AIM:To report and analyze cases of sterile intraocular inflammation(IOI)following intravitreal faricimab injections in patients treated for neovascular age-related macular degeneration(nAMD)and diabetic macular edema(...AIM:To report and analyze cases of sterile intraocular inflammation(IOI)following intravitreal faricimab injections in patients treated for neovascular age-related macular degeneration(nAMD)and diabetic macular edema(DME).METHODS:This double-center case series included nine eyes of six patients who developed uveitis after faricimab therapy.Comprehensive clinical evaluation was performed,including slit-lamp examination,intraocular pressure(IOP)measurement,fluorescein and indocyanine green angiography(ICGA),and laboratory tests.Inflammatory responses were treated with topical or systemic corticosteroids,and patients were monitored for visual acuity and inflammatory activity.RESULTS:The incidence of IOI was 0.8%per patient(Innsbruck)and 0.23%(Czechia),with inflammation typically occurring between the third and sixth injection(mean interval:10d post-injection).Inflammator y presentations ranged from anterior uveitis to posterior segment involvement.One notable case demonstrated novel choroidal hypofluorescent lesions on angiography,suggesting deeper ocular involvement.The mean patient age was 76y;five of six affected patients were female.All cases responded to local and systemic corticosteroids,with full recovery of initial visual acuity.CONCLUSION:Sterile IOI after faricimab appears to be a rare but relevant adverse event.Although the incidence falls within expected ranges for anti-vascular endothelial growth factor(anti-VEGF)agents,the observed choroidal involvement represents a potentially new safety signal.Prompt diagnosis and corticosteroid therapy are effective in all cases.Our findings support the need for vigilant post-marketing surveillance and further studies to better understand the underlying mechanisms and risk factors of faricimab-associated inflammation.展开更多
BACKGROUND Congenital hypothyroidism(CH)is a common condition in both preterm and term infants characterized by either thyroid gland absence or hypofunctionality.The clinical association of refractory lactic acidosis ...BACKGROUND Congenital hypothyroidism(CH)is a common condition in both preterm and term infants characterized by either thyroid gland absence or hypofunctionality.The clinical association of refractory lactic acidosis and heart failure has rarely been observed in cases of pediatric patients with CH pathology.Here,we explored the etiological relationship between CH,heart failure,and refractory lactic acidosis to reflect the importance of thyroid function screening in neonates with heart disease.CASE SUMMARY A 33-day-old extremely premature female infant presented with tachypnea,respiratory distress,recurrent infections,and abdominal distension postnatal.On admission to our facility,she had cardiomegaly,hepatomegaly,and lactic acidosis(revealed on blood gas analysis),with lactate progressively rising to 25 mmol/L.Chest radiographs showed pulmonary congestion,while echocardiography revealed cardiac enlargement,left ventricular wall thickening,and pericardial effusion.Initial management aimed at correcting acidosis and treating heart failure proved ineffective.After reassessment,thyroid function tests showed significantly decreased triiodothyronine,free triiodothyronine,thyroxine,and free thyroxine levels,with a significantly increased thyroidstimulating hormone level,confirming a CH diagnosis.Levothyroxine was administered,resulting in rapid correction of lactic acidosis and gradual improvement of thyroid function and systemic symptoms,culminating in full recovery and discharge.We also reviewed the relevant literature on thyroid and cardiac dysfunctions in order to explore their deeper association.CONCLUSION This case links CH-induced heart failure with refractory lactic acidosis,urging prompt thyroid screening in affected neonates to reduce mortality.展开更多
BACKGROUND Edwardsiella tarda(E.tarda)belongs to the family Enterobacteriaceae and is generally seen to cause infections mainly in fish,but is also capable of infecting humans.Extraintestinal infections occur in patie...BACKGROUND Edwardsiella tarda(E.tarda)belongs to the family Enterobacteriaceae and is generally seen to cause infections mainly in fish,but is also capable of infecting humans.Extraintestinal infections occur in patients with certain risk factors,including immunocompromised status.We recently diagnosed a case of spontaneous bacterial peritonitis(SBP)due to E.tarda in an immuno-compromised dialysis patient.CASE SUMMARY Patient was a 55-year-old male,with a history of diabetic nephropathy being treated with hemodialysis three times a week.He was referred to our hospital due to an increased volume of ascites,and blood examination revealed increased inflammatory reaction.At our emergency department,he developed fever,disturbance of consciousness,abdominal distension,and abdomen-wide pain.In addition,a dialysis shunt was confirmed in his right forearm,and the shunt site showed no signs of inflammation.No wounds were confirmed on or in his body.A blood examination revealed increased values of white blood cells,C-reactive protein,and creatinine.Plain chest and abdominal computed tomography scanning revealed increased ascites volume.Abdominal paracentesis was performed and a Gram stain revealed Gramnegative bacillus.These findings prompted diagnosis of SBP.The patient was admitted and treated with cefmetazole,causing fever resolution and symptom improvements.Later,E.tarda was identified in ascites culture.The patient improved with decreased inflammatory response and was discharged on the 12th day of hospitalization.The antibiotic was terminated after 14 days of treatment.SBP in this case may have developed from chronic renal failure and diabetes mellitus.CONCLUSION We report the first known case of SBP due to E.tarda in an immuno-compromised dialysis patient.展开更多
