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1.
Pediatr Surg Int ; 40(1): 106, 2024 Apr 13.
Artigo em Inglês | MEDLINE | ID: mdl-38613719

RESUMO

PURPOSE: To explore the influence of postoperative Hirschsprung-associated enterocolitis (post-HAEC) on long-term outcomes and to identify risk factors of post-HAEC. METHODS: The medical records of 304 eligible patients diagnosed with Hirschsprung's disease (HSCR) were reviewed. We analyzed the clinical characteristics of post-HAEC and its influence on long-term outcomes. Furthermore, risk factors for early and recurrent HAEC were identified separately. RESULTS: The overall incidence of post-HAEC was 29.9% (91/304). We categorized early HAEC as occurring within postoperative 3 months (n = 39) and recurrent HAEC as occurring ≥ 3 episodes within postoperative 6 months (n = 25). Patients with early HAEC were more likely to experience worse nutritional status, defecation function, and quality of life compared to those with late or no episodes (P < 0.05). Similarly, the adverse influences of recurrent HAEC on these outcomes were also significant (P < 0.05). The risk factors for early HAEC included preoperative undernutrition, long-segment HSCR, and postoperative Grade 3-4 complications within 30 days. For recurrent HAEC, risk factors were preoperative malnutrition, non-parental caregivers, long-segment HSCR, and postoperative Grade 3-4 complications within 30 days. CONCLUSION: Classification of post-HAEC based on the first episode time and frequency was necessary. The earlier or more frequent episodes of post-HAEC have detrimental influences on long-term outcomes. Furthermore, risk factors for early and recurrent HAEC were different.


Assuntos
Enterocolite , Doença de Hirschsprung , Desnutrição , Criança , Humanos , Estudos Retrospectivos , Qualidade de Vida , Enterocolite/epidemiologia , Enterocolite/etiologia , Doença de Hirschsprung/complicações , Doença de Hirschsprung/cirurgia , Complicações Pós-Operatórias/epidemiologia , Centros de Atenção Terciária
2.
Int Arch Allergy Immunol ; 185(4): 334-342, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38194937

RESUMO

INTRODUCTION: Food protein-induced enterocolitis syndrome (FPIES) is a form of non-IgE-mediated gastrointestinal food allergy. FPIES is considered a rare food allergy disorder and is often under-recognized. Therefore, clinicians should have a better understanding of its manifestations and maintain a high index of suspicion for a correct diagnosis. To this end, information about differences in the characteristics of caregiver-reported and physician-diagnosed FPIES is important. METHODS: The present, national, multicentric, prospective birth cohort study, called the Japan Environment and Children's Study (JECS), enrolled a general population of 104,062 fetal records. The characteristics of FPIES in 1.5-year-old children were categorized as cases reported by caregivers or as those diagnosed by a physician using questionnaire data. RESULTS: The prevalence of caregiver-reported and physician-diagnosed FPIES cases was 0.69% and 0.06%, respectively. Among the former, the most common causative food was hen's egg (HE), and the second most common causative food was cow's milk (CM) (51.0% and 17.1% of patients responded to HE and CM, which accounted for 46% and 15% of all the causative foods, respectively). Conversely, among the physician-diagnosed cases, the most common causative food was CM followed by HE (57.7% and 36.5% of patients responded to CM and HE, which accounted for 46% and 29% of all the causative foods, respectively). CM accounted for a significantly higher proportion of causative foods in physician-diagnosed FPIES while HE accounted for a significantly higher proportion of caregiver-reported FPIES (p < 0.05). CONCLUSION: A discrepancy was found in reports of the most common causative food between caregiver-reported and physician-diagnosed cases of FPIES.


Assuntos
Enterocolite , Hipersensibilidade Alimentar , Bovinos , Humanos , Feminino , Animais , Lactente , Pré-Escolar , Cuidadores , Estudos de Coortes , Estudos Prospectivos , Galinhas , Japão/epidemiologia , Hipersensibilidade Alimentar/diagnóstico , Hipersensibilidade Alimentar/epidemiologia , Hipersensibilidade Alimentar/complicações , Enterocolite/diagnóstico , Enterocolite/epidemiologia , Enterocolite/etiologia , Alérgenos , Proteínas na Dieta/efeitos adversos
4.
J Pediatr Surg ; 59(2): 220-224, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37981542

