Diagnostic Criteria Research Articles

Managing crawling-type gastric adenocarcinoma with endoscopic techniques and postoperative monitoring

Crawling-type gastric adenocarcinoma is a rare subtype of gastric cancer with diagnostic and therapeutic challenges due to its flat, ill-defined lesions. Advanced diagnostic techniques, such as narrow-band imaging and linear endoscopic ultrasonography, improve detection, but endoscopic submucosal dissection poses a risk of incomplete resection. Despite negative resection margins, vigilant postoperative monitoring is crucial due to the potential for recurrence. This letter highlights the importance of refined diagnostic criteria, individualized treatment approaches, and continuous follow-up to optimize management of this unique gastric cancer subtype.

Expert consensus on diagnosis and treatment of spontaneous intracranial hypotension(2024)

Spontaneous intracranial hypotension is a disease characterized by orthostatic headache caused by spinal cerebrospinal fluid leakage. Patients have to remain bedridden, which significantly affects their quality of life. In severe cases, complications such as subdural hematomas may occur. Clinical practice reveals a high rate of misdiagnosis and insufficient implementation of diagnostic and treatment measures. To improve clinical physicians' understanding of spontaneous intracranial hypotension and to standardize the diagnosis and treatment, the Neurologist Branch of the Chinese Medical Doctor Association and the Headache and Sensory Disorders Committee of the Chinese Research Hospital Association organized experts to develop a total of 30 recommendations on the clinical identification, examination processes, diagnostic criteria, treatment, and follow-up of spontaneous intracranial hypotension based on domestic and international clinical practices and recent literature evidence after extensive discussions.

Diagnosis and Treatment of Polycythemia Vera

ImportancePolycythemia vera (PV), a myeloproliferative neoplasm characterized by an increased red blood cell mass and increased risk of thrombosis, affects approximately 65 000 people in the US, with an annual incidence of 0.5 to 4.0 cases per 100 000 persons.ObservationsErythrocytosis (hemoglobin >16.5 mg/dL in men or >16.0 mg/dL in women) is a required diagnostic criterion, although thrombocytosis (53%) and leukocytosis (49%) are common. Patients may have pruritus (33%), erythromelalgia (5.3%), transient visual changes (14%), and splenomegaly (36%) with abdominal discomfort. More than 95% of patients have a JAK2 gene variant, which helps distinguish PV from secondary causes of erythrocytosis, such as tobacco smoking or sleep apnea. Among 7 cohorts (1545 individuals), the median survival from diagnosis was 14.1 to 27.6 years. Prior to or at the time of PV diagnosis, arterial thrombosis occurred in 16% of patients and 7% had venous thrombotic events, which could involve unusual sites, such as splanchnic veins. PV is also associated with an increased bleeding risk, especially in patients with acquired von Willebrand disease, which can occur with extreme thrombocytosis (platelet count, ≥1000 × 109/L). All patients with PV should receive therapeutic phlebotomy (goal hematocrit, <45%) and low-dose aspirin (if no contraindications). Patients who are at higher risk of thrombosis include those aged 60 years or older or with a prior thrombosis. These patients and those with persistent PV symptoms may benefit from cytoreductive therapy with hydroxyurea or interferon to lower thrombosis risk and decrease symptoms. Ruxolitinib is a Janus kinase inhibitor that can alleviate pruritus and decrease splenomegaly in patients who are intolerant of or resistant to hydroxyurea. About 12.7% of patients with PV develop myelofibrosis and 6.8% develop acute myeloid leukemia.Conclusions and RelevancePV is a myeloproliferative neoplasm characterized by erythrocytosis and is almost universally associated with a JAK2 gene variant. PV is associated with an increased risk of arterial and venous thrombosis, hemorrhage, myelofibrosis, and acute myeloid leukemia. To decrease the risk of thrombosis, all patients with PV should be treated with aspirin and therapeutic phlebotomy to maintain a hematocrit of less than 45%. Cytoreductive therapies, such as hydroxyurea or interferon, are recommended for patients at high risk of thrombosis.

