Fever among Children with Sickle-Cell Disease: Findings from the General Pediatric Ward of the Owendo Pediatric Hospital in Libreville, Gabon

Sickle-cell disease (SCD) represents a substantial public health problem in Gabon. Fever is one of the principal reasons for the hospitalization of children afflicted by major sickle-cell disorder, since it can be a clinical reflection of severe infections that have the potential to become life threatening. Objectives: Identification of the main causes of fever in children with SCD in our clinical setting, with the aim of optimizing treatments. Patients and Methods: This is a retrospective study of all the medical files for children with SCD that were admitted to our ward, over a two year period, due to fever (>38.5°C) lasting more than 24 hours. Only those files that contained at least the following five fundamental medical examinations were retained for further evaluation: Complete Blood Count (CBC), blood smear, blood culture, urine culture and chest X-ray. Out of a total of 118 admissions (103 patients), 87 (73.7%) were due to the incidence of fever. The medical files of 11 patients were deemed to be unusable. Seventy-six episodes of fever were observed among 69 children, of which 42 were male and 27 female (sex ratio of 1.5). Among these, seven (10%) were admitted twice. Results: The age groups that were most affected included 12 - 18 year-olds (30 cases: 43.5%) and 6 - 12 year-olds (26 cases: 37.7%). The most common accompanying symptoms were bone and joint pain (43.4%), asthenia (22.4%), cough (19.7%), vomiting (17%) and headache (15.8%). The specific cause of the fever could not be pinpointed in 29 cases (38.1%). Aside from these cases, the main causes of fever were malaria (30.3%) and bronchopulmonary infections (22.4%). The white blood cell count was >20,000/mm3 in 47% of respiratory infections, 43.5% of the cases involving malaria and 55.2% of cases of fever with unknown cause. Hemoglobin levels were <5g/dl for 52.2% of the cases involving malaria and 22.6% for those of unknown origin. For four patients, all less than 10 years of age, the disease was fatal. Conclusion: For the majority of fever episodes, the underlying cause could not be determined. Nonetheless, malaria was identified as one of the principal identifiable causes of fever among children with SDC in Libreville. Treatment for malaria upon admission, and the promotion of preventative measures, therefore seems to be appropriate for our clinical setting. In light of the large number of unresolved cases, systematic prescription of broad-spectrum antibiotics may also be called for.

Pediatric obesity prevention:From na?ve examination of energy imbalance towards strategies that influence the competition for nutrient resources among tissues

Current pediatric obesity interventions have collectively yielded relatively unsuccessful results. In this Field of Vision, we present plausible physiologic underpinnings fostering ineffectiveness of conventional strategies grounded in requisite induction of negative energy imbalance. Moreover, such recommendations exacerbate the underlying metabolic dysfunction by further limiting metabolic fuel availability, lowering energy expenditure, and increasing hunger(recapitulating the starvation response amid apparent nutritional adequacy) which precede and promote obesity during growth and development. The qualitative aspects of musculoskeletal system(i.e., endocrine response, muscle functional capacity) are likely to improve metabolic function and increase nutrient delivery and utilization. An intricate and complex system including multiple feedback mechanisms operates to homeostatically regulate energy balance and support optimal body composition trajectories and metabolic health, during growth and development. Thus, ignoring the interdependencies of regulatory growth processes initiates a nuanced understanding of energy regulation and thus misguided attempts at preventive strategies. Importantly, these gains are not dependent upon weight-loss, rather we suggest can be achieved through resistance training. Collectively, optimizing musculoskeletal health via resistance training elicits augmentation of competitive capacity across systems. Further, substantial gains can be achieved in skeletal muscle mass, strength, and functional capacity through resistance training in a relatively short period of time.

