Inflammatory myofibroblastic tumor of the midesophagus

An inflammatory myofibroblastic tumor (IMFT) is a rare entity that can arise in a multiplicity of organs including the lung, liver, and at any location within the gastrointestinal tract. Typically, an IMFT presents as a localized mass with clinical symptoms dependent upon its site of origin. IMFTs pathologically resemble a neoplastic process but are theorized to arise from an unknown inflammatory event. We present a case of a midesophageal IMFT in a 12-year-old female.     

Radiological appearance of inflammatory myofibroblastic tumour

Inflammatory myofibroblastic tumour (IMFT) is a distinct entity with variable clinical presentation and therapeutic options. We present three paediatric cases of IMFT, originated from the lung, bladder and ovary respectively. All lesions were heterogeneous, with mixed solid/cystic components and infiltrative pattern, and were interpreted as aggressive malignant neoplasms initially due to their bizarre imaging appearance. The definitive diagnosis was derived from characteristic histopathological features. Pediatr Blood Cancer 2010;54:1029–1031 © 2010 Wiley‐Liss, Inc.     

Inflammatory polyps following successful HLA-matched cord blood transplantation in a patient with X-linked lymphoproliferative syndrome

Gastrointestinal complications following HSCT are numerous and include a variety of issues resulting in hepatic, biliary, pancreatic, and intestinal compromise. In the context of an underlying state of immune dysregulation, novel complications may arise including autoimmunity. To our knowledge, this is the first report of a patient with XLP who was successfully treated with HSCT using an HLA-matched unrelated cord blood unit that was complicated by the development of inflammatory polyps of the colon. Given the underlying diagnosis of XLP and its associated immune dysregulation, the challenge of understanding unique gastrointestinal manifestations of autoimmunity following HSCT is discussed.     

Stertor in the newborn due to congenital nasal pyriform aperture estenosis: case series

Stertor is a noise generated by the disturbance of the air flow passing through the nose. Its main cause -in newborns and infants- is inflammatory or infectious rhinitis. Congenital, neoplastic, traumatic or iatrogenic causes are less frequent. Congenital stenosis of the pyriform aperture is a rare etiology of nasal obstruction in the neonates. Early diagnosis and appropriate treatment are essential because of their exclusive nasal breathing. Suspicion might arise when a difficulty or even an impossibility to pass a probe of 2.8 mm (K30 tube) through anterior nares, exists. Diagnosis should be confirmed by a computed tomography of the craniofacial massif. The therapeutic behavior will depend on the severity of symptoms. We describe our experience with nine patients with this condition whose surgical correction was successful.     

Mechanisms of autoimmune hepatitis

Autoimmune hepatitis (AIH) is a progressive inflammatory liver disease with a female preponderance, responsive to immunosuppressive treatment. Two types of AIH are described: type 1 AIH is characterized by positivity for smooth muscle and/or antinuclear antibody, while type 2 AIH is positive for liver kidney microsomal type 1 antibody. The putative mechanisms leading to the development of this condition include genetic predisposition to autoimmunity through possession of specific human leukocyte antigen alleles, immune reactions to liver cell antigens, possibly triggered by a mechanism of molecular mimicry, where immune responses to external pathogens, e.g. viruses, become directed toward structurally similar self-components, and an impairment in immune regulation. AIH has been described to arise de novo after liver transplantation. The mechanisms leading to post-transplant autoimmunity remain to be defined.     

Gastrointestinal polyposis in infancy and childhood

The clinical and pathological features in 12 children with symptomatic gastrointestinal polyposis are presented. Five children with Peutz-Jeghers syndrome all presented with intussusception. Three children with Gardner syndrome presented with multiple large epidermoid cysts of the scalp. The other 4 with juvenile polyposis all presented with anaemia, often associated with growth retardation and extragastrointestinal abnormalities. One of these 4, as well as her mother, had Osler disease with pulmonary arteriovenous malformation. Some degree for overlap in the histology was noted between the polyps in the three conditions, and confusion may arise if only small areas are examined. The histological evolution of small lesions in juvenile polyposis and the surgical observation of friability of adjacent non-polypoid mucosa and association with multiple disorders, local as well as generalised, mechanical as well as inflammatory, suggest these to be the result of non-specific ulceration followed by granulation tissue formation, inflammatory infiltration, and irregular epithelial regeneration.     

