Omphalitis

Muniraman H, et al Disorders of the Umbilical Cord. Pediatr Rev. 2018 Jul;39(7):332-341.

The umbilical cord, a vital conduit between the placenta and the fetus, loses much of its significance after birth. However, newborns can often present with various abnormalities of the umbilicus, such as benign granulomas or more serious lesions due to persistent remnants, many of which can change the normal course of cord separation and may be associated with significant morbidities if left unrecognized and uncorrected. Although not uncommon, sanguineous drainage from the umbilical stump can be quite alarming to new parents. Parental counseling regarding normal umbilical cord changes, as well as abnormal findings, such as discharge and skin changes, are important for the recognition and timely treatment of potentially significant umbilical cord disorders.

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Painter K, Feldman J. Omphalitis. 2018 Jun 27. StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2018 Jan-.

Omphalitis is an infection of the umbilicus and/or surrounding tissues, occurring primarily in the neonatal period. It is a true medical emergency that can rapidly progress to systemic infection and death, with an estimated mortality rate between 7% and 15%. Early recognition and treatment are essential to prevent the morbidity and mortality associated with omphalitis.

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Orbital cellulitis and subperiosteal orbital abscess

Wong SJ, Levi J. Management of pediatric orbital cellulitis: A systematic review. Int J Pediatr Otorhinolaryngol. 2018 Jul;110:123-129.

RESULTS: Pre-septal and post-septal cellulitis can generally be managed non-surgically, while orbital abscess and cavernous sinus thrombosis are managed surgically. For subperiosteal abscess, non-surgical medical management has been successful in certain patients. Results of the literature review were summarized, and subsequently developed into a comprehensive algorithm for management, including criteria for age, location, and volume of abscess on imaging.

CONCLUSIONS: Orbital cellulitis, particularly subperiosteal abscesses, in children is not an absolute indication for immediate surgical intervention. Conservative measures can be safe and effective if appropriately used, depending on patient characteristics, clinical course, and imaging.

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Transfusion-related acute lung injury (TRALI)

Cho MS, Sharma S. Transfusion, Acute Lung Injury. 2018 Jun 14. StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2018 Jan-.

Diagnostic criteria for TRALI is if the symptoms develop during or within 6 hours of transfusion without any risk factors for developing acute lung injuries such as sepsis from pneumonia, aspiration, and shock. Physical symptoms include fever, hypotension, and tachycardia. Clinical findings include exudative bilateral infiltrates on chest radiograph, no evidence of pulmonary vascular overload, and hypoxemia of SpO2 less than 90% on room air with a ratio of partial pressure of oxygen to a fractional inspired oxygen concentration of less than 300 mmHg. Possible TRALI is when there are other risk factors for acute lung injury. Delayed TRALI is when transfusion is completed after 6 to 72 hours, and it is associated with higher mortality. Transfusion-related circulatory overload (TACO) needs to be ruled out as it can be on differential diagnosis due to the similarity of pulmonary edema picture, but due to actual volume overload.

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Oakley FD, et al. Transfusion reactions in pediatric compared with adult patients: a look at rate, reaction type, and associated products. Transfusion. 2015 Mar; 55(3):563-70.

CONCLUSION: To our knowledge this is the first study to provide detailed comparisons of acute transfusion reactions to all blood products between pediatric and adult populations at a single institution and supported by a single transfusion service and culture. Collectively these data provide insight into pediatric transfusion reactions and demonstrate a general increase in the incidence of transfusion reactions within the pediatric compared to adult population.

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Suprasellar tumors (with focus on pituitary adenomas)

McCrea HJ, et al. Pediatric Suprasellar Tumors. J Child Neurol. 2016 Oct;31(12):1367-76.

The various childhood suprasellar tumors, while pathologically distinct, present similar clinical and surgical challenges as a result of their common anatomic location. These lesions are in close proximity to or may invade the optic nerve and chiasm, pituitary gland and infundibulum, hypothalamus, and third ventricle, leading to presenting features including visual field loss, impairment in visual acuity, endocrine dysfunction, and hydrocephalus. Though many suprasellar lesions are relatively benign in pathology, treatment may be complicated by high surgical morbidity resulting from damage to the hypothalamic-pituitary axis. Here we review the most frequent pediatric lesions occurring in the suprasellar region: craniopharyngioma, chiasmatic glioma, germ cell tumor, Rathke cleft and arachnoid cysts, pituitary adenoma, and histiocytosis. This review outlines both common presenting features and differentiating aspects of these lesions. It also includes classic radiographic presentations and treatment considerations for each lesion.

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Tubulointerstitial nephritis

Joyce E, et al. Tubulointerstitial nephritis: diagnosis, treatment, and monitoring. Pediatr Nephrol. 2017 Apr;32(4):577-587.

