Global variations in funding and use of hemodialysis accesses: an international report using the ISN Global Kidney Health Atlas.

BACKGROUND: There is a lack of contemporary data describing global variations in vascular access for hemodialysis (HD). We used the third iteration of the International Society of Nephrology Global Kidney Health Atlas (ISN-GKHA) to highlight differences in funding and availability of hemodialysis accesses used for initiating HD across world regions. METHODS: Survey questions were directed at understanding the funding modules for obtaining vascular access and types of accesses used to initiate dialysis. An electronic survey was sent to national and regional key stakeholders affiliated with the ISN between June and September 2022. Countries that participated in the survey were categorized based on World Bank Income Classification (low-, lower-middle, upper-middle, and high-income) and by their regional affiliation with the ISN. RESULTS: Data on types of vascular access were available from 160 countries. Respondents from 35 countries (22% of surveyed countries) reported that > 50% of patients started HD with an arteriovenous fistula or graft (AVF or AVG). These rates were higher in Western Europe (n = 14; 64%), North & East Asia (n = 4; 67%), and among high-income countries (n = 24; 38%). The rates of > 50% of patients starting HD with a tunneled dialysis catheter were highest in North America & Caribbean region (n = 7; 58%) and lowest in South Asia and Newly Independent States and Russia (n = 0 in both regions). Respondents from 50% (n = 9) of low-income countries reported that > 75% of patients started HD using a temporary catheter, with the highest rates in Africa (n = 30; 75%) and Latin America (n = 14; 67%). Funding for the creation of vascular access was often through public funding and free at the point of delivery in high-income countries (n = 42; 67% for AVF/AVG, n = 44; 70% for central venous catheters). In low-income countries, private and out of pocket funding was reported as being more common (n = 8; 40% for AVF/AVG, n = 5; 25% for central venous catheters). CONCLUSIONS: High income countries exhibit variation in the use of AVF/AVG and tunneled catheters. In low-income countries, there is a higher use of temporary dialysis catheters and private funding models for access creation.

Effect of metformin adjunct therapy on cardiometabolic parameters in Indian adolescents with type 1 diabetes: a randomized controlled trial.

Introduction: Insulin resistance is being increasingly reported in type-1 Diabetes (T1D) and is known to accelerate microvascular complications. The Asian Indian population has a higher risk of double diabetes development compared to Caucasians. Hence, we studied the effect of adding Metformin to standard insulin therapy on glycemic control, insulin sensitivity (IS), cardiometabolic parameters and body composition in Indian adolescents with T1D. Methods: A Randomized controlled trial was conducted spanning 9 months (Registration number:CTRI/2019/11/022126). Inclusion: Age 10-19 years, T1D duration>1year, HbA1c>8% Exclusion: Uncontrolled vascular complications/comorbidities, Metformin intolerance, concomitant drugs affecting insulin sensitivity. Participants were randomized to Metformin/Placebo (n=41 each) groups and age, sex, duration-matched. Assessments were performed at baseline, 3 and 9 months. Results: 82 participants aged 14.7 ± 3years (40 females) were enrolled, with a mean diabetes duration of 5.2 ± 2.3 years. Over 9 months, HbA1c decreased significantly by 0.8 (95% confidence interval: -1.2 to -0.3) from 9.8 ± 1.8% to 9.1 ± 1.7% on Metformin but remained largely unchanged (difference of 0.2, 95% confidence interval: -0.7 to 0.2) i.e. 9.9 ± 1.6% and 9.7 ± 2.2% on placebo. HbA1c improvement correlated negatively with baseline IS (EGDR:r= -0.3;SEARCH:r = -0.24, p<0.05) implying better HbA1c-lowering in those with decreased initial IS. CGM-based glycemic variability (standard deviation) reduced by 6.3 mg/dL (95% confidence interval: -12.9 to 0.2) from 100.2 ± 19.1 mg/dL to 93.7 ± 19.9 mg/dL in those on Metformin (p=0.05) but not placebo (94.0 ± 20.5; 90.0 ± 22.6 mg/dL). Insulin sensitivity: CACTIexa & SEARCH scores demonstrated no change with Metformin but significant worsening on placebo. Significant increase in LDL-C(42%), total cholesterol(133.6 to 151.1 mg/dL), triglyceride (60.0 to 88.0 mg/dL) and carotid intima-media thickness was noted on placebo but not Metformin. Weight, BMI, fat Z-scores increased significantly on placebo but not Metformin. Adverse events (AE) were minor; AE, compliance and safety parameters were similar between the two groups. Conclusion: Metformin as an adjunct to insulin in Asian Indian adolescents with T1D demonstrated beneficial effect on glycemic control, glycemic variability, IS, lipid profile, vascular function, weight and body fat, with a good safety profile when administered for 9 months.

