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Cutaneous tuberculosis is a rare manifestation of extrapulmonary tuberculosis caused by Mycobacterium tuberculosis in most cases and rarely by Mycobacterium bovis. Diagnosis may be challenging due to a wide range of clinical findings and similarities to other chronic dermatoses, leading to delayed treatment. We present a case of scrofuloderma in a 4-year-old girl that arose from a contiguous spread from the anterior mediastinum with associated pulmonary involvement., (© 2024 Wiley Periodicals LLC.)

Background: Celiac trunk compression by the median arcuate ligament (MAL) increases the risk of ischemic complications following gastrointestinal surgical procedures. Previous studies suggest increased risk of hepatic artery thrombosis (HAT) in orthotopic liver transplant (OLT) recipients. The aim of this study is to investigate the impact of untreated MAL compression (MAL-C) on biliary complications in OLT., Methods: Contrast-enhanced imaging was used to classify celiac trunk stenosis by MAL-C. Medical records were reviewed to extract pre-transplant, transplant and post-transplant data. Patients were divided into two groups: no MAL compression (nMAL-C) and MAL-C. The primary endpoint was biliary complications. Secondary endpoints were HAT and graft survival., Results: 305 OLT were performed from 2010 to 2021, of which 219 were included for analysis: 185 (84.5%) patients without and 34 (15.5%) with MAL-C. The incidence of HAT was 5.9% in both groups. Biliary complications were more common in the MAL-C group (35.3% vs. 17.8%, p = 0.035). Graft survival was decreased in patients with MAL-C (p = 0.035)., Conclusions: MAL-C of the celiac trunk was associated with increased risk of biliary complications and inferior graft survival in OLT patients. These findings highlight the importance of preoperative screening and treatment of MAL in this population., (Copyright © 2023 International Hepato-Pancreato-Biliary Association Inc. Published by Elsevier Ltd. All rights reserved.)

The uterine artery pulsatility index (PI) assessed by Doppler ultrasound reflects the impedance to the blood flow in the vessel distal to the sampling point. We aimed to assess the accuracy of the uterine artery PI for the diagnosis of puberty in girls. A PRISMA-ScR-compliant scoping review was performed in the MEDLINE and Embase databases with the search terms "puberty" and "Doppler ultrasonography". Studies that included girls aged 0-18 years who underwent pelvic Doppler ultrasound with calculation of uterine artery PI were eligible. Ten studies comprising 1385 girls aged 1.2-18 years were included. The selected studies included participants from Italy, Brazil, Iran, Belgium and Denmark, and were published between 1996 and 2021. Six studies selected girls who were referred for evaluation of pubertal disorders, while four studies included only healthy girls. Nine studies found a significant difference in Doppler signal pattern and PI according to pubertal stage, with PI cutoff points ranging from 2.5 to 4.6 for the diagnosis of puberty, with a sensitivity of 77%-94%, specificity of 85%-100%, and accuracy of 79%-98%. Doppler assessment of the uterine arteries with PI calculation is a useful noninvasive tool in the diagnosis of pubertal onset in girls., (© 2023. The Author(s).)

Objective: This study aimed to estimate the performance of single-phase-enhanced computed tomography and ultrasonography examinations in the preoperative evaluation of solid abdominal tumors and their relationship with relevant adjacent structures in children., Methods: This retrospective study included 50 pediatric patients with malignant solid abdominal tumors treated with surgical resection between 2009-2017. Preoperative computed tomography and ultrasonography were compared to operative findings (gold standard) in the diagnosis of invasion or encasement of adjacent structures. Accuracy, sensitivity, specificity, and positive and negative predictive values were evaluated., Results: Renal (20.4%) and neuroblastic (19.4%) tumors were the most common. Complete surgical resection with negative margins was achieved in 44 (88%) patients. The comparison between single-phase-enhanced computed tomography and ultrasonography findings showed the following results: sensitivity = 90.3% vs 86.6%, specificity = 86.8% vs 94.6%, negative predictive value = 95.3% vs 94.4%, positive predictive value = 75.3% vs 86.9%, and accuracy = 87.9% vs 92.2%. The correlation (kappa index) between computed tomography and ultrasonography examinations was 0.72 (p < 0.001). In 14% (7/50) of the patients, the invasion of adjacent structures was diagnosed by ultrasonography but not by computed tomography (1 patient had 2 invaded structures)., Conclusion: Ultrasonography can be considered a complementary method to single-phase-enhanced computed tomography in the preoperative evaluation of children with an abdominal tumor. The present study showed that ultrasonography and single-phase-enhanced computed tomography each possess a high accuracy in the preoperative planning of resection of solid abdominal tumors in children. Thus, it seems that the combination of both imaging methods would be enough for the evaluation of most abdominal tumors in the pediatric population., Competing Interests: Conflicts of interest The authors declare no conflicts of interest., (Copyright © 2022 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.)

