Insight into prevalence, etiology, and modalities of pediatric chronic dialysis: a comprehensive nationwide analysis.

BACKGROUND: This study aimed to determine the prevalence and etiology of kidney failure (KF) among children below 15 years of age receiving chronic dialysis in Saudi Arabia and describe their dialysis modalities. METHODS: This cross-sectional descriptive study was conducted on 8 August 2022, encompassing all 23 pediatric dialysis centers in Saudi Arabia. Data gathered comprised patient demographics, causes of KF, and the dialysis methods employed. Collected data underwent analysis to determine prevalence of children undergoing chronic dialysis, discern underlying causes of KF, and evaluate distribution of patients across different dialysis modalities. RESULTS: The prevalence of children on chronic dialysis is 77.6 per million children living in Saudi Arabia, equating to 419 children. The predominant underlying cause of KF was congenital anomalies of the kidneys and urinary tract (CAKUT), representing a substantial 41% of cases. Following this, others or unknown etiologies accounted for a noteworthy 25% of cases, with focal segmental glomerulosclerosis (FSGS) comprising 13%, glomerulonephritis at 11%, and congenital nephrotic syndrome contributing 10% to etiological distribution. Regarding dialysis modalities employed, 67% of patients were on peritoneal dialysis (PD), while the remaining 33% were on hemodialysis (HD). CONCLUSIONS: This first nationwide study of pediatric chronic dialysis in Saudi Arabia sheds light on the prevalence of children undergoing chronic dialysis and underlying causes of their KF, thereby contributing to our understanding of clinical management considerations. This research serves as a stepping stone for the development of national registries.

Outcomes of Endoscopic Resection in Pediatric Skull Base Chordoma: A Systematic Review.

The endoscopic approach has been recommended as a primary option for treating chordomas, and it is associated with better resection rates and fewer surgical complications than transcranial surgery. This review aimed to assess the long-term consequences and evidence in the current literature regarding the endoscopic approach's efficacy in treating skull-base chordoma in children. A systematic review was conducted based on the PubMed, Web of Science, and EMBASE databases to examine the clinical outcomes of endoscopic endonasal surgery for pediatric skull base chordoma tumors. The review included studies published in English that employed specific research designs and reported on pediatric patients with skull base chordoma. Of the 268 studies initially considered, 25 met our eligibility criteria and were included in the final analysis. The average age of the patients was 11.5 years, with approximately equal number of males and females. The endoscopic endonasal approach (EEA) was the most commonly used modality. Gross total resection (GTR) was achieved in 62.7% of patients, while 18.09% had a subtotal resection (STR), and 13.83% had near-total resection only. Most patients showed significant to moderate improvement from their baseline condition and had no recurrence during their follow-up. Our findings further endorse that the endoscopic approach is a viable primary treatment option for pediatric skull base chordoma.

Successful Management of a Broken Spinal Needle During Difficult Spinal Anesthesia: A Case Report.

Spinal anesthesia is the most common mode of anesthesia utilized during caesarian sections worldwide. Despite its many advantages over general anesthesia in the pregnant cohort, uncommon and even catastrophic complications could occur due to patient-related, equipment-related, and procedure-related complications. One such uncommon event of a broken spinal needle during failed spinal anesthesia for a caesarian section and subsequent successful management is described here.

Dermatofibrosarcoma Protuberans of the Scalp Mimicking Trichilemmal Cyst: A Case Report.

We present a case of a 47-year-old female with a swelling on her scalp that was at first thought to be trichilemmal cysts. After two years, she returned to her general practitioner with a larger scalp mass. Following a biopsy, histological analysis revealed dermatofibrosarcoma protuberans (DFSP). She then had the tumor completely removed, resulting in clean margins.

Relationship Between Asymptomatic Helicobacter Pylori Infection and BMI Among Patients Undergoing Bariatric Surgery in Saudi Arabia.

