Hereditary hemochromatosis: Temporal trends, sociodemographic characteristics, and independent risk factor of hepatocellular cancer - nationwide population-based study.

BACKGROUND: Hereditary hemochromatosis (HH) has an increased risk of hepatocellular cancer (HCC) both due to genetic risks and iron overload as iron overload can be carcinogenic; HH impacts the increasing risk of HCC, not only through the development of cirrhosis but concerning hepatic iron deposition, which has been studied further recently. AIM: To evaluate HH yearly trends, patient demographics, symptoms, comorbidities, and hospital outcomes. The secondary aim sheds light on the risk of iron overload for developing HCC in HH patients, independent of liver cirrhosis complications. The study investigated HH (without cirrhosis) as an independent risk factor for HCC. METHODS: We analyzed data from National Inpatient Sample (NIS) Database, the largest national inpatient data collection in the United States, and selected HH and HCC cohorts. HH was first defined in 2011 International Classification of Disease - 9th edition (ICD-9) as a separate diagnosis; the HH cohort is extracted from January 2011 to December 2019 using 275.01 (ICD-9) and E83.110 (ICD-10) diagnosis codes of HH. Patients were excluded from the HH cohort if they had a primary or secondary diagnostic code of cirrhosis (alcoholic, non-alcoholic, and biliary), viral hepatitis, alcoholic liver disease, non-alcoholic fatty liver disease (NAFLD), and non-alcoholic steatohepatitis (NASH). We removed these patients from the HH cohort to rule out bias or ICD-10 diagnostic errors. The HCC cohort is selected from January 2011 to December 2019 using the ICD-9 and ICD-10 codes of HCC. We selected a non-HCC cohort with the 1:1 fixed ratio nearest neighbor (greedy) propensity score method using the patients' age, gender, and race. We performed multivariate analysis for the risk factors of HCC in the HCC and non-HCC matched cohort. We further analyzed HH without cirrhosis (removing HH patients with a diagnosis of cirrhosis) as an independent risk factor of HCC after adjusting all known risk factors of HCC in the multivariate model. RESULTS: During the 2011-2019 period, a total of 18031 hospitalizations with a primary or secondary diagnosis of HH (excluding liver diseases) were recorded in the NIS database. We analyzed different patients' characteristics, and we found increments in inpatient population trend with a Ptrend < 0.001 and total hospital cost of care trend from $42957 in 2011 to $66152 in 2019 with a Ptrend < 0.001 despite no change in Length of Stay over the last decade. The multivariate analyses showed that HH without cirrhosis (aOR, 28.8; 95%CI, 10.4-80.1; P < 0.0001), biliary cirrhosis (aOR, 19.3; 95%CI, 13.4-27.6; P < 0.0001), non-alcoholic cirrhosis (aOR, 17.4; 95%CI, 16.5-18.4; P < 0.0001), alcoholic cirrhosis (aOR, 16.9; 95%CI, 15.9-17.9; P < 0.0001), hepatitis B (aOR, 12.1; 95%CI, 10.85-13.60; P < 0.0001), hepatitis C (aOR, 8.58; 95%CI, 8.20-8.98; P < 0.0001), Wilson disease (aOR, 4.27; 95%CI, 1.18-15.41; P < 0.0001), NAFLD or NASH (aOR, 2.96; 95%CI, 2.73-3.20; P < 0.0001), alpha1-antitrypsin deficiency (aOR, 2.10; 95%CI, 1.21-3.64; P < 0.0001), diabetes mellitus without chronic complications (aOR, 1.17; 95%CI, 1.13-1.21; P < 0.0001), and blood transfusion (aOR, 1.80; 95%CI, 1.69-1.92; P < 0.0001) are independent risk factor for liver cancer. CONCLUSION: Our study showed an increasing trend of in-hospital admissions of HH patients in the last decade. These trends were likely related to advances in diagnostic approach, which can lead to increased hospital utilization and cost increments. Still, the length of stay remained the same, likely due to a big part of management being done in outpatient settings. Another vital part of our study is the significant result that HH without cirrhosis is an independent risk factor for HCC with adjusting all known risk factors. More prospective and retrospective large studies are needed to re-evaluate the HH independent risk in developing HCC.

Pancreatic Ganglioneuroma Presenting in an Octogenarian.

