BACKGROUND:Pacemaker-induced cardiomyopathy (PICM) occurs in a subset of patients exposed to chronic right ventricular pacing (RVP) and results in left ventricular dysfunction due to pacing-related dyssynchrony. Left bundle branch area pacing (LBBAP) has emerged as a physiologic pacing strategy capable of restoring conduction system activation and reversing PICM. However, the acute effects of LBBAP on ventricular repolarization in PICM remain unclear. This study evaluated immediate repolarization changes after LBBAP in PICM compared with a control cohort of non-ischemic dilated cardiomyopathy (DCM) patients undergoing LBBAP. METHODS:We retrospectively analyzed clinical, electrocardiographic, and pacing data from patients receiving successful LBBAP. Baseline and post-implant ECG intervals (QT, QTc, Tp-Te) were manually measured; T-wave memory (TWM) was qualitatively assessed. Procedure parameters (RWPT, V6-V1 interpeak interval, output-dependent QRS transition), procedural success, and complications were recorded. RESULTS:Sixty patients were included (28 PICM vs. 32 DCM). Baseline LVEF was higher in patients with PICM compared to DCM (35 ± 8% vs. 29 ± 7%; p = 0.003). LBBAP success was similar (93% vs. 96%). PICM patients had wider paced QRS (149 ms vs. 128 ms; p = 0.02) and longer V6-V1 interval (46 ± 12 vs. 38 ± 10 ms; p = 0.02), but repolarization metrics showed no adverse changes and remained comparable between groups. QT shortened similarly (PICM: 476 → 437 ms; DCM: 466 → 432 ms), QTc minimally increased without group interaction, Tp-Te decreased, and TWM was frequent in both cohorts (63% vs. 64%). Complications rate was also comparable. CONCLUSION:LBBAP does not induce unfavorable acute repolarization changes in PICM and yields repolarization responses comparable to DCM controls, supporting its safety and physiologic benefit in PICM upgrades.
Background: Total parenteral nutrition (TPN) is widely used after major gastrointestinal surgery; however, its early systemic metabolic effects and temporal adaptation patterns remain incompletely characterized. This study applied a longitudinal plasma metabolomics approach to investigate time-dependent metabolic changes during early TPN administration. Methods: Plasma samples were collected from patients undergoing gastrointestinal surgery before TPN initiation (baseline, T0) and at 24 h (T1), 48 h (T2), and 72 h (T3). Untargeted metabolomic profiling was performed using complementary gas chromatography-mass spectrometry (GC-MS) and liquid chromatography-mass spectrometry (LC-MS) platforms. In total, 111 metabolites were detected. Analysis of variance (ANOVA) with baseline (T0) as the reference identified time-point-specific metabolic alterations during TPN administration. Results: At 24 h (T1), nominally significant increases were observed in glycine, tryptophan, isoleucine, and methionine, accompanied by decreases in sarcosine and oxalic acid. At 48 h (T2), elevated levels of glycine, isoleucine, valine, and phenylalanine persisted, while sarcosine, oxalic acid, and myo-inositol remained decreased. By 72 h (T3), sustained increases in glycine, isoleucine, valine, phenylalanine, proline, alanine, and tryptophan were accompanied by reduced levels of sarcosine, oxalic acid, and glucopyranose, reflecting coordinated alterations across multiple metabolite classes. Conclusions: Overall, the results demonstrated a distinct longitudinal metabolomic pattern characterized by increases in circulating amino acids and time-dependent changes in carbohydrate- and lipid-related metabolites within the first 72 h of TPN. This exploratory, time-resolved metabolomic study in 37 patients highlights the utility of untargeted metabolomics for characterizing early metabolic adaptation to parenteral nutrition and supporting postoperative metabolic monitoring.
