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Progressive familial intrahepatic cholestasis type 3 (PFIC-3) is a rare autosomal recessive disorder with poor prognosis. It is caused by pathogenic variants of the ATP binding cassette subfamily B member 4 () gene and usually progresses from chronic cholestasis with or without jaundice to portal hypertension and end-stage liver disease within the first to second decade of life. Few reported PFIC-3 patients presented with atypical clinical symptoms, therefore, often misdiagnosed if without family history. Herein, we report a 16-year-old male who was admitted to our hospital due to acute episodes of jaundice and intense pruritus, subsequently progressed to end-stage liver disease. Laboratory examinations showed no evidence of liver injury caused by viral, autoimmune, drug or liver tumors. Ursodeoxycholic acid and dexamethasone did not relieve his symptoms and he underwent liver transplantation successfully. Targeted next-generation sequencing identified that the patient was a compound heterozygote for two missense mutations (c.959C > T/c.1429C > A) in the gene. The mutation c.1429C > A (p.Q477K) is a novel heterozygous mutation. We constructed a three-dimensional model of this novel pathogenic variant using the SWISS MODEL program and found that the patient's protein is an ATP hydrolysis deficient mutant. The postoperative pathological diagnosis showed intrahepatic cholestasis with progression to cirrhosis. Negative liver tissue immunohistochemistry of MDR3 was found in the explanted liver. The patient was diagnosed with PFIC-3, and his symptoms improved dramatically with liver transplantation. In conclusion, for young patients with acute cholestasis, pruritus, jaundice, growth retardation, and enlargement of the liver and spleen, the possibility of inherited metabolic liver diseases should be considered, detailed medical and family history should be collected, and metabolic screening tests as well as gene tests are necessary for correct diagnosis. Increasing the coverage of PFIC3 is meaningful and thus can improve the current understanding of this disease.

There are several methods to control pain, especially in traumatic patients with rib fractures. Intrapleural analgesia (IPA) and intercostal block methods are recommended in patients with rib fractures to control pain. Here, we aimed to evaluate and compare the effects of IPA and intercostal block on patients' clinical conditions.

To analyze the clinical manifestations and imaging features of a hospitalized patient with intermittent headache who was finally diagnosed with von Hippel-Lindau (VHL) syndrome and to perform whole-exon gene detection to improve the understanding of the diagnosis and treatment strategies of the disease.

Dual-tasking (DT) while walking is common in daily life and can affect both gait and cognitive performance depending on age, attention prioritization, task complexity and medical condition. The aim of the present study was to investigate the effects of DT on cognitive DT cost (DTC) (i) in a dataset including participants of different age groups, with different neurological disorders and chronic low-back pain (cLBP) (ii) at different levels of cognitive task complexity, and (iii) in the context of a setting relevant to daily life, such as combined straight walking and turning.

Inflammation plays an important role in the occurrence and development of neuropathic pain. Immune-responsive gene 1 (IRG1) decarboxylates -aconitate to produce itaconate in the mitochondria. Itaconate serves as an immunomodulator of macrophages and represses inflammation in infectious diseases. Recently, a study showed that an itaconate derivative inhibits neuroinflammation and reduces chronic pain in mice. However, the function and molecular mechanisms of endogenous itaconate in neuropathic pain have not been fullyelucidated. In this study, the content of itaconate in the ipsilateral spinal cord after nerve-injured mice was detected with mass spectrometry. The mouse was constructed to determine the role of endogenous itaconate in the chronic constriction nerve injury (CCI) model. The analgesic effect of exogenous itaconate was assessed with intraperitoneal and intrathecal administration in both male and female CCI mice. The spinal application of 4-OI also reduced the evoked responses of wide dynamic range neurons in CCI mice. The potential analgesic mechanism of itaconate was explored through molecular biology experiments and verified in Interleukin mice. We found the levels of itaconate and IRG1 in the spinal cord significantly increased after CCI. deficiency aggravated the mechanical and heat hypersensitivity, while the exogenous administration of the itaconate derivative 4-OI alleviated the neuropathic pain in male and female CCI mice. Mechanistically, the treatment of 4-OI increased the level of IL-10 and activates STAT3/β-endorphin pathway in the spinal cord, and the analgesia effect of itaconate was impaired in mice. Finally, we showed that the upregulation of IL-10 induced by 4-OI was mainly from spinal neurons through Nrf2 pathway. This study demonstrated the analgesic effect of endogenous and exogenous itaconate in the neuropathic pain model, suggesting that the spinal IL-10/STAT3/β-endorphin pathway might mediate the analgesia effect of itaconate.

