Managing children and adolescents with type 1 diabetes and coexisting celiac disease: Real-world data from a global survey.

OBJECTIVES: Celiac disease (CD) is among the diseases most commonly associated with type 1 diabetes (T1D). This study aimed to evaluate the worldwide practices and attitudes of physicians involved in pediatric diabetes care regarding diagnosing and managing CD in children with T1D. METHODS: The 30-item survey was conducted between July and December 2023 aimed at targeting pediatricians with special interest in T1D and CD. It was shared by the JENIOUS- young investigators group of the International Society of Pediatric and Adolescent Diabetes (ISPAD) and the YES- early career group of the European Society for Pediatric Endocrinology (ESPE). RESULTS: 180 physicians (67.8% female) from 25 countries responded. Among respondents, 62.2% expected sustaining optimal glycemic control in children with T1D and CD (T1D+CD) to be more difficult than in children with T1D alone. Majority (81.1%) agreed that more specific guidelines are needed. The follow-up routine for patients with T1D+CD differed, and one-quarter of physicians scheduled more frequent follow-up checkups for these patients. Seventy percent agreed multidisciplinary outpatient clinics for their follow-up is needed. In the multivariate ordinal logistic regression model, a statistically significant predictor of a higher degree of practice according to ISPAD 2022 guidelines was a higher level of country income (OR=3.34; p<0.001). CONCLUSIONS: These results showed variations in physicians' practices regarding managing CD in children with T1D, emphasising the need for more specific guidelines and intensive education of physicians in managing this population, especially in lower-income countries. Our data also suggest the implementation of multidisciplinary outpatient clinics for their follow-up.

Quality of Life of Elementary School Students with Type 1 Diabetes in a Developing Country during the COVID Pandemic.

Type 1 diabetes (T1D) is a condition that affects all aspects of life, and thus is closely related to the quality of life itself. Dealing with it during the COVID-19 pandemic is a big challenge. A case-control study conducted in Montenegro at the end of 2021 included 87 elementary school students with T1D and 248 of their peers as controls matched by gender. Standardized questionnaires were distributed to participants (Peds-QL Generic core 4.0 questionnaire for all participants and Peds-QL Diabetes Module 3.2 only for cases). Based on them, the results of obtained scores were measured and compared using non-parametric statistical methods in relation to gender, region and type of household. Children with T1D reported lower quality of life comparing to matching controls with lower scores in almost all domains. Differences in the same domains among patients and their classmates were also observed in the different gender subgroups, environment type subgroups and in the central region. Results of the study provide insights to prioritizing actions for children with diabetes care as well as for public healthcare planning.

Trends in nationwide incidence of pediatric type 1 diabetes in Montenegro during the last 30 years.

Significant and unexplained variations in type 1 diabetes (T1D) incidence through the years were observed all around the world. The update on this disorder's incidence is crucial for adequate healthcare resource planning and monitoring of the disease. The aim of this study was to give an update on the current incidence of pediatric T1D in Montenegro and to analyze incidence changes over time and how the exposure to different factors might have affected it. This retrospective cohort study included a total of 582 patients younger than 15 years who were newly diagnosed with T1D during the past 30 years. The average age at diagnosis was 8.4 ± 3.91 years. The mean annual incidence of T1D in the Montenegro population during the whole study period of 30 years was 15.2/100,000 person-years. Slightly higher incidence rates were observed in male compared to female individuals, and the incidence increased with age, with the highest incidence in the 10-14 age group. If the model is observed as one without jointpoints, the annual percentage change (APC) for the total population is 3.1 (1.8-4.4); for male individuals, 3.8 (2.1-5.5); and for female individuals, 2.1 (0.6-3.5). In 2020, the first year of the coronavirus disease of 2019 (COVID-19) pandemic, in comparison to 2019, the incidence rate increased from 19.7/100,000 to 21.5/100,000, with the highest increase in the age group of 5-9 years. This is the first nationwide report on a 30-year period of T1D incidence trend in Montenegro. It suggests that T1D incidence among Montenegrin children is rising again and that there is a short-term influence of COVID-19 on new-onset T1D.

What information does YouTube offer on laparoscopic pyloromyotomy?

