Clinical characteristics and outcomes of the multisystem inflammatory syndrome in children (MIS-C) following COVID-19 infection in Iran: A multicenter study.

OBJECTIVES: This study aimed to assess the clinical characteristics, treatment and outcomes of the multisystem inflammatory syndrome in children (MIS-C) following COVID-19 in five different geographical regions of Iran. METHODS: In this multicenter observational study, patients <21 years were included between March 2020 and October 2021. By Disease Control and Prevention (CDC) checklist, demographic characteristics, comorbidities, clinical signs and symptoms, laboratory and radiology findings, and treatment were collected. Statistical analysis was using Chi-square and t-test in STATA14. RESULTS: In total 225 patients with median age of 55 (26-96) months were included that 59.56% boys. 57.33% were admitted to the PICU with a median of 7 days (4-10). 95.56% of patients were discharged with recovery and the rest died. All of the patients in our study were included based on the MIS-C criteria. However, some patients had Kawasaki symptoms, so we compared the clinical and epidemiological characteristics of the two groups. Conjunctival injection, cervical lymphadenopathy>1.5 cm diameter, and strawberry tongue in Kawasaki-like MIS-C patients were higher than of MIS-C patients, and this difference was significant(p<0.001). The most common comorbidity was obesity (24.86%). Most patients tested for COVID-19 and about 60% of the patients had a positive test by serology or reverse transcription-polymerase chain reaction (RT-PCR). Gastrointestinal (88.89%) and hematologic signs (84.44%) were most common. Most drugs used in patients were IVIG and steroids. 88.07% and 61.29% of the patients had at least one problem in echocardiography and lung CT, respectively. CONCLUSIONS: The best outcome was seen in patients who were treated with both IVIG and steroids on the first days of admission. Myocarditis was common in two groups of patients. According to most patients had echocardiography abnormal, screening of heart function is recommended for patients.

Non-effectiveness of Ivermectin on Inpatients and Outpatients With COVID-19; Results of Two Randomized, Double-Blinded, Placebo-Controlled Clinical Trials.

Background: Ivermectin which was widely considered as a potential treatment for COVID-19, showed uncertain clinical benefit in many clinical trials. Performing large-scale clinical trials to evaluate the effectiveness of this drug in the midst of the pandemic, while difficult, has been urgently needed. Methods: We performed two large multicenter randomized, double-blind, placebo-controlled clinical trials evaluating the effectiveness of ivermectin in treating inpatients and outpatients with COVID-19 infection. The intervention group received ivermectin, 0.4mg/kg of body weight per day for 3 days. In the control group, placebo tablets were used for 3 days. Results: Data for 609 inpatients and 549 outpatients were analyzed. In hospitalized patients, complete recovery was significantly higher in the ivermectin group (37%) compared to placebo group (28%; RR, 1.32 [95% CI, 1.04-1.66]; p-value = 0.02). On the other hand, the length of hospital stay was significantly longer in the ivermectin group with a mean of 7.98 ± 4.4 days compared to the placebo receiving group with a mean of 7.16 ± 3.2 days (RR, 0.80 [95% CI, 0.15-1.45]; p-value = 0.02). In outpatients, the mean duration of fever was significantly shorter (2.02 ± 0.11 days) in the ivermectin group versus (2.41 ± 0.13 days) placebo group with p value = 0.020. On the day seventh of treatment, fever (p-value = 0.040), cough (p-value = 0.019), and weakness (p-value = 0.002) were significantly higher in the placebo group compared to the ivermectin group. Among all outpatients, 7% in ivermectin group and 5% in placebo group needed to be hospitalized (RR, 1.36 [95% CI, 0.65-2.84]; p-value = 0.41). Also, the result of RT-PCR on day five after treatment was negative for 26% of patients in the ivermectin group versus 32% in the placebo group (RR, 0.81 [95% CI, 0.60-1.09]; p-value = 0.16). Conclusion: Our data showed, ivermectin, compared with placebo, did not have a significant potential effect on clinical improvement, reduced admission in ICU, need for invasive ventilation, and death in hospitalized patients; likewise, no evidence was found to support the prescription of ivermectin on recovery, reduced hospitalization and increased negative RT-PCR assay for SARS-CoV-2 5 days after treatment in outpatients. Our findings do not support the use of ivermectin to treat mild to severe forms of COVID-19. Clinical Trial Registration: www.irct.ir IRCT20111224008507N5 and IRCT20111224008507N4.

