A different starting line for allergic march: food protein-induced allergic proctocolitis.

OBJECTIVE: The aim of this study is to investigate the long-term prognosis of food protein--induced allergic proctocolitis (FPIAP) patients, the risk of developing both allergic and gastrointestinal diseases, and to evaluate whether it leads to allergic march. METHODS: A total of 149 children who were diagnosed with FPIAP and developed tolerance at least 5 years prior to the study and 41 children (with no history of food allergy) as a control group were enrolled. Both groups were re-evaluated for allergic diseases as well as gastrointestinal disorders. RESULTS: The mean age of diagnosis for the FPIAP group was 4.2 ± 3.0 months, while the mean age of tolerance was 13.9 ± 7.7 months. The mean age of both FPIAP and control groups at the last visit was 101.6 ± 24.4 and 96.3 ± 24.1 months, respectively (P = 0.213). At the final evaluation of both groups, the comorbid allergic disease was significantly higher in the FPIAP group (P < 0.001). There was no significant difference between the two groups in terms of functional gastrointestinal disorders (FGIDs), eosinophilic gastrointestinal diseases, and inflammatory bowel disease (P = 0.198, 0.579, and 0.579, respectively).In the FPIAP group, the allergic disease was significantly higher at the final visit in patients with comorbid allergic disease at diagnosis (P < 0.001). In the FPIAP group, FGID was significantly higher in the group that developed allergic diseases in the future, compared to the group that did not develop allergic diseases in the future (P = 0.034). The proportion of both FGID and allergic diseases was significantly higher in subjects that developed tolerance at >18 months, compared to subjects that developed tolerance at >18 months (P < 0.001 and <0.001, respectively). CONCLUSIONS: Patients with FPIAP may develop allergic diseases as well as FGID in the long term.

Evaluation of the Clinical Characteristics of Patients with Food Protein-Induced Enterocolitis Syndrome: A Multicenter Study.

INTRODUCTION: Food protein-induced enterocolitis syndrome (FPIES) is a rare non-IgE, cell-mediated food allergy disorder. We aimed to report the demographic characteristics, clinical features, and management of pediatric patients with FPIES. METHODS: This retrospective study included all children diagnosed with FPIES at the pediatric allergy departments of the participating twelve study centers from January 2015 to November 2020. RESULTS: A total of 73 patients (39 males, 53.4%) with a male/female ratio of 1.1 were included in the study. The median (interquartile ranges) age at symptom onset was 6 months (0.5-168, 4-9.5). The most frequent offending foods were cow's milk, egg's yolk, fish, and egg's white, identified in 38.4% (n = 28), 32.9% (n = 24), 21.9% (n = 16) and 20.5% (n = 15) of the patients, respectively. The total number of reported FPIES episodes was 290 (3.9 episodes per child). Oral food challenge (OFC) was performed in 54.8% (n = 40) of the patients, and tolerance was detected in 17 OFCs (42.5%) at a median age of 15 months (range 8-132 months). CONCLUSION: FPIES is a non-IgE-mediated food hypersensitivity that commonly affects infants and is often misdiagnosed. The pathophysiology of the disease remains unclear and the low awareness of FPIES among physicians and parents highlights the need for more education.

Hearing status in vitamin B12-deficient children.

AIM: The aim of this study is to investigate the effects of vitamin B12 deficiency on hearing in school-aged children by pure-tone audiometry. METHODS: Forty-three vitamin B12-deficient children and 37 age-matched control subjects were enrolled in the study. Tympanometric evaluations and pure-tone audiometry including high frequencies were performed on the subjects. The results were compared between the two groups. RESULTS: Both right and left ear pure-tone hearing thresholds (PTHTs) at 0.25-4 kHz, and four-frequency pure-tone average values were significantly better in the control group compared with the patient group (P < 0.05). However, PTHTs at 8-16 kHz were not different between the two groups (P > 0.05). Vitamin B12 level also did not show any significant correlation with the PTHTs at 0.25-16 kHz (P > 0.05). CONCLUSION: This study indicates that vitamin B12 deficiency may contribute to hearing impairment at low frequencies as a possible aetiological factor in children.

Is Oral Food Challenge (OFC) test safe for preschool children?

