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.

Terminal ileitis and cytotoxic lesion of corpus callosum as the presenting features of Multisystem inflammatory syndrome in children (MIS-C): a case report.

BACKGROUND: Multisystem inflammatory syndrome in children (MIS-C) is a post-viral inflammatory vasculopathy characterized by persistent fever, multiorgan dysfunction, significant laboratory markers of inflammation, lack of an alternative diagnosis, and prior SARS-CoV-2 infection or exposure in children and adolescents. The most common early symptoms include a prolonged fever, as well as dermatologic, mucocutaneous, and gastrointestinal symptoms such abdominal pain, vomiting, and diarrhea. CASE PRESENTATION: We present a pediatric patient with multisystem inflammatory syndrome with the development of abdominal pain and seizure who was found to have a circumferential wall thickening of the terminal ileum and ileocecal junction in abdominal CT scan. The brain MRI of the patient showed cytotoxic lesions of the corpus callosum (CLOCC) which had hypersignal intensity with a few diffusion restrictions in the splenium of the corpus callosum. CONCLUSION: This case is being reported to raise awareness of MIS-C presenting characteristics. Given the rising number of MIS-C patients and a lack of understanding regarding early diagnostic clinical characteristics and therapy, further research into clinical presentations, treatment, and outcomes is urgently needed.

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.

Changing face of Candida colonization pattern in pediatric patients with hematological malignancy during repeated hospitalizations, results of a prospective observational study (2016-2017) in shiraz, Iran.

BACKGROUND: Surveillance of current changes in the epidemiology of Invasive Fungal Diseases (IFDs) as an important component of the antifungal stewardship programs (ASP), requires careful regular monitoring, especially in high-risk settings such as oncology centers. This study aimed to examine Candida colonization status and corresponding current changes in children with malignancy during repeated admissions and also investigate the possible epidemiological shifts after the implementation of ASP. METHODS: In this prospective observational study, all eligible patients younger than 18 years were recruited during 2016-2017 at Amir Medical Oncology Center (AMOC) in Shiraz, Iran. Totally, 136 patients were enrolled and 482 samples were collected from different sites (oral/nasal discharges, urine and stool). Weekly regular sampling was carried out during hospitalization. Candida colonization status and epidemiological changes were monitored during repeated admissions. Samples were cultivated on Sabouraud Dextrose agar medium and identified by Polymerase Chain Reaction -Restriction Fragment Length Polymorphism (PCR-RFLP). RESULTS: Estimated Candida colonization incidence was 59.9% (82/136) in our patients. Candida colonization was found to be higher in oral cavity and rectum than that in nasal cavity. Among those long-term follow ups and repetitive hospitalizations, a significant number of patients exhibited changes in their colonization patterns (37.7%). Candida colonization did not reveal any significant relationship with age, sex, oncologic diseases and degree of neutropenia. C. albicans (72.0%) was found as the most common Candida species in colonized patients, followed by C. krusei, C. kefyr, C. glabrata and C. parapsilosis. CONCLUSION: Given the high incidence of Candida infections in children with cancers, close monitoring of epidemiologic changes is essential for judicious management, based on local surveillance data and improvement of overall quality of care in high risk patients.

Case Report: Gastrointestinal Basidiobolomycosis in a Young Girl with Cystic Fibrosis and Celiac Disease.

Basidiobolomycosis is an uncommon fungal infection that has been reported in the literature mainly as a cause of infection in the skin and subcutaneous tissue. Intraabdominal infections have been reported in tropical and subtropical areas in the Middle East, such as Iran and Saudi Arabia, and in the United States. Our patient was a 6-year-old girl with cystic fibrosis and celiac disease who was referred to our department with a history of chronic abdominal pain. In the imaging studies of the abdomen and pelvis, a large retroperitoneal mass was reported in the right upper part of the abdomen with involvement of the duodenum and the mesentery of the small and large intestines, as well as the superior mesenteric vessels, and was diagnosed as basidiobolomycosis through biopsy. Because of the large unresectable mass, the patient was first treated with antifungal drugs for 2 months and then surgical resection was performed. The main point in the management of these patients is a combination of antifungal therapy and surgical resection. In some patients, complex surgeries such as the Whipple procedure may be performed to appropriately manage intraabdominal infections.

Comparing clinical presentation, viremia, and immunological factors at various severity presentations in hospitalized children affected by COVID-19: A cross-sectional study.

Background and Aims: Although SARS-CoV-2 infection usually leads to mild COVID-19 in children, sometimes it causes serious complications, especially in those with underlying diseases. Several factors have been identified in determining disease severity in adults, and limited studies have been conducted in children. The prognostic implications of SARS-CoV-2 RNaemia as an important factor in determining disease severity in children are not well understood. Methods: In this study, we aimed to prospectively assess the relationship between disease severity and immunological factors and viremia in 47 COVID-19 hospitalized children. In this research, 76.5% of children experienced mild and moderate COVID-19, while 23.5% experienced severe and critical forms of the disease. Results: The presence of underlying diseases in different groups of pediatric patients differed significantly from each other. On the other hand, clinical symptoms such as vomiting and chest pain as well as laboratory parameters including erythrocyte sedimentation rate were significantly different in different groups of patients. Viremia was seen in only two children, and this had no significant relationship with the severity of COVID-19. Conclusion: In conclusion, our data confirmed that COVID-19 severity differed in SARS-CoV-2 infected children. Some clinical presentation and lab data parameters were different in various presentation of patients. Viremia was not associated with severity in our study.

