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Competing Interests: Conflicts of interest None disclosed.

Background: External Jugular Thrombophlebitis (EJT) is a rare clinical phenomenon with few reports in the literature, especially in the pediatric population. This is a report of an unusual case of right-sided EJT in a pediatric patient secondary to acute pharyngitis with sinusitis most prominent on the left side., Case Presentation: A 13-year-old presented to the emergency department with worsening upper respiratory infectious (URI) symptoms and facial swelling, cough, throat pain, and emesis. The patient had traveled to Switzerland and received amoxicillin for strep throat 6 weeks before this hospitalization. Physical examination revealed nasal purulence, allodynia over the right side of the face without overlying erythema, and oropharyngeal exudate. CT scan revealed left-sided predominate sinusitis and right external jugular vein thrombosis. Blood cultures confirmed the presence of group A streptococcus infection. Treatment included IV antibiotics, non-steroidal anti-inflammatory drugs (NSAIDs), IV steroids, and anticoagulation. Follow-up imaging demonstrated improvement in thrombosis, cellulitis, and sinus disease. The patient was discharged on antibiotics for 6 weeks and anticoagulation for 10 weeks. Follow-up imaging at 6 months revealed no EJT, and medications were discontinued., Conclusions: EJT is a rare condition, and to our knowledge, no reports of EJT with sinusitis most pronounced on the contralateral side have been published. Physicians will benefit from noting clinical signs of EJT such as facial edema, headache, erythema, and palpable neck mass, especially if these symptoms occur with URI symptoms refractory to treatment. The use of anticoagulation is controversial for internal jugular vein thrombosis, and while no guidelines for EJT exist, anticoagulation is likely not necessary save for severe complications., (© 2024. The Author(s).)

Monogenic diseases of immune dysregulation should be considered in the evaluation of children presenting with recurrent neutrophilic dermatoses in association with systemic signs of inflammation, autoimmune disease, hematologic abnormalities, and opportunistic or recurrent infections. We report the case of a 2-year-old boy presenting with a neutrophilic dermatosis, found to have a novel likely pathogenic germline variant of the IKAROS Family Zinc Finger 1 (IKZF1) gene; the mutation likely results in a loss of function dimerization defective protein based on reports and studies of similar variants. IKZF1 variants could potentially lead to aberrant neutrophil chemotaxis and development of neutrophilic dermatoses. Long-term surveillance is required to monitor the development of hematologic malignancy, autoimmunity, immunodeficiency, and infection in patients with pathogenic IKZF1 germline variants., (© 2024 Wiley Periodicals LLC.)

Objective: This study aimed to present 2 children clinically diagnosed with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome and treated with intracapsular tonsillectomy with adenoidectomy (ITA)., Methods: We conducted a retrospective analysis of 2 children who were referred for an otolaryngology consultation between 2019 and 2022 for surgical treatment of PFAPA syndrome. Both patients had symptoms strongly suggestive of PFAPA and were at risk for total tonsillectomy (TT) complications. ITA was performed using a microdebrider. Both patients were followed up postoperatively to assess for symptomatic resolution and complications., Results: Two children exhibited recurrent febrile episodes prior to ITA. The procedure was efficacious in both patients, with neither experiencing postoperative complications or recurring PFAPA symptoms for over 1 year after surgery., Conclusion: Our study reported on the use of ITA as a surgical treatment option for PFAPA. We showed that ITA eliminated febrile attacks and was safely performed without postoperative complications in 2 pediatric patients after 1-year follow-up. Future studies involving larger cohorts of PFAPA patients and lengthier follow-ups will need to be conducted to further evaluate ITA as a surgical option. Laryngoscope, 134:1967-1969, 2024., (© 2023 The Authors. The Laryngoscope published by Wiley Periodicals LLC on behalf of The American Laryngological, Rhinological and Otological Society, Inc.)

