Introduction:Secondary hyperparathyroidism (SHPT) is an insidious disease that develops early in the course of chronic kidney disease (CKD) and increases in severity as the glomerular filtration rate deteriorates. Recent studies have identified fibroblast growth factor-23 (FGF23) as a new protein with phosphaturic activity. It is mainly secreted by osteoblasts and is now considered the most important factor for regulation of phosphorus homeostasis. It is not yet proven if there is any direct relation between parathyroid hormone (PTH) and FGF23. The present study aims to evaluate the relation between serum FGF23, phosphorus, and PTH in end-stage renal disease in patients with SHPT on regular hemodialysis.Materials and Methods:Forty-six consecutive CKD adult patients (case group) and 20 healthy adults (control group) were included in the study. All patients had SHPT and were on regular hemodialysis. Both groups were subjected to full medical history, clinical examination and biochemical studies. Serum phosphorus, calcium, ferritin, hemoglobin level, blood urea, creatinine, PTH, and FGF23 were analyzed.Results:Levels of FGF23 were significantly higher in the case group in comparison with those in the control group, viz., 4-fold, and positively correlated with PTH. Phosphorus levels in the case group were significantly high in spite of the increasing levels of FGF23. Both PTH and FGF23 were positively correlated with phosphorus and negatively with hemoglobin levels.Conclusion:SHPT and FGF23 may have a partial role in the development of anemia in patients with CKD. FGF23 could be a central factor in the pathogenesis of SHPT. Its role in controlling hyperphosphatemia in CKD is vague.
Background and aim Autoimmune hepatitis (AIH) has variable clinical manifestations and should be considered in the diagnostic work-up of any patient with cryptogenic liver disease. The aim of the study was to determine the clinical, biochemical, histopathological characteristics and treatment outcome of AIH in Egyptian children. Patients and methods This observational study was conducted at the Pediatric Hepatology Unit at Cairo University Pediatric Hospital, Egypt. All children (<18 years of age) presenting from 2009 to 2016 with established diagnosis of AIH were included. Medical history, clinical examination, and results of investigations were retrieved from patients’ files. The main outcome measures included the rate of remission, relapses, and mortality. Results The study included 34 children with AIH. Twenty patients (58%) presented with chronic liver disease. There was a history of concomitant autoimmune diseases in 5 patients. Transaminases were elevated in all patients. There was synthetic dysfunction in 58%. Twenty-four patients (70.5%) had AIH-1, while nine patients (26.4%) had AIH-2 and one patient (2.9%) had autoantibody negative AIH. Piecemeal necrosis was observed in the liver biopsy of 79% of our cohort. Approximately 80% achieved biochemical remission (88% received combined therapy of prednisolone and azathioprine). About half of the patients developed relapses. One patient died of liver cell failure. Conclusion In children with liver disease, a diagnosis of AIH should be considered. In those patients, AIH-1 is more common than AIH-2. Prednisolone monotherapy or combined with azathioprine could achieve remission, but relapse is still common. Treatment non-adherence is the main risk factor for relapse.
Background Adherence of patients with End-Stage Renal Disease (ESRD) to Hemodialysis (HD), prescribed medications, diet and fluid restrictions is essential to get the desirable outcome and prevent complications. During COVID-19 pandemic, ESRD patients became more concerned with attending the HD sessions and following the protective measures because of the potential for increased susceptibility to COVID-19. The aim of this study was to evaluate the impact of the pandemic on patients' adherence to HD and medical regimens. Methods Two hundred five ESRD patients on HD were interviewed with the ESRD Adherence Questionnaire (ESRD-AQ) and the Fear-of-COVID-19 Scale (FCV-19S). Clinical and laboratory correlates of adherence were retrieved from patients' records. Results Self-reported adherence to HD showed that 19.5% were not adherent to HD during the pandemic compared to 11.7% before the pandemic (p < 0.001), with a significant agreement with the actual attendance of HD sessions (Kappa = 0.733, p < 0.001). Twenty-five patients (12.2%) had a history of COVID-19. The FCV-19S had a mean score of 18.8 and showed significant positive correlations with the pre-dialysis phosphorus and potassium. Multivariate analysis showed that the main predictors of non-adherence were the history of COVID-19, understanding and perception scores, and the Fear-of-COVID score. Conclusions The COVID-19 pandemic adversely affected the adherence of ESRD patients to HD and medical regimen. Strategies to mitigate patients' fears of COVID-19 and improve their understanding and perceptions of adherence to HD and medical regimen should be adopted in HD centers during the pandemic.
Systemic lupus erythematosus (SLE) is a complex systemic autoimmune disease characterized by a wide spectrum of clinical manifestations with varying severity. The dysregulation of cytokines contributes strongly to disease pathogenesis. Recently, it has been shown that the imbalanced antagonist/agonist profile of interleukin (IL)-36 cytokines, might play a role in autoimmune disorders. Our aim is to investigate mRNA expression of Interleukin-36α (IL-36α) in the peripheral blood of Egyptian systemic lupus erythematosus (SLE) patients and its association with disease activity and organs involvement. We assessed the relative expression of IL-36α mRNA by real time polymerase chain reaction and the comparative CT method on the peripheral blood of 49 SLE patients, and 40 healthy controls. Patients were subjected to thorough history and clinical examination, in addition to routine hematological, biochemical, and serological studies. Disease activity was evaluated using the SLE Disease Activity Index (SLEDAI). The relative expression of IL-36α mRNA was significantly higher in SLE patients compared to healthy controls (4.3±2.9 vs 1, respectively, P<0.0001). Fold change of IL-36α expression was significantly increased in patients with moderate and high disease activity (SLEDAI>5) than patients with mild disease activity (SLEDAI≤5) (4.3±1.2 vs 2.7±2.0, respectively, P=0.006) and positively correlated with SLEDAI (r=0.505, P=0.001). Regarding major organ involvement, the mean±SD expression of IL-36α in patients with arthritis and mucocutaneous involvement was significantly higher compared to those without organ involvement (4.2±1.5 vs 2.8±1.8, P=0.039 and 4.1±1.4 vs 2.8±2.1, P=0.041, respectively). In conclusion, the IL-36α is significantly more expressed in SLE patients compared to healthy controls and correlated with SLE disease activity and arthritis. IL-36α expression could be a useful biomarker for SLE disease activity in Egyptian patients with SLE.
Background: During the COVID-19 pandemic, there was limited data about the appropriate management of acute myeloid leukemia (AML) with COVID-19 infection and the possible post-COVID-19 complications reported in acute leukemia patients. Case presentation: We report a 52-year-old lady with AML and confirmed twice SARS-CoV-2 infection. The first infection was just after the diagnosis of AML before the administration of induction therapy, and the second infection was just after she received the salvage therapy. The COVID-19 infection was confirmed by qRT-PCR and highresolution non-contrast computerized tomography of the chest. Unfortunately, the patient developed post-covid neurological complications, disturbed consciousness level, and encephalopathy. The COVID-19 infection may have triggered encephalopathy or exaggerated the neurological toxicity of cytarabine even in a small dose. Another possible explanation is the exaggeration of cytokine storm by the administration of granulocyte-stimulating factors used in salvage therapy. Conclusion: Management of COVID-19 infection in AML patients faces many challenges. These patients are more vulnerable and susceptible to many complications and high mortality rates. The treatment approach needs to be tailored to overcome the interaction between the treatment adverse events and the biology of covid-19 infection.
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