Familial thrombotic microangiopathy in a child with coenzyme Q10 deficiency-associated glomerulopathy.

We report a child with biallelic COQ6 variants presenting with familial thrombotic microangiopathy (TMA). A Chinese boy presented with steroid-resistant nephrotic syndrome at 8 months old and went into kidney failure requiring peritoneal dialysis at 15 months old. He presented with hypertensive encephalopathy with the triad of microangiopathic haemolytic anaemia, thrombocytopenia, and acute on chronic kidney injury at 25 months old following a viral illness. Kidney biopsy showed features of chronic TMA. He was managed with supportive therapy and plasma exchanges and maintained on eculizumab. However, he had another TMA relapse despite complement inhibition a year later. Eculizumab was withdrawn, and supportive therapies, including ubiquinol (50 mg/kg/day) and vitamins, were optimized. He remained relapse-free since then for 4 years. Of note, his elder sister succumbed to multiple organ failure with histological evidence of chronic TMA at the age of 4. Retrospective genetic analysis revealed the same compound heterozygous variants in the COQ6 gene.

Renal vascular lesions in childhood-onset lupus nephritis.

BACKGROUND: This study aimed to determine the clinical significance of renal vascular lesions (RVLs) in childhood-onset lupus nephritis (cLN). METHODS: We retrospectively reviewed all children with biopsy-proven cLN between 2004-2020 to evaluate the prevalence of RVLs on kidney biopsy and its associated factors and long-term outcomes. The composite kidney outcome was defined as advanced chronic kidney disease (CKD) stage 3-5, kidney failure and death. RESULTS: 107 biopsies from 84 Chinese patients were analysed. RVLs were observed in 19 patients (22.6%), including non-inflammatory necrotizing vasculopathy (NNV, n = 6), thrombotic microangiopathy (TMA, n = 4), arterial sclerosis (AS, n = 3), concurrent NNV with AS (n = 4), concurrent NNV with TMA (n = 1) and concurrent true renal vasculitis with AS (n = 1). The presence of RVLs was associated with lower estimated glomerular filtration rate (eGFR) (66.9 ± 40.3 vs. 95.6 ± 39.4 ml/min/1.73m2, p = 0.005), haemoglobin level (9.1 ± 1.9 vs. 10.4 ± 1.9 g/dL, p = 0.008) and platelet count (150.1 ± 96.4 vs. 217.2 ± 104.8 × 109/L, p = 0.01). LN classes and activity/chronicity indices were similar. Patients with RVLs had poorer composite kidney outcomes, though not reaching statistical significance (log-rank test, p = 0.06). The presence of NNV was associated with inferior survival free from composite kidney outcome (log-rank test, p = 0.0018), compared to other forms of RVLs and those without RVLs. Univariate analysis revealed NNV (HR 7.08, 95% CI 1.67-30.03) was predictive of composite kidney outcome. CONCLUSION: RVLs are present in one-fifth of cLN patients and are associated with severe presentation. NNV is associated with worse long-term kidney outcome. Routine evaluation of RVLs is warranted and should be incorporated into future classification criteria.

Three-dimensional endoscopy in lumbar spine surgery as a novel approach for degenerative pathologies: a case report.

Endoscopic spine surgery has evolved exponentially. However, the two-dimensional (2D) view results in lack of stereoscopic vision and depth perception, contributing to the steep learning curve. This case report recounts a world first trial of a three-dimensional (3D) endoscopic system that converts 2D to 3D images and explores its potential role in the surgical management of degenerative lumbar spine diseases. The 3D endoscopic system was used for two patient cases and both 2D and 3D images were displayed side by side and compared. Advantages of the 3D endoscopic system include increased perception of depth, rapid identification of bleeding points, and greater visualization of anatomical details. Field of view and exposure were identical in 2D and 3D views. Limitations include costs and need for additional equipment. Overall, 3D endoscopy improved depth perception, instrument manoeuvrability, and recognition of anatomical details. This case report can guide further research and training in endoscopic spine surgery.

Feeding Issues in Infants Referred for Frenotomy.

