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Historically, children evaluated for vomiting and diarrhea secondary to viral enteritis have symptoms lasting 2–4 days and respond to supportive care, including oral rehydration and anti-emetics if required. Recently, within a 14-day timespan, we encountered three children with severe diarrhea who rapidly became dehydrated and went into hypotensive shock. Although SARS-CoV-2 molecular tests were negative by nasopharyngeal swab, all were later found to have MIS-C. This small case series underscores features reported in previous larger studies and emphasizes the rapid clinical evolution of this condition. We highlight the importance of early recognition of cardinal laboratory findings characteristic of MIS-C (i.e., lymphopenia, markedly elevated acute phase reactants, and hypoalbuminemia). We also show serologic evidence that the pathophysiological mechanism of SARS-CoV-2 related diarrhea may differ from other causes of dehydrating vomiting and diarrhea, with no serologic evidence of villus cell injury.

Made available in DSpace on 2022-04-29T08:30:47Z (GMT). No. of bitstreams: 0 Previous issue date: 2021-07-01 Objective: Sjögren syndrome in children is a poorly understood autoimmune disease. We aimed to describe the clinical and diagnostic features of children diagnosed with Sjögren syndrome and explore how the 2016 ACR/EULAR classification criteria apply to this population. Methods: An international workgroup retrospectively collected cases of Sjögren syndrome diagnosed under 18 years of age from 23 centres across eight nations. We analysed patterns of symptoms, diagnostic workup, and applied the 2016 ACR/EULAR classification criteria. Results: We identified 300 children with Sjögren syndrome. The majority of patients n = 232 (77%) did not meet 2016 ACR/EULAR classification criteria, but n = 110 (37%) did not have sufficient testing done to even possibly achieve the score necessary to meet criteria. Even among those children with all criteria items tested, only 36% met criteria. The most common non-sicca symptoms were arthralgia [n = 161 (54%)] and parotitis [n = 140 (47%)] with parotitis inversely correlating with age. Conclusion: Sjögren syndrome in children can present at any age. Recurrent or persistent parotitis and arthralgias are common symptoms that should prompt clinicians to consider the possibility of Sjögren syndrome. The majority of children diagnosed with Sjögren syndromes did not meet 2016 ACR/EULAR classification criteria. Comprehensive diagnostic testing from the 2016 ACR/EULAR criteria are not universally performed. This may lead to under-recognition and emphasizes a need for further research including creation of paediatric-specific classification criteria. Division of Pediatric Rheumatology Department of Pediatric and Adolescent Medicine Mayo Clinic Division of Rheumatology Department of Pediatrics University of Utah School of Medicine Division of Rheumatology The Children's Hospital of Philadelphia Department of Internal Medicine and Pediatrics The University of Chicago Medical Center Department of Pediatrics Duke University School of Medicine Department of Pediatrics Hematology and Oncology Medical University of Gdansk Children's Mercy Hospital University of Missouri-Kansas City Division of Rheumatology Irccs Ospedale Pediatrico Bambino Gesù Department of Pediatric Rheumatology Institute of Rheumatology Department of Pediatrics Columbia University Medical Center Division of Rheumatology Children's National Hospital George Washington University School of Medicine and Health Sciences National Institutes of Health National Institute of Arthritis and Musculoskeletal and Skin Diseases Department of Pediatrics University of Texas McGovern Medical School at Houston Division of Pediatric Rheumatology Children's Healthcare of Atlanta Emory University School of Medicine Instituto de Puericultura e Pediatria Martagão Gesteira Universidade Federal Do Rio de Janeiro Pediatric Rheumatology Hospital Sant Joan de Déu Universitat de Barcelona Barcelona Rheumatology Unit Department of Medicine School of Medical Science University of Campinas Division of Rheumatology Department of Pediatrics University of Indiana School of Medicine Riley Hospital for Children at Indiana University Health Pediatric Rheumatology Unit São Paulo State University (UNESP), Botucatu Rheumatology Service Royal Children's Hospital Melbourne and Murdoch Children's Research Institute Division of Pediatric Allergy Immunology and Rheumatology Department of Pediatrics College of Medicine University of Florida Division of Oral Medicine Department of Oral and Maxillofacial Diagnostic Sciences College of Dentistry University of Florida Rheumatology Department Hospital General Universitario Gregorio Marañón Division of Immunology Boston Children's Hospital Department of Pediatrics Harvard Medical School Department of Rheumatic Diseases Tokyo Metropolitan Tama Medical Center Division of Rheumatology Allergy and Immunology Stead Family Department of Pediatrics Carver College of Medicine University of Iowa Pediatric Rheumatology Unit São Paulo State University (UNESP), Botucatu

