Abstract

Sickle cell disease (SCD) is a common hemoglobinopathy in the world with progressive multi organ failure. Many organs have been affected in this disease, but sickle cell nephropathy (SCN) is a major complication which affects the quality of life in these patients. SCN has a wide range of renal manifestations such as asymptomatic microalbuminuria, hyposthenuria, hematuria, frank proteinuria, nephrotic syndrome and end stage renal disease (ESRD). Nowadays novel biomarkers such as N-acetyl-beta-D-glucosaminidase (NAG), kidney injury molecule-1 (KIM-1) and transforming growth factor β (TGF-β) has allowed the early detection of the kidney involvement in sickle cell disease. The angiotensin converting enzyme (ACE) inhibitor drugs such as captopril or enalapril and also ACE receptor blockers (losartan) have beneficial effects in albuminuria or proteinuria. Also endothelin-1 (ET-1) receptor antagonists have a promising role in glomerular injury in SCD. In sickle patients who develop ESRD, renal replacement therapy can be life- saving. In recent years, kidney transplantation is the only curative treatment for advanced chronic kidney disease in these patients.

Keywords: Sickle cell; Nephropathy; Kidney.

Background and Aim: To determine the spectrum of congenital anomalies of kidney and urinary tract (CAKUT) in Indian children, and to evaluate its clinico-epidemiological characteristics.

Methods: A tertiary-care, hospital-based cross-sectional study conducted (November 2016-October 2018) at SCBMCH & SVPPGIP Hospital included children aged ≤14 years with a diagnosis of CAKUT. Incidence of individual congenital anomalies and clinico-epidemiological characteristics of each anomaly thus considered were evaluated.

Results: In 140 children, 9 types of congenital anomalies were reported; posterior urethral valve was most common (37.85%) followed by pelvi-ureteric junction obstruction (32.14%) and vesico-ureteric reflux (14.28%). Hypospadiasis (5%), renal agenesis (2.86%), multicystic kidney disease (2.86%), duplication of pelvi-calyceal system (2.14%), horseshoe kidney (1.42%) and ureterocele (1.42%) were other CAKUTs. A total of 68.57% were boys (girls=31.43%). The CAKUTs were observed more in children with: age 1-5 years (32.14%), unilateral disease (45%), and term delivery (67.86%), and in children of mother aged >30 years (58.57%). Diabetes was present in mothers of 4 children (gestational: 2, pre-gestational: 2), hypertension in 3, tobacco use in 5, oligohydramnios in 7 and polyhydramnios in 3 mothers. Majority of the children had asymptomatic (37.86%) manifestation followed by burning micturition (31.43%). Non-renal congenital anomalies were present in 15.71% children (gastrointestinal malformations: 5.71% were most common). Positive family history for CAKUT was seen in 7.8% cases.

Conclusion: The most frequent CAKUTs were posterior urethral valve and pelvi-ureteric junction obstruction in our study. The study also provided the clinico-epidemiological characteristics of CAKUT. These data can be helpful for prenatal counseling and management of CAKUTs.

Keywords: CAKUT; congenital anomalies; child; Indian.

PBackground and Aim: Routine clinical and laboratory assessments facilitate the diagnosis of erythropoietin (EPO) resistant anemia. Resistance to erythropoietin (EPO) rarely occurs in children with chronic kidney disease (CKD). It may be caused by several factors, such as poor compliance, hyperparathyroidism, malnutrition, inadequate dosages, and medication. This study was conducted to evaluate the frequency and causes of EPO resistant anemia in CKD children.

Methods: This observational retrospective study was performed in Ali Asghar Children’s Hospital in 2008. Children who were treated by EPO and still had hemoglobin concentrations (Hb) below 10 g/dl or required very high erythropoietin doses (more than 150 IU/kg/week) were considered as EPO resistant anemic cases. Therefore, their data including demographic data, EPO dosage, medications, underlying diseases, mode of dialysis, and lab test results were collected from their medical records.

Results: The Hb concentration was below 8 g/dl in eight cases (40%). The male to female ratio was 1.66 and the median age was 7.9 years old. The median hemoglobin concentration was 6.25 g/dL. Three patients (37.5%) were true cases of resistance in EPO (two patients had bone marrow fibrosis due to hyperparathyroidism and one had EPO Ab) while five cases (62.5%) received inadequate doses of EPO (anemia improved upon an acceptable EPO dosage).

