Case Report: Multiple autoimmune syndrome (MAS)-An unusual combination.

This is a case report of a Chinese adolescent boy who had multiple autoimmune syndrome (MAS) of a rare combination comprising type 1 diabetes, Hashimoto thyroiditis and childhood-onset systemic lupus erythematosus (SLE). He developed SLE within one year of symptom onset, presenting with necrotising histiocytic lymphadenitis and hepatitis. We highlight the importance for physicians to be aware of the need for continued surveillance for developing new autoimmune diseases in patients with multiple autoimmune diseases. It is also essential to remain vigilant for overlap syndrome as autoimmune diseases commonly share similar subphenotypes and non-specific autoantibodies. Long-term follow-up is warranted to define the final phenotype.

Motor Vehicle Collision and Acute Kidney Injury in Children.

OBJECTIVE: Our study aimed to determine the prevalence of acute kidney injury (AKI) in pediatric patients who sustained trauma due to motor vehicle collision (MVC) and identify factors associated with its development. METHODS: We retrospectively reviewed the clinical information and laboratory tests of pediatric patients who were admitted because of MVC from January 2014 to April 2021. The occurrence of AKI was our primary outcome, which was defined by the Kidney Disease Improving Global Outcomes criteria. A serum creatine kinase (CK) level of 1000 IU/L or greater was defined as rhabdomyolysis. RESULTS: Sixty-four patients (median age, 9.6 years) were included in the study. Twenty three of the patients (35.9%) developed AKI: stage 1 AKI in 18 (78.3%) and stage 2 AKI in 5 (21.7%) patients. Acute kidney injury patients had higher injury severity scores (no AKI: 5.0; stage 1 AKI: 10.5; stage 2 AKI: 26.0). Rhabdomyolysis was present in 21.1% of the patients and was associated with the overall AKI risk (odds ratio [OR], 7.3; 95% confidence interval [CI], 1.6-32.6) and stage 2 AKI (OR, 15.0; 95% CI, 1.4-163.2). Blood tests from AKI patients showed lower serum bicarbonate and base excess, higher leukocyte and CK levels on admission, and a higher peak CK in the first 72 hours of admission. Intubation in emergency department (OR, 11.1; 95% CI, 1.2-102.1) and surgical interventions (OR, 3.2; 95% CI, 1.0-9.8) were associated with AKI. Acute kidney injury patients required longer stay in pediatric intensive care unit (median 1.0 vs 2.3 days, P < 0.001) and hospital (median 4.1 vs 7.0 days, P = 0.010). CONCLUSIONS: Approximately one third of pediatric patients who sustained trauma from MVC developed AKI. Initial blood results, including higher CK and leukocyte count, and lower bicarbonate and base excess, were associated with the development of AKI. Rhabdomyolysis occurred in approximately one fifth of the patients and was significantly associated with AKI.

What and who should we focus in pediatric injury prevention - An analysis of critical pediatric trauma in a major trauma center in Hong Kong.

BACKGROUND: Understanding trauma epidemiology is essential in formulating region-specific strategies for injury prevention. Our study aimed to evaluate the characteristics and clinical outcomes of critical pediatric trauma requiring intensive care in Hong Kong. METHODS: A retrospective analysis was performed on pediatric patients who were injured and admitted to the pediatric intensive care unit (PICU), Queen Elizabeth Hospital, Hong Kong between 2014 and 2018. Clinical features of patients who sustained injuries before and after 2 years old were compared. RESULTS: 141 patients were admitted to the PICU due to trauma during the study period. Most patients sustained injuries due to fall (48.2%) or road traffic injuries (34.0%), with the latter more common in older patients. Two (1.4%) patients died due to the trauma. The majority (95.0%) of the survivors had good recovery assessed by Glasgow Outcome Scale. Patients younger than 2 years old were mostly injured at home due to fall while left unattended. Children in this age group were more likely to receive interventions, including mechanical ventilation (OR 2.61; 95% CI 1.15-5.95), anti-epileptic medications (OR 2.61; 95% CI 1.17-5.83), blood transfusion (OR 5.37; 95% CI 2.29-12.60) and inotropic support (OR 4.08; 95% CI 1.31-12.70), and require longer hospitalization (PICU stay 2.5 vs. 1.5 days, p = 0.011; hospital stay 10.9 vs. 6.9 days, p = 0.012). CONCLUSION: Fall injuries and road traffic injuries were common etiologies of critical pediatric trauma in Hong Kong. Patients younger than 2 years old had worse clinical outcomes. Parental education on home safety and importance of close supervision should be emphasized in this age group.

Acute kidney injury in pediatric non-traumatic rhabdomyolysis.