1. Leigh A. Stubbs, MD, MPH* 2. Sam N. Russo, MD† 3. Baruch R. Goldberg, MD‡ 4. Ankur A. Kamdar, MD‡ 1. *Division of Community and General Pediatrics, 2. †Division of Neurology, Department of Pediatrics, 3. ‡Division of Pulmonary, Allergy, Immunology, and Rheumatology, McGovern Medical School at The University of Texas Health Science Center at Houston, Houston, TX 1. Address correspondence to Leigh Anna Stubbs, MD, MPH, Feigin Tower, 1102 Bates, Ste. 330, Houston, TX 77030. E-mail: leigh.stubbs{at}bmc.edu An 8-year-old African American, fully vaccinated girl with a past medical history of febrile seizures presents to the general pediatric ward with altered mental status (AMS), ataxia, and ophthalmoplegia. Over the course of 5 days before admission, she developed bilateral otalgia with upper respiratory symptoms, fever, headache, neck pain, and fatigue. Her mother denies that the patient has had any weight loss, hallucinations, seizures, significant family history, travel, insect bites, ingestions, undercooked or unpasteurized food, or known contacts with tuberculosis. On physical examination, she is febrile (103°F [39.4°C]) and tachycardic (126 beats/min) without tachypnea or blood pressure abnormalities. She is ill-appearing, sleepy, and irritable, but she arouses with stimulation and follows simple commands. Her pupils are 3 mm, symmetrical, and equally reactive to light. She has a wide-based gait and ophthalmoplegia characterized by bilateral disconjugate gaze with esotropia. She exhibits difficulty with forward flexion of her neck but otherwise normal range of motion. The rest of her cranial nerve examination, deep tendon reflexes, tone, strength, and bulk are normal. Her initial cerebrospinal fluid (CSF) analysis shows a white blood cell count of 470/μL (0.47 × 109/L) with 50% lymphocytes and 50% atypical mononuclear cells, red blood cell count of 110/μL (0.11 × 109/L), protein of 84 mg/dL (0.84 g/L), and glucose of 44 mg/dL (2.4 mmol/L) with concurrent serum glucose of 100 mg/dL (5.6 mmol/L). Her complete blood cell count is significant …

Purpose: The purpose of the article is to describe a novel case of idiopathic central nervous system inflammatory disease with bilateral human leukocyte antigen (HLA)-B27-positive anterior ...

Background Anti–N-Methyl-D-Aspartate receptor (NMDAR) encephalitis is an autoimmune disorder that often affects women of childbearing age, and maternal-fetal transfer of anti-NMDAR antibodies during pregnancy has been documented in both symptomatic and asymptomatic women. The effects of these antibodies on the fetus, however, are incompletely understood. Patient Description This term infant exhibited depressed respiratory effort, poor feeding, and abnormal movements after birth. Magnetic resonance imaging revealed diffuse cerebral edema with ischemic and hemorrhagic injury. Her mother had experienced anti-NMDAR encephalitis secondary to an ovarian teratoma 18 months earlier. The baby's serum NMDAR antibody titer was elevated at 1:320. Intravenous immunoglobulin did not result in clinical improvement, and care was withdrawn on day of life 20. Her mother had an elevated serum NMDAR antibodies (1:80), positive CSF antibody titers, and a new ovarian teratoma. Conclusion Routine testing of NMDAR antibodies in pregnant women with a previous history of anti-NMDAR encephalitis may be warranted. Infants born to these mothers should be closely monitored throughout pregnancy and after birth.

Background Idiopathic inflammatory myopathies (IIM) are a group of systemic autoimmune disorders primarily affecting skeletal muscle. Pediatric coronary artery dilation is frequently discussed in Kawasaki disease. However, it has yet to be reported in the IIMs or antisynthetase syndrome. We report a unique case of a patient with IIM, antisynthetase syndrome and coronary artery dilation. Case presentation We report an adolescent presenting with joint symptoms, fever, and eye swelling with a clinical diagnosis of Juvenile Dermatomyositis. He subsequently developed diastolic hypotension with evidence of coronary artery dilation. He received steroids and immunoglobulin and followed by immunosuppressants with mild improvement in his symptoms. The adolescent later developed dyspnea and cough with CT lungs evident for cystic changes; lung biopsy showed interstitial fibrosis and inflammation, and muscle biopsy was abnormal as well. The anti-pl-12 antibody was positive. Following several weeks of treatment, an echocardiogram showed improvement in coronary artery dilation. His joint symptoms, muscle strength and respiratory symptoms have also improved. Conclusions Coronary artery dilation is not well understood in IIMs or antisynthetase syndrome. Pathobiology of coronary artery involvement, its treatment and prognosis, and association with IIM and antisynthetase syndrome needs further exploration.

