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.

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.

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.

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.

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.

Attitude and levels of awareness toward organ donation and transplantation among healthcare providers: A cross-sectional study.

End-stage organ damage is the major cause of death worldwide. The number of donors is low, and one of the challenging phases in organ donation is the availability of organ donors. There are many studies that suggest a strong correlation between knowledge and beliefs toward organ donation. A study conducted among Health-Care Providers in the Intensive Care Units at a Tertiary Center at Riyadh reported that only 57% of the health-care providers in the Intensive Care Unit were willing to donate their organs. The objective of our study was to evaluate the knowledge, attitude, and awareness of organ donation and transplantation among health-care providers at different hospitals around the Kingdom of Saudi Arabia. The data of this cross-sectional descriptive study were collected between February and July 2018 in different hospitals all around the Kingdom of Saudi Arabia. The investigators formulated a questionnaire based on several published studies. Ethical approval was obtained from the Unit of Biomedical Ethics, Research Committee at King Abdul Aziz University. Of the 241 participants, 130 (53.9%) were female. In addition, 110 (45.6%) of them were medical residents. Moreover, 224 (92.9%) participants were aware of the concept of organ donation. The overall level of knowledge is good (55.2%).The study showed 62.2% have participated in the organ donation program for their close relatives only. In conclusion, the level of perception and knowledge about organ donation among health-care providers was inadequate, although they showed positive attitudes toward this issue.

Comparison of quality of life in children undergoing peritoneal dialysis versus hemodialysis.

OBJECTIVES: To record pediatric end stage renal disease (ESRD) patients' quality of life (QOL) in relation to peritoneal dialysis (PD) and hemodialysis (HD). Chronic kidney disease is a rising global epidemic yielding worldwide prevalence of 11-13%. It could possibly lead to ESRD, thus imposing serious burdens on patients and reducing their QOL. These burdens may affect their family members as well. Methods: This cross-sectional study examined 23 pediatric ESRD patients aged 2-18 years who were undergoing peritoneal dialysis and hemodialysis at King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia, in July 2018. Data were collected using the validated Pediatric Quality of Life InventoryTM 3.0 ESRD Module questionnaire. Results: The sample included HD (n=9, 40.9%) and PD (n=14, 60.9%) patients. According to the parent-proxy report, we found that the QOL among PD pediatric patients was significantly higher than HD patients (p=0.045). Also, male HD patients had a significantly better QOL on the interaction subscale (70.83±15.95 compared to 30.00±24.00 for females [p=0.023]). Conclusion:   Quality of life was found to be better among PD pediatric patients in King Abdulaziz University Hospital, Kingdom of Saudi Arabia.

Awareness of Vitamin D Deficiency among the General Population in Jeddah, Saudi Arabia.

BACKGROUND: During the last decade, vitamin D status was a major concern in the health and biomedical fields. This study assessed the level of awareness and knowledge regarding vitamin D and investigated the factors associated with the level of awareness such as knowing general facts about vitamin D, sources, benefit, and consequences of its deficiency. METHODS: This cross-sectional study was conducted among the general population in Jeddah, Saudi Arabia, above the age of 18 years who were in 3 malls during the period of August and September 2017. A self-administrated questionnaire in paper forms was utilized and was divided into two parts to collect data. It was designed by the authors after revising the previous studies and validated by three pediatric consultants. An ethical approval was obtained from the ethical committee in King Abdulaziz University. We did an initial sample and tested it with the Cronbach test. The questionnaire had 2 parts with 19 questions. The first part was demographic information, and the second part was general information about vitamin D. An ethical approval was obtained from the ethical committee in King Abdulaziz University. Each participant received explanations about the aim of the study, and a verbal consent was taken from participants. The scoring system was as follows: for each right answer, 1 score was given and for wrong answer, 0 score was given, and the overall score was 14. The collected data were statistically analyzed using descriptive statistics using IBM SPSS statistics for windows version 20.0 (SPSS Inc., Chicago, IL). RESULT: Out of 1022 participants, 472 (46.1%) were aged 18-28 years, 830 (82.1%) were of Saudi ethnicity, 702 (68.7%) had a university degree, 275 (26.9%) attended high school, more than half were married (55.6%), and 54.4% had children. The majority of the participants agreed that vitamin D is important in the maintenance of bone and tooth health (88.4%). It is important in the maintenance of calcium and phosphates (76.6%), and it strengthens immunity (69.4%). Of the total participants, 86.2% were aware that sunlight exposure encourages vitamin D production in the skin. The overall knowledge mean score was 5.9 ± 1.2 (39.3%). CONCLUSION: This study highlighted a high level of inadequate knowledge of vitamin D deficiency among participants. There was a significant association between knowledge level and education level. The awareness of vitamin D deficiency is high regarding its benefits. The study revealed that participants who did not have children had the highest score for benefits. Nongovernment organizations and social workers may work together with government health-care organization to teach parents and children about the uses and benefits of vitamin D.