BACKGROUND: YouTube is overloaded with various medical videos and has become a common source for surgeons and the general public alike to update on surgical procedures. This study determined the quality and benefits of information with regards to laparoscopic pyloromyotomy on YouTube. METHODS: A search was performed on YouTube using the key words "laparoscopic pyloromyotomy." All of the videos were viewed and evaluated by 2 surgeons watching the first 20 seconds and selected segments of each video to determine the content of the videos. The exclusion criteria were videos not related to laparoscopic pyloromyotomy, Microsoft-PowerPoint slide presentations and repeated videos. RESULTS: The search revealed 471 videos and video playlists, and of the 350 watched videos only 34 were on the topic of laparoscopic pyloromyotomy. Eight videos had an institutional source and 26 were private uploads. Among the analyzed videos, 11 were in high-definition (HD) and 23 non-HD. Regarding the country of origin, most of them were from India (N.=6) and the USA (N.=6), and regarding language, English was most common 28. The videos were uploaded between the years 2007-2016.The number of views ranged from 21 to 14,783 and mean duration of the videos was 2.55 min. There was a noticeable paucity of information as the largest number of videos offered a brief text description below only. CONCLUSIONS: Social media exposure of laparoscopic pyloromyotomy on YouTube can be summarized as follows: one-quarter of the videos are institutional uploads, English language videos top the list, mean video durations are 2.5 minutes with an average number of views >2500 and the majority of surgery videos focus on the main part of the procedure.

Effectiveness of different therapy protocols in preemies with intestinal hypomotility.

OBJECTIVE: Intestinal hypomotility delays achievement of full enteral feeds and normalization of stooling patterns in preemies. We hypothesized that introduction of prokinetic drug in addition to enemas would improve intestinal motility. PRIMARY OUTCOME: time needed to achieve full enteral feeds and normal stooling pattern. Secondary outcome: day when start of minimal enteral feeding was feasible, necrotizing enterocolitis incidence, length of hospitalization and whether daily meconium evacuation is more effective than evacuation in presence of clinical symptoms only. STUDY DESIGN: A randomized controlled trial was conducted from December 1st, 2015. until December 1st, 2016. in level III neonatal unit on 67 preterm infants ≤ 32 gestational weeks and intestinal hypomotility. Infants were allocated to: Group 1 - treated with saline enemas twice daily until normal stooling pattern was achieved; and Group 2 - treated with erythromycin and enemas. Infants with intestinal hypomotility, hospitalized from December 1st, 2014. to December 1st, 2015. were assigned to group 3, and were treated with enemas only when symptoms of abdominal distension or absence of stool for 48 hours were observed. RESULTS: Total of 127 neonates was included in this study; 33 were assigned to Group 1, 34 to Group 2, and 60 to Group 3. There was no significant difference in number of days needed to reach full enteral feeds: 25 vs. 26 days and normal stooling pattern: 18 vs. 15 between groups 1 and 2. Time needed to achieve full enteral feeds and normal stooling pattern in groups 1 and 2 were significantly shorter when compared to group 3. No difference in length of hospitalization between the groups was observed. CONCLUSIONS: Erythromycin did not improve the patient outcome, although therapy protocol in group 1 and group 2 were more effective than therapy used in patients in group 3.

Clinical and genetic characteristics of patients with congenital hyperinsulinism in 21 non-consanguineous families from Serbia.

