Gastrointestinal neuromuscular pathology in alpers disease

Alpers disease is a recessive mitochondrial disorder caused by mutations in POLG1 and characterized primarily by progressive neurological and hepatic degeneration. Intestinal dysmotility is a frequent symptom, but it is often overshadowed by other clinical manifestations. The onset and progression of Alpers disease vary; however, most patients die during childhood, often before a specific diagnosis has been established. The gastrointestinal neuromuscular pathology of 4 patients, obtained largely from postmortem specimens, showed distinctive eosinophilic cytoplasmic granules in a subset of enteric ganglia and patchy atrophy of small intestinal muscularis externa. The cytoplasmic inclusions corresponded to abnormal mitochondria, which have been reported previously in another mitochondrial disorder (mitochondrial neurogastrointestinal encephalomyopathy) but not in Alpers disease. Recognition of these distinctive light microscopic findings, in an appropriate clinical setting, should prompt the evaluation of an underlying primary mitochondriopathy.     

Adrenaline injection plus argon plasma coagulation versus adrenaline injection plus hemoclips for treating high-risk bleeding peptic ulcers: A prospective, randomized trial

BACKGROUND/OBJECTIVE:

Several combination endoscopic therapies are currently in use. The present study aimed to compare argon plasma coagulation (APC) + adrenaline injection (AI) with hemoclips + AI for the treatment of high-risk bleeding peptic ulcers.

METHODS:

In a prospective randomized trial, 172 patients with major stigmata of peptic ulcer bleeding were randomly assigned to receive APC + AI (n=89) or hemoclips + AI (n=83). In the event of rebleeding, the initial modality was used again. Patients in whom treatment or retreatment was unsuccessful underwent emergency surgery. The primary end point of rebleeding rate and secondary end points of initial and definitive hemostasis need for surgery and mortality were compared between the two groups.

RESULTS:

The two groups were similar in all background variables. Definitive hemostasis was achieved in 85 of 89 (95.5%) of the APC + AI and 82 of 83 (98.8%) of the hemoclips + AI group (P=0.206). The mean volume of adrenaline injected in the two groups was equal (20.7 mL; P=0.996). There was no significant difference in terms of initial hemostasis (96.6% versus 98.8%; P=0.337), rate of rebleeding (11.2% versus 4.8%; P=0.124), need for surgery (4.5% versus 1.2%; P=0.266) and mortality (2.2% versus 1.2%; P=0.526). When compared for the combined end point of mortality plus rebleeding and the need for surgery, there was an advantage for the hemoclip group (6% versus 15.7%, P=0.042).

CONCLUSION:

Hemoclips + AI has no superiority over APC + AI in treating patients with high-risk bleeding peptic ulcers. Hemoclips + AI may be superior when a combination of all negative outcomes is considered.     

A correlation study on diagnostic endoscopic ultrasound-guided fine-needle aspiration of lymph nodes with histological and clinical diagnoses, the UCLA Medical Center experience

Endoscopic ultrasound guided (EUS) FNA procedure has two aspects, the endoscopic sampling and the FNA interpretation. The two aspects of the procedure are performed in two different disciplines; gastroenterology (EUS) and pathology (FNA). The aim of this study was to evaluate the concordance, sensitivity, specificity, positive predictive (PPV), and negative predictive values (NPV).Sixty-one EUS-FNA procedures of the lymph nodes were analyzed by correlating the FNA results with histological or clinical diagnoses. The lymph nodes were divided in five groups; mediastinal, gastrohepatic, peripancreatic, portal, and perirectal.The study showed a concordance of 92% in mediastinal, 80% in gastrohepatic, 81% in peripancreatic, 95% in portal, and 100% in perirectal lymph nodes with an overall sensitivity of 84%, specificity of 92%, PPV of 88%, and NPV of 89%. In conclusion, EUS-FNA offers an invaluable approach for diagnostic examination of the internal lymph nodes where percutaneous FNA is either difficult or impossible. impossible.     

Functioning adrenocortical oncocytoma: a case report and review of literature

Adrenocortical oncocytoma is very rare. Less than five functioning types of them are reported and most of the reported cases are incidentally found. We herein report a case of functioning adrenocortical oncocytoma of the left adrenal cortex in a young woman.     

Chemoradiation in nasopharyngeal carcinoma: a 6-year experience in Tehran cancer institute

To determine the addition of value of neoadjuvant, concurrent and adjuvant chemotherapy to radiation in the treatment of nasopharyngeal carcinoma with regard to the overall survival (OS) and disease free survival (DFS) within a six year period in Tehran cancer institute. Files of all patients with nasopharyngeal carcinoma treated by radiotherapy with or without concurrent chemotherapy in a curative setting in Tehran cancer institute during the period of 1999-2005 were retrospectively reviewed.. A total of 103 patients with nasopharyngeal carcinoma had been treated during the study period with radiotherapy or chemoradiotherapy in our institute. There were 29 (28.2%) females and 74 (71.8%) males. The median age at the time of radiotherapy was 47 years old (range 9-75 years). The patients were followed 2 to 76 months with a median follow-up of 14 months. Time of first recurrence after treatment was 3-44 months with a median of 10 months.. Survival in 2 groups of patients treated with radiotherapy alone or chemoradiation did not have a significant difference (P>0.1). Two-year survival in patients treated with or without adjuvant chemotherapy and had local recurrence after treatment did not have significant difference (P>0.1). Two-year survival in patients with or without local recurrence after treatment did not have significant difference (P>0.1). A beneficial affect or a survival benefit of adjuvant/neoadjuvant chemotherapy and concurrent chemoradiation was not observed in Iranian patients.