Nonfamilial cherubism in a 6-month-old infant: a case report.

BACKGROUND: Cherubism is known as a very rare autosomal dominant familial disorder of childhood caused by a mutation in the SH3BP2 gene on 4p16.3. It has not yet been observed at birth and is usually diagnosed in children aged 2-7. Here, we present a non-hereditary case of cherubism at a very early age. CASE PRESENTATION: A 6-month-old girl presented with bilateral progressive jaw enlargement. On physical examination, bilateral asymmetrical jaw enlargement, predominantly on the left side, and some enlarged, non-tender, mobile submandibular lymph nodes were detected. No other abnormality was observed. Further investigations with radiology suggested cherubism and Burkitt's lymphoma as differential diagnoses. Later on, histopathologic evaluations were suggestive of cherubism. No surgical interventions were indicated, and the child is on regular follow-ups. CONCLUSION: Non-hereditary Cherubism, despite scarcity, can present in children below two years of age, even as early as the beginning of primary dentition. Accurate and swift diagnosis is essential to avert physical and psychological complications. Our case report shows the importance of keeping cherubism in mind as a differential diagnosis of bone disease, even in children under a year old, and the value of interdisciplinary collaboration in dealing with rare genetic disorders.

Unintended impact of COVID-19 pandemic on the rate of catheter related nosocomial infections and incidence of multiple drug resistance pathogens in three intensive care units not allocated to COVID-19 patients in a large teaching hospital.

BACKGROUND: The prevalence of resistant hospital infections in the intensive care unit (ICU) increases mortality and antibiotic resistance. COVID-19 pandemic may have unintended impact on nosocomial infections (NI) and the prevalence of resistant microorganism. METHODOLOGY: The present non-interventional study was performed by a pre and a post survey each lasting 8 months before (March-October 2019) and after (March-October 2020) the onset of COVID-19 pandemic in three ICU's, not allocated to COVID-19 patients, in Nemazee Hospital, Shiraz, Iran. The rates of the following nosocomial infections were compared at pre- and post-pandemic period: ventilator associated pneumonia (VAP), central line associated blood stream infection (CLABSI), catheter-associated urinary tract infections (CAUTI) and incidence of multiple drug resistance (MDR) pathogens. RESULTS: Pre-pandemic and pandemic incidence of VAP was 23.5 and 17.2 cases per 1000 device-days, respectively; an absolute decrease of 27%. The main reason for the decrease in the rate of VAP during the pandemic was a significant decrease in the rate of VAP caused by Acinetobacter baumannii; from 39 to 17% in total VAP episodes. The rate of VAP associated with other microorganisms remained relatively unchanged from 14.2 cases in pre-pandemic period to 14.3 cases per 1000 MV-days during the pandemic (P = 0.801). Pre-pandemic incidence of CLABSI was 7.3 cases and, in pandemic period, was 6.5 cases per 1000 device-days (IRR = 0.88, 95% CI 0.43-1.73, P = 0.703). Pre-pandemic incidence of CAUTI was 2 and in pandemic period, was 1.4 cases per 1000 device-days (IRR = 0.70, 95% CI 0.22-1.98, P = 0.469). CONCLUSION: The results of the present study showed a decrease in the incidence of VAP in critically ill non-COVID-19 patients during the pandemic compared to before the pandemic, especially regarding Acinetobacter baumannii.

Thromboprophylaxis Outcome in Childhood SARS-CoV-2 Infection: A Single-Center Experience.

BACKGROUND: The SARS-CoV-2 infection has been associated with potentially endothelial damage and coagulation cascade activation that cause thrombosis. There is limited information on thrombosis and anticoagulant therapy in children with coronavirus disease 2019 (COVID-19). AIMS: This study evaluates the outcome of thromboprophylaxis in children younger than 18-year old with COVID-19 infection. METHODS: A retrospective study was conducted on 184 hospitalized pediatric patients with confirmed COVID-19 infection. A designed questionnaire was made to collect all demographic, clinical, and laboratory data. According to World Health Organization, the patients were classified as asymptomatic/mild, moderate, severe, and critically ill. RESULTS: The mean age of the patients was 7.04±5.9 (1 wk to younger than 18 y). Overall, 33 patients received anticoagulant therapy. All patients who passed away (n=19) belonged to the critical group. One patient (1.28%) was complicated with deep vein thrombosis despite taking thromboprophylaxis, and 1 (1.28%) with pulmonary thromboembolism while the patient did not take an anticoagulant. CONCLUSIONS: Our data showed a lower rate of thrombosis (1.4%) than adult patients with COVID-19. It may underline the role of anticoagulants in moderate to severe/critically ill children with COVID-19 infection. Expert opinion and personal experience are necessary, while we have a significant knowledge gap in understanding COVID-19-associated coagulopathy and thrombotic risk in children.

