migraine and constipation were treated concurrently, whereas in the control group, only migraine was addressed. The outcomes of the two groups were then monitored and compared.

 After the treatment, the intervention group experienced a significant reduction in the average number of monthly headache attacks (3 ± 2.4) compared to the control group (7.1 ± 6.9) with a P-value of 0.016. The average duration of each headache attack was shorter in the intervention group (2.3 ± 4.8 hours) compared to the control group (2.9 ± 4.2 hours), although this difference was not statistically significant. Parental satisfaction with the treatment was higher in the intervention group than in the control group, but this difference also lacked statistical significance. Constipation treatment was successful in all children in the study.

 The study's findings indicate that treating constipation in children with both migraine and functional constipation significantly reduces the frequency of headache attacks. However, it does not have a significant impact on the duration of headache attacks or on parental satisfaction with the treatment.

 Familial Mediterranean fever (FMF) is an autosomal recessive disorder caused by a mutation in the MEFV gene and characterized by recurrent episodes of fever and polyserositis.

 This study aimed to identify the frequency and distribution of MEFV mutations in children with FMF in northeastern Iran and determine clinical examinations.

 Our study was a descriptive and analytical cross-sectional study conducted among 29 patients under the age of 18 who visited the pediatric rheumatologist between April 2014 and 2021. After clinical diagnosis, the patients underwent genetic evaluation. The mutations related to each patient were identified using Sanger sequencing of the entire MEFV gene sequence. The rest of the information was extracted from the checklist. Finally, the data were analyzed using SPSS v. 16.

 Fever was the most common symptom, followed by abdominal pain. During the acute attack period, laboratory inflammatory factors increased in all patients. None of the patients had complications. There was no significant relationship between the demographic variables in the groups (benign, variant of uncertain significance [VUS], pathogen) except for the rate of hospitalization (P-value = 0.039). Moreover, 34.5% of the studied patients had pathogenic mutations. The most common mutation was E148Q, which was reported to be benign, followed by pathogenic mutations (M680I), with a frequency of 10.2%.

 The FMF is rare in Northeastern Iran, and the number of pathogenic mutations is lower compared to Northwest Iran and other studies. It is necessary to conduct a genetic examination and treatment of affected patients to control the course of the disease and its complications.

 The most frequent cause of coronary artery aneurysm in children is Kawasaki disease (KD). Recently, limited studies on procalcitonin (PCT) were performed to find a biomarker for the diagnosis or prognosis of children with KD.

 This study aimed to compare serum PCT levels between complete and incomplete KD and testify to the predictive validity of PCT for intravenous immunoglobulin (IVIG) resistance and predicting coronary artery lesions (CALs).

 This cross-sectional study was conducted at Namazi hospital in Shiraz, Iran, in 2019. All KD patients admitted to the hospital were included, with parental consent obtained. Kawasaki disease patients were categorized as complete KD (cKD) or incomplete KD (iKD). Two-dimensional echocardiography was performed, and peripheral venous blood was examined for PCT levels and other markers. All patients received IVIG and aspirin as standard treatment. The presence of coronary artery abnormalities (CAAs) was determined based on coronary artery size and morphology. We used Stata software version 14.0 for data analysis. Logistic regression models identified predictive factors for coronary complications. This study determined the optimal PCT cut-off point for predicted coronary complications using receiver operating characteristic (ROC) curve analysis. Approval was obtained from the Research Ethics Committees of Shiraz University of Medical Sciences.

 Procalcitonin values in 38 children hospitalized with acute KD ranged from 0.2 ng/mL to 10 ng/mL, with a mean of 2.65 ng/m. There was no correlation of PCT with patients’ age or gender, incomplete KD, or IVIG resistance. The serum PCT concentration was higher in patients with CAL (P = 0.009). The best PCT cut-off value for CAL prediction was 2.5 ng/mL (corresponding sensitivity = 81.8% and specificity = 68.7%), considering CAL prevalence as the studied group.

 Supplementary research is needed to determine the sensitivity and specificity of PCT for the diagnosis and prognosis of KD. Procalcitonin might be of value in predicting which children are at increased risk for CALs to intensify therapy.

Context:

 Bardet-Biedl syndrome is a rare genetic disorder with variable prevalence rates across populations, characterized by symptoms such as retinal degeneration and intellectual disability. In this study, researchers investigated renal cystic epithelia from patients with PKD1 mutations. This study identified the upregulation of genes related to the Jak-STAT pathway and NF-κB signaling in these renal cells. These pathways appear to be crucial in regulating immune responses within cystic epithelial and renal cell types in PKD-affected kidneys.

Evidence Acquisition:

 This study was carried out through a literature search with the keywords of polycystic kidney disease (PKD), Newborn, and Bardet-Biedl syndrome (BBS), combined with Drug Therapy in Scopes, PubMed, and Web of Science. This study included relevant articles (i.e., randomized controlled trials, observational studies, guidelines, and reviews) written in English and published between 2000 and 2023.

 Recent genome-wide expression analyses have provided valuable insights into the molecular mechanisms associated with PKD. The Jak-STAT system, essential for immune signaling, can be activated by cytokines, such as interleukin 6 (IL-6) and interferon-gamma (IFN-γ).

