Hepatotoxicity associated with the use of teriflunomide in a patient with multiple sclerosis: A case report

Rationale:Teriflunomide is an inhibitor of pyrimidine synthesis available as a first-line treatment for relapsing-remitting multiple sclerosis. Drug-induced liver damage is a relevant problem in clinical practice, representing a frequent cause of treatment discontinuation. This case report describes the occurrence of liver injury, with a 33.7-fold increase in the upper limit of normality of the liver enzyme alanine aminotransferase during treatment with teriflunomide 14 mg.Patient concern:A 44-year-old woman receiving teriflunomide 14 mg for the treatment of multiple sclerosis presented symptoms suggestive of liver dysfunction 54 days after starting treatment. The patient had no history of using disease-modifying therapy, neither previous liver disease nor other comorbidities.Diagnostics:The suggested diagnosis was drug-induced liver injury, classified as hepatocellular. Other possible hepatic and autoimmune etiologies were ruled out.Interventions:Replacement of teriflunomide treatment with glatiramer acetate and follow-up of the disease.Outcomes:Signs and symptoms regressed after treatment with teriflunomide 14 mg was discontinued, with normalization of liver enzyme activity in ∼5 months. The causality assessment of the adverse drug reaction was determined by the Naranjo scaling system, resulting in probable, with a final score of 7.Conclusions:Teriflunomide-induced liver injury in patients with multiple sclerosis is a serious adverse reaction. The report of this case contributes to updating knowledge about the safety aspects of treatment with teriflunomide and planning of monitoring strategies and patient risk management.     

Laparoscopic treatment of a giant seminal vesicle cyst with hemorrhage: A case report

Rationale:A seminal vesicle cyst is a benign lesion of the seminal vesicle that is usually asymptomatic. However, when a giant seminal vesicle cyst ruptures and bleeds, it can cause obvious clinical symptoms. To our knowledge, no single giant seminal vesicle cyst with hemorrhage has been reported in current studies, and surgery is the primary method to treat seminal vesicle hemorrhage with obvious symptoms.Patient concerns:A 31-year-old man presented with urination pain but without obvious urination frequency and urgency, dysuria, and discomfort. Rectal palpation in the chest-knee position revealed a hard mass palpable in the upper right with a smooth surface and mild tenderness, and the upper edge of the mass could not be palpated.Diagnosis:The results of the B-mode ultrasound indicated a mixed echogenic lump between the bladder and prostate, with a size of 81 × 76 mm. The computer tomography scan showed an “S” tubular lump in the right side of the pelvic cavity. The mass has a computer tomography value of 58 to 70 HU, and uneven reinforcement can be observed. On the basis of the results of the magnetic resonance imaging of the urinary bladder, the lump has T1 and T2 signals of equal lengths.Interventions:The patient was diagnosed with a huge right seminal vesicle cyst with hemorrhage and was treated via laparoscopic surgery.Outcomes:The patient recovered quickly after the operation, and the symptoms of urination pain were significantly improved.Lessons:Seminal vesicle hemorrhage is clinically rare, and laparoscopic treatment is an effective and safe surgical method for the treatment of seminal vesicle cysts.     

Favorable response to multimodal treatment in hepatocellular carcinoma with inferior vena cava and right atrial tumor thrombus and left adrenal gland metastasis: A case report and literature review

