scholarly journals Kikuchi-Fujimoto disease: unusual presentation of rare disease

2016 ◽  
Vol 6 (1) ◽  
Author(s):  
Duy Vu ◽  
Srini Reddy ◽  
Lynn Day ◽  
Nail Aydin ◽  
Subhasis Misra
Keyword(s):  
Lupus Erythematosus ◽  
Unusual Presentation ◽  
Chronic Infections ◽  
Young Females ◽  
Inappropriate Treatment ◽  
Increased Risk ◽  
Long Term Follow Up ◽  
Autoimmune Processes

Kikuchi-Fujimoto disease (KFD) is a rare, benign disorder that typically follows a selflimiting natural course and was initially described in young females of Asian descent. Its clinical presentation may mimic lymphoproliferative disorders, connective tissue disorders, and chronic infections. This often leads to misdiagnosis and inappropriate treatment. The exact cause of this condition remains unknown although autoimmune processes and certain infectious agents have been associated with the disease. The diagnosis of KFD is made histopathologically. Treatment is supportive and long-term follow-up is recommended due to increased risk of future development of systemic lupus erythematosus. Here we are presenting a case of a patient with an unusual presentation of KFD.

scholarly journals Damage accrual and mortality over long-term follow-up in 300 patients with systemic lupus erythematosus in a multi-ethnic British cohort

Rheumatology ◽  
2019 ◽  
Author(s):  
Beatriz Tejera Segura ◽  
Brett Sydney Bernstein ◽  
Thomas McDonnell ◽  
Chris Wincup ◽  
Vera M Ripoll ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
High Dose ◽  
Concomitant Disease ◽  
Systemic Lupus ◽  
Increased Risk ◽  
Damage Accrual ◽  
Long Term Follow Up

Abstract Objective Damage in patients with systemic lupus erythematosus is irreversible change in organs due to disease activity, concomitant disease or medication side-effects. It is measured using the Systemic Lupus International Collaborative Clinics Damage Index (SDI) and is associated with increased mortality. Previous reports have suggested associations between damage accrual and various ethnic, disease and treatment factors, but there is a dearth of long-term follow-up data from large multi-ethnic cohorts. We describe a study of damage and mortality in 300 patients from London, UK followed for up to 40 years. Methods We carried out retrospective analysis of medical records and SDI scores of 300 patients followed for up to 40 years (median 13.3 years). Characteristics of the groups who did and did not develop damage and those who died or survived to the end of follow-up were compared using univariable and multivariable analysis. Kaplan-Meier analysis was used to analyse factors affecting mortality and accrual of damage. Results Damage developed in 231/300 (77%) of patients. There was a linear accrual of damage over 40 years follow-up. Factors associated with damage were African/Caribbean ethnicity, renal and cerebral involvement, early use of high-dose corticosteroids or immunosuppressants, anti-RNP and antiphospholipid antibodies. Damage was strongly associated with mortality. Of 87 patients who died, 93% had damage compared with 70% of survivors (P < 0.001). Conclusion Development of damage is strongly associated with increased mortality. We identified groups at increased risk of developing damage, including those treated with high-dose steroids and immunosuppressants within the first two years.

scholarly journals Prognostic role of neoplastic markers in Takotsubo syndrome

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Francesco Santoro ◽  
Tecla Zimotti ◽  
Adriana Mallardi ◽  
Alessandra Leopizzi ◽  
Enrica Vitale ◽  
...  
Keyword(s):  
Serum Levels ◽  
Takotsubo Syndrome ◽  
Ca 15.3 ◽  
Risk Of Death ◽  
Increased Risk ◽  
Ca 19.9 ◽  
Long Term Follow Up

AbstractTakotsubo syndrome (TTS) is an acute heart failure syndrome with significant rates of in and out-of-hospital mayor cardiac adverse events (MACE). To evaluate the possible role of neoplastic biomarkers [CA-15.3, CA-19.9 and Carcinoembryonic Antigen (CEA)] as prognostic marker at short- and long-term follow-up in subjects with TTS. Ninety consecutive subjects with TTS were enrolled and followed for a median of 3 years. Circulating levels of CA-15.3, CA-19.9 and CEA were evaluated at admission, after 72 h and at discharge. Incidence of MACE during hospitalization and follow-up were recorded. Forty-three (46%) patients experienced MACE during hospitalization. These patients had increased admission levels of CEA (4.3 ± 6.2 vs. 2.2 ± 1.5 ng/mL, p = 0.03). CEA levels were higher in subjects with in-hospital MACE. At long term follow-up, CEA and CA-19.9 levels were associated with increased risk of death (log rank p < 0.01, HR = 5.3, 95% CI 1.9–14.8, HR = 7.8 95% CI 2.4–25.1, respectively, p < 0.01). At multivariable analysis levels higher than median of CEA, CA-19.9 or both were independent predictors of death at long term (Log-Rank p < 0.01). Having both CEA and CA-19.9 levels above median (> 2 ng/mL, > 8 UI/mL respectively) was associated with an increased risk of mortality of 11.8 (95% CI 2.6–52.5, p = 0.001) at follow up. Increased CEA and CA-19.9 serum levels are associated with higher risk of death at long-term follow up in patients with TTS. CEA serum levels are correlated with in-hospital MACE.

