Epidemiological and clinical risk factors related to severe COVID-19 in Iran: a multi-center study.

BACKGROUND: Iran was one of the first countries to be affected by COVID-19. Identifying factors associated with the severity of COVID-19 is effective in disease management. This study investigated the epidemiological and clinical features and factors related to the severity of COVID-19 in one of the less privileged areas in Iran. METHODS: In a multi-center study, all patients admitted to Zahedan University of Medical Sciences hospitals in southeastern Iran were investigated from February 29 to April 31, 2020. Demographic, epidemiological, and clinical data of patients were extracted from medical records. Bivariate and multivariate logistic regression models were used to explore the risk factors associated with the severity of COVID-19. RESULTS: Among the 413 patients, 55.5% were male, and 145 (35.10%) were in a severe condition at admission time. Multivariate analysis showed that the adjusted odds of the disease severity increased in patients with older age (OR 2.27; 95% CI 1.41-3.65), substance abuse (OR 2.49; 95% CI 1.14-5.43), having one underlying disease (OR 1.52; 95% CI 0.90-2.55), having two underlying disease (OR 2.31; 95% CI 1.19-4.50), and having three or more underlying disease (OR 2.60; 95% CI 1.19-5.66). CONCLUSIONS: COVID-19 was more severe in older patients, patients with a history of substance abuse, and patients with the underlying disease. Understanding the factors affecting the disease severity can help the clinical management of COVID-19, especially in less privileged areas where fewer resources are available.

A retrospective study on clinical manifestations of neonates with FXIII-A deficiency.

We assessed clinical presentations and the rate of central nervous system (CNS) bleeding in neonates with FXIIID who exhibited bleeding diathesis in the early days of their lives. A total of 27 neonates presented bleeding or abnormal clinical symptoms, diagnosed with FXIII deficiency were evaluated. Factor XIII concentrate was initiated as the first-line of treatment, and prophylactic therapy was given to all patients. Umbilical cord bleeding, delayed detachment of umbilical stunt, seizure, hematoma, and ecchymosis were concurrent complications in 27 (100%), 5 (18.5%), 5 (18.5%), 3 (11.1%), and 1 (3.7%) of the patients, respectively. History of having CNS bleeding was detected in 13 (48.1%) patients. There was no significant association between CNS bleeding and gender, familial history of FXIIID, or other clinical presentations. Also, there was no significant difference in the mean age of the patients who had CNS bleeding (3.4 ±â€¯0.9 days) and without CNS bleeding (2.9 ±â€¯0.7 days). However, a near significant threshold difference between the patients with and without CNS bleeding was found regarding the mean number of suspicious FXIIID death in their family (1.8 ±â€¯0.5 and 0.7 ±â€¯0.1, respectively, P = 0.05). Therefore, a suggested diagnostic algorithm based on prenatal diagnosis could be useful for timely detection of FXIII deficiency in neonates.

Correlation of bleeding score with frequency and severity of bleeding symptoms in FXIII deficiency assessing by the ISTH Bleeding Assessment Tool.

OBJECTIVES: The ISTH bleeding assessment tool (ISTH-BAT) is developed for standardization of bleeding symptoms in bleeding disorders. The aim of this study is to apply this bleeding score for FXIII deficient patients and its relation to the frequency and severity of symptoms. METHODS: In this cross-sectional study, 63 patients with severe FXIII deficiency were evaluated for the assessment of bleeding score according to the standard ISTH-BAT questionnaire. All patients were registered at two major thrombosis and hemostasis centers in Iran affiliated to Zahedan University of medical sciences (50 patients) and Shiraz University of medical sciences (13 patients). RESULTS: Significant correlations between the bleeding score and number of symptoms (r = 0.668, P < 0.001) and with a number of severe symptoms (r = 0.938, P < 0.001) were detected. There was no significant relationship between the mean bleeding score and CNS bleeding (P = 0.390). CONCLUSION: The ISTH-BAT score is an acceptable bleeding assessment tool for standardization and evaluation of patients with FXIII deficiency.

Leishman-Donovan bodies at bone marrow examination.

