Rapidly progressing pediatric Lupus with Diffuse Alveolar Hemorrhage and CNS involvement.

Key Clinical Message: Systemic Lupus Erythematosus (SLE) can have an insidious onset and a fatal prognosis in children. Patients presenting without typical signs of SLE should undergo further evaluation if they are not responding to the initial diagnosis and treatment. This is especially true for patients with rapidly progressing symptoms and deterioration in spite of treatment. Abstract: Pediatric Systemic Lupus Erythematosus is a chronic autoimmune disorder with various organ involvement pulmonary involvement in the course of this disorder is a rare yet potentially life-threatening complication. In this case report we highlight the findings of a 16-year-old girl acutely and initially presenting with cough and fever, eventually complicating to diffuse alveolar hemorrhage and gradual loss of consciousness. Although the patient was started on immunosuppressive treatment after the diagnosis of lupus, based on renal and hematological impairment, was made and initially responded, she eventually deteriorated.

Cardiac Outcome of Children With SARS-CoV-2 Related Multisystem Inflammatory Syndrome.

OBJECTIVE: To study the cardiac outcomes of patients with multisystem inflammatory syndrome in children (MIS-C) after 6-month of diagnosis. METHODS: This review of hospital records was conducted on MIS-C patients (aged <21 year) who completed a six-month follow up. The baseline demographic, clinical, laboratory, and treatment characteristics during the acute phase, and echocardiographic findings during follow-up were collected. RESULTS: 116 patients (61.2% male, median age 7 years) with MIS-C were included in the study. At the time of admission, cardiac abnormalities were present in 70.7% of MIS-C patients, and the most common cardiac abnormalities were valve failure (50.9%), followed by ventricular dysfunction (39.7%), and pericardial effusion (23.3%). Six month after diagnosis, cardiac abnormalities were found in 10.3% of patients, and patients had lower rates of ventricular dysfunction (P<0.001), valve failure (P<0.001), pericardial effusion (P<0.001), and coronary involvement (P<0.001) as composed to the baseline. Intravenous immunoglobulin (IVIG) and steroid treatment significantly reduced the odds of occurrence of ventricular dysfunction (P=0.002), valve failure (P=0.004), and low ejection fraction (P=0.002) in comparison to IVIG treatment. CONCLUSION: While most MIS-C patients had abnormal echocardiographic findings at admission, only 10.3% of patients had cardiac abnormalities during follow up.

Coronavirus disease 2019-related Kawasaki syndrome: a case report.

BACKGROUND: Coronavirus disease 2019 has changed the pattern of some diseases in the world, especially in pediatrics. Despite data suggesting that the pediatric population is less affected by coronavirus disease-19 infection, new concerns have been raised owing to reported cases with hyperinflammatory conditions such as Kawasaki disease. CASE PRESENTATION: We report herein the case of a pediatric patient diagnosed and treated for classic Kawasaki disease in the setting of confirmed coronavirus disease 2019 infection. She was an 8-year-old, previously healthy, and fully immunized Iranian girl who initially presented to the pediatric emergency department with 5 days of intermittent fever, followed by abdominal pain, nausea, and vomiting. She was admitted for fever and abdominal pain to the surgery service of Akbar Hospital with suspected appendicitis. CONCLUSIONS: This case report may serve as a useful reference to other clinicians caring for pediatric patients affected by coronavirus disease 2019 infection. Standard therapeutic interventions for Kawasaki disease must be performed to prevent critical coronary aneurysm-related complications in the coronavirus disease 2019 era.

Bilateral expansion of mandible and maxillae in a 5-year-old girl.

A 5-year-old girl with symmetrical expansion of mandible and both maxillae, lack of eruption of several teeth an hypertrophied palate was presented to our clinic. upon detailed laboratory, imaging and histopathological evaluation, the diagnosis of "Cherubism" a rare autoinflammatory bone disease was confirmed.

The Negative Predictive Value of Harada Scoring for Coronary Artery Dilatation or Aneurysm in Children with Kawasaki Disease: A Cross-sectional Study.