Clinical Presentation, Investigation Findings, and Outcomes of IgG4-Related Pachymeningitis

ImportanceImmunoglobulin G4 (IgG4)–related disease is an increasingly recognized fibroinflammatory condition that can involve multiple organs, including the pachymeninges. The understanding of IgG4-related pachymeningitis (IgG4-RP) remains limited because of its rarity and the predominance of knowledge derived from case reports and case series.ObjectiveTo systematically review and synthesize the clinical presentation, investigation findings, and prognosis of IgG4-RP to better understand its diagnosis and management.Evidence ReviewA comprehensive systematic review was conducted following guidelines from the Preferred Reporting Items for Systematic Reviews and Meta-analyses. PubMed/MEDLINE, Embase, and Scopus were searched from their inception until May 30, 2023, using terms related to IgG4-related disease and pachymeningitis without language or publication restrictions. Case reports and series that met the 2020 Revised Comprehensive Diagnostic Criteria or the 2019 American College of Rheumatology/European League Against Rheumatism classification criteria were included. Data on clinical presentations, investigation findings, and treatment outcomes were extracted and summarized.FindingsA total of 148 case reports contributed data from 208 patients. Their median (IQR) age was 52 (39-62) years; 132 patients were male (63.5%) and 76 female (36.5%). Headache and cranial nerve dysfunctions were the most common neurological manifestations. Systemic involvement was identified in nearly half of the patients. Diagnostic imaging often showed preferential involvement of cavernous sinus and middle fossa. Laboratory results highlighted elevated serum IgG4 levels in 97 of 147 patients (65%) of patients and cerebrospinal fluid pleocytosis in 43 of 82 patients (52%). Storiform fibrosis or obliterating phlebitis were uncommon pathological findings. Mortality was below 1% (1/134; 0.7%), but only a third of patients presented complete clinical improvement, and the recurrence rate was 60 patients (40%) in a median (IQR) follow-up time of 9 (1-20) months. Glucocorticoids were the most commonly prescribed treatment, in 143 of 169 patients (85%); rituximab was prescribed as maintenance therapy in 53 of 169 patients (31%).Conclusions and RelevanceIgG4-RP commonly presents with headaches and cranial nerve dysfunction, posing diagnostic challenges due to the significant absence of systemic manifestations, low IgG4 serum levels, and atypical pathological findings. Current treatment outcomes are limited by incomplete recovery and frequent relapses underscoring the necessity for new treatment strategies.

Neuroradiological Findings in Cerebral Amyloid Angiopathy with a Particular Consideration of the Boston Criteria 2.0: An Imaging Review

In the elderly, cerebral amyloid angiopathy (CAA) is the most common cause for intracranial lobar hemorrhages. CAA is caused by the accumulation of amyloid-β fibrils in cortical and leptomeningeal vessels. In 2022, the Boston Criteria 2.0 became the new diagnostic standard for CAA, following the Modified Boston Criteria of 2010. The diagnostic criteria are a composite of clinical, imaging and histopathological findings. In the latest version of the Boston Criteria, neuroradiological imaging findings were even expanded compared to the previous version. Crucially, the correct application of the diagnostic criteria is necessary to avoid over- and underdiagnosis. The aim of this review is to demonstrate the diagnostic criteria for CAA with an emphasis on typical imaging findings which are part of the Boston Criteria 2.0 and other imaging findings suggestive of CAA.

Advanced treatment-resistant ovarian germ cell tumor (yolk sac tumor) with successful fertility realization 20 years after combined treatment: a case report

Background. Yolk sac tumors (YST) are classified as rare malignant germ cell tumors of the ovaries, most commonly found in young patients interested in preserving fertility. YST is characterized by large size, predominantly unilateral involvement, and increased production of alpha-fetoprotein, which can be used as an important diagnostic criterion for this condition. Among all germ cell tumors, YST has the least favorable prognosis. The primary goals of YST treatment are not only to improve prognosis and quality of life but also to enable the patient to fulfill her reproductive plans in the future.Description of the clinical case. We present a clinical case of an aggressive course of yolk sac tumor in the ovary of a 15-year-old girl, requiring multiple surgeries, repeated courses of high-dose drug therapy, and bone marrow transplantation.Conclusion. This is a rare case of YST with successful preservation of reproductive function due to modern medical technologies despite aggressive treatment.

