Etiological Groups Research Articles

Clinical, etiological profile and outcomes of convulsive refractory and non-refractory status epilepticus at a tertiary care centre

Introduction: Epidemiological data on childhood status epilepticus (SE) limited in India. This study depicts clinical profiles, etiology, and outcomes of convulsive refractory SE (RSE) and compares the results with cases of nonrefractory SE (NRSE). Subjects and Methods: This observational, cross-sectional study enrolled 300 children aged 1 month to 18 years, presenting with convulsive SE at a tertiary care hospital. Details of children who progressed to RSE were compared to those without RSE. Results: In the acute symptomatic etiology group, common causes for RSE were central nervous system infections (33.8%), cryptogenic etiology (13.8%), and remote symptomatic etiology (7%). RSE iwas associated with a higher rate of complications (58% Vs 24%) as compared to NRSE. Thirty percent of cases that progressed to RSE needed ventilator support as compared to only 8% of cases in the NRSE group. RSE was also associated with higher rates of shock (25%), AKI (16%), transaminitis (25%), acute liver failure (5.69%), multiple organ dysfunction score (12.6%), and acidosis (53%) as compared to NRSE. RSE was associated with higher mortality (28%) and morbidity (32%) as compared to NRSE cases. Cryptogenic etiology led to the highest mortality (33.3%) in cases of RSE while acute symptomology was associated with the highest morbidity (62%). Conclusions: RSE is a serious pediatric emergency that requires prompt recognition and management. Clinical knowledge and early administration of appropriate antiepileptic drugs at health-care facilities is the key to reduce morbidity and mortality.

Influence of confirmed viral infection on adult acute fulminant myocarditis supported with extracorporeal membrane oxygenation.

The impact of etiologies of acute fulminant myocarditis (AFM), which requires extracorporeal membrane oxygenation (ECMO), on clinical outcomes remains unknown. This study aimed to investigate the risk factors for ECMO weaning and mortality among patients with AFM due to viral etiologies in a tertiary referral medical center. We included 33 adults with AFM who received ECMO and were admitted between January 2002 and January 2021. General demographics, laboratory data, echocardiography findings, and long-term outcomes were analyzed for confirmed viral etiology and unconfirmed etiology groups. The overall hospital survival rate was 54.5%. The age, sex, severity of the hemodynamic condition, and cardiac rhythm were similar between the two groups. Multivariate Cox regression analysis revealed that a confirmed viral etiology (HR 4.201, 95% CI 1.061-16.666), peri-ECMO renal replacement therapy (RRT) (HR 9.804, 1.140-83.333) and a high positive end-expiratory pressure (PEEP) in the ventilator settings at 24 h after ECMO (HR 1.479, 1.020-2.143) were significant prognostic factors for in-hospital mortality. Peri-ECMO RRT was also a significant negative prognostic factor for successful ECMO weaning (OR 0.061, 0.006-0.600) in the multivariate logistic model. Among AFM patients receiving ECMO support, RRT use was associated with a decreased chance of survival to ECMO weaning. Multiple organ dysfunction and a high PEEP were also predictive of a lower chance of hospital survival. Those with a confirmed diagnosis of viral myocarditis may require more medical attention due to the higher risk of hospital mortality than those without a definite diagnosis.

Lengthening Behavior of Magnetically Controlled Growing Rods in Early-Onset Scoliosis: A Multicenter Study.

The "law of diminishing returns" is described for traditional growing rods. Magnetically controlled growing rods (MCGRs) have become a preferred implant for the surgical treatment of early-onset scoliosis (EOS). We examined a large cohort of patients with EOS to determine whether the law of diminishing returns applies to MCGRs. A prospectively collected, multicenter registry was queried for patients with EOS treated with MCGRs. Patients with only spine-based implants and a minimum of 2 years of follow-up were included; patients with congenital scoliosis, single rods, <3 lengthenings, or >25% missing data were excluded. Patients were analyzed in 3 cohorts: primary MCGR (pMCGR) had first-time MCGR implants, secondary MCGR (sMCGR) were converted from an MCGR to a new MCGR, and conversion MCGR (cMCGR) were converted from a non-MCGR implant to MCGR. A total of 189 patients in the pMCGR group, 44 in the cMCGR group, and 41 in the sMCGR group were analyzed. From post-MCGR placement to the most recent follow-up or pre-definitive procedure, there were no differences in the changes in major Cobb angle, T1-S1 height, or T1-T12 height over time between the pMCGR and cMCGR groups. There was a decrease in length achieved at subsequent lengthenings in all cohorts (p < 0.01), and the sMCGR group had a significantly poorer ability to lengthen at each subsequent lengthening versus the pMCGR and cMCGR groups (p < 0.02). The 1-year survival rate was 90.5% for pMCGR, 84.1% for sMCGR, and 76.4% for cMCGR; 2-year survival was 61.5%, 54.4%, and 41.4%, respectively; and 3-year survival was 37.6%, 36.7%, and 26.9%, respectively. Excluding MCGRs still expanding, 27.6% of pMCGRs, 8.8% of sMCGRs, and 17.1% of cMCGRs reached the maximum excursion. Overall, 21.7% reached the maximum excursion. Within the pMCGR cohort, idiopathic and neuromuscular etiologies had a decline in lengthening achieved over time (p < 0.001), while syndromic EOS demonstrated a preserved ability to lengthen over time (p = 0.51). When the etiological groups were compared with each other, the neuromuscular group had the least ability to lengthen over time (p = 0.001 versus syndromic, p = 0.02 versus idiopathic). The MCGR experiences the law of diminishing returns in patients with EOS. We found that only 21.7% of rods expanded to within 80% of the maximum excursion. Therapeutic Level III . See Instructions for Authors for a complete description of levels of evidence.

