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1.
JPEN J Parenter Enteral Nutr ; 47(6): 718-728, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-37004208

RESUMO

As the majority of children with short bowel syndrome (SBS) and intestinal failure (IF) are now surviving into adulthood, there is a paradigm shift from short-term management to long-term outcomes and a growing need to focus on healthcare transition (HCT). It is imperative that adolescents and young adults with SBS and IF receive disease education, empowerment, and support as they navigate the transition from pediatric to adult care. Furthermore, both pediatric and adult healthcare providers who manage these patients should be aware of the challenges faced by this population, barriers to their HCT, and strategies to overcome them. This article reviews the literature on HCT in children with chronic illnesses, discusses barriers to HCT in SBS/IF, identifies the important constituents of the transition process in SBS/IF, and provides recommendations for the successful and smooth transition of the pediatric patient to the adult healthcare environment. Structured and multicomponent HCT programs should become the standard of care to ensure uninterrupted high-quality care across the life span for patients with SBS/IF.


Assuntos
Insuficiência Intestinal , Síndrome do Intestino Curto , Transição para Assistência do Adulto , Adolescente , Adulto Jovem , Humanos , Criança , Síndrome do Intestino Curto/terapia , Doença Crônica
3.
J Pediatr Gastroenterol Nutr ; 72(3): 474-486, 2021 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-33399327

RESUMO

ABSTRACT: Intestinal failure requires the placement and maintenance of a long-term central venous catheter for the provision of fluids and/or nutrients. Complications associated with this access contribute to significant morbidity and mortality, while the loss of access is an increasingly common reason for intestinal transplant referral. As more emphasis has been placed on the prevention of central line-associated bloodstream infections and new technologies have developed, care for central lines has improved; however, because care has evolved independently in local centers, care of central venous access varies significantly in this vulnerable population. The present position paper from the Intestinal Failure Special Interest Group of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN) reviews current evidence and provides recommendations for central line management in children with intestinal failure.


Assuntos
Infecções Relacionadas a Cateter , Cateterismo Venoso Central , Cateteres Venosos Centrais , Gastroenterologia , Infecções Relacionadas a Cateter/prevenção & controle , Cateterismo Venoso Central/efeitos adversos , Cateteres Venosos Centrais/efeitos adversos , Criança , Humanos , Intestinos , Opinião Pública , Estudos Retrospectivos
5.
J Pediatr Hematol Oncol ; 38(7): e243-7, 2016 10.
Artigo em Inglês | MEDLINE | ID: mdl-27571123

RESUMO

Severe congenital neutropenia type IV (SCN IV) is a syndrome of severe neutropenia, cardiac and urogenital defects, prominent superficial veins, facial dysmorphism, failure to thrive (FTT), and intermittent thrombocytopenia, caused by a glucose-6-phosphatase catalytic subunit 3 (G6PC3) gene mutation. SCN IV has been linked to glycogen storage disease type 1b as both disorders involve disruption of the glucose-6-phosphatase/glucose-6-phosphate transporter complex, leading to arrested neutrophil maturation. Emerging evidence suggests that neutrophil function plays an important role in intestinal integrity, evidenced by inflammatory bowel disease in certain neutropenic patients. Here, we report 3 unrelated Hispanic males from the Dominican Republic with classic features of SCN IV found to share an identical inherited canonical splice-site mutation of the G6PC3 gene (c.218+1G>A). All 3 patients presented with severe FTT and gastrointestinal manifestations. Two of the patients had significant improvement in growth and resolution of gastrointestional symptoms with initiation of granulocyte colony-stimulating factor. We hypothesize that the gene variant described represents a founder mutation in the Dominican Republic, the first to be described in this geographical region. We discuss the potential associations between neutropenia and gastrointestinal disease with FTT and the role of granulocyte colony-stimulating factor in improving neutrophil count and intestinal integrity and growth.


Assuntos
Gastroenteropatias/genética , Glucose-6-Fosfatase/genética , Mutação , Neutropenia/congênito , Adolescente , Criança , Pré-Escolar , Síndrome Congênita de Insuficiência da Medula Óssea , Insuficiência de Crescimento/etiologia , Fator Estimulador de Colônias de Granulócitos/uso terapêutico , Humanos , Masculino , Neutropenia/tratamento farmacológico , Neutropenia/genética , Fenótipo
7.
Can J Gastroenterol ; 24(9): 552-6, 2010 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-21152460

RESUMO

BACKGROUND: Halitosis is a common human condition for which the exact pathophysiological mechanism is unclear. It has been attributed mainly to oral pathologies. Halitosis resulting from gastrointestinal disorders is considered to be extremely rare. However, halitosis has often been reported among the symptoms related to Helicobacter pylori infection and gastroesophageal reflux disease. OBJECTIVE: To retrospectively review the experience with children and young adults presenting with halitosis to a pediatric gastroenterology clinic. METHODS: A retrospective chart review of patients diagnosed with halitosis as a primary or secondary symptom was conducted. All endoscopies were performed by the same endoscopist. RESULTS: A total of 94 patients had halitosis, and of the 56 patients (59.6%) who were recently examined by a dental surgeon, pathology (eg, cavities) was found in only one (1.8%). Pathology was found in only six of 27 patients (28.7%) who were assessed by an otolaryngology surgeon. Gastrointestinal pathology was found to be very common, with halitosis present in 54 of the 94 (57.4%) patients. The pathology was noted regardless of dental or otolaryngological findings. Most pathologies, both macroscopically and microscopically, were noted in the stomach (60% non-H pylori related), followed by the duodenum and the esophagus. Fifty-two of 90 patients (57.8%) were offered a treatment based on their endoscopic findings. Of the 74 patients for whom halitosis improvement data were available, some improvement was noted in 24 patients (32.4%) and complete improvement was noted in 41 patients (55.4%). CONCLUSIONS: Gastrointestinal pathology was very common in patients with halitosis regardless of dental or otolaryngological findings, and most patients improved with treatment.


Assuntos
Endoscopia Gastrointestinal , Gastroenteropatias/complicações , Halitose/etiologia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Gastroenteropatias/diagnóstico , Gastroenteropatias/patologia , Halitose/diagnóstico , Humanos , Lactente , Masculino , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
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