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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Neonatal hyperbilirubinemia

Itoh S, et al. Phototherapy for neonatal hyperbilirubinemia. Pediatr Int. 2017 May 31.

About 60 years ago in England, phototherapy for neonatal hyperbilirubinemia was actually used in clinical practice. It was introduced in Japan about 50 years ago. However, the mechanism of how the serum bilirubin concentration was decreased by the phototherapy was still unknown. The mechanism was identified by chemists, biochemists, and pediatricians. Clarification started with the report that unconjugated bilirubin was excreted into bile after the photoirradiation of Gunn rats. After the molecular conformation of bilirubin by X-ray analysis, the mechanism for bile excretion of unconjugated bilirubin was verified based on geometric configurational photoisomers in the Gunn rat. Finally, the reaction and excretion of structural bilirubin photoisomers was proved to be the main mechanism for the decrease in serum bilirubin during phototherapy for neonatal hyperbilirubinemina, which differs from the mechanism in the Gunn rat. However, the most effective and safest light source and the optimal method to evaluate phototherapy remain unknown. Moreover, as for bronze baby syndrome which is a well-known adverse reaction to phototherapy, the substances as the etiological origin are unclear. Hence, we review phototherapy for hyperbilirubinemia including a fundamental understanding of the bilirubin photochemical reactions, and discuss the subclinical carcinogenic risk of phototherapy and the increased mortality rate of extremely very low birth weight infants due to aggressive phototherapy, which is becoming a problem an increasing.

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Maisels MJ. Managing the jaundiced newborn: a persistent challenge. CMAJ. 2015
Mar 17;187(5):335-43.

“In this review, I present an approach to managing the jaundiced newborn that is based on published guidelines. [2-5] The aim is to help clinicians identify and manage jaundice in the newborn, intervene when appropriate and, when possible, prevent bilirubin-induced brain damage. It would be ideal if the published guidelines for the management of hyperbilirubinemia, including treatment with phototherapy and exchange transfusion, were based on estimates of when the benefit of these interventions exceeded their risks and costs. These estimates should come from randomized trials or high-quality, systematic observational studies, but such studies are rare. Guidelines must therefore rely on relatively uncertain estimates of risk and benefits, often from conflicting results.”

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Adverse events associated with antipsychotic medications

See also: Lithium toxicity.


Garcia G, Logan GE, Gonzalez-Heydrich J. Management of psychotropic medication side effects in children and adolescents. Child Adolesc Psychiatr Clin N Am. 2012
Oct;21(4):713-38.

“An evidence-based review of the published literature on the side effects of psychotropic medications on children and adolescents is provided. Due to the large scope of side effects, some of the most commonly presenting side effects are focused on and the rare but serious effects where controversy still exists around monitoring and management (eg, suicidality and metabolic syndrome) are highlighted. Emphasis is placed on the presentation of side effects, prevalence, treatment, and management of the adverse effects associated with psychotropic medication.”

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Complementary therapies of irritable bowel syndrome (focus on hypnotherapy)

Leiby A, Vazirani M. Complementary, integrative, and holistic medicine: integrative approaches to pediatric irritable bowel syndrome. Pediatr Rev. 2016 Apr;37(4):e10-5.

“Once a diagnosis of IBS is established, a suggested approach is to help the family understand that coping strategies are important for symptom management because psychological and environmental factors play a role in the disease. Validation and reassurance may be sufficient treatment for many children and families, but psychological, pharmacologic, or dietary treatment may also be needed for some. Strong evidence supports the efficacy of CBT, and emerging data suggest that clinical HT and yoga also may be beneficial. Probiotics and certain herbs may be useful adjuncts to biobehavioral therapy. Above all, acknowledging that the patient’s symptoms are genuine is most important for strengthening the patient-parent-physician bond, assuring the patient of the physician’s belief in the patient’s pathology, and initiating discussions about the “brain – gut” connection.”

