Biopsy In Children Research Articles

Relationship between the timing of chemotherapy and surgical complications following surgical biopsy in children with malignant solid tumors.

Biopsies for diagnosis before chemotherapy is common in children with malignant solid tumors. Wound healing is delayed by chemotherapy; however, the ideal interval between biopsy and chemotherapy remains unknown. We aimed to summarize the relationship between chemotherapy timing and postoperative surgical complications. We retrospectively reviewed patients with malignant solid tumors who underwent chemotherapy after surgical biopsy at our institution between January 2014 and August 2020. The primary outcomes were postoperative surgical complications (within 30days) and the timing of chemotherapy. Forty-three patients were analyzed. The types of tumors were neuroblastoma (n=20), hepatoblastoma (n=10), Ewing sarcoma (n=5), germ cell tumor (n=3), angiosarcoma (n=1), clear cell sarcoma (n=1), ganglioneuroblastoma (n=1), rhabdoid tumor (n=1), and rhabdomyosarcoma (n=1). The operative procedures were thoracoscopy (n=5), laparotomy (n=17), laparoscopy (n=14), and superficial (n=7). The median time [range] to chemotherapy after biopsy was 4 [0-21] days. No surgical complications occurred before chemotherapy, and two (4.7%) patients experienced complications after chemotherapy. These included postoperative hemorrhage (grade 3) and surgical site infection (grade 1). Chemotherapy was initiated 1 and 6days after biopsy, respectively, in these cases. Complications occurred 10 and 23days after biopsy, respectively. The rate of postoperative surgical complications related to biopsy seems acceptable, even when chemotherapy was initiated in the early postoperative period. Early initiation of chemotherapy after biopsy may be a suitable option, particularly in children with bulky or symptomatic malignant solid tumors.

Skin Biopsy in the Pediatric Age Group

Abstract Skin biopsy has a crucial place in arriving at a definitive diagnosis in many pediatric dermatologic conditions. The need to perform a skin biopsy on a child may provoke apprehension in the parents/caregivers, which can be alleviated by establishing a good rapport with them. The details of the procedure, including benefits and risks involved, and the expected result should be communicated to the parents in an easy-to-understand language to have an informed decision. In this review, literature search was done across the PubMed database from January 1992 to January 2022 using the following keywords “skin biopsy in children,” “skin biopsy in pediatric age group,” “pediatric dermatopathology,” “dermatologic surgery in children,” “dermatologic surgery in pediatric age group,” “pediatric dermatologic surgery,” and “procedures in pediatric dermatology.” Articles not written in the English language were excluded.

OA32 Minimally invasive, ultrasound-guided tissue biopsies of synovial tissue in children with Juvenile Idiopathic Arthritis for research: a feasibility study

Introduction/BackgroundWhen investigating disease mechanisms, site-specific differences in immune cell phenotype and function have highlighted the need to analyse cellular and molecular mechanisms at the tissue site directly. In adults, the ability to obtain synovial tissue biopsies using ultrasound-guided techniques, combined with advanced tissue analytics, has revolutionised our understanding of the cellular ecosystem that operates within the joint and how it contributes to disease. However, a similar approach in paediatric disease is lacking. Description/MethodAims: 1) To describe the protocol for undertaking minimally invasive ultrasound-guided synovial tissue biopsies in children and young people with arthritis, for the purpose of research, alongside routine clinical care. 2) To investigate whether high-quality synovial tissue can be obtained that is suitable for downstream applications including single cell profiling technologies, histology and digital spatial profiling.Treatment-naïve children with a diagnosis of Juvenile Idiopathic Arthritis, who were being referred for a corticosteroid joint injection were recruited from two large UK Paediatric Rheumatology centres. We established a workflow pipeline for performing synovial tissue biopsies in child and young people with arthritis, using standardised procedures for biopsy and sample processing. Procedures were performed by experienced paediatric interventional radiologists with experience of joint biopsy for diagnostic purposes. Following a general anaesthetic, required as part of routine clinical care and the establishment of sterility, synovial fluid was aspirated. Needle-biopsies were undertaken from the same needle insertion site and subsequently corticosteroid was injected into the joint. Thickened synovium was graded via ultrasonography. Participating families completed questionnaires prior to and following synovial biopsy.Discussion/Results11 participants were recruited to the study over a nine month period, with a median age of 7 years (range 1-16 years); 91% were female. Samples obtained included core synovial biopsies, paired synovial fluid and peripheral blood. Synovial tissue fragments were processed for histology by formalin fixation and cryopreserved for downstream applications, including RNA sequencing and cell culture. Quality control indices included histological analysis to ensure the biopsied material was characteristically synovium and to grade the severity of inflammation. No significant complications were reported; however, one child had a mild haemarthrosis controlled with cold saline wash out and cold compresses.Key learning points/ConclusionObtaining biopsies of synovial tissue in children with Juvenile Idiopathic Arthritis for the purpose of research, alongside clinical care is feasible. Analysis of tissue direct from the site of inflammation with single-cell RNA sequencing in children is achievable.