Inflammatory myofibroblastic tumor of the lung in children: anaplastic lymphoma kinase (ALK) expression and clinico-pathological correlation

The inflammatory myofibroblastic tumor (IMT) is a rare neoplastic lesion with a high incidence in children and young people, and may arise in lungs, soft tissue, or viscera. It is recognized as a borderline tumor with the possibility to recur, undergo malignant transformation, and metastasize. IMT is composed of fascicles of bland myofibroblastic cells admixed with an inflammatory infiltrate consisting of lymphocytes, plasma cells, and eosinophils. We reviewed pulmonary IMT diagnosed at Garrahan Hospital in Buenos Aires, Argentina, during 12 years and examined the clinical, laboratory, and pathological features as well as molecular genetics. Eight pediatric cases were evaluated with a male-to-female ratio of 5:3 and a median age of 6 years at diagnosis. The most common lung localization was the upper lobe. All cases underwent surgical excision and no local recurrences were found. Five out of eight patients, including two cases with metastatic/multifocal lesions in the central nervous system (CNS), are alive and disease free after a median follow-up of 30 months. Anaplastic lymphoma kinase (ALK) expression was negative in all pulmonary samples by immunohistochemistry (IHC), however, rearrangement for ALK locus by fluorescence in situ hybridization was found in one lung and in two CNS samples. These findings may reflect higher sensitivity of the molecular biologic procedure compare to traditional IHC practice. In our pediatric experience, 25% of patients with lung IMT developed CNS lesions; therefore we consider that CNS screening in these patients should be considered, at diagnosis and later during follow up.     

Ernährung und Chirurgie als Säulen der CED-Behandlung

Nutritional interventions and surgical therapy play an important role in the treatment of children and adolescents suffering from inflammatory bowel disease (IBD), especially because of the limited efficacy of many drugs and their possible side-effects. In all cases nutritional and calorific deficiencies have to be identified and corrected. Exclusively enteral nutrition with liquid formula for children with Crohn’s disease has the potential to induce remission in approximately 80% of cases, irrespective of disease location and severity. It has no side-effects and promotes growth. Surgical treatment of children suffering from IBD is mainly indicated in severe, treatment-resistant cases and when complications arise. Colectomy has the potential to heal the underlying ulcerative colitis but because of the surgical risks and postoperative problems it is only indicated in severe cases and in toxic megacolon. In Crohn’s disease surgery is important for the treatment of strictures and fistulas when other treatments fail. A common problem is the high recurrence rate after resection and a preventive treatment should be instituted. The article gives an overview on indications and modalities of non-pharmacological treatment of IBD in children.     

Histological assessment of bile lake formation after hepatic portoenterostomy for biliary atresia

Bile lakes develop after hepatic portoenterostomy in some patients with biliary atresia, and have been regarded as an indication of poor prognosis. We reported that bile lakes have no epithelium of the bile duct on their wall, and are surrounded by bile ducts; however, the mechanism of bile lake formation is little known. We investigated histologically how bile ducts are formed using whole removed liver, and the characteristics of bile ducts around bile lakes. From April 1980 to July 2006, we encountered 84 patients with biliary atresia. Bile lakes were analyzed histologically in 11 patients who underwent liver transplantation in our hospital. Bile lakes had a fibrotic cyst wall and lacked epithelia. In most cases, bile stasis, calculi formation, damaged bile ducts, and invasion of inflammatory cells were observed around the bile lakes. Bile ducts around bile lakes were not stained by CD56, but bile ducts around liver lobuli were stained by CD56. The present study speculates that bile lakes would arise from original bile ducts, which are damaged, and fuse together after calculi are formed in bile ducts.     

