A Retrospective Study on Vitamin D Status and Its Association With Cardiometabolic Risk Factors Among Children With Chronic Kidney Disease at King Abdulaziz University Hospital.

BACKGROUND: Vitamin D deficiency is a significant global health issue. It is prevalent in chronic kidney disease (CKD) patients, which is an important cause of death among children. Many studies have found a link between low vitamin D status in CKD patients and cardiovascular disease (CVD) risk factors. However, there are no data on this relationship in children with CKD in Saudi Arabia. AIMS: We aimed to demonstrate this association among children with CKD admitted to the King Abdulaziz University Hospital (KAUH) in Jeddah, Saudi Arabia. MATERIALS AND METHODS: Data were collected between June and August 2020 from a convenience sample of pediatric patients. RESULTS: In total, 153 pediatric patients with CKD stages 2-5 were admitted to the KAUH between 2010 and 2019, and 67.3% had CKD stage 5. Approximately 4.6% and 10.5% of the participants were overweight or obese, respectively. Patients who fell into the lower 25-hydroxyvitamin D (25[OH]D) tertile were older, had higher body mass index (BMI) values, and had higher blood pressure than those in the upper two tertiles; however, these differences were not statistically significant. There was a significant inverse association of 25(OH)D levels with BMI, blood pressure, and serum creatinine levels. CONCLUSIONS: The results of this retrospective study suggest that patients with CKD and lower vitamin D levels have a higher BMI and blood pressure and are therefore at higher risk of developing CVD. Future prospective studies with a larger sample size are needed to confirm these findings. Randomized clinical trials are also needed to investigate the effect of sufficient vitamin D status on reducing CVD in patients with CKD.

Necrotizing Fasciitis: A Side Effect of Rituximab Administration in Steroid-Dependent Nephrotic Syndrome.

Steroid-dependent nephrotic syndrome (SDNS) is a common type of childhood nephrotic syndrome. Remission following steroid therapy is achieved in 80-90% of the patients, while the remainder show steroid-resistant nephrotic syndrome (SRNS). Rituximab is an anti-CD20 chimeric monoclonal antibody with proven therapeutic effects in several diseases and has been used with great success in the treatment of NS since its discovery. We report a case of a 4-year-old girl diagnosed with SDNS at the age of 3. As treatment with steroids, enalapril, and mycophenolate failed to produce complete remission, rituximab was initiated, and remission was successfully achieved after administration of the first dose. Due to this response, rituximab therapy was continued; however, a day after being admitted to the nephrology ward for the second dose, she started to develop a high fever, which reached up to 40°C. In addition, she also displayed symptoms of upper respiratory tract infection and an ulcerated wound on her left cheek. The patient became drowsy with reactive pupils, cold peripheries, and weak peripheral pulses. The capillary refill time was prolonged to 3-4 seconds and it was decided to withhold the second dose of rituximab. The patient was shifted to the PICU as a case of septic shock secondary to facial cellulitis and started on inotropes (epinephrine and norepinephrine), meropenem, vancomycin, and hydrocortisone 15 mg. Thereafter, surgical debridement of the wound was carried out. The patient remained in remission with regard to nephrotic syndrome and was discharged in a healthy condition. In conclusion, rituximab used in conjunction with steroids and other immunosuppressants may increase the risk of serious infections like necrotizing fasciitis (NF). Further studies are needed to explore the relationship between rituximab and NF.

Renal stone prevalence and risk factors in Jeddah and Riyadh.

Introduction: Nephrolithiasis is a common problem worldwide, especially in hot areas like Saudi Arabia. This retrospective study investigated the current prevalence and risk factors of renal stones in Jeddah and Riyadh, Saudi Arabia. Methods: A non-interventional cross-sectional study was conducted from November 2018 to June 2019 at King Abdulaziz University Hospital, Jeddah, Saudi Arabia. This study included 1031 participants (age ≥18 years) from Jeddah (n = 652, 63.30%) and Riyadh (n = 379, 36.80%). Of them, 169 (16.40%) had renal stones while 862 (83.60%) had no renal stones. All participants filled out a questionnaire distributed via social media, which contained questions regarding eating and drinking habits. Results: Significant differences were found between the groups regarding frequencies of intake of black tea and orange/lemon juice. The number of patients who consumed cow meat was significantly higher among those without renal stones than among those with renal stones. Significant high risk for renal stones was found in those who consumed energy drink >1 can/day, black tea >1 cup/day, and orange/lemon juice >1 glass/day. Conclusion: This study revealed that some eating habits play a major role in the development of urinary stones in the Saudi population. Therefore, a dietary intervention on a large scale and health, education in this regard may be helpful in preventing the 25 related to renal stones.