An evaluation of patients who present to the emergency department with dizziness

This study aimed to analyze the data of patients presenting with dizziness at our hospital’s emergency department (ED) by determining the etiology of dizziness and to contribute to the medical data of our country and the literature. This prospective study was conducted with 116 volunteering patients who presented to the Emergency Department of Afyon Kocatepe University, Faculty of Medicine for one year with dizziness complaints. After the anamnesis, physical examination, diagnostic tests (blood tests, Dix-Hallpike maneuver, audiological-vestibular tests, carotid-vertebral artery color Doppler ultrasonography, and computerized brain tomography), and psychiatric interrogation, the patients were divided into four etiology groups: Peripheral, central, psychogenic, and other causes of dizziness. Clinical features were compared between peripheral and central dizziness groups. A total of 116 (3.4%) of patient admissions to the emergency department complained of dizziness. Forty-nine (42.2%) of these patients were male, 67 (57.8%) were female. In the cohort, 33.9% were under 40, and dizziness increased with older age. Seventy (60.3%) patients had no formal education or were primary school graduates. The initial evaluation at the ED revealed that dizziness mostly shifted with the position (71.6%) and was mainly accompanied by headache (67.2%). Based on their diagnosis, 50 patients (43.1%) were in the peripheral group, 30 patients (25.8%) were in the central group, 22 patients (19%) were in the psychogenic dizziness group, and 14 patients (12%) were in the other causes group. Intergroup analysis between peripheral and central vertigo groups identified that the hearing loss, ear fullness, recent upper respiratory tract infection (URTI), nausea, vomiting, and shifting position was statistically significant (p&lt;0.05). Dizziness is one of the common complaints of admission to the ED and may arise from different etiologies. Our study demonstrated that psychogenic dizziness was common in this patient cohort.

Clinical outcomes among distinct groups of patients with severe tricuspid valve regurgitation

Abstract Introduction Severe tricuspid valve regurgitation (TR) is associated with increased 1-year morbidity and mortality. Characterization by valve etiology (primary, secondary, and lead-associated), a classification borrowed from mitral valve disease, has not been universally shown to correlate with outcomes. Purpose Among a large, racially diverse cohort with newly identified severe TR, we aimed to 1) characterize outcomes of severe TR by etiology, and 2) assess whether unsupervised phenoclustering or supervised outcome-driven prediction trees were more effective in establishing subgroups of TR with differential clinical risk profiles. Methods We retrospectively analyzed outcomes of 5-year all-cause death and a composite of death or heart failure hospitalization (HFH) among adult patients with new severe TR identified by echocardiography between 2007 to 2018 at a large academic tertiary referral center in the United States. Patients were initially categorized by etiology of TR, including primary, secondary, and lead-associated. Second, we separately applied unsupervised hierarchical clustering to identify distinct clusters using demographics, clinical, and echo data at the time of diagnosis. Third, we applied a supervised recursive partitioning algorithm (survival trees) by each outcome to identify distinct TR subgroups. We estimated the cumulative incidence of death and composite death or HFH over 5 years by 1) etiology of TR, 2) distinct clusters, and 3) groups identified by supervised learning (prediction trees). Results Among 2,379 consecutive patients with newly identified severe TR, the median age was 70 years, 61% were female, and 40% were Black. Event rates (95% CI) were 30.9 (29.0 to 32.8) events/100 PY for death and 49.0 (45.9 to 52.2) events/100 PY for composite death or HFH over median follow-up of 1.6 years. Event rates were similarly high across TR etiology groups for both death and composite death or HFH (Figure 1). Multiple methods of unsupervised clustering did not yield distinct clusters by patient demographic and imaging characteristics. After applying supervised survival tree modeling, four phenoclusters with distinct clinical prognoses were separately identified for death and composite death or HFH (Figure 2). Variables identified to partition the cohort to discriminate both death and composite death or HFH were age, albumin, blood urea nitrogen, right ventricular contractility, and right ventricular systolic pressure (all p&amp;lt;0.05). Conclusions Five-year cumulative incidence of adverse events among patients with newly diagnosed severe TR was 69% for death and 80% for composite death or HFH. TR etiology did not stratify prognosis, while supervised survival tree models identified phenoclusters with distinct clinical risk. The identified subgroups of severe TR with differential outcomes offer insights towards enrichment in clinical trials of TR and risk/benefit analysis in patients undergoing TR therapies. Funding Acknowledgement Type of funding sources: Private company. Main funding source(s): Abbott