Rationale:Hepatocellular carcinoma (HCC) is the fourth most common cause of cancer-related deaths and the sixth most commonly diagnosed cancer globally. Interdisciplinary and multimodal treatment strategies are essential for a successful therapy in HCC. Established therapies for HCC treatment include surgical resection, liver transplantation, local ablative therapies, transarterial chemoembolization (TACE), tyrosine kinase inhibitors (TKIs), immunotherapy, and radiotherapy (RT).Patient concerns:A 52-year-old male patient did an ultrasound scan and found a large mass within the right lobe of the liver and gallstones in December 2018. He had a history of chronic hepatitis C virus infection (30 years) and was treated with sofosbuvir (400 mg, q.d.) for 1 year. The patient never had any symptoms of gallstones. Enhanced abdominal computed tomography of this patient showed a heterogeneous irregular mass with the largest measurement of up to 13.7 × 11.1 cm in size in the right lobe of the liver, meanwhile also had inferior vena cava (IVC) tumor thrombus, right atrial (RA) tumor thrombus, and left adrenal gland metastasis. The laboratory test data revealed that the serum tumor marker α-fetoprotein was 2.63 ng/mL, cancer antigen 19-9 (CA 19-9) was 34.40 U/mL, and protein induced by Vitamin K absence was 391.94 mAU/mL.Diagnosis:HCC with IVC tumor thrombus, RA tumor thrombus, and left adrenal gland metastasis, and gallstones.Interventions:He was hospitalized and received TACE treatment, oral TKIs, intravenous drip programmed cell death-1 (PD-1) inhibitor and RT.Outcomes:The patient showed a favorable response after consecutive treatment with TACE, TKIs, PD-1 inhibitor, and RT. Until now, the patient has survived 34 months since the diagnosis of the disease.Lessons:Our case suggests that TACE combined with TKIs, PD-1 inhibitor, and RT may be a suitable treatment option for advanced HCC patients with IVC tumor thrombus and/or RA tumor thrombus, and/or adrenal gland metastasis.     

Acute respiratory distress syndrome associated with macrophage activation syndrome in systemic lupus erythematosus: A case report and literature review

Rationale:Previous treatment for macrophage activation syndrome (MAS) includes high-dose intravenous methylprednisolone along with intravenous immunoglobulin G. If MAS worsened, second-line therapy consisted of anakinra; if the disease remained refractory, third-line therapy with etoposide was considered. In addition, cyclosporine A plays a role in early MAS and in preventing recurrence. Some studies have reported the use of cytokine-targeting agents other than anakinra, such as canakinumab, tocilizumab, abatacept, and tofacitinib.Patient concerns:The patient with systemic lupus erythematosus (SLE) had an uncommon combination of intermittent fever, hyperferritinemia, hypertriglyceridemia, jaundice, and significantly abnormal liver function test results. The patient reported a history of daily fever of 38 to 39°C, painful oral ulcer, anorexia, abdominal bloating, diarrhea, and malar rash progression for 2 weeks, and jaundice, tea-colored urine, and clay-colored stool for 1 week preceding hospital admission.Diagnosis:SLE flareups in the patient were initially suspected. However, the final diagnosis was acute respiratory distress syndrome (ARDS) associated with MAS.Interventions:The treatment included disease-modifying antirheumatic drugs (DMARDs), such as azathioprine, and titrated steroid doses of methylprednisolone (40 mg q8 h) and dexamethasone (15 mg q8 h), after the patient had ARDS and was intubated.Dose-adjusted monotherapy with dexamethasone was found to be effective; this may be attributed to some DMARDs being unsuitable for cytokine storms, that is, some DMARDs may cause complications in cytokine storms.Outcomes:After dexamethasone 15 mg q8 h treatment, the patient''s fever subsided within 2 days, and liver function became normal within 3 weeks. The patient regularly attended scheduled outpatient follow-up visits after discharge. After 2 years, the patient reported no symptoms or signs of SLE with 2 mg/d oral dexamethasone.Lessons:Early diagnosis of MAS and dexamethasone treatment for MAS with ARDS appear to be crucial for these patients.     

Uterine leiomyoma with fumarate hydratase deficiency: A case report

Rationale:Hereditary leiomyomatosis and renal cell carcinoma is an uncommon autosomal dominant disease caused by mutations in the fumarate hydratase (FH) gene. They usually demonstrated multiple uterine myomas and preformed surgical procedures for myomectomy and/or hysterectomy 10 years earlier than sporadic myomas due to early development. This case report describes a woman with multiple uterine leiomyomas diagnosed with FH deficiency.Patient concerns:A 37-year-old woman visited a gynecological clinic for the discovery of uterine leiomyoma for more than 1 year. The size of the largest grew from 42 × 27 × 46 to 98 × 85 × 113 mm in 1 year. She had a history of surgery for breast cancer and thyroid cancer but denied a history of uterine leiomyoma in her family.Diagnosis and Interventions:The patient underwent successful transabdominal hysterectomy. The pathological results showed multiple uterine leiomyomas (partly cellular leiomyomas) with scattered large bizarre giant cells. Immunohistochemistry results demonstrated FH deficiency.Outcomes:On follow-up, the patient did not have any complications. She was finally referred to the oncologists and urologists for follow-up.Lessons:Gynecologists should be aware that early onset uterine leiomyoma presenting as large, multiple, and symptomatic lesion, may be associated with FH deficiency.     

