Pediatric Kidney Biopsies in Oman: A Retrospective Study.

Objectives: To determine the spectrum of kidney diseases in Omani children < 13 years of age and to evaluate the complications following kidney biopsy. Methods: This study retrospectively investigated the hospital data of children who underwent kidney biopsies from January 2014 to June 2019 at Royal Hospital, Muscat, Oman. Results: The subjects comprised of 78 children with a median age of 8.0 years (range = 0-13 years). Histopathology showed minimal change disease in 15 (19.2%) children, lupus nephritis in 13 (16.7%), and focal segmental glomerulosclerosis in 13 (16.7%). The most common post-biopsy complications were pain that required analgesia (38; 49.4%) followed by gross hematuria (10; 13.0%). No patient required blood transfusion or surgical intervention. Conclusions: Minimal change disease was the most common histopathological finding in this cohort of Omani children. The records did not mention any major complications following the renal biopsy procedure.

Genetic analysis and outcomes of Omani children with steroid-resistant nephrotic syndrome.

BACKGROUND: Nephrotic syndrome (NS) is one of the most common kidney disorders seen by pediatric nephrologists and is defined by the presence of heavy proteinuria (>3.5 g/24 h), hypoalbuminemia (<3.5 g/dL), edema, and hyperlipidemia. Most children with NS are steroid-responsive and have a good prognosis following treatment with prednisolone. However, 10%-20% of them have steroid-resistant nephrotic syndrome (SRNS) and fail to respond to treatment. A significant proportion of these children progress to kidney failure. METHODS: This retrospective study aimed to determine the underlying genetic causes of SRNS among Omani children below 13 years old, over a 15-year period and included 77 children from 50 different families. We used targeted Sanger sequencing combined with next-generation sequencing approaches to perform molecular diagnostics. RESULTS: We found a high rate of underlying genetic causes of SRNS in 61 (79.2%) children with pathogenic variants in the associated genes. Most of these genetically solved SRNS patients were born to consanguineous parents and variants were in the homozygous state. Pathogenic variants in NPHS2 were the most common cause of SRNS in our study seen in 37 (48.05%) cases. Pathogenic variants in NPHS1 were also seen in 16 cases, especially in infants with congenital nephrotic syndrome (CNS). Other genetic causes identified included pathogenic variants in LAMB2, PLCE1, MYO1E, and NUP93. CONCLUSION: NPHS2 and NPHS1 genetic variants were the most common inherited causes of SRNS in Omani children. However, patients with variants in several other SRNS causative genes were also identified. We recommend screening for all genes responsible for SRNS in all children who present with this phenotype, which will assist in clinical management decisions and genetic counseling for the affected families.

Pediatric Renal Transplantation in Oman: A Single-center Experience.

OBJECTIVES: This study sought to report 22 years experience in pediatric kidney transplantation in Oman. METHODS: Electronic charts of all Omani children below 13 years of age who received a kidney transplant from January 1994 to December 2015 were reviewed. Data collected included patient demographics, etiology of end-stage kidney disease, modality and duration of dialysis, donor type, complication of kidney transplantation (including surgical complications, infections, graft rejection) graft and patient survival, and duration of follow-up. RESULTS: During the study period transplantation from 27 living related donors (LRDs), 42 living unrelated donors (LURDs), also referred to as commercial transplant, and one deceased donor were performed. The median age at transplantation was nine years for both groups. The most common primary diagnosis was congenital anomalies of the kidney and urinary tract in 32.8% of patients followed by familial nephrotic syndrome in 20.0% and polycystic kidney disease in 18.5%. Almost half the patients were on hemodialysis before transplantation, 35.7% were on peritoneal dialysis, and 14.2% received preemptive renal transplantation. Children who received LURD kidneys had high surgical complications (42.8%) compared to the LRDs group (17.8%). Five patients from LURDs group had early graft nephrectomy and four patients developed non-graft function or delayed graft function. In addition, patients in the LURDs group had a higher incidence of hypertension and acute rejection. Graft and patient survival were both better in the LRDs than the LURDs group. CONCLUSIONS: Although our pediatric kidney transplant program is a young program it has had successful patient outcomes comparable to international programs. Our study provides evidence that in addition to legal and ethical issues with commercial transplant, it also carries significantly higher morbidity and reduced graft and patient survival.