 Promising developments in the treatment of PKD have emerged from studies involving immune-modulating drugs in animal models. Glucocorticoids and rosmarinic acid exhibited positive effects, reducing cystic indices and preserving renal function in PKD mice and rats. Mycophenolate mofetil, an immunosuppressive drug, showed effectiveness in reducing cyst area, inflammation, and fibrosis in rat models. Additionally, COX-2 inhibitors, PPARγ agonists, and vasopressin V2 receptor antagonists demonstrated potential in slowing cystic disease progression.

 Tracheal tumors are rare in the pediatric population. Primary tumors within the trachea often present with non-specific symptoms during the initial stages, leading to a delayed diagnosis. Prompt diagnosis of a tracheal tumor is crucial due to the risk of life-threatening airway obstruction it poses. This article discusses the case of a twelve-year-old girl who experienced 8years of breathing difficulties without an adequate response to treatment.

 The patient was referred to Mofid Children's Hospital after experiencing 8 years of difficulty with dyspnea. Her dyspnea was classified as functional class 2 and was accompanied by dry coughs. Additionally, a biphasic stridor was observed during her auscultation. Further investigations revealed an intra-tracheal mass, identified as a mucous gland adenoma (MGA). Imaging and pathology showed no signs of malignancy. Following her surgery, she was completely free of symptoms, and her follow-up spirometry showed no abnormalities.

 In this article, we present a detailed case of MGA. Despite the low incidence of intra-tracheal tumors, conducting further studies in patients who respond poorly to treatment is crucial. The potential complications from these tumors can be catastrophic. Thus, enhancing our knowledge and expertise in this area is of great importance.

 Hypermobility spectrum disorder (HSD), likely a multifactorial condition characterized by musculoskeletal pain, joint instability, and reduced bone mineral density (BMD), is the focus of this study.

 The aim of this study was to assess and compare bone density in children diagnosed with HSD and those with benign hypermobility.

 This case-control study involved 73 pediatric patients diagnosed with hypermobility spectrum disorder and benign hypermobility, who were referred to the Rheumatology clinic of Mofid Hospital in Tehran between February and November 2022. Bone mineral density was measured using Z-scores from L1-L4 and the whole body less head via dual-energy X-ray absorptiometry (DEXA).

 Significant differences were observed in the frequency distribution of spine BMD Z-scores between the two groups (P = 0.002). There was no significant correlation between spine and whole body BMD Z-scores with age and gender (P > 0.05). A notable correlation was found between spine and whole body BMD Z-scores (P < 0.001 and r = 0.64). Among the HSD group, 18 patients (81.8%) had normal BMD, while 4 patients (18.2%) exhibited low bone density, with no cases of osteoporosis. In contrast, osteoporosis was observed in 7.3% of patients in the benign hypermobility group.

 Given the high prevalence of osteoporosis in patients with benign hypermobility, clinicians should be vigilant for tendencies toward osteopenia when treating such patients.

 The full scope of coronavirus disease 2019 (COVID-19) remains unknown, and a definitive treatment for children has yet to be established. N-acetylcysteine (NAC), beyond its mucolytic effect in lung disorders, operates through various mechanisms, such as enhancing the immune system, inhibiting viral replication, and reducing inflammation. These pharmacological properties of NAC suggest it is a potential therapeutic agent for COVID-19.

 Our goal was to evaluate whether NAC could improve outcomes in hospitalized children presenting with acute respiratory symptoms due to COVID-19.

 Fifty-eight patients with moderate COVID-19 symptoms were randomly allocated to receive either 1200 mg/day of NAC or a placebo for 7 days. We monitored NAC-related side effects, C-reactive protein (CRP) levels, white blood cell (WBC) count, serum creatinine, oxygen saturation, hospital stay duration, and clinical symptoms.

 All measured variables in both groups showed significant improvement by the end of the study. However, the analysis indicated that the changes in CRP and WBC levels in the NAC group, compared to the placebo, were not significant (P = 0.659 and 0.067, respectively). There was a notable improvement in oxygen saturation in the NAC group versus the placebo group at the study's conclusion (P = 0.001). The length of hospital stay and CRP levels significantly decreased in the NAC group compared to the placebo group (P-value = 0.001 and P-value ≤ 0.001, respectively). Additionally, the mortality rate was 0.0% in the intervention group versus 7.4% in the placebo group (P-value = 0.491).

 The findings from this study support the potential of NAC in shortening hospital stay durations and enhancing oxygen saturation among children with COVID-19.

 The social, economic, political, and emotional (SEPE) environment plays a crucial role in shaping human development both pre- and post-birth, with direct and independent effects on growth, as research shows.

 This study examines the growth of twins and singletons and their association with maternal education as one of the social-economic (SE) variables.

 This cross-sectional study utilized Nutritional Status Monitoring for Children under 5 Years Old in Bandung District in September 2019. A total of 158 healthy children aged < 5 years, involving 35 twins (70 children) and 88 singletons, were included. Data on weight and length at birth and weight and length/height taken at age 8.6 to 60 months were plotted according to WHOCGS 2006. Statistical analyses and visualizations, including SE variables analysis, were conducted using density plots, correlation plots, St. Nicolas House Analysis (SNHA), and ANOVA.