Successful management of the hepatocellular carcinoma with inferior vena cava tumor thrombus: A case report

Rationale:Hepatocellular carcinoma (HCC) with inferior vena cava tumor thrombus (IVCTT) is traditionally considered an advanced-stage cancer with a poor prognosis. There is no standard treatment for patients diagnosed as HCC with IVCTT.Patient concerns:A 52-year-old man was admitted to our hospital because of suspected hepatic mass during a health examination.Diagnoses:Computed tomography (CT) showed a hepatic mass approximately 4.3 cm × 6.3 cm in size located in segment VII of the liver, with thrombus in the inferior vena cava. The mass exhibited a pattern of early enhancement and washout on contrast-enhanced CT. Alpha-fetoprotein was 614.1 ng/mL (normal value, <8 ng/mL). The preoperative diagnosis was HCC with IVCTT.Interventions:Two months after stereotactic body radiotherapy combined with sorafenib therapy, a planned open anatomical resection of the right posterior lobe of the liver was performed.Outcomes:The patient is alive without disease 12 months after surgery, and the level of alpha-fetoprotein is normal.Lessons:The patient diagnosed as HCC with IVCTT was successfully treated by stereotactic body radiotherapy combined with molecularly targeted drugs followed by surgical treatment. If confirmed in future studies, this would suggest a promising strategy for the management of HCC with IVCTT.     

Management of complex liver cystic hydatidosis: challenging benign diseases for the hepatic surgeon: A case series report from an endemic area

Hydatid cysts of the liver are benign lesions which require a wide range of surgical strategies for their treatment. We hypothesized that cysts larger than 15 cm, or compressing main vascular structures, or located in both hemilivers should be considered, as well as complicated cysts, in the category of complex hydatid cysts.In a retrospective study including 55 patients, we evaluated the characteristics of complex hydatid cysts, and compared surgical outcomes between patients operated on for complex cysts (Complex Group) and those operated on for non-complex cysts (non-Complex Group).In the Complex Group, 19% of patients had cysto-biliary communication with recurrent cholangitis, 9.5% had cysts eroding the diaphragm or chest wall, or communicating with the bronchial tree, 31% had cysts with contact with main vascular structures, 11.9% had multiple bilobar cysts, 14.3% had giant cysts with organ displacement, and 14.3% had a combination of the above-mentioned types. Type of surgical treatment was different between the two groups (P < .001). Additional procedures were statistically more frequent in the Complex Group (P = .02). Postoperative morbidity was higher in the Complex Group, although not in a significant manner (P = .07). Median hospital stay was longer in the Complex Group (12 vs 7 days, P < .001). No 30-day mortality occurred. Four patients (7.3%), all belonging to the Complex Group, required reoperation for postoperative complications.Surgery for complex hydatid cysts of the liver is potentially burdened by serious complications. This kind of benign liver disease requires skill-demanding procedures and should be treated in centers with expertise in both hepato-biliary surgery and hydatid disease management.     

An update on the pathophysiology and management of polycystic liver disease

Introduction: Polycystic liver disease (PLD) is characterized by the presence of multiple cholangiocyte-derived hepatic cysts that progressively replace liver tissue. They are classified as an inherited ciliopathy /cholangiopathy as pathology exists at the level of the primary cilia of cholangiocytes. Aberrant expression of the proteins in primary cilia can impair their structures and functions, thereby promoting cystogenesis.

Areas covered: This review begins by looking at the epidemiology of PLD and its natural history. It then describes the pathophysiology and corresponding potential treatment strategies for PLD.