Objectives: Data on multidisciplinary programs dedicated to home parenteral nutrition (HPN) in Latin America are limited. This study describes the results of the first multidisciplinary pediatric intestinal rehabilitation program for HPN at a public tertiary hospital in Brazil., Methods: We retrospectively reviewed patients aged 0-18 years with intestinal failure (IF) who required parenteral nutrition (PN) for >60 days between January/2014 and December/2020., Results: Fifty-four patients were discharged on HPN (15 achieved enteral autonomy, 34 continued on HPN at the end of the study, 1 underwent intestinal transplantation, and 4 died). The median (IQR) age at the study endpoint of patients who achieved enteral autonomy was 14.1 (9.7-19) versus 34.7 (20.4-53.9) months in those who did not achieve enteral autonomy. Overall prevalence of catheter-related thrombosis was 66.7% and catheter-related bloodstream infection rate was 0.39/1000 catheter-days. Intestinal failure-associated liver disease (IFALD) was present in 24% of all patients; none of the patients who achieved enteral autonomy had IFALD. All patients showed significant improvement in anthropometric parameters during the HPN period. The sociodemographic characteristics of the patients' family members were mothers less than 20 years old (7.5%), schooling time more than 10 years (55.5%), and household income between 1 and 3 times the minimum wage (64.8%). The 5-year survival rate for HPN is 90%, and 27.7% of patients achieve enteral autonomy., Conclusion: The treatment of pediatric patients with IF followed by a multidisciplinary pediatric intestinal rehabilitation program with HPN is feasible and safe in the Brazilian public health system., Competing Interests: The authors report no conflict of interest., (Copyright © 2022 The Author(s). Published by Wolters Kluwer on behalf of European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Purpose: To evaluate the accuracy of the uterine artery pulsatility index (PI) for the diagnosis of pubertal onset in girls., Methods: Cross-sectional study of girls with normal pubertal development. Puberty was diagnosed by the presence of Tanner breast development score ≥2. All girls underwent pelvic ultrasound and Doppler imaging of the uterine arteries. We evaluated the uterine artery PI and uterine, endometrial, and ovarian measurements. We used ROC curves with cutoffs determined by Youden index for data analysis., Results: We included 169 girls aged 5-16 years who underwent 202 pelvic ultrasound examinations. Prepubertal girls had a significantly higher mean PI (6.70 ± 2.15) than girls in initial puberty (4.14 ± 1.55) and in late puberty (2.81 ± 1.05) (P < 0.001 for all comparisons), which reflects a progressive increase in blood flow to the uterus with the progression of puberty. ROC curve analysis showed that the PI was able to identify the onset of puberty with a mean area under the curve of 0.838 ± 0.04 (P < 0.001), and the PI cutoff point of 5.05 had a sensitivity of 77%, specificity of 85%, positive predictive value (PPV) of 92%, and accuracy of 79%. The combination of PI < 5.05 plus uterine volume >3.75 cm³ had a sensitivity of 73%, specificity of 95%, PPV of 97%, and accuracy of 79% to detect initial puberty., Conclusions: We found a significant reduction in the PI during pubertal development, which can possibly be a valuable noninvasive tool in the evaluation of pubertal disorders, alone or in combination with uterine and ovarian volumes., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

This report describes an adolescent with Mixed Gonadal Dysgenesis and unexpected mosaicism [karyotype 46,X,mar(Y)/ 47,X, mar(Y),+mar(Y)].). Diagnosis with 1 month of age due to atypical genitalia. He presented a right streak gonad, which was removed due to the risk for germ cell tumor, and a left testis with epididymis barely connected and without vas deferens. Left testis maintenance was sufficient for him to undergo spontaneous puberty. The patient was non-responsive to growth hormone. Webbed neck was the only dysmorphic feature. To the best of our knowledge, there were no similar cases reported with spontaneous pubertal progress reported in the literature., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Objective: Patients with cystic fibrosis (CF) have a high incidence of pubertal and growth delay. In girls with CF, pubertal delay has an important psychological impact. Still, only a few studies have explored the occurrence of pubertal delay in girls with CF. The aims of this study were to compare the pubertal development of girls with CF compared with healthy controls regarding Tanner staging and pelvic ultrasound and, in girls with CF, correlate the findings with those of spirometry, body mass index, Shwachman-Kulczycki score (SKS), and genotyping., Methods: This was a cross-sectional, case-control study including 35 girls with CF aged 6-17 years and following up at the Pediatric Pulmonology Outpatient Clinic of a tertiary hospital. These patients were compared with 59 healthy controls who had undergone pelvic ultrasound as part of another study conducted by the same group. Girls with CF were consecutively enrolled in the study during their annual routine check-up visit. Data collected in the CF group included spirometry and anthropometric results, SKS values, bone age, occurrence of current cystic fibrosis-related diabetes (CFRD) and Pseudomonas aeruginosa colonization, history of meconium ileus, genotype, ultrasound parameters, and Tanner stage., Results: Pelvic ultrasound findings and Tanner stage reflected less pubertal development in girls with CF compared with healthy controls. Pubertal stage in patients with CF who presented CFRD (3.17 ± 1.16), had chronic colonization by Pseudomonas aeruginosa (3.10 ± 1.10), or were homozygous for the F508del mutation (1.91 ± 1.30) was more delayed than in controls (3.41 ± 1.41). Tanner stage correlated with age at menarche, bone age, and anthropometric and ultrasound data., Conclusion: Girls with CF presented a delay in pubertal development evaluated by Tanner stage and ultrasound parameters, which was more evident in the presence of comorbidities.

