Comparison of Urinary Biomarkers in Diagnosis of Ureteropelvic Junction Obstruction and Differentiation from Nonobstructive Dilatation.

Background and Aims: Differentiation of nonobstructive dilatation (NOD) from ureteropelvic junction obstruction (UPJO) is a challenge in children with antenatally detected hydronephrosis. The aim of this study is to compare the utility of urinary biomarkers: carbohydrate antigen (CA 19-9), neutrophil gelatinase-associated lipocalin (NGAL), and kidney injury molecule (KIM) in diagnosing UPJO. Methods: A prospective study was conducted after obtaining ethical clearance between 2021 and 2022. Group 1 - control group (n = 30): children with normal antenatal ultrasound with no urinary symptoms. Group 2 - study group (n = 48): children with unilateral hydronephrosis: Group 2a - NOD (n = 24): children stable on ultrasound and diuretic renogram and Group 2b - UPJO (n = 24): children who worsened to Grade 4 hydronephrosis on ultrasound/worsening of differential renal function (10% drop) on renogram who underwent pyeloplasty. Urinary biomarkers NGAL, KIM-1, and CA 19-9 were measured using the enzyme-linked immune absorbent assay method. Results: The urine CA 19-9 level was 128.05 ± 4.08 U/mL in the UPJO group, and this was significantly higher (P = 0.001) than NOD, 70.29 ± 4.41, and controls, 1.91 ± 1.57. The urine NGAL level was 21.41 ± 4.44 pg/mL in UPJO, and this was significantly higher than controls, 2.669 ± 0.513, but not NOD, 24.55 ± 2.67. The urine KIM level was 817 ± 15.84 pg/mL in the UPJO group, and this was significantly higher than controls, 285 ± 8.10, but not NOD, 768.23 ± 15.12. Receiver operating characteristic analysis of CA 19-9 revealed a urine biomarker cutoff of 95 U/mL for diagnosing UPJO (sensitivity 95%; specificity 96%; and area under the curve 0.99). Conclusions: CA 19-9 is a superior marker compared to NGAL and KIM in differentiating UPJO from NOD. Further studies with larger numbers are warranted.

Differential urinary microRNA expression analysis of miR-1, miR-215, miR-335, let-7a in childhood nephrotic syndrome.

BACKGROUND: Recently, urinary exosomal miRNAs are gaining increasing attention as their expression profiles are often associated with specific diseases and they exhibit great potential as noninvasive biomarkers for the diagnosis of various diseases. The present study was aimed to evaluate the expression status of selected miRNAs (miR-1, miR-215-5p, miR-335-5p and let-7a-5p) in urine samples from children with NS [steroid sensitive (SSNS)] and [steroid resistant (SRNS)] along with healthy control group. METHODS: MicroRNA isolation was carried out in urine samples collected from SSNS (100 nos), SRNS (100 nos), and healthy controls (50 nos) using MiRNeasy Mini Kit, followed by cDNA conversion for all the four selected miRNAs using Taqman advanced miRNA cDNA synthesis kit and their expression was quantified by Taqman Advanced miRNA assay kits using Real Time PCR Machine and Rotogen-Q in SSNS and SRNS patients and healthy control subjects. RESULTS: Quantification of all the four miRNAs (miR-1, mir-215, miR-335, let-7a) were found to be upregulated in both SSNS and SRNS as compared to control group. Further, the comparison of microRNAs within the case groups revealed significant downregulation of three microRNAs-miR-1, miR-215, miR- 335 and upregulation of let-7a in SRNS group as compared to SSNS. The t-test performed for all the four miRNAs was found to be statistically significant. CONCLUSIONS: The aberrant expression of all the four microRNAs in both SSNS and SRNS as compared to healthy subjects may serve as novel biomarkers to distinguish between NS and healthy controls. The differential expression of microRNA let-7a is useful to discriminate SSNS and SRNS.

The Association Between Vitamin D and Urinary Tract Infection in Children: A Case-Control Study.

Background and objective Urinary tract infection (UTI) is one of the common causes of febrile illness in young children. Vitamin D influences the levels of endogenous cathelicidin, an antimicrobial peptide, which improves bladder wall immunity and prevents UTIs. In light of this, we conducted this study to determine the association between vitamin D deficiency and UTIs in children and to identify whether vitamin D deficiency is one of the risk factors for UTIs. Materials and methods This was a case-control study of children aged between one to five years. Eighty-two children with the first episode of febrile culture-proven UTI as cases and 82 healthy children as a control group were included in this study. The sera were analyzed for 25-hydroxy vitamin D levels and classified as vitamin-D deficient if their level was below 30 ng/mL. Descriptive statistics were presented as numbers and percentages. Continuous data were expressed as means and standard deviations (SD). Pearson's chi-square test was used to test the significance of the differences in variables between the two groups. Multiple logistic regression equation methods were used to predict the relationship between the dependent and independent variables. Results The mean age of the study and the control group was 2.36 ±1.42 years and 2.57 ±1.26 years, respectively. The mean serum 25-hydroxy vitamin D levels in the patients and controls were 24.27 ±9.70 ng/mL and 31.97 ±10.7 ng/mL (p<0.001), respectively. Vitamin D deficiency was present in 34 (41.5%) patients and 10 (2.2%) in the control group (p<0.001). Conclusion Based on our findings, vitamin D deficiency might be one of the risk factors for UTIs in children. Vitamin D deficiency is significantly associated with febrile UTIs in children between one to five years of age.

