Giant Coronary Aneurysms with Multiple Large Resistant Thromboses in an 8-Month-Old Boy with IVIg-Resistant Kawasaki Disease: A Case Report.

Kawasaki disease is an acute self-limiting systemic vasculitis in childhood, resulting in arterial swelling or inflammation and eventually leading to cardiovascular problems, such as coronary artery aneurysms. Based on previous studies, serum sodium ≤133 mmol/L, albumin ≤3.2 g/dL, alanine transaminase ≥80 U/L, and neutrophil percentage ≥80% at diagnosis are risk factors for intravenous immunoglobulin (IVIg). However, the prevalence of resistance to Ig among children with Kawasaki disease varies among different countries due to diversity in evaluation, treatment, and diagnosis. Approximately, 10% to 20% of patients have IVIg-resistant Kawasaki disease. As the probability of coronary artery damage associated with IVIg-resistant Kawasaki disease is higher than that with IVIg-sensitive Kawasaki disease, the early detection and appropriate treatment of IVIg-resistant Kawasaki disease can decrease the probability of damage to coronary arteries and hospital lengths of stay and cost. Kawasaki disease in early infancy is uncommon, and sometimes it occurs with thrombosis and peripheral gangrene. A positive genetic background may play a role in susceptibility to thrombosis. We herein describe a patient suffering from an IVIg-resistant Kawasaki disease with severe coronary artery thrombosis and positive genetic mutation. Medical treatment resolved the thrombosis, but the coronary arteries remained dilated.

Methylprednisolone pulses as an initial treatment in hyperinflammatory syndrome after COVID-19 in children: evaluation of laboratory data, serial echocardiography and outcome: a case series.

BACKGROUND: Hyper-inflammatory syndrome in children and young adult occur 2-6 weeks after COVID-19 infection or closed contact with COVID-19 persons. In this study, the laboratory data and echocardiography and abdominal ultrasonography assessments were evaluated before and after Methylprednisolone pulse as an initial treatment of hyper-inflammatory syndrome. Therefore, the aim of this study is to assessment the clinical manifestations and laboratory data and outcome after methylprednisolone pulse as an initial treatment. METHOD: In this retrospective study, the demographic status, clinical features, laboratory data, echocardiography, abdominal ultrasound, treatment and outcome of 31 pediatric patients under 16 years old, with inflammatory process after COVID-19 were evaluated. The clinical assessments, laboratory data, sonography and echocardiography were evaluated before and after methylprednisolone pulse. The patients were divided in two age group < and ≥ 7 years old and the clinical manifestations were compared with each other. The Mann-Whitney U test was used to assess the difference in quantitative variables between two groups. To compare pre- and post- treatment values, Wilcoxol test was used. To assess the correlation between qualitative variables chi-square test was used. The level of significant was considered 0.05. These patients with fever and hyper-inflammation features admitted to the referral pediatric rheumatology ward in Children Medical Center of Tehran University of medical sciences, from April 2020 to May 2021 were assessed. RESULT: The mean age ± SD were (5.94 ± 3) and 51.6% (16) patients were male and 48.4% (15) patients were female. The most documented of previous COVID infection were antibody positive in about 27 (87%) patients. Moreover, 1 (3.8%) was PCR positive, 2 (7.7%) were positive in both PCR and serology and 3(11.5%) had closed contact with COVID-19 patients. About 9(29%) of patients were admitted in Intensive Care Unit (ICU). There were significant correlation between days of delay in starting treatment and ICU admission (P-value = 0.02). The mortality rate was negative in patients and no re-hospitalization was documented. There were significant differences (P-value < 0.05) between lymphocytes, platelet, Erythrocyte Sedimentation rate, C-reactive protein, Aspartate transaminase, Alanine transaminase and ferritin before and after treatment. Skin rashes and cardiac involvement totally as carditis (myocarditis, vulvulitis and pericarditis) (33.3%) and coronary involvements (53.3%) were the most prominent initial presentation in patients. There were near significant correlation (P-value = 0.066) between ferritin level and carditis before treatment. Cervical lymphadenopathy was seen significantly more in ≥ 7 years old (P-value = 0.01). CONCLUSION: Multisystem inflammatory system in children as a hyperinflammatory syndrome could be treated with first step methylprednisolone pulse with decreasing inflammation in laboratory data and cardiac involvements and good outcome. Furthermore, the ferritin level may be one of the predictor of severe hyper-inflammatory syndrome leading to aggressive and urgent treatment with methylprednisolone pulse.