Expert commentary: Traditionally, therapies for symptomatic PLD have been limited to symptomatic management and surgical interventions. Such techniques are not completely effective, do not alter the natural history of the disease, and are linked with high rate of re-accumulation of cysts. As a result, there has been a push for drugs targeted at abnormal cellular signaling cascades to address deregulated proliferation, cell dedifferentiation, apoptosis and fluid secretion. Currently, the only available drug treatments that halt disease progression and improve quality of life in PLD patients are somatostatin analogues. Numerous preclinical studies suggest that targeting components of the signaling pathways that influence cyst development can ameliorate growth of hepatic cysts.     

Graves disease and metastatic hormonal-active Hürthle cell thyroid cancer: A case report

Rationale:A hormone-active metastatic Hürthle cell thyroid carcinoma (HCTC) and Graves disease (GD) present a therapeutic challenge and is rarely reported.Patient concerns:We present a 64-year-old male patient, who had dyspnea and left hip pain lasting 4 months. He had clinical signs of hyperthyroidism and a tumor measuring 9 cm in diameter of the left thyroid lobe, metastatic neck lymph node and metastases in the lungs, mediastinum, and bones.Diagnosis:Laboratory findings confirmed hyperthyroidism and GD. Fine-needle aspiration biopsy and cytological investigation revealed metastases of HCTC in the skull and in the 8th right rib. A CT examination showed a thyroid tumor, metastatic neck lymph node, metastases in the lungs, mediastinum and in the 8th right rib measuring 20 × 5.6 × 4.5 cm, in the left acetabulum measuring 9 × 9 × 3 cm and parietooccipitally in the skull measuring 5 × 4 × 2 cm. Histology after total thyroidectomy and resection of the 8th right rib confirmed metastatic HCTC.Interventions:The region of the left hip had been irradiated with concomitant doxorubicin 20 mg once weekly. When hyperthyroidism was controlled with thiamazole, a total thyroidectomy was performed. Persistent T3 hyperthyroidism, most likely caused by TSH-R-stimulated T3 production in large metastasis in the 8th right rib, was eliminated by rib resection. Thereafter, the patient was treated with 3 radioactive iodine-131 (RAI) therapies (cumulative dose of 515 mCi). Unfortunately, the tumor rapidly progressed after treatment with RAI and progressed 10 months after therapy with sorafenib.Outcomes:Despite treatment, the disease rapidly progressed and patient died due to distant metastases. He survived for 28 months from diagnosis.Lessons:Simultaneous hormone-active HCTC and GD is extremely rare and prognosis is dismal. Concomitant external beam radiotherapy and doxorubicin chemotherapy, followed by RAI therapy, prevented the growth of a large metastasis in the left hip in our patient. However, a large metastasis in the 8th right rib presented an unresolved problem. Treatment with rib resection and RAI did not prevent tumor recurrence. External beam radiotherapy and sorafenib treatment failed to prevent tumor growth.     

Effectiveness of perampanel in managing chronic pain caused by the complex regional pain syndrome: A case report

Rationale:The α-amino-3-hydroxy-5-methy-4-isoxazole propionate (AMPA) receptor plays a critical role in the development and persistence of pain, and AMPA receptor antagonists are considered possible therapeutic targets for controlling pain. This report describes a patient with complex regional pain syndrome (CRPS) type I in the right lower leg and foot who responded well to perampanel, an AMPA receptor antagonist, for managing the chronic pain.Patient concern:A 61-year-old woman complained of pain in her right lower leg and foot over a period of 7 year (numeric rating scale: 8) due to CRPS type I.Diagnosis:CRPS type 1.Interventions:Despite the combination of 300 mg pregabalin, 225 mg/1950 mg tramadol/acetaminophen, and 10 mg nortriptyline per day, her right lower leg and foot were nearly disabled due to the severity of the pain. High-dose prednisolone was found to be ineffective. Then, perampanel (4 mg; 2 mg twice) was administered to this patient daily.Outcomes:The day after treatment with perampanel, her pain completely disappeared. Additionally, at day 7 and 1 month follow-up, she reported no pain in the right lower leg and foot. Moreover, no adverse effects were reported after the application of perampanel.Lessons:These results suggest that perampanel may potentially be used to treat centralized pain.     

