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1.
Perm J ; : 1-8, 2024 May 03.
Article in English | MEDLINE | ID: mdl-38698715

ABSTRACT

OBJECTIVE: This study aimed to evaluate misinformation surrounding infertility and the COVID-19 vaccine on X (formerly known as Twitter) by analyzing the prevalence and content of this misinformation across a sample of posts on X. METHODS: This study is a retrospective review of posts on X (formerly known as tweets) from the COVID-19-TweetIDs dataset from July 2021 and November 2021. Included posts were from crucial time points in the COVID-19 vaccine discourse and contained at least one word related to COVID-19 vaccination and fertility. Posts were analyzed and categorized based on factuality, common words, and hashtags. Descriptive statistics on total followers, account verification status, and engagement were obtained. Differences between posts on X classified as factual and misinformation were examined using analysis of variance or χ2 tests. Sentiment analysis determined if post content was generally positive, neutral, or negative. RESULTS: A total of 17,418 relevant posts on X were reviewed: 11,436 from timeframe 1 (July 2021) and 5982 from timeframe 2 (December 2021). Misinformation posts rose from 29.9% in July 2021 to 45.1% in November 2021. In both timeframes, accounts sharing factual information had more followers (p < 0.001), and verified users were more likely to share accurate posts (p ≤ 0.001). Factual and misinformation posts had similar engagement. Sentiment analysis identified that real posts were more positive and misinformation posts were more negative (p < 0.001). CONCLUSIONS AND RELEVANCE: Misinformation about the COVID-19 vaccine and fertility is highly prevalent on X and threatens vaccine uptake in patients desiring future fertility. Accounts sharing factual information were likely to have more followers and be verified; therefore, verifying more physicians sharing accurate information is critical.

2.
Pediatr Res ; 93(4): 801-809, 2023 03.
Article in English | MEDLINE | ID: mdl-36202969

ABSTRACT

BACKGROUND: The accumulation of short-chain fatty acids (SCFAs) from bacterial fermentation may adversely affect the under-developed gut as observed in premature newborns at risk for necrotizing enterocolitis (NEC). This study explores the mechanism by which specific SCFA fermentation products may injure the premature newborn intestine mucosa leading to NEC-like intestinal cell injury. METHODS: Intraluminal injections of sodium butyrate were administered to 14- and 28-day-old mice, whose small intestine and stool were harvested for analysis. Human intestinal epithelial stem cells (hIESCs) and differentiated enterocytes from preterm and term infants were treated with sodium butyrate at varying concentrations. Necrosulfonamide (NSA) and necrostatin-1 (Nec-1) were used to determine the protective effects of necroptosis inhibitors on butyrate-induced cell injury. RESULTS: The more severe intestinal epithelial injury was observed in younger mice upon exposure to butyrate (p = 0.02). Enterocytes from preterm newborns demonstrated a significant increase in sensitivity to butyrate-induced cell injury compared to term newborn enterocytes (p = 0.068, hIESCs; p = 0.038, differentiated cells). NSA and Nec-1 significantly inhibited the cell death induced by butyrate. CONCLUSIONS: Butyrate induces developmental stage-dependent intestinal injury that resembles NEC. A primary mechanism of cell injury in NEC is necroptosis. Necroptosis inhibition may represent a potential preventive or therapeutic strategy for NEC. IMPACT: Butyrate induces developmental stage-dependent intestinal injury that resembles NEC. A primary mechanism of cell injury caused by butyrate in NEC is necroptosis. Necroptosis inhibitors proved effective at significantly ameliorating the enteral toxicity of butyrate and thereby suggest a novel mechanism and approach to the prevention and treatment of NEC in premature newborns.


Subject(s)
Enterocolitis, Necrotizing , Infant, Newborn , Animals , Mice , Humans , Enterocolitis, Necrotizing/chemically induced , Enterocolitis, Necrotizing/prevention & control , Enterocolitis, Necrotizing/drug therapy , Butyric Acid/pharmacology , Butyric Acid/metabolism , Butyric Acid/therapeutic use , Necroptosis , Intestinal Mucosa/metabolism , Intestines
3.
Nutrients ; 14(17)2022 Aug 28.
Article in English | MEDLINE | ID: mdl-36079804

