Convincing History Research Articles

Diagnostic Decision Points of Specific Immunoglobulin E Concentrations for Seafood Allergies in Korean Children: A Nationwide Multicenter Study.

Shellfish and fish are common causes of food allergy (FA) in Asia, with significant prevalence rates reported across various countries. This study aimed to identify the diagnostic decision points (DDPs) for shrimp, crab, and cod allergies in Korean children. We enrolled participants aged 18 years or younger with suspected shrimp, crab, or cod allergies through a retrospective review of medical records from a nationwide 14-center study between January 2018 and March 2022. FA was diagnosed using an open oral food challenge or a reproducible and convincing history with positive serum specific immunoglobulin E (sIgE) levels. The sIgE cut-off values for shrimp, crab, and cod were determined by analyzing receiver operating characteristic curves. Out of 266 children, 90 (33.8%) were confirmed to have a shrimp allergy. Crab allergies were found in 37 (39.8%) of 93 children. Of 66 children, 15 (22.7%) were found to be allergic to cod. The optimal sIgE cut-off values based on the largest area under the curve were 1.43, 3.25, and 2.05 kU/L for shrimp, crab, and cod, respectively. Levels of sIgE with more than 90% positive predictive value (PPV) were 33.8, 47.5, and 29.9 kU/L for shrimp, crab, and cod, respectively. Levels of sIgE with greater than 90% negative predictive value (NPV) were 0.36, 0.04, and 3.93 kU/L for shrimp, crab, and cod, respectively. Our results suggest that DDPs of sIgE levels with high PPV and NPV can be used to diagnose shrimp, crab, and cod allergies in Korean children.

FP3.3 - Sensitivity of Faecal Immunochemical Testing

Abstract Aim The use of faecal immunochemical testing (FIT) in the diagnosis of colorectal cancers (CRC) has become a routinely utilised and reliable investigation. In the symptomatic population, FIT may identify those patients who require urgent colorectal investigation. It is also commonly used as a triaging tool in bowel cancer screening. In this study we sought to assess the sensitivity of FIT in detecting CRC in our centre. Method Patients with histologically confirmed CRC were identified from a data set gathered from the hospital colorectal specialist services between January 2021 to January 2022. Thereafter, the hospital electronic systems were used to retrospectively gather the value of FIT tests for each CRC patient found. Results From 407 patients with CRC identified, only 256 patients who we successfully managed to obtain their pre diagnosis FIT result were included. From those, 235 patients were FIT positive and 21 patients had a false negative result. Therefore, this suggests a FIT sensitivity of 91.79%. Furthermore, we identified 14 patients who had a repeat FIT preformed. From which, one patient who was originally fit negative had a positive result on the repeat test. Conclusion While faecal immunochemical testing is highly sensitive and a useful screening tool, it's important to remember that it is not always accurate. Therefore if a patient has convincing history or symptoms, we should keep a high index of suspicion and investigate further. It also remains to be seen if the use of double FIT will help increase the sensitivity of detecting CRC.

Helminth Infestation Causing Anaemia in Pregnancy Attending Primary Care Clinic: A Case Report

Anaemia in pregnancy is among the commonest medical disorders encountered antenatally. However, the condition has sometimes been overlooked and not corrected accordingly given wrong or unidentified exact causes. Helminth infestation, even though rare generally nowadays, is still an important cause that needs to be ruled out in case of anaemia. We reported a case of an antenatal woman who presented with unresolved anaemia. However, she benefited from the helminth infestation screening and was able to be treated well. Her condition tremendously improved after the eradication of the infection. This case highlighted that anaemia should always include stool for ova and cyst despite no convincing history towards the infection. The symptoms could be mild or even not recognised.

Sensitization to α-gal and omega-5-gliadin in patients with chronic urticaria: clinical implications and need for targeted testing.