Lupus anti-coagulant hypoprothrombinemia syndrome (LAHPS) is a rare condition that can be difficult to treat. It increases the risk of thrombosis and bleeding due to the presence of lupus anti-coagulant and factor II deficiency, respectively. There are a limited number of cases described in the literature. Herein we describe a case of LAHPS with bleeding symptoms as a first clinical manifestation of systemic lupus erythematosus (SLE) in an 8-year-old female. She has had multiple recurrences of her bleeding symptoms, requiring treatment with steroids, cyclophosphamide, mycophenolate mofetil, and rituximab. Her course was later complicated by development of arthritis and lupus nephritis. Her complicated course provides a new perspective on the clinical course and treatment of LAHPS. We also present a comprehensive literature review which demonstrates the difficulty in treating patients with LAHPS with underlying SLE and the variability of the clinical course and management of LAHPS depending on the age at presentation., (© 2023. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

Background: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is a clinical syndrome of unclear etiology. PFAPA has generally been considered a non-hereditary fever syndrome; however, this has been called into question with recent reports of family clustering. Few reports have been published describing siblings with PFAPA. To our knowledge, this is the first report of siblings with near simultaneous onset of disease followed by synchronous disease flares., Case Presentation: We describe the case of near simultaneous onset of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis in siblings followed by synchronous disease flares of clear frequency and nearly identical character. Flares were characterized predominantly by fever, aphthous ulceration, cervical lymphadenitis, and the absence of infection. The fever episodes demonstrated a robust response to glucocorticoids and recurred in the same staggered manner every four weeks, with complete absence of symptoms and normal growth and development between episodes. Nine months after onset, the older sibling, a 5-year-old female, underwent tonsillectomy resulting in dramatic resolution of episodes. At the same time, her 2-year-old sister experienced resolution of her fever episodes, though she did not undergo tonsillectomy herself., Conclusion: This is an unusual case of simultaneous onset PFAPA followed by synchronous disease flares. PFAPA is an uncommon clinical syndrome, and it is rarely diagnosed in siblings. The etiology of PFAPA remains unclear. Though the disease is classically considered sporadic, there is a growing body of evidence to suggest that PFAPA may be heritable., (© 2022. The Author(s).)

Importance: To date, no study has characterized the mucocutaneous features seen in hospitalized children with multisystem inflammatory syndrome in children (MIS-C) or the temporal association of these findings with the onset of systemic symptoms., Objective: To describe the mucocutaneous findings seen in children with MIS-C during the height of the coronavirus disease 2019 (COVID-19) pandemic in New York City in 2020., Design, Setting, and Participants: A retrospective case series was conducted of 35 children admitted to 2 hospitals in New York City between April 1 and July 14, 2020, who met Centers for Disease Control and Prevention and/or epidemiologic criteria for MIS-C., Main Outcomes and Measures: Laboratory and clinical characteristics, with emphasis on mucocutaneous findings, of children who met criteria for MIS-C. The characterization of mucocutaneous features was verified by 2 board-certified pediatric dermatologists., Results: Twenty-five children (11 girls [44%]; median age, 3 years [range, 0.7-17 years]) were identified who met definitional criteria for MIS-C; an additional 10 children (5 girls [50%]; median age, 1.7 years [range, 0.2-15 years]) were included as probable MIS-C cases (patients met all criteria with the exception of laboratory test evidence of severe acute respiratory syndrome coronavirus 2 [SARS-CoV-2] infection or known exposure). The results of polymerase chain reaction tests for SARS-CoV-2 were positive for 10 patients (29%), and the results of SARS-CoV-2 immunoglobulin G tests were positive for 19 patients (54%). Of the 35 patients, 29 (83%) exhibited mucocutaneous changes, with conjunctival injection (n = 21), palmoplantar erythema (n = 18), lip hyperemia (n = 17), periorbital erythema and edema (n = 7), strawberry tongue (n = 8), and malar erythema (n = 6) being the most common findings. Recognition of mucocutaneous findings occurred a mean of 2.7 days (range, 1-7 days) after the onset of fever. The duration of mucocutaneous findings varied from hours to days (median duration, 5 days [range, 0-11 days]). Neither the presence nor absence of mucocutaneous findings was significantly associated with overall disease severity., Conclusions and Relevance: In this case series of hospitalized children with suspected MIS-C during the COVID-19 pandemic, a wide spectrum of mucocutaneous findings was identified. Despite their protean and transient nature, these mucocutaneous features serve as important clues in the recognition of MIS-C.

