Chylothoraces are rare and although the vast majority are exudative in nature, it is important to be aware of the possible transudative causes such as heart failure, nephrotic syndrome and liver cirrhosis https://bit.ly/4ujq4rO.

Congenital complete atrioventricular block (CCAVB) is a well-recognized manifestation of neonatal lupus erythematosus (NLE), caused by transplacental transfer of maternal anti-Ro/Sjögren's Syndrome Type A (SSA) and anti-La/Sjögren's Syndrome Type B (SSB) antibodies. Although non-cardiac manifestations of NLE typically resolve within the first year of life, the long-term autoimmune risk in affected offspring remains unclear. We report a 24-year-old woman with CCAVB secondary to maternal Sjögren's disease who presented with acute heart failure, nephrotic-range proteinuria, bicytopenia, pericardial effusion, and findings concerning for Libman-Sacks endocarditis. Despite negative conventional lupus serologies, renal biopsy demonstrated Class IV/V lupus-like immune-complex glomerulonephritis with full-house immune complex deposition, leading the multidisciplinary team to favor seronegative lupus nephritis. This case demonstrates the diagnostic limitations of serology-based approaches in atypical lupus presentations and underscores the critical role of tissue biopsy. The case also raises questions regarding long-term autoimmune risk after autoimmune congenital heart block, an area for which prospective data and evidence-based surveillance strategies remain limited.

Renal disease in people living with HIV extends beyond classical HIV-associated nephropathy to include immune-mediated and vasculitic lesions, of which pauci-immune crescentic glomerulonephritis is among the rarest and most easily missed. We describe two biopsy-proven cases with anti-myeloperoxidase anti-neutrophil cytoplasmic antibody positivity. The first, established on antiretroviral therapy, presented with a pulmonary-renal syndrome after empirical anti-tubercular treatment had failed; biopsy showed cellular crescents without chronic change, and corticosteroids with rituximab achieved sustained clinical, biochemical and serological remission. The second, antiretroviral-naive, had glomerular haematuria and proteinuria documented at HIV diagnosis and an ischaemic stroke soon afterwards, but declined nephrological assessment; biopsy 3 years later was dominated by global glomerulosclerosis and interstitial fibrosis and he remained dialysis-dependent. The divergence turned on the histological stage at diagnosis rather than antiretroviral status and argues for early biopsy whenever unexplained renal decline with an active urinary sediment occurs in HIV.

A 12-year-old girl presented with steroid-resistant nephrotic syndrome and was found on kidney biopsy to have membranous nephropathy with a full-house immunofluorescence pattern. At presentation, she had marked hypercholesterolemia, with an LDL-C level of 589 mg/dL, and a family history suggestive of familial hypercholesterolemia. Multiple immunosuppressive therapies resulted in only transient or insufficient improvement in proteinuria, while conventional lipid-lowering agents failed to control hypercholesterolemia. On hospital day 98, evolocumab was initiated for refractory hypercholesterolemia. LDL-C levels declined rapidly, and proteinuria subsequently improved during the clinical course. Corticosteroids were tapered and discontinued. Evolocumab was administered for three doses, and nephrotic remission was achieved during the subsequent clinical course and maintained for nearly 1 year. This case illustrates that evolocumab may be an effective lipid-lowering option for selected pediatric patients with nephrotic syndrome complicated by severe refractory hypercholesterolemia. The subsequent improvement in proteinuria should be interpreted cautiously as a temporal association.

A 62-year-old man with biopsy-proven focal segmental glomerulosclerosis presented with nephrotic syndrome and enlarging bilateral perinephric masses. Contrast-enhanced CT showed large low-density lesions surrounding and compressing both kidneys, raising concern for retroperitoneal malignancy. Needle biopsy revealed bland spindle cells with low proliferative activity in myxoid stroma. Because he declined repeat biopsy, dual-tracer PET/CT was performed, showing diffuse mild-to-moderate 18F-FDG uptake (SUVmax, 6.5) and moderate 68Ga-FAPI uptake (SUVmax, 8.5), without focal markedly increased uptake. Integration of these imaging and pathologic findings supported the diagnosis of perinephric myxoid pseudotumor of fat and guided conservative management.