Short- and Mid-Term Outcomes of Stenting in Patients with Isolated Distal Internal Carotid Artery Stenosis or Post-Surgical Restenosis.

The aim was to evaluate the outcome of stenting in patients with isolated distal internal carotid artery (ICA) stenosis or post-surgical restenosis, as no data are currently available in the literature. Sixty-six patients (men, N = 53; median age: 66 [IQR, 61–73] years) with ≥50% distal ICA (re)stenosis were included in this single-center retrospective study. The narrowest part of the (re)stenosis was at least 20 mm from the bifurcation in all patients. Patients were divided into two etiological groups, atherosclerotic (AS, N = 40) and post-surgical restenotic (RES, N = 26). Postprocedural neurological events were observed in two patients (5%) in the AS group and in two patients (7.7%) in the RES group. The median follow-up time was 40 (IQR, 18–86) months. Three patients (7.5%) in the AS group had an in-stent restenosis (ISR) ≥ 50%, but none in the RES group. Three patients (7.5%) in the AS group and seven patients (26.9%) in the RES group died. None of the deaths in the RES group were directly related to stenting itself. The early neurological complication rate of stenting due to distal ICA (re)stenoses is acceptable. However, the mid-term mortality rate of stenting for distal ICA post-surgical restenoses is high, indicating the vulnerability of this subgroup.

Heart Rate and Mortality After Resuscitation in Patients With Out-of-Hospital Cardiac Arrest - Insights From the SOS-KANTO Registry.

Heart rate (HR) predicts outcomes in patients with acute coronary syndrome (ACS), whereas the impact of HR on outcomes after out-of-hospital cardiac arrest (OHCA) remains unclear. This study aimed to investigate the impact of HR after resuscitation on outcomes after OHCA and whether the impact differs with OHCA etiology. Of 16,452 patients suffering from OHCA, this study analyzed 741 adults for whom HR after resuscitation was recorded by 12-lead electrocardiogram upon hospital arrival. Etiology of OHCA was categorized into 3 groups: ACS, non-ACS, and non-cardiac. Patients in each etiology group were further divided into tachycardia (>100 beats/min) and non-tachycardia (≤100 beats/min). The impact of HR on outcomes was evaluated in each group. Among the 741 patients, the mean age was 67.6 years and 497 (67.1%) patients were male. The primary outcome - 3-month all-cause mortality - was observed in 55.8% of patients. Tachycardia after resuscitation in patients with ACS was significantly associated with higher all-cause mortality at 3 months (P=0.002), but there was no significant association between tachycardia and mortality in non-ACS and non-cardiac etiology patients. In a multivariate analysis model, the incidence of tachycardia after resuscitation independently predicted higher 3-month all-cause mortality in OHCA patients with ACS (hazard ratio: 2.17 [95% confidence interval: 1.05-4.48], P=0.04). Increased HR after resuscitation was associated with higher mortality only in patients with ACS.

Extreme Hyperferritinemia: Causes and Prognosis.