The pediatric surgeon-scientist: An evolving Breed or endangered phenotype?

INTRODUCTION: National Institute of Health (NIH) funding is a "gold-standard" of achievement; we examined trends in NIH-funded pediatric surgeons. METHODS: NIH Research Portfolio Online Reporting Tools (RePORT) was queried for American Pediatric Surgical Association (APSA) members (2012 vs 2022). Demographics and time-to-award (TTA) from fellowship were compared. Number of grants, funding allotment, award classification, administering institutes/centers, research type were studied. RESULTS: Thirty-eight (4.6%) APSA members were NIH-funded in 2012 compared to 37 (2.9%) in 2022. Of funded surgeons in 2022, 27% were repeat awardees from 2012. TTA was similar (12 vs 14years, p=0.109). At each point, awards were commonly R01 grants (40 vs 52%, p â€‹= â€‹0.087) and basic science-related (76 vs 63%, p = â€‹0.179). Awardees were predominantly men (82% in 2012 vs 78% in 2022, p=0.779) and White (82% in 2012 vs 76% in 2022, p=0.586). Median amount per grant increased: $254,980 (2012) to $364,025 (2022); by $96,711 for men and $390,911 for women. Median awards for White surgeons increased by $215,699 (p=0.035), and decreased by $30,074 for non-White surgeons, though not significantly (p=0.368). CONCLUSION: The landscape of NIH-funded pediatric surgeons has remained unchanged between time points. With a substantial number of repeat awardees, predominance of R01 grants, and a median TTA over a decade after fellowship graduation, the phenotypes of early career pediatric surgeon-scientists are facing academic endangerment.

Validating a New Surgical Gouge for Autogenous Ear Reconstruction During Simulated Microtia Workshops.

Autologous ear reconstruction remains a gold standard surgical technique for the treatment of external ear deformities. This highly technical procedure requires experience, an understanding of aesthetic principles, and a surgical approach that can consistently produce optimal results. As an experienced microtia surgeon having trained under Dr Satoru Nagata, the senior author has emphasized the importance of appropriate surgical tools during this procedure. Here, we present results of a novel surgical handle and gouge meant to optimize complex cartilage carving. The senior author regularly holds microtia workshops to help train individuals around the United States. During 2 of such workshops held in 2022, participants were given access to both the standard, commercially available surgical gouge as well as a prototype of a novel surgical gouge developed by the authors. Participants were then given a Likert-scale survey to assess their subjective feedback for both tools. Twenty-seven total participants completed the postworkshop survey. Cumulatively, the results demonstrated that participants rated the custom gouge significantly higher than its counterpart (4.2 versus 3.2, P<0.001). They also had a significantly higher likelihood of using the custom gouge again (4.1 versus 3.2, P=0.023). The custom gouge designed by the senior author demonstrated higher subjective ratings when compared with what is currently available on the market. This serves as a primary validation study that demonstrates feasibility for further assessment in a true operative setting.

Assessing the Current State of Microtia Reconstruction in the United States.