Iron and the liver

Iron is an important bio-catalyst of oxidation-reduction reactions in the cell and is essential for life. Paradoxically, it may also be lethal when the fraction of redox-active metal ions exceeds that sequestered in specialized proteins or cellular compartments, and uncontrolled production of free radical species may arise. The liver is the main body site for iron stores and central in the regulation of iron homeostasis. Important iron-proteins, such as hepcidin, the iron regulatory hormone, are specifically produced by the liver. Pathogenic mutations in hepatic iron transporters and regulators lead to hereditary iron overload diseases, including hemochromatosis. Iron toxicity depends on its excessive accumulation and is due to promotion of oxidant stress: free radicals and membrane oxidation by-products cause hepatocellular death by triggering organelle dysfunction, or by activating cells involved in hepatic inflammation and fibrogenesis, such as Kupffer cells and hepatic stellate cells. Xenobiotics and hepatotoxins as well as immunological and host defense mechanisms may cause subtle changes in the pool of redox-active metal ions and in metal compartmentalization that potentially contribute to hepatotoxic, inflammatory and fibrogenic events. The hepatotoxic and profibrogenic potential of metal ions, particularly iron, is dramatic at moderate levels of tissue metal overload in concomitance with other inciting insults, such as alcohol abuse and viral hepatitis. Removal of metal excess from the liver in iron overload diseases is beneficial and prevents progression toward cirrhosis. The development of drugs able to block catalytically active metals, particularly iron, may prove effective in other chronic liver diseases in which inflammatory, degenerative and fibrogenic processes are fueled by redox-active metal ions.     

Dorsalgies révélant une maladie de Takayasu

Takayasu arteritis is an uncommon inflammatory arteritis especially in children. We report a case. CASE REPORT: A 11-year-old boy presented dorsalgia with inflammatory syndrome. One year later, the investigation of an hypertension with asymmetric blood pressure revealed an aortic coarctation and a bilateral renal arteries stenosis leading to Takayasu's arteritis diagnosis. CONCLUSION: Takayasu's arteritis must be evokated in young children in case of associated hypertension and inflammatory syndrome.     

脂氧素与肾脏病研究进展

脂氧素能广泛调节多种炎症细胞的功能和炎症相关基因的表达,并作用于多种细胞促进炎症的消退.脂氧素在肾脏病的发病机制、临床治疗中起重要作用.     

Rathke's cleft cyst abscess

Pituitary abscesses are rare. Occasionally they will arise in pre-existing pituitary pathology. We report such an occurrence within a Rathke's cleft cyst. On the basis of history and imaging, this was indistinguishable from more commonly encountered pituitary pathology.     

INFLAMMATORY MYOFIBROBLASTIC TUMOR OF THE GASTROESOPHAGEAL JUNCTION IN CHILDHOOD

We report an unusual case of an inflammatory myofibroblastic tumor arising at the gastroesophageal junction in a 14-year-old girl. The bland histologic appearance with concurrent infiltration into adjacent structures made diagnostic interpretation difficult, but suggested a neoplastic process. A literature review was undertaken to address diagnostic and management issues raised in this case. Although the anatomic location was unusual, clinical, gross, histopathologic, and immunohistochemical data substantiated the diagnosis of inflammatory myofibroblastic tumor. The bland histologic appearance was consistent with the most widely accepted view of inflammatory myofibroblastic tumor as a low-grade neoplasm. Wide surgical excision was performed. This is considered the preferred treatment given the potential risk of recurrence and aggressive behavior, most frequently noted with extrapulmonary disease. Although inflammatory myofibroblastic tumor represents an heterologous spectrum of benign to malignant neoplastic proliferations, the prognosis is good in cases with benign histologic features.     

