Extensive perineal ecthyma gangrenosum in leukocyte adhesion deficiency type 1 associated with Staphylococcus hominis bacteremia.

Leukocyte adhesion deficiency (LAD), a disorder of neutrophil function, is characterized by a defect in leukocyte adhesion to the endothelium. Recurrent infections in the skin, soft tissue, gingiva, and lungs due to Staphylococcus aureus, Pseudomonas aeruginosa, and Klebsiella sp. are common in these patients. Ecthyma gangrenosum (EG) is an ulcer of skin and subcutaneous tissue with a black eschar and surrounding erythematous halo secondary to a bacterial infection. Here, we report an unusual presentation of LAD type-1 with extensive EG of perineum secondary to Staphylococcus hominis bacteremia treated successfully with combination of granulocyte transfusion and diversion colostomy.

Management dilemma in pelvi-ureteric junction obstruction: is transit time the answer?

PURPOSE: Pelvi-ureteric junction obstruction (PUJO) is the leading cause for hydronephrosis in children. Diuretic renography used in the pre- and post-operative evaluation of pyeloplasty may not be reliable in predicting the outcome. METHODS: A prospective study was conducted from October 2018 to November 2020, including all children with unilateral PUJO undergoing Pyeloplasty. Renal diuretic renography scans done in the pre and post operative periods were assessed for transit time. The time taken for the isotope to reach the renal pelvis was noted by a single observer as the transit time, from the dynamic frames of the diuretic renogram. RESULTS: Of the 65 children, 74% were boys. The transit time (median) in the preoperative renography was 10 min, whereas the same was 4 min 45 s in the postoperative period, the decrease was statistically significant. On evaluation of transit time with ROC (receiver operating characteristics) curve, a cut-off of 4 min and 25 s was arrived at, which could identify the delayed drainage reliably. CONCLUSION: Improvement in transit time is a reliable predictor of renal function improvement after pyeloplasty, comparing favorably in relation to split renal function.

Recurrent Hemangioma of the Penile Urethra - An Unusual Etiology of Gross Hematuria Managed Successfully with Holmium Laser Coagulation.

Urethral hemangioma is a benign uncommon vascular tumor. It presents with hematuria and macroscopic urethrorrhagia. Urethrocystoscopy is the main diagnostic modality. We share the case of a 14-year-old male child who presented with recurrent massive hematuria and was treated with urethra-cystoscopic holmium laser coagulation.

Assessment of Antenatal and Postnatal Prognostic Indicators in the Outcome of Neonatal Congenital Diaphragmatic Hernia: A Prospective Observational Study.

Context: Despite advances in neonatal intensive care, surgical methods, and anesthesia, congenital diaphragmatic hernia (CDH) is still associated with significant mortality. Predicting which babies will have poorer outcomes is essential to identify the high-risk babies and to give targeted care and accurate prognosis to the parents, especially in a resource crunch set-up. Aims: The aim of this study is to evaluate the antenatal and postnatal prognostic factors in neonatal CDH that can be used to predict the outcome. Settings and Design: This was a prospective observational study in a tertiary care center. Subjects and Methods: Neonates presented with CDH within 28 days of life were included in the study. Bilateral disease, recurrent diseases, and babies operated outside were excluded from the study. The data were collected prospectively, and babies were followed until discharge or death. Statistical Analysis Used: Data were expressed in mean with standard deviation or median with range based on normality. All the data were analyzed using the SPSS software version 25. Results: Thirty babies with neonatal CDH were studied. There were three right-sided cases. The male-to-female ratio was 2.3:1, and 93% of babies were antenatally diagnosed. Seventeen out of the 30 babies underwent surgery. Nine (52.9%) underwent laparotomy, and 8 (47%) underwent thoracoscopic repair. Overall mortality was 53.3%, and operative mortality was 17.6%. Demographic characteristics were comparable between expired versus survived babies. The significant predictors of outcome identified were - Persistent pulmonary hypertension (PPHN), mesh repair, high-frequency oscillatory ventilation (HFOV), use of inotropes, 5-min APGAR, ventilator index (VI), and HCO3 levels. Conclusions: We conclude that the prognostic indicators associated with poor prognosis are low 5-min APGAR, high VI, low HCO3 levels in venous blood gas analysis, mesh repair, HFOV, inotropes usage, and PPHN. None of the antenatal factors studied showed any statistical significance. Further prospective studies with a larger sample size are recommended to confirm the findings.