RESUMO

PURPOSE: Hirschsprung Disease (HD) is typically diagnosed in the neonatal period. A small subset of patients have a prolonged course of abdominal distention and constipation prior to diagnosis. Late HD is defined as having been diagnosed at greater than or equal to one year of age. The literature is limited and offers conflicting data on the implications of a late diagnosis. We aim to investigate the presentation, operative approach, and functional outcomes of a large cohort of patients with a late HD diagnosis. METHODS: All patients with a late diagnosis of HD (after 1 year of age) at our institution between 1997 and 2021 were included. RESULTS: Twenty-eight patients were diagnosed with HD at a median age of 3.4 years. Chronic constipation, failure to thrive, and enterocolitis occurred in 100 %, 31 %, and 14 %, respectively. All patients underwent contrast enema and biopsies during their workup, identifying primarily rectosigmoid disease (n = 27) and total colonic aganglionosis (n = 1). Surgical intervention was performed in 27 patients, with 4 patients (15 %) needing a stoma (3 with plan for staged pull-through, 1 long-term stoma) and 23 patients (85 %) undergoing a single-stage pull-through. Postoperative complications included Hirschsprung-associated enterocolitis (n = 5), ostomy prolapse and revision (n = 2), abdominal distention requiring ileostomy creation (n = 2), redo pull-through (n = 2), retroperitoneal hematoma (n = 1), and cecostomy tube placement (n = 1). At a median follow-up of 5.4 years, 83 % of eligible patients achieved fecal continence with 43 % needing laxatives for persistent constipation. CONCLUSION: Recognizing a late presentation of HD requires a high index of suspicion. Patients with a late diagnosis did not experience an increased rate of permanent stoma, complications, or redo surgery compared to rates reported for the larger HD population. Similar long-term functional outcomes were achieved compared to the larger HD population. LEVEL OF EVIDENCE: IV.


Assuntos
Enterocolite , Doença de Hirschsprung , Recém-Nascido , Humanos , Lactente , Pré-Escolar , Doença de Hirschsprung/diagnóstico , Doença de Hirschsprung/cirurgia , Doença de Hirschsprung/epidemiologia , Resultado do Tratamento , Diagnóstico Tardio , Constipação Intestinal/epidemiologia , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Enterocolite/diagnóstico , Enterocolite/etiologia , Enterocolite/epidemiologia , Estudos Retrospectivos
5.
Allergol Int ; 73(2): 275-281, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38151409

RESUMO

BACKGROUND: Adults with food-protein-induced enterocolitis syndrome (FPIES) often develop severe abdominal symptoms after eating seafood. However, no investigation of a food elimination strategy for adult FPIES patients has been performed to date. METHODS: We conducted a retrospective cohort study of seafood-avoidant adults by telephone interview, based on the diagnostic criteria for adult FPIES reported by González et al. We compared the clinical profiles, abdominal symptoms, and causative seafoods between FPIES and immediate-type food allergy (IgE-mediated FA) patients. We also profiled the detailed intake-status of seafoods in adult FPIES patients. RESULTS: Twenty-two (18.8 %) of 117 adults with seafood-allergy were diagnosed with FPIES. Compared with the IgE-mediated FA patients, FPIES patients had an older age of onset, more pre-existing gastrointestinal and atopic diseases, more episodes, longer latency and duration of symptoms, more nausea, abdominal distention, and severe abdominal pain, and more frequent vomiting and diarrhea. In particular, abdominal distention-reflecting intestinal edema and luminal fluid retention-may be the most distinctive characteristic symptom in adult FPIES (p < 0.001). Bivalves, especially oysters, were the most common cause of FPIES. Strikingly, intake-status profiling revealed that many FPIES patients can safely ingest an average of 92.6 % of seafood species other than the causative species. CONCLUSIONS: There are many differentiators between FPIES and IgE-mediated FA, which may reflect differences in the underlying immunological mechanisms. Although seafood FPIES is unlikely to induce tolerance, many patients can ingest a wide variety of seafood species after a long period from onset.