Mast cells and eosinophils of the intestinal mucosa compared with data on food allergy and food sensitization in children with inflammatory bowel disease

Aim of the study. To investigate the frequency and clinical significance of detecting increased serum levels of IgE to food proteins, eosinophils (EOs) and mast cells (MCs) in colon biopsies in children with inflammatory bowel disease (IBD) in remission or with a low degree of activity.Methods and materials. 165 children with IBD and 81 children of the control group were examined. The level of serum sIgE to cow’s milk and wheat was determined. To detect cow’s milk protein allergy (CMPA), we used the questionnaire «Food Allergy» previously developed by us and validated on a group of patients with IBD. We performed the microscopy of histological preparations of the intestinal mucosa with counting of EOs and MCs.Results. The frequency increased sIgE levels to CMP in children with CD was higher than in children with UC, but there were no differences in the frequency of sensitization in the comparison group of children without allergies and IBD. An increased number of MCs was present in 37.0 % of IBD patients: 37.9 % of children with CD and 35.3 % of children with UC. Intestinal eosinophilia was observed in 15.2 % of children with IBD: 10.3 % with CD and 23.5 % with UC. A correlation was found between the average number of MCs in the biopsy specimen and the questionnaire score sums, in UC, as well as between the average number of EOs in the biopsy specimen and the questionnaire score sums and between the average number of MCs in the biopsy and the questionnaire score sums. In patients with CD, there is a correlation between the average number of MCs in the biopsy and the questionnaire score sums. Differences at the trend level were found between the average values of MCs in intestinal biopsy specimens in children with IBD with and without FA.Conclusion. The found correlation between the average number of MCs and EOs in the biopsy and the the questionnaire score sums shows that increased levels of intestinal MCs and EOs can be considered a diagnostic criterion for FA in patients with IBD with the presence of clinical data.

Epitope detection in monocytes (EDIM) for liquid biopsy including identification of GD2 in childhood neuroblastoma—a pilot study

BackgroundNeuroblastoma (NB) is the most common paediatric extracranial solid malignancy. We analysed the role of the epitope detection in monocytes (EDIM) technique for liquid biopsy in NB patients.MethodsTumour epitopes transketolase-like 1 (TKTL1), Apo10 (DNaseX) and GD2 were assessed: expression levels in seven NB tumour samples and five NB cell lines were analysed using RT-PCR and flow cytometry. LAN-1 cells were co-cultured with blood and assessed using EDIM. Peripheral blood macrophages of patients with neuroblastoma (n = 38) and healthy individuals (control group, n = 37) were labelled (CD14+/CD16+) and assessed for TKTL1, Apo10 and GD2 using the EDIM technology.ResultsmRNA expression of TKTL1 and DNaseX/Apo10 was elevated in 6/7 NB samples. Spike experiments showed upregulation of TKTL1, Apo10 and GD2 in LAN-1 cells following co-culturing with blood. TKTL1 and Apo10 were present in macrophages of 36/38 patients, and GD2 in 15/19 patients. The 37 control samples were all negative. EDIM expression scores of the three epitopes allowed differentiation between NB patients and healthy individuals.ConclusionsThe EDIM test might serve as a non-invasive tool for liquid biopsy in children suffering from NB. Future studies are necessary for assessing risk stratification, tumour biology, treatment monitoring, and early detection of tumour relapses.