Anemia in Juvenile Idiopathic Arthritis (JIA) and Other Pediatric Rheumatologic Diseases: A Retrospective Study

Objectives: The present study estimated the prevalence of anemia among children and adolescents with pediatric rheumatological diseases in a referral center, and analyzed the associated clinical and biological parameters. Methods: A retrospective chart review included 49 children with rheumatological diseases, who were diagnosed by a pediatric rheumatologist and classified according to the International League of Associations for Rheumatology (ILAR) guidelines and criteria endorsed by the American College of Rheumatologists. Anemia was defined as hemoglobin level lower than the 5th centile for the corresponding age and gender. Disease activity was indicated by serum levels of C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR), where available. Results: Participants were aged 2 - 18 years (mean ± SD = 10.41 ± 4.23 years), 38 (77.6%) of them had JIA, and 8 (16.3%) had systemic lupus erythematosus. The most frequent subtype of JIA was the polyarticular (16 out of 38, 42.1%), followed by systemic (14, 36.8%). The prevalence of anemia was 46.9% (95% CI = 32.5% - 61.7%), with no significant difference between JIA and other diseases or between the different JIA subtypes. Nevertheless, anemia was more frequently observed in younger patients (age 2 - 6 years: 69.2% vs <48%) and those with elevated ESR (68.8% vs 33.3%) or CRP (60.0% versus 45.2%), compared with their counterpart respectively;however, only the association with ESR was statistically significant (p = 0.049). No agreement was found between CRP and ESR (Kappa = 0.140). Conclusion: Anemia is frequent in JIA and other rheumatologic diseases in children, concerning approximately 50% of the patients and responding to anemia of inflammation as the major pathophysiological mechanism. Further research is warranted to provide more accurate insight into the pathophysiological mechanisms and clinical characteristics of anemia in pediatric rheumatological disease and to measure its morbidity, to provide efficient and evidence-based management strategies.

Hemodynamic Profiling Using a Cardiac Index–Systemic Vascular Resistance Plot in Patients with Fontan Circulation

Background: Elevated Fontan pressure (FP) alone cannot fully predict clinical outcomes. We hypothesized thathemodynamic profiling using a cardiac index (CI)-systemic vascular resistance (SVR) plot could characterize clinicalfeatures and predict the prognosis of post-Fontan patients. Methods: We included post-Fontan patients whounderwent cardiac catheterization at age < 10 years. Patients were classified into four categories: A, CI ≥ 3, SVRindex (SVRI) ≥ 20;B, CI < 3, SVRI ≥ 20;C, CI ≥ 3, SVRI < 20;and D, CI < 3, SVRI < 20. The primary outcome wasfreedom from the combined endpoint: new onset of protein-losing enteropathy or plastic bronchitis, heart transplant,and death. Clinical and hemodynamic variables and freedom from the endpoint were compared betweenthe hemodynamic categories and outcome predictors were evaluated. Results: Eighty-three patients wereincluded. Median follow-up was 5.3 years. Category A/B/C/D consisted of 4/15/53/11 patients, respectively. Allthe patients in category A were New York Heart Association I/II and had a significantly lower pulmonary vascularresistance index (PVRI). Patients in category C had lower pulmonary/systemic blood flow. Patients in category Dhad a higher PVRI and had the poorest freedom from the endpoint (44% at 5 years). Elevated FP and category Dwere outcome predictors. Conclusions: CI-SVR plots was a novel adjunctive method for Fontan hemodynamicprofiling.

Situs inversus totalis in an asymptomatic adolescent-importance of patient education:A case report