Knowledge and attitude of children safety at home among population in Saudi Arabia.

Background: Unintentional injury to children at home is a public health problem and it is a significant cause of death and disabilities. It accounts for 40% of all child deaths. To our knowledge, there have been no similar studies done on Arabic countries that show the prevalence of home injuries among children. Aim: We aimed to assess the adults' knowledge and attitude toward child safety at home in Saudi Arabia. Methods: A cross-sectional study was conducted among 1,301 participants, during August 2017, who are older than 18 years old and have at least one child in the family. Data were collected by an online questionnaire assessing the believes, knowledge, and behavior of the participants. Analysis was carried out using IBM SPSS Software version 21, using descriptive analysis, t-test, and one-way ANOVA. Results: It was found that gender, age, marital status, employment status, and the type of accommodation played key roles in the awareness of children safety at home, the study showed that females and age group above 25, married, employees, and those who lived in flats had more awareness than the other groups, respectively. Conclusion: The majority of the population had good awareness about children's safety at home. Despite the good level of awareness, more public health education is recommended to improve the awareness and the prevention methods of these injuries.

Postoperative Intracardiac Thrombus in a Child with Nephrotic Syndrome.

Background: Thromboembolic events are a known complication of nephrotic syndrome in children. Intracardiac thrombi are a rare location for thrombus formation but can lead to severe clinical consequences. Case Presentation: We report an intracardiac left atrial thrombus in a child with new onset steroid-resistant nephrotic syndrome and recently repaired atrial septal defect and cor triatriatum. The thrombus was successfully treated with surgical excision. Conclusion: Intracardiac thrombus is a rare but serious complication of nephrotic syndrome in children, especially in children with risk factors for potentially increased intracardiac thrombogenicity such as history of recent intervention.

Sickle cell nephropathy: A review of novel biomarkers and their potential roles in early detection of renal involvement.

Whether the underlying mutations are homozygous, heterozygous, or co-inherited with other hemoglobinopathies, sickle cell disease is known to afflict the kidneys, leading to the clinical entity known as sickle cell nephropathy (SCN). Although common, SCN remains diagnostically elusive. Conventional studies performed in the context of renal disorders often fail to detect early stage SCN. This makes the quest for early diagnosis and treatment more challenging, and it increases the burden of chronic kidney disease-related morbidity among patients. Novel diagnostic tools have been employed to overcome this limitation. In this study, we discuss various biomarkers of SCN, including those employed in clinical practice and others recently identified in experimental settings, such as markers of vascular injury, endothelial dysfunction, tubulo-glomerular damage, and oxidative stress. These include kidney injury molecule-1, monocyte chemoattractant protein-1, N-acetyl-B-D-glucosaminidase, ceruloplasmin, orosomucoid, nephrin, and cation channels, among others. Furthermore, we explore the potential of novel biomarkers for refining diagnostic and therapeutic approaches and describe some obstacles that still need to be overcome. We highlight the importance of a collaborative approach to standardize the use of promising new biomarkers. Finally, we outline the limitations of conventional markers of renal damage as extensions of the pathogenic process occurring at the level of the organ and its functional subunits, with a discussion of the expected pattern of clinical and biochemical progression among patients with SCN.

The Association between Adenotonsillectomy and Frequency of Vaso-Occlusive Crises in Patients with Sickle Cell Disease.