Assuntos
Enterocolite , Hipersensibilidade Alimentar , Adulto , Humanos , Lactente , Estudos Retrospectivos , Proteínas na Dieta/efeitos adversos , Síndrome , Enterocolite/diagnóstico , Enterocolite/epidemiologia , Alérgenos , Alimentos Marinhos/efeitos adversos , Imunoglobulina E
6.
Bol Med Hosp Infant Mex ; 80(6): 331-338, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-38150718

RESUMO

BACKGROUND: Surgeons create a neorectum to repair patients with Hirschsprung's disease (HD), which should be formed from a normoganglionic bowel. However, the neorectum is occasionally created with a transition zone (TZ) bowel. A neorectum created with a TZ has been postulated as a cause of postoperative enterocolitis or constipation. This study compares the incidence of enterocolitis and constipation in patients with TZ neorectum and normoganglionic bowel. METHODS: We conducted a retrospective review of patients with rectosigmoid HD who underwent primary pull-through. Patients were divided into normoganglionic neorectum (NNR) and TZ neorectum. The diagnosis was based on the final histopathologic report of the proximal margin. The incidence of enterocolitis and constipation was compared between these two groups. RESULTS: A total of 98 HD patients were analyzed. Seventy-one patients fulfilled the inclusion criteria. 65 (92%) had a NNR, and six patients (8%) had a TZ neorectum. From these patients, 42 (59%) presented with enterocolitis or constipation. However, there was no significant difference between both groups. CONCLUSION: The present study showed no difference in the incidence of enterocolitis or postoperative constipation in HD patients with normoganglionic or TZ neorectum. These results suggest that TZ neorectum does not cause postoperative obstructive symptoms.


INTRODUCCIÓN: Los cirujanos crean un neo-recto para tratar a los pacientes con enfermedad de Hirschsprung (EH), que debe formarse con intestino normogangliónico; sin embargo, en ocasiones el neo-recto se forma con intestino de la zona de transición. Se ha postulado que un neo-recto en zona de transición causa enterocolitis o estreñimiento postoperatorio. El objetivo de este estudio fue comparar la frecuencia de enterocolitis y estreñimiento en pacientes con neo-recto en zona de transición y con neo-recto normogangliónico. MÉTODOS: Se llevó a cabo una revisión retrospectiva de pacientes con EH recto sigmoideo que se sometieron a descenso primario. Los pacientes se dividieron en el grupo neo-recto normogangliónico y el grupo con neo-recto en zona de transición. El diagnóstico del neo-recto se estableció con el informe histopatológico definitivo del margen proximal. Se comparó la frecuencia de enterocolitis y estreñimiento entre estos dos grupos. RESULTADOS: Se analizó un total de 98 pacientes con EH, de los cuales 71 pacientes cumplieron los criterios de inclusión; 65 (92%) con neo-recto normogangliónico y seis (8%) con neo-recto en zona de transición. Posteriormente, 42 (59%) pacientes presentaron enterocolitis asociada a Hirschsprung (HAEC) o estreñimiento; sin embargo, no hubo diferencia significativa entre ambos grupos. CONCLUSIONES: El presente estudio no demostró una diferencia en la frecuencia de HAEC o estreñimiento postoperatorio en pacientes con EH con neo-recto normogangliónico o en zona de transición. Estos resultados sugieren que un neo-recto en zona de transición no causa síntomas obstructivos postoperatorios.


Assuntos
Enterocolite , Doença de Hirschsprung , Humanos , Doença de Hirschsprung/cirurgia , Doença de Hirschsprung/diagnóstico , Doença de Hirschsprung/patologia , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/patologia , Constipação Intestinal/etiologia , Constipação Intestinal/complicações , Reto/cirurgia , Reto/patologia , Enterocolite/epidemiologia , Enterocolite/etiologia , Enterocolite/patologia
7.
J Allergy Clin Immunol Pract ; 11(10): 3179-3186.e2, 2023 10.
Artigo em Inglês | MEDLINE | ID: mdl-37380072

