The prevalence, epidemiological characteristics and mortality trends of inflammatory myopathies patients in Oman: the Prevision study.

OBJECTIVES: This research aims to investigate the prevalence, epidemiological characteristics, mortality rates, survival rates and the rate of malignancy in patients diagnosed with inflammatory myopathies (IIM) in Oman. METHODS: This is a longitudinal study, that covered a span of 16 years at eight rheumatology centres in Oman. The study included all adults and paediatric patients diagnosed with different types of idiopathic inflammatory myopathies (IIM) and who fulfil either the Bohan classification criteria or the 2017 EULAR/ACR classification criteria. RESULTS: The study included a total of 116 patient with an average age of 38.78 (±17.61 SD) years. The most prevalent form of myositis was found to be dermatomyositis (DM) 48 (41.38%), followed by polymyositis (PM) 36 (31.03%) and juvenile myositis (JDM) 18(15.52%). However, inclusion body myositis and necrotising myopathy were relatively rare conditions. The prevalence rates for DM, PM and JDM were determined as 2.2, 2.2, and 1.14 per 100,000 population respectively. Cardiac complications were observed in 14.66% of cases. Among the individuals studied, a history of malignancy was present in around 1.72% of cases. ANA antibodies were present in 71.55% of the cases, anti-Jo 1 and anti-RNP/SM antibodies were detected in 8.62%, and Anti-Ro antibodies in 24.14%. The overall mortality rate was found to be 6.90% with a rate of 11.1% among JDM cases. The five-year survival rates for PM, DM and JDM were found to be 94.4%, 91.7% and 89.0% respectively. These rates decline over a 10-year period to 67%, 69% and 83.3% respectively. CONCLUSIONS: The study highlights the prevalence, mortality, and survival rates of IIM in Oman. Patients with JDM had a higher mortality rate. This underscores the significance of using novel healthcare strategies to improve clinical outcomes and meet special requirements for this group of patients.

Consensus recommendations on fasting during Ramadan for patients with kidney disease: review of available evidence and a call for action (RaK Initiative).

Ramadan fasting (RF) involves abstaining from food and drink during daylight hours; it is obligatory for all healthy Muslims from the age of puberty. Although sick individuals are exempt from fasting, many will fast anyway. This article explores the impact of RF on individuals with kidney diseases through a comprehensive review of existing literature and consensus recommendations. This study was conducted by a multidisciplinary panel of experts.The recommendations aim to provide a structured approach to assess and manage fasting during Ramadan for patients with kidney diseases, empowering both healthcare providers and patients to make informed decisions while considering their unique circumstances.

Therapeutic plasma exchange: A potential therapeutic modality for critically ill adults with severe acute respiratory syndrome coronavirus 2 infection.

BACKGROUND: Severe acute respiratory syndrome coronavirus 2 infection can be severe and fatal due to cytokine storm. Therapeutic plasma exchange (TPE) potentially mitigates the harmful effects of such cytokines. We investigated the use of TPE, as rescue therapy, in patients with severe Coronavirus disease 2019 (COVID-19) infection. STUDY DESIGN AND METHODS: A retrospective analysis on COVID-19 patients admitted to the intensive care unit and treated with TPE from April 17, 2020 to July 2, 2020. This group was compared with COVID-19 patients who received standard therapy without TPE. The following outcomes were analyzed: changes in laboratory parameters, length of hospital stay (LOS), days on mechanical ventilation, mortality at days 14 and overall mortality. RESULTS: A total of 95 patients were included, among whom 47% (n = 45) received TPE. Patients who received TPE had reductions in C-reactive protein (P = .002), ferritin (P < .001) and interleukin-6 (P = .013). After employing entropy-balancing matching method, those on TPE were also more likely to discontinue inotropes (72% vs 21%; P < .001). However, they were more likely to be associated with longer LOS (23 vs 14 days; P = .002) and longer days on ventilatory support (14 vs 8 days; P < .001). Despite marginal mortality benefit at 14-days (7.9% vs 24%; P = .071), there was no significant differences in overall mortality (21% vs 31%; P = .315) between the groups. CONCLUSIONS: TPE was effective in reducing inflammatory markers in patients with severe COVID-19 infection, however, further research is warranted.

