[DEVELOPMENT AND VALIDATION OF QUALITY OF LIFE QUESTIONNAIRE FOR PARENT BURDEN OF CHILDREN WITH FOOD ALLERGY].

OBJECTIVE: Quality of life (QOL) questionnaires for parents of children with food allergies have been developed in the United States and Europe. However, no original Japanese QOL questionnaire has been developed till date. We aimed to develop an original questionnaire to evaluate the QOL in parents of children with food allergies in Japan. METHODS: We collected QOL-related questions from parents of children with food allergies aged 0-15 years, and created a primary questionnaire. Responses to the primary questionnaire were obtained from the parents again, and question items were reduced using factor analysis to create a secondary questionnaire comprising eight items. In addition to the secondary questionnaire, responses to the Food Allergy QOL Questionnaire-Parent Form (FAQLQ-PF) Japanese version, Parent reported Health-Related QOL in children and adolescents (KINDL) and Health-related QOL (SF-8) were obtained from parents to assess the validity of the secondary questionnaire. RESULTS: A total of 407 parents completed all questionnaires. The secondary questionnaire scores were positively correlated with those of FAQLQ-PF and weakly negatively correlated with the KINDL and SF-8 mental component summary scores. Parents of children with food allergies with ≥3 culprit foods or severe reactions to daily foods, a history of anaphylaxis, and those carrying adrenaline autoinjectors scored higher and had lower QOL. CONCLUSION: The developed original questionnaire is a valid QOL questionnaire for Parents of children with food allergies.

[EVALUATION OF ADRENAL CORTICAL FUNCTION USING SALIVARY CORTISOL MEASUREMENTS IN CHILDREN WITH BRONCHIAL ASTHMA].

BACKGROUND: When corticosteroids are used for a long time in patients with bronchial asthma, a decrease in adrenal cortex function occurs. We investigated the use of salivary cortisol measurements as a simple, noninvasive method for the evaluation of the adrenal cortex function in pediatric asthmatic patients. METHODS: Plasma and salivary cortisol levels were measured from 8:00 am to 10:00 am in 248 pediatric asthmatic patients aged 0-18 years that were under long-term care management at the National Hospital Organization Fukuoka National Hospital in 2011-2013. We determined the correlation between plasma and salivary cortisol levels and calculated a salivary cortisol cut-off value for screening adrenocortical function. RESULTS: There was a significant positive correlation between salivary cortisol levels and plasma cortisol levels in all patients, patients under age 3, and patients over age 3 (r=0.759, r=0.563, r=0.827, respectively). Salivary cortisol cut-off values were 0.015µg/dL for plasma cortisol levels below 3µg/dL (sensitivity 83%, specificity 82%), and 0.045µg/dL those below 5µg/dL (sensitivity 83%, specificity 75%). 5µg/dL or less is an abnormal value, and 3µg/dL or less is considered to be adrenal insufficiency. CONCLUSION: Salivary cortisol levels were positively correlated with plasma cortisol levels in pediatric asthmatic patients. This is a useful method for frequently monitoring young children with adrenal dysfunction or severe asthma that are treated with high doses of inhaled corticosteroids.

Risk prediction of severe reaction to oral challenge test of cow's milk.

Cow's milk is one of the most common food allergens among children. Oral food challenge tests determine the threshold dose of allergens, but have not been standardized. To reduce the severe reactions, we developed a practical model of the test. We studied 111 high-risk patients who underwent a first milk oral food challenge on the risk-stratified dose between 2011 and 2017 for predicting the severe reaction risk. Severe reactions were defined as showing > 3 of Sampson's classification grade. Twenty-eight patients (25%) showed severe reactions without death. Prior to oral food challenge, severe reaction patients experienced milk avoidance (71% vs. 45%, p = 0.02) or bronchial asthma (61% vs. 28%, p = 0.003) more frequently and showed higher milk-specific IgE levels (median 28.3 vs. 7.7 UA/mL, p < 0.0001) than non-severe reaction patients. Multivariate logistic regression analyses established a formula including severe reaction-associated factors; increased levels of milk-specific IgE (odds ratio 11.61, p = 0.001), milk avoidance (odds ratio 3.88, p = 0.02), and bronchial asthma (odds ratio 3.75, p = 0.02). This model had 86% sensitivity and 56% specificity (cut-off 0.25) for risk. Five patients with < 25% probability developed severe reactions, which started in > 3 grade dyspnea up to 20 mL of challenge.Conclusion: This model could effectively reduce the severe reaction development on the first milk oral food challenge test according to the individual needs. What is Known: •Higher levels of milk-specific IgE values, bronchial asthma, and complete milk avoidance are independent risk factors of severe reactions during the cow's milk oral food challenge. What is New: •Statistical analyses of our milk oral food challenge records for 111 patients helped us develop a model formula predicting severe reactions at the first test with high specificity and sensitivity. •This simple risk-stratified protocol is useful for minimizing the adverse events in the first milk challenge.

