The effect of continuous care model on parents’ knowledge and controlling symptoms and recurrence in children with nephrotic syndrome

Background and aims: Nephrotic syndrome is the most common manifestation of glomerular damage in children. Due to being a chronic disease as well as the need to continuing the provision cares to reduce the recurrence of disease, this research was aimed to determine the effect of continuous care model on parents’ knowledge and controlling symptoms and recurrence in children with nephrotic syndrome. Methods: This research is an experimental study which 66 children with nephrotic syndrome qualified for inclusion in the study, were randomly classified into 2 groups: Case and control. Data were collected by a researcher-designed questionnaire, and checklist for recording blood pressure, weight, blood lipids and the presence or absence of recurrence in children in the 2 groups before and after intervention. Continuous care model was carried out on test group for 6 months. Data were analyzed by SPSS software using descriptive statistics, Chi-square, and Independent t-test and McNamara tests. Results: The proportion of disease recurrence after the intervention in both case and control groups showed no significant difference (P=0.787), but mean of systolic blood pressure in both groups after the intervention showed significant differences (P=0.011). Also, the level of parents' knowledge ratio in both groups after the intervention showed significant differences (P=0.0001). Conclusion: According to the study results, it is suggested that children care needs with nephrotic syndrome will be met by using the implementation of continuous care mode

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

Acute kidney injury in Hemiscorpius lepturus scorpion stung children: Risk factors and clinical features

Acute kidney injury (AKI) is frequently seen in Hemiscorpius lepturus scorpion stung children. We have previously reported several victims with hemolytic uremic syndrome (HUS) and a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 deficiency. Hence, we conducted this study to identify predictive factors and clinical features of AKI in H. lepturus scorpion stung patients. We included all 215 H. lepturus scorpion stung children with no previous renal diseases in two groups (with and without AKI) and compared them based on their clinical and laboratory findings. AKI was found in 27.4% of patients, they were significantly younger and with lower body weight (P = 0.006, P = 0.011, respectively). There was a significant difference between groups with and without AKI in findings such as fever (P = 0.003), hypertension (P <0.001), hemolytic anemia (P <0.001), thrombocytopenia (P <0.001), massive proteinuria (P <0.001), hemoglobinuria (P <0.001), pyuria (P <0.001), and hematuria (P = 0.004). HUS was in 5.5% and disseminated intravascular coagulation in 14.6% which had a significant association with AKI (P <0.001).There were several independent predictors for AKI in a multivariate regression model including thrombocytopenia (P = 0.002), pyuria (P = 0.01), proteinuria (P =0.01), and fever (P = 0.02). Hemodialysis was performed in four patients but kidney function improved in all patients and there was no findings of renal impairment after three months follow-up. We found several predictors for AKI in children following H. lepturus scorpion sting including younger age, delay in receiving medical care, pigmenturia, microangiopathic hemolytic anemia, proteinuria, and pyuria

Bardet-biedl syndrome in a child with chronic kidney disease

A 4-year old boy was referred for evaluation of renal failure, posterior urethral valve (PUV) and urinary tract infection. His parents added complaints of polyuria, polydipsia, enuresis, shortness of stature, and inappropriate obesity. Serum blood urea nitrogen and creatinine levels were 45 and 3.5 mg/dL, respectively. Urine culture was positive for <i> Pseudomonas aeruginosa, </i> and abdominal ultrasound revealed bilateral small kidneys. The patient&#x2032;s history included mild to moderate mental retardation and postaxial polydactyly of both lower limbs amputated two years ago. The combination of mental retar-dation, obesity, postaxial polydactyly, and bilateral renal hypoplasia were compatible with the diagnosis Bardet-Biedl syndrome (BBS). The combination of PUV and BBS is a rare condition that caused this early onset of renal failure and inappropriate obesity guided us to the diagnosis

Clinical correlation between hypercalciuria and nocturnal enuresis

Hypercalciuria may present with dysuria, urinary incontinence and nocturnal enuresis (NE). To determine the frequency of hypercalciuria in NE patients and normally continent children, we studied 122 consecutive pre- school children with NE referred to our nephrology clinic during two years, from September 2007 to August 2009. We measured the 24- hour urinary calcium. Furthermore, we compared the response to nasal desmopressin in hypercalciuric and normocalciuric patients. Hypercalciuria was found in 26 (21.3 %) of the NE patients as compared with five (4.5%) of 110 continent children [(P 0.05). The response to desmopressin above 90% occurred within one month of therapy without a significant change in the levels of hypercalciuria. We conclude that these results suggest that hypercalciuria has a significant association with NE and does not interfere with the desmopressin therapy

How to Reach Rapid Diagnosis in Sickle Cell Disease?