BACKGROUND Rhabdomyosarcoma(RMS)is a type of malignant tumor originating from rhabdomyocytes or mesenchymal cells differentiating into rhabdomyocytes.Hepatic pleomorphic RMS is a rare malignant liver tumor.Hepatic sar...BACKGROUND Rhabdomyosarcoma(RMS)is a type of malignant tumor originating from rhabdomyocytes or mesenchymal cells differentiating into rhabdomyocytes.Hepatic pleomorphic RMS is a rare malignant liver tumor.Hepatic sarcomatoid carcinoma is also a rare epithelial malignant tumor originating from the liver;it is characterized by the coexistence of both carcinomatous and sarcomatoid spindle cell components.CASE SUMMARY This paper reports a special case of an elderly woman whose initial liver puncture biopsy showed pleomorphic RMS.After chemotherapy with the vincristine+doxorubicin+cyclophosphamide regimen,the alpha-fetoprotein level increased significantly.Therefore,a second liver puncture was performed,the pathological result of which was hepatic sarcomatoid carcinoma.Next-generation sequencing revealed MET gene amplification with an average copy number of 9 in the tumor tissue;however,both fluorescence in situ hybridization and immunohistochemical tests were negative for MET amplification.The treatment regimen was adjusted to chemotherapy combined with immunotherapy;however,the disease progressed rapidly,and the overall survival was only 6 months.CONCLUSION By sharing the diagnosis and treatment process of this patient and reviewing the relevant literature,we aim to help clinicians enhance their understanding of two rare diseases,namely pleomorphic RMS and sarcomatoid carcinoma of the liver.展开更多
BACKGROUND Sorafenib has been the conventional treatment for advanced hepatocellular carcinoma(HCC)since 2008.While radiological complete responses are extremely rare,improved supportive care and multidisciplinary app...BACKGROUND Sorafenib has been the conventional treatment for advanced hepatocellular carcinoma(HCC)since 2008.While radiological complete responses are extremely rare,improved supportive care and multidisciplinary approaches in clinical practice may explain the recent increase in case reports and retrospective series documenting such responses.CASE SUMMARY This case series describes 3 patients with advanced HCC who achieved durable complete responses using first-line sorafenib therapy,even in the presence of portal vein thrombosis or extrahepatic spread,and highlights the potential for sustained remission in selected patients.Dermatologic toxicity and non-viral etiology may correlate with favorable outcomes;however,reliable predictive biomarkers for sorafenib response are lacking.CONCLUSION Future research into the etiology and molecular differences in HCC is necessary to develop more personalized therapy options.展开更多
BACKGROUND Ankle fractures are well-documented in snow sports,but concomitant Achilles tendon and peroneal tendon ruptures are rare.This case report presents a previously unreported combination of Achilles tendon rupt...BACKGROUND Ankle fractures are well-documented in snow sports,but concomitant Achilles tendon and peroneal tendon ruptures are rare.This case report presents a previously unreported combination of Achilles tendon rupture,peroneal tendon rupture,and fibular fracture in a snowboarder,highlighting the complex nature of diagnosis,management,and rehabilitation.CASE SUMMARY A 50-year-old male snowboarder presented with severe right ankle pain following a high speed tumbling crash.Initial evaluation revealed an Achilles tendon rupture and a non-displaced distal lateral malleolus fracture.Subsequent magnetic resonance imaging confirmed complete tears of the Achilles tendon and both peroneus longus and brevis tendons,along with a Weber A lateral malleolus fracture.Surgical intervention included a 4-suture core Kraków repair of the Achilles tendon with calcaneal docking,open reduction and internal fixation of the distal fibula fracture,and primary repair of both peroneal tendons.Postoperatively,a modified Achilles repair protocol was implemented.At 16 weeks post-surgery,radiographs showed a well-healed fibular fracture,and physical examination confirmed intact Achilles and peroneal tendon repairs.By 6 months,the patient had regained full daily and work activities,including recreational pursuits.CONCLUSION This case underscores the importance of maintaining a high index of suspicion for concomitant injuries in high-energy ankle trauma during snow sports.Timely advanced imaging and a comprehensive surgical approach are crucial for optimal outcomes in such complex cases.展开更多
BACKGROUND SMARCB1/INI1-deficient pancreatic undifferentiated rhabdoid carcinoma is a highly aggressive tumor,and spontaneous splenic rupture(SSR)as its presenting manifestation is rarely reported among pancreatic mal...BACKGROUND SMARCB1/INI1-deficient pancreatic undifferentiated rhabdoid carcinoma is a highly aggressive tumor,and spontaneous splenic rupture(SSR)as its presenting manifestation is rarely reported among pancreatic malignancies.CASE SUMMARY We herein report a rare case of a 59-year-old female who presented with acute left upper quadrant abdominal pain without any history of trauma.Abdominal imaging demonstrated a heterogeneous splenic lesion with hemoperitoneum,raising clinical suspicion of SSR.Emergency laparotomy revealed a pancreatic tumor invading the spleen and left kidney,with associated splenic rupture and dense adhesions,necessitating en bloc resection of the distal pancreas,spleen,and left kidney.Histopathology revealed a biphasic malignancy composed of moderately differentiated pancreatic ductal adenocarcinoma and an undifferentiated carcinoma with rhabdoid morphology and loss of SMARCB1 expression.Immunohistochemical analysis confirmed complete loss of SMARCB1/INI1 in the undifferentiated component,along with a high Ki-67 index(approximately 80%)and CD10 positivity.The ductal adenocarcinoma component retained SMARCB1/INI1 expression and was positive for CK7 and CK-pan.Transitional zones between the two tumor components suggested progressive dedifferentiation and underlying genomic instability.The patient received adjuvant chemotherapy with gemcitabine and nab-paclitaxel and maintained a satisfactory quality of life at the 6-month follow-up.CONCLUSION This study reports a rare case of SMARCB1/INI1-deficient undifferentiated rhabdoid carcinoma of the pancreas combined with ductal adenocarcinoma,presenting as SSR-an exceptionally uncommon initial manifestation of pancreatic malignancy.展开更多
BACKGROUND Salvage of the infected long stem revision total knee arthroplasty is challenging due to the presence of well-fixed ingrown or cemented stems.Reconstructive options are limited.Above knee amputation(AKA)is ...BACKGROUND Salvage of the infected long stem revision total knee arthroplasty is challenging due to the presence of well-fixed ingrown or cemented stems.Reconstructive options are limited.Above knee amputation(AKA)is often recommended.We present a surgical technique that was successfully used on four such patients to convert them to a knee fusion(KF)using a cephalomedullary nail.CASE SUMMARY Four patients with infected long stem revision knee replacements that refused AKA had a single stage removal of their infected revision total knee followed by a KF.They were all treated with a statically locked antegrade cephalomedullary fusion nail,augmented with antibiotic impregnated bone cement.All patients had successful limb salvage and were ambulatory with assistive devices at the time of last follow-up.All were infection free at an average follow-up of 25.5 months(range 16-31).CONCLUSION Single stage cephalomedullary nailing can result in a successful KF in patients with infected long stem revision total knees.展开更多