[POLLEN FOOD ALLERGY SYNDROME IN A FIVE-YEAR-OLD BOY AFTER MOVING HOUSE AT THE FOOT OF THE MOUNTAIN: A CASE REPORT].

We report the case of a boy with a history of atopic dermatitis starting in infancy. At the age of four, his family moved into a newly built house at the foot of a mountain. One year later, he was diagnosed with Japanese Cedar pollinosis. During the same year, in March, he began to experience oral symptoms, hoarseness, and coughing, after eating multiple types of fruits and vegetables, like soybeans, apples, etc. His tests for Bet v1 and the pathogenesis-related protein-10 (PR-10) of the corresponding foods were positive; accordingly, he was diagnosed with Pollen Food Allergy Syndrome (PFAS). In order to investigate the relationship between pollen and food allergies, we counted the pollen grains dispersed at the patient's house during a period of one year and measured his specific IgE titers for pollen and food allergens every three months. We found a large amount of Japanese cedar, cypress, oak, and various other species of pollen dispersed at the patient's house. All counts were higher than the average pollen counts in the city of Fukuoka. After the seasonal dispersal of oak pollen, the patient's specific IgE antibody titers against Alder, Oak, Bet v1, Gly m4, and PR-10 protein group of fruits increased, although alder pollen was not detected. We thus inferred that the patient had developed PFAS by exposure to a large amount of Fagales species pollen, including oak.

Infantile hypophosphatasia combined with vitamin B6-responsive seizures and reticular formation lesions on magnetic resonance imaging: A case report.

BACKGROUND: Hypophosphatasia (HPP) is a rare genetic disorder characterized by rachitic bone manifestations and a low serum alkaline phosphatase (ALP) level. It is caused by mutations in the tissue non-specific alkaline phosphatase (TNSALP) gene, which encodes the tissue non-specific isozyme of ALP. HPP patients exhibit various presentations depending on their age at onset, such as infantile HPP combined with vitamin B6-responsive seizures. CASE PRESENTATION: A newborn with infantile HPP presented with tonic convulsions from day 5 after birth and received intravenous vitamin B6 (10mg/kg/day pyridoxal phosphate). Eleven days later, frequent apneic episodes occurred, and head magnetic resonance imaging (MRI) showed bilateral reticular formation lesions in the brain stem, including the medulla oblongata. After the pyridoxal phosphate dose was increased (to 40mg/kg/day), the patient's seizures and apnea resolved, and her MRI findings also improved. Genetic testing revealed that she was homozygous for the 1559delT mutation of TNSALP. CONCLUSIONS: High-dose pyridoxal phosphate is a useful treatment for HPP-induced seizures and might improve reticular formation lesions.

Age-associated effect of kestose on Faecalibacterium prausnitzii and symptoms in the atopic dermatitis infants.

BACKGROUND: Although Faecalibacterium prausnitzii is a major bacterium in the intestine of adults, which is known to have anti-inflammatory effects, the development in infants or the response to prebiotics remains unclear. METHODS: The counts of F. prausnitzii in the feces were examined by real-time polymerase chain reaction (PCR). Fecal samples were obtained from 65 atopic dermatitis (AD) infants who participated in a randomized controlled clinical trial to investigate the therapeutic effect of kestose, the smallest fructooligosaccharide. RESULTS: Although the F. prausnitzii count was undetectable level in most 0- to 1-y-old infants, the count reached a level comparable to that in adults in 2- to 5-y-old infants. The bacterial number increased about 10-fold by oral administration of kestose every day for 12 wk in the younger infants, but not so much in the older infants. This bacterial increase was significantly correlated with an improvement in the AD symptoms in the older infants. CONCLUSION: The F. prausnitzii population in the intestine reaches a level comparable to that in adult at approximately 2 y of age. Kestose efficiently stimulates the growth of this bacterium in the intestine, which might lead to an improvement in AD symptoms in infants.

Blue rubber bleb nevus syndrome with knee joint disorder.

Blue rubber bleb nevus syndrome (BRBNS) involves cutaneous vascular malformation characterized by multiple venous malformations. This commonly affects the skin and gastrointestinal tract. BRBNS is associated with anemia and occasionally involves orthopedic manifestations. A 6-year-old boy was referred to hospital for evaluation of anemia. He presented with a rubber-like soft-tissue mass in the left knee and the right side of the neck, recurrent pain, and fixed flexion contracture of the knee. Blood examination indicated consumption coagulopathy and anemia caused by not only iron-deficiency anemia but also microangiopathy. Endoscopy of the gastrointestinal tract indicated multiple bluish-black sessile venous malformations. Ultrasonography and magnetic resonance imaging of the knee showed intra-articular and intramuscular involvement. Based on these findings, BRBNS with knee joint disorder was diagnosed. With regard to vascular malformations, like other diseases such as inflammatory arthropathy, ultrasonography of the joint may become a new diagnostic approach for evaluating orthopedic manifestations.

