Determination of Bone Density by DEXA Method Based on Bone Age and its Comparison with Chronological Age in Chronic Patients.

Background and Objective: Given the growing awareness about the important role of children's age in building bone for a person's life, physicians need to assess bone health in high-risk children for bone density disorders more than before to optimize their bones' density and prevent osteoporosis in future. The aim of this study was to evaluate bone density based on chronological and bone age. Materials and Methods: In this cross-sectional study, 80 Patients who have been referred for bone density to the Osteoporosis Centre of the Children's Medical Centre over a one-year period (spring 98 to spring 99) were studied. Bone density was performed for all patients by using DEXA method. Results: The z-score mean chronological age for the lumbar spine was -0.8± 1.85 years and bone age was -0.58±1.64 years. The z-score mean chronological age for femoral bone was -1.6±1.02 years and bone age was -1.32± 1.4 years. Conclusion: Results showed that in all patients, the difference in the mean Z score of chronological age and bone age of the spine between patients was not significant but for femur was significant. Also, use of corticosteroids leads to significant difference between the two age groups' z-score in femur and spine.

Children with SARS-CoV-2 infection during the novel coronaviral disease (COVID-19) outbreak in Iran: an alarming concern for severity and mortality of the disease.

BACKGROUND: The rapid worldwide spread of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections led to public health crises globally and the number of pediatric patients with Coronavirus Disease 2019 (COVID-19) is still rising. The aim of this study was to describe the epidemiological, clinical, laboratory, and imaging features of hospitalized patients with COVID-19 at an Iranian referral pediatrics hospital and to compare these parameters between hospitalized patients with and without severe disease, multisystem inflammatory syndrome in children (MIS-C) and children with acute COVID-19, as well as deceased and discharged cases. METHODS: This study included hospitalized children and adolescents (≤ 18 years) with suspected COVID-19 who had positive results for SARS-CoV-2. RESULTS: Among the 262 patients with suspected COVID-19, 142 confirmed COVID-19 cases were included in the study. A total of 11 children were diagnosed as MIS-C. The majority of the cases with MIS-C were male, (n = 9, 82%) which is significantly higher than children (n = 61, 47%) with acute COVID-19 (P = 0.03). Fifty patients (35%) were shown to have a more severe form of COVID-19. Ninety percent of the cases (n = 45) with severe COVID-19 had comorbidities that was significantly higher than cases with non-severe or mild disease (n = 41, 45%; P < 0.0001). A mortality rate of 10% was reported (n = 14). Ninety-three percent of the deceased cases (n = 13) had comorbidities that were significantly higher than discharged patients (n = 73, 57%; P = 0.009). CONCLUSION: The increasing number of children with severe COVID-19 is cause for great concern. Underlying diseases, mainly cardiovascular diseases, cancer, and malignancies, are associated with greater risk of development of severe COVID-19 and even death in children. On the other hand, pediatric patients with MIS-C usually develop a milder form of the disease. However, evaluation specific immunological responses in children to explore the delayed inflammatory syndrome are highly recommended.

Evaluating the value of different sonographic findings in diagnosis of acute appendicitis in children.

INTRODUCTION: Appendicitis is one of the most common paediatric surgical emergencies occurring in about 7% of healthy children. To make a definitive diagnosis preferably avoiding unnecessary X-ray radiation exposure, ultrasound is the ideal modality. The aim of this study is to evaluate the diagnostic value of sonographic findings in children with acute appendicitis and comparing them with surgical findings to demonstrate the safety, simplicity and accuracy of this procedure in emergency departments as the first diagnostic procedure. MATERIALS AND METHODS: One hundred and eight children aged 1-15 years suspected of acute appendicitis in our tertiary hospital emergency department enrolled the study. Patients presenting as acute abdomen suspected as having acute appendicitis underwent abdominal ultrasonography (US) at first. Sonographic findings were compared to surgical and pathologic results, and sensitivity and specificity of each sonographic parameter in paediatric appendicitis were evaluated. RESULTS: The analysis of sonographic results showed that 67.6% of patients had acute appendicitis, 13.9% had perforated appendicitis and 18.5% had normal appendix. On the other hand, there were acute appendicitis in 63.9% of patients, perforated appendicitis in 12% and normal appendix in 8.3% in surgical reports. Sensitivity of uncompressible appendicitis, appendicitis, maximal outer diameter (MOD) above 6 mm, maximal mural thickness (MMT) above 3 mm, round appendix was 98.68%, 28.04%, 94.74%, 61.84% and 68.42%, respectively. Specificity of incompressible appendicitis, appendicitis, MOD above 6 mm, MMT above 3 mm, round appendix was 64.71%, 96.15%, 64.71%, 82.35% and 94.12%, respectively. Overall sensitivity and specificity of US in appendicitis were 97.56% and 69.23%, respectively. CONCLUSION: According to the findings of this study, sensitivity of US in diagnosing appendicitis is higher than other studies, but its specificity was lower. Ultrasonographic accuracy and efficacy to diagnose acute appendicitis in children are high enough to allow clinicians to do it as an imaging modality of first choice, and also, in problematic cases to assist correct clinical diagnosis avoiding unnecessary X-ray exposure, decreasing negative appendectomies, decreasing perforation rate and lowering the cost of patients. Furthermore, negative US do not justify immediate computed tomography because clinical re-evaluation and a second US can help greatly the clinicians in the correct diagnosis.

