Unilateral Cervical Lymphadenopathy Research Articles

Scrotal and penile edema in a patient with incomplete kawasaki disease: a case report and brief literature review

BackgroundKawasaki disease (KD) is a medium artery vasculitis that predominantly affects children under age 5. Prompt diagnosis and treatment with IVIG and moderate dose aspirin is required to prevent the formation of coronary artery aneurysms. While scrotal edema and erythema have been seen in KD, here we present a distinctive case of incomplete Kawasaki with these features as well as penile edema.Case presentationA 2-year-old, unvaccinated, African American male presented with 4 days of fever, bilateral limbic sparing conjunctivitis, a papular rash, unilateral shotty cervical lymphadenopathy, mild right-hand edema, and scrotal and penile edema and erythema. His labs were significant for sterile pyuria, elevated ALT, anemia for age, and hypoalbuminemia. He was diagnosed with incomplete Kawasaki disease and was treated with IVIG and moderate dose aspirin. Echocardiogram was negative for coronary aneurysms. His symptoms resolved and he was discharged home with low dose aspirin. At his 2-week follow up, he remained well-appearing with no refractory Kawasaki symptoms.ConclusionThis is a unique case of penile edema in KD which to our knowledge has not been previously reported in literature. An understanding of genitourinary symptoms in Kawasaki disease can help timely diagnosis and treatment of the disease.

Whole Genome Sequence of a Treponema pallidum Strain From a Formalin-Fixed, Paraffin-Embedded Fine Needle Aspirate of a Cervical Lymph Node.

A patient with unilateral cervical lymphadenopathy suspicious for malignancy underwent a fine needle aspiration. Histology demonstrated mixed inflammatory infiltrates with abundant spirochetes. Sufficient spirochete DNA was extracted from paraffin-embedded tissue sections to obtain the near-complete genome sequence of a macrolide-resistant strain belonging to the SS14 omega strain of Treponema pallidum .

Neinfektivna unilateralna cervikalna limfadenopatija u djece - prikaz pacijenata

Cervical lymphadenopathy in children is often the reason to seek medical attention. According to the literature, 90% of children aged 4-9 years has cervical lymphadenopathy, mainly caused by acute infections, that is treated in the out-of-hospital setting. In a small, but significant number of cases, cervical lymphadenopathy is caused by malignancy and wide range of other clinical entities, that require more detailed clinical approach. Three patients with non-infectous unilateral cervical lymphadenopathy are presented; adolescent boy with unicentric Castleman disease, young adult with progressive transformation of germinal centers, and an infant with viral parotitis mistaken for cervical lymphadenopathy. Unicentric Castleman disease was diagnosed by pathohistological assesment of excised lymph node to a 14-year-old boy after 7 months of persistent cervical lymphadenopathy, and close follow-up was suggested as no disease progression was verified by PETCT. Histopathological-clinical diagnosis of progressive transformation of germinal centers was established in an adolescent with 3-month history of lymphadenopathy, after extensive radiological and infectious assesment. Chronic reccurent course of the disease was once again confirmed, as this patient awaits his third extirpation in the last year. Viral parotitis is one of the conditions that can mimic cervical lymphadenopathy as showed with the third presented patient – a 6-month-old male infant with the submandibular swelling lasting for three days. This article emphasises the importance of a detailed history taking and clinical assesment, but is also a reminder of a less often, noninfectious causes of lymphadenopathy in children, where systematic approach is the key to a correct diagnosis and treatment.

Clinicopathologic Analysis of Kikuchi-Fujimoto Disease and Etiologic Exploration Using Metagenomic Next-Generation Sequencing.

