Osama Y. Safdar

Rituximab for troublesome cases of childhood nephrotic syndrome

Nephrotic syndrome (NS) is the most common glomerular disease of childhood. Steroid-dependent and steroid-resistant nephrotic syndrome present challenges in their pharmaceutical management; patients may need several immunosuppressive medication for optimum control, each of which medication has its own safety profile. Rituximab (RTX) is a monoclonal antibody that targets B cells and has been used successfully for management of lymphoma and rheumatoid arthritis. Recent clinical studies showed that rituximab may be an efficacious and safe alternative for the treatment of complicated nephrotic syndrome. In this review article, we aim to review the efficacy and safety of RTX therapy in nephrotic syndrome. We reviewed the literature pertaining to this topic by searching for relevant studies on PubMed and Medline using specific keywords. The initial search yielded 452 articles. These articles were then examined to ensure their relevance to the topic of research. We focused on multicenter randomized controlled trials with relatively large numbers of patients. A total of 29 articles were finally identified and will be summarized in this review. The majority of clinical studies of RTX in complicated pediatric NS showed that rituximab is effective in approximately 80% of patients with steroid-dependent NS, as it decreases the number of relapses and steroid dosage. However, RTX is less effective at achieving remission in steroid-resistant NS. RTX use was generally safe, and most side effects were transient and infusion-related. More randomized, double-blinded clinical studies are needed to assess the role of RTX in children with nephrotic syndrome.

Left Ventricular Hypertrophy in Obese Children

Childhood obesity is one of the most troubling health conditions worldwide, and it is associated with many diseases that eventually lead to serious morbidities and mortality in adulthood, such as insulin resistance, type 2 diabetes mellitus, high blood pressure, dyslipidemia, obstructive sleep apnea and subsequent renal, and liver and cardiovascular diseases. Cardiovascular disease carries the most risk of all of the long-term outcomes of obesity because it is associated with serious complications in adult life, such as heart failure, acute coronary syndrome, and premature sudden death. This review aims to address childhood obesity as a worldwide health concern and focuses on the cardiovascular risk and adverse outcomes associated with this condition. This review presents details of obesity-related cardiac structural and functional changes, such as left ventricular hypertrophy (LVH) and dysfunction, respectively. A search of multiple medical databases was performed, and the results yielded many studies related to pediatric left ventricular hypertrophy; seventeen studies were found on left ventricular hypertrophy in obese children from 1980 to 2015. Most of these studies demonstrated that obesity and its comorbidities are important predictors for left ventricular hypertrophy in the pediatric age group. Furthermore, weight reduction is an important measure to reverse these structural changes and reduce the associated risks.

Antibiotic Resistance Pattern in Healthy Children Diagnosed withCommunity Acquired Respiratory Tract Infections in King AbdulazizUniversity Hospital

Objectives: To identify the prevalent organisms associated with respiratory tract infections according to age groups. To identify the most common antibiotics prescribed among pediatric age groups. Methods: A retrospective cohort study conducted on patients aged 0-18 years, admitted to the inpatient department (IPD) diagnosed with respiratory tract infection between Jan 1 2009 to Jan 31 2015 in King Abdulaziz University Hospital (KAUH). Demographic information, diagnosis, date of admission, laboratory investigations, and treatment were collected from patients’ electronic and paper records then analyzed. Exclusion criteria were based on congenital defects, chronic diseases, and compromised immunity. Results: 122 patients with respiratory tract infections were selected for the study (73 males and 49 females). 45.9% of which were infants-toddlers (n=56). The most common diagnosis was pneumonia unspecific (n=32), followed by acute tonsillitis (n=24) and otitis media (n=22). 52 microorganisms were isolated from clinical samples, 14% being Pseudmonas aeruginosa and 13% Streptococcus pneumoniae. 171 antimicrobials were prescribed during the study period. Penicillins were the most frequent (n=78), followed by Cephalosporins (n=45) and Macrolides (n=22). 6.897% resistance was detected among 87 files with culture and sensitivity tests, 30% of which were positively cultured, ranking resistance with 20%. Conclusions: Despite the results showing minimal resistance, antibiotic resistant organisms remain a global concern that needs to be addressed to prevent the spread of antimicrobial resistant infections.

NEUTROPHIL GELATINASE-ASSOCIATED LIPOCALIN AS AN EARLY MARKER FOR THE DIAGNOSIS OF URINARY TRACT INFECTIONS IN SAUDI CHILDREN

Background: Urinary tract infections (UTI) are widespread infections in the pediatric age group which presents non-specific symptoms. “Neutrophil gelatinase-associated lipocalin” (NGAL) is one of a biomarker in determining kidney injury. The early urinary tract infections diagnosis is essential for the purpose of preventing enduring consequences. Aim: To evaluate the use of NGAL as an early predictor of UTI in clinical practice. Patients and methods: A cross-sectional study examined specificity and sensitivity of urinary NGAL in the early UTI diagnosis. All febrile children admitted to KAAUH (age: 0-14 years) were included. Mann-Whitney U test, Shapiro- Wilk’s test, and Spearman correlation was used to analyze the data. Results: In terms of mean urinary NGAL, no significant difference was observed between urinary NGAL levels in patients with and without UTIs (P-value=0.17). The receiver operator characteristic (ROC) curve demonstrated 0.594 sensitivity, and 0.683 specificity. For serum CRP (ROC) curve demonstrated 0.53 sensitivity and 0.47 specificity. Spearman’s correlation was undertaken to examine an association between NGAL and CRP levels. There was a positive monotonic correlation between NGAL and CRP levels (rs= 0.503 n = 73, p < .001). Conclusion: Urinary NGAL is a poor biomarker for the diagnosis of febrile UTI. Further investigations are required with larger groups of patient to confirm the results of this study.

Paradoxical increase in blood pressure following bilateral native nephrectomy.

Hypertension with Chronic kidney disease is often difficult to control medically. In such patients, nephrectomy can help to control blood pressure (BP). We describe a case of a 6‐year‐old boy with autosomal recessive polycystic kidney disease who showed a paradoxical increase in BP following bilateral nephrectomy.

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.

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.

Unusual Case of an Infant with Urinary Tract Infection Presenting as Cholestatic Jaundice

Neonatal jaundice is considered one of the most common reasons for admission to the pediatric medical ward. We report a case of a 1-month-old infant who presented with jaundice but no fever or any other signs of systemic illnesses. Laboratory test results revealed high direct hyperbilirubinemia, and urine culture showed a urinary tract infection with Enterobacter cloacae as the causative agent. He was admitted to the pediatric medical ward where he was treated with a course of antibiotics for 14 days, and cholestasis resolved completely following a course of antibiotics. We conclude that direct hyperbilirubinemia can be related to urinary tract infection in neonates. It is unusual for urinary tract infection to present clinically and biochemically as cholestatic jaundice.

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.