Background: The Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) is a reliable outcome measure for cutaneous lupus erythematosus (CLE) in adults used in clinical trials. However, it has not been validated in children, limiting clinical trials for paediatric CLE., Objectives: This study aimed to validate the CLASI in paediatrics., Methods: Eleven paediatric patients with CLE, six dermatologists and six rheumatologists participated. The physicians were trained to use the CLASI and Physician's Global Assessment (PGA), and individually rated all patients using both tools. Each physician reassessed two randomly selected patients. Within each physician group, the intraclass correlation coefficient (ICC) was calculated to assess the reliability of each measure., Results: CLASI activity scores demonstrated excellent inter- and intrarater reliability (ICC > 0·90), while the PGA activity scores had good inter-rater reliability (ICC 0·73-0·77) among both specialties. PGA activity scores showed excellent (ICC 0·89) and good intrarater reliability (ICC 0·76) for dermatologists and rheumatologists, respectively. Limitations of this study include the small sample size of patients and potential recall bias during the physician rerating session., Conclusions: CLASI activity measurement showed excellent inter- and intrarater reliability in paediatric CLE and superiority over the PGA. These results demonstrate that the CLASI is a reliable and valid outcome instrument for paediatric CLE., (© 2018 British Association of Dermatologists.)

Juvenile idiopathic arthritis (JIA) includes several forms of chronic arthritis in childhood with no apparent cause. JIA is the most common rheumatic disease in children, and may result in pain, joint deformity, and growth im- pairment, with possible persistent active arthritis into adulthood. Prior treatment involved non-specific agents, several with significant adverse effects. The recent use of biologics now provides target-specific therapy, which may be better tolerated. Through continued translational research and clinical trials, one better understands the biology mediating disease, with the hope of offering safer, more effective medicine, and potential cure. This review will outline the clinical features of JIA, as well as provide the latest updates in treatment.

Objective: Behcet's is an idiopathic multi-organ syndrome, which may have onset during childhood. Vascular involvement is uncommon, with rarely reported coronary aneurysm formation. We present a case report of a teenager girl who developed recalcitrant life-threatening Behcet's vasculitis, involving both small and large venous and arterial systems including a giant coronary aneurysm., Case Report: De-identified data were collected retrospectively in case report format. Although our sixteen year old female with Behcet's vasculitis had resolution of many arterial aneurysms, she had persistent venous thrombosis of large vessels, as well as persistent, giant arterial aneurysms requiring intra-arterial coiling of a lumbar artery and coronary bypass grafting despite intensive immunosuppression including glucocorticoids, cyclophosphamide, infliximab, methotrexate, azathioprine and intravenous immunoglobulin., Conclusions: Vascular manifestations may be seen in Behcet's syndrome, including asymptomatic coronary aneurysm, which may be refractory to immunosuppression and ultimately require surgical intervention. Increased awareness is essential for prompt diagnosis and management.

Objective: To assess the prevalence of neurocognitive impairment (NCI) in childhood-onset systemic lupus erythematosus (cSLE) by comparing published classification criteria, and to examine associations between NCI, disease characteristics, psychosocial well-being, and intelligence., Methods: cSLE patients and ethnicity- and age-matched healthy controls completed a neuropsychological research battery, and results were categorized by 3 different NCI classification criteria with different cutoff scores (e.g., >2, 1.5, or 1 SD below the mean) and the number of required abnormal tests or domains., Results: Forty-one cSLE subjects and 22 controls were included. Subjects were predominantly female (~70%) and Hispanic (∼70%). Executive functioning, psychomotor speed, and fine motor speed were most commonly affected. Method 1 classified 34.1% of cSLE subjects with NCI compared to method 2 (14.6% with decline and 7.3% with NCI) and method 3 (63.4% with NCI). The prevalence of NCI was not significantly different between the controls and patients using any of the categorization methods. NCI was not associated with SLE disease activity or characteristics or with depression. Using method 3, patients in the cognitive impairment group reported significantly lower quality of life estimates (69.7 versus 79.3; P = 0.03). Below average intellectual functioning (intelligence quotient <90) differentiated the number of test scores >1 and >1.5 SDs, but not >2 SDs below the mean., Conclusion: NCI was prevalent in cSLE, but varied according to the chosen categorization method. A similar proportion of cSLE patients and controls had NCI, reinforcing the importance of studying an appropriate control group. Categorical classification (i.e., impaired/nonimpaired) may oversimplify the commonly observed deficits in cSLE., (Copyright © 2011 by the American College of Rheumatology.)