Kikuchi-Fujimoto lymphadenitis, also known as Kikuchi-Fujimoto disease (KFD), is a self-limited lymphoproliferative disease, with no definitive causative agent confirmed by traditional methods. To further explore the clinicopathologic features of KFD and clarify related pathogenic factors. A retrospective analysis was performed in a collection of KFD cases to review the clinical and histopathologic features, and metagenomic next-generation sequencing (mNGS) was used in 64 formalin-fixed, paraffin-embedded (FFPE) tissues from patients with KFD. One hundred five of the 170 patients with KFD (61.8%) were female; 10 patients had autoimmune diseases. Four pathologic subtypes were classified: necrotic (45.9%, 78 of 170), phagocytic (32.4%, 55 of 170), proliferative (17.1%, 29 of 170), and xanthomatous (4.7%, 8 of 170). Patients younger than 40 years with unilateral cervical lymphadenopathy and small vessel fibrinous degeneration accounted for significant differences among the 4 pathologic subtypes (P < .05). Among 64 patients with KFD, 9 had detectable bacterial or viral DNA-of 6 bacterial cases, 1 involved Chlamydia psittaci; while of 3 viral cases, 1 involved human beta herpesvirus 6B and 2 involved Epstein-Barr virus. No significant relationships were found between the pathologic subtypes and specific pathogens. Only a small proportion of patients with KFD had autoimmune diseases or infections from specific pathogens, suggesting that KFD is likely a reactive lesion of lymph nodes to various circumstances. To our knowledge, this is the first and the largest study to detect pathogens with the use of mNGS on FFPE samples in KFD. Our study also further confirms that mNGS can be used on FFPE samples to detect potentially infectious agents in clinical settings.

Kawasaki Disease Associated with Streptococcal Infection and Facial Nerve Palsy: A Case Report

ABSTRACT Background: Kawasaki disease (mucocutaneous lymph node syndrome), an acute febrile vasculitis of childhood that affects medium and small-sized arteries, is uncommonly reported in the West African sub-region. Its diagnosis relies on the presence of a constellation of clinical signs which could mimic or coexist with infectious viral or bacterial agents, thereby requiring a high index of suspicion. Case presentation: We report a two-year, ten months-old boy who presented with prolonged high-grade fever for over 3 three weeks; non-purulent conjunctivitis, unilateral cervical lymphadenopathy, cracked lips, reddish tongue, diffuse oedema, erythema of his palms and soles; skin desquamation over the tips of his digits and left-sided facial nerve palsy. He had leukocytosis, thrombocytopenia, and elevated inflammatory markers. Throat swab yielded Streptococcus pyogenes species; however, serial echocardiography was unrevealing. He was managed with aspirin, steroids, bed rest, and antibiotics (based on culture sensitivity), but intravenous immunoglobulin (IVIG) was not readily available and therefore was not administered. His inpatient clinical course showed fever persisting into the second week of inpatient management that gradually became undulating by the third week before lysing. Conclusion: The rare complication of facial nerve palsy is highlighted, while the evidence for acute bacterial infection posed further diagnostic challenges in this child with clinical features of Kawasaki disease. The unavailability of IVIG also portends a prolonged course for the acute stages, which are largely unresponsive to antipyretics and antibiotics. The importance of long-term follow-up for potential coronary artery aneurysms in the face of risk factors is further emphasized.

Blood routine risk factors for coronary artery aneurysm in infants younger than 8\u2009months with Kawasaki disease

ObjectiveThe aims of this study were to characterize the evolution of routine blood values within the first 10 days of illness and coronary artery outcome in infants < 8 months with Kawasaki disease (KD) and to identify risk factors for coronary artery aneurysm (CAA).MethodsLaboratory data, clinical features and coronary artery outcomes from 78 infants < 8 months old and 86 patients between 8 months and 7 years old were retrospectively analyzed. Logistic regression analysis was conducted to evaluate the potential risk factors for CAA.ResultsInfants < 8 months old were more likely to present with incomplete KD (37.2% vs 4.7%, P < 0.001), erythema and induration at the BCG inoculation site (24.4% vs 3.5%, P < 0.001) and CAA (47.4% vs 15.1%, P < 0.001) even with timely diagnosis and treatment with intravenous immunoglobulin (IVIG) compared with patients ≥8 months old. Clinical feature related to diagnostic criteria for KD including bilateral conjunctival injection, oral changes, unilateral cervical lymphadenopathy and extremity changes were less common in the younger group. During the acute phase, the percentage neutrophils and neutrophil to lymphocyte ratio [NLR] peaked on median illness day 3, followed by white blood cell (WBC) and CRP on median illness day 4, hemoglobin on median illness day 7 and platelet count on median illness day 9. CAA occurred on median illness day 6 and regressed on median illness day 28. Multivariate logistic regression analysis revealed that the peak percentage neutrophils (odds ratio [OR] per 0.1: 1.597, 95% confidence interval [CI]: 1.041–2.452, P = 0.032) and the peak platelet count (OR per 10 × 109/L: 1.029, 95% CI: 1.004–1.055, P = 0.024) were independent risk factors for CAA. Hemoglobin on the 5th day was associated with persistent CAA at 1 year after KD onset.ConclusionFactors associated with CAA include a high peak percentage neutrophils, increased peak platelet count, and reduced hemoglobin within 4–6 days during the acute phase of KD. Therefore, this population should receive primary therapy with IVIG and adjunctive anti-inflammatory medications.