Developing a National Minimum Data Set for Kawasaki Disease Registry in Iran.

Background: Kawasaki Disease is an acute and self-limited systemic inflammatory and febrile illness, which is the most common cause of acquired heart disease in children in developed countries. The incidence of KD in Asian countries is high. But, data is not available from the Middle East. So, the aim of this study was to develop an MDS to set up a national registry for KD to estimate the burden of disease in Iran. Materials and Methods: This cross-sectional and descriptive study was conducted in 2020. Literature review, data collection from patients medical records, and expert panel approach were used to design this MDS. Data elements with a Content Validity Ratio (CVR) of more than 0.56 were selected as the MDS of the registry. Results: Overall, 99 data elements were recognized. Of which, 51 and 48 data elements were verified and rejected, respectively. Moreover, 17 data elements were added as required by experts. Eventually, 68 data elements were chosen as the MDS of the national KD registry of IRAN; of which, 17 and 51 data elements were classified as administrative and clinical data, respectively. Conclusions: These precise, integrated, and comprehensive developed data elements and the national KD registry will lead to effective disease management and thus, improve the quality of care and, consequently, improve public health.

Epidemiological data of national Kawasaki disease registry in Iran, 2007-2019.

Introduction: Kawasaki disease(KD) is a vasculitis of childhood that tends to influence the coronary arteries. There is no national data about the prevalence of KD in Iran. This study aimed to perform a national registry in Iran for 13 years. Methods: In this retrospective study, the data for KD extracted from medical records of <19 year-old patients admitted to tertiary hospitals in Iran between 2007 and 2019 were recorded in the national KD registry system. Age, admission date, gender, location, and presence of KD criteria, laboratory and echocardiography findings, and treatment modalities were evaluated. Complete KD was considered if ≥4 clinical criteria of the KD existed and otherwise, incomplete KD was considered. Results: Data from 1,682 KD patients including 999(59.39%) boys and 683(40.61%) girls and male/female ratio of 1.46 were evaluated. The mean age was 3.08 ± 2.49 years and 1465(87%) were living in urban regions. The yearly incidence of the disease was between 2.62 to 3.03 from 2015 to 2019. The highest age-specific incidence was observed in children <1-year-old. Incomplete and resistant KD included 1,321(78.54%) and 9(0.54%) patients, respectively. Abnormal echocardiography was detected in 619(36.80%) patients. Leukocytosis, with dominancy of neutrophils, anemia, thrombocytosis and increased ESR and CRP were the most noticeable laboratory findings. No death due to KD disease was reported. Conclusion: Based on this study, most of the KD cases are presented with atypical presentation in Iran. So, increasing awareness of primary healthcare workers by educating and updating their data is very important in timely diagnosis and management of the disease.

Comparing Watchful Waiting Approach vs. Antibiotic Therapy in Children with Nonsevere Acute Otitis Media: A Randomized Clinical Trial.