Leishmaniasis is a parasitic disease and often seen in developing countries and tropic areas. Visceral leishmaniasis (VL) is the most severe form of this disease, which is fatal if left untreated. In this report, we describe an 11-month-old infant with poor growth, pancytopenia, and splenomegaly. Microscopic examination of bone marrow revealed intracellular and extracellular Leishmania amastigotes. VL should be considered when a child presents with fever, failure to thrive, organomegaly, and pancytopenia.

Quality of death certificates completion for COVID-19 cases in the southeast of Iran: A cross-sectional study.

Background and Aim: Death certificate (DC) data provides a basis for public health policies and statistics and contributes to the evaluation of a pandemic's evolution. This study aimed to evaluate the quality of the COVID-19-related DC completion. Methods: A descriptive-analytical study was conducted to review a total of 339 medical records and DCs issued for COVID-19 cases from February 20 to September 21, 2020. A univariate analysis (χ 2 as an unadjusted analysis) was performed, and multiple logistic regression models (odd ratio [OR] and 95% confidence interval [CI] as adjusted analyses) were used to evaluate the associations between variables. Results: Errors in DCs were classified as major and minor. All of the 339 examined DCs were erroneous; more than half of DCs (57.8%) had at least one major error; all of them had at least one minor error. Improper sequencing (49.3%), unacceptable underlying causes of death (UCOD) (33.3%), recording more than one cause per line (20.1%), listing general conditions instead of specific terms (11.2%), illegible handwriting (8.3%), competing causes (6.2%), and mechanisms (3.8%) were most common major errors, respectively. Absence of time interval (100%), listing mechanism allying with UCOD (51.6%), using abbreviations (45.4%), missing major comorbidities (16.5%), and listing major comorbidities in part I (16.5%) were most common minor errors, respectively. Conclusion: The rate of both major and minor errors was high. Using automated tools for recording and selecting death cause(s), promoting certifiers' skills on DC completion, and applying quality control mechanisms in DC documentation can improve death data and statistics.

Congenital Bleeding Disorders amid the COVID-19 pandemic: Open questions and recommendations.

This article reviews the current knowledges of congenital bleeding disorders (CBD) amid the COVID-19 pandemic. It appears that CBD is not associated with higher risk of getting COVID-19 and so the prevalence of COVID-19 among them seems not higher compared to the general population. In absence of specific therapeutic recommendations, it is essential to make a correct assessment of the risk of haemorrhage/thrombosis. Based on expert opinion, strategies for outpatient management include adherence to prescribed regimens, telemedicine, and communication about COVID-19 in patients with CBD. More data should be also collected to better characterize the impact of COVID-19 on patients with CBD. The current findings encourage further studies to determine the prevalence of SARS-CoV2 infection in CBD patients to understand more fully the burden of this novel pathogen and to develop adequate preventive measures against this infection.

Comparison of the Effect of Phenobarbital versus Sodium Valproate in Management of Children with Status Epilepticus.

OBJECTIVES: Acute prolonged seizure is the most common neurological emergency in children. This research was conducted to compare the effect of intravenous phenobarbital and Sodium valproate in control of seizure in children with status epilepticus, referred to emergency ward from Mar to Nov 2013. MATERIALS & METHODS: In this randomized clinical trial, registered with the code number IRCT2015051722300N1, 80 children aged 6 months to 10 years with prolonged seizure and with no response to one dose of diazepam (0.2 mg/kg) administered through IV injection during the five min were selected. Children were randomly divided into two groups, intervention, and control through permutation blocks. In intervention group, intravenous Sodium valproate (20 mg/kg) and in control group, intravenous phenobarbital (20 mg/kg) were prescribed. Data such as age, gender, history of previous seizure, seizure type, and recovery time after receiving drug was recorded in the form. Data analysis was done through descriptive statistics, Chi-square and Independent t- test. RESULTS: Two groups were the same in terms of age and gender and had no statistically significant difference, but they were different in terms of seizure type. In valproate group, 18 patients (45%) and in phenobarbital group, 32 patients (80%) had positive response to the treatment and the chi-square test showed the significant difference. CONCLUSION: With regards to this point that in phenobarbital group, patients had more rapid response to drug in comparison with patients in Sodium valproate group, phenobarbital is a suitable and effective drug for controlling of seizure in children.

Chronic Myelogenous Leukemia Presenting with Facial Nerve Palsy in an Infant.