Comparative analysis of image quality and diagnostic performance among SS-EPI, MS-EPI, and rFOV DWI in bladder cancer.

To compare image quality and diagnostic performance among SS-EPI diffusion weighted imaging (DWI), multi-shot (MS) EPI DWI, and reduced field-of-view (rFOV) DWI for muscle-invasive bladder cancer (MIBC). This retrospective study included 73 patients with bladder cancer who underwent multiparametric MRI in our referral center between August 2020 and February 2023. Qualitative image assessment was performed in 73; and quantitative assessment was performed in 66 patients with maximum lesion diameter > 10mm. The diagnostic performance of the imaging finding of muscle invasion was evaluated in 47 patients with pathological confirmation of MIBC. T2-weighted imaging, SS-EPI DWI, MS-EPI DWI, rFOV DWI, and dynamic contrast-enhanced imaging were acquired with 3T-MRI. Qualitative image assessment was performed by three readers who rated anatomical distortion, clarity of bladder wall, and lesion conspicuity using a four-point scale. Quantitative assessment included calculation of SNR and CNR, and grading of the presence of muscle layer invasion according to the VI-RADS diagnostic criteria. Wilcoxon matched pairs signed rank test was used to compare qualitative and quantitative image quality. McNemar test and receiver-operating characteristic analysis were used to compare diagnostic performance. Anatomical distortion was less in MS-EPI DWI, rFOV DWI, and SS-EPI DWI, in that order with significant difference. Clarity of bladder wall was greater for MS-EPI DWI, SS-EPI DWI, and rFOV DWI, in that order. There were significant differences between any two combinations of the three DWI types, except between SS-EPI DWI and MS-EPI in Reader 1. Lesion conspicuity, diagnostic performance, SNR and CNR were not significantly different among the three DWI types. Among the three DWI sequences evaluated, MS-EPI DWI showed the least anatomical distortion and superior bladder wall delineation but no improvement in diagnostic performance for MIBC. MS-EPI DWI may be considered for additional imaging if SS-EPI DWI is of poor quality.

The Possible Role of Rosuvastatin Therapy in HFpEF Patients—A Preliminary Report

Background: An increasing number of heart failure with preserved ejection fraction (HFpEF) syndromes has been reported in tandem with increasing age and burdens of obesity and cardiometabolic disorders. Identifying possible risk and modulatory HFpEF factors has significant epidemiological and clinical value. This study aimed to assess the prevalence of echocardiographic diagnostic criteria of left ventricular dysfunction in patients with chronic coronary syndrome depending on rosuvastatin therapy. Method: There were 81 (33 (41%) male) consecutive patients with a median age of 70 (62–75) years, presenting with stable heart failure symptoms according to the New York Heart Association (NYHA) classification I to III. They presented with chronic coronary syndrome and were hospitalized between March and August 2024. Patients were divided according to the type of long-term lipid-lowering therapy into patients with rosuvastatin and with other statin therapy. The echocardiographic analysis based on diastolic dysfunction evaluation was performed on admission and compared with demographical, clinical, and laboratory results. Results: In the multivariable model for diastolic dysfunction prediction in the analyzed group based on three echocardiographic parameters, septal E’ below 7 cm/s, lateral E’ below 10 cm/s, and LAVI above 34 mL/m2, the following factors were found to be significant: sex (male) (OR: 0.19, 95% CI: 0.04–0.83, p = 0.027), obesity (defined as BMI > 30) (OR: 12.78, 95% CI: 2.19–74.50, p = 0.005), and rosuvastatin therapy (OR: 0.09, 95% CI: 0.02–0.51, p = 0.007). Conclusions: Rosuvastatin therapy can be regarded as a possible protective therapy against left ventricular diastolic dysfunction in chronic coronary syndrome.