The risk of medically uncontrolled secondary hyperparathyroidism depends on parathyroid hormone levels at haemodialysis initiation.

BACKGROUND: Optimal parathyroid hormone (PTH) control during non-dialysis chronic kidney disease (ND-CKD) might decrease the subsequent risk of parathyroid hyperplasia and uncontrolled secondary hyperparathyroidism (SHPT) on dialysis. However, the evidence for recommending PTH targets and therapeutic strategies is weak for ND-CKD. We evaluated the patient characteristics, treatment patterns and PTH control over the first year of haemodialysis (HD) by PTH prior to HD initiation. METHODS: We studied 5683 incident HD patients from 21 countries in Dialysis Outcomes and Practice Patterns Study Phases 4-6 (2009-18). We stratified by PTH measured immediately prior to HD initiation and reported the monthly prescription prevalence of active vitamin D and calcimimetics over the first year of HD and risk of PTH >600 pg/mL after 9-12 months on HD. RESULTS: The 16% of patients with PTH >600 pg/mL prior to HD initiation were more likely to be prescribed active vitamin D and calcimimetics during the first year of HD. The prevalence of PTH >600 pg/mL 9-12 months after start of HD was greater for patients who initiated HD with PTH >600 (29%) versus 150-300 (7%) pg/mL (adjusted risk difference: 19%; 95% confidence interval : 15%, 23%). The patients with sustained PTH >600 pg/mL after 9-12 months on HD were younger, more likely to be black, and had higher serum phosphorus and estimated glomerular filtration rates at HD initiation. CONCLUSIONS: Increased PTH before HD start predicted a higher PTH level 9-12 months later, despite greater use of active vitamin D and calcimimetics. More targeted PTH control during ND-CKD may influence outcomes during HD, raising the need for PTH target guidelines in these patients.

Birthweight predicts glomerular filtration rate in adult-life: population based cross sectional study.

INTRODUCTION: Worldwide, there is a global progressive rise of chronic kidney disease. In parallel, children born after intra-uterine growth retardation are surviving to adult-life and beyond. This study describes the association of birthweight with and estimated glomerular filtration rate (eGFR). METHODS: Australian Diabetes, Obesity and Lifestyle (AusDiab) study participants were asked to complete a birthweight questionnaire. The associations between birthweight and eGFR were determined. RESULTS: A total of 4502 reported information related to their birthweight, with the other responders did not provide a value. The birthweight of the participants ranged from 0.4 to 7.0 kg with a mean-(SD) of 3.37 (0.7) kg. The mean (95%CI) birthweight was lower for females, 3.28 (0.6) kg, when compared to males, 3.5 (0.7) kg. Eight percent had a birthweight less than 2.5 kg. The eGFR was strongly and positively associated with birthweight, with people in the lowest sex-specific birthweight-quintiles having the lowest mean eGFR. This relationship persisted with adjustment for confounding factors. The OR(CI) for eGFR <10th-percentile (<61.4 ml/min for females and <73.4 for males) for people in the lowest vs. the higher birthweight-quintile was 2.19 (95%CI 1.14-4.2) for females and 2.37 (1.1-5.3) for males, after adjustment for other factors. CONCLUSIONS: Birthweight had a positive relationship with eGFR. Possible explanations include an association of birthweight with nephron-endowment. From a global health perspective but more in developing countries and in populations in epidemiologic transition, where substantially lower birthweights coexist with recently improved infant and adult survivals, the overall impact of this phenomenon on the population health profile could be more substantial.

Clinical and genetic characteristics of autosomal recessive polycystic kidney disease in Oman.

