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Immunoglobulin A nephropathy is a predominant cause of chronic kidney disease in the pediatric population. Despite standard-of-care treatments, a significant subset of children remains refractory to conventional immunosuppressive therapies, necessitating exploration of novel targeted interventions. Telitacicept, a recombinant fusion protein targeting both B lymphocyte stimulator and a proliferation-inducing ligand, has demonstrated efficacy in adult immunoglobulin A nephropathy; however, real-world evidence in pediatric cases remains limited. This exploratory retrospective case series evaluated the efficacy and safety of telitacicept in seven pediatric patients with refractory immunoglobulin A nephropathy. Telitacicept was administered subcutaneously once weekly using a weight-stratified dosing regimen (80 mg for patients weighing <40 kg and 160 mg for patients weighing ≥40 kg) over a 24-week period. At the end of follow-up, clinical remission (defined as urine protein-to-creatinine ratio <0.2 g/g) was achieved in five of the seven patients (71.4%). Notably, a rapid therapeutic response was observed, with 85.7% of patients achieving at least partial remission by month 3. Although estimated glomerular filtration rate remained stable throughout the study, significant reductions were observed in serum immunoglobulin A, immunoglobulin G, and immunoglobulin M levels (all p < 0.05), correlating with clinical improvement. However, two patients with specific pathological features, including minimal change disease or advanced chronic lesions, showed limited response to treatment. The treatment was well tolerated, with only one instance of mild upper respiratory tract infection and no serious adverse events reported. These preliminary findings suggest that weight-stratified telitacicept is a promising and safe adjuvant therapy for pediatric patients with refractory immunoglobulin A nephropathy, although its efficacy may be influenced by individual clinicopathological characteristics.

Purpose Obtaining an objective, reproducible, and accurate assessment of volume status is one of the more difficult tasks in the perioperative arena. Since its advent in 2020, the Venous Excess Ultrasound (VExUS) score has gained popularity in the minimally invasive assessment of venous congestion. The VExUS exam has been well described as an additional series of images (hepatic vein, portal vein, and intrarenal vein) obtained with a phased-array probe during a transthoracic echocardiogram. Nevertheless, there are no descriptions of comprehensive VExUS exams performed using transesophageal echocardiography (TEE)—a modality that is routinely employed in patients undergoing cardiac surgery. Clinical features We describe techniques to acquire and interpret a comprehensive TEE-supported VexUS exam, which may be used to optimize the perioperative care of cardiac surgical patients. Conclusion Given the risks of fluid overload in critically ill cardiac surgery patients, TEE-supported VExUS examination may be a way to reduce morbidity in this population.

PurposeBrain death/death by neurologic criteria (BD/DNC) may be determined in many countries by a clinical examination that shows coma, brainstem areflexia, and apnea, provided the conditions causing reversible loss of brain function are excluded a priori. To date, accounts of recovery from BD/DNC in adults have been limited to noncompliance with guidelines.Clinical featuresWe report the case of a 72-yr-old man with a combined primary infratentorial (hemorrhagic) and secondary global (anoxic) brain lesion in whom decompressive craniectomy of the posterior fossa and six-hour therapeutic hypothermia (33–34°C) followed by 8-hour rewarming to ≥ 36°C were conducted. Thirteen hours later, clinical findings of brain function loss were documented in addition to guideline-compliant exclusion of reversible causes (arterial hypotension, intoxication, depressant drug effects, relevant metabolic or endocrine disequilibrium, chronic hypercapnia, neuromuscular disorders, and administration of a muscle relaxant). Since a primary infratentorial brain lesion was present, German guidelines required further ancillary testing. Doppler ultrasonography revealed some preserved cerebral circulation, and BD/DNC was not diagnosed. Approximately 24 hr after rewarming to ≥ 36°C, the patient exhibited respiratory efforts. He continued with assisted respiration until final asystole/apnea, without regaining additional brain function other than mild signs of hemispasticity. Follow-up computed tomography showed partial herniation of the cerebellum through the craniectomy gap of the posterior fossa, alleviating caudal brain stem compression.ConclusionsTherapeutic decompressive craniectomy of the posterior fossa may allow for delayed reversal of apnea. In these patients, proof of cerebral circulatory arrest should be mandatory for diagnosing BD/DNC.