Cohort Profile: Shiraz Pediatric Liver Cirrhosis Cohort (SPLCCS).

Liver diseases in children and adolescents are a significant and arising public health issue and should be surveyed from different dimensions (clinical and para-clinical, psychological, socio-economic) and in diverse populations. Shiraz Liver Transplant Center, Shiraz, Iran is the only center for pediatric liver transplantation and its pre-operative evaluations. This provides a unique and valuable situation for studying this vulnerable population. The Shiraz Pediatric Liver Cirrhosis Cohort Study (SPLCCS) was established to assess cirrhotic children, the course of their disease, and treatment over time. This cohort study aimed to prospectively evaluate the natural course and factors that contributed to complications and death of children with chronic liver disease in the region. SPLCCS was launched in September 2018 after obtaining ethical approval; until August 2022, 370 children with end-stage liver disease were enrolled and followed every six months. Here, the cohort's features, the included population's baseline characteristics, and primary outcomes are reported.

Persistent cough and asthma-like symptoms post COVID-19 hospitalization in children.

BACKGROUNDS: Respiratory viruses are the main triggers of asthma. Coronavirus is shown to contribute to respiratory tract infections that can lead to prolonged cough and asthma. OBJECTIVES: Present study aimed to determine the risk of developing Persistent cough and asthma-like symptoms in hospitalized children due to COVID-19. METHODS: This prospective study was carried out in a tertiary referral center. During the COVID-19 pandemic, 69 hospitalized pediatric patients admitted with COVID-19 were observed from February 2020 to January 2021. Clinical and laboratory data were recorded, and after discharge, patients were followed and visited for cough and asthma evaluation one, 2 and 6 months later. Patients with asthma-like diagnoses in follow up defined as asthma-like groups, and patients without any sign of asthma were categorized as the non-asthma group. Asthma-like co-morbids and risk factors were evaluated and compared between the two groups. RESULTS: In follow-up, most of the COVID-19 hospitalized patients (N = 42) (58.5%) were not affected by asthma-like symptoms. 60.9% of the COVID-19 patients were male. The asthma-like group cases had a significantly familial history of asthma (63.0%), past medical history of asthma (33.3%), and Allergic rhinitis (85.2%). Rates of signs and symptoms during hospitalization were significantly higher in patients with COVID-19 and past medical history of asthma. CONCLUSIONS: We found an asthma-like prevalence of 41.5% in the cohort of COVID-19 hospitalized children. Family history of asthma and previous history of asthma and allergic rhinitis are risk factors for asthma-like after COVID-19 hospitalization. COVID-19 presentations are more severe in the asthma-like group.

Multisystem Inflammatory Syndrome with Features of Atypical Kawasaki Disease during the COVID-19 Pandemic.

BACKGROUND: Although Kawasaki disease (KD) is the most common self-limited systemic vasculitis in pediatrics, the exact etiology of the disease, its association with other diseases, and pathogens is still unknown. In order to achieve a better understanding and management of the disease, documentation and reporting of atypical cases is justified, particularly with the growing number of children with inflammatory syndrome with clinical features simulating KD during the COVID-19 pandemic. Here, we present a case of an atypical case of KD presenting as multisystem inflammatory syndrome (MIS) during the COVID-19 pandemic. Case Presentation. The patient is a 7-year-old girl who developed fever (39°C) and erythematous multiform rash on the abdomen and along with erythema and edema on the extremities. Laboratory evaluation revealed neutrophilia and lymphopenia along with elevated C-reactive protein, erythrocyte sedimentation rate, troponin, lactate dehydrogenase, ferritin, and D-dimer. Although the patient did not fulfill the KD criteria, based on approved guidelines and approaches regarding atypical KD and multisystem inflammatory syndrome in children (MIS-C) during the COVID-19 pandemic, intravenous immunoglobulin along with aspirin was administered for the patients. The patient's symptoms resolved with an uneventful postdischarge course. CONCLUSION: Early diagnosis and treatment of patients meeting full or partial criteria for KD are critical to preventing end-organ damage and other long-term complications, especially during times of public crisis and global health emergencies, such as the novel coronavirus pandemic.

Assessment of the HScore as a predictor of disease outcome in patients with COVID-19.