BACKGROUND Well-differentiated small bowel mesenteric liposarcoma(LPS)is rare,with high malignancy,poor prognosis,and high preponderance to local recurrence.CASE SUMMARY Here we described a 71-year-old male,who compla...BACKGROUND Well-differentiated small bowel mesenteric liposarcoma(LPS)is rare,with high malignancy,poor prognosis,and high preponderance to local recurrence.CASE SUMMARY Here we described a 71-year-old male,who complains of persistent abdominal distension for a month.The clinical manifestation is a huge abdominal mass occupying almost the entire abdomen.Physical examination indicated palpable massive mass in the abdomen,hard texture,indefinable boundary,poor mobility.The abdominal enhanced computed tomography at another hospital scan showed multiple abdominal masses originating from the small bowel mesentery.Abdominal and pelvic magnetic resonance imaging at our hospital showed multiple masses in the abdominal and pelvic cavities,indicating that the tumor originated from the mesentery or peritoneum.Results of exploratory laparotomy indicated that the tremendous mass primarily results from the mesentery of the small intestine,occupying the entire abdominal cavity in a polymorphic and lobulated shape.The patient underwent complete surgical resection of the tumor,and the weight of the tumor was approximately 11 kg.The histopathological examination of the resected specimens confirmed the diagnosis of well-differentiated LPS of the small bowel mesentery.CONCLUSION Completed surgical resection was cornerstone,and histopathological and molecular confirmations were crucial.The necessity of adjuvant therapy should be phrased as a potential consideration to improve patient’s survival time.展开更多
BACKGROUND Hepatocellular carcinoma(HCC)is a major type of liver cancer worldwide.In advanced stages,portal vein tumor thrombosis(PVTT)and jaundice are common,whereas obstructive jaundice(OJ)is relatively rare.Both co...BACKGROUND Hepatocellular carcinoma(HCC)is a major type of liver cancer worldwide.In advanced stages,portal vein tumor thrombosis(PVTT)and jaundice are common,whereas obstructive jaundice(OJ)is relatively rare.Both conditions markedly reduce survival and increase therapeutic complexity.Recently,hepatic artery infusion chemotherapy(HAIC)in combination with targeted immunotherapy has shown promise for advanced HCC.CASE SUMMARY We report a 47-year-old male with advanced HCC complicated by PVTT and OJ,who was admitted with marked jaundice of the skin and sclera.Imaging revealed a large hepatic mass(14.5 cm×11.3 cm)in the right lobe with associated portal vein tumor thrombus.The tertiary bile duct was only mildly dilated,making percutaneous transhepatic cholangiography drainage infeasible.The patient underwent reduced-dose HAIC,which resulted in significant tumor shrinkage and marked reduction in serum bilirubin.This improvement enabled sequential treatment with lenvatinib and camrelizumab.After six cycles,both liver function and alphafetoprotein levels improved.The patient achieved a progression-free survival of 20 months and an overall survival of 29 months.CONCLUSION HAIC can treat high-bilirubin HCC with PVTT and OJ,allowing for subsequent targeted immunotherapy.展开更多
BACKGROUND While varices and variceal bleeds are well-known and feared complications of advanced cirrhosis and portal hypertension,omental variceal bleed are a rare sequala even in patients with known esophageal or ga...BACKGROUND While varices and variceal bleeds are well-known and feared complications of advanced cirrhosis and portal hypertension,omental variceal bleed are a rare sequala even in patients with known esophageal or gastric varices.While rare,omental varices pose a risk for hemoperitoneum if ruptured,which is a lifethreatening complication with high mortality rates despite surgical intervention.CASE SUMMARY This report reviews the case of a patient 36-year-old female with alcohol related cirrhosis decompensated by ascites,but no history of varices admitted for hemorrhagic shock from spontaneous rupture of omental varices requiring emergency surgery.She underwent the first documented successful orthotopic liver transplantation the same admission.CONCLUSION This case report and literature review stresses the importance of early consideration and identification of intraabdominal variceal sources in cirrhotic patients with refractory shock.展开更多
It is challenging to diagnose isolated hyperbilirubinemia with rare and complex etiologies under the constraints of traditional testing conditions.Herein,we present a rare case of coexisting Gilbert syndrome(GS)and er...It is challenging to diagnose isolated hyperbilirubinemia with rare and complex etiologies under the constraints of traditional testing conditions.Herein,we present a rare case of coexisting Gilbert syndrome(GS)and erythropoietic protoporphyria(EPP),which has not been previously documented.CASE SUMMARY We present a rare case of coexisting GS and EPP in a 23-year-old Chinese male with a long history of jaundice and recently found splenomegaly.Serial nonspecific hemolysis screening tests yielded inconsistent results,and investigations for common hemolytic etiologies were negative.However,Levitt’s carbon monoxide breath test,which measures erythrocyte lifespan(the gold-standard marker of hemolysis),demonstrated significant hemolysis,revealing a markedly shortened erythrocyte lifespan of 11 days(normal average 120 days).Genetic testing subsequently confirmed EPP with a homozygous ferrochelatase gene mutation and GS with a heterozygous uridine diphosphate glucuronosyl trans-ferase 1A1 gene mutation.CONCLUSION The rapid,non-invasive Levitt’s carbon monoxide breath test resolved the diagnostic challenge posed by a rare and complex cause of hyperbilirubinemia.展开更多