Post-Marketing Surveillance of a generic Oxaliplatin (AlvoxalⓇ) in Iranian Patients with Cancer.

BACKGROUND: CRC is the second and third most common cancer in women and men, respectively. The national comprehensive cancer network guidelines recommend oxaliplatin-based chemotherapy as a preferred regimen for patients with advanced or metastatic colon cancer. Oxaliplatin is also used in the off-label treatment of gastric cancer. FDA uses post-marketing study commitments to gather additional information about a product's safety, efficacy, or optimal use. It is necessary to perform safety monitoring after marketing authorization is received; such monitoring can be done by means of characterizing the safety of drugs in patients being treated in real-world clinical practice settings. OBJECTIVES: This Phase IV study aimed to evaluate the safety profile of a brand-name formulation of the generic drug oxaliplatin (AlvoxalⓇ, NanoAlvand, Tehran, Iran) in Iranian patients diagnosed with either colorectal or other, different types of cancer. METHODS: Patients with colorectal cancer, gastric cancer, or other malignancies receiving oxaliplatin as a part of their treatment were included in this open-label, multicenter, observational Phase IV study. This study aimed to assess the safety profile of oxaliplatin in patients diagnosed with different cancers. FINDINGS: A total of 483 patients from 16 cities in Iran were enrolled. The most common malignancy was colorectal cancer (55.49%), followed by gastric cancer (28.16%). Based on the results, 405 patients experienced at least 1 adverse event. Most of these adverse events were grade 1 or 2, and the most commonly reported adverse event was anemia (60.66%). During the study, 26 serious adverse events occurred in 15 (3.11%) patients, which were perhaps related to oxaliplatin. There were no remarkable differences in the incidences of adverse events in the system organ classes of blood and lymphatic system disorders, gastrointestinal disorders, or nervous system disorders among patients with different malignancies (ie, colorectal cancer, gastric cancer, and other malignancies) or between genders. The results of this open-label, multicenter, observational, postmarketing surveillance study demonstrated no unexpected safety findings from the use of this oxaliplatin product (AlvoxalⓇ) in Iranian patients diagnosed with different types of cancer. CONCLUSIONS: This Phase IV study provides data on the safety profile of a number of chemotherapy regimens containing an oxaliplatin product given to Iranian patients diagnosed with different types of cancer.

Little Hearts Are Affected by COVID19: Importance of the Myocardial Systolic Evaluation.

Background: Identifying the cardiac changes could help design measures to recover the cardiovascular system and lessen the mortality and morbidity rate. Accordingly, this cross-sectional study was performed to evaluate the echocardiography indices which are indicators of the cardiac alterations of the children with COVID19 infection. Methods: This study was performed as a cross-sectional study evaluating echocardiography indices in children infected with COVID19. Fifteen children, known cases of the COVID19, and 14 healthy children were enrolled. Evaluated parameters include left ventricle ejection fraction (LVEF), left ventricle end-diastolic diameter (LVED), mitral valve Sa (MV Sa), Tricuspid annular plane systolic excursion (TAPSE), and laboratory parameters. Results: The participants' mean age and weight were 62.8 (±48.0) months and 19.95 (±15.67) kg, respectively. None of the laboratory and echocardiography parameters differed between males and females, between patients with and without positive past medical history, between the patients with and without respiratory tract symptoms, and between patients with and without GI tract symptoms (P.0.05). Patients had significantly higher TAPSE (p = 0.027), although MV Sa (p = 0.01) was significantly higher among healthy children. LV EF (p = 0.425) and LVED diameter (p = 0.603) were not different significantly. None of the patients had pericardial effusion, pleural effusion, and cardiac tamponade. Conclusion: The heart can be involved during the disease course in children, even at the level of echocardiography indices. This could contribute to a worse prognosis, higher morbidity, and mortality rate, especially in patients with overt myocardial involvement. Non-classic indicators, including LVEF, may not be conclusive for cardiac involvement in non-symptomatic patients.

Giant intracardiac thrombosis in an infant with leukaemia and prolonged COVID-19 viral RNA shedding: a case report.