Complications of Ultrasonography Guided Percutaneous Renal Biopsy in Children: A Retrospective Study in a Tertiary Hospital in Nepal

Introduction: Percutaneous renal biopsy is done under ultrasonographic guidance in recent times due to which the diagnostic yield has increased and complications have been severely curtailed. We aimed to retrospectively evaluate the complications following kidney biopsy in children. Methods: After obtaining ethical clearance, retrospective evaluation of sixty pediatric renal biopsies done in BPKIHS from October 1, 2018, to September 30, 2020, were included in the study. Biopsies were done by 18G biopsy guns under ultrasound guidance and the complications were evaluated. Results: Of sixty patients, 13.33% had gross hematuria, 3.33% had a perirenal hematoma and the biopsy failure rate was 3.3%. None of the patients required blood transfusion and had major complications requiring further intervention. Conclusions: Percutaneous renal biopsy under ultrasonographic guidance is a safe procedure with a better success rate.

SURG-04. Robotic alignment system Cirq (Brainlab) for navigated brain tumor biopsies in children

BACKGROUND: With the incorporation of the robotic alignment system Cirq (Brainlab, Munich, Germany) into our neurosurgical armamentarium, we deemed it important to study application accuracy of this new device. As a baseline, we retrospectively reviewed our historical data on brain tumor biopsies using the non-robotic alignment instrument Varioguide (Brainlab, Munich, Germany). Because of relatively high target registration errors in this historical series, we sought to improve our registration with the introduction of Cirq. AIMS: 1.Share our experience with the new robotic alignment system Cirq for navigated brain tumor biopsies. 2. Compare patient-to-image registration methods. 3. Evaluate the accuracy of Cirq robotic system as compared to the non-robotic instrument Varioguide. METHODS: All patients (0-19 years old) who underwent a brain tumor biopsy at the Princess Máxima Center, Utrecht, were included. Over the period 2018-2020, data were collected retrospectively (cohort “Varioguide” in which patient-to-image registration was based on preoperative MRI with adhesive scalp fiducials). From 2021 onwards, data were collected prospectively (cohort “Cirq” in which patient-to-image registration was done using 1. A preoperative MRI with adhesive scalp fiducials and 2. An intraoperative CT head scan with bone screw fiducials). For both cohorts, Euclidian distances were calculated between the intended target and the obtained target using postoperative MRI scans. PRELIMINARY RESULTS : Nineteen patients were biopsied with Varioguide and nine patients with Cirq. The Cirq robotic system was convenient and safe in use with no intraoperative complications. Patient-to-image registration was more accurate when based on bone screw fiducials as compared to scalp fiducials. The Cirq robotic system, combined with bone fiducial registration, resulted in a high target accuracy with a maximum target error of 2 millimeters. CONCLUSION: The Cirq robotic alignment system, combined with bone screw fiducial registration, provides a safe and highly accurate method for brain tumor biopsies in children.

Perioperative use of antibiotics in full-thickness rectal biopsies for Hirschsprung’s disease

BackgroundThere is no consensus on the use of perioperative antibiotic in full-thickness rectal biopsies (FTB) in children suspected for Hirschsprung disease. The primary objective of this study was to examine the effect of perioperative antibiotics on infectious and overall complications rate in FTB performed in children under the age of 1 year.MethodsA retrospective chart review. Two time periods were compared—one with and one without the routine use of perioperative antibiotics. The treatment included cefuroxime 100 mg/kg and metronidazole 20 mg/kg administered intravenously at anesthesia induction followed by peroral administration of amoxicillin 50 mg/kg with Clavulanic acid 12.5 mg/kg three times a day t.i.d. for 3 days postoperative.ResultsIn the group with perioperative antibiotics the infectious complications rate was 3.3% compared to 13.4% in the group without (p = 0.03) with fever as the most common. The overall complication rate was 11.3% and 15.2%, respectively (p = 0.43).ConclusionPerioperative antibiotics significantly reduced the rate of infectious complications, but not the rate of overall complication rate after a full thickness rectal biopsy in children under the age of 1 year.

Safety and Adequacy of Ultrasound-Guided Percutaneous Renal Biopsy in Children: A Single-Center Experience.