OBJECTIVE: Microtia is a congenital ear deformity with variability in surgical techniques and tools across surgeons pursuing an autologous reconstruction. Different techniques have emerged over time, and surgeons opt for various tools to aid in creating the complex three-dimensional cartilaginous ear framework. The purpose of this study was to understand the current state of microtia reconstruction in the United States. METHODS: Microtia surgeons affiliated with the nonprofit, Ear Community, were invited to complete a 20-item survey. Data were collected on demographic information regarding surgeons, considerations when approaching microtia repair in patients, and techniques and comfort levels. Additional data were collected on materials, tools, flaps, and skin grafts used for reconstruction. RESULTS: Twenty-two surgeons responded to the survey reporting 3 different techniques learned and utilized in practice including the Brent, Nagata, and Firmin techniques. About two-thirds of surgeons were "extremely comfortable" with their techniques and one-third were "extremely uncomfortable" or "somewhat uncomfortable." Most respondents reported using a tunneled temporoparietal fascial flap or a posterior fascial flap along with a full-thickness skin graft for the second stage (ear elevation). Most surgeons utilized a combination of scalpels and gouges when carving the ear framework along with sutures or wire. CONCLUSIONS: This study highlights the current state of autogenous microtia reconstruction underscoring the variability in approaches and preferences. These data may guide future directions that aim to improve patient outcomes. Surgeons may gain insight into different practices and choose to adopt different aspects to enhance their surgical approach.

Mechanisms of spinophilin-dependent pancreas dysregulation in obesity.

Spinophilin is an F-actin binding and protein phosphatase 1 (PP1) targeting protein that acts as a scaffold of PP1 to its substrates. Spinophilin knockout (Spino-/-) mice have decreased fat mass, increased lean mass, and improved glucose tolerance, with no difference in feeding behaviors. While spinophilin is enriched in neurons, its roles in non-neuronal tissues, such as beta cells of the pancreatic islets, are unclear. We have corroborated and expanded upon previous studies to determine that Spino-/- mice have decreased weight gain and improved glucose tolerance in two different models of obesity. We have identified multiple putative spinophilin interacting proteins isolated from intact pancreas and observed increased interactions of spinophilin with exocrine, ribosomal, and cytoskeletal protein classes that normally act to mediate peptide hormone production, processing, and/or release in Leprdb/db and/or high fat-fed (HFF) models of obesity. Additionally, we have found that spinophilin interacts with proteins from similar classes in isolated islets, suggesting a role for spinophilin in the pancreatic islet. Consistent with a pancreatic beta cell type-specific role for spinophilin, using our recently described conditional spinophilin knockout mice, we found that loss of spinophilin specifically in pancreatic beta cells improved glucose tolerance without impacting body weight in chow-fed mice. Our data further support a role for spinophilin in mediating pathophysiological changes in body weight and whole-body metabolism associated with obesity. Our data provide the first evidence that pancreatic spinophilin protein interactions are modulated by obesity and that loss of spinophilin specifically in pancreatic beta cells impacts whole-body glucose tolerance.

Long term clinical follow up of four patients with Wolfram syndrome and urodynamic abnormalities.

OBJECTIVES: Wolfram syndrome is characterised by insulin-dependent diabetes (IDDM), diabetes insipidus (DI), optic atrophy, sensorineural deafness and neurocognitive disorders. The DIDMOAD acronym has been recently modified to DIDMOAUD suggesting the rising awareness of the prevalence of urinary tract dysfunction (UD). End stage renal disease is the commonest cause of mortality in Wolfram syndrome. We present a case series with main objective of long term follow up in four children having Wolfram syndrome with evaluation of their urodynamic profile. METHODS: A prospective follow up of four genetically proven children with Wolfram syndrome presenting to a tertiary care pediatric diabetes clinic in Pune, India was conducted. Their clinical, and urodynamic parameters were reviewed. RESULTS: IDDM, in the first decade, was the initial presentation in all the four children (three male and one female). Three children had persistent polyuria and polydipsia despite having optimum glycemic control; hence were diagnosed to have DI and treated with desmopressin. All four patients entered spontaneous puberty. All patients had homozygous mutation in WFS1 gene; three with exon 8 and one with exon 6 novel mutations. These children with symptoms of lower urinary tract malfunction were further evaluated with urodynamic studies; two of them had hypocontractile detrusor and another had sphincter-detrusor dyssynergia. Patients with hypocontractile bladder were taught clean intermittent catheterization and the use of overnight drain. CONCLUSIONS: We report a novel homozygous deletion in exon 6 of WFS-1 gene. The importance of evaluation of lower urinary tract malfunction is highlighted by our case series. The final bladder outcome in our cases was a poorly contractile bladder in three patients.

