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  • Long-Term Quality of Life after Endoscopic Pituitary Adenoma Surgery with Nasoseptal Flap Reconstruction

    Introduction: Endoscopic endonasal transsphenoidal surgery (EETS) on the pituitary gland is considered safe and efficacious. The nasoseptal flap (NSF) is sometimes used to prevent or repair postoperative cerebrospinal fluid (CSF) leaks. Few investigators have quantified long-term quality-of-life (QOL) outcomes regarding sinonasal measures after EETS, with or without involvement of the NSF. This study assesses whether the septal flap affects sinonasal QOL outcomes for patients receiving EETS for pituitary adenoma. Methods and Materials: This is a retrospective study of patients who underwent EETS between 2013 and 2018. A total of 62 adults completed the Sinonasal Outcome Test-22 (SNOT-22) at least one year after the surgery. Outcome measures were compared between patients who underwent EETS with and without septal flap reconstruction. Results: For the entire cohort, there were 14 patients (22.6%) who had septal flap reconstruction and 48 patients (77.4%) who did not. Patient demographics, tumor characteristics, surgical outcomes, and duration between surgery and completion of the questionnaire were similar for both groups. The mean SNOT-22 scores in the no reconstruction (NR) group and the nasoseptal flap reconstruction (NSFR) group were similar (P=0.9). In terms of SNOT-22 subdomains (rhinologic symptoms, extranasal rhinologic symptoms, ear/facial symptoms, psychological dysfunction, and sleep dysfunction), no significant differences were found when comparing the groups. Conclusion: As compared with no reconstructive involvement, NSF utilization does not affect the QOL and nasal symptoms of patients undergoing EETS.
  • COVID-19 and Treatment and Immunization of Children—The Time to Redefine Pediatric Age Groups is Here

    Children are infected with coronavirus disease 2019 (COVID-19) as often as adults, but with fewer symptoms. During the first wave of the COVID-19 pandemic, multisystem inflammatory syndrome (MIS) in children (MIS-C), with symptoms similar to Kawasaki syndrome, was described in young minors testing positive for COVID-19. The United States (US) Centers for Disease Control and Prevention (CDC) defined MIS-C as occurring in <21-year-olds, triggering hundreds of PubMed-listed papers. However, postpubertal adolescents are no longer children biologically; the term MIS-C is misleading. Furthermore, MIS also occurs in adults, termed MIS-A by the CDC. Acute and delayed inflammations can be triggered by COVID-19. The 18th birthday is an administrative not a biological age limit, whereas the body matures slowly during puberty. This blur in defining children leads to confusion regarding MIS-C/MIS-A. United States and European Union (EU) drug approval is handled separately for children, defined as <18-year-olds, ascribing non-existent physical characteristics up to the 18th birthday. This blur between the administrative and the physiological meanings for the term child is causing flawed demands for pediatric studies in all drugs and vaccines, including those against COVID-19. Effective treatment of all conditions, including COVID-19, should be based on actual physiological need. Now, the flawed definition for children in the development of drugs and vaccines and their approval is negatively impacting prevention and treatment of COVID-19 in minors. This review reveals the necessity for redefining pediatric age groups to rapidly establish recommendations for optimal prevention and treatment in minors.
  • Alternating and Concurrent True Hyperkalemia and Pseudohyperkalemia in Adult Sickle Cell Disease

    Sickle cell disease (SCD) predisposes the patient to recurrent episodes of acute painful hemolytic crisis. Sickle cell nephropathy (SCN) is not uncommon in adult patients, and renal manifestations of SCN include renal ischemia, microinfarcts, renal papillary necrosis, and renal tubular abnormalities with variable clinical presentations. Intravascular hemolysis and reduced glomerular filtration rate with renal tubular dysfunction predispose to true hyperkalemia. Hemolytic crisis can be complicated by sepsis, leading to significant degrees of thrombocytosis, and thrombocytosis is a well-defined cause of pseudohyperkalemia. We describe a 40-year-old African American male patient with sickle cell anemia who exhibited alternating episodes of true hyperkalemia and pseudohyperkalemia, during consecutive hospital admissions. Clearly, true hyperkalemia is a potentially lethal condition. At the same time, the institution of inappropriate and intensive treatment of pseudohyperkalemia leading to severe hypokalemia is also potentially lethal. The need for this caution is most imperative with the recent introduction of the safer and more potent potassium binders, patiromer and sodium zirconium cyclosilicate.
  • Circulating Proprotein Convertase Subtilisin/Kexin Type 9 Levels Predict Future Cardiovascular Event Risks in Hemodialyzed Black African Patients