OBJECTIVE: To compare both approaches for the treatment of nonsevere acute otitis media (AOM) in Iran. METHODS: This randomized clinical trial was performed at a pediatric infectious diseases clinic in Buali tertiary hospital in Sari, north of Iran, from 2016 to 2018. All participants in this study were previously healthy children with AOM diagnosis, who were 6 months to 6 years old. The patients were randomly assigned into two groups: the intervention (80 mg/kg/day amoxicillin for 7-10 days) and the control group (watchful waiting approach). AOM recovery and adverse drug reactions were evaluated after 72 hours, and the patients were followed for the frequency of AOM and middle ear effusion 1 and 3 months' postintervention. RESULTS: A total of 396 children have participated in this study. AOM recovery was significantly different in the two groups (73% vs. 44% in the intervention and control groups, respectively). Recurrence of AOM and middle ear effusion (MEE) persistence, one month following the intervention, have not shown any significant differences between the two groups. However, the AOM recurrence between 1 and 3 months was more frequent in the control group. The frequency of diarrhea was also higher in the intervention group compared to the control but no significant difference was found between the two groups regarding vomiting and skin rash. CONCLUSION: The faster recovery from AOM is achieved when an antibiotic treatment regimen is applied, although the risk of potential side effects should be considered.

Clinical Characteristics and Outcomes of COVID-19 in Children in Northern Iran.

OBJECTIVE: Since December 2019, the coronavirus disease 2019 (COVID-19) has been spread rapidly all over the world, infecting all age groups with this novel virus. In this manuscript, we report characteristics of children with COVID-19 in Mazandaran province, northern Iran. METHOD: From 12 February to 28 July 2020, medical records of 100 children diagnosed with COVID-19 admitted to the hospitals of Mazandaran province were collected. Patients' age, gender, clinical symptoms, and signs, in addition to therapeutic management and outcomes, were reported. RESULTS: 57 (57%) boys and 43 girls with the mean age of 104.63 ± 79.14 months were evaluated. 20 patients (20%) were transferred to the PICU (pediatric intensive care unit), and 13 children experienced a severe form of the disease, pediatric inflammatory multisystem syndrome (PIMS). The mean duration of hospitalization was 5.3 ± 4.7 days. Fever (81%), respiratory (79%), gastrointestinal (47%), and neurologic complaints (29%) were experienced by the patients in addition to skin rash (14%). Sixty-two patients needed supplemental oxygen, and 6 of them underwent endotracheal intubation. Leukopenia was reported in 7, anemia in 24, and thrombocytopenia in 12 patients. 4 patients with underlying diseases including chronic renal failure, Down syndrome with cerebral palsy, and morbid obesity died. CONCLUSION: COVID-19 can cause symptoms in children in two stages. In the first week, upper and lower respiratory symptoms can occur which has lower severity and prevalence compared to adults. But after 2-3 weeks following infection, symptoms of MIS-C or multisystem involvement can occur and COVID-19 should be considered. The most common indication for admission is fever, rash, and respiratory problems.

Effects of Ivermectin in Patients With COVID-19: A Multicenter, Double-blind, Randomized, Controlled Clinical Trial.

PURPOSE: Given the coronavirus disease 2019 (COVID-19) pandemic, there is a global urgency to discover an effective treatment for patients withthis disease. This study aimed to evaluate the effects of the widely used antiparasitic drug ivermectin on outcomes in patients with COVID-19. METHODS: In this randomized, double-blind clinical trial, patients with COVID-19 admitted to 2 referral tertiary hospitals in Mazandaran, Iran, were randomly divided into 2 groups: intervention and control. In addition to standard treatment for COVID-19, the intervention group received a single weight-based dose (0.2 mg/kg) of ivermectin; the control group received the standard of care. Demographic, clinical, laboratory, and imaging data from participants were recorded at baseline. Patients were assessed daily for symptoms and disease progression. The primary clinical outcome measures were the durations of hospital stay, fever, dyspnea, and cough; and overall clinical improvement. FINDINGS: Sixty-nine patients were enrolled (mean [SD] ages: ivermectin, 47.63 [22.20] years; control, 45.18 [23.11] years; P = 0.65). Eighteen patients (51.4%) in the ivermectin group and 18 (52.9%) in control group were male (P = 0.90). The mean durations of dyspnea were 2.6 (0.4) days in the ivermectin group and 3.8 (0.4) days in the control group (P = 0.048). Also, persistent cough lasted for 3.1 (0.4) days in the ivermectin group compared to 4.8 (0.4) days in control group (PP = 0.019). The mean durations of hospital stay were 7.1 (0.5) days versus 8.4 (0.6) days in the ivermectin and control groups, respectively (P = 0.016). Also, the frequency of lymphopenia decreased to 14.3% in the ivermectin group and did not change in the control group (P = 0.007). IMPLICATIONS: A single dose of ivermectin was well-tolerated in symptomatic patients with COVID-19, and important clinical features of COVID-19 were improved with ivermectin use, including dyspnea, cough, and lymphopenia. Further studies with larger sample sizes, different drug dosages, dosing intervals and durations, especially in different stages of the disease, may be useful in understanding the potential clinical benefits ivermectin. Iranian Registry of Clinical Trials identifier: IRCT20111224008507N3.

