Transperineal sonographic anal sphincter complex evaluation in chronic anal fissures.

OBJECTIVES: The purpose of this study was to assess the role of transperineal sonography in assessment of pathologic changes to the anal sphincter complex in patients with chronic anal fissures. METHODS: We conducted a prospective case-control study of 100 consecutive patients of any age and both sexes with chronic anal fissures who presented to a colorectal clinic between January 2012 and August 2013 (group A) and 50 healthy volunteers (group B). RESULTS: The most common patterns of radiologic changes to anal sphincters associated with chronic anal fissures were circumferential thickening of the anal sphincter complex in 5 patients (5%), circumferential thickening of the internal anal sphincter in 3 patients (3%), preferential thickening of the internal anal sphincter at the 6-o'clock position in 80 patients (80%) and the 12-o'clock position in 7 patients (7%), preferential thickening of the internal and external anal sphincters in 3 patients (3%), and thinning of the internal anal sphincter in 2 patients (2%). CONCLUSIONS: Chronic anal fissures cause differential thickening of both internal and external anal sphincters, with a trend toward increased thickness in relation to the site of the fissure. Routine preoperative transperineal sonography for patients with chronic anal fissures is recommended, and it is mandatory in high-risk patients.

Manifestations of severe vitamin D deficiency in adolescents: effects of intramuscular injection of a megadose of cholecalciferol.

We recorded the manifestations of severe vitamin D deficiency (VDD) in 40 adolescents before and 3 and 6 months after treatment with a mega dose of cholecalciferol (10 000 IU kg(-1), max 600 000 IU). Significant improvement of symptoms related to VDD was reported in 34/40. Three months after the injection, serus calcium, phosphate, alkaline phosphatase and parathormone were normal in all adolescents with VDD with 25-hydroxyvitamin D (25OHD) level = or >20 ng ml(-1). After 6 months, the majority had 25OHD level <20 ng ml(-1). Two patterns of radiological changes have been recorded with complete healing achieved in all patients after a year of therapy. A mega dose of cholecalciferol is an effective therapy for treatment of VDD in adolescents for 3 months but not for 6 months. Radiographs of the ends of long bones are still valuable tool for diagnosis and follow-up of these patients.

Clinical responses to a mega-dose of vitamin D3 in infants and toddlers with vitamin D deficiency rickets.

OBJECTIVES: Was to investigate the effect of treatment with an IM injection, a mega dose of vitamin D3 (10,000 IU/kg) on the clinical, biochemical and radiological parameters of 40 rachitic children with vitamin D deficiency (VDD) over a period of 3 months. DESIGN: In this prospective study we evaluated the clinical, biochemical and radiological responses of an IM injection of cholecalciferol (10,000 IU/kg) for 3 months. RESULTS: At presentation, the most frequent manifestations were enlarged wrist joints, hypotonia, irritability, cranial bossing, wide anterior fontanel, bow legs, delayed teething and walking and Harrison's sulcus with chest rosaries. Short stature (length SDS < -2) was recorded in 30% of patients. Craniotabes and hypocalcemic tetany were the least common presentations. In VDD children the most frequent biochemical abnormality was high alkaline phosphatase (ALP) (100%), followed by low phosphate (PO(4)) (75%) and low calcium (Ca) (12.5%). One month after treatment, serum Ca, PO(4) and 25(OH)D concentrations were normal. Three months after the injection, serum level of ALP and parathormone (PTH) decreased to normal. The majority of patients (87.5%) had serum 25(OH)D level >or= 20 ng/ml, but some (12.5%) had level <20 ng/ml. Hypercalcemia was not recorded in any patient during the 3-month-period. Significant cure of all symptoms and signs related to vitamin D deficiency had been achieved in all children. Leg bowing showed significant improvement in all patients but was still evident in one third. Complete healing of the radiological evidence of rickets was achieved in 95% of all children. CONCLUSION: An IM injection of a mega dose of cholecalciferol is a safe and effective therapy for treatment of VDD rickets in infants and toddlers with normalization of all the biochemical parameters and healing of radiological manifestations. Measurement of serum 25(OH)D level is highly recommended in all short children with a clear need for a general vitamin D supplementation for all infants and young children in Qatar.

Proposed Scoring System for Evaluating Clinico-radiological Severity of COVID- 19 using Plain Chest X- ray (CXR) changes (CO X-RADS): Preliminary results.