BACKGROUND Early renal artery thrombosis after kidney transplantation is rare but often leads to graft loss.Prompt diagnosis and intervention are essential,particularly in patients with inherited thrombophilias such a...BACKGROUND Early renal artery thrombosis after kidney transplantation is rare but often leads to graft loss.Prompt diagnosis and intervention are essential,particularly in patients with inherited thrombophilias such as factor V Leiden(FVL)mutation.CASE SUMMARY A kidney transplant recipient with FVL mutation developed an acute transplant renal artery thrombosis.The immediate post-operative Doppler ultrasonography revealed thrombosis of the main and inferior polar renal arteries.Emergent thrombectomy and separate arterial re-anastomoses were performed after cold perfusion with heparinized saline and vasodilator solution.Reperfusion was successful with immediate urine output and gradual improvement in renal function.The patient was discharged on direct oral anticoagulation therapy.CONCLUSION Early detection and surgical intervention can preserve graft function in posttransplant renal artery thrombosis even in patients at high risk.展开更多
BACKGROUND With the increasing use of laparoscopic techniques in living-donor kidney transplantation,limitations in donor vessel length,particularly of the right renal vein,pose significant challenges for vascular ana...BACKGROUND With the increasing use of laparoscopic techniques in living-donor kidney transplantation,limitations in donor vessel length,particularly of the right renal vein,pose significant challenges for vascular anastomosis to the recipient’s external iliac vein.These anatomical constraints can complicate graft implantation and increase the risk of postoperative complications.CASE SUMMARY To address the issue of short right renal veins,several surgical strategies have been proposed.In this report,we describe our experience with three cases in which venous extension was successfully achieved using a venous cuff interposition technique during back-table reconstruction.This approach was used to facilitate secure vascular anastomosis and improve graft positioning in anatomically complex transplant scenarios.CONCLUSION Venous cuff interposition represents an effective technique for managing short renal veins in living-donor kidney transplantation.It provides additional length and flexibility,easing anastomotic tension and supporting successful transplantation.展开更多
BACKGROUND Ulcerative colitis(UC)is a chronic and debilitating inflammatory bowel disease.Cumulative evidence indicates that excess hydrogen peroxide,a potent neutrophilic chemotactic agent,produced by colonic epithel...BACKGROUND Ulcerative colitis(UC)is a chronic and debilitating inflammatory bowel disease.Cumulative evidence indicates that excess hydrogen peroxide,a potent neutrophilic chemotactic agent,produced by colonic epithelial cells has a causal role leading to infiltration of neutrophils into the colonic mucosa and subsequent development of UC.This evidence-based mechanism identifies hydrogen peroxide as a therapeutic target for reducing agents in the treatment of UC.CASE SUMMARY Presented is a 41-year-old female with a 26-year history of refractory UC.Having developed steroid dependence and never achieving complete remission on treatment by conventional and advanced therapies,she began treatment with oral R-dihydrolipoic acid(RDLA),a lipid-soluble reducing agent with intracellular site of action.Within a week,rectal bleeding ceased.She was asymptomatic for three years until a highly stressful experience,when she noticed blood in her stool.RDLA was discontinued,and she began treatment with oral sodium thiosulfate pentahydrate(STS),a reducing agent with extracellular site of action.After a week,rectal bleeding ceased,and she resumed oral RDLA and discontinued STS.To date,she remains asymptomatic with normal stool calprotectin while on RDLA.CONCLUSION STS and RDLA are reducing agents that serve as highly effective and safe therapy for the induction and maintenance of remission in UC,even in patients refractory or poorly controlled by conventional and advanced therapies.Should preliminary findings be validated by subsequent clinical trials,the use of reducing agents could potentially prevent thousands of colectomies and represent a paradigm shift in the treatment of UC.展开更多
BACKGROUND Complement-mediated thrombotic microangiopathy(TMA)is a rare endothelial injury syndrome caused by dysregulated activation of the alternative complement pathway,often linked to genetic abnormalities in comp...BACKGROUND Complement-mediated thrombotic microangiopathy(TMA)is a rare endothelial injury syndrome caused by dysregulated activation of the alternative complement pathway,often linked to genetic abnormalities in complement factor H(CFH),complement factor I,or complement factor H-related(CFHR)proteins.Both renal transplantation and pregnancy are independent triggers for recurrence.This case highlights a genetically high-risk patient who achieved a successful term pregnancy after renal transplantation without complement inhibition,emphasizing individualized risk stratification,close surveillance,and multidisciplinary management for favourable maternal and graft outcomes.CASE SUMMARY A 32-year-old woman with end-stage renal disease secondary to genetically confirmed complement-mediated TMA—homozygous CFH exon 17 deletion and CFHR3-CFHR1 duplication—was maintained on dialysis for 2.5 years before undergoing a successful live-donor kidney transplant from her mother.Post-transplant immunosuppression included tacrolimus,mycophenolate mofetil,and prednisolone,later modified to azathioprine during pregnancy planning.One-year post-transplant,she conceived spontaneously.Pregnancy was complicated by transient gestational hypertension,controlled with nifedipine,labetalol,and amlodipine.Proteinuria remained<150 mg/day;white blood cell counts 5.8-7.2×109/L without cytopenia.Serum creatinine ranged 0.9-1.1 mg/dL,and tacrolimus trough levels 5-7 ng/mL.At 36 weeks,she delivered a healthy 3 kg infant by elective caesarean section.Postpartum follow-up at three months confirmed stable maternal and graft function.CONCLUSION High-risk complement-mediated TMA patients can achieve successful pregnancy post-transplant through individualized care without mandatory complement blockade.展开更多
1.About CASEE The Chinese Academy of Science and Education Evaluation(CASEE),also known as the Chinese Academy of Evaluation Sciences(CAES),is a key evaluation and research institute established by Hangzhou Dianzi Uni...1.About CASEE The Chinese Academy of Science and Education Evaluation(CASEE),also known as the Chinese Academy of Evaluation Sciences(CAES),is a key evaluation and research institute established by Hangzhou Dianzi University in 2017.Prof.Junping Qiu,a well-known Chinese bibliometrican who has expertise in evaluation management and subsidized by the State Council of China,is the founder and the first president of CASEE.Under the leadership of Prof.Qiu,the CASEE attempts to achieve the following objectives.展开更多