Background Obesity has become a major health concern associated with several comorbidities. Obesity has been connected to numerous variables. Furthermore, multiple studies were done worldwide to identify the relationship between obesity and Helicobacter pylori (H. pylori), and there was controversy. However, the relationship between H. pylori infection and obesity in our community is still not clear, and there is a knowledge gap. Aim To determine the relationship between asymptomatic H. pylori infection and body mass index (BMI) among patients who underwent bariatric surgery in Saudi Arabia, King Fahad Specialist Hospital - Buraidah (KFSH-B). Method An observational retrospective cohort study was conducted at KFSH-B. Patients with high BMI (>30 kg/m2) who underwent bariatric surgery between January 2017 and December 2019 were included. Gender, age, BMI, and upper GI endoscopy reports of preoperative mapping were collected from electronic health records. Results The sample size was 718, and the mean BMI (standard deviation) was 45 kg (6.8). Patients with positive H. pylori results were 245 (34.1%) and patients with negative H. pylori results were 473 (65.9%). The t-test showed the mean BMI of patients with negative H. pylori results to be 45.36 (SD 6.6). Positive H. pylori 44.95 (SD 7.2) p-value was not significant (0.44). Conclusion The data showed that patients who had undergone bariatric surgery had negative pre-operative histopathological results of H. pylori more than those who had positive results, which is consistent with the prevalence of H. pylori infection among the general population. Therefore, we found no correlation between H. pylori infection and high BMI.

A Multicenter Study Evaluating the Discontinuation of Eculizumab Therapy in Children with Atypical Hemolytic Uremic Syndrome.

BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening thrombotic microangiopathy (TMA), which has been treated successfully with eculizumab. The optimal duration of eculizumab in treating patients with aHUS remains poorly defined. METHODS: We conducted a multicenter retrospective study in the Arabian Gulf region for children of less than 18 years of age who were diagnosed with aHUS and who discontinued eculizumab between June 2013 and June 2021 to assess the rate and risk factors of aHUS recurrence. RESULTS: We analyzed 28 patients with a clinical diagnosis of aHUS who had discontinued eculizumab. The most common reason for the discontinuation of eculizumab was renal and hematological remission (71.4%), followed by negative genetic testing (28.6%). During a median follow-up period of 24 months after discontinuation, 8 patients (28.5%) experienced HUS relapse. The risk factors of recurrence were positive genetic mutations (p = 0.020). On the other hand, there was no significant relationship between the relapse and age of presentation, the need for acute dialysis, the duration of eculizumab therapy before discontinuation, or the timing of eculizumab after the presentation. Regarding the renal outcomes after discontinuation, 23 patients were in remission with normal renal function, while 4 patients had chronic kidney disease (CKD) (three of them had pre-existing chronic kidney disease (CKD) before discontinuation, and one case developed a new CKD after discontinuation) and one patient underwent transplantation. CONCLUSIONS: The discontinuation of eculizumab in patients with aHUS is not without risk; it can result in HUS recurrence. Eculizumab discontinuation can be performed with close monitoring of the patients. It is essential to assess risk the factors for relapse before eculizumab discontinuation, in particular in children with a positive complement variant and any degree of residual CKD, as HUS relapse may lead to additional loss of kidney function. Resuming eculizumab promptly after relapse is effective in most patients.

Management of Psychotic Symptoms in a Patient With Parkinson's Disease Maintained on Levodopa-Carbidopa Intestinal Gel by Paliperidone Long-Acting Injection: A Case Report.

This case presents a 47-year-old man, without known past psychiatric history who developed psychotic symptoms including delusions of infidelity and had homicidal plans against his wife after 10 months of Levodopa-Carbidopa intestinal gel insertion (LCIG). The patient was diagnosed with Parkinson's disease at age 34, which is being managed with LCIG. Patient Parkinson's symptoms were not well controlled with other pharmacological and surgical interventions tried previously. Despite the current guidelines in treating Parkinson's disease psychosis, the treating teams have faced many difficulties with the management of this patient's psychotic symptoms. After trying Risperidone Consta on August 24, 2018, the patient improved gradually, then he was shifted to Paliperidone long-acting injection (LAI) on September 12, 2018. One month later, the patient was seen in the outpatient department with much improvement in Paliperidone LAI. Reporting this case as the patient was seen on November 29, 2021, the patient is stable and doing well overall in terms of absent psychotic symptoms with minimal resting tremors. The success story of using LAIs such as our patient's response to Paliperidone LAI can help other psychiatrists expand their treatment options when facing such difficulties.