The classic definition of precocious sexual maturation is the development of secondary sexual characteristics before 8 years of age in girls and before 9 years of age in boys. It is classified as central precocious puberty when premature maturation of the hypothalamic-pituitary-gonadal axis occurs, and as peripheral precocious puberty when there is excessive secretion of sex hormones, independent of gonadotropin secretion. Precocious sexual maturation is more common in girls, generally central precocious puberty of idiopathic origin. In boys, it tends to be linked to central nervous system abnormalities. Clinical evaluation should include a detailed history and physical examination, including anthropometric measurements, calculation of growth velocity, and evaluation of secondary sexual characteristics. The main sign to suspect the onset of puberty is breast tissue development (thelarche) in girls and testicular enlargement (≥4 mL) in boys. Hormonal assessment and imaging are required for diagnosis and identification of the etiology. Genetic testing should be considered if there is a family history of precocious puberty or other clinical features suggestive of a genetic syndrome. Long-acting gonadotropin-releasing hormone analogs are the standard of care for central precocious puberty management, while peripheral precocious puberty management depends on the etiology.Conclusion: The aim of this review is to address the epidemiology, etiology, clinical assessment, and management of precocious sexual maturation. What is Known: • The main sign to suspect the onset of puberty is breast tissue development (thelarche) in girls and testicular enlargement (≥4 mL) in boys. The classic definition of precocious sexual maturation is the development of secondary sexual characteristics before 8 years of age in girls and before 9 years of age in boys. • Long-acting gonadotropin-releasing hormone agonist (GnRHa) is the standard of care for CPP management, and adequate hormone suppression results in the stabilization of pubertal progression, a decline in growth velocity, and a decrease in bone age advancement. What is New: • Most cases of precocious sexual maturation are gonadotropin-dependent and currently assumed to be idiopathic, but mutations in genes involved in pubertal development have been identified, such as MKRN3 and DLK1. • A different preparation of long-acting GnRHa is now available: 6-month subcutaneous injection., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Congenital mid ureteric valve (MUV) stenosis is a very rare cause of ureteric obstruction and hydronephrosis (HN) in children. We describe how we manage laparoscopically one case of a patient with congenital MUV. We describe a 6-month-old boy born with antenatal left HN, with an anteroposterior diameter (APD) of 1,5 cm. Follow-up renal ultrasound at 3 months of age showed an increase in left kidney HN, approximately 2.5 cm below the PUJ associated with distal stenosis. Renal scintigraphy with DTPA showed an obstructive pattern. Urography scan showed an abrupt reduction in the caliber of the mid left ureter Based on a preoperative diagnosis of MUV stenosis, we performed a laparoscopic left ureteroplasty. Abrupt tapering of the left ureter at 3 cm from the PUJ was identified. Proximal ureter repair was performed before the resection of the narrowing segment. The two edges of the ureter were spatulated and continuous anastomosis was performed without tension. An antegrade JJ stent was inserted after the posterior part of the anastomosis was done. Patient made an uneventful recovery and was discharged on the first postoperative day. Laparoscopic ureteroplasty is a complex but feasible option to treat patients with congenital MUV stenosis., (Copyright © 2020 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Aims: To evaluate the accuracy of dynamic ultrasonography (DUS), as a feasible alternative diagnostic method to identify detrusor overactivity (DO) in patients with neurogenic bladder., Methods: We performed concurrent analysis of 81 pairs of urodynamic study (UDS) and DUS, in 63 patients with myelomeningocele (MMC), from June 2014 to February 2017. The assessment focused on bladder behavior during the filling phase, DO evaluation, DO with leakage, compliance, and maximum cystometric capacity (MCC)., Results: Patient age ranged from 3 months to 34 years (median, 84 months); 47.6% were male. Overall, 9.5% of patients had chronic kidney disease, 20.6% had recurrent urinary tract infection, 19.05% had vesicoureteral reflux, and 69.8% had constipation. Anticholinergic therapy was used by 41.3% of patients. DO was observed in 45.67% of patients and DO with leakage in 42.6%. Mean bladder compliance was 10.39 mL/cmH 2 O and normal MCC was 56.79%. DUS had 91.89% sensitivity in identifying DO, 88.64% specificity, 87.18% positive predictive value, 92.86% negative predictive value, and 90.12% accuracy, with a kappa coefficient of 0.8 (P < 0.001)., Conclusion: MMC follow-up is essential because urinary parameters can change during patient growth. The standard examination is invasive and has related complications, making noninvasive evaluation a desirable alternative, like DUS. Our data suggest that DO and MCC can be evaluated using DUS in patients with MMC. UDS should be performed in patients with abnormal findings on ultrasound evaluation or those with worsening of urinary tract function., (© 2018 Wiley Periodicals, Inc.)