Thyroid Dysfunction and Kidney Dysfunction.

Objectives: The incidence and prevalence of chronic kidney disease (CKD) are rising worldwide. It is becoming more common in the developing world with the increasing impact of non-communicable diseases in these countries. Also, autoimmune disorders, including thyroid dysfunction are more common and may worsen the clinical status of patients with CKD. We sought to determine the thyroid status in patients with CKD and explore the clinical, biochemical, immunological, and hematological parameters that can be affected by thyroid dysfunction among CKD patients. Methods: We conducted a cross-sectional observational study at the Royal Hospital, Muscat. The data was progressively collected for all newly diagnosed CKD patients with no known history of thyroid disease from January 2018 to December 2019. Assessment of thyroid status was performed at their initial diagnosis. Results: During the study period, 121 females (40.3%) and 179 males (59.7%) were diagnosed with CKD with no prior thyroid diseases. The mean age for females and males were 53.6±16.5 and 49.5±16.5 years, respectively. There were 35 patients with thyroid dysfunction with a prevalence of 11.7%. Of these, 22 patients (62.9%) had subclinical hypothyroidism, and 13 (37.1%) had subclinical hyperthyroidism. Total cholesterol and low-density lipoprotein were higher in hypothyroid patients. Urea was higher in hyperthyroid patients with CKD, and hemoglobin level was significantly lower. Conclusions: Thyroid dysfunction was not uncommon among CKD patients, with subclinical hypothyroidism more common than subclinical hyperthyroidism. Thyroid dysfunctions coexisted with kidney dysfunction. These hormonal axis dysfunctions may not be apparent at first presentation; and therefore, may require close clinical and laboratory evaluations.

Epidemiology of Cancer Among Chronic Kidney Disease Patients Compared to The General Population.

BACKGROUND: Kidney disease has a major effect on global health, both as direct cause of morbidity and mortality and as an important factor for other comorbid diseases including malignancy. Different studies report a higher risk of cancer development in patients with chronic kidney disease (CKD), but the impact of less sever CKD on risk of cancer is uncertain. However, data concerning the cancer risk in Oman CKD including dialysis patients is scarce. More importantly, there is lack of information about the cancer-specific mortality in CKD and dialysis patients. METHODS: During January 2006 to December 2019, all patients with CKD and those on regular dialysis who are admitted or follow up in Royal Hospital were included for evaluation of malignancy. RESULTS: During the study, a total of 2500 patients with CKD were included, of which 25 patients were found to have different types of cancers, of which 13 were male (52%) and 12 were female (48%).Most of patients 13 (52%) were senior adult (>65 years), then 11 (44%) were adult (19:64- ys) and only one case (4%) was child (<18years.). Cancer was detected in the ovaries (16%), stomach, multiple myeloma and renal (12%) each, while breast and colorectal (1 male/1 female) (8%), parathyroid, thyroid, uterus, cervix, prostate, skin, liver, lymphoma, pituitary gland, and myelofibrosis were present in 4% each. In general population, the breast cancer is the most common cancer among females, followed by thyroid cancer, and colorectal cancer while the most common cancer types among males are colorectal cancer followed by the prostate and then the Non-Hodgkin lymphoma disease. CONCLUSION: These epidemiologic findings should prompt clinicians and health authorities to assess strategies for cancer screening in high-risk population of CKD patients. Additional studies are needed to explain the reasons for this association and represent the potential use of cancer screening in patients with CKD in Oman. KEY WORDS: cancer, chronic kidney disease, epidemiology, hemodialysis.

The role of thymectomy in myasthenia gravis: A programmatic approach to thymectomy and perioperative management of myasthenia gravis.