Kikuchi disease: An unusual cause of cervical lymphadenopathy in a young female

Kikuchi-Fujimoto disease (KD), also known as histiocytic necrotizing lymphadenitis, is a rare cause of unilateral cervical lymphadenopathy usually described in adolescents and young adults with female preponderance. Clinically patients present with palpable lymphadenopathy, fever, and loss of weight. Hence, it should be differentiated from other causes of lymphadenopathy such as tuberculosis, lymphoma, or malignancy. Although the majority resolve spontaneously without treatment some may require non-steroidal anti-inflammatory drug or steroids. It may also be associated with systemic lupus erythematosus. Hence early detection, prompt diagnosis, and follow-up of the patient are essential in all cases of unilateral cervical lymphadenopathy in young adults. Here, we describe a 22-year old female who presented with unilateral neck swelling and was clinically diagnosed initially as a case of tuberculous lymphadenitis which on biopsy turned out to be KD.

Evaluation of paediatric lymphadenopathy at a rural tertiary care centre

Background: The aim and objectives of the present study was to determine the various etiologies of lymphadenopathy in children in our region and its associated clinical findings and prevalence of malignancy in children presented with significant lymphadenopathy.Methods: Sixty-five patients with significant lymphadenopathy charts were reviewed retrospectively from department of Paediatrics and Paediatric Surgery during the period of September 2018-2020. Patient’s records were evaluated in terms of age, gender, complaints, and characteristics of lymph nodes in terms of site, consistency, discharge, sinus and clinical course of a patient. Physical findings of all the cases were noted. Hematological and serological tests were done to know the source of infection. Clinical findings and laboratory results were corroborated with pathological diagnostic tests like FNAC and Excision biopsies in children with significant lymphadenopathyResults: The etiology was confirmed in 94% children and couldn’t be diagnosed in 6% children presented with significant lymphadenopathy. On evaluation commonest etiology was infection with reactive hyperplasia in 58% of children secondary to bacterial or viral infections, Kawasaki disease, suppurative lymphadenitis in 17%, tuberculous lymphadenitis in 14%, and malignancy in 11%. Majority of children presented with cervical lymphadenopathy. An unusual presentation of Non Hodgkin lymphoma as intestinal obstruction with no significant mass per abdomen, a case of nasopharyngeal carcinoma presented as torticollis due to massive unilateral cervical lymphadenopathy were diagnosedConclusions: The most widely encountered cause of lymphadenopathy in children was infection. Most of them are secondary to non specific viral or bacterial infections. The most important concern in children presenting with complaints of lymphadenopathy is the detection of underlying malignant disease. There was significant malignancy rate in our study in children with lymphadenopathy with few atypical presentations. Excisional biopsy is the gold standard method to confirm the diagnosis.

Analysis of temporal clusters: A new approach to unraveling the mystery of Kawasaki disease

Kawasaki disease is an acute vasculitis of childhood characterized by high fever, rash, bilateral nonexudative conjunctival injection, erythema of the oral mucosa, unilateral cervical lymphadenopathy, and erythema and edema of the hands and feet in the acute phase, or periungual desquamation in the subacute phase. Patients who lack full features of Kawasaki disease may have incomplete Kawasaki disease. Whereas the presenting signs of Kawasaki disease are transient, long-term morbidity and mortality may ensue because of an associated necrotizing arteritis that causes coronary artery aneurysms.