RESUMO

BACKGROUND: Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated food allergy treated by trigger food avoidance and supportive care. Whether the prevalence of different trigger foods is changing with evolving food introduction patterns is unknown. The rate and nature of subsequent reactions after initial diagnosis have not been fully studied. OBJECTIVE: We sought to characterize how trigger foods have changed over time and investigate the nature of subsequent reactions after initial diagnosis. METHODS: We collected data regarding patients' FPIES reactions from 347 patients seen in the University of Michigan Allergy and Immunology clinic for FPIES from 2010 to 2022. Inclusion criteria consisted of pediatric patients diagnosed with FPIES by an allergist based on international consensus guidelines. RESULTS: Most foods including less commonly cited FPIES triggers increased in frequency over time. The most common index trigger was oat. A total of 32.9% (114 of 347) patients experienced a subsequent reaction after education on trigger avoidance and safe home introduction of new foods, with 34.2% (41 of 120) of subsequent reactions to new triggers at home and 45% (54 of 120) to known triggers at home. Of patients reacting subsequently, 28% (32 of 114) experienced a subsequent reaction necessitating an emergency department visit. The most common new subsequent reaction triggers were egg and potato, whereas peanut most commonly triggered reactions on oral food challenge. CONCLUSIONS: The risk profile of FPIES triggers may be evolving over time, though high-risk FPIES foods remain common. The subsequent reaction rate after counseling indicates that home food introduction poses risk. This study highlights the need for improved safety of new food introduction and/or prediction methods for FPIES to help prevent potentially dangerous home FPIES reactions.


Assuntos
Enterocolite , Hipersensibilidade Alimentar , Criança , Humanos , Lactente , Hipersensibilidade Alimentar/diagnóstico , Hipersensibilidade Alimentar/epidemiologia , Síndrome , Alimentos/efeitos adversos , Enterocolite/diagnóstico , Enterocolite/epidemiologia , Alérgenos , Proteínas na Dieta/efeitos adversos
8.
Int J Surg ; 109(8): 2509-2524, 2023 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-37288551

RESUMO

BACKGROUND: The incidence of Hirschsprung disease (HSCR) is nearly 1/5000 and patients with HSCR are usually treated through surgical intervention. Hirschsprung disease-associated enterocolitis (HAEC) is a complication of HSCR with the highest morbidity and mortality in patients. The evidence on the risk factors for HAEC remains inconclusive to date. METHODS: Four English databases and four Chinese databases were searched for relevant studies published until May 2022. The search retrieved 53 relevant studies. The retrieved studies were scored on the Newcastle-Ottawa Scale by three researchers. Revman 5.4 software was employed for data synthesis and analysis. Stata 16 software was employed for sensitivity analysis and bias analysis. RESULTS: A total of 53 articles were retrieved from the database search, which included 10 012 cases of HSCR and 2310 cases of HAEC. The systematic analysis revealed anastomotic stenosis or fistula [ I2 =66%, risk ratio (RR)=1.90, 95% CI 1.34-2.68, P <0.001], preoperative enterocolitis ( I2 =55%, RR=2.07, 95% CI 1.71-2.51, P <0.001), preoperative malnutrition ( I2 =0%, RR=1.96, 95% CI 1.52-2.53, P <0.001), preoperative respiratory infection or pneumonia ( I2 =0%, RR=2.37, 95% CI 1.91-2.93, P <0.001), postoperative ileus ( I2 =17%, RR=2.41, 95% CI 2.02-2.87, P <0.001), length of ganglionless segment greater than 30 cm ( I2 =0%, RR=3.64, 95% CI 2.43-5.48, P <0.001), preoperative hypoproteinemia ( I2 =0%, RR=1.91, 95% CI 1.44-2.54, P <0.001), and Down syndrome ( I2 =29%, RR=1.65, 95% CI 1.32-2.07, P <0.001) as the risk factors for postoperative HAEC. Short-segment HSCR ( I2 =46%, RR=0.62, 95% CI 0.54-0.71, P <0.001) and transanal operation ( I2 =78%, RR=0.56, 95% CI 0.33-0.96, P =0.03) were revealed as the protective factors against postoperative HAEC. Preoperative malnutrition ( I2 =35 % , RR=5.33, 95% CI 2.68-10.60, P <0.001), preoperative hypoproteinemia ( I2 =20%, RR=4.17, 95% CI 1.91-9.12, P <0.001), preoperative enterocolitis ( I2 =45%, RR=3.51, 95% CI 2.54-4.84, P <0.001), and preoperative respiratory infection or pneumonia ( I2 =0%, RR=7.20, 95% CI 4.00-12.94, P <0.001) were revealed as the risk factors for recurrent HAEC, while short-segment HSCR ( I2 =0%, RR=0.40, 95% CI 0.21-0.76, P =0.005) was revealed as a protective factor against recurrent HAEC. CONCLUSION: The present review delineated the multiple risk factors for HAEC, which could assist in preventing the development of HAEC.