Correlation between the estimated GFR and SWRD score in patients with posterior urethral valves at King Abdul-Aziz University Hospital.

OBJECTIVE: The aim was to establish the reliability of the SWRD score as a predictor of both renal and bladder outcomes in posterior urethral valves. This retrospective study included 67 patients with PUVs at King Abdul-Aziz University Hospital. The score was calculated from voiding cystourethrogram before and after the relief of obstruction, and estimated glomerular filtration rates (eGFRs) were calculated as well. RESULTS: Based on Spearman correlations, both baseline eGFRs and SWRD scores can be possible predictors of long-term renal outcomes, as a significant positive correlation between the baseline eGFRs and the last eGFRs was found (p = 0.005). A significant negative correlation was also found between the SWRD score calculated before the intervention and the last eGFRs (p = 0.02). Additionally, the baseline SWRD scores can be possible predictors of short-term bladder outcomes, as the correlation analysis showed a positive relationship between the baseline SWRD scores and the SWRD scores calculated within 2 months after the intervention (p < 0.0001). A significant decrease in SWRD scores and eGFRs was found from before to after the intervention, regardless of the type of intervention. In conclusion, the SWRD scoring system proved to be a potentially promising tool in the anticipation of the clinical outcomes of PUVs.

Nephrotic syndrome co-existing with type 1 diabetes in a 12-year-old boy: Case report and literature review.

Occurrence of early nephrotic syndrome in type 1 diabetes mellitus patients is extremely rare. Herein, we report the case of a 12-year-old boy who presented to our pediatric nephrology clinic with generalized edema. He had been diagnosed with type 1 diabetes mellitus at age 9 and had been treated with regular insulin. Examinations revealed normal kidney function, hypoalbuminemia, proteinuria (4+), hyperlipidemia, and low protein-to-creatinine ratio. The patient was diagnosed with idiopathic nephrotic syndrome and was empirically administered prednisolone for 12 weeks. Subsequently, prednisolone was tapered over 10-12 weeks. The patient showed good response to treatment. In conclusion, co-existence of nephrotic syndrome and type 1 diabetes mellitus may suggest an immunological basis; therefore, further studies are needed to investigate the relationship between these two conditions.

Genetics of congenital and infantile nephrotic syndrome.

BACKGROUND: Congenital and infantile nephrotic syndrome (CNS and INS) are rare inherited defects in glomerular filtration involving a variety of gene mutations. This study aimed to analyze all genetic mutations associated with congenital and infantile nephrotic syndrome treated at our institution. We also discussed our different approach secondary to culture and resources. METHODS: A retrospective single-center study of all children diagnosed as NS before the age of 1 year over a duration of over one decade. RESULTS: Twenty-nine children (12 boys) were included in the study. Their median age (range) was 2.4 (0.1-12) months (20 CNS and 9 INS). Consanguinity was present in 90% of children. The genetic analysis' results were only available for 20 children. An underlying causative homozygous mutation was detected in 18 children (90%): NPHS1 (9), NPHS2(2), LAMB2(3), PLCE1(1), WT1(1), and ITSN1 novel mutation (2). One child had heterozygous mutation of NPHS2 and another child had heterozygous mutation of NPHS1 which could not explain the disease. All CNS cases were all managed with intermittent intravenous albumin infusion, ACEi, diuretics, and indomethacin. None of the children were managed by nephrectomy followed by peritoneal dialysis (PD) because of limited resources. Only one child achieved partial remission, while 15 children died at a median (range) age of 5.8 (1.25-29) months. The remaining 14 children were followed up for an average of 36 (3.9-120) months. Three children progressed to end-stage kidney disease and PD was performed in only two children. CONCLUSIONS: NPHS1 is the main underlying cause of CNS and INS in our study population. CNS and INS were associated with high morbidity and mortality.

A Rare Benign Tumor in a 14-Year-Old Girl.

BACKGROUND: Oncocytomas are the second most common benign renal neoplasm but, unfortunately, they are difficult to differentiate from renal cell carcinoma. Renal oncocytomas are rare and have mostly been reported in adults. To our knowledge, this is only the sixth pediatric reported case of renal oncocytoma worldwide. CASE PRESENTATION: A 14-year-old Yemeni girl with a recurrent history of urinary tract infections came to our clinic complaining of left flank pain with a frontal headache. Ultrasound showed a 3 cm, well-defined echogenic lesion with mild vascularity. This lesion increased in size on her subsequent follow-ups. Computed tomography showed no intralesional fat, vessels invasion, or enlarged lymph nodes. The patient underwent laparoscopic radical nephrectomy, and a pathology report confirmed the diagnosis of renal oncocytoma. CONCLUSION AND RECOMMENDATIONS: We present the rare occurrence of renal oncocytoma in a pediatric patient and highlight the importance of considering oncocytomas in the diagnosis of a renal mass.