BACKGROUND: There is a high prevalence of rare genetic disorders in the Middle East, and their study provides unique clinical and genetic insights. Autosomal recessive polycystic kidney disease (ARPKD) is one of the leading causes of kidney and liver-associated morbidity and mortality in Oman. We describe the clinical and genetic profile of cohort of ARPKD patients. METHODS: We studied patients with a clinical diagnosis of ARPKD (n = 40) and their relatives (parents (n = 24) and unaffected siblings (n = 10)) from 32 apparently unrelated families, who were referred to the National Genetic Centre in Oman between January 2015 and December 2018. Genetic analysis of PKHD1 if not previously known was performed using targeted exon PCR of known disease alleles and Sanger sequencing. RESULTS: A clinical diagnosis of ARPKD was made prenatally in 8 patients, 21 were diagnosed during infancy (0-1 year), 9 during early childhood (2-8 years) and 2 at later ages (9-13 years). Clinical phenotypes included polycystic kidneys, hypertension, hepatic fibrosis and splenomegaly. Twenty-four patients had documented chronic kidney disease (median age 3 years). Twenty-four out of the 32 families had a family history suggesting an autosomal recessive pattern of inherited kidney disease, and there was known consanguinity in 21 families (66%). A molecular genetic diagnosis with biallelic PKHD1 mutations was known in 18 patients and newly identified in 20 other patients, totalling 38 patients from 30 different families. Two unrelated patients remained genetically unsolved. The different PKHD1 missense pathogenic variants were: c.107C > T, p.(Thr36Met); c.406A > G, p.(Thr136Ala); c.4870C > T, p.(Arg1624Trp) and c.9370C > T, p.(His3124Tyr) located in exons 3, 6, 32 and 58, respectively. The c.406A > G, p.(Thr136Ala) missense mutation was detected homozygously in one family and heterozygously with a c.107C > T, p.(Thr36Met) allele in 5 other families. Overall, the most commonly detected pathogenic allele was c.107C > T; (Thr36Met), which was seen in 24 families. CONCLUSIONS: Molecular genetic screening of PKHD1 in clinically suspected ARPKD cases produced a high diagnostic rate. The limited number of PKHD1 missense variants identified in ARPKD cases suggests these may be common founder alleles in the Omani population. Cost effective targeted PCR analysis of these specific alleles can be a useful diagnostic tool for future cases of suspected ARPKD in Oman.

Missed Hemodialysis Treatments: International Variation, Predictors, and Outcomes in the Dialysis Outcomes and Practice Patterns Study (DOPPS).

RATIONALE & OBJECTIVE: Missed hemodialysis (HD) treatments not due to hospitalization have been associated with poor clinical outcomes and related in part to treatment nonadherence. Using data from the Dialysis Outcomes and Practice Patterns Study (DOPPS) phase 5 (2012-2015), we report findings from an international investigation of missed treatments among patients prescribed thrice-weekly HD. STUDY DESIGN: Prospective observational study. SETTING & PARTICIPANTS: 8,501 patients participating in DOPPS, on HD therapy for more than 120 days, from 20 countries. Longitudinal and cross-sectional analyses were performed based on the 4,493 patients from countries in which 4-month missed treatment risk was > 5%. PREDICTORS: The main predictor of patient outcomes was 1 or more missed treatments in the 4 months before DOPPS phase 5 enrollment; predictors of missed treatments included country, patient characteristics, and clinical factors. OUTCOMES: Mortality, hospitalization, laboratory measures, patient-reported outcomes, and 4-month missed treatment risk. ANALYTICAL APPROACH: Outcomes were assessed using Cox proportional hazards, logistic, and linear regression, adjusting for case-mix and country. RESULTS: The 4-month missed treatment risk varied more than 50-fold across all 20 DOPPS countries, ranging from < 1% in Italy and Japan to 24% in the United States. Missed treatments were more likely with younger age, less time on dialysis therapy, shorter HD treatment time, lower Kt/V, longer travel time to HD centers, and more symptoms of depression. Missed treatments were positively associated with all-cause mortality (HR, 1.68; 95% CI, 1.37-2.05), cardiovascular mortality, sudden death/cardiac arrest, hospitalization, serum phosphorus level > 5.5mg/dL, parathyroid hormone level > 300pg/mL, hemoglobin level < 10g/dL, higher kidney disease burden, and worse general and mental health. LIMITATIONS: Possible residual confounding; temporal ambiguity in the cross-sectional analyses. CONCLUSIONS: In the countries with a 4-month missed treatment risk > 5%, HD patients were more likely to die, be hospitalized, and have poorer patient-reported outcomes and laboratory measures when 1 or more missed treatments occurred in a 4-month period. The large variation in missed treatments across 20 nations suggests that their occurrence is potentially modifiable, especially in the United States and other countries in which missed treatment risk is high.

