SMSJ Peds.
It's time to revise the role of positive D- penicillamine challenge test in diagnosis of Wilson disease

fereshteh karbasian, Amirhossein Hosseini, Naghi Dara, Sarah Javdani Yekta, Negar Imanzadeh, Aliakbar Sayyari

Hepatic involvement in Wilson disease consists of isolated elevated liver enzyme, asymptomatic hepatomegaly, cirrhosis and acute liver failure. Here, we report three patients with unexplained elevated liver enzymes. By considering the level of urinary excretion of copper after penicillamine challenge test, we had some problems in the process of diagnosis. Therefore, we thought of cautiously applying the diagnostic cut-off in the mentioned challenge test

Vaginal foreign body in a 4 year old girl: a case report

Niloufar Bineshfar, Alireza Mirahmadi, Fereshteh Karbasian, Amirhossein Hosseini, Leily Mohajerzadeh, Soheila Vaghefi

Vaginal foreign body is an uncommon clinical entity in the pediatric population but it is more common in mentally retarded and young children. In this case report, we present a 4-year-old girl who was brought to the emergency room with a history of self-insertion of a pen cap into the vaginal orifice which had no vaginal bleeding, vaginal discharge, abdominal pain or urinary symptoms.

A Rare Case Report of Duodenal Adenocarcinoma Accompanied by Cerebellar Medulloblastoma

Siamak Afaghi, Nastaran Khoshpour, Amirhossein Hosseini, Farzad Esmaeili Tarki, Fatemeh Sadat Rahimi, Yalda Nilipour, Naghi Dara, Negar Imanzadeh

A case of a 6-year-old female with a diagnosis of Duodenal Adenocarcinoma is presented by the authors, in which 24 months subsequent to undergoing radiation therapy for a Cerebellar Medulloblastomas, the mentioned diagnosis has been confirmed. Although, the chance of a secondary malignancy has been recognized to be elevated in children formerly undergone radiation therapy, the incidence of duodenal adenocarcinoma is a highly infrequent condition. As a consideration, observing manifestations like weight loss, vomiting, and icterus in a pediatric patient who has been administered earlier on with radiation therapy, must alert physicians that the risk of a gastrointestinal malignancy exists.

Autoimmune pancreatitis as a very rare cause of recurrent pancreatitis in children; a case report and review of literature

Niloufar Shashaani, Amirhossein Hosseini, Negar Imanzadeh, Naghi Dara, Rahman Matani, Aliakbar Sayyari

Autoimmune pancreatitis as chronic inflammation of the pancreas due to an autoimmune mechanism is a rare type of pancreatitis. A 14 years old girl presented with multiple episodes of abdominal pain, nausea with elevation of amylase and lipase suspicions of acute recurrent pancreatitis since 3 years of age. After through evaluation about secondary causes of recurrent and familial pancreatitis finally she responded to corticosteroid treatment. Although very rare but autoimmune processes should be considered in teenagers with recurrent pancreatitis.

Prognosis of Mechanical Ventilation in Very Low Birth Weight Neonates: A Single-Center Study in Tehran.

Fereshteh Karbasian, Sara Jafarian, Ali Zafari, Niloufar Bineshfar, Seyed Abolfazl Afjehi, Amirhossein Hosseini, Minoo Fallahi

Background and Aims: Approximately 4–7 percent of all live births are led to a very low birth weight (VLBW) situation where the morbidity and mortality rate are very high. A large number of VLBW newborns in intensive care unit (ICU) require mechanical ventilation due to various conditions. To reduce mortality in this group, identification of risk factors is important. This study aimed to determine the prognosis of mechanical ventilation in VLBW neonates at Mahdiye hospital in Tehran.

Materials and Methods: This study is a prospective cohort study. VLBW neonates who consecutively were put on mechanical ventilation during the study period were enrolled.  Then, the enrolled neonates were divided into two groups: neonates who died after implementing the ventilator were in group-I and neonates who survived after receiving mechanical ventilation were in group-II. Demographic, clinical and paraclinical variables were gathered to find out the predictors of mortality of ventilated neonates. The data were analyzed by SPSS software version 21.

Results: During the study period, a total of 177 neonates were ventilated due to different causes. 56% were male with a male to female ratio 1.27:1. Mean birth weight and gestational age were 1024.8 ± 247.5 grams and 27.9±2.2 weeks respectively. Out of 177 mechanically ventilated VLBW neonates enrolled for this study, 53% died. Significant factors determining mortality rate were mean weight, mean gestational age, pulmonary hemorrhage, advance resuscitation and duration of hospital stay (p<0.05). APGAR score, gender, Pneumothorax, IVH>II, Sepsis and Maternal Disease were not significantly associated with mortality in VLBW neonates requiring mechanical ventilation (P>0.05).