Objective: To describe the course of patients with juvenile dermatomyositis (JDM) treated effectively without systemic corticosteroids., Study Design: A retrospective study of 38 patients with JDM treated at a tertiary care children's hospital identified 8 patients who had never received corticosteroids. Disease presentation and course, pharmacologic, and ancillary treatments were recorded., Results: Patients in the no corticosteroid group were followed for a median of 2.8 years (range, 2.1 to 9.5 years). Treatment was primarily with intravenous immunoglobulin (IVIG) (75%) and methotrexate (50%), with favorable response in all. No serious treatment complications were observed; headaches were reported by 3 patients receiving IVIG. Two patients had a myositis flare after discontinuing all medications for more than 1 year; complete resolution of symptoms was observed after either 1 or 2 further doses of IVIG. Two patients had calcinosis (at 1 and 9 years of disease); however, no patient had joint contractures, muscle atrophy, lipodystrophy, or functional limitations., Conclusions: Systemic corticosteroids can be avoided in a select group of patients with JDM. Alternative agents such as methotrexate and IVIG may be prescribed to effectively treat JDM and prevent complications., (Copyright 2010 Mosby, Inc. All rights reserved.)

In 2000, soldiers from a Special Operations Force had airborne lead exposures 20 to 38 times the permissible exposure limit. Their average blood lead level was 13.9 microg/dL. Immediate implementation of pertinent Occupational Safety and Health Administration regulations was recommended. In 2003, investigators learned that the unit also used an outdoor firing range with exposures exceeding the permissible exposure limit. Attempts to conduct more detailed evaluations and control measures were hindered by frequent deployments in the unit and a strong commitment to realistic training. Despite these challenges, the controls implemented resulted in limited success. The average blood lead level in the unit was 6.8 microg/dL in 2005, a reduction of 51%. Proper initial range construction is essential to controlling lead exposures at firing ranges. Occupational health specialists caring for these soldiers must identify, assess, and mitigate exposures from firing ranges while respecting the importance of realistic training.

Prostaglandins are presumed to have cytoprotective properties and may play a role in the pathogenesis of duodenal ulcer and its complications. To evaluate this hypothesis, 35 patients with either duodenal ulcer bleeding (18 patients) or gastric outlet obstruction (17 patients) were investigated. Biopsies were taken from gastroduodenal tissues and secretions for prostaglandin E2 (PGE2) levels. These levels were compared to those taken from the same areas during a later endoscopy. A correlation was found between the severity of the clinical endoscopic findings and PGE2 levels. Increased levels of PGE2 were found in the quiescent phase and decreased levels found during the deteriorated phase. These differences of PGE2 levels were found to be of significant value (P less than 0.002). Furthermore, the patients in which the PGE2 levels were decreased at second endoscopy needed surgery. PGE2 may, thus, be a factor in duodenal ulcer pathogenesis and its complications, and be used as a prognostic marker and guide.

Palliative surgery has a role in nonresectable pancreatic carcinoma. We attempted to evaluate the efficacy of palliative surgery followed by synchronous therapy in such cases. A group of 92 patients was studied. Sixty-six patients (Group 1) with biliary obstruction underwent surgical biliary bypass. Twenty-six patients (Group 2) underwent explorative laparotomy only. Thirty patients (45.5%) in Group 1 and 10 patients (38.5%) in Group 2 received synchronous therapy consisting of 10 intravenous administrations of 5-fluorouracil (750 mg/m2) over 8 h, followed 8 h later by radiation with 400 rads repeated every 4 days. The mean survival for the entire group was 8.9 months. Those in Group 1 who received synchronous therapy had a mean survival of 13.5 months; those who did not 8.9 months (P less than 0.01). Patients in Group 2 who received synchronous therapy survived with a mean of 5.4 months, those who did not 2.7 months (P less than 0.01). Toxicity due to synchronous therapy was minimal. Of the 40 patients receiving synchronous therapy, 37 suffered from abdominal pains prior to initiation of therapy, 29 of which (78.4%) were free of pain upon completion of therapy. In nonresectable carcinoma of the pancreas, surgical palliation provides an acceptable survival. When combined with synchronous therapy, it results in prolonged survival and an ameliorated quality of life.

A hidden loop jejunostomy, the placement of a proximal small bowel loop under the skin of dogs, is described. A feeding tube was inserted in the loop at a later date, which enabled feeding for at least 6 weeks. This procedure was well tolerated by the 10 dogs involved in this experimental model. It should be considered as a possible surgical procedure at initial explorative laparotomy in patients with advanced cancer originating at the gastric cardia or esophagogastric junction.