Knowledge, attitude and practice of Sari birth cohort members during early weeks of COVID-19 outbreak in Iran.

BACKGROUND: It has been determined that the coronavirus disease 2019 (COVID-19) pandemic needs social distancing and proper measures to prevent its spreading. This study aimed to determine COVID-19 knowledge, attitude, and practice among Sari Birth Cohort (SBC) members. METHODS: In this cross-sectional study linked to the SBC in north of Iran, mothers living in Sari and its suburbs from March 28 to April 8, 2020 were evaluated. The measurement tool was an online researcher-made, self-reported knowledge, attitude, and practice questionnaire related to COVID-19. RESULTS: In total, 1449 mothers with a mean age of 31.51 ± 5.73 years participated. Of them, 82.4% had good knowledge (98.6% in healthcare workers and 79.2% in housewives, p = 0.000). Most of them were worried about spread of the disease in the country (97.4%) and agreed that COVID-19 will finally be successfully controlled around the world (72.2%). Sleep disturbance was reported in 42.7% of mothers. Eighty-eight percent of cases wore masks and gloves when leaving home, 99.4% washed their hands frequently while 12.9% went to any crowded places. People with better knowledge followed safer practices (p = 0.000) and were more worried about the spread of the disease in the country and infection (among themselves and their first-degree relatives) (p = 0.000). CONCLUSIONS: Most of the SBC members had a good level of knowledge about COVID-19 but were worried about a long-term pandemic period. They also had good practices regarding the prevention of the disease.

COVID-19-Associated Multisystem Inflammatory Syndrome Complicated with Giant Coronary Artery Aneurysm.

In the early stages of the outbreak of the novel coronavirus disease 2019 (COVID-19), it was assumed that this infection is very mild and uncommon in children. However, recent reports have shown that children may also develop the disease and its severe complications. These complications included shock, multisystem inflammatory syndrome in children (MIS-C), and pneumonia in children. A previously healthy 14-month-old boy presented with fever, irritability, and skin rash, besides changes in the lips, conjunctiva, and tongue. His medical history, clinical presentations, treatment, laboratory data, and follow-up information were recorded. He was treated according to the diagnosis of Kawasaki disease (KD). He had a history of close contact with a COVID-19 patient. However, the result of reverse transcription-polymerase chain reaction (RT-PCR) assay for COVID-19 was negative. Immunoglobulin M for COVID-19 was positive (1.20), while immunoglobulin G was negative (0.37). Three weeks later, seroconversion of COVID-19 immunoglobulin G (1.42) occurred. Despite treatment with two doses of intravenous immunoglobulin and methylprednisolone, coronary artery ectasia was detected. On the sixth day of hospitalization, the patient experienced hypotension, which necessitated treatment with inotropic drugs and resulted in a change of diagnosis to MIS-C. The later echocardiography showed evidence of coronary artery aneurysm (CAA), which finally changed to giant CAA. Although the patient was treated with infliximab, the size of CAA showed a significant decrease in the one-month follow-up. This is the first report of MIS-C during the COVID-19 pandemic in Iran, accompanied by KD, which was complicated with giant CAA.