BACKGROUND: COVID-19 can induce thrombotic disease both in the venous and arterial circulations, as a result of inflammation, platelet activation, endothelial dysfunction, and stasis. Although several studies have described the coagulation abnormalities and thrombosis in adult patients with COVID-19, there is limited data in children. Here, we present an 18-month-old boy with a prolonged SARS-CoV-2 RNA shedding and chronic right atrial and superior vena cava (SVC) thrombosis. CASE PRESENTATION: An 18-month-old boy with acute lymphoblastic leukemia (ALL) (pre-B cell ALL) and a history of chemotherapy was referred to our center due to intermittent fever with unknown origin. a positive nasopharyngeal PCR for COVID-19 was reported and stayed positive for eight consecutive weeks The high-resolution computed tomography (HRCT) showed no sign of pulmonary embolism. Initial echocardiography indicated a semilunar thrombotic mass extending from right SVC into the right atrium without coronary or myocardial involvement. Enoxaparin was administered with continuous monitoring of the level of anti-Xa activity. The serial echocardiographic studies found a slow but continuous reduction in the mass size. CONCLUSIONS: Our case shows that, as already described in adult patients, clinically relevant thrombosis can complicate the course of pediatric patients as well. In view of the specific and milder manifestations of COVID-19 in children, these complications may pose considerable diagnostic and therapeutic challenges.

Mitochondrial Polymorphisms, in The D-Loop Area, Are Associated with Brain Tumors.

OBJECTIVE: This study was carried out to evaluate the relationship between mtDNA D-loop variations and the pathogenesis of a brain tumor. MATERIALS AND METHODS: In this experimental study, 25 specimens of brain tumor tissue with their adjacent tissues from patients and 454 blood samples from different ethnic groups of the Iranian population, as the control group, were analysed by the polymerase chain reaction (PCR)-sequencing method. RESULTS: Thirty-six variations of the D-loop area were observed in brain tumor tissues as well as the adjacent normal tissues. A significant difference of A750G (P=0.046), T15936C (P=0.013), C15884G (P=0.013), C16069T (P=0.049), T16126C (P=0.006), C16186T (P=0.022), T16189C (P=0.041), C16193T (P=0.045), C16223T (P=0.001), T16224C (P=0.013), C16234T (P=0.013), G16274A (P=0.009), T16311C (P=0.038), C16327T (P=0.045), C16355T (P=0.003), T16362C (P=0.006), G16384A (P=0.042), G16392A (P=0.013), G16394A (P=0.013), and G16477A (P=0.013) variants was found between the patients and the controls. CONCLUSION: The results indicated individuals with C16069T [odds ratio (OR): 2.048], T16126C (OR: 2.226), C16186T (OR: 3.586), G16274A (OR: 4.831), C16355T (OR: 7.322), and T16362C (OR: 6.682) variants with an OR more than one are probably associated with a brain tumor. However, given the multifactorial nature of cancer, more investigation needs to be done to confirm this association.

Pre-operative laparoscopic staging of gastric cancer in patients who are candidates for neo-adjuvant chemotherapy: A Cross Sectional Study.

Objective The aim of this study is to evaluate the laparoscopic method in gastric cancer staging before surgery in patients undergoing Neo-adjuvant chemotherapy. Methods This was a cross-sectional study on patients with T3-T4 and node positive non-metastatic gastric adenocarcinoma who was candidates for neo-adjuvant chemotherapy. The patients were excluded from the study when they were not in the condition to continue the study. All eligible patients underwent metastases work up by abdominal, pelvic and chest CT scans and then received neo-adjuvant chemotherapy. Pre-operative laparoscopy was performed before definitive surgery. The collected data was analyzed using SPSS software. The statistical significance, sensitivity, specificity and predictive values were calculated from this data. Results In this study, out of 50 patients, 26 (52%) were male. The mean age of the patients was 62.44 ± 12.136 years. Most tumors were located in one-third distal of stomach (21 patients, 42%). The accuracy of the laparoscopic method, to determine the degree of involvement of the lymph nodes, revealed that the method had a sensitivity of 84%, a specificity of 47.62%, a positive likelihood ratio (PLR) of 1.6, a negative likelihood ratio (NLR) of 0.33%, a positive predictive value of 65.62% and a negative predictive value of 71.43%. The accuracy of the laparoscopic method, regarding determining the presence or absence of metastasis, revealed that the method had a sensitivity, specificity, positive predictive value, and a negative predictive value of 100%. Conclusion Based on the evidence of this study, and other studies in this field, it seems that the use of a step-by-step combination methods in gastric cancer staging would be logical. Alone, no singular method would be sufficient in providing the physician with sufficient information to successfully stage the tumor and thus determine the appropriate treatment. Therefore, the combination of methods should be used which, in view of the economic constraints of health systems, would be recommended for high-risk patients for metastasis (N+/ T3-T4), diagnostic pre-operative laparoscopy.