Utility of Urinary Biomarkers Neutrophil Gelatinase-Associated Lipocalin and Kidney Injury Molecule-1 as a Marker for Diagnosing the Presence of Renal Scar in Children with Vesicoureteral Reflux (VUR): A Cross-Sectional Study.

Aim: To explore the possibility of using urinary biomarkers neutrophil gelatinase-associated lipocalin (NGAL) and kidney injury molecule-1 (KIM-1) to assess the presence of renal scars in children with Vesicoureteric Reflux (VUR). Materials and Methods: This cross-sectional study was conducted in 94 children aged 0-16 years diagnosed with VUR in the Department of Pediatric Surgery, JIPMER. Urinary biomarkers were measured using the enzyme-linked immunosorbent assay kits, normalized with urinary creatinine (Cr) and compared with severity of VUR (low grade [I and II] and high grade [III, IV, and V]), presence or absence of renal scar in VUR patients and severity of renal scar. Independent Student's t-test, Mann-Whitney U-test, and analysis of variance Kruskal-Wallis test were used for comparison, and receiver operating characteristic (ROC) curve analysis for predicting the accuracy of biomarkers in detecting the presence of renal scars. Results: The median urinary NGAL (uNGAL) value was higher in children with renal scar (1.49 ng/mL) than those without renal scar (0.58 ng/mL) and was statistically significant (<0.001). Whereas median uNGAL/Cr was higher in children with renal scar (0.07) than those without renal scar (0.03) but was not statistically significant (P = 0.06). Urinary KIM-1 and urinary KIM-1/urinary Cr (uKIM-1/Cr) was not found to be a significant predictor of renal scar. The difference of uNGAL/Cr was comparable between the grades of renal scar but was not statistically significant. On ROC curve analysis, uNGAL had area under the ROC curve (AUC) of 0.769 with 71% of both specificity and sensitivity, whereas uNGAL/Cr was found to be a poor predictor of renal scar with AUC of 0.611, 60% sensitivity, and 61.2% specificity. Conclusion: uNGAL can serve as a noninvasive marker for diagnosing the presence of renal scar in children with VUR and a multicentric more extensive cohort study may be needed to strengthen or negate its role.

Fetus in Fetu: Case Report and Brief Review of Literature on Embryologic Origin, Clinical Presentation, Imaging and Differential Diagnosis.

BACKGROUND: Fetus in fetu (FIF) is a rare entity in which a malformed diamniotic monochorionic parasitic fetal twin develops inside a normal co-twin's body, most commonly in the abdominal cavity. FIF is differentiated from the teratoma by the presence of vertebral column often with an appropriate arrangement of other organs or limbs around it. CASE REPORT: A two-and-a-half-year-old girl presented with a painless abdominal swelling in the right hypochondrium. On imaging, a heterogenous soft tissue mass with internal calcific densities was noted in the retroperitoneum. The mass had vertebral organization, limb and pelvic bones. The presence of a fetiform teratoma was suspected and surgery revealed an encapsulated mass with an anencephalic head, spine, upper and lower limb buds. Histopathology confirmed the presence of a fetus in fetu. The postoperative period was uneventful with no evidence of recurrence. CONCLUSIONS: FIF is a pediatric rarity. Cross-sectional imaging helps in differentiating it from a teratoma, meconium peritonitis and abdominal ectopic pregnancy. Surgical excision is the treatment of choice for this benign condition, which requires a follow-up only in certain cases. This case report describes a retroperitoneal fetus in fetu and discusses its clinical presentation, differential diagnosis and embryologic origin.

Malignant rhabdoid tumor of liver.

Malignant rhabdoid tumor (MRT) is a rare, but aggressive tumor commonly arising from the kidney in young children. Extrarenal MRT has been reported in the literature in various other sites including the liver, pelvis, CNS, abdomen, heart and other soft-tissues. Reported herein are the presentation, radiology, histopathology, immunohistochemistry, treatment and outcome of a 6 month infant with primary MRT of liver.

