The prevalence of multidrug-resistant Gram-negative infections, particularly carbapenem-resistant strains, has become a significant global health concern. Ceftazidime-avibactam (CZA) has emerged as a promising treatment option. However, data on its efficacy and safety in children are scarce, necessitating further investigation. We conducted a descriptive case series at a tertiary hospital in Spain from February 2019 to January 2022. Pediatric patients (<16 years) treated with CZA for confirmed or suspected multidrug-resistant Gram-negative infections were included. The clinical and microbiological characteristics, treatment approaches, and outcomes were examined. Eighteen children received CZA treatment. All had complex chronic conditions, with the most frequent underlying main diseases being liver transplantation (n = 8) and biliary atresia (n = 4). The predominant type of infection for which they received CZA was intra-abdominal infection caused or suspected to be caused by OXA-48-producing Klebsiella pneumoniae. CZA was generally well tolerated. Within the first month of starting CZA therapy, two patients died, with one case directly linked to the infection\'s fatal outcome. Some patients needed repeated courses of therapy due to recurrent infections, yet no resistance development was noted. In summary, the use of CZA showed effectiveness and safety, while the lack of resistance development highlights CZA\'s potential as a primary treatment option against OXA-48-producing infections.

Biliary ascariasis, resulting from the infestation of the biliary system by the roundworm Ascaris lumbricoides, is a rare but important cause of biliary blockage in children. In this case report, we present the clinical and radiological findings of a child who presented with symptoms and signs of ascending cholangitis, which followed biliary ascariasis. Double tube sign, typical for biliary ascariasis, was visualized on real time abdominal ultrasound examination.

BACKGROUND: Achromobacter xylosoxidans is a Gram-negative opportunistic aerobe, usually causing nosocomial infections in immunocompromised patients with manifestations including bacteremia, pneumonia, and catheter-related infections. However, A. xylosoxidans have not yet been reported to cause biliary system infections.
METHODS: A 72-year-old woman presented to the outpatient department of our hospital with a chief complaint of jaundice. Computed tomography of her abdomen revealed the presence of a mass of approximately 2.4 cm in the hilar portion of the common hepatic duct, consistent with hilar cholangiocarcinoma. We performed endoscopic retrograde cholangiopancreatography (ERCP) to decompress the obstructed left and right intrahepatic ducts (IHDs) and placed 10 cm and 11 cm biliary stents in the left and right IHDs, respectively. However, the day after the procedure, the patient developed post-ERCP cholangitis as the length of the right IHD stent was insufficient for proper bile drainage. The blood culture of the patient tested positive for A. xylosoxidans. Management measures included the replacement of the right IHD stent (11 cm) with a longer one (12 cm) and administering culture-directed antibiotic therapy, solving the cholangitis-related complications. After the cholangitis had resolved, the patient underwent surgery for hilar cholangiocarcinoma and survived for 912 d without recurrence.
CONCLUSIONS: A. xylosoxidans-induced biliary system infections are extremely rare. Clinical awareness of physicians and endoscopists is required as this rare pathogen might cause infection after endoscopic procedures.

UNASSIGNED: Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is frequently accompanied with type 1 autoimmune pancreatitis (AIP). Isolated IgG4-SC which is not accompanied with AIP is uncommon in clinical practice, and its manifestations are similar to those of hilar cholangiocarcinoma.
UNASSIGNED: A 55-year-old male presented with persistent aggravation of icteric sclera and skin. He was initially diagnosed with hilar cholangiocarcinoma and underwent surgery. However, positive IgG4 plasma cells were found in the surgical specimens. Thus, a pathological diagnosis of IgG4-SC was established. After that, steroid therapy was given and initially effective. But he was steroid dependent, and then received rituximab therapy twice. Unfortunately, the response to rituximab therapy was poor.
UNASSIGNED: It is crucial to differentiate isolated IgG4-SC from hilar cholangiocarcinoma to avoid unnecessary surgery. Future studies should further explore effective treatment strategy in patients who do not respond to steroids therapy. It is also required to develop novel and accurate diagnostic approaches to avoid unnecessary surgical procedures.