BackgroundUltrasound-guided percutaneous renal biopsy (PCRB) is a commonly used technique to obtain renal tissue for histopathological diagnosis in children and adolescents. The objectives of this study include determining the indications for renal biopsy, documenting the safety and efficacy of ultrasound-guided PCRB, and documenting its complications along with histopathological findings in children.MethodologyThe Ethical Review Committee approved this cross-sectional study. Data of all children with either nephrotic or nephritic syndrome from January 2017 to September 2020 (at The Kidney Center Post Graduate Training Institute Karachi) who underwent ultrasound-guided PCRB were collected and analyzed. An ultrasonic examination was performed both before and after the biopsy.ResultsDuring the research period, 104 individuals underwent PCRB. The average age of the children biopsied was 7.44 ± 4.12 years (range = 1-17 years). The most prevalent reason for biopsy was nephrotic syndrome. Almost 94% of PCRBs were effective. Post-biopsy complications were detected in 16 cases, with peri-nephric hematoma being the most prevalent.ConclusionsIn children, ultrasound-guided PCRB can safely be performed under sedation in experienced hands with an automated biopsy gun needle. The use of real-time ultrasound guidance as well as the automated biopsy gun ensures good outcomes.

Percutaneous ultrasound-guided renal biopsies in a paediatric population: comparison of coaxial and non-coaxial techniques using 18-gauge core biopsy needles.

Percutaneous ultrasound-guided biopsy is performed in paediatric patients for evaluation of diffuse renal parenchymal disease. When compared with the non-coaxial technique, the coaxial technique has the advantages of obtaining multiple tissue cores via a single capsular puncture and post-biopsy tract embolisation. To compare the coaxial and non-coaxial techniques of percutaneous ultrasound (US)-guided biopsy of native kidney parenchyma in children and adolescents with renal disease. We retrospectively identified consecutive patients who underwent percutaneous US-guided renal biopsy using an 18-gauge core biopsy needle from July 2019 to July 2021 in a single tertiary paediatric nephrology centre. Focal renal tumour biopsy and transplant kidney biopsy were excluded. The total glomerular yield, specimen adequacy, complication rate and procedural time between the coaxial and non-coaxial groups were compared. There were 34 percutaneous US-guided renal biopsies: 22 using a coaxial technique and 12 using a non-coaxial technique. The total median glomerular yield obtained was higher in the coaxial group (coaxial=37.9; non-coaxial=22.2; P=0.02). No statistically significant difference was noted between specimen adequacy (coaxial=100%; non-coaxial=91.7%; P=0.35). While no statistically significant difference was detected for overall complication rates (coaxial=13.6%; non-coaxial=41.7%; P=0.09), the coaxial group had a lower rate of haemorrhagic complications (coaxial=4.5%; non-coaxial=41.7%; P=0.01). One patient in the non-coaxial group had post-biopsy haemorrhage requiring embolisation. The procedural time was shorter in the coaxial group (coaxial=26.3 ± 7.0min; non-coaxial=51.3 ± 11.5min; P<0.001). Percutaneous US-guided renal biopsy in children using the coaxial technique has significantly higher total glomerular yield, shorter procedural time and fewer haemorrhagic complications, compared to biopsies using the non-coaxial technique.

Percutaneous ultrasound-guided cholecystocholangiography with microbubbles combined with liver biopsy for the assessment of suspected biliary atresia.