Atrial fibrillation associated with increased risk of venous thromboembolism

2015 ◽  
Vol 113 (01) ◽  
pp. 185-192 ◽  
Author(s):  
Chun-Cheng Wang ◽  
Cheng-Li Lin ◽  
Guei-Jane Wang ◽  
Chiz-Tzung Chang ◽  
Fung-Chang Sung ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
Venous Thromboembolism ◽  
Incidence Rates ◽  
Vein Thrombosis ◽  
Kaplan Meier ◽  
Increased Risk ◽  
Long Term Follow Up ◽  
Deep Vein

SummaryWhether atrial fibrillation (AF) is associated with an increased risk of venous thromboembolism (VTE) remains controversial. From Longitudinal Health Insurance Database 2000 (LHID2000), we identified 11,458 patients newly diagnosed with AF. The comparison group comprised 45,637 patients without AF. Both cohorts were followed up to measure the incidence of deep-vein thrombosis (DVT) and pulmonary embolism (PE). Univariable and multivariable competing-risks regression model and Kaplan-Meier analyses with the use of Aelon-Johansen estimator were used to measure the differences of cumulative incidences of DVT and PE, respectively. The overall incidence rates (per 1,000 person-years) of DVT and PE between the AF group and non-AF groups were 2.69 vs 1.12 (crude hazard ratio [HR] = 1.92; 95 % confidence interval [CI] = 1.54-2.39), 1.55 vs 0.46 (crude HR = 2.68; 95 % CI = 1.97-3.64), respectively. The baseline demographics indicated that the members of the AF group demonstrated a significantly older age and higher proportions of comorbidities than non-AF group. After adjusting for age, sex, and comorbidities, the risks of DVT and PE remained significantly elevated in the AF group compared with the non-AF group (adjusted HR = 1.74; 95 %CI = 1.36-2.24, adjusted HR = 2.18; 95 %CI = 1.51-3.15, respectively). The Kaplan-Meier curve with the use of Aelon-Johansen estimator indicated that the cumulative incidences of DVT and PE were both more significantly elevated in the AF group than in the non-AF group after a long-term follow-up period (p<0.01). In conclusion, the presence of AF is associated with increased risk of VTE after a long-term follow-up period.

scholarly journals Repetition of deliberate self-harm and subsequent suicide risk: Long-term follow-up study of 11 583 patients

2004 ◽  
Vol 185 (1) ◽  
pp. 70-75 ◽  
Author(s):  
Daniel Louis Zahl ◽  
Keith Hawton
Keyword(s):  
Relative Risk ◽  
Suicide Risk ◽  
Single Episode ◽  
Deliberate Self Harm ◽  
Young Females ◽  
Follow Up Study ◽  
Increased Risk ◽  
Self Harm

BackgroundRepetition of deliberate self-harm (DSH) is a risk factor for suicide. Little information is available on the risk for specific groups of people who deliberately harm themselves repeatedly.AimsTo investigate the long-term risk of suicide associated with repetition of DSH by gender, age and frequency of repetition.MethodA mortality follow-up study to the year 2000 was conducted on 11583 people who presented to the general hospital in Oxford between 1978 and 1997. Repetition of DSH was determined from reported episodes prior to the index episode and episodes presenting to the same hospital during the follow-up period. Deaths were identified through national registers.ResultsThirty-nine percent of patients repeated the DSH. They were at greater relative risk of suicide than the single-episode DSH group (2.24; 95% CI 1.77–2.84). The relative risk of suicide in the repeated DSH group compared with the single-episode DSH group was greater in females (3.5; 95% C11.3–2.4) than males (1.8; 95% C1 2.3–5.3) and was inversely related to age (up to 54 years). Suicide risk increased further with multiple repeat episodes of DSH in females.ConclusionsRepetition of DSH is associated with an increased risk of suicide in males and females. Repetition may be a better indicator of risk in females, especially young females.