Accuracy and Performance Characteristics of Administrative Codes for the Diagnosis of Autoinflammatory Syndromes

Objective The aim of this study was to evaluate and validate the accuracy and performance characteristics of administrative codes in diagnosing autoinflammatory syndromes (AISs). Methods We identified potential AIS patients from the electronic medical records at the University of Iowa Hospital and Clinics and the Stead Family Children's Hospital using a screening filter based on the 10th edition of the International Classification of Diseases (ICD-10) codes and interleukin-1 antagonists. Diagnostic criteria for adult-onset Still disease, systemic juvenile idiopathic arthritis, Behçet disease (BD), familial Mediterranean fever (FMF), cryopyrin-associated periodic syndrome (CAPS), and SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome and chronic nonbacterial osteomyelitis (SAPHO-CNO) were reviewed for each patient. Patients who did not meet the diagnostic criteria were categorized as non-AIS. In this cross-sectional study, we calculated the sensitivity, specificity, positive predictive value, negative predictive value, and area under the receiver operating characteristic curve for the ICD codes in diagnosing AIS. Results Out of the 502 patients with potential AIS, 338 patients (67%) had a true AIS diagnosis. Sensitivity ranged from 80% (SAPHO-CNO) to 100% (BD and FMF), and positive predictive value ranged from 15% (FMF) to 80% (SAPHO-CNO). Specificity ranged from 81% (FMF) to 99% (CAPS and SAPHO-CNO), whereas negative predictive value ranged from 98% (adult-onset Still disease) to 100% (systemic juvenile idiopathic arthritis, BD, FMF, and CAPS). All ICD codes or code combinations for the diagnosis of specific AIS subtypes showed high accuracy with areas under the receiver operating characteristic curve ≥0.89. Conclusions This study validated the accuracy of administrative codes for diagnosing AIS, supporting their use in constructing AIS cohorts for clinical outcomes research.

An overview of invasive micropapillary carcinoma of the breast: past, present, and future

Invasive micropapillary carcinoma of the breast (IMPC) exhibits a unique micropapillary structure and “inside-out” growth pattern. Despite its extremely low incidence, IMPC has attracted considerable attention owing to its poor prognosis. Since Siriaunkgul and Tavassoli first proposed the term IMPC in 1993 to describe its morphological characteristics, with tumor cell clusters arranged in a pseudopapillary structure within the glandular cavity, its diagnostic rate has substantially increased. Based on the in-depth study of IMPC, a more comprehensive understanding of its epidemiology, clinicopathological features, and diagnostic criteria has been achieved in recent years. The pathogenesis and specific therapeutic targets of IMPC remain unclear. However, numerous studies have delved into its high-risk biological behavior. This review discusses the opportunities and challenges associated with IMPC.

Renovascular hypertension - a primer for the radiologist.

Renovascular hypertension (RVHT) is an important cause of secondary hypertension, accounting for approximately 75% of cases. This pictorial review describes the imaging modalities used to diagnose RVHT, including ultrasound, computed tomography angiography, and magnetic resonance angiography, provides their benefits and limitations, and explores the imaging findings, diagnostic criteria, and management of multiple causes of RVHT. Atherosclerosis is the most common cause of RVHT, particularly in older individuals, while fibromuscular dysplasia is more prevalent in younger females. Less common discussed etiologies include polyarteritis nodosa, extrinsic compression, Page kidney, dissection, renal artery (RA) thrombus, and RA aneurysms. This paper also highlights the importance of recognizing anatomical variants and rare conditions that can impact diagnosis and management. While RVHT represents a minority of hypertension cases, its potential reversibility makes accurate diagnosis crucial. Treatment approaches vary based on etiology and may include medical management, angioplasty, stenting, or surgical intervention. This review aims to enhance radiologists' understanding of RVHT, facilitating improved diagnosis and patient care.

