Information Registry System on Congenital Hypothyroidism: A Systematic Review

Background: Congenital hypothyroidism is the most common congenital disorder of the endocrine system, leading to preventable mental retardation. Objectives: We aimed to evaluate the current status of congenital hypothyroidism information registry systems. Methods: In this systematic review 290 papers identified. A total of 254 articles were screened, of which 17 qualified articles were selected through the databases of Scopus, science direct ProQuest, PubMed, as well as the search engine Google scholar (no restriction on date of publication) up to 2020, were searched. Inclusion criteria were the Articles with the English language that examining information registry systems on congenital hypothyroidism. Articles whose full texts were not accessible, case reports, and letters to the editor had to be excluded. Results: This study showed that the primary goal of nearly half (50%) of the information registry systems for congenital hypothyroidism is to evaluate the efficiency and effectiveness of the screening program. Other information registry systems for congenital hypothyroidism have examined the epidemiology of the disease in 4 studies (23.6%), monitoring iodine deficiency in 3 studies (18%), surveillance disease in 1 study (6%), and describing the clinical and familial features of the patient in 1 study (6%). Conclusions: Few studies have been done on developing information registry systems, and most studies have focused on the use of information systems in this field. Further comprehensive reviews are recommended to investigate the infrastructure of the commission national registry and world network to record information units on congenital hypothyroidism.

Digital Clubbing and Hodgkin Disease in Children: A Case Report and Review of Literature

Introduction: Digital clubbing (hypertrophic osteoarthropathy) as the initial presentation of lymphoma is rarely reported, particularly in children. In this study, we report a patient with intrathoracic Hodgkin Disease (HD) and digital clubbing as the first presentation, and we will review the literature regarding the same condition. Case Presentation: A 10-year-old boy presented with a 2-month history of cough, mild dyspnea, and night sweats, with prominent digital clubbing. A chest x-ray and a computed tomography scan of the chest showed multiple mediastinal masses. A mediastinal lymph node biopsy was done. Pathologic examination was indicative of nodular sclerosis HD. Conclusions: In patients with digital clubbing, intrathoracic malignancies should be considered a differential diagnosis and must be ruled out by precise examination and paraclinical help.

Acute Generalized Exanthematous Pustulosis Due to Cephalexin: A Case Report and Literature Review

Introduction: Acute Generalized Exanthematous Pustulosis (AGEP) is a cutaneous reaction that may appear after using certain medications, such as cephalexin. This disease is characterized by non-follicular sterile pustules, erythematous, urticaria, fever over , and leukocytosis. Cephalexin belongs to the family of β-lactam antibiotics, which are widely used to treat infections. However, cephalexin skin sensitivities have been reported rarely. Herein, in this case, we aimed to report a patient presented with AGEP due to cephalexin usage. Case Presentation: A 12-year-old boy presented with warm skin lesions that gradually appeared on the limbs, trunk, face, and neck after using cephalexin powder on his left leg injury. Because of his symptoms, acetaminophen, fexofenadine hydrochloride, loxoprofen sodium, and ointment, including difluprednate and hydrocortisone, were prescribed. Over time, the patient’s fever subsided, and 8 days later, the symptoms of AGEP, including urticaria, erythematous, and pustules in the neck and trunk, disappeared. Conclusions: Cephalexin is one of the antibiotics that doctors and patients consider when there is a possibility of infection. AGEP is a rare but severe reaction that can manifest as skin rashes in any age and sex following the use of cephalexin, so the patient should be careful when using this antibiotic.

Peritrochanteric Unicameral Bone Cyst in A 9-year-old Girl: A Case Report and Review of Literature

Introduction: The Unicameral Bone Cyst (UBC) is a benign osteolytic lesion primarily found in the metaphyseal part of long bones in children. It is important as it can involve growth plate involvement, cause pathological fractures and deformities of the affected limb. We report this case to emphasize that hip area pathologies can be represented with knee pain and discomfort. Case Presentation: The patient was a 9-year-old girl with a bone cyst in the right proximal femur, with functional knee pain and limping. The diagnosis was made after two years of pain in the knee area. As the cyst was symptomatic and the signs of impending pathological fracture were seen, the lesion was managed by curettage and fibular strut allograft and proximal humerus locking plate. Conclusions: The patient has been examined for knee joint problems for a long time, and her femur bone cyst was diagnosed after two years of pain. However, it could become a pathologic fracture or involve the growth plate and stop the limb’s growth, thus affecting the patient’s quality of life. Therefore, in children with chronic knee pain, careful assessment of the hip area is recommended.