Comparison of diagnostic accuracy of models combining the renal biomarkers in predicting renal scarring in pediatric population with vesicoureteral reflux (VUR).

INTRODUCTION: Renal scarring is prominently observed in children with vesicoureteral reflux (VUR) and can lead to complicated renal outcomes. Although biopsy is the gold standard to detect renal scarring, it is an invasive procedure. There are established renal biomarkers which can help detect renal scarring. Individual biomarkers have not shown to have extensively good discriminatory ability for this. AIM: This paper aims at combining the values of multiple biomarkers in models to detect renal scarring. METHODOLOGY: Secondary data with the values of renal biomarkers like kidney injury molecule-1, neutrophil gelatinase-associated lipocalin (NGAL), and urinary creatinine along with the renal scarring status was considered. Logistic regression, discriminant analysis, Bayesian logistic regression, Naïve Bayes, and decision tree models were developed with these markers. The discriminatory ability of individual biomarkers along with the models was assessed using the area under the curve from ROC curve. Sensitivity, specificity, and misclassification rates were estimated and compared. RESULTS: NGAL was the most predominant renal biomarker in classifying the patients with renal scarring (AUC: 0.77 (0.67, 0.87); p value < 0.001). Each of the model performed better than individual biomarkers. Decision tree (AUC: 0.83 (0.74, 0.91); p value < 0.001) and Naïve Bayes model (misclassification rate = 20.2%) performed the best amongst the models. CONCLUSION: Combining the values of renal biomarkers through a statistical or machine learning model to detect renal scarring is a better approach as compared to considering individual renal biomarkers.

Utility of Ischemia-Modified Albumin as a Biomarker for Acute Appendicitis: A Systematic Review and Meta-Analysis.

BACKGROUND: Acute appendicitis is a frequently encountered surgical emergency. Despite several scoring systems, the possibility of delayed diagnosis persists. In addition, a delayed diagnosis leads to an increased risk of complicated appendicitis. Hence, there is a need to identify biological markers to help clinicians rapidly and accurately diagnose and prognosticate acute appendicitis with a high sensitivity and specificity. Although several markers have been evaluated, the pressing concern is still the low specificity of these markers. One such marker is serum ischemia-modified albumin (IMA), which can be a novel biomarker for accurately diagnosing and prognosticating acute appendicitis. METHODS: The authors conducted a systematic search of the PubMed, EMBASE, Web of Science, and Scopus databases through February 2023 as per the PRISMA guidelines. The difference in the levels of IMA between patients with acute appendicitis vs. healthy controls, and the difference in the levels of IMA between patients with complicated vs. non-complicated acute appendicitis were taken as the outcome measures. Statistical analysis was performed using a random effects model and mean difference (MD) was calculated. The methodological quality of the studies was assessed by utilizing the Newcastle-Ottawa scale. RESULTS: A total of six prospective comparative studies were included in the meta-analysis. The analysis revealed that the mean level of serum IMA was significantly raised in the acute appendicitis group (MD 0.21, 95% CI 0.05 to 0.37, p = 0.01). Similarly, the mean serum IMA levels were also raised in the complicated appendicitis group compared to the non-complicated appendicitis group (MD 0.05, 95% CI 0.01 to 0.10, p = 0.02). Three of the studies included were, however, of poor methodological quality. CONCLUSIONS: Serum IMA is a viable potential marker for diagnosing and prognosticating acute appendicitis. However, due to the limited methodological quality of available studies, further prospectively designed and adequately powered studies are needed.

Oncocytic adrenal cortical neoplasm of uncertain malignant potential - A rare case report.

Adrenal oncocytic tumors are rarely encountered. Most of the oncocytic tumors are benign and rarely malignant. Here we report a case of a 10-year-old male child presented with abdominal mass whose Contrast-Enhanced Computed Tomography (CECT) shows well-circumscribed tumor. Right adrenalectomy was carried out. Histopathologically, it was diagnosed as adrenal cortical oncocytic neoplasm of uncertain malignant potential (AONUMP). This case highlights that although adrenal oncoytic tumors are rare, we should consider this as a differential diagnosis while evaluating the patient for adrenal masses. The case is presented in view of its rarity, and distinguishing gross and microscopy appearance, the diagnosis of which was further confirmed with immunohistochemistry markers.