ABSTRACT

Objective: To assess the longitudinal metabolic patterns during the evolution of bronchopulmonary dysplasia (BPD) development. Methods: A case-control dataset of preterm infants (<32-week gestation) was obtained from a multicenter database, including 355 BPD cases and 395 controls. A total of 72 amino acid (AA) and acylcarnitine (AC) variables, along with infants' calorie intake and growth outcomes, were measured on day of life 1, 7, 28, and 42. Logistic regression, clustering methods, and random forest statistical modeling were utilized to identify metabolic variables significantly associated with BPD development and to investigate their longitudinal patterns that are associated with BPD development. Results: A panel of 27 metabolic variables were observed to be longitudinally associated with BPD development. The involved metabolites increased from 1 predominant different AC by day 7 to 19 associated AA and AC compounds by day 28 and 16 metabolic features by day 42. Citrulline, alanine, glutamate, tyrosine, propionylcarnitine, free carnitine, acetylcarnitine, hydroxybutyrylcarnitine, and most median-chain ACs (C5:C10) were the most associated metabolites down-regulated in BPD babies over the early days of life, whereas phenylalanine, methionine, and hydroxypalmitoylcarnitine were observed to be up-regulated in BPD babies. Most calorie intake and growth outcomes revealed similar longitudinal patterns between BPD cases and controls over the first 6 weeks of life, after gestational adjustment. When combining with birth weight, the derived metabolic-based discriminative model observed some differences between those with and without BPD development, with c-statistics of 0.869 and 0.841 at day 7 and 28 of life on the test data. Conclusions: The metabolic panel we describe identified some metabolic differences in the blood associated with BPD pathogenesis. Further work is needed to determine whether these compounds could facilitate the monitoring and/or investigation of early-life metabolic status in the lung and other tissues for the prevention and management of BPD.


Subject(s)
Bronchopulmonary Dysplasia , Birth Weight , Case-Control Studies , Gestational Age , Humans , Infant , Infant, Newborn , Infant, Premature
4.
Surgery ; 171(1): 155-159, 2022 01.
Article in English | MEDLINE | ID: mdl-34924179

ABSTRACT

BACKGROUND: The Afirma Genomic Sequencing Classifier uses whole transcriptome RNA sequencing to identify thyroid nodules as benign or suspicious. The Afirma Xpression Atlas became available in 2018 and reports findings across 593 genes, including 905 variants and 235 fusions. When an alteration is identified, its risk of malignancy and associated neoplasm type is listed. We report the results of Afirma Xpression Atlas testing at our institution during its first 2 years of clinical use. METHODS: All patient charts with indeterminate thyroid nodules and Afirma Xpression Atlas results at our institution were reviewed. Thyroid nodule characteristics, cytology, Afirma Genomic Sequencing Classifier results, Afirma Xpression Atlas results, and final histopathology were reported. RESULTS: Afirma Xpression Atlas was performed on 136 indeterminate nodules since May 2018, and 103 met inclusion criteria. Forty-three nodules had positive Afirma Xpression Atlas results, and of these, 83.7% were follicular cell-derived thyroid cancer on surgical histopathology. This is similar to the overall 82.5% positive predictive value among Afirma Genomic Sequencing Classifier-suspicious indeterminate nodules during the same time period. Of the 60 nodules with negative Afirma Xpression Atlas, 73.3% were follicular cell-derived thyroid cancer on surgical histopathology. CONCLUSION: Afirma Xpression Atlas positivity is predictive of follicular cell-derived thyroid cancer, but its positive predictive value is similar to that of Genomic Sequencing Classifier-suspicious results alone at our institution, which is higher than previously published. Specific mutations likely predict follicular cell-derived thyroid cancer with higher accuracy, but our current sample size of any given mutation is too small to evaluate this further. Larger studies are needed to determine whether Afirma Xpression Atlas results predictably inform the risk of malignancy and tumor characteristics in thyroid nodules.