BACKGROUND Acute hyperglycemia due to insulin resistance is common in critically ill patients,typically managed with insulin infusion.However,the occurrence of transient extreme insulin resistance(EIR)requiring except...BACKGROUND Acute hyperglycemia due to insulin resistance is common in critically ill patients,typically managed with insulin infusion.However,the occurrence of transient extreme insulin resistance(EIR)requiring exceptional high-dose insulin is rare.CASE SUMMARY We present the case of a 68-year-old woman with pneumonia who suffered an out-of-hospital cardiac arrest,subsequently developing transient EIR following a new episode of sepsis.Remarkably,insulin resistance rapidly reversed when the insulin infusion rate peaked at 960 units/hour(a total of 18224 units on that day),and it was promptly titrated down to zero upon achieving the target glucose level.CONCLUSION Exceptional high-dose insulin infusion may be required in critically ill patients with stress-related EIR,which is typically transient.Clinicians should be aware of the phenomenon and cautious to avoid hypoglycemia and fluid overload during the steep titration of high-dose insulin infusion.展开更多
文摘AIM:To report and analyze cases of sterile intraocular inflammation(IOI)following intravitreal faricimab injections in patients treated for neovascular age-related macular degeneration(nAMD)and diabetic macular edema(DME).METHODS:This double-center case series included nine eyes of six patients who developed uveitis after faricimab therapy.Comprehensive clinical evaluation was performed,including slit-lamp examination,intraocular pressure(IOP)measurement,fluorescein and indocyanine green angiography(ICGA),and laboratory tests.Inflammatory responses were treated with topical or systemic corticosteroids,and patients were monitored for visual acuity and inflammatory activity.RESULTS:The incidence of IOI was 0.8%per patient(Innsbruck)and 0.23%(Czechia),with inflammation typically occurring between the third and sixth injection(mean interval:10d post-injection).Inflammator y presentations ranged from anterior uveitis to posterior segment involvement.One notable case demonstrated novel choroidal hypofluorescent lesions on angiography,suggesting deeper ocular involvement.The mean patient age was 76y;five of six affected patients were female.All cases responded to local and systemic corticosteroids,with full recovery of initial visual acuity.CONCLUSION:Sterile IOI after faricimab appears to be a rare but relevant adverse event.Although the incidence falls within expected ranges for anti-vascular endothelial growth factor(anti-VEGF)agents,the observed choroidal involvement represents a potentially new safety signal.Prompt diagnosis and corticosteroid therapy are effective in all cases.Our findings support the need for vigilant post-marketing surveillance and further studies to better understand the underlying mechanisms and risk factors of faricimab-associated inflammation.
文摘BACKGROUND Congenital hypothyroidism(CH)is a common condition in both preterm and term infants characterized by either thyroid gland absence or hypofunctionality.The clinical association of refractory lactic acidosis and heart failure has rarely been observed in cases of pediatric patients with CH pathology.Here,we explored the etiological relationship between CH,heart failure,and refractory lactic acidosis to reflect the importance of thyroid function screening in neonates with heart disease.CASE SUMMARY A 33-day-old extremely premature female infant presented with tachypnea,respiratory distress,recurrent infections,and abdominal distension postnatal.On admission to our facility,she had cardiomegaly,hepatomegaly,and lactic acidosis(revealed on blood gas analysis),with lactate progressively rising to 25 mmol/L.Chest radiographs showed pulmonary congestion,while echocardiography revealed cardiac enlargement,left ventricular wall thickening,and pericardial effusion.Initial management aimed at correcting acidosis and treating heart failure proved ineffective.After reassessment,thyroid function tests showed significantly decreased triiodothyronine,free triiodothyronine,thyroxine,and free thyroxine levels,with a significantly increased thyroidstimulating hormone level,confirming a CH diagnosis.Levothyroxine was administered,resulting in rapid correction of lactic acidosis and gradual improvement of thyroid function and systemic symptoms,culminating in full recovery and discharge.We also reviewed the relevant literature on thyroid and cardiac dysfunctions in order to explore their deeper association.CONCLUSION This case links CH-induced heart failure with refractory lactic acidosis,urging prompt thyroid screening in affected neonates to reduce mortality.
文摘BACKGROUND Edwardsiella tarda(E.tarda)belongs to the family Enterobacteriaceae and is generally seen to cause infections mainly in fish,but is also capable of infecting humans.Extraintestinal infections occur in patients with certain risk factors,including immunocompromised status.We recently diagnosed a case of spontaneous bacterial peritonitis(SBP)due to E.tarda in an immuno-compromised dialysis patient.CASE SUMMARY Patient was a 55-year-old male,with a history of diabetic nephropathy being treated with hemodialysis three times a week.He was referred to our hospital due to an increased volume of ascites,and blood examination revealed increased inflammatory reaction.At our emergency department,he developed fever,disturbance of consciousness,abdominal distension,and abdomen-wide pain.In addition,a dialysis shunt was confirmed in his right forearm,and the shunt site showed no signs of inflammation.No wounds were confirmed on or in his body.A blood examination revealed increased values of white blood cells,C-reactive protein,and creatinine.Plain chest and abdominal computed tomography scanning revealed increased ascites volume.Abdominal paracentesis was performed and a Gram stain revealed Gramnegative bacillus.These findings prompted diagnosis of SBP.The patient was admitted and treated with cefmetazole,causing fever resolution and symptom improvements.Later,E.tarda was identified in ascites culture.The patient improved with decreased inflammatory response and was discharged on the 12th day of hospitalization.The antibiotic was terminated after 14 days of treatment.SBP in this case may have developed from chronic renal failure and diabetes mellitus.CONCLUSION We report the first known case of SBP due to E.tarda in an immuno-compromised dialysis patient.
