The association of fecal calprotectin and respiratory exacerbation in cystic fibrosis patients.

BACKGROUND: CF patients experience several episodes of pulmonary exacerbations and reduction in their lung function progressively. Lung function is not the only diagnostic index by physicians to decide if CF patients require antibiotic therapy following pulmonary exacerbations. Non-invasive fecal indicators are increasingly being used to assess intestinal inflammation. Calprotectin is the most extensively utilized fecal biomarker in recent CF researches. METHODS: In this longitudinal study, 30 CF patients (1-18 years) without current infectious gastroenteritis were recruited from Mofid Children's Hospital and Masih Daneshvari Hospital, Tehran, Iran. Then, fecal calprotectin levels were evaluated before treatment, two weeks after systemic antibiotic administration, as well as recurrence of pulmonary exacerbation after first post-hospital discharge. RESULTS: The initial fecal calprotectin level in CF patients receiving antibiotics was 651.13 ± 671.04, significantly decreasing two weeks after antibiotic therapy and following recurrence (171.81 ± 224.40, 607.93 ± 549.89, respectively; P < 0.01). Following systemic antibiotic treatment, the patient's respiratory and GI symptoms improved (P < 0.01). CONCLUSION: Our findings revealed that fecal calprotectin modifications are associated with CF pulmonary exacerbations and antibiotic treatment could reduce calprotectin levels. Therefore, the fecal calprotectin level could be considered as a diagnostic tool and an index to follow the response to treatment in CF pulmonary exacerbations.

Re-positive PCR of SARS-CoV-2 in health care persons during COVID-19 pandemic.

Reinfection rate with SARS-CoV-2 and degree of protection by the induced antibody after the first episode of the infection is not well known, so it makes a big dilemma for health care personnel (HCP) who work in the front line of combating SARS-CoV-2. In this study, we investigated the frequency of SARS-CoV-2 redetection among HCP after the initial onset of the infection in a children's hospital during one year. Out of 131 seropositive HCP, 13.7% of them were symptomatic and PCR positive during 74-360 days after first sampling. Analysis of demographic data of seropositive HCP showed a correlation between a higher number of family members, higher body mass index, and the existence of underlying diseases with SARS-CoV-2 redetection. In conclusion, reinfection is one of the important problems in the SARS-CoV-2 pandemic. Research on this topic can help us to find answers to questions for estimating the duration of human protection with produced immunity after the infection or vaccination.

Persistent elevation of aspartate aminotransferase in a child after incomplete Kawasaki disease: a case report and literature review.

BACKGROUND: Interpretation of abnormalities in liver function tests, especially in asymptomatic children, is a common problem faced by clinicians. Isolated elevation of aspartate aminotransferase may further puzzle physicians. Macro-aspartate aminotransferase (AST) results from complexes AST produces with other plasma components, such as immunoglobulin. To our knowledge, this is the first report on a case of macro-AST-associated incomplete Kawasaki disease (KD). It is to make physicians aware of this benign condition and help to prevent extensive, unnecessary investigations and invasive workups. CASE PRESENTATION: A 16-month old boy with a 7-day history of fever was admitted to our pediatric ward for pyrexia workup. After complete investigations, KD was confirmed by a pediatric rheumatologist. During his admission and serial follow-up tests, an isolated AST elevation was noted. Comprehensive tests were performed and using the polyethylene glycol (PEG) precipitation method, macro-AST was confirmed. The patient has been followed up for 3 years, and so far, the benign nature of this condition has been confirmed. CONCLUSION: Clinicians should consider testing for macro-AST when elevated AST is the only abnormal lab finding. Although an uncommon finding, macro-AST may be seen in both children and adults. There are many reasons for this phenomenon, including resolved acute hepatitis or in some cases, inflammatory bowel disease, hepatic malignancy, monoclonal gammapathy, celiac disease, or KD; however, it may be observed in asymptomatic healthy children as well. Using the PEG precipitation method, a definitive diagnosis can be made. In none of these conditions does macro-AST have any prognostic significance. An appreciation of macro-AST may prevent the need for more invasive investigations to which patients may be unnecessarily subjected. It is important to recognize this condition as benign and assure patients that no specific treatment is required.