A major health problem facing immigrant children: nutritional rickets

2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Erdal Kurnaz ◽  
Semra Çetinkaya ◽  
Selin Elmaoğulları ◽  
Aslıhan Araslı Yılmaz ◽  
Nursel Muratoğlu Şahin ◽  
...  
Keyword(s):  
Vitamin D ◽  
Immigrant Children ◽  
High Dose ◽  
Pediatric Endocrinology ◽  
Major Health Problem ◽  
Nutritional Rickets ◽  
Increased Risk ◽  
Long Term Follow Up

Abstract Objectives Nutritional rickets (NR) is still an important problem and one which increasing influxes of immigrants are further exacerbating. This study evaluated cases of mostly immigrant children followed up with diagnoses of NR in our pediatric endocrinology clinic. Methods Details of 20 cases diagnosed with NR between 2017 and 2020 were retrieved from file records. Results Twenty (11 male) cases were included in the study. Three (15%) were Turkish nationals and the others (85%) were immigrants. Hypocalcemia and hypophosphatemia were detected in 17 and 13, respectively. Alkaline phosphatase (ALP) values were normal in two cases, while ALP and parathyroid hormone (PTH) values were elevated in all other cases, and PTH levels were very high (473.64 ± 197.05 pg/mL). 25-hydroxyvitamin D levels were below 20 ng/mL in all cases. Patients with NR received high-dose long-term vitamin D or stoss therapy. Six patients failed to attend long-term follow-up, while PTH and ALP levels and clinical findings improved at long-term follow-up in the other 14 cases. Conclusions The elevated PTH levels suggest only the most severe cases of NR presented to our clinic. Clinically evident NR is therefore only the tip of the iceberg, and the true burden of subclinical rickets and osteomalacia remains unidentified. Public health policies should therefore focus on universal vitamin D supplementation and adequate dietary calcium provision, their integration into child surveillance programs, adequate advice and support to ensure normal nutrition, exposure to sunlight, and informing families of the increased risk not only for resident populations but also for refugee and immigrant children.

Abstract 15934: Higher Midlife Serum Levels of Kidney-injury Molecule-1 (KIM-1) are Associated With Incident Fatal CHD Over Very Long-term Follow-up Among Men

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Rachel H Mackey ◽  
Greg G Grandits ◽  
Lewis H Kuller ◽  
Joel Estis ◽  
John A Todd ◽  
...  
Keyword(s):  
Risk Factor ◽  
Limit Of Detection ◽  
Kidney Injury ◽  
Multiple Risk Factor ◽  
Serum Samples ◽  
Increased Risk ◽  
Long Term Follow Up ◽  
Kidney Injury Molecule 1

Introduction: Higher levels of kidney-injury molecule-1 (KIM-1) measured in urine are associated with presence and progression of acute renal disease. A recent study reported similar results for KIM-1 measured in blood. Hypothesis: We hypothesized that KIM-1 measured in stored serum from middle-aged men who participated in the Multiple Risk Factor Intervention Trial (MRFIT) would differentiate very long-term risk of fatal CHD vs. survival to a mean age of 80 over approximately 30 year follow-up. Methods: We conducted a nested case-control study within MRFIT, which in 1973-76 randomized 12,866 high risk but CVD free men ages 35-57 to risk factor intervention vs. usual care. Serum samples were collected at baseline and stored for future use. The trial concluded in 1982 but long-term mortality follow-up was ascertained through 2005 using the National Death Index. From MRFIT participants with stored serum from baseline, we sampled 100 men who died of CHD (mean age 47.3 at baseline and 73.9 at death), and 100 men who survived to 2005 (mean age =48.4 at baseline and 80.1 in 2005.) KIM-1 was assayed from stored serum samples using high sensitivity single-molecule counting technology (Erenna ® Immunoassay System, Singulex), with limit of detection (LoD)=0.5 pg/ml, and lower limit of quantification (LLoQ)=2.0 pg/ml. Results were compared between cases and controls using Wilcoxon rank tests and logistic regression. Results: Inter-assay %CVs were 8%. Median KIM-1 was higher for smokers vs. non-smokers and for men with vs. without hypertension, but was not associated with high cholesterol. KIM-1 was significantly higher in cases (183 pg/ml (IQR: 137-239) versus controls, (161 pg/ml (IQR:109-212), p=0.03; OR (95%CI)for Q4 versus Q1 was 2.26 (1.02 - 5.02) Adjusted for age and smoking the OR(95%CI) of fatal CHD for Q4 vs. Q1 was 2.34 (1.02- 5.37), and further adjusted for diastolic BP and serum cholesterol at baseline, was 2.0 (95% CI: 0.8-4.7). Conclusions: Higher serum KIM-1 levels at midlife were associated with a ∼2-fold increased risk of fatal CHD vs. survival over ∼30 years of follow-up. This is the first report of a longitudinal association of circulating KIM-1 levels with fatal CHD in long-term follow-up.