The significance of extreme hyperferritinemia and its association with certain diagnoses and prognoses are not well characterized. We performed a retrospective analysis of adult patients with at least one total serum ferritin (TSF) measurement ≥ 5000 µg/L over 2 years, in three university hospitals. Conditions associated with hyperferritinemia were collected, and patients were classified into 10 etiological groups. Intensive care unit (ICU) transfer and mortality rates were recorded. A total of 495 patients were identified, of which 56% had a TSF level between 5000 and 10,000 µg/L. There were multiple underlying causes in 81% of the patients. The most common causes were infections (38%), hemophagocytic lymphohistiocytosis (HLH, 18%), and acute hepatitis (14%). For TSF levels > 10,000 µg/L, there were no solid cancer or hematological malignancy without another cause of hyperferritinemia. Isolated iron-overload syndromes never exceeded TSF levels > 15,000 µg/L. Extreme hyperferritinemia (TSF levels > 25,000 µg/L) was associated with only four causes: HLH, infections, acute hepatitis and cytokine release syndromes. A total of 32% of patients were transferred to an ICU, and 28% died. Both ICU transfer rate and mortality were statistically associated with ferritin levels. An optimized threshold of 13,405 μg/L was the best predictor for the diagnosis of HLH, with a sensitivity of 76.4% and a specificity of 79.3%. Hyperferritinemia reflects a variety of conditions, but only four causes are associated with extreme hyperferritinemia, in which HLH and acute hepatitis are the most common. Extreme hyperferritinemia has a poor prognosis with increased mortality.

A comprehensive evaluation of pre- and post-processing sperm parameters for predicting successful pregnancy rate following intrauterine insemination with the husband’s sperms

BackgroundTo determine the predictive values of sperm parameters pre- and post-processing by density gradient centrifugation for clinical pregnancy rates (CPRs) following artificial insemination by husband (AIH) in infertile Chinese couples.MethodsA total of 3,522 AIH cycles from 1,918 couples were retrospectively analyzed. The parameters were compared between the pregnant and non-pregnant groups and further between different etiological groups (Male-factor, Both-male-and-female-factor, and Other-factor). Multivariate logistic regression analysis was performed to create models for predicting the CPRs of each etiological group.ResultsThe overall CPR was 13.3%. There were significant improvements for most sperm parameters after DGC. Multivariate logistic regression analysis indicated that, in overall AIH cases, the top parameters significantly influencing the CPR of AIH were pre-STR (OR = 1.037; P = 0.048) and post-VSL (OR = 1.036; P = 0.011). In the Male-factor Group, the top influencing parameters were pre-VCL (OR = 2.096; P = 0.008), pre-LIN (OR = 1.930; P = 0.002) and post-VSL (OR = 1.316; P = 0.023). In the Both-factor Group, the top influencing parameters were pre-VCL (OR = 1.451; P = 0.008) and post-motility (OR = 1.218; P = 0.049). In the Other-factor Group, the top influencing parameters were pre-VAP (OR = 1.715; P = 0.024), pre-STR (OR = 1.20; P = 0.011) and post-VSL (OR = 1.04; P = 0.017). Moreover, receiver operating characteristic analysis showed that the logistic regression models of the Male- and Both-factor Groups had greater powers for prognostic classification than those of other groups.ConclusionsThis study demonstrated that some sperm parameters have a collinearity relationship in predicting the CPR following AIH. Moreover, the predictive capacity of a multivariate logistic regression model is better than those of individual parameters, especially for the Male- and Both-factor Groups. In these cases, pre-VCL is the common top influencing factor.

High reproducibility of spleen stiffness measurement by vibration-controlled transient elastography with a spleen-dedicated module.

Spleen stiffness measurement (SSM) by vibration‐controlled transient elastography (VCTE) is a noninvasive technique for estimating portal hypertension in patients with chronic liver disease (CLD), with its reproducibility yet to be established and its feasibility still unknown beyond CLD. We have studied 420 participants from two tertiary referral centers for liver diseases (Novara, Milan): 297 patients with CLD (32% with cirrhosis) of different etiology (Group A), 63 Philadelphia‐negative myeloproliferative neoplasms (Group B), and 60 heathy volunteers (Group C). All underwent SSM by VCTE with a spleen‐dedicated module (SSM@100 Hz) and liver stiffness measurement (LSM), blindly performed by 2 different operators. In total, 1680 VCTE examinations for SSM were performed (1000 in Novara, 680 in Milan), with an overall 3.2% failure rate. Median SSM was 26.5 kPa (interquartile range [IQR] 20.0–42.3) in Group A, 26.3 kPa (IQR 22.3–33.6) in Group B, and 16.1 kPa (IQR 14.6–18.7) in Group C. In Group A, the median LSM was 6.8 kPa (IQR 4.9–11.3) in Novara and 8.3 kPa (IQR 7.1–10.8) in Milan, the proportion of patients with cirrhosis being 34% in Novara and 31% in Milan. The Group A interobserver agreement ICC was 0.90 (0.88–0.92), significantly lower in the absence of splenomegaly (ICC 0.87 vs. 0.91) and in absence of cirrhosis (ICC 0.84 vs. 0.90); overweight slightly, but not significantly reduced the interobserveragreement. The intra‐observer agreement ICC ranged from 0.91 to 0.96 for the four operators. The Group B interobserver agreement ICC was 0.90 (0.83–0.94). In conclusion, SSM measured by the new spleen‐dedicated VCTE module is a feasible, reliable, and highly reproducible tool in patients with CLD and hematological disorders, and in healthy volunteers.