基金Supported by Shaanxi Provincial Natural Science Basic Research Program,No.2020JQ-951.
文摘BACKGROUND Rhabdomyosarcoma(RMS)is a type of malignant tumor originating from rhabdomyocytes or mesenchymal cells differentiating into rhabdomyocytes.Hepatic pleomorphic RMS is a rare malignant liver tumor.Hepatic sarcomatoid carcinoma is also a rare epithelial malignant tumor originating from the liver;it is characterized by the coexistence of both carcinomatous and sarcomatoid spindle cell components.CASE SUMMARY This paper reports a special case of an elderly woman whose initial liver puncture biopsy showed pleomorphic RMS.After chemotherapy with the vincristine+doxorubicin+cyclophosphamide regimen,the alpha-fetoprotein level increased significantly.Therefore,a second liver puncture was performed,the pathological result of which was hepatic sarcomatoid carcinoma.Next-generation sequencing revealed MET gene amplification with an average copy number of 9 in the tumor tissue;however,both fluorescence in situ hybridization and immunohistochemical tests were negative for MET amplification.The treatment regimen was adjusted to chemotherapy combined with immunotherapy;however,the disease progressed rapidly,and the overall survival was only 6 months.CONCLUSION By sharing the diagnosis and treatment process of this patient and reviewing the relevant literature,we aim to help clinicians enhance their understanding of two rare diseases,namely pleomorphic RMS and sarcomatoid carcinoma of the liver.
文摘BACKGROUND Sorafenib has been the conventional treatment for advanced hepatocellular carcinoma(HCC)since 2008.While radiological complete responses are extremely rare,improved supportive care and multidisciplinary approaches in clinical practice may explain the recent increase in case reports and retrospective series documenting such responses.CASE SUMMARY This case series describes 3 patients with advanced HCC who achieved durable complete responses using first-line sorafenib therapy,even in the presence of portal vein thrombosis or extrahepatic spread,and highlights the potential for sustained remission in selected patients.Dermatologic toxicity and non-viral etiology may correlate with favorable outcomes;however,reliable predictive biomarkers for sorafenib response are lacking.CONCLUSION Future research into the etiology and molecular differences in HCC is necessary to develop more personalized therapy options.
文摘BACKGROUND Ankle fractures are well-documented in snow sports,but concomitant Achilles tendon and peroneal tendon ruptures are rare.This case report presents a previously unreported combination of Achilles tendon rupture,peroneal tendon rupture,and fibular fracture in a snowboarder,highlighting the complex nature of diagnosis,management,and rehabilitation.CASE SUMMARY A 50-year-old male snowboarder presented with severe right ankle pain following a high speed tumbling crash.Initial evaluation revealed an Achilles tendon rupture and a non-displaced distal lateral malleolus fracture.Subsequent magnetic resonance imaging confirmed complete tears of the Achilles tendon and both peroneus longus and brevis tendons,along with a Weber A lateral malleolus fracture.Surgical intervention included a 4-suture core Kraków repair of the Achilles tendon with calcaneal docking,open reduction and internal fixation of the distal fibula fracture,and primary repair of both peroneal tendons.Postoperatively,a modified Achilles repair protocol was implemented.At 16 weeks post-surgery,radiographs showed a well-healed fibular fracture,and physical examination confirmed intact Achilles and peroneal tendon repairs.By 6 months,the patient had regained full daily and work activities,including recreational pursuits.CONCLUSION This case underscores the importance of maintaining a high index of suspicion for concomitant injuries in high-energy ankle trauma during snow sports.Timely advanced imaging and a comprehensive surgical approach are crucial for optimal outcomes in such complex cases.
文摘BACKGROUND SMARCB1/INI1-deficient pancreatic undifferentiated rhabdoid carcinoma is a highly aggressive tumor,and spontaneous splenic rupture(SSR)as its presenting manifestation is rarely reported among pancreatic malignancies.CASE SUMMARY We herein report a rare case of a 59-year-old female who presented with acute left upper quadrant abdominal pain without any history of trauma.Abdominal imaging demonstrated a heterogeneous splenic lesion with hemoperitoneum,raising clinical suspicion of SSR.Emergency laparotomy revealed a pancreatic tumor invading the spleen and left kidney,with associated splenic rupture and dense adhesions,necessitating en bloc resection of the distal pancreas,spleen,and left kidney.Histopathology revealed a biphasic malignancy composed of moderately differentiated pancreatic ductal adenocarcinoma and an undifferentiated carcinoma with rhabdoid morphology and loss of SMARCB1 expression.Immunohistochemical analysis confirmed complete loss of SMARCB1/INI1 in the undifferentiated component,along with a high Ki-67 index(approximately 80%)and CD10 positivity.The ductal adenocarcinoma component retained SMARCB1/INI1 expression and was positive for CK7 and CK-pan.Transitional zones between the two tumor components suggested progressive dedifferentiation and underlying genomic instability.The patient received adjuvant chemotherapy with gemcitabine and nab-paclitaxel and maintained a satisfactory quality of life at the 6-month follow-up.CONCLUSION This study reports a rare case of SMARCB1/INI1-deficient undifferentiated rhabdoid carcinoma of the pancreas combined with ductal adenocarcinoma,presenting as SSR-an exceptionally uncommon initial manifestation of pancreatic malignancy.
