Maximum Surgical Blood Ordering Schedule in Common Pediatric Cardiac Surgeries in a Tertiary Center.

Background & Objective: Unnecessary pre-operative ordering of red blood cells (RBCs) in elective surgeries increases costs and waste of blood inventory. Maximum surgical blood order schedule (MSBOS) is a helpful strategy in the estimation of blood units needed for surgery and the prevention of overconsumption. In this study, an MSBOS for pediatric cardiac surgeries is designed. Methods: In this cross-sectional study, we included all pediatric patients who underwent elective cardiac surgery in Children's Medical Center in Tehran, Iran, from March 21, 2019, to September 22, 2019. Data consisted of the type of surgery and the number of blood units transfused and units cross-matched, based on which cross-match to transfusion ratio (CTR), the transfusion index (TI), and transfusion probability (T%) were calculated. Results: Overall 205 pediatric patients were included in the study. Four hundred and ten RBCs units were cross-matched, and 262 were transfused. The overall results of the CTR, T%, and TI for all the eight types of cardiac surgery were 1.56 (410/262), 76% (157/205), and 1.28 (262/205), respectively. The raw MSBOS for cardiac surgeries included ventricular septal defect, tetralogy of fallot, dextro-transposition of the great arteries, atrial septal defect, aortic coarctation, patent ductus arteriosus, pulmonary stenosis, and pacemaker insertion, which were 1.58, 1.03, 1.54, 1.66, 0.77, 0, 1.25, and 0 unit, respectively, and the figures were rounded up. Conclusion: Accurate MSBOS protocols reduce cross-match workload in laboratories, lead to the appropriate use of blood stocks with less wastage, save human and economic resources, and eventually, promote patient safety.

Central nervous system Aspergillus quadrilineatus infection in a COVID-19 patient, a case report and literature review.

BACKGROUND: Viral pneumonia such as COVID-19-associated aspergillosis could increase susceptibility to fungal super-infections in critically ill patients. METHODS: Here we report a pediatric case of Aspergillus quadrilineatus cerebral infection in a recently diagnosed COVID-19-positive patient underlying acute lymphocytic leukemia. Morphological, molecular methods, and sequencing were used to identify this emerging species. RESULTS: Histopathological examination showed a granulomatous necrotic area containing dichotomously branching septate hyphae indicating a presumptive Aspergillus structure. The species-level identity of isolate growing on brain biopsy culture was confirmed by PCR sequencing of the ß-tubulin gene as A. quadrilineatus. Using the CLSI M38-A3 broth microdilution methodology, the in vitro antifungal susceptibility testing demonstrated 0.032 µg/mL MIC for posaconazole, caspofungin, and anidulafungin and 8 µg/mL against amphotericin B. A combination of intravenous liposomal amphotericin B and caspofungin therapy for 8 days did not improve the patient's condition. The patient gradually continued to deteriorate and expired. CONCLUSIONS: This is the first COVID-19-associated cerebral aspergillosis due to A. quadrilineatus in a pediatric patient with acute lymphocytic leukemia. However, comprehensive screening studies are highly recommended to evaluate its frequency and antifungal susceptibility profiles. Before being recommended as first-line therapy in high-risk patients, more antifungal susceptibility data are needed.

Antimicrobial susceptibility and genotyping of microorganisms isolated from sputum culture of children with cystic fibrosis in an Iranian referral children's hospital.

