Background and Aim:Ureteropelvic junction obstruction (UPJO) is a common cause of hydronephrosis and is typically diagnosed antenatally. About 70% of these cases will self-resolve; however, symptomatic children may present with abdominal pain (i.e. Dietl crisis), vomiting, rash, or fever. Case Presentation:A 14-year-old male patient presented with cyclical vomiting every two months for the past two years. The patient's mother also described an eight-year history of headaches and intermittent abdominal pain. A renal magnetic resonance urography scan revealed decreased right kidney function and delayed cortex to ureter transit time. A pyeloplasty and stent placement were performed to correct the obstructing vessel. Conclusion:Clinical guidelines for abdominal pain and cyclical vomiting earlier in the patients' healthcare may have led to an appropriate workup and treatment years before. We recommend physicians consider UPJO as a differential diagnosis in adolescent patients with cyclical vomiting and abdominal pain and consult the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHN) guidelines to better guide the diagnosis.
Background and Aim: Recent recommendations are to use isotonic fluid as maintenance intravenous fluid (mIVF) in children. The most commonly prescribed fluid is 0.9% NS (normal saline) but there are concerns about hypernatremia, fluid overload and hyperchloremic metabolic acidosis leading to increased morbidity and even mortality mainly due to its adverse effects on kidneys. This study was conducted to assess the clinical and biochemical effects of 0.9% sodium chloride as an isotonic mIVF in the general non-critically ill pediatric patients. Methods: This observational study was conducted on admitted children requiring mIVF for a minimum 24 hours. Changes in the blood pressure and biochemical parameters like serum sodium, serum chloride and bicarbonate were evaluated and the incidence of hypo/hypernatremia, hyperchloremia and metabolic acidosis was calculated at 24 and 48 hours from baseline. Results: Two hundred and fifty children were analyzed. The mean age of the patients was 3.79±3.2 years with majority (43.6%) in the age group 2 months- 1 year. There was no significant change in BP at any time point. The mean serum chloride level was 103.81±4.717, 104.5±4.581, and 105.28±4.545 at baseline, 24 hours and 48 hours respectively with a significant rise at 48 hours of mIVF (p-value< 0.001) and among 3 time points (p-value<0.01]. There was a significant decrease in the bicarbonate level at 48 hours from baseline (p<0.05). Conclusion: According to the results, 0.9% NS as mIVF in non-critically ill pediatric patients causes a significant increase in the serum chloride level leading to hyperchloremia and metabolic acidosis.
Backgroundand Aim:Rituximab is a novel therapy that can help patients with steroid-dependent or resistant nephrotic syndrome.The aim of this study was to evaluate the efficacy of rituximab in children with corticosteroid-dependent and resistant nephrotic syndrome and to determine the factors associated with its efficacy. Methods:In this study, 40 children with corticosteroid-dependent or resistant nephrotic syndrome who were treated with rituximab in Dr. Sheikh Hospital,Mashhad,between 2014 and 2018 were enrolled. Patients with a history of hematuria, severe urinary tract infection, or secondary nephrotic syndrome were excluded. Results:The mean age of patients was 11.9 ± 5.04 years, and 55% were female. The most common underlying pathology of nephrotic syndrome was focal segmental glomerulonephritis (FSGS) (42.5%) followed by membranoproliferative glomerulonephritis (MPGN) and minimal change disease (MCD). Most of the participants (62.5%) were steroid-dependent and the rest (27.5%) were steroid resistant. Only 10% of the patients showed complications following rituximab administration and 57.5% went into complete remission. A negative family history and steroid-dependent nephrotic syndrome were significantly associated with a better treatment response. Moreover, patients with steroid-resistant nephrotic syndrome were more likely to have a positive family history, while factors associated with steroid response included underlying pathology, gender, and family history. Conclusion: Rituximab can cause remission in more than half of the patients with steroid-resistant or dependent nephrotic syndrome. Moreover, the only factors that reduce response to rituximab are a history of corticosteroid resistance and a positive family history of nephrotic syndrome.
Severe gastrointestinal manifestations and rashes of Henoch Schonlein Purpura (HSP) may not always respond to prednisolone or other immunosuppressive agent. Though reduced factor XIII may play a role in gastrointestinal manifestations but its role in management of skin rashes is uncertain. Here, we reported a case treated with different immunosuppressive agents but didn`t respond. Thereafter, despite having the normal factor XIII assay, the unremitting GI symptoms and skin rashes, has improved after prescribing colchicines & infusion of Cryoprecipitate containing factor XIII.
