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.