Pediatric pulmonologists are frequently consulted on the child with “too many” respiratory infections. Considering the volume of air, particulates, chemicals, and potential airborne pathogens inspired with each breath, the question should actually be “Why don't we get more infections?” The large surface area of the conducting airways and alveolar surfaces (>70 m 2 in the adult) that is available for these particles to deposit poses a great challenge for the lung defenses. The anatomy of the lung, lung products, cell receptors (and signaling mechanisms), and host cellular responses all contribute to the normal lung defense and repair. A defect in any of these defenses can result in an increased susceptibility to infection in the absence of “classic” immunodeficiency. In this chapter, we will review defenses that have evolved in the lung. In this chapter, we discuss three major categories of lung defense: intrinsic defenses (airway anatomy, physiology, secretory products that trap inhaled particles), innate defenses (various pattern recognition receptors [PRRs] and secreted proteins that provide first line antimicrobial and proinflammatory responses), and adaptive responses (cellular and antibody immunity). Finally, we will discuss cellular processes that help control inflammatory responses and allow repair.

Postural orthostatic tachycardia syndrome and neurocardiogenic syncope are clinical manifestations of autonomic nervous system dysfunction (dysautonomia) that can lead to impaired daily functions. We report two young patients presenting with dysautonomia and autoimmune disease who both received autologous adipose stem cells (ASCs) infusions. This report is the first description of ASCs therapy for patients with combined dysautonomia and autoimmune disease. Case 1: A 21-year-old female presented at 12 years of age with escalating severe dysautonomia with weight loss and gastrointestinal symptoms. She had elevated autoantibodies and cytokines and received multiple immune modulation therapies. Her dysautonomia was treated by volume expanders, vasoconstrictors, and beta blockers with mild improvement. She received ASCs about 2 years before this report with dramatic improvement in her dysautonomia and autoimmune symptoms with a 10 kg weight gain. Case 2: A 7-year-old boy presented at 2 years of age with polyarthritis. At 5 years of age, he manifested orthostatic intolerance. He received immune modulatory therapies with mild improvement. He received ASCs and showed marked improvement of his dysautonomia and immune symptoms. Dysautonomia symptoms of these two patients improved significantly after modulation of autoimmune components by ASC therapy. Favorable clinical responses of these two cases warrant further case-control studies.

Purpose: To describe four cases of orbital inflammatory syndrome (OIS) with associated anterior uveitis that have presented within 2 years to our practice.Methods: Charts of patients diagnosed with OIS from June 2013 to May 2015 were reviewed.Results: Four patients, three children and one adult, presented with orbital swelling, pain, and varying degrees of vision loss. Treatment with intravenous methylprednisolone resulted in significant symptomatic improvement in all cases initially; when symptoms recurred, the patients had evidence of anterior uveitis. With continued systemic therapy and the addition of topical prednisolone, the patients all achieved control of their uveitis and OIS and are well controlled with regular outpatient follow-up.Conclusions: Reports of OIS-associated with uveitis are relatively rare. The presentation of three pediatric patients and one adult patient to the same practice with OIS and secondary uveitis within a 2-year period may indicate that the association is underrep...

Outcomes in children with proliferate lupus nephritis (PLN) show 9-15% progress to end-stage renal disease (ESRD) at 5 years. Immunosuppression improves outcome, but significant side effects are possible. Clinical and laboratory analyses are poor predictors of class and progression in PLN. We describe 28 patients with systemic lupus erythematosus (SLE), between 1990 and 2005, whose initial biopsy (Bx1) showed PLN and who received nine monthly doses of intravenously administered cyclophosphamide (CYP) (500-750 mg/m(2) up to 1 g to maintain their absolute neutrophil count (ANC) > 3,000). Continued therapy with additional quarterly intravenous (i.v). administration of CYP was dictated by repeat renal biopsy (Bx2). Bx1 was done 1 +/- 1.6 years after diagnosis of SLE. Bx2 showed histological improvement by WHO classification in 20/25 children; 3/25 were unchanged, 1/25 was categorized as new class V, and 1/25 was worse. Four patients (14%) had infectious complications requiring hospitalization (one of these died). Mean follow-up (f/u) after Bx2 was 3.5 +/- 2.3 years. At last follow-up, 26 patients had normal glomerular filtration rate (GFR), with a mean of 126 +/- 42.8 ml/min per 1.73 m(2) body surface area, one non-compliant patient had ESRD, and one had chronic renal failure. At last follow-up, most patients had minimal to no proteinuria. Clinical and biopsy results greatly improved after 9 monthly intravenously administered CYP pulses in most children with class IV PLN. Those who did not improve are at risk for flares and progression of disease. The tailoring of therapies based on findings from a biopsy after induction may improve outcomes.

Congenital indifference to pain is a rare and debilitating congenital disease. Individuals with the disorder may have one or a combination of sensory or autonomic deficits, which can range from lack of mechanical nociception, diminished ability to detect heat and cool stimulation, to the devastating and fatal form which includes autonomic dysfunction. It is important for radiologists to be able to recognize the radiographic presentations of this rare disorder, as delay in diagnosis can lead to extensive and sometimes unnecessary workup. We present a case of congenital indifference to pain initially interpreted as a mass of the distal femur.