[Treatment of facial lesion in infantile and childhood atopic dermatitis].

BACKGROUND: Treatment of infantile and childhood atopic dermatitis needs special consideration compared to adult's counterpart. Repeated exposure to irritation by slaver or rubbing makes facial lesions difficult to treat in infants and children. We considered the deterioration factor and the treatment that we performed. METHODS: We considered 6 infantile and childhood atopic dermatitis who had severe condition on their face. RESULT: All the patients needed enough application of topical steroids. Exacerbation of atopic dermatitis masked concomitant skin infection such as impetigo or Kaposi's varicelliform eruption. Guardian usually hesitated periorbital and perioral application of topical steroids and tacrolimus. In order to obtain the guardian's understanding and cooperation, it was very important to establish a good doctor/guardian relationship by educational approach including an enough explanation about treatment policy. CONCLUSION: It was important for the treatment of infantile and childhood atopic dermatitis to identify and eliminate triggering factors. And we need to obtain the guardian's understanding and cooperation for our treatment policy.

Lung sound analysis in a patient with vocal cord dysfunction and bronchial asthma.

OBJECTIVE: Vocal cord dysfunction (VCD) is a condition characterized by adduction of the vocal cords, resulting in narrowing or even closure of the glottis during inspiration. This can cause wheezing that originates at the site of narrowing. Some patients have both VCD and asthma. In such cases, an acute episode of VCD can be difficult to differentiate from that of asthma. We tested the usefulness of lung sound analysis (LSA) in such a condition. METHODS: We performed an LSA in a patient with asthma and coexisting VCD diagnosed using laryngoscopy. RESULTS AND CONCLUSION: The LSA during an acute VCD episode revealed monophonic continuous adventitious sounds that were distributed symmetrically over both lung fields. The time domain analysis revealed that the adventitious sounds originated in the neck. These LSA findings clearly indicated that the acute episode was not due to asthma but due to VCD. This case illustrates that the LSA may be a useful tool to differentiate between an acute episode of asthma and that of VCD.

[Effect of weight reduction on respiratory function in obese children with asthma].

BACKGROUND: Though the epidemiological relationship between obesity and asthma has been widely investigated, the mechanism is less known. Several studies have examined that weight loss improved lung function in adults. However, it has not been systematically studied in children. We investigated the effect of weight reduction on respiratory function in obese children with asthma. METHODS: We selected 10 obese children aged 7-13 years with physician diagnosed asthma who was subjected the weight reduction program in Fukuoka National Hospital in 2003-2007. Obesity was defined by BMI >90 percentile of the same age population. Before and after the program, they performed the spirometry. And they were measured the height, the weight, the %Fat, and the Fat volume. Logistic linear analysis was conducted to determine the correlation between relative change in each obesity markers and relative change in each respiratory parameter. RESULTS: Reduction of BMI, %Fat, and Fat volume correlated with relative changes in %FVC and %FEV1. Reduction of %Fat and Fat volume also correlated with relative changes in %PEF and %V50. CONCLUSION: Reduction of BMI, %Fat, and Fat volume can improve respiratory function in obese children with asthma. Obesity may be contribute to aggravate the symptoms of asthma.

[Relationship between compliance with inhaled corticosteroids and changes in the fraction of exhaled nitric oxide during summer camp for asthmatic children].

BACKGROUND: Recently, the fraction of exhaled nitric oxide (FENO), which can be measured easily and noninvasively even in children, has attracted attention as a method of evaluating airway inflammation. The aim of this study was to investigate the relationship between compliance with inhaled corticosteroids (ICS) before a summer camp for asthmatic children and the changes in the FENO during camp. METHODS: Fifty asthmatic children (26 boys and 24 girls) aged 6-12 years old were recruited from the Fukuoka National Hospital Summer Camp between 2008 and 2010. We measured their FENO on the first and last day of camp. The children were taught appropriate inhalation methods by pediatric pulmonologists and nurses and performed ICS under their supervision every day during the camp. Before the camp, we asked the participants to complete a questionnaire regarding the use of ICS. The participants were classified into four groups according to their adherence to this therapy. The changes in FENO were then examined and compared among the groups. RESULTS: The FENO measured on the last day of camp was significantly lower than that measured on the first day of camp among children who tended to forget to perform ICS. However, no significant difference in the FENO measurements were observed during the camp, when the children were performing ICS every day. CONCLUSION: The FENO decreased significantly after only four days of camp in the poor compliance group. Therefore, teaching children the appropriate method for inhaling ICS is important, especially for children who tend to forget to perform ICS.