Evaluating the association between neonatal mortality and maternal high blood pressure, heart disease and gestational diabetes: A case control study.

BACKGROUND: Prevention of noncommunicable diseases (NCDs) during pregnancy is recommended due to severe complications for mothers and infants. Considering that NCDs have a significant impact on infant mortality, this study was conducted to investigate the relationship between mothers' underlying diseases and gestational diabetes and infant mortality in Iran. MATERIALS AND METHODS: Mothers who referred to the health centers in nine provinces of Iran were included. This case-control study used data collected from pregnant women. There were 1162 cases and 1624 controls. The required data were collected from mothers' health records and through interviews. RESULTS: The chances of neonatal mortality in women with a body mass index (BMI) of 30-35, 1.7 times (odds ratio [OR] = 1.7, confidence interval [CI]: 1.19-2.44, P = 0.003) was higher compared with women with a normal BMI. The chance of neonatal mortality among mothers with high blood pressure was three times higher compared with healthy mothers (OR = 3.04, 95% CI: 1.98-4.65, P < 0.001). The chance of neonatal mortality in women with kidney disease was also 1.64 times higher than mothers without kidney problems (OR = 1.64, 95% CI: 1.1-2.45, P = 0.015). In the study of gestational diabetes, the chance of neonatal mortality among the mothers who had at risk was 1.63 times higher than mothers without gestational diabetes (OR = 1.63, 95% CI: 0.84-3.16, P = 0.014). Furthermore, the chance of neonatal mortality among the mothers who had heart disease was 1.10 times higher than mothers without heart disease (OR = 2.10, 95% CI: 0.88-4.99, P = 0.014). CONCLUSION: This study showed that undiagnosed underlying diseases were related to neonatal mortality, which highlights the importance of caring for and counseling about the underlying diseases, screening, and controlling blood sugar levels before and during pregnancy to prevent infant mortality by all means possible.

Food allergy among Iranian children with inflammatory bowel disease: A preliminary report.

BACKGROUND: Evidence has shown a link between allergic disease and inflammatory bowel diseases (IBDs). We investigated food allergy in Iranian pediatric IBD patients. MATERIALS AND METHODS: A cross-sectional study was conducted on a consecutive sample of children with newly diagnosed IBD referring to Mofid Children's University Hospital in Tehran (Iran) between November 2013 and March 2015. Data on age, gender, history of cow's milk allergy (CMA), IBD type, routine laboratory tests, and colonoscopic and histopathological findings were gathered. Food allergy was assessed with the skin prick test (SPT). RESULTS: A total of 28 patients including 19 ulcerative colitis (UC), 7 Cronh's disease (CD), and two with unclassified colitis with a mean age of 8.3 ± 4.4 years. (57.1% females, 42.9% were studied. History of CMA was present in eight patients (28.6%). Seventeen patients (60.7%) had at least one food allergy (68.4% of UC vs. 42.9% of CD, P = 0.230). Ten patients (35.7%) had multiple food allergies (36.8% of UC vs. 42.9% of CD, P > 0.999). Common allergic foods were cow's milk (28.6%), beef, seafood, albumen, wheat, and walnuts (each 10.7%), and peanuts and chestnuts (each 7.1%). The SPT showed CMA in 68.4% (8/17) of UC but none of the CD patients (P = 0.077). CONCLUSION: Food allergy is frequent in Iranian pediatric IBD patients with CMA being the most common observed allergy. The CMA seems to be more frequent in UC than in CD patients.

Blood lead level evaluation in children presenting with chronic constipation in Tehran-Iran: a cross-sectional study.

