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Purpose: Congenital retinal macrovessels (CRM) are large aberrant blood vessels that cross the horizontal raphe. Visual acuity may be negatively affected in cases afflicted with CRM due to macular serous detachment, hemorrhage, exudation, foveolar cyst formation and retinal artery occlusion. Even the presence of an anomalous vessel itself running through the foveal avascular zone may compromise the visual acuity. In this case report, we discuss the multimodal imaging characteristics of a case of symptomatic unilateral CRM-related cystoid macular edema and serous macular detachment in a 13-year-old boy.Observations: Optical coherence tomography angiography (OCTA) imaging of the superficial retina revealed the aberrant vessel and anomalous arteriovenous communication between the aberrant vessel and the retinal artery. The foveal avascular zone also appeared partly distorted.Conclusions: The present case reports the second-youngest patient afflicted with unilateral CRM and associated macular edema. There was spontaneous resolution of macular edema within six weeks. In light of the observations in this case and the literature review, the need to refrain from rapid therapeutic intervention in the eyes of patients affected with CRM and macular edema must be emphasized.

Objective: Henoch-Schoenlein purpura (HSN), an IgA-mediateddisease is the most common form of systemic vasculitis seen inchildhood and long-term prognosis depends on renal involvement.We aimed to investigate the influence of renal IgA accumulationon clinical and histopathological findings in childrenwith HSN.Materials and Methods: Seventeen patients who were diagnosedas HSN based on renal biopsy were included in the study. Theclinical assessment at baseline and last visit were done accordingto a classification adapted from that of Goldstein et al and renalhistopathological changes were graded according to the classificationof International Study of Kidney Disease in Childhood. IgAaccumulation was quantified as +1 to +4. Based on this, patientswere divided into group 1 (+1 or +2 accumulation) and group 2(+3 or +4 accumulation). All parameters were compared betweenthe groups.Results: There was no significant difference between the groupswith respect to clinical findings and histopathological changes.The amount of IgA accumulation was not correlated with clinicalor histopathological status.Conclusion: The amount of renal IgA deposition is not associatedwith clinical or histological findings in our sample. These resultsneed to be confirmed in larger samples. [ABSTRACT FROM AUTHOR]

Children who had undergone adenotonsillectomy for recurrent adenotonsillitis showing no signs of clinical or radiological obstructive manifestations were evaluated with pulmonary function tests before, and one month after the operation. In relation to the result obtained by function tests, 60% of 45 cases (27) had the findings of mild obstructive pulmonary disease whereby these findings were in transient character that vanished after the operation. The following parameters were measured and found that they were all increased, mean FVC from 82.22 +/- 6.82 to 93.11 +/- 7.81 (p < 0.01), mean PEF from 77.60 +/- 8.38 to 88.60 +/- 5.57 (P < 0.01), mean FEVI from 74.28 +/- 11.68 to 90.15 +/- 7.28 (p < 0.01), mean FEF 25 from 71.44 +/- 11.53 to 83.53 +/- 6.40 (p < 0.01), mean FEF 50 from 69.53 +/- 14.53 to 84.37 +/- 7.72 (p < 0.01), mean FEF 75 from 70.08 +/- 12.15 to 85.48 +/- 7.15 (p < 0.01). In conclusion, pulmonary function tests could reveal the obstructive effects of adenotonsillar hypertrophy with no clinical or radiological obstructive findings, and could be useful in surgical indications of adenotonsillar hypertrophy dur to recurrent infections in children. [ABSTRACT FROM AUTHOR]

Presents a letter to the editor about the incidence of nonlupus nephritis in an adolescent with systemic lupus erythematosus.

Reports on a case of a girl with congenital erythropoietic porphyria. Description of the disease; Physical manifestations of the disease; Effect of phototherapy administered during the neonatal period.