BACKGROUND: Chronic bacterial infections of the airways are present in most patients with cystic fibrosis (CF). Although most pathogens are acquired from the environment, there is great evidence of patient-to-patient transmission. Therefore, evaluating the genetic variation of strains isolated from CF patients is recommended for the purpose of examining hospital infection. AIM: The aim of this study was to determine the antibiotic susceptibility pattern and genotyping of Staphylococcus aureus and Pseudomonas aeruginosa strains isolated from sputum samples of children with CF referred to a single pediatric CF center in Tehran, Iran. METHODS: In this cross-sectional study, the antimicrobial susceptibility profiles of strains isolated from patients with CF during 1 year were determined. Pseudomonas aeruginosa and S. aureus isolates were genotyped using the random amplified polymorphic DNA polymerase chain reaction method and were analyzed using GelCompar II software. RESULTS: Of 534 patients with CF, 384 had negative sputum cultures (72%), and 94 strains of P. aeruginosa (18%) and 53 strains of S. aureus (10%) were isolated. The mean age of the patients was 8.22 ± 5.7 years (range, 2 months to 18 years). The P. aeruginosa strains showed high sensitivity to ceftazidime (96%), piperacillin/tazobactam (96%), and imipenem (94%). All strains of S. aureus were susceptible to vancomycin, and 13% of the strains were methicillin-resistant S. aureus. High resistance to penicillin (92%) and erythromycin (88.5%) were reported. The results of P. aeruginosa genotyping revealed that there were six major clusters in this hospital. Also, based on the analysis of genotyping results, S. aureus strains were obtained from five clusters, most of which were located in cluster B1 (34 isolates, 64%). CONCLUSION: The results of this study show the possibility of strains being transferred from one part of the hospital to another (especially from the respiratory ward to other areas). Hence, high attention should be paid to the basic methods of preventing infection.

Distribution of human papillomavirus genotypes in suspected women cytological specimens from Tehran, Iran.

Background and Objectives: The human papillomavirus (HPV) is associated with more than 70% of the cervical neoplasm. The current study aims to evaluate the distribution of HPV genotypes in suspected women cytological specimens from Tehran, Iran. Materials and Methods: In the current cross-sectional study, HPV genotype prevalence was investigated in 433 subject women. DNA extraction was performed by High Pure Viral Nucleic Acid kit. A semi-automatically hybriSpot 24™ (HS24) setting was used for HPV typing and data interpreted by hybriSoft™ software according to instructions. Results: Pathologic data showed 181 (41.8%) had non-malignant lesions, 212 (49%) had inflammation and 40 (9.2%) reported LSIL in primary Pap-smear result. HPV was found in 143 (33%) specimens and the most comment high-risk and low-risk HPV types were HPV-16 and -6, respectively. Also, 62 (43%) were co-infected with multiple genotypes includes, 34 (24%) cases had co-infection with two HPV types, 17 (12%) cases had co-infection with three HPV types, 6 (4%) cases had co-infection with four HPV types and 5 (3%) cases had co-infection with five HPV types. There was statistically different domination on high-risk genotype in most of the co-infected samples (p<0.01). Conclusion: Current study indicates that the lesion pathology assessment was significantly associated with the HPV infection (p<0.01). Furthermore, the age group assessment shows that most of the HPV positive cases were 21 to 40 (p<0.01). The HPV infection prevalence in the current study was 33% and the most frequently reported high-risk and low-risk HPV types were 16 and 6, respectively.

Case Report: Encephalitis Caused by Balamuthia mandrillaris in a 3-Year-Old Iranian Girl.

It is about half a century since free-living amoebae were recognized as pathogenic organisms, but there is still much we should learn about these rare fatal human infectious agents. A recently introduced causative agent of granulomatous amoebic encephalitis, Balamuthia mandrillaris, has been reported in a limited number of countries around the world. A 3-year-old girl was referred to our tertiary hospital because of inability to establish a proper diagnosis. She had been experiencing neurologic complaints including ataxia, altered level of consciousness, dizziness, seizure, and left-sided hemiparesis. The patient's history, physical examination results, and laboratory investigations had led to a wide differential diagnosis. Computed tomography (CT) scan and magnetic resonance imaging analyses revealed multiple mass lesions. As a result, the patient underwent an intraoperative frozen section biopsy of the brain lesion. The frozen section study showed numerous cells with amoeba-like appearances in the background of mixed inflammatory cells. Medications for free-living amoebic meningoencephalitis were administered. PCR assay demonstrated B. mandrillaris as the pathogenic amoeba. Unfortunately, the patient died 14 days after her admission. To our knowledge, this is the first report of B. mandrillaris meningoencephalitis in the Middle East and the first time we have captured the organism during a frozen-section study.

Comparing predictive values of carbohydrate antigen 19-9, neutrophil gelatinase-associated lipocalin, and kidney injury molecule-1 in 161 patients with ureteropelvic junction obstruction.