Abstract Postoperative epilepsy patients may present with new neurological symptoms that raise concern for autoimmune or infectious etiologies. In this case, a 25-year-old man with intellectual disability and left hippocampal sclerosis underwent left temporal lobectomy and hippocampectomy. After a seizure-free interval of 45 days, he developed multiple generalized tonic–clonic seizures in the setting of a postoperative subdural hematoma. Following hematoma evacuation, he received intravenous antiseizure therapy that was complicated by a medication dosing error. Shortly thereafter, he exhibited prominent involuntary orofacial movements, confusion, and persistent electrographic abnormalities. Neuroimaging showed new, mild bilateral posterior cortical enhancement, and electroencephalography (EEG) revealed lateralized periodic discharges. The constellation of movement disorder, seizures, and EEG changes raised concern for an autoimmune encephalitis, which can occur after epilepsy surgery and requires timely immunotherapy. However, cerebrospinal fluid analysis and a comprehensive autoimmune antibody panel were unremarkable. Over the subsequent 2 weeks, symptoms resolved in parallel with normalization of antiseizure drug levels, without the use of immunotherapy. This case underscores the importance of considering medication toxicity in the differential diagnosis of acute postoperative neurological syndromes. Recognition of drug-induced phenomena can prevent both misdiagnosis of autoimmune encephalitis and unnecessary treatment delays in patients who truly require immunotherapy.
Thymic carcinoma (TC) is a rare and aggressive malignancy with limited evidence guiding second-line treatment after prior systemic therapy. We evaluated real-world outcomes of second-line systemic therapy in a multicenter cohort. Adults with unresectable, recurrent, or metastatic TC who received second-line systemic therapy after prior systemic therapy between 2010 and 2023 were retrospectively analyzed. Treatments were categorized as multidrug chemotherapy, gemcitabine monotherapy, PD-1 inhibitor therapy, or targeted therapy (sunitinib). For the primary comparison of multidrug chemotherapy versus gemcitabine monotherapy, propensity scores based on age, sex, ECOG performance status, Masaoka–Koga stage IV, prior surgery, and calendar year were used to generate overlap weights. Weighted Cox models used robust standard errors clustered by treatment center. A sensitivity propensity-score model additionally included first-line carboplatin–paclitaxel exposure (yes/no). A total of 107 patients from 20 treatment centers were included. Median age was 53.2 years (IQR, 41.5–58.0), 78 (72.9
Abstract Background Glycogen storage disease type III (GSD III) is a rare autosomal recessive metabolic disorder caused by mutations in the AGL gene, leading to a defect in the glycogen debranching enzyme. It primarily affects the liver and skeletal muscle, with childhood manifestations like hepatomegaly, fasting hypoglycemia, and elevated transaminases. Endocrine complications are exceptionally rare and the coexistence of hypogonadotropic hypogonadism and infertility has been scarcely reported. In this case report, we present a male patient with genetically confirmed GSD type III who developed hypogonadotropic hypogonadism and infertility, emphasizing the importance of recognizing multisystem involvement in metabolic diseases. Case Presentation A 28-year-old man with a known diagnosis of glycogen storage disease type III since childhood was admitted with infertility and decreased libido. Physical examination revealed short stature, reduced facial and body hair, and small testes. Laboratory studies showed markedly low total testosterone (78 ng/dL), low luteinizing hormone (LH) and follicle-stimulating hormone (FSH) levels, but normal prolactin and thyroid hormone levels, consistent with hypogonadotropic hypogonadism. Liver enzyme levels were persistently elevated (AST 154 U/L, ALT 132 U/L), and ultrasonography of the abdomen revealed hepatomegaly. Serum triglyceride levels were elevated as well. Scrotal ultrasonography demonstrated bilateral testicular atrophy, and semen analysis confirmed azoospermia. Magnetic resonance imaging (MRI) of the pituitary gland demonstrated pituitary stalk hypoplasia. Genetic testing reconfirmed homozygous AGL mutation consistent with GSD type III. Testosterone replacement therapy was begun, leading to significant improvement in libido, energy, and general well-being. The patient was referred for reproductive counseling for assisted fertility options. Conclusions This case highlights an uncommon endocrine manifestation of glycogen storage disease type III, in which chronic metabolic decompensation and hepatic dysfunction, possibly related to infundibular hypoplasia, may contribute to secondary hypogonadotropic hypogonadism and infertility. Recognition of endocrine complications in GSD is crucial for timely hormonal replacement and fertility counseling. Adult survivors of GSD should be followed up in multidisciplinary care settings to monitor and manage late-onset endocrine and metabolic complications effectively.
