BACKGROUND Telomere syndromes have their most common manifestation in idiopathic pulmonary fibrosis and emphysema. The short telomere defect in these patients may manifest systemically as bone marrow ...failure and liver disease. We sought to understand the causes of dyspnea in telomerase and telomere gene mutation carriers who have no parenchymal lung disease. METHODS Clinical and pathologic data were reviewed as part of a Johns Hopkins-based natural history study of short telomere syndromes including dyskeratosis congenita. RESULTS Hepatopulmonary syndrome (HPS) was diagnosed in nine of 42 cases (21%). Their age at presentation was significantly younger than that of cases initially presenting with pulmonary fibrosis and emphysema (median, 25 years vs 55 years; P « .001). Cases had evidence of intra- and extrapulmonary arteriovascular malformations that caused shunt physiology. Nodular regenerative hyperplasia was the most frequent histopathologic abnormality, and it was seen in the absence of cirrhosis. Dyspnea and portal hypertension were progressive, and the median time to death or liver transplantation was 6 years (range, 4-10 years; n = 6). In cases that underwent liver transplantation, dyspnea and hypoxia improved, but pulmonary fibrosis subsequently developed. CONCLUSIONS This report identifies HPS as a frequent cause of dyspnea in telomerase and telomere gene mutation carriers. While it usually precedes the development of parenchymal lung disease, HPS may also co-occur with pulmonary fibrosis and emphysema. Recognizing this genetic diagnosis is critical for management, especially in the lung and liver transplantation setting.
Background Although sarcopenia has been identified as a predictor of poor, postoperative, clinical outcomes, the financial impact of sarcopenia remains undetermined. We sought to evaluate the ...relationship between sarcopenia and hospital finances among a cohort of patients undergoing a hepato-pancreatico-biliary or colorectal resection. Methods Clinical, financial, and morphometric data were collected for 1,169 patients undergoing operative resection between January 2011 and December 2013 at the Johns Hopkins Hospital. Multivariable regression analysis was performed to assess the relationship between sarcopenia and total hospital costs. Results Using sex-specific cutoffs for total psoas volume, 293 patients were categorized as sarcopenic. The presence of sarcopenia was associated with a $14,322 increase in the total hospital cost (median covariate-adjusted cost, sarcopenia versus no sarcopenia: $38,804 vs $24,482, P < .001). Patients who presented with sarcopenia demonstrated a higher total hospital cost within the subgroup of patients who developed a postoperative complication (sarcopenia versus no sarcopenia: $65,856 vs $59,609) and among those patients who did not develop a postoperative complication (sarcopenia versus no sarcopenia: $26,282 vs $23,763, both P < .001). Similarly, total hospital costs were higher among patients presenting with sarcopenia regardless of the length of stay for index admission (observed:expected, length of stay < 1: sarcopenia versus no sarcopenia: $25,038 vs $22,827; observed:expected, length of stay > 1: sarcopenia versus no sarcopenia: $43,283 vs $38,679, both P < .001). Conclusion As measured by sarcopenia, patient frailty is inversely related to total hospital costs. Sarcopenia represents a novel tool for forecasting patient outcomes and operative costs and can be used to inform quality improvement and cost containment strategies.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK, ZRSKP
Abstract Primary liver cancer is the sixth most common cancer overall and the second most common cause of cancer mortality worldwide. Hepatocellular carcinoma accounts for up to 90% of all primary ...hepatic malignancies and represents a major international health problem. While surgical resection and transplantation is the cornerstone of therapy in early-stage hepatocellular carcinoma, locoregional therapy and sorafenib are beneficial in those who are not surgical candidates or have more advanced disease. At times, both surgical therapy and locoregional therapy may be integrated. Hence, hepatocellular carcinoma requires a multidisciplinary approach to determine the most appropriate form of treatment as well as the timing of various treatments for optimal outcomes.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK, ZRSKP
Multidisciplinary care has been associated with improved survival in patients with primary liver cancers. We report the practice patterns and real world clinical outcomes for patients presenting to ...the Johns Hopkins Hospital (JHH) multidisciplinary liver clinic (MDLC). We analyzed hepatocellular carcinoma (HCC, n = 100) and biliary tract cancer (BTC, n = 76) patients evaluated at the JHH MDLC in 2019. We describe the conduct of the clinic, consensus decisions for patient management based on stage categories, and describe treatment approaches and outcomes based on these categories. We describe subclassification of BCLC stage C into 2 parts, and subclassification of cholangiocarcinoma into 4 stages. A treatment consensus was finalized on the day of MDLC for the majority of patients (89% in HCC, 87% in BTC), with high adherence to MDLC recommendations (91% in HCC, 100% in BTC). Among patients presenting for a second opinion regarding management, 28% of HCC and 31% of BTC patients were given new therapeutic recommendations. For HCC patients, at a median follow up of 11.7 months (0.7-19.4 months), median OS was not reached in BCLC A and B patients. In BTC patients, at a median follow up of 14.2 months (0.9-21.1 months) the median OS was not reached in patients with resectable or borderline resectable disease, and was 11.9 months in patients with unresectable or metastatic disease. Coordinated expert multidisciplinary care is feasible for primary liver cancers with high adherence to recommendations and a change in treatment for a sizeable minority of patients.