Calyx to Parenchymal Ratio (CPR): An unexplored tool and its utility in the follow-up of pyeloplasty.

INTRODUCTION: Antero-posterior trans pelvic diameter (APD) and renal scintigraphy play a significant role in the diagnosis of pelvi-ureteric junction (PUJ) obstruction and postoperative follow-up following pyeloplasty. However, the APD varies irrespective of improvement, deterioration, or preserved function in a hydronephrotic kidney and is not a reliable parameter due to various factors (hydration status, compliance, and reduction pyeloplasty). Calyx to Parenchymal Ratio (CPR) is the ratio of the depth of the calyx and parenchymal thickness measured on ultrasound (USG) in coronal image. We assessed the utility of CPR in the follow up of pyeloplasty and compared it with the commonly used APD of the pelvis and renal scintigraphy. MATERIAL AND METHODS: A prospective cohort study was done from July 2016 to October 2017. During this period 73 pyeloplasties were done, and 62 cases meeting the inclusion criteria were enrolled. All the children underwent ultrasound and Technetium-99 m Ethylene dicysteine isotope renogram (EC) scan before and after pyeloplasty. APD and CPR values were measured on USG and compared with isotope renogram outcomes in these children in the preoperative versus postoperative period. Two defined objective variables ΔAPD, percent ΔAPD and ΔCPR, percent ΔCPR were compared with categorical variables that would predict the surgical outcome as - failed, successful or equivocal. Multinomial logistic regression analysis and receiver operating curve (ROC) analysis was used to identify predictive accuracy. RESULTS: The mean (range) APD value recorded in the preoperative period was 3.67 cm (1.40-8.00 cm), which decreased to 1.67 cm (0.40-6.50) postoperatively, which was 54.2% lower (P=<0.001). The mean (range) CPR value decreased from 5.96 (1.20-20.00) in the preoperative period to 2.57 (0.43-10.90) postoperatively, which was 56.8% lower (P=<0.001). On multinomial logistic regression analysis, ΔCPR was found to be a significant predictor of outcome with an overall accuracy of 95.1%, change in CPR was a better predictor of success after pyeloplasty as compared to change in APD, which had an overall accuracy of 85.2% (p = 0.01). Further, on ROC curve analysis, we observed that ΔCPR and %ΔCPR can strongly predict successful pyeloplasty with a sensitivity of each with 96% and 98% respectively and AUC of 0.897 and 0.799 respectively. DISCUSSION: USG (APD) and renogram are the most widely used investigation in follow-up of pyeloplasty; however, APD has its own limitations like operator variability and slower improvement. CPR has the advantages that neither calyceal depth nor parenchymal thickness is directly altered during the surgery, and early resolution of calyceal dilatation and rapid parenchymal growth following pyeloplasty and thus a surgeon independent parameter. Our results have shown that ΔCPR can identify successful pyeloplasty with strong prediction than ΔAPD and thus renal scans can be avoided if there is visible improvement in CPR on follow-up. CONCLUSIONS: Our study identified a change in CPR, i.e., ΔCPR as a strong predictor of surgical outcome, as it is not influenced by extent of pelvis reduction during pyeloplasty and early to change. Using this parameter, we can avoid unnecessary repeated nuclear scans based on persistent high APD values and optimize resource utilization. We recommend the use of CPR in routine practice in the preoperative and postoperative follow-up of PUJ obstruction following pyeloplasty.

Screening for Renal and Urinary Tract Anomalies in Asymptomatic First Degree Relatives of Children with Congenital Anomalies of the Kidney and Urinary Tract (CAKUT).