Sickle cell disease (SCD) typically manifests in early childhood as attacks of pain known as vaso-occlusive crises. Infection and hypoxemia have been linked with these recurrent episodes and with prolonged hospitalization in SCD patients. However, adenoids and tonsils as sources of infection and causes of hypoxemia have not been adequately investigated in association with vaso-occlusive crises in SCD. To assess the association between adenotonsillectomy and frequency of vaso-occlusive crisis in SCD patients who underwent this procedure at our Hospital, and between adenotonsillectomy and frequency of blood transfusions and emergency department and intensive care unit admissions. We used medical record data to conduct a retrospective review of SCD patients who underwent adenoidectomy and/or tonsillectomy between 2005 and 2017. Eligible subjects were assessed for frequency of vaso-occlusive crises, blood transfusions, and emergency department and intensive care unit admissions. Using the Wilcoxon signed rank test, we compared the frequencies of each outcome preoperatively and 1, 3, 5, and 10 years postoperatively. Of 524 records reviewed, 40 eligible patients were included in the study. Minimal reduction was observed in the frequency of vaso-occlusive crisis episodes within 1 and 3 years after adenotonsillectomy (p = 0.337 and p = 0.549, respectively). Although the 5- and 10-year postoperative vaso-occlusive crisis frequency tended to be higher than that in the preoperative period, none of the results reached statistical significance. The number of emergency department admissions showed a statistically significant increase 3 years postoperatively compared with that in the preoperative period (P = 0.043). There were no statistically significant differences in perioperative blood transfusion frequency or number of intensive care unit admissions in any period. Adenotonsillectomy in SCD patients does not seem to be related to the frequency of vaso-occlusive crises, blood transfusions, or emergency department or intensive care unit admissions. Prospective studies with larger sample sizes are recommended to further evaluate these findings.

Nutritional Assessment and Management in Paediatric Chronic Kidney Disease.

Nutrition in paediatrics has always been one of the most important factors for optimal growth. Children with chronic kidney disease (CKD) need special consideration for better long-term outcomes, including nutritional status, optimal height, and cognitive function. Nonetheless, there are many obstacles to overcome to attain optimal linear growth and nutritional status in children with CKD. This review highlights the need for tools to assess the growth parameters in CKD. In addition, recommendations for dietary intake play a major role in controlling electrolyte disturbances in patients with CKD. For example, it is still unclear whether it is better to restrict phosphate sources in inorganic, organic, or food additives. The review also summarises different factors such as fluid intake, route of feeding, and essential nutrients that require particular attention in paediatric patients with CKD. In summary, a multidisciplinary team is needed to devise individual nutritional plans to achieve the best outcome and improve the quality of life of patients.

Renal Stones and Risk Factors in Jeddah and Riyadh.

Saudi Arabia has extremely hot climate for most of the year; this is associated with the risk of developing nephrolithiasis. This retrospective research aimed to investigate the current prevalence, manifestation, mode of treatment, and risk factors of renal stones in Jeddah and Riyadh, Saudi Arabia. A cross-sectional study was conducted from November 2018 to June 2019 at King Abdulaziz University Hospital, Jeddah, Saudi Arabia. This study included 1031 participants aged ≥18 years from Jeddah (n=652, 63.3%) and Riyadh (n=379, 36.8%). Data were expressed as mean ± standard deviation (minimum-maximum) or number (%). Comparisons between patients with and without renal stones were made using the Chi-square test and unpaired Student's t-test as appropriate. The odds ratio (OR) with a 95% confidence interval (95% CI) was determined for the risk factors of renal stones. The prevalence of renal stones was 16.9% among the participants. A significantly high risk for renal stones was associated with male sex (2.96; 95% CI: 2.08-4.20, P = 0.0001) and age group of 34-40 years (OR: 1.44; 95% CI: 1.005-2.103, P = 0.047). Hypertension was more common in patients with renal stones than those without renal stones (9.5% vs.4.6%, P = 0.013). The percentage of patients who took diuretics was significantly higher among those with renal stones than among those without renal stones (11.2% vs. 3.5%, P = 0.001). Of the 169 patients with renal stones, 58.0% had a positive family history of renal stones, and 23.7% had a history of urinary tract infections (UTIs). The symptoms of renal stones were pain (86.4%), hematuria (11.2%), fever (2.4%), and others (0.6%). Of all the patients, 43.8% took medication. For most patients, the stones passed spontaneously (67.5%), while 23.7% underwent lithotripsy, and 1.5% received stents. In this study, we found a higher prevalence of renal stones two major cities in Saudi Arabia in Jeddah and Riyadh, at 16.4%. Risk factors included male sex and age group of 34-40 years. A significant number of patients with renal stones have a family history of renal stones and a history of UTI. Loin pain and hematuria were the two major clinical presenting symptoms for renal stones. In the majority of patients, the renal stones passed spontaneously without the need for lithotripsy or surgery.