Time to address gender inequalities against female physicians.

Although the health care system depends heavily on female physicians, it discriminates against women and tends to concentrate female physicians' work in lower status occupations. Gender discrimination has structural, social, and cultural dimensions. Such discrimination is perceived differently by various stakeholders and the public. In addition, there is reluctance to publicly acknowledge gender discrimination, especially in the culturally conservative Middle East region. Gender discrimination leads to underrepresentation of female physicians in leadership roles and certain specialties and hence leads to less attention and understanding of the working conditions of female physicians and their roles in the health care system. The lack of accessible data in the region regarding gender discrimination among physicians leads to stakeholders failing to recognize the existence and magnitude of this type of discrimination. This article takes up the relatively neglected issue of gender discrimination in the health care workforce among the stakeholders of the Ministry of Health and Prevention of the United Arab Emirates. Future research should explore the extent of gender discrimination among physicians and the gender remuneration gap, together with other sorts of discrimination, perception of equal opportunity, and dominant stereotypes of men and women working in health care in relation to job obligation, promotion, retention, remuneration, and education.

Health workforce in the United Arab Emirates: analytic point of view.

A strong health system is impossible without health workers who are the ultimate resource. Money and medical supplies are needed, but these inputs require an efficient workforce. Challenges with respect to human resources vary greatly between and within countries, and are associated with the political, economical, cultural and societal context of a country. Moreover, the gaps in the workforce do not generally relate to doctors but to nurses and other classes of health worker who make up the bulk of health workforce. The difficulties caused by low staff numbers are compounded by morale problems, skill imbalances and geographical maldistribution. This paper will discuss how it is difficult for the United Arab Emirates (UAE, a Middle East federation country) to wrestle effectively with the demands of a good health system, exploring how they lack the basis of health systems-motivated, trained and supported people. Additionally, we looked at how the UAE health system further challenged by negative work environment, and weak knowledge-base, out-migration and inadequate investment. At the end of our discussion, we are providing some suggestions to manage human resource problems in the UAE. Highlighting how a national workforce strategic plan is important to guide investments in human resources as the core component of strengthening the UAE national health system.

Performance of Modification of Diet in Renal Disease and Chronic Kidney Disease Epidemiology Collaboration Equations Versus 99Tc-DTPA-Renogram in Assessing Kidney Function.