Palliative Care Training During Surgical Critical Care Fellowship: A Preliminary Needs Assessment at a Major Academic Center.

Surgical palliative care (PC) facilitates communication between surgeons and patients/family about prognosis, symptom control, and therapeutic goals. Surgical critical care (SCC) fellows are at the forefront of the intensive care team; thus, we aim to assess previous and ongoing experiences in delivering PC by surveying fellows at a large academic center. Seventeen surveys were completed in which 59% of fellows reported no previous PC education. Six fellows (35%) reported participating in goals of care/end-of-life (GOC/EOL) discussions "a few times a year" during residency, while 41% responded the same for transitioning patients to comfort-focused care (CFC). When asked if respondents felt comfortable facilitating GOC/EOL discussions prior to fellowship, 7 (41%) answered "disagree" or "strongly disagree." Most fellows reported that more training in navigating GOC/EOL discussions (88%) and transitioning patients to CFC (76%) is needed. This assessment demonstrates variability in fellows' prior PC exposure and a strong desire for more structured training.

Impact of Prior Abdominal Procedures on Peritoneal Dialysis Catheter Outcomes: Findings From the North American Peritoneal Dialysis Catheter Registry.

RATIONALE & OBJECTIVE: A history of prior abdominal procedures may influence the likelihood of referral for peritoneal dialysis (PD) catheter insertion. To guide clinical decision making in this population, this study examined the association between prior abdominal procedures and outcomes in patients undergoing PD catheter insertion. STUDY DESIGN: Retrospective cohort study. SETTING & PARTICIPANTS: Adults undergoing their first PD catheter insertion between November 1, 2011 and November 1, 2020, at 11 institutions in Canada and the US participating in the International Society for Peritoneal Dialysis (ISPD) North American Catheter Registry. EXPOSURE: Prior abdominal procedure(s), defined as any procedure that enters the peritoneal cavity. OUTCOMES: Primary outcome: time to the first of abandonment of the PD catheter, or interruption/termination of PD. SECONDARY OUTCOMES: rates of emergency room visits, hospitalizations, and procedures. ANALYTICAL APPROACH: Cumulative incidence curves were used to describe the risk over time and an adjusted Cox proportional hazards model was used to estimate the association between the exposure and primary outcome. Models for count data were used to estimate the associations between the exposure and secondary outcomes. RESULTS: A total of 855 patients met the inclusion criteria. Thirty-one percent had a history of a prior abdominal procedure and 20% experienced at least one PD catheter-related complication that led to the primary outcome. Prior abdominal procedures were not associated with an increased risk of the primary outcome [Adjusted HR 1.12 (95% CI 0.68-1.84)]. Upper abdominal procedures were associated with a higher adjusted hazard of the primary outcome, but there was no dose-response relationship concerning the number of procedures. There was no association between prior abdominal procedures and other secondary outcomes. LIMITATIONS: Observational study and cohort limited to sample of patients felt to be potential candidates for PD catheter insertion. CONCLUSION: A history of prior abdominal procedure(s) does not appear to influence catheter outcomes following PD catheter insertion. Such a history should not be a contraindication to peritoneal dialysis.

Identifying Community Safety and Policy-Based Injury Prevention Opportunities to Reduce Golf Cart Injuries.

BACKGROUND: In small US communities, golf cart utilization has become increasingly more common. In the past 3 years, the incidence and severity of pediatric golf cart-related trauma evaluated at our trauma center has noticeably increased. Thus, the aim of this study was to analyze trends, identify risk and protective factors, and provide community-level recommendations to improve golf cart safety for children in a coastal community. METHODS: A retrospective cross-sectional study of our institutional trauma registry was performed. The registry was queried for golf cart injuries between 2012 -2022. Demographics, accident details, hospital course and outcomes were reviewed. Data analysis involved quantitative statistics. Incident locations were mapped, including additional data from the County emergency medical service. Additionally, customer education at four prominent golf rental shops was observed. RESULTS: Annual golf cart-related traumas doubled starting in 2020. Of 235 total patients, 105 (46%) were children. Median age was 11.5 years (range 2-17). 55% were female and 67% were non-Hispanic White. 80% were out-of-county residents. The most common injury location was extremity (56%). Median ISS was 4, and 3% died. Only 10% of children were restrained. 41% were ejected and most (84%) were front-facing passengers. Ejection was associated with more severe injury (OR 4.13, p = 0.01). Most injuries occurred during 5-10 pm (47%), weekends and summertime. Nighttime injuries were more severe than daytime (p = 0.04). A hotspot of crashes was identified in a zone where golf carts were restricted. Rental stores provided education on seat belt use, car seat use for infants, and off-limit zones. However, rules were not enforced. CONCLUSION: Our results inform the following golf cart injury prevention opportunities: raising awareness of injury risks to children in high-tourist areas, partnering with rental stores to enforce rules, improving signage, adding protected lanes, and adopting a no nighttime operation policy. LEVEL OF EVIDENCE: IV.