Endoscopically assisted transoral resection of a Bailey type IV second branchial cleft cyst: A case report

Rationale:The diagnosis of type IV branchial cleft cyst (BCC) according to the Bailey classification is very challenging due to lack of specific clinical manifestations in the early stage of the disease. Here, we present the transoral surgical route of endoscopic resection of second BCC in the parapharyngeal space (PPS) with good outcomes.Patient concerns:A 21-year-old man with a 1-year history of snoring complained about sore throat for 1 month and a fever that lasted for 3 days.Diagnoses:On admission, physical examination revealed a temperature of 39°C, pain when swallowing accompanied with a lump sensation in the throat, and inability to open mouth more than 3 cm. Blood testing revealed 19.29 × 10⁹ white blood cells (WBCs)/L and 14.94 × 10⁹ neutrophils/L. A cervical computed tomography (CT) examination revealed a mass with liquid density of 6.2 × 4.0 × 7.7 cm³ in the left parapharyngeal space (PPS) and pharyngeal cavity stenosis. Postoperative pathology showed the existence of lymphoepithelial cysts (left PPS), which was in accordance with the diagnosis of BCC.Interventions:The patient was administered 1.5 g ceftazidime every 12 hours, anti-inflammatory drugs, and incision drainage was performed subsequently. Then, endoscopy-assisted resection of the left PPS was performed via the transoral route. We used low-temperature plasma and an 8-Fr Foley catheter with a water capsule during the surgery.Outcomes:After resection of the mass, the patient''s blood results returned to within the normal range and his symptoms improved. Five days postoperatively, the incision made in the palatine arch of the pharynx opened up by 1 cm, and eventually the wound and laceration healed. Normal oral eating was restored, and no complications were observed.Lessons:Magnetic resonance imaging (MRI), and color Doppler ultrasound can be useful to diagnose BCC in PPS, which rarely occurs in the clinical setting. Extended endoscopy provides a satisfactory surgical field for trans-oral resection allowing complete resection of the BCC without serious postoperative complications.     

Nivolumab plus regorafenib in patients with small bowel adenocarcinoma: A case report

Introduction:Small bowel adenocarcinomas (SBAs) are rare cancers that have a distinct clinical characteristic and genetic profile. The only potentially curative treatment for localized SBAs is surgery, and treatment options are limited for patients in the advanced stage of disease.Patient concerns:A 39-year-old woman presented in October 2015 with a complaint of persistent vomiting for 8 months.Diagnosis:The patient had obstruction caused by a 3 × 2 cm mass at the ascending part of the duodenum and suspected metastasis in the right adnexal region. Postoperative pathology showed a moderately differentiated adenocarcinoma with serosal invasion. The diagnosis was stage IV duodenum adenocarcinoma with right adnexal metastasis.Interventions:After the failure of multi-line treatment with chemotherapy and targeted therapy, she was treated with the immune checkpoint inhibitor nivolumab plus regorafenib.Outcomes:Disease control lasted for 15 months with markedly improved symptoms.Conclusion:To the best of our knowledge, this is the first case of small bowel adenocarcinoma that has been treated with nivolumab combined with regorafenib. This case highlights the potential efficacy of combining nivolumab and regorafenib in the treatment of SBAs.     