Severe coronavirus disease 2019 (COVID-19) accompanies hypercytokinemia, similar to secondary hemophagocytic lymphohistiocytosis (sHLH). We aimed to find if HScore could predict disease severity in COVID-19. HScore was calculated in hospitalized children and adult patients with a proven diagnosis of COVID-19. The need for intensive care unit (ICU), hospital length of stay (LOS), and in-hospital mortality were recorded. The median HScore was 43.0 (IQR 0.0-63.0), which was higher in those who needed ICU care (59.7, 95% CI 46.4-72.7) compared to those admitted to non-ICU medical wards (38.8, 95% CI 32.2-45.4; P = 0.003). It was also significantly higher in patients who died of COVID-19 (105.1, 95% CI 53.7-156.5) than individuals who survived (41.5, 95% CI 35.8-47.1; P = 0.005). Multivariable logistic regression analysis revealed that higher HScore was associated with a higher risk of ICU admission (adjusted OR = 4.93, 95% CI 1.5-16.17, P = 0.008). The risk of death increased by 20% for every ten units increase in HScore (adjusted OR 1.02, 95% CI 1.00-1.04, P = 0.009). Time to discharge was statistically longer in high HScore levels than low levels (HR = 0.41, 95% CI 0.24-0.69). HScore is much lower in patients with severe COVID-19 than sHLH. Higher HScore is associated with more ICU admission, more extended hospitalization, and a higher mortality rate. A modified HScore with a new cut-off seems more practical in predicting disease severity in patients with severe COVID-19.

Orbital mucormycosis in immunocompetent children; review of risk factors, diagnosis, and treatment approach.

BACKGROUND: Orbital mucormycosis is a rare but potentially severe and troublesome invasive fungal infection that could be occurred even in healthy individuals. The initial clinical presentation is similar to bacterial pre-septal or septal cellulitis, especially in early stages. CASE PRESENTATION: Herein, we describe the successful management of a series of five cases presenting with orbital mucormycosis in previously healthy children. CONCLUSIONS: Orbital mucormycosis is extremely rare in healthy children and maybe life-threatening when diagnosis delayed given a similar clinical presentation with bacterial septal cellulitis. Intravenous antifungal therapy with amphotericin B and timely surgical drainage is live-saving.

A Case of COVID 19 and Staphylococcus Coinfection.

Since December 2019, we have seen a significant number of cases of a novel coronavirus (2019-nCov), first identified in Wuhan China. Coronavirus might coexist with other infections such as Staphylococcus.

Successful Treatment of Disseminated Cutaneous Leishmaniasis With Liposomal Amphotericin B and Miltefosine in an Eight-year-old Girl.

This case report presents an 8-year-old girl, from Fars province in Iran, diagnosed with cutaneous leishmaniasis in the form of multiple nodular, ulcerative and crusted lesions disseminated on the face, trunk and extremities. The result of direct smear of ulcers was positive for Leishmania parasite. The patient had no immunodeficiency or relevant family history making her susceptible for disseminated cutaneous leishmaniasis. The skin lesions failed to respond to multiple treatment courses of meglumine antimoniate or amphotericin B but were successfully treated with simultaneous miltefosine and liposomal amphotericin B.

Gastro-intestinal basidiobolomycosis in a 2-year-old boy: dramatic response to potassium iodide.

Gastro-intestinal basidiobolomycosis (GIB) is a rare fungal infection caused by Basidiobolus ranarum. Treatment includes surgical resection and long-term antifungal therapy. A 2.5-year-old boy presented with a 10-day history of abdominal pain, fever and diarrhoea, and a palpable abdominal mass was detected. Resection was undertaken and histology confirmed basidiobolomycosis. Treatment with amphotericin B and itraconazole was commenced, but the infection progressed and spread to involve the intestines, liver, ribs and lung, and also the abdominal wall after 6 months, requiring four operative procedures. Because of unresponsiveness to amphotericin and itraconazole, oral potassium iodide was added which resulted in complete resolution of the infection. Potassium iodide is an essential component of the treatment of systemic B. ranarum.

Analysis of hospital records of children with hydatid cyst in south of Iran.

