Intracardiac Thrombus in Renovascular Hypertension: A Case Report

Thromboembolism is a rare complication of primary nephortic syndrome. Both venous and arterial thrombosis might occur in steroid responsive and steroid resistant nephrotic syndrome. This is the report of an infant with nephrotic syndrome and renovascular hypertension, complicated with asymptomatic intracardiac thrombus and managed appropriately with medical treatment

Urine Neutrophil Gelatinase Associated Lipocalin as a Predictor of Vesicoureteral Reflux and Renal Parenchymal Damage: A Systematic Review

Background: Vesicoureteral reflux (VUR) is the most common congenital urinary tract abnormality in children. Renal parenchymal damage is the most devastating complication of severe undiagnosed VUR. Different diagnostic biomarkers have been introduced as alternatives for radiologic evaluation in these patients. This review article aimed to increase the knowledge about the role of urine neutrophil gelatinase-associated lipocalin (uNGAL) in children affected by primary VUR and renal parenchymal damage. Methods: A systematic review of PubMed, Scopus, Web of Science, ProQuest, and Ovid was conducted in September 2022 to retrieve studies that investigated the correlation between uNGAL or uNGAL/Cr excretion and primary VUR in male/female patients younger than 18 years of age. Patients with secondary VUR, age older than 18 years, infectious or inflammatory disorders, obstructive uropathies, and acute or chronic kidney diseases were excluded. Two reviewers independently screened the titles and abstracts of the search results and then assessed the full texts selected from the pertinent studies.  Results: Eighteen research articles with a total sample of 699 patients were found to measure uNGAL in VUR or renal scarring. UNGAL or uNGAL/Cr had increased excretion in the majority of children with primary VUR or RPD, with a positive or no correlation to the severity of VUR.  Conclusions: Several studies addressed uNGAL and uNGAL/Cr as putative biomarkers for the prediction of VUR or reflux-associated RPD

Assesment, treatment and prevention of atypical hemolytic uremic syndrome

Hemolytic uremic syndrome (HUS) is a heterogeneous group of hemolytic disorders. Different terminologies have been described in HUS, which are as follows: (1) D+ HUS: Presentation with a preceding diarrhea; (2) typical HUS: D+ HUS with a single and self-limited episode; (3) atypical HUS (aHUS): Indicated those with complement dysregulation; (4) recurrent HUS: Recurrent episodes of thrombocytopenia and/or microangiopathic hemolytic anemia (MAHA) after improvement of hematologic abnormalities; and (5) familial HUS: Necessary to distinct synchronous outbreaks of D+ HUS in family members and asynchronous disease with an inherited risk factor. aHUS is one of the potential causes of end-stage renal disease (ESRD) in children. It has a high recurrence after renal transplantation in some genetic forms. Therefore, recognition of the responsible mechanism and proper prophylactic treatment are recommended to prevent or delay the occurrence of ESRD and prolong the length of survival of the transplanted kidney. A computerized search of MEDLINE and other databases was carried out to find the latest results in pathogenesis, treatment, and prevention of aHUS

Validity of Serum Sodium and Calcium Screening in Children

Febrile convulsion (FC) is the most common seizure disorder in young children. Different predisposing factors have been suggested to enhance the susceptibility to febrile seizure and its recurrence. The main objective of this study was to identify the adverse effect of electrolytes disturbance in FC and its recur-rence. The medical records of 175 children with convulsive disorders were reviewed. Patients were divided into 3 groups. Group A (n=71) with simple febrile convulsion (FC) and group B (n=54) with recurrent FCs. Fifty children (group C) with non-FC served as control. Serum sodium and calcium concentrations were significantly lower in groups A and B compared to the control group. Serum sodium level was not significantly different between group A and B patients (134.4 vs. 134.7 mEq/l) but was significantly lower in group A than the control group (P= 0.014). Serum calcium concentration did not differ among the 3 groups. Minor abnormal levels of serum sodium concentration were detected in children with febrile convulsions. Thus, routine serum electrolytes screening are not recommended in febrile seizure

Clinical Course and Effective Factors of Primary Vesicoureteral Reflux

Vesicoureteral reflux (VUR) is one of the most important causes of urinary tract infection and renal failure in children. It is a potentially self-limited disease. The aim of this study was to evaluate the clinical course and significant factors in children with primary VUR. The medical charts of 125 infants and children (27.2 % males, 72.8% females) with all grades of primary VUR were retrospectively reviewed. Mean age at diagnosis was 22.3 ± 22.9 months. 52% of patients had bilateral VUR. Mild reflux (Grade I, II) was the most common initial grade. 53.6% of patients achieved spontaneous resolution. 30.1% of patients had decreased renal function on initial DMSA renal scan, significantly in males and severe VUR. Reflux nephropathy occurred in 17.6% of patients, especially in renal damage and male sex. No significant association was observed between recurrent urinary tract infection with the severity of VUR, and the presence of renal damage at admission. Age at diagnosis, gender, grade, laterality, the absence of recurrent urinary tract infection and renal damage had a significant correlation between spontaneous VUR resolution. Spontaneous resolution of primary VUR occurred significantly in female patients, age ≤ 30 months at diagnosis, mild-to-moderate VUR, unilateral reflux, the absence of recurrent urinary tract infection, and renal damage

Epidermal Nevus Syndrome and Dysplatic Kidney Disease

Epidermal nevus syndrome is a rare congenital disorder, characterized by epidermal nevi and multiple organ involvement. Multicystic kidney disease has been very rarely reported in this syndrome. Here is the report of a boy presented with multiple epidermal nevi, cardiac anomaly, seizure attack, hemi hypertrophy, and multicystic dysplastic kidney complicated with Wilms' tumor. According to this association, it is suggested to search for dysplastic kidney disease in patients with neurocutaneous disorders

Plasma Volume to Achieve Remission in Atypical Hemolytic Uremic Syndrome

Introduction: Atypical hemolytic uremic syndrome (HUS) is defined as a heterogeneous group of disorders. Plasma infusion or plasma exchange is the rescue therapy for this life-threatening syndrome. There is no evidence for the volume of plasma required to induce remission. Materials and Methods: Between 2007 and 2018, Forty – two patients (M=20, F=22) with a diagnosis of recurrent or familial atypical hemolytic uremic syndrome (aHUS) who were admitted to Ali-Asgar Children’s Hospital were enrolled in this observational retrospective study. The total volume of plasma required for normalizing platelet (>150000) and LDH (<500 IU), eliminating hemolysis, and decreasing serum creatinine at first presentation of disease was calculated. Patients with TTP, vasculitis, and post infectious HUS were excluded. Results: The mean age of the patients was 53 months (3-144 m). The majority of patients achieved remission at first presentation by plasma infusion (5 under peritoneal dialysis and 4 under hemodialysis) but ten patients required plasmapheresis. A total of 980 units of FFP perfused with a total volume of 195.975 L. The median (range) total plasma volume required for remission was 166 ml/kg (43-2850 ml/kg).Conclusions: This study showed that the required plasma volume for the acute phase of atypical HUS for controlling the first attack of disease.Keywords: Atypical Hemolytic Uremic Syndrome; Plasma Volume; Remission Induction; Child