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Respiratory care in familial dysautonomia: Systematic review and expert consensus recommendations

Kazachkov, Mikhail; Palma, Jose-Alberto; Norcliffe-Kaufmann, Lucy; Bar-Aluma, Bat-El; Spalink, Christy L; Barnes, Erin P; Amoroso, Nancy E; Balou, Stamatela M; Bess, Shay; Chopra, Arun; Condos, Rany; Efrati, Ori; Fitzgerald, Kathryn; Fridman, David; Goldenberg, Ronald M; Goldhaber, Ayelet; Kaufman, David A; Kothare, Sanjeev V; Levine, Jeremiah; Levy, Joseph; Lubinsky, Anthony S; Maayan, Channa; Moy, Libia C; Rivera, Pedro J; Rodriguez, Alcibiades J; Sokol, Gil; Sloane, Mark F; Tan, Tina; Kaufmann, Horacio
BACKGROUND:Familial dysautonomia (Riley-Day syndrome, hereditary sensory autonomic neuropathy type-III) is a rare genetic disease caused by impaired development of sensory and afferent autonomic nerves. As a consequence, patients develop neurogenic dysphagia with frequent aspiration, chronic lung disease, and chemoreflex failure leading to severe sleep disordered breathing. The purpose of these guidelines is to provide recommendations for the diagnosis and treatment of respiratory disorders in familial dysautonomia. METHODS:We performed a systematic review to summarize the evidence related to our questions. When evidence was not sufficient, we used data from the New York University Familial Dysautonomia Patient Registry, a database containing ongoing prospective comprehensive clinical data from 670 cases. The evidence was summarized and discussed by a multidisciplinary panel of experts. Evidence-based and expert recommendations were then formulated, written, and graded using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system. RESULTS:Recommendations were formulated for or against specific diagnostic tests and clinical interventions. Diagnostic tests reviewed included radiological evaluation, dysphagia evaluation, gastroesophageal evaluation, bronchoscopy and bronchoalveolar lavage, pulmonary function tests, laryngoscopy and polysomnography. Clinical interventions and therapies reviewed included prevention and management of aspiration, airway mucus clearance and chest physical therapy, viral respiratory infections, precautions during high altitude or air-flight travel, non-invasive ventilation during sleep, antibiotic therapy, steroid therapy, oxygen therapy, gastrostomy tube placement, Nissen fundoplication surgery, scoliosis surgery, tracheostomy and lung lobectomy. CONCLUSIONS:Expert recommendations for the diagnosis and management of respiratory disease in patients with familial dysautonomia are provided. Frequent reassessment and updating will be needed.
PMCID:6084453
PMID: 30053970
ISSN: 1532-3064
CID: 3216612

Dexmedetomidine for refractory adrenergic crisis in familial dysautonomia

Dillon, Ryan C; Palma, Jose-Alberto; Spalink, Christy L; Altshuler, Diana; Norcliffe-Kaufmann, Lucy; Fridman, David; Papadopoulos, John; Kaufmann, Horacio
OBJECTIVE: Adrenergic crises are a cardinal feature of familial dysautonomia (FD). Traditionally, adrenergic crises have been treated with the sympatholytic agent clonidine or with benzodiazepines, which can cause excessive sedation and respiratory depression. Dexmedetomidine is a centrally-acting alpha 2-adrenergic agonist with greater selectivity and shorter half-life than clonidine. We evaluated the preliminary effectiveness and safety of intravenous dexmedetomidine in the treatment of refractory adrenergic crisis in patients with FD. METHODS: Retrospective chart review of patients with genetically confirmed FD who received intravenous dexmedetomidine for refractory adrenergic crises. The primary outcome was preliminary effectiveness of dexmedetomidine defined as change in blood pressure (BP) and heart rate (HR) 1 h after the initiation of dexmedetomidine. Secondary outcomes included incidence of adverse events related to dexmedetomidine, hospital and intensive care unit (ICU) length of stay, and hemodynamic parameters 12 h after dexmedetomidine cessation. RESULTS: Nine patients over 14 admissions were included in the final analysis. At 1 h after the initiation of dexmedetomidine, systolic BP decreased from 160 +/- 7 to 122 +/- 7 mmHg (p = 0.0005), diastolic BP decreased from 103 +/- 6 to 65 +/- 8 (p = 0.0003), and HR decreased from 112 +/- 4 to 100 +/- 5 bpm (p = 0.0047). The median total adverse events during dexmedetomidine infusion was 1 per admission. Median hospital length of stay was 9 days [interquartile range (IQR) 3-11 days] and median ICU length of stay was 7 days (IQR 3-11 days). CONCLUSIONS: Intravenous dexmedetomidine is safe in patients with FD and appears to be effective to treat refractory adrenergic crisis. Dexmedetomidine may be considered in FD patients who do not respond to conventional clonidine and benzodiazepine pharmacotherapy.
PMCID:5292083
PMID: 27752785
ISSN: 1619-1560
CID: 2279892

Conduction Disturbances and Ventricular Arrhythmias Associated with High-Dose Loperamide

Leung, Galen; Altshuler, Diana; Goldenberg, Ronald; Fridman, David; Yuriditsky, Eugene
Although loperamide has been widely used for the treatment of diarrhea, there is growing popularity over its abuse potential in alleviating opioid-withdrawal symptoms and achieving euphoria. Toxic levels of loperamide have been associated with life-threatening ventricular tachyarrhythmias and cardiac arrest. We report a case of high-dose loperamide ingestion in a patient presenting initially with unstable bradycardia followed by episodes of polymorphic ventricular tachycardia, and an unmasked Brugada ECG pattern. This is the first such report of the Brugada pattern being unmasked on ECG with loperamide ingestion. The patient stabilized with supportive care without the need for inotropic support. We discuss potential mechanisms of toxicity leading to conduction abnormalities and provide a literature review of all published cases of loperamide toxicity to describe proposed treatment options. Recognition of the abuse potential and hazards of this over-the-counter anti-diarrheal therapy will alert the clinician of associated toxidromes and management strategies
ORIGINAL:0011816
ISSN: 2161-0495
CID: 2492962