This study aimed to evaluate the efficacy of external fenestration using a reserved guide wire and a Viabahn-covered stent in Stanford type B aortic dissection that involves the left subclavian artery. Eight patients with Stanford type B aortic dissection involving the left subclavian artery were treated from March 2023 to May 2024 at Yinzhou People’s Hospital using a reserved guide wire external fenestration and a Viabahn-covered stent. This study compared a novel external fenestration technique (reserved guide wire + Viabahn stent) with traditional in-situ fenestration for Stanford type B aortic dissection involving the left subclavian artery. Clinical data showed that the new method achieved full technical success in vessel reconstruction, with no postoperative deaths, minimal complications, and shorter hospital stay versus the conventional approach. The fenestration complication incidence was also significantly reduced during a follow-up period of 1–3 months. Studies on larger samples can be performed in the future to verify the present results. External stent fenestration with a reserved guide wire demonstrated significant efficacy in treating large-branch artery disease, resulting in substantial improvements in prognosis in terms of complications. Therefore, this technique is worthy of further exploration.

Signet ring cell carcinoma (SRCC) of the colon is a rare histologic subtype of colorectal cancer, accounting for <1% of cases, and is associated with aggressive clinical behavior and poor prognosis. We report two cases with advanced colonic SRCC. The first case involved a 58-year-old female with obstructing rectal SRCC and extensive pelvic invasion with adnexal metastasis. The second is a 45-year-old male with an obstructing splenic flexure SRCC and peritoneal dissemination, found to have microsatellite instability and multiple oncogenic alterations. Both cases required aggressive surgical intervention and illustrate the need for individualized, multimodal treatment strategies. Colonic SRCC is an uncommon but highly aggressive malignancy that frequently presents at an advanced stage. Early recognition, comprehensive pathologic and molecular evaluation, and coordinated multimodal management are critical to optimizing outcomes.

Lipoprotein(a) (Lp(a)) is an atherogenic low-density lipoprotein (LDL)-like particle that is currently regarded as a non-modifiable risk factor for atherosclerotic cardiovascular disease. The number of patients detected with elevated Lp(a) concentrations has been increasing in recent years, although the implication of this finding is unclear for patients and physicians. We screened our lipid clinic database for patients aged >65 years with very high Lp(a) concentrations, which were defined as >230 nmol/L, and cardiovascular outcomes were assessed. The patients’ (n = 16) mean (±standard deviation) age was 72.2 ± 7.1 years and the mean Lp(a) concentration was 313 ± 68 nmol/L. After a cumulative 129.0 patient-year follow-up (mean: 8.1 ± 4.2 years), the mean age was 80.3 ± 7.0 years. We observed a low baseline prevalence of cardiovascular events, with only two patients having a history of cardiovascular events. Furthermore, zero incident adverse cardiovascular events were recorded over the follow-up. Therefore, very high Lp(a) concentrations and disease-free old age are not mutually exclusive. Our aggregated clinical experience is that there is only a modest association between elevated Lp(a) concentrations and adverse outcomes. Nonetheless, we still advise treating modifiable risk factors in these patients.

Subacute thyroiditis is a condition with varied and often misleading symptoms, which can lead to diagnostic delays and inappropriate treatment. This case series focuses on 32 patients who presented with unilateral pharyngalgia as the primary symptom and were ultimately diagnosed with subacute thyroiditis. The patients, aged 28–52 years with a female predominance (23 females and nine males), reported pharyngeal pain that lasted from 7 days to 3 months, with 19 having left-sided and 13 having right-sided initial pain. Of these patients, 43.7% (14/32) were initially misdiagnosed, primarily as pharyngitis or tonsillitis. Key diagnostic findings included thyroid tenderness, abnormal thyroid function, elevated erythrocyte sedimentation rate, characteristic manifestations on thyroid ultrasound, and decreased radioactive iodine uptake. Treatment varied by pain severity. In conclusion, unilateral pharyngalgia is frequently mistaken as other common throat conditions. Thyroid palpation should be routine in patients with unilateral pharyngalgia; positive findings warrant further tests (C-reactive protein, ultrasound, radioactive iodine uptake) to confirm subacute thyroiditis.