Rosai-Dorfman Disease: A Rare Clinicopathological Presentation and Review of the Literature.

Rosai-Dorfman disease (RDD) is a rare and self-limiting disease process that presents most commonly in young patients as massive, painless, cervical lymphadenopathy. Extranodal involvement may also occur. Histopathologic evaluation is the main diagnostic modality. We report an unusual presentation of RDD with cervical lymphadenopathy and an incidentally discovered sinonasal mass, clinically worrisome for malignancy. We emphasize that a high index of clinical suspicion is critical for accurate diagnosis of RDD. Clinicians and pathologists should consider RDD in a differential diagnosis of cervical lymphadenopathy, especially in young patients.

The Coronavirus Disease 2019 (COVID-19) in Children: A Study in an Iranian Children's Referral Hospital.

BACKGROUND: Despite the worldwide spread of the coronavirus disease 2019 (COVID-19), the epidemiological and clinical patterns of the COVID-19 infection remain largely unclear, particularly among children. In this study, we explored the epidemiological characteristics, clinical patterns, and laboratory and imaging findings of pediatric patients with COVID-19. MATERIALS AND METHODS: From March 7 to March 30, 2020, there were a total of 35 patients who had confirmed COVID-19 infection by laboratory virus nucleic acid test (RT-PCR) assay with throat swab samples or typical chest CT manifestation compatible with COVID-19, in addition to a history of close contact with suspected or confirmed SARS-CoV-2 in family members. Information recorded included demographic data, medical history, exposure history, underlying comorbidities, symptoms, signs, laboratory findings and radiologic assessments, severity of disease, treatment, and mortality. RESULTS: The median age of the patients was 7.5 years (IQR=4-11; range=4 months to 15 years). A total of 63% were male. Cough was present in 80% of the patients, followed by fever (77%), nausea or vomiting (29%), diarrhea (26%), shortness of breath (29%), headache (20%), and myalgia (14%). Lymphopenia was present in 43% of the patients, thrombocytopenia in 9%, neutopenia in 8%, and leucopenia in 26%. We reported severe pneumonia in 40% of the hospitalized patients and 18 (51%) had underlying diseases. Of 35 patients, 11 had positive RT-PCR results (31%). The chest CT images of 24 patients (69%) suggested COVID-19, while their RT-PCR assays from throat swab samples were negative. CONCLUSION: This study demonstrates different clinical findings of pediatrics compared to the previous reports of children. Since a high rate of false negative RT-PCR test was observed, early detection of children with COVID-19 infection by CT is conducive to reasonable management and early treatment.

Transgluteal ultrasonography in spica cast in postreduction assessment of developmental dysplasia of the hip.