本文报道了1例原发性胆汁性胆管炎-自身免疫性肝炎重叠综合征（PBC-AIH OS）合并下肢软组织感染患者的诊治经过。患者为老年女性，因肝功能异常就诊入院，在完善肝穿刺病理后确诊PBC-AIH OS。治疗过程中出现下肢软组织感染，及时停用免疫抑制剂并针对副作用进行积极治疗后，创面愈合良好，在后续随访中再次使用免疫抑制剂未发现不良反应，目前复查肝功能、免疫指标均正常。本文通过该病例的诊治经过回顾总结该病的临床特点和联合免疫抑制治疗过程中处理、预防不良事件的经验，希望能提高我们对该病的认识和处理药物不良反应的经验。.

Alanine aminotransferase (ALT) is an enzyme that catalyzes the transfer of amino groups from alanine to ketoglutaric acid. ALT is an established marker of liver diseases. Occasionally, ALT levels may be abnormally low due to various factors, making accurate assessment difficult. To date, no studies have documented ALT alterations following Living donor liver transplantation (LDLT) in patients with low ALT levels. Here, we present a case of abnormally low ALT levels that were ameliorated by LDLT. A 27-year-old woman underwent LDLT for refractory cholangitis with biliary atresia. The patient\'s preoperative ALT level was 1 IU/L. Following graft reperfusion, ALT levels increased (peak value, 456 IU/L), primarily attributed to the donor liver. After LDLT, ALT levels consistently surpassed the lower limit. The differential diagnosis of abnormally low ALT levels suggested a genetic mutation as the most probable underlying cause. Even after LDLT, ALT levels in organs other than the transplanted liver would remain abnormally low. Therefore, to prevent underestimating liver damage, the standard ALT range for such cases should be set lower than the typical range.

Ampullary adenocarcinoma is a rare malignancy that originates in the ampulla of Vater. It typically presents in the seventh decade of life. This condition shares overlapping features with periampullary tumors such as pancreatic cancer, but treatment modalities and prognosis vary. Histology will demonstrate either intestinal or pancreato-biliary epithelial subtype in ampullary adenocarcinoma. Despite its rare occurrence, ampullary adenocarcinoma should be included as a differential in elderly patients presenting with biliary obstruction. This case presentation is unique as it highlights the importance of histopathological findings and their progression. In this case, initial histology results revealed tubulovillous adenomatous polyps, but later biopsies revealed adenocarcinoma cells. These findings suggest that ampullary adenocarcinoma and several gastrointestinal cancers share a similar mechanism of action as it is related to the adenoma-to-carcinoma sequence. This case presentation aims to highlight the rare occurrence of this phenomenon at the ampulla of Vater.

先天性肝纤维化目前仍被认为是一种罕见的常染色体隐性遗传性疾病，该病与胆管板畸形所致的肝内胆管遗传发育障碍有关。现以1例多囊肾/多囊肝病变1基因突变致胆管炎型先天性肝纤维化患者为例，探讨该病发病原因、临床表现、诊断要点以及治疗进展，以期能够在一定程度上提高肝胆科医师对该病的认识，从而有效提高早期诊断率。.

BACKGROUND Hydatid disease is a common parasitic infection in many areas of Asia, South America, and Africa. It can affect any organ, most commonly the liver. The hydatid is often asymptomatic and the diagnosis is made when complications arise. The most common complication of this disease is opening in the bile ducts, which is a life-threatening condition causing serious acute cholangitis. We report a case of acute cholangitis caused by hydatid cyst rupture into the right bile duct. CASE REPORT A 33-year-old woman, with no medical or surgical history, presented to our Emergency Department with abdominal pain, jaundice, and fever for 3 days prior to admission. The patient was hemodynamically stable. In the examination, we noticed right upper-quadrant tenderness with guarding, icterus sclera, and negative Murphy sign. A CT scan showed a liver hydatid cyst of the 4th and 8th of segments, with intrahepatic and extrahepatic biliary duct dilation. The cyst communicated with the right hepatic bile duct via a large fistula. A diagnosis of acute cholangitis was made and she underwent conservative treatment with external drainage of the pericystic cavity through the biliary duct. The postoperative course was uncomplicated and she was discharged 15 days later. CONCLUSIONS The surgical approach to hepatic hydatid must be customized based on the specific characteristics of the cyst and associated complications. Acute hydatid cholangitis is a rare but serious complication of a hydatid cyst, which requires early diagnosis and adequate surgical management.

The report describes a middle-aged woman with acute cholangitis combined with acute myeloid leukaemia, and examination suggesting that she was also a patient with a rare case of total visceral inversion. The analysis of this case helps clinicians to deepen the differential diagnosis of rare diseases and improve the timeliness and accuracy of diagnosis.