Chronic urticaria (CU) is defined as the presence of recurring hives and/or angioedema lasting more than 6 weeks. The precise impact of food allergies, particularly to galactose-α-1,3-galactose (α-gal) and omega-5-gliadin (O5G), on the development of CU is not well established. The study examines the frequency of sensitisation to these allergens and their clinical significance in individuals with CU. We recruited 100 adults newly diagnosed with CU to evaluate the presence of specific immunoglobulin E antibodies and allergies to α-gal and O5G. The patients were categorised into three groups based on their specific IgE levels: those sensitised to α-gal, those sensitised to O5G, and those not sensitised to either. Demographic, clinical, and laboratory variables were compared across the groups. The findings indicated that 5% of the cohort was sensitised to α-gal, and 4% showed sensitivity to O5G, while only 4% of patients had a convincing history of food allergy (two to red meat and two to wheat). The sensitised groups exhibited the greatest levels of total IgE, and had a higher proportion of males. The α-gal group showed higher levels of specific IgE to Ascaris lumbricoides than the group without sensitisation. The groups had similar features in all other assessed aspects. Our CU cohort's sensitivity and convincing food allergy to α-gal and O5G were comparatively modest. While routine screening may not be essential, focused testing of individuals with severe or uncommon symptoms might enhance the accuracy of diagnosis and treatment.

Cashew Allergy Prevalence and Sensitization in 1-Year-Old Infants

Cashew allergy is the most common tree nut allergy in Australia, but there is limited data on population-level prevalence and risk factors. Describe the prevalence of cashew sensitization and allergy in 12-month-old infants and identify risk factors. Data was from the EarlyNuts cohort, a population-based sample of infants recruited in Melbourne, Australia. Families completed a questionnaire and infants underwent skin prick test (SPT) to cashew. Infants with positive SPTs were offered food challenges. Questionnaires collected demographic data and allergy risk factors. Allergy outcomes were determined by challenge outcomes or convincing history of an allergic reaction. Weights were used to adjust estimated prevalence to reflect the distribution of risk factors among the combined sample of participants and non-participants. 1933 participants were recruited, with 1414 cashew allergy outcomes identified. Of these, 1.96% (95%CI 1.28-2.99%) had a SPT ≥3mm and 1.49% (95%CI 0.91-2.44%) were cashew allergic. Infants with eczema or peanut allergy in the first year of life were more likely to be cashew allergic (adjusted odds ratio 5.75, 95%CI 2.08-15.88, p=0.001 and 19.30, 95%CI 5.44-68.43, p<0.001 respectively). 25% of participants introduced cashew before 12 months (95%CI 22.7-27.8%). There was no association between timing of cashew introduction and cashew allergy. This is the first study describing prevalence and risk factors of cashew allergy in a population-based infant cohort. Eczema and peanut allergy were associated with an increased risk of cashew allergy. Few infants were introduced to cashew prior to age 12 months, suggesting infant feeding guidelines have not yet translated to earlier introduction of all allergens.

Horner’s Syndrome as an Atypical Presentation of Neuroborreliosis: A Case Report and Review of Literature (P1-9.006)

<h3>Objective:</h3> To localize and report postganglionic Horner’s syndrome as an unusual presentation of neuroborreliosis. <h3>Background:</h3> Lyme disease is the most common tick-born infection in the United Sates with ~30,000 reportable cases yearly, 10–15% involving the nervous system. Neuroborreliosis has a heterogenous spectrum of clinical manifestations. Currently, there are few case reports of associated Horner’s syndrome. Localization has not been well described with no cases reporting positive imaging findings. <h3>Design/Methods:</h3> Case report. <h3>Results:</h3> A 50-year-old healthy woman presented with four days of high-grade fever and left-sided headache followed by one day of left droopy eyelid. Symptoms developed two weeks after an outdoor summer trip to Massachusetts with exposure to insect bites. Her neurologic exam demonstrated 2mm left-sided ptosis, miosis with a briskly reactive right pupil from 6-to-2mm and left pupil from 3-to-2mm in the dark, and 2mm-difference in pupil size in the light with a smaller left pupil. She also developed macular patches over the head and trunk consistent with multifocal erythema migrans. Initial laboratory work-up showed mild anemia, thrombocytopenia, and elevated transaminase levels. MRI of brain and orbits with contrast revealed asymmetric enhancement within the left cavernous sinus and superior orbital fissure with mild edema of the left extraocular muscles. MRA/MRV did not show evidence of cavernous sinus thrombosis, carotid-cavernous fistula, or other abnormalities. Cerebrospinal fluid analysis showed lymphocytic pleocytosis with negative Borrelia species detection by PCR. Further testing revealed elevated serum total Lyme antibody and Western blot IgM suggestive of Lyme neuroborreliosis. Patient was treated with oral doxycycline with resolution of symptoms. <h3>Conclusions:</h3> Neuroborreliosis has a highly variable clinical presentation. This case illustrates and localizes an isolated Horner’s syndrome as a masquerading presentation of neuroborreliosis. Therefore, clinical suspicion and early recognition are important, especially in the setting of a convincing history, to prompt early diagnosis and initiation of treatment and to prevent disease progression. <b>Disclosure:</b> Dr. Bou Reslan has nothing to disclose. Dr. Park has nothing to disclose. Dr. Sabadia has nothing to disclose. Dr. Grossman has nothing to disclose. The institution of Dr. Zakin has received research support from American Board of Psychiatry and Neurology.

