PA and MAA have numerous nonspecific presentations, potentially leading to delayed diagnosis or misdiagnosis. In this paper, we present the clinical and biochemical characteristics of MMA and PA patients at initial presentation. Results. This is a retrospective review of 20 patients with PA ( n = 10 ) and MMA ( n = 10 ). The most observed symptoms were vomiting (85%) and refusing feeding (70%). Ammonia was 108.75 ± 9.3  μmol/l, showing a negative correlation with pH and bicarbonate and positive correlation with lactate and anion gap. Peak ammonia did not correlate with age of onset ( r = 0.11 and p = 0.64 ) or age at diagnosis ( r = 0.39 and p = 0.089 ), nor did pH ( r = 0.01 , p = 0.96 ; r = − 0.25 , p = 0.28 ) or bicarbonate ( r = 0.07 , p = 0.76 ; r = − 0.22 , p = 0.34 ). There was no correlation between ammonia and C3 : C2 ( r = 0.1 and p = 0.96 ) or C3 ( r = 0.23 and p = 0.32 ). The glycine was 386 ± 167.1  μmol/l, and it was higher in PA ( p = 0.003 ). There was a positive correlation between glycine and both pH ( r = 0.56 and p = 0.01 ) and HCO3 ( r = 0.49 and p = 0.026 ). There was no correlation between glycine and ammonia ( r = − 0.435 and p = 0.055 ) or lactate ( r = 0.32 and p = 0.160 ). Conclusion. Clinical presentation of PA and MMA is nonspecific, though vomiting and refusing feeding are potential markers of decompensation. Blood gas, lactate, and ammonia levels are also good predictors of decompensation, though increasing levels of glycine may not indicate metabolic instability.

Hydroxychloroquine (HCQ) is an antimalarial agent used to treat mucocutaneous, musculoskeletal, constitutional manifestations of systemic lupus erythematosus (SLE). This study assessed the efficacy and side effects of HCQ in children with proliferative lupus nephritis (LN). This double-blind, randomized, placebo-controlled trial study was conducted on 60 children with proliferative LN classes III and IV treated with steroids and a mycophenolate (MMF) regimen. Patients were categorized into two groups, the HCQ group (n = 30) and the placebo group (n = 30). They were evaluated initially at 6- and a 12-month follow-up by mucocutaneous, ophthalmological examination, and investigations (BUN, creatinine, 24 h proteinuria, triglycerides (TG), cholesterol, Antids-DNA, C3, C4). Disease activity was assessed using the SLE disease activity index (SLEDAI-2 k). After 12 months, TG, cholesterol, 24 h proteinuria, Antids-DNA, and SLEDAI score were significantly decreased in the HCQ group (P: 0.002, 0.012, 0.031, 0.001, respectively). After 12 months, the cumulative probabilities of developing primary end-points (LN partial and complete remission) were 40% and 60% in the HCQ group versus 53.3% and 36.7% in the placebo group (P: 0.002). After 12 months, the HCQ group experienced mucocutaneous alopecia (3.3%), hyperpigmentation (10%), and ophthalmological mild retinal changes (6.7%), but they did not differ significantly from the placebo group.  Cunclusion: HCQ improved the disease and LN activity in children with proliferative LN, with documented skin hyperpigmentation and mild retinal changes following HCQ use in a few cases. This study was registered on http://www.clinicaltrials.gov/ with trial registration number (TRN): NCT03687905, September 2018 “retrospectively registered.” What is Known: • Hydroxychloroquine (HCQ) is documented as an adjunctive treatment in children with systemic lupus erythematosus (c-SLE) LN with efficacy in improving lupus musculoskeletal and mucocutaneous manifestations. • Due to the paucity of studies, its effects and side effects in children with LN remain unclear. What is New: • This pilot randomized clinical trial assessed the efficacy and adverse effects of HCQ in children with proliferative LN. • HCQ had numerous advantages for LN, including rapid and sustained remission, antilipidemic effect, and rapid improvement of kidney functions.

Background Down syndrome (DS) is a chromosomal disease in which all or part of a third copy of chromosome 21 is present. From the age of 6–9 months postnatal, endogenous growth hormone induces the production of insulin-like growth factor 1 (IGF-1), which has a significant influence on development. As a result, when growth hormone takes over as the primary regulator of development, the growth retardation associated with DS becomes more apparent. Aim The study’s purpose was to compare IGF-1 levels in children with DS to those in healthy children. Patients and methods This prospective cross-sectional research was conducted on 20 children with DS as the study group who attended the outpatient clinic of the Tanta University Pediatric Department’s Genetics Unit. Twenty healthy normal children with matched age and sex served as the control group. All included children were given a full history, clinical examination, and blood IGF-1 test. Results Serum levels of IGF-1 were considerably lower in children with DS (122 ± 83.3 ng/ml) when compared with healthy controls (169.4 ± 33.3 ng/ml). The median IGF-1 levels were significantly lower in the DS patient group (2–6 years) than that in the age group 7–12 years (60.4 vs. 153, respectively). IGF-1 was significantly positively correlated with age and BMI. Conclusion IGF-1 level in children with DS was substantially lower than that in healthy children, especially in the age group 2–6 years. Moreover, IGF-1 positively correlated with age and BMI.

Background: Pneumonia is the most frequent reason for morbidity and mortality in children worldwide. As many as ten times as many children die from childhood pneumonia in developing countries, compared to developed countries. Aim: This study was conducted to investigate severe recurrent pneumonia in children. Subjects and methods: This prospective & descriptive research were conducted on 200 patients at Tanta University Hospital, Pediatrics Department during the period from the beginning of May 2019 to April 2022. We recruited all patients with advanced recurrent pneumonia (RP). Results: Mean age of advanced RP children is 15.4 months, while the age at 1st episode of pneumonia was 12.7 months. Between children with advanced RP, the majority were males, being twice compared with females. Cough was the prevalent symptom in 99% of cases then wheezing (63%) and fever (60%). Respiratory abnormalities were the most prevalent (28%) as underlying causes of advanced RP patients, then immune disorders (22%) and congenital heart diseases (16%). The pulmonary hemorrhagic syndrome was the least prevalent cause as it was observed in only 2 cases. Conclusion:Children with underlying diseases had more serious diseases & poorer clinical results because they were more likely to get recurrent pneumonia & were susceptible to resistant microorganisms. As a result, more emphasis can be placed on clinical severity & therapeutic plan.