Stem Cell Therapy in Children With Sensorineural Hearing Loss: A Narrative Review

Context: One of the research areas is using stem cell transplantation for treating children’s sensorineural hearing loss. Preclinical studies and testing of the stem cell types have been performed in this field, and relative improvement has been achieved. Objectives: This narrative review has been prepared to study the advancements in hearing regeneration with stem cell transplantation. Data Sources: The English articles with full-text were searched in PubMed, Scopus, and Google scholar from 2000 to 2020 using keywords of sensory neural hearing loss and stem cell. Results: In 2018, the first human study was performed with stem cells from the human umbilical cord, which has promising results regarding the safety of the method and its positive effects on hearing. Conclusions: Autologous stem cell transplantation had induced relative improvement without serious adverse events in children with acquired sensorineural hearing loss. To obtain more evidence, further studies are required with larger sample sizes and in different patients groups.

Potts Shunt in Children With Familial Primary Pulmonary Hypertension: A Case Report and Brief Literature Review

Introduction: The primary concern about Familial Pulmonary Arterial Hypertension (FPAH) is the development of right heart failure, which ultimately leads to sudden death. Despite recent advances in pharmacological therapy, the mortality rate in children is still high, emphasizing the importance of novel treatments. Moreover, there is still no definitive treatment for children with severe pulmonary hypertension. The consequences of right heart failure led to the proposal of a surgical approach, the Potts shunt, to decompress the right ventricle, providing shunting of deoxygenated blood to lower extremities and improving overall cardiac output. This surgical technique creates an anastomosis between the left pulmonary artery and the descending aorta, providing a palliative treatment to off-load the right ventricle. Case Presentation: We report an 11-year-old girl with familial pulmonary arterial hypertension and right ventricular failure who benefited from a Potts shunt with good mid-term results. The patient was diagnosed at the age of 5 when she had a history of fainting and persistent syncope. The patient was under medical treatment therapy with bosentan, sildenafil, acetylsalicylic acid, and warfarin for six years. The results of molecular genetic testing, which was confirmed with direct sequencing of the Bone Morphogenetic Protein Receptor Type 2 (BMPR2) gene, revealed a heterozygous pathogenic mutation. Since she was diagnosed with Pulmonary Arterial Hypertension (PAH), she lost her grandmother, an aunt, and father because of PAH. Her 14-year-old sister also had mutated the BMPR2 gene without developing FPAH. Conclusions: The Potts shunt provides an interventional step for palliation of patients with familial pulmonary hypertension and severe right heart failure refractory to medical treatment. It opens the door to the possibility of lung transplantation in the future. We did not see any complications within 6 years after placing the Potts shunt.

Evaluation of the Drug Hypersensitivity Reactions Prevalence in Hospitalized Children in Sari City from 2014 to 2018: An Original Research

Background: Drug allergy is a subtype of Adverse Drug Reaction (ADR) mediated by the immunologic system. Extreme drug allergy is poorly known in children that contribute to hospitalization in our region. Objectives: This research aimed to study major drug hypersensitivity reactions in the north of Iran among the hospitalized children. Methods: This cross-sectional study was conducted at Bou Ali Sina Hospital in the north of Iran. We analyzed the medical files of 140 children admitted to the hospital with drug allergies from 2014 to 2018. The inclusion criteria were based on the DRESS (drug reaction with eosinophilia and systemic symptoms) scoring system, and those who scored more than four were enrolled. Patients with incomplete medical charts were excluded. The sampling methodology was based on the consecutive census method. Results: In the current study, 62 patients were females (44.29%), and 78 were males (55.71%). Ninety-six cases (68.57%) were children under five, and 44 cases (31.43%) were over five years old. Most ADRs were seen in patients from urban areas (58.57%) than in rural areas (41.43%). In 116 children (82.6%), maculopapular rashes were seen. Phenobarbital and cefalexin had the highest levels of drug reactions, including 85 persons (55.19%) and 18 persons (11.69%), respectively. Pruritus, fever, and scaling were the most common symptoms observed in the patients. The most commonly used treatment was antihistamines. Conclusions: The most causative medication inducing ADR was phenobarbital. The maculopapular rash was the most common skin manifestation, and there were no relationships between sex, age, medication type, and type of hypersensitivity reactions.