Nephromegaly in childhood can result from a wide variety of causes, yet iron-deficiency anemia has not been previously described. We report a child with severe iron-deficiency anemia associated with transient bilateral nephromegaly. We hypothesize that increased renal production of erythropoietin as well as greater cardiac output with subsequent glomerular hyperfiltration secondary to critical anemia explained the observed nephromegaly. The normalization of kidney size following resolution of anemia supports this hypothesis. Our case report suggests an additional etiology of bilateral nephromegaly in the pediatric population and thus merits attention among physicians.
Background and Aim: Malnutrition is a common disorder worldwide. Growth of visceral organs is affected by various factors including nutrition. Kidney size is an important parameter to assess renal growth. Kidney size in malnourished children is not well studied. We compared kidney size between severe acute malnourished (SAM) and normal children. Methods: This prospective case control study was carried out at a tertiary care center in North India. Children with SAM (n=124) were enrolled as cases and 86 age and sex matched healthy children were enrolled as controls. All children were subjected to anthropometric and sonographic kidney size measurement. Various renal size parameters were compared between cases and controls. Linear correlation coefficients of kidney dimensions with anthropometric parameters were derived. P-values <0.05 were considered significant. Results: The kidney length was significantly lower in malnourished cases compared to healthy controls. There was no difference in renal width and combined kidney volume (CKV) between the two groups. However, renal depth, combined relative kidney volume, CKV/body height ratio, and CKV/BSA ratio were significantly higher in malnourished child compared to healthy controls. Kidney size parameters (kidney length, width, depth, CKV) had significant correlations with anthropometric measurements in both groups but the correlations were stronger in healthy controls compared to malnourished children. Conclusion: Malnourished children had a significantly smaller kidney length. This point should be remembered while evaluating kidney size in these children to avoid unnecessary invasive investigations.
Background: Urinary tract infection (UTI) in neonates might be associated with bacteremia and congenital anomalies of the kidney and urinary tract (CAKUT), that in complicated situations can lead to renal parenchymal scarring and chronic kidney disease. Aim: To determine occurrence of UTI in neonatal sepsis. Methods: This cross sectional study was conducted on 500 term, extramural hospitalized neonates, suspected to have infection underwent, a detailed history, physical examination and a comprehensive sepsis workup. The diagnosis of neonatal UTI was based, on positive urine culture, obtained by suprapubic aspiration (SPA). Results: Out of 500 neonates included in our study (324 boys and 176 girls), blood culture was positive in 84 (36.5%) neonates in early-onset sepsis group (n=230), while in late-onset sepsis group (n=270) blood culture was positive in130 (48.1%), p-value < 0.05. In both early-onset sepsis (EOS) and late-onset sepsis (LOS) groups, blood culture was found to be positive in a greater proportion of male (41.3%) than female (29.3%) patients, p-value <0.05. The most common organism isolated from blood and urine culture was Klebsiella, followed by E-coli. Urine culture was positive in 34 (6.8%) patients, among whom 29 (85%) culture-positive cases were from the LOS group, a p-value of <0.05. Conclusions: UTI is not uncommon among the hospitalized neonates, and UTI evaluation among septic neonates can prove beneficial for the prevention of long-term sequelae of neonatal UTI.
Rhabdoid tumor of the kidney (RTK) mimics other renal tumors histologically, but a rhabdoid tumor mimicking an anaplastic Wilms’ tumor has been rarely reported. Wilms’ tumor classically comprises three histological components, including a blastema, epithel ium, and stroma. The degree of maturation of these components makes the histological appearance of this tumor unique. Anaplasia is defined by the presence of extreme nuclear and mitotic atypia. Most of these lesions can be differentiated on the basis of light microscopic histological investigations. Herein we report a case of pediatric anaplastic Wilms’ tumor that was difficult to differentiate from a mesenchymal lesion histologically. The application of immunohistochemistry and extensive sampling of the lesion was critical for accurate diagnosis. Keywords: Wilm’s Tumor; Child; Rhabdoid Tumor.
Background and Aim: Acute Kidney Injury (AKI) is an important complication of Nephrotic Syndrome (NS) associated with adverse outcome. The frequency of AKI has increased to almost double in the last decade. To determine the incidence of AKI, risk factors and its association with outcome in hospitalized children with NS. Methods – All children aged 1-18 years with diagnosis of NS from 01 November 2018 to 31 May 2020 were enrolled for the study. AKI was diagnosed using the KIDIGO 2012 guidelines and classified according to pediatric RIFLE definition. Results –The mean age of children in this cohort was 4.7± 2.8 years. Complications were observed in 67% cases only. Among all complications the most frequent complication was anemia (25%). The incidence of AKI in hospitalized children with NS was 18.6%. According to pRIFLE criteria of AKI; 11.6% of children met Stage 1 (risk) criteria, 4.6% met Stage 2 (injury) criteria and 2.3% met Stage 3 (failure) respectively. Among all NS children, 53% received nephrotoxic drugs during the hospital stay. On applying multivariate logistic regression analysis, only male gender, associated anemia and Vancomycin use in NS patients came out to be significant independent risk factor for acute kidney injury. Conclusions: AKI is more frequent in first episode of NS rather than in any type of relapses. Although, nephrotoxic drug and male gender are known independent risk factors for development of AKI, associated anemia is till yet not considered as independent risk factor for AKI in children with NS.