...the authors combined patients who met the TFC (>=4 diagnostic points) with patients who had only 3 diagnostic points. Structural abnormalities in children with ARVD/C are often mild, and involve ...focal subtricuspid dyskinesia with preserved global function rather than severe RV enlargement.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
To compare liver cancer resectability rates before and during the COVID-19 pandemic.
Liver cancers usually present with nonspecific symptoms or are diagnosed through screening programs for at-risk ...patients, and early detection can improve patient outcomes. In 2020, the COVID-19 pandemic upended medical care across all specialties, but whether the pandemic was associated with delays in liver cancer diagnosis is not known.
We performed a retrospective review of all patients evaluated at the Johns Hopkins Multidisciplinary Liver Cancer Clinic from January 2019 to June 2021 with a new diagnosis of suspected or confirmed hepatocellular carcinoma (HCC) or biliary tract cancer (BTC).
There were 456 liver cancer patients (258 HCC and 198 BTC). From January 2019 to March 2020 (pre-pandemic), the surgical resectability rate was 20%. The subsequent 6 months (early pandemic), the resectability rate decreased to 11%. Afterward from October 2020 to June 2021 (late pandemic), the resectability rate increased to 27%. The resectability rate early pandemic was significantly lower than that for pre-pandemic and later pandemic combined (11% lower; 95% confidence interval CI, 2%-20%). There was no significant difference in resectability rates pre-pandemic and later pandemic (7% difference; 95% CI, -3% to 16%). In subgroup analyses, the early pandemic was associated with a larger impact in BTC resectability rates than HCC resectability rates. Time from BTC symptom onset until Multidisciplinary Liver Clinic evaluation increased by over 6 weeks early pandemic versus pre-pandemic (Hazard Ratio, 0.63; 95% CI, 0.44-0.91).
During the early COVID-19 pandemic, we observed a drop in the percentage of patients presenting with curable liver cancers. This may reflect delays in liver cancer diagnosis and contribute to excess mortality related to the COVID-19 pandemic.
Abstract Background Incomplete penetrance and variable expressivity of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) complicate family screening. Objectives The objective of the ...present study was to determine the optimal approach to longitudinal follow-up regarding: 1) screening interval; and 2) testing strategy in at-risk relatives of ARVD/C patients. Methods We included 117 relatives (45% male, age 33.3 ± 16.3 years) from 64 families who were at risk of developing ARVD/C by virtue of their familial predisposition (72% mutation carriers 92% plakophilin-2; 28% first-degree relatives of a mutation-negative proband). Subjects were evaluated by electrocardiography (ECG), Holter monitoring, signal-averaged ECG, and cardiac magnetic resonance (CMR). Disease progression was defined as the development of a new criterion by the 2010 Task Force Criteria (not the “Hamid criteria”) at last follow-up that was absent at enrollment. Results At first evaluation, 43 subjects (37%) fulfilled an ARVD/C diagnosis according to the 2010 Task Force Criteria. Among the remaining 74 subjects (63%), 11 of 37 (30%) with complete re-evaluation experienced disease progression during 4.1 ± 2.3 years of follow-up. Electrical progression (n = 10 27%, including by ECG 14%, Holter monitoring 11%, or signal-averaged ECG 14%) was more frequently observed than structural progression (n = 1 3% on CMR). All 5 patients (14%) with clinical ARVD/C diagnosis at last follow-up had an abnormal ECG or Holter monitor recording, and the only patient with an abnormal CMR already had an abnormal ECG at enrollment. Conclusions Over a mean follow-up of 4 years, our study showed that: 1) almost one-third of at-risk relatives have electrical progression; 2) structural progression is rare; and 3) electrical abnormalities precede detectable structural changes. This information could be valuable in determining family screening protocols.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