Tubulointerstitial nephritis (TIN) is a frequent cause of acute kidney injury (AKI) that can lead to chronic kidney disease (CKD). TIN is associated with an immune-mediated infiltration of the kidney interstitium by inflammatory cells, which may progress to fibrosis. Patients often present with nonspecific symptoms, which can lead to delayed diagnosis and treatment of the disease. Etiology can be drug-induced, infectious, idiopathic, genetic, or related to a systemic inflammatory condition such as tubulointerstitial nephritis and uveitis (TINU) syndrome, inflammatory bowel disease, or immunoglobulin G4 (IgG4)-associated immune complex multiorgan autoimmune disease (MAD). It is imperative to have a high clinical suspicion for TIN in order to remove potential offending agents and treat any associated systemic diseases. Treatment is ultimately dependent on underlying etiology. While there are no randomized controlled clinical trials to assess treatment choice and efficacy in TIN, corticosteroids have been a mainstay of therapy, and recent studies have suggested a possible role for mycophenolate mofetil. Urinary biomarkers such as alpha1- and beta2-microglobulin may help diagnose and monitor disease activity in TIN. Screening for TIN should be implemented in children with inflammatory bowel disease, uveitis, or IgG4-associated MAD.

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Staphylococcal scalded skin syndrome

Leung AKC, et al. Staphylococcal-scalded skin syndrome: evaluation, diagnosis, and management. World J Pediatr. 2018 Apr;14(2):116-120.

SSSS is caused by toxigenic strains of Staphylococcus aureus. Hydrolysis of the amino-terminal extracellular domain of desmoglein 1 by staphylococcal exfoliative toxins results in disruption of keratinocytes adhesion and cleavage within the stratum granulosum which leads to bulla formation. The diagnosis is mainly clinical, based on the findings of tender erythroderma, bullae, and desquamation with a scalded appearance especially in friction zones, periorificial scabs/crusting, positive Nikolsky sign, and absence of mucosal involvement. Prompt empiric treatment with intravenous anti-staphylococcal antibiotic such as nafcillin, oxacillin, or flucloxacillin is essential until cultures are available to guide therapy. Clarithromycin or cefuroxime may be used should the patient have penicillin allergy. If the patient is not improving, critically ill, or in communities where the prevalence of methicillin-resistant S. aureus is high, vancomycin should be used.

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The use of steroids for post-extubation stridor

Khemani RG, Randolph A, Markovitz B. Corticosteroids for the prevention and treatment of post-extubation stridor in neonates, children and adults. Cochrane
Database Syst Rev. 2009 Jul 8;(3):CD001000. Edited/Substantively amended: 04 June 2014; Edited (no change to conclusions) this issue.

AUTHORS’ CONCLUSIONS: Using corticosteroids to prevent (or treat) stridor after extubation has not proven effective for neonates or children. However, given the consistent trends towards benefit, this intervention does merit further study, particularly for high risk children or neonates. In adults, multiple doses of corticosteroids begun 12-24 hours prior to extubation do appear beneficial for patients with a high likelihood of post extubation stridor.

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Neuromyelitis optica

Tenembaum S, et al. Neuromyelitis optica spectrum disorders in children and adolescents. Neurology. 2016 Aug 30;87(9 Suppl 2):S59-66.

Neuromyelitis optica (NMO) is a severe autoimmune disease of the CNS characterized by recurrent inflammatory events primarily involving the optic nerves and spinal cord. NMO is infrequent in children, but early recognition is important to start adequate treatment. In this article, we review the evolving diagnostic criteria of NMO and provide an update on the clinical and neuroimaging spectrum of the disorder in pediatric patients, including current knowledge on immunopathogenesis and treatment recommendations for children with NMO.

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Eczema herpeticum

Hsu DY, et al. Epidemiology of Eczema Herpeticum in Hospitalized U.S. Children: Analysis of a Nationwide Cohort. J Invest Dermatol. 2018 Feb; 138(2):265-272.

Eczema herpeticum (EH) is characterized by viral infection of a preexisting inflammatory dermatosis and is associated with considerable morbidity. In this cross-sectional study, we sought to determine the incidence, risk factors, comorbidities, costs of care, length of stay, and mortality in hospitalized U.S. children with EH.

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Blanter M, et al. Eczema herpeticum: would you know it if you saw it? Pediatr Emerg Care. 2015 Aug;31(8):586-8.

Eczema herpeticum is an easily missed entity most commonly seen in the pediatric population and carries the risk of systemic compromise and a 10% mortality rate. Clinicians should maintain high clinical suspicion when encountering children or young adults with a history of atopic dermatitis or other erosive dermatoses and who present with vesicular lesions, punched-out erosions, and systemic symptoms. We present 3 severe cases of eczema herpeticum that were potentially overlooked and demonstrate the need for elevated awareness to avoid potential pitfalls.

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Article of interest: Corticosteroids for the treatment of Kawasaki disease in children.

Wardle AJ, et al. Corticosteroids for the treatment of Kawasaki disease in children. Cochrane Database Syst Rev. 2017 Jan 27;1:CD011188.

AUTHORS’ CONCLUSIONS: Moderate-quality evidence shows that use of steroids in the acute phase of KD can be associated with improved coronary artery abnormalities, shorter duration of hospital stay and a decreased duration of clinical symptoms. High-quality evidence shows reduced inflammatory marker levels. There were insufficient data available regarding incidence of adverse effects attributable to steroids, mortality and long-term (> 1 year) coronary morbidity. Certain groups, including those based in Asia, those with higher risk scores, and those receiving longer steroid treatment may have greater benefit from steroid use, especially with decreasing rates of heart problems, but more tests are needed to answer these questions. Evidence presented in this study suggests that treatment with a long course of steroids should be considered for all children diagnosed with KD until further studies are performed.

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See also: Incomplete and atypical Kawasaki disease.