The anesthetic management of a child with ohtahara syndrome and severe stridor: a case report.

BACKGROUND: Ohtahara syndrome is a progressive developmental and epileptic encephalopathy that manifests in the early infantile period. This rare condition is characterized by intractable seizures, psychomotor retardation, and poor prognosis. To date, there are a handful of case reports regarding the anesthetic management of children with Ohtahara syndrome. However, limited reports exist of patients with Ohtahara syndrome who present with difficult airways. This report describes our airway findings and general anesthetic management of a pediatric patient with Ohtahara syndrome undergoing diagnostic bronchoscopy for severe inspiratory stridor. CASE PRESENTATION: A 14-month-old, 9 kg, male patient with Ohtahara syndrome presented with a year-long history of severe inspiratory stridor and was scheduled for bronchoscopy with lavage. On exam, the patient had noisy breathing, was non-verbal with developmental delay, and had poor head control with significant central hypotonia. The patient was induced with ketamine and general anesthesia was maintained with propofol. Bronchoscopic evaluation was completed uneventfully and revealed a diagnosis of laryngotracheomalacia. The patient's breathing was maintained spontaneously throughout the procedure and no seizures were noted. In the post anesthesia care unit, the patient's respiratory and cardiovascular function were stable. CONCLUSIONS: This report documents the unusual finding of severe inspiratory stridor in a 14-month-old child diagnosed with Ohtahara syndrome and our anesthetic management during their diagnostic bronchoscopy. Currently, documentation of complex airway pathology present in patients with Ohtahara syndrome is limited and should be further evaluated. This will assist pediatric anesthesiologists as these patients may require careful preoperative assessment, thoughtful airway management, and surgical alternatives on standby.

Exploring Use of Endoscopy Simulation in North American Pediatric Gastroenterology Fellowship Training Programs.

OBJECTIVES: Increasing evidence supports simulation-based training; however, limited data exist regarding its use in pediatric gastroenterology (GI). We explored the use of simulation-based endoscopy training in pediatric GI fellowship programs across North America. METHODS: GI fellowship program directors (PDs) from the United States and Canada were surveyed between August to November 2018. The pretested, electronic survey comprised 3 sections: program demographics; details of current simulation-based training; and PDs' perceptions of endoscopy simulation. Responses were analyzed using descriptive statistics. RESULTS: Forty-three of 71 (61%) PDs responded (6 Canadian, 37 US). Programs were predominantly academic (95%) and enrolled 1.87 ±â€Š1.01 fellows/yr. Twenty-four programs (56%) reported using simulation for endoscopy training, whereas 8 (19%) used simulation for nonprocedural education. Only 2 programs (5%) used endoscopy simulation for assessment. Of those using simulation (n = 24), upper endoscopy and colonoscopy were trained most frequently, and mechanical simulators were used most commonly. Eight programs (33%) required simulation training prior to clinical performance. Although 10 programs (42%) provided protected training time, only 2 (8%) tracked hours. Three programs (13%) reported having an organized curriculum and 6 (25%) train their endoscopic trainers. Cost, time constraints, and lack of a standardized curriculum were perceived as key barriers to integration. Most PDs reported a need for endoscopy simulation to train both technical and nontechnical skills; however, they felt simulation cannot replace clinical experience. CONCLUSION: PDs recognize the potential importance of endoscopy simulation, particularly for novices; however, only 56% report using it. Perceived barriers indicate the need for inexpensive portable simulators and a validated pediatric simulation curriculum to promote uptake.

A Rare Cause of Septic Shock in the Emergency Department in an Intellectually-Disabled Child.

BACKGROUND: Sepsis in older children is often associated with the presence of developmental abnormalities and cerebral palsy. While relatively uncommon, surgical abdomen in these patients is associated with a high rate of mortality. Few reports have been described of sepsis caused by isolated cecal necrosis. CASE REPORT: We report a 13-year-old child with cerebral palsy and global developmental delay who presented to the emergency department with acute worsening abdominal distention that the mother attributed to chronic constipation. Clinical evaluation revealed that she was in severe septic shock and needed immediate stabilization after which she underwent an exploratory laparotomy. Operative findings revealed cecal necrosis that necessitated an ileocecectomy. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Children with intellectual disabilities presenting with sepsis to the emergency department can be particularly challenging given the communication barriers and the time-sensitive nature of the condition. When evaluating these patients, a thorough history and examination are often the only tools that assist in the early identification of the infectious source, leading to improved clinical outcomes.© 2019 Elsevier Inc.

An Unusual Cause of Persistent Crying in a Toddler.