Clinical characteristics of 10 children with a pediatric inflammatory multisystem syndrome associated with COVID-19 in Iran.

BACKGROUND: Although symptoms and signs of COVID-19 (Coronavirus disease 2019) in children are milder than adults, there are reports of more severe cases which were defined as pediatric inflammatory multisystem syndrome (PIMS). The purpose of this report was to describe the possible association between COVID-19 and PIMS in children. METHODS: From 28 March to 24 June 2020, 10 febrile children were admitted with COVID-19 infection showing characteristics of PIMS in Buali tertiary hospital of Sari, in Mazandaran province, northern Iran. Demographic and clinical characteristics, laboratory and imaging findings, and therapeutic modalities were recorded and analyzed. RESULTS: The mean age of the patients was 5.37 ± 3.9 years (13 months to 12 years). Six of them were boys. Kawasaki disease, myocarditis, toxic shock syndrome, appendicitis, sepsis, urosepsis, prolonged febrile seizure, acute hemorrhagic edema of infancy, and COVID-19-related pneumonia were their first presentation. All of them had increased C-reactive protein levels, and most of them had elevated erythrocyte sedimentation rate, lymphopenia, anemia, and hypoalbuminemia. Three of them had thrombocytopenia(PLT < 106). Six of them were serologically or polymerase chain reaction positive for COVID-19, and 4 of them were diagnosed as COVID-19 just by chest computed tomography scan. Most of the patients improved without a residual sequel, except one who died with multiorgan failure and another case was discharged with a giant coronary aneurysm. CONCLUSIONS: Children with COVID-19 may present symptoms similar to Kawasaki disease and inflammatory syndromes. PIMS should be considered in children with fever, rash, seizure, cough, tachypnea, and gastrointestinal symptoms such as vomiting, diarrhea, and abdominal pain.

Fever with Rash is One of the First Presentations of COVID-19 in Children: A Case Report.

INTRODUCTION: Clinical presentations of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vary among patients, which make a confusing challenge for clinicians to diagnose the disease. While the disease is not rare, but the symptoms may be atypical in children. CASE PRESENTATION: A 12-month-old boy with fever and rash without respiratory complaints at the onset of the disease presented to the hospital. His illness was then accompanied by severe anemia, hypoalbuminemia, hypoxemia, and pleural effusion. The nasopharyngeal swab polymerase chain reaction test was positive for the novel coronavirus. Despite primary normal lung imaging, the second imaging was typical for the disease on the third day of admission. DISCUSSION: Dermatologic manifestations of the novel coronavirus infection are not rare during the illness but rarely occur at the onset of the disease. Information about the cutaneous manifestations of SARS-CoV-2 and its time interval from the onset of the disease in children are not clear yet. Severe anemia, hypoalbuminemia, and pleural effusion are not reported as classic findings of the novel SARS-CoV-2. They may be considered as a consequence of the hyperinflammatory response or direct effect of the virus. CONCLUSION: As a different manifestation of SARS-CoV-2, infected children may initially present fever and rash. More studies are needed to discover the cause of these complications. The correct answer may lead to better insight and more effective treatment.

Familial Mediterranean Gene (MEFV) Mutation in Parents of Children with Familial Mediterranean Fever: What Are the Exceptions?