INTRODUCTION: The diagnosis of ankyloglossia has increased significantly around the world over the last decade. Frenotomy is indicated in infants with ankyloglossia to improve breastfeeding, although there is little scientific evidence of its efficacy. The purpose of this study is to evaluate whether infants being referred for frenotomy had feeding issues prior to the procedure. METHODS: A retrospective chart review was undertaken for all infants under one year of age referred with ankyloglossia to a pediatric otolaryngology practice or a pediatric hospital between 2018 and 2020. Data included age at referral, gender, comorbidities, feeding issues, whether ankyloglossia was diagnosed, and whether frenotomy was done. Frequencies and non-parametric comparisons were calculated. RESULTS: Of the 646 consultations made for tongue tie, a diagnosis of ankyloglossia was made in 94.7% (N=612) of the patients based on clinical judgment. The most common feeding complaints were poor latch (57.1%, N=369) and painful latch (50.3%, N=325). Eighty one (12.5%) patients did not have a reported feeding difficulty. Most patients had an anterior tongue tie (85.8%, N=554), with some showing signs of restricted tongue movement (30.1%, N=184). Ankyloglossia was 4.03 times more likely to be diagnosed (p<.001) and frenotomy was 1.76 times more likely to be performed (p<.001) in the hospital setting compared to the clinic setting.  Conclusion: Children under the age of one referred to otolaryngology for ankyloglossia were often diagnosed concordantly, although some lacked feeding issues that would indicate frenotomy. There are still knowledge gaps about infantile ankyloglossia in referring medical personnel.

A Comparison of Drug-Induced Sleep Endoscopy Findings in Infants and Toddlers.

OBJECTIVE: To compare patterns of obstruction in infants and toddlers with obstructive sleep apnea (OSA) by analyzing drug-induced sleep endoscopy (DISE) examinations. STUDY DESIGN: Retrospective chart review. SETTING: An academic tertiary care pediatric hospital at a single institution. METHODS: Children aged ≤3 years who underwent DISE with propofol titration from 2020 to 2022 were included in the study. Data extracted included age, body mass index, comorbidities, and polysomnography parameters. The sites of anatomic obstruction during DISE were recorded using a validated 10-site scoring system. RESULTS: A total of 86 children underwent DISE, including 15 infants (<1 year) and 71 toddlers (>1≤3 years). Sixty-two percent were male and 37% were female. The mean age of the infant group was 9.6 months (95% confidence interval [CI]: 7.92, 11.28 months), and the mean age of the toddler group was 2.3 years (95% CI: 2.06, 2.50 years). The mean total number of sites of obstruction was not significantly different between infants (N = 2.5) and toddlers (N = 3.2, P = .086). The most common subsite of significant obstruction in both infants and toddlers was the aryepiglottic folds (46.7% and 36.8%, respectively; P = .578). The sites of obstruction and frequency of their occurrence were not significantly different between the 2 groups. CONCLUSION: Multisite airway obstruction is common among children with OSA, especially at the epiglottis, aryepiglottic folds, and tongue base. There is no difference in sites of obstruction and their frequency between toddlers and infants.

White noise use among children undergoing sound field audiometry: A preliminary study.

OBJECTIVE: To describe the prevalence of routine white noise exposure in children who undergo sound field audiometry. METHODS: A retrospective cohort study was conducted at a pediatric otolaryngology clinic affiliated with an academic tertiary care hospital. The medical records of children who underwent sound field audiometry were reviewed and data was collected regarding demographics and audiogram results. The group was divided into two cohorts based on routine exposure to white noise. Children exposed to white noise were tested with warble tones, while those not exposed were tested with narrow-band noise. RESULTS: 127 patients underwent sound field audiometry testing, of which 96 (75.6%) were reported by their parents to use white noise for sleep. The mean age at time of testing was 1.6 years (95% Confidence Interval [CI] 1.5-1.7). 104 (81.9%) children were able to respond to at least four of the sound field thresholds, and there was no significant difference between the children exposed to white noise and those who were not (P = 0.459). Mean pure tone average (PTA) was 26.2 dB (95% CI 25.2-27.2) and mean speech reception threshold (SRT) was 19.2 dB (95% CI 18.2-20.2). The sound field response rate, PTA, and SRT were similar between these two groups. CONCLUSIONS: The routine use of white noise therapy was extremely common in this pediatric population. The use of warble tones as the audiometric stimuli for children exposed to white noise resulted in similar testing success compared to the use of narrow-band noise in children not exposed to white noise.