Experience of Cat Scratch Disease (CSD) in Rawalpindi, Pakistan – Could Physician's vigilance help in detection and case management?

Background: Cat scratch disease, caused by Bartonella henselae is more likely found in young cats, transmitted to humans through a scratch, bite, or contamination of wound or mucous membrane. Reporting the first documented case in Pakistan, in a young boy with painful ‘lumps’ on his neck. Case description: A 7-year-old boy, from rural area of Rawalpindi presented with malaise, anorexia, fever for 3 weeks and painful development of a ‘lumps’ on the right side of his neck in August 2018. The patient was treated with Amoxicillin-Clavulanate and Cephradine. On examination, grade-I malnutrition and multiple tender enlarged lymph nodes on the right angle of Mandibula was noted. All labs including TB and Rheumatoid screening were normal. Neck sonography revealed multiple enlarged matted lymph-nodes at level II B, III and IV. Findings were suggestive of chronic inflammation, Indiafection process. He was given Anti Tuberculosis treatment (ATT) on clinical grounds but no response after 4months. Epidemiological history revealed contact with young cats. Serology and histopathology confirmed Cat Scratch Disease. Azithromycin for 14 days resulted with notable improvement and was followed up for the next 6 months for progression/remission. Discussion: CSD,is a bacterial cutaneous and systemic zoonosis of cats. Contact with cats is almost invariably reported. The present case was diagnosed within 6 months of the initial symptoms and had only fever, malaise and unilateral cervical lymphadenopathy. There was no involvement of bones, joints or musculoskeletal system except raised ALT levels. Treatment with azithromycin showed to hasten the resolution of the lymphadenopathy as compared with placebo (Manfredi & Sabbatani, 2006). In our case, the patient was treated with Tab. Azithromycine for 10 days following ATT of 4 months that led to complete recovery. Conclusion: One of the most common causes of benign regional lymphadenopathy in children. Physicians must rule out CSD among patients with lymphadenopathy and history of cats contact. Surgical excision and Azithromycin should be considered. Health education & awareness in public, pet owners as well as in health practitioners is recommended.

KAWASAKI DISEASE: A CASE REPORT

Kawasaki disease (KD) is a systemic vasculitis mostly affecting medium-sized arteries. Main symptoms include fever, conjunctivitis, skin and mucous membrane affection, and cervical lymphadenopahty. KD begins with acute-onset high fever, reduced general condition and frequently reduced cooperativity of children which can complicate physical examination. Further symptoms include generalized polymorphic exanthema (&gt;90%), palmoplantar erythema (80%), symmetric non purulent conjunctivitis (80–90%), usually unilateral cervical lymphadenopathy (&gt;1.5 cm; 50%), and mucosal enanthema with red and/or chapped lips (80–90%). A female patient of 8 months and weight 10.2kg was bought to the hospital on 17/1/2020 with the complaints of prolonged high grade fever since last 10 days, previously the baby was treated with antibiotics but the fever was not subsided. On further evaluation the child was diagnosed with KD and symptomatic treatment given along with standard immunoglobulin and aspirin. Patient was treated well and discharged. Keywords: Kawasaki ,medium sized articles, chapped lips, fever

Distribution of lymphadenopathy in patients with Kikuchi disease.

Cervical lymphadenopathy is among the cardinal manifestations of Kikuchi disease (KD). The incidences and locations of extra-cervical lymph nodes (LNs) involvement in KD have not been comprehensively reported. From 2003 to 2016, 60 patients with pathologically confirmed KD and with computed tomography and/or whole-body inflammation scans at diagnosis were retrospectively identified. The locations, sizes and characteristics of all affected LNs were analyzed by extensive review of the image studies. The clinical and laboratory parameters were abstracted from medical records and the associations with extra-cervical LNs involvement were identified. Female accounted for 35 (58.3%) patients and the median age of all patients was 21.3 years (ranges, 3-64 years). Of 59 patients with evaluable neck images, 42 (71.2%) and 16 (27.1%) patients presented with unilateral and bilateral nodal involvement, respectively, with the most common locations at level II, III and IV by Som's classification. The largest LNs appeared most commonly in level II. The incidences of extra-cervical lymphadenopathy in abdomen, pelvis, inguina, axilla and mediastinum with available images were respectively 52.9% (9/17), 47.1% (8/17), 41.2% (7/17), 30.6% (11/36) and 14.3% (8/56). When compared to cases with solitary cervical lymphadenopathy, the cases with extra-cervical lymphadenopathy had significantly greater incidences of bilateral cervical lymphadenopathy (P=.0379) and leukopenia (P=.0173). Unilateral cervical lymphadenopathy was the most frequent form of LNs involvement of KD. Extra-cervical lymphadenopathy was not uncommon and was associated with the appearance of bilateral distribution of cervical LNs and leukopenia.