PURPOSE: The position of the femoral head in spica cast after the reduction of developmental dysplasia of the hip (DDH) should be examined and followed up closely and regularly. The study aimed to use the transgluteal ultrasonography approach for this purpose and compare its accuracy with the results of CT scan, which is the most commonly used modality. METHODS: Twenty-three patients with an average age of 20-21 months were examined for 1 year after the reduction of DDH, both closed and open. Ultrasonography and CT scan were performed on the patients on the same day, and the results were interpreted by different radiologists. Transgluteal ultrasonography in spica cast was performed while the legs were abducted, internally rotated, and flexed. A blanket was placed under the patient to elevate the cast. RESULTS: Thirty cases of proper reduction (81%) and 7 cases of dislocated hip (19%) were reported in transgluteal ultrasonography, and 29 cases of proper reduction (78%) and 8 cases of dislocated hip (22%) were reported in the CT scan. The rate of agreement between the results of ultrasonography and CT scan was 91%. CONCLUSION: Transgluteal ultrasonography can be used as an excellent modality to examine the position of the femoral head in relation to the posterior rim of the acetabulum in spica cast. The position of the femoral head can be viewed properly needless of perineal opening in the cast. Thus, transgluteal ultrasonography can replace the CT scan to assess the position of the femoral head. Sonography does not expose patients to radiation and does not require sedation.

Evaluation of Lower Extremities' Vascular Characteristics in Myelomeningocele Patients: A Case-Control Study.

OBJECTIVE: Myelomeningocele (MMC) is the most common neural tube defect. Patients with MMC have multiple risk factors for venous thrombosis, but this complication rarely occurs. This lower rate of venous thrombosis in MMC children could be related to some characteristics of the vessels in the lower extremities. This study aimed at finding explanations for this dilemma. METHODS: A case-control study was designed in the Children's Hospital Medical Center, Tehran considering paraplegic patients with MMC as the case group and nonparaplegic MMC patients as a control group. Doppler ultrasound was performed to evaluate femoral and popliteal arterial and venous properties. RESULTS: Patients aged from 8 months to 12 years were evaluated. The mean diameter of the femoral arteries was 3.73 ± 0.23 and 4.72 ± 0.39 mm among paraplegic and nonparaplegic MMC patients, respectively (p = 0.02). The femoral artery flow was 0.52 ± 0.08 and 0.75 ± 0.06 L/min, respectively in the case and control groups (p = 0.015). The diameters of the femoral veins were 4.85 ± 0.34 and 5.13 ± 0.32 mm in the case and control groups, respectively (p > 0.05). Besides, the blood flows of the case and control groups' femoral veins were 0.27 ± 0.08 and 0.14 ± 0.01 L/min, respectively (p = 0.6). It turned out that lower extremities' arteries in the case group had significantly lower blood flow and diameter compared to those of the control group. However, the same venous properties did not show any significant differences. CONCLUSION: The decreased arterial flow along with the unchanged venous properties leads to less stasis and better drainage of the blood, which in turn might result in a lower incidence of deep vein thrombosis.

International Interdisciplinary Consensus Meeting on the Evaluation of Developmental Dysplasia of the Hip.

In September 2018, an international meeting of doctors of various disciplines, with expertise in the detection and treatment of DDH, was held in Csolyospalos, Hungary. The aim was to achieve consensus on the detection and early treatment of the condition and to develop a standardized system of teaching and training for hip ultrasound. There was strong agreement that US screening is essential. Specifically the Graf technique was selected as the technique of choice. Universal US screening was strongly favored. Screening should be carried out as soon as possible, but not later than the sixth week of age. US screening is cost-effective, does not result in overtreatment, and contributes to a reduction of long-term consequences. The essential principle of treatment is timely application of a device to achieve reduction, retention and maturation, by holding the hips in flexion, and a safe degree of abduction. It was agreed that the effectiveness of any screening policy depends on the correct scanning technique. Therefore, standardization of teaching and training of the Graf technique is mandatory. A unified teaching policy and materials should be developed for this purpose. Certification, re-certification and audit were discussed. The group, which has been formalized as the International Interdisciplinary Consensus Committee On DDH Evaluation (ICODE), will continue to meet and work towards establishing international consensus on DDH, standardizing and developing teaching and training of the Graf technique for hip US, and maintaining standards for detection and management.

Systemic Amyloidosis in a Teenage Boy With Inflammatory Bowel Disease.

Systemic amyloidosis is a very rare complication of inflammatory bowel disease (IBD). The reported cases of secondary amyloidosis in children with IBD are much fewer than those reported in adults. Herein, a teenage boy with Crohn's disease is presented who developed nephrotic syndrome due to renal involvement secondary to amyloidosis, whereas the patient was under treatment with corticosteroid and 6-mercaptopurine. To our best knowledge, this is the first reported case of secondary amyloidosis in a teenage Iranian boy with Crohn's disease.

Caustic Agent Ingestion by a 1.5-Year-Old Boy.