The clinical manifestations of hydatidosis are various and related to anatomic location. Defining frequent symptoms and signs of the disease is imperative for early management of it. The aim of this report was to analyse the clinical features of infected children with hydatid cysts located in different organs. In this study, medical charts of 57 children between 3 and 16 years of age with hydatid cyst admitted to Pediatric Wards of Nemazee Hospital were evaluated over a 12 year period (from 2003 to 2014, prospectively). All the epidemiologic, clinical, paraclinical and therapeutic data were collected. The frequencies of hydatidosis in males and females were 42.1 and 56.1%, respectively. Hydatid cysts were found in the liver and lungs in 59.6 and 33.3% patients respectively and 2 patients had an asymptomatic cyst in the heart with concomitant liver and lung cysts. The right upper quadrant pain (100%) was the most common symptom in the liver cysts. Phlegm (78.9%), Dyspnea (57.9%), acute (47.4%) and chronic cough (47.4%) were mostly seen in lung hydatid cysts. Some symptoms such as fever (68.4%) and weakness (59.6%) were the most common presenting symptoms in both groups. All children were treated through surgical approaches plus medical treatment. In the present report, liver was the most common site of involvement in children. Liver hydatidosis should be considered in children with upper quadrant pain and pulmonary hydatidosis in children complaining of phlegm and dyspnoea.

Diagnostic value of lactate, procalcitonin, ferritin, serum-C-reactive protein, and other biomarkers in bacterial and viral meningitis: A cross-sectional study.

There are many difficulties distinguishing bacterial from viral meningitis that could be reasonably solved using biomarkers. The aim of this study was to evaluate lactate, procalcitonin (PCT), ferritin, serum-CRP (C-reactive protein), and other known biomarkers in differentiating bacterial meningitis from viral meningitis in children.All children aged 28 days to 14 years with suspected meningitis who were admitted to Mofid Children's Hospital, Tehran, between October 2012 and November 2013, were enrolled in this prospective cross-sectional study. Children were divided into 2 groups of bacterial and viral meningitis, based on the results of cerebrospinal fluid (CSF) culture, polymerase chain reaction, and cytochemical profile. Diagnostic values of CSF parameters (ferritin, PCT, absolute neutrophil count [ANC], white blood cell count, and lactate) and serum parameters (PCT, ferritin, CRP, and erythrocyte sedimentation rate [ESR]) were evaluated.Among 50 patients with meningitis, 12 were diagnosed with bacterial meningitis. Concentrations of all markers were significantly different between bacterial and viral meningitis, except for serum (P = .389) and CSF (P = .136) PCT. The best rates of area under the receiver operating characteristic (ROC) curve (AUC) were achieved by lactate (AUC = 0.923) and serum-CRP (AUC = 0.889). The best negative predictive values (NPV) for bacterial meningitis were attained by ANC (100%) and lactate (97.1%).The results of our study suggest that ferritin and PCT are not strong predictive biomarkers. A combination of low CSF lactate, ANC, ESR, and serum-CRP could reasonably rule out the bacterial meningitis.

A 25-year surveillance of disseminated Bacillus Calmette-Guérin disease treatment in children in Southern Iran.

Disseminated Bacillus Calmette-Guérin (BCG) disease is one of the most serious complications of BCG vaccination, mainly among immunocompromised children with high morbidity and mortality.Currently, there is no any consensus with regard to the standard regimen of antituberculosis (anti-TB) agents and duration of treatment in healthy or immunocompromised host in children. The aim of this study is to investigate the effect of various combination treatment strategies for disseminated BCG disease in children.In this cross-sectional study, the outcome of 3 different combination protocols was investigated in 59 patients.All patients were younger than 6 years old. Both possible immunocompetent and proven immunodeficient children were included in a period of 25 years (1991-2014) in a Nemazee referral teaching hospital.The minimum age was 1 month and the maximum was 60 months. The average age of patients was 8 months (8.26 ±â€Š9.73). Out of 59 cases, 32 (54.2%) were female and 27 (45.8%) were male. Based on the primary work up, 52.5% of cases were classified as definite immunodeficient and 47.5% were classified as possible immunocompetent. Overall mortality rate was 50.8%. Mortality rate of disseminated BCG disease in immunocompetent and immunodeficient children was 28.6% and 71%, respectively. The mortality rate was not statistically different between patients treated with different treatment protocols. These results were not affected by immune status and the type of immunodeficiency.More than 2 anti-TB drugs combination will not change outcome of patient with disseminated BCG disease.

Impact of WHO Hand Hygiene Improvement Program Implementation: A Quasi-Experimental Trial.

Objectives. As affirmed by the World Health Organization (WHO), hand hygiene is the most powerful preventive measure against healthcare-associated infections (HCAIs) and, thus, it has become one of the five key elements of patient safety program. The aim is to assess the effect of implementation of the WHO's Multimodal Hand Hygiene Improvement Strategy among healthcare workers of a tertiary teaching hospital in a developing country. Methods. Hand hygiene compliance was assessed among healthcare workers, according to five defined moments for hand hygiene of the WHO, before and after implementation of the WHO's Multimodal Hand Hygiene Improvement Strategy in fourteen wards of a tertiary teaching hospital in Shiraz, Iran. We used direct observation method and documented the results in WHO hand hygiene observation forms. Results. There was a significant change in compliance before and after implementation of WHO's Multimodal HH Improvement Strategy (29.8% and 70.98%, resp.). Conclusions. Implementing WHO hand hygiene program can significantly improve hand hygiene compliance among nurses.

