Eosinophilia in infants with food protein-induced enterocolitis syndrome in Japan

Background

Many Japanese infants with food protein-induced enterocolitis syndrome (FPIES) show eosinophilia, which has been thought to be a characteristic of food protein-induced proctocolitis (FPIP).

Cytokine profile after oral food challenge in infants with food protein-induced enterocolitis syndrome

BackgroundAlthough food protein-induced enterocolitis syndrome (FPIES) is supposed to be caused by inflammation, the role of cytokines has not yet been clarified.MethodsTo elucidate the role of cytokines in the development of symptoms and abnormal laboratory findings at an oral food challenge (OFC), changes in serum cytokine levels were analyzed for 6 OFCs in 4 patients with FPIES. The result of OFC was judged positive if any gastrointestinal (GI) symptoms (vomiting, diarrhea, or bloody stool) were induced.ResultsAmong 11 cytokines profiled, serum levels of interleukin (IL)-2, IL-5, and IL-8 were clearly increased in all 4 positive OFCs in which elevations of the serum level of C-reactive protein (CRP) and peripheral blood neutrophilia were also seen. The level of serum IL-10 also rose in 2 positive OFCs. Remarkable increases in the serum level of interferon-gamma (IFN-γ), tumor necrosis factor-alpha (TNF-α), IL-6, and IL-12 were observed in a positive OFC where the serum level of CRP rose markedly (6.75 mg/dL). The serum levels of IL-5 were also elevated in 2 negative OFCs. No apparent specific correlations were found between cytokines and GI symptoms.ConclusionsThese results suggest that IL-2 and IL-8 are involved in the antigen-specific immune responses in most patients with FPIES. Further studies are needed to elucidate the significance of these cytokine in the pathogenesis of FPIES.     

Absorption of food protein antigen in infants with food protein-induced enterocolitis

Increased gastrointestinal absorption of intact antigen with systemic immunization has been considered a major etiologic factor in the development of food sensitivity. We attempted to test this hypothesis in infants with suspected food protein-induced entercolitis by measuring serum ovalbumin (OVA) concentrations after ingestion of egg white (prior to the performance of good challenges to establish this diagnosis). We first noted significant underestimation of serum OVA concentrations in the presence of even low serum anti-OVA antibody concentrations (greater than 1:12). Next, using selected noninhibitory sera, we found that all infants studied absorbed some OVA, there was no correlation between serum OVA levels and age (3-11 months), and there was no significant difference between serum OVA concentrations in infants who subsequently had positive oral food challenge responses (120 +/- 67 ng/ml) and a matched group with negative challenges (102 +/- 80). These data do not support the hypothesis that "intestinal closure" (antigen exclusion) occurs in the neonatal period or the role of antigen absorption as the major etiological factor in the development of food sensitivity. Better methods of quantitating macromolecular absorption must be developed before the role of antigen absorption in food sensitivity can be assessed. Of note, urinary excretion of intact OVA also occurred. This varied greatly from one voiding to the next and continuing for at least 13 hr after ingestion.     

Comparison of methemoglobin levels in food protein-induced enterocolitis syndrome and other gastrointestinal diseases in neonates

Introduction and objectivesMethemoglobinemia has been reported to be associated with severe food protein-induced enterocolitis syndrome (FPIES). However, no reports have evaluated methemoglobin (MHb) levels in FPIES without symptomatic methemoglobinemia or the usefulness of MHb measurement for the diagnostic prediction of FPIES. To evaluate the MHb levels of patients with neonatal-onset FPIES and determine whether MHb levels are higher in FPIES than in other gastrointestinal diseases.Patients and methodsEleven neonates with severe acute FPIES (FPIES group) and 139 neonates with other gastrointestinal diseases (non-FPIES group) were included in this study. Patient characteristics, symptoms, and venous blood test values (MHb, pH, HCO₃⁻, and C-reactive protein) were evaluated.ResultsThe median age at onset was 16 days vs. 1 day; males comprised 64% vs. 46%, the median gestational age was 38 weeks vs. 38 weeks, the median birth weight was 2710 g vs. 2880 g, and the median hospitalization duration was 31 days vs. 6 days for the FPIES vs. non-FPIES groups, respectively. MHb (%) was higher in the FPIES group than in the non-FPIES group [median (range), 1.1 (0.6–10.9) and 0.6 (0.3–1.2), respectively, p < 0.001]. There were no differences in terms of pH, HCO₃⁻, and C-reactive protein (p > 0.05). In the receiver operating characteristic analysis for FPIES diagnosis based on MHb (%), the area under the curve was 0.885, specificity was 97.1%, and sensitivity was 72.7% at a MHb cutoff of 1.0.ConclusionHigh MHb levels may help diagnose severe acute FPIES in neonates, but careful evaluation is needed.     

