Gastrointestinal Disease in Patients with Common Variable Immunodeficiency: A Retrospective Observational Study.

BACKGROUND: Gastrointestinal (GI) symptoms are common among patients with common variable immunodeficiency disorder (CVID) yet remain poorly understood. AIMS: The aim of this study was to characterize the demographic, clinical, endoscopic and histologic features of patients with CVID and GI symptoms. METHODS: We conducted a retrospective observational study of all patients with CVID at a large Canadian tertiary care centre between January 2000 and May 2018. RESULTS: We included 95 patients with CVID. The mean age of patients at the time of CVID diagnosis was 38.2(±16.0). Fifty-three (56%) patients were female. Sixty-four (67%) patients had GI symptoms, with a mean age of onset for GI symptoms of 43.4(±15.1) years. The most common symptoms were bowel movement changes (n = 55 [58%]) and abdominal pain (n = 44 [46%]). Patients with GI symptoms were more likely to have anemia (n = 23 [36%] versus n = 3 [10%], P = 0.0129), iron deficiency (n = 16 [25%] versus n = 2 [7%], P = 0.0481), and have received GI antibiotics (n = 37 [58%] versus n = 0, P < 0.0001) and proton pump inhibitors for reflux (n = 24 [38%] versus n = 3 [10%], P = 0.0067). The most common GI infections were Giardia lamblia (n = 14 [15%]) and Clostridium difficile (n = 4 [4%]). Forty-three (45%) patients with GI symptoms underwent colonoscopy, esophagogastroduodenoscopy or both. The most common findings were inflammation, nodular lymphoid hyperplasia, reduced plasma cells and increased intraepithelial lymphocytes. CONCLUSIONS: This is the largest study on CVID patients in a North American setting. The majority of patients experienced GI symptoms. Future studies should study response to treatment for GI disease among patients with CVID.

Clinical, endoscopic, and histologic characteristics of lymphocytic esophagitis: a systematic review.

OBJECTIVE: Lymphocytic esophagitis (LyE) is a novel, yet poorly described, clinicopathologic entity. The aim of this systematic review was to characterize the demographic, clinical, endoscopic, and histologic features of LyE in observational studies of adult and pediatric patients. DESIGN: We searched the Embase, MEDLINE, and SCOPUS databases for relevant studies in 2018. Two authors reviewed and extracted data from studies that met the inclusion and exclusion criteria. RESULTS: We identified 20 studies for analysis of demographic, clinical, and endoscopic features of LyE. The mean age ranged from 9 to 67 years. When pooled, there were 231 (52.7%) patients with LyE that were female. The most common presenting symptom was dysphagia reported in 191 (48.8%) patients. On endoscopy, most patients with LyE tended to have abnormal findings (69.0%), which included erosive esophagitis, multiple esophageal rings, linear furrows, and narrow-caliber esophagus. In the 31 studies used to assess the histologic definition, the cut-off number of intraepithelial lymphocytes (IELs) was reported in 16 (51.6%) studies, peripapillary IEL specification in 18 (58.1%) studies, and presence of spongiosis in 6 (19.4%) studies. CONCLUSION: We identified a spectrum of demographic, clinical, and endoscopic findings characteristic of patients with LyE. A consensus on the diagnostic criteria of LyE is required.

Association between troponin-I levels and outcome in critically ill patients admitted to non-cardiac intensive care unit with high prevalence of cardiovascular risk factors.

BACKGROUND: The association of troponin-I levels and outcome in medical-surgical ICU patients has been studied before in populations with low to moderate prevalence of cardiovascular risk factors. The objective in this article is to examine the association of troponin-I levels with hospital mortality in patients with high prevalence of cardiovascular risk factors who were admitted with medical-surgical indications to a non-cardiac intensive care unit. METHODS: This was a retrospective study of adult patients admitted to a tertiary medical-surgical ICU between July 2001 and November 2011. Data were extracted from prospectively collected ICU and clinical laboratory databases. Patients were stratified based on the highest troponin-I level in the first 72 h of admission into four groups (Group I < 0.03, Group II = 0.03-0.3, Group III = 0.3-3 and Group IV > 3 ng/mL). Hospital mortality was the primary outcome. To study the association between elevated troponin-I and hospital mortality, we carried out multivariate logistic regression analyses with Group I as a reference group. RESULTS: During the study period, 3368 patients had troponin-I levels measured in the first 72 h, of whom 1293 (38.3%) were diabetic and 1356 (40.2%) were chronically hypertensive. Among the study population, 2719 (81%) had elevated troponin-I levels (0.03 ng/mL and higher). Hospital mortality increased steadily as the troponin-I levels increased. Hospital mortality was 23.4% for Group I, 33.2% for Group II (adjusted odds ratio (aOR) 1.08, 95% confidence interval (CI) 0.84, 1.38), 49.6% for Group III (aOR = 1.64, 95% CI 1.24, 2.17), and 57.4% for Group IV (aOR 1.80, 95% CI 1.30, 2.49). The association of increased mortality with increased troponin level was observed whether patients had underlying advanced heart failure or not. Subgroup analysis showed an increased mortality in patients aged < 50 years, non-diabetics and not on vasopressors. CONCLUSION: In a population with high prevalence of diabetes and hypertension, elevated troponin-I was frequently observed in medical-surgical critically ill patients, and showed a level-dependent association with hospital mortality.

Effect of Gender on Postoperative Morbidity and Mortality Outcomes: A Retrospective Cohort Study.