COMPARATIVE STUDY OF DISEASE SEVERITY, COURSE, COMPLICATIONS, AND CLINICAL OUTCOME BETWEEN ALCOHOL-INDUCED PANCREATITIS AND GALL STONE-INDUCED PANCREATITIS

Objective:To assess whether the etiological factors of alcohol and gall stones affect the disease severity, course, disease-associated complications, and clinical outcome in terms of length of hospital stay, infection/organ failure rates, intervention rates, and mortality. Materials and Method: We conducted a hospital-based prospective study in the Department of Radio-Diagnosis, Assam Medical College, where 75 cases with acute pancreatitis were screened and subdivided into three etiological groups: (i) alcoholism (33 cases), (ii) cholelithiasis (23 cases), and (iii) other causes (19 cases), out of which 56 cases with etiology of alcoholism and gall stones were included in our study. The severity was scored using the Modified CT Severity Index. Clinical follow-up was done for both alcohol-induced Pancreatitis and Gall stone-induced pancreatitis groups to assess the disease course, disease-associated complications&amp; clinical outcome. Statistical analysis: We analyzed data using the computer program Statistical Package for Social Sciences (SPSS for Windows, version 21.0. Chicago, SPSS Inc.) and Microsoft Excel 2010. Results: In a total of 75 cases screened for our study, alcohol-induced pancreatitis was the leading cause of acute pancreatitis (33 cases), followed by gall stone-induced pancreatitis (23 cases). Together, they accounted for 56 cases and constituted our study population. The majority of cases in our study were categorized under Moderate acute Pancreatitis in both alcohol-induced acute pancreatitis and gall stone-induced Pancreatitis as per the Modified CT Severity Index. The occurrence of ascites was more in alcohol-induced A.P. No significant difference was noted between the two groups in parameters such as peripancreatic or pancreatic fluid collection, necrosis, pleural effusion, extrapancreatic parenchymal abnormality, vascular complications &amp; G.I. complications. There was no statistically significant difference in clinical outcome between the two groups regarding the length of hospital stay, infection/organ failure evidence, need for intervention, and mortality. Conclusion: Our study found no significant difference between the two etiological groups regarding disease severity, course &amp; complications, and clinical outcome except for the increased occurrence of ascites in alcohol-induced pancreatitis.

Prevalence and clinical characteristics of autoimmune liver disease in hospitalized patients with cirrhosis and acute decompensation in China.

BACKGROUNDAutoimmune liver disease (AILD) has been considered a relatively uncommon disease in China, epidemiological data for AILD in patients with cirrhosis and acute decompensation (AD) is sparse.AIMTo investigate the prevalence, outcome and risk factors for AILD in cirrhotic patients complicated with AD in China.METHODSWe collected data from patients with cirrhosis and AD from two prospective, multicenter cohorts in hepatitis B virus endemic areas. Patients were regularly followed up at the end of 28-d, 90-d and 365-d, or until death or liver transplantation (LT). The primary outcome in this study was 90-d LT-free mortality. Acute-on-chronic liver failure (ACLF) was assessed on admission and during 28-d hospitalization, according to the diagnostic criteria of the European Association for the Study of the Liver (EASL). Risk factors for death were analyzed with logistic regression model.RESULTSIn patients with cirrhosis and AD, the overall prevalence of AILD was 9.3% (242/2597). Prevalence of ACLF was significantly lower in AILD cases (14%) than those with all etiology groups with cirrhosis and AD (22.8%) (P < 0.001). Among 242 enrolled AILD patients, the prevalence rates of primary biliary cirrhosis (PBC), autoimmune hepatitis (AIH) and PBC-AIH overlap syndrome (PBC/AIH) were 50.8%, 28.5% and 12.0%, respectively. In ACLF patients, the proportions of PBC, AIH and PBC/AIH were 41.2%, 29.4% and 20.6%. 28-d and 90-d mortality were 43.8% and 80.0% in AILD-related ACLF. The etiology of AILD had no significant impact on 28-d, 90-d or 365-d LT-free mortality in patients with cirrhosis and AD in both univariate and multivariate analysis. Total bilirubin (TB), hepatic encephalopathy (HE) and blood urea nitrogen (BUN) were independent risk factors for 90-d LT-free mortality in multivariate analysis. The development of ACLF during hospitalization only independently correlated to TB and international normalized ratio.CONCLUSIONAILD was not rare in hospitalized patients with cirrhosis and AD in China, among which PBC was the most common etiology. 90-d LT-free mortality were independently associated with TB, HE and BUN.