A case of patient with renal lupus with an initial presentation of hemolytic uremic syndrome triggered by streptococcal infection.

Systemic lupus erythematosus (SLE) is a systemic disease that is presented in a myriad of ways. Renal involvement is common in SLE and usually presents clinically as glomerulonephritis. We describe patients with SLE presented initially with hemolytic uremic syndrome which is a distinctive initial presentation.

Assessment of the etiologies and renal outcomes of rapidly progressive glomerulonephritis in pediatric patients at King Abdulaziz University Hospital, Jeddah, Saudi Arabia.

OBJECTIVES: To investigate the etiologies and outcomes of rapidly progressive glomerulonephritis (RPGN) in pediatric patients at King Abdulaziz University Hospital (KAUH) in Jeddah, Saudi Arabia. METHODS: A retrospective study was conducted in 19 pediatric patients who were diagnosed with RPGN between 2006 and 2016 at the Department of Pediatric Medicine at KAUH. Associations between variables were evaluated using independent t-test, one-way analysis of variance (ANOVA) and Chi-squared tests. RESULTS: Majority of patients were male, (68.4%), with a mean±SD age at diagnosis of 8.52±3.15 years. The most common underlying etiologies were post-infectious glomerulonephritis (PIGN) (63.2%) and lupus nephritis (21.1%). Thirteen patients exhibited a good clinical prognosis (68.4%), with 6 exhibiting a poor prognosis (31.6%), 4 of whom progressed to end-stage renal disease (ESRD), one experiencing a relapse and one developing chronic kidney disease. Post-infectious glomerulonephritis was associated with the best clinical outcome overall. Treatment was implemented early in most patients and continued for 3 months. Among the 19 patients, 2 died and one underwent hemodialysis. CONCLUSION: Post-infectious glomerulonephritis was the most common etiology of RPGN, with these patients achieving a good clinical prognosis overall. Early identification and treatment of RPGN is important to preserve renal function, which is a key factor for achieving a good prognosis.

Is the light at the end of the tunnel nigh? A review of ADPKD focusing on the burden of disease and tolvaptan as a new treatment.

Autosomal dominant polycystic kidney disease (ADPKD) causes pathological cystic changes to the kidney and is characterized by numerous renal and systemic manifestations. ADPKD is the fourth most common renal disease requiring renal replacement therapy. In this report, we present a detailed review of ADPKD, with a particular focus on its major economic, psychological, and social burden in affected patients. Treatment of this disease has been based on prophylactic and supportive measures. However, in recent years, new drugs have emerged as promising agents that may retard the progression of ADPKD, such as tolvaptan. In this report, we provide an in-depth discussion of tolvaptan, which has shown an effect in decreasing annual total kidney volume growth and renal function decline, thus slowing disease progression. The mechanism of action, side effects, and available data on cost-effectiveness are discussed together with the results of the first clinical trials and the most recent trials with regard to its efficacy and safety. Tolvaptan has recently received approval and been granted marketing authorization in Japan, Canada, Korea, Switzerland, and Europe. A demand for widely accepted guidelines for its use has emerged since its approval. The currently available series of recommendations and guidelines as to when to start treatment with tolvaptan, as well as which patients should be treated, are also reviewed in this report. We lastly offer some considerations for future trials, and raise unanswered questions.

Outcome of pediatric acute kidney injury: a multicenter prospective cohort study.

BACKGROUND: Acute kidney injury (AKI) is a common problem encountered in critically ill children with an increasing incidence and evolving epidemiology. AKI carries a serious morbidity and mortality in patients requiring admission to a pediatric intensive care unit (PICU). METHODS: We undertook a prospective cohort study of PICU admissions at three tertiary care hospitals in the Kingdom of Saudi Arabia over 2 years. The Kidney Disease Improving Global Outcomes (KDIGO) definition was used to diagnose AKI. RESULTS: A total of 1367 pediatrics PICU admissions were included in the study. AKI affected 511 children (37.4%), with 243 children (17.8%) classified as stage I (mild), 168 patients (12.3%) stage II (moderate), and 100 children (7.3%) were classified as stage III (severe). After adjustment for age, sex, and underlying diagnosis, in-hospital mortality was six times more likely among patients with AKI as compared to patients with normal renal function (adjusted OR: 6.5, 95% CI: 4.2-10). AKI was also a risk factor for hypertension (adjusted OR: 4.1, 95% CI: 2.8-5.9) and prolonged stay in the PICU and hospital, as it increased the average number of admission days by 10 (95% CI: 8.6-11) days in the PICU and 12 (95% CI: 10-14) days in the hospital. CONCLUSIONS: One-third of PICU admissions were complicated with AKI. AKI was associated with increased hospital mortality and the length of stay in both PICU and hospital.