A 13-year-old girl was admitted to our hospital because of fatigue, oliguria, and edema of lower extremities. Her laboratory workup showed an acute renal failure (BUN= 225 mg/dl, Creatinine= 13.25 mg/dl). She also had a massive proteinuria. A renal biopsy showed cellular crescentic glomerulonephritis and positive C-ANCA. The serum Creatinine level decreased after treatment with high-dose methylprednisolone, rituximab and plasmapheresis. She did not require dialysis. This is a report of a rare case of GPA presenting with 100% cellular crescentic glomerulonephritis.
Introduction This case-series describes a short term follow-up of Renal Transplantation (RT) in three children at the Zenith Medical and Kidney Centre (ZMKC), Abuja, Nigeria. Methods The immediate allograft function (IAF) and recipients’ survival at 6-24 months of follow-up from January 2018 to January 2020 were described. RT involved the use of basiliximab and thymoglobulin for induction therapy and oral prednisolone, tacrolimus and mycophenolate mofetil for maintenance immunosuppressive therapy. Results Recipients were 9 years to 17 years (average age of 12 years) of age. Primary steroid-resistant focal segmental glomerulosclerosis nephrotic syndrome, congenital posterior urethral stricture and lupus nephritis were the causes of the end-stage kidney diseases. The IAF was excellent in all the cases. All the 3 children are alive with good allograft functions at 6-24 months of the follow-up period. Conclusion This case series shows that a successful paediatric RT programme is feasible in a resource-constrained setting like Nigeria
Background and Aim: Urinary tract infection is one of the most common childhood diseases. The results of studies investigating discontinuation or continuation of antibiotics in children with recurrent urinary tract infections and urinary reflux are controversial. Therefore, this study was conducted to compare prophylactic antibiotic treatment twice a week versus every night in the recurrence of urinary tract infections in children. Methods: This clinical trial was conducted using non-random simple sampling. Group A was given a single daily dose of cephalexin 10 mg/kg and group B was given cotrimoxazole at a dose of 5 mg/kg. Both groups were followed for ten months. Recurrences of urinary tract infections were compared between the two groups. Results: The mean age of the participants was 3.53±2.04 years. Most of the subjects were female (n=37, 61.7%). Urinary reflux was unilateral in 65% of the cases (n=39) and bilateral in the rest. There was no significant difference in age distribution, sex, and type of reflux between groups A and B. The frequency of recurrent urinary tract infection was 8.3% in group A and 6.7% in group B indicating no significant difference (p = 0.500). Conclusion: The results of this study showed that the frequency of recurrence of urinary tract infections in children who received prophylactic antibiotic treatment twice a week was not significantly different compared to the group of children who received continuous antibiotic prophylaxis.
Methemoglobinemia is a rare disease characterized by the elevated levels of methemoglobin in the blood. It may be congenital or acquired. Co-trimoxazole is an antibiotic that belongs to the sulfone group. Sulfone group drugs may produce drug-induced acquired methemoglobinemia. Methemoglobin is an oxidized form of hemoglobin that has an increased affinity to oxygen and a reduced ability to release oxygen to tissues. High levels of methemoglobin inred blood cells cause tissue hypoxia. This disorder may present with several symptoms such as cyanosis, fatigue, dyspnea, and headache. Because it is a rare cause of cyanosis and hypoxemia, the diagnosis of methemoglobinemia is often delayed. We herein discuss a five-year-old boy with steroid-resistant nephrotic syndrome who presented with exertional dyspnea and cyanosis and was later diagnosed as a case of co-trimoxazole-induced methemoglobinemia.