Local Action for Sexual and Reproductive Health of Street Children: A Systematic Review

Context: Street children may expose to high-risk behaviors such as unwanted pregnancy, abortion, and Sexually Transmitted Diseases (STDs). Objectives: This study systematically reviewed and presented preventive strategies for promoting Sexual and Reproductive Health (SRH) against unsafe abortion, STDs, and unwanted pregnancy in street children. Data Source: In this systematic review, we searched databases of ScienceDirect, Web of Science (ISI), PubMed, Scopus, Scientific Information Database (SID), and Google Scholar. Published articles between 1990 and 2020 were retrieved. Finally, 41 articles were used to compile the results of this study. Study Selection: A total of 1522 potentially relevant articles were identified; 275 articles were removed due to duplication and in abstract screening, and 766 articles did not meet the inclusion criteria or focus on the research question. Next, the full-text of 481 remained articles were screened. Studies in languages other than English or Persian, studies presented in conferences, and those with no information regarding the SRH among street children were removed. Finally, 41 studies were included in this systematic review. Data Extraction: Data were extracted from the articles according to the selection criteria by two independent reviewers. Results: Results of this study were categorized into 2 tables. Table 1 presents the strengths, weaknesses, opportunities, and threats of SRH in street children were shown. Table 2 presents the preventive strategies regarding unwanted pregnancy, unsafe abortion, and STDs in the street children in four categories of 1) expansion program and promotion of educational services, 2) expansion program and promotion of educational services, 3) creating secure umbrella and social welfare, and 4) expansion program and promotion of social protection. Conclusions: Because of no information on street children regarding SRH, the government should consider adequate sexual education programs such as protected sexual intercourse and counseling on preventing STDs and unsafe abortion. It is essential to allocate affordable and accessible healthcare centers and facilities to provide services with no judgment on these children.

The Association Between Antioxidant Status and Excess Weight in Children: A Systematic Review and Meta-analysis

Background: The prevalence of childhood obesity with its complications has increased in the world. Obesity, along with antioxidants deficiency (due to an unhealthy diet), might change the balance in favor of oxidative stress. Objectives: The current study aims to assess the literature on the relationship between obesity and antioxidant status through a systematic review and meta-analysis Results: β-Carotene levels was significantly lower in obese children than non-obese ones (mean difference: 0.13, 95%CI: 0.09-0.16, P<0.001), with significant heterogeneity (P<0.001, I2=85%). There was a significant difference between obese and non-obese children in both α-tocopherol (pooled mean difference respectively: 0.36, 95% CI: 0.04-0.96, P<0.001 ) with non-significant heterogeneity (P>0.05, I2=0.0% ) and α-tocopherol per lipoid (pooled mean difference: 0.42, 95%CI: 0.28-0.55, P<0.001), with significant heterogeneity (P=0.048, I2=58.8%). There was no significant association between vitamin E level and obesity (pooled mean difference: 0.40, 95%CI: -0.05-0.85, P>0.05), with significant heterogeneity (P<0.001, I2=84.5%). There was significant association between zinc, magnesium, copper, and selenium level and obesity (P>0.05), with significant heterogeneity (P<0.001). Conclusions: This review revealed a significant inverse relationship between childhood obesity and serum antioxidant levels. More studies are necessary to find the underlying mechanisms and clinical impacts of this finding. Data Sources: This systematic review and meta-analysis were performed among English language articles published until September 2020 without any time limit. An electronic search was conducted in international databases of Google Scholar, PubMed, Web of Science, Scopus, Medline, and Cochrane. Study Selection: First, 1255 papers were found. After removing duplicates and quality assessment, 46 were used in the systemic review, and 19 articles were entered into the meta-analysis. Data Extraction: Two researchers independently searched the following keywords in the databases: “Vitamin C”, “Vitamin E”, “Vitamin A”, “Carotenoids”, “Antioxidants”, “Selenium”, “Magnesium”, “Copper”, “Zinc”, “Ascorbic acid”, “Tocopherol”, “Obesity”, “Overweight”, “Childhood”, “Pediatric”, and “Adolescence”. Articles that examined the association between obesity and antioxidant status were included in the study. The research on animals, interventional studies, case studies, case reports, and irrelevant studies were excluded. The research team determined the quality of studies using the STROBE (strengthening the reporting of observational studies in epidemiology) checklist. Heterogeneity of studies was evaluated using index (I2) and probability of diffusion bias by funnel plot and Begg’s and Egger’s tests.

Cardiac Involvement in a Fetus With Vein of Galen Aneurysmal Malformation, Diagnosis, and Treatment: A Case Report

Vein of Galen Aneurysmal Malformations (VGAMs) are severe and rare congenital brain vasculature anomalies, which causes high mortality and morbidity in fetuses, neonates, and infants. It can be identified in the pregnancy period with fetal echocardiography. We report a case of VGAMs with severe cardiac manifestations in the fetus of a pregnant woman at the 28th week of gestational age.