Hyponatremia in Kawasaki disease

Lim GW, et al. Hyponatremia and syndrome of inappropriate antidiuretic hormone secretion in kawasaki disease. Korean Circ J. 2010 Oct;40(10):507-13.

The pathogenesis of hyponatremia (serum sodium <135 mEq/L) in Kawasaki disease (KD) remains unclear. We investigated the clinical significance of hyponatremia, and the role of interleukin (IL)-6 and IL-1β in the development of hyponatremia and syndrome of inappropriate antidiuretic hormone secretion (SIADH) in KD.

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Kaneko K, et al. Immunoglobulin preparations affect hyponatremia in Kawasaki disease. Eur J Pediatr. 2010 Aug;169(8):957-60.

Hyponatremia frequently occurs in Kawasaki disease (KD). The aim of this study was to investigate the effect of Na content of the intravenous immunoglobulin (IVIG) preparation on serum Na levels in KD. Seventy-eight subjects, of whom 27 had hyponatremia, were split up into two groups: group A receiving IVIG preparations containing high Na (0.9%) and group B receiving IVIG preparations containing trace Na. While the data before IVIG therapy revealed no significant differences in the median serum Na between the groups, an administration of IVIG preparations increased the serum levels of Na in group A (P < 0.01) but not in group B (P > 0.05). Furthermore, the median serum Na level was significantly higher in group A than that in group B (139.0 vs 137.0 mEq/L, respectively, P < 0.01). No significant difference was found in the prevalence of coronary artery lesions between the groups. In conclusion, we should keep it in mind that the IVIG products without Na have an adverse affect on hyponatremia in KD though their efficacy seems to be equivalent to those containing high Na.

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Somatic symptom disorders

Malas N, et al. Pediatric Somatic Symptom Disorders. Curr Psychiatry Rep. 2017 Feb;19(2):11.

Somatic symptom disorder (SSD) is a common disorder encountered in pediatric medicine. It involves the presentation of physical symptoms that are either disproportionate or inconsistent with history, physical examination, laboratory, and other investigative findings. SSDs result in significant impairment with considerable increase in healthcare utilization, school absenteeism, and the potential for unnecessary diagnostic evaluation and treatment intervention. Patients and families often feel dismissed and may worry that a serious condition has been missed. Primary care providers are frequently frustrated due to a lack of a successful approach to patients and families impacted by SSD. The result is often a cycle of disability, frustration and missed opportunities for collaboration towards enhanced patient functionality. This review summarizes the current evidence-based understanding, as well as insights from clinician experience, on the evaluation and management of pediatric SSD.

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Evaluation of abnormal liver function tests

Lamireau T, et al. A practical approach to the child with abnormal liver tests. Clin Res Hepatol Gastroenterol. 2014 Jun;38(3):259-62.

The presence of elevated aminotransferases on routine blood tests can reveal liver diseases of various severities. In children, etiologies are more numerous and complex than those usually considered in adults. Information derived from family and personal history, physical examination and basic laboratory data are necessary to reach a timely and correct diagnosis. A stepwise approach is proposed to guide the timing of more specific investigations that are often required.

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Autoimmune hemolytic anemia

Liebman HA, Weitz IC. Autoimmune Hemolytic Anemia. Med Clin North Am. 2017 Mar;101(2):351-359.

Autoimmune hemolytic anemia is an acquired autoimmune disorder resulting in the production of antibodies directed against red blood cell antigens causing shortened erythrocyte survival. The disorders can present as a primary disorder (idiopathic) or secondary to other autoimmune disorders, malignancies, or infections. Treatment involves immune modulation with corticosteroids and other agents.

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Sankaran J, et al. Autoimmune Hemolytic Anemia in Children: Mayo Clinic Experience. J Pediatr Hematol Oncol. 2016 Apr;38(3):e120-4.

We studied 35 pediatric patients with autoimmune hemolytic anemia seen at Mayo Clinic from 1994 to 2014. The median age was 10.0 years and 65.7% were males. Most had warm antibodies (80.0%) and some secondary to viral (14.3%) or autoimmune disorders (31.4%). Seven (20.0%) patients presented with Evans syndrome, 3 of whom also had common variable immunodeficiency. The median hemoglobin at diagnosis was 6.1 g/dL and 62.8% patients required red cell transfusions. The severity of anemia was worse among children below 10 years (median 5.5 vs. 7.0 g/dL, P=0.01). Steroid was the initial treatment for 88.5% patients, with overall response rate of 82.7% (68.5% complete, 14.2% partial) and median response duration of 10.7 months (range, 0.2 to 129.7+ mo). After median follow-up of 26.6 months, 8 (22.8%) patients relapsed. Salvage treatments included splenectomy, intravenous immunoglobulin, rituximab, and mycophenolate mofetil. Infectious complications occurred in 9 (25.7%) patients and 1 patient died of cytomegalovirus infection. Four patients had cold agglutinin disease and 3 (75.0%) responded to steroids. Autoimmune hemolytic anemia is a rare disorder in pediatric population and most respond well to steroids regardless of the type of antibody. Infectious complications are common and screening for immunodeficiency is recommended among those with Evans syndrome.