Objectives: To evaluate the performance of measurement of glomerular filtration rate (GFR) using Modification of Diet in Renal Disease equations (MDRD186, MDRD175) and Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) equations, in comparison with technetium-99m diethylenetriaminepentaacetic acid (99Tc-DTPA) renogram method, the gold standard. A related aim was to correlate the three equations to estimate GFR and their impact on reclassifying the stages of CKD in adult Omani patients. Methods: This cross-sectional study recruited two groups of patients diagnosed with CKD during a 10-month period from January to October 2021. The first group comprised 48 patients who underwent a 99Tc-DTPA renogram procedure for GFR measurement, and the second group comprised 30 348 adult patients who did not undergo the same procedure; estimated GFR was calculated using the three equations. Results: The median of the reference GFR was 106.0 mL/min/1.73 m2, whereas the median estimated GFR for the MDRD175, MDRD186, and CKD-EPI equations were 92.5, 98.3, and 102.1, respectively. All three equations correlated moderately with the reference GFR (0.428, 0.428, 0.523, respectively; p < 0.010). The CKD-EPI showed lesser bias (3.7 vs. 12.9 and 7.5 for MDRD175 and MDRD186, respectively) and more accuracy (95.8% vs. 91.7% and 93.8%); however, it was the least precise (25.1 vs. 22.3 and 23.8). The MDRD186 performed similarly to the CKD-EPI equation at CKD stages 3a-5 and differed significantly at stages 1-2. Whereas the MDRD175 differed significantly with both equations at stages 1-3b and was similar to them at stages 4-5. Conclusions: The CKD-EPI equation had the highest accuracy and the least bias and precision in the general population. The MDRD186 CKD classification differed significantly from the CKD-EPI equation at CKD-stages 1-2 only. The CKD-EPI equation is preferred to MDRD for the detection and classification of early CKD stages.

Simultaneous Pancreatic and Kidney Transplant in Adult with Autosomal Dominant Polycystic Kidney Disease and Type I Diabetes Mellitus: Post Surgical Events and Genetic Review.

Autosomal dominant polycystic kidney disease (ADPKD) is a common inherited condition characterized by the growth of multiple bilateral cysts in the kidneys. We describe the case of a 35-year-old male with combined ADPKD and type 1 diabetes mellitus with a strong family history of both. At the age of 32, he developed end-stage kidney disease for which he underwent preemptive simultaneous pancreatic and kidney transplant, which in turn led to multiple perioperative complications. Evaluation of familial clustering of genetic disease is critical in genetic epidemiology and precision medicine as it enables estimation of lifetime disease risk and early assessment as well as detection of the disease among one's siblings.

Unusual Presentation of Aggressive Atypical Hemolytic Uremic Syndrome With Brugada Syndrome.

Hemolytic uremic syndrome (HUS) is part of a spectrum of disorders known as thrombotic microangiopathies. These disorders are characterized by giving rise to platelet microthrombi, which subsequently develop hemolytic anemia and thrombocytopenia. In HUS, the kidneys are destroyed, mainly due to damage to the renal blood vessels. HUS can be typical or atypical, depending on the cause, and can lead to significant mortality rates. We herein report an unusual case of atypical HUS in a 15-year-old female who presented with fatigue, abdominal pain with nausea and vomiting, loss of appetite, and urine discoloration. Further tests showed low platelets with significant anemia. She was diagnosed with atypical HUS after discovering that she had no previous bloody diarrhea episode with a negative E. coli strain, O157:H7, alongside valid ADAMTS13 activity. The diagnosis was confirmed by genetic testing, and a variant of uncertain significance was found in the CFH gene. The patient, therefore, was started on eculizumab, and a follow-up was done once or twice a month through blood testing. She showed significant improvement. Due to non-compliance with the eculizumab treatment, the patient showed deterioration numerous times. A kidney biopsy was subsequently done, showing signs of acute to chronic thrombotic microangiopathy with moderate tubular atrophy and interstitial fibrosis. After many hemodialysis and plasma exchange sessions and being put on several treatments, such as prednisolone and rituximab, the patient faced death after one year.

COVID-19 Associated Acute Viral Myocarditis and Thyroid Gland Follicular Neoplasm in a Hemodialysis Patient.

