Refractory Henoch-Schönlein purpura

Lei WT, et al. Incidence and risk factors for recurrent Henoch-Schönlein purpura in children from a 16-year nationwide database. Pediatr Rheumatol Online J. 2018 Apr 16;16(1):25.

Ours was the first study to describe the incidence of recurrent HSP, and added evidence of increased associations of recurrent HSP and allergic rhinitis, renal involvement, and steroid treatment for > 10 days. These results can be considered observations from real-world conditions. Patients with the aforementioned clinical features were suggested to receive longer periods of follow-up than previously suggested for the early identification and management of recurrent HSP. In those with a second HSP episode, perhaps 6 more months of monitoring is also suggested for the early recognition of a third HSP recurrence. Further study is needed to clarify the underlying pathogenic mechanisms of these associations.

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Alfredo CS, et al. Henoch-Schönlein purpura: recurrence and chronicity. J Pediatr (Rio J). 2007 Mar-Apr;83(2):177-80.

“Our data reiterates the need for periodic follow-up of all children with HSP, especially in cases with renal involvement, such as, for example, those with persistent hematuria and proteinuria or arterial hypertension. Urinary sediments should be monitored, even in asymptomatic patients. Referral to a specialist (pediatric rheumatologist or nephrologist) is recommended in cases where presentation is atypical.”

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Pertussis leukocytosis

Palvo F, et al. Severe pertussis infection: A clinicopathological study. Medicine (Baltimore). 2017 Dec;96(48):e8823.

We aimed to investigate the cliniopathological features of pertussis in children admitted to a tertiary-care university hospital in Brazil. This was a retrospective cohort study of all pediatric hospital admissions with pertussis from January 1, 2008 to December 31, 2014. We also reported the autopsy findings in children who died. Fifty-five patients admitted to the hospital over the study period had laboratorial confirmation of Bordetella pertussis infection, 17 (30.9%) needed pediatric intensive care unit (PICU) admission and 6 (10.9%) died. All patients who died were younger than 60 days old and unvaccinated for pertussis; 50% of them had coinfection with respiratory syncytial virus. Leukocyte count ≥40,000/mm at hospital admission was an independent risk factor for PICU admission. Mean heart rate during hospitalization ≥160 bpm was an independent risk factor for death. A cut-off point of 41,200 leukocytes/mm at hospital admission had sensitivity of 64.7% and specificity of 89.5% to predict PICU admission (area under the curve 0.75) and sensitivity of 100% and specificity of 81.6% to predict death (area under the curve 0.93). Autopsy showed medial thickening of small pulmonary arteries in 80% of patients who had pulmonary hypertension; intravascular aggregates of leukocytes or pulmonary thrombosis were not observed. Immunohistochemical staining of tissue samples obtained at autopsy identified B pertussis and respiratory syncytial virus in pulmonary and extra-pulmonary sites. Marked leukocytosis at presentation was associated with morbidity and mortality in children hospitalized with pertussis. Implementation of preventive strategies is crucial to diminish the incidence of the disease, especially in young unimmunized infants.

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Post-exposure prophylaxis of pertussis

Chapter 129: Pertussis and Other Bordetella Infections in Feigin and Cherry’s Textbook of Pediatric Infectious Diseases, 8th ed. (2019), pg.1175.

Pertussis: Isolation and Prophylactic Measures

“Erythromycin, azithromycin, or clarithromycin treatment in the index case shortens the duration of communicability of the organisms and limits spread of the disease. During the first few days of treatment, contact with susceptible persons should be avoided. Close contacts (e.g., household members, those in childcare centers, playmates) of the index case should be protected from infection. Protection can be implemented by the prophylactic use of erythromycin for 14 days, azithromycin for 5 days, or clarithromycin for 7 days. [9,80,367,522] Active immunization of all exposed persons (i.e., children, adolescents, and adults) who are not adequately vaccinated should be conducted.

The use of prophylactic antibiotics in adolescents and adults in exposure situations such as classrooms and hospital settings frequently is recommended. This approach often involves many people and considerable expense.

Because of the side effects of erythromycin and other macrolides, adult compliance is poor. These antibiotics should not be used prophylactically in large group settings but should be used for treatment only at the first sign of respiratory illness in those exposed.”