The prevalence of renal stones among local residents in Saudi Arabia.

BACKGROUND: Urolithiasis is the most common urological problem worldwide. It is a recurrent multifactorial problem that is caused by the interaction of several environmental and genetic factors. This study aimed to assess the prevalence of renal stones among local residents in Saudi Arabia in order to renew the statistics of renal stones occurrence in the current Saudi population. METHODS: A cross-sectional study was conducted using an electronic questionnairethat was distributed randomly through phones and social media to reach the local residents in Saudi Arabia. We then reviewed the published papers in Saudi journals for patients with renal stones. RESULTS: From a total of 580 responders to the electronic questionnaire, the prevalence of renal stones was 9.1% (n = 64). The median age at diagnosis was 29 years and the mean age at diagnosis was 36.91 years (SD = 18.66, Range of 20-99). Two peaks of age were observed, the first peak was at the (21-25) age group representing 34.4% of the kidney stones patients. The second peak was older than 47 years. The majority of those diagnosed with kidney stones had normal BMI (n = 29, 45.3%), and the family history of kidney stones among first degree relatives was found in 35.9% of the cases (n = 23). CONCLUSION: Kidney stones is a common health problem with the local incidence being underreported. In our sample, the prevalence was 9.1%. We also observed a relatively high percentage of positive family history among renal stone patients (34.9%) that could be attributed to the high rates of consanguinity. We encourage more local epidemiological studies to describe the patterns and the contributing factors of the development of kidney stones.

Coronavirus disease in children: A multicentre study from the Kingdom of Saudi Arabia.

BACKGROUND: The COVID-19 global pandemic caused by severe acute respiratory syndrome coronavirus 2 infection, warranted attention for whether it has unique manifestations in children. Children tend to develop less severe disease with a small percentage present with clinical manifestations of paediatric multisystem inflammatory syndrome and have poor prognosis. We studied the characteristics of COVID-19 in children requiring hospitalisation in the Kingdom of Saudi Arabia and assessed the clinical presentation and the risk factors for mortality, morbidity, and paediatric intensive care (PICU) admission. METHODS: We conducted a retrospective analysis of COVID-19 patients under 15 years hospitalised at three tertiary academic hospitals between 1 March and 30 June 2020. RESULTS: Eighty-eight children were enrolled (>20% were infants). Seven (8%) were in critical condition and required PICU admission, and 4 (4.5%) died of which 3 met the full diagnostic criteria of multi-system inflammatory syndrome and had a high Paediatric Risk of Mortality (PRISM) score at the time of admission. The initial polymerase chain reaction (PCR) test result was positive for COVID-19 in most patients (97.7%), and the remaining two patients had positive result in the repeated confirmatory test. In a subset of patients (20 subjects), repeated PCR testing was performed until conversion to negative result, and the average duration for conversion was 8 (95% CI: 5.2-10.5) days Children requiring PICU admission presented with signs of respiratory distress, dehydration, and heart failure. Most had fever (71.4%) and tonsillitis; 61.4% were discharged within 7 days of hospitalisation. Risk factors for mortality included skin rash, hypotension, hypoxia, signs of heart failure, chest radiograph suggestive of acute respiratory distress syndrome, anaemia, leucocytosis, hypernatraemia, abnormal liver enzymes, and high troponin I, and risk factors for prolonged hospitalisation (>7 days) included the presence of comorbidities, leucopaenia, hyponatraemia, and elevated C-reactive protein. CONCLUSIONS: The majority of hospitalised children had a brief febrile illness and made a full recovery, but a minority had severe disease.

Incidence and Outcome of Community-Acquired Acute Kidney Injury in Pediatric Patients Seen at an Emergency Department: A Retrospective Cohort Study.