    Context and Objective: Cardiovascular diseases are the leading cause of mortality in patients. In this context, proprotein convertase subtilisin/kexin type 9 (PCSK9) appears to be the new biomarker identified as interfering in lipid homeostasis. This study aimed to investigate the association between PCSK9, dyslipidemia, and future risk of cardiovascular events in a population of black Africans. Methods: A cross-sectional study was conducted between August 2016 and July 2020 in six hemodialysis centers in the city of Kinshasa, Democratic Republic of the Congo. Serum PCSK9 was measured by ELISA; lipid levels of 251 chronic kidney disease grade 5 (CKD G5) hemodialysis patients and the Framingham predictive instrument were used for predicting cardiac events. Results: Total cholesterol (TC), low-density lipoprotein cholesterol (LDL-c), and triglycerides (TG) were significantly increased in the tertile with the highest PCSK9. By contrast, high-density lipoprotein cholesterol (HDL-c) was significantly decreased in the same tertile. A strong positive and significant correlation was found between PCSK9 and TC, TG, and LDL-c. Negative and significant correlation was observed between PCSK9 and HDL-c. The levels of PCSK9, smoking, overweight, and atherogenic dyslipidemia were associated with future risks for cardiovascular events in univariate analysis. After adjustment, all these variables persisted as independent determinants of future risk for cardiovascular events. The probability of having a cardiovascular event in this population was independently associated with PCSK9 levels. Compared to the patients having lowest PCSK9 tertile, patients with PCSK9 levels in the middle (aOR 5.9, 95% CI 2.06-17.3, P<0.001) and highest tertiles (aOR 8.9, 95% CI 3.02-25.08, P<0.001) presented a greater risk of cardiac event. Conclusion: Increased PCSK9 serum levels are associated with higher levels of TC, LDL-c, and TG and lower levels of HDL-c in black African hemodialysis patients. Serum PCSK9 levels in these patients predict increased risk of cardiovascular events, independent of traditional potential confounders.
  • Incidence of Cardiac Manifestations in Children with Dengue Fever: A Cross Sectional Study

    Objective: The aim of our study was to explore the incidence of cardiac involvement in children with dengue infection admitted in a tertiary care hospital and to evaluate the features of cardiac involvement with the severity of dengue fever. Methods: This was a cross-sectional study conducted from September 2014 to August 2016. A total of 130 patients with confirmed dengue NS1 antigen or IgM antibody positivity between the ages of 1 month and 18 years were evaluated. On the third day of admission, blood samples for cardiac markers were collected, and electrocardiograms (ECG), and echocardiograms were performed for each patient. Results: Of the 130 dengue patients in the study, 60 (46.2%) were males and 70 (53.8%) were females (male to female ratio, 1:1.16). Cardiac involvement was present in 60 (46.2%) children and was more prominent in children with severe dengue (72.7%), followed by dengue with warning symptoms (53.8%) and dengue fever (28.6%). There was no significant correlation between cardiac involvement and primary/secondary dengue. Both ECG and echocardiography changes were significantly correlated with dengue severity, as opposed to cardiac markers. Conclusions: Cardiac involvement was present in children with dengue. Evaluation with ECG, echocardiography, and cardiac markers such as CPK-MB are required for the management of cardiac complications in children with dengue. Our study showed an association between cardiac involvement and the severity of dengue. Further studies should be framed, and follow-up of dengue patients with cardiac involvement is necessary for therapeutic management.
  • Salivary Biomarker Levels and Oral Health Status of Children with Cerebral Palsy and Their Healthy Siblings: A Comparative Study

    Background: The dental needs of cerebral palsy children are an area of study much in need of attention. The neglect of this aspect should be rectified, and simpler diagnostic methodologies should be established and used to serve this purpose. Aim: This study aimed to determine oral health status and salivary biomarkers (salivary flow rate, pH, buffering capacity) among children with cerebral palsy (CP), to compare their data with that of their healthy siblings, and to evaluate the relationship between salivary biomarkers and dental caries. Methods: A total of 30 CP children (study group) and 30 normal healthy siblings (controls) were selected between the ages of 5 and 12 years. Salivary biomarkers were assessed, and oral health status was examined. Statistical Analysis: Chi-square test was used for comparison of oral health status. “Unpaired t test was used to compare caries indexes (decay/filled teeth–primary dentition [dft] and decay/missing/filled teeth–permanent dentition [DMFT]) and salivary biomarkers between the groups. Pearson correlation was used to find the correlation between salivary biomarkers and caries. Results: The dft scores were significantly higher in the study group (P<0.05). The pH values and salivary flow rates were significantly lower in the study group (P<0.05 and P<0.001, respectively). There was a significant correlation between DMFT scores and salivary flow rate in the study group (P<0.05). Conclusion: Low pH and low salivary flow rate might be risk factors for dental caries in CP populations; moreover, the significant correlation between DMFT score and salivary flow rate suggests that salivary flow rate could be used as a screening tool for assessing at-risk subjects in such populations.
  • First Admission Neutrophil–Lymphocyte Ratio May Indicate Acute Prognosis of Ischemic Stroke