Full extent of gender differences on postoperative outcomes has never been studied on large scale, specifically postoperative complications. This study aims to assess the effect of gender on 30-day morbidity and mortality after major surgery. A retrospective cohort study was carried out using data of patients undergoing major surgeries from the American College of Surgeons' National Surgical Quality Improvement Program database between 2008 and 2011. Demographics, pre- and perioperative risk factors, as well as 30-day morbidities, both overall and specific, were reviewed. The 30-day mortality data were also assessed. Multivariate logistic regression analyses, basic (Adj1) and extended (Adj2), were used to assess the association between gender and outcomes. Out of 1,409,131 patients, 57.2 per cent were females. Females had lower prevalence of most system-specific risk variables. Overall morbidities were also lower in females versus males, even after adjustment for variables [total overall morbidity: ORadj2 = 0.9 (0.89-0.92), P < 0.0001] except in some cases such as after cardiac surgeries [ORadj2 = 1.29 (1.14-1.44), P < 0.0001] and vascular surgeries [ORadj2 = 1.14 (1.10-1.18), P < 0.0001], where overall morbidities of females were higher. Specific morbidities were also lower in females than in males in all types of complications except central nervous system-related postoperative complications [ORadj2 = 1.15 (1.08-1.22), P < 0.0001] and return to the operating room [ORadj2 = 1.06 (1.04-1.08), P < 0.0001]. The 30-day mortality rate for females was lower than males [ORadj2 = 0.99 (0.96-1.03), P = 0.94]. Female gender was associated with less perioperative morbidity and mortality versus males, but they did worse after cardiovascular procedures and had more central nervous system-related complications. These outcomes should be taken into consideration by surgeons and should be evaluated further in future studies.

The Effect of Preoperative Pneumonia on Postsurgical Mortality and Morbidity: A NSQIP Analysis.

BACKGROUND: Currently, only indirect evidence suggests that preoperative pneumonia is a significant risk factor for poor postsurgical outcomes. Although this relationship is clinically intuitive, this is the first study that aims to quantify the extent to which pneumonia impacts morbidity and mortality. The objective of this study was to determine the impact of preoperative pneumonia on 30-day mortality and morbidity among both elective and emergency surgical patients. METHODS: We conducted a retrospective cohort study using 2008-2012 data from the American College of Surgeons National Surgical Quality Improvement Program database. Patients with preoperative pneumonia were matched to controls without preoperative pneumonia. Patient demographics and postoperative outcomes were extracted from the database, including 30-day mortality, specific morbidities (wound, cardiac, respiratory, urinary, central nervous system, thromboembolism and sepsis), composite morbidity, number of blood transfusions and number of patients that returned to the OR. Mortality and composite morbidity were further stratified. RESULTS: We obtained data for 137,174 patients, of whom 6933 (0.50%) had preoperative pneumonia. Overall, 6111 were successfully matched to 24,444 patients with no pneumonia. Postoperative mortality and composite morbidity were both higher in patients with pneumonia than in those without pneumonia, with an odds ratio of 1.37 (95% CI 1.26-1.48) and 1.68 (95% CI 1.58-1.79), respectively. CONCLUSION: Preoperative pneumonia significantly increased the rate of postoperative morbidity and mortality across several surgical settings and patient groups. It is our recommendation that elective surgery be delayed until after the pneumonia resolves.

Preoperative INR and postoperative major bleeding and mortality: A retrospective cohort study.

Little research has been done on the current cut-off international normalized ratio (INR) value of 1.5 for patients undergoing surgery. The objectives of this study are to assess the association between INR and postoperative major bleeding and mortality in patients undergoing surgery and to identify an ideal pre-operative INR for surgical patients. We analyzed data from the American College of Surgeons' National Surgical Quality Improvement Program database between 2008 and 2011 (636,231 patients). The primary outcomes were major bleeding and mortality at 30 days postoperatively. Multivariate logistic regression analyses were carried out to assess these associations. Compared to an INR of <1, the adjusted odds ratio (aOR) for major bleeding was 1.22 (95 % CI 1.18-1.25) for INR 1-1.49, 1.48 (95 % CI 1.40-1.56) for INR 1.5-1.9, and 1.49 (95 % CI 1.39-1.60) for INR ≥2. The aOR for mortality at 30 days post-operation compared to INR of <1 was 1.51 (95 % CI 1.41-1.62), 2.31 (95 % CI 2.12-2.52), and 2.81 (95 % CI 2.56-3.10) for INR 1-1.49, 1.5-1.9, and ≥2, respectively. The ideal pre-operative INR value to predict an increased risk for major bleeding was 1.10 and 1.13 for mortality. In conclusion, preoperative INR is significantly and independently associated with postoperative major bleeding and mortality.

Novel mutation causing partial biotinidase deficiency in a Syrian boy with infantile spasms and retardation.

We report a case of partial biotinidase deficiency (plasma biotinidase levels: 1.30 nm/minute/mL) in a 7-month-old boy who presented with evidence of perinatal distress followed by developmental delay, hypotonia, seizures, and infantile spasms without alopecia or dermatitis. His neurologic symptoms improved markedly on biotin supplementation and antiepileptic drug therapy. DNA mutational analysis revealed that the patient was homozygous for a novel E64K mutation and his parents were heterozygous for the same mutation. Whereas preexisting perinatal distress probably contributed to the severity of the patient's symptoms, the described mutation is novel and is possibly responsible for at least some of his clinical manifestations.