OBJECTIVES: Acute kidney injury (AKI) has significant morbidity and mortality rates among young patients. This study aimed to determine the incidence and outcome of community-acquired AKI among pediatric patients seen in the emergency department of King Abdulaziz University Hospital for more than 3 years. METHODS: This retrospective study reviewed electronic medical records for all pediatric patients aged 1 month to 18 years who visited the emergency department of King Abdulaziz University Hospital from January 1, 2015, until December 31, 2017. Acute kidney injury was diagnosed and classified according to the Kidney Disease: Improving Global Outcomes criteria. RESULTS: Of 6038 patients, 1581 were included. Acute kidney injury occurred in 135 patients (8.5%), of which 77 (57%) were in stage 1, 42 (31.1%) were in stage 2, and 16 (11.9%) were in stage 3. Mortality was higher in the AKI group (4.4%) than in the non-AKI group (0.2%; P < 0.01). On long-term observation, 14.8% did not return for follow-up, 58.5% of survivors recovered completely, and 22.2% progressed to chronic kidney disease. The most affected age group was 1 month to 2 years (26%). Common admission causes were chemotherapy-induced AKI (31.9%) and pneumonia (10.4%). There was a significant inverse relationship between AKI and age group (P < 0.001) and a positive association between AKI and death (P < 0.001). However, no association was found between AKI stages and outcomes. CONCLUSIONS: Community-acquired AKI remains a common condition affecting the pediatric population. It is associated with a higher mortality rate. Infants were more susceptible to AKI, and a significant number of patients with AKI progressed to chronic kidney disease.

Hypertension in pediatric patients admitted to inpatient ward at King Abdulaziz Universty Hospital in Saudi Arabia: Prevalence, causes, and outcomes.

BACKGROUND: The secondary hypertension (HTN) is the predominant form of HTN in pediatrics. Renal diseases and renovascular anomalies are the most commonly reported causes. In this study, we aimed to identify the prevalence, causes, and outcomes of secondary HTN in Saudi Arabia. METHODS: A retrospective study was conducted among 3,640 pediatric patients aged between 0 and 18 years, admitted to the pediatric nephrology ward at King Abdulaziz University Hospital, Jeddah, Saudi Arabia. The study has been approved by the ethics review committee of King Abdulaziz University. RESULTS: Prevalence of secondary HTN due to renal disease was (77.0%). Most of the cases were diagnosed with stage 5 renal disease (78.3%). Small kidney size was frequently diagnosed (n = 29, 11.9%), followed by large kidney size (n = 26, 10.7%). One third of the cases (n = 79, 32.4%) were under control, 49 (20.1%) lost follow-up, and 24 (10.1%) deceased. A total of 61 (33.1%) patients progressed to end-stage renal disease and patientswere managed by different types of treatments. CONCLUSION: The prevalence of secondary HTN due to renal disease is considered to be high in pediatric patients admitted to King Abdulaziz University. Several renal diseases in the renal system are associated with secondary HTN mostly attriubuted to renal malformation. In addition, renal affection, cerebral infarction, bleeding, left ventricular hypertrophy, and valvular lesion are the highest reported complications in our population. Follow-up with ECHO and brain CT is highly recommended in pediatric HTN. Future studies on a larger sample and vigorous follow-up are recommended.

Bilateral Pulmonary Embolism in a 12-Year-Old Girl with Steroid-Resistant Nephrotic Syndrome.

Nephrotic syndrome is the most common glomerular disease among children. Although most cases respond to steroid therapy, approximately 10-20% of patients exhibit resistance to conventional steroid therapy and are labeled as steroid-resistant. Such patients are at risk of complications, including infection, thrombosis, and chronic kidney disease. Nephrotic syndrome is considered a thrombogenic condition. Pulmonary embolism is associated with high mortality, and early treatment is essential for the survival of patients. Here, we report the case of a 12-year-old girl with late steroid resistance who developed bilateral pulmonary embolism.

Assessment of the Etiologies and Outcomes of Antenatal Hydronephrosis in Patients at King Abdulaziz University Hospital.