BACKGROUND Situs inversus totalis(SIT)may be an incidental finding in asymptomatic children.Patients may not understand the implications of this condition and the importance of relaying the diagnosis to their healthcare providers.CASE SUMMARY We report an asymptomatic seventeen-year-old adolescent with previouslydiagnosed SIT who presented for a routine well-child visit.During history taking,he denied any past medical conditions,including cardiovascular conditions.Only when physical exam revealed point of maximal impulse and heart sounds on the right side,did he convey that he had been diagnosed with SIT incidentally at age of 12 years.He was not aware of associated conditions or the potential implications of his diagnosis,nor did he realize it is pertinent medical history to be relayed to healthcare providers.Chest X-ray confirmed dextrocardia and abdominal X-ray showed right-sided stomach.Abdomen sonogram showed left-sided liver and right-sided spleen.Echocardiogram showed normal valvular structure and function.A comprehensive discussion was provided to address the patient’s lack of understanding that SIT is a medical diagnosis with potential implications.CONCLUSION While SIT is rare and mostly asymptomatic,affected patients may not comprehend the importance of the diagnosis and its potential ramifications.Recognition of the patient’s lack of awareness allows the healthcare provider to educate the patient and hopefully can prevent potential medical and surgical complications.

Eosinophilic gastroenteritis with cytomegalovirus infection in an immunocompetent child

A 3-year-old boy developed transient protein-losing gastroenteropathy associated with cytomegalovirus (CMV) infection. Both IgG and IgM antibodies to CMV were positive in a serologic blood test. Upper gastrointestinal endoscopy showed multiple erosions throughout the body of the stomach, without enlarged gastric folds. Histological examination of the biopsy specimens indicated eosinophilic gastroenteritis and CMV infection. The patient had complete resolution without specific therapy for CMV in four weeks. An allergic reaction as well as CMV infection played important roles in the pathogenesis of this case.

Therapy-refractory gastrointestinal motility disorder in a child with c-kit mutations

Constipation and fecal impaction are frequent and distressing complaints in pediatric gastroenterology. Especially in neurologically handicapped children, treatment of severe forms of slow-transit constipation (STC) can be difficult. In the majority of cases, STC is of unknown etiology. However, in recent years, there is growing evidence that interstitial cells of Cajal (ICCs), which serve as electrical pacemakers and generate spontaneous electrical slow waves in the gastrointestinal tract, might play an important role in the pathophysiology of STC. It remains unclear whether morphological ICC alterations seen in affected patients are based on congenital developmental anomalies, or whether they are a consequence of long-term constipation with secondary damage of the gastrointestinal nervous system. To the best of our knowledge, we present the first case of a patient with histological alterations in ICC morphology who displayed multiple alterations of c-kit at the level of mRNA. The protein encoded by c-kit is the receptor tyrosine kinase Kit (CD117), which is crucial for development and function of ICCs. Therefore, these findings provide a new explanation for congenital alterations of ICC development that result in gastrointestinal motility disorders.

Differential diagnosis in patients with suspected bile acid synthesis defects

AIM: To investigate the clinical presentations associated with bile acid synthesis defects and to describe identification of individual disorders and diagnostic pitfalls. METHODS: We describe semiquantitative determination of 16 urinary bile acid metabolites by electrospray ionization-tandem mass spectrometry. Sample preparation was performed by solid-phase extraction. The total analysis time was 2 min per sample. We determined bile acid metabolites in 363 patients with suspected defects in bile acid metabolism. RESULTS: Abnormal bile acid metabolites were found in 36 patients. Two patients had bile acid synthesis defects but presented with atypical presentations. In 2 other patients who were later shown to be affected by biliary atresia and cystic fibrosis the profile of bile acid metabolites was initially suggestive of a bile acid synthesis defect. Three adult patients suffered from cerebrotendinous xanthomatosis. Nineteen patients had peroxisomal disorders, and 10 patients had cholestatic hepatopathy of other cause. CONCLUSION: Screening for urinary cholanoids should be done in every infant with cholestatic hepatopathy as well as in children with progressive neurological disease to provide specific therapy.