Incidental finding of acquired cystic kidney disease associated renal cell carcinoma in an adolescent with kidney failure.

Acquired cystic kidney disease (ACKD) can occur in patients with chronic kidney disease and kidney failure, and its incidence increases with the duration of dialysis. In adults, ACKD is less common in the pre-dialysis group (~ 7%), but its incidence can be as high as 80% for those who are on dialysis for more than ten years. There is, however, very little information about the prevalence of ACKD in children. We report a case of malignant transformation of ACKD following a kidney transplant, highlighting the importance of surveillance of the native kidneys in paediatric patients who have been in long-term kidney replacement therapy.

A case report of multicentric carpotarsal osteolysis syndrome: Depiction of a debilitating disease course.

Multicentric carpotarsal osteolysis syndrome (MCTO) is a rare skeletal disorder characterized by progressive osteolysis involving the carpal and tarsal bones, and often associated with nephropathy. It is caused by heterozygous mutation in the MAF bZIP transcription factor B (MAFB) gene. Heterogeneous clinical manifestation and wide spectrum of disease severity have been observed in patients with MCTO. Here, we report a case of a male patient who presented with kidney failure in childhood with progressive disabling skeletal deformity. He was diagnosed with MCTO at 31-years-old, where a de novo pathogenic heterozygous variant in NM_005461.5:c.212C>A: p.(Pro71His) of the MAFB gene was identified. While there has been little data on the long-term prognosis and life expectancy of this disease, this case report sheds light on the debilitating disease course with multiple significant morbidities of a patient with MCTO throughout his lifetime of 33 years.

Renal relapse in children and adolescents with childhood-onset lupus nephritis: a 20-year study.

OBJECTIVES: There is little data on renal relapse in childhood-onset LN (cLN). We investigate the incidence, predictive factors and outcomes related to renal relapse. METHODS: We conducted a retrospective cohort study of all cLN diagnosed at ≤18 years between 2001-2021 to investigate the incidence and outcomes related to renal relapse. RESULTS: Ninety-five Chinese cLN patients (91% proliferative LN) were included. Induction immunosuppression was prednisolone and CYC [n = 36 (38%)] or MMF [n = 33 (35%)]. Maintenance immunosuppression was prednisolone and MMF [n = 53 (54%)] or AZA [n = 29 (31%)]. The rates of complete remission/partial remission (CR/PR) at 12 months were 78.9%/7.4%. Seventy renal relapses occurred in 39 patients over a follow-up of 10.2 years (s.d. 5.9) (0.07 episode/patient-year). Relapse-free survival was 94.7, 86.0, 80.1, 71.2, 68.3, 50.3 and 44.5% at 1, 2, 3, 4, 5, 10 and 20 years, respectively. Multivariate analysis showed that LN diagnosis <13.1 years [adjusted hazard ratio (HRadj) 2.59 995% CI 1.27, 5.29), P = 0.01], AZA maintenance [HRadj 2.20 (95% CI 1.01, 4.79), P = 0.05], PR [HRadj 3.9 (95% CI 1.03, 9.19), P = 0.01] and non-remission [HRadj 3.08 (95% CI 1.35, 11.3), P = 0.04] at 12 months were predictive of renal relapse. Renal relapse was significantly associated with advanced chronic kidney disease (stages 3-5) and end-stage kidney disease (17.9% vs 1.8%, P < 0.01). Furthermore, patients with renal relapse showed an increased incidence of infections (30.8% vs 10.7%, P = 0.02), osteopenia (38.5% vs 17.9%, P = 0.04) and hypertension (30.8% vs 7.1%, P < 0.01). CONCLUSION: Renal relapse is common among cLN, especially among young patients, and is associated with an increased incidence of morbidity and mortality. Attaining CR and the use of MMF appear to decrease the incidence of renal relapse.

