Obinutuzumab as a viable therapeutic strategy in rituximab-refractory childhood frequently relapsing, steroid-dependent nephrotic syndrome that relapsed during B-cell depletion.

A subgroup of children with frequently-relapsing, steroid-dependent nephrotic syndrome relapse during B-cell depletion after rituximab. A 15-year-old boy with focal segmental glomerulosclerosis became rituximab-refractory after 5 courses of treatments, with a relapse-free period shortened to 1 month. Circulating total and memory B-cells were undetectable at the time of relapse.

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.

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.

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.

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.

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.

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.

Therapeutic drug monitoring in childhood idiopathic nephrotic syndrome: a state of the art review.

Immunosuppressants are commonly used as steroid-sparing agents in childhood idiopathic nephrotic syndrome (NS) to induce and sustain remissions. These drugs have narrow therapeutic indices with high inter- and intra-patient variability. Therapeutic drug monitoring (TDM) would therefore be essential to guide the prescription.

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 m).

Use of Rituximab in Childhood Idiopathic Nephrotic Syndrome.

Rituximab is an established therapy in children with idiopathic nephrotic syndrome to sustain short- to medium-term disease remission and avoid steroid toxicities. Recent trials focus on its use as a first-line agent among those with milder disease severity. Rituximab is used in multidrug refractory nephrotic syndrome and post-transplant disease recurrence, although the evidence is much less substantial.

Outcomes and perception of cloud-based remote patient monitoring in children receiving automated peritoneal dialysis: a prospective study.

Background: Remote patient monitoring (RPM) for automated peritoneal dialysis (APD) may improve clinical outcomes. Paediatric data, however, remain extremely scarce.

Methods: We conducted a prospective observational study of children (0-18 years) receiving APD with cloud-based RPM over two 24-week periods (pre- and post-RPM).

Epidemiology and outcomes of pediatric transplant-associated thrombotic microangiopathy in Hong Kong.

Background: Transplant-associated thrombotic microangiopathy (TA-TMA) is an under-recognized yet potentially devastating complication of hematopoietic stem cell transplantation (HSCT) which had increased awareness in recent years. This report summarizes the demographics and outcomes of pediatric TA-TMA in Hong Kong.

Methods: All patients aged below 18 years who underwent HSCT in the Hong Kong Children's Hospital and were diagnosed to have TA-TMA during the 2-year period from April 1, 2019 to March 31, 2021 were included.

Embolisation of an aneurysmal high-flow renal arteriovenous fistula in a paediatric patient: simultaneous arterial and venous approach.

Background: A large aneurysmal renal arteriovenous fistula (AVF) can cause hypokalaemic hypertension due to activation of renin-aldosterone system due to steal effect from renal parenchyma. In comparison to nephrectomy, endovascular embolisation of renal AVF is minimally invasive and can be nephron sparing, thus preserving renal function. However, such embolisation is technically challenging and can be associated with high risk of embolic migration.

Long-Term Efficacy and Safety of Repeated Rituximab to Maintain Remission in Idiopathic Childhood Nephrotic Syndrome: An International Study.

Background: Long-term outcomes after multiple courses of rituximab among children with frequently relapsing, steroid-dependent nephrotic syndrome (FRSDNS) are unknown.

Methods: A retrospective cohort study at 16 pediatric nephrology centers from ten countries in Asia, Europe, and North America included children with FRSDNS who received two or more courses of rituximab. Primary outcomes were relapse-free survival and adverse events.

Demographics and long-term outcomes of children with end-stage kidney disease: A 20-year territory-wide study.

Aim: To evaluate the demographics and long-term patient outcomes of children with end-stage kidney disease in Hong Kong.

Methods: We conducted a cohort study at the Paediatric Nephrology Centre, the designated site providing kidney replacement therapy (KRT) for children in Hong Kong. The clinical characteristics and outcomes of all children who initiated chronic KRT before 19 years, between 2001 and 2020, were analysed.

When should we start and stop ACEi/ARB in paediatric chronic kidney disease?

Renin-angiotensin-aldosterone inhibitors (RAASi) are the mainstay therapy in both adult and paediatric chronic kidney disease (CKD). RAASi slow down the progression of kidney failure by optimization of blood pressure and reduction of proteinuria. Despite recommendations from published guidelines in adults, the evidence related to the use of RAASi is surprisingly scarce in children.

Vertical gaze palsy and intraoral numbness in a patient with neuro-psychiatric systemic lupus erythematosus: A case report and literature review.

We report, to the best of our knowledge, the first case of neuropsychiatric systemic lupus erythematosus with clinical presentation of bilateral upward gaze palsy and intraoral numbness. Magnetic resonance imaging of the brain was able to identify the pathogenic lesion at the left side of midbrain, involving the vertical gaze center and sensory pathways for innervating the buccal and hard palate mucosa. A course of aggressive immunosuppressive treatment resulted in prompt resolution of gaze palsy and the midbrain lesion.