Subject(s)
Adenocarcinoma, Follicular/diagnosis , Biomarkers, Tumor/genetics , Gene Expression Profiling/methods , Thyroid Neoplasms/diagnosis , Thyroid Nodule/diagnosis , Adenocarcinoma, Follicular/genetics , Adenocarcinoma, Follicular/pathology , Biopsy, Fine-Needle , Data Accuracy , Diagnosis, Differential , Feasibility Studies , Female , Gene Expression Profiling/statistics & numerical data , Humans , Male , Middle Aged , Mutation , Predictive Value of Tests , Retrospective Studies , Thyroid Gland/pathology , Thyroid Neoplasms/genetics , Thyroid Neoplasms/pathology , Thyroid Nodule/genetics , Thyroid Nodule/pathology
6.
Nutrients ; 12(5)2020 Apr 30.
Article in English | MEDLINE | ID: mdl-32365850

ABSTRACT

Necrotizing Enterocolitis (NEC) is associated with prematurity, enteral feedings, and enteral dysbiosis. Accordingly, we hypothesized that along with nutritional variability, metabolic dysfunction would be associated with NEC onset. Methods: We queried a multicenter longitudinal database that included 995 preterm infants (<32 weeks gestation) and included 73 cases of NEC. Dried blood spot samples were obtained on day of life 1, 7, 28, and 42. Metabolite data from each time point included 72 amino acid (AA) and acylcarnitine (AC) measures. Nutrition data were averaged at each of the same time points. Odds ratios and 95% confidence intervals were calculated using samples obtained prior to NEC diagnosis and adjusted for potential confounding variables. Nutritional and metabolic data were plotted longitudinally to determine relationship to NEC onset. Results: Day 1 analyte levels of alanine, phenylalanine, free carnitine, C16, arginine, C14:1/C16, and citrulline/phenylalanine were associated with the subsequent development of NEC. Over time, differences in individual analyte levels associated with NEC onset shifted from predominantly AAs at birth to predominantly ACs by day 42. Subjects who developed NEC received significantly lower weight-adjusted total calories (p < 0.001) overall, a trend that emerged by day of life 7 (p = 0.020), and persisted until day of life 28 (p < 0.001) and 42 (p < 0.001). Conclusion: Premature infants demonstrate metabolic differences at birth. Metabolite abnormalities progress in parallel to significant differences in nutritional delivery signifying metabolic dysfunction in premature newborns prior to NEC onset. These observations provide new insights to potential contributing pathophysiology of NEC and opportunity for clinical care-based prevention.


Subject(s)
Amino Acids/metabolism , Enterocolitis, Necrotizing/etiology , Infant Nutritional Physiological Phenomena/physiology , Infant, Premature/metabolism , Metabolic Diseases/etiology , Nutrition Disorders/etiology , Nutritional Status , Data Analysis , Databases as Topic , Enterocolitis, Necrotizing/prevention & control , Female , Humans , Infant, Newborn , Longitudinal Studies , Male , Metabolic Diseases/metabolism , Multicenter Studies as Topic , Nutrition Disorders/metabolism
7.
Best Pract Res Clin Endocrinol Metab ; 34(3): 101408, 2020 05.
Article in English | MEDLINE | ID: mdl-32265101

ABSTRACT

Adrenocortical carcinoma (ACC) is a rare malignancy that is frequently asymptomatic at presentation, yet has a high rate of metastatic disease at the time of diagnosis. Prognosis is overall poor, particularly with cortisol-producing tumors. While the treatment of ACC is guided by stage of disease, complete surgical resection is the most important step in the management of patients with primary, recurrent, or metastatic ACC. Triphasic chest, abdomen, and pelvis computer tomography (CT) scans and 18F flourodeoxyglucose positron emission tomography CT scanning are essential for accurate staging; moreover, MRI may be helpful to identify liver metastasis and evaluate the involvement of adjacent organs for operative planning. Surgical resection with negative margins is the single most important prognostic factor for survival in patients with ACC. To achieve the highest rate of R0 resection, open adrenalectomy is the gold standard surgical approach for confirmed or highly suspected ACC. It is extremely important that the tumor capsule is not ruptured, regardless of the surgical approach used. The best post-operative outcomes (complications and oncologic) are achieved by high-volume surgeons practicing at high-volume centers.


Subject(s)
Adrenal Cortex Neoplasms/surgery , Adrenalectomy/methods , Adrenocortical Carcinoma/surgery , Adrenal Cortex Neoplasms/diagnosis , Adrenal Cortex Neoplasms/pathology , Adrenalectomy/standards , Adrenocortical Carcinoma/diagnosis , Adrenocortical Carcinoma/pathology , Humans , Liver Neoplasms/secondary , Magnetic Resonance Imaging , Neoplasm Metastasis , Neoplasm Staging , Positron Emission Tomography Computed Tomography , Prognosis , Time Factors , Tomography, X-Ray Computed
8.
J Gen Intern Med ; 34(11): 2536-2541, 2019 11.
Article in English | MEDLINE | ID: mdl-31520229