BACKGROUND: Oral food challenges (OFCs) are performed for diagnosis of a food allergy in cases where the allergy is not supported by patient history, or when a newly developed tolerance level needs to be established. OBJECTIVE: We aimed to investigate the prevalence and severity of reactions during OFCs in preschool children. METHODS: A retrospective study was conducted on children younger than 5 years, for whom OFC had been performed with milk, egg white and egg yolk. All children had been admitted to the Department of Pediatric Allergy at Behçet Uz Children's Hospital between 1 January 2010 and 31 December 2014. Any symptoms developed during the OFC were classified and recorded. RESULTS: A total of 122 patients who underwent an OFC were included in this study. The patients included 85 males (69.7%), and 50.8% of patients (n = 62) had a history of IgE-mediated food allergy. Co-existing allergies were found for 57.4% (n = 70) of patients. Of the OFCs performed, tests for milk, egg white and egg yolk made up 46.5, 30.5 and 23.0%, respectively. Of these, 19% (n = 33) were mild and 4.5% (n = 7) were moderate allergies in terms of symptom development. It was determined that the skin prick test (SPT) wheal size and the food-specific IgE levels did not effect in determining whether the allergic reaction would develop by OFC if the SPT wheal size and the food-specific IgE levels were below the cut-off point of a 95% positive predictive value (p > 0.05). CONCLUSION: The severity of egg and milk allergy symptoms resulting from the frequently used OFC in preschool children are generally mild and easy to manage, particularly if the OFC is only conducted if serum-specific IgE or SPT wheal size is below the cut-off point.

Can mean platelet volume be used as a biomarker for asthma?

INTRODUCTION: Platelets play important roles in airway inflammation and are activated in inflammatory lung diseases, including asthma. AIM: We evaluated the mean platelet volume (MPV), used as a marker of platelet activation, in asthmatic patients during asymptomatic periods and exacerbations compared to healthy controls to determine whether MPV can be used as an indicator of inflammation. MATERIAL AND METHODS: Our patient group consisted of 95 children with exacerbation of asthma who were admitted to our allergy clinic. The control group consisted of 100 healthy children matched for age, gender, and ethnicity. Mean platelet volume values of the patient group obtained during exacerbation of asthma were compared to those of the same group during the asymptomatic period and with the control group. We investigated factors that can affect the MPV values of asthma patients, including infection, atopy, immunotherapy treatment, and severity of asthma exacerbation. RESULTS: The patient group consisted of 50 (52.6%) boys and 45 (47.4%) girls with a mean age of 125 ±38 months old. Mean MPV values in the exacerbation period, the healthy period, and in the control group were 8.1 ±0.8 fl, 8.1 ±1.06 fl, and 8.2 ±0.9 fl, respectively; there were no significant differences between groups (p > 0.05). The severity of asthma, severity of asthma exacerbation, immunotherapy, coinfection, eosinophil count, and IgE level also had no effect on MPV (p > 0.05). CONCLUSIONS: Although platelets play a role in the pathophysiology of asthma, MPV measurement is insufficient to detect inflammation through platelets.

Coexistence of hereditary angioedema in a case of familial Mediterranean fever with partial response to colchicine.

Hereditary angioedema (HAE) is a very rare and potentially life-threatening genetic disease characterised by episodes of edema in various parts of the body, including the extremities, face, and airway. The disease is usually associated with attacks of abdominal pain. On the other hand, familial Mediterranean fever (FMF) is an inherited condition characterised by recurrent episodes of painful inflammation in the abdomen, chest, or joints. In this report, we present a child with FMF and undiagnosed HAE, which made him a partial responder to colchicine treatment. Consequently, HAE must be considered in differential diagnosis of cases in which a partial response is obtained from FMF treatment, particularly in countries where FMF is frequently encountered, because early diagnosis of HAE can facilitate prevention of life-threatening complications, such as upper airway obstruction. To our knowledge, our patient is the first patient reported in the literature with the diagnosis of HAE and FMF together.

Screening for urinary tract infection with the Sysmex UF-1000i urine flow cytometer.

