Pediatric Hypertension and End-Stage Renal Disease.

Pediatric hypertension is not an uncommon diagnosis, affecting about 3.5% of all children. Most childhood hypertension is associated with obesity, but elevated blood pressure can also be the presenting symptom of a secondary disease process. Moreover, no matter the cause of hypertension, early identification can improve long-term health outcomes as childhood hypertension predicts hypertension in adulthood. In 2017, the American Academy of Pediatrics revised their 2004 guidelines regarding blood pressure screening for all children. Here, we discuss an illustrative case of a 16-year-old girl with hypertension and underlying nephrotic syndrome whose diagnosis was delayed due to inadequate blood pressure screening. Given the varying practices regarding the interpretation of blood pressure data in the outpatient setting, it is important for primary care providers to understand the updated guidelines and the indications for referral. [Pediatr Ann. 2020;49(6):e258-e261.].

Paraneoplastic opsoclonus myoclonus syndrome associated with inflammatory myofibroblastic tumor in a pediatric patient.

Opsoclonus myoclonus syndrome (OMS) is a rare neurological syndrome caused by a paraneoplastic autoimmune process that affects children with neuroblastic tumors. Treatment includes corticosteroids, intravenous gamma globulin (IVIG), rituximab, and other immunosuppressive therapies. Here, we describe a patient diagnosed with OMS associated with a localized inflammatory myofibroblastic tumor. The patient has no evidence of tumor recurrence following surgical resection with 8-month follow-up. The neurologic symptoms resolved with corticosteroids and IVIG. This case demonstrates that in children, neoplasms other than neuroblastoma may be associated with this paraneoplastic syndrome, and highlights the importance of evaluating patients with OMS for underlying malignancies.

Pleuroparenchymal fibroelastosis in an 8-year old treated for neuroblastoma.

Pleuroparenchymal fibroelastosis (PPFE), which is primarily diagnosed in adults, is a progressive lung pathology associated with significant morbidity and mortality. PPFE is characterized by pleural and subpleural parenchymal disease causing dyspnea, cough, and recurrent pneumothoraces. PPFE can be precipitated by autoimmune disorders, recurrent respiratory infections, chemotherapy, and transplant. We describe the youngest recorded patient to develop PPFE, whose symptoms began several years after treatment for neuroblastoma. Her symptoms were initially mistaken for worsening asthma, and multiple comorbidities developed during the prolonged time to recognition of PPFE and she progressed to fatal lung disease before potentially curative lung transplantation could occur.

Pediatric Anti-NMDA-R Encephalitis: Presentation, Diagnosis, and Management.

Immune- mediated encephalitis is the most common cause of encephalitis after infection in children and adults. Although this disease process was identified nearly 20 years ago, the variety of clinical presentations and the lack of specific diagnostic criteria can make the identification of anti-N-methyl-D-aspartate receptors (NMDA-R) encephalitis challenging. Moreover, identifying NMDA-R antibodies in blood or cerebrospinal fluid can take days to weeks, and thus clinicians need to have a high index of suspicion to investigate for this disease in patients who may appear to have an overlap of neurologic and psychiatric symptomatology. In this article, the authors describe three illustrative cases of anti-NMDA-R encephalitis in children age 3 to 16 years. The discussion reviews our current understanding of the clinical presentation, diagnostic criteria, and inpatient therapeutic management of anti-NMDA-R encephalitis, as well as illuminates the unique and often perplexing presentations of this disease process versus other organic and psychiatric causes of altered mental status. [Pediatr Ann. 2019;48(10):e387-e390.].

Pediatric anti-NMDA receptor encephalitis with catatonia: treatment with electroconvulsive therapy.

BACKGROUND: Anti-NMDA receptor encephalitis, an autoimmune disease associated with antibodies against N-methyl-D-aspartate (NMDA) receptors, is being diagnosed more frequently, especially in children and young adults. Acute neurological and psychiatric manifestations are the common presenting symptoms. Diagnosing anti-NMDA receptor encephalitis is often challenging given the wide range of clinical presentation, and may be further complicated by its overlap of symptoms, brain MRI changes, and CSF findings with other entities affecting the brain. Even though diagnosis can be made by identifying antibodies in immune-mediated encephalitis, the diagnosis may be delayed by weeks to months. Delay in initiation of treatment with immune suppressive therapies is shown to be associated with adverse outcomes. Malignant catatonia is a severe and life-threatening state associated with anti-NMDA receptor encephalitis. It is often inadequately assessed and may not respond to immunosuppressive treatment. CASE PRESENTATION: We present a confirmed case of anti-NMDA receptor encephalitis in a 16 year old girl who had severe critical neurological and psychiatric manifestations, including malignant catatonia and autonomic instability. Our patient continued to manifest malignant catatonia despite the initiation of prompt, aggressive immune suppressive therapies, including corticosteroids, plasmapheresis, intravenous gammaglobulin and rituximab, as well as treatment with high-dose benzodiazepines. Once electroconvulsive therapy (ECT) began, she had a robust response with resolution of her catatonia. Six weeks after treatment with eight ECT cycles, she had returned to her normal baseline cognitive and motor function. CONCLUSIONS: ECT was an effective and well-tolerated therapy in our patient, and should be considered for the treatment of children with anti-NMDA receptor encephalitis whose catatonia does not respond to immunosuppression and benzodiazepines.