Childhood-onset systemic lupus erythematosus

Bundhun PK, et al. Differences in clinical features observed between childhood-onset versus adult-onset systemic lupus erythematosus: A systematic review and meta-analysis. Medicine (Baltimore). 2017 Sep;96(37):e8086.

Significant differences were observed between childhood-onset and adult-onset SLE. Childhood-onset SLE was associated with significantly higher adverse clinical features whereby neurological involvement, renal involvement, oral ulcers, malar rash, vasculitis, fever, ocular, and hematological manifestations were significantly higher, whereas pulmonary involvement, Raynaud phenomenon, and photosensitivity were significantly higher with adult-onset SLE. However, no significant difference was observed in gastrointestinal involvement, cardiovascular involvement, discoid rash, psychosis, alopecia, serositis, and arthritis.

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Pneumatosis intestinalis

Nellihela L, et al. Management of pneumatosis intestinalis in children over the age of 6 months: a conservative approach. Arch Dis Child. 2017 Oct 7.

“Once PI is diagnosed, clinical correlation is essential in order to select the right treatment option. In cases of benign PI, pneumatosis is transient and the gas gradually migrates out of the gut wall resolving with conservative management. Incidental PI in asymptomatic individuals does not require active intervention and is likely to resolve spontaneously. [21] Symptomatic PI requires careful clinical and laboratory assessment to identify patients with ‘red flag’ features who may benefit from early surgical intervention. Studies in adult patients have suggested that high serum lactate, hypotension, peritonitis and acute renal failure are high-risk features requiring immediate surgical exploration. [7 25 26] None of our patients had any of those red flag signs and to the best of our knowledge, there is no such stratification algorithm for children with PI.

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Necrotizing fasciitis

VanderMeulen H, et al. A 10-Year Review of Necrotizing Fasciitis in the Pediatric Population: Delays to Diagnosis and Management. Clin Pediatr (Phila). 2017 Jun;56(7) :627-633.

“The importance of prompt diagnosis stems from its role in the initiation of therapy. Multiple reports support the association of both timely antibiotic initiation and
timely surgical debridement with reduced morbidity and mortality. [3,9,10] This process begins with proper selection of antibiotic agents. Given the delay associated with
receiving results of tissue and blood cultures, empiric therapy should be started immediately. Our data suggests that while the antibiotics initially prescribed often
covered the causative organism, the majority did not provide protection against worrisome bacteria such as MRSA. We argue that coverage against MRSA is warranted
given that it is contributing to an increasing number of cases in North America. [11,12] While various suggestions have been made in the literature, we recommend a combination of clindamycin, vancomycin, and piperacillin-tazobactam. This affords a broad-spectrum coverage of the organisms most likely to be responsible, including as streptococcal species, staphylococcal species, bacteroides species and gram-negative enterobacteriaceae. [13]”

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Diagnostic imaging of vertebral artery dissection

Shakir HJ, et al. Carotid and Vertebral Dissection Imaging. Curr Pain Headache Rep. 2016 Dec;20(12):68.

Carotid or vertebral artery dissection is the result of a tear in the vessel lining wherein the intima separates the media. This creates a false or pseudo lumen, often accompanied by hemorrhage into the arterial wall. Dissection of these craniocervical vessels often manifests with pain alone but, if untreated, may result in severe neurologic compromise. The causes of dissection are multifactorial, including spontaneous, iatrogenic, and traumatic insults. Regardless of etiology, treatment consists primarily of anticoagulation, whereas endovascular therapy is reserved for cases with persistent thrombus or flow limitation. Given the high risk of neurological compromise or death and the propensity of these injuries to occur in younger individuals, early diagnosis of carotid and vertebral artery dissections is critical. Although angiography remains the criterion standard for diagnosis, advances in noninvasive imaging have placed magnetic resonance and computed tomography at the forefront of diagnosis. This article examines the current imaging modalities used to diagnose this under-recognized entity.

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Methylphenidate-induced psychosis

Martínez-Aguayo JC, et al. Brief psychosis induced by methylphenidate in a child with attention deficit disorder: a case report and literature review. Medwave. 2017 Jun 21; 17(5):e6980.

Methylphenidate-induced psychosis has been scarcely studied due to bioethical and neurobiological issues regarding its research. Although some authors have hypothesized that there might be a vulnerability for the development of a major psychiatric disorder in the long term, there is no agreement about the predictive value of this type of psychosis in children and adolescents, and its origin is also uncertain. It has been suggested that higher dopamine levels in some cerebral regions and a family history of mental disorders might increase the risk of psychosis secondary to psychostimulants. We present the case of a nine-year-old child diagnosed with attention deficit disorder and oppositional defiant disorder, who exhibited visual and auditory hallucinations and delirious ideas about harm during methylphenidate treatment. The patient’s symptoms regressed after drug removal. We discuss the key issues related to the origin, causality, management, and prognosis of psychostimulant-induced psychosis.