Evaluation of the minor clinical features of Hanifin and Rajka amongst children with atopic dermatitis, visiting a tertiary care hospital in North Eastern India

Atopic Dermatitis (AD) is a commonly encountered, extremely itchy, chronic and relapsing skin disorder, which is characterized by inflammation in the skin. It typically has its onset in childhood, presenting with eczematous lesions; and can also be associated with personal or family history of “atopy”, usually most often. The criterion put by Hanifin and Rajka, encompassing 4 major plus 23 minor criteria, is not infrequently used as a diagnostic criterion for this disease in a hospital setup; however, the frequency of occurrence of the minor criteria usually varies in the population.The study aims at estimating the frequency of the minor diagnostic criteria of the Hanifin and Rajka in children with AD. This was a hospital-based cross-sectional study, that was conducted on 70 children (less than 18yrs of age) by taking their history, doing clinical and ophthalmological examination and some basic tests.The most common occurrence amongst the minor criteria estimated from our study was xerosis i.e., in 45(64.2%). The other common minor criteria observed were pityriasis alba (42.8%) facial erythema (40%), orbital darkening (37.14%) Early age of onset was seen in children (35.7%). There is variation in the clinical features of AD according to geography and population. The prevalence and severity of the disease are dependent on several factors like ethnicity/race and environment. Hence, it is important for dermatologists to know the usual and common ways the disease presents, in a given population for the diagnosis so as to diagnose and treat the patients at an earlier stage and before significant discomfort.

Relevance of Atopic Dermatitis Diagnostic Criteria in Context to Indian Paediatric Population: A Cross-sectional Study

Relevance of Atopic Dermatitis Diagnostic Criteria in Context to Indian Paediatric Population: A Cross-sectional Study

Overlap syndrome of hereditary hemorrhagic telangiectasia and juvenile polyposis syndrome: ten years follow-up-case series and review of literature.

Hereditary Hemorrhagic Telangiectasia (HHT) is an autosomal vascular dysplasia characterized by the presence of mucocutaneous telangiectasia and arteriovenous malformations in solid organs. The Curaçao criteria and/or detection of ALK1, ENG, and SMAD4 gene mutations are used for diagnosis. Juvenile Polyposis Syndrome (JPS) is diagnosed according to the number and localization of juvenile polyps, and family history of JPS. Both entities have a low prevalence. Mutation of SMAD4 leads to a combined syndrome of these two conditions called HHT-JPS Overlap Syndrome. We aim to describe the clinical characteristics associated with this condition focusing on long term follow up and review of the literature. A cross-sectional descriptive study of HHT-JPS cases from an HHT Institutional Registry was designed. Patients were eligible for this case series if they fulfilled both HHT and JPS diagnostic criteria and/or mutation on SMAD4. A comprehensive review was conducted on the clinical phenotype associated with HHT and its gastrointestinal involvement. Fourteen patients from eleven families in 788 previously HHT-diagnosed patients met the inclusion criteria. The ages ranged between 25 and 70 years old and 12 were females. In addition to the typical signs/symptoms of HHT, two distinct phenotypes were observed. Nine patients predominantly exhibited initially upper, while five showed predominantly initially lower gastrointestinal involvement. Numerous musculoskeletal and cardiovascular anomalies were also identified. The observed phenotypic diversity, particularly in gastrointestinal involvement, underscores the need for tailored clinical approaches. Comprehensive assessments identified associated musculoskeletal and cardiovascular anomalies, emphasizing the systemic nature of HHT-JPS.

The association of limited or painful spinal range of motion with lumbar disc degeneration: the back complaints in older adults (BACE) study.

