Pulmonary Lymphangiomyomatosis: A Rare Disease responsive to Progesterone

Lymphangiomyomatosis (LAM) is a rare multisystem disorder in women of child-bearing age. We present a case of a 28 year old lady who presented with cough and breathlessness. She had been diagnosed as a case of lymphangiomyomatosis by computer tomography of chest. She showed dramatic clinical improvement with hormonal therapy

Gastrointestinal Anastomosis with Lumen Apposing Metal Stent

Gastric outlet obstruction, afferent or efferent limb obstruction, and biliary obstruction among patients with altered anatomy often require surgical intervention which is associated with significant morbidity and mortality. Endoscopic dilation for benign etiologies requires multiple sessions, whereas self-expandable metal stents used for malignant etiologies often fail due to tumor in-growth. Lumen apposing metal stents, placed endoscopically with the intent of creating a de-novo gastrointestinal anastomosis bypassing the site of obstruction, can potentially achieve similar efficacy, with a much lower complication rate. In our study cohort (n=79), the composite technical success rate and clinical success rate was 91.1% (72/79) and 97.2% (70/72), respectively. Five different techniques were used: 43% (34/79) underwent the balloon-assisted method, 27.9% (22/79) underwent endoscopic ultrasound-guided balloon occluded gastro-jejunostomy bypass, 20.3% (16/79) underwent the direct technique, 6.3% (5/79) underwent the hybrid rendezvous technique, and 2.5% (2/79) underwent natural orifice transluminal endoscopic surgery (NOTES)-assisted procedure. All techniques required an echoendoscope except NOTES. In all, 53.2% (42/79) had non-cautery enhanced Axios stent, 44.3% (35/79) had hot Axios stent, and 2.5% (2/79) had Niti-S spaxus stent. Symptom-recurrence was seen in 2.8%, and 6.3% had a complication (bleeding, abdominal pain or peritonitis). All procedures were performed by experts at centers of excellence with adequate surgical back up

International external validation of a stratification tool to identify branch-duct intraductal papillary mucinous neoplasms at lowest risk of progression

BACKGROUND: Identifying branch‐duct intraductal papillary mucinous neoplasms (BD‐IPMNs) at lowest risk of progression may allow for a reduced intensity of surveillance. OBJECTIVE: We aimed to externally validate the previously developed Dutch‐American Risk stratification Tool (DART‐1; https://rtools.mayo.edu/DART/), which identifies cysts at low risk of developing worrisome features (WFs) or high‐risk stigmata (HRS). METHODS: Three prospective cohorts of individuals under surveillance for BD‐IPMNs were combined, independent from the original development cohort. We assessed the performance (discrimination and calibration) of DART‐1, a multivariable Cox‐proportional logistic regression model with five predictors for the development of WFs or HRS. RESULTS: Of 832 individuals (mean age 77 years, SD 11.5) under surveillance for a median of 40 months (IQR 44), 163 (20%) developed WFs or HRS. DART‐1's discriminative ability (C‐statistic 0.68) was similar to that in the development cohort (0.64–0.72) and showed moderate calibration. DART‐1 adequately estimated the risk for patients in the middle risk quintile, and slightly underestimated it in the lowest quintiles. Their range of predicted versus observed 3‐year risk was 0%–0% versus 0%–3.7% for Q1; 0.3%–0.4% versus 3%–11% for Q2; and 2.6%–3% versus 2.4%–9.8% for Q3. The development of WFs or HRS was associated with pancreatic cancer (p < 0.001). Vice versa, in absence of WFs or HRS, the risk of malignancy was low (0.3%). CONCLUSIONS: The performance of DART‐1 to predict the development of WFs or HRS in BD‐IPMN was validated in an external international cohort, with a discriminative ability equal as in the development cohort. Risk estimations were most accurate for patients with BD‐IPMNs in the middle risk quintile and slightly underestimated in the lowest quintiles

Effect of insulin therapy and obesity on colon adenoma and advanced adenoma among type II diabetes mellitus population

Insulin is a debatable risk factor for colon adenoma (Ad) among type II diabetes mellitus (DM II) patients. Obesity is an important confounding variable. The study involved chart review of DM II patients undergoing screening colonoscopy. Study population was divided into obese [body mass index (BMI)≥30] and nonobese (BMI&lt;30) groups which were further divided into insulin and non-insulin subgroup. Colonoscopy and pathology reports were used to calculate Ad detection rate (ADR) and AAd detection rate (AADR). A total of 538 subjects satisfied the inclusion and exclusion criteria. The study population composed of 52.8% obese and 47.2% non-obese subjects. Obese group had 28.9% insulin and 71.1% non-insulin subjects. Non-obese group composed of 29.9% insulin and 70.1% non-insulin subjects. ADR for non-obese insulin and non-insulin subgroup was 31.6% and 37.1% respectively. AADR for non-obese insulin and non-insulin subgroup was 13.2% and 11.2% respectively. ADR for obese insulin and non-insulin subgroup was 41.5% and 34.2% respectively. AADR for obese insulin and non-insulin subgroup was 15.9% and 16.3% respectively. Insulin exposure lacked statistically significant association with ADR or AADR among obese and non-obese DM II subjects

Pulmonary foreign body granulomatosis in a chronic user of powder cocaine

ABSTRACT We describe the case of a 33-year-old man, a chronic user of powder cocaine, who presented with dyspnea, fever, night sweats, and significant weight loss. Chest HRCT revealed centrilobular nodules, giving an initial impression of miliary tuberculosis. Therefore, he was started on an empirical, four-drug antituberculosis treatment regimen. Four weeks later, despite the tuberculosis treatment, he continued to have the same symptoms. We then performed transbronchial lung biopsy. Histopathological analysis of the biopsy sample revealed birefringent foreign body granuloma. A corroborative history of cocaine snorting, the presence of centrilobular nodules, and the foreign body-related histopathological findings led to a diagnosis of pulmonary foreign body granulomatosis. This report underscores the fact that pulmonary foreign body granulomatosis should be included in the differential diagnosis of clinical profiles resembling tuberculosis

Contrast Enhanced Harmonic Endoscopic Ultrasound: A Novel Approach for Diagnosis and Management of Gastrointestinal Stromal Tumors

The histologic analysis of gastrointestinal stromal tumors (GISTs) is a common method to detect the mitotic activity and to subsequently determine the risk of GISTs for malignancy. The potential false negative error due to inadequate yield of specimens and actual determination of malignancy risk requires analysis of the whole tumor. We aimed to assess the role of contrast enhanced endoscopic ultrasound (CE-EUS) in the management of GISTs. Two authors individually did review of English literatures to identify nine peer-reviewed original articles using keywords- contrast endoscopic ultrasound, GIST and submucosal tumor. Studies were heterogeneous in their aims looking either at differentiating submucosal lesions from GISTs, estimating malignant potential of GISTs with histologic correlation or studying the role of angiogenesis in malignant risk stratification. CE-EUS had moderate to high efficacy in differentiating GISTs from alternative submucosal tumors. CE-EUS had a higher sensitivity than EUS-guided fine needle aspiration, contrast computed tomography and Doppler EUS for detection of neo-vascularity within the GISTs. However, the evidence of abnormal angiogenesis within GIST as a prognostic factor needs further validation. CE-EUS is a non-invasive modality, which can help differentiate GISTs and provide valuable assessment of their perfusion patterns to allow better prediction of their malignant potential but more experience is needed