ABSTRACT

BACKGROUND: Patient satisfaction with healthcare is associated with clinical outcomes, provider satisfaction, and success of healthcare organizations. As the clinic day progresses, provider fatigue, deterioration with communication within the care team, and appointment spillover may decrease patient experience. OBJECTIVE: To understand the relationship between likelihood to recommend a primary care practice and scheduled appointment time across multiple practice settings. DESIGN: Retrospective cohort. PARTICIPANTS: A retrospective cohort was created of all patients seen within four primary care practices between July 1, 2016, and September 30, 2017. MAIN MEASURES: We looked at scheduled appointment time against patient likelihood to recommend a practice as a measure of overall patient experience collected routinely for clinical practice improvement by the Press Ganey Medical Practice Survey®. Adjusted mixed effects logistic regression models were created to understand the relationship between progressing appointment time on patient likelihood to recommend a practice. We constructed locally weighted smoothing (LOESS) curves to understand how reported patient experience varied over the clinic day. RESULTS: We had a response rate of 14.0% (n = 3172), 80.2% of whom indicated they would recommend our practice to others. Appointment time scheduling during the last hour (4:00-4:59 PM) had a 45% lower odds of recommending our practice when compared to the first clinic hour (adjusted OR = 0.55, 95% CI 0.35-0.86) which is similar when controlling for patient-reported wait time (aOR = 0.59, 95% CI 0.37-0.95). LOESS plots demonstrated declining satisfaction with subsequent appointment times compared with the first session hour, with no effect just after the lunch hour break. CONCLUSIONS: In primary care, appointment time of day is associated with patient-reported experience.


Subject(s)
Patient Satisfaction/statistics & numerical data , Primary Health Care/statistics & numerical data , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Office Visits/statistics & numerical data , Primary Health Care/standards , Retrospective Studies , Surveys and Questionnaires , Time Factors
9.
BMC Pediatr ; 19(1): 172, 2019 May 28.
Article in English | MEDLINE | ID: mdl-31138158

ABSTRACT

Following publication of the original article [1], the authors reported error on the images/figures used which also resulted in un-sequential order. The updated figures and captions are provided below.

10.
BMC Pediatr ; 19(1): 93, 2019 04 05.
Article in English | MEDLINE | ID: mdl-30953480

ABSTRACT

BACKGROUND: Total colonic and small bowel aganglionosis (TCSA) occurs in less than 1% of all Hirschsprung's disease patients. Currently, the mainstay of treatment is surgery. However, in patients with TCSA, functional outcomes are often poor. A characteristic transition zone in TCSA can be difficult to identify which may complicate surgery and may often require multiple operations. CASE PRESENTATION: We present the case of a male infant who was diagnosed with biopsy-proven total colonic aganglionosis with extensive small bowel involvement as a neonate. The patient was diverted at one month of age based on leveling biopsies at 10 cm from the Ligament of Treitz. At 7 months of age, during stoma revision for a prolapsed stoma, intra-operative peristalsis was observed in nearly the entire length of the previously aganglionic bowel, and subsequent biopsies demonstrated the appearance of mature ganglion cells in a previously aganglionic segment. CONCLUSIONS: TCSA remains a major challenge for pediatric surgeons. Our case introduces new controversy to our understanding of aganglionosis. Our observations warrant further research into the possibility of post-natal ganglion maturation and encourage surgeons to consider a more conservative surgical approach.


Subject(s)
Ganglia/pathology , Hirschsprung Disease/surgery , Intestine, Small/innervation , Biopsy , Colon/abnormalities , Colon/pathology , Colon/surgery , Hirschsprung Disease/pathology , Humans , Infant, Newborn , Intestinal Diseases/diagnostic imaging , Intestinal Diseases/pathology , Intestine, Small/diagnostic imaging , Intestine, Small/pathology , Intestine, Small/surgery , Male , Radiography
11.
Pediatr Surg Int ; 34(12): 1293-1298, 2018 Dec.
Article in English | MEDLINE | ID: mdl-30317375