Abstract 13943: Hypertrophic Cardiomyopathy with Left Ventricular Apical Aneurysm Represents a High-Risk Subgroup Necessitating Aggressive Preventive Therapy: A Long-term Follow-Up Study

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Ethan J Rowin ◽  
Barry J Maron ◽  
Tammy S Haas ◽  
John R Lesser ◽  
Mark S Link ◽  
...  
Keyword(s):  
Heart Failure ◽  
Hypertrophic Cardiomyopathy ◽  
High Risk ◽  
Sudden Death ◽  
Left Ventricular ◽  
Increased Risk ◽  
Long Term Follow Up ◽  
Apical Aneurysm

Background: Increasing penetration of high spatial resolution cardiovascular magnetic resonance (CMR) imaging into routine cardiovascular practice has resulted in more frequent identification of a subset of hypertrophic cardiomyopathy (HCM) patients with thin-walled, scarred left ventricular (LV) apical aneurysms. Prior experience involved relatively small numbers of patients with short follow-up and therefore the risk associated with this subgroup remains incompletely defined. Therefore, we assembled a large HCM cohort with LV apical aneurysms and long-term follow-up in order to clarify clinical course and prognosis. Methods and Results: Of 2,400 HCM patients, 60 (2.5%) were identified by CMR with LV apical aneurysm, 24 to 86 years of age, including 19 (32%) <45 years old; 70% male, and followed for 5.6 ± 3.5 years. Over the follow-up period, 24 patients experienced 31 adverse disease-related complications including: appropriate implantable cardioverter-defibrillator discharge for VT/VF (n=11), received or listed for heart transplant (n=6), heart failure death (n=5), nonfatal thromboembolic events (n=4), resuscitated out-of-hospital cardiac arrest (n=3), and sudden death (n=2). In addition, an intracavitary thrombus was identified in the apical aneurysm in 9 patients without a thromboembolic history. Combined HCM-related death and aborted life threatening event rate was 8.6% per year, nearly 6-fold greater than the 1.5% annual mortality rate reported in the general HCM population. Conclusions: Patients with LV apical aneurysms represent a high-risk subgroup within the diverse HCM spectrum, associated with substantial increased risk for disease-related morbidity and mortality, including advanced heart failure, thromboembolic stroke and sudden death. Identification of this unique HCM phenotype should prompt consideration for primary prevention ICD, and anticoagulation for stroke prophylaxis.

Abstract 4784: The Ross Operation: Lessons From Long Term Follow-up

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Laurent de Kerchove ◽  
Jean Rubay ◽  
Agnès Pasquet ◽  
Alain Poncelet ◽  
Munir Boodhwani ◽  
...  
Keyword(s):  
Valve Replacement ◽  
Bleeding Event ◽  
Right Ventricular Outflow Tract ◽  
Aortic Insufficiency ◽  
Bleeding Events ◽  
Ross Operation ◽  
Increased Risk ◽  
Long Term Follow Up

Background: The Ross operation is considered the procedure of choice for aortic valve replacement in children and represents an attractive alternative in selected young adults. However, long term follow-up has demonstrated high rates of pulmonary autograft failure raising concern regarding its clinical utility. We analyze long term outcomes of our Ross series, focusing on predictors of autograft failure. Methods: Between 1991 and 2006, 260 consecutive patients underwent Ross operation. Mean age was 35±15 years (range: 1 day to 64 years) and 75% were male. The technique for autograft implantation was a full root replacement in 70% (n=183), inclusion cylinder in 28% (n=73) and subcoronary in 2% (n=4). Right ventricular outflow tract (RVOT) was reconstructed with a pulmonary homograft in 94% (n=245) and with a xenograft in 6% (n=15). Mean clinical follow-up (97% complete) was 97±46 months (13 to 196 months). Echocardiographic follow-up was available in 234 survivors (94%). Results: Hospital mortality was 1.9% (n=5) and 7 patients died during follow-up (3 non cardiac). Twelve year overall survival was 93±4% and freedom from autograft reoperation and from autograft valve replacement was 83±7% and 94±5% respectively. Twenty-five patients (9.6%) underwent autograft reoperation either for insufficiency (n=8), autograft or ascending aorta dilatation (n=6), or both (n=11) with no mortality. In 76% (n=19) of these patients, the autograft valve was preserved. Predictors for autograft dilatation (≥45 mm) were preoperative aortic insufficiency and full root technique. RVOT reintervention was required in 10 (4%) patients (reoperations n=7, balloon dilatation n=3). Twelve year freedom from RVOT reintervention was 95±4. Twelve year freedom from thrombo-embolic and bleeding events was 99±1% (1 stroke, 1 TIA, 1 bleeding event). Conclusions: This long terms study confirms the excellent survival after Ross operation with an extremely low rate of thrombo-embolic and bleeding events. Autograft reoperation rate remains acceptable. Full root technique is associated with increased risk of autograft dilatation, and should be avoided especially in adults with preoperative aortic insufficiency. Autograft valve preservation is feasible in autograft reoperation.

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