Sixty patients were treated in the emergency ward for biliary colic. Cholelithiasis was proven by ultrasonography. Twenty patients (group I) were treated by placebo. Twenty patients (group II) were treated by papaverine, and 20 patients were treated by diclofenac sodium (Voltaren) (group III). Twenty more patients (group IV) with low back pain (LBP) were treated with diclofenac sodium (Voltaren) as a control to assess the analgesic effect of Voltaren. Two interesting observations were made: Voltaren was proven more efficient for pain relief (P less than 0.002), and none of the patients treated with Voltaren were in need of hospitalization and immediate surgery. In comparison, nine patients of the other two groups progressed to acute cholecystitis and needed surgical intervention. The possible anticolic and anti-biliary inflammation properties and the indications for use of Voltaren are discussed.

In the phenomenon of leukergy, white blood cells agglomerate in peripheral blood slides. This agglomeration has been described in inflammatory infections of various causes. This study assesses this phenomenon in inflammatory bowel disease. A correlation was found between the severity of inflammatory bowel disease activity and the percentage of leukergy. Leukergy was found to parallel the clinical and endoscopic findings of inflammatory bowel disease. Furthermore, leukergy was found to be more accurate than white blood count and erythrocyte sedimentation rate. It is also found to accurately assess the course of the disease when clinical and other laboratory tests were masked by steroid and antibiotic administrations. Leukergy is a quick, inexpensive test that can easily be performed at the patient's bedside.

Prostaglandins are presumed to have many cytoprotective properties that play a role in the pathogenesis of duodenal ulcer and its complications where decreased levels of prostaglandin E2 (PGE2) impair gastric motility, oppose ionic membrane influx, and enhance obstructive changes. These are just some of the mechanisms that may cause pyloric obstruction and may result from decreased PGE2 levels. To evaluate this hypothesis, 17 patients with duodenal ulcer complicated by pyloric stenosis were examined. Biopsy specimens were obtained from the duodenal bulb, ulcer margins, gastric antrum, fundus, and gastric secretions. Prostaglandin E2 levels were measured and compared with those taken from the same areas during a second endoscopy in a later quiescent or exacerbated phase. During the active phase of pyloric stenosis, decreased levels of PGE2 were found in the gastroduodenal tissues and secretions were compared with levels found during convalescence. These level differences were statistically significant. A correlation between the severity of the clinical and endoscopic findings and the PGE2 levels was found. A further decrease in PGE2 levels in the second endoscopy were indicative of the presence of scar tissue, representing an irreversible obstructive peptic disease.

The most common causes for morbidity and mortality in colorectal resections are anastomotic leaks. In low anterior resection, the incidence of anastomotic leakage ranges from 17 to 50%. With the use of the stapler technique, leakage incidence rate remains high and ranges from 10 to 25%. Colostomy formation and closures are associated with considerable morbidity and mortality. Due to the high incidence of anastomotic leakage rate in low anterior resection, and the additional complications of diverting colostomy formation and closure, the use of a rectal stent-intrarectal bypass graft has been instituted. This is carried out by means of a silastic graft, which prevents the fecal stream and gas pressure from coming into contact with the anastomotic site at the low rectum. The efficacy of intrarectal bypass graft was examined in two high-risk surgical situations, the first in very low anterior resection and the other, after early sigmoid obstruction. In both situations the intrarectal bypass graft provided for a safe anastomosis. Even when dehiscence and early obstructions occur, the tube may prevent leakage. This procedure presents effective practical implications which obviate the need for a proximal colostomy formation, thereby eliminating the physical and psychological stress that accompanies colostomies.

In a retrospective study, 153 male patients who underwent surgical treatment of the colon and rectum presented with complaints indicating obstruction of the prostate gland and clear indications for preoperative administration of alpha-blockers. Between the years 1982 and 1984, the alpha-blocker phenoxybenzamine hydrochloride (PB-HCl) was prohibited from use by the Ministry of Health because of suspicions of its carcinogenic properties. This enabled a comparative study between two groups of patients. Seventy-five patients (group 2a1) were prohibited from receiving PB-HCl. Urinary retention occurred in 54.7 per cent and urinary tract infection in 65.3 per cent. Seventy-eight patients (group 2a2) were administered PB-HCl. In this group of patients, urinary retention occurred in 19.2 per cent and urinary tract infection in 15.4 per cent. Forty-one patients with urinary retention of group 2a1 who were not administered PB-HCl were compared with 50 patients with urinary retention (group 3) who received PB-HCl. Spontaneous relief was observed in 21.9 and 76.0 per cent, respectively. PB-HCl was shown to be extremely effective, both as a preventative and therapeutic agent, when complaints indicating the prostate gland are present in patients undergoing surgical treatment of the colon and rectum.