SARS-CoV-2 precipitated Kasabach-Merritt syndrome in a child with angioma infantile and acute lymphoblastic leukemia: Case Report

Background: We describe an 8-month-old boy with leukemia and SARS-CoV-2 infection who developed Kasabach-Merritt phenomenon. He had a positive SARS-COV-2 RT-PCR sample. Hematologic tests showed coagulopathy and intestinal involvement. She was managed in emergency receiving transfusion support and in hospitalization with social isolation measures, she started propanolol and corticotherapy as initial treatment of infantile angiomas. She presented with symptoms of intestinal obstruction and underwent surgery and evidence of hemorrhagic infarction with foci of intestinal ischemic necrosis, ending in ileostomy. We tried to understand a pathophysiological explanation of the dermatologic and gastrointestinal tract involvement by the virus and the atypical form of COVID-19. Given the emerging evidence of endothelial and vascular involvement in COVID-19, the development of tests to detect vascular lesions may be critical to guide the use of new therapeutic strategies.

Compromiso gastrointestinal inusual en paciente pediátrico con vasculitis asociada a anticuerpos anticitoplasma de neutrófilos. Reporte de un caso

Vasculitis is characterized by presenting inflammation of the wall of blood vessels, one type of these diseases are those associated with anti-neutrophil cytoplasm antibodies. They usually occur in adulthood and are rare in childhood. The disease generally affects the lung, kidney, and skin, with gastrointestinal involvement being rare. Here we describe the case of a pediatric patient with gastrointestinal bleeding secondary to ANCA positive vasculitis. Endoscopy revealed patchy erythematous lesions and wall hematoma at the level of the colon. Although we report a fairly infrequent clinical condition, it is not without complications. We believe it is appropriate to suspect it, first of all, intestinal involvement where the underlying inflammatory process is not clear, even more so when it involves other organs.

SARS-CoV-2 precipitated Kasabach-Merritt syndrome in a child with Kaposiform Hemangiendothelioma and acute lymphoblastic leukemia

Kaposiform hemangiendothelioma usually occurs in children under two years of age and develops thrombocytopenia secondary to sequestration of platelets within (Kasabach-Merritt phenomenon) and is complicated by secondary consumption of fibrinogen and clotting factors. SARS-CoV-2 produces cutaneous endothelitis as a direct effect of the presence of the virus and the host inflammatory response. We describe an 8-month-old boy with leukemia and SARS-CoV-2 infection who developed Kasabach-Merritt phenomenon, coagulopathy, and intestinal involvement. Given the emerging evidence of endothelial and vascular involvement in COVID-19, the development of tests to detect vascular injury may be critical to guide the use of new therapeutic strategies.

Giant Mesenteric Hemangioma without Small Intestinal Involvement Report of a Case and Review of Literature

there were approximately twenty cases reported in the English literature, of which five cases were designated as giant hemangioma. We reported another giant mesenteric hemangioma, measuring 35x20x11 cm. with a weight of 5,5 Kilograms. The etiology of mesenteric hemangioma was still debatable. In our case, the patient only experienced mild abdominal pain, without gastrointestinal hemorrhage. Therefore, we were in favor of mesenteric origin as opposed to gastrointestinal origin. The symptoms were non-specific, ranging from abdominal pain, nausea, vomiting, and gastrointestinal bleeding if Gastrointestinal tract was involved. Preoperative diagnosis was very challenging despite the modern images’ technics have become available, such as computed tomography magnetic resonance and ultrasonography. The final diagnoses of mesenteric hemangioma have been exclusively established by histological examination after surgical removal of the tumor. Surgical resection with clear margin is the treatment of choice.

Aggressive infantile myofibromatosis with intestinal involvement

Background Infantile myofibromatosis (IM) is the most common cause of multiple fibrous tumors in infancy. Multicentric disease can be associated with life-threatening visceral lesions. Germline gain-of-function mutations in PDGFRB have been identified as the most common molecular defect in familial IM. Case presentation We here describe an infant with PDGFRB-driven IM with multiple tumors at different sites, including intestinal polyposis with hematochezia, necessitating temporary chemotherapy. Conclusions PDGFRB-driven IM is clinically challenging due to its fluctuating course and multiple organ involvement in the first years of life. Early molecular genetic analysis is necessary to consider tyrosine kinase inhibitor treatment in case of aggressive visceral lesions.