FREQUENCY AND DISTRIBUTION OF DIFFERENT TYPES OF CERVICAL LYMPHADENOPATHY IN PATIENTS IN D.I.KHAN DISTRICT, PAKISTAN

Background: Cervical lymphadenopathy is a common medical problem. The objective of the study was to determine the frequency and distribution of different types of cervical lymphadenopathy in patients in D.I.Khan, KP, Pakistan. Material &amp; Methods: This cross sectional study was conducted at Department of ENT, Gomal Medical College, Dera Ismail Khan from April 2013 to March 2016. Sample size was 80, selected through consecutive sampling technique. Patients having enlarged lymph nodes of either sex and of any age of at least 2 months duration were included. Patients having generalized lymphadenopathy were excluded from the study. Gender and age groups were the demographic variables. The research variables were types of cervical lymphadenopathy (tuberculous lymphadenopathy/ reactive hyperplasia/ metastatic carcinoma/ lymphpoma) and side of the neck involved (right/ left). All variables being categorical, the frequencies and percentages were calculated. descriptive statistical analysis was done using SPSS version 10. Results: Out of 80 patients, 42 were males and 38 females. Majority of the patients were in the first decade. Chronic granulomatous diseases especially the tuberculous cervical lymphadenopathy was the most frequent histological findings in 31 patients. Lymphoma was noted in 21, reactive hyperplasia in 18 and metastatic malignancy in 10 cases. Lymph nodes involvement on the right side was more common 36 than on the left side 32. Conclusion: Unilateral tuberculous cervical lymphadenopathy was most common found especially in young males.

SIALOLITHIASIS: CLINICAL AND RADIOGRAPHIC APPROACH

The sialolithiasis is characterized by the formation of a calcified structure within the salivary gland or its ductal system. In general, the submandibular gland is the most prevalent, being most common in adults over 40 years. Clinical tests and imaging studies are essential to confirm the diagnosis. A 41-year-old female patient attended the Regional Health Unit of the Municipality of Serra, complaining of difficulty feeding, swallowing and phonation for over 3 months, with a significant worsening in the previous 3 days. Clinically, it was observed unilateral left cervical lymphadenopathy and a hardened nodule was pointed out in the floor of the mouth region. The occlusal radiographic examination revealed the presence of a radiopaque mass. Based on clinical and radiographic observations, the diagnostic sialolithiasis was confirmed. Postoperative follow-up was proven to normal salivary flow and also the anatomic aspects of oral floor after surgical treatment.

Kawasaki disease: State of the art.

Kawasaki disease is an acute febrile arteritis of childhood that can result in coronary artery aneurysms if untreated in the first 10 and ideally 7 days of illness. Kawasaki disease begins as a necrotizing arteritis with neutrophilic infiltrate, followed by subacute/chronic changes and luminal myofibroblastic proliferation that can cause coronary artery stenosis. Manifestations include the presence of ≥5 days of fever, together with clinical criteria of extremity changes, rash, conjunctivitis, oral changes, and unilateral cervical lymphadenopathy. Echocardiography should be performed at the time of diagnosis, then 1-2 weeks and 4-6 weeks later, with more frequent studies in individuals with coronary artery dilation or persistent fever. Coronary artery dimensions are characterized both as z-scores and absolute measurements, and coronary architecture evolves over time in children who have aneurysms in the first weeks of illness. Systematic follow-up and therapies are tailored to the degree of coronary disease and to coronary ischemia.

