THE ANAPHYLAXIS HYPOTHESIS OF SUDDEN INFANT DEATH SYNDROME (SIDS): MAST CELL DEGRANULATION IN COT DEATH REVEALED BY ELEVATED CONCENTRATIONS OF TRYPTASE IN SERUM

PEDIATRICS ◽  
1996 ◽  
Vol 98 (2) ◽  
pp. 320-320
Author(s):  
Mary E. Bollinger ◽  
Robert A. Wood
Keyword(s):  
Mast Cell ◽  
Infant Death ◽  
Cell Activation ◽  
Sudden Infant Death ◽  
Mast Cell Activation ◽  
Sudden Infant ◽  
Specific Marker ◽  
Serum Tryptase ◽  
Cell Degeneration ◽  
Cot Death

This study found that elevated concentrations of serum tryptase are associated with sudden unexplained infant death, and this may be consistent with anaphylaxis as a mechanism in some cases of SIDS. Further studies are needed to determine if mast cell degeneration in these cases is secondary to terminal respiratory failure or some other event common to most cases of SIDS. The measurement of 9α, 11β-PGF2, as a specific marker of mast cell activation was not helpful under the conditions of this study.

scholarly journals Patient Reported Symptoms and Tryptase Levels in WHO-Defined Systemic Mastocytosis (SM) Versus Mast Cell Activation Syndrome (MCAS) Versus Neither

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 3204-3204
Author(s):  
Darci Zblewski ◽  
Ramy A. Abdelrahman ◽  
Dong Chen ◽  
Joseph H. Butterfield ◽  
Ayalew Tefferi ◽  
...  
Keyword(s):  
Mast Cell ◽  
Symptom Score ◽  
Cell Activation ◽  
Total Symptom Score ◽  
Mast Cell Activation ◽  
Median Score ◽  
Serum Tryptase ◽  
Tryptase Level ◽  
Patient Reported ◽  
Symptom Scores

Abstract Introduction: The utility of patient reported symptoms and serum tryptase levels in distinguishing those with systemic mastocytosis (SM) versus mast cell activation syndrome (MCAS) versus those not meeting formal diagnostic criteria for SM or MCAS has not been systematically examined. Methods: This study was approved by our institutional review board. Patients were referred for suspected SM based on symptoms of mast cell activation, osteopenia, skin rash, etc., or had an established diagnosis of SM. All patients were given a Mastocytosis Symptom Assessment Form (MastSAF) to complete at their initial evaluation. The MastSAF is comprised of 36 symptom questions to be graded on a scale of 0 (absent) to 10 (worst imaginable), and is organized around 9 symptom clusters: gastrointestinal (8 questions), constitutional (3 questions), musculoskeletal (5 questions), cutaneous (4 questions), neuropsychological (6 questions), genitourinary (3 questions), respiratory (4 questions), angioedema (1 question), and cardiovascular (2 questions). All patients underwent bone marrow biopsy and serum tryptase level assessment. SM was diagnosed by 2008 WHO criteria. In the presence of characteristic symptoms, if clonal or abnormal mast cells were not identified, and if serum/urine mast cell mediator levels were increased, then non-SM associated, non-monoclonal MCAS was diagnosed. Results: A total 53 patients were studied. 1) SM: Of 28 patients, 13 had indolent SM (ISM), 9 aggressive SM (ASM) and 6 SM with associated hematological disease (SM-AHD) The median total symptom score was 47 (range 8-159). The median (range) for SM subgroups was: ISM 51 (9-159), ASM 55 (19-157) and SM-AHD 27 (8-131) (p=0.2). The normalized median score for individual symptom categories (total median score/no. of symptoms per category) in order of severity was cutaneous 1.9, gastrointestinal 1.8, constitutional 1.7, neuropsychological 1.3, respiratory 1.3, musculoskeletal 0.9, cardiovascular 0.3, genitourinary 0.3, and angioedema 0. Symptom severity was not significantly different among the 3 SM subgroups, except for constitutional symptoms (median score ASM 9, ISM 4, SM-AHD 2.5, p=0.02). The median (range) tryptase level was 48.4 ng/mL (8.8-282); four patients (14%) had a baseline level <20 ng/mL. The median (range) tryptase level among SM subgroups was: ISM 46.9 (8.8-225), ASM 103 (29.4-282), and SM-AHD 43.5 (28.5-233) (p=0.1). When considering patients with tryptase level ≥50 versus <50 ng/mL, symptom scores were not significantly higher in the former group with the exception of constitutional (p=0.02) and genitourinary symptoms (p=0.04). 2) MCAS: 15 patients The median total symptom score was 127 (range 2-248). The normalized median score for individual symptom categories in order of severity was neuropsychological 4.5, musculoskeletal 4.2, cutaneous 4.0, constitutional 3.3, gastrointestinal 2.1, respiratory and cardiovascular 2.0 each, genitourinary 1.7, and angioedema 1.0. The median (range) serum tryptase level at referral was 12.7 ng/mL (1.7-25.8); five patients (33%) had a baseline level >20 ng/mL. 3) Neither SM/MCAS: 10 patients The median total symptom score was 119 (range 45-177). The normalized median score for individual symptom categories in order of severity was musculoskeletal 4.2, gastrointestinal 3.6, constitutional 3.3, neuropsychological 3.3, cutaneous 3.0, cardiovascular and genitourinary 2.0 each, respiratory 1.3, and angioedema 0. The median (range) serum tryptase level at referral (n=9) was 4.8 ng/mL (2.9-6.6). 4) Comparison: MCAS vs. SM: Symptom scores were significantly higher in MCAS as compared to SM (p<0.05), except for genitourinary and respiratory symptoms, which were not significantly different. ‘Neither SM/MCAS’ vs. SM: Symptom scores (total, gastrointestinal, constitutional, musculoskeletal, cutaneous, and neuropsychological) were significantly higher in the former group (p<0.05). Other symptom scores were not significantly different. Conclusions: The spectrum and severity of patient reported symptoms was broadly similar among WHO subcategories of SM, and serum tryptase level had limited if any correlation with symptom scores. Despite the significantly higher overall symptom burden in MCAS versus SM, tryptase levels in the former group were significantly lower with values >30 ng/mL unusual. Despite overlap, the top ranked symptoms in the 3 groups were different. Disclosures No relevant conflicts of interest to declare.