IgE-mediated fish allergy in Singaporean children.

Fish is one of the common causes of food allergy and there is limited literature about fish allergy in Singapore. We aimed to describe the demographics, clinical features, and natural history of children with IgE-mediated fish allergy. A retrospective review was conducted for children diagnosed with fish allergy in a tertiary pediatric hospital in Singapore between 2015 and 2020. The diagnosis of fish allergy was made in 108 patients based on a convincing history of IgE-mediated allergic reaction and a positive skin prick test. The median age at first reaction was 12 months (range 6-168) with most reacting on first ingestion (62.0%). The most common fish causing reactions were threadfin (48.1%), salmon (33.3%) and cod (31.5%). Majority presented with cutaneous symptoms (97.2%). Anaphylaxis occurred in 6.5%. Five were mono-sensitized (4.6%), 77 were oligo-sensitized (71.3%) and 26 were polysensitized (24.1%). Most can tolerate another species of fish (75.9%), most commonly salmon (37.0%), tuna (24.1%) and cod (22.2%). Median duration of follow up was 24 months (range 0-176). Twenty-eight out of 108 children (25.9%) acquired natural tolerance to index fish at a median age of 60 months (range 18-159). Most children with fish allergy can tolerate at least one other species of fish and resolution of fish allergy is possible. Thus, it is important to follow-up with an allergist to evaluate which fish species can be included in their diet to avoid unnecessary dietary restrictions.

Study on Effects of Sublingual Immunotherapyin Allergic Rhinitis Patients.

Allergic Rhinitis is one of the most common allergic disease and characterized by sneezing, rhinorrhea, nasal congestion and nasopharyngeal itching. The initial management includes pharmacological treatment and the patients who are refractory to pharmacological treatment are then reffered for immunotherapy. SLIT has been widely used for treatment of allergic rhinitis and has proven its clinical efficacy. The objective of the present study was to assess the clinical effects, safety and tolerability of sublingual immunotherapy (SLIT) among the patients suffering from allergic rhinitis. The study was conducted from Aug 2018 to April 2021 and 40 patients with convincing history, positive skin prick test to one or more allergen extracts were recruited. SLIT was conducted with antigens (mix), namely dust mites, tree pollens, grass pollens and weed pollens in patients of allergic rhinitis for 1 year. There was significant improvement in quality of life and symptoms severity(Nasal and Non-Nasal) from base line to end of 1 year. SLIT lowers the total IgE, absolute eosinophilic count and medication requirement. Sublingual Immunotherapy for specific allergens decreases clinical symptoms in patients with allergic rhinitis and sensitivity to multiple allergen.

COVID vaccination can be performed in patients with a history of allergic reactions to the vaccines or their components: experience from a specialist clinic in South Australia.

BackgroundThe development of vaccines against SARS‐CoV2 has been a key public health response to the COVID‐19 pandemic. However, since their introduction, there have been reports of anaphylactic reactions to vaccines in individuals with history of allergic reactions to other vaccines, excipients or to COVID vaccines.AimA dedicated adult COVID vaccine allergy clinic with a standardised allergy testing protocol was set up to investigate safety and suitability of available COVID vaccines in Australia.MethodsPatients referred to a state‐wide COVID‐19 vaccine allergy clinic between March and August 2021 with a history of allergy underwent skin‐prick testing and intradermal testing to both available vaccine formulations (BNT162b2 and ChAdOx1‐S), excipients (polyethylene glycol and polysorbate 80), excipient‐containing medications and controls. Basophil activation testing was conducted in few subjects with convincing history of immediate type reactions.ResultsFifty‐three patients underwent testing for possible excipient allergy (n = 19), previous non–COVID vaccine reaction (n = 13) or previous reaction to dose 1 of COVID‐19 vaccine (n = 21). Patients were predominantly female (n = 43, 81%), aged 18–83 (median 54) years. Forty‐four patients tested negative and 42 of these received at least their first dose of a COVID‐19 vaccine. Nine patients tested positive to excipients or excipient‐containing medication only (n = 3), or vaccines (n = 6). Five patients were positive to just BNT162b2, 3/5 have been vaccinated with ChAdOx1‐S. One who was skin test positive to both vaccines, but negative BAT to ChAdOx1‐S was successfully vaccinated with ChAdOx1‐S.ConclusionEven in a high‐risk population, most patients can be vaccinated with available COVID‐19 vaccines. This paper reports local experiences using a combined allergy testing protocol with skin testing and BAT during the pandemic.