New insights into the pathogenesis of pulmonary inflammation in preterm infants

Chronic lung disease (CLD) and bronchopulmonary dysplasia are associated with a significant inflammatory response of the airways and the interstitium of the lungs. Besides inflammatory cells, various cytokines, lipid mediators, proteolytic enzymes and toxic oxygen radicals may play an essential role in the pathogenesis of this disease. Intrauterine exposure to chorioamnionitis or proinflammatory cytokines has been shown to induce a pulmonary and systemic inflammatory response in the fetus. In this subgroup, antenatal infection may prime the lung such that minimally injurious postnatal events provoke an excessive inflammatory response in the airways and the pulmonary tissue. Inflammation and lung injury most certainly affect normal alveolization and pulmonary vascular development in preterm infants with CLD.     

In utero origins of childhood leukaemia

Chimaeric fusion genes derived by chromosome translocation are common molecular abnormalities in paediatric leukaemia and provide unique markers for the malignant clone. They have been especially informative in studies with twins concordant for leukaemia and in retrospective scrutiny of archived neonatal blood spots. These data have indicated that, in paediatric leukaemia, the majority of chromosome translocations arise in utero during foetal haemopoiesis. Chromosomal translocations and preleukaemic clones arise at a substantially higher frequency ( approximately 100x) before birth than the cumulative incidence or risk of disease, reflecting the requirement for complementary and secondary genetic events that occur postnatally. A consequence of the latter is a very variable and occasionally protracted postnatal latency of disease (1-15 years). These natural histories provide an important framework for consideration of key aetiological events in paediatric leukaemia.     

No evidence for a clear link between active intestinal inflammation and autism based on analyses of faecal calprotectin and rectal nitric oxide

AIM: Due to parental concern regarding the child's bowel habits and the ongoing discussion whether there might be an association between autism and intestinal inflammation, two inflammatory markers were analysed in a group of children with autism. METHODS: Twenty-four consecutive children with autism (3-13 years) of unknown aetiology were investigated with respect to faecal calprotectin and rectal nitric oxide (NO). RESULTS: One child who previously had a severe Clostridium difficile infection displayed raised levels of both these inflammatory markers and one child with extreme constipation for whom only calprotectin was possible to measure had raised levels. The remaining children displayed results that did not indicate an active inflammatory status in the intestine when the two inflammatory markers were combined. CONCLUSION: By the use of two independent markers of inflammatory reactions in the gut, i.e. rectal NO and faecal calprotectin we were not able to disclose evidence of a link between the autistic disorder and active intestinal inflammation.     

硫化氢在炎症中的作用研究进展

硫化氢(H2S)是继一氧化氮和一氧化碳之后发现的气体信号分子,有着广泛的生物学活性.H2S在炎症细胞、炎症介质、血管张力和信号通路等方面参与了炎症反应的各个环节,发挥双向的调节作用,包括抗炎和促炎作用.不同的动物炎症模型中H2S所起的作用不同.深入探讨H2S在炎症中的效应及作用机制,可为炎症疾病的治疗提供新的防治措施.     

Editorial: Hypothesis and Empiricism

The article by S. F. Cramer, The Melanocytic Differentiation Pathway in Congenital Melanocytic Nevi: Theoretical Considerations (page 253), is an exercise in hypothesis. Cramer has constructed an elegant and detailed model for the pathogenesis of melanocytic nevi in this and foregoing papers.^1,2 His hypothesis recognizes that skin melanocytes develop from the migration of neural crest precursors into the epidermis. The melanocyte precursors are presumed to arise directly from the neural crest components in the perineural sheaths of dermal nerve endings. He proposes that nevi arise by an aberration or disruption of this migratory process, which he defines as the “melanocyte differentiation pathway”. The form and nature of the nevi are dependent on the stage of development and the extent of the derangement of this migratory and differentiation process.     

Inflammatory fibroid polyp of the stomach as an unusual differential diagnosis of epigastric tumor

We describe the case of a 5 months old infant with an inflammatory fibroid polyp of the stomach. The girl presented with inflammatory symptoms and a tumor in the upper abdomen which was confirmed by sonography and computer-tomography. This very rare benign tumor has not yet been described in an infant.