Background: Elevated C-reactive protein (CRP) was proposed as a biomarker instead of renal biopsy in drug-induced acute tubulointerstitial nephritis (D-ATIN) in adults. However, there is no study in which patients were followed up with serial CRP without kidney biopsy. We evaluated the significance of CRP levels in predicting the clinical course of D-ATIN in children., Materials and Methods: Children with a clinical diagnosis of D-ATIN were evaluated for sex, age, symptoms, offending drug(s), latent period after exposure, blood pressure, urine output, urinalysis, eosinophiluria, urine albumin/total protein, serum creatinine, and CRP at presentation. In addition, serial CRP and creatinine levels were recorded daily until discharge and thereafter. Correlations of CRP with creatinine and of peak CRP with creatinine normalization time were evaluated., Results: There were 13 patients (8 female, median age 15 years). None had oliguria or hypertension. Median CRP and creatinine at presentation were 36 mg/L and 1.6 mg/dL, respectively. Median times to peak CRP and creatinine were 6 and 7 days after drug exposure, respectively. The decrease in CRP preceded the fall in creatinine. Median CRP and creatinine normalization times after their peaks were 5 and 14 days, respectively. None required renal biopsy or corticosteroid treatment. CRP was correlated with creatinine throughout the study period. However, peak CRP was not correlated with creatinine normalization time., Conclusion: CRP was increased at presentation and decreasing CRP predicted favorable outcome. Renal biopsy and corticosteroid treatment could be postponed safely in these children.

Many ventilator-dependent children have comorbid conditions including urinary tract disorders. We aimed to present a focused review of the literature describing the causes and management of urinary system problems in children with long-term home mechanical ventilation. We performed a literature search in PubMed/MEDLINE, Scopus, and Web of Science with keywords "children," "home mechanical ventilation," "urinary system," "urinary tract," "neurogenic bladder," "clean intermittent catheterization," "urinary tract infection," "urolithiasis," and "acute kidney injury." We included original articles, reviews, guidelines, and case reports published in English. Ventilator-dependent children may have neurogenic bladder/bowel dysfunction which renders them prone to urinary tract infection, high bladder pressure, vesicoureteral reflux, hydronephrosis, and renal dysfunction. These children require bladder catheterization, medications affecting parasympathetic/sympathetic nervous systems, or surgical procedures to prevent urinary infections, and to maintain continence and renal functions. However, bladder catheterization or surgical procedures like augmentation cystoplasty may also be complicated with urinary infections, urolithiasis, or urethral strictures. Urolithiasis frequency is also increased due to immobilization-related hypercalciuria, hypocitraturia caused by antiepileptic drugs, urinary stasis, and urinary infections. On the other hand, mechanical ventilation can impair renal function by reduction of cardiac output, redistribution of intrarenal blood flow and stimulation of sympathetic and hormonal pathways. Children requiring long-term invasive home mechanical ventilation may have other comorbid conditions, including urinary system diseases, which become manifest as these patients are being kept alive due to the advances in ventilation strategies. These children must be carefully observed for urological complications and managed accordingly to prevent kidney injury., (© 2024 The Authors. Pediatric Pulmonology published by Wiley Periodicals LLC.)

Background: Breast cancer has been a disease in which treatment strategy has changed over time under the influence of different hypotheses and evidence for more than a century. We analyzed the contribution of radiotherapy to disease-free survival and overall survival by classifying according to stage, 1-3 lymph node involvement, and molecular subgroups., Methods: Following the approval of the Institutional Review Board, records of patients with breast cancer who were admitted to University School of Medicine Departments of Radiation Oncology and Medical Oncology between July 1999 and December 2020 were reviewed. Using data propensity score matching was performed between the groups that did and did not receive radiotherapy using an optimal matching algorithm (optimum, 1:1). Disease-free survival and overall survival after propensity score matching were calculated using the Kaplan-Meier method. Univariate and multivariate Cox regression analysis was used to estimate hazard ratios., Results: In the radiotherapy and non-radiotherapy groups, disease-free survival was 257.42 ± 5.46 (246.72- 268.13), 208,96 ± 8,15 (192,97-224,94) months respectively, (p = < 0.001), overall survival was 272,46 ± 8,68 (255,43-289,49), 219,05 ± 7,32 (204,70-233,41) months respectively (p = .002). We compared the 19 N1 patient groups who received radiotherapy with the 19 patients who did not receive radiotherapy and calculated the disease-free survival times was 202,21 ± 10,50 (181,62-222,79) and 148,82 ± 24,91 (99,99-197,65) months respectively (p = .011) and overall survival times was 200,85 ± 12,79 (175,77-225,92) and 166,90 ± 20,39 (126,93-206,82) months respectively (p = .055). We examined disease-free survival and overall survival times in both groups according to Luminal A, Luminal B, TNBC, and HER2-enriched subgroups. In the Luminal B subgroup, the disease-free survival duration in the groups receiving radiotherapy and not receiving radiotherapy was 264.83 ± 4.95 (255.13-274.54) and 187.09 ± 11.06 (165.41-208.78) months (p < .001), and overall survival times were 252.29 ± 10.54 (231.62-272.97) and 197.74 ± 9.72 (178.69-216.80) months (p = .001) respectively., Conclusions: Thanks to studies proving that RT increases long-term survival rates in breast cancer as a result of reducing locoregional recurrence and systemic metastasis rates, it has been understood that the spectrum hypothesis is the hypothesis that most accurately describes breast cancer to date. We found that patients with Luminal B invasive breast cancer benefited significantly more from RT compared to other subgroups., (© 2024. The Author(s).)