Frequency of involvement different parts of the gastrointestinal tract in the pathological process in children with biliary dysfunction

The close anatomical and physiological connection of the digestive organs leads to a significant spread of functional disorders in various diseases. Purpose — to investigate the frequency and features of the clinical course of biliary dysfunction (BD) in children. Materials and methods. 66 children aged 10–18 years with BD were examined. The study included a complete clinical examination of children, laboratory and instrumental methods. To assess the severity of clinical symptoms in the examined patients was used traditional score scale of symptoms (0–3 points) and the frequency index (FI). Results. In most children, the database was combined with other functional and organic lesions of the digestive tract (n=56, 84.8%). The database was most often combined with chronic gastritis and duodenitis, as well as with functional motor disorders. FI in children with BD involved in the pathological process of the stomach and duodenum was the highest (0.59), and IR in children with BD and intestinal involvement was the lowest (0.23). The relationship between the frequency of combined pathology and the frequency of cases of increase in the size of the gallbladder (χ2=22.87 at a critical value of χ2=9.33 for the significance level p<0.01). Hyper- or hypofunctions of the biliary tract occurred with the same frequency. Conclusions. Biliary dysfunction in children is significantly more often combined with chronic gastritis and duodenitis, as well as with other functional disorders of the gastrointestinal tract (duodenogastric reflux). In children with biliary dysfunction and involvement in the pathological process of other parts of the gastrointestinal tract there is an increase in the frequency of exacerbations, more pronounced signs of dyspeptic syndrome on the background of pain with the same intensity and more often ultrasound reveals signs of gallbladder dysformation. The research was carried out in accordance with the principles of the Helsinki declaration. The study protocol was approved by the Local Ethics Committee of all participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: children, biliary dysfunction.

POS0427 CLINICAL CHARACTERISTICS OF PATIENTS WITH SYSTEMIC SCLEROSIS AND GASTRIC ANTRAL VASCULAR ECTASIA (GAVE)

Background:Gastric antral vascular ectasia (GAVE) is one of the gastrointestinal (GI) manifestations related to systemic sclerosis (SSc). It can be presented as iron deficiency anemia or even upper gastrointestinal bleeding. GAVE is diagnosed by endoscopy observing an image of confluent vascular ectasias that is oriented longitudinally on the folds of the antrum in the appearance of “watermelon”. The definitive treatment for this manifestation consists in endoscopy guided fulguration when the clinical situation allows it.Objectives:The objective was to study a cohort of SSc patients at their first endoscopy. The clinical characteristics, laboratory tests and treatments received from SSc patients with GAVE were compared to those without this GI manifestation.Methods:From the cohort of patients with SSc in Hospital Universitari Vall d’Hebron, a total of 269 patients who had undergone at least one endoscopy during follow-up were selected. Twenty seven were diagnosed with GAVE. We compared the clinical, analytical and treatment characteristics of these patients with the remaining 242 who did not present GAVE. The statistical study was carried out using the SPSS 20.0 package (Chicago, IL), a p <0.05 was considered as statistical significance.Results:The prevalence of GAVE in SSc patients was 10.0%. Patients with GAVE had a higher median age SSc onset taking into account the first non-Raynaud’s phenomenon (RP) symptom attributable to the disease (56.6 vs 48.0 years, p = 0.001). The median age at first endoscopy was 56.5 years in GAVE group compared with 61.7 in the group without GAVE.Compared with SSc patients without GAVE, patients with GAVE had a higher prevalence of Barrett’s esophagus (14.8% vs. 3.7%, p = 0.011), intestinal involvement (37% vs. 18.6%, p = 0.024) and a trend towards a lower prevalence of interstitial lung disease (25.9% vs 45.0%, p = 0.057).No difference was identified in the prevalence of scleroderma renal crisis. Patients with GAVE presented a higher frequency of early or active Cutolo capillaroscopy pattern with a predominance of enlarged capillaries or megacapillaries (84.6% vs 62.4%, p = 0.025), greater frequency of anti-centromere antibodies (63.0% vs. 42.1%, p = 0.039) and a trend towards a lower proportion of anti-topoisomerase I (3.7% vs. 18.6%, p = 0.052). No difference was found in prevalence of anti-RNA polymerase III antibodies between groups. Patients with GAVE were treated less frequently with non-glucocorticoid immunosuppressants prior to diagnostic endoscopy (0% vs 20.2%, p = 0.010). The 33.3% of patients with GAVE were treated with endoscopic fulguration, and 66.7% of them received supplementary treatment with oral iron.Conclusion:SSc patients with GAVE had higher age at SSc onset, more frequency of Barrett’s esophagus and intestinal involvement, prevalence of anti-centromere antibodies, early or active Cutolo scleroderma pattern and lower prior non-glucocorticoids treatment.References:[1]Ghrénassia E, Avouac J, Khanna D, T.Derk C, Distler O, Suliman Y, et al. Prevalence, Correlates and Outcomes of Gastric Antral Vascular Ectasia in Systemic Sclerosis: A EUSTAR Case-control Study. The Journal of Rheumatology. 2014; 41:1.Disclosure of Interests:None declared