Assuntos
Enterocolite , Doença de Hirschsprung , Humanos , Doença de Hirschsprung/complicações , Doença de Hirschsprung/cirurgia , Enterocolite/epidemiologia , Enterocolite/etiologia , Enterocolite/terapia , Fatores de Risco , Incidência , Morbidade
9.
J Pediatr Surg ; 58(8): 1578-1581, 2023 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-37221126

RESUMO

BACKGROUND: Hirschsprung-associated enterocolitis (HAEC) is the most common cause of morbidity and mortality amongst patients with Hirschsprung disease (HD); rectal Botulinum toxin (Botox) has been reported a possible prevention strategy. We aimed to evaluate our institution's historic cohort of HD patients, first to determine our incidence of HAEC and second to begin assessing the effect of Botox on HAEC incidence. METHODS: Patients with HD seen at our institution between 2005 and 2019 were reviewed. Incidence of HD and frequencies of HAEC and Botox injections were tallied. Associations between initial Botox treatment or transition zone and HAEC incidence were evaluated. RESULTS: We reviewed 221 patients; 200 were included for analysis. One hundred thirteen (56.5%) patients underwent primary pull-through at a median age of 24 days (IQR 91). Eighty-seven (43.5%) patients with initial ostomy had their intestinal continuity reestablished at a median of 318 days (IQR 595). Ninety-four (49.5%) experienced at least one episode of HAEC and 62 (66%) experienced multiple episodes of HAEC. Nineteen (9.6%) patients had total colonic HD and had an increased total incidence of HAEC compared to patients without total colonic HD (89% vs 44%, p < 0.001). Six (2.9%) patients received Botox injections at the time of pull-through or ostomy takedown; one experienced an episode of HAEC (versus 50.7% of the patients who were confirmed to have not received Botox injections at their surgery, p = 0.102). CONCLUSION: Further prospective study on Botox's effect on Hirschsprung-associated enterocolitis is required and is the next step in our investigation. LEVEL OF EVIDENCE: Level III.


Assuntos
Toxinas Botulínicas Tipo A , Enterocolite , Doença de Hirschsprung , Humanos , Lactente , Estudos Retrospectivos , Estudos Prospectivos , Toxinas Botulínicas Tipo A/uso terapêutico , Doença de Hirschsprung/complicações , Doença de Hirschsprung/cirurgia , Enterocolite/epidemiologia , Enterocolite/etiologia , Enterocolite/cirurgia , Reto , Complicações Pós-Operatórias/epidemiologia
11.
J Pediatr Surg ; 58(9): 1694-1698, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-36890100

RESUMO

BACKGROUND: The Coronavirus Disease 2019 pandemic provided a natural experiment to study the effect of social distancing on the risk of developing Hirschsprung's Associated Enterocolitis (HAEC). METHODS: Using the Pediatric Health Information System (PHIS), a retrospective cohort study of children (<18 years) with Hirschsprung's Disease (HSCR) across 47 United States children's hospitals was performed. The primary outcome was HAEC admissions per 10,000 patient-days. The exposure (COVID-19) was defined as April 2020-December 2021. The unexposed (historical control) period was April 2018-December 2019. Secondary outcomes included sepsis, bowel perforation, intensive care unit (ICU) admission, mortality, and length of stay. RESULTS: Overall, we included 5707 patients with HSCR during the study period. There were 984 and 834 HAEC admissions during the pre-pandemic and pandemic periods, respectively (2.6 vs. 1.9 HAEC admissions per 10,000 patient-days, incident rate ratio [95% confidence interval]: 0.74 [0.67, 0.81], p < 0.001). Compared to pre-pandemic, those with HAEC during the pandemic were younger (median [IQR]: 566 [162, 1430] days pandemic vs. 746 [259, 1609] days pre-pandemic, p < 0.001) and more likely to live in the lowest quartile of median household income zip codes (24% pandemic vs. 19% pre-pandemic, p = 0.02). There were no significant differences in rates of sepsis (6.1% pandemic vs. 6.1% pre-pandemic, p > 0.9), bowel perforation (1.3% pandemic vs. 1.2% pre-pandemic, p = 0.8), ICU admissions (9.6% pandemic vs. 12% pre-pandemic, p = 0.2), mortality (0.5% pandemic vs. 0.6% pre-pandemic, p = 0.8), or length of stay (median [interquartile range]: 4 [(Pastor et al., 2009; Gosain and Brinkman, 2015) 2,112,11 days pandemic vs. 5 [(Pastor et al., 2009; Tang et al., 2020) 2,102,10 days pre-pandemic, p = 0.4). CONCLUSIONS: The COVID-19 pandemic was associated with significantly decreased incidence of HAEC admissions across US children's hospitals. Possible etiologies such as social distancing should be explored. LEVEL OF EVIDENCE: II.