OBJECTIVES: Familial Mediterranean Fever (FMF) is one of the most prevalent periodic fever syndromes; MEFV, the responsible gene for the disease, is in the short arm of chromosome16. In the considerable count of the FMF patients, only one mutation is found in the MEFV and parents, who were the obligatory carriers for that mutation, were asymptomatic. The aim of this study was to evaluate these asymptomatic parents in regard to mutation in MEFV gene and similarity between parents and offspring patients. METHODS: In this cross-sectional study, asymptomatic parents of FMF patients enrolled the study were referred to periodic fever clinic or pediatric rheumatology clinic of Tehran University of Medical Sciences. The patients should have at least one mutation in MEFV gene and none of them had any family history of autoinflammatory disease. Twelve mutations in MEFV gene were assessed in the parents by Vienna Lab FMF Strip Assay kit by MAS PCR/Reverse hybridization. RESULTS: Forty-three patients and their parents participated in the study. Sixty-three percent (27) of patients were male. Onset of disease symptoms in 31 patients (72%) was before 4 years of old. Nine (21%) of the patients had homozygote, 16 (37%) compound heterozygote, and 17(40%) heterozygote for MEFV mutation; there was a case of complex alleles mutations (2%). M694V/M694V in 4 patients (9%) was the most homozygote genotype, and M694V/R761H in 4 (9%) and E148Q in 7 (16%) were the most compound heterozygote and heterozygote genotype, respectively. M694V, M680I, and E148Q were the most mutation in the parents. Overall, 41 patients had mutations similar to their parents' mutation, except 2 whose parents had no mutation, but a patient did. CONCLUSION: It seems that occurrence of new mutations in offspring is not prevalent among FMF patients and there are other reasons for different clinical presentation in similar mutation carriers. On the other hand, in ethnicities with high prevalence of FMF, new mutation in descendant may occur, infrequently.

Pseudotumor Cerebri as the First Manifestation of Juvenile Systemic Lupus Erythematosus.

INTRODUCTION: Headache is a common neuropsychiatric manifestation of juvenile systemic lupus erythematous (JSLE). Pseudotumor cerebri (PTC) is an uncommon cause of headache, presenting especially in active JSLE. In this paper, we report a case of missed intractable headache that was eventually diagnosed as PTC and presented as the first manifestation of JSLE. CASE PRESENTATION: A 9-year-old girl with a history of progressive headache for four months, fever, fatigue, myalgia, arthralgia, small-joint arthritis of the hands, and recent diplopia was referred to our clinic. The diagnosis of PTC in the background of JSLE was made based on her history, physical examination, positive laboratory findings, and increased intracranial pressure, with normal neuroimaging. Treatment with high-dose prednisolone led to dramatic resolution of the headache. CONCLUSIONS: It seems that a complete neurologic examination in newly diagnosed SLE patients is mandatory, especially in the presence of any neuropsychiatric manifestations, such as headache.

Spectrum of bone marrow failures of myeloid series: new report of neutropenic patients from a referral pediatric center in Iran.

Neutropenia is a reduction of the absolute neutrophil count (ANC), which could be seen in different conditions, while its association with a number of primary immunodeficiency diseases has been reported. This study was performed in all neutropenic patients who were admitted in a referral pediatric hospital during a 6-year period (2006-2011). One hundred and forty patients with ANC of below 1500/mm(3) were investigated in this study. The most common causes of neutropenia were severe congenital neutropenia (41%), aplastic anemia (19%), cyclic neutropenia (11%), hyperimmunoglobulin M syndrome (9%), and fanconi anemia (7%). The patients experienced their first manifestation at a median age of 1 year, while the median diagnostic age was 21 months. Parental consanguinity was present in about half of the cases. The most common clinical manifestations of the patients were sinusitis (62 cases), periodontitis (51 cases), acute diarrhea (39 cases), pneumonia (38 cases), abscess (36 cases), skin rashes (35 cases), and otitis media (31 cases). Twenty two patients (16%) died during the study period. Considering the differential diagnosis of neutropenia, making the diagnosis and appropriate treatments are the keys in management of patients with neutropenia to avoid further complications.

Clinical manifestations, laboratory findings, and therapeutic regimen in hospitalized children with brucellosis in an Iranian Referral Children Medical Centre.