Background Antenatal hydronephrosis (ANH) is the most common congenital abnormality. It is often detected during pregnancy through an antenatal ultrasound (US) examination. This condition is defined as the dilatation of the renal pelvis and/or calyces in neonates. Little is known about ANH and its associated etiology and outcomes, especially in the Middle East. This study aims to determine the outcome of patients diagnosed with ANH between 2011 and 2017. Methods The current study was a retrospective analysis of data collected from the medical records of 64 ANH patients (45 male, 19 female). We collected data regarding the demographic characteristics, fetal parameters, laboratory and radiological parameters, and medical and surgical interventions. Moreover, based on radiological reports patients were classified into one of the following two groups: good prognosis, including patients with improved or resolved ANH and poor prognosis, including patients with progressing ANH and associated complications such as parenchymal scarring, chronic kidney disease complicated by secondary hypertension. Results Overall, 48.4% of patients exhibited good prognosis, whereas 51.6% exhibited poor prognosis. Among the 64 patients, 53.1% of patients exhibited unilateral ANH, and 46.9% exhibited bilateral ANH. Also, unilateral ANH and bilateral ANH had good prognoses in 41.2% and 56.7%, respectively, while unilateral and bilateral ANH had poor prognosis in 58.8% and 43.3%, respectively. Conclusions Additional prophylactic measures should be taken to avoid complications, such as urinary tract infection (UTI), as our study found that patients who have ANH are more prone to develop UTI. Patients with several ANH comorbidities are associated with poor prognosis.

Correction to: Short-term outcome associated with disease severity and electrolyte abnormalities among critically ill children with acute kidney injury.

Following publication of the original article [1].

Rituximab versus cyclophosphamide as first steroid-sparing agent in childhood frequently relapsing and steroid-dependent nephrotic syndrome.

BACKGROUND: Approximately 50% of children with steroid-sensitive nephrotic syndrome (SSNS) will suffer from frequent relapses or steroid dependency, prompting the use of so-called steroid-sparing drugs. In this pilot study, we compare the efficacy and safety of rituximab to oral cyclophosphamide as first-line steroid-sparing medications. METHODS: A prospective open-label non-randomized study of children with frequent relapsing or steroid-dependant SSNS. Exclusion criteria were steroid-resistant disease, prescription of immunosuppressive agents other than prednisolone or levamisole, evidence of impaired kidney function, leucopenia, or active infection. The recruited children were allocated either to the oral cyclophosphamide (3 mg/kg/day for 8 weeks) or intravenous rituximab treatment (two doses of 375 mg/m2/dose, 2 weeks apart) and were monitored for relapses and side effects for 12 months. RESULTS: Forty-six subjects were included from two centers; 27 received cyclophosphamide and 19 received rituximab. One-year relapse-free survival was reached in 17 (58.6%) patients treated with cyclophosphamide compared to 16 (84.2%) with rituximab (adjusted HR 0.36; 95% CI 0.09-1.45; p = 0.151). The mean interval to relapse was 6.9 months in the cyclophosphamide group (N = 10) and 6.3 months in the rituximab group (N = 3). Both treatments were associated with a significant (p < 0.001) reduction in prescribed dose of oral alternate-day steroid from 1.02 to 0.36 mg/kg (cyclophosphamide) and 0.86 to 0.08 mg/kg (rituximab). Importantly, a significantly (p = 0.003) higher percentage of patients achieved complete withdrawal of steroid within 3 months of commencing study treatment in the rituximab (73.7%) versus cyclophosphamide (29.6%) group. Transient leucopenia was the most frequent adverse effect observed in the cyclophosphamide group (18.5%) and one patient (3.4%) had acute hepatotoxicity besides severe leucopenia and neutropenia in the 7th week of treatment with complete recovery with the withdrawal of cyclophosphamide and maintenance of remission. A minor infusion-related reaction in the form of a generalized macular skin rash was observed in one patient (5%) in the rituximab group. CONCLUSIONS: Rituximab is non-inferior to cyclophosphamide and safe as a first-line steroid-sparing agent in children with SSNS. A larger multicenter study is required to assess superiority over cyclophosphamide. Graphical abstract.

Acute Kidney Injury in Pediatric Patients Treated with Vancomycin and Piperacillin-Tazobactam Versus Vancomycin and Cefotaxime: A Single-center Study.