OBJECTIVE: To estimate the frequency of renal and urinary tract anomalies in first-degree relatives of children with Congenital anomalies of kidney and urinary tract (CAKUT). METHODS: This descriptive study was conducted on parents and siblings of 138 children with CAKUT. Renal ultrasonogram, radionuclide diuretic renogram and micturating cysturethrogram were the tools used for screening these family members. RESULTS: Asymptomatic first-degree relatives of 138 children [total of 270 first-degree relatives (95 fathers, 97 mothers and 78 siblings)] were screened, with new anomalies detected in 11 first-degree relatives (4% out of 270 first-degree relatives screened) from 11 families (7.9% out of 138 families screened). The anomalies detected were vesicoureteric reflux (VUR) (n = 2), non-obstructive non-refluxing hydronephrosis (n = 2), pelviureteral junction obstruction (PUJO) (n = 3), Duplex collecting system (n = 1), hypodysplastic kidney (n = 1), single kidney (n = 1) and horseshoe kidney (n = 1). Most of the anomalies were discordant to the index anomaly (66.6%). Among 95 fathers screened, 5 (5.2%) had renal anomalies. Among 97 mothers screened, 2 (2.1%) had renal anomalies. Among the 78 siblings screened, 4 (5.1%) had renal anomalies. CONCLUSIONS: Familial clustering was noted in 7.9% of the 138 families (of the index cases) screened. The anomalies detected were mostly discordant to the index anomaly.

Use of fosfomycin combination therapy to treat multidrug-resistant urinary tract infection among paediatric surgical patients - a tertiary care centre experience.

With increasing resistance to currently used antibiotics, antibiotic combinations are being resorted to. The present study deals with five children with complicated urinary tract infection (UTI) whose urine cultures grew multidrug-resistant (MDR) organisms. In all of these five cases, MDR organisms were the causative agents for UTI and the currently available antibiotics, including colistin, were ineffective, although the organisms were sensitive in vitro. In all of these cases, the isolates reverted to being susceptible to the quinolones and cephalosporins tested, namely ceftriaxone and ceftazidime. All were treated using a combination of fosfomycin with other antibiotics, since it has no interference with other classes of antibiotics. Our observations suggest that the use of a combination of fosfomycin with either a carbapenem or an aminoglycoside in a clinical setting would be a reasonable choice to treat UTIs caused by MDR organisms, especially in complicated cases that require chronic therapy.

Bilateral primary renal lymphoma with orbital metastasis in a child.

Primary renal lymphoma (PRL) is a rare condition and bilateral PRL even rarer. Most of these bilateral PRL have been reported in adults. We describe a 3-year-old male with bilateral primary renal B cell lymphoma with orbital metastases. We discuss the difficulties in diagnosis and management of this rare presentation of lymphoma.

Clinical Spectrum of Congenital Anomalies of Kidney and Urinary Tract in Children.

OBJECTIVES: To evaluate the clinical spectrum and patterns of clinical presentation in congenital anomalies of kidney and urinary tract. METHODS: We enrolled 307 consecutively presenting children with congenital anomalies of kidney and urinary tract at the pediatric nephrology clinic. Patients were evaluated clinically, with serum biochemistry, appropriate imaging and radionuclide scans. RESULTS: The most common anomaly was primary vesicoureteric reflux (VUR) (87, 27.3%), followed by pelviureteral junction obstruction (PUJO) (62,20.1%), multicystic dysplastic kidney (51 16.6%), non-obstructive hydronephrosis (32, 10.4%) and posterior urethral valves (PUV) (23, 7.4%). 247 (80.4%) anomalies had been identified during the antenatal period. Another 33 (10.7%) were diagnosed during evaluation of urinary tract infection, and 21 (6.8%) during evaluation for hypertension at presentation. Obstructive anomalies presented earlier than non-obstructive (7 (3, 22.5) vs 10 (4, 24) mo: (P=0.01)). The median (IQR) ages of presentation for children with PUV (n=23), VUR (n=87) and PUJO (n=62) were 4 (2, 14) mo, 10 (5, 27) mo, and 7 (3, 22.5) mo, respectively. Nine (2.9%) children had extrarenal manifestations. CONCLUSIONS: The median age at clinical presentation for various subgroups of anomalies indicates delayed referral. We emphasize the need for prompt referral in order to initiate appropriate therapeutic strategies in children with congenital anomalies of kidney and urinary tract.