Background: One of the most common bacterial infections in childhood is urinary tract infection (UTI). Toll-like receptors (TLRs) contribute to immune response against UTI recognizing specific pathogenic agents. Our aim was to determine whether soluble TLR4 (sTLR4), soluble TLR5 (sTLR5) and interleukin 8 (IL-8) can be used as biomarkers to diagnose UTI. We also aimed to reveal the relationship between urine Heat Shock Protein 70 (uHSP70) and those biomarkers investigated in this study., Methods: A total of 802 children from 37 centers participated in the study. The participants (n = 282) who did not meet the inclusion criteria were excluded from the study. The remaining 520 children, including 191 patients with UTI, 178 patients with non-UTI infections, 50 children with contaminated urine samples, 26 participants with asymptomatic bacteriuria and 75 healthy controls were included in the study. Urine and serum levels of sTLR4, sTLR5 and IL-8 were measured at presentation in all patients and after antibiotic treatment in patients with UTI., Results: Urine sTLR4 was higher in the UTI group than in the other groups. UTI may be predicted using 1.28 ng/mL as cut-off for urine sTLR4 with 68% sensitivity and 65% specificity (AUC = 0.682). In the UTI group, urine sTLR4 levels were significantly higher in pyelonephritis than in cystitis (p < 0.0001). Post-treatment urine sTLR4 levels in the UTI group were significantly lower than pre-treatment values (p < 0.0001)., Conclusions: Urine sTLR4 may be used as a useful biomarker in predicting UTI and subsequent pyelonephritis in children with UTI. A higher resolution version of the Graphical abstract is available as Supplementary information., (© 2023. The Author(s), under exclusive licence to International Pediatric Nephrology Association.)

Background: The accuracy of conventional urinalysis in diagnosing urinary tract infection (UTI) in children is limited, leading to unnecessary antibiotic exposure in a large fraction of patients. Urinary heat shock protein 70 (uHSP70) is a novel marker of acute urinary tract inflammation. We explored the added value of uHSP70 in discriminating UTI from other infections and conditions confused with UTI., Methods: A total of 802 children from 37 pediatric centers in seven countries participated in the study. Patients diagnosed with UTI (n = 191), non-UTI infections (n = 178), contaminated urine samples (n = 50), asymptomatic bacteriuria (n = 26), and healthy controls (n = 75) were enrolled. Urine and serum levels of HSP70 were measured at presentation in all patients and after resolution of the infection in patients with confirmed UTI., Results: Urinary (u)HSP70 was selectively elevated in children with UTI as compared to all other conditions (p < 0.0001). uHSP70 predicted UTI with 89% sensitivity and 82% specificity (AUC = 0.934). Among the 265 patients with suspected UTI, the uHSP70 > 48 ng/mL criterion identified the 172 children with subsequently confirmed UTI with 90% sensitivity and 82% specificity (AUC = 0.862), exceeding the individual diagnostic accuracy of leukocyturia, nitrite, and leukocyte esterase positivity. uHSP70 had completely normalized by the end of antibiotic therapy in the UTI patients. Serum HSP70 was not predictive., Conclusions: Urine HSP70 is a novel non-invasive marker of UTI that improves the diagnostic accuracy of conventional urinalysis. We estimate that rapid urine HSP70 screening could spare empiric antibiotic administration in up to 80% of children with suspected UTI. A higher resolution version of the Graphical abstract is available as Supplementary information., (© 2022. The Author(s), under exclusive licence to International Pediatric Nephrology Association.)