Use of modern three-dimensional imaging models to guide surgical planning for local control of pediatric extracranial solid tumors.

INTRODUCTION: In complex pediatric surgical oncology, surgical planning is contingent upon data gathered from preoperative imaging. Three-dimensional (3D) modeling and printing has been shown to be beneficial for adult presurgical planning, though pediatric literature is less robust. The study reviews our institutional experience with the use of 3D image segmentation and printed models in approaching resection of extracranial solid tumors in children. METHODS: This is a single institutional series from 2021 to 2023. Models were based on computed tomography and magnetic resonance imaging studies, optimized for 3D imaging. The feasibility and creation of the models is reviewed, including specific techniques, software, and printing materials from our institution. Clinical implications for surgical planning are also described, along with detailed preoperative and intraoperative images. RESULTS: 3D modeling and printing was performed for four pediatric patients diagnosed with extracranial solid tumors. Diagnoses included Ewing sarcoma, hepatoblastoma, synovial sarcoma, and osteosarcoma. No intraoperative complications or discrepancies with the preoperative 3D-printed model were noted. No evidence of local recurrence was identified in any patient thus far. CONCLUSION: Our institutional series demonstrates a wide spectrum of clinical application for 3D modeling and printing technology within pediatric surgical oncology. This technology may aid in surgical planning for both resection and reconstruction, can be applied to a diverse breadth of diagnoses, and may potentially augment patient and/or family education about their condition.

Effect of Calcium and Vitamin D Supplementation (Dairy vs. Pharmacological) on Bone Health of Underprivileged Indian Children and Youth with Type-1 Diabetes: A Randomized Controlled Trial.

BACKGROUND: Bone health is affected by chronic childhood disorders including type-1 diabetes mellitus (T1DM). We conducted this randomized controlled trial with the objective of investigating the effect of 1-year supplementation of vitamin-D with milk or with pharmacological calcium on bone mass accrual in underprivileged Indian children and youth with T1DM. METHODS: 5 to 23year old (n = 203) underprivileged children and youth with T1DM were allocated to one of three groups: Milk (group A-received 200 ml milk + 1000 international unit (IU) vitamin-D3/day), Calcium supplement (group B-received 500 mg of calcium carbonate + 1000 IU of vitamin-D3/day) or standard of care/control (group C). Anthropometry, clinical details, biochemistry, diet (3-day 24-h recall), physical activity (questionnaires adapted for Indian children) and bone health parameters (using dual-energy X-ray absorptiometry and peripheral quantitative computed tomography- DXA and pQCT respectively) were evaluated at enrolment and end of 12 month intervention. RESULTS: Total body less head(TBLH) bone mineral content (BMC(g)) and bone mineral density (BMD(gm/cm2)) were significantly higher at end of study in girls in both supplemented groups (TBLHBMC-A-1011.8 ±â€¯307.8, B-983.2 ±â€¯352.9, C-792.8 ±â€¯346.8. TBLHBMD-A-± 0.2, B-0.8 ±â€¯0.2, C-0.6 ±â€¯0.2, p < 0.05). Z score of lumbar spine bone mineral apparent density of supplemented participants of both sexes was significantly higher than controls (Boys- A-0.7 ±â€¯1.1, B-0.6 ±â€¯1.4, C- -0.7 ±â€¯1.1; Girls- A-1.1 ±â€¯1.1, B-0.9 ±â€¯3.4, C- -1.7 ±â€¯1.3, p < 0.05). A significantly higher percentage increase was found in cortical thickness in girls in both supplemented groups (A-17.9 ±â€¯28.6, B-15.3 ±â€¯16.5, C-7.6 ±â€¯26.2); the differences remained after adjusting for confounders. CONCLUSION: Supplementation with milk or pharmacological calcium (+vitaminD3) improved bone outcomes-particularly geometry in children with T1DM with more pronounced effect in girls. Pharmacological calcium may be more cost effective in optimising bone health in T1DM in resource limited settings.

