Improvement of Encapsulating Peritoneal Sclerosis After Medical Treatment and Successful Deceased Donor Kidney Transplant in a Child: A Case Report.

BACKGROUND: Encapsulating peritoneal sclerosis (EPS) is an uncommon complication of long-term peritoneal dialysis (PD). Despite its rarity, EPS significantly increases morbidity and mortality in patients undergoing prolonged peritoneal dialysis. In children on PD, the incidence of EPS ranges from 0.5% to 7.3%. CASE: We present the case of a 13-year-old Omani boy diagnosed with end-stage kidney disease (ESKD) secondary to steroid-resistant nephrotic syndrome due to diffuse mesangial sclerosis at the age of 2 years. He was started on automated peritoneal dialysis (APD) on the same year and experienced four episodes of peritonitis, which were treated successfully with intraperitoneal (IP) antibiotics. In January 2023, he developed intermittent abdominal pain and chronic constipation, which progressed to daily vomiting, reduced oral intake, and weight loss. He later developed subacute intestinal obstruction which was managed conservatively. A CT scan of the abdomen revealed findings consistent with EPS. Following the diagnosis of EPS, peritoneal dialysis (PD) was discontinued, and the patient transitioned to hemodialysis. Treatment for EPS began with steroids and Tamoxifen. Subsequently, he underwent deceased donor kidney transplantation and was started on multiple immunosuppressive medications. During subsequent follow-up appointments, he was maintained on total parenteral nutrition (TPN) along with a soft diet. His overall condition improved significantly, enhancing his quality of life. CONCLUSION: This case highlights the risk of encapsulating peritoneal sclerosis (EPS) in patients undergoing long-term peritoneal dialysis. Transitioning to hemodialysis and kidney transplantation, combined with targeted treatments such as steroids and Tamoxifen, significantly improved the patient's condition and quality of life. Early diagnosis and intervention are crucial for effective management of EPS in children.

Vogt-Koyanagi-Harada Syndrome (VKHS): First Two Cases Reported in Pediatric Age Group in Oman.

The Vogt-Koyanagi-Harada syndrome (VKHS) is a unique form of granulomatous autoimmune disease that mostly impacts the pigmented tissues of the body. The main feature is bilateral granulomatous panuveitis, which is detected on ophthalmologic examination, along with additional systemic signs such as vitiligo, white hair, neurological involvement, or hearing loss. This study aims to report two cases of Vogt-Koyanagi-Harada syndrome presented in the children age group, which is unusual and very rare, to improve recognition of this disease to avoid complications and delay referral.

Further phenotypic delineation of Alazami syndrome.

Alazami syndrome (AS) is an autosomal recessive condition characterized by the cardinal features of severe growth restriction, moderate to severe intellectual disability, and distinctive facial features. Biallelic pathogenic variants of the LARP7, encoding a chaperone of 7SK noncoding RNA, is implicated in this disease. There are <35 reported cases in the literature. All reported cases share the same three cardinal features of the syndrome. Herein, we report on 12 patients with a confirmed diagnosis of AS from eight unrelated families. The cohort shares the same key feature of the syndrome. Moreover, we report additional phenotypic features, including genito-renal anomalies, ophthalmological abnormalities, and congenital heart disease. Whole-exome sequencing was used in all reported cases, implicating a clinical under-recognition of the syndrome. This report further expands the clinical and molecular characteristics of Alazami syndrome.

Severe Pneumonitis in Omani Infants During An In-Hospital Measles Outbreak: A report of three cases.

Measles is a highly contagious infectious disease. Despite aggressive national initiatives to eradicate measles, outbreaks have occurred in recent years. We report three infants who presented to a tertiary care hospital in Muscat, Oman, in 2019 with measles and then developed pneumonitis, received intensive care treatment and made full recoveries. Infants can have an atypical presentation and develop severe symptoms. Pneumonitis is a serious complication and the management strategies are controversial. The early detection of measles and isolation of affected individuals play major roles in the elimination of measles outbreaks.