Epidemiological Features and Clinical Manifestations of Patients With Somatoform Disorder at a Tertiary Medical City in Riyadh, Saudi Arabia.

Background Somatoform disorder (SD), known as the presence of physical symptoms suggesting a physical condition, for which there are no demonstrable organic findings or established physiological mechanisms with positive evidence that the symptoms are related to psychological causes. The aim of this study was to highlight the epidemiological characteristics, demographic features, comorbidities, and clinical presentations of patients with SD. Materials and methods This was a retrospective study of SD patients at King Abdulaziz Medical City in Riyadh, Saudi Arabia. We reviewed the patients' electronic health records from January 2015 to December 2020 for collecting the patients' demographic information, including gender, age, marital status, and occupation. The types of SD, presenting symptoms of each disorder, department to which patients initially presented, comorbidities, and management were also documented. The diagnosis of SD was based on the International Classification of Diseases, Tenth Revision (ICD-10). Results In total, 89 patients were included in the study. The majority (n=50, 56.2%) were female, with a mean age of 42.7±17.1 years. More than half of the sample was married (n=54, 60.7%). The most common subtype of SD was somatization disorder followed by conversion disorder, pain disorder, and hypochondriasis, diagnosed in 69 (77.5%), 12 (13.5%), 5 (5.6%), and three (3.4%) patients, respectively. Neurological symptoms and pain were the most frequent presenting symptoms for all the somatoform patients. More than half of the sample (n=48, 53.9%) initially presented at an outpatient clinic. Conclusions The number of SD patients was less than expected, and a third did not receive any treatment. This emphasizes the need for more SD awareness among clinicians in various medical specialties. Appropriate SD and other mental disorders education for physicians may support achieving a better identification of SD and subsequently an improved quality of life for the patients.

Clinical improvement of encapsulating peritoneal sclerosis after challenging course and 6 months of total parenteral nutrition in child with nephronophthisis: a case report.

BACKGROUND: Encapsulating peritoneal sclerosis is a rare but potentially lethal complication of long-term peritoneal dialysis that is associated with significant morbidity and mortality. The occurrence of encapsulating peritoneal sclerosis varies worldwide, but is increased in patients maintained on peritoneal dialysis for 5-8 years. The etiology of encapsulating peritoneal sclerosis remains unidentified, and a high index of clinical suspicion is required for diagnosis. CASE PRESENTATION: We report a 5-year-old Saudi female with end-stage renal disease secondary to nephronophthisis type 2. She underwent peritoneal dialysis for 30 months, with four episodes of peritonitis. She presented with clinical signs of peritonitis. Three days later, she developed septic shock, which required pediatric intensive care unit admission. The peritoneal dialysis catheter was removed because of refractory peritonitis. Her course was complicated by small bowel perforation, and severe adhesions were revealed on abdominal ultrasound and computed tomography, consistent with a diagnosis of EPS. This finding was later confirmed by diagnostic laparotomy performed twice and complicated by recurrent abdominal wall fistula. She received total parenteral nutrition for 6 months and several courses of antibiotics. The patient received supportive treatment including nutritional optimization and treatment for infection. No other treatments, such as immunosuppression, were administered to avoid risk of infection. Following a complicated hospital course, the patient restarted oral intake after 6 months of total parenteral nutrition dependency. Her abdominal fistula resolved completely, and she was maintained on hemodialysis for few years before she received a kidney transplant. CONCLUSION: When treating patients using peritoneal dialysis, it is important to consider encapsulating peritoneal sclerosis with refractory peritonitis, which is not always easy to identify, particularly if the patient has been maintained on peritoneal dialysis for less than 3 years. Early identification of encapsulating peritoneal sclerosis and appropriate conservative treatment, including nutritional optimization and treatment of infections, are essential to achieve a better prognosis.