Assuntos
COVID-19 , Enterocolite , Doença de Hirschsprung , Perfuração Intestinal , Humanos , Criança , Incidência , Estudos Retrospectivos , Perfuração Intestinal/epidemiologia , Pandemias , COVID-19/epidemiologia , Enterocolite/epidemiologia , Enterocolite/etiologia , Doença de Hirschsprung/complicações , Hospitais Pediátricos
12.
Acta Paediatr ; 112(7): 1558-1564, 2023 07.
Artigo em Inglês | MEDLINE | ID: mdl-36869615

RESUMO

AIM: To describe clinical presentation and development of tolerance among children with Food protein-induced enterocolitis syndrome (FPIES) in a population in northern Sweden. METHODS: A retrospective review of medical records of children presenting with FPIES symptoms from 1 January 2004, to 31 May 2018. RESULTS: Sixty children (65% boys) with FPIES were included. The estimated incidence gradually increased to 0.45% in 2016-2017. The most common food triggers were cow's milk (40%), fish (37%) and oat (23%). Symptoms presented in 31 (60%) children before 6 months and in 57 (95%) before 1 year of age. The median age for FPIES diagnosis was 7 (range 3-134) months and for fish FPIES 13 (range 7-134) months. By 3 years of age, 67% of children with FPIES to milk and oat but none of the children with fish FPIES had developed tolerance. Allergic conditions like eczema and asthma were reported in 52% of the children. CONCLUSION: The cumulative FPIES incidence was 0.45% in 2016-2017. Most children presented with symptoms before 1 year of age, but the diagnosis was often delayed, especially for FPIES to fish. Tolerance development occurred at an earlier age when FPIES was triggered by milk and oat compared to fish.


Assuntos
Enterocolite , Hipersensibilidade Alimentar , Feminino , Animais , Bovinos , Hipersensibilidade Alimentar/epidemiologia , Hipersensibilidade Alimentar/etiologia , Estudos Retrospectivos , Suécia/epidemiologia , Leite , Enterocolite/epidemiologia , Enterocolite/etiologia , Alérgenos , Proteínas na Dieta/efeitos adversos
13.
J Pediatr Surg ; 58(5): 856-861, 2023 May.
Artigo em Inglês | MEDLINE | ID: mdl-36801072

RESUMO

BACKGROUND/PURPOSE: A small number of Hirschsprung disease (HD) patients develop inflammatory bowel disease (IBD)-like symptoms after pullthrough surgery. The etiology and pathophysiology of Hirschsprung-associated IBD (HD-IBD) remains unknown. This study aims to further characterize HD-IBD, to identify potential risk factors and to evaluate response to treatment in a large group of patients. METHODS: Retrospective study of patients diagnosed with IBD after pullthrough surgery between 2000 and 2021 at 17 institutions. Data regarding clinical presentation and course of HD and IBD were reviewed. Effectiveness of medical therapy for IBD was recorded using a Likert scale. RESULTS: There were 55 patients (78% male). 50% (n = 28) had long segment disease. Hirschsprung-associated enterocolitis (HAEC) was reported in 68% (n = 36). Ten patients (18%) had Trisomy 21. IBD was diagnosed after age 5 in 63% (n = 34). IBD presentation consisted of colonic or small bowel inflammation resembling IBD in 69% (n = 38), unexplained or persistent fistula in 18% (n = 10) and unexplained HAEC >5 years old or unresponsive to standard treatment in 13% (n = 7). Biological agents were the most effective (80%) medications. A third of patients required a surgical procedure for IBD. CONCLUSION: More than half of the patients were diagnosed with HD-IBD after 5 years old. Long segment disease, HAEC after pull through operation and trisomy 21 may represent risk factors for this condition. Investigation for possible IBD should be considered in children with unexplained fistulae, HAEC beyond the age of 5 or unresponsive to standard therapy, and symptoms suggestive of IBD. Biological agents were the most effective medical treatment. LEVEL OF EVIDENCE: Level 4.


