Severe non-hepatic hyperammonaemic encephalopathy in an immunocompromised adolescent with enterocolitis.

Non-hepatic causes of hyperammonaemia are uncommon relative to hepatic aetiologies. An adolescent female was admitted to the hospital with a diagnosis of very severe aplastic anaemia. During her treatment with immunosuppressive therapy, she developed neutropenic enterocolitis, pseudomonal bacteraemia and hyperammonaemia. A combination of intermittent haemodialysis and high-volume continuous veno-venous haemodiafiltration (CVVHDF) was required to manage the hyperammonaemia. Despite a thorough investigation, there were no hepatic, metabolic or genetic aetiologies identified that explained the hyperammonaemia. The hyperammonaemia resolved only after the surgical resection of her inflamed colon, following which she was successfully weaned off from the renal support. This is a novel case report of hyperammonaemia of non-hepatic origin secondary to widespread inflammation of the colon requiring surgical resection in an immunocompromised patient. This case also highlights the role of high-volume CVVHDF in augmenting haemodialysis in the management of severe refractory hyperammonaemia.

A fatal SARS-coronavirus-2 induced bone marrow aplasia complicated with invasive fungal infection and severe neutropenic enterocolitis.

BACKGROUND: Immunization against the coronavirus disease 2019 (COVID-19) began in January 2021 in Iran; nonetheless, due to a lack of vaccination among children under 12, this age group is still at risk of SARS-CoV-2 infection and its complications. CASE PRESENTATION: SARS-CoV-2 infection was diagnosed in a 6-year-old girl who had previously been healthy but had developed a fever and pancytopenia. The bone marrow aspiration/biopsy demonstrated just hypocellular marrow without signs of leukemia. She was worked up for primary and secondary causes of pancytopenia. Except for a repeated reactive HIV antibody/Ag P24 assay, all test results were inconclusive. After a thorough diagnostic investigation, the cross-reactivity of the HIV antibody/Ag P24 test with SARS-CoV-2 antibodies was confirmed. The patient did not develop any COVID-19-related signs and symptoms, but she did get a severe invasive fungal infection and neutropenic enterocolitis. She died as a result of disseminated intravascular coagulopathy. CONCLUSION: It is critical to recognize children infected with SARS-CoV-2 who exhibit atypical clinical manifestations of COVID-19, such as persistent pancytopenia. SARS-CoV-2 infection can cause severe and deadly consequences in children; thus, pediatricians should be aware of COVID-19's unusual signs and symptoms mimicking other conditions such as aplastic anemia.

Gastrointestinal Manifestations of Immunodeficiency: Imaging Spectrum.

There is a wide spectrum of hereditary and acquired immunodeficiency disorders that are characterized by specific abnormalities involving a plethora of humoral, cellular, and phagocytic immunologic pathways. These include distinctive primary immunodeficiency syndromes due to characteristic genetic defects and secondary immunodeficiency syndromes, such as AIDS from HIV infection and therapy-related immunosuppression in patients with cancers or a solid organ or stem cell transplant. The gut mucosa and gut-associated lymphoid tissue (the largest lymphoid organ in the body), along with diverse commensal microbiota, play complex and critical roles in development and modulation of the immune system. Thus, myriad gastrointestinal (GI) symptoms are common in immunocompromised patients and may be due to inflammatory conditions (graft versus host disease, neutropenic enterocolitis, or HIV-related proctocolitis), opportunistic infections (viral, bacterial, fungal, or protozoal), or malignancies (Kaposi sarcoma, lymphoma, posttransplant lymphoproliferative disorder, or anal cancer). GI tract involvement in immunodeficient patients contributes to significant morbidity and mortality. Along with endoscopy and histopathologic evaluation, imaging plays an integral role in detection, localization, characterization, and distinction of GI tract manifestations of various immunodeficiency syndromes and their complications. Select disorders demonstrate characteristic findings at fluoroscopy, CT, US, and MRI that permit timely and accurate diagnosis. While neutropenic enterocolitis affects the terminal ileum and right colon and occurs in patients receiving chemotherapy for hematologic malignancies, Kaposi sarcoma commonly manifests as bull's-eye lesions in the stomach and duodenum. Imaging is invaluable in treatment follow-up and long-term surveillance as well. Online supplemental material is available for this article. ©RSNA, 2022.

Outcome and Determinants of Neutropenic Enterocolitis in Pediatric Cancer Patients.