Radiological response and inflammation scores predict tumour recurrence in patients treated with transarterial chemoembolization before liver transplantation

To investigate the prognostic value of the radiological response after transarterial chemoembolization (TACE) and inflammatory markers in patients affected by hepatocellular carcinoma (HCC) awaiting liver transplantation (LT).METHODSWe retrospectively evaluated the preoperative predictors of HCC recurrence in 70 patients treated with conventional (n = 16) or doxorubicin-eluting bead TACE (n = 54) before LT. The patient and tumour characteristics, including the static and dynamic alpha-fetoprotein, neutrophil-to-lymphocyte ratio and platelet-to-lymphocyte ratio (PLR) measurements, were recorded. Treatment response was classified according to the modified Response Evaluation Criteria in Solid Tumours (mRECIST) and the European Association for the Study of the Liver (EASL) criteria as complete response (CR), partial response (PR), stable disease or progressive disease. After examination of the explanted livers, histological necrosis was classified as complete (100% of the cumulative tumour area), partial (50%-99%) or minimal (< 50%) and was correlated with the preoperative radiological findings.RESULTSAccording to the pre-TACE radiological evaluation, 22/70 (31.4%) and 12/70 (17.1%) patients were beyond Milan and University of San Francisco (UCSF) criteria, respectively. After TACE procedures, the objective response (CR + PR) rates were 71.4% and 70.0% according to mRECIST and EASL criteria, respectively. The agreement between the two guidelines in defining the radiological response was rated as very good both for the overall and target lesion response (weighted k-value: 0.98 and 0.93, respectively). Complete and partial histological necrosis were achieved in 14/70 (20.0%) and 28/70 (40.0%) patients, respectively. Using histopathology as the reference standard, mRECIST criteria correctly classified necrosis in 72.9% (51/70) of patients and EASL criteria in 68.6% (48/70) of cases. The mRECIST non-response to TACE [Exp(b) = 9.2, p = 0.012], exceeding UCSF criteria before TACE [Exp(b) = 4.7, p = 0.033] and a preoperative PLR > 150 [Exp(b) = 5.9, p = 0.046] were independent predictors of tumour recurrence.CONCLUSIONThe radiological response and inflammatory markers are predictive of tumour recurrence and allow the proper selection of TACE-treated candidates for LT.

Expression of Clock genes in the pineal glands of newborn rats with hypoxic-ischemic encephalopathy

Clock genes are involved in circadian rhythm regulation, and surviving newborns with hypoxic-ischemic encephalopathy may present with sleep-wake cycle reversal. This study aimed to determine the expression of the clock genes Clock and Bmall, in the pineal gland of rats with hypoxic-ischemic brain damage. Results showed that levels of Clock mRNA v^re not significantly changed within 48 hours after cerebral hypoxia and ischemia. Expression levels of CLOCK and BMAL1 protein were significantly higher after 48 hours. The levels of Bmall mRNA reached a peak at 36 hours, but were significantly reduced at 48 hours. Experimental findings indicate that Clock and Bmall genes were indeed expressed in the pineal glands of neonatal rats. At the initial stage （within 36 hours） of hypoxic-ischemic brain damage, only slight changes in the expression levels of these two genes were detected, followed by significant changes at 36-48 hours. These changes may be associated with circadian rhythm disorder induced by hypoxic-ischemic brain damage.

Using quality improvement methods to increase use of pain prevention strategies for childhood vaccination