Percutaneous ultrasound (US)-guided cholecystocholangiography is effective in diagnosing biliary atresia for infants with a gallbladder >1.5cm in length on US. However, whether it is still effective for other types of gallbladders needs further clarification. To evaluate the diagnostic performance and safety of percutaneous US-guided cholecystocholangiography combined with liver biopsy in children with suspected biliary atresia and with different types of gallbladders on US. Sixty-five infants were referred for percutaneous US-guided cholecystocholangiography with microbubbles and liver biopsy after an equivocal (n=39) or highly suspected (n=26) US diagnosis of biliary atresia. Two radiologists evaluated US and percutaneous US-guided cholecystocholangiography images in consensus. One pathologist independently evaluated liver specimens. We used the unpaired t-test, Mann-Whitney U test and chi-square test to analyze the data. Of the 65 infants, 59 (90.8%) underwent a successful percutaneous US-guided cholecystocholangiography, with both sensitivity and specificity of 100%. All six infants for whom puncture failed had contracted gallbladders. The sensitivity and specificity of liver biopsy in the diagnosis of biliary atresia were 89.7% (26/29) and 83.3% (30/36), respectively. When percutaneous US-guided cholecystocholangiography and liver biopsy were combined, all infants gained correct diagnosis, and in 35 infants (97.2%, 35/36) biliary atresia could be excluded without intraoperative cholangiography. Twenty-two of 65 infants (33.8%) had fluid collections around the liver related to puncture. None of these complications needed treatment. Percutaneous US-guided cholecystocholangiography combined with liver biopsy appears safe and effective for excluding or confirming biliary atresia in cholestatic infants with a dilated gallbladder on US.

Collaborative studies of U.S.-China neurologists on acute motor axonal neuropathy.

Collaborative studies of U.S.-China neurologists on acute motor axonal neuropathy.

POS-485 Percutaneous ultrasound-guided renal biopsy in children: A retrospective review of a tertiary hospital experience in Lagos, Nigeria

Percutaneous renal biopsy (PRB) is an invasive procedure in nephrology that is extremely important in making diagnosis, prognostication and evaluation of treatment outcome in children with renal diseases. Currently the use of real time ultrasound guided PRB has greatly increased the tissue yield and also reduced the complications associated with the procedure. The prevalence of renal diseases as well as mortality associated with renal diseases is increasing worldwide and especially in developing countries like Nigeria.

Renal biopsy in children with IgA vasculitis.

IntroductionHenoch-Schönlein purpura nephritis (HSN) is defined as Henoch-Schönlein purpura with kidney involvement, including hematuria and/or proteinuria. The aim of this study was to evaluate the data of HSN patients who underwent renal biopsy, and compare the main clinical and laboratory parameters that may affect renal biopsy findings, treatment protocols, and short- and long-term outcome of those patients.MethodsBiopsies performed in 72 HSN patients between January 2007 to January 2017 were retrospectively evaluated. They were divided into two groups according to renal biopsy classification of the International Study of Kidney Disease in Children. Renal outcome, clinical and laboratory parameters, treatment protocols, and outcome were compared between groups. Short- and long-term follow-up of patients were evaluated.ResultsOf 72 patients, 47 were male (65.3%) and 44 (61.1%) were ≤10 years of age. Neutrophil-lymphocyte ratio was found higher in patients with scrotal involvement (p=0.042). Short-term unfavorable outcome was significantly higher in patients with scrotal involvement (p=0.038). Patients with hypertension and decreased creatinine clearance were found to have more unfavorable outcomes in long-term follow-up (p=0.029, p=0.040).ConclusionCyclosporin-A and cyclophosphamide could be effective in steroid unresponsive HSN patients. Patients with scrotal involvement, decreased creatinine clearance, and hypertension should be closely monitored for sequelae of HSN.

Sedative and Analgesic Effects of Intravenous Midazolam and Ketamine Combination in Bone Marrow Aspiration and Biopsy of Childhood Cancer

Background: Various drugs, including ketamine and midazolam, are used for sedation in children. Objectives: This study aimed to evaluate the effectiveness of intravenous midazolam and ketamine combination in bone marrow aspiration and biopsy in children with cancer. Methods: This descriptive cross-sectional study was conducted on 100 children aged six months to 17 years candidates for bone marrow aspiration or biopsy. Sampling was easy, accessible, and sequential. Children were injected with 0.05 mg/kg midazolam and 1 mg/kg ketamine for sedation and analgesia. Then, sedation rate, restlessness, nausea, vomiting, laryngospasm, and decreased oxygen saturation were assessed. Data were analyzed using SPSS version 18 software. Results: The mean age of children was 6.8 ± 4.3 years. The mean degree of sedation based on the modified Ramsey score was 5.2 ± 0.74. Nausea and vomiting were observed in nine (9%) children. Six (6%) children had arterial saturation of less than 90%. Twelve (12%) children showed restlessness when waking up. There were no cases of laryngeal spasms. Conclusions: The present study showed that the intravenous midazolam and ketamine combination provides suitable sedation and analgesia to children, with low and negligible side effects.