Conclusion: This study showed that among the analyzed factors weight <1000gm, gestation <28weeks, pulmonary hemorrhage and complications during ventilation were the most significant predictors of mortality in ventilated VLBW neonates in the intensive care unit.

Foreign body aspiration(FBA) is a common life-threatening condition in pediatric emergency medicine that can lead to airway obstruction, asphyxia, and severe respiratory distress. An 18-month old female infant referred with the complaint of dry cough and wheezing with a sudden onset of dyspnea. At the time of admission, her oxygen saturation was  89%. Chest X-ray (CXR) showed a significant opacity in the left lung and the mediastinum shifting to the left side. Complete collapse seen in the left lung was associated with consolidation in the residual lung parenchyma. Rigid bronchoscopy revealed the corn kernels, which were taken out accordingly. The air trapped in the involved lung parenchyma was absorbed, and signs of shrinkage and collapse as consequences of the obstruction occurred in distal parts of the obstruction point. An emergent therapeutic decision is crucial in the early stages of management.

The Late presenting Bochdalek hernia; A Case report and review of literature

Farid Imanzadeh, Sepand Tehrani Fateh, Shaya Alimoghadam, Naghi Dara, Mohsen Rouzrokh, Amirhossein Hosseini

We report a 22-month-old boy who referred due to nausea, vomiting, abdominal pain and watery non-bloody diarrhea and after thorough evaluation, a large defect in the left postero-lateral side of diaphragm and presence of bowel loops, spleen, stomach and left lobe of liver in the left hemi-thorax were detected. So, he was operated and managed with the impression of Bochdalek hernia. We have also reviewed the similar case reports in the past 10 years, briefly, in order to map the presentations and clinical course of cases with Bochdalek hernia which were diagnosed late, for giving physicians a better insight on this issue.

Vestibular neuritis in pediatrics: a mini review

Fargol Farahmandi, Ali Nikkhah, Mohammad Reza Khalilian, Parynaz Parhizgar

Background and aims: Vestibular neuritis (VN) is a clinical feature, portrayed by acute and prolonged vertigo from peripheral source without associated auditory symptoms. the appearance of vestibular disease may also lead to cognitive deficits, including attention deficits. Therefore, the purpose of this mini-review is to describe the clinical characteristics, etiology, prevalence and treatment of vestibular neuritis in children.

Materials and methods: The present study was conducted in January 2021. The study was performed based on the preferred reporting items for systematic reviews and meta-analyses (PRISMA) checklist. The comprehensive search was conducted in the literature of the international databases (PubMed, Scopus, and Google Scholar) and national databases including scientific information database (SID), Magiran and iranDoc until January 13, 2021. Search keywords included "vestibular neuritis", "vestibular disorder", "balance disorder", and "pediatrics" or "children" or "adolescent" or "infants". The time limit for publishing articles was from 2010 onwards.

Results: In the initial search, 17 articles were extracted. After reviewing the title and abstract, 6 studies were excluded. In the evaluation and full-text review, 2 articles were excluded due to low quality and lack of access to the full text of another article. Finally, 9 studies were included in the present study.

Conclusion: We conclude that vestibular neuritis should always be considered in pediatric patients presenting with vertigo, and especially with nasopharyngeal viral infection, in addition to other differential diagnoses. Vestibular neuritis is one of the most common diseases in vestibular disorders in children, and different prevalence rates have been reported in studies so it needs further investigation. Once the diagnosis of vestibular neuritis is confirmed, symptomatic treatment should be started for the child.

Background and Aims: vitamin D insufficiency is a common disorder worldwide and children are involved with this disorder. In this study we aimed to evaluate of effectiveness of treatment of vitamin D insufficiency with vitamin D 50,000 unit in children who had vitamin D insufficiency.

Materials  and  Methods: children’ vitamin D levels were evaluated and if they had vitamin D insufficiency, 1 pearl of vitamin D 50,000 unit was prescribed weekly for 6 weeks, and after 6 weeks, vitamin D levels were checked again. Then all data were analyzed in SPSS 20 software.

Results: ninety-seven children were evaluated and 61.9% were boys. The average age of children was 101.01 ± 19.27 months. The average BMI of these children was 15.46 ± 2.52 kg/m2. Initial vitamin D level was 18.21 ± 6.42 ng / mL with a range of 4.2 to 29.8. After treatment, this level reached 41.08 ± 14.61ng / mL (12.2 - 98.4 ng / mL) and this increase was statistically significant (P-value< 0.005). There was no correlation between age, gender, height, and BMI with the increase of efficacy of vitamin D level with 1 pearl of vitamin D 50000 unit.

Conclusion: using one pearl of vitamin D 50000 unit weekly for 6 weeks is a good method for vitamin D insufficiency treatment in children.