Constipation is a common reason for children seeking medical care worldwide. Abdominal complaints and constipation are also common in lead-poisoned children. This study evaluates the prevalence of abnormal blood lead levels (BLL) among pediatric and adolescent patients and examines the association of constipation with elevated BLL. This was a prospective data collection of patients younger than 18 years old with the chief complaint of constipation seen in the Mofid Children's Hospital gastroenterology clinic and Loghman Hakim pediatric and pediatric gastroenterology clinics were eligible for enrollment in this study. Constipation was defined as infrequent or difficult defecation according to ROME IV criteria lasting 2 months or more. BLL was measured with a fresh capillary whole blood capillary sample. The LeadCare II device assays BLL using an electrochemical technique (anodic stripping voltammetry). A total of 237 patients were enrolled in the study. 122 (51.48%) were female and 115 (48.52%) were male. About one fifth of patients (49; 20.67%) had BLL ≥ 5 µg/dL. The mean BLL in the sample was 3.51 µg/dL. Abdominal pain was the most common symptom accompanying constipation (134; 56%). Multivariate analysis found endoscopic evaluation (P values 0.024, OR 3.646, 95% CI 1.189-11.178), muscle pain (P values 0.020, OR 24.74, 95% CI 1.67-365.83), and maternal education (P values 0.02, OR 4.45, 95% CI 1.27-15.57) with significant differences in groups of patients with normal and elevated BLL. Elevated BLL necessitates an assessment and plans to reduce childhood lead exposure. BLL screening in childhood constipation with refractory chronic abdominal pain may also eradicate the need for invasive procedures like endoscopic evaluation.

Right-sided aortic arch with aberrant left subclavian artery diagnosed in an infant with regurgitation.

Right-sided aortic arch with aberrant left subclavian artery is a rare congenital anomaly of the aorta that occurs in less than 0.1% of the population. Patients are asymptomatic in most cases, and the anomaly is found incidentally; however, symptoms can occur due to the compression of other structures, mostly the trachea and esophagus. In this report, we present a case of esophageal compression by a right-sided aortic arch with aberrant left subclavian artery that mimicked gastro-esophageal reflux in a 3-month-old (87-day-old) infant with complaint of regurgitation, vomiting, and failure to gain weight who was diagnosed through a barium meal study.

Increased regulatory T cells in peripheral blood of children with eosinophilic esophagitis.

AIM: Considering the allergic basis of Eosinophilic esophagitis (EoE), this study was conducted to evaluate peripheral blood Tregs in children with EoE. BACKGROUND: Eosinophilic esophagitis (EoE) is an allergic inflammatory disease of gastrointestinal tract. Regulatory T cells (Tregs) have a confirmed role in allergic disorders. METHODS: Children with EoE, gastroesophageal reflux disease (GERD) and healthy controls (HC) (10 subjects in each group) were recruited after diagnosis by a pediatric gastroenterologist and allergist. After obtaining informed written consent, peripheral blood was obtained. Peripheral blood mononuclear cells were isolated by Ficoll gradient centrifugation. Flowcytometry was used to enumerate peripheral blood Tregs (CD4 +CD25 +FOXP3+ gated lymphocytes were considered as Tregs). RESULTS: CD4+ gated lymphocytes significantly increased in EoE and GERD groups compared to HC group (p= 0.018). Tregs also was significantly increased in EoE in comparison to HC group (p=0.016). There were no statistically significant differences in Tregs of EoE as compared to GERD subjects (p=0.085). CONCLUSION: Peripheral blood Tregs increase in patients with EoE as compared to healthy controls, which may be indicative of a feedback mechanism to regulate inflammatory responses.

Diagnostic Challenges in the Early Onset of Inflammatory Bowel Disease: A Case Report.

Inflammatory bowel disease (IBD) with very early onset manifestations (younger than six years of age) is an essential pediatric gastrointestinal disease that encompasses a group of diverse and rare genetic defects. It may be associated with chronicity, premalignant nature, and high morbidity and mortality during childhood. Because of overlapping phenotypes, the definitive diagnosis based on conventional strategies is frequently a challenge. However, many patients with different molecular pathologies are treated with the same therapeutic strategy. In this context, it is essential to define a more reliable method to provide an opportunity for a rapid and accurate diagnosis. Here we report a novel homozygous exonic variant in a patient with an IBD-like lesion in the colon during the infancy period. A 7 months old boy who was born of a consanguineous marriage developed gastrointestinal disorders early in life. After complete diagnostic workups, this case underwent conventional therapy of IBD for five months; but clinical remission was not achieved. We identified a novel homozygous mutation (c.684C>T p(=)) in exon 7 of IL-12RB1 gene that in silico studies indicated its significance in the splicing process. At the 14th month of age, this case died. Our finding reveals the importance of genetic screening as an early diagnostic tool in the identification of the underlying causes of IBD with very early onset manifestations, particularly infantile (< 2 years of age) IBD. This strategy makes an opportunity in prompt diagnosis and targeted therapy.