Primary hyperoxaluria type 1 in 18 children: genotyping and outcome.

Background. Primary hyperoxaluria belongs to a group of rare metabolic disorders with autosomal recessive inheritance. It results from genetic mutations of the AGXT gene, which is more common due to higher consanguinity rates in the developing countries. Clinical features at presentation are heterogeneous even in children from the same family; this study was conducted to determine the clinical characteristics, type of AGXT mutation, and outcome in children diagnosed with PH1 at a tertiary referral center in Oman. Method. Retrospective review of children diagnosed with PH1 at a tertiary hospital in Oman from 2000 to 2013. Result. Total of 18 children were identified. Females composed 61% of the children with median presentation age of 7 months. Severe renal failure was initial presentation in 39% and 22% presented with nephrocalcinosis and/or renal calculi. Family screening diagnosed 39% of patients. Fifty percent of the children underwent hemodialysis. 28% of children underwent organ transplantation. The most common mutation found in Omani children was c.33-34insC mutation in the AGXT gene. Conclusion. Due to consanguinity, PH1 is a common cause of ESRD in Omani children. Genetic testing is recommended to help in family counseling and helps in decreasing the incidence and disease burden; it also could be utilized for premarital screening.

2015 Oman Heart Association guidelines for the management of hypertension : practical recommendations from the Oman Heart Association (OHA).

Hypertension (HTN) is a major independent risk factor for the development of stroke, coronary artery disease (CAD), peripheral arterial disease (PAD), heart failure (HF) and chronic kidney disease (CKD). HTN is a growing public health problem in Oman, almost certainly the most prevalent modifiable risk factor for cardiovascular disease (CVD). The risk of CVD in patients with HTN can be greatly reduced with lifestyle modifications and effective antihypertensive therapy. Randomized trials have shown that blood pressure (BP) lowering produces rapid reductions in CV risk. Several studies have shown that the majority of the hypertensive patients remain uncontrolled. It is well established that the observed poor control of the disease is not only related to poor adherence to medications, but also to limited awareness and adherence to evidence-based management of hypertension among physicians. Several guidelines for the management of patients with hypertension have been published. However, the aim of this document is to provide the busy physicians in Oman with more concise and direct approach towards implementing these guidelines into clinical practice.

2014 Oman Heart Association protocol for the management of acute atrial fibrillation.

Although atrial fibrillation (AF) is the most common cardiac arrhythmia, there is variation in practice with regard to the management of acute AF among the hospitals and even within the same hospital in Oman. This variation likely reflects a lack of high-quality evidence. Standard guidelines and textbooks do not offer clear evidence-based direction for physicians to guide the management of acute AF. Particularly controversial is the issue of using rhythm control or rate control. This stimulated Oman Heart Association (OHA) to issue a simplified protocol for the management of acute AF to be applied by the entire cardiac caregivers all over the country. The priorities for acute management of AF include stabilizing the patient's hemodynamic status, symptom control, treatment of the underlying and precipitating cause, and more importantly protecting the brain.

Clinical Pathways for Non-ST Elevation Acute Coronary Syndrome in Oman: An Oman Heart Association Protocol for Hospital Quality Improvement Initiative.

In 2012, Oman Heart Association (OHA) published its own guidelines for the management of patients with unstable angina/non-ST-elevation myocardial infarction, the aim was not to be comprehensive but rather simplified and practical in order to reduce the gap between the long comprehensive guidelines and our actual practice. However, we still feel that the busy registrars and residents need simpler and direct clinical pathways or protocol to be used in the emergency departments, coronary care units and in the wards. Clinical pathways are now one of the main tools used to manage the quality in healthcare concerning the standardization of care processes. It has been shown that their implementation reduces the variability in clinical practice and improves outcomes in acute care.

2012 Oman Heart Association simplified guidelines for the management of patients with unstable angina/non-ST-elevation myocardial infarction.

Although current practice guidelines provide an evidence-based approach to the management of acute coronary syndromes, application of the evidence by individual physicians has been suboptimal. This gap between comprehensive guidelines and actual practice stimulated Oman Heart Association to issue a simplified series for the management of the common cardiac abnormalities to be applied by the entire cardiac caregivers all over the country. This simplified approach for the management of non-ST-elevation acute coronary syndrome provides a practical and systematic means to implement evidence-based medicine into clinical practice.