How Pediatric Sleep Disordered Breathing Impacts Parental Fatigue.

OBJECTIVE: Previous research has indicated that sleep disordered breathing (SDB) can lead to a decreased quality of life in children and their families as compared to children who do not have SDB. The purpose of this study was to examine fatigue levels in parents who had young children who were impacted by sleep symptoms as determined by the OSA-18 scale. STUDY DESIGN: Survey. SETTING: Three pediatric otolaryngology clinics associated with a tertiary care children's hospital in Buffalo, NY. METHODS: Fatigue levels for parents of children with OSA-18 ≥ 60 were assessed using the Fatigue Severity Scale and the Chalder Fatigue Scale. Consecutive parents with at least one child between the ages of 1 and 10 were recruited. Parents scored their youngest child on the OSA-18 scale. RESULTS: Of the 261 respondents included, 37 parents had a child with an OSA-18 score ≥60. The majority, 211 (82.1%), of participants reported 2 caregivers in the household while 30 (11.7%) had 1 caregiver in the household. Parents of children with OSA-18 ≥60 had a significantly higher mean fatigue score, 16.5 ± 5.8, compared to their counterparts, 11.9 ± 5.2, on the Chalder Fatigue Scale (P < .001). Similar results were reported for the total score on the Fatigue Severity Scale, 34.7 ± 10.8 compared to 28.9 ± 12.0 (P = .004). CONCLUSION: Parents of children with OSA-18 score ≥60 are significantly more fatigued than parents of children with lower scores. Recognition of this is important for the health care community as it impacts not just the child with OSA but also their family.

The Changing Faces of Leadership in Surgery: Study on Presidents of Major Surgical Organizations.

INTRODUCTION: To gain an understanding of the changing faces of leadership in surgery, we examined trends in the demographics, additional degrees pursued, and scientific publication characteristics of the past presidents of three major surgery organizations. METHODS: We queried the BoardCertifiedDocs and Web of Science databases for the demographics, as well as the quantity and quality of publications, of the past presidents of the Association for Academic Surgery, Society of University Surgeons, and American College of Surgeons from 1970 to 2020. Data were analyzed by decade to identify any trends. RESULTS: We identified a total of 140 presidents from the organizations. The proportion of female presidents significantly increased from the 1990s to the 2010s (10% versus 33%, P < 0.05). The percentage of non-White presidents increased from the 1970s to the 2010s (3.33% versus 21.2%, P = 0.024). The percentage of presidents with additional degrees also increased from the 1970s to the 2010s (10.0% versus 48.8%, P = 0.039). During this same time period, the most common area of expertise of presidents shifted from cardiothoracic surgery to surgical oncology. The ratio of presidents' postinduction to preinduction publications was significantly increased among all three organizations in the 2010s compared to the 1970s (P < 0.05). Co-cluster analysis revealed a research topic change from the 1970s to the 2010s. CONCLUSIONS: The faces of surgical leadership have changed in terms of gender equality, racial diversity, surgical subspecialty, and additional degrees held. Such a transformation mirrors evolving diversity, equity, and inclusion initiatives, and it further highlights the adaptability of surgical leadership to the ever-changing landscape of surgery.

Managing Lupus Nephritis in Children and Adolescents.

Lupus nephritis is an important manifestation of systemic lupus erythematosus, which leads to chronic kidney disease, kidney failure, and can result in mortality. About 35%-60% of children with systemic lupus erythematosus develop kidney involvement. Over the past few decades, the outcome of patients with lupus nephritis has improved significantly with advances in immunosuppressive therapies and clinical management. Nonetheless, there is a paucity of high-level evidence to guide the management of childhood-onset lupus nephritis, because of the relatively small number of patients at each centre and also because children and adolescents are often excluded from clinical trials. Children and adults differ in more ways than just size, and there are remarkable differences between childhood- and adult-onset lupus nephritis in terms of disease severity, treatment efficacy, tolerance to medications and most importantly, psychosocial perspective. In this article, we review the 'art and science' of managing childhood-onset lupus nephritis, which has evolved in recent years, and highlight special considerations in this specific patient population.