We present a case of caustic ingestion by a 1.5-year-old boy. The caustic agent was drain opener which is a strong alkaline substance. Children in Iran and many other countries are still exposed to not "child proof" (child resistant packaging) toxic substance containers. Ingestion of caustic agents may lead to necrosis, perforation, and strictures. Substances that are ingested more frequently are liquid alkali material which causes severe, deep liquefaction necrosis. Common signs and symptoms of caustic agents are vomiting, drooling, refusal to drink, oral burns, stridor, hematemesis, dyspnea, dysphagia and abdominal pain. Even if no oropharyngeal lesion is seen, a significant esophageal injury which can lead to perforation and stricture cannot be ruled out. If abdominal pain or rigidity, substernal, chest or back pain exists, visceral perforation should be considered. The first thing to be checked is airway assessment. A lot of patients should be admitted to intensive care unit, and endoscopic evaluation, surgical intervention, long-term hospitalization, and worsening quality of life or among the complications. Preventive measures especially at the country level and approving proper legislation for obligating the related industries to produce child proof containers for house hold toxic products are the urgent measures to be followed by all of us.

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.

Drainage-related ultrasonography (DRUS): a novel technique for discriminating obstructive and nonobstructive hydroureters in children.

BACKGROUND: Despite advances in urologic imaging, the paucity of an optimal technique that accurately clarifies obstructive and nonobstructive hydroureter exists. OBJECTIVE: This study was conducted to introduce a novel and modified ultrasonographic technique, known as drainage-related ultrasonography (DRUS), discriminating obstructive and nonobstructive, nonrefluxing hydroureter. MATERIALS AND METHODS: A total of 358 children (mean age, 3.7 years) with 418 nonrefluxing hydroureter were included. These children were composed of two groups of obstructive nonrefluxing (141 children with 157 dilated ureters) and nonobstructive, nonrefluxing (217 children with 261 hydroureter). The definite diagnosis regarding the subtype of hydroureter was derived from appropriate investigation. The maximum diameter of the dilated ureter, which was observed on ultrasonography, was recorded before and after 3 h of catheterization, as D1 and D2, respectively. To assess the D ratio, a formula was developed, that is, [(|D1 - D2|)/D1] × 100. Values were recorded and cutoff points were set to discriminate between subtypes. RESULTS: Obstructive versus nonobstructive subtypes of nonrefluxing hydroureter were clarified with 78.5 % sensitivity and 83.4 % specificity, by setting a cutoff point of 22 % for the D ratio. Regardless of the cutoff point assigned to the reduction in D (D2 compared with D1), DRUS revealed 93.9 % sensitivity, 80.6 % specificity, 63.2 % positive predictive value, and 97.4 % negative predictive value in discriminating upper from lower obstruction. CONCLUSION: DRUS affords favorable results in terms of differentiating between obstructive and nonobstructive, nonrefluxing hydroureter, as well as between upper and lower obstruction in obstructive cases. It has the potential to become an efficient imaging modality in the diagnostic algorithm of hydroureter.

Concomitant anterior and posterior urethral valves in pediatrics: A single center experience over 12 years and long-term follow up after endoscopic treatment.

OBJECTIVE: To report our 12-year experience with endoscopic management of patients with concomitant anterior and posterior urethral valves. METHODS: We retrospectively reviewed the charts of patients referred to us for management of urethral valves from 2000 to 2012 to find cases with concomitant anterior and posterior valves. The diagnosis of valves was first suspected on voiding cystourethrography and confirmed by urethrocystoscopy. We collected available data on patients' age at diagnosis, clinical presentations, ultrasound and urodynamic findings, and surgical treatments. The final outcome at last follow up was also recorded. RESULTS: From 38 cases with anterior urethral valve, six (15.8%) presented concomitant anterior and posterior valves. The age at diagnosis in these patients ranged from antenatal diagnosis to 13 years. Initial presenting symptoms were recurrent urinary tract infection, incontinence, urosepsis and poor urinary stream. All valves were ablated by transurethral fulguration/resection using small-sized urethrocystoscopes. Among those with concomitant anterior and posterior valves, four patients had vesicoureteral reflux at presentation that resolved in two patients after valve ablation. One patient progressed to renal failure and required dialysis. Bladder hypercontractility and detrusor overactivity were the main urodynamic patterns in these patients. CONCLUSIONS: Concomitant anterior and posterior valves seem to be more prevalent than previously assumed, and might be missed on initial assessment. Oblique view voiding cystourethrography with full-length delineation of the urethra is of paramount diagnostic importance when obstruction is suspected. A meticulous urethrocystoscopy should follow for confirming the diagnosis and endoscopic ablation/resection of the valves.