 At birth, z-scores of the body length, weight, and body mass index (BMI) of twins were lower than those of singletons (P < 0.05). After birth, z-scores of twins' length/height, weight, and BMI adjusted to those of singletons, with maternal education being the strongest among SE variables of early childhood growth adjustment.

 Twins were shorter, lighter, and had a lower average BMI at birth than singletons. There was no apparent association between birth measurements and SE variables. However, after birth, maternal education starts to gain a central position in regulating child growth.

 Henoch-Schönlein purpura (HSP) is the most common type of vasculitis in children. Children with HSP often experience gastrointestinal symptoms, such as abdominal pain, nausea/vomiting, gastrointestinal bleeding, and intussusception. These symptoms are estimated to occur in 50 - 75% of cases.

 In this study, we evaluated the prevalence of gastrointestinal manifestations in children with HSP and identified associated predictive factors.

 For this cross-sectional study, we collected data from 295 children with HSP, aged 1 to 16, who were treated at Mofid Children's Hospital in Tehran, Iran, between 2013 and 2022. We gathered the following information from hospital records: Laboratory results for ALT, AST, bilirubin, stool exam (S/E), WBC, RBC, and occult blood (OB), as well as demographic data. Clinical symptoms evaluated included fever, rash, abdominal pain, distension, tenderness, nausea/vomiting, diarrhea, anorexia, and icterus.

 Our study included 295 children diagnosed with HSP, consisting of 46.77% females and 53.23% males. The average age was 5.3 ± 2.8 years for female patients and 6.2 ± 2.9 years for male patients. Further analysis indicated that anorexia was the most common symptom, followed by abdominal pain, diarrhea, nausea/vomiting, and bloody stool. We observed no significant differences in symptoms based on sex. Physical examination findings, including abdominal distension and tenderness, were similar across both sexes. Elevated levels of AST and ALT, as well as hyperbilirubinemia, were noted in some patients. Stool analysis revealed positive results for RBC, WBC, and occult blood in certain cases, with 21% testing positive for RBC, 24.85% for WBC, and 23.25% for occult blood. Anorexia showed a correlation with increased AST levels, while bloody stool was associated with higher ALT levels, hyperbilirubinemia, and direct hyperbilirubinemia. Logistic regression analysis confirmed a significant association between bloody stools and elevated ALT levels.

 In this study, we examined the clinical manifestations and laboratory findings in children with small vessel vasculitis to identify associated predictive factors. Our findings indicated that anorexia and abdominal pain were the most common clinical manifestations, with bloody stool also being a prevalent symptom. Additionally, logistic regression analysis demonstrated that the presence of bloody stool is a significant predictive factor for elevated ALT levels.

 Bipolar disorder (BD) is a prevalent psychological disorder associated with depressive symptoms. Transcranial direct current stimulation (tDCS) is a portable and non-invasive technique for brain stimulation. The present study was conducted to determine the effectiveness of the combined treatment of tDCS and routine medication on the symptoms of depression, anxiety, and anger in BD adolescents.

 In this double-blind randomized clinical trial study, forty adolescents with BD referred to the outpatient clinic of child and psychiatry of Golestan Ahvaz Teaching Hospital were included. Eligible patients aged 12 - 18 years were randomly divided into 2 groups receiving routine medications plus active tDCS (intervention group) or routine medications plus sham tDCS (control group). Transcranial direct current stimulation intervention with an intensity of 2 mA was applied to the dorsolateral prefrontal cortex in two sessions for 20 minutes each day, for 5 consecutive days. Data were collected at baseline, one week, and one month after the start of the intervention using the Hamilton Depression Rating Scale (HDRS-21), Hamilton Anxiety Rating Scale (HDRS), and State-Trait Anger Expression Inventory.

 Based on our findings, age and gender were not considerably different between the two groups (P = 0.592, P = 0.1, respectively). In both groups, scores of depression (control: 12.25 ± 3.97, intervention: 0.75 ± 1.44, mean [SD]), anxiety (13.55 ± 2.58, 25.0 ± 0.55), and anger (52.65 ± 8.27, 47.25 ± 4.86) improved significantly one month after treatment (P < 0.0001). After one month of treatment, the severity of bipolar symptoms in the intervention group (slightly: 10%, moderately: 50%, and markedly: 40%) improved significantly compared to the control group (slightly: 15%, moderately: 10%, and markedly: 0%) (P < 0.0001). The improvement rate of HDRS in the intervention group was 81.29% and 95.24% at one week and one month after treatment, while these values were 8.41% and 23.04% in the control group, respectively (P < 0.0001). All patients tolerated the treatment well without serious side effects. There was no significant difference between the side effects observed in both groups (P = 0.185).

 The combination of tDCS with routine medications can reduce depressive symptoms and improve bipolar symptoms. Therefore, tDCS add-on could be an effective, safe, and tolerable intervention for bipolar depression.