We present a rare case of Mollaret's meningitis in a young patient with seven prior episodes of recurrent meningitis. The patient presented with headache, fever, neck stiffness, nausea, and vomiting. Brain imaging revealed no acute abnormalities. Lumbar puncture revealed elevated nucleated cells with lymphocytic predominance. The patient was started on antimicrobials including acyclovir. Cerebrospinal fluid polymerase chain reaction was positive for herpes simplex virus type 2. Her 2-day hospital course was uncomplicated, and she was discharged in good condition. Mollaret's meningitis, also known as recurrent benign lymphocytic meningitis, is a rare clinical disorder characterized by at least three recurrent episodes of meningitis associated with spontaneous recovery with or without antiviral therapy. Herpes simplex virus type 2 has frequently been implicated in the setting of this illness.

Postoperative poor sleep quality and decreased gastrointestinal motility function are common clinical problems. This study investigated the effects of dexmedetomidine (DEX) combined with sufentanil for patient-controlled analgesia (PCA) on postoperative sleep quality and gastrointestinal motility function after surgery in patients with colorectal cancer. Patients undergoing colorectal cancer surgery were randomly divided into three groups, DEX 0, 200, or 400 μg, each combined with sufentanil 150 μg for PCA immediately after surgery. The primary outcome was sleep quality in the first 7 days after surgery based on the Athens Insomnia Scale (AIS) score. The secondary outcome was postoperative gastrointestinal motility recovery evaluated by the time of first flatus, first feces and first diet. Postoperative pain intensity, side effects and the length of postoperative hospital stay were also compared among groups. The study was registered with the Chinese Clinical Trial Registry (https://www.chictr.org.cn/enIndex.aspx, ChiCTR2000032601). Ultimately, 210 cases were included. Sleep quality was better in the DEX 200 μg group and DEX 400 μg group than in the DEX 0 μg group. Overall, in the DEX 200 μg group and DEX 400 μg group, the AIS score ( < 0.05) and the incidence of sleep disturbance (7.3%, 4.5% vs. 19.6%, < 0.001) were lower than those in the DEX 0 μg group in the first 7 days after surgery. There were no significant differences in postoperative gastrointestinal motility among the three groups in the total surgical categories ( > 0.05). In the laparoscopic surgery patients of each group, the time of postoperative first flatus ( = 0.02) and first feces ( = 0.01) was significantly longer in the DEX 400 μg group than in the DEX 0 μg group. There were no differences in postoperative pain intensity, side effects or length of postoperative hospital stay ( > 0.05). The continuous infusion of DEX (200 or 400 μg) for PCA significantly improved postoperative sleep quality after colorectal cancer surgery. DEX (200 μg) was better at improving postoperative sleep quality without affecting gastrointestinal motility function than DEX (400 μg) in patients who underwent laparoscopic colorectal cancer surgery.

To explore changes of depression, anxiety and sleep disturbance in patients with episodic cluster headache inside and outside the attack period and assess the therapy to improve the treatment.

Propofol combined with remifentanil is the most common anesthesia method in laparoscopic hysteromyomectomy. However, whether the combination of the two is helpful to patients undergoing hysteromyomectomy still requires unclear.

In Gamma Knife (GK) radiosurgery, dose rate decreases during the life cycle of its radiation source, extending treatment times. Prolonged treatments influence the amount of sublethal radiation injury that is repaired during exposure, and is associated with decreased biologically-equivalent dose (BED). We assessed the impact of treatment times on clinical outcomes following GK of the trigeminal nerve – a rare clinical model to isolate the effects of treatment times. This is a retrospective analysis of 192 patients with facial pain treated across three source exchanges. All patients were treated to 80 Gy with a single isocenter. Treatment time was analyzed in terms of patient anatomy-specific dose rate, as well as BED calculated from individual patient beam-on times. An outcome tool measuring pain in three distinct domains (pain intensity, interference with general and oro-facial activities of daily living), was administered before and after intervention. Multivariate linear regression was performed with dose rate/BED, brainstem dose, sex, age, diagnosis, and prior intervention as predictors. BED was an independent predictor of the degree of improvement in all three dimensions of pain severity. A decrease in dose rate by 1.5 Gy/min corresponded to 31.8% less improvement in the overall severity of pain. Post-radiosurgery incidence of facial numbness was increased for BEDs in the highest quartile. Treatment time is an independent predictor of pain outcomes, suggesting that prescription dose should be customized to ensure iso-effective treatments, while accounting for the possible increase in adverse effects at the highest BEDs.