Cot death and myocarditis

1992 ◽  
Vol 2 (3) ◽  
pp. 266-271 ◽  
Author(s):  
Caroline Rambaud ◽  
Cécile Cieuta ◽  
Danielle Canioni ◽  
Christine Rouzioux ◽  
Jean Lavaud ◽  
...  
Keyword(s):  
Sudden Infant Death Syndrome ◽  
Infant Death ◽  
Inflammatory Cells ◽  
Sudden Infant Death ◽  
Neurological Involvement ◽  
Sudden Infant ◽  
Cot Death ◽  
Cellular Necrosis ◽  
Characteristic Features ◽  
Epidemiological Characteristics

SummaryWe have investigated the hearts from 153 infants found dead in their cots at ages ranging from one month to one year. The deaths occurred over a period of five years (January 1986 to December 1990) and were studied in a center for the study of the sudden infant death syndrome located in Paris. The epidemiological characteristics of this group are:male predominance (sex ratio 1.43), age less than four months (82%), and a predominance of winter deaths. These are the characteristic features ofthe sudden infant death syndrome. Ofthe 143 children studied with the permission of their parents, 24 (16.8%–12 girls and 12 boys) had histological lesions consistentwith myocarditis according to the Dallas criteria. The histological diagnosis of myocarditis is based on the association of cellular necrosis with a mononuclear or mixed inflammatory infiltrate. Cytoplasmic vacuolization, the presence of inflammatory cells in myocytes, and rupture of the cell walls of myocardial fibres are the equivalent histological signs of cellular necrosis. Myocarditis was diversely associated with respiratory, hepatic, muscular, gastrointestinal and/or neurological involvement. Twelve infants had previously been ill. Two died during the course of a serious illness. In only four cases was a viral association demonstrated. This incidence of myocardial involvement, similar to thatdescribed elsewhere in the literature, suggests that myocarditis could be a pathogenic explanation of some deaths thought on general autopsy investigation to be sudden and unexplained.

Clothing and bedding and its relevance to sudden infant death syndrome: Further results from the New Zealand Cot Death Study

1994 ◽  
Vol 30 (6) ◽  
pp. 506-512 ◽  
Author(s):  
C. A. WILSON ◽  
B. J. TAYLOR ◽  
R. M. LAING ◽  
S. M. WILLIAMS ◽  
E. A. MITCHELL ◽  
...  
Keyword(s):  
New Zealand ◽  
Sudden Infant Death Syndrome ◽  
Infant Death ◽  
Sudden Infant Death ◽  
Sudden Infant ◽  
Cot Death

Distinguishing Sudden Infant Death Syndrome From Child Abuse Fatalities

PEDIATRICS ◽  
1994 ◽  
Vol 94 (1) ◽  
pp. 124-126 ◽  
Author(s):  
Keyword(s):  
Child Abuse ◽  
Sudden Infant Death Syndrome ◽  
Infant Death ◽  
Clinical History ◽  
Sudden Infant Death ◽  
The United States ◽  
Sudden Infant ◽  
Death Scene ◽  
Battered Child ◽  
Cot Death

Public and professional awareness of sudden infant death syndrome (SIDS) has increased in the 28 years since the establishment of the National Sudden Infant Death Foundation, now called the National SIDS Alliance.1 Similarly, awareness of child abuse has increased in the 30 years since the publication of the first article on the battered child.2 In the majority of cases, when an infant younger than 1 year dies suddenly and unexpectedly, the cause is SIDS. Sudden infant death syndrome is far more common than infanticide. In a few difficult cases, legitimate investigations for possible child abuse have resulted in an insensitive approach to grieving parents or caretakers. This statement provides professionals with information and guidelines to avoid distressing or stigmatizing families of SIDS victims while allowing accumulation of appropriate evidence in the uncommon case of death by infanticide. INCIDENCE AND EPIDEMIOLOGY Sudden infant death syndrome, also called crib or cot death, is "the sudden death of an infant under 1 year of age which remains unexplained after a thorough case investigation, including performance of a complete autopsy, examination of the death scene, and a review of the clinical history." 3 Sudden infant death is the most common cause of death between I and 12 months of age. Eighty percent of cases occur before age 5 months, with a peak incidence between 2 and 4 months of age. Sudden infant death syndrome occurs in 1.5 to 2 per 1000 live births, resulting in 6000 to 7000 infant deaths each year in the United States.4

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