Pancreatic ganglioneuromas occur mostly in children and rarely in young adults, with no cases reported in adults older than 60 years. An 86-year-old-woman, with active advanced multiple myeloma, presented with epigastric pain for 2 days. Abdominal and pelvic computed tomography demonstrated a distended gallbladder, mildly dilated biliary tree, and a 13 × 8-mm hypodense mass in pancreatic body, without extrapancreatic invasion at endoscopic ultrasound. Fine-needle endoscopic ultrasound-guided core biopsy revealed characteristic histopathology of ganglioneuroma, as confirmed by immunohistochemical positivity for S100, SOX-10, and synaptophysin. This demonstrates novel finding of pancreatic ganglioneuroma occurring in the elderly. Lesion inclusion in the differential diagnosis may mandate tissue for pathologic diagnosis and complete lesion resection.

Novel Case Report: A Previously Reported, but Pathophysiologically Unexplained, Association Between Collagenous Colitis and Protein-Losing Enteropathy May Be Explained by an Undetected Link with Collagenous Duodenitis.

Collagenous colitis (CC) is associated with non-bloody, watery diarrhea, which is pathophysiologically reasonable because normal colonic absorption (or excretion) of water and electrolytes can be blocked by the abnormally thick collagen layer in CC. However, CC has also been associated with six previous cases of protein-losing enteropathy (PLE), with no pathophysiologic explanation. The colon does not normally absorb (or excrete) amino acids/proteins, which is primarily the function of the small bowel. Collagenous duodenitis (CD) has not been associated with PLE. This work reports a novel case of CD (and CC) associated with PLE; a pathophysiologically reasonable mechanism for CD causing PLE (by the thick collagen layer of CD blocking normal intestinal amino acid absorption); and a novel association of PLE with severe COVID-19 infection (attributed to relative immunosuppression from hypoproteinemia, hypoalbuminemia, hypogammaglobulinemia, and malnutrition from PLE).

Proposed characterization of the syndrome of epidural pneumatosis (pneumorrhachis) in patients with forceful vomiting from diabetic ketoacidosis as a clinico-radiologic pentad based on systematic literature review & an illustrative case report.

BACKGROUND: Previous literature on epidural pneumatosis (pneumorrhachis, or air in epidural cavity) associated with forceful vomiting in a patient with diabetic ketoacidosis (DKA) has consisted of individual case reports without comprehensive syndrome characterization due to syndromic rarity, with the largest previous literature review comprising 6 cases. Presumed pathophysiology is air escaping from alveolar rupture from forceful vomiting via tissue planes to cause epidural pneumatosis. AIM: Systematically review literature to facilitate syndromic diagnosis, evaluation, and treatment. A new illustrative case is reported. METHODS: Systematic review of literature using 2 independent readers, 2 computerized databases, and the following medical terms/keywords: ["epidural pneumatosis" OR "pneumorrhachis"] AND ["diabetes" OR "diabetic ketoacidosis" or "DKA"]. Discrepancies between 2 readers were resolved by consensus using prospectively developed study inclusion criteria. Two readers independently abstracted case report. Prospective review protocol and patients, problems, intervene, comparison group, outcomes discussed in Methods section of paper. RESULTS-SYSTEMATIC-LITERATURE-REVIEW: Revealed 10 previously reported cases plus 1 new case (see below) that shows this syndrome presents rather stereotypically with the tentatively proposed following pentad (% of patients fulfilling individual criterion): 1-forceful vomiting (100%), 2-during DKA (100%), 3-pneumomediastinum from forceful alveolar rupture (100%), 4-epidural pneumatosis from air escape from pneumomediastinum (100%), and 5-no complications of Boerhaave syndrome or of focal neurological deficits (100%). Pentad is pathophysiologically reasonable because forceful vomiting can cause alveolar rupture, pneumomediastinum, and air entry into epidural space. RESULTS-ILLUSTRATIVE-CASE-REPORT: Epidural pneumatosis occurred in a 33-year-old-male with poorly controlled diabetes mellitus type 1 who presented with forceful vomiting while in DKA. Radiologic findings also included subcutaneous emphysema, pneumomediastinum, and small pneumothorax. The patient rapidly improved while receiving acute therapy for DKA, and was discharged after 2 hospital days. STUDY LIMITATIONS: Limited number of analyzed, retrospectively reported cases. Case reports subject to reporting bias. Specificity, positive predictive value, and negative predictive value not meaningfully analyzed in this homogeneous population. CONCLUSIONS: Based on systematic review, syndrome is tentatively proposed as a pentad with: 1-forceful vomiting, 2-during DKA, 3- pneumomediastinum, 4-epidural pneumatosis, and 5-no complications of Boerhaave syndrome or focal neurological deficits. Proposed pentad should be prospectively tested in a larger population including patients with this versus closely related syndromes.