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Incomplete and atypical Kawasaki disease

Singh S, et al. Diagnosis of Kawasaki disease. Int J Rheum Dis. 2017 Nov 13.

Kawasaki disease (KD) is a medium vessel vasculitis with predilection for coronary arteries. Due to lack of a reliable confirmatory laboratory test, the diagnosis of KD is based on a constellation of clinical findings that appear in a typical temporal sequence. These diagnostic criteria have been modified from time to time and the most recent guidelines have been proposed by the American Heart Association (AHA) in 2017. However, several children may have incomplete or atypical forms of KD and the diagnosis can often be difficult, especially in infants and young children. In this review, we have detailed the steps involved in arriving at a diagnosis of KD and also highlight the important role of echocardiography in diagnosis and management of children with KD.

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Mansoor S, et al. Weighing evidence and art: a challenging case of early-onset atypical Kawasaki disease. Hosp Pediatr. 2015 Nov;5(11):591-6.

We present a case of a high-risk young patient (<6 months of age) with subacute KD who was successfully treated with a tapering course of systemic steroids along with a concomitant standard treatment protocol. Learning points to be taken from this case include the following:

  • The clinician’s index of suspicion needs to remain heightened to make the diagnosis of early-onset KD, which not uncommonly presents with incomplete diagnostic features.
  • Despite the lack of evidence-based guidance from the literature, important informed treatment decisions need to be made for KD patients diagnosed late in the subacute period who have documented coronary artery aneurysms.
  • Steroids may be an effective and safe therapeutic tool to supplement conventional treatment in the high-risk infant population with atypical KD if used judiciously.

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Globus pharyngeus

Doody J, Fenton JE. Troublesome Throat Awareness (tTA) as a contemporary alternative to ‘globus pharyngeus’. Surgeon. 2017 Aug;15(4):183-185.

““Globus pharyngeus” is a tainted term suggesting that it is time to retire the title, as was done with “globus hystericus” two centuries ago. We suggest that the feeling of having a lump in the throat on dry swallow is a normal sensation that everyone experiences to some degree, is accentuated by an ‘event’ and perhaps some are predisposed to perceive this “regular” sensation more strongly than others. It is our opinion that an alternate and contemporary approach is required on this topic. We propose the new term of “troublesome Throat Awareness” (tTA) which is clear, unambiguous with an inherent and therefore therapeutic reassurance to the patient.”

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Jones D, Prowse S. Globus pharyngeus: an update for general practice. Br J Gen
Pract. 2015 Oct;65(639):554-5.

“Globus pharyngeus is a common condition frequently presenting to primary care. Its aetiology remains unclear; however, gastro-oesophageal reflux may play a role in a subset of patients. It is important to consider red flags and ensure prompt referral to secondary care if present.

Management of this condition includes reassurance, vocal hygiene, and treatment of reflux if this is appropriate. Speech and language therapy and cognitive behavioural therapy may also have a role.”

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Abdominal migraines

Irwin S, et al. Recurrent Gastrointestinal Disturbance: Abdominal Migraine and Cyclic Vomiting Syndrome. Curr Neurol Neurosci Rep. 2017 Mar;17(3):21.

Primary headache disorders, including migraine, are some of the most common neurological disorders presenting to hospital. Episodic syndromes that may be associated with migraine, including recurrent gastrointestinal disturbances such as abdominal migraine and cyclic vomiting, often pre-date or co-occur with the onset of migraine in a child who is at risk of developing the headache condition. The purpose of this review is to evaluate the two most common episodic syndromes, abdominal migraine and cyclic vomiting syndrome, including their pathophysiology, common presentations, and diagnostic criteria. Differential diagnosis and “red flag” features are outlined, and an approach to diagnostic work-up is offered. Finally, we provide an evidence-based review of management options and long-term prognosis. Future research should include randomized trials for the acute and preventive treatment of these disorders, as well as research as to whether early intervention can prevent progression to migraine and/or mitigate migraine severity.


Napthali K, et al. Abdominal migraine. Cephalalgia. 2016 Sep;36(10):980-6.

AM is relatively common, affecting up to 4% of the paediatric population. Whilst AM is not believed to continue into adulthood for the majority of children, it has the propensity to develop into probable migraine and recurrent abdominal pain in adulthood. The pathophysiology of this condition remains unclear and as a result treatment for this condition is suboptimal with avoidance of triggers and prophylactic treatment currently recommended when an episode begins.

