Pediatric Nephrology最新文献

Background: Takayasu arteritis (TAK) is a granulomatous large-vessel vasculitis typically affecting young adult females. Pediatric cases are rare, and infantile onset is exceptional. Management relies on immunosuppression, with surgery reserved for severe complications.

Case report: We describe a now 5.5-year-old boy diagnosed with TAK at six months of age, presenting with hypertensive encephalopathy and kidney dysfunction. Despite treatment with corticosteroids and anti-TNFα, his kidney function deteriorated, leading to kidney failure and dialysis. At nearly three years of age, he underwent abdominal aorto-aortic bypass and bilateral nephrectomy due to progressive vascular narrowing and refractory hypertension. At age four, he successfully received a deceased-donor kidney transplant. Eighteen months post-transplant, he maintains excellent graft function and shows no signs of TAK recurrence.

Clinical significance: This case underscores the complexity of diagnosing and managing infantile TAK with multiorgan involvement. To our knowledge, he is among the youngest reported TAK patients to undergo successful kidney transplantation following major vascular surgery. His course demonstrates the potential for long-term remission and safe transplantation under standard immunosuppression, without continued anti-TNFα therapy. The literature is sparse regarding kidney failure and transplantation in TAK, particularly in infants.

Key management points: This case highlights key management dilemmas in infantile TAK, including clinical diagnosis, timing of surgery and transplantation, choice of immunosuppression, and long-term monitoring. It emphasizes the importance of a multidisciplinary approach and the need for collaborative research to address knowledge gaps in this rare but complex condition.

Background: Urinary pH is known to influence the solubility and excretion of lithogenic substances, yet its relationship with other metabolic parameters in pediatric stone formers remains underexplored. This study investigated the association between urinary pH and lithogenic risk factors in children and adolescents with urolithiasis compared to healthy controls.

Methods: A total of 400 pediatric patients (ages 3-18 years) with urinary stones and 372 age- and sex-matched healthy controls were included. All participants completed a 24-h urine collection for comprehensive metabolic analysis. Parameters assessed included urinary pH, BMI z-score, urine volume, osmolality, excreted creatinine, GFR, and urinary excretion of calcium, ionized calcium, oxalate, phosphate, magnesium, citrate, and uric acid. Two lithogenic risk indices were also evaluated: Bonn Risk Index (BRI) and Upper Metastable Limit osmolality (UMLOsm).

Results: Stone-formers demonstrated significantly higher urine volume, oxalate, calcium, ionized calcium, uric acid, and BRI compared to controls. In contrast, controls exhibited higher levels of urinary citrate, osmolality, and UMLOsm. Lower urine pH was associated with higher BMI z-scores and reduced urine volume. Calcium excretion increased with urine pH up to 7 before declining, whereas citrate and magnesium excretion rose at pH levels between 6.75 and 7.0, indicating a potential protective effect.

Conclusions: Our findings highlight the importance of individualized dietary and lifestyle guidance in pediatric stone formers, with a focus on maintaining a healthy BMI, adequate hydration, and urine pH between 6.75 and 7.0 to support protective factors like citrate and magnesium.

Background: Varicella zoster (VZV) vaccination pre-kidney transplant (Tx) can help prevent severe disseminated VZV in immunosuppressed recipients; however, studies have shown loss of humoral immunity post-Tx.

Methods: A retrospective analysis of 45 pediatric kidney Tx recipients with positive pre-Tx VZV IgG (>1.09 index). VZV IgG was assessed annually and compared with the induction agent used, the number of VZV vaccines received, and the interval between the last dose of VZV vaccine and Tx.

Results: Median age at Tx was 16.7 years (IQR 12.7-18.5). 11 of 45 (24.4%) patients lost immunity to VZV at a median of 12.6 months post-Tx. Those who lost VZV immunity were younger at the time of Tx, 12.4 years vs. 17.3 years (P = 0.05) and more likely to be on steroid-based immunosuppression 81.8% vs. 32.4% (P = 0.006). There were no differences between the induction agents used and the ability to maintain VZV IgG antibodies. Subjects who required ≥3 doses of VZV vaccine to develop VZV IgG seropositivity were at a higher risk for losing their anti-varicella antibody post-Tx (HR 3.81, 95% CI 1.09-13.30, P = 0.04). Receiving VZV vaccination <1 year prior to kidney Tx was associated with a higher risk for losing anti-varicella antibody after Tx (HR 6.97, 95% CI 2.08-23.34).

Conclusion: In this small cohort, pediatric kidney Tx recipients are more likely to lose VZV IgG in those who were younger at the time of Tx, on steroid-based immunosuppression, required 3 or more doses of VZV vaccination to seroconvert, or received VZV vaccine <1 year before Tx.