Limited English Proficiency can Negatively Impact Disease/Treatment in Children With Cancer Compared to Those Who are English Proficient-an Institutional Study.

BACKGROUND: In 2013, 25.5 million people in the United States self-identified as having limited English proficiency (LEP). LEP in adults has been associated with longer hospital stays, increased adverse events, increased emergency room visits, and decreased understanding of medications prescribed. This study aims to define the relationship between LEP and outcomes in a pediatric oncologic population. METHODS: We performed a matched case-control study utilizing data from our institutional cancer database (children

Rare cause of recurrent hypocalcaemia and functional hypoparathyroidism due to hypomagnesaemia caused by TRPM6 gene mutation.

Magnesium is essential for the functioning and release of parathyroid hormone. Therefore, its deficiency can present as functional hypoparathyroidism. This case report describes a rare inherited disorder called congenital hypomagnesaemia with secondary hypocalcaemia due to TRPM6 gene mutation. This disease clinically and biochemically mimics hypoparathyroidism. However, unlike hypoparathyroidism, it can be treated only by long-term oral magnesium supplements. The patient presented to us with recurrent hypocalcaemic convulsions. The laboratory picture in each admission was similar to that of hypoparathyroidism. However, the hypocalcaemia persisted, and it was noticed to be associated with persistent hypomagnesaemia. A defect in the tubular magnesium reabsorption was postulated and a genetic analysis of the patient was done, which revealed a TRPM6 mutation causing hypomagnesaemia by excessive renal excretion of magnesium. The child responded well to oral magnesium supplements and is currently developmentally appropriate for her age and thriving well.

Clinical Practice Patterns in Facelift Surgery: A 15-Year Review of Continuous Certification Tracer Data from the American Board of Plastic Surgery.

BACKGROUND: This study evaluates change in practice patterns in facelift surgery based on a 15-year review of tracer data collected by the American Board of Plastic Surgery as part of the Continuous Certification process. METHODS: Tracer data for facelift was reviewed from 2006 to 2021. The 15-year collection period was divided into an "early cohort (EC)" from 2006 to 2014 and a "recent cohort (RC)" from 2015 to 2021. RESULTS: Of 3400 facelifts (1710 EC/1690 RC) performed, 18% were done in hospital and 76% were done in an accredited office facility. Ninety one percent of patients were female with an average age of 61 years. There was an increase in the number of secondary facelifts (4% EC vs 18% RC; p < 0.001) and an increased number of patients concerned about volume loss/deflation (25% EC vs 37% RC; p < 0.001). The surgical approach to the SMAS involved plication (40%), flaps (35%), SMASectomy (22%) and MACS lift (6%). One percent of facelifts were subperiosteal and 8% skin-only. Significantly more surgeons used the lateral SMAS flap (14% EC vs 18% RC, p < 0.005), while less used an extended SMAS flap (21% vs 18%; p = 0.001) and MACS lift (10% EC vs 6% RC; p = 0.021) techniques. The concomitant use of facial fat grafting is becoming more common (15% EC vs 24% RC, p = 0.0001). CONCLUSIONS: A 15-year review of ABPS tracer data provides an excellent venue for the objective assessment of the current status of facelift surgery, and key changes in practice patterns during that time. LEVEL OF EVIDENCE III: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Validation of ChatGPT 3.5 as a Tool to Optimize Readability of Patient-facing Craniofacial Education Materials.

