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Chronic Liver Disease
Published in Praveen S. Goday, Cassandra L. S. Walia, Pediatric Nutrition for Dietitians, 2022
Julia M. Boster, Kelly A. Klaczkiewicz, Shikha S. Sundaram
Cirrhosis leads to a state of hypermetabolism, with affected children having higher energy needs than expected. Furthermore, due to the increased resistance to blood flow through the fibrotic liver, portal hypertension with progressive organomegaly and ascites develops. Organomegaly and fluid accumulation contribute to perceived weight gain, confounding measurements of weight and BMI or weight-for-length. Anthropometric measurements, including MUAC and TSFT, are critical to assess growth in this population. Sodium restriction is necessary to effectively manage ascites associated with portal hypertension. Limiting daily sodium intake to 2 mEq/kg can help control ascites.
Paper 3
Published in Aalia Khan, Ramsey Jabbour, Almas Rehman, nMRCGP Applied Knowledge Test Study Guide, 2021
Aalia Khan, Ramsey Jabbour, Almas Rehman
Which one statement is not true about acute lymphoblastic leukaemia (ALL)? White blood cell count >50 × 109/L is associated with a worse prognosis.Female gender is associated with a worse prognosis.Bulky organomegaly is associated with a worse prognosis.Central nervous disease is associated with a worse prognosis.Bulky lymphadenopathy is associated with a worse prognosis.
System Imaging in Unexplained Fever
Published in Benedict Isaac, Serge Kernbaum, Michael Burke, Unexplained Fever, 2019
Significant organomegaly, particularly the enlarged spleen, is reliably assessed by nuclear medicine, ultrasound, and CT. The recently introduced faster and more sophisticated CT scanners, in combination with intravenous bolus contrast enhanced dynamic liver and splenic scans, may disclose diffuse infiltrative organ pathology. The combination of hepatosple-nomegaly and retroperitoneal lymphadenopathy revealed by ultrasound or CT should alert the clinician to the diagnosis of a lymphoma as the cause of fever in the patient.
PPARG, GNG12, and CD19 are potential independent predictors of central nerve recurrence in childhood acute lymphoblastic leukemia
Published in Hematology, 2023
Shan Zhang, Yansong Tu, Hurong Lai, Huijun Chen, Huaijun Tu, Jian Li
Studies have reported that sex, hepatomegaly, central nervous system status and age [6–8], neuronal-glial antigen-2 (NG2) expression [9], and CNS microenvironment [10] are all associated with CNS relapse of childhood ALL. Another study showed that the up-regulation of the PBx1 gene in B-cell leukemia in mouse CNS microenvironment could enhance the chemotherapy-resistance and self-renewal characteristics of leukemia cells [11]. Organomegaly at diagnosis was a highly significant clinical predictor for relapse [12]. In addition, acute leukemia patients with CNSL, which showed positive CD19 expression in tumor cell immunotyping and remission after anti-CD19 CAR T cell therapy [13]. It could also be a potential predictor. However, as these studies were based only on clinical data or animal experiments, there is still no independent predictor and lack of a corresponding prediction model for CNS relapse in childhood ALL.
Chronic myelomonocytic leukemia - a review
Published in Expert Review of Hematology, 2021
Thomas P. Thomopoulos, Anthi Bouhla, Sotirios G. Papageorgiou, Vasiliki Pappa
Recently, the MDS/MPN International Working Group, proposed new response criteria for CMML and other MDS/MPN overlap syndromes, incorporating reduction in blast percentage, improvement of anemia and thrombocytopenia as well as correction of WBC, monocyte, and peripheral IMC counts and regression of splenomegaly [98]. A novel feature of the criteria is introduction of an entity named ‘clinical benefit’ based on improvement evaluated by the myeloproliferative Neoplasm Symptom Assessment Form (MPN-SAF) scoring system [99]. Although, the new criteria have been successfully validated in a small retrospective CMML cohort, prospective validation in large cohorts is in need [100]. In clinical practice, periodical evaluation of patients should include general symptoms, complete blood count, and physical examination for organomegaly. Bone marrow, including cytogenetic analysis, should be performed, if indicated by alterations in the aforementioned aspects [92].
Diagnostic, prognostic and predictive values of miR-100 and miR-210 in pediatric acute lymphoblastic Leukemia
Published in Hematology, 2020
Naglaa M. Hassan, Lobna A. Refaat, Ghada N. Ismail, Mona Abdellateif, Sayed A. Fadel, Rania S. AbdelAziz
The current study included 85 patients, out of them 47 (55.3%) were males and 38 (44.7%) were females, with a median age of 6 years (range: 1–17) and mean ± SD of 7.6 ± 5.07 years. BM findings showed that 71 patients (83.5%) presented with hypercellular BM, 11 patients (12.9%) with normocellular BM, and only 3 (3.5%) patients presented with hypocellular BM. Of all patients, BM blasts ranged from 26% to 99% with a median of 91%. Blast percentage was <90% in 28 (32.9%) patients, while it was ≥90% in 57 (67.1%) patients. Immunophenotyping analysis showed that 50 (58.8%) patients had (Pre-B) phenotype, 17 (20%) had common ALL (C-ALL), and 18 (21.2%) patients had T-ALL phenotype. Molecular and genetic data were available for 84 patients, where 68/84 patients (80.9%) had normal karyotype, 5/84 (6%) patients had t(9;22) p190, 3/84 (3.6%) patients had t(9;22) p210, 3/84 (3.6%) patients had t(12;21), 1/84 (1.2%) patient had t(1;19), 3 (3.5%) patients had hyperdiploidy (53xx, 54xx, and 58xy), and only 1(1.17%) patient had Hypodiploidy (45, xx). There were 14/85 (16.5%) patients presented with hepatomegaly, 7/85 (8.2%) patients had splenomegaly, 42/85 (49.4%) had hepatosplenomegaly and 22/85 (25.9%) patients did not have any organomegaly, while lymphadenopathy was present in 54/85 (63.5%) of the cases. Patients were classified according to risk stratification of ALL [19] into low risk (LR) in 33 (38.8%) patients, standard risk (SR) in 45 (52.9%) patients, and high risk (HR) in 7 (8.2%) patients (Table 1).