文摘BACKGROUND Salvage of the infected long stem revision total knee arthroplasty is challenging due to the presence of well-fixed ingrown or cemented stems.Reconstructive options are limited.Above knee amputation(AKA)is often recommended.We present a surgical technique that was successfully used on four such patients to convert them to a knee fusion(KF)using a cephalomedullary nail.CASE SUMMARY Four patients with infected long stem revision knee replacements that refused AKA had a single stage removal of their infected revision total knee followed by a KF.They were all treated with a statically locked antegrade cephalomedullary fusion nail,augmented with antibiotic impregnated bone cement.All patients had successful limb salvage and were ambulatory with assistive devices at the time of last follow-up.All were infection free at an average follow-up of 25.5 months(range 16-31).CONCLUSION Single stage cephalomedullary nailing can result in a successful KF in patients with infected long stem revision total knees.
文摘BACKGROUND Well-differentiated small bowel mesenteric liposarcoma(LPS)is rare,with high malignancy,poor prognosis,and high preponderance to local recurrence.CASE SUMMARY Here we described a 71-year-old male,who complains of persistent abdominal distension for a month.The clinical manifestation is a huge abdominal mass occupying almost the entire abdomen.Physical examination indicated palpable massive mass in the abdomen,hard texture,indefinable boundary,poor mobility.The abdominal enhanced computed tomography at another hospital scan showed multiple abdominal masses originating from the small bowel mesentery.Abdominal and pelvic magnetic resonance imaging at our hospital showed multiple masses in the abdominal and pelvic cavities,indicating that the tumor originated from the mesentery or peritoneum.Results of exploratory laparotomy indicated that the tremendous mass primarily results from the mesentery of the small intestine,occupying the entire abdominal cavity in a polymorphic and lobulated shape.The patient underwent complete surgical resection of the tumor,and the weight of the tumor was approximately 11 kg.The histopathological examination of the resected specimens confirmed the diagnosis of well-differentiated LPS of the small bowel mesentery.CONCLUSION Completed surgical resection was cornerstone,and histopathological and molecular confirmations were crucial.The necessity of adjuvant therapy should be phrased as a potential consideration to improve patient’s survival time.
文摘BACKGROUND Hepatocellular carcinoma(HCC)is a major type of liver cancer worldwide.In advanced stages,portal vein tumor thrombosis(PVTT)and jaundice are common,whereas obstructive jaundice(OJ)is relatively rare.Both conditions markedly reduce survival and increase therapeutic complexity.Recently,hepatic artery infusion chemotherapy(HAIC)in combination with targeted immunotherapy has shown promise for advanced HCC.CASE SUMMARY We report a 47-year-old male with advanced HCC complicated by PVTT and OJ,who was admitted with marked jaundice of the skin and sclera.Imaging revealed a large hepatic mass(14.5 cm×11.3 cm)in the right lobe with associated portal vein tumor thrombus.The tertiary bile duct was only mildly dilated,making percutaneous transhepatic cholangiography drainage infeasible.The patient underwent reduced-dose HAIC,which resulted in significant tumor shrinkage and marked reduction in serum bilirubin.This improvement enabled sequential treatment with lenvatinib and camrelizumab.After six cycles,both liver function and alphafetoprotein levels improved.The patient achieved a progression-free survival of 20 months and an overall survival of 29 months.CONCLUSION HAIC can treat high-bilirubin HCC with PVTT and OJ,allowing for subsequent targeted immunotherapy.
文摘BACKGROUND While varices and variceal bleeds are well-known and feared complications of advanced cirrhosis and portal hypertension,omental variceal bleed are a rare sequala even in patients with known esophageal or gastric varices.While rare,omental varices pose a risk for hemoperitoneum if ruptured,which is a lifethreatening complication with high mortality rates despite surgical intervention.CASE SUMMARY This report reviews the case of a patient 36-year-old female with alcohol related cirrhosis decompensated by ascites,but no history of varices admitted for hemorrhagic shock from spontaneous rupture of omental varices requiring emergency surgery.She underwent the first documented successful orthotopic liver transplantation the same admission.CONCLUSION This case report and literature review stresses the importance of early consideration and identification of intraabdominal variceal sources in cirrhotic patients with refractory shock.
文摘It is challenging to diagnose isolated hyperbilirubinemia with rare and complex etiologies under the constraints of traditional testing conditions.Herein,we present a rare case of coexisting Gilbert syndrome(GS)and erythropoietic protoporphyria(EPP),which has not been previously documented.CASE SUMMARY We present a rare case of coexisting GS and EPP in a 23-year-old Chinese male with a long history of jaundice and recently found splenomegaly.Serial nonspecific hemolysis screening tests yielded inconsistent results,and investigations for common hemolytic etiologies were negative.However,Levitt’s carbon monoxide breath test,which measures erythrocyte lifespan(the gold-standard marker of hemolysis),demonstrated significant hemolysis,revealing a markedly shortened erythrocyte lifespan of 11 days(normal average 120 days).Genetic testing subsequently confirmed EPP with a homozygous ferrochelatase gene mutation and GS with a heterozygous uridine diphosphate glucuronosyl trans-ferase 1A1 gene mutation.CONCLUSION The rapid,non-invasive Levitt’s carbon monoxide breath test resolved the diagnostic challenge posed by a rare and complex cause of hyperbilirubinemia.