Perioperative hypothermia in colorectal surgeries: are we doing enough to prevent it?

OBJECTIVE: To determine the incidence of hypothermia in patients undergoing colorectal surgery, and to identify factors that increase vulnerability to perioperative hypothermia. METHODS: The retrospective study was conducted at the Aga Khan University Hospital, Karachi, and comprised medical records from May 2012 to June 2017 related to all patients aged >16 years of either gender who underwent colorectal procedures. Analysis about predictors of perioperative hypothermia was done using Stata 12. RESULTS: Of the 100 patients, 69(69%) were males. The overall mean age was 50.2±16.7 years. Majority cases had elective presentation 72(72%). Incidence of perioperative hypothermia was noted in 74(74%) patients. Postoperative morbidity was 16(16%), while mortality was 4(4%). Elective presentation and hypothermia before surgery were significantly associated with occurrence of intraoperative hypothermia (odds ratio: 4.5 and 1.3 respectively). CONCLUSIONS: Perioperative incidence of hypothermia was found to be quite high despite appropriate measures. Factors responsible need to be explored and rectified.

Does academic assessment system type affect levels of academic stress in medical students? A cross-sectional study from Pakistan.

INTRODUCTION: Stress among medical students induced by academic pressures is on the rise among the student population in Pakistan and other parts of the world. Our study examined the relationship between two different systems employed to assess academic performance and the levels of stress among students at two different medical schools in Karachi, Pakistan. METHODS: A sample consisting of 387 medical students enrolled in pre-clinical years was taken from two universities, one employing the semester examination system with grade point average (GPA) scores (a tiered system) and the other employing an annual examination system with only pass/fail grading. A pre-designed, self-administered questionnaire was distributed. Test anxiety levels were assessed by The Westside Test Anxiety Scale (WTAS). Overall stress was evaluated using the Perceived Stress Scale (PSS). RESULTS: There were 82 males and 301 females while four did not respond to the gender question. The mean age of the entire cohort was 19.7 ± 1.0 years. A total of 98 participants were from the pass/fail assessment system while 289 were from the GPA system. There was a higher proportion of females in the GPA system (85% vs. 59%; p < 0.01). Students in the pass/fail assessment system had a lower score on the WTAS (2.4 ± 0.8 vs. 2.8 ± 0.7; p = 0.01) and the PSS (17.0 ± 6.7 vs. 20.3 ± 6.8; p < 0.01), indicating lower levels of test anxiety and overall stress than in students enrolled in the GPA assessment system. More students in the pass/fail system were satisfied with their performance than those in the GPA system. CONCLUSION: Based on the present study, we suggest governing bodies to revise and employ a uniform assessment system for all the medical colleges to improve student academic performance and at the same time reduce stress levels. Our results indicate that the pass/fail assessment system accomplishes these objectives.

Depression is linked to uncontrolled hypertension: a case-control study from Karachi, Pakistan.

BACKGROUND: High blood pressure is an important risk factor for cardiovascular disease and may be more prevalent in persons with psychopathology. OBJECTIVE: To determine the association between uncontrolled hypertension with depressive and anxiety disorders in two tertiary care centers in Karachi. METHODS: It was a case-control study conducted in two hospitals in Karachi. Cases were patients with uncontrolled hypertension and controls were patients with controlled hypertension. History of anxiety or depression as measured by Hospital Anxiety and Depression Scale (HADS) was the exposure. HADS of >8 was suggestive of having anxiety or depression. RESULTS: A total of 700 participants were approached, out of whom 590 fulfilled the inclusion criteria and consented to participate, 323 (54.7%) participants were enrolled as cases and 267 (45.3%) as controls. Mean (SD) age was 54.98 (12.38) years, 229 (38%) were males. Odds ratio (OR) (95% CI) of having uncontrolled hypertension and being depressed (HADS-D > 8) was 2.02 (1.44-2.83), p value ≤ 0.001. The association remained significant even after adjusting for age and gender in Model 1 OR (95% CI): 1.82 (1.27-2.60), p value = 0.001, ethinicity and education in Model 2 OR (95% CI): 1.87 (1.29-2.71), p value = 0.001 and comorbids, history of hospitalization body mass index in Model 3 OR (95% CI): 1.94 (1.31-2.85), p value = 0.001. CONCLUSION: There is association between uncontrolled hypertension and depression that is independent of sociodemographic factors, comorbids and history of hospitalization.