BACKGROUND: Excessive or persistent crying is a common presentation to the pediatric emergency department, and often poses a diagnostic dilemma to emergency physicians. There are several reasons for excessive or persistent crying in children, ranging from benign causes like hunger, to life-threatening causes such as intussusception. CASE REPORT: We report an interesting case of a toddler whose cause of excess crying, with no detectable clinical clues, was eventually attributed to a foreign body in the esophagus. A brief review of diagnostic approach to excess crying and management of ingested foreign bodies is presented. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Ingested foreign body is a potential cause of persistent crying, and early recognition can result in definitive treatment and prevention of potential mortality and morbidity.

Hospital Utilization, Treatment Modalities, and Mortality Using Different Biopsy Methods in Infants With Biliary Atresia.

Objectives To present a nationwide retrospective analysis of the sequelae and aftereffects of different liver biopsy methods in the care of pediatric patients with biliary atresia. Methods The National Inpatient Sample 2001-2013 database was queried for a primary diagnosis of biliary atresia and stratified based on biopsy type including percutaneous, surgical, laparoscopic, and transjugular. Patient demographics, length of stay, hospital costs, type of treatment, and mortality were compared by biopsy type. One-way analysis of variance test and multivariable logistic regression were used for analysis with α < 0.05. Results A total of 4,306 patients with biliary atresia were identified, of whom 2,293 underwent no biopsy, and 723 and 1,080 underwent a percutaneous or surgical biopsy, respectively. Significant differences in socio-demographics were demonstrated between the biopsy types. The length of stay and hospital charges were statistically significantly different between the biopsy types where patients without biopsies had the smallest length compared to percutaneous, surgical, and combination of biopsies. Overall, the Kasai procedure was done more frequently compared to direct liver transplantation, and compared to other biopsy types, undergoing a combination of biopsies had the highest odds of undergoing either procedure. Conclusions When comparing different biopsy methods, surgical biopsies of the liver outperformed percutaneous biopsies in hospital utilization and progression to definitive treatments with the Kasai procedure. Our research indicated that vulnerable populations such as minorities or the indigent may undergo inferior treatments or infrequently undergo definitive treatment. The need for definitive diagnostic guidelines is understated in patients with biliary atresia.

Aberrant responses to TLR agonists in pediatric IBD patients; the possible association with increased production of Th1/Th17 cytokines in response to candida, a luminal antigen.

Toll like receptors (TLR) regulate innate immune responses sensing byproducts of intestinal microbiota. We examined responses to TLR agonists in children with inflammatory bowel disease (IBD). Peripheral blood mononuclear cells (PBMC) obtained from children with IBD [Crohn's disease (CD, n = 10), ulcerative colitis (UC, n = 10)], children with non-IgE-mediated food allergy (NFA, n = 20), and controls (n = 15) were tested for their production of proinflammatory and counter-regulatory cytokines with TLR agonists in comparison with their cytokine production against milk protein and candida. IBD patients were all in the inactive state. IBD PBMC produced more IL-6 with all the TLR agonists tested than controls. CD PBMC produced more counter-regulatory cytokines with TLR agonists, while UC PBMC produced more IL-1ss and IL-10 with TLR 7/8 agonist than controls. Cytokine production by NFA PBMC did not differ from controls. CD but not UC PBMC produced more IFN-gamma and IL-17 with candida. Aberrant responses to TLR agonists may be associated with increase in IFN-gamma/IL-17 production against candida in CD children.

Role of intestinal transporters in neonatal nutrition: carbohydrates, proteins, lipids, minerals, and vitamins.

To support rapid growth and a high metabolic rate, infants require enormous amounts of nutrients. The small intestine must have the complete array of transporters that absorb the nutrients released from digested food. Failure of intestinal transporters to function properly often presents symptoms as "failure to thrive" because nutrients are not absorbed and as diarrhea because unabsorbed nutrients upset luminal osmolality or become substrates of intestinal bacteria. We enumerate the nutrients that constitute human milk and various infant milk formulas, explain their importance in neonatal nutrition, then describe for each nutrient the transporter(s) that absorbs it from the intestinal lumen into the enterocyte cytosol and from the cytosol to the portal blood. More than 100 membrane and cytosolic transporters are now thought to facilitate absorption of minerals and vitamins as well as products of digestion of the macronutrients carbohydrates, proteins, and lipids. We highlight research areas that should yield information needed to better understand the important role of these transporters during normal development.

Overall severities of gastrointestinal symptoms in pediatric outpatients with and without autism spectrum disorder.