BACKGROUND: To evaluate and compare the efficacy of urinary carbohydrate antigen 19-9 (CA19-9), neutrophil gelatinase-associated lipocalin (NGAL), and kidney injury molecule-1 (KIM-1) biomarkers as predictive factors to determine the surgery requirement in patients with ureteropelvic junction obstruction. METHODS: We obtained urine samples from 161 patients at diagnosis and evaluated their levels of the three biomarkers. The patients were under observation for 2 years; subsequently, they were divided into two groups based on their requirement of pyeloplasty. We determined the correlation between the urinary concentration of the biomarkers and surgical interventions, as well as the kidney function deterioration and sonography outcomes. RESULTS: The non-surgery group included 60 male and 22 female patients with mean age of 21 months. The surgery group comprised 58 boys and 21 girls with mean age of 26.9 months with no significant difference of age and gender between the two groups. The outcomes were indicative of higher efficacy of CA19-9 level with a sensitivity and specificity of 84.2% and 73.2% at the cutoff point of 59.09 U/ml. Also, a significant negative correlation was detected between the kidney function and the concentrations of CA19-9 and NGAL. CONCLUSIONS: Our evaluations demonstrate the higher efficacy of CA19-9 to predict the requirement of surgical intervention in comparison with the other biomarkers, as well as a significant correlation between kidney function deterioration and urinary CA19-9 and NGAL. The outcomes of this investigation could pave the way for more extensive clinical application of these urinary biomarkers, besides future research determining the association between markers and kidney fibrosis.

Genotype-phenotype relationship in Iranian patients with cystic fibrosis.

BACKGROUND/AIMS: Cystic fibrosis (CF), the most common hereditary, life-threatening disease, is caused by a mutation in the CFTR gene. Because different mutations can affect clinical manifestations of patients, this study was conducted to investigate the possible genotype-phenotype relationship in a group of Iranian patients with CF. MATERIALS AND METHODS: This case-series study was conducted in 30 patients with CF who were referred to a tertiary pediatric hospital in Tehran. In this study, the DNA of the patients was evaluated for delta F508 mutation, whereas some parameters such as the age at diagnosis, the sweat chloride level, and clinical manifestations related to pancreatic insufficiency and pulmonary involvement were also assessed. RESULTS: Among all the studied patients, 16.6% had a delta F508 mutation, either homozygote or heterozygote. The mean age at diagnosis was lower in patients with the delta F508 mutation, but the sweat chloride level tended to be higher in these patients. All the patients with the delta F508 mutation had exocrine pancreatic insufficiency, which tended to be higher than 84% in those without this mutation. In addition, all of these patients had pulmonary involvement, which tended to be higher than 64% in those with negative delta F508 mutation. CONCLUSION: According to the results of this study, the frequency of delta F508 mutations in Iranian patients appears to be much lower than what is seen in American and the European patients. In those with the delta F508 mutation, pulmonary involvement and pancreatic insufficiency are more common; the sweat chloride level tended to be higher, but the age at diagnosis was lower, all of which resemble a more severe form of disease.

Low incidence of alpha-1-antitrypsin deficiency in Iranian patients with neonatal cholestasis.

BACKGROUND/AIMS: There is little data concerning the incidence of alpha-1-antitrypsin"(AAT) deficiency, the most common genetic cause of liver disease, among children with neonatal cholestasis in Iran. Thus, this study was performed to analyze AAT deficiency in this group of patients. MATERIALS AND METHODS: DNA samples from patients with neonatal cholestasis were investigated for Pi S and Pi Z alleles, using polymerase chain reaction-restriction fragment length polymorphism. RESULTS: Thirty patients with neonatal cholestasis were enrolled. Among those who underwent biopsies, the results revealed neonatal hepatitis in 19, bile duct paucity in 1, steatohepatitis in 1, bile duct proliferation in 1, cirrhosis in 2, fibrosis in 2, and extrahepatic biliary atresia in 1 patient. No mutant allele was found in any patient. CONCLUSION: The incidence of AAT deficiency is very low in Iran; therefore, screening for AAT is not recommended for patients with neonatal cholestasis in Iran.

Screening for thalassemia carriers in populations with a high rate of iron deficiency: revisiting the applicability of the Mentzer Index and the effect of iron deficiency on Hb A2 levels.