BACKGROUND: Neutropenic enterocolitis (NEC) is a dreaded complication of chemotherapy. There is scant literature regarding incidence, clinical features, and determinants. The understanding of gut dysbiosis in NEC and pediatric cancer is evolving. METHODS: Pediatric cancer patients with neutropenia and gastrointestinal symptoms were evaluated for NEC with contrast-enhanced computed tomography abdomen. Clinical, imaging, and laboratory features were analyzed. Fecal samples were analyzed for fecal calprotectin by sandwich enzyme-linked immunoassay and gut microbiota by conventional culture and compared with healthy controls and children without NEC. RESULTS: NEC was diagnosed in 44 children based on clinical and imaging features with incidence of 7.4% (4 had recurrent episodes). Common manifestations included fever (98%), pain abdomen (88%), and diarrhea (83%). Hypoalbuminemia was observed in 78% of patients. Large bowel involvement (94%) with diffuse bowel involvement (63%) and pancolitis (64%) were common. Fecal calprotectin was significantly elevated in NEC group than non-NEC group and healthy controls (median: 87, 53, and 42 µg/g, respectively). A higher degree of gut dysbiosis was observed in children with NEC with higher isolation of Bacteroides and infrequent isolation of Lactobacilli. Mortality rate of 23% was observed. Only the presence of free fluid predicted higher mortality. Though levels of fecal calprotectin and gut dysbiosis were higher in NEC, they did not increase mortality. Isolation of Bacteroides and absence of Lactobacilli predicted a longer duration of intravenous alimentation. CONCLUSIONS: NEC caused significant morbidity and mortality in pediatric cancer patients. Gut dysbiosis was significantly higher in NEC group suggesting a role in pathogenesis and influencing outcome. This highlights the role of targeted interventions towards gut dysbiosis like prebiotics and probiotics.

Abdominal Pain in the Immunocompromised Patient.

Abdominal pain in an immunocompromised patient represents a common clinical scenario that may have uncommon causes. Evaluation relies first on identifying the immunocompromise, whether due to congenital immunodeficiencies, malignancy, hematopoietic stem cell transplant, solid organ transplant, or human immunodeficiency virus/acquired immunodeficiency syndrome. Based on this determination, the emergency physician may then build a focused differential of pathophysiologic possibilities. Careful evaluation is necessary given the absence of classic physical examination findings, and liberal use of laboratory and cross-sectional imaging is prudent. Conservative evaluation and disposition of these high-risk patients is important to consider.

Enterocolitis neutropénica: reporte de un caso en un paciente con SIDA / Neutropenic enterocolitis report of an AID’s patient case

La enterocolitis neutropénica es una enteropatía necrotizante en pacientes con neutropenia severa relacionada con tratamientos quimioterápicos. Se presenta el caso de un paciente HIV positivo que desarrolló una enterocolitis neutropénica en el contexto de un tratamiento quimioterápico por Linfoma de Hodgkin. Presentaba leucopenia 400 leucocitos/mm3 y cuadro clínico compatible. La ecografía mostró engrosamiento de las paredes del ciego, del colon ascendente, y del íleon terminal. No hubo aislamiento microbiológico. Recibió tratamiento empírico con cefepime, vancomicina, y metronidazol con buena respuesta clínica y ecográfica. En el paciente con SIDA, diversas entidades pueden presentarse con dolor abdominal y engrosamiento de las paredes intestinales, como por ejemplo Citomegalovirus, Clostridium difficile, tuberculosis, histoplasmosis y linfomas, entre otras. El avance tecnológico, y las características ponderales de estos pacientes (bajo peso) permiten utilizar transductores de alta frecuencia para evaluar el tracto gastrointestinal y detectar mínimas lesiones en otros órganos abdominales, aportando al diagnóstico diferencial.

[19-year old male with Crohn's disease complicated by retroperitoneal abscesses and coxarthritis dexter]. / Choroba Lesniowskiego-Crohna u 19-letniego mezczyzny powiklana ropniami zaotrzewnowymi i zapaleniem stawu biodrowego prawego.

The article presents the case of a patient with Crohn's disease in whom an infection of the complex ileo-caecal be spread into the retroperitoneal space and the right hip.

Diarrhea in the immunocompromised patient.