When a child presents with cranial nerve palsy and a bulging fontanel, a pediatric neurologist is often consulted to determine the cause of increased intracranial pressure. This report describes an infant with chronic myelogenous leukemia (CML) referred to Ali-bin-Abitaleb Hospital, Zahedan, eastern Iran in 2013 who presented with seventh cranial nerve palsy and bulging fontanel. Chromosomal analysis and peripheral blood smear confirmed the diagnosis of CML.

Immune Thrombocytopenic Purpura and Hemolytic Anemia Secondary to Hepatitis A.

Hepatitis A is common in children and usually is a self-limiting disease. Although extrahepatic and hematological immune manifestations following acute hepatitis A virus (HAV) infection have rarely been reported, they are frequently observed in other viral hepatitis. In this paper, we report the case of a 3-year-old girl who developed immune thrombocytopenic purpura (ITP) and hemolytic anemia after HAV infection. She was presented with malaise, pallor, ecchymosis, petechiae and purpura on the trunk and extremities.

Prevalence of G6PD deficiency in Children with Hepatitis A.

Introduction: Hepatitis A virus is the most prevalent viral hepatitis. It is globally a major public health problem with different clinical symptoms. This study aimed at investigating the clinical findings and prevalence of glucose 6-phosphate dehydrogenase (G6PD) deficiency in children with hepatitis A. Materials and Methods: In this prospective study, demographical information, clinical findings, and G6PD level of hepatitis A patients, who were visited at Pediatric Hematology clinic, were entered into the database. The diagnosis of hepatitis A infection was based on the presence of anti-HAV IgM antibody. The activity of G6PD enzyme was measured with florescent spot test. Results: Of the 117 children with hepatitis A, 52 (44.4%) were male and 65 (55.6%) were female. The mean age of these patients was 2.79±5.39 years. The most prevalent clinical manifestations were dark yellow urine and anorexia. G6PD deficiency was observed in 26 (26.3%) out of 99 patients whose G6PD levels were measured. Conclusion: Given the high prevalence of G6PD deficiency in this study, the measurement of G6PD level along with other liver and biochemical markers in areas with endemic hepatitis A is recommended. In addition, it is recommended that patients undertake precise monitoring for hemolysis and renal function.

Comparison of iron chelation effects of deferoxamine, deferasirox, and combination of deferoxamine and deferiprone on liver and cardiac T2* MRI in thalassemia maior.

BACKGROUND: Cardiac complications due to iron overload are the most common cause of death in patients with thalassemia major. The aim of this study was to compare iron chelation effects of deferoxamine, deferasirox, and combination of deferoxamine and deferiprone on cardiac and liver iron load measured by T2* MRI. METHODS: In this study, 108 patients with thalassemia major aged over 10 years who had iron overload in cardiac T2* MRI were studied in terms of iron chelators efficacy on the reduction of myocardial siderosis. The first group received deferoxamine, the second group only deferasirox, and the third group, a combination of deferoxamine and deferiprone. Myocardial iron was measured at baseline and 12 months later through T2* MRI technique. RESULTS: The three groups were similar in terms of age, gender, ferritin level, and mean myocardial T2* at baseline. In the deferoxamine group, myocardial T2* was increased from 12.0±4.1 ms at baseline to 13.5±8.4 ms at 12 months (p=0.10). Significant improvement was observed in myocardial T2* of the deferasirox group (p<0.001). In the combined treatment group, myocardial T2* was significantly increased (p<0.001). These differences among the three groups were not significant at the 12 months. A significant improvement was observed in liver T2* at 12 months compared to baseline in the deferasirox and the combination group. CONCLUSION: In comparison to deferoxamine monotherapy, combination therapy and deferasirox monotherapy have a significant impact on reducing iron overload and improvement of myocardial and liver T2* MRI.

Marital Status and Fertility in Adult Iranian Patients with ß-Thalassemia Major.