Background: The most common cause of coronary artery aneurysms in children is Kawasaki disease (KD). One of the risk ratings used to predict coronary artery aneurysms is the Harada score. This study aims to assess the negative predictive value (NPV) of Harada scoring in children with KD. Methods: This cross-sectional study was conducted in Mashhad (Iran) from January 2014 to December 2017. The Harada score was generated for each patient using demographic, laboratory, and echocardiography data retrieved from their medical records. The patients were divided into typical and atypical cases, and the sensitivity, specificity, positive predictive value (PPV), and NPV were calculated. The Chi square test, independent t test, Mann-Whitney U test, and Fisher exact test were used to analyze the data in SPSS software (version 23.0). P≤0.05 was considered statistically significant. Results: The study involved 168 individuals with a mean age of 29.91±19.52 months, including 103 typical and 65 atypical cases. Regarding cardiac complications, the atypical group had a significantly greater rate of coronary artery tapering (P=0.030). Out of 101 patients with cardiac complications, the Harada score was positive in 57 cases, while it was falsely negative in the remaining 44 patients. The calculated sensitivity, specificity, PPV, and NPV were 56.44%, 53.73%, 64.77%, and 45%, respectively. Conclusion: The findings revealed that the Harada scoring is ineffective in detecting coronary artery aneurysms or dilation in KD patients.

Cardiac Tamponade: A Rare Manifestation of Familial Mediterranean Fever.

Familial Mediterranean fever (FMF) typically presents with recurrent attacks of fever and serosal inflammation with peritoneum, pleura, and synovium. We usually do not expect pericardial involvement at the early stages. FMF is an autoinflammatory disease, usually inherited with an autosomal recessive pattern. The patients typically have biallelic mutations in the MEFV gene, located on chromosome 16. Colchicine is the first-line treatment of FMF, which not only plays a crucial prophylactic role regarding the attack episodes, but also prevents amyloidosis. Colchicine resistance and intolerance in FMF patients have been rarely reported. Alternative anti-inflammatory agents are understood to be helpful in such cases. We describe a 13-year-old boy referred to our pediatric department complaining of chest pain, dyspnea, and tachycardia. Due to the massive pericardial and pleural effusion, a pericardiocentesis was performed, and a chest tube was inserted. Cardiac tamponade was considered as the initial diagnosis. After a month, he faced another episode of pleuritic chest pain, fever, tachycardia, and pleural and pericardial effusion. Evaluation for probable differential diagnoses including infection, malignancy, and collagen vascular disease showed no remarkable results. Finally, the mutation found by whole exome sequencing was confirmed by direct Sanger sequencing revealing a heterozygote c.44G > C (p.Glu148Gln) mutation in exon 2, confirming the clinical diagnosis of familial Mediterranean fever. Since he seemed to be nonresponsive to the maximum standard dose of colchicine, 100 mg of daily dapsone was added to his treatment regimen, which controlled the attack episodes well. FMF, while rarely initiated with cardiac manifestation, should be considered in patients with any early signs and symptoms of cardiovascular involvement.

COVID-19 Associated Multisystem Inflammatory Syndrome: A Systematic Review and Meta-analysis.

The prevalence of multisystem inflammatory syndrome in children (MIS-C) has increased since the coronavirus disease 2019 (COVID-19) pandemic started. This study was aimed to describe clinical manifestation and outcomes of MIS-C associated with COVID-19. This systematic review and meta-analysis were conducted on all available literature until July 3rd, 2020. The screening was done by using the following keywords: ("novel coronavirus" Or COVID-19 or severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) or coronavirus) and ("MIS-C" or "multisystem inflammatory" or Kawasaki). Data on gender, ethnicity, clinical presentations, need for mechanical ventilation or admission to intensive care unit (ICU), imaging, cardiac complications, and COVID-19 laboratory results were extracted to measure the pooled estimates. Out of 314 found articles, 16 articles with a total of 600 patients were included in the study, the most common presentation was fever (97%), followed by gastrointestinal symptoms (80%), and skin rashes (60%) as well as shock (55%), conjunctivitis (54%), and respiratory symptoms (39%). Less common presentations were neurologic problems (33%), and skin desquamation (30%), MIS-C was slightly more prevalent in males (53.7%) compared to females (46.3%). The findings of this meta-analysis on current evidence found that the common clinical presentations of COVID-19 associated MIS-C include a combination of fever and mucocutaneous involvements, similar to atypical Kawasaki disease, and multiple organ dysfunction. Due to the relatively higher morbidity and mortality rate, it is very important to diagnose this condition promptly.