AIM To increase evidence-based pain prevention strategy use during routine vaccinations in a pediatric primary care clinic using quality improvement methodology.METHODS Specific intervention strategies(i.e.,comfort positioning,nonnutritive sucking and sucrose analgesia,distraction) were identified,selected and introduced in three waves,using a Plan-Do-Study-Act framework.System-wide change was measured from baseline to post-intervention by:(1) percent of vaccination visits during which an evidence-based pain prevention strategy was reported as being used; and(2) caregiver satisfaction ratings following the visit.Additionally,self-reported staff and caregiver attitudes and beliefs about pain prevention were measured at baseline and 1-year post-intervention to assess for possible long-term cultural shifts.RESULTS Significant improvements were noted post-intervention.Use of at least one pain prevention strategy was documented at 99% of patient visits and 94% of caregivers were satisfied or very satisfied with the pain prevention care received.Parents/caregivers reported greater satisfaction with the specific pain prevention strategy used [t(143) = 2.50,P ≤ 0.05],as well as greater agreement that the pain prevention strategies used helped their children's pain [t(180) = 2.17,P ≤ 0.05] and that they would be willing to use the same strategy again in the future [t(179) = 3.26,P ≤ 0.001] as compared to baseline.Staff and caregivers also demonstrated a shift in attitudes from baseline to 1-year post-intervention.Specifically,staff reported greater agreement that the pain felt from vaccinations can result in harmful effects [2.47 vs 3.10; t(70) =-2.11,P ≤ 0.05],less agreement that pain from vaccinations is "just part of the process" [3.94 vs 3.23; t(70) = 2.61,P ≤ 0.05],and less agreement that parents expect their children to experience pain during vaccinations [4.81 vs 4.38; t(69) = 2.24,P ≤ 0.05].Parents/caregivers reported more favorable attitudes about pain prevention strategies for vaccinations across a variety of areas,including safety,cost,time,and effectiveness,as well as less concern about the pain their children experience with vaccination [4.08 vs 3.26; t(557) = 6.38,P ≤ 0.001],less need for additional pain prevention strategies [3.33 vs 2.81; t(476) = 4.51,P ≤ 0.001],and greater agreement that their doctors' office currently offers pain prevention for vaccinations [3.40 vs 3.75; t(433) =-2.39,P ≤ 0.05].CONCLUSION Quality improvement methodology can be used to help close the gap in implementing pain prevention strategies during routine vaccination procedures for children.

Health-Related Quality of Life of Children and Adolescents with Juvenile Idiopathic Arthritis in Western Saudi Arabia

Objectives: To evaluate the effect of Juvenile Idiopathic Rheumatoid Ar-thritis (JIA) on the health-related quality of life (HRQOL) in Saudi children. Methods: A cross-sectional study was conducted in a tertiary hospital in Jeddah, Saudi Arabia to evaluate the HRQOL of children aged ≤ 18 years who had JIA using the childhood health assessment questionnaire modified for Arab children (CHAQ-MAC). Such questionnaire investigates 34 activities of daily life (ADL) classified into 8 life domains. Children or their parents were invited for face-to-face interview, and a phone interview was done for patients who missed their appointments during the period between February and July 2017. A statistical model was used to calculate a total CHAQ.MAC score (range = 0 - 33;Cronbach’s alpha = 0.966);with higher values indicating poorer HRQOL. Results: Of a total of 44 children (male ratio = 0.63;mean ± SD age = 9.95 ± 5.44), Systemic-onset JIA was the most frequent type (27.3%), followed by polyarticular (15.9%) and oligoarticular (13.6%). Pain was reported among 43.2% (frequently in the knee, in 27.3%) whereas morning stiffness was reported in 20.5%. The mean CHAQ.MAC score = 2.89 (75th centile = 3.00). With respect of ADLs, up to 22.7% of the children complained of difficulty;and 31.8% reported a difficulty in at least one of the 34 investigated ADLs. With respect of the life domain, children reported difficulties for activities (27.3%), dressing & grooming and hygiene (13.6%), and eating (6.82%). According to the life domain, 4.5% to 13.6% of the children needed help to execute the related ADLs and up to 9.1% used aids or devices. Poor HRQOL was associated with articular pain (p = 0.003) and specific medication (p = 0.043). Con-clusion: Children with arthralgia and those on specific treatment are at higher risk of impaired QOL, which emphasizes the need for systematic screening for treatment adverse effects and joint pain and implementation of efficient management to improve HRQOL.