Necrotizing enterocolitis in an infant with Omenn syndrome.

Omenn syndrome (OS) is a rare disorder within the combined immunodeficiency family that is characterized by a diffuse exudative, erythematous rash, lymphadenopathy, hepatosplenomegaly, alopecia, and failure to thrive. Specific lab findings unique to OS include hypereosinophilia, elevated IgE, excess production of oligoclonal T-cells and near-to-absent B-cells. Much remains elucidated about the underlying genetic cause of OS. Until recently, it was felt that the disease was primarily caused by mutations of the RAG1 or RAG2 genes. The type of mutation of the RAG1 and RAG2 genes in patients with OS affects the degree of functioning variable (diversity) joining [V(D)J] recombination activity, which is critical to the development of lymphoid cell receptor diversity. New work has also shown that thymic tissue in OS patients demonstrates a severe defect in the expression of the autoimmune regulator element. This may contribute to the development of autoreactive T-cells that are felt to be the causative agent of a number of the clinical hallmarks unique to OS. The genetic spectrum of OS was further expanded when a patient with clinical and immunologic features consistent with OS, without RAG mutation, was found to have mutations in both alleles coding for ARTEMIS, a key V(D)J recombination/DNA repair factor. Regardless of the underlying cause, early recognition is critical because patients die at a very young age without bone marrow transplantation. We describe an infant diagnosed with OS post-mortem in which death was directly related to the development of necrotizing enterocolitis.     

Loss of tolerance for fishes previously tolerated in children with fish food protein induced enterocolitis syndrome

We describe two case reports presenting some novel information on fish FPIES. Fish FPIES to one fish does not always start at the same time to other fish. Additionally, development of tolerance to the index fish do not necessarily imply tolerance to other reactive fish. This reflects on the best management of children with FPIES fish.     

Auranofin (SK + F 39162) induced enterocolitis in rheumatoid arthritis. A case report

A case of gold-induced enterocolitis in a 69-year-old female with rheumatoid arthritis who had received the new gold compound by oral administration (Auranofin (AF) for 4 weeks) is reported. Mild diarrhoea is a commonly described side effect accompanying AF, but in this case a severe colitis developed. The course of this case is very similar to those reported regarding Myochrysin. The pathogenesis is discussed. It might be a direct toxic effect on the mucosa of the bowel, evidenced by the relatively high excretion of oral gold with faeces.     

Powdered infant formula as a source of Salmonella infection in infants.

Powdered infant formula is not sterile and may be intrinsically contaminated with pathogens, such as Salmonella enterica, that can cause serious illness in infants. In recent years, at least 6 outbreaks of Salmonella infection in infants that have been linked to the consumption of powdered infant formula have been reported. Many of these outbreaks were identified because the Salmonella strains were unique in some way (e.g., a rare serotype) and a well-established Salmonella surveillance network, supported by laboratories capable of serotyping isolates, was in place. Another common feature of the outbreaks was the low level of salmonellae detected in the implicated formula (salmonellae may be missed in routine testing). These outbreaks likely represent only a small proportion of the actual number of Salmonella infections in infants that have been linked to powdered infant formula. Managing this problem requires a multidimensional approach in which manufacturers, regulators, and caregivers to infants can all play a role.     