Background: To address patient health literacy, the American Medical Association recommends that readability of patient education materials should not exceed a sixth grade reading level; the National Institutes of Health recommend no greater than an eigth-grade reading level. However, patient-facing materials in plastic surgery often remain at an above-recommended average reading level. The purpose of this study was to evaluate ChatGPT 3.5 as a tool for optimizing patient-facing craniofacial education materials. Methods: Eighteen patient-facing craniofacial education materials were evaluated for readability by a traditional calculator and ChatGPT 3.5. The resulting scores were compared. The original excerpts were then inputted to ChatGPT 3.5 and simplified by the artificial intelligence tool. The simplified excerpts were scored by the calculators. Results: The difference in scores for the original excerpts between the online calculator and ChatGPT 3.5 were not significant (P = 0.441). Additionally, the simplified excerpts' scores were significantly lower than the originals (P < 0.001), and the mean of the simplified excerpts was 7.78, less than the maximum recommended 8. Conclusions: The use of ChatGPT 3.5 for simplification and readability analysis of patient-facing craniofacial materials is efficient and may help facilitate the conveyance of important health information. ChatGPT 3.5 rendered readability scores comparable to traditional readability calculators, in addition to excerpt-specific feedback. It was also able to simplify materials to the recommended grade levels. With human oversight, we validate this tool for readability analysis and simplification.

Follow up of a rare case of adrenal insufficiency due to NNT mutation.

Hypoglycaemia is one of the most common causes of convulsions in neonatal period. Repeated hypoglycaemic convulsions have to be addressed with utmost urgency to prevent its morbid sequelae. Repeated ketotic hypoglycaemia in the infantile period needs detailed endocrine evaluation. Our patient is a boy in the third year of his life, had presented in infancy with hypoglycaemic convulsions and hyperpigmentation of skin and mucous membrane. Investigations revealed ketotic hypoglycaemia, hypocortisolaemia with high adrenocorticotropic hormone (ACTH) and normal aldosterone, 17-hydroxyprogesterone (17-OHP) and testosterone levels. This suggested isolated glucocorticoid deficiency without mineralocorticoid deficiency. He responded well to hydrocortisone therapy with resolution of symptoms and normalisation of lab parameters. Genetic study confirmed the diagnosis of familial glucocorticoid deficiency (FGD) with homozygous mutation in NNT (nicotinamide nucleotide transhydrogenase) gene with a novel p.Thr578lle variant. This is the first case of FGD with NNT mutation to be reported from the Indian subcontinent.

The Association of Intra-Abdominal Adhesions with Peritoneal Dialysis Catheter-Related Complications.

BACKGROUND: This study investigated the association of intra-abdominal adhesions with the risk of peritoneal dialysis (PD) catheter complications. METHODS: Individuals undergoing laparoscopic PD catheter insertion were prospectively enrolled from eight centers in Canada and the United States. Patients were grouped based on the presence of adhesions observed during catheter insertion. The primary outcome was the composite of PD never starting, termination of PD, or the need for an invasive procedure caused by flow restriction or abdominal pain. RESULTS: Seven hundred and fifty-eight individuals were enrolled, of whom 201 (27%) had adhesions during laparoscopic PD catheter insertion. The risk of the primary outcome occurred in 35 (17%) in the adhesion group compared with 58 (10%) in the no adhesion group (adjusted HR, 1.64; 95% confidence interval [CI], 1.05 to 2.55) within 6 months of insertion. Lower abdominal or pelvic adhesions had an adjusted HR of 1.80 (95% CI, 1.09 to 2.98) compared with the no adhesion group. Invasive procedures were required in 26 (13%) and 47 (8%) of the adhesion and no adhesion groups, respectively (unadjusted HR, 1.60: 95% CI, 1.04 to 2.47) within 6 months of insertion. The adjusted odds ratio for adhesions for women was 1.65 (95% CI, 1.12 to 2.41), for body mass index per 5 kg/m 2 was 1.16 (95% CI, 1.003 to 1.34), and for prior abdominal surgery was 8.34 (95% CI, 5.5 to 12.34). Common abnormalities found during invasive procedures included PD catheter tip migration, occlusion of the lumen with fibrin, omental wrapping, adherence to the bowel, and the development of new adhesions. CONCLUSIONS: People with intra-abdominal adhesions undergoing PD catheter insertion were at higher risk for abdominal pain or flow restriction preventing PD from starting, PD termination, or requiring an invasive procedure. However, most patients, with or without adhesions, did not experience complications, and most complications did not lead to the termination of PD therapy.