Persistent hypoglycaemia in newborns and infants is most commonly caused by congenital hyperinsulinism (CHI). Most CHI studies report outcomes in children from both consanguineous and non-consanguineous families which can affect the phenotype-genotype analysis. The aim of this study was to analyze characteristics of patients with CHI in 21 non-consanguineous families from Serbia. This retrospective cohort study included a total of 21 patients with CHI treated in the Mother and Child Healthcare Institute of Serbia during the past 20 years. The prevalence of macrosomia at birth was very low in our cohort (4.8%). Median age at presentation was 6 days, with seizures as the presenting symptom in 76% of patients. Only four patients (19%) were diazoxide unresponsive, and eventually underwent pancreatectomy. Genetic testing was performed in 15 patients and genetic diagnosis was confirmed in 60%, with all patients being heterozygous for detected mutations. The ABCC8 gene mutations were detected in 55.6%, GLUD1 in three patients (33.3%) with HIHA syndrome and one patient had HNF4A gene mutation and unusual prolonged hyperglycaemia lasting 6 days after diazoxide cessation. Neurodevelopmental deficits persisted in 33% of patients.Conclusion: This is the first study regarding CHI patients in Serbia. It suggests that in countries with low consanguinity rate, majority of CHI patients are diazoxide responsive. The most common mutations were heterozygous ABCC8, followed by GLUD1 and HNF4A mutations, suggesting the potential benefit of population-tailored genetic analysis approach, targeting the mutations causing CHI via dominant inheritance model in regions with low consanguinity rates. What is Known: • Persistent hypoglycaemia during infancy and early childhood is most commonly caused by congenital hyperinsulinism (CHI). • Consanguinity is a very important factor regarding the genetics and phenotype of CHI, increasing the risk of autosomal recessive genetic disorders, including the severe, diazoxide-unresponsive forms caused by recessive inactivating mutations in ABCC8 and KCNJ11. What is New: • Results of the present study which included CHI patients from 21 non-consanguineous families suggest that in countries with low consanguinity rates, majority of CHI patients can be diazoxide responsive, with most common mutations being heterozygous ABCC8, followed by GLUD1 and HNF4A mutations. • Unusually prolonged hyperglycaemic reaction to diazoxide treatment in a patient with HNF4A mutation was also described in the present study.

Review of Laparoscopic Management of Mature Cystic Teratoma of Ovaries in Children.

Ovarian cystic mature teratomas (OCMTs) are the most frequent ovarian tumors in childhood. This review aimed to determine the feasibility and safety of laparoscopic management of OCMT. Literature was searched for terms "mature," "ovarian," "teratomas," and "laparoscopy." Primary endpoints were age at surgery, laparoscopic and surgical technique, intraoperative complications, postoperative morbidity, and associated pathology. Literature search revealed 11 articles published between 1998 and 2014 that met the inclusion criteria. There were 105 (n = 95 unilateral; n = 10 bilateral) patients for this analysis, with mean age at surgery being 13 years. Four laparoscopic approaches were opted: gasless transumbilical laparoendoscopic single-site (LESS) surgery (n = 19), gasless multiport surgery (n = 24), single-incision laparoscopic surgery (SILS) (n = 3), and pneumoperitoneum multiport laparoscopy (n = 59). The 10 patients with bilateral OCMT underwent ovary-sparing surgery: LESS-assisted extracorporeal bilateral cystectomy in which tumors were punctured by a balloon catheter (n = 2), intracorporeal cystectomy for gasless multiport laparoscopy (n = 5) with use of endobags to prevent spillage, and transperitoneal multiport laparoscopy (n = 3). OCMT was associated with ipsilateral and unilateral ovarian torsion in five and bilateral ovarian torsion in one patient with bilateral OCMT. In four patients with unilateral OCMT, salpingo-oophorectomy was performed. Intraoperative complications were laceration of utero-ovarian ligament and bladder injury during a suprapubic port placement. The mean follow-up was 31.9 months. Patients with unilateral or bilateral OCMT can be offered ovarian-sparing surgery laparoscopically with one of the following techniques: LESS, SILS or multiport laparoscopy with pneumoperitoneal or gasless. Long-term follow-up of these techniques has shown no recurrence with low postoperative morbidity and low intraoperative complications.

Child Migrant Health: The Role of Pediatric Surgery in the Migrants' Route across Balkan Countries to North-West Europe.

INTRODUCTION: Child migrants are the most vulnerable population, prone to various health conditions due to trauma and the bad living conditions that they experience during their migration. The objective of this study was to determine the pediatric surgical conditions of migrant children treated in our hospital on their way toward North-West Europe. MATERIALS AND METHODS: A retrospective analysis was performed on all admitted migrants in one tertiary and one secondary level hospital from 2016 to 2018. Only migrant children with surgical issues who had been hospitalized or treated in outpatient clinics were included in the study. RESULTS: There were 47 migrants admitted to hospitals and outpatient clinics; 32 from Afghanistan, 11 from Iraq, 1 from Syria, 2 from Iran, and 1 from Algeria. There were 27 boys and 20 girls, average age 7.96 years (range: 3 months-17 years). The average length of hospitalization was 14.1 days (range: 1-48), and average stay in the intensive care unit (ICU) was 7.4 days (range: 1-15). There were 29 migrants treated in outpatient clinics, and 18 were hospitalized. The cause of seeking surgical treatment was trauma in 33 and non-traumatic surgical problems in 14. The most common types of trauma were isolated fractures which occurred in one, followed by isolated head injury in eight. Polytrauma occurred in five. A lethal outcome occurred in one 9-year-old boy from Afghanistan due to severe polytrauma. CONCLUSION: Trauma, predominantly fractures and head injuries, was the leading cause of morbidity in migrant children treated in these two hospitals.