Differentiating between ß-thalassemia (ß-thal) minor and iron deficiency has important implications in thalassemia carrier screening. Several complete blood count (CBC)-based equations have been proposed for differentiating these two conditions. The applicability of these equations in populations with high rates of iron deficiency and ß-thal minor, where patients can have both conditions, is limited. In addition, there have been conflicting reports on the possible effect of iron deficiency on Hb A2 level with possible consequences for thalassemia screening programs. Here, we demonstrate that in our population the Mentzer Index separates individuals with ß-thal minor from those without ß-thal minor, regardless of their iron status. Iron deficiency also does not reduce Hb A2 levels in ß-thal minor patients. Correction of iron deficiency is not required for diagnosis of ß-thal minor using high performance liquid chromatography (HPLC).

Sensitivity and specificity of adenosine deaminase in diagnosis of juvenile idiopathic arthritis.

BACKGROUND: Juvenile Idiopathic Arthritis (JIA) is one of the most common chronic rheumatic diseases inchildren with unknown etiology and pathogenesis. It also has no diagnostic test and its clinical diagnosis ismade through ruling out other types of arthritis. The aim of this study was to evaluate the level of ADA (AdenosineDeaminase) in the serum of JIA patients and to compare it with that of patients with Reactive Arthritis(RA). Evaluation of sensitivity and specificity of serum ADA level in JIA was another objective. METHODS: The study included 120 children with JIA (mean age= 7.6 ± 4.3 years) and 40 children with RA(mean age= 5.5 ± 3.1 years). The ADA was measured in the active phase of both diseases. RESULTS: The mean ADA serum level was obtained as 15.8 ± 11.8 U/l in JIA patients and 14.3 ± 7.5 U/l in RApatients. The difference was statistically insignificant (p= 0.4). Another finding of this study was the significantspecificity (77.5%) of this laboratory parameter for JIA in comparison with its low sensitivity (36.7%). Positivepredictive value was 83% and negative predictive value 29%. CONCLUSION: Determination of ADA serum levels is a noninvasive reliable and easy biomarker for diagnosis ofJIA and it can be used as alternative parameters representing disease activity.

Is There any Association Between Passive Smoking and Esophagitis in Pediatrics?

OBJECTIVE: Exposure to environmental tobacco smoke (ETS) is one of the major factors of predisposing children to develop several hazardous health problems. We decided to investigate the association between nicotinine, one of the nicotine metabolites and esophagitis in children with gastroesophageal reflux disease (GERD). METHODS: In a case control study 46 children suffering from esophagitis referred to endoscopy ward were recruited. The control group consisted of 45 healthy children. Urine samples were collected and urinary cotinine level (UCL) measured. FINDINGS: The mean age of esophagitis and control groups were 5.11±2.93 and 6.72±2.8 respectively. Sixty children were passive smokers; 31 of them had non-smoker parents. In control group, 32 (71.1%) children and in esophagitis group 29 (63%) children had non-smoker parents. The mean value of UCL in patients suffering from esophagitis was significantly higher than those in normal group (P=0.04, 24.98±6.4 ng/ml vs. 15.16 ± 3.9 ng/ml). Considering 50ng/ml as a cutoff point for UCL, it was significantly higher in passive smoker group than in non smoker group (P=0.02). The mean cotinine level differed significantly in esophagitis and control group. CONCLUSION: Our results indicate the increased risk of developing esophagitis in children with ETS exposure.

The discovery of a Persian family with a form of Birt-Hogg-Dubé syndrome lacking the typical cutaneous stigmata of the syndrome.

PURPOSE: This study was performed in 24 members of a family with spontaneous pneumothorax to test clinical suspicion of Birt-Hogg-Dubé syndrome (BHDS). METHODS: Computed tomography scan was performed for confirmation of pneumothorax, while genetic tests were done using real-time quantitative polymerase chain reaction. RESULTS: Genetic studies showed a deletion of exon 1 in the FLCN gene in the index case as well as nine other individuals, including two with clinical phenotypes of pneumothorax and seven who are symptom-free to date. CONCLUSIONS: Proper imaging and taking accurate family history could be the keys to test clinical suspicion in some syndromes, including BHDS.