Since the first cases were reported in Wuhan, China, COVID-19 has spread swiftly worldwide and is caused by SARS-CoV-2. The development of myocardial injury is associated with significantly worse clinical course and increased mortality. However, currently, it is unclear whether cardiac injury occurred in COVID-19 patients. Histological results obtained directly from the viral infection of the myocardium (i.e., SARS-CoV-2 viral myocarditis) or indirectly from the complications of COVID-19, showed that only a portion of patients infected with the virus developed viral myocarditis. Therefore, it is possible that with more autopsy evidence of SARS-CoV-2 and more correlation with the severity of the viral infection, viral myocarditis will emerge. Although COVID-19 manifests primarily as respiratory disease, few cases of cardiac injury without respiratory involvement or febrile illness have been reported. The pathogenesis of cancer and viral infections is due to the inability of the immune system to distinguish between self and non-self. Several oncogenic (hepatitis B virus, hepatitis C virus, human papilloma virus, Epstein-Barr virus, and HIV) and oncolytic viruses (coxsackievirus, reovirus, vaccinia virus, and adenovirus) are known to cause and regress various cancer types. We report a case of atypical manifestation of COVID-19-induced acute myocarditis and thyroid gland follicular neoplasm in a hemodialysis patient with no respiratory symptoms. This case illustrates that COVID-19 can present atypically and affect non-respiratory organ systems.

Acute Pulmonary Embolism in Oman: A Situational Analysis.

Objectives: Pulmonary embolism (PE) is increasingly prevalent in Oman. The aim of this situational analysis of PE among Omani patients was to study its predisposing factors, diagnostic issues, and optimal management. Methods: In this retrospective cross-sectional situational analysis, the subjects were Omani patients who were diagnosed with acute PE using computed tomography pulmonary angiogram from 2010-2021. The required data was collected from the hospital database and statistically analyzed. Results: The subjects were 438 patients diagnosed with PE, with a mean age of 53.3±18.5 years and mean body mass index of 29.7±7.3 kg/m2. Males were in a slight majority (223; 50.9%) and were older (55.0±18.5 years) than females (51.5±18.3 years). Two-thirds of the PE patients had hypertension and one-third had diabetes mellitus. Most patients presented with dyspnea and chest pain. Syncope was more common in females compared to males who mostly presented with hemoptysis. Nearly half of the patients had abnormal echocardiogram, and males had lower mean ejection fraction compared to females. Different modalities of management were used to treat patients' PE. Heparin was the most used anticoagulant followed by warfarin and direct oral anticoagulants. Out of the 122 PE patients who died during the study period, PE was the direct cause of death of 68 patients. The mortality was higher in males than in females. Conclusions: The incidence of acute PE was similar among both male and female Omani patients. However, there were sex differences in risk factors, symptoms, investigations, and management of PE. Females presented with more severe PE, but mortality was higher in males.

Mycophenolate Induced Colitis: One-year Post-kidney Transplantation.

The incidence of end-stage kidney disease (ESKD) has been increasing over the past few years as a direct result of the growing percentages of individuals with metabolic syndrome. From 2001 to 2015 there were 2805 individuals diagnosed with ESKD in Oman with a growing number of patients undergoing renal transplant as the gold standard management of renal replacement therapy. Mycophenolate mofetil (MMF) is one of the most frequently used medications as a part of immunosuppressive medications in renal transplant specifically and solid organ transplant generally. We are reporting a case of MMF-induced colitis in a young female patient that underwent a living-related kidney transplant. She presented with a three-month history of watery non-bloody and afebrile diarrhea. Investigations confirmed the diagnosis of MMF-induced colitis. Histopathological examination of colonic biopsies obtained during the colonoscopy procedure showed mildly increased crypt apoptosis, mild architectural disarray, and focal crypt attenuation; features consistent with MMF-induced colitis. The patient was treated by stopping the causative agent and replacing it with another immunosuppressive medication, which led to complete resolution of the symptoms on follow-up appointments. In this case report, we highlighted the underlying mechanism, pathogenesis, and clinical features of MMF-induced colitis.