Barrett's esophagus in children: what is the evidence?

BACKGROUND: This study systematically reviewed etiology, prevalence, treatment and outcome of Barrett's esophagus (BE) in the pediatric population. METHODS: PubMed® was searched for terms "Barrett's esophagus" and "children". End points were age of patients, etiology, association with other syndromes, treatment, incidence of carcinoma and outcome. This review was conducted according to the PRISMA guidelines. Data were collected, entered and analyzed into a Microsoft Excel® spreadsheet database. RESULTS: Search revealed 278 articles published between 1984 and 2017, of which 18 met the inclusion criteria. There were 130 patients for analysis with a mean age 10.6 years (0.8-17.2 years). BE was diagnosed in 80 patients with confirmed gastroesophageal reflux (GER) only; further 20 patients were neurologically impaired and had GER, 13 after esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) repair with associated GER, 6 post-chemotherapy, 1 after post caustic burns, 1 after esophageal replacement with stomach, 1 after peptic esophageal stricture, 1 with secretory diarrhea, 1 with Fanconi anemia, 1 tetralogy of Fallot, and 5 healthy children. Regarding treatment, 26 were on medical treatment only, 16 had surgeries combined with medical treatment, 80 patients underwent surgery only, 1 was on diet management, 4 were on surveillance only and 2 were never treated for BE as death occurred because of associated conditions. Fundoplication was the most commonly performed surgery (82.2%). Adenocarcinoma was found in one 23-year-old patient. Mean follow-up was 3.45 years (10 months-13 years) and long-term outcome showed recurrences in 8 and esophago-mediastinal fistula and proximal esophagus ulcer in 1. There were 7 lethal outcomes which were not directly associated with BE. CONCLUSIONS: Although BE is considered a premalignant condition; incidence of carcinoma in pediatric population is low. Long-term follow-up with endoscopies and biopsies seems to be advisable for BE evidence and malignant alterations.

Laparoscopic management of Müllerian duct remnants in the paediatric age: Evidence and outcome analysis.

BACKGROUND: This study performed a literature analysis to determine outcomes of laparoscopic management in Müllerian duct remnants (MDRs). PATIENTS AND METHODS: Literature was searched for terms 'Müllerian' 'duct' 'remnants' and 'laparoscopy'. Primary end points were age at surgery, laparoscopic technique, intraoperative complications and postoperative morbidity. RESULTS: The search revealed 10 articles (2003-2014) and included 23 patients with mean age of 1.5 years (0.5-18) at surgery. All patients were 46XY, n = 1 normal male karyotype with two cell lines. Explorative laparoscopy was performed in n = 2 and surgical management in n = 21. The 5-port technique was used in n = 10, 3-port in n = 9 and robot-assisted laparoscopic approach in n = 1 (n = 1 technique not described). Complete MDRs removal in n = 9, complete dissection and MDRs neck ligation with endoscopic loops in n = 11 and n = 1 uterus and cervix were split in the midline. After MDRs removal, there were n = 2 bilateral orchidopexy, n = 3 unilateral orchidopexy, n = 1 Fowler-Stephens stage-I and n = 1 orchiectomy. Mean operative time was 193 min (120-334), and there were no intraoperative complications. Mean follow-up was 20.5 months (3-54) and morbidity included 1 prostatic diverticula. There were 13 associations with hypospadias, of which 3 had mixed gonads and 3 bilateral cryptorchidism. Other associations were unilateral cryptorchidism and incarcerated inguinal hernia n = 1, right renal agenesis and left hydronephrosis n = 1 and n = 2 with transverse testicular ectopy. CONCLUSION: This MDRs analysis suggests that the laparoscopic approach is an effective and safe method of treatment as no intraoperative complication has reported, and there is low morbidity in the long-term follow-up.