Assuntos
Síndrome de Down , Enterocolite , Doença de Hirschsprung , Doenças Inflamatórias Intestinais , Criança , Humanos , Masculino , Lactente , Pré-Escolar , Feminino , Doença de Hirschsprung/complicações , Doença de Hirschsprung/cirurgia , Doença de Hirschsprung/diagnóstico , Síndrome de Down/complicações , Estudos Retrospectivos , Opinião Pública , Enterocolite/epidemiologia , Enterocolite/etiologia , Enterocolite/diagnóstico , Doenças Inflamatórias Intestinais/complicações , Fatores Biológicos
14.
J Allergy Clin Immunol ; 151(1): 43-46, 2023 01.
Artigo em Inglês | MEDLINE | ID: mdl-36608982

RESUMO

Food protein-induced enterocolitis syndrome (FPIES) was first described in detail in the late 20th century as a non-IgE-mediated food allergy characterized by delayed gastrointestinal symptoms after ingestion of a trigger food. Although the initial case series reported infants reacting to cow's milk- and soy-based formulas, we now recognize that FPIES affects patients across the age spectrum. This brief review highlights our evolving understanding of FPIES with a discussion of triggers, epidemiology, food challenges, and pathophysiology.


Assuntos
Enterocolite , Hipersensibilidade Alimentar , Feminino , Animais , Bovinos , Hipersensibilidade Alimentar/epidemiologia , Síndrome , Leite , Enterocolite/epidemiologia , Alérgenos , Proteínas na Dieta/efeitos adversos
15.
Allergy ; 78(6): 1595-1604, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-36635218

RESUMO

BACKGROUND: The microbiome associations of food protein-induced enterocolitis syndrome (FPIES) are understudied. We sought to prospectively define the clinical features of FPIES in a birth cohort, and investigate for the evidence of gut dysbiosis. METHODS: We identified children diagnosed with FPIES in the Gastrointestinal Microbiome and Allergic Proctocolitis Study, a healthy infant cohort. Children were assessed and stools were collected at each well child visit. The clinical features of the children with FPIES were summarized. Stool microbiome was analyzed using 16S rRNA sequencing comparing children with and without FPIES. RESULTS: Of the 874 children followed up for 3 years, 8 FPIES cases (4 male) were identified, yielding a cumulative incidence of 0.92%. The most common triggers were oat and rice (n = 3, each) followed by milk (n = 2). The children with FPIES were more likely to have family history of food allergy (50% vs. 15.9% among unaffected, p = .03). The average age of disease presentation was 6 months old. During the first 6 months of life, stool from children with FPIES contained significantly less Bifidobacterium adolescentis, but more pathobionts, including Bacteroides spp. (especially Bacteroides fragilis), Holdemania spp., Lachnobacterium spp., and Acinetobacter lwoffii. The short-chain fatty acid (SCFA)-producing Bifidobacterium shunt was expressed significantly less in the stool from FPIES children. CONCLUSIONS: In this cohort, the cumulative incidence over the 3-year study period was 0.92%. During the first 6 months of life, children with FPIES had evidence of dysbiosis and SCFA production pathway was expressed less in their stool, which may play an important role in the pathogenesis of FPIES.


Assuntos
Enterocolite , Hipersensibilidade Alimentar , Criança , Humanos , Lactente , Masculino , Estudos Prospectivos , Disbiose , RNA Ribossômico 16S/genética , Proteínas na Dieta/efeitos adversos , Síndrome , Hipersensibilidade Alimentar/diagnóstico , Enterocolite/epidemiologia , Enterocolite/etiologia , Enterocolite/diagnóstico , Alérgenos
17.
J Pediatr Surg ; 58(8): 1458-1462, 2023 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-36371352