We currently lack clear definitions for structural and symptomatic spinal osteoarthritis (OA). To define spinal OA and create diagnostic criteria for this condition, it is necessary to determine the relationship between clinical signs and symptoms of back pain and radiographic features of OA. Notably, recent studies suggest a defining sign of spinal OA could be a limited or painful spinal range of motion (ROM). Therefore, our objective was to assess the association between restricted or painful active spinal ROM, and multilevel structural features of lumbar disc degeneration (LDD) (i.e., disc space narrowing, osteophytes) on radiographs. We used the baseline data from the'Back Complaints in Older Adults' (BACE) study. The association between a limited or painful active spinal ROM, and multilevel disc space narrowing or osteophytes was assessed using multivariable logistic regression adjusted for age, mean pain intensity in the previous week, sex, BMI, and the Fear Avoidance Belief Questionnaire score. Primary analyses were performed on imputed datasets. We included 675 patients with a mean age of 66.52years (SD 7.69). Limited latero-flexion (fingertip that at best reached halfway up the upper leg) was associated with increased odds of having multilevel osteophytes (OR 1.85; 95% CI: 1.13 to 3.01). However, a limited or painful ROM in other directions (i.e., rotation, ante-flexion) was not associated with multilevel osteophytes, and a limited or painful active ROM in any direction was found to have no association with multilevel disc space narrowing. Our study indicates that a restricted ROM in latero-flexion could potentially be a defining sign in future definitions of symptomatic spinal OA. However, since our results partially differ from previous studies, further research is needed to explore the association between active ROM and LDD.

The Clinical Significance of Interstitial Pneumonia with Autoimmune Features in Cryptogenic Organizing Pneumonia: A Prospective Multicenter Observational Study

Background: There are cases of idiopathic interstitial pneumonias (IIPs) that do not meet the diagnostic criteria for connective tissue disease but have clinical features suggestive of autoimmune process. Interstitial pneumonia with autoimmune features (IPAF) was recently proposed as a research concept for these patients. Although several prospective studies on IPAF have been conducted, its clinical significance in cryptogenic organizing pneumonia (COP) remains unclear. Methods: Patients aged ≥20 years with suspected COP were prospectively enrolled between June 2018 and December 2022. Among the enrolled patients, those diagnosed with COP based on computed tomography (CT) and bronchoalveolar lavage (BAL) findings were compared between the IPAF and non-IPAF groups. Results: A total of 56 patients were enrolled in this study. Of these, 30 were diagnosed with COP and included in the analysis. Clinical and serological features were positive in two and six patients, respectively. Each feature was exclusive, and eight patients (26.7%) were diagnosed with IPAF. There were no differences between the IPAF and non-IPAF groups in terms of clinical features, including BAL findings, laboratory data, CT findings, and clinical course. During the one-year follow-up period, the frequency of COP exacerbation did not differ between the IPAF and non-IPAF groups, and no cases of systemic autoimmune disease or death occurred in either group. Conclusions: The COP characteristics of the IPAF and non-IPAF groups are similar in all aspects, and distinguishing between the two groups may be of little significance.

Clinicopathologic feature and treatment progress of high-grade ovarian neuroendocrine tumors.

High-grade Ovarian neuroendocrine tumors represent a rare subset of ovarian neoplasms characterized by aggressive behavior, poor prognosis, and early metastasis. Despite their clinical significance, the management of these tumors lacks consensus due to their low incidence. This comprehensive review encompasses literature spanning from 1991 to 2024, focusing on the clinical presentation, diagnostic criteria, differential diagnosis, prognostic indicators, treatment modalities, and recent advancements in the understanding of this condition. Notably, a substantial proportion of affected individuals present during the perimenopausal period with unilateral lesions displaying mixed histological components. Biomarkers such as CA125, CA199, and NSE hold promise for aiding in the diagnosis and screening of ovarian neuroendocrine tumors. Unfortunately, patients exhibit a dismal prognosis even diagnosed at an early stage. Primary treatment strategies predominantly involve surgical intervention coupled with etoposide-cisplatin combination chemotherapy. In cases of recurrence, second-line chemotherapeutic agents including paclitaxel, irinotecan, and doxorubicin are commonly employed alongside localized radiotherapy. While specific genetic mutations remain elusive, emerging evidence suggests potential therapeutic effect involving mTOR inhibitors, PD-1 monoclonal antibodies, and antiangiogenic agents based on isolated case reports. The exploration of representative set of mutations will help for precise targeted therapies and remains a focal point of our ongoing research efforts.

Managing Multiorgan Failure in Acute on Chronic Liver Failure.