文摘BACKGROUND Early renal artery thrombosis after kidney transplantation is rare but often leads to graft loss.Prompt diagnosis and intervention are essential,particularly in patients with inherited thrombophilias such as factor V Leiden(FVL)mutation.CASE SUMMARY A kidney transplant recipient with FVL mutation developed an acute transplant renal artery thrombosis.The immediate post-operative Doppler ultrasonography revealed thrombosis of the main and inferior polar renal arteries.Emergent thrombectomy and separate arterial re-anastomoses were performed after cold perfusion with heparinized saline and vasodilator solution.Reperfusion was successful with immediate urine output and gradual improvement in renal function.The patient was discharged on direct oral anticoagulation therapy.CONCLUSION Early detection and surgical intervention can preserve graft function in posttransplant renal artery thrombosis even in patients at high risk.
文摘BACKGROUND With the increasing use of laparoscopic techniques in living-donor kidney transplantation,limitations in donor vessel length,particularly of the right renal vein,pose significant challenges for vascular anastomosis to the recipient’s external iliac vein.These anatomical constraints can complicate graft implantation and increase the risk of postoperative complications.CASE SUMMARY To address the issue of short right renal veins,several surgical strategies have been proposed.In this report,we describe our experience with three cases in which venous extension was successfully achieved using a venous cuff interposition technique during back-table reconstruction.This approach was used to facilitate secure vascular anastomosis and improve graft positioning in anatomically complex transplant scenarios.CONCLUSION Venous cuff interposition represents an effective technique for managing short renal veins in living-donor kidney transplantation.It provides additional length and flexibility,easing anastomotic tension and supporting successful transplantation.
文摘BACKGROUND Ulcerative colitis(UC)is a chronic and debilitating inflammatory bowel disease.Cumulative evidence indicates that excess hydrogen peroxide,a potent neutrophilic chemotactic agent,produced by colonic epithelial cells has a causal role leading to infiltration of neutrophils into the colonic mucosa and subsequent development of UC.This evidence-based mechanism identifies hydrogen peroxide as a therapeutic target for reducing agents in the treatment of UC.CASE SUMMARY Presented is a 41-year-old female with a 26-year history of refractory UC.Having developed steroid dependence and never achieving complete remission on treatment by conventional and advanced therapies,she began treatment with oral R-dihydrolipoic acid(RDLA),a lipid-soluble reducing agent with intracellular site of action.Within a week,rectal bleeding ceased.She was asymptomatic for three years until a highly stressful experience,when she noticed blood in her stool.RDLA was discontinued,and she began treatment with oral sodium thiosulfate pentahydrate(STS),a reducing agent with extracellular site of action.After a week,rectal bleeding ceased,and she resumed oral RDLA and discontinued STS.To date,she remains asymptomatic with normal stool calprotectin while on RDLA.CONCLUSION STS and RDLA are reducing agents that serve as highly effective and safe therapy for the induction and maintenance of remission in UC,even in patients refractory or poorly controlled by conventional and advanced therapies.Should preliminary findings be validated by subsequent clinical trials,the use of reducing agents could potentially prevent thousands of colectomies and represent a paradigm shift in the treatment of UC.
文摘BACKGROUND Complement-mediated thrombotic microangiopathy(TMA)is a rare endothelial injury syndrome caused by dysregulated activation of the alternative complement pathway,often linked to genetic abnormalities in complement factor H(CFH),complement factor I,or complement factor H-related(CFHR)proteins.Both renal transplantation and pregnancy are independent triggers for recurrence.This case highlights a genetically high-risk patient who achieved a successful term pregnancy after renal transplantation without complement inhibition,emphasizing individualized risk stratification,close surveillance,and multidisciplinary management for favourable maternal and graft outcomes.CASE SUMMARY A 32-year-old woman with end-stage renal disease secondary to genetically confirmed complement-mediated TMA—homozygous CFH exon 17 deletion and CFHR3-CFHR1 duplication—was maintained on dialysis for 2.5 years before undergoing a successful live-donor kidney transplant from her mother.Post-transplant immunosuppression included tacrolimus,mycophenolate mofetil,and prednisolone,later modified to azathioprine during pregnancy planning.One-year post-transplant,she conceived spontaneously.Pregnancy was complicated by transient gestational hypertension,controlled with nifedipine,labetalol,and amlodipine.Proteinuria remained<150 mg/day;white blood cell counts 5.8-7.2×109/L without cytopenia.Serum creatinine ranged 0.9-1.1 mg/dL,and tacrolimus trough levels 5-7 ng/mL.At 36 weeks,she delivered a healthy 3 kg infant by elective caesarean section.Postpartum follow-up at three months confirmed stable maternal and graft function.CONCLUSION High-risk complement-mediated TMA patients can achieve successful pregnancy post-transplant through individualized care without mandatory complement blockade.
文摘1.About CASEE The Chinese Academy of Science and Education Evaluation(CASEE),also known as the Chinese Academy of Evaluation Sciences(CAES),is a key evaluation and research institute established by Hangzhou Dianzi University in 2017.Prof.Junping Qiu,a well-known Chinese bibliometrican who has expertise in evaluation management and subsidized by the State Council of China,is the founder and the first president of CASEE.Under the leadership of Prof.Qiu,the CASEE attempts to achieve the following objectives.
文摘BACKGROUND Acute hyperglycemia due to insulin resistance is common in critically ill patients,typically managed with insulin infusion.However,the occurrence of transient extreme insulin resistance(EIR)requiring exceptional high-dose insulin is rare.CASE SUMMARY We present the case of a 68-year-old woman with pneumonia who suffered an out-of-hospital cardiac arrest,subsequently developing transient EIR following a new episode of sepsis.Remarkably,insulin resistance rapidly reversed when the insulin infusion rate peaked at 960 units/hour(a total of 18224 units on that day),and it was promptly titrated down to zero upon achieving the target glucose level.CONCLUSION Exceptional high-dose insulin infusion may be required in critically ill patients with stress-related EIR,which is typically transient.Clinicians should be aware of the phenomenon and cautious to avoid hypoglycemia and fluid overload during the steep titration of high-dose insulin infusion.