Peritoneal dialysis in the Arabian Gulf countries: Challenges and opportunities.

The Gulf Cooperation Council (GCC) is a regional organisation, consisting of six Arab countries that share common objectives and cultural identities, with a total population of 57.3 million. The prevalence of patients requiring dialysis in GCC countries is increasing, with a current mean prevalence of 551 per million population. Despite the several patient-level and healthcare system benefits of peritoneal dialysis (PD) compared to in-centre haemodialysis, the growth in PD utilisation has been limited. This is related to several factors, including deficiencies in modality education for chronic kidney disease patients, nephrology training and governmental policies advocating for this dialysis modality. Establishing a detailed PD registry in GCC countries is an important step towards understanding our patients' characteristics, outcomes, current PD practices and challenges in order to increase the use of PD and to facilitate future initiatives aimed at optimising the management of PD patients in this part of the world. This article reviews common challenges around PD practices and utilisation in GCC countries and provides possible solutions to overcome these challenges. It should be noted that the literature on PD patients, outcomes and treatment practices in GCC countries is limited, and as a result, many of our recommendations and discussion are based on clinical observations, experience and data when available.

Epidemiology, Management, and Outcome of Atypical Hemolytic Uremic Syndrome in an Omani Cohort.

Objectives: Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening disease of chronic uncontrolled complement pathway activation that leads to thrombotic-microangiopathy, along with severe organ damage, including end-stage kidney disease. This study aimed to evaluate the epidemiology, management, and outcome of aHUS in an Omani population. Methods: This retrospective descriptive cohort study assessed all cases of aHUS diagnosed and followed up at two tertiary care centers in Oman from January 2008 to December 2019, based on clinical features, complement pathway assays, histopathological, and genetic testing. Results: The study accrued 19 patients who fulfilled the inclusion criteria, of whom 11 (57.9%) were male. The participants' median age was 25.0 years (range = 0.1-69.0). Most (15; 78.9%) patients presented in the acute phase of the disease. The triad of hemolytic anemia, acute kidney injury, and thrombocytopenia was present in all patients. A trigger factor (e.g., infection) was identified in 68.4% of cases. Of the 14 (73.7%) patients who underwent kidney biopsy, 10 (71.4%) were found to have aHUS in native kidneys and three in grafted kidneys. Of the 11 (57.9%) patients who underwent genetic analysis, five (45.5%) were found to have a known pathogenic variant in their aHUS susceptibility genes. Plasma exchange followed by eculizumab was the treatment method in 11 (57.9%) cases. Complete renal recovery was achieved in seven (36.8%) patients, while four (21.1%) passed away during the study period. Conclusions: The wide spectrum and multiple expressions of aHUS make it a challenge to diagnose and consequently may delay the commencement of the targeted treatment. Eculizumab is considered the first-line therapy and should be commenced as early as possible.

Quality of Life and Health-related Quality of Life in Patients with End-stage Kidney Disease Undergoing Hemodialysis: A Literature Review.

Measurements of quality are intended to drive improvements in care and provide accountability regarding costs and quality. Quality of life (QoL) and health-related QoL (HRQoL) comprise personal perceptions, health, and socioenvironmental dimensions. This structured integrative review aimed to present and analyze the nature and significance of the predictors of QoL and HRQoL in patients with end-stage kidney disease (ESKD). The articles found through searching the main databases were assessed for sample size, design, and methodological limitations. The revised Wilson-Cleary conceptual framework of HRQoL and the World Health Organization's definition of QoL guided this review. Forty-five articles were selected (36 were observational or cross-sectional studies; nine were prospective). These articles reported a range of factors related to QoL and HRQoL characterized as physical, mental, socioeconomic, biological, and symptomatic. Few studies considered spiritual beliefs and cultural beliefs. There was a lack of consistency in the use of measures of QoL and HRQoL in ESKD. The most validated measures of HRQoL and QoL identified were the Short-Form 36 v2, the QoL Index - Dialysis, the Hospital, Anxiety, and Depression Scale, the Fatigue Severity Scale, the Itch Scale, the Spiritual Well-being Scale, and the Schedule for the Evaluation of QoL - Direct Weighting. Most studies were conducted in developed countries, with only two from the Middle East. The possible measures of QoL and HRQoL are health status, disease-specific, symptom-specific, spiritual, and individualized QoL measures. This set of measures is expected to capture the patients' own perceptions concerning their QoL and HRQoL.