ABSTRACT

BACKGROUND/PURPOSE: The proposed benefits of laparoscopic inguinal hernia repair in the pediatric population include easier access to the contralateral groin and avoidance of manipulation of the spermatic cord; however, some studies also report higher recurrence rates. Due to these differences, the traditional open technique is still used by many pediatric surgeons. The objective of this study is to compare the outcomes of two institutions that employed different techniques. METHODS: We retrospectively reviewed pediatric patients who had open repair of inguinal hernias at hospital A or laparoscopic repair at hospital B. Data collection included age of patients, laterality, operative time, and complications. RESULTS: From 2010 to 2015, 154 patients underwent open repair at hospital A and 220 patients underwent laparoscopic repair at hospital B. The mean operative time was 52 min for the open technique and 23 min for the laparoscopic technique (p < 0.01). There were 2.6% complications and 0.65% recurrences with the open technique, compared to 4.6% complications and 2.7% recurrences with the laparoscopic technique (p > 0.2). CONCLUSION: Laparoscopic hernia repairs at hospital B are associated with shorter operative times and have similar outcomes as open repairs at hospital A. A prospective study with both techniques done at the same institution is warranted.


Subject(s)
Hernia, Inguinal/surgery , Herniorrhaphy/methods , Laparoscopy/methods , Child, Preschool , Female , Humans , Infant , Male , Operative Time , Recurrence , Retrospective Studies , Treatment Outcome
12.
Mayo Clin Proc ; 93(10): 1431-1439, 2018 10.
Article in English | MEDLINE | ID: mdl-30244811

ABSTRACT

OBJECTIVE: To evaluate the impact of opioid controlled substance agreements (CSAs) enrollment on health care utilization. PATIENTS AND METHODS: We retrospectively evaluated health care utilization changes among 772 patients receiving long-term opioid therapy for chronic noncancer pain enrolled in a CSA between July 1, 2015, and December 31, 2015. We ascertained patient characteristics and utilization 12 months before and after CSA enrollment. Decreased utilization was defined as a decrease of 1 or more hospitalizations or emergency department visits and 3 or more outpatient primary and specialty care visits. Multivariate modeling assessed demographic characteristics associated with utilization changes. RESULTS: The 772 patients enrolled in an opioid CSA during the study period had a mean ± SD age of 63.5±14.9 years and were predominantly female, white, and married. The CSA enrollment was associated with decreased outpatient primary care visits (odds ratio [OR], 0.16; 95% CI, 0.14-0.19) and increased diagnostic radiology services (OR, 1.22; 95% CI, 1.02-1.47). After CSA enrollment, patients with greater comorbidity (Charlson Comorbidity Index score >3) were more likely to have reduced hospitalizations (adjusted OR, 2.8; 95% CI, 1.3-6.0; P=.008), reduced outpatient primary care visits (adjusted OR, 2.0; 95% CI, 1.2-3.2; P=.005), and reduced specialty care visits (adjusted OR, 2.0; 95% CI, 1.2-3.3; P=.006). CONCLUSION: For patients receiving long-term opioid therapy for chronic noncancer pain, CSA enrollment is associated with reductions in primary care visits and increased radiologic service utilization. Patients with greater comorbidity were more likely to have reductions in hospitalizations, outpatient primary care visits, and outpatient specialty clinic visits after CSA enrollment. The observational nature of the study does not allow the conclusion that CSA implementation is the primary reason for these observed changes.


Subject(s)
Analgesics, Opioid/administration & dosage , Chronic Pain/drug therapy , Hospitalization/statistics & numerical data , Long-Term Care/statistics & numerical data , Patient Acceptance of Health Care/statistics & numerical data , Primary Health Care/statistics & numerical data , Aged , Chronic Pain/diagnosis , Chronic Pain/epidemiology , Chronic Pain/etiology , Comorbidity , Controlled Before-After Studies , Controlled Substances/administration & dosage , Female , Humans , Male , Middle Aged , Outpatients/statistics & numerical data , United States/epidemiology
13.
J Laparoendosc Adv Surg Tech A ; 28(5): 617-621, 2018 May.
Article in English | MEDLINE | ID: mdl-29446701

