Thalassemia syndromes are the most prevalent hereditary hemoglobinopathy in the world. Reduction or absence of β-chain synthesis is the hallmark of β-thalassemia syndromes, resulting in an excess of alpha chains. In all of the patients with thalassemia major (TM) and some patients with thalassemia intermedia (TI), repeated and regular transfusions are the main part of treatment. In β-thalassemia minor, the patients are asymptomatic and diagnosis is often based upon hematologic parameters on CBC and hemoglobin electrophoresis. Both tubular and glomerular dysfunctions have been reported in three forms of β-thalassemia, but these changes are more prominent in TM and TI. Iron overload due to repeated transfusions, chronic anemia, and the toxicity of iron chelator drugs such as deferoxamine and deferasirox are the main factors in the pathogenesis of nephropathy in patients with transfusion-dependent thalassemia (TDT). In this review, the mechanism of renal disease in thalassemia, comparative assessment of kidney dysfunction in three variant forms of β-thalassemia, and markers of tubular and glomerular dysfunction have been discussed.

Keywords: Thalassemia; Anemia; Kidney; child.

Original Research Papers

Pediatric Long-term follow up for renal disorders in ALL children by evaluating urine NGAL

Gholamreza Bahoush Mehdiabadi, Hasan Otukesh, Rozita Hosseini Shamsabadi, Mahdie Emami Arjomand

Journal of Pediatric Nephrology, Vol. 7 No. 2 (2019), 31 August 2019, Page 1-5

Introduction: Recent Developments in cancer treatment could provide a better survival rate for Acute Lymphoblastic Leukemia (ALL) patients. Survivors faced different long term complication after treatment, for instance cardiac, neurologic and kidney complications. Assessment of the late complications could be useful in the optimization of treatment protocols. The objective of this study was to evaluate the late kidney complications, renal function in ALL patients after therapy.

Material & Methods: In this study, we used 46 children. The treatment preformed based on ICBFM protocol. The mean age at the start of the treatment was 53±23 and the mean follow up time was 48±11 months. The tubular damage in these patients was evaluated by urinary NGAL level and the renal function was assessed by GFR.

Results: in this study 56.7% of the patients were male. The NGAL level shows abnormally high in 8.9% of patients and the mean urine NGAL was 63±113ng/mL. Also, the mean GFR at the time of the diagnosis and at the time of the start of the follow up were 102.8 ±25.6 mL/min/1.73 m2 and 93.6 ±29.1 mL/min/1.73 m2, respectively. The study indicated GFR were less than 60 mL/min/1.73 m2 in 13.3% of patients.

Conclusion: this study indicated the long term follow up of the ALL survivors for kidney disorders are an important manner. The urinary NGAL level shows that 8.9% of the patients are tubular damage by using this treatment protocol. The study concluded that ICBFM protocol is a safe protocol with little long term damage. 

Clinical Effects of Methylphenidate Compared to Oxybutynin on Management of Giggle Incontinence

Yalda Ravanshad, Anoush Azarfar, Mohammad Esmaeeli, Aida Foroutan, Houra Marhamati, Zahra Mostafavian, Sahar Ravanshad, Azadeh Zare Feizabad, Mahmood reza Khazaee, Mojtaba Fazel

Journal of Pediatric Nephrology, Vol. 7 No. 2 (2019), 31 August 2019, Page 1-3

Background and Aim: Giggle incontinence, also known as “enuresis risoria”, is characterized by unexpected, involuntary, and complete bladder voiding in response to laughter. The cause is unknown; however, there are different assumptions based on several case studies. This research discusses the effectiveness of methylphenidate for giggle incontinence in children.

Methods: Fifteen girls who met the giggle incontinence criteria were randomly divided to two groups: group A (n=8 girls) and group B (n=7 girls). The participants in group A took Oxybutynin chloride and the patients in group B received methylphenidate for one month. The response of the two groups to drugs was assessed.

Results: Group A included 8 girls (mean age: 7.7 years) and group B comprised 7 girls (mean age: 8.4 years). Only one patient in group A showed complete response to Oxybutynin chloride. No wetting was reported by six patients in group B.

