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Uvod. IgA vaskulitis (IgAV) najčešći je sistemski vasulitis u djece. Kožne promjene, koje su obvezni klasifikacijski kriterij, najčešće su tipične, ali u oko 2% djece mogu se pojaviti teže promjene koje uključuju hemoragijske vezikule i bule koje mogu progredirati do ulceracija i nekroza. Pitanje je jesu li teže kožne promjene povezane i s težom kliničkom slikom. Ispitanici i metode. U ovo istraživanje uključena su djeca iz 8 međunarodnih sveučilišnih centara koja su u sklopu IgAV-a razvila teže kožne manifestacije. Podaci su analizirani deskriptivno i primjenom Fisherova testa. Rezultati. Prikupljeni su podaci o 40 bolesnika s težim kožnim manifestacijama koji su uspoređeni s kohortom od 611 bolesnika s IgAV-om iz nacionalne baze podataka. Od ukupnog broja bolesnika 65% bilo je muškog spola, a medijan (raspon) dobi pri dijagnozi iznosio je 8, 5 (1, 5– 17, 08) godina. Najznačajniji okidač bolesti bile su infekcije, prisutne u 57, 5% bolesnika, najviše respiratorne. Najčešći prvi simptom bolesti bile su kožne promjene (60%), a tipične i teže kožne promjene dominantno su zahvaćale donje ekstremitete (45% odnosno 70%). Medijan (raspon) od prvog simptoma do prve teže kožne manifestacije iznosio je 4.5 (0–180) dana, a njihovog trajanja 10 (4–780) dana dok je od prve teže kožne promjene do početka liječenja proteklo 1 (0–37) dan. U 75% bolesnika nisu zabilježene trajne sekvele na koži dok su u 12, 5% zaostali trajni ožiljci, a u 10% promjene pigmentacije. Nefritis je razvilo 40% bolesnika, a najčešći nalaz bila je kombinacija hematurije i proteinurije. Bolesnici s težim kožnim promjenama statistički su značajno češće razvili nefritis (p = 0, 0085) i bili su značajno češće liječeni sistemskim glukokortikoidima (p < 0, 00001), a većina bolesnika imala je i gastrointestinalne (67, 5%) i zglobne manifestacije (62, 5%). Svi su bolesnici liječeni, pri čemu ih je 75% liječeno primjenom metilprednizolona s medijanom (rasponom) kumulativne doze od 33 (4–170, 46) mg/kg i medijanom (rasponom) trajanja liječenja od 17 (3– 298) dana, a 40% liječeno je primjenom nesteroidnih protuupalnih lijekova u trajanju od 5 (2–30) dana. Ostali su lijekovi primijenjeni sporadično. Zaključak. Bolesnici s IgAV-om i težim kožnim manifestacijama statistički su značajno češće razvili nefritis i bili liječeni sistemskim glukokortikoidima. Potpora: Projekt Hrvatske zaklade za znanost, IP- 2019-04-8822.

È proprio il caso di dirlo: rara ma non troppo. La corea reumatica non è una condizione che abbiamo studiato a scuola e che non vedremo mai. Può capitare a ognuno di imbattersi in un caso e non sarebbe perdonabile non ri- conoscerla prontamente (non averne ben chiare le peculiarità cliniche, le modalità di comparsa, la frequente as- sociazione con la cardite) e non saperla trattare adeguatamente (cortisone). Tre “C” appunto (Corea, Cardite, Cortisone) che sostanzialmente ci aiutano a ricordare tutto.

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Neuroimaging is a key tool in the diagnostic process of various clinical conditions, especially in pediatric endocrinology. Thanks to continuous and remarkable technological developments, magnetic resonance imaging can precisely characterize numerous structural brain anomalies, including the pituitary gland and hypothalamus. Sometimes the use of radiological exams might become excessive and even disproportionate to the patients’ medical needs, especially regarding the incidental findings, the so-called “incidentalomas”. This unclarity is due to the absence of well-defined pediatric guidelines for managing and following these radiological findings. We review and summarize some indications on how to, and even if to, monitor these anomalies over time to avoid unnecessary, expensive, and time-consuming investigations and to encourage a more appropriate follow-up of brain MRI anomalies in the pediatric population with endocrinological conditions.

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In healthy adolescents, delayed pubarche is generally a benign condition that is caused by a physiological discrepancy between gonadarche and adrenarche. In presence of other clinical signs and symptoms, delayed pubarche can be caused by single or multiple hormones deficiency (such as adrenal insufficiency, panhypopituitarism and hypothyroidism) and/or genetic conditions (Turner syndrome, androgen insensitivity syndrome). Exposition to endocrine disruptors has also been described as a possible cause of delay of pubic hair development. Basic blood tests, karyotype and first level imaging studies are helpful in the differential diagnosis.

Background: Total body irradiation (TBI) is a mandatory step for patients with acute lymphoblastic leukemia (ALL), undergoing allogeneic hematopoietic stem cell transplantation (HSCT). In the past, amylases have been reported to be a possible sign of TBI toxicity. We investigated the relationship between total amylases (TA) and transplant-related outcomes in pediatric recipients. Methods: We retrospectively analyzed the medical records of all the patients who underwent allogeneic HSCT between January 2000 and November 2019. The inclusion criteria were the following: recipient’s age between 2 and 18, diagnosis of ALL, no previous transplantation, and use of TBI-based conditioning. The serum total amylase and pancreatic amylase were evaluated before, during, and after transplantation. Cytokines and chemokines assays were retrospectively performed. Results: 78 patients fulfilled the inclusion criteria. Fifty-seven patients were treated with fractionated TBI, and 21 with a single-dose regimen. The overall survival (OS) was 62.8%. Elevated values of TA were detected in 71 patients (91%). The TA were excellent in predicting the OS (AUC = 0.773, 95% CI = 0.66–0.86, p &lt, 0.001). TA values below 374 U/L were correlated with a higher OS. The highest mean TA values (673 U/L) were associated with a high disease-progression mortality rate. The TA showed a high predictive performance for disease progression-related death (AUC = 0.865, 95% CI = 0.77–0.93, 0.0001). Elevated TA values were also connected with significantly higher levels of proinflammatory cytokines, such as TNF-α, IL-6, and RANTES (p &lt, 0.001). Conclusions: this study shows that TA is a valuable predictor of post-transplant OS and increased risk of leukemia relapse.