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Woo TM. Postexposure Management of Vaccine-Preventable Diseases. J Pediatr Health Care. 2016 Mar-Apr;30(2):173-82; quiz 183-4

“Prophylactic antibiotic treatment is recommended for all household and other close contacts of the index case, regardless of their immunization status. Close contacts are defined by the AAP (2015e) recommendations as children in day care; face-to-face exposure within 3 feet of the index case; direct contact with nasal or respiratory secretions of the infected person; or sharing close or confined space for 1 hour or longer with the index case. Postexposure prophylaxis should be provided within 21 days of exposure to persons at high risk of developing severe disease or who will have close contact with a person at risk of developing severe disease ( CDC, 2015e ). Persons considered high risk for developing severe disease include infants and patients with health conditions that would be worsened by pertussis, such as moderate to severe asthma or immunocompromised persons. Persons who are exposed to high-risk patients, particularly infants and pregnant women in the third trimester of pregnancy, should be given prophylaxis treatment. Providers need to balance the health and safety of high-risk patients with the potential for overuse of antibiotics. Table 1 provides recommendations for postexposure prophylaxis.”

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Nesterova G, Gahl WA. Cystinosis. 2001 Mar 22 [updated 2017 Dec 7]. In: Adam MP, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2018.

CLINICAL CHARACTERISTICS: Cystinosis comprises three allelic phenotypes: Nephropathic cystinosis in untreated children is characterized by renal Fanconi syndrome, poor growth, hypophosphatemic/calcipenic rickets, impaired glomerular function resulting in complete glomerular failure, and accumulation of cystine in almost all cells, leading to cellular dysfunction with tissue and organ impairment. The typical untreated child has short stature, rickets, and photophobia. Failure to thrive is generally noticed after approximately age six months; signs of renal tubular Fanconi syndrome (polyuria, polydipsia, dehydration, and acidosis) appear as early as age six months; corneal crystals can be present before age one year and are always present after age 16 months. Prior to the use of renal transplantation and cystine-depleting therapy, the life span in nephropathic cystinosis was no longer than ten years. With these interventions, affected individuals can survive at least into the mid-forties or fifties with satisfactory quality of life. Intermediate cystinosis is characterized by all the typical manifestations of nephropathic cystinosis, but onset is at a later age. Renal glomerular failure occurs in all untreated affected individuals, usually between ages 15 and 25 years. The non-nephropathic (ocular) form of cystinosis is characterized clinically only by photophobia resulting from corneal cystine crystal accumulation.

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JAMAevidence: Does This Patient Have Infectious Mononucleosis?

Does This Patient Have Infectious Mononucleosis?

Listen on iTunes to an interview with Dr. Christina Minami from JAMAevidence The Rational Clinical Examination: Using Evidence to Improve Care.

Instructions: To listen to an audio podcast, mouse over the title and click Play. Open iTunes to download and subscribe to podcasts.

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Blood coagulation disorders

Rodriguez V, Warad D. Pediatric Coagulation Disorders. Pediatr Rev. 2016

After completing this article, the reader should be able to:

  1. Describe the physiology of hemostasis in the pediatric patient.
  2. List clinical signs and symptoms suggestive of a congenital or acquired bleeding disorder.
  3. Understand laboratory testing and indications in the diagnosis of a bleeding disorder and timing of subspecialty referral.
  4. Describe the clinical management of bleeding disorders.
  5. Become familiar with congenital and acquired thrombophilic disorders and their diagnosis and management recommendations.

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Juvenile idiopathic arthritis

Shenoi S. Juvenile Idiopathic Arthritis – Changing Times, Changing Terms, Changing Treatments. Pediatr Rev. 2017 May;38(5):221-232.

After completing this article, readers should be able to:

  1. Recognize the clinical findings associated with the various categories of juvenile idiopathic arthritis (JIA).
  2. Recognize the laboratory findings associated with the different categories of JIA and its complications.
  3. Formulate a differential diagnosis for children with joint pain.
  4. Recognize the long-term complications associated with JIA.
  5. Plan the appropriate management of JIA while recognizing adverse effects of some therapies.