Assessing the Prevalence and Treatment of Malnutrition in Hospitalized Children in Mofid Children's Hospital During 2015-2016.

BACKGROUND: Malnutrition in hospitalized patients causes problems in treatment and increases hospitalization duration. The aim of this research was to determine the prevalence of malnutrition in hospitalized children. METHODS: Children aged 1 month to 18 years (n = 1186) who were admitted to medical and surgery wards of Mofid children's hospital from November 2015 to February 2016, entered the study. We measured different anthropometric variables in patients with malnutrition. Also, nutritional counseling was performed and three months follow-up was done. RESULTS: Patient data were registered in questionnaires particularly for children 2 years old and less. 597 children under 2 years of age and 607 children over two years entered the study. The data analysis was done by SPSS version 22.0 (Chicago, IL, USA). The t test inferential method was used in comparing variables. P values less than 0.05 were considered statistically significant. Based on the body mass index (BMI) Z score, and in accordance with the World Health Organization (WHO) cut-off, among children over 2 years, 9% were diagnosed as overweight or obese, 54% were within the normal range and 37% were underweight at time of admission. In the underweight group, 43% were mildly, 21.2% were moderately and 35.8% were severely underweight. Based on the weight for length Z score in patients less than 2 years of age at time of admission, 6% were overweight, 60% were in normal range and 34% were underweight. Among children with malnutrition, 21% had mild, 3.0% had moderate and 10% had severe malnutrition. No significant meaningful relation was found between prevalence of malnutrition and severity of illness. In the moderate to severe undernutrition group, nutritionist counseling was done. Comparison of BMI and weight, before and after admission (the baseline and the follow up visits), was done by means of repeated measurements. Comparison of the patient's weight at time of admission with weight at 1, 2 and 3 months after the first nutritional consultation showed statistically meaningful difference (P value < 0.05). CONCLUSION: Growth indices need to be evaluated in every hospitalized child. Nutritional consultation is useful in children with malnutrition. The main purpose of early diagnosis of malnutrition is to prevent its progression, and also to design a useful, applicable and cost-effective nutritional intervention for malnutrition treatment.

Very early onset inflammatory bowel disease: Investigation of the IL-10 signaling pathway in Iranian children.

BACKGROUND & AIM: Comparing to adult inflammatory bowel disease (IBD), those with early onset manifestations have different features in terms of the underlying molecular pathology, the course of disease and the response to therapy. We investigated the IL-10 signaling pathway previously reported as an important cause of infantile (Very Early Onset) IBD to find any possible variants. METHOD: With the next generation sequencing technique we screened IL-10, IL-10RA and IL10RB genes of 15 children affected by very early onset-GI (gastrointestinal) disorders. Additionally, we analyzed them based on Thermo Fisher immune deficiency panel for genes either having a known role in IBD pathogenesis or cause the disorders with overlapping manifestations. We performed multiple functional analyses only for the cases showing variants in IL-10- related genes. RESULT: In 3 out of 15 patients we identified variants including a homozygous and heterozygote mutations in IL-10RA and a novel homozygous mutation in IL-12RB1. Our functional studies reveal that in contrast to the IL-10RA heterozygote mutation that does not have deleterious effects, the homozygous mutation abrogates the IL-10 signaling pathway. CONCLUSION: Our study suggests we need to modify the classical diagnostic approach from functional assays followed by candidate- gene or genes sequencing to the firstly parallel genomic screening followed by functional studies.

Methodology of the Comprehensive Program on Prevention and Control of Overweight and Obesity in Iranian Children and Adolescents: The IRAN-Ending Childhood Obesity (IRAN-ECHO) Program.