Background Obese patients may present with metabolic abnormalities that impact liver regeneration. We sought to assess the impact of body mass index (BMI) on liver volume regeneration index (RI) and ...kinetic growth rate (KGR) among patients undergoing liver resection. Methods The study included 102 patients undergoing major hepatectomy (≥3 segments) between July 2004 and April 2015 and stratified the patients by preoperative BMI, number of segments resected, and postoperative remnant liver volume (RLVp ) to total liver volume ratio. Resected volume at operation was subtracted from total liver volume to calculate postoperative RLVp . RI was defined as the relative increase in RLV within 2 months (RLV2m –RLVp )/RLVp and 7 months (RLV7m –RLVp )/RLVp postoperatively; KGR was calculated as RI divided by time postoperatively (weeks). Results Median patient age was 59.6 years (interquartile range 48.1–68.7 years), and most patients were men (52.0%). Liver failure was associated with the KGR at 2 months (KGR2m ) and was greater among patients with KGR2m <2.5% per week (KGR <2.5%, 18.5% vs KGR ≥ 2.5%, 4.6%; P = .04). Although RI and KGR within 2 and 7 months postoperatively were similar among all patients, after excluding patients with fibrosis, obese (0.42% per week) and overweight patients (0.29% per week) had lesser KGR2–7m compared with patients of normal BMI (0.82% per week; P < .05). Additionally, risk of a major complication was greatest among obese patients (normal weight, 8.1% vs overweight, 12.9% vs obese, 29.4%; P = .04). Conclusion BMI did not impact liver regeneration during the first 2 months. In contrast, KGR per week between 2 and 7 months postoperatively was less among overweight and obese patients.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK, ZRSKP
The ACR Incidental Findings Committee (IFC) presents recommendations for managing pancreatic cysts that are incidentally detected on CT or MRI. These recommendations represent an update from the ...pancreatic component of the JACR 2010 white paper on managing incidental findings in the adrenal glands, kidneys, liver, and pancreas. The Pancreas Subcommittee-which included abdominal radiologists, a gastroenterologist, and a pancreatic surgeon-developed this algorithm. The recommendations draw from published evidence and expert opinion, and were finalized by informal iterative consensus. Algorithm branches successively categorize pancreatic cysts based on patient characteristics and imaging features. They terminate with an ascertainment of benignity and/or indolence (sufficient to discontinue follow-up), or a management recommendation. The algorithm addresses most, but not all, pathologies and clinical scenarios. Our goal is to improve quality of care by providing guidance on how to manage incidentally detected pancreatic cysts.
Objectives The aim of this study was to identify the incremental value and optimal role of cardiac magnetic resonance (CMR) imaging in arrhythmic risk stratification of arrhythmogenic right ...ventricular dysplasia/cardiomyopathy (ARVD/C)–associated desmosomal mutation carriers without histories of sustained ventricular arrhythmia. Background Risk stratification of ARVD/C mutation carriers is challenging. Methods Sixty-nine patients (mean age 27.0 ± 15.3 years, 42% men) harboring ARVD/C-associated pathogenic mutations (83% plakophilin 2) without prior sustained ventricular arrhythmias were included. Electrocardiographic and 24-h Holter monitoring findings closest to presentation were analyzed for electrical abnormalities per revised task force criteria. CMR studies were done to identify abnormal cardiac structure and function according to the revised task force criteria. Results Overall, 42 patients (61%) presented with electrical abnormalities on the basis of electrocardiography and Holter monitoring, of whom 20 (48%) had abnormal results on CMR. Only 1 of 27 patients (4%) without electrical abnormalities at initial evaluation had abnormal CMR results. Over a mean follow-up period of 5.8 ± 4.4 years, 11 patients (16%) experienced sustained ventricular arrhythmias, exclusively in patients with both electrical abnormalities (electrocardiography and/or Holter monitoring) and abnormal CMR results. Conclusions These results suggest that electrical abnormalities on electrocardiography and Holter monitoring precede detectable structural abnormalities in ARVD/C mutation carriers. Therefore, evaluation of cardiac structure and function using CMR is probably not necessary in the absence of baseline electrical abnormalities. Among ARVD/C mutation carriers, the presence of both electrical and CMR abnormalities identifies patients at high risk for events and thus patients who might benefit from prophylactic implantable cardioverter-defibrillator placement.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
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