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Polymicrogyria

Shain C, et al. Polymicrogyria-associated epilepsy: a multicenter phenotypic study from the Epilepsy Phenome/Genome Project. Epilepsia. 2013 Aug;54(8):1368-75.

Polymicrogyria (PMG) is an epileptogenic malformation of cortical development. We describe the clinical epilepsy and imaging features of a large cohort with PMG-related epilepsy.

Participants with PMG had both focal and generalized onset of seizures. Our data confirm the involvement of known topographic patterns of PMG and suggest that more extensive distributions of PMG present with an earlier age of seizure onset and increased prevalence of developmental delay prior to seizure onset.

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Barkovich AJ. Current concepts of polymicrogyria. Neuroradiology. 2010 Jun;52(6):479-87.

Polymicrogyria is one of the most common malformations of cortical development. It has been known for many years and its clinical and MRI manifestations are well described. Recent advances in imaging, however, have revealed that polymicrogyria has many different appearances on MR imaging, suggesting that is may be a more heterogeneous malformation than previously suspected. The clinical and imaging heterogeneity of polymicrogyria is explored in this review.

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Concurrent viral infections and Kawasaki disease

Song E, et al. Clinical and virologic characteristics may aid distinction of acute adenovirus disease from Kawasaki disease with incidental adenovirus detection. J Pediatr. 2016 Mar;170:325-30.

Incidental adenovirus detection in Kawasaki disease (KD) is important to differentiate from acute adenovirus disease. Twenty-four of 25 children with adenovirus disease and mimicking features of KD had <4 KD-like features, predominance of species B or E, and higher viral burden compared with those with KD and incidental adenovirus detection.

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Turnier JL, et al. Concurrent respiratory viruses and Kawasaki disease. Pediatrics. 2015 Sep;136(3):e609-14.

“Overall, our study supports earlier evidence that a large number of patients with KD have respiratory symptoms and evidence of viral nucleic acid in the nasopharynx. This study showed that a large percentage of patients with KD have a concurrent or recent history of respiratory viral infections and suggests that clinicians should not dismiss the diagnosis of KD based on the presence of respiratory or gastrointestinal symptoms or solely on the results of a positive respiratory viral PCR test. Furthermore, our data support the recommendation that a positive respiratory virus test result, regardless of the virus detected, should not be used to exclude the diagnosis of KD.4,16 Continued research is needed to elucidate the etiology and/or discover a more sensitive and specific diagnostic test for this important pediatric disease.”

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Lead poisoning

Dapul H, Laraque D. Lead poisoning in children. Adv Pediatr. 2014 Aug;61(1):313-33.

“The management of patients with lead exposure involves not only the pharmacologic management of toxicity, but also strategies for intervention and prevention of further exposure. Once an elevated lead level is found, the local health department should be notified and a home risk assessment should be performed to determine the need for abatement strategies. With the gradual lowering of the “BLL of concern” by the CDC, the threshold for action has decreased as well. The Pediatric Environmental Health Specialty Unit Network has made recommendations on further evaluation and/or intervention based on the BLL, as outlined in Table 6 [46] .”

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Acute Ataxia

Caffarelli M, Kimia AA, Torres AR. Acute Ataxia in Children: A Review of the Differential Diagnosis and Evaluation in the Emergency Department. Pediatr Neurol. 2016 Dec;65:14-30.

Acute ataxia in a pediatric patient poses a diagnostic dilemma for any physician. While the most common etiologies are benign, occasional individuals require urgent intervention. Children with stroke, toxic ingestion, infection, and neuro-inflammatory disorders frequently exhibit ataxia as an essential-if not the only-presenting feature. The available retrospective research utilize inconsistent definitions of acute ataxia, precluding the ability to pool data from these studies. No prospective data exist that report on patients presenting to the emergency department with ataxia. This review examines the reported causes of ataxia and attempts to group them into distinct categories: post-infectious and inflammatory central and peripheral phenomena, toxic ingestion, neurovascular, infectious and miscellaneous. From there, we synthesize the existing literature to understand which aspects of the history, physical exam, and ancillary testing might aid in narrowing the differential diagnosis. MRI is superior to CT in detecting inflammatory or vascular insults in the posterior fossa, though CT may be necessary in emergent situations. Lumbar puncture may be deferred until after admission in most instances, with suspicion for meningitis being the major exception. There is insufficient evidence to guide laboratory evaluation of serum, testing should be ordered based on clinical judgement-recommended studies include metabolic profiles and screening labs for metabolic disorders (lactate and ammonia). All patients should be reflexively screened for toxic ingestions.

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Thakkar K, Maricich SM, Alper G. Acute Ataxia in Childhood: 11-Year Experience at a Major Pediatric Neurology Referral Center. J Child Neurol. 2016 Aug;31(9):1156-60.