Conclusion: Giggle incontinence is a rare form of incontinence. The pathophysiology is unfortunately unknown yet. Methylphenidate may be suggested for symptomatic relief compared to Oxybutynin chloride until the etiology of the disease is more clearly defined.

Keywords: Giggle incontinence; Giggle micturition; Laughter incontinence; Enuresis.

Background and Aim: Despite the spread of Henoch-Schonlein purpura (HSP) in all societies, especially in Asian children, no comprehensive study on HSP has been conducted in Iranian children and most of these reports are limited to disease cases or exclusively to patients with HSP. Therefore, this study was conducted to describe the clinical, diagnostic, and therapeutic approaches in children with HSP in Iran.

Methods: This historical cohort study was performed in all children suffering from HSPN hospitalized at Ali-Asghar Children’s Hospital, Tehran between April 2006 and March 2017. The patients' baseline characteristics including demographics, clinical symptoms and laboratory parameters were all collected from hospital files. The patients were followed up for at least six months of initiating treatment and also for 12 to 120 months after treatment.  

Results: Of 100 patients with HSP, 18 (11 boys and 7 girls) had indications for biopsy that were included in the study. The mean age of the participants was 7.72 ± 2.69 years. Nephrotic syndrome was found in 44.4% and nephritic syndrome in 61.1% of the patients. Hematuria was found in 66.7%, proteinuria in 66.7%, and hypertension in 38.9% of the patients. The mean serum creatinine was 1.0 ± 0.6 mg/dl with a mean GFR of 95 ± 5ml/min. Regarding pathological classification, 33.3% had class II and 66.7% had class III. With respect to therapeutic regimen, 61.1% were treated only with steroids while others were treated with a combination of steroids and immunosuppressant drugs. During the follow-up time, all patients were treated successfully with the mentioned regimens. In all treated subjects, proteinuria disappeared in all urine samples. Due to complete improvement in all patients, repeated renal biopsy was not indicated.

Conclusion: Kidney involvement occurs as nephritic syndrome in about two thirds of patients and as nephrotic syndrome in the remaining cases. In the majority of patients, treatment with steroids alone is successful although combined therapy with immunosuppressant drugs is required in the remaining patients. In summary, the therapeutic protocols are associated with a significant long-term recovery (a five-year recovery of 87.5% in our study).

Keywords: Henoch-Schonlein purpura; Nephritis; Nephrotic syndrome; Child.

A Comparative Evaluation of Salivary Changes and Oral Indices in Pediatric Patients Having Chronic Kidney Disease and Juvenile Diabetes with Healthy Controls

Shweta Chaudhary, Manasi Rajendra Shimpi, Rajendra Kashinath Shimpi, Laxmi Lakade, Shweta Jajoo, Sneha Desai

Journal of Pediatric Nephrology, Vol. 7 No. 2 (2019), 31 August 2019, Page 1-8

Background and Aim: Chronic Kidney disease is a common condition seen in Juvenile diabetes with 90% of renal impairment patients displaying a wide spectrum of oral manifestations in the hard and soft tissues including changes of the salivary composition and flow rate. There is an increase in the serum cystatin-C, urea and creatinine levels in these patients, which is reflected in the saliva. This study was conducted to assess the changes in salivary levels of cystatin-C, urea, and creatinine as well as oral – Decayed, Missing and Filled Teeth Index (DMFT) and gingival indices in pediatric patients suffering from chronic renal disease and juvenile diabetes and compare them with healthy individuals.

Methods: Fifteen patients with juvenile diabetes suffering from chronic renal disease and 15 healthy controls aged 2-18 years were included in the study. Their saliva was analyzed for creatinine, cystatin-C and urea levels using an auto-analyzer and correlated with their existing serum levels. DMFT, gingival index, gingival bleeding and gingival enlargement indices were also assessed.
Results: Increased levels of salivary cystatin C, urea (p value <0.001) and creatinine (p value =0.001) were seen in the cases. The deft value was significantly lower (p value <0.001) while the gingival index, gingival bleeding index, and gingival enlargement index were significantly higher in the subjects with renal impairment. 