Acute-on-chronic liver failure (ACLF) is defined as a clinical syndrome that develops in patients with chronic liver disease characterized by the presence of organ failure and high short-term mortality, although there is still no worldwide consensus on diagnostic criteria. Management of ACLF is mainly based on treatment of "precipitating factors" (the most common are infections, alcohol-associated hepatitis, hepatitis B flare, and bleeding) and support of organ failure, which often requires admission to the intensive care unit. Liver transplantation should be considered in patients with ACLF grades 2 to 3 as a potentially life-saving treatment. When a transplant is not indicated, palliative care should be considered after 3 to 7 days of full organ support in patients with at least four organ failures or a CLIF-C ACLF score of >70. This review summarizes the current knowledge on the management of organ failure in patients with ACLF, focusing on recent advances.

Sclerosing mucoepidermoid carcinoma of salivary glands.

Sclerosing mucoepidermoid carcinoma (SMEC) of the salivary glands is a rare variant of low-grade mucoepidermoid carcinoma with scanty cellular atypia characterized by marked fibrosis/sclerosis and a rich inflammatory infiltrate. Herein, we report 25 unpublished cases of SMEC, two of them with prominent eosinophilia (2/25; 8%) and three with abundant IgG4-positive plasma cells (3/25; 12%). In our series of salivary SMEC, molecular analysis using fluorescence in situ hybridization (FISH) and/or next-generation sequencing (NGS) provided evidence of MAML2 gene rearrangement in 18 cases of the 21 analyzable cases tested (86%), while this gene locus was intact in 3 cases (14%). This study focuses on the diagnostic criteria of salivary SMEC given its challenge of abundant collagenous stroma, minimal residual neoplastic areas, and inconspicuous mucous cells. Follow-up data of our cases indicate that salivary SMECs have favorable outcomes. Molecular analysis for MAML2 gene rearrangement suggests that SMECs of salivary glands represent a rare variant of conventional low-grade MECs of salivary glands. In contrast, SMECs of the thyroid gland are genetically distinct from salivary-type thyroid MECs.

Expert consensus for pertussis in children: new concepts in diagnosis and treatment.

Pertussis resurgence has been reported worldwide in the past two decades. Pertussis is still endemic and difficult to control though with universal vaccination in children. The resurgence may be related to multiple variables, such as increased disease awareness and laboratory tests, waning of immunity following vaccination, and/or genetic mutations of Bordetella pertussis. For better pertussis prevention, diagnosis, and management, we called up an expert panel to develop this expert consensus to provide new concepts in diagnosis and treatment for clinical practice. The expert groups collected clinical evidence, summarized their clinical experiences, evaluated preliminary recommendations or guidelines, and then organized open-ended discussions to form the recommendations. This consensus was developed by reviewing the literature and studies in databases, including PubMed, Cochrane, EMBASE, the China Biomedical Database, and the Chinese Journal Full-text Database up to May 2024. The search terms included "pertussis" or "whooping cough", "children", "diagnosis", and "treatment". The burden of pertussis has also changed from infants to schoolchildren and adults, and these age groups have consequently become the main source of infection for vulnerable population including infants and newborns. In China, a high prevalence of erythromycin-resistant Bordetella pertussis (ERBP) has been reported in the past decade. ERBP may lead to failed clinical empirical treatment with macrolides, which poses a great challenge for pertussis management and control. For better management of pertussis, a flow diagram for diagnosis and treatment of pertussis was presented in this consensus. This consensus also described the diagnostic criteria for pertussis, high-risk cases, and severe pertussis. Macrolides can still be used to treat confirmed erythromycin-sensitive B. pertussis (ESBP) infections, whereas oral trimethoprim-sulfamethoxazole therapy is the initial treatment option for children older than twomonths. For infants younger than twomonths, severe patients, or those exhibiting a high degree of sulfonamide allergy, intravenous administration of piperacillin or cefoperazone-sulbactam is advised. This expert consensus provides a comprehensive guidance and a reference for the diagnosis and treatment of pertussis in children.