TULAREMIA IN SOUTH-EASTERN SERBIA IN TWELVE-YEAR FOLLOW-UP

Tularemia is a serious bacterial zoonosis caused by the highly infectious agent Francisella tularensis. Microbiological diagnosis of tularemia mainly relies on serology. The occurrence of a tularemia epidemic in the Southeast of Serbia in 1998/999 initiated an epidemiological as well as a clinical and microbiological research in this area. Objective was establishing the correlation between the clinical-epidemiological and serological diagnosis of tularemia as well as the clinical and serological follow-up of patients in the period from 1 to 12 years since the disease onset. From the beginning of 1999 until the end of 2011, 113 patients diagnosed with tularemia were examined. The control group was formed of 111 patients with lymphadenopathy of different origins. The following serological methods were used: microagglutination test (MAT), immunoensyme assays: ELISA (VMA, Belgrade), Serion ELISA IgG i IgM, Serazym ELISA and ELISA in house and immunochromatographic test (ICT). Clinical-epidemiological diagnosis of tularemia was confirmed serologically in all 113 patients. The high sensitivity and specifity were found for all the examined tests. IgG Virion ELISA demonstrated the highest sensitivity (97.4%) and specificity (93.1%). IgG and IgM class of antibodies remained positive in the serum in a high percentage, even as long as 12 years from the infection. Oropharyngeal form (93.8%), with predominant unilateral cervical lymphadenopathy (91.5%), was the most common clinical form. Complications, such as suppurative lymphadenitis and recurrent lymphadenitis, were seen in 41.6% of patients. A positive correlation between clinical-epidemiological and serological diagnosis of tularemia has been established. Serological findings must be interpreted only within the clinical picture of tularemia. A finding of IgM and IgG class antibodies or total antibodies of F. tularensis in the sera of patients without clinical disease manifestations, from one to 12 years from the disease onset, does not indicate an acute but a past infection.

Ultrasonographic findings of Kikuchi cervical lymphadenopathy in children.

PurposeThe purpose of this study was to analyze the ultrasonographic (USG) findings of Kikuchi cervical lymphadenopathy in pediatric patients.MethodsBetween April 2007 and September 2016, 84 children (42 male and 42 female; mean±standard deviation age, 12.9±3.2 years; range, 5 to 18 years) confirmed with Kikuchi disease were enrolled. Clinical findings and USG findings of Kikuchi cervical lymphadenopathy were retrospectively reviewed. Localized symptoms, systemic symptoms, and laboratory findings including the white blood cell count, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) were analyzed. An analysis of the USG findings included evaluation of the location, size, and presence of intranodal abscess; intranodal calcification; perinodal fat swelling; localized fluid collection; and loss of nodal echogenic hilum.ResultsAmong the patients, 49 (58%) showed localized tenderness at the cervical lymphadenopathy. Fever was present in 55 (66%), while 27 (32%) had prolonged fever. Of 74 with lab results, 54 (73%) had leukopenia but none had leukocytosis. Among the same 74, there was a high ESR (>50 mm/hr) in 10 (14%) and a high CRP level (>5 mg/dL) in seven (9%). The USG findings of most of the patients (n=72, 86%) showed unilateral neck involvement, especially in the left side neck (45 of 72, 63%). The most common site of Kikuchi lymphadenopathy involvement was the area at cervical lymph node level V, at the posterior triangle (n=77, 92%). Conglomerated nodal distribution (n=57, 68%), preserved central nodal echogenic hilum (n=84, 98%), and perinodal fat swelling (n=55, 65%) were common USG findings in the children with Kikuchi. In addition, multiple cervical lymph nodes showed a relatively even size distribution (n=73, 87%).ConclusionThe common USG findings of Kikuchi disease in the pediatric population of our study were multiple conglomerated unilateral cervical lymphadenopathy showing perinodal fat swelling and even size distribution.