    Objectives: Our study aimed to determine the relationship between serum periostin levels, and the neutrophil–lymphocyte ratio (NLR) with ischemic stroke subtypes, clinical stroke scales, and acute prognosis in patients with acute ischemic stroke. Materials and Methods: Forty-two ischemic stroke patients and 39 age- and sex-matched healthy volunteers were included in our study. Demographic characteristics including age and gender were recorded. Blood serum periostin and NLR values were evaluated in the first 24 hours after admission. Serum periostin levels were compared with healthy controls of similar age and sex. Lesion localization was determined by cranial CT or diffusion MRI of the patients. Stroke scales were recorded on days 1 and 7 of hospitalization in the study group. Results: The mean serum periostin levels were higher than in the control group, but no statistically significant difference was found. There was no correlation between serum periostin levels and prognosis of stroke. First admission NLRs were statistically higher than in the control group. The first admission NLRs were positively correlated with the first admission National Institute of Health Stroke Scale score and the day 7 modified Rankin score. Conclusion: Our study is the first study to evaluate both NLR and serum periostin levels in all types of acute ischemic stroke. While our study did not show that first admission serum periostin levels can be used as a biomarker in ischemic stroke, it did indicate that the first admission NLR can be used for acute prognosis of ischemic stroke.
  • Bone Involvement in Hyperphosphatemic Familial Tumoral Calcinosis: A New Phenotypic Presentation

    Mutations in FGF23, KL, and GALNT3 have been identified as the cause for the development of hyperphosphatemic familial tumoral calcinosis (HFTC). Patients with HFTC typically present in childhood or adolescence with periarticular soft tissue deposits that eventually progress to disrupt normal joint articulation. Mutations in the GALNT3 gene were shown to account for the hyperphosphatemic state in both HFTC and hyperostosis-hyperphosphatemia syndrome (HHS), the latter characterized by bone involvement. We present the case of a patient of a Druze ethnic origin with known HFTC that presented to our department with the first documented case of pathologic fracture occurring secondary to the disease. Our report introduces this new phenotypic presentation, suggests a potential role for prophylactic bone screening, and highlights the need for preconception genetic screening in selected populations.
  • Non-immune Hemolysis in Gaucher Disease and Review of the Literature

    Gaucher disease (GD) is an autosomal recessive disease characterized by the buildup of glucocerebrosides in macrophages, resulting in the formation of “Gaucher cells.” These cells predominantly infiltrate the liver, spleen, and bone marrow leading to hepatosplenomegaly, cytopenia, and bone pain. Anemia in GD is typically considered to result from non-hemolytic processes. Although rare, a higher rate of hemolytic anemia of the autoimmune type has been reported in GD than in the general population. The literature on non-immune hemolytic anemia in GD is scarce. We review the literature on hemolytic anemia in GD and report on a case of non-immune hemolytic anemia secondary to GD. We believe this is the first description of a patient with confirmed GD and symptomatic non-immune hemolytic anemia that responded to GD-specific treatment.
  • Inpatient Rehabilitation is Effective for Severe Daily Activity Deficits Related to Chronic Low Back Pain

    Background and Objective: Chronic low back pain disability (CLBPD) is a syndrome that includes pain, disability, emotional impairments, and social problems. The study was conducted to examine the effect of an inpatient rehabilitation program on the performance of everyday life tasks (daily activities), and report on pain in CLBPD patients with primary activities of daily living (ADL) deficits. Methods: In a retrospective cohort study, demographic and clinical data were retrieved from records of inpatients admitted consecutively to the program. Scores of the Spinal Pain Independence Measure (SPIM) and of changes in reported pain levels were used to assess improvement in the performance of daily activities and pain reduction. T-tests were used to assess the significance of score changes. Spearman’s correlations and analysis of variance were used to assess relationships of SPIM gain and affecting factors. Results: Ninety-nine patients were included. Daily task performance improved in 71 patients (71.7%). The SPIM score increased from 48.7 (SD 16.3) at admission to the rehabilitation program to 57.8 (SD 12.5) at discharge (P<0.001). The SPIM score at admission negatively affected SPIM gain (P<0.001). The SPIM gain was significant for admission SPIM scores of 50 or lower (P<0.05), but differences in SPIM scores were not as noticeable for patients with admission SPIM scores above 50. Relief in pain was reported in 59 patients (59.6%) and was not associated with function. Conclusions: The multidisciplinary rehabilitation program, which improved function and provided limited pain relief in inpatients with CLBPD primary ADL deficits, can be effective for the most severe CLBPD cases.