Humoral and Cellular Immunogenicity of 3 Doses of BNT162b2 in Children With Kidney Diseases.

Introduction: Patients with severe kidney diseases are at risk of complications from COVID-19; however, little is known about the effectiveness of COVID-19 vaccines in children and adolescents with kidney diseases. Methods: We investigated the immunogenicity and safety of an accelerated 3-dose primary series of COVID-19 vaccination among 59 pediatric patients with chronic kidney disease (CKD) (mean age 12.9 years; 30 male) with or without immunosuppression, dialysis, or kidney transplant. Dosage was 0.1 ml BNT162b2 to those aged 5 to 11 years, and 0.3 ml BNT162b2 to those aged 11 to 18 years. Results: Three doses of either vaccine type elicited significant antibody responses that included spike receptor-binding domain (S-RBD) IgG (90.5%-93.8% seropositive) and surrogate virus neutralization (geometric mean sVNT% level, 78.6%-79.3%). There were notable T cell responses. Weaker neutralization responses were observed among those on immunosuppression, especially those receiving higher number of immunosuppressants or on mycophenolate mofetil. Neutralization was reduced against Omicron BA.1 compared to wild type (WT, i.e., ancestral) (post-dose 3 sVNT% level; 82.7% vs. 27.4%; P < 0.0001). However, the T cell response against Omicron BA.1 was preserved, which likely confers protection against severe COVID-19. Infected patients exhibited hybrid immunity after vaccination, as evidenced by the higher Omicron BA.1 neutralization response among these infected patients who received 2 doses compared with those who were uninfected. Generally mild or moderate adverse reactions following vaccines were reported. Conclusion: An accelerated 3-dose primary series with BNT162b2 is immunogenic and safe in young children and adolescents with kidney diseases.

Exposure to Otolaryngology: Impact on Female Students Considering Surgery.

INTRODUCTION: Otolaryngology continues to be dominated by men. As of 2019, only 18.4% of practicing otolaryngologists were women. The goal of this project was to introduce female students to otolaryngology as a career. METHODS: A Women in Otolaryngology event was held in September 2021. Participants included undergraduate and first- or second-year medical students from the University at Buffalo. During the event, students rotated through three skills stations working with female otolaryngology residents and attending physicians. Participants completed pre-and post-course surveys regarding their attitudes toward women in surgery and their perceptions of surgery and otolaryngology. RESULTS: A total of 17 students that completed both the pre- and post-course surveys were included. The mean age was 22.6 years (range 18-25 yr). Specifically, 13 (76.5%) of the participants were Caucasian, three (17.6%) were Asian, and one (5.9%) was Hispanic, and 15 (88.2%) were medical students. On the pretest, 10 (55.6%) participants strongly agreed or agreed that otolaryngology as a career is open to females, while on the posttest, 16 (88.9%) participants strongly agreed or agreed (p=0.002). Nine (50.0%) participants strongly agreed or agreed that they have access to resources to help make a decision if they want to pursue a career in otolaryngology before the event, while, after the event, 16 (88.9%) participants strongly agreed or agreed (p=0.007). Five (27.8%) participants strongly agreed or agreed prior to the event that they felt confident in their knowledge of what otolaryngology includes, while afterward 15 (83.3%) strongly agreed or agreed (p=0.002). CONCLUSION: The Women in Otolaryngology event increased participants' confidence in understanding otolaryngology, promoted understanding of resources available, and demonstrated the openness of the specialty to women.

Neonatal Temporomandibular Joint Dislocation: A Case Report and Literature Review.