Food allergy: Symptoms and diagnosis.

Food allergy is common and has increased in prevalence over time. Although there are many types of reactions to foods, immunoglobulin E (IgE) mediated food allergy is acute in onset and resolves quickly, and is reproducible in nature. Anaphylaxis is the most severe form of IgE-mediated food allergy and has a range of clinical manifestations. First-line food allergy diagnostic testing is sensitive but not specific and should only be done in the context of a convincing history of a reaction. Oral food challenge is the criterion standard in diagnosis but carries the risk of a reaction. The only therapy for anaphylaxis is immediate intramuscular epinephrine. Traditional management of food allergy consists of avoidance management, with strict avoidance, reading labels, and carrying an epinephrine autoinjector at all times. Although effective, accidental reactions do occur, and this management strategy has a profound impact on quality of life.

Economic burden of food allergy in Canada: Estimating costs and identifying determinants

BackgroundLimited data exist on the economic burden of food allergy (FA). ObjectiveTo assess FA-related direct (healthcare and out-of-pocket) and indirect (lost productivity) costs and their determinants in Canadian children and adults self-reporting FA. MethodsFA-individuals self-reporting a convincing history or physician diagnosis were recruited through FA registries, an anaphylaxis registry, and advocacy associations, and electronically surveyed regarding FA-related healthcare use, out-of-pocket expenditures, and time lost from paid and unpaid labor. Direct and indirect costs (2020 Canadian dollars [CAD]) were stratified on severe reaction vs mild, moderate or no reaction, and children vs adults; multivariate regressions assessed the association between costs and sociodemographic and disease characteristics. ResultsBetween May 2018 and July 2019, 2692 eligible individuals responded (2189 convincing history and 503 physician diagnosis only); 1020 experienced a severe reaction; 1752 were children. Per FA-individual, annual healthcare, out-of-pocket, and indirect costs were $1267, $2136, and $7950. Those with a severe reaction had higher healthcare and out-of-pocket costs than those with mild, moderate or no reaction. FA-children vs FA-adults had higher healthcare and out-of-pocket costs, and lower indirect costs. Multivariate results showed that lower age, a severe reaction ever, multiple FAs, and fair or poor general health were associated with higher healthcare and out-of-pocket costs. Higher age, lower household education and income, and fair or poor general health were associated with higher indirect costs. ConclusionThe economic burden of FA in Canada is substantial, particularly for those with a severe reaction ever, multiple FAs, and fair or poor general health. It is crucial that those most adversely affected are allocated appropriate resources to support disease management.

Prevalence of IgE-mediated cow milk, egg, and peanut allergy in young Singapore children.

BackgroundThe rising prevalence of food allergy reported in the United States, UK, and Australia may be attributable to the rise in peanut allergy prevalence. The food allergy prevalence in other parts of the world such as Asia is, however, less well documented.ObjectiveThis study aimed to evaluate the prevalence of cow’s milk, egg, and peanut allergies in a general population of Singaporean children below 30 months of age.MethodsA total of 4,115 children from the general population who attended well-baby visits between 2011 and 2015 completed standardized questionnaires to elicit a convincing history of food allergy to estimate the population prevalence of food allergies.ResultsThe prevalence of a convincing history of cow’s milk allergy was 0.51% (95% confidence interval [CI], 0.3–0.7), hen’s egg allergy 1.43% (95% CI, 1.1–1.8), and peanut allergy 0.27% (95% CI, 0.12–0.42). Of the 15 of 59 children with a convincing history of hen’s egg allergy who consented, 12 (80%) had corroborative positive skin prick tests.ConclusionThe prevalence of food allergy, in particular peanut allergy, in children below 2 years of age is lower in this South East Asian population than reported in Western cohorts. Further research should focus on deciphering differential risk factors for food allergy across different geographical locations.