Ultrasound-guided percutaneous liver biopsy in children. Five-year experience at a tertiary care center

Introduction and aimsPercutaneous liver biopsy with histopathologic analysis is a valuable tool for the diagnosis, prognosis, and treatment evaluation of liver diseases. Its ultrasound-guided performance is useful, making the procedure safer and reducing the risk for complications and hospital stay. Our aim was to describe the indications, histopathologic study, and complications associated with the performance of ultrasound-guided percutaneous liver biopsy in pediatric patients. Material and methodsThe study included 102 ultrasound-guided percutaneous liver biopsies performed on patients <16 years of age, within the time frame of January 2014 and December 2019. The information was obtained from electronic files and histopathologic studies and the data were analyzed through descriptive statistics. ResultsA total of 102 procedures were carried out on 99 patients. Mean patient age was 72 months and 58.8% of the patients were female. Over 65% of the indications for liver biopsy included autoimmune hepatitis (23.5%), elevated liver enzymes (21.5%), and chronic liver disease (20.5%). Four patients presented with immediate complications (3.9%), three of which were major (2.9%), concurring with that reported in the international literature. ConclusionsOur study corroborates the importance of ultrasound-guided liver biopsy in the diagnosis and follow-up of pediatric patients. The procedure also had a low complication rate of only 3.9%.

Magnetic resonance imaging-guided biopsies in children.

BackgroundMagnetic resonance imaging (MRI) is used far less as an imaging-guided method for percutaneous biopsies than computed tomography (CT) and ultrasound (US), despite its imaging benefits, particularly in children.PurposeTo evaluate the feasibility, accuracy and safety of MRI-guided biopsies in paediatric patient population.Material and MethodsThe retrospective study included 57 consecutive paediatric patients (<18 years old). A percutaneous core needle biopsy (PCNB) or trephine biopsy was performed in 53 cases, and an additional fine-needle aspiration biopsy (FNAB) in 26 cases. In 4 cases, a stand-alone FNAB was taken. Biopsies were performed with 0.23 T open and 1.5 T closed MRI scanners. Statistical methods used for confidence intervals and p-values were Wilson score method and chi-square test.ResultsThe overall diagnostic accuracy of histologic biopsy was 0.94, with sensitivity 0.82, specificity 1.00, positive predictive value (PPV) 1.00 and negative predictive value (NPV) 0.92. In histological bone biopsies, diagnostic accuracy was 0.96, with sensitivity 0.86, specificity 1.00, PPV 1.00 and NPV 0.94. The FNAB sample diagnosis was associated with the histological diagnosis in 79% of cases. There were no major primary complications and only a few late complications. After biopsy, 83% of the children were ambulatory in 6 h. Anti-inflammatory drugs and paracetamol provided satisfactory pain relief in 96% of the patients after biopsy. Most outpatients (71%) were discharged from hospital either on the same day or 1 day later.ConclusionMRI is a technically feasible, accurate and safe guidance tool for performing percutaneous biopsies in children.

Analysis of buccal mucosa as a prognostic tool in children with arrhythmogenic cardiomyopathy

BackgroundThe diagnosis of arrhythmogenic cardiomyopathy (ACM) is challenging especially in children at risk of adverse events. Analysis of cardiac myocyte junctional protein distribution may have diagnostic and prognostic implications, but its utility is limited by the need for a myocardial sample. We previously reported that buccal mucosa cells show junctional protein redistribution similar to that seen in cardiac myocytes of adult patients with ACM. ObjectivesWe aimed to determine when junctional protein distribution abnormalities first occur in children with ACM variants and whether they correlate with progression of clinically apparent disease. MethodsWe analyzed buccal mucosa samples of children and adolescents with a family history of ACM (n = 13) and age-matched controls (n = 13). Samples were immunostained for plakoglobin, desmoplakin, plakophilin-1 and connexin43 and analyzed by confocal microscopy. All participants were swabbed at least twice with an average interval of 12–18 months between samplings. ResultsJunctional protein re-localization in buccal mucosa cells did not correlate with the presence of ACM-causing variants but instead occurred with clinical onset of disease. No changes in protein distribution were seen unless and until there was clinical evidence of disease. In addition, progressive shifts in the distribution of key proteins correlated with worsening of the disease phenotype. Finally, we observed restoration of junctional signal for Cx43 in patient with a favorable response to anti-arrhythmic therapy. ConclusionsDue to ethical concerns about obtaining heart biopsies in children with no apparent disease, it has not been possible to analyze molecular changes in cardiac myocytes with the onset/progression of clinical disease. Using buccal smears as a surrogate for the myocardium may facilitate future studies of mechanisms and pathophysiological consequences of junctional protein redistribution in ACM. Buccal cells may also be a safe and inexpensive tool for risk stratification and potentially monitoring response to treatment in children bearing ACM variants.