Comparison of Clinical Symptoms and Cardiac Lesions in Children with Typical and Atypical Kawasaki Disease.

The present study was performed to evaluate the clinical symptoms and cardiovascular complications in patients with typical and atypical Kawasaki disease (KD). This retrospective study was conducted on the medical records of 176 patients with KD for three years. The study population was divided into two groups of typical and atypical based on the KD clinical criteria. The two groups were compared in terms of demographic data, clinical symptoms, cardiac lesions, and laboratory markers. Based on the diagnostic criteria, 105 (60%) and 71 (40%) patients were diagnosed with typical and atypical KD, respectively. The mean age of the typical patients (38.16 months) was higher than that of the atypical group (24.03 months) at the time of diagnosis (p < 0.05). The results revealed no significant difference between the two groups regarding the seasonal distribution of KD onset (p = 0.422). However, the most common season for the diagnosis of the disease was spring, followed by winter. There was no significant difference between the two groups in terms of fever duration (p = 0.39). Furthermore, vomiting was more common in the atypical patients than in the typical group (p = 0.017). In terms of the cardiac lesions, ectasia (p = 0.005) and lack of tapering of the distal coronary vessels (p = 0.015) were more frequently detected in the atypical group than in the typical group. Considering the laboratory findings, thrombocytosis (p = 0.010) and anemia (p = 0.048) were more common in the atypical group, compared to those in the typical group. On the other hand, the typical group had a higher serum alanine aminotransferase level (adjusted for age) (p = 0.012) and Hyponatremia (serum sodium concentration ≤130 mmol/L) (p = 0.034). Based on the findings of the current study, the fever duration from onset to diagnosis was slightly more in atypical KD patients than in the typical group, but not statistically significant, possibly due to more timely diagnosis of atypical KD. There was no difference in coronary aneurysm between the two groups at the time of diagnosis. The atypical group had a higher frequency of coronary ectasia and lack of tapering, indicating cardiac involvement. Consequently, these conditions should be given more attention in the atypical patients. Furthermore, the higher frequency of anemia and thrombocytosis in the atypical patients can be useful for diagnosis of this kind of KD.

Protective role of HLA-DRB1*11 against juvenile idiopathic arthritis living in North Eastern Iran.

OBJECTIVES: Juvenile idiopathic arthritis (JIA) is one of the most common chronic rheumatic diseases in children. The complex nature of this immune-mediated disease owes itself to several predisposing genes and environmental factors affecting its pathogenesis. Conducted in Iran, this study was originally intended to investigate every possible association between HLA DRB1 alleles and a susceptibility to JIA. MATERIALS AND METHODS: In this case-control study, 45 patients with a definite diagnosis of JIA based on International League against Rheumatism (ILAR) criteria were compared against 46 healthy controls. DNA samples taken from both groups were analyzed using PCR-sequence specific primers (PCR-SSP) method. Data analysis including parametric and nonparametric test and multivariate analysis was undertaken using the SPSS 11.5 software. A P-value< 0.05 was regarded as statistically significant. RESULTS: Mean ages in case group and healthy controls were 14.64±6.21 and 13.73±6.39, respectively with no significant difference between the two groups (P=0.515). Sex difference between JIA group and healthy controls was also not significant (P=0.068). The frequency of HLA-DRB1*01 was found the most frequent HLA-RB1 in our patients (33.3%). No significant statistical correlation between various HLA-DRB1 alleles and clinical subtypes of the disease could be established from the data. HLA-DRB1*11 was shown to raise protection to JIA (P=0.035, OR=2.755, 95% CI=0.963-8.055) in northeastern Iran. In addition, we found that HLA-RB1*09 is nominally associated with an increased risk of JIA (P=0.56, OR=2, 05, 95% CI=0.18-23.63). CONCLUSION: HLA-DRB1*11 was shown to raise protection to JIA in northeastern Iran. The disparity of findings in other ethnicities prompts further investigations with larger sample sizes.