Secondary Hemophagocytic Lymphohistiocytosis - A Common Ramification of Different Diseases.

Hemophagocytic lymphohistiocytosis is a rare and potentially fatal disorder caused by immune dysregulation. It can occur as a primary genetic disease or secondarily due to various causes including infections, malignancies or autoimmune diseases. In this case report, we present two cases of Hemophagocytic lymphohistiocytosis which were secondary to typhoid and dengue fever. While primary disease occurs predominantly in infants, secondary hemophagocytic lymphohistiocytosis can occur in any age group. Both primary and secondary hemophagocytic lymphohistiocytosis are characterised by fever, hepatosplenomegaly, pancytopenia and multiorgan dysfunction. But unusual persistence of fever and other organ involvement should need further workup for hemophagocytic lymphohistiocytosis. Secondary hemophagocytic lymphohistiocytosis may resolve on treating the underlying disorder. But severe cases need treatment with immunosuppressive/immunomodulation therapy to prevent morbidity. Early clinical suspicion, prompt diagnosis and treatment of hemophagocytic lymphohistiocytosis are essential to prevent deleterious effects to health.

Analysis of Clinicopathological Characteristics and Its Correlation With the Prognosis of Pediatric Lupus Nephritis: A Tertiary Care Center Experience.

Aim To study the various pathological patterns of pediatric lupus nephritis (LN) by renal biopsies and to correlate the histopathological data with the clinical and biochemical outcomes. Methods This is a retrospective study in children between 1 month and 18 years of age with renal biopsy-proven lupus nephritis, conducted between January 2015 and December 2019. Various pathological and clinical parameters were compared between the groups with lupus nephritis activity and those without activity. Results Of 38 biopsy-proven lupus nephritis cases, 30 (78.9%) were in the adolescent age group, and the female gender was predominantly affected (n=30; 78.9%). Class IV proliferative lupus nephritis (n=17, 44.7%) was the most common biopsy finding, and the activity score for endocapillary hypercellularity, neutrophil infiltration, fibrinoid necrosis, hyaline deposits, and interstitial inflammation was significantly high in classes III and IV. Overall, attaining remission was less, and the risk of progression of chronic kidney disease (CKD) was higher in class IV (n=3; 7.8%). Mortality was reported in 1 out of 38 (2.6%) children. Conclusion Light microscopy and immunofluorescence studies play an important role in defining the extent of renal damage in the form of activity and chronicity indices, which are the key factors in the decision-making of lupus nephritis treatment. The prognostic relevance of the histological scoring has been evaluated, and it is evident that the activity index and chronicity index go a long way in therapeutic intervention.

Nephrotic Syndrome and Hodgkins Lymphoma - an Unusual Association.

Background: An association between nephrotic syndrome and extrarenal neoplasia was described for the first time in 1922. The reported incidence of nephrotic syndrome in Hodgkin lymphoma is less than 1%. Clinical description: We present a 13 year old boy who was admitted with complaints of abdominal pain, vomiting and loose stools for 2 months. He had a history of significant weight loss of 5kg in a couple of months.On examination, he had bilateral pedal oedema and right cervical lymphadenopathy. Cervical lymph node biopsy revealed nodular sclerosis type of Hodgkin lymphoma. He also had hypoalbuminemia, massive proteinuria and hypercholesterolemia.Secondary nephrotic syndrome due to Hodgkin's lymphoma was made as a clinical diagnosis. Management and outcome: He had been started on chemotherapy (with Prednisolone, Vincristine, Doxorubicin, Etoposide) for stage 3B Hodgkin lymphoma. He tolerated the chemotherapy well. Though he had symptomatic edema, managed conservatively as the urine output was adequate. On follow up, he attained spontaneous remission of nephrotic syndrome. Conclusion: Overt proteinuria might be the manifestation of paraneoplastic syndrome in children with Hodgkin lymphoma and with the management of the primary disease, proteinuria resolves spontaneously.

A study on parental awareness of feeding practices in children in the age-group 12-24 months.