BACKGROUND: Myasthenia gravis is a rare autoimmune disease caused by antibodies that probably originate from the thymus glands. This study examined the epidemiology of patients with MG, who underwent thymectomy over the last three decades. METHODS: The objectives of this observational study were to investigate the clinicopathological features, treatment modalities, and prognostic factors for patients with thymic masses, over three decades at the Royal Hospital, Muscat, Oman. RESULTS: There were 100 patients who underwent thymectomy with a mean (SD) age of 32.0 (8.6) years, of which 20% were men and 80% were women. Their follow up period, cardiac and neurology clinics, ranged from 1.5 to 12.0 years with a mean (SD) of 6.0 (3.0) years. Small percentage of MG patients had diabetes and hypertension and 10% of patients have positive family history of MG. Symptoms at the onset of the disease were ophthalmoplegia in 75%, limb weakness in 39%, bulbar symptoms in 57% and respiratory symptoms in 39% of patients. The Osserman grading was Grade I - 5%, Grade IIA - 39%, Grade IIB - 34%, and Grade III - 22%. Post thymectomy, 21% of patients had complete clinical remission, 76% of patients had significant clinical improvement and 3% had no apparent improvement in their clinical status. Histologically, hyperplasia was found in 57% and involuted thymus in 18% of patients. CONCLUSION: Thymectomy can reduce patient's need for medication and reduce the severity of MG regardless of age, sex, severity, or length of sickness, or thymic masses. The early-onset, sever M.G, female, thymic hyperplasia benefit the most. Patients classified as Osserman Class IIA and IIB benefit most from this procedure.

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.

Awareness, knowledge, and perception of chronic kidney disease patients at renal medicine outpatients' clinic.

Chronic kidney disease (CKD) is a major cause of morbidity and mortality. Awareness and education of CKD patients positively influence its medical management and significantly diminish the economic burden on public health. We conducted a pretested, structured, questionnaire-based survey to assess the awareness, knowledge, and perception of CKD patients at Renal Medicine Outpatients' Clinic. The questionnaire was distributed to all CKD patients of at least one-year duration after their diagnosis. A total of 200 patients completed this study, mainly from the capital region. The mean age was 50 (17.1) years for males and 42.3 (16) years for females. The majority (74%) knew the reason for their referral to a nephrologist. Nearly 38% of the patients with CKD reported their shock and anger when informed about their kidney status by the nephrologist at the first encounter. Almost 64% of the patients reported having no knowledge of their CKD status and only 18% of the participants were aware that they were in CKD Stage 5, but according to the medical health computerized system, 44% were in CKD Stage 5. In addition, only 50% of them were aware of renal replacement therapy options. Patients and their family should receive enough information and education regarding the nature of kidney disease and the treatment options to allow them to make an informed decision about the management of their CKD. A well-informed decision-making in the treatment of CKD can provide health professionals with evidence on how to best deliver education programs for patients and their families. It may also enhance communication and improve the capacity of patient and family involvement in shared decision-making.

Commercial Kidney Transplantation: Attitude, Knowledge, Perception, and Experience of Recipients.

INTRODUCTION: Kidney transplantation is the gold standard for patients with end-stage kidney disease. In view of shortages of available organs, long wait times for possible transplantation, and strict regulation, many patients opt for commercial transplantation. This study elicits the reasons and motivations for patients with end-stage kidney disease to elect for commercial transplant. METHODS: A questionnaire-based evaluation was conducted during the period from July 2015 until late December 2015. It consisted of 29 multiple choice questions and was distributed to all patients who underwent commercial kidney transplantation. RESULTS: One hundred and fifty patients were approached to participate and 106 agreed. Of the participants, 60% were male with an average age of 41.5 (SD 14.8) years and ranged from 18 to 83 years. The majority (82%) of our participants were educated ranging from primary to college level. The major reason (71%) for these participants to obtain commercial transplants was stated as the unavailability of a live related donor. Thirteen percent stated that they objected to getting a kidney donated from a family member, and 9% stated that they were worried about taking a kidney from a family member. Finally, 3% of participants stated that they needed prompt transplant and could not wait for a long time for transplant investigations and the workup associated with this program. DISCUSSION: The study showed that the most common underlying cause for seeking commercial transplantation is the unavailability of a national transplant program, particularly transplantation from deceased sources. All western ethical arguments turn out to become of vital importance in developing countries, because transplantation is the cheapest renal replacement therapy. However, it must be emphasized that commercial transplants should not be an alternative to building a national transplant initiative. The national diseased program must be a priority with full financial and administrative support. All government agencies including religious affairs must work together to support the program and to provide the citizens with a good transplantation service and ameliorate the impact of commercial transplantation.