IgG4-related disease simulating Hodgkin lymphoma in a child

Immunoglobulin (Ig) G4-related disease is a recently described syndrome characterized by mass forming lymphoplasmacytic tissue infiltration and elevated serum IgG4 concentrations usually affecting middle-aged or older individuals. Lymphadenopathy is frequently observed and is sometimes the first or only manifestation of the disease. We report a case of IgG4-related disease mimicking Hodgkin lymphoma in a 13-year-old girl. The patient presented with progressive unilateral cervical lymphadenopathy of several months duration. Biopsy showed follicular hyperplasia with progressive transformation of germinal centers. Interfollicular areas were expanded by small lymphocytes, histiocytes, eosinophils and fibrosis with occasional CD30 positive cells initially concerning for interfollicular Hodgkin lymphoma. Immunohistochemical analysis revealed an intrafollicular plasmacytosis with an IgG4-positive/IgG-positive plasma cell ratio of 50% supporting a diagnosis of IgG4-related lymphadenopathy, progressively transformed germinal centers type. Laboratory studies were supportive with elevated serum IgG4 (178mg/dL) and IgE (30.40kU/L) levels along with an elevated serum IgG4/IgG ratio (0.16). Very few cases of IgG4-related disease have been described in children. Within this age group, there is considerable clinical overlap between IgG4-related disease associated lymphadenopathy and Hodgkin lymphoma. In addition, lymphadenopathy secondary to IgG4-related disease demonstrates substantial histologic diversity with the potential to simulate the inflammatory background and fibrosis of Hodgkin lymphoma. The importance of accurate diagnosis is underscored by the prognostic implications considering the marked response of the syndrome to steroid therapy. In addition, appropriate follow up is critical to monitor for relapse and additional organ involvement.

Abstract 185: Is Lymph-Node-First Kawasaki Disease a High-risk Group for Coronary Aneurysm?

Cervical lymphadenopathy (CLA) is the least common main feature in patients with Kawasaki disease (KD), comprising only 42%-65% of all diagnoses. Nonetheless, several studies have shown that KD patients who first present with remarkable CLA and fever (NF-KD) are older in age and exhibit stronger inflammation than that of typical KD (tKD) patients. Therefore, whether NF-KD patients are also a high-risk group for intravenous immunoglobulin resistance (rIVIG) and coronary arterial aneurysm (CAA), and whether tKD with CLA (tKD-CLA) is associated with higher inflammatory indices than tKD without CLA warrants investigation. Previous retrospective (R) and prospective (P) studies have shown varied results (Table). In this study, we reviewed 10 years of medical records from a tertiary referral hospital and identified 42 NF-KD patients. These NF-KD patients were then compared with 113 tKD-CLA and 105 tKD patients from the same database over the same period. Significant trends of increasing values were noted in age, neutrophils, CRP, ESR, GOT, and doses of IVIG among these 3 groups of patients ( p = 0.03 to &lt; 0.0001). NF-KD patients presented with larger cervical LN (3.7 ± 7.0 cm) complicated by higher CAA (12/42 vs. 25/218, p = 0.008) and by moderate to extremely high CAA (6/42 vs. 4/218, p = 0.002), and suffered more CHF or shock at the acute stage (6/42 vs. 6/218, p &lt; 0.001) than all of the tKD patients. The size of LN did not correlate with CAA occurrence, but the presence of bilateral CLA showed a higher risk for CAA than unilateral CLA did (6/10 vs. 6/32, p = 0.02). Author(Hospital No.) Method NF-KD tKD-CLA tKD LN(cm) CAA rIVIG Nomura Y (1) R 16 106 65 &gt; 1.5 + + Kanegaye JT (1) P 53 . 287 3.0 ± 1.0 - - Kubota M (1) R 29 62 107 &gt; 1.5 - - Sung RYT (14) R . 170 526 &gt; 1.5 - - Chang JS (1) R 42 113 105 3.7 ± 7.0 + + This study showed that NF-KD patients, particularly those with larger lymph nodes, are a high risk group for CAA complications. The tKD-CLA patients also exhibited a tendency for higher inflammation. Previous pathological studies of swollen CLA have reported tissue edema in the sinus and paracortical areas of LN, as well as vessel thrombi in severe cases. Further study of swollen CLA is warranted to determine the pathological correlation between CLA severity and CAA complications in KD patients.

Progressive Unilateral Cervical Lymphadenopathy With Elevated LDH in a School-Aged Female.

Progressive Unilateral Cervical Lymphadenopathy With Elevated LDH in a School-Aged Female.