ABSTRACT

PURPOSE: Benign masses of the eyebrow and forehead are common in pediatric patients and can result in facial asymmetry, discomfort, or super-infection. Excision is classically conducted via an incision directly over the mass, which can produce sub-optimal cosmesis. Recently, an endoscopic approach using pediatric brow-lift equipment has been adopted. We reviewed our center's experience with endoscopic removal of benign facial lesions and compared these cases with an equivalent series of open cases. MATERIALS AND METHODS: A retrospective chart review was conducted to identify pediatric cases of endoscopic and open removal of benign eyebrow or forehead lesions at our institution from 2009 to 2016. Clinical and cosmetic outcomes were reviewed. RESULTS: A total of 40 endoscopic and 25 open cases of excision of benign facial lesions in children were identified. For the patients who underwent endoscopic excision, the majority (85%) presented with a cyst located at the eyebrow. Histologic examination revealed 36 dermoid cysts (90%), 2 epidermal cysts, and 2 pilomatrixomas. Of the 36 cases with post-operative follow-up, 32 patients (89%) had an uncomplicated recovery with good cosmesis. Two patients had an eyebrow droop that resolved without intervention. One patient had localized numbness overlying the site, but no motor deficits. One patient presented with a recurrent dermoid cyst that required open resection. For the patients who underwent open excision, the majority (52%) had dermoid cysts located at the eyebrow. Of the 22 cases with follow-up, 20 of the patients had an uncomplicated recovery (90%). Comparing the rate of complications, there was no statistically significant difference between the two groups (P = 1.0). CONCLUSION: Endoscopic excision of benign forehead and eyebrow lesions in pediatric patients is feasible and yields excellent cosmetic results. When compared with open excision, complication rates are similar between both approaches and a facial scar can be avoided with an endoscopic approach.


Subject(s)
Dermoid Cyst/surgery , Endoscopy/methods , Facial Neoplasms/surgery , Hair Diseases/surgery , Pilomatrixoma/surgery , Skin Neoplasms/surgery , Adolescent , Child , Child, Preschool , Cicatrix/etiology , Endoscopy/adverse effects , Epidermal Cyst/surgery , Eyebrows , Female , Forehead , Humans , Infant , Infant, Newborn , Male , Postoperative Complications/etiology , Retrospective Studies , Treatment Outcome
14.
J Pediatr Surg ; 53(8): 1532-1536, 2018 Aug.
Article in English | MEDLINE | ID: mdl-28827050

ABSTRACT

BACKGROUND: Achalasia is a primary esophageal motility disorder characterized by aperistalsis of the esophagus and failed relaxation of the lower esophageal sphincter that presents rarely in childhood. The peroral endoscopic myotomy (POEM) procedure is an emerging treatment for achalasia in adults that has recently been introduced into pediatric surgical practice. METHODS: This is a prospective case series of all children referred to Stanford University Lucile Packard Children's Hospital with manometry-confirmed achalasia who underwent a POEM procedure from 2014 to 2016. RESULTS: We enrolled 10 subjects ranging in age from 7 to 17years (M=13.4). The mean pre- and 1-month post-procedure Eckardt scores were 7 (SD=2.5) and 2.4 (SD=2) (p<0.001), respectively. The median procedure time for the entire cohort was 142min (range 60-259min) with ongoing improvement with increased experience (R2=0.6, p=0.008). There were no major adverse events. CONCLUSION: The POEM procedure can be successfully completed in children for the treatment of achalasia with demonstrated short-term post-operative improvement in symptoms. The adoption of advanced endoscopic techniques by pediatric surgeons may enable development of unique intraluminal approaches to congenital anomalies and other childhood diseases. LEVEL OF EVIDENCE: Treatment Study - Level IV.


Subject(s)
Esophageal Achalasia/surgery , Myotomy/methods , Natural Orifice Endoscopic Surgery/methods , Adolescent , Child , Esophageal Sphincter, Lower/surgery , Esophagoscopy/methods , Female , Hospitals, Pediatric , Humans , Male , Manometry/methods , Mouth/surgery , Prospective Studies , Treatment Outcome
15.
Pediatr Surg Int ; 34(1): 113-116, 2018 Jan.
Article in English | MEDLINE | ID: mdl-29170900

ABSTRACT

We present the case of a 14-year-old male with a history of small bowel transplantation for long segment Hirschsprung's disease who underwent Duhamel ileorectal pull-through procedure. In post-transplant, the patient had no restrictions and was not TPN-dependent. To improve his quality of life, he and his family were interested in closing the ileostomy and undergoing pull-through surgery. The complexity of the case includes the presence of an aganglionic rectal segment-a short root of the mesentery due to the small bowel transplant-and significant immunosuppression. At the moment, he is continent, doing well, and has not had any remarkable complications.