Clinical Correlation Between Pre and Post ERCP Laboratory Values

Abstract Background: Endoscopic retrograde cholangiopancreatography (ERCP) has evolved from a diagnostic modality to a primarily therapeutic procedure for pancreatic as well as biliary disorders. However, several complications were described post-procedure such as pancreatitis, perforation, cholangitis, post-sphincterotomy bleeding, etc. Data concerning variation in laboratory values before and after ERCP and its clinical significance with respect to endoscopic findings and possible complications is lacking in the literature. Aim: To analyze the clinical significance of laboratory values in patients before and after ERCP. Methods: From a total of 723 patients, 363 with different sets of findings on ERCP were eligible to be included in the study and were divided into 8 different groups. Serum levels of alanine aminotransferase (ALT), aspartate aminotransferase (AST), Gamma-glutamyl transferase (GGT), Alkaline phosphatase (ALKP), bilirubin, amylase, lipase, c-reactive protein (CRP), white blood count (WBC), neutrophil, lymphocyte, monocyte, eosinophils, basophils, platelets counts and creatinine were determined preoperatively as well as postoperatively in these patients. Results: AST and direct bilirubin showed a significant difference in all patients between pre and post-ERCP (p-value&lt;0.01 and p-value&lt;0.05, respectively). Liver tests were significantly higher in the malignant obstruction group than in the bile duct stones group (P &lt;0.05) and decrease more significantly (P &lt;0.05) after the procedure. A significant increase in lipase (p-value&lt;0.05) among all groups was found, and interestingly, the lymphocytic count showed a significant decrease (p-value&lt;0.01). Conclusion: In conclusion, (1) ERCP significantly decreases AST, direct bilirubin, lymphocytes, and monocytes count post procedure among all stratified groups of obstructive etiology thus proving its therapeutic value for biliary system obstructions. (2) Higher baseline disturbances in laboratory values at T0, especially in liver function tests such as ALT, AST, GGT, and ALKP as well as a bigger decrease in lymphocyte count at T1 are noted to be linked with malignant obstructions (tumor group), rather than benign obstructions (stone, sludge, stone+ sludge, and stricture). (3) Finally, stone and stricture groups are at the highest risk of post-ERCP pancreatitis owing to those groups having the highest pancreatic enzyme levels post ERCP, and thus should be the best candidates for a pre-ERCP pharmacologic prophylaxis (such as diclofenac, etc) and post ERCP close monitoring.

Cohort study of infantile epileptic spasms syndrome: etiological analysis and treatment of corticosteroids

BackgroundInfantile epileptic spasms syndrome (IESS) is the most common type of severe epilepsy in infants. However, etiological frequency and optimized therapy, particularly corticosteroid regimen and dose, remain unknown. MethodsAn ambispective study of an IESS-diagnosed cohort was conducted. Etiologies were evaluated based on the 2017 International League Against Epilepsy classification system. Patients received intravenous dexamethasone or methylprednisolone for 3–5 consecutive days, followed by usual-dose (2 mg/kg/d) oral prednisone for 60–90 days with tapering doses for 1–2 months or high-dose (4 mg/kg/d) oral prednisone for 9–11 days with tapering doses for 2–4 weeks. Treatment responses were compared between the usual and high-dose prednisone groups after propensity score matching. Correlation analysis between treatment responses and underlying etiology was performed. ResultsOf the 441 included participants, 218 (49.4%) cases had proven etiologies. The most common etiology of IESS was acquired-structural (23.6%), followed by genetic (15.4%) and congenital-structural (7.0%). Hypoxic-ischemic encephalopathy (55, 52.8%) was the most common acquired-structural etiology. Among the 242 patients administered corticosteroids, 95 received usual-dose oral prednisone and 147 received high-dose oral prednisone. After propensity score matching, 54 patients were included in the usual-dose and high-dose groups, respectively, and treatment effectiveness was compared. There were no significant differences in seizure freedom at days 13–14 (55.6% vs. 51.9%, p = 0.700) and continued seizure freedom between days 14–42 (29.6% vs. 38.9%, p = 0.311) post corticosteroid administration between the usual- and high-dose prednisone groups. The proportion of children achieving seizure cessation at days 13–14 (χ2 = 1.470, p = 0.698) and days 14–42 (χ2 = 0.928, p = 0.836) was similar in the different etiological subgroups. Unknown etiological group showed significantly higher resolution of hypsarrhythmia than other etiological groups (χ2 = 10.761, p = 0.009). Both usual-dose and high-dose group showed tolerance to full-dose corticosteroids and similar adverse events over the observation period. ConclusionIESS etiology was primarily related to structural causes. Clinical response in short-term follow-up was independent of prednisone dosage and underlying etiology. Better EEG responses may occur in patients with unknown etiology.