Conclusion: True resistance to erythropoisis stimulatin agent is not frequent and because of there are heterogeneous causes for Lack of appropriate response to EPO, so full investigation is needed especially complete history to find the underlying reason.

Keywords: Anemia; Chronic Kidney Disease; Erythropoietin.

Background and Aim: Antenatal hydronephrosis refers to the dilation of renal pelvis during fetal development. This condition is commonly diagnosed during intrauterine ultrasonography. According to available statistics, fetal anomalies are seen in about 0.5-2.5% of intrauterine ultrasound examinations. The most common anomaly is hydronephrosis. The severity of renal pelvic dilatation in the first sonography after birth may help to diagnose the underlying cause of antenatal hydronephrosis. On the other hand, knowledge of the etiology of hydronephrosis can help to understand the clinical course of the disease and to determine an appropriate therapeutic protocol for patients.

Methods: In this descriptive cross-sectional study, all infants with antenatal hydronephrosis referred to the pediatric nephrology clinic of Imam Khomeini Hospital, Ilam, Iran were evaluated. Considering a hydronephrosis frequency of 50%, confidence interval of 95%, and error rate of 13%, the sample size was calculated at 60 subjects.

Results: This study was performed in 61 neonates with antenatal hydronephrosis, including 43 (70.5%) males and 18 (29.5%) females. The frequency of hydronephrosis was significantly higher in boys than girls. Non-obstructive hydronephrosis was the most common underlying etiology in the patients. Most of the infants had a mild hydronephrosis. UPJO (ureteropelvic junction obstruction) was the most common cause of hydronephrosis. Furthermore, most of the patients had bilateral hydronephrosis.

Conclusion: Non-obstructive hydronephrosis accounted for about 50% of antenatal hydronephrosis cases in this study. The intensity of the pelvic dilatation was directly associated with obstructive hydronephrosis, which can be used as a diagnostic guide.

Keywords: Hydronephrosis; Etiology; Neonates; Severity.

Background and Aim: Urinary tract infection (UTI) is one of the most common and important childhood diseases that may lead to complications such as acute pyelonephritis, renal scaring, and renal failure if not diagnosed and treated properly. The antibiotic resistance rate has increased recently because of unnecessary antibiotic consumption. In order to prescribe an appropriate drug as empirical therapy, it is necessary to know the prevalence of pathogens causing UTI and their antibiotic resistance rate in the community. Therefore, this study was conducted to present an appropriate guideline for UTI empirical therapy through evaluating the rate of antibiotic resistance in a sample of hospitalized patients suffering from UTI.

Methods: This retrospective descriptive analytical study was conducted in 130 children with UTI whose medical records included antibiogram results in hospitals affiliated with Shahid Beheshti University during 2016-2017.

Results: Of 130 patients, 73.07% were girls and 26.92% were boys. About 50% of the patients were under one-year-old, 33.07% were 1-3 years old, and 16.15% were over 3 years old. The detected pathogens were E. coli in 63.07%, Enterococcus in 14.61%, Klebsiella in 13.07%, Pseudomonas aeruginosa in 5.38%, and Enterobacter in 3.84%.

Conclusion: Based on the data of the present study, ampicillin, co-trimoxazole and first-generation cephalosporins are not appropriate for UTI empirical therapy.

Keywords: Antibiogram; Antibiotic resistance; Urinary tract infection.

Hydrothorax is a rare but serious complication of peritoneal dialysis. We report a recurrent pleural effusion in a case of ESRD (end stage renal disease) treated by pleurodesis using tetracycline.

 Key words: Sweet pleural effusion. Peritoneal Dialysis. Pleurodesis

Keywords: Sweet pleural effusion; Peritoneal Dialysis; Pleurodesis; Child.

An 8-year-old boy presented with primary nocturnal enuresis as the first clinical sign of ureterocele in the absence of dysuria, urinary incontinence or urinary tract infections. A prolonged history of bedwetting prompted subsequent clinical and laboratory evaluations, leading to the correct diagnosis of ureterocele.  Primary and persistent nocturnal enuresis as an initial manifestation of ureterocele has not been reported previously. The present patient showed a feature not previously described in children with ureterocele. Although rare, ureterocele should be considered in the differential diagnosis of children presenting with primary nocturnal enuresis.

Keywords: Primary nocturnal enuresis; Simple orthotopic ureterocele; Single-system ureter.