Safety of COVID-19 vaccination in patients with polyethylene glycol allergy: A case series

Safety of COVID-19 vaccination in patients with polyethylene glycol allergy: A case series

Separating Fact from Fiction in the Diagnosis and Management of Food Allergy

Separating Fact from Fiction in the Diagnosis and Management of Food Allergy

A case series of neonatal human parecho-virus encephalitis with a seasonality and endemicity

Human parecho viruses are small, non-enveloped, single stranded RNA viruses within parechovirus genus of the large picornaviridae family. In new-born it can cause gastroenteritis, sepsis and encephalitis. We report case series of parecho-virus encephalitis admitted to a tertiary care centre, Perinthalmanna. Parechoviral encephalitis is an endemic disease seasonally present (May-July) in certain regions of Northern part of Kerala. HPeV are viruses that cause CNS infection in the neonatal period, resulting in white matter lesions that can be visualised with MRI. In a retrospective study, 4 newborn babies of neonatal HPeV encephalitis diagnosed on the basis of clinical and radiological findings with a seasonality were assessed. HPeV encephalitis needs to be in differential diagnosis when neonates and young infants present with seizure and sepsis. HPeV infection must be considered in infants with specific pattern of white matter change but no convincing history of a perinatal hypoxic-ischaemic insult.

Les tests cutanés sont-ils utiles pour le diagnostic des réactions d’hypersensibilité aux antalgiques-antipyrétiques-anti-inflammatoires non stéroïdiens ? Contre

Reactions to nonopioid analgesics, antipyretics and nonsteroidal anti-inflammatory drugs (NSAIDs) may result from a specific/allergic hypersensitivity (HS), either IgE-mediated (immediate and very early reactions) or mediated by T-lymphocytes (delayed reactions), or from a non-specific/non-allergic HS (pharmacological « intolerance »), with a frequent cross-reactivity between the various chemical families of drugs, including paracetamol and, sometimes, coxibs. In patients with suspected allergic HS, diagnosis is usually based on a convincing clinical history and/or provocation tests (PT) showing that the patients react to the suspected drug (±other drugs in the same chemical family) but tolerate other (families of) drugs. However, although immediate and non-immediate-reading skin tests (ST) are specific when performed with non-irritant concentrations, they have a low sensitivity (≤25–30 %) and are not validated. In patients with non-allergic HS, skin tests are useless and diagnosis is based on a convincing clinical history and/or responses in PT. Considering also the low diagnostic value of in vitro tests, diagnosis of HS to analgesics-antipyretics-NSAIDs, whether allergic or non-allergic, is primarily based on clinical history of the patients and their responses in PT, and international consensus do not recommend skin tests.

Children of Asian ethnicity in Australia have higher risk of food allergy and early-onset eczema than those in Singapore.

In Western countries, Asian children have higher food allergy risk than Caucasian children. The early-life environmental exposures for this discrepancy are unclear. We aimed to compare prevalence of food allergy and associated risk factors between Asian children in Singapore and Australia. We studied children in the Growing Up in Singapore Towards healthy Outcomes (GUSTO) birth cohort (n=878) and children of Asian ancestry in the HealthNuts cohort (n=314). Food allergy was defined as a positive SPT ≥3mm to egg or peanut AND either a convincing history of IgE-mediated reaction at 18months (GUSTO) or a positive oral food challenge at 14-18months (HealthNuts). Eczema was defined as parent-reported doctor diagnosis. Food allergy prevalence was 1.1% in Singapore and 15.0% in Australia (P<0.001). Egg introduction was more often delayed (>10months) in Singapore (63.5%) than Australia (16.3%; P<0.001). Prevalence of early-onset eczema (<6months) was lower in Singapore (8.4%) than Australia (30.5%) (P<0.001). Children with early-onset eczema were more likely to have food allergy than those without eczema in Australia [aOR 5.11 (2.34-11.14); P<0.001] and Singapore [aOR4.00 (0.62-25.8); P=0.145]. Among Asian children, prevalence of early-onset eczema and food allergy was higher in Australia than Singapore. Further research with larger sample sizes and harmonized definitions of food allergy between cohorts is required to confirm and extend these findings. Research on environmental factors influencing eczema onset in Australia and Singapore may aid understanding of food allergy pathogenesis in different parts of the world.