The immunological response among COVID-19 patients with acute respiratory distress syndrome.

Coronavirus disease 2019 (COVID-19) often results in pneumonia and can lead to acute respiratory distress syndrome (ARDS). ARDS is one of the most significant causes of death in patients with COVID-19. The development of a "cytokine storm" in patients with COVID-19 causes progression to ARDS. In this scoping review, we investigated the effect of pro-inflammatory cytokines in inducing moderate and severe ARDS outcomes. A comprehensive search was performed using PubMed and Google Scholar to implement a broad query that captured all the relevant studies published between December 2019 and September 2020.We identified seven studies that evaluated the immune response in COVID-19 patients with ARDS. The white blood cell counts (WBCs), CRP, and IL-6 were higher in the moderately presenting ARDS patients, critically ill patients, and those with more severe ARDS. This study may contribute to better patient management and outcomes if tailored immune marker interventions are implemented in the near future.

Factitious Disorder in Seven Patients: A Saudi Experience.

Background Factitious disorder (FD) is a psychiatric disease where signs and symptoms are produced, falsified, or exaggerated consciously in the absence of clear external motivations. Through needless medical visits, costly investigatory testing, and potentially long hospital stays, patients with FD waste valuable time and resources, which affects both the patient and the healthcare system. It can be very challenging for physicians who have never encountered patients with FD to recognize them promptly as symptoms of FD vary greatly. Methodology This was a retrospective study of patients diagnosed with FD attending King Abdulaziz Medical City in Riyadh, Saudi Arabia, a tertiary care military hospital and one of the most prominent academic and referral medical institutions in the country. Using the BESTCare health information system, we reviewed patients' electronic health records from January 2015 to December 2020. The diagnosis of FD was based on the International Classification of Diseases and Related Health Problems 10th edition. Results A total of seven patients were included in the study, of whom five (71%) were males and two (29%) were females. Of the total seven patients, three were 21 years old and younger, one was 38 years old, and three were 56 years old and older. Three (43%) patients were married and four (57%) were single. In terms of occupation, three (43%) patients were retired, one (14%) worked in a private company, one (14%) was unemployed, and two (29%) were students. A total of four (57%) patients initially presented to the Emergency Department (ED), and only three (43%) presented to the outpatient clinics. Induced disease or injury was found in five (71%) patients. Induced skin injury was found in four (57%) patients. Counseling and psychotherapy were only offered to one (14%) patient. Conclusions FD remains a rare psychiatric condition that is difficult to recognize. Despite a small sample size, FD in the present study showed a male predominance, affecting patients of all age groups. About half of the patients presented initially to the ED. Induced disease or injury was the most commonly reported pattern of clinical presentation. Induced skin injury was the most common clinical presentation found in around half of the patients. We believe that the number of FD patients in the present study is likely underreported and is expected to be higher. This highlights the need for better awareness of FD among physicians in different medical fields. We emphasize that there is a need for better professional training in the identification of FD.

Swimming through the sands of the Sahara and Arabian deserts: Phylogeny of sandfish skinks (Scincidae, Scincus) reveals a recent and rapid diversification.

Large parts of the Sahara Desert and Arabia are covered by sand seas and sand dunes, which are inhabited by specialized animal communities. For example, many lizards have developed adaptations to life in loose sand, including sand-swimming behavior. The best-known sand swimmers of the Saharo-Arabia are the sandfish skinks (genus Scincus). Although there are currently only four Scincus species recognized, their phylogenetic relationships have not yet been addressed in detail. We use eight genetic markers (three mitochondrial, five nuclear) and a complete sampling of species to infer the relationships within the genus. We employ multiple phylogenetic approaches to reconstruct the evolutionary history of these skinks and to assess the level of reticulation at the onset of their radiation. Our results indicate the presence of five strongly supported species-level lineages, four represented by the currently recognized species and the fifth by S. scincus conirostris, which does not form a clade with S. scincus. Based on these results we elevate the Iranian and northern Arabian S. conirostris to the species level. The two Saharan species, S. albifasciatus and S. scincus, are sister in all analyses. Deeper relationships within the genus, however, remained largely unresolved despite the extensive genetic data set. This basal polytomy, together with the fact that we detected no sign of hybridization in the history of the genus, indicates that the diversification of the five Scincus species was rapid, burst-like, and not followed by secondary hybridization events. Divergence time estimations show a Middle Pliocene crown radiation of the genus (3.3 Mya). We hypothesize that the aridification of the Saharo-Arabia that began in the Late Miocene triggered the initial diversification of Scincus, and that the subsequent expansion of sand deserts enabled their dispersal over the large Saharan and Arabian range. We discuss the evolution of body form in sand swimming lizards and ponder how Scincus retained their fully limbed morphology despite being sand swimmers that are typically limbless.