We categorized the causes of acute ataxia in the pediatric population-referred to the Division of Neurology-at a large, urban pediatric medical center. Of the 120 cases identified over the past 11 years, post-infectious cerebellar ataxia was the most commonly diagnosed (59%), followed by drug intoxication, opsoclonus-myoclonus ataxia syndrome, episodic ataxia, acute cerebellitis, cerebellar stroke, ADEM, meningitis, cerebral vein thrombosis, Leigh’s disease, Miller-Fisher syndrome, and concussion. Among the patients with post-infectious cerebellar ataxia, 85% were 1-6 years old and all had a history of antecedent viral illness. CSF pleocytosis was present in 40% of patients; all had normal brain MRIs. The majority (91%) recovered within 30 days. We conclude that post-infectious cerebellar ataxia remains the most common cause of acute ataxia in childhood and that it carries a good prognosis. We also differentiate acute post-infectious cerebellar ataxia from other causes with similar presentations.

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CNS abnormalities with Cornelia de Lange Syndrome

Deardorff MA, Noon SE, Krantz ID. Cornelia de Lange Syndrome. 2005 Sep 16 [Updated 2016 Jan 28]. In: Pagon RA, Adam MP, Ardinger HH, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2017.


Roshan Lal TR, et al. Cornelia de Lange syndrome: Correlation of brain MRI findings with behavioral assessment. Am J Med Genet C Semin Med Genet. 2016 Jun; 172(2):190-7.

“Neurobehavioral and developmental issues with a broad range of deficits are prominent features of Cornelia de Lange syndrome (CdLS), a disorder due to disruption of the cohesin protein complex. The etiologic relationship of these clinical findings to anatomic abnormalities on neuro-imaging studies has not, however, been established. Anatomic abnormalities in the brain and central nervous system specific to CdLS have been observed, including changes in the white matter, brainstem, and cerebellum. We hypothesize that location and severity of brain abnormalities correlate with clinical phenotype in CdLS, as seen in other developmental disorders.

Ten of fifteen (67%) of CdLS patients had abnormal findings on brain MRI, including cerebral atrophy, white matter changes, cerebellar hypoplasia, and enlarged ventricles. Other findings included pituitary tumors or cysts, Chiari I malformation and gliosis. Abnormal behavioral scores in more than one behavioral area were seen in all but one patient. All 5 of the 15 (33%) patients with normal structural MRI studies had abnormal ABC scores. All normal ABC scores were noted in only one patient and this was correlated with moderately abnormal MRI changes. Although our cohort is small, our results suggest that abnormal behaviors can exist in individuals with CdLS in the setting of relatively normal structural brain findings.”

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Fever of unknown origin (FUO) and fever without a source (FWS)

Definitions:

  • FUO – Children with fever >101ºF (38.3ºC) of at least eight days’ duration, in whom no diagnosis is apparent after initial outpatient or hospital evaluation that includes a careful history and physical examination and initial laboratory assessment.
  • FWS – Children with fever lasting for one week or less without adequate explanation after a careful history and thorough physical examination.

From UpToDate.


Antoon JW, et al. Pediatric fever of unknown origin. Pediatr Rev. 2015 Sep;36(9):380-90; quiz 391.

  • On the basis of strong clinical evidence, the causes of FUO are broad and include both benign and life-threatening medical conditions. (12)
  • On the basis of observational studies, most cases of FUO have shifted to noninfectious etiologies over the past several decades. (10)
  • On the basis of observational studies, completely normal physical examination findings at the time of the initial FUO evaluation suggest a benign underlying cause. (13)
  • On the basis of consensus and expert opinion, a stepwise, tiered approach to FUO should be implemented to decrease cost and time to diagnosis. (13)

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Cunha BA, ET AL. Fever of unknown origin: a clinical approach. Am J Med. 2015 Oct; 128(10):1138.e1-1138.e15.

Fevers of unknown origin remain one of the most difficult diagnostic challenges in medicine. Because fever of unknown origin may be caused by over 200 malignant/neoplastic, infectious, rheumatic/inflammatory, and miscellaneous disorders, clinicians often order non-clue-based imaging and specific testing early in the fever of unknown origin work-up, which may be inefficient/misleading. Unlike most other fever-of-unknown-origin reviews, this article presents a clinical approach. Characteristic history and physical examination findings together with key nonspecific test abnormalities are the basis for a focused clue-directed fever of unknown origin work-up.

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Arora R, Mahajan P. Evaluation of child with fever without source: review of literature and update. Pediatr Clin North Am. 2013 Oct;60(5):1049-62.

“Fever is one of the most common reasons for a visit to the primary care provider or the emergency department. Traditionally, clinicians have used various risk-stratification strategies to identify serious bacterial infections (SBI) without an obvious source in febrile children, because missed bacterial infections in such children can result in meningitis, sepsis, and death; therefore, early and accurate identification of SBIs is critical. Infants aged less than 60 to 90 days are at greatest risk of SBI. The epidemiology of SBI continues to evolve, especially after the successful introduction of conjugate vaccines against Streptococcus pneumoniae and Haemophilus influenzae.”

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