Conclusion: Chronic Kidney disease results in many metabolic changes in the body, necessitating frequent biochemical blood analysis. Saliva, being a non-invasive, simple and rapid adjunctive tool, can be used for diagnosing and staging the disease and to check the progression of the condition.

Keywords: Chronic Kidney Disease; Renal Dysfunction; Saliva; Cystatin-C; Diagnosis.

Background and Aim: Being the most common pathologic cause of hydronephrosis in children, we characterized and evaluated the long-term outcome of Ureteropelvic junction obstruction (UPJO) at a tertiary hospital in South Africa

Methods: Children confirmed to have UPJO between 1985 and 2016 were characterized based on demographic and baseline clinical data. Long term outcomes were need for surgical intervention, loss to follow up rates, anthropometric measures, renal outcomes (Glomerular Filtration Rate and Blood pressure).

 Results: Of 107 children, 74.8% were male, 47% had hydronephrosis identified antenatally, 47.7% had the left kidney unilaterally affected, 31.8% had an additional urogenital anomaly, 19.6% presented with an abdominal mass, and 37.4% had a urinary tract infection. On enrolment, 54.2% and 30.8% had normal systolic and diastolic BP, 59.8% had normal BMI for age and 72% had normal length/height for age. The median follow-up time was 35(9.0 - 191.0) months, 65% had surgery with a median time to surgery of 2 (0 - 6.8) months. Children lost to follow-up had a higher proportion of extrinsic causes of UPJO (12.0% vs. 0%, P=0.041) and other urogenital anomalies (38.7% vs. 15.6%, P=0.019). There was no significant effect of time or surgical intervention on mean BMI and height for age, blood pressure percentile and eGFR for age.

Conclusion: In our setting, UPJO has an early presentation, with an early time to surgery. Long-term outcome is favorable, but loss to follow-up presents a significant drawback that needs to be addressed.

Keywords: Ureter; Hypertension; Congenital anomalies of kidney and urinary tract; CAKUT; Urological Diseases; Hydronephrosis.  

Case Reports

Acute Kidney Injury and Anemia in Infants with Primary Hyperoxaluria: Two Case Reports

Mohammad Amin Fallahzadeh, Elham Vahhab, Babak Shirazi Yeganeh, Mohammad Hossein Fallahzadeh

Journal of Pediatric Nephrology, Vol. 7 No. 2 (2019), 31 August 2019, Page 1-4

Primary hyperoxaluria is a rare hereditary disease that usually presents with renal stone, nephrocalcinosis and renal insufficiency. Anemia is usually expected secondary to chronic kidney disease or bone marrow oxalosis following hyperoxalemia. However, sudden onset of renal insufficiency and anemia is very unusual and not easily explained. In this report, we present two infants with histopathologic diagnosis of hyperoxaluria who presented with sudden onset of anuria and severe anemia that required blood transfusion. Both patients had normal body growth indices. Kidneys and urinary bladder x-rays in both infants and serial ultrasound evaluations in one of them did not reveal any renal stone or nephrocalcinosis. The other patient had multiple microcalculi in both kidneys on ultrasound. We may conclude that in any infant with unexplained acute kidney injury and anemia, hyperoxaluria must be considered and kidney biopsy can be conclusive.

Keywords: Primary hyperoxaluria; Anemia; Infant; Acute kidney injury; Renal insufficiency.

Hypermagnesemia and Dilated Cardiomyopathy in a 10-year-old Male Patient on Hemodialysis, A Case Report

Mohsen Akhavansepahi, Rozita Hoseini

Journal of Pediatric Nephrology, Vol. 7 No. 2 (2019), 31 August 2019, Page 1-3

A combination of hypermagnesemia, dilated cardiomyopathy, hypertension, and chronic renal frailer is a rare presentation in children. One of important and rare side effects of chronic renal frailer is hypermagnesemia. In this article, we discuss the association between hypermagnesemia and cardiomyopathy and how patients with none oliguric chronic renal failure develop hypermagnesemia. A 10-year-old boy presented to our PICU ward with chief complaints of respiratory distress, lethargy, and weakness since three days ago. On physical examination, the patient had respiratory distress and was hypertensive and ill.

Keywords: Hypertension; Hypermagnesemia; Cardiomyopathy.

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