Bilateral basal ganglia germinomas are exceptionally rare intracranial germ cell tumors associated with significant diagnostic delays and irreversible neurological impairment. This study addressed this diagnostic challenge by analyzing the largest dedicated cohort of histopathologically confirmed bilateral basal ganglia germinomas (n = 8). We identified alarming diagnostic delays (median, 1.2 years) in 62.5% of cases, culminating in irreversible deficits such as paralysis and cognitive impairment. Key early clinical indicators included adolescent predominance (mean age, 12.4 years), marked male predominance (7:1), cognitive decline (87.5%), and hemiparesis or monoparesis (62.5%). Magnetic resonance imaging detected cerebral atrophy in 87.5% of patients, with 25.0% showing concomitant mass lesions and atrophy, a distinctive radiological pattern not previously emphasized in unilateral basal ganglia germinomas. Cerebrospinal fluid beta-human chorionic gonadotropin analysis demonstrated superior sensitivity to serum testing (57.1% vs 28.6%). To mitigate devastating outcomes, we propose integrated cerebrospinal fluid beta-human chorionic gonadotropin screening combined with magnetic resonance imaging, with an emphasis on atrophy detection. In patients exhibiting progressive deficits or serial radiological progression despite negative biomarker results, stereotactic biopsy should be prioritized to enable early intervention and preserve neurological function.

PurposeProlonged postoperative neuromuscular respiratory paralysis after administration of a nondepolarizing neuromuscular blocking agent is a serious concern during anesthetic management of patients with Charcot-Marie-Tooth disease (CMTD). Some recent reports have described rocuronium use without respiratory paralysis in CMTD patients when sugammadex was used for its reversal. We report a case in which an induction dose of rocuronium caused a prolonged respiratory paralysis in a patient with undiagnosed type 1A CMTD (CMT1A).Clinical featuresA 63-yr-old-male with an American Society of Anesthesiologists Physical Status score of III underwent a left hip arthroplasty under general anesthesia for osteoarthritis. Preoperative pulmonary function testing indicated a restrictive impairment. Anesthesia was induced with fentanyl, remifentanil, propofol, and 0.73 mg·kg-1 of rocuronium. The train-of-four (TOF) count was 0 for the 273-min duration of surgery. After repeated doses of sugammadex failed to recover the TOF count and spontaneous respirations, a total of 1,200 mg (17.3 mg·kg-1) of sugammadex, which was assumed to be a sufficient amount for capturing the residual rocuronium, was administered. Although the patient expressed that he was awake via eye blinking, he could not breathe. Thus, he was placed on mechanical ventilation for 18 hr after surgery. A postoperative neurology consultation revealed a delayed nerve conduction velocity of 20 m·sec-1 and a mutated duplication of the PMP22 gene; a diagnosis of CMT1A was made.ConclusionsOur case shows that rocuronium can cause a prolonged neuromuscular respiratory paralysis refractory to sugammadex in patients with CMT1A and impaired respiratory function. Our case may also indicate that restrictive pulmonary impairment and low nerve conduction velocity of 20 m·sec-1 are predictive factors that cause prolonged neuromuscular respiratory paralysis refractory to sugammadex in CMT1A.

A group of four healthy Canadian travellers visited a cenote in the Yucatán peninsula in April 2024 and subsequently developed symptomatic histoplasmosis. Diagnosis was made in the acute period with a positive urine antigen test in three of the cases. Two developed severe presentations and were treated with itraconazole, including a three-year-old child with disseminated disease. The sensitivity of different modalities for diagnostics depends on the timing and severity of illness, with urine antigen being most sensitive in early infection, serology converting 4-8 weeks following exposure and cultures generally of low sensitivity. Treatment depends on the disease manifestations and host immunologic status. Many patients have relatively mild, self-limited, influenza-like illness and the diagnosis may be overlooked. Given the number of Canadian tourists travelling to the Yucatán peninsula and the popularity of visiting cenotes, awareness of the risk of histoplasmosis associated with this exposure should be promoted.