Background Previous literature showed a higher incidence of acute kidney injury (AKI) in pediatric patients using vancomycin + piperacillin-tazobactam compared to cefepime + vancomycin. Our aim was to compare the incidence of developing AKI during the use of vancomycin + cefotaxime with that during the use of vancomycin + piperacillin-tazobactam in pediatric patients. Methods This was a retrospective, matched cohort study that used electronic records from May 1, 2015 through April 30, 2018 for all patients aged less than 16 years who received intravenous (IV) vancomycin + piperacillin-tazobactam or cefotaxime + vancomycin for at least 72 hours. AKI was defined by Kidney Disease Improving Global Outcomes (KDIGO) guidelines. Each patient from the vancomycin + piperacillin-tazobactam group was matched 1:1 with those in the vancomycin + cefotaxime group according to their age, chronic disease, gender, and the number of concomitant nephrotoxic agents. A total of 64 cases were included. Statistical analysis was performed using descriptive statistics and binary logistic regression. Results AKI developed in 10 of 32 patients (31.25%) who were using vancomycin + piperacillin-tazobactam. On the other hand, 13 of 32 patients (40.62%) were using cefotaxime + vancomycin (p = 0.047). Of the 10 patients who were on vancomycin + piperacillin-tazobactam regimen, 80% developed AKI Stage I. Of the 13 patients who were using cefotaxime + vancomycin, 46% developed AKI Stage II, although no statistical significance was noted in all stages.  Conclusion Our study showed that patients treated with cefotaxime and vancomycin showed a higher incidence of AKI than patients treated with vancomycin and piperacillin-tazobactam, although the study showed no statistical significance.

Prevalence and Risk Factors for Microalbuminuria in Children with Sickle Cell Disease at King Abdulaziz University Hospital: A Retrospective Cross-sectional Study.

OBJECTIVES: Previous studies have not addressed microalbuminuria in pediatric patients with sickle cell disease (SCD) in Jeddah, Saudi Arabia. This study aimed to determine the prevalence of microalbuminuria and to identify associated risk factors in children with SCD at King Abdulaziz University Hospital. RESULTS: Overall, 42.5% of the patients enrolled were Saudi Arabian and 51% were male. The mean age was 12.4 years, and the highest percentage (40%) was in the age group of 15-18 years. The prevalence of microalbuminuria was 9.6%, and hematuria was present in 8% of cases. The percentage of patients with hematuria was significantly higher in the microalbuminuria group (22.6%) than in the nonmicroalbuminuria group (6.5%; P = 0.007). The percentage of patients with acute chest syndrome was also higher in the microalbuminuria group (26%) than in the nonmicroalbuminuria group (8%; P = 0.005). The percentage of patients with gallbladder stones was higher in the microalbuminuria group (13%) than in the nonmicroalbuminuria group (2.4%; P = 0.014). However, the mean number of blood transfusions was higher in the nonmicroalbuminuria group than in the microalbuminuria group (P = 0.002). Sickle cell nephropathy manifests as microalbuminuria, begins at an early age, occurs in all types of SCD, and is associated with disease severity.

Exome sequencing and metabolomic analysis of a chronic kidney disease and hearing loss patient family revealed RMND1 mutation induced sphingolipid metabolism defects.

Mitochondrial disorders (MIDs) shows overlapping clinical presentations owing to the genetic and metabolic defects of mitochondria. However, specific relationship between inherited mutations in nuclear encoded mitochondrial proteins and their functional impacts in terms of metabolic defects in patients is not yet well explored. Therefore, using high throughput whole exome sequencing (WES), we screened a chronic kidney disease (CKD) and sensorineural hearing loss (SNHL) patient, and her family members to ascertain the mode of inheritance of the mutation, and healthy population controls to establish its rare frequency. The impact of mutation on biophysical characteristics of the protein was further studied by mapping it in 3D structure. Furthermore, LC-MS tandem mass spectrophotometry based untargeted metabolomic profiling was done to study the fluctuations in plasma metabolites relevant to disease causative mutations and kidney damage. We identified a very rare homozygous c.631G > A (p.Val211Met) pathogenic mutation in RMND1 gene in the proband, which is inherited in an autosomal recessive fashion. This gene is involved in the mitochondrial translational pathways and contribute in mitochondrial energy metabolism. The p.Val211Met mutation is found to disturb the structural orientation (RMSD is -2.95 Å) and stability (ΔΔG is -0.552 Kcal/mol) of the RMND1 protein. Plasma metabolomics analysis revealed the aberrant accumulation of metabolites connected to lipid and amino acid metabolism pathways. Of these metabolites, pathway networking has discovered ceramide, a metabolite of sphingolipids, which plays a role in different signaling cascades including mitochondrial membrane biosynthesis, is highly elevated in this patient. This study suggests that genetic defects in RMND1 gene alters the mitochondrial energy metabolism leading to the accumulation of ceramide, and subsequently promote dysregulated apoptosis and tissue necrosis in kidneys.