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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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Practice Based Learning: Sharp Object Ingestion

David Greenky

 

 

 

 

By David Greenky MD (PGY1)

Foreign body Ingestion: General

  • At least 80% of foreign object ingestions will pass on their own
  • Surgical intervention is needed ~12% of the time
  • Vast majority of cases are pediatric, and most between ages of 6 m – 6 y
  • Trouble happens most often at GI angles or narrowing
  • Previous surgery and congenital malformations = greater risk

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Pericarditis

Bergmann KR, et al. Myocarditis and pericarditis in the pediatric patient: validated management strategies. Pediatr Emerg Med Pract. 2015 Jul;12(7):1-22; quiz 23.

Myocarditis and pericarditis are inflammatory conditions of the heart commonly caused by viral and autoimmune etiologies, although many cases are idiopathic. Emergency clinicians must maintain a high index of suspicion for these conditions, given the rarity and often nonspecific presentation in the pediatric population. Children with myocarditis may present with a variety of symptoms, ranging from mild flu-like symptoms to overt heart failure and shock, whereas children with pericarditis typically present with chest pain and fever. The cornerstone of therapy for myocarditis includes aggressive supportive management of heart failure, as well as administration of inotropes and antidysrhythmic medications, as indicated. Children often require admission to an intensive care setting. The acute management of pericarditis includes recognition of tamponade and, if identified, the performance of pericardiocentesis. Medical therapies may include nonsteroidal anti-inflammatory drugs and colchicine, with steroids reserved for specific populations. This review focuses on the evaluation and treatment of children with myocarditis and/or pericarditis, with an emphasis on currently available medical evidence.

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Lithium toxicity

Baird-Gunning J, et al. Lithium poisoning. J Intensive Care Med. 2017 May;32(4):249-263.

Lithium is a commonly prescribed treatment for bipolar affective disorder. However, treatment is complicated by lithium’s narrow therapeutic index and the influence of kidney function, both of which increase the risk of toxicity. Therefore, careful attention to dosing, monitoring, and titration is required. The cause of lithium poisoning influences treatment and 3 patterns are described: acute, acute-on-chronic, and chronic. Chronic poisoning is the most common etiology, is usually unintentional, and results from lithium intake exceeding elimination. This is most commonly due to impaired kidney function caused by volume depletion from lithium-induced nephrogenic diabetes insipidus or intercurrent illnesses and is also drug-induced. Lithium poisoning can affect multiple organs; however, the primary site of toxicity is the central nervous system and clinical manifestations vary from asymptomatic supratherapeutic drug concentrations to clinical toxicity such as confusion, ataxia, or seizures. Lithium poisoning has a low mortality rate; however, chronic lithium poisoning can require a prolonged hospital length of stay from impaired mobility and cognition and associated nosocomial complications. Persistent neurological deficits, in particular cerebellar, are described and the incidence and risk factors for its development are poorly understood, but it appears to be uncommon in uncomplicated acute poisoning. Lithium is readily dialyzable, and rationale support extracorporeal treatments to reduce the risk or the duration of toxicity in high-risk exposures. There is disagreement in the literature regarding factors that define patients most likely to benefit from treatments that enhance lithium elimination, including specific plasma lithium concentration thresholds. In the case of extracorporeal treatments, there are observational data in its favor, without evidence from randomized controlled trials (none have been performed), which may lead to conservative practices and potentially unnecessary interventions in some circumstances. More data are required to define the risk-benefit of extracorporeal treatments and their use (modality, duration) in the management of lithium poisoning.

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Clinical management of acute hypertension

Webb TN, Shatat IF, Miyashita Y. Therapy of acute hypertension in hospitalized children and adolescents. Curr Hypertens Rep. 2014 Apr;16(4):425.

Acute hypertension (HTN) in hospitalized children and adolescents occurs relatively frequently, and in some cases, if not recognized and treated promptly, it can lead to hypertensive crisis with potentially significant morbidity and mortality. In contrast to adults, where acute HTN is most likely due to uncontrolled primary HTN, children and adolescents with acute HTN are more likely to have secondary HTN. This review will briefly cover evaluation of acute HTN and various age-specific etiologies of secondary HTN and provide more in-depth discussion on treatment targets, potential risks of acute HTN therapy, and available pediatric data on intravenous and oral antihypertensive agents, and it proposes treatment schema including unique therapy of specific secondary HTN scenarios.

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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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