在新生儿重症监护室同伴咨询者对母乳喂养率的影响:一项随机对照试验结果

Objective:To determine whether peer counselors impacted breastfeeding duration among premature infants in an urban population. Design:This was a randomized controlled clinical trial. Setting:The trial was conducted in the Newborn Intensive Care Unit at Boston Medical Center,an innercity teaching hospital with approximately 2000 births per year. Participants:One hundred eight mother-infant pairs were enrolled between 2001 and 2004. Pairs were eligible if the mother intended and was eligible to breastfeed per the 1997 guidelines from the American Academy of Pediatrics and if the infant was 26 to 37 weeks’ gestational age and otherwise healthy. Intervention:Subjects were randomized to either a peer counselor who saw the mother weekly for 6 weeks or to standard of care. Main Outcome Measure:The main outcome measure was any breastmilk feeding at 12 weeks postpartum. Results:Intervention and control groups were similar on all measured sociodemographic factors. The average gestational age of infants was 32 weeks(range,26.3-37 weeks) with a mean birth weight of 1875 g(range,682-3005 g). At 12 weeks postpartum,women with a peer counselor had odds of providing any amount of breastmilk 181%greater than women without a peer counselor (odds ratio,2.81 95%confidence interval,1.11-7.14 ; P=0.01). Conclusions:Peer counselors increased breastfeeding duration among premature infants born in an innercity hospital and admitted to the neonatal intensive care unit. Peer counseling programs can help to increase breastfeeding in this vulnerable population.

Factors Affecting Japanese HPV-Vaccination: Findings from the Semi-Structured Interviews with Adolescent Girls and Caregivers

The objective of the present study was to qualitatively assess the obstructive and facilitative factors affecting adolescent girls and their caregivers when the adolescent had received or was considering receiving the Human Papilloma Virus (HPV) vaccination. Using these data, we propose recommendations for medical and nursing staff concerned with HPV vaccination. Participants were 20 adolescent girls (aged 10 - 19 years) and their caregivers, who had visited any of the 3 pediatric clinics in the Tokyo metropolitan area during a specified period since HPV vaccination began in Japan. The girls and their caregivers were separately interviewed by 2 child and/or family nursing care specialists with a semi-structured interview. The responses were qualitatively analyzed by 2 specialists, and the obstructive and facilitative factors affecting participants’ decision to receive HPV vaccination were extracted from the responses. Among the 20 sets of participants, 7 adolescents had completed HPV vaccination, 9 were going to receive vaccination, and 4 had not received any vaccination. The obstructive/facilitative factors related to considering or receiving HPV vaccination and actual vaccination were extracted and 4 main categories of factors were identified. Facilitators toward HPV-vaccination of daughters included clear future self-image and visions, fear Cervical Cancer (CC) and desire to escape from CC, having discussion with mothers about HPV-vaccination and CC, and to have a boyfriend. Barriers toward vaccination included the mothers’ reluctance to explain the sexual matters about HPV-vaccination to their daughters and difficulty with find the appropriate clinic or hospital to HPV-vaccination. Relevant factors about vaccination included positive family attitudes toward vaccination, having family system allowing consultation and having a public financial support for vaccination for daughters. Our conceptual model adapted from the Katz, et al. conceptual framework integrated the key barriers and facilitators as factors within each of four domains. These four domains have an important link. Especially, the environmental factors and the structural and sociocultural factors domain affect the individual adolescent and the caregiver factors domain, respectively. The results of present study suggest that medical/nursing activities centered on promoting HPV vaccination in Japan should comprehensively cover CC/vaccination/sex education in an integrated fashion, while schools and public health centers should provide opportunities for caregivers and adolescents to jointly participate in awareness education on HPV vaccination.