High grade renal trauma due to blunt injury in children: do all require intervention?

INTRODUCTION: The aim of this study was to analyze the presentation and management of major grade renal trauma in children. METHOD: A retrospective study was performed including data collected from the patients who were admitted in Pediatric surgery with major grade renal injury (grade 3 and more) from January 2015 to August 2018. Demography, clinical parameters, management, duration of hospital stay and final outcome were noted. RESULTS: Out of 13 children (9 males and 4 females), with age range 2-12 years (mean of 8 years), reported self-fall was the commonest mode of injury followed by road traffic accident. The majority (10/13, 75%) had a right renal injury. Eight children had a grade IV injury, one had a grade V injury, and four children had grade III injury. Duration of hospital stay varied from 3 to 28 (mean of 11.7) days. Three children required blood transfusion. One child required image guided aspiration twice and two required pigtail insertion for perinephric collection. All the 13 children improved without readmission or need for any other surgical intervention. CONCLUSION: Children with major grade renal trauma due to blunt injury can be successfully managed without surgical intervention and minimal intervention may only be needed in select situations.

Clinico-Biochemical Profile of Children with Congenital Anomalies of the Kidney and Urinary Tract: A Cross-Sectional Study.

BACKGROUND: Congenital anomalies of the kidney and urinary tract (CAKUT) are a group of disorders responsible for the majority of pediatric end-stage renal disease cases. There are only a few studies on CAKUT. OBJECTIVES: A study was conducted to determine the clinical and biochemical profile of children with CAKUT and to estimate the prevalence and the factors associated with hypertension in CAKUT. METHODS: A cross-sectional study was conducted in a tertiary center for 18 months from March 2014 to August 2015. Demographic data were recorded, and clinical examination including blood pressure measurement was performed. Various biochemical parameters including plasma renin activity (PRA), urinary beta-2-microglobulin (B2M), and microalbuminuria were evaluated. RESULTS: A total of 81 patients with CAKUT were studied. Twenty-two (27%) patients were underweight, 4 (5%) patients were stunted, and 26 (32%) were both underweight and stunted. Children with bilateral disease had a higher incidence of underweight (21/44 vs. 8/37; p = 0.04; 95% CI; Fisher exact test), and both underweight and stunted (25/44 vs. 10/37; p = 0.006; 95% CI; Fisher exact test) compared to children with unilateral disease. Hypertension was found in 27% cases. No association was found between hypertension and PRA, BM2, or microalbuminuria in our study. PRA was inversely proportional to the estimated glomerular filtration rate (eGFR) (Pearson test; 95% CI; p = 0.006). CONCLUSIONS: Bilateral disease in CAKUT was significantly associated with poor somatic growth. PRA was inversely proportional to eGFR. The prevalence of hypertension was higher in children with CAKUT than in normal children and is possibly multifactorial as it was not associated with elevated PRA, B2M, or microalbuminuria.

Solitary functioning kidney in children: clinical implications.

INTRODUCTION: Children with solitary functioning kidney (SFK) are prone to develop long term problems, which are not well represented in the literature. The extent to which the presence of associated congenital anomalies of kidney and urinary tract (CAKUT) further de-stabilize renal function is to be addressed. OBJECTIVE: This study was conducted to evaluate the etiology, presentation, presence of CAKUT, and renal damage in children with SFK. METHODS: All children with SFK who presented to the department of pediatric surgery from March 2014 to May 2016 were included in the study. Children with malignancy were excluded from the study. RESULTS: Of the 20 patients with SFK, 14 (70%) had primary SFK (8 with agenesis and 6 with multicystic dysplastic kidney), 6 (30%) belonged to secondary SFK group, among them 3 had pelviureteric junction obstruction, 2 had posterior urethral valves and 1 had vesicoureteric reflux. Eight (40%) had associated CAKUT, 4 (20%) were asymptomatic while 8 (40%) had UTI and 6 (30%) had hypertension. Ten (50%) patients had reduced glomerular filtration rate (GFR) suggesting compromised renal function. CONCLUSION: Children with SFK have high morbidity especially when associated with ipsilateral CAKUT. Long-term periodical follow up is essential in these patients to improve clinical outcome.