Introduction: Nutrition plays an integral part in growth and development of a child. Age-appropriate feeding is known to improve the child's well-being and reduce the risk of specific diseases. The present study aimed to assess the awareness of parents regarding breastfeeding and complementary feeding practices. Methodology: This health-based prospective observational study was conducted in a tertiary care hospital enrolling 95 parents with children in the age group 1-2 years. The data was analyzed using SPSS version 26 and Microsoft excel. Results: In the present study, the prevalence of exclusive breastfeeding was 73.68%. Eighty-six (90.53%) parents initiated complementary feeds at 6 months. However, only 45.26% of children were consuming adequate quantity of complementary foods. The association of child's calorie consumption with maternal age and occupation was found to be statistically significant. Conclusion: Adequate nutrition during childhood and infancy is a key factor influencing growth and development. In the present study, the overall breastfeeding and complimentary feeding practices were satisfactory. However, the quantity of complementary feeding was inadequate. Counselling the mothers on appropriate breastfeeding and complementary feeding practices during antenatal and postnatal visits may have a positive impact on infant feeding practices.

A Puffy Child - A Rare Case of Steroid Resistant Nephrotic Syndrome with ANLN Mutation.

Recent advances in genomics have uncovered the molecular mechanisms involved in the broad spectrum of variation associated with steroid-resistant nephrotic syndrome. Over 50 monogenic causes of steroid-resistant nephrotic syndrome have been discovered; however, these genes are implicated in only a small proportion of cases. Using a combination of whole-exome sequencing and genome-wide linkage studies, a missense mutation in anillin (ANLN) has been identified as a cause of focal segmental glomerulosclerosis, a pattern of glomerular injury associated with steroid-resistant nephrotic syndrome. We report a case of 2-year-6-month-old male child, who presented with severe edema and oliguria for 6 weeks. He was found to be an early steroid non-responder, hence renal biopsy and genetic testing were ordered. These findings were in favour of focal segmental glomerulosclerosis, a common cause of childhood steroid-resistant nephrotic syndrome. It is important to identify the causative agent to avoid unnecessary immunosuppressive therapy and its associated risks.

Asymptomatic Hypernatremia in an Infant with Midline Defects.

Holoprosencephaly is a developmental abnormality caused due to incomplete cleavage of the rostral neural tube (basal forebrain) structures during early embryogenesis. This defect causes incomplete separation of the right and left cerebral hemispheres. Children manifest a wide spectrum of clinical manifestations, the extent of which depends upon the degree of hemispheric nonseparation. We describe an infant with midline cleft referred for preoperative evaluation in whom, asymptomatic electrolyte abnormalities and holoprosencephaly were identified. On further evaluation, the infant was diagnosed to have isolated central diabetes insipidus and she responded well to oral desmopressin therapy. Cleft lip and palate is one of the commonest congenital malformations and midline clefts are likely to be associated with significant pituitary abnormalities. Awareness about the syndromic associations with clefts and the associated anomalies are important for early diagnosis and intervention in these children.

Renal Manifestations in Children with Dengue Fever Hospitalized in Pediatric Intensive Care Unit.

OBJECTIVE: To study the pattern of acute kidney injury in children with dengue infection hospitalized in an intensive care unit. METHODS: This is a retrospective study from January 2019 through December 2019. Various renal manifestations of dengue were studied and compared between the severity of dengue fever. RESULTS: Three hundred nineteen children with dengue fever were hospitalized and 127 needed intensive care admission. Among the 127 patients, 26 (20.5%) children developed acute kidney injury (AKI). Children with severe dengue developed a higher number of AKI (n = 20; 28.6%), as compared to dengue with warning sign group (n = 6; 11.8%). Colloid infusion, inotropic support, ventilatory requirement and presence of secondary hemophagocytic lymphohistiocytosis were the risk factors for AKI. Nine children underwent dialysis. Among the AKI group, 23 recovered and 3 died and all three had multi organ dysfunction syndrome. CONCLUSIONS: It is essential to recognize the various renal manifestations of dengue AKI which is associated with increased mortality and morbidity.

Efficacy of levamisole in children with frequently relapsing and steroid-dependent nephrotic syndrome.

OBJECTIVE: To assess the efficacy of levamisole in frequently relapsing nephrotic syndrome and steroid-dependent nephrotic syndrome. STUDY DESIGN: Retrospective analysis of hospital case records. SETTING: Pediatric nephrology department of a tertiary referral pediatric hospital. PARTICIPANTS: 62 children with frequently relapsing nephrotic syndrome and 35 children with steroid-dependent nephrotic syndrome. METHODS: Case records of children who were diagnosed as steroid-dependant or frequently-relapsing nephrotic syndrome from June 2004 to June 2011, were reviewed. Levamisole was given daily (2 mg/kg/d) along with tapering doses of alternate day steroids after remission on daily steroids. RESULTS: Levamisole was effective in 77.3% children with a better (80.6%) efficacy in frequently relapsing nephrotic syndrome. A total of 34 children completed 1 year follow-up post levamisole therapy. The cumulative mean (SD) steroid dose 1-year before therapy was 4109(1154) mg/m2 and 1-year post therapy was 661 (11) mg/m2 (P<0.001). The relapses were also less during the period of post-levamisole therapy. CONCLUSION: Levamisole is an effective alternative therapy in frequently relapsing and steroid-dependent nephrotic syndrome.