Temporal Trends of Neonatal Surgical Conditions in Texas and Accessibility to Pediatric Surgical Care.

INTRODUCTION: Texas consistently accounts for approximately 10% of annual national births, the second highest of all US states. This temporal study aimed to evaluate incidences of neonatal surgical conditions across Texas and to delineate regional pediatric surgeon accessibility. METHODS: The Texas Birth Defects Registry was queried from 1999 to 2018, based on 11 well-established regions. Nine disorders (30,476 patients) were identified as being within the operative scope of pediatric surgeons: biliary atresia (BA), pyloric stenosis (PS), Hirschsprung's disease, stenosis/atresia of large intestine/rectum/anus, stenosis/atresia of small intestine, tracheoesophageal fistula/esophageal atresia, gastroschisis, omphalocele, and congenital diaphragmatic hernia. Annual and regional incidences were compared (/10,000 births). Statewide pediatric surgeons were identified through the American Pediatric Surgical Association directory. Regional incidences of neonatal disorder per surgeon were evaluated from 2010 to 2018 as a surrogate for provider disparity. RESULTS: PS demonstrated the highest incidence (14.405/10,000), while BA had the lowest (0.707/10,000). Overall, incidences of PS and BA decreased significantly, while incidences of Hirschsprung's disease and small intestine increased. Other diagnoses remained stable. Regions 2 (48.24/10,000) and 11 (47.79/10,000) had the highest incidence of neonatal conditions; Region 6 had the lowest (34.68/10,000). Three rural regions (#2, 4, 9) lacked pediatric surgeons from 2010 to 2018. Of regions with at least one surgeon, historically underserved regions (#10, 11) along the Texas-Mexico border consistently had the highest defect per surgeon rates. CONCLUSIONS: There are temporal and regional differences in incidences of neonatal conditions treated by pediatric surgeons across Texas. Improving access to neonatal care is a complex issue that necessitates collaborative efforts between state legislatures, health systems, and providers.

Development of a simplified new method of bone age estimation using three bones of the hand and wrist.

Though the Greulich and Pyle (GP) method is easy, inter-observer variability, differential maturation of hand bones influences ratings. The Tanner-Whitehouse (TW) method is more accurate, but cumbersome. A simpler method combining the above, such that it utilizes fewer bones without affecting accuracy, would be widely used and more applicable in clinical practice. OBJECTIVES: 1. Devising a simplified method utilizing three bones of the hand and wrist for bone age (BA) assessment. 2. Testing whether the 3 bone method gives comparable results to standard methods (GP,TW2,TW3) in Indian children. METHODS: Developmental stages and corresponding BA for radius, hamate, terminal phalanx (left middle finger) epiphyses combining stages from GP,TW3 atlases were described; BA were rated by two blinded observers. 3 bone method ratings were compared with the same dataset analyzed earlier using GP,TW2,TW3 (4 raters). RESULTS: Radiographs analysed:493 (Girls=226). Mean chronological age:9.4 ± 4.6 yrs, mean BA 3 bone:9.8 ± 4.8 yrs, GP:9.6 ± 4.8 yrs, TW3:9.3 ± 4.5 yrs, TW2:9.9 ± 5.0 yrs. The 3 bone method demonstrated no significant inter-observer variability (p = 0.3, mean difference = 0.02 ± 0.6 yrs); a strong positive correlation (p < 0.0001) with GP (r = 0.985), TW3 (r = 0.983) and TW2 (r = 0.982) was noted. Bland-Altman plots demonstrated good agreement; the root mean square errors between 3 bone and GP,TW3,TW2 ratings were 0.6,0.7,0.6 years; mean differences were 0.19,0.49,-0.14 years respectively. Greatest proportion of outliers (beyond ±1.96 SD of mean difference) was between 6 and 8 years age for difference in 3 bone and GP, and between 4-6 years for difference in 3 bone and TW3,TW2. CONCLUSION: The 3 bone method has multiple advantages; it is easier, tackles differential maturation of wrist and hand bones, has good reproducibility, without compromising on accuracy rendering it suitable for office practice.