Neonatal temporomandibular joint (TMJ) dislocation is rare. The purpose of this study is to describe a case of neonatal TMJ dysfunction and to review the literature on this topic. A six-day-old female was seen with both parents for evaluation of a dislocating jaw. Her mother had been breastfeeding successfully but noticed that there was a noticeable click every time the baby swallowed. Her jaw came out and down as she fed and then returned to the normal position. Over the last few days, her mother felt that only one side was involved as her jaw movement seemed asymmetrical. Her primary care physician had witnessed the click during the sucking reflex. The patient had a normal appearance and was otherwise healthy. The pediatric otolaryngologist observed deviation of the jaw toward the left with a palpable click upon mouth opening and spontaneous reduction with mouth closing. The symptoms resolved over the following month. The literature review showed few cases of TMJ dislocation in infants, most of which described fixed dislocation related to vomiting or crying. Due to the development of the TMJ in infancy characterized by joint laxity and a flat mandibular fossa, malfunctioning of the hinge joint mechanism could be expected to be more common early in life.

Long-term outcomes with rituximab as add-on therapy in severe childhood-onset lupus nephritis.

BACKGROUND: Long-term data pertaining to rituximab as add-on therapy in childhood-onset lupus nephritis (cLN) is scarce. METHODS: A retrospective cohort study was conducted on all patients with proliferative cLN, diagnosed ≤ 18 years and between 2005 and 2021, who received rituximab for LN episodes that were life/organ threatening and/or treatment resistant to standard immunosuppression. RESULTS: Fourteen patients with cLN (female, n = 10) were included, with median follow-up period of 6.9 years. LN episodes (class III, n = 1; class IV, n = 11; class IV + V, n = 2) requiring rituximab occurred at 15.6 years (IQR 12.8-17.3), urine protein:creatinine ratio was 8.2 mg/mg (IQR 3.4-10.1) and eGFR was 28 mL/min/1.73 m2 (IQR 24-69) prior to rituximab treatment. Ten and four patients received rituximab at 1500 mg/m2 and 750 mg/m2, which were given at 46.5 days (IQR 19-69) after commencement of standard therapies. Treatment with rituximab resulted in improvements in proteinuria (ps < 0.001), eGFR (ps < 0.01) and serological parameters, including haemoglobin levels, complement 3 levels and anti-dsDNA antibodies, compared with baseline. Rates of complete/partial remission at 6-, 12- and 24-month post-rituximab were 28.6/42.8%, 64.2/21.4% and 69.2/15.3%. All three patients who required acute kidney replacement therapy became dialysis-free after rituximab. Relapse rate following rituximab was 0.11 episodes/patient-year. There was no lethal complication or severe infusion reaction. Hypogammaglobulinaemia was the most frequent complication (45%) but was mostly asymptomatic. Neutropenia and infections were observed in 20% and 25% of treatments. Upon last follow-up, three (21%) and two (14%) patients developed chronic kidney disease (stage 2, n = 2; stage 4; n = 1) and kidney failure, respectively. CONCLUSION: Add-on rituximab is an effective and safe rescue therapy for cLN patients with life-/organ-threatening manifestations or treatment-resistance. A higher resolution version of the Graphical abstract is available as Supplementary information.

First Branchial Cleft Cyst Confined to the Pinna.

First branchial cleft cysts (FBCCs) arise due to an incomplete fusion of the cleft between the first and second branchial arches. Classically, they are found inferior to the pinna or along the external auditory canal. This report presents a unique case of a nine-month-old male with a first branchial cleft cyst completely within the pinna. The patient presented with a left auricular pit and pinna mass. Ultrasound revealed a homogeneous hypoechoic mass isolated to the pinna. Surgical resection revealed the cyst to be anterior to the inferior pinna cartilage, with the tract projecting anteriorly and inferiorly. Final pathology revealed a benign cyst lined by squamous epithelium and a rim of cartilage, confirming an FBCC. To our knowledge, FBCCs isolated to the pinna have not been previously reported. Awareness of the various presentations of this rare anomaly is essential for a prompt and accurate diagnosis.

Acute kidney injury in children with haematological malignancy: a territory-wide study.