Brucellosis is considered a known widespread zoonotic disease and is endemic in Mediterranean region, like Iran. This study reviewed the clinical manifestations, laboratory findings, and therapeutic regimen in childhood brucellosis in Iran. In this retrospective study, we reviewed hospital-records of 34 consecutive children with a confirmed diagnosis of brucellosis among a total number of 10,864 patients admitted to Children's Medical Center, Tehran, Iran, between 2002 and 2010. Among the patients diagnosed with brucellosis, 22 (65%) were admitted during spring and summer. Clinical findings of these patients at admission were arthritis, splenomegaly, hepatomegaly, lymphadenopathy, maculopapular skin rashes, and fever. Anaemia (53%) and leukopenia (33%) were the most common findings in the children. Only one patient had presented with leukocytosis. Four children (12%) were thrombocytopenic, and none of patients had pancytopenia. Blood cultures were positive in 5 patients (23%). Only one patient underwent bone-marrow aspiration and had positive culture for Brucella spp. Positive titres were found in 33 cases (97%) in Wright test, 23 cases (96%) in Coombs test, and 16 patients (72.7%) in 2ME (2-Mercaptoethanol) test. In one case, Wright and Coombs test titres were below 1:80 while Brucella spp. were isolated from blood at the same time. It is concluded, prolonged fever with joint involvement and organomegaly may increase possibility of infection with Brucella spp. Appropriate treatment regimen by more tolerable oral drugs, with a duration of at least 8 weeks, is recommended.

Sildenafil and ventriculo-arterial coupling in Fontan-palliated patients: a noninvasive echocardiographic assessment.

The fundamental role of pulmonary vascular resistance in the Fontan circulation is obvious. Medications decreasing this resistance may have an impact on the fate of this population. Hence, we assessed noninvasively the effect of oral sildenafil on the ventriculo-arterial coupling in patients with Fontan circulation. In a single-center, prospective case series study, 23 patients with fenestrated extracardiac total cavopulmonary connection age 12-31 years were enrolled in this study. Clinical characteristics and echocardiographic examination were performed before and after a 1 week course of sildenafil at 0.5 mg/kg every 8 h. Sildenafil had no effect on heart rate and blood pressure. However, oxygen saturation was significantly increased with sildenafil (87.6 ± 4.3 vs. 90.1 ± 3.6; P < 0.0001). The calculated noninvasive ventricular end-systolic elastance (Ees) was greater after sildenafil compared with the pre-sildenafil values (1.59 ± 0.17 vs. 1.72 ± 0.27 mm Hg/ml; P = 0.001). Moreover, significant decreases in arterial elastance (Ea) (1.62 ± 0.53 vs. 1.36 ± 0.43 mm Hg/ml; P < 0.0001), ventricular end-diastolic elastance (Eed) (0.05 ± 0.021 vs. 0.04 ± 0.013; P = 0.002), and, finally, ventriculo-arterial coupling index (0.99 ± 0.26 vs. 0.76 ± 0.15; P < 0.0001) were found after sildenafil administration. The intolerable side effects that led to stopping the sildenafil occurred only in one (4 %) patient. Sildenafil has increased ventricular systolic elastance and improved ventriculo-arterial coupling in patients palliated with Fontan circulation. Short-term sildenafil was well tolerated in most of the patients with only minor side effects.

Apparent life-threatening events in neonatal period: clinical manifestations and diagnostic challenges in a pediatric referral center.