BACKGROUND: Our study aimed to determine the prevalence of acute kidney injury (AKI) in pediatric non-traumatic rhabdomyolysis, and to identify factors associated with its development. METHODS: Clinical information and laboratory tests of children with rhabdomyolysis who were admitted between 2009 and 2018 were reviewed retrospectively. Rhabdomyolysis was defined by a peak serum creatine kinase (CK) level > 1000 IU/L within the first 72 h of admission. The primary outcome was the occurrence of AKI within the first 7 days of admission, which was determined by the KDIGO criteria. RESULTS: A total of 54 patients with a median age of 7.8 years old were included. Ten (18.5%) patients developed AKI. AKI was relatively rare in children with viral myositis (2.6%), whereas all patients with rhabdomyolysis related to seizure or irritability/dystonia developed AKI. Patients with AKI had higher white cell count (10.6 vs. 4.5 × 109/L) and lower serum bicarbonate (19.4 vs. 25.5 mmol/L) on admission, with higher peak serum CK (23,086.0 vs. 3959.5 IU/L). The AKI group was more likely to present with positive urine results (myoglobinuria, dipstick heme or protein ≥ 2+). Peak serum CK had a good discriminatory power for stage 2-3 AKI (AUC 0.930, p = 0.005), with an optimal cut-off of 15,000 IU/L identified from the ROC analysis. CONCLUSIONS: The overall prevalence of AKI in pediatric non-traumatic rhabdomyolysis was 18.5%. Positive urine tests (myoglobinuria, dipstick heme or protein ≥ 2+), high white cell count, lower serum bicarbonate on admission, and high peak serum CK were associated with development of AKI.

Sequential use of hemoperfusion and single-pass albumin dialysis can safely reverse methotrexate nephrotoxicity.

BACKGROUND: High-dose methotrexate therapy (HDMTX) is a common form of chemotherapy used in children with high-grade malignancy such as osteosarcoma. Treatment with HDMTX requires careful monitoring of drug levels with folinic acid (leucovorin) rescue therapy. Toxicity from methotrexate is not uncommon and sometimes causes significant morbidity and mortality. CASE-DIAGNOSIS/TREATMENT: We report an 11-year-old child whose 24-h post-HDMTX serum level was 651.8 µmol/L (recommended level <20 µmol/L), which was complicated by septic shock and progressive renal and liver failure. As carboxypeptidase (glucarpidase) was not available locally, she was treated with the sequential use of charcoal hemoperfusion (CHP) and single-pass albumin dialysis (SPAD). The patient recovered without complications. Both liver and renal function recovered with no significant late sequelae. CONCLUSION: CHP and SPAD are effective extracorporeal methods of removing methotrexate. They provide alternative treatment options for critical care nephrologists in the management of methotrexate toxicity.

Paradoxical hypertension and salt wasting in Type II Bartter syndrome.

Ante/neonatal Bartter syndrome (BS) is a rare hereditary disorder. It is characterized by renal salt wasting, hypokalaemic metabolic alkalosis, high renin and aldosterone but normal blood pressure. We report a low birth weight newborn baby who presented with repeated apnoea shortly after birth as well as hyponatraemia, hypochloraemia, hyperkalaemia and metabolic acidosis. Her biochemical features mimicked pseudohypoaldosteronism but with initial hypertension, which had not been described in BS. Her subsequent genetic study confirmed two novel heterozygous mutations in the Exon 5 of KCNJ1 compatible with Type II BS.

Evaluating different imaging strategies in children after first febrile urinary tract infection.

We conducted a retrospective multicenter review to estimate the prevalence of urological abnormalities in Chinese children with first febrile urinary tract infection (UTI) and to evaluate the selective imaging strategy recommended by the NICE guideline for detecting underlying abnormalities. Atypical UTI was defined as in the NICE UTI guideline. Overall, 576 boys and 244 girls aged below 24 months were reviewed. All underwent ultrasound (US) and micturating cystourethrogram (MCUG) and 612 underwent DMSA scans. US was abnormal in 73 (8.9%) and vesicoureteral reflux was shown in 195 patients (23.8%). A total of 126 patients were considered to have remediable urological abnormalities requiring additional surgical or medical interventions. The NICE guideline yielded excellent negative predictive values (NPV) of 100-94.4% in girls but 91% in boys. If all boys underwent US and DMSA and only those with atypical UTI or abnormal US or DMSA proceeded to MCUG, then the NPV increased to 95.2% and 97.4% for boys aged below and above 6 months, respectively. These revised strategies would substantially save invasive studies-DMSA and MCUG in 27 and 74% of girls aged below and above 6 months, respectively, or MCUG in 23 and 59% of boys aged below and above 6 months, respectively.

Membranous lupus nephritis in Chinese children--a case series and review of the literature.

We retrospectively reviewed the cases of 13 lupus nephritis children with pure membranous glomerulonephritis (MGN; Group A) and ten children with mixed proliferative and membranous nephritis (Group B). The children were identified through a territory-wide survey of patients between 1990 and 2003. All were ethnic Chinese. Age at diagnosis ranged from 3.7 to 18.6 years (Group A) and from 9.6 to 22.1 years (Group B). Female-to-male ratios were 12:1 (Group A) and 9:1 (Group B). Group A patients were more often nephrotic than Group B patients (11/13 vs. 5/10, p = 0.17). The glomerular filtration rate (GFR) at presentation was normal in all but two patients (one from each group). For induction, Group B patients consistently received prednisolone and cyclophosphamide; in contrast, the cytotoxic regimens in Group A patients varied from cyclophosphamide (five patients), mycophenolate mofetil (two patients), azathiorpine plus cyclosporine (one patient), and azathioprine alone (one patient). After a median follow-up of 7.6-7.8 years, one Group A patient had died of fulminant lupus. One survivor in Group B had a GFR < 90 ml/min per 1.73 m(2). Proteinuria persisted in five Group A patients and two Group B patients. In conclusion, Group B patients had good prognosis in terms of survival and proteinuria control. The only death occurred in Group A, and five of the 12 survivors in this group had persistent proteinuria. Further studies are needed to define the best treatment for pure lupus MGN.