We herein report an unusual case of lupus with bleeding diathesis in a Chinese adolescent boy. In the presence of lupus anticoagulant and hypoprothrombinemia, the diagnosis of lupus anticoagulant-hypoprothrombinemia syndrome was made. He responded promptly to immunosuppressive agents and achieved disease remission.

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Our results showed that most children with OSAS underwent T&A without complications. The respiratory complication rate was 7%, and desaturation was the most common post-operative complication. Children with higher BMI z score were more likely to have desaturation after T&A (p = 0.014). Hence, careful monitoring with pulse oximeter after T&A should be offered to those who are obese.

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. Results One hundred forty‐seven children (50% male) received KRT at a mean age of 11.4 ± 5.7 years. The incidence of ESKD was 6.28 per million age‐related population (pmarp). The leading cause of ESKD was congenital anomalies (33%). Ten children (7%) had pre‐emptive kidney transplants, 104 (71%) and 33 (22%) patients received automated peritoneal dialysis and haemodialysis as initial KRT. The incidence of ESKD increased over time, and were 4.38, 5.07, 6.15 and 9.17 pmarp during 2001–2005, 2006–2010, 2011–2015 and 2016–2020, respectively (p = .005). Ninty‐seven patients (66%) received kidney transplants and the median time to receive a kidney graft was 3.7 years (95% CI 3.1–4.3). Only 10 patients had pre‐emptive kidney transplants. The mortality rate was 9.1 deaths per 1000‐patient‐years (95%CI 4.6–16.2). The survival probabilities at 1‐, 5‐, 10‐ and 15‐year were 100%, 94.8% (95%CI 90.7–98.9%), 89.7% (95% CI 83.4%–95.9%), 87.1% (95% CI 79.3%–94.9%), respectively. Standardised mortality ratio was 54.5. 72% of deaths were due to infections. Young infants and those without kidney transplants were associated with worse survival (p < .01). Multivariate analysis demonstrated that dialysis was the only factor associated with significantly increased risk of death (HRadj 12.9, 95% CI 2.7–63.2, p = .002). Conclusion We observed an increasing incidence of paediatric ESKD in Hong Kong with considerable waiting time to kidney transplant. Mortality risk is comparable to other developed countries and is highest among dialysis population. Efforts should be made to facilitate early access to paediatric kidney transplantation in Hong Kong.

NOTE: This preprint reports new research that has not been certified by peer review and should not be used to guide clinical practice. CoronaVac and BNT162b2 in pediatric kidney patients 2 SIGNIFICANCE STATEMENT Little is known about the effectiveness of COVID-19 vaccines in children and adolescents with kidney diseases. This paper describes the antibody and T cell responses of 3 doses of CoronaVac or BNT162b2, the top 2 COVID-19 vaccines distributed worldwide, by an accelerated regimen in patients with kidney diseases aged 1-18 years. Antibody and T cell responses were significantly elicited by either vaccine. Neutralization was reduced against Omicron while T cell response was preserved, which likely confer protection against severe COVID-19. Rate of severe adverse reactions was low in the study. Results confirm that accelerated 3-dose primary series with CoronaVac and BNT162b2 is safe and immunogenic in young children and adolescents with kidney diseases.

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. Case presentation We present a case of successful embolisation of a large aneurysmal renal AVF in a 11-year-old girl. The AVF was initially treated with coil embolization via transarterial route, resulting in partial migration of coil into inferior vena cava. After removal of the migrated coil via transvenous snaring, coils were deployed simultaneously via transarterial and transvenous routes to prevent migration. AVF flow dampened but residual flow persisted at 1 month follow up. A second embolization session with additional coil deployment and N-butyl cyanoacrylate (NBCA) injection resulted in successful occlusion of the AVF. At 3 months follow up, the girl’s blood pressure and serum potassium level have normalized without need of antihypertensive agent or potassium supplements. Conclusion Endovascular embolisation can be an effective nephron sparing treatment for large aneurysmal renal AVF. This is particularly important in paediatric patients as most renal function can be preserved with their expected longer life span. Risk of coil migration can be controlled by simultaneous transarterial and transvenous deployment. Complete occlusion of AVF can be aided by additional use of NBCA.

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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.Results: A total of 73 transplants (51 allogeneic and 22 autologous) in 63 patients had been performed. Six patients (four males and two females) developed TA-TMA at a median duration of 2.5 months post-HSCT. The incidence rate was 9.52%. Of the six TA-TMA patients, five underwent allogenic one underwent autologous HSCT, respectively. Three of them were histologically proven. All four patients with cyclosporine had stopped the drug once TA-TMA was suspected. Median six doses of eculizumab were administered to five out of six patients. Three patients died (two due to fungal infection and one due to acute-on-chronic renal failure) within 3 months upon diagnosis of TA-TMA. Among three survivors, two stabilized with mild stage 2 chronic kidney disease (CKD) while the other suffered from stage 5 end-stage CKD requiring lifelong dialysis. Conclusion:In conclusion, recognition and diagnosis of TA-TMA are challenging. Early recognition and prompt administration of complement blockage with eculizumab may be beneficial in selected cases. Further prospective research studies are recommended to improve the management and outcomes of TA-TMA.