Predictors of mortality in neonates with giant omphaloceles.

INTRODUCTION: This analysis performed a review of giant-omphaloceles to determine the predictors of mortality. EVIDENCE ACQUISITION: PubMed and KoBson databases were searched for terms "giant," "omphalocele," and "mortality." Primary end points included mortality correlation with gestational age (GA), birth weight (BW), eviscerated organs, associated anomalies and management. To calculate mean and median values IBM SPSS v. 23.0 was used. EVIDENCE SYNTHESIS: After de-duplication and review search revealed 42 articles of which 23 met the inclusion criteria with 396 giant-omphaloceles for this analysis. Median gestational age (GA) was 36 weeks for all neonates (range 21-41); 21 neonates were reported as premature with median GA 33.5 (range 21-36). Overall median birth weight (BW) was 3100 g (range 1100-4100 g). The diameter of abdominal wall defect was 4-15 cm with the average size of 7.6 cm except for non-giant giant omphaloceles (N.=7) where the defect was measuring between 2.7 and 4 cm. Amniotic sac contents beside intestines included liver (N.=154), stomach (N.=11), spleen (N.=2), pancreas (N.=1), gallbladder (N.=5), and 5 giant omphaloceles were reported to contain only liver; sac was ruptured in 22. Giant omphaloceles were associated with a variety of other anomalies, most often with cardiac anomalies (N.=93; 23.4%) and pulmonary hypoplasia and/or pulmonary hypertension (N.=39; 9.8%). Management included conservative treatment N.=264 (66.6%), primary closure (N.=17; 4.3%), staged closures (N.=98; 24.7%) primary or staged closure (N.=17; 4.3%). The most frequent complication was sepsis (N.=52). There were 90 (22.7%) lethal outcomes, 6 lethal outcomes in neonates even before final closure could be achieved and 12 in prematures. Leading cause of mortality was sepsis (N.=51; 56.6%), the cause of lethal outcome was not reported in 8 cases. CONCLUSIONS: Giant-omphaloceles have a lethal outcome in one-fifth of neonates. Predictors of mortality included pulmonary hypoplasia and respiratory failure with prematurity and ruptured sacs implicated within this group. Sepsis was the independent iatrogenic factor in mortality.

Asynchronus bilateral ovarian torsions in girls-systematic review.

BACKGROUND: Bilateral ovarian torsions with complete loss of ovaries is devastating. This study analyzed the literature on bilateral ovarian torsions in girls to evaluate surgical options and outcomes. METHODS: Literature was searched on Pubmed® (1987-2014) using terms "bilateral", "adnexal", "ovary", "torsion" and "children". Data were collected on age, surgical preference, pathology and outcomes. RESULTS: Thirteen articles were identified, and 9 met the inclusion criteria (5 case reports, 4 original articles); and analyzed 17 girls (mean age: 8.75 years, range: 1-16). Bilateral oophorectomies (n=4), ipsilateral oophorectomy of severely affected ovary and contralateral oophoropexy (n=10), and detorsion of bilateral ovaries and bilateral oophoropexy (n=3) were performed. One torsion recurrence occurred after two oophoropexies. Laparoscopy and open surgery was done in 2 and 15 girls, respectively. Considering etiology, there were simple tubo-ovarian torsions (n=8), polycystic ovary (n=1), polycystic ovary associated with Down syndrome (n=1) and corpus luteum cyst (n=1). No tumors were reported. Serial ultrasound follow-ups of ipsilateral oophorectomy and contralateral oophoropexy (n=5) confirmed follicular function (n=4) and viability and position of the ovary (n=1). CONCLUSIONS: Though extremely rare, school age girls present bilateral ovarian torsion. Ipsilateral oophorectomy and contralateral detorsion with oophoropexy has been the preferred approach.

Thoracoschisis associated with Limb Body Wall Complex.

Thoracoschisis is a rare condition. A female newborn presented with right-sided thoracoschisis, associated with diaphragmatic hernia and protrusion of an accessory liver lobe through the chest wall defect along with deformity of the right forearm and hand duplication. Diagnosed as part of the limb-body wall complex (LBWC), management included resection of the exteriorized liver lobe followed by right hemidiaphragm and thoracic wall reconstruction.