Objective: Sickle cell disease (SCD) is a common hereditary disease in Iran. In developed countries, newborn screening programs have been established to ensure early diagnosis, but in most developing countries, screening is not performed and the diagnosis is often delayed. The aim of the present work was to investigate the clinical presentation of SCD in Iran and comparison of its hematologic indices with normal children. Methods: The study included 44 pediatric patients (26 boys and 18 girls) with sickle cell anemia (SS), 27 sickle /β°-thalassemia (Sβ°), and 21 sickle /β+-thalassemia (Sβ+). Fifty seven healthy individuals matched with the patients were randomly selected as controls. Findings: Mean age at diagnosis in SS group was 4.3 years. At the time of diagnosis all patients were anemic, 89% complained of painful crises. Hemoglobin(Hb) concentration, red blood cell (RBC) count and Hb×RBC product in SS group was significantly lower than in control group (P<0.001), mean corpuscular volume (MCV) and mean corpuscular hemoglobin (MCH) showed no significant differences. Hb×RBC product below 45 and MCH/RBC above 7 have the best sensitivity and specificity for differenting SS group and the control normal group (91 and 98% for Hb×RBC and 89 and 100% for MCH/RBC respectively). Mean age at diagnosis in Sβ+ group was higher than in SS and Sβ° groups (7.45 year vs 4.26 and 4.25 year) (P<0.001). In addition, Sβ° and Sβ+ groups had significantly lower MCV, MCH, and Hb×RBC indices compared with control group. Conclusion: We suggest that in an anemic patient with history of pain crises, normochrome normocytic anemia, Hb×RBC <45 and MCH/RBC ≥7, SCD should be considered and the patient evaluated accordingly to confirm the diagnosis

ADAMTS-13 deficiency following Hemiscorpius lepturus scorpion sting

Hemiscorpius lepturus is a lethal scorpion with potentially cytotoxic venom. Various degrees of local and systemic toxicity have been observed after its envenomation ranging from local erythema to disseminated intravascular coagulation, renal failure and severe pulmonary hemorrhage. In this case report, we report on a seven-year-old patient who developed the hemolytic uremic syndrome (HUS) after being stung by the scorpion H. lepturus. This condition is characterized by microangiopathic hemolytic anemia, thrombocytopenia, increased serum levels of lactate dehydrogenase and uremia. We evaluated the causes of HUS and found that the levels of C3, C4, CH50 and H factors were normal, but the activity of Von Willebrand factor cleaving protease was decreased (less than 5% of the normal activity). The patient improved after administering therapy with plasma exchange

Chronic Kidney Disease in Southwestern Iranian Children

Objective: The aim of the study was to determine the etiology of Chronic Kidney Disease (CKD) among children attending the pediatric nephrology service at Abuzar children's hospital in Ahvaz city, the referral center in Southwest of Iran. Methods: We reviewed the records of 139 children, diagnosed to have CKD over a 10-year period. CKD was defined a glomerular filtration rate (GFR) below 60 ml/1.73 m2/min persisting for more than 3 months. Findings: Among 139 children 81 (58%) were males. The mean age at diagnosis of CKD in the patients was 4.2 (±3.6) years. Mean level of serum creatinine at presentation was 1.9 (±1.4) mg/dl. The mean GFR at presentation was 33.5 (±15.4) ml/1.73m2/min while 22% of the patients were already at end stage renal failure indicating that these children were referred too late. Congenital urologic malformation was the commonest cause of CKD present in 70 (50.4%) children [reflux nephropathy (23.1%), hypo/dysplastic kidney (15.8%), obstructive uropathy (10.8%), and prune belly syndrome (0.7%)]. Other causes included hereditary nephropathies (17.2%), chronic glomerulo-nephritis (6.5%), multisystemic diseases (4.3%), miscellaneous and unknown (each one 10.8%). The mean duration of follow-up was 26 (±24.67) months. Peritoneal or hemodialysis was performed in 10 patients. Six patients underwent (4 live-related and 2 non-related) renal transplantation. The rest have died or received standard conservative management for CKD. Conclusion: The commonest causes of CKD were reflux nephropathy, hypo/dysplastic kidney, hereditary nephropathy and obstructive uropathy. Patients presented late, had severe CKD and were malnourished and stunted