BACKGROUND: The World Health Organization program on Ending Childhood Obesity (WHO-ECHO) has developed a comprehensive and integrated package of recommendations to address childhood obesity. The present study, entitled IRAN-ECHO, was designed and implemented in the framework of the WHO-ECHO program. METHODS: The IRAN-ECHO program is implementing multicomponent interventions by considering life course dimensions. The program has two parts: a population approach and an individual approach. The population approach considers different periods in life, including prenatal, infancy, childhood, and adolescence, as well as family and society. The individual approach targets those children or adolescents with overweight or obesity; this part is conducted as a referral system that is now integrated in the current national health system. As part of the population approach, a quasi-experimental study was conducted in six provinces to compare the status before and after implementing parts of the interventions. By intersectoral collaboration with different organizations, multicomponent interventions are conducted for different age groups. RESULTS: The IRAN-ECHO program is being conducted in six provinces, and will be considered in all provinces in the near future. Its main effects could be assessed in future years. Part of this program that was conducted as a quasi-experimental survey comprised 7149 students and showed that a high percentage of students had acceptable knowledge about adverse health effects of overweight and obesity. However, the knowledge about the low nutritional value of unhealthy snacks such as potato chips, puffs, industrial juices, and carbonated drinks was not appropriate. Many participants had the undesirable attitude of skipping one of the main meals when attempting to lose weight. CONCLUSIONS: The IRAN-ECHO program is presenting the feasibility of conducting the WHO-ECHO recommendations in Iran. The scope of potential policy recommendations to decrease childhood obesity is extensive and includes various elements. This program considers multisectoral interventions through population and individual approaches. The multicomponent interventions of this program address the obesogenic environment by considering the life course dimensions. It is expected that, by its life course interventions, it could help in primordial and primary prevention of noncommunicable diseases.

Simultaneous Presentation of Wilson's Disease and Autoimmune Hepatitis; A Case Report and Review of Literature.

INTRODUCTION: Coexistence of Wilson's disease and autoimmune hepatitis has been rarely reported in English literature. In this group of patients, there exist features of both diseases and laboratory and histopathological studies may be misleading. Medical treatment for any of these entities, per se, may result in poor response. Therefore, by considering the acute hepatitis resembling Wilson's disease and autoimmune hepatitis, simultaneous therapy with immunosuppressive and penicillamine may have a superior benefit. CASE PRESENTATION: We present the case of a 10-year-old boy with nausea, vomiting, yellowish discoloration of skin and sclera, abdominal pain and tea-color urine. Physical examination showed mild hepatomegaly and right upper quadrant tenderness. Laboratory and histochemical studies and atomic absorption test were done and the results were highly suggestive of both Wilson's disease and autoimmune hepatitis, in him. CONCLUSIONS: This case study highlights, although rare, the coexistence of Wilson's disease and autoimmune hepatitis and the need to maintain a high level of awareness of this problem. Therefore, it is reasonable to consider this type of hepatitis in rare patients, with dominant features of both diseases at the same time.

Hepatic encephalopathy: early diagnosis in pediatric patients with cirrhosis.

OBJECTIVE: As acute liver failure (ALF) and chronic liver disease (cirrhosis) continue to increase in prevalence, we will see more cases of hepatic encephalopathy. Primary care physician are often the first to suspect it, since they are familiar with the patient's usual physical and mental status. This serious complication typically occurs in patients with severe comorbidities and needs multidisciplinary evaluation and care. Hepatic encephalopathy should be considered in any patient with acute liver failure and cirrhosis who presents with neuropsychiatric manifestations, decrease level of consciousness (coma), change of personality, intellectual and behavioral deterioration, speech and motor dysfunction. Every cirrhotic patient may be at risk; potential precipitating factors should be addressed in regular clinic visits. The encephalopathy of liver disease may be prominent, or can be present in subtle forms, such as decline of school performance, emotional outbursts, or depression. "Subtle form" of hepatic encephalopathy may not be obvious on clinical examination, but can be detected by neurophysiologic and neuropsychiatric testing.

Helicobacter pylori infection and pediatric asthma.