OBJECTIVE: Apparent Life-Threatening Events (ALTEs) is an episode that is frightening to the observer and is characterized by some combination of apnea, color change, altered muscle tone, choking, and gagging. This study was designed to evaluate and follow up neonates who presented with clinical manifestation of an ALTE in a year. METHODS: In this prospective observational study, all of the neonates with episode of ALTE who were admitted to the Children's Medical Center (CMC) in Tehran, from June 15(th) 2010 to May 14(th) 2011 were enrolled in the study. Data from patients consisting of history, physical examinations, and paraclinical findings were recorded in a checklist and all followed up 3 to 6 months after discharge. FINDINGS: During the study period 18 neonates were admitted due to ALTE episode(s) with mean age of 15±13 days. Nine (50%) neonates had previous attacks of ALTE. The most frequent complaint was cyanosis in 12 (67%) and apnea in 8 (44%) patients. In 10 (56%) the event lasted less than one minute, 13 (72%) were awake, 17 (95%) in supine position and 13 (72%) on their parent's lap. Primary antagonistic impression on admission was sepsis in 11 (61%) and concomitant seizure in 5 (28%). The most common final diagnosis according to repeated physical examinations, result of paraclinical investigations and follow up was sepsis 4 (22%) and aspiration 9 (50%). ALTE recurred in none of the neonates during follow up. CONCLUSION: The rate of ALTE seems to be higher than in this study owing to high incidence of recurrent ALTE. Although most of these attacks regress spontaneously, more attention should be paid for the underlying diseases.

Clinical presentation of cystic fibrosis at the time of diagnosis: a multicenter study in a region without newborn screening.

BACKGROUND/AIMS: Cystic fibrosis is the most common inherited lethal disease, which could be frequently identified late in regions without newborn screening. There are dramatically better outcomes in the early diagnosis of cystic fibrosis patients. This study aimed to evaluate the spectrum of manifestations of cystic fibrosis at first admission leading to diagnosis. MATERIALS AND METHODS: This study was performed in a multi-referral pediatrics center in Iran. Data of patients with cystic fibrosis at the time of diagnosis were recorded based on a checklist denoting demographic characteristics, clinical and laboratory features. All of the patients had two documented sweat chloride tests. RESULTS: One hundred and ninety seven patients with cystic fibrosis were enrolled in this study. Among them, 119 patients (74%) were less than six months and 34 patients (21%) were between 6 and 12 months of age. The most common clinical findings were failure to thrive, recurrent pulmonary infections, and steatorrhea in 178 (90%), 139 (71%), and 135 (69%) patients, respectively. The most common radiologic abnormality was hyperaeration. In patients with salty tasting skin, steatorrhea, metabolic alkalosis, radiologic findings, and liver function abnormalities, the mean age at the time of diagnosis was significantly low than in the subjects without these findings. CONCLUSION: This study suggests that some conditions such as failure to thrive, recurrent respiratory infections, steatorrhea, metabolic alkalosis, and salty tasting skin should be considered as clinical screening tools for cystic fibrosis, especially in regions with high rate of cystic fibrosis. In these regions, awareness and clinical suspicion of medical professionals are crucial for early diagnosis of cystic fibrosis patients in the pre-diagnostic period.

The impact of preload alteration on the myocardial performance index through implementing positive end expiratory pressure.

Load independent methods should be used for the assessment of ventricular function. Debate still exists regarding whether tissue Doppler imaging (TDI) indices are influenced by preload. Here, we evaluated the effect of positive end expiratory pressure (PEEP) related preload reduction on both conventional pulsed Doppler (PD) and TDI myocardial performance index (MPI). Thirty-eight mechanically ventilated patients of 3 months to 12 years old (mean ± SD age of 30 ± 11 months) without overt heart disease were enrolled. Doppler mitral inflow velocities, isovolumetric contraction and relaxation times and aortic ejection time in addition to TDI peak systolic, early and late diastolic velocities from the basal segment of left ventricular lateral wall were determined for each patient before and after applying high PEEP (10 cmH(2) O).PD-MPI was load dependent (0.61 ± 0.22 vs. 0.78 ± 0.25, P = 0.002). However, TDI-MPI did not significantly change after the use of high PEEP declining the left ventricular volume loading (0.78 ± 0.21 vs. 0.84 ± 0.22, P = 0.23). Hence, regarding various interfering pathophysiologic factors particularly preload reduction, it seems that TDI-MPI would be a more reliable index for the assessment of ventricular function.