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Article of interest: TGA and coarctation of the aorta in an infant presenting with bronchiolitis…

Al-Mukhaini KS, Mohamed AM. Transposition of the Great Arteries and Coarctation of the Aorta in an Infant Presenting with Bronchiolitis: An incidental finding. Sultan Qaboos Univ Med J. 2017 Aug;17(3):e348-e351.

The transposition of the great arteries (TGA) is a complex congenital heart disease which usually presents as cyanosis in neonates with limited mixing between the systemic and pulmonary circulatory systems. A delayed diagnosis of TGA can lead to ventricular failure. We report a six-week-old infant who was admitted to the paediatric Intensive Care Unit of the Royal Hospital, Muscat, Oman, in 2016 for bronchiolitis. During admission, she was incidentally diagnosed with TGA and coarctation of the aorta. Postnatal screening, including the use of pulse oximetry, plays a significant role in avoiding a late diagnosis of TGA; however, this screening tool is not widely used in Oman. Moreover, the common practice of applying a pulse oximetry probe only to the foot may have been limited in the current case due to the reverse differential saturation between the upper and lower limbs caused by this particular combination of lesions.

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Yap SH, et al. Reversed differential cyanosis in the newborn: a clinical finding in the supracardiac total anomalous pulmonary venous connection. Pediatr Cardiol. 2009 Apr;30(3):359-62.

The newborn can experience two types of differential cyanosis (DC). The common type of DC occurs when oxygen saturation in the right hand is greater than in the foot. The second type of DC, reversed differential cyanosis (RDC), occurs when oxygen saturation is lower in the right hand than in the foot. This phenomenon is observed in transposition of the great arteries (TGA) with patent ductus arteriosis (PDA) and elevated pulmonary vascular resistance or in TGA with PDA and preductal aortic interruption or coarctation. This report describes a case of RDC not previously described involving an infant with supracardiac total anomalous pulmonary venous connection (TAPVC). In supracardiac TAPVC, RDC results from streaming of highly saturated superior vena cava (SVC) blood into the right ventricle, out the main pulmonary artery, through a PDA, and to the descending aorta, with streaming of more desaturated blood from the inferior vena cava (IVC) into the left atrium across the atrial septal defect (ASD)/foramen ovale. Therefore, as part of a neonatal examination to rule out congenital heart disease (CHD), simultaneous pre- and postductal oxygen saturations should be documented. The presence of RDC should initiate immediate full cardiac evaluation for CHD. Supracardiac TAPVC should be included in the differential diagnosis if RDC is observed.

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Articles of interest: Diagnosing pneumonia

Shah SN, et al. Does This Child Have Pneumonia?: The Rational Clinical Examination Systematic Review. JAMA. 2017 Aug 1;318(5):462-471. Erratum in: JAMA. 2017 Oct 3;318(13):1284.

Bottom Line: Among children with cough or fever, the general appearance of the patient and the oxygen saturation level appear to be key determinants in evaluating for the presence of pneumonia. Clinical observations such as hypoxemia and increased work of breathing (grunting, nasal flaring, and retractions) outweigh the importance of tachypnea and auscultatory findings in the diagnosis of pneumonia. In settings where chest radiography is not readily available, increased work of breathing and hypoxemia can be used to identify children with pneumonia. In settings where pneumonia is commonly diagnosed radiographically, these clinical findings can be used to guide judicious use of chest radiography. Future research should evaluate the predictive value of combining clinical features and pulse oximetry among children with suspected pneumonia.

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Article of interest: Cerebrospinal Fluid Reference Values for Young Infants Undergoing Lumbar Puncture.

Thomson J, et al. Cerebrospinal Fluid Reference Values for Young Infants Undergoing Lumbar Puncture. Pediatrics. 2018 Feb 2. pii: e20173405.

“In this study, we establish age-specific reference values for CSF WBC counts, CSF protein concentrations, and CSF glucose concentrations in young infants. We determined that infants ≤28 days of age have higher CSF WBC counts, higher CSF protein concentrations, and lower CSF glucose concentrations than infants 29 to 60 days of age. We also illustrate the age-related decline in CSF WBC counts and protein concentrations and the age-related increase in CSF glucose concentrations over the first 2 months of life.”

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