Acute Coronary Artery Thrombosis Precipitated By Eltrombopag, A Thrombopoetin Receptor Agonist [Meeting Abstract]

Burke, DW; Mulliken, JS; Skolnick, AH; Fridman, D
ISI:000390749606717
ISSN: 1535-4970
CID: 2414902

A Unique Case Of Obstructive Shock [Meeting Abstract]

Lesko, MB; Mukherjee, V; Maulion, C; Fridman, D
ISI:000390749606744
ISSN: 1535-4970
CID: 2414932

Phenibut Overdose in Combination with Fasoracetam: Emerging Drugs of Abuse

Merchan, Cristian; Morgan, Ryan; Papadopoulos, John; Fridman, David
The widespread availability of non-traditional dietary supplements and pharmacologically active substances via the Internet continues to introduce mechanisms for inadvertent toxidromes not commonly seen. Consumers are virtually unrestricted in their ability to acquire products purporting augmentation of normal physiology for the purposes of enhancement, recreation, and/or potential abuse. The safety profiles at standard or toxic doses remain largely unknown for many agents that can be purchased electronically. We report a case of mixed toxicity related to phenibut and fosaracetam, both of which are readily available for consumer purchase from online retailers. Written and verbal consent was obtained for this case presentation
ORIGINAL:0012267
ISSN: n/a
CID: 2713332

Black Pleural Effusion: A Unique Presentation of Metastatic Melanoma

Chhabra, Akansha; Mukherjee, Vikramjit; Chowdhary, Mudit; Danckers, Mauricio; Fridman, David
Metastatic melanoma is a rare form of skin cancer, but one that comes with a high mortality rate. Pulmonary involvement is frequently seen in metastatic melanoma with only 2% of malignant melanoma patients with thorax metastasis presenting with pleural effusions. Herein, we report an extremely rare case of black pleural effusion from thoracic metastasis of cutaneous malignant melanoma. A 74-year-old man with known metastatic melanoma presented with a 1-month history of worsening lower back and hip pain and was found to have extensive osseous metastatic disease and multiple compression fractures. The patient underwent an uneventful kyphoplasty; however, the following day, he became acutely hypoxic and tachypneic with increased oxygen requirements. Radiographic evaluation revealed new bilateral pleural effusions. Bedside thoracentesis revealed a densely exudative, lymphocyte-predominant black effusion. Cytological examination showed numerous neoplastic cells with melanin deposition. A diagnosis of thoracic metastasis of malignant melanoma was established based on the gross and microscopic appearance of the pleural fluid. To the best of our knowledge, this is the first reported case of black pleural effusions secondary to metastatic melanoma in the United States. Despite the rarity of this presentation, it is important to determine the etiology of the black pleural effusion and to keep metastatic melanoma as a differential diagnosis.
PMCID:4464038
PMID: 26078741
ISSN: 1662-6575
CID: 1640392

Black Pleural Effusion [Meeting Abstract]

Chhabra, A; Mukherjee, V; Mahmoudi, M; Fridman, D
ISI:000377582808296
ISSN: 1535-4970
CID: 2214922

Lipiodol embolism following transarterial chemoembolization: an atypical case

Taupin, Daniel; Mukherjee, Vikramjit; Nathavitharana, Ruvandhi; Green, David A; Fridman, David
OBJECTIVE: Transarterial chemoembolization is a widely used therapy for the treatment of hepatocellular carcinoma. A rare adverse event is acute respiratory distress syndrome from pulmonary embolization of Lipiodol, an iodinated oil commonly used during the procedure. The objective of this report is to describe an atypical case of acute respiratory distress syndrome from Lipiodol embolization in a patient who underwent transarterial chemoembolization for hepatocellular carcinoma 9 days prior to presentation, despite having received relatively small amounts of Lipiodol (5.5 mL). Although this diagnosis has classically been based on radiological findings, we established a diagnosis after lipid-laden macrophages were detected in bronchial alveolar lavage fluid. DESIGN: Case report. SETTING: ICU of a major metropolitan academic medical center. PATIENTS: Single case. INTERVENTIONS: Diagnostic interventions included noncontrast CT scan of the chest and cytologic examination of bronchial alveolar lavage fluid with oil red O staining. Therapeutic interventions included mechanical ventilation and methylprednisolone infusions. MEASUREMENTS AND MAIN RESULTS: Noncontrast CT demonstrated nonspecific diffuse ground glass opacification, most prominent within the upper lobes. Mechanical ventilation was begun for hypoxemic respiratory failure. Cytologic examination of bronchial alveolar lavage fluid revealed a high proportion of lipid-laden macrophages, findings consistent with Lipiodol embolism. Despite infusions of methylprednisolone, the patient expired on hospital day 8. CONCLUSIONS: Acute respiratory distress syndrome from Lipiodol embolization following transarterial chemoembolization can occur even with small Lipiodol volumes. Cytologic examination of bronchial alveolar lavage fluid with oil red O staining is a useful diagnostic modality, especially when imaging studies are equivocal.
PMID: 24607940
ISSN: 0090-3493
CID: 1004772

An Unusual Cause Of Acute Respiratory Failure [Meeting Abstract]

Mukherjee, V; Postelnicu, R; Esaian, D; Fridman, D
ISI:000209838205805
ISSN: 1535-4970
CID: 2492882