Hip Surgery in Quiescent or Active Tubercular Hip Arthritis; Is Reactivation Risk Really a Matter.

About 15 % of all musculoskeletal tuberculosis (TB) have hip involvement. Early anti-tubercular drug therapy and other conservative treatment including short-term traction and mobilization programs could prevent hip joint destruction. Reactivation of TB accounts for a significant of active TB incidence, especially in the developed countries with a low TB prevalence. The risk of TB activation for population with the latent form of disease is about 5-10%. According to the existing literature surgery in tubercular hip arthritis would be safe once sufficient debridement and precise anti tubercular chemotherapy has been done.

Chronic Recurrent Multifocal Osteomyelitis in a 9-year-old Boy.

Chronic recurrent multifocal osteomyelitis (CRMO) is a rare aseptic, auto-inflammatory bone disorder. CRMO presents with bone pain with or without fever. The diagnosis of CRMO is a diagnosis of exclusion and should be included in the differential diagnosis of chronic inflammatory bone lesions in children. Cultures of the bone are typically sterile, antibiotic therapy does not result in clinical improvement whereas anti-inflammatory drugs improve the condition. Furthermore, biopsy should be considered in chronic and relapsing bone pain and swelling unresponsive to treatment. Herein, we present a nine-year-old boy complaining of recurrent pain in his upper and lower extremities. On examination he had mild fever and cervical lymphadenopathy. He also had experienced bone pain and weight loss in the recent month. Based on biopsy and bone scan he was finally diagnosed with CRMO. Naproxen and Pamidronate was prescribed and he was getting better and returned to normal life and activity without need to corticosteroids.

Clinical course and outcomes of Iranian children with juvenile dermatomyositis and polymyositis.

This study evaluated the clinical features, course, and outcomes of Iranian children with juvenile dermatomyositis (JDM), juvenile polymyositis (JPM), and other uncommon connective tissue disorders. A chart review of 85 Iranian children with JDM and JPM was performed during a 10-year period from 2003 to 2013. The patients' clinical signs and symptoms, laboratory data, and other factors affecting clinical outcomes were recorded using questionnaires. Statistical analysis was performed using SPSS software version 20. In all, 40 boys and 45 girls were included in the study (F/M, 1.1:1). Disease frequency was significantly higher in boys aged <5 years (F/M, 0.4:1) and girls aged >5 years (F/M, 1.6:1). The combined mean age at diagnosis was 7.5 years. Muscle weakness, particularly in the proximal muscles of lower extremities (96 %); fatigue (83 %); and heliotrope rash (71 %) were the most frequently recorded symptoms. Elevated lactate dehydrogenase level was the most common enzyme disturbance (98 %). Monocyclic course was seen in 60 % of patients. The mean treatment duration was 3 years. The incidence rate of complications such as calcinosis, lipodystrophy, and growth disturbances was 20, 9, and 30 %, respectively. The occurrence of these complications in patients with monocyclic disease was significantly lower. Vital organ involvement led to the death of four patients. The incidence of calcinosis was significantly lower in patients having a shorter interval between disease onset and treatment. Two important complications, failure to thrive and lipodystrophy, were significantly higher in patients having antinuclear antibodies. The incidence of the above three complications was higher in patients with polycyclic or continuous chronic disease. Respiratory failure was the most common cause of patient mortality.

Infantile Systemic Hyalinosis: Report of 17-year Experience.

BACKGROUND: Infantile Systemic Hyalinosis (ISH) is a very rare autosomal recessive disorder characterized by connective tissue involvement as hyaline deposition in skin, gastrointestinal tract, muscles, glands and other organs. CASES PRESENTATION: We report eight Iranian children (4 male and 4 female) with ISH referred to our hospital from 1996 to 2013. The illness had been diagnosed by clinical manifestations and disease progression. Six of them died and two are alive but very sick. CONCLUSION: ISH is a very rare disorder with poor prognosis. Seventy five percent of our 8 patients died before 2 years old due to severe diarrhea, malabsorption and/or infection.