Improved Renal Recovery with Eculizumab Therapy among Children with High Prevalence of Mutation-Associated Atypical Hemolytic Uremic Syndrome: A Retrospective Cohort Study.

INTRODUCTION: Genetic defects that determine uncontrolled activation of the alternative complement pathway have been well documented, which account for approximately 40-60% of atypical hemolytic uremic syndrome (aHUS) cases worldwide. In Saudi Arabia, nearly half of the marriages are consanguineous, resulting in a high prevalence of such genetic diseases. Recent studies have demonstrated the effectiveness of eculizumab against aHUS. OBJECTIVE: We report our experience of using plasma therapy or/and eculizumab to treat children with aHUS in a tertiary care center in Saudi Arabia and to compare their clinical characteristics, genetic mutations, and treatment outcomes. METHODS: A retrospective cohort study was conducted between January 2010 and May 2017. Data, including demographic parameters, clinical presentation, hospital stay duration, need for dialysis, renal recovery, genetic mutations, and outcomes, were obtained from electronic medical records of all eligible patients. RESULTS: Overall, 21 children with aHUS were included, of which 12 (57.1%) received eculizumab therapy and 9 (42.9%) received only plasma therapy. End-stage renal disease occurred in 7 children (33.3%), of which 4 (57.1%) received only plasma therapy and 3 (42.9%) received eculizumab therapy whose genetic mutations were not related to the complement dysregulation system. No child who received eculizumab therapy showed recurrence; however, 3 children (33.3%) who received plasma therapy alone showed recurrence. Genetic mutations were detected in 12/20 (60%) of those who underwent genetic screening. CONCLUSIONS: Children who received eculizumab therapy showed good renal recovery and maintained remission compared with children who received plasma therapy alone. Genetic mutations were detected in 60% of the patients, which was associated with a high prevalence of consanguineous marriages.

Spectrum of pediatricbiopsy-proven renal diseases: A single center experience.

Glomerular diseases are considered to be a significant cause of chronic kidney disease. Kidney biopsy continues to be an essential diagnostic tool. We review the renal biopsies which were done on children below the age of 14 years in the past 10 years (from January 2008 to September 2018) in a single tertiary pediatric hospital in Saudi Arabia to determine the patterns of renal disease among Saudi children as well-correlating clinical presentation with histopathological diagnosis. A total of 203 pediatric kidney biopsies were performed. The mean age was 7.3 ± 3.9 years (3 months to 14 years). There were 105 males and 98 females. The most frequent indication for renal biopsy was nephrotic syndrome in 58.9% of patients, followed by acute glomerulo- nephritis in 20.8%. Other indications included significant proteinuria, persistent microscopic hematuria, acute kidney injury of uncertain etiology, in the remaining 20% of biopsies. Clinical diagnosis was consistent with histopathological diagnosis in 92% of the cases. Minimal change disease was the most common cause of primary glomerular diseases in 37.4%, followed by focal segmental glomerulosclerosis in 20.2%. Lupus nephritis represents the most common cause of the secondary renal disease (8.4%). Complications of kidney biopsy were observed in only 16.3% of patients, of whom 9.9% had perirenal hematomas and 6.4% of the patients developed either microscopic hematuria or macroscopic hematuria.