The diagnosis of urinary tract infection (UTI) by urine culture is time-consuming and can produce up to 60 to 80% negative results. Fast screening methods that can reduce the necessity for urine cultures will have a large impact on overall turnaround time and laboratory economics. We have evaluated the detection of bacteria and leukocytes by a new urine analyzer, the UF-1000i, to identify negative urine samples that can be excluded from urine culture. In total, 1,577 urine samples were analyzed and compared to urine culture. Urine culture showed growth of ≥10(3) CFU/ml in 939 samples (60%). Receiver operating characteristics (ROC) curves and ROC decision plots were been prepared at three different gold standard definitions of a negative urine culture: no growth, growth of bacteria at <10(4) CFU/ml, and growth of bacteria at <10(5) CFU/ml. Also, the reduction in urine cultures and the percentage of false negatives were calculated. At the most stringent gold standard definition of no growth, a chosen sensitivity of 95% resulted in a cutoff value of 26 bacteria/µl, a specificity of 43% and a reduction in urine cultures of only 20%, of which 14% were false negatives. However, at a gold standard definition of <10(5) CFU/ml and a sensitivity of 95%, the UF-1000i cutoff value was 230 bacteria/µl, the specificity was 80%, and the reduction in urine cultures was 52%, of which 0.3% were false negatives. The applicability of the UF-1000i to screen for negative urine samples strongly depends on population characteristics and the definition of a negative urine culture. In our setting, however, the low workload savings and the high percentage of false-negative results do not warrant the UF-1000i to be used as a screening analyzer.

Severe Cutaneous Adverse Drug Reactions in Pediatric Patients: A Multicenter Study.

BACKGROUND: The severe cutaneous adverse drug reactions (SCARs) are rare but could be life-threatening. These include drug reaction with eosinophilia and systemic symptoms (DRESS), Stevens-Johnson syndrome, toxic epidermal necrolysis (TEN), and acute generalized exanthematous pustulosis. OBJECTIVE: The purpose of this study was the evaluation of the clinical characteristics of patients with the diagnosis of SCARs. METHODS: Patients who were diagnosed with SCARs between January 2011 and May 2016 by pediatric allergy clinics in the provinces of Ankara, Trabzon, Izmir, Adana, and Bolu were included in this multicenter study. Clinical and laboratory findings, the time between suspected drug intake and development of clinical findings, treatments they have received, and length of recovery time were recorded. RESULTS: Fifty-eight patients with SCARs were included in this study. The median age of the patients was 8.2 years (interquartile range, 5.25-13 years) and 50% (n = 29) were males. Diagnosis was Stevens-Johnson syndrome/TEN in 60.4% (n = 35), DRESS in 27.6% (n = 16), and acute generalized exanthematous pustulosis in 12% (n = 7) of the patients. In 93.1% of the patients, drugs were the cause of the reactions. Antibiotics ranked first among the drugs (51.7%) and antiepileptic drugs were the second (31%) most common. A patient who was diagnosed with TEN developed lagophthalmos and a patient who was diagnosed with DRESS developed secondary diabetes mellitus. Only 1 patient with the diagnosis of TEN died. CONCLUSIONS: SCARs in children are not common but potentially serious. Early diagnosis and appropriate treatment of SCARs will reduce the incidence of morbidity and mortality.

Changing epidemiology of non-cystic fibrosis bronchiectasis.

Non-cystic fibrosis bronchiectasis again becomes a major health problem due to inappropriate antibiotic use and increasing frequency of protracted bacterial bronchitis. The aim was to determine the changes in etiology of bronchiectasis. Patients who admitted to Behçet Uz Children Hospital between 2005 and 2015 (n=110) were retrospectively examined. The etiology of bronchiectasis was detected as; primary ciliary dyskinesia 26.4%, protracted bacterial bronchitis 22.8%, primary immune deficiency 11.8%, bronchiolitis obliterans 8.2%, lung disease secondary to gastro-esophageal reflux 3.7%, foreign body aspiration 2.7%, tuberculosis %2.7, congenital malformation 1.8% and asthma 1.8%, respectively. In 15.4% of cases, etiology was not identified clearly. 91% of the patients were medically treated. In ten years, the frequency of asthma and tuberculosis in etiology had decreased but primary ciliary dyskinesia and primary immune deficiency had increased. Non-cystic fibrosis bronchiectasis can be followed up for a long time with medical treatment.

Primary hyperparathyroidism as an extremely rare cause of secondary myelofibrosis in childhood.

Primary hyperparathyroidism (PHP) and myelofibrosis are rare entities in childhood. Myelofibrosis secondary to PHP is also extremely rare. We report a 15-year-old boy presented with generalized weakness, vomiting, and pallor. A parathyroid adenoma was detected on the left distal parathyroid gland. PHP was diagnosed together with hepatosplenomegaly and pancytopenia. Bone marrow biopsy revealed grade 3-4 reticulin fibrosis. As early as 2 months after the left distal parathyroidectomy, hematologic parameters improved without any other intervention. His liver and spleen also gradually decreased in size. We concluded that the pancytopenia was as a result of myelofibrosis from PHP.