Expecting a family is an important component and a great goal for better quality of life for most of adults with ß-thalassemia major. The aim of the present study was to examine the marital status of adults with ß-thalassemia major. This cross-sectional study examined the marital status of patients with transfusion-dependent ß-thalassemia aged over 15 years. Patients' demographic characteristics including age, gender, marital status, duration of marriage, divorce, having or not having children and spouse's health status were recorded. Information about the disease including cardiac and endocrine complications, ferritin level, splenectomy and viral hepatitis were also recorded. Of 228 patients with transfusion-dependent ß-thalassemia major aged over 15 years who were treated at this medical center, 32 (14 %) were married. The mean age of married patients was 25.18 ± 4.74 years. Among the married patients, 8 (25 %) were females and 24 (75 %) patients were males. The mean age of marriage was 22.76 ± 4.16 years. The minimum and maximum marriage age was 15 and 33 years, respectively. The median duration of marriage was one year with the range from 3 months to 11 years. Only 8 (25 %) patients (one female and seven males) had children. Therapeutic advances have led to significantly increased survival and improved quality of life and fertility of patients with ß-thalassemia major. According to the results, 14 % of patients over 15 years were married which was slightly higher as compared with other similar studies.

Bilineal Acute Leukemia Associated With Fanconi Syndrome: The First Case Report.

Fanconi syndrome is a metabolic disorder involving dysfunction of the renal proximal tubules, resulting in excessive urinary excretion of several metabolites. Various factors may lead to Fanconi syndrome, as it may be a genetic disease with primary or secondary etiologies, or may be acquired. In this study, we report a unique case of Fanconi syndrome with development of a relatively rare acute leukemia, a condition that has not been reported before. The case was an 8-year-old boy with familial occurrence of Fanconi syndrome, presenting with pallor, asthenia, recurrent infections, growth failure, and a variety of biochemical and hematological abnormalities. After physical examination, radiographic studies, and comprehensive laboratory analyses, Fanconi syndrome associated with bilineal acute leukemia, of myeloid and T-lymphoid lineages, was diagnosed.

Brain Metastasis in Wilms' Tumor: a Case Report.

Wilms' tumor is the most common abdominal tumor of childhood, and its cerebral metastasis is apparently very rare. The authors report an 18-month-old girl with Wilms' tumor and brain metastasis.

Comparison of the Efficacy of Combination Therapy of Prednisolone - Acyclovir with Prednisolone Alone in Bell's Palsy.

OBJECTIVE: Bell's palsy is a rapid onset, usually, unilateral paralysis of the facial nerve that causes significant changes in an individual's life such as a decline in personal, social, and educational performance. This study compared efficacy of combined prednisolone and acyclovir therapy with prednisolone alone. MATERIALS & METHODS: This study is a randomized controlled trial conducted on 43 Children (2-18 years old) with Bell's palsy. The first group of 23 patients was treated with prednisolone and the remaining patients were treated with a combination of prednisolone and acyclovir. The required data were extracted, using an informational form based on the House-Brackmann Scale, which grades facial nerve paralysis. The data were analyzed with Mann-Whitney test using SPSS version 16. RESULTS: The mean age of the first and second group were 8.65 ± 5.07 and 8.35 ± 4.92 years, respectively, (p=0.84). Sixty one percent and 39% of patients in the first group, and 45% and 55% of patients in the second group were male and female, respectively. No significant differences exist between the groups in terms of age and gender. The rate of complete recovery was 65.2% in group I and 90% in the group II (p=0.04). CONCLUSION: The results of this study showed that the combined prednisolone and acyclovir therapy of patients with Bell's palsy is far more effective than treatment with prednisolone alone. Actually, age and gender had no impact on the rate of recovery.

Severe hypercalcemia: a rare and unusual presentation of childhood acute lymphoblastic leukemia.

Hypercalcemia in children is a medical emergency and often manifests as nonspecific symptoms such as nausea, vomiting, weight loss, and anorexia. Severe hypercalcemia is a rare complication of malignancy in children, while it can be seen in various types of malignant tumors. It is usually associated with significant morbidity and may be severe enough to threaten life. Incidence of hypercalcemia in hematopoietic malignancies including acute lymphoblastic leukemia (ALL) is very rare and unusual, especially as the initial manifestation of the disease. In this paper a 6-year-old boy who had severe hypercalcemia and gastrointestinal symptoms before the onset of common and usual manifestations of ALL is introduced.

Rhabdomyosarcoma with skin metastasis: report of a case.

Rhabdomyosarcomas are the most common soft tissue sarcoma in adult and children that accompany with skeletal muscle differentiation. Skin metastasis of rhabdomyosarcomas is unusual and has only been sporadically reported in literature. In this paper we present a case of skin metastasis of rhabdomyosarcoma in an 8-year-old girl that has treated with chemotherapy.