Comparison of classic sweat test and crystallization test in diagnosis of cystic fibrosis.

OBJECTIVE: Sweat chloride measurement is considered a standard diagnostic tool for cystic fibrosis (CF). This study was performed to compare sweat chloride values obtained by quantitative pilocarpine iontophoresis (classic test) with sweat crystallization detected by direct observation of a drop of perspiration under light microscopy in patients with and without CF. METHODS: The tests using both techniques were performed simultaneously in patients with and without CF. Cutoff values of ≥60 mmol/L of chloride concentration for the classic sweat test was considered for diagnosis of CF. In crystallization method, observation of typical dendritic forms of salt crystals under light microscopy was interpreted positive. FINDINGS: Sixty patients suspected to CF (31 males and 29 females) with age range of 9 months to 2 years underwent the sweat test using both techniques. Median sweat chloride values was 26.13+10.85 in group with negative and 72.76+12.78 mmol/L in group with positive sweat test, respectively. All the patients who had positive sweat test in classic method showed typical dendritic forms of salt crystal in sweat crystallization test, which provided the test with 100% sensitivity (95%CI: 93.1-100). Only one of the 31 subjects with negative results for classic sweat test had positive result for crystallization sweat test, which provided the test with 96.7% specificity (95%CI: 92.9-100). Time spent to perform the crystallization test was significantly shorter than the classic method whereas its cost was also lower than the second method. CONCLUSION: There was a good correspondence between two studied methods of sweat test. These results suggested the sweat crystallization test as an alternative test for detecting CF disease with high sensitivity and specificity.

Analysis of colonic mucosa mast cell count in patients with chronic diarrhea.

BACKGROUND/AIM: Chronic diarrhea is defined as a decrease in fecal consistency lasting for four or more weeks. Prevalence of this complication in the general population is 5%. Mast cells that play an important role in the regulation of gastrointestinal visceral sensitivity and vascular permeability may be involved in functional chronic diarrhea. In this study we tried to evaluate mast cells density in colonic mucosa of patients with chronic diarrhea. PATIENTS AND METHODS: 50 patients with chronic diarrhea and 50 persons as control group were investigated. All specimens were immunohistochemically stained for mast cell tryptase (MCT) with monoclonal mouse anti-human MCT as well as toluidine blue. Mean number of mucosal mast cells were counted in 10 high power microscopic fields of patients and control groups. RESULTS: In patients group (age range, 15-78 years; 26 females), the number of mast cells per high power field in the immunohistochemistry staining was 21.3 ± 4.8 compared to 14.2 ± 3.4 in the control group (age range, 18-78 years; 24 females) [P < 0.001]. Also number of mast cells in toluidine blue staining was 10.3 ± 3.6 per high power field in cases and 7.1 ± 2.4 in the control group (P < 0.001). CONCLUSION: Elevated number of colonic mast cells exist in patients with chronic diarrhea. Further research should be considered on application of these findings for new therapeutic opportunities.

Colorectal polyps: a clinical, endoscopic and pathologic study in Iranian children.

OBJECTIVE: To study the clinical presentation, histology and colonoscopic features of lower gastrointestinal polyps in Iranian children. MATERIAL AND METHODS: Medical reports of children with colorectal polyps were retrospectively reviewed from 1996 to 2005 at the Children's Medical Center Hospital, Iran. A total of 563 cases were studied. Data related to age, sex, family history, signs and symptoms, the size, location, polyp types and associated lesions were collected and analyzed. RESULTS: The mean age of children was 5.66 +/- 2.88 years (range 2 months to 17 years), with a male-to-female ratio of 1.61:1.0. The highest incidence was between ages 2 and 10 years (85.1%). Rectal bleeding was the presenting symptom in 78.5% cases. The polyps were solitary in 94% of cases. A majority of polyps (86.3%) were juvenile and 86.7% located in the rectosigmoid area. Three percent of cases had a positive family history. One case of Turcot syndrome was also identified. CONCLUSION: Juvenile polyps remain the most common polyps in Iranian children. Although the presence of a solitary polyp in the rectosigmoid colon is more prevalent, in a significant number of cases they are multiple and located in proximal parts. Polyps must be removed even when asymptomatic because of their probable neoplastic potential.