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Seizures in fetal alcohol spectrum disorders

Boronat S, et al. Seizures and electroencephalography findings in 61 patients with fetal alcohol spectrum disorders. Eur J Med Genet. 2017 January; 60 (1): 72-78.

Fetal alcohol spectrum disorders (FASD) cause neurodevelopmental abnormalities. However, publications about epilepsy and electroencephalographic features are scarce. In this study, we prospectively performed electroencephalography (EEG) and brain magnetic resonance (MR) imaging in 61 patients with diagnosis of FASD. One patient had multiple febrile seizures with normal EEGs. Fourteen children showed EEG anomalies, including slow background activity and interictal epileptiform discharges, focal and/or generalized, and 3 of them had epilepsy. In one patient, seizures were first detected during the EEG recording and one case had an encephalopathy with electrical status epilepticus during slow sleep (ESES). Focal interictal discharges in our patients did not imply the presence of underlying visible focal brain lesions in the neuroimaging studies, such as cortical dysplasia or polymicrogyria. However, they had nonspecific brain MR abnormalities, including corpus callosum hypoplasia, vermis hypoplasia or cavum septum pellucidum. The latter was significantly more frequent in the group with EEG abnormal findings (p < 0.01).

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Nicita F, et al. Seizures in fetal alcohol spectrum disorders: evaluation of clinical, electroencephalographic, and neuroradiologic features in a pediatric case series. Epilepsia. 2014 Jun;55(6):e60-6.

Seizures are observed with a frequency of 3-21% in children with fetal alcohol spectrum disorders (FASD). However, clinical, neuroradiologic, and electroencephalography (EEG) features are poorly described. In this study, 13 patients with FASD and epilepsy or seizures were identified retrospectively from the databases of seven Italian pediatric neurology divisions. Eleven children were affected by epilepsy, and two had at least one documented seizure. Both generalized and focal seizures were observed. EEG showed diffuse or focal epileptic activity; two children developed electric status epilepticus during sleep (ESES). Structural brain anomalies, including polymicrogyria, nodular heterotopia, atrophy, and Arnold-Chiari type 1 malformation, were discovered in almost 50% of patients. Control of seizures was not difficult to obtain in 11 cases; one patient showed pharmacoresistant epilepsy. EEG and clinical follow-up are recommended in children with FASD and epilepsy, since severe conditions requiring aggressive treatment, such as in ESES, may develop. Neuroradiological evaluation is warranted because several types of brain anomalies could be associated with maternal alcoholconsumption during pregnancy.

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Juvenile dermatomyositis

Papadopoulou C, Wedderburn LR. Treatment of Juvenile Dermatomyositis: An Update. Paediatr Drugs. 2017 May 26.

The idiopathic inflammatory myopathies of childhood consist of a heterogeneous group of autoimmune diseases characterised by proximal muscle weakness and pathognomonic skin rashes. The overall prognosis of juvenile myositis has improved significantly over recent years, but the long-term outcome differs substantially from patient to patient, suggestive of distinct clinical phenotypes with variable responses to treatment. High doses of corticosteroids remain the cornerstone of therapy along with other immunosuppressant therapies depending on disease severity and response. The advent of biological drugs has revolutionised the management of various paediatric rheumatologic diseases, including inflammatory myopathies. There are few data from randomised controlled trials to guide management decisions; thus, several algorithms for the treatment of juvenile myositis have been developed using international expert opinion. The general treatment goals now include elimination of active disease and normalisation of physical function, so as to preserve normal growth and development, and to prevent long-term damage and deformities. This review summarises the newer and possible future therapies of juvenile inflammatory myopathies, including evidence supporting their efficacy and safety.

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McCann LJ, Pain CE. A Practical Approach to Juvenile Dermatomyositis and Juvenile Scleroderma. Indian J Pediatr. 2016 Feb;83(2):163-71.

Juvenile dermatomyositis and juvenile scleroderma are rare multisystem autoimmune disorders. Although they share some pathognomonic hallmarks with adult onset myositis or scleroderma, there are significant differences in presentation, characteristics and associated features when the diseases present in childhood. In view of this, and the rarity of the conditions, it is important for care to be led by teams with expertise in pediatric rheumatology conditions. Prognosis has improved significantly in the West; likely due to early diagnosis and aggressive treatment with immunosuppressive medications. However, this trend is not replicated in the developing world. Early recognition of these diseases is crucial to achieve rapid and sustained remission and prevent disease or medication associated complications. This article aims to provide a practical overview for recognition, diagnosis and treatment of these conditions.

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