Subject(s)
Digestive System Surgical Procedures , Hirschsprung Disease/surgery , Ileostomy , Intestine, Small/transplantation , Adolescent , Humans , Immunosuppressive Agents/therapeutic use , Male
16.
J Pediatr Surg ; 53(3): 503-507, 2018 Mar.
Article in English | MEDLINE | ID: mdl-28549685

ABSTRACT

BACKGROUND: Minimal access surgery (MAS) has gained popularity in infants less than 5kg, however, significant challenges still arise in very low weight infants. STUDY DESIGN: A retrospective chart review was performed to identify all infants weighing less than 3kg who underwent an advanced MAS or equivalent open procedure from 2009 to 2016. Advanced case types included Nissen fundoplication, duodenal atresia repair, Ladd procedure, congenital diaphragmatic hernia repair, esophageal atresia/tracheoesophageal fistula repair, diaphragmatic plication, and pyloric atresia repair. A comparative analysis was performed between the MAS and open cohorts. RESULTS: A total of 45 advanced MAS cases and 17 open cases met the inclusion criteria. Gestational age and age at operation were similar between the cohorts, while infants who underwent open procedures had significantly lower weight at operation (p=0.003). There were no deaths within 30days related to surgery in either group. Only 3 MAS cases required unintended conversion to open. There were 2 (4.4%) postoperative complications related to surgery in the MAS cohort and 2 (11.8%) in the open cohort. CONCLUSION: Advanced MAS may be performed in infants weighing less than 3kg with low mortality, acceptable rates of conversion, and similar rates of complications as open procedures. TYPE OF STUDY: Prognosis study. LEVEL OF EVIDENCE: Level III.


Subject(s)
Birth Weight , Minimally Invasive Surgical Procedures , Diaphragm/surgery , Esophageal Atresia/surgery , Fundoplication/adverse effects , Gestational Age , Hernias, Diaphragmatic, Congenital/surgery , Humans , Infant , Infant, Newborn , Intestinal Atresia/surgery , Minimally Invasive Surgical Procedures/adverse effects , Postoperative Complications , Pylorus/abnormalities , Pylorus/surgery , Retrospective Studies , Tracheoesophageal Fistula/surgery
17.
Pediatr Surg Int ; 33(5): 631-635, 2017 May.
Article in English | MEDLINE | ID: mdl-28160093

ABSTRACT

We present the case of a 15-year-old female with a right perineal mass that was found to be pleomorphic myxoid liposarcoma, a recently recognized, rare subtype of liposarcoma. The patient had a strong family history of malignancy and genetic screening revealed a pathogenic TP53 mutation consistent with Li-Fraumeni syndrome.


Subject(s)
Genital Neoplasms, Female/diagnostic imaging , Genital Neoplasms, Female/surgery , Li-Fraumeni Syndrome/complications , Liposarcoma, Myxoid/complications , Liposarcoma, Myxoid/surgery , Adolescent , Female , Humans , Perineum/diagnostic imaging , Perineum/surgery , Tomography, X-Ray Computed
18.
J Pediatr ; 181: 80-85.e1, 2017 02.
Article in English | MEDLINE | ID: mdl-27836286

ABSTRACT

OBJECTIVE: To evaluate the association between newborn acylcarnitine profiles and the subsequent development of necrotizing enterocolitis (NEC) with the use of routinely collected newborn screening data in infants born preterm. STUDY DESIGN: A retrospective cohort study was conducted with the use of discharge records for infants born preterm admitted to neonatal intensive care units in California from 2005 to 2009 who had linked state newborn screening results. A model-development cohort of 94 110 preterm births from 2005 to 2008 was used to develop a risk-stratification model that was then applied to a validation cohort of 22 992 births from 2009. RESULTS: Fourteen acylcarnitine levels and acylcarnitine ratios were associated with increased risk of developing NEC. Each log unit increase in C5 and free carnitine /(C16 + 18:1) was associated with a 78% and a 76% increased risk for developing NEC, respectively (OR 1.78, 95% CI 1.53-2.02, and OR 1.76, 95% CI 1.51-2.06). Six acylcarnitine levels, along with birth weight and total parenteral nutrition, identified 89.8% of newborns with NEC in the model-development cohort (area under the curve 0.898, 95% CI 0.889-0.907) and 90.8% of the newborns with NEC in the validation cohort (area under the curve 0.908, 95% CI 0.901-0.930). CONCLUSIONS: Abnormal fatty acid metabolism was associated with prematurity and the development of NEC. Metabolic profiling through newborn screening may serve as an objective biologic surrogate of risk for the development of disease and thus facilitate disease-prevention strategies.