No Correlation between <i>AVPR</i>1A Promoter Polymorphisms and Prepulse Inhibition in Patients with Nocturnal Enuresis

Introduction: A correlation between AVPR1A promoter polymorphisms and prepulse inhibition (PPI) of startle reflexes has been described in healthy adults. Many children with nocturnal enuresis (NE) have a reduced PPI and treatment with desamino arginine vasopressin (dDAVP), a ligand of the arginine vasopressin receptor 1A (AVPR1A), and both improve clanical symptoms and significantly increase PPI. Methods: In 17 children (median 9.1 years, range 6.4-17.3) with NE, promoter repeats within the RS1 and RS3 regions of AVPR1A were quantified and correlated to PPI (native and age-adjusted). Results: No direct correlation was found between the number of promoter repeats at RS1 and PPI (correlation coefficient—0.240, p = 0.346) or RS3 and PPI (correlation coefficient—0.0192, p = 0.936), with no change through age-adjustment of PPI. The different RS3 length subgroups did not show differences in PPI, nor did differentiation of NE according to clinical subtype or treatment response to dDAVP show differences in the number of promoter repeats. Conclusion: The missing reproducibility of the correlation between AVPR1A promoter polymorphisms and PPI in a group with wide range of PPI suggests a more complex interaction. Therefore, further investigations are needed to analyze this very plausible interaction. Conditions with a reduced PPI, such as enuresis, schizophrenia or autism, are particularly interesting for this research.

Should Empyema with or without Necrotizing Pneumonia in Children Be Managed Differently?

Background: Necrotizing pneumonia (NP) is an increasing lung infection mostly associated with pleural empyema. Objectives: We aimed to compare children with empyema with and without concomitant NP, in terms of risk factors, management and outcome. Methods: We retrospectively included children hospitalized between 2005-2014 with empyema to whom a computed tomography was performed. We recorded patient characteristics, clinical, biological (blood and pleural fluid) and radiological findings, medical and surgical treatments, and clinical, radiological and functional follow-up. Results: 35 children with empyema were included, including 25 with a concomitant NP. Patients with or without NP were undistinguishable, in terms of characteristics, symptoms at admission or detected pathogens. Pleural leucocytes were significantly higher in the empyema group (p = 0.0002) as pleural LDH (p = 0.002), and pleural/blood LDH ratio (p = 0.0005). Medical and surgical managements were similar between both groups. Complications occurred in 1/10 children with empyema alone (pneumatocele) and 5/25 with concomitant NP (bronchopleural fistula (n = 3), lobectomy, pneumothorax). The hospital length of stay and delay for chest X-ray normalization were similar in both groups. Conclusion: Except for minor biological parameters, the presence of concomitant NP in case of empyema does not change the presentation, clinical features, management and outcome, suggesting that the presence of additional NP to empyema should not be managed differently. Therefore, in case of empyema with suspected concomitant NP, chest CT should probably be restricted to abnormal worsening or when mandatory for surgical treatment.

An Unusual and Previously Unreported Association between Tyrosinemia Type 1 and an Extremely Rare Variation of Congenital Cystic Dilatation: TODANI’s VI Cystic Duct Cyst: Report of a Case

Cystic duct cysts are rare lesions, and type VI of TODANI’s modified classification is the rarest subtype with only sporadic case reports in the literature. The following report describes the coexistence of this entity and type 1 tyrosinemia in a one month-old infant referred to our department for etiological investigations of prolonged neonatal cholestasis. To the best of our knowledge, we report herein the first case in the literature describing this association. Cystic duct abnormalities should be considered in the differential diagnosis of neonatal cholestases, however further investigations should be performed to exclude associated life-threatening conditions such as metabolic disorders including tyrosinemia.

Effectiveness of Rituximab Therapy on Severe Calcinosis in 4 Children with Juvenile Dermatomyositis