Late Presentation for Kidney Biopsy: Clinical Presentations and Laboratory Findings.

Although the number of patients reaching end-stage kidney disease without a biopsy- proven diagnosis is increasing, kidney biopsies play a key role in diagnosing kidney disease. We analyzed prospective data from patients with kidney disease who underwent percutaneous native kidney biopsies from January 2006 to December 2017. Demographic data, clinical presentations, and the laboratory and radiological findings at the time of biopsy were analyzed. Of 530 patients, 42.8% were male. The mean age was 33.9 (32.8-34.9.2) years; 66.3% were aged 25-64 years. Edema was the main clinical presentation (61.9%), with clinical urine changes seen in 66.7%. Most (89.6%) were nondiabetic; 46.8% had high blood pressure or were on antihypertensive therapy. Most patients (77.5%) were in Stages I, II, and III, and 12.3% underwent hemodialysis at the time of admission. Most (54.4%) were obese. Low hemoglobin (31.8%), high triglycerides (30%), high total cholesterol (58.2%), low serum albumin (73.9%), nephrotic proteinuria (61.8.6%), and microscopic hematuria (79.8%) were the main laboratory findings. The immunological investigations showed that antinuclear antibodies, positive anti-double-stranded DNA (anti-dsDNA), and extractable nuclear antigens were positive in 29.6%, 20.7%, and 19.7%, respectively. Perinuclear antineutrophil cytoplasmic antibodies (ANCA) were positive in 9.6% and cytoplasmic ANCA were positive in 5.4%, whereas immunoglobulin A was detected in 4.6%. More than one- third of the patients had reached advanced chronic kidney disease (CKD) Stages IIIB, IV, and V. This indicates the need to increase awareness about CKD, greater utilization of kidney biopsies, and earlier investigations to enable accurate diagnoses, and proper and timely management.

Hepatitis Status and its Effect upon the Progression of Chronic Kidney Disease: Presentations, Associations and Complications.

Chronic kidney disease (CKD), hepatitis B virus (HBV), and hepatitis C virus (HCV) have a high prevalence in Oman. This study aimed to examine the association between CKD and viral hepatitis through an observational cohort study conducted at the Royal Hospital of the Sultanate of Oman to evaluate the relationship of HBV and HCV with CKD. During the study, 233 patients were identified, 112 with chronic HBV (Group 1), 112 with chronic HCV (Group 2), and nine with HBV and HCV coinfection (Group 3). The population was predominantly male, especially in Groups 1 and 3. The difference in age between Groups 1 and 2 was significant, with the mean age being 48 ± 14.6 years and 55 ± 12.6 years, respectively (P <0.05). This study revealed that the prevalence of CKD in Group 1 is 51%, in Group 2 was 78%, and in Group 3 was 56%. The mean estimated glomerular filtration rate (eGFR) was 79.7 mL/min/1.73 m2 in Group 1, 73.2 mL/min/1.73 m2 in Group 2, and 57.6 mL/min/1.73 m2 in Group 3. CKD had the highest prevalence in Group 2. The lowest eGFR was found in Group 3. Group 2 showed the highest rate of declining renal function over time despite treatment. This study found a significant and independent association between viral hepatitis and the risk of CKD, especially in cases of coinfection and HCV infection. This warrants close monitoring of kidney function during screening and follow-up. Patients with CKD should be screened for viral hepatitis.