Hemoadsorption devices represent a significant advancement in extracorporeal organ support therapies, enabling the targeted removal of molecules that are not cleared by conventional kidney replacement therapies. Several hemoadsorption devices are currently available, each with distinct characteristics, mechanisms of action, and molecular adsorption profiles that enable their targeted use in a broad range of clinical scenarios in critically ill patients. Their application has recently been explored in pediatric patients with conditions such as septic shock, acute liver failure, hyperinflammatory syndromes, rhabdomyolysis, and intoxications. Preliminary findings suggest both clinical and biochemical improvements, including reduction in severity scores and circulating inflammatory mediators. However, the current body of evidence remains limited, primarily consisting of case reports and small case series, and often lacks robust clinical trial data. In the pediatric population, several challenges persist, including concerns about device sizing, the unintended removal of essential substances, such as medications, nutrients, albumin, and fibrinogen, and the procedural invasiveness, particularly for younger children. Future research should focus on patient stratification to identify those most likely to benefit from hemoadsorption, and on conducting large, multicenter studies to validate its effectiveness and utility in the pediatric setting.

Background: Renal scar can lead to hypertension and cardiovascular disease in children. Arterial stiffness is an important endpoint in the development of cardiovascular diseases and is a guide in the recognition of subclinical cardiovascular events. This study aimed to investigate oscillometric arterial stiffness data and sclerostin and galectin-3 levels in children with renal scar.

Methods: Patients aged 5-18 years with renal scar and controls were included. Arterial stiffness parameters were measured by oscillometric method. Sclerostin, galectin-3 and NGAL levels were determined by ELISA method.

Results: 108 patients and 66 controls were included. The patients had higher C-reactive protein (CRP) values and lower glomerular filtration rate (GFR) (p = 0.013, p = 0.005, respectively). Serum sclerostin levels, urine NGAL/creatinine (cr) and sclerostin/cr, galectin-3/cr, augmentation index, reflection magnitude and augmentation pressure were higher in patients (p = 0.026, p = 0.047, p = 0.000, p = 0.000, p = 0.013, p = 0.03, p = 0.02, respectively). Serum sclerostin was positively correlated with CRP and augmentation index (r = 0.22, p = 0.044; r = 0.355, p = 0.003, respectively). Urinary galectin-3/cr and NGAL/cr values were positively correlated with each other (r = 0.544, p = 0.000). Urinary galectin-3/cr was higher in patients with proteinuria than in those without proteinuria (p = 0.000). A ROC analysis showed that galectin-3/cr was predictive of proteinuria (area under the curve:0.726 ± 0.075, p = 0.048).

Conclusion: The detection of higher augmentation index and reflection magnitude in scarred patients might support the onset of subclinical atherosclerosis in these children. Higher serum sclerostin levels in scarred patients might be due to increased inflammation or arterial stiffness. Urinary galectin-3/cr could be used as tubular damage markers.

Background: As a rare autosomal recessive disorder, primary hyperoxaluria type 3 (PH3) presents diagnostic challenges. Our comparative analysis of clinical characteristics between patients with PH3 and non-PH patients revealed distinct characteristics that may facilitate the diagnosis of PH3.

Methods: Clinical data from pediatric patients with urolithiasis who had undergone whole-exome sequencing from 2016-2024 were analyzed. Patients were divided into PH3 group and non-PH group on the basis of genetic testing.

Results: Compared with non-PH patients, PH3 patients presented earlier onset (0.9 vs. 2.0 years, P = 0.021), higher incidence of nephrocalcinosis (22.22% vs. 3.17%, P = 0.008), higher serum calcium (2.55 vs. 2.49 mmol/L, P = 0.007), higher urinary oxalate levels (333.70 vs. 170.84 µg/mg, P = 0.008), higher urinary citrate levels (195.22 vs. 123.13 µg/mg, P = 0.015), lower urinary uric acid levels (838.44 vs. 1177.42 µg/mg, P = 0.040), and lower urinary calcium levels (113.27 vs. 352.21 µg/mg, P < 0.001). Subgroup analyses revealed that patients with PH3 had higher urinary oxalate levels (333.70 vs. 170.84 µg/mg, P = 0.042) than patients with cystinuria. Compared with patients in the other stone-related gene mutation groups, patients in the PH3 group presented earlier onset (0.9 vs. 2.5 years, P = 0.029), higher urinary oxalate levels (333.70 vs. 105.30 µg/mg, P = 0.045), higher urinary citrate levels (195.22 vs. 59.36 µg/mg, P < 0.001), and lower urinary calcium levels (113.27 vs. 421.24 µg/mg, P = 0.003). Patients with PH3 had greater incidence of nephrocalcinosis (22.22% vs. 0, P = 0.007), higher serum calcium levels (2.55 vs. 2.49 mmol/L, P = 0.030), higher urinary oxalate levels (333.70 vs. 182.74 µg/mg, P = 0.048) and lower urinary calcium levels (113.27 vs. 368.14 µg/mg, P = 0.004) than patients with negative molecular diagnoses.

Conclusion: Pediatric patients with PH3 are characterized by early onset, nephrocalcinosis, increased urinary oxalate excretion and lower urinary calcium excretion, which could provide guidance for earlier diagnosis of patients with PH3.