Hyperostosis-hyperphosphatemia syndrome (HHS): report of two cases with a recurrent mutation and review of the literature.

Hyperostosis-hyperphosphatemia syndrome (HHS) is a rare autosomal recessive metabolic disorder caused by mutations in the GALNT3 and FGF23 genes. The main features of this disorder include painful swelling of long bones, increased renal reabsorption of phosphate but normal renal function and vitamin D and parathormone levels. Previously, we reported a novel missense mutation in the FGF23 gene in a patient suffering from HHS. In the present report, we demonstrated the same mutation (c.471C>A) in two other cases of HHS with similar clinical manifestations. As this nucleotide change has not been reported previously, it can be a population specific mutation in Iran that can facilitate carrier testing and prenatal diagnosis of HHS.

First description of a patient with Vici syndrome due to a mutation affecting the penultimate exon of EPG5 and review of the literature.

Vici syndrome is a rare autosomal recessively inherited multisystem disorder characterized by agenesis of the corpus callosum, cataracts, cardiomyopathy, combined immunodeficiency, psychomotor delay, and hypopigmentation. Cullup et al. recently identified mutations in the gene EPG5 as the cause of Vici syndrome. EPG5 is involved in autophagy, an evolutionarily conserved lysosomal degradation process that is essential for cell homeostasis. Following the first description in 1988 by Vici et al., 24 other cases of Vici syndrome have been published with variable expression of the defining features. Here, we report on a further case of Vici syndrome with a homozygous truncating mutation of EPG5, identified by whole-exome sequencing. The mutation in our patient is the first reported affecting the penultimate exon of EPG5 and presenting with typical clinical manifestations of Vici syndrome. Additionally, we present a detailed clinical analysis of Vici syndrome comprising all cases previously described in the literature.

Posterior mediastinal mass: an unusual presentation of pediatric tuberculosis.

Mediastinal mass is a rare presentation of tuberculosis in children. The sources of posterior mediastinal mass are usually neurogenic tumors, infections or vascular lesions. Herein, a 12-year-old girl is presented who manifested with a posterior mediastinal mass extending to the right paravertebral space from T3 to T8 with extension to retrocardiac and subcarinal spaces. She suffered from chronic cough, prolonged fever, and weight loss. The results of the tuberculin skin test and biopsy were compatible with tuberculosis. Mediastinal tuberculosis was confirmed histologically and by polymerase chain reaction. The patient was treated with anti-tuberculosis drugs and surgical intervention.

Computed tomography findings of a patient with severe dysplasia of the inner ear and recurrent meningitis: a case report of gusher ear in a five-year old boy.

Communication between subarachnoid and perlymphatic spaces can be due to a deficiency of lamina cribrosa (stapes gusher). Recognition of the condition may alter the course of treatment that can avoid perilymph gushing. A five-year-old boy presented with a history of congenital hearing loss and recurrent meningitis. The computed tomography (CT) of the temporal bone showed severe bilateral dysplasia in the inner ears in favor of gusher disease.

Hypothalamic hamartoma in an unusual case with delayed puberty.

Hypothalamic hamartoma (HH) is a rare intracranial lesion that usually presents with classic triad of central precocious puberty, gelastic epilepsy, and developmental delay. Herein, a 14-year old boy is presented in whom the diagnosis of HH was made by magnetic resonance imaging. While he did not have any complain of precocious puberty, he surprisingly suffered from delay in puberty. The definite diagnosis of HH can only be made by appropriate imaging, in a case with atypical feature of delay in puberty and in the absence of gelastic epilepsy. To our best knowledge, this is the first case of HH who is presented with delay in puberty as of first manifestation.

Sketched x-rays: Calcinosis universalis.

Calcinosis universalis (CU) is a known complication of dermatomyositis manifesting as calcified nodules and plaques localized in subcutaneous tissue, fascial planes, tendons, or intramuscular regions. We report a case and image of CU in a 9-year-old boy diagnosed with juvenile dermatomyositis (JDM).