OBJECTIVE: Childhood infectious diseases are one of the most known environmental pathogenic causes of childhood asthma. The high prevalence of both Helicobacter pylori infection and asthma in our country prompted us to assess anyprobable association between them in childhood. METHODS: This cross-sectional study recruited 196 children aged 6 to 12 years old comprising 98 asthmatic (case group) and 98 healthy (control group) individuals. Urea breath test was performed for all of the children and H. pylori infection was compared between the two groups according to the urea breath test results. RESULTS: Urea breath test was positive in 18 asthmatic (18.36) and 23 (23.36) healthy subjects but was not significantly different between the case and controls (p = 0.380). Further analysis in the asthmatic group revealed association of H. pylori infection withage (p < 0.001) and duration of asthma (p = 0.010). However, no significant correlation was found between sex, severity of asthma, controled asthma or abnormal pulmonary function tests with H. pylori infection (p= 0.804, 0.512, 0.854 and 0.292, respectively). CONCLUSION: Given the results of the study, H. pylori infection was not significantly different between asthmatic and healthy children. In asthmatic patients, there was no significant association between H. pylori infection and sex, severity of disease, control status of disease and normal or abnormal pulmonary function tests. H. Pylori infection had a significant association with increasing age and duration of asthma.

Clinical evaluation of disc battery ingestion in children.

BACKGROUND The purpose of this study was to evaluate the characteristics, management, and outcomes of disc battery ingestion in children. METHODS We reviewed the medical records of children admitted to Mofid Children's Hospital due to disc battery ingestion from January 2006 to January 2010. Clear history, clinical symptoms and results of imaging studies revealed diagnosis of disc battery ingestion in suspected patients. The clinical data reviewed included age, gender, clinical manifestation, radiologic findings, location of disc battery, duration of ingestion, endoscopic results and surgical treatment. RESULTS We found 22 cases (11 males and 11 females) of disc battery ingestion with a mean age of 4.3 years (range: 9 months to 12 years). Common symptoms were vomiting, cough, dysphagia, and dyspnea. The mean duration of ingestion was 2.7 days (4 hours to 1.5 months). A total of 19 patients had histories of disc battery ingestion, but three cases referred with the above symptoms, and the batteries were accidentally found by x-ray. Only three cases had batteries impacted in the esophagus. Twelve batteries were removed endoscopically, 6 batteries spontaneously passed through the gastrointestinal (GI) tract within 5 to 7 days, and 4 patients underwent surgery due to complications: 3 due to tracheo-esophageal fistula (TEF) and 1 due to intestinal perforation. There was no mortality in our study. CONCLUSION Most cases of disc battery ingestion run uneventful courses, but some may be complicated. If the battery lodges in the esophagus, emergency endoscopic management is necessary. However, once in the stomach, it will usually pass through the GI tract.

Celiac disease in children with diarrhea is more frequent than previously suspected.

BACKGROUND: Celiac disease (CD) may be missed or diagnosed late in children with chronic diarrhea. In this study the authors estimated the frequency of CD among pediatric patients with chronic diarrhea based on serologic and pathologic examinations. METHODS: During a 6-year period, all patients with chronic diarrhea of more than 6 weeks referred to the authors' department were included. For each patient, an asymptomatic control was enrolled from among the patients referred to our clinic for other reasons. Serologic tests for CD including immunoglobulin A endomysial antibody and immunoglobulin A antigliadin antibody were performed in all patients and controls. If positive, duodenal biopsy was performed to confirm the diagnosis. Patients subsequently diagnosed as CD were placed on a gluten-free diet and reevaluated after 6 months. RESULTS: 825 cases of diarrhea and 825 controls were enrolled. CD was diagnosed in 54 (6.5%) of the diarrhea patients and seven (0.8%) of the controls. After 6 months of gluten-free diet, 48 (88.8%) patients had significant improvement in symptoms and of these 41 (76.1%) were totally asymptomatic. Forty-two patients allowed repeat endoscopy after 6 months of gluten-free diet and 40 (95.2%) showed improvement in histologic findings. CONCLUSION: CD is common among patients labeled as chronic diarrhea. In this subgroup, gluten-free diet may lead to a significant improvement in symptoms. Routine testing for CD may be indicated in all patients being evaluated for chronic diarrhea.