In order to determine the effectiveness of a Gastrointestinal Severity Index to screen for gastrointestinal disorders, the Gastrointestinal Severity Index was administered to 135 children with autism spectrum disorders and 146 comparisons with and without gastrointestinal disorders. The mean Gastrointestinal Severity Index scores of the groups were 3.53 ± 1.78, 3.15 ± 1.99, 0.81 ± 1.25, and 0.29 ± 0.76 (comparative pediatric patients with gastrointestinal disorder = autism spectrum disorder + gastrointestinal disorder > autism spectrum disorder-gastrointestinal disorder > comparative pediatric patients without gastrointestinal disorder, respectively), Ps < 0.05. Receiver operating characteristic curves and areas under the receiver operating characteristic curves were calculated to ascertain which Gastrointestinal Severity Index cutoff scores yielded the highest sensitivity and specificity rates for the diagnosis of gastrointestinal disorders. The area under the receiver operating characteristic curve (0.97) for the comparison group was higher (P < 0.001) than the area under the receiver operating characteristic curve (0.85) for autism spectrum disorder children indicating that the Gastrointestinal Severity Index was more effective in screening for gastrointestinal disorders in comparisons. However, the same Gastrointestinal Severity Index cutoff score of 2 and above yielded, respectively, sensitivity and specificity rates of 92% and 93% for comparisons and 80% and 79% for autism spectrum disorder children. The negative and positive predictive values based on these sensitivity and specificity rates were calculated for a range of prevalences of gastrointestinal disorders and indicated that the Gastrointestinal Severity Index may be useful for screening children with and without autism spectrum disorder for gastrointestinal symptoms.

Effects of Immigration on Infant Feeding Practices in an Inner City, Low Socioeconomic Community.

OBJECTIVE: Infant feeding practices have been shown to differ between immigrants and non-immigrants in the United States. Our study characterizes feeding practices of infants of immigrant versus American-born mothers followed in an inner city Pediatric continuity clinic serving predominantly low socioeconomic status families. METHODS: A survey was given to 102 parents of infants ranging from 12 to 15 months of age who attended the clinic. Parents were asked about their country of origin, ethnic background and time since immigration to the US. They were also asked about their breastfeeding and early infant feeding practices. Statistical significance was estimated using chi-squared tests. RESULTS: Not only were breastfeeding rates higher among immigrant mothers compared to American-born mothers (88% vs. 63%, p-value 0.008) but they introduced commercially available baby food less frequently (37% vs. 52%, p-value 0.03) and rarely fed their infants fast food (22% vs. 50%, p-value<0.001). Moreover, breast feeding rates decreased with duration of residence in the United States. 53% of immigrant mothers who have been in the United States for less than 5 years breastfed for over 6 months versus 22% of immigrant mothers who resided more than 5 years in the United States (p-value 0.02). The vast majority of immigrant mothers who switched to formula did so because they felt their milk production was insufficient (93%) whereas the vast majority of American-born mothers stopped breastfeeding because they perceived it to be painful (64%, p value 0.001). CONCLUSIONS: Infant feeding practices differ between immigrant and American-born mothers and the differences diminish the longer the mothers reside in the United States. These differences stem from differences in cultural perceptions of breastfeeding. Therefore, in educating mothers about infant feeding, physicians should strongly consider cultural and ethnic factors.

Tc-99m red blood cell scintigraphic detection of CMV-induced small bowel hemorrhage.

A 4-month-old female child, recently diagnosed with parentally-acquired HIV complicated by moderate immunosuppression and pneumocystis carinii pneumonia (PCP), presented with hematochezia. A Tc-99m bleeding study was performed and demonstrated a site of brisk bleeding in the distal ileum. The patient was taken to the operating room and resection of the distal ileum revealed the presence of cytomegalovirus (CMV) enteritis. In immunocompromised children presenting with gastrointestinal (GI) hemorrhage, CMV enteritis should be considered.

Diagnosis and management of inflammatory bowel disease in children.

Inflammatory bowel diseases (IBD), including Crohn's disease and ulcerative colitis, are lifelong conditions that often begin in childhood. The implications of IBD are of particular importance in children because of the potential negative effects on growth, development, psychosocial function, and overall wellbeing. The key management strategy is to achieve sustained control of intestinal inflammation and monitor for potential complications of the disease and side effects of therapies. Overall, the evidence on the management of IBD in children is less extensive than in adults, but good quality multicenter studies and various guidelines and society consensus statements are available. This review summarizes the evidence on the pathophysiology, diagnosis, and approaches to management of children and adolescents with IBD.