Diarrhea is a common problem in patients with immunocompromising conditions. The etiologic spectrum differs from patients with diarrhea who have a normal immune system. This article reviews the most important causes of diarrhea in immunocompromised patients, ranging from infectious causes to noninfectious causes of diarrhea in the setting of HIV infection as a model for other conditions of immunosuppression. It also deals with diarrhea in specific situations, eg, after hematopoietic stem cell or solid organ transplantation, diarrhea induced by immunosuppressive drugs, and diarrhea in congenital immunodeficiency syndromes.

Management of neutropenic enterocolitis in children with cancer.

AIM: To describe the symptoms, clinical management and short-term outcome in a series of paediatric oncology patients with severe typhlitis following conservative treatment. METHODS: Twelve episodes of severe typhlitis in 11 children with cancer treated at the paediatric oncology ward at Queen Silvias Children's Hospital between 1995 and 2006 were analysed retrospectively. Data on symptoms, radiological findings, laboratory status and treatment as well as outcome were collected and analysed. RESULTS: In all episodes, the classical signs of neutropenia, fever, abdominal pain and thickening of the bowel wall were present. All were successfully treated with bowel rest, broadspectrum antibiotics and supportive care. After recovery from typhlitis, three patients needed surgical intervention because of complications. CONCLUSION: A high clinical suspicion combined with radiological imaging aids early diagnosis. Predisposing factors for developing typhlitis were haematologic malignancy and treatment with chemotherapy within 3 weeks of onset. Supportive care, bowel rest including parenteral nutrition, correction of cytopenias and aggressive antimicrobial treatment is essential. Measurements of C-reactive protein in blood may be of benefit when assessing the clinical course.

The surgical management of abdominal pain in the multiple myeloma patient.

BACKGROUND: The purpose of this retrospective study was to characterize the presentation, treatment, and outcomes of patients with multiple myeloma requiring surgical evaluation for abdominal pain. METHODS: Medical records of patients with myeloma and abdominal pain evaluated by surgery over a period of 18 months were examined. RESULTS: Twenty-one patients underwent surgical evaluation, with 23 diagnoses. Neutropenic enterocolitis (n = 5 [22%]) and ileus (n = 4 [17%]) were common diagnoses. Eleven patients (52%) were neutropenic. Peritonitis was noted in only 1 patient. Eastern Cooperative Oncology Group performance status was either 3 or 4 in most patients (67%). Surgery was performed in 5 patients. The 90-day mortality rate for all patients was 43%, with all deaths secondary to sepsis in patients managed without surgery. CONCLUSIONS: Patients with myeloma requiring surgical evaluation for abdominal pain have a unique differential diagnosis, with notable findings at presentation including the presence of cytopenia, lack of peritoneal signs, and low performance status.

Significance of abdominal ultrasound in inflammatory bowel disease.

Transabdominal ultrasound is most commonly used to examine the liver, hepatobiliary-pancreatic and urogenital tract. Its use for imaging the intestinal tract is less well established and has been considered more difficult in the past. Improvements in technology and increasing experience with sonographic findings in a variety of intestinal diseases including inflammatory bowel disease (IBD), however, have contributed to firmly establishing the role of ultrasound as a clinically important, non-invasive and widely available imaging modality. In addition, newer techniques such as harmonic imaging and contrast-enhanced ultrasound have recently gained attention. Transabdominal ultrasound is clinically useful in the initial diagnosis of IBD by evaluating bowel wall thickness and surrounding structures including peri-intestinal inflammatory reaction, extent and localization of involved bowel segments and detection of extraluminal complications such as fistula, abscesses, carcinoma and ileus. Transabdominal ultrasound is currently accepted as a clinically important first-line tool in assessing patients with Crohn's disease irrespective of their clinical symptoms and/or disease activity. It helps to better characterize the course of the disease in individual patients and can guide therapeutic decisions. The topic of this review is to provide an updated overview of the role of transabdominal ultrasound in IBD including Crohn's disease, ulcerative colitis, tuberculosis and neutropenic colitis while summarizing the results of recent studies with special reference to sensitivity/specificity in detecting the disease and sonomorphologic features to evaluate disease activity and its luminal and extraluminal complications.

[Prevalence of neutropenic enterocolitis in adults with severe neutropenia and associated mortality.]. / Prevalencia de enterocolitis neutropénica en adultos con neutropenia grave y mortalidad asociada.