Background: Calcinosis is an important sequela of JDM which may cause significant morbidity and mortality. There is no standard curative treatment for calcinosis but different agents were used with variable efficacy. We report the favorable outcome of rituximab on severe calcinosis in 4 JDM patients and present their clinical data. Patients and Methods: A retrospective chart review of 4 children with JDM and severe calcinosis who received rituximab for relapsing or polycyclic JDM course. Diagnosis and follow up of calcinosis was clinically and by X-ray. Review data included: age of patients at onset of JDM symptoms and diagnosis, clinical and laboratory criteria at diagnosis, disease course and duration of follow up. Data about calcinosis onset, sites, severity and its progression were also included. Further data about rituximab therapy included: dosage, side effects, other treatment used before, during or after this drug and outcome and duration of follow up of calcinosis after therapy. Results: 4 patients (2 male, 2 female), interval between onset of symptoms and diagnosis was 6 - 12 months, course of JDM was polycyclic or relapsing, duration of follow up was 5 - 7 years. Calcinosis was severe causing ulceration, recurrent skin infections and joint limitation. It was not improving despite treatment with different DMARDs and/or bisphosphonates, colchicine and warfarin. Reason to start rituximab was inadequate disease control with conventional DMARDs. All patients received steroids and more than one DMARD before starting rituximab and were continued thereafter, follow up after rituximab was 3 to 5 years. All patients had improvement in disease activity and frequency of admission especially due to complications of calcinosis. One patient had complete clearance of calcinosis for the last 5 years. Others had significant improvement in calcinosis with no new lesions, decreased sites and density and decreased calcinosis related contractures. There were no serious side effects to rituximab. Conclusion: Our study showed the favorable effect of rituximab in treatment of calcinosis in 4 patients with JDM-associated severe calcinosis when it was used with other conventional DMARDs.

Giant Omphalocele Ruptured: An Operated Case and Review of the Literature

Background: The omphalocele is a congenital defect of closure of the abdominal wall at the level of the umbilical ring with evisceration of the intra-abdominal organs in the form of a bag covered with a membrane. This membrane can rupture and become an extreme emergency that can put the infant’s life at stake. Aim: The purpose of this case report is to present a case of ruptured omphalocele operated successfully and describe the factors of good prognosis. Case Presentation: A female new-born with a giant omphalocele. This omphalocele ruptured during conservative treatment. An emergency surgery was organised and the defect was closed by using a prosthesis. The postoperative course was simple. During the 12 months follow-up, we have seen no complication. Conclusion: The management of ruptured omphaloceles is a surgical and anesthetic challenge. The mortality of this condition can be improved in our context by setting up well-equipped neonatal resuscitation units.

Manifestations of COVID-19 infection in children with malignancy:A single-center experience in Jordan

BACKGROUND The coronavirus disease 2019(COVID-19)has been the cause of a global health crisis since the end of 2019.All countries are following the guidelines and recommendations released by the World Health Organization to decrease the spread of the disease.Children account for only 3%-5%of COVID-19 cases.Few data are available regarding the clinical course,disease severity,and mode of treatment in children with malignancy and COVID-19.AIM To evaluate the treatment plan and outcome of children with malignancy who contracted COVID-19.METHODS A retrospective study of the medical files of patients with malignancy who contracted COVID-19 between July 2020 and June 2021 was performed.The following data were reviewed for all patients:primary disease,laboratory data,admission ward,clinical status upon admission,disease course,treatment plan,and outcome.Eligible patients were those with malignancy who tested positive for COVID-19 by reverse transcription polymerase chain reaction.RESULTS A total of 40 patients who had malignancy contracted COVID-19 from July 1,2020 to June 1,2021.Their primary diseases were as follows:34 patients(85%)had hematological malignancies(30 had acute lymphoblastic leukemia,2 had acute myeloblastic leukemia,and 2 had Hodgkin lymphoma),whereas 6 patients(15%)had solid tumors(2 had neuroblastoma,2 had rhabdomyosarcoma,and 2 had central nervous system tumors).Twelve patients(30%)did not need hospitalization and underwent home isolation only,whereas twenty-eight patients(70%)required hospitalization(26 patients were admitted in the COVID-19 ward and 2 were admitted in the pediatric intensive care unit).CONCLUSION COVID-19 with malignancy in the pediatric age group has a benign course and does not increase the risk of having severe infection compared to other children.