Nutritional and feeding challenges in aerodigestive patients.

PURPOSE OF REVIEW: Aerodigestive disorders encompass conditions that affect both the airway and gastrointestinal tract. These include conditions such as acquired and congenital defects of the airway and esophagus as well as neuromuscular disorders. Patients often suffer from dysphagia, aspiration, and respiratory disorders. This article will provide a review of current practices in the management of feeding disorders, oropharyngeal dysphagia, and nutritional support in the aerodigestive population. RECENT FINDINGS: Oral aversion, aspiration, and feeding-tube dependence are all commonly encountered problems in the aerodigestive population. Intensive inpatient and outpatient programs along with use of appetite stimulants and psychotropic medications may help to improve feeding-related disorders. Aspiration affects many patients and requires close monitoring of clinical symptoms along with routine assessment with video fluoroscopy. Developments in blenderized feeds and formula supplementation have also provided new options for patients with feeding intolerance. SUMMARY: Patients with aerodigestive disorders require complex medical care, and multidisciplinary teams are the most effective in addressing their medical needs. Advances in feeding, occupational, and pharmacologic therapy have allowed healthcare providers to better address the needs of these patients.

Obstructing Sigmoid Volvulus: An Unusual Complication in a Pediatric Patient With Ulcerative Colitis.

Acute colonic dilation in pediatric patients with ulcerative colitis (UC) raises a concern for toxic megacolon, but other rare conditions such as sigmoid volvulus may present in a similar manner. We report a rare case of a teenager with UC without prior surgery who developed an obstructing sigmoid volvulus managed with endoscopic detorsion and decompression. Colonic inflammation in patients with UC may result in a volvulus in the absence of other predisposing factors and should be considered in the differential diagnosis of patients with UC who present with obstructive symptoms with an atypical presentation.

Overview of Physical, Neurocognitive, and Psychosocial Outcomes in Pediatric Intestinal Failure and Transplantation.

PURPOSE OF REVIEW: Intestinal failure and transplantation may significantly impact physical, neurocognitive, and psychosocial development in pediatric patients. Currently, there is a paucity of literature on the effects of intestinal failure and transplantation on these aspects of development. This article will review the current literature and discuss the short and long-term impacts as well as interventions to improve clinical outcomes in children with intestinal failure or those undergoing transplantation. RECENT FINDINGS: Psychological disorders, neurodevelopmental delay, and social maladaptation are frequently encountered in this patient population. While the main focus is often on medical management, equal emphasis should be placed on other aspects of development such as increasing social support and improving school performance. The transition to adulthood also presents many obstacles for patients and healthcare providers should anticipate challenges such as childbirth, employment, and raising a family. The pre-operative, perioperative, and post-operative periods all represent opportunities for medical intervention. Frequent monitoring of physical, psychosocial, and neurocognitive status helps to improve clinical outcomes and long-term quality of life. Future research should emphasize continued development of multidisciplinary programs and specialized services to help address the physical and psychosocial needs of children with intestinal failure as well as transplant recipients.

Measurement of Quality-of-Life Outcomes in Pediatric and Young Adult Patients Treated for Eosinophilic Esophagitis.

Eosinophilic esophagitis is a chronic, immune-mediated esophageal condition that may lead to impairment of quality of life in pediatric and young adult patients. We performed a prospective, cross-sectional study on 40 patients between the ages of 2-21 years with an established diagnosis of eosinophilic esophagitis. The study evaluated physical, emotional, social, and school functioning in patients undergoing treatment with proton pump inhibitors, dietary elimination, or swallowed corticosteroids. There were no statistically significant differences in total or domain-specific quality of life scores between proton pump inhibitors, dietary elimination, and swallowed corticosteroid therapy. Overall, total and domain-specific quality of life were well-preserved in patients with eosinophilic esophagitis, with the highest scores reported in social functioning. There were also no statistically significant associations between clinical, endoscopic, and histologic features and quality-of-life measures.

Overview of Albumin Physiology and its Role in Pediatric Diseases.

PURPOSE OF REVIEW: Albumin plays a critical role in a wide range of disease processes; however, the role of albumin in pediatric patients has not been well described. This article aims to review albumin physiology and kinetics in children, albumin's impact on pediatric diseases, and the utility of albumin as a predictor of clinical outcome. RECENT FINDINGS: Hypoalbuminemia is seen in a wide range of conditions, including protein-losing enteropathy, hepatic synthetic failure, malnutrition, inflammatory states, and renal disease. While the impact of hypoalbuminemia has been more extensively studied in adult patients, there is a relative paucity of literature in the pediatric population. Hypoalbuminemia is a marker of poor outcome in critically ill children and those undergoing a wide range of medical interventions. Albumin infusions may be an effective therapy for fluid resuscitation and for patients with severe hypoalbuminemia.

Rare genetic mutation triggering acute liver failure in a toddler requiring a liver transplant.

APS-1 is an extremely rare, autosomal recessive condition that often presents with candidiasis, adrenal insufficiency, and hypoparathyroidism. This condition is associated with autoimmune hepatitis in less than 20% of cases, and there have only been a few reports of children with the condition who developed ALF. We present a unique case of an infant with APS-1 who developed ALF and subsequently required liver transplantation.

Correction to: Gallbladder Adenomyomatosis in an Infant.

[This corrects the article DOI: 10.14309/crj.0000000000000433.].

Gallbladder Adenomyomatosis in an Infant.

Gallbladder adenomyomatosis is a rare condition that is predominantly seen in adults, and only a few cases have been described in the pediatric population. Although it is generally benign, it may present a diagnostic challenge for physicians. Advances in imaging have led to an increase in its detection. Nevertheless, the characteristics and management of this condition in pediatric patients have not been well described. We present a case of a 6-week-old infant boy who was found to have gallbladder adenomyomatosis.

A Review of Autoimmune Enteropathy and Its Associated Syndromes.

Autoimmune enteropathy is an extremely rare condition characterized by an abnormal intestinal immune response which typically manifests within the first 6 months of life as severe, intractable diarrhea that does not respond to dietary modification. Affected individuals frequently present with other signs of autoimmunity. The diagnosis is made based on a characteristic combination of clinical symptoms, laboratory studies, and histological features on small bowel biopsy. Autoimmune enteropathy is associated with a number of other conditions and syndromes, most notably immunodysregulation polyendocrinopathy enteropathy X-linked (IPEX) syndrome and autoimmune polyglandular syndrome type 1 (APS-1). Diagnosis and treatment is challenging, and further research is needed to better understand the pathogenesis, disease progression, and long-term outcomes of these conditions.

Bullous Henoch-Schönlein purpura in children.

Henoch-Schönlein purpura (HSP) is a systemic vasculitis that is common in the pediatric population and often presents with the classical triad of palpable purpura, arthralgia, and abdominal pain. We describe a case of HSP in a 14-year-old adolescent girl who presented with atypical features of painful hemorrhagic bullae. The patient was treated with high-dose steroids, dapsone, and supportive therapy with remarkable improvement.