Impact of antifungal stewardship interventions on the susceptibility of colonized Candida species in pediatric patients with malignancy.

There is a worldwide concern regarding the antimicrobial resistance and the inappropriate use of antifungal agents, which had led to an ever-increasing antifungal resistance. This study aimed to identify the antifungal susceptibility of colonized Candida species isolated from pediatric patients with cancer and evaluate the clinical impact of antifungal stewardship (AFS) interventions on the antifungal susceptibility of colonized Candida species. Candida species colonization was evaluated among hospitalized children with cancer in a tertiary teaching hospital, Shiraz 2017-2018. Samples were collected from the mouth, nose, urine, and stool of the patients admitted to our center and cultured on sabouraud dextrose agar. The isolated yeasts identified by polymerase chain reaction-restriction fragment length polymorphisms (PCR-RFLP). DNA Extracted and PCR amplification was performed using the ITS1 and ITS4 primer pairs and Msp I enzyme. The broth microdilution method was used to determine the minimum inhibitory concentrations (MICs) for amphotericin B, caspofungin, and azoles. The prevalence of Candida albicans in the present study was significantly higher than other Candida species. Candida albicans species were completely susceptible to the azoles. The susceptibility rate of C. albicans to amphotericin B and caspofungin was 93.1% and 97.1%, respectively. The fluconazole MIC values of Candida albicans decreased significantly during the post-AFS period (P < 0.001; mean difference: 72.3; 95% CI of the difference: 47.36-98.62). We found that 52.5% (53/117) of the isolated C. albicans were azole-resistant before AFS implementation, while only 1.5% (2/102) of the isolates were resistant after implementation of the AFS program (P < 0.001). C. albicans fluconazole and caspofungin resistant rate also decreased significantly (P < 0.001) after implementation of the AFS program [26 (32.9%) versus 0 (0.0%) and 11 (10.9%) versus 1 (0.9%), respectively]. Besides, fluconazole use (p < 0.05) and fluconazole expenditure reduced significantly (about one thousand US$ per year) after the AFS program. Our results confirm the positive effect of optimized antifungal usage and bedside intervention on the susceptibility of Candida species after the implementation of the AFS program. C. albicans and C. glabrata exhibited a significant increase in susceptibility after the execution of the AFS program.

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.

Serum insulinlike growth factor-1 and its binding protein-3 levels in children with cirrhosis waiting for a liver transplant.

OBJECTIVES: Investigate the prognostic value of serum insulinlike growth factor-1 (IGF-1) and its binding protein 3 (IGFBP-3) in pediatric patients with liver cirrhosis, and investigate the correlation between these parameters and other available prognostic factors including Child-Pugh scoring, Pediatric End-Stage Liver Disease, and Mayo End-Stage Liver Disease scoring. MATERIALS AND METHODS: This prospective, case-controlled study was done at the Nemazee hospital for 12 months from August 2009 to August 2010. It included 45 pediatric patients (< 18 years) diagnosed with liver cirrhosis and 38 healthy age and sex-matched controls. The extent and severity of the liver disease was evaluated by the Child-Pugh classification and Pediatric End-Stage Liver Disease/Mayo End-Stage Liver Disease scores. Serum levels of IGF-1 and IGFBP-3 were determined and were compared to controls and their correlation with Child-Pugh and Pediatric End-Stage Liver Disease/Mayo End-Stage Liver Disease scores were investigated. RESULTS: The most-common cause of liver cirrhosis was biliary atresia being found in 11 patients (24.4%) followed by tyrosinemia in 8 (17.8%). IGF-1 serum levels were significantly lower in cirrhotic patients compared with controls (3.85 ± 3.69 nmol/L vs 41.79 ± 16.03 nmol/L; P < .001). Serum levels of IGFBP-3 also were significantly lower in patients with liver cirrhosis compared with healthy controls (46.66 ± 30.57 nmol/L vs 205.63 ± 25.52 nmol/L; P < .001). Serum levels of IGF-1 were significantly lower in patients with stage B (P = .047) and C (P = .036) of Child-Pugh classification compared with stage A. Serum levels of IGF-1 (r ≈ 0.227; P = .034) and IGFBP-3 (r ≈ 0.389; P = .008) were negatively correlated with Pediatric End-stage Liver Disease / Mayo End-stage Liver Disease scores. CONCLUSIONS: The serum levels of IGF-1 and IGFBP-3 are decreased in children with liver cirrhosis. The stage of liver dysfunction is correlated to serum levels of IGF-1 and IGFBP-3 in children. Thus, these 2 factors can be used for assessing the prognosis and outcome in those children with liver cirrhosis.