Calcineurin inhibitors and renal biopsy in children with idiopathic nephrotic syndrome.

Idiopathic nephrotic syndrome (NS) is common in children, and most patients respond to corticosteroid therapy. Patients who relapse may need additional immunosuppression with cyclophosphamide, mycophenolate mofetil, calcineurin inhibitors (CNI), or rituximab. Many such patients undergo protocol renal biopsies before and after the initiation of CNI therapy. The main objective of our study was to assess the role of protocol renal biopsies in the monitoring of CNI-induced nephrotoxicity in patients with steroid-dependent (SD)/frequent relapse (FR) NS. We did an Institutional Review Board (IRB)-approved retrospective chart review of patients who were diagnosed with NS at the Children's Hospital of Michigan from January 2000 to June 2019. Study inclusion criteria were a diagnosis of NS, age 1 - 21 years at initial diagnosis, SD/FR clinical course, patients with initial steroid resistance with renal biopsy showing minimal change disease, and renal biopsy before and after CNI initiation. The data is presented on 24 patients who met study inclusion criteria. Only 3 patients (12.5%) showed evidence of chronic CNI nephrotoxicity after a median treatment 66.5 months (range 12 - 153 months). Our study revealed that a baseline renal biopsy before starting CNI therapy for children with FR/SDNS is not necessary. A renal biopsy may be considered after 2 - 3 years of CNI administration in selected few cases in whom the diagnosis of CNI nephrotoxicity might help change the management.

Нейтропенії в дітей: набуті нейтропенії

Neutropenia is defined as a reduction in the absolute neutrophil count (ANC) below 1,500 cells/mcl in the blood. Neutropenia is a common laboratory finding in children. It is important to distinguish transient and benign causes from severe congenital neutropenia. Neutropenia can be classified in asymptomatic (mild), moderate, and severe form, the susceptibility to infection depending on ANC. Neutropenia can be either acquired or congenital. Infection, drugs, and immune disorders are the most common acquired causes while congenital causes are rare and confined mostly to infants and children. Transient neutropenia often accompanies viral infections in children, manifested in the period of acute viremia. Young children are characterized by autoimmune neutropenia (AIN), which has a benign course and a favorable prognosis. Autoimmune neutropenia is characterized by a decrease in the number of neutrophils as a result of the destruction of neutrophils in the peripheral blood by antineutrophil antibodies. The duration of AIN is usually 3–5 years. This is a self-limiting disease that in most cases does not require treatment. Despite the benign course, serious infectious complications can occur. Treatment of myeloid growth factors should be started after a previous bone marrow aspiration biopsy in children with severe infections or requiring surgical intervention. High doses of intravenous immunoglobulin and corticosteroids may be effective in treating AIN in patients with life-threating infections. The danger to the patient depends on the etiology, ANC and bone marrow status. The risk of infections is significantly increased in patients with ACN less than 500 cells/μ1. The most common loci of infections include the oral mucosa, skin, perirectal area, perineum. Oral ulcers and gingivitis are characteristic signs of clinically significant neutropenia, requiring the exclusion of its congenital causes. Severe infections in patients with neutropenia are caused by pyogenic or intestinal bacteria and Candida species. It is important to distinguish between transient or benign causes and severe congenital neutropenia or neutropenia associated with serious haematological or systemic disease. Clarification of the cause of neutropenia is important for determining management and prognosis. No conflict of interest was declared by the author. Key words: neutropenia, children, immunodeficiency, autoimmune neutropenia.