Subject(s)
Carnitine/analogs & derivatives , Enterocolitis, Necrotizing/diagnosis , Enterocolitis, Necrotizing/metabolism , Infant, Premature , Biomarkers/analysis , California , Carnitine/analysis , Carnitine/blood , Cohort Studies , Confidence Intervals , Enterocolitis, Necrotizing/epidemiology , Female , Follow-Up Studies , Gestational Age , Humans , Incidence , Infant, Newborn , Intensive Care Units, Neonatal , Male , Multivariate Analysis , Neonatal Screening/methods , Odds Ratio , Reproducibility of Results , Retrospective Studies , Risk Assessment , Vulnerable Populations
19.
Pediatr Surg Int ; 33(3): 341-345, 2017 Mar.
Article in English | MEDLINE | ID: mdl-27873010

ABSTRACT

PURPOSE: The use of general anesthesia in young children has come under increasing scrutiny due to its potential long-term neurotoxic effects. Meanwhile, regional anesthesia for surgical procedures in neonates has many advantages, including preservation of respiratory status and faster return to feeding. We describe the successful use of 3% 2-chloroprocaine administered via continuous caudal infusion as the sole anesthetic agent during elective surgical procedures in infants. METHODS: A retrospective chart review of all patients who underwent elective surgical procedures under continuous caudal regional anesthetic at a single institution was performed. Thirty patients (27 males, three females) were identified: 28 patients underwent inguinal hernia repairs. Caudal anesthesia was established via continuous infusion of 3% 2-chloroprocaine through an indwelling catheter. RESULTS: Successful analgesia by regional block alone was achieved in all patients for the duration of each surgical procedure without need for rescue anesthesia. Mean operative time was 49 min. Patients were able to return to feeding immediately after surgery and were ready for discharge home within that day. CONCLUSION: Continuous caudal infusion of chloroprocaine is a safe and effective way to maintain adequate analgesia for elective surgeries in infants. This successful regional approach obviates the use of general anesthetic which reduces post-operative recovery time and avoids concerns for neurotoxicity.


Subject(s)
Anesthesia, Conduction/methods , Hernia, Inguinal/surgery , Procaine/analogs & derivatives , Anesthesia, Caudal/methods , Anesthetics, Local/administration & dosage , Female , Humans , Infant, Newborn , Infusions, Intravenous , Male , Procaine/administration & dosage , Retrospective Studies
20.
J Laparoendosc Adv Surg Tech A ; 26(5): 413-7, 2016 May.
Article in English | MEDLINE | ID: mdl-26913816

ABSTRACT

INTRODUCTION: Surgical intervention for duodenal atresia most commonly entails duodenoduodenostomy in the neonatal period. Occasionally, type I duodenal atresia with incomplete obstruction may go undiagnosed until later in life. Endoscopic approach to dividing intestinal webs has been reported as successful in patients as young as 7 days of age, and can be a useful modality particularly in patients with comorbidities who may not tolerate open or laparoscopic surgery. METHODS: A 2-year-old female with a history of trisomy 21 and tetralogy of Fallot underwent laparoscopic and endoscopic exploration of intestinal obstruction as seen on upper gastrointestinal series for symptoms of recurrent emesis and weight loss. After laparoscopy confirmed a duodenal web as the cause of intestinal obstruction, endoscopic division of the membrane was carried out with a triangle tip electrocautery knife and 15 mm radially dilating balloon. RESULTS: The patient tolerated the procedure well, and also tolerated full age-appropriate diet by time of discharge on postoperative day 2. She remains asymptomatic as of 6 months postoperatively. CONCLUSIONS: This report describes a successful endoscopic approach for definitive treatment of a duodenal web in a 2-year-old girl with trisomy 21, and laparoscopy confirmed no intraabdominal obstructive process or complication from endoscopy. Endoscopy enables minimal recovery time and suggests an improved method of duodenal web division over pure surgical intervention.


Subject(s)
Down Syndrome/diagnosis , Duodenal Obstruction/etiology , Laparoscopy/methods , Anastomosis, Surgical , Child, Preschool , Duodenal Obstruction/complications , Duodenal Obstruction/diagnosis , Duodenal Obstruction/surgery , Duodenum/surgery , Female , Fluoroscopy , Humans , Intestinal Atresia , Postoperative Period , Stomach/surgery
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