A giant biliary cyst of Todani IA in a young woman: A case report

Rationale:Biliary cysts (BC) are rare dilatations of various parts of a biliary tract. They account for approximately 1% of all benign biliary diseases. Total cyst excision and Roux-Y hepaticojejunostomy is the treatment method of choice in most patients. In this paper, a novel surgical treatment with the use of internal biliary and pancreatic catheters was presented.Patient concerns:A 21-years-old woman with a giant choledochal cyst of Todani IA type presenting with abdominal pain, nausea, fever and palpable abdominal mass. It had been previously drained as a misdiagnosed pancreatic cyst in another hospital.Diagnosis:A very high amylase level (107140,0 U/l) in drain fluid was noted in laboratory tests. Endoscopic retrograde cholangiopancreatography revealed a biliary cyst located in the distal common bile duct and a pancreaticobiliary anomaly was suggested. A cholangiography per catheter inserted to the biliary cyst showed a large round contrast-filled cyst. A cholangiography following cyst decompression revealed a very long, tortuous bile duct entering the duodenum.Interventions:Cholecystectomy, cyst resection, Roux-Y hepaticojejunostomy, and implantation of catheters into pancreatic and bile duct were performed. The postoperative course was uneventful and she was discharged on 12th day without any complications. Histopathology revealed a cyst wall partially lined with biliary-type and mucinous epithelium, with dysplasia ranging from low to high grade (biliary intraepithelial neoplasia, high grade), without invasion.Outcomes:The biliary and pancreatic catheters were removed during endoscopic retrograde cholangiopancreatography 8 weeks following surgery without any complications. Fourteen months later, the patient reported good health.Lessons:Diagnosis of the abdominal cyst should be very precise in order to avoid misdiagnosis and inadequate management. The early diagnosis and proper treatment of BC are needed in order to avoid serious complications. The cholangiocarcinoma is the most dangerous potential complication of BC due to dysplasia within the cyst wall as in our young female patient.     

Primary Squamous Cell Carcinoma of Liver: Case Series and Review of Literatures

Primary squamous cell carcinoma (SCC) of liver is rare, and its prognosis is extremely poor. This study aims at reviewing the clinical data of all pathologically diagnosed liver cancer in our institute, and discussing the clinical presentation, diagnosis, treatment, and prognosis of our cases of SCC and the literatures reported previously.All the patients undergoing liver surgery or biopsy for liver cancers from 2002 to 2013 in our hospital were reviewed, and the liver specimens were examined pathologically. A literature search for case reports of primary SCC of liver published until December 31, 2014, was performed on PubMed, MEDLINE, Scopus Elsevier, Cochrane, and Google Scholar. The primitive data of the case reports were all included and analyzed if available.From January 2002 to October 2013, 2210 cases of liver cancer were diagnosed pathologically in our hospital. Among, 4 cases (0.2%) were diagnosed as primary SCC of liver. All were negative for hepatitis B infection, but present with liver cyst and/or hepatolithiasis. One patient underwent radical resection, but died of tumor recurrence 18 months postoperatively. One patient received transcatheter arterial chemoembolization and 1 patient received laparotomy and alcohol injection, but died 9 and 4 months after surgery, respectively. The last patient received only biopsy and supportive treatment, and finally died of tumor metastasis 6 months later. From 1970 to 2014, 31 cases of primary liver SCC have been published in English previously. Thirty one cases and the 4 cases in the present study were included. The average age of the patients were 54 years (range 18–83), with a male to female ratio of 19:16. Twenty patients had liver cysts, 7 had bile duct stones, and 2 cases had both. Patients undergoing radical surgery had better prognosis than those undergoing palliative treatments (median survival 17 vs 5 months, P = 0.005, log-rank test). Patients with liver cysts seemed to have worse prognosis than those with bile duct stones (median survival 7 vs 18 months, P = 0.090, log-rank test).Primary liver SCC seems to be mostly originated from liver cyst or hepatolithiasis. Radical surgery should be firstly recommended, although the prognosis might be unfavorable.     

Pazopanib-induced severe acute liver injury: A case report

Rationale:Drug-induced liver injury (DILI) is the most common cause of acute liver failure in the United States. Painkillers and fever antipyretics are the most common cause of DILI. Hepatic injury can be provoked by DILI as hepatocellular or cholestatic type.Patient concerns:A 48-year-old woman presented jaundice accompanied by nausea and vomiting. The patient was an inactive hepatitis B carrier with low viral titer and was diagnosed renal cell carcinoma (RCC) with hepatic metastasis requiring pazopanib treatment. Prior to administration of pazopanib, tenofovir administration was started to prevent exacerbation of hepatitis B. The patient was referred to clinic of gastroenterology department due to sudden elevation of bilirubin after 5 weeks of pazopanib treatment.Diagnoses:Abdominal ultrasound and computed tomography showed non-specific finding other than metastatic nodule in the liver and liver cirrhosis. After then, the patient was performed liver biopsy, and the biopsy result was acute cholestatic hepatitis with centrilobular area necrosis and portal inflammation. Therefore, considering the clinical history and biopsy results, the patient was diagnosed as DILI due to pazopanib.Interventions:After the biopsy, empirical steroid therapy was initiated and after 7 weeks of pazopanib discontinuation.Outcomes:The total bilirubin level returned to normal from peak level of 24.61 to 1.52 mg/dL.Lessons:In patients with renal cell carcinoma, pazopanib treatment requires clinical caution as it causes rare complications such as severe jaundice and acute cholestatic hepatitis.     