Exercise-food challenge test in patients with wheat-dependent exercise-induced anaphylaxis.

Wheat-dependent exercise-induced anaphylaxis (WDEIA) is a severe and potentially life-threatening food allergy. Diagnosis of WDEIA is challenging because reactions are not always reproducible. This study aimed to evaluate the positivity rate of exercise-food challenge test at our allergy unit in order to confirm the diagnosis, and to investigate the effect on the episode of reactions after the test. This retrospective evaluation included patients aged 5-60 years who presented at the pediatric and adult allergy units of Siriraj Hospital during 2014-2018 with a convincing history of WDEIA and who underwent a 4-day exercise-food challenge test. Demographic data, challenge test result, and episodes of the reaction before and after the challenge test were obtained. Fourteen of the 17 patients that were enrolled were included in the analysis. The 3 excluded patients were found to have IgE-mediated wheat allergy. Median age and time to diagnosis was 18.3 years (range: 10.5-43.4) and 1.8 years (range: 0.3-6.2). History of recurrent acute urticaria before the onset of anaphylaxis was reported in 5 patients (35.7%). Exercise-food challenge test was positive in 10 patients (71.4%). Median mean number of exacerbations per year before and after the confirmation test was 2 (range: 1-10) and 1 (range: 0-3), respectively. For WDEIA, time to diagnosis was delayed, and one-third of patients had recurrent acute urticaria proceeding anaphylaxis onset. Our exercise-food challenge test could be utilized safely in both children and adult and able to elicit symptoms in two-third of patients.

WHEAT AND PEANUT FOOD ALLERGY IN MUGWORT (ARTEMESIA VULGARIS) SENSITIZED PATIENTS: A CASE SERIES OF FOUR CASES

Non-specific lipid transfer proteins (LTPs) are the major food allergens in mugwort-Pollen Food Syndrome and are associated with systemic allergic reactions. We evaluated the relationship between mugwort pollen with peanut and wheat. The diagnosis was based on a convincing history of an allergic reaction after their food consumption and positive skin prick test with elevated food-specific immunoglobulin E (IgE) by Immunocap. We speculate that the causative allergenic protein Art V3 nsLTP in artemisia, Arh 9 nsLTP in peanut, and Tria 14 nsLTP in wheat are the major allergenic epitopes which are thermostable and resistant to peptic digestion, causing for systemic reaction in our cases.

Youngest reported child with tryptase >20ug/L, found to have hereditary alpha tryptasemia (HAT)

Guidelines recommend bone marrow biopsy for tryptase >20ug/L, but systemic mastocytosis (SM) is exceedingly rare in children. Hereditary alpha-tryptasemia (HAT) is caused by a duplication or triplication of the alpha-tryptase gene (TPSAB1), leading to overproduction of alpha-tryptase. HAT affects 6% of the population, with autosomal dominant inheritance. We report the youngest child with tryptase level >20ug/L, who is also the youngest child reported with HAT. Bone marrow biopsy was performed and assessed for mast cell infiltrates and KIT mutation, in addition to staining for tryptase and CD117. Serum and urine PGD2 levels were sent to rule out mast cell activation. Genetic testing was performed from a buccal swab using DNA amplification via digital droplet polymerase chain reaction for TPSAB1. A 3-month-old girl with mosaic Turner syndrome presented to her pediatrician with recurrent episodes of flushing, and gastroesophageal reflux requiring omeprazole. She was otherwise well with no features of SM. Tryptase level at 8 months was 19.8ug/L (normal <11.1ug/L), and 25.3ug/L when repeated. Complete blood count, liver, and renal function tests were normal. Bone marrow biopsy did not reveal evidence of mast cell infiltrate or KIT mutation. Immunohistochemical staining for tryptase and CD117 was negative. Serum and urine PGD2 levels were normal. Amplification of TPSAB1 revealed duplication, consistent with HAT. We propose that in the absence of convincing history of SM, children with elevated tryptase should first have HAT genetic testing prior to bone marrow biopsy, due to the rarity of childhood SM and genetic testing being less invasive.