BACKGROUND: Neutropenic enterocolitis (NEC) is characterized by inflammatory damage and necrosis of the intestinal mucosa, mainly of the terminal ileum and the cecum. It is more frequent in patients with leukemia and/or undergoing antineoplastic chemotherapy, and the main risk factor is neutropenia <1000/mm3. OBJECTIVE: To know the prevalence of NEC and the mortality associated with it in adults with hematologic conditions and neutropenia <1000/mm3. MATERIAL AND METHODS: All adult patients who were hospitalized for malignant hematologic conditions with neutropenia <1000/mm3 were enrolled in the study; those with neutropenia >1000/mm3 were excluded. The diagnosis of NEC was based on the clinical data and imaging tests (abdominal plain X-rays and CT scan). Demographics of all patients were collected, as well as the data related with the course and treatment of the underlying hematologic condition and the NEC. RESULTS: 117 patients were enrolled in the study; 75.2% of them with some type of acute leukemia. The diagnosis of NEC was made in 8 patients (6.8%). NEC occurred in 10.5% of the patients with acute myeloid leukemia and in 8.0% of those with acute lymphocytic leukemia. Three patients died, which resulted in a 37.5% mortality rate. No association was found between the severity of neutropenia and the onset of NEC or NEC-related mortality. CONCLUSIONS: The prevalence of NEC in patients with hematologic conditions admitted for severe neutropenia is 6.8% and the mortality rate associated with this complication is 37.5%.

Gastrointestinal bleeding in the cancer patient.

Gastrointestinal bleeding is a common occurrence in patients with cancer and is a frequent indicator of a gastrointestinal malignancy. Rapid evaluation and treatment is key for the hemodynamically unstable patient. Endoscopy remains the cornerstone of diagnosis and management for cancer patients with gastrointestinal bleeding. The emergency physician should also be aware of other diagnostic and treatment modalities that may be needed to take care of these patients.

Challenges in surgical management of abdominal pain in the neutropenic cancer patient.

BACKGROUND: Abdominal pain in neutropenic cancer patients presents a unique clinical challenge for surgeons. The purposes of this retrospective study were to characterize the clinicopathologic factors associated with the presentation of neutropenia and abdominal pain, examine the treatment strategies used, and define associated outcomes for these patients. METHODS: We identified patients with concomitant neutropenia (absolute neutrophil count <1000 cells/microL) and abdominal pain who had been evaluated by surgical oncologists over a period of more than 6 years. A Cox proportional hazards regression model was used to analyze the association between clinicopathologic factors and overall survival time. RESULTS: Sixty patients were included in this analysis. After our clinical and radiographic evaluations, we determined that the most frequent causes of the abdominal pain were neutropenic enterocolitis (28%) and small bowel obstruction (12%); the cause remained uncertain in 35%. Surgical interventions had been performed in 9 patients. The 30- and 90-day mortality rates for all patients were 30% and 52%, respectively. Multivariate analysis revealed that severe sepsis, a relatively long duration of neutropenia, and the lack of surgical intervention were significant adverse prognostic factors for overall survival. CONCLUSIONS: Abdominal pain as a symptom in neutropenic patients continues to be a diagnostic and therapeutic challenge and is associated with a high mortality rate. Based on our results, we conclude that efforts should focus on improving neutrophil counts and on treating the frequent and serious comorbidities found in these patients. Surgery should be delayed, when possible, to allow for neutrophil recovery.

Neutropenic enterocolitis in children with acute leukemia or aplastic anemia.

Neutropenic enterocolitis (NE) and acute appendicitis are life-threatening conditions that develop in children with severe or prolonged neutropenia secondary to acute leukemia and lymphoma. The medical records of 118 patients who were treated for acute lymphoblastic leukemia (69 patients), acute myelogenous leukemia (22 patients), or aplastic anemia (27 patients) between 1997 and 2006 in our hospital pediatric hematology department were examined retrospectively. NE was diagnosed in 11 patients (age range, 2.5-16 years) on the basis of clinical and laboratory features. Two of these 11 patients had appendicitis in addition to NE. Conservative treatment was favored for all patients, but 1 patient with acute appendicitis underwent surgery. Neutropenic patients with a hematologic malignancy and abdominal pain should receive their diagnoses immediately and undergo treatment. NE and acute appendicitis should always be considered in the differential diagnosis of abdominal pain. Conservative treatment must be chosen initially for patients with NE, and these patients should be evaluated carefully for surgery. The criteria for the surgical process are the same as those for immunocompetent children. In addition, the close monitoring of hematologic factors is necessary.