Necrotizing enterocolitis: an unusual cause of rectal bleeding in a term infant

A 5-day-old term infant presented to the emergency department with a seven-hour history of rectal bleeding. He had had an uncomplicated prenatal course with no significant risk factors during the third trimester or perinatal period. The infant presented in minimal distress but was admitted for further workup and evaluation. The workup revealed necrotizing enterocolitis, which ultimately required surgical management. The infant was discharged with a colostomy, and then was readmitted ten weeks later for reanastomosis.     

Alcohol as a risk factor for sudden infant death syndrome (SIDS)

Aim To test whether alcohol is a risk factor for sudden infant death syndrome (SIDS). Design and setting US epidemiological study using computerized death certificates, linked birth and infant death dataset, and Fatality Analysis Reporting System. Participants All SIDS cases (n = 129 090) and other infant deaths (n = 295 151) from 1973–2006; all persons involved in late‐night alcohol‐related crashes (n = 135 946) from 1994–2008. Measurements Three measures were used: the expected number of deaths on New Year versus the observed number (expected values were determined using a locally weighted scatterplot smoothing polynomial), the average number of weekend deaths versus the average number of weekday deaths, and the SIDS death rate for children of alcohol‐consuming versus non‐alcohol‐consuming mothers. Findings These measures indicate that the largest spikes in alcohol consumption and in SIDS (33%) occur on New Year, alcohol consumption and SIDS increase significantly on weekends, and children of alcohol‐consuming mothers are much more likely to die from SIDS than are children of non‐alcohol‐consuming mothers. Conclusions Alcohol consumption appears to be a risk factor for sudden infant death syndrome, although it is unclear whether alcohol is an independent risk factor, a risk factor only in conjunction with other known risk factors (like co‐sleeping), or a proxy for other risk factors associated with occasions when alcohol consumption increases (like smoking). Our findings suggest that caretakers and authorities should be informed that alcohol impairs parental capacity and might be a risk factor for sudden infant death syndrome; in addition, future research should further explore possible connections between sudden infant death syndrome and alcohol.     

Swyer-James (MacLeod's) syndrome following pertussis infection in an infant

Pertussis is a potentially severe disease, especially in infancy. The case of an 8-month-old infant is described who presented the typical radiographic image of unilateral hyperlucent lung or Swyer-James (MacLeod's) syndrome. The infant suffered from pertussis at 20 d of age. A rare postinfectious complication of pertussis is reported.     

Gulf War syndrome as a part of the autoimmune (autoinflammatory) syndrome induced by adjuvant (ASIA)

Gulf War syndrome (GWS) is a multi-symptom condition comprising a variety of signs and symptoms described in the literature, which not been fully resolved. The various symptoms of the condition include muscle fatigue and tiredness, malaise, myalgia, impaired cognition, ataxia, diarrhoea, bladder dysfunction, sweating disturbances, headaches, fever, arthralgia, skin rashes, and gastrointestinal and sleep disturbances. In addition, excessive chemical sensitivity and odour intolerance is reported. The aetiology of the condition is unclear, but many reviews and epidemiological analyses suggest association with pyridostigmine bromide (PB), certain vaccination regimes, a variety of possible chemical exposures, including smoke from oil-well fires or depleted uranium from shells, as well as physical and psychological stress. Recently, Shoenfeld et al. suggested that four conditions--siliconosis, macrophagic myofaciitis (MMF), GWS and post-vaccination phenomena--that share clinical and pathogenic resemblances, may be incorporated into common syndrome called 'Autoimmune (Autoinflammatory) Syndrome induced by Adjuvants' (ASIA). Symptoms and signs of the four conditions described by Shoenfeld et al. show that at least eight out of ten main symptoms are in correlation in all four conditions. Namely, myalgia, arthralgias, chronic fatigue, neurological cognitive impairment, gastrointestinal symptoms, respiratory symptoms, skin manifestations and appearance of autoantibodies. Regardless of the aetiology of GWS, be it exposure to environmental factors or chemical drugs, vaccinations or the adjuvants in them, GWS fits well with the definition of ASIA and is included as part of 'Shoenfeld's syndrome'.