BACKGROUND: In onco-nephrology, data on acute kidney injury (AKI) among children with haematological malignancies are scarce. METHODS: A retrospective cohort study of all patients in Hong Kong diagnosed with haematological malignancies from 2019 to 2021 before 18 years of age, was conducted to investigate the epidemiology, risk factors and clinical outcomes of AKI during the first year of treatment. AKI was defined according to the Kidney Disease: Improving Global Outcomes (KDIGO) criteria. RESULTS: We included 130 children with haematological malignancy at median age of 9.4 years (IQR, 3.9-14.1). Of these patients, 55.4% were acute lymphoblastic leukemia (ALL), 26.9% were lymphoma and 17.7% were acute myeloid leukemia (AML). Thirty-five patients (26.9%) developed 41 AKI episodes during the first year of diagnosis, corresponding to 32 episodes per 100-patient-year. A total of 56.1% and 29.2% of the AKI episodes occurred during induction and consolidation chemotherapy respectively. Septic shock (n = 12, 29.2%) was the leading cause of AKI; 21 episodes (51.2%) were stage 3 AKI; 12 episodes (29.3%) were stage 2 AKI; and 6 patients required continuous kidney replacement therapies. Tumor lysis syndrome and impaired baseline kidney function were significantly associated with AKI on multivariate analysis (P = 0.01). History of AKI was associated with chemotherapy postponement (37.1% vs. 16.8%, P = 0.01), worse 12-month patient survival (77.1% vs. 94.7%, log rank P = 0.002) and lower disease remission rate at 12-month (68.6% vs. 88.4%, P = 0.007), compared to patients without AKI. CONCLUSION: AKI is a common complication during treatment of haematological malignancies which is associated with worse treatment outcomes. A regular and dedicated surveillance program for at-risk patients should be studied in children with haematological malignancies for prevention and early detection of AKI. A higher resolution version of the Graphical abstract is available as Supplementary information.

Long-Term Outcomes of Children and Adolescents With Biopsy-Proven Childhood-Onset Lupus Nephritis.

Introduction: Long-term data pertaining to childhood-onset lupus nephritis (cLN) remain extremely scarce. Methods: We conducted a retrospective cohort study of biopsy-proven cLN with onset age <18 years diagnosed from 2001 to 2020 to ascertain the long-term patient and kidney survival rates, and the incidence of advanced chronic kidney disease (CKD) (estimated glomerular filtration rate [eGFR] <60 ml/min per 1.73 m2). Results: A total of 92 subjects (78 female; age 13.7 ± 3.3 years; all Chinese) were included, with follow-up duration of 10.3 years (interquartile range, 5.8-15.9). Of these, 83 children (90%) had proliferative lupus nephritis (LN) (Class III/IV ± V). Mycophenolate was used for induction in 36%, whereas 34% received cyclophosphamide (CYC); 55% received mycophenolate as maintenance immunosuppression. The rates of complete remission (CR) and partial remission (PR) at 6 months and 12 months, respectively, were 65% and 20% and 78% and 8%. Two patients died (mortality rate 2.1/1000 patient-years), with a standardized mortality ratio of 22.3. Three patients (3.2%) developed end-stage kidney disease (ESKD), and advanced CKD occurred in 5 patients (5.4%). Survival rates without advanced CKD, ESKD, or death were 96.7%, 94.2%, 92.7%, 83.2% and 83.2% at 1 year, 5 years, 10 years, 15 years, and 20 years, respectively. Multivariate analysis revealed that severe kidney failure necessitating dialysis at presentation (adjusted hazard ratio 37.7, 95% confidence interval [CI] 4.0-355.6, P = 0.002), nonresponse (NR) after 12 months of treatment (adjusted hazard ratio 11.2, 95% CI 2.3-54.9, P = 0.003), and multiple nephritis flares (adjusted hazard ratio 2.6, 95% CI 1.1-6.2, P = 0.03) were predictive of advanced CKD, ESKD, or death. Other adverse outcomes included infections (2.9 episodes/100 patient-years), osteopenia (32%), hypertension (17%), short stature (14%), and avascular necrosis (7%). Conclusion: The long-term outcomes of cLN appeared to have improved in the present era with effective immunosuppression, cautious drug tapering, and assurance of medication adherence. There is still an unacceptably high prevalence of adverse outcomes.