Explaining Postdischarge Care Needs of Cancer Patients: A Qualitative Study.

AIM: This study aimed to explain the postdischarge care needs of cancer patients. MATERIALS AND METHODS: In this qualitative content analysis, nine cancer patients presenting to the department of chemotherapy or an oncologist's office, four of patients' accompaniments, four nurses, and two oncologists were selected purposefully until data saturation. They underwent deep individual interviews. Data were collected from October 2016 to June 2017. Data were analyzed using Zhang's method with an inductive approach to extract the themes. RESULTS: Data analysis led to the formation of two main categories of healing and relaxation needs (with subcategories of "respect and friendship," "trust and confidence," "relief," "patience and tolerance," "empowerment," and "protection from tension and excessive pressure") and monitoring and balancing conditions (with subcategories of "care monitoring" and "protection"). CONCLUSIONS: Postdischarge needs are a set of needs for primary exposure to diagnosis, outpatient attendance at the hospital and treatment centers, access to the treatment team, family readiness, and care systems and community needs for acceptance and care of these patients. The discharge plan should be designed in accordance with the needs set out above, so that the patients can meet the needs of the acute and disease encounter phase and also can manage the emerging postdischarge needs, such as empowerment.

Prevalence of Abelson murine leukemia viral oncogene homolog-breakpoint cluster region fusions and correlation with peripheral blood parameters in chronic myelogenous leukemia patients in Lorestan Province, Iran.

CONTEXT: Chronic myelogenous leukemia (CML) is a chronic malignancy of myeloid linage associated with a significant increase in granulocytes in bone marrow and peripheral blood. CML diagnosis is based on detection of Philadelphia chromosome and "Abelson murine leukemia viral oncogene homolog" (ABL)-"breakpoint cluster region protein" fusions (ABL-BCR fusions). AIMS: In this study, patients with CML morphology were studied according to ABL-BCR fusions and the relationship between the fusions and peripheral blood cell changes was examined. MATERIALS AND METHODS: All patients suspected to chronic myeloproliferative disorders in Lorestan Province visiting subspecialist hematology clinics who were confirmed by oncologist were studied over a period of 5 years. After completing basic data questionnaire, blood samples were obtained with informed consent from the patients. Blood cell count and morphology were investigated and RNA was extracted from blood samples. cDNA was synthesized from RNA and ABL-BCR fusions including b3a2 and b2a2 (protein 210 kd or p210), e1a2 (protein 190 kdor p190), and e19a2 (protein 230 kdor p230) were studied by multiplex reverse transcription polymerase chain reaction method. Coexistence of e1a2 and b2a2 (p210/p190) fusions was also studied. The prevalence of mutations and their correlation with the blood parameters were statistically analyzed. RESULTS: Of 58 patients positive for ABL-BCR fusion, 18 (30.5%) had b2a2 fusion, 37 (62.71%) had b3a2 fusion and three (3.08%) had e1a2 fusion. Coexistence of e1a2 and b2a2 (p210/p190) was not observed. There was no significant correlation between ABL-BCR fusions and white blood cell count, platelet count, and hemoglobin concentration. CONCLUSIONS: The ABL-BCR fusions in Lorestan Province were similar to other studies in Iran, and b3a2 fusion had the highest prevalence in the studied patients studied.

A 15-Month-Old Boy With Respiratory Distress and Parapharyngeal Abscess: A Case Report.

Parapharyngeal abscess is a life-threatening disease. Upper respiratory tract infection is the main cause in children. We present a 15-month-old boy admitted to the emergency ward with the chief complaint of difficulty in breathing caused by parapharyngealabscess. His condition deteriorated gradually, and he transferred to the operation theater quickly for abscess drainage and because of the difficulty in orotracheal intubation; a tracheostomy was performed. His respiratory condition deteriorated 2 days after PICU admission, and the medical team noticed an unexplainable respiratory distress. A chest x ray obtained and showed a right side pneumothorax and subcutaneous emphysema around theneck area. The case presented here, had not been diagnosed at the first examination; however, there were enough clinical clues (such as respiratory distress, drooling, torticollis, bulging of theneck, previous viral respiratory infection, possible pharyngeal trauma). The story of this case reminds us the importance of the precise physical exam and history taking which could be life-saving.