Respiratory Burst Process in Diabetic Children.

BACKGROUND: Increased rate of infections in diabetes mellitus (DM) is an accepted fact. Pathophysiologically, several tasks of the immune system could be involved including polymorphonuclear (PMN) functions. OBJECTIVES: The aim of this research was to evaluate the respiratory burst process of PMNs that is an essential part of phagocytosis, in children with DM. PATIENTS AND METHODS: Fifty two children with insulin dependent diabetes and 29 non-diabetic children were enrolled in this cross sectional study from 2010 to 2011. Nitroblue tetrazolium (NBT) test was done on PMNs taken from their heparinized blood. The resultant data was analyzed by SPSS version 16. P values were considered significant when it was under 0.05. RESULTS: Mean NBTs were 72.1 ± 15.84 and 94.68 ± 5.31 in diabetics and non-diabetics, respectively (P < 0.001). Using Pearson correlation, there was no significant correlation between the NBT level and age, gender, duration of diabetes, daily insulin usage and blood HbA1C level. CONCLUSIONS: Compared to non-diabetics, respiratory burst process of polymorphonuclears is obviously decreased in diabetic children. This can explain one of the mechanisms involved in the increased rate of infections in DM.

Surveillance of Antibiotic Consumption Point Prevalence Survey 2014: Antimicrobial Prescribing in Pediatrics Wards of 16 Iranian Hospitals.

BACKGROUND: Antibiotics are among the most commonly prescribed drugs in pediatrics. Due to lack of uniformity in pediatric antimicrobial prescribing and the emergence of antibiotic resistance, appropriate drug utilization studies have been found to be crucial to evaluate whether these drugs are properly used. METHODS: Data were collected between January 2014 and February 2014 in 16 Iranian pediatric hospitals using a standardized method. The point prevalence survey included all inpatient beds. RESULTS: Of 858 children, 571 (66.6%) received one or more antimicrobials. The indications were therapeutic in 60.6%. The parenteral route was used in 92.5% of therapeutic indications. Ceftriaxone was the most prescribed antimicrobials for therapeutic indications (32.4%) and combination-therapy was the most type of therapy in pediatric intelligent care unit (PICU). CONCLUSION: According to results of this study, antibiotics' prescribing in pediatrics wards of Iranian hospitals is empirical. Therefore, for quality improvement of antimicrobial use in children continuous audit process and antibiotic prescriptions require further investigation.

Molecular Epidemiology of Enteric Adenovirus Gastroenteritis in under-Five-Year-Old Children in Iran.

Background. Acute gastroenteritis is one of the major sources of morbidity and mortality among young children in developed and developing countries. The aim of this study was to determine the prevalence of human adenovirus- (HAdV-) 40 and HAdV-41 in children hospitalized with gastroenteritis in five different health centers of Iran. Methods. In a cross-sectional epidemiological study, we studied 2682 fecal specimens that were collected from children under the age of 5 years in five educational and therapeutic pediatric centers in Iran from February 2012 to February 2013. Samples were tested for HAdV-40 and HAdV-41, using a specific pair of primers in polymerase chain reaction (PCR) method. Results. HAdV-40 and HAdV-41 were detected in 132 (5.18%) of the patients with diarrhea. A significantly higher prevalence of HAdV-40 and HAdV-41 (58.3%) was observed in children under 12 months of age, compared to other age groups. The male to female ratio was 1.7. Conclusion. The results of this study demonstrated that HAdV-40 and HAdV-41 could be considered etiological agents for acute gastroenteritis among children in Iran. The PCR as a rapid test may increase the chance for a relatively mild course of the disease followed by a complete recovery and avoiding administration of unnecessary antibiotics.

A case of hepatic fasciolosis presented with prolonged Fever in southern iran.

BACKGROUND: Human fasciolosis is deemed as an emerging/re-emerging infection, hence making it an important human parasitic disease. In contrast to northern parts of Iran, human cases of fasciolosis in southern Iran are rare and sporadic. We report a sporadic case of fasciolosis in southern Iran (Fars Province) who presented with prolonged fever. Our report could suggest that there might be new foci emerging in the region, which indicates the need for further investigations.