Objective: To evaluate the efficacy and safety of obinutuzumab (OBZ) in children with refractory nephrotic syndrome (RNS) who have a poor response to rituximab (RTX) therapy. Methods: This retrospective cohort study enrolled children with primary RNS who received OBZ because of poor response to RTX at the Children's Medical Center of Peking University First Hospital from July 2024 to December 2025. For each patient, outcomes during the OBZ treatment phase were compared with those during their preceding RTX treatment phase. Comparative parameters included duration of sustained B-cell depletion, duration of urinary protein remission, tapering of glucocorticoids and immunosuppressants, relapse frequency, and adverse events. Paired t-test, Wilcoxon signed-rank test, Mann-Whitney U test, χ[2] test and Fisher's exact test were adopted for statistical analysis according to the data type and distribution characteristics. Results: Thirteen children (10 males, 3 females) were enrolled. The age at disease onset was (47±13) months, and the age at first OBZ administration was (121±35) months. There were 3 cases of steroid-resistant and 10 cases of steroid-sensitive but frequently relapsing nephrotic syndrome. Compared with the RTX phase, the OBZ phase was associated with significantly longer durations of sustained B-cell depletion, sustained urinary protein remission, and annual glucocorticoid and calcineurin inhibitor (CNI) withdrawal (all P<0.05). The annual relapse frequency was significantly lower during OBZ treatment than during RTX treatment (0 (0, 0.3) vs. 1.9 (1.4, 2.6), Z=3.06, P=0.002). Regarding safety, 2 children developed mild transient infusion reactions (gastrointestinal discomfort and elevated blood pressure), which resolved with reduced infusion rate; 1 child discontinued OBZ due to concurrent pneumonia and otitis media; and 7 developed asymptomatic hypogammaglobulinemia. No severe adverse events, such as severe neutropenia, thrombocytopenia or organ dysfunction, were documented. Conclusions: For children with RNS who experience short B-cell depletion duration and frequent relapse after RTX therapy, OBZ provides more durable B-cell depletion, effectively prolongs urinary protein remission, reduces relapse risk, and facilitates safer glucocorticoid tapering. These findings suggest that OBZ may serve as a viable alternative therapeutic option for this specific pediatric population.

Inferior vena cava (IVC) agenesis is a rare congenital anomaly (prevalence 0.0005%-1%) often asymptomatic but can be complicated by deep vein thrombosis, particularly in the presence of acquired thrombophilia. There are 2 major presentations reported in literature, infrahepatic IVC agenesis associated mostly with lower-limb thrombosis and intrahepatic IVC agenesis associated frequently with splanchnic thrombosis and pulmonary embolism. We report the case of a 38-year-old man presenting with chronic bilateral leg ulcers evolving for 3 years. Cross-sectional imaging demonstrated partial agenesis of intra-hepatic IVC and complete agenesis of the infra-hepatic IVC up to the iliac bifurcation, a massive compensatory collateral network involving the azygos vein, hemiazygos, lumbar, and abdominal parietal systems, as well as multiple venous thrombosis of the portal vein, mesenteric vein, azygos vein and lower-limbs. The iliac veins drained via ascending lumbar veins to the hypertrophied azygos system. Morphology CT analysis showed a focal right renal infarct, but no atrophy was noted. Laboratory workup revealed a probable antiphospholipid syndrome (APS) and severe antithrombin III deficiency (22%), nephrotic syndrome, and alteration in glomerular filtration. This case illustrates the importance of recognizing radiological signs of IVC agenesis, particularly in the presence of atypical thrombosis or unexplained leg ulcers. The association with probable APS, multiple visceral thrombosis and renal function altered, makes this case exceptionally rare and educationally important.

Immune checkpoint inhibitors (ICIs) have transformed cancer therapy but produced immune-related adverse events across multiple organs. Renal toxicities are uncommon yet significant, and secondary serum amyloid A (AA) amyloidosis has emerged as a rare, underrecognized complication reflecting sustained systemic inflammation rather than direct immune-mediated kidney injury.