Effect of the stellate ganglion block on symptoms of ulcerative colitis: A case report

Rationale:Chronic ulcerative colitis is an autoimmune disease in which epithelial injury continuously occurs in the colonic mucosa. While mesalazine (5-aminosalicylic acid) is used to treat ulcerative colitis, it can also cause liver failure, headaches, and abdominal pain; therefore, an alternative treatment is required. The purpose of this study was to evaluate the effectiveness of 80 stellate ganglion blocks in reducing pain and other symptoms in a patient with chronic ulcerative colitis.Patient concerns:A 54-year-old female patient with a history of ulcerative colitis was concerned with worsening symptoms, such as abdominal discomfort and bloody-mucous stools, over the past 3 years.Diagnoses:Oozing mucosal bleeding and a small amount of exudate were observed on colonoscopy; a diagnosis of ulcerative colitis was made upon histologic examination.Interventions and outcomes:A total of 80 stellate ganglion blocks were administered, after which the patient''s symptom and pain level was decreased from 6 to 4 points on the numeric rating scale (11-point, 0 = no pain, 10 = worst pain imaginable). Improved clinical signs were observed on colonoscopy at a follow-up assessment.Lessons:The stellate ganglion block may be effective for the reduction of pain and other symptoms in patients with chronic ulcerative colitis.     

Cyst Infections in Patients with Autosomal Dominant Polycystic Kidney Disease

Background and objectives: Cyst infection is a complex diagnostic and therapeutic issue in patients with autosomal dominant polycystic kidney disease (ADPKD); however, published data regarding the diagnosis and the management of cyst infections in patients with ADPKD are sparse.Design, setting, participants, & measurements: A retrospective study was conducted in a referral center for patients with ADPKD in Paris, France. We identified using a computerized database all patients who had ADPKD and were admitted in the nephrology department of Hôpital Necker between January 1998 and August 2008 with likely or definite renal and/or hepatic cyst infection. Medical files of all included patients were reviewed.Results: Among 389 identified patients with ADPKD, 33 (8.4%) had 41 episodes of cyst infection, including eight definite and 33 likely cases. The incidence of cyst infections in patients with ADPKD was 0.01 episode per patient per year. Microbiological documentation was available for 31 episodes (75%), Escherichia coli accounting for 74% of all retrieved bacterial strains. Positron emission tomography scan proved superior to ultrasound, Computed tomography scan, and magnetic resonance imaging for the detection of infected cysts. Clinical efficacy of initial antibiotic treatment was noted in 71% of episodes. Antibiotic treatment modification was more frequently required for patients who were receiving initial monotherapy compared with those who were receiving bitherapy. Large (diameter >5 cm) infected cysts frequently required drainage.Conclusions: Positron emission tomography scan will probably make the diagnosis of cyst infections easier and more accurate. Antibiotic association, including a fluoroquinolone, and the drainage of large infected cysts remain the main treatment for cyst infections.Autosomal dominant polycystic kidney disease (ADPKD) represents the most common inherited disorder affecting one in 500 to one in 1000 live births and accounting for 4 to 10% of dialysis patients. The most striking feature of ADPKD is the occurrence of numerous renal and hepatic cysts, which arise from various renal tubule segments and lead to an increased kidney size. Cysts are also associated with some of the most common complications of ADPKD: Intracystic bleeding, gross hematuria, obstruction mainly caused by liver cysts, and, most important, infections. Kidney and liver cyst infection is a complex diagnostic and therapeutic challenge; however, the literature on the diagnosis and the management of urinary tract infections and particularly cyst infections in patients with ADPKD is relatively sparse. The clinical, microbiological, and radiologic features of cyst infections as well as treatment regimens remain ill-defined (1–3). We conducted a retrospective, single-center study to assess the clinical and radiologic presentation and treatment outcomes of cyst infections in patients with ADPKD.     