Background and Aim: Nephrotic syndrome (NS) is a common disease in children characterized by proteinuria, hypoalbunemia and hyperlipidemia. In some cases, NS is resistant to steroid therapy and may have frequent relapses. The aim of this study was to determine the prognostic role of gestational age and birth weight in the clinical outcomes of NS. Methods: This retrospective cross-sectional study was conducted on 77 patients. The patients’ data such as history of relapse and steroid resistance, birth weight, gestational age, and pathological variant were collected. Data was analysed using the SPSS software. Results: Twenty-three patients were females and 54 were males. There was no significant association between the number of recurrences and premature birth (P value= 0.99). Mann-Whitney U test showed no significant difference between birth weight of patients who recurred less than two times during six months and those who recurred more than two times in six months (P= 0.336). Besides, Fisher’s exact test showed no significant association between premature birth and the chance of developing steroid resistance (P value = 0.643). Moreover, there was no significant association between birth weight and steroid resistance (P-value = 0.768). Conclusion: Low birth weight and premature birth did not have a role in the prognosis of nephrotic syndrome in our study population. Other factors including uterine-placental disorders, maternal underlying diseases, quality of weight gain in the first years of life, and ethnicity should also be examined in further investigations including larger samples of different ethnic groups.
Cerebral salt wasting syndrome (CSWS) is an important cause of persistent hyponatremia in children admitted to the intensive care unit. It needs to be promptly differentiated from the syndrome of inappropriate antidiuretic hormone (SIADH) secretion as a cause of hyponatremia in pediatric neurological patients. These two entities often have similar presenting symptoms however the treatment of both can be drastically different, which makes the distinction critical. We present a 6-month-old male child with hydrocephalus secondary to aqueductal stenosis, a blocked VP shunt, meningitis, and hyponatremia. A diagnosis of CSWS was considered and fludrocortisone was started. The patient improved gradually with a reduction in urine output and a gradual increase in serum sodium levels. A diagnosis of CSWS should be strongly considered in hyponatremic pediatric patients with significant natriuresis and suitable treatment should be initiated promptly to prevent long-term neurologic sequelae.
Coronavirus disease 2019 (COVID-19) may present as acute abdomen although the pathophysiology remains obscure. This is a report of an 11-year-old boy, presented to the emergency department at our hospital with myalgia and gastrointestinal symptoms including non- bloody diarrhea and abdominal pain associated with nausea and intractable vomiting during the course of the disease. He also had a fever and dry cough. On initial evaluation, his vital signs were unremarkable. Oxygen saturation was 92% in the room air. His temperature was 38˚C and his respiratory rate was 17/min. An initial physical examination showed abdominal tenderness in the right lower quadrant. Laboratory analysis revealed leukocytosis and a high ESR and CRP. Therefore, he underwent appendectomy and was discharged after a few days in good condition by observing health protocols.
A 3- year- old boy presented with abdominal pain and distension, lower extremities and facial edema, and anuria for several days that was associated with ureteropelvic junction obstruction (UPJO). Initial ultrasonography showed the presence of a massive bilateral hydronephrosis with UPJO and a computerized tomography (CT) scan without contrast revealed a massive hydronephrosis and hydroureter (grade 3). A follow-up study, after relief of ureteral obstruction, showed the reversal of this pattern. Blood biochemical tests revealed a severe acidosis, hyperkalemia, and a BUN/Cr ratio of more than 20. The patient underwent emergency nephrostomy. The general condition of the patient improved. Abdominal distention reduced, levels of urea and creatinine decreased, and acidosis resolved. A CT scan revealed an almost complete disappearance of the hydronephrosis and a normal ureter. Ureteropelvic junction obstruction (UPJO) was established as the cause of giant hydronephrosis.
Background and Aim: Quality of life has been improved by kidney transplantation (KT), especially in pediatric patients. Children have a higher risk of rejection and graft loss compared to adults. It is important to determine the pediatric graft outcome and prevent early graft loss, if possible. This study was done to evaluate graft and patient survival in pediatric patients after kidney transplantation. Methods: This cross-sectional study was done on all children below 18 years old that underwent kidney transplantation in Shahid Beheshti Kidney Transplant Center. Sex, time of transplantation, serum creatinine, acute rejection, viral infection (cytomegalovirus), and graft survival and patient survival were recorded. Results: Fifty-eight patients underwent kidney transplantation. The mean serum creatinine level was 0.89±0.88 mg/dl, 0.7±1.29 mg/dl, 058±0.92 mg/dl, 0.9±0.83 mg/dl and 1.32±0.91 mg/dl on day 1, 2, and 3 post KT, discharge day, and the last follow-up visit, respectively. Graft survival was 93.1%, 89.5% and 86.5% at 1, 5 and 10 years, respectively. During the study, five patients expired and therefore patient survival was 99.6%, 92.3% and 92.3% at 1, 5 and10 years, respectively. Conclusion: This study, compared to other studies, showed that the graft and patient survival were acceptable. More attention should be paid to improve the outcome.