Acquired Penile Epidermoid Cysts in Children.

BackgroundIn this study, we aim to present the experience of a tertiary center regarding penile epidermoid cysts over 15 years.MethodologyPatient files of those who underwent surgical excision for penile epidermoid cysts between 2005 and 2019 were reviewed retrospectively. The demographics, clinical characteristics, etiological factors, cyst features, surgical techniques, complications, and follow-up data were analyzed.ResultsIn total, 24 penile epidermoid cysts were excised in 21 boys. The median age at the time of surgery was 52 (15-204) months. The median duration between previous surgery and cyst excision was 40 (1-180) months. In total, 11 cases had a history of circumcision, and 10 had undergone hypospadias surgery. There was no significant difference between these two etiologic groups (p > 0.05). The main symptom was an asymptomatic penile mass. The average cyst size was 9.4 ± 6.7 mm. All cysts were completely excised with incisions made over old scars, except one. No complications were observed during a median follow-up period of 50 (12-120) months, and only one recurrence was noted.ConclusionsAcquired penile epidermoid cysts may present as an early or late complication after penile surgery. Complete excision with an incision along the existing scars seems to be an effective solution for preventing new scars.

Acute Abdominal Complications in Deeply Neutropenic Onco-Hematology Patients: A Retrospective Series of 105 Cases.

Acute abdominal complications (AAC) in patients with deep neutropenia (DN) is challenging to manage because of the expected influence of AAC on oncological prognosis and higher surgical complication rate in a period of DN. In practice, these parameters are difficult to appreciate. This study reported our experience in managing these patients. All consecutive patients treated in our tertiary care cancer center between 2010 and 2020 who developed AAC in the context of a DN were retrospectively analyzed. AAC was defined as an infection (intra-abdominal, perineal, or cutaneous), bowel obstruction, or intra-abdominal hemorrhage. Among 105 patients, 18 (17%) required emergent surgery (group 1), 34 patients had a complication requiring surgical oversight (group 2), and 53 patients had a non-surgical etiology (group 3). Fifteen patients underwent surgery in the group 1, three in group 2, and one in group 3. Overall, 28 patients died during hospitalization. Mortality was statistically different between the groups (p = 0·01), with a higher rate in group 1 (n = 9/18, 50%) than in group 2 (n = 11/34, 32%) and group 3 (n = 8/53, 15%). All groups together had a median overall survival (OS) of 14months and disease-free survival (DFS) of 10months. OS was not comparable between the groups, and the median length of survival in group 1 was 6months versus 8months in group 2 and 23months in group 3. In group 1, five patients (5/18, 28%) did not relapse at the end of the follow-up compared to 13 in group 2 (13/34, 38%) and 25 in group 3 (25/53, 47%). After discharge, OS and DFS were similar between the groups. The advent of an AAC necessitating surgery in the context of DN is a deadly event associated with a 50% mortality; nonetheless, in case of unpostponable emergencies, surgery can provide long-term survival in selected patients.

Adult intussusception: still a challenging diagnosis for the surgeon

Introduction and aimIntussusception is rare in adults and can occur in the small bowel and colon. Its atypical presentation makes the diagnosis difficult. The aim of the present study was to evaluate the causes, clinical characteristics, and treatment outcomes of adult intussusception and to determine whether there was an association between etiology and clinical presentation. Materials and methodsA retrospective study was carried out on patients above 18 years of age that were treated for intussusception at a tertiary care hospital, between 2000 and 2020. The findings were summarized utilizing descriptive and inferential statistics. ResultsTwenty-eight cases were identified. Median patient age was 46 years (18-80) and median symptom duration was 18 days. Abdominal pain was the most frequent symptom (96.42%). The intussusceptions registered were enteroenteric (14), ileocecal (4), ileocolonic (4), colocolonic (5), and colorrectal (1). Intussusception etiology was benign in 15 cases, 9 were associated with malignancy, and 4 were idiopathic. Surgery was performed on 11 patients with enteroenteric intussusception and on all the cases of ileocecal, ileocolonic, colocolonic, and colorectal intussusception. There were 2 events of perioperative mortality (8%) and 8 of postoperative morbidity (32%). No significant differences were found regarding symptom duration or length of hospital stay, when the etiologic groups were compared. ConclusionsIntussusception is rare in adults. Diagnosis is a challenge because of the nonspecific signs and symptoms. Surgical resection should be considered in the definitive treatment and management should be individualized according to the patient’s comorbidities, clinical presentation, and risk of malignancy.