Application of ex vivo liver resection and autotransplantation in treating Budd-Chiari syndrome secondary to end-stage hepatic alveolar echinococcosis: A case series

Background:Secondary Budd-Chiari syndrome (BCS) occurs due to a blockage in the liver caused by invasion or compression by a large lesion. Conventional treatments for BCS do not solve practical problems, wherease liver transplantation has been only applied as a last-resort therapy and as the only opportunity for a radical cure. We explored the feasibility of applying ex vivo liver resection and autotransplantation (ELRA) for the new indications of treating patients with end-stage hepatic alveolar echinococcosis (HAE). Our center has firstly proposed the idea and successfully treated the 49 patients with HAE. This article for the first time reports the application of ELRA in treating patients with BCS secondary to HAE.Methods:According to the degree of lesion invasion and surgical options, 11 patients were divided into 4 types. These 11 patients had large lesions that invaded the second and third hilum of the caudate lobe and involved the confluence of the hepatic vein and the inferior vena cava, suprahepatic vena cava, or at least 2 hepatic veins and led to secondary BCS. The aim of the present work was to report 11 patients with life-threatening diseases who underwent ELRA (ex vivo liver resection and autotransplantation) for secondary BCS, to propose a classification system for secondary BCS, and to suggest that secondary BCS is an indication for ELRA.Results:Eleven patients successfully underwent ELRA without intraoperative mortality. The median autograft weight was 690 g (440–950 g); operative time was 12.5 hours (9.4–16.5 hours); Postoperative hospital stay was 15 days (7–21 days). Clavien-Dindo grade IIIa or higher postoperative complications occurred in only 5 patients.Conclusions:This article for the first time reports the application of ELRA in treating patients with BCS secondary to HAE, not only provides new ideas for alternative treatments of secondary BCS, but also provides a classification system for secondary BCS. This article describes the technical process of outflow tract reconstruction and the experience for expanding the indications for ELRA. Our study demonstrated that ELRA is well feasible for treatment of BCS secondary to advanced HAE.     

Primary extranodal natural Killer/T-cell lymphoma in a child in the colon: A case report

Rationale:Primary extranodal natural killer (NK)/T-cell lymphoma (ENKTL) rarely occurs in childhood and adolescence. To the best of our knowledge, ENKTL of childhood in the gastrointestinal (GI) tract has not been reported yet.Patient concerns:A 12-year-old Chinese boy complained of abdominal pain and persistent fever for 1 month.Diagnosis:Grossly an ulcerated tumor with perforation was located at the proximal ascending colon, 5 cm × 4 cm × 1.5 cm in diameter. The tumor was poorly circumscribed, tan-white and solid. Histological evaluation revealed medium-sized atypical lymphoid cells with large areas of necrosis distributed throughout all layers of the colon. Small blood vessels with destroyed walls were surrounded by lymphoid cells. Immunohistochemistry (IHC) highlighted tumor cells as strongly positive for CD3, CD56, CD5, CD2, CD8, CD4, CD43, T-cell restricted intracellular antigen 1 (TIA-1) and granzyme B. The proliferation index, measured by Ki-67 expression was high with 60%. The In situ hybridization (ISH) for EBER was positive. TCR was negative. Therefore, the final diagnosis was ENKTL of childhood in the colon.Interventions:The patient underwent right hemicolectomy and ileocolostomy.Outcomes:We recommended further evaluation and treatment, but the patient and patient family rejected further treatment of his condition. The patient died within 1 month after being discharged from hospital as a result of his disease.Lessons:ENKTL of childhood in the GI tract is extremely rare. Due to the non-specific clinical symptoms, it is easy it is easy not to think of this differential diagnosis at early stage. If patients have GI symptoms, ENKTL cannot easily be ignored. It is necessary to diagnose ENKTL of childhood in the GI tract by morphology and immunohistochemistry, and to differentiate from the GI T-cell lymphomas. We hope this case may serve as a reference improving clinical diagnosis and treatment.