RESUMO

BACKGROUND: Hirschsprung-associated enterocolitis (HAEC) is the most common cause of morbidity and mortality in patients with Hirschsprung disease (HD). The objective of this study was to examine the association of social determinants of health (SDOH) with HAEC. METHODS: A review of patients who underwent primary pull through for HD at our institution from 2014 to 2021 was performed. Clinical, surgical, and SDOH data were collected. HAEC was defined by an international scoring system. Categorical variables were analyzed via Fisher's exact tests and continuous variables with Mood's median tests. RESULTS: One hundred patients were identified with 29 patients (29%) having at least one episode of HAEC during a median follow-up of 31 months (IQR: 11.7-55.7). Children who utilized public transportation for clinic visits, had one or more missed appointments, had any reported safety concerns, were involved with Child Protective Services, had parents/guardians who were not married, lived with people other than their immediate family, or had mothers who reported drug use or lack of prenatal care were found to have a higher likelihood of developing HAEC (p<0.04 for all). Age at HD diagnosis, age at pull through, operative approach, length of aganglionic colon, and Trisomy 21 were not significant predictors of HAEC. CONCLUSIONS: In our series of 100 patients undergoing primary pull through, there was a significant correlation of HAEC with several social determinants of health elements while anatomical and clinical factors were not associated with HAEC. Attention to social determinants of health and identifying high-risk patients may serve to prevent morbidity and mortality from HAEC. LEVEL OF EVIDENCE: III.


Assuntos
Síndrome de Down , Enterocolite , Doença de Hirschsprung , Criança , Humanos , Lactente , Determinantes Sociais da Saúde , Doença de Hirschsprung/complicações , Doença de Hirschsprung/cirurgia , Enterocolite/epidemiologia , Enterocolite/etiologia , Enterocolite/diagnóstico , Síndrome de Down/complicações , Fatores de Risco
18.
Rev Alerg Mex ; 70(4): 222-224, 2023 Dec 31.
Artigo em Espanhol | MEDLINE | ID: mdl-38506860

RESUMO

The prevalence of food allergy has increased in some regions of the world, and with it the incidence, according to geographical variability, in the phenotype and clinical manifestations. Food allergy arises from the specific immune response induced by exposure to the proteins of a certain food. Food intolerance refers to non-immune reactions, caused by unique physiological characteristics of the individual, including metabolic, toxic, pharmacological and undefined mechanisms. Adverse reactions to foods are classified as: IgE-mediated: Type I Hypersensitivity, non-IgE-mediated: Type IV Hypersensitivity, mixed: Types I and IV Hypersensitivity Non-Allergic; toxic, pharmacological, metabolic, intolerances. These types of alterations are rare but have increased in recent years; These include protein-induced enterocolitis syndrome, which can cause emesis, diarrhea and hypotension, and shock, which begins two hours after ingestion of the allergen. Protein-induced allergic proctocolitis is a condition that includes allergy to cow's milk protein. Delayed reactions usually affect the digestive system, are more insidious in their onset and are not immediately controlled, even with the suspension of food. There are eight foods responsible for 90% of food allergies: milk, eggs, soy, wheat, peanuts, walnuts, fish, and shellfish.


La prevalencia de alergia alimentaria se ha incrementado en algunas regiones del mundo, y con ello la incidencia, según la variabilidad geográfica, en el fenotipo y manifestaciones clínicas. La alergia alimentaria surge de la respuesta inmune específica inducida por la exposición a las proteínas de cierto alimento. La intolerancia alimentaria se refiere a reacciones no inmunitarias, causadas por características fisiológicas únicas del individuo, que incluyen mecanismos metabólicos, tóxicos, farmacológicos e indefinidos. Las reacciones adversas a los alimentos se clasifican en: mediada por IgE: Hipersensibilidad Tipo I, no mediada por IgE: Hipersensibilidad Tipo IV, mixtas: Hipersensibilidad Tipos I y IV No Alérgicas; tóxicas, farmacológicas, metabólicas, intolerancias. Este tipo de alteraciones son poco frecuentes, pero se ha incrementado en los últimos años; entre estas se encuentra el síndrome de enterocolitis inducida por proteínas, que puede producir emesis, diarrea e hipotensión, y estado de shock, que inicia dos horas después de la ingestión del alergeno. La proctocolitis alérgica inducida por proteínas es una afectación que incluye la alergia a la proteína de leche de vaca. Las reacciones retardadas suelen afectar el aparato digestivo, son más insidiosas en su inicio y no se controlan inmediatamente, aún con la suspensión del alimento. Existen ocho alimentos responsables del 90% de alergia alimentaria: leche, huevo, soya, trigo, cacahuate, nuez, pescados y mariscos.


Assuntos
Enterocolite , Hipersensibilidade Alimentar , Hipersensibilidade Imediata , Animais , Bovinos , Feminino , Diarreia , Enterocolite/epidemiologia , Enterocolite/etiologia , Leite
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