Cervical gland area: a new sonographic marker in predicting preterm delivery.

PURPOSE: Preterm delivery is one of the important causes of morbidity and mortality in newborns. Nowadays, a new sonographic marker is suggested for prediction of preterm delivery which is termed "Cervical Gland Area" (CGA). Absence of normal mucosal glands of the cervix can be utilized as a predictor in preterm delivery. This study was performed to evaluate the role of absence of CGA for the prediction of preterm delivery <37 weeks. METHODS: Trans-vaginal ultrasonography was performed on 600 pregnant women at 16-19 weeks of gestational age. Cervical gland area and cervical length were evaluated. Women with cervical length <25 mm were conducted to cerclage and excluded from the study. Age, number of pregnancies and history of previous preterm deliveries were recorded in questionnaires. The date of delivery was recorded. Statistical analysis was performed by version 18 SPSS and P value <0.05 was significant. RESULTS: Spontaneous preterm delivery was seen in 6.7% of all women. There was no significant difference in age, cervical length, the number of pregnancies and history of previous preterm deliveries between term pregnancies and preterm cases. CGA was detected in 77.1% of term pregnancies comparing with 55% in preterm group; which was significantly different (P value = 0.002). CONCLUSIONS: Non detection of CGA at second trimester ultrasonography is a predictor of preterm delivery.

Pneumothorax after mechanical ventilation in newborns.

OBJECTIVE: Air leak syndromes including pneumothorax, pneumomediastinum and pulmonary interstitial emphysema are frequent in neonatal period. Mechanical ventilation with positive pressure is one of the most common causes of these syndromes. The aim of this study was to evaluate predisposing factors and incidence of pneumothorax in newborns under mechanical ventilation. METHODS: This descriptive cross sectional study was performed in 400 newborns under mechanical ventilation in intensive care unit of a teaching hospital in Iran from April 2004 to December 2008. Predisposing factors leading to ventilation and incidence of air leak syndromes were studied. Sex, gestational age, birth weight, type of delivery, history of surfactant replacement therapy, ventilator settings and mortality rate were recorded. Statistical analysis was done using SPSS software. Univariate analysis and regression analysis were considered. FINDINGS: Among 400 patients under mechanical ventilation, 102 neonates developed pneumothorax (26%). Fifty six (54.9%) of them were boys and 46 (45.1%) girls. 54.9% of newborns with pneumothorax were preterm and 45.1% term. Birth weight less than 2500g was recorded in 59.8%. Fifty two percent of these neonates were born by cesarean section vs. 32% of newborns without pneumothorax. The most common type (62.7%) of ventilation leading to pneumothorax was Inspiratory Positive Pressure Ventilation (IPPV). Surfactant replacement therapy was recorded in 32.4% of cases with pneumothorax compared to 60.4% of neonates under ventilation without pneumothorax, which was significantly different (P= 0.017). CONCLUSION: In newborns surfactant replacement therapy can reduce the risk of pneumothorax caused by mechanical ventilation.

Cervicofacial emphysema and pneumomediastinum following dental extraction: case report.

BACKGROUND: Pneumomediastinum is commonly caused by iatrogenic injury such as surgery on the cervical planes and chest or by tracheostomy. It is also well known that emphysema may occur after dental treatments using an air turbine drill, but there have been few cases of emphysema which extended to the mediastinum. CASE PRESENTATION: A 16-year-old boy is presented with subcutaneous emphysema and pneumomediastinum which developed 24 hours after surgery for extraction of an inferior second molar. We first describe history, clinical presentation and radiologic appearance of our patient and then review the literature about oral surgery causing pneumomediastinum. CONCLUSION: Surgical procedures in oral cavity can lead to the development of emphysema and pneumomediastinum when air turbine dental drills are used. To avoid these complications, air turbine high speed drills should be used only in necessary cases.