Gene expression adjustment of inflammatory mechanisms in dairy cow mammary gland parenchyma during host defense against staphylococci

Abstract The aim of the study was to identify differences in the expression of splice variants of the PRMT2, LTF and C4A genes in the mammary glands of healthy dairy cows and those infected with staphylococci. An expression study was conducted on 38 Polish Holstein-Friesian dairy cows who were removed from the herd owing to subclinical and chronic mastitic or reproductive issues. Two days before slaughter, milk samples were taken for microbiological analysis and examined for the presence of bacteria. The mammary gland parenchyma samples with a predominance of secretory tissue were taken; these were divided into three groups according to the health status of the mammary gland: H (without pathogenic bacteria in milk), CoNS (with coagulase-negative staphylococci in milk), and CoPS (with coagulase-positive staphylococci in milk). Two of the investigated genes, LTF and C4A, demonstrated variants unequivocally expressed in infected tissue. Two LTF gene variants were found to be associated with cow health status, and with the type of bacteria causing mastitis (CoPS or CoNS). In addition, the expression of C4A isoforms differed with regard to mastitis etiology groups. The comprehensive evaluation of PRMT2 transcript suggested that the gene may also be involved in course of mastitis: two of four PRMT2 transcripts showed increased expression in the mammary gland of the CoPS group compared to controls. The obtained results are important for the knowledge on the etiology of bovine mastitis. The effects of the identified mastitis-relevant splice variants need to be further explored on the protein level to verify the suitability of splice variants and recognize their contribution towards the disease phenotypes and course.

Comparative study of visual outcome of newly designed scleral tuck lens and suture-fixated lens for rehabilitation of aphakia in various aetiologies.

Purpose:Visual rehabilitation in aphakia can be performed using several modalities. However, these modalities could be either technically difficult or expensive. Herein, we developed a scleral tuck lens to provide a simple and cost-effective solution for aphakia and compared its outcome with standard methods.Methods:A specially designed posterior chamber self-sustaining lens was implanted in patients with aphakia without capsular support because of different primary etiologies. The visual outcomes, as well as intraoperative and postoperative complications, were examined. The data were retrieved from electronic medical records, and visual outcome and complication rates were compared. The outcomes were also compared according to the etiology and age groups (pediatric and adults).Results:We found significant improvement in preoperative and postoperative visual outcome. We did not find any significant difference in visual outcome amongst suture-supported scleral fixated lens with scleral tuck lens.Conclusion:Satisfactory visual outcomes were noted with minimal complications; and comparable with gold standard suture fixated lens, however long-term follow-up is required.

Intususcepción en adultos: todavía un reto diagnóstico para el cirujano

Introduction and aimsIntussusception is rare in adults and can occur in the small bowel and colon. Its atypical presentation makes the diagnosis difficult. The aim of the present study was to evaluate the causes, clinical characteristics, and treatment outcomes of adult intussusception and to determine whether there was an association between etiology and clinical presentation. Material and methodsA retrospective study was carried out on patients above 18 years of age that were treated for intussusception at a tertiary care hospital, between 2000 and 2020. The findings were summarized utilizing descriptive and inferential statistics. ResultsTwenty-eight cases were identified. Median patient age was 46 years (18-80) and median symptom duration was 18 days. Abdominal pain was the most frequent symptom (96.42%). The intussusceptions registered were enteroenteric (14), ileocecal (4), ileocolonic (4), colocolonic (5), and colorrectal (1). Intussusception etiology was benign in 15 cases, 9 were associated with malignancy, and 4 were idiopathic. Surgery was performed on 11 patients with enteroenteric intussusception and on all the cases of ileocecal, ileocolonic, colocolonic, and colorectal intussusception. There were 2 events of perioperative mortality (8%) and 8 of postoperative morbidity (32%). No significant differences were found regarding symptom duration or length of hospital stay, when the etiologic groups were compared. ConclusionsIntussusception is rare in adults. Diagnosis is a challenge because of the nonspecific signs and symptoms. Surgical resection should be considered in the definitive treatment and management should be individualized according to the patient's comorbidities, clinical presentation, and risk of malignancy.