BACKGROUND: Plain chest radiograph (CXR), although less sensitive than chest CT, is usually the first-line imaging modality used for patients with symptomatic SARS-CoV-2 infection. The relation between radiological changes in CXR and clinical severity of the disease in symptomatic patients with COVID 19 has not been fully studied and there is no scoring system for the severity of the lung involvement, using the plain CXR. AIM OF THE STUDY: Current COVID-19 radiological literature is dominated by CT and a detailed description CXR appearances in relation to the disease time course is lacking. We propose an easy scoring system (CO X-RADS) to describe the severity of chest involvement in symptomatic COVID 19 patients using CXR and to correlate the radiological changes with the clinical severity of the disease. PATIENTS AND METHODS: The clinical manifestations and CXR findings were recorded in 500 symptomatic COVID-19 positive patients who were admitted to Hamad Medical Corporation (HMC) COVID-19 designated facility Center from January to June 2020. The severity and outcome of the disease included: intensive care unit admission, need for oxygen therapy, mechanical ventilation. and mortality rate. RESULTS: Most of our symptomatic patients (86.8%) had mild and moderate clinical manifestations. The remaining 13.2% had severe manifestations, including: fever, persistent dry cough, shortness of breath, dyspnea, abdominal and generalized body pains. Based on our radiological scoring system (0 to 10) patients were distributed according to their CXR findings into different categories and according to our suggested (CO X-RADS) severity system into five categories (0 to IV). Patients with mild clinical manifestations showed low scoring in CXR (score 0 up to 4) and they represented 72% of our patients. Patients with moderately severe clinical manifestations showed mainly GGO (scoring 5 and 6) and represented about 14.8% of patients. Patients presented with severe clinical manifestations had obvious lung consolidations at the time of presentation with CXR scoring system ≥ 7 and represented about 13.2% of patients. CONCLUSION: We proposed a simple CXR reporting scoring system (CO X-RADS) to categorize COVID-19 patients according to their radiological severity. This radiological score was correlated well with the clinical severity score of patients. We encourage other centers to test this scoring system in correlation with the clinical status of patients.

Linear growth, growth-hormone secretion and IGF-I generation in children with neglected hypothyroidism before and after thyroxine replacement.

We studied growth hormone (GH) stimulation and insulin-like growth factor -I (IGF-I) generation tests in 15 children with neglected congenital hypothyroidism (CH) (age = 6.4 +/- 4.2 years) and measured their growth parameters for >1 years after starting thyroxine (T4) replacement. One year after treatment, height SDS (HtSDS) increased from -4.3 +/- 2.5 to -2.7 +/- 2.3. Peak GH response to clonidine increased from 3.2 +/- 1.2 ng ml(-1) to 7.62 +/- 1.38 ng ml(-1) after treatments. Basal and peak IGF-I response to GH increased from (34.66 +/- 17.3 ng ml(-1) and 58.4 +/- 36.99 ng ml(-1), respectively) before treatment to (130.6 +/- 97.8 ng ml(-1) and 193.75 +/- 122.5 ng ml(-1), respectively). HtSDS increments were correlated significantly with basal free T4 concentrations (r = 0.622, P < 0.01). In summary, after long period of hypothyroidism, T4 replacement produced significant, although incomplete, catch-up growth through a partial recovery of GH- IGF-I axis.

Review of radiologic skeletal changes in thalassemia.

The modern radiologist is unlikely to encounter the classic radiographic features of thalassemia other than in teaching files. The main pathological change that leads to radiological skeletal changes in beta thalassemia major is extensive marrow proliferation. The severity of the skeletal responses is related to the type of thalassemia, the extent and duration of the disease, the type of treatment and the volume of blood transfusions given to the patient, as well as the side effects of transfusion-chelation therapy, and also depends on the bone involved. The radiographic features can be divided into those affecting the skeleton (axial and appendicular) and those occurring extra-medullary. Axial skeletal changes mainly include skull and facial bones, paranasal sinuses, vertebral bodies, weight-bearing bones, while appendicular skeleton manifestations are more pronounced in peripheral bones, mainly hands and feet as well as ribs. Patients on repeated blood transfusions and iron-chelation therapy may demonstrate variable range of manifestations than the scope of untreated patients.

Pros and cons for the medical age assessments in unaccompanied minors: a mini-review.

Unaccompanied minors refer to immigrants who are under the age of 18 and are not under the care of a parent or legal guardian. Age assessment is used in Europe mainly to establish whether or not an individual is under 18 years of age and therefore eligible for protection under the United Nations' Convention on the Rights of the Child (UN - CRC). EU Member States use a combination of techniques to determine the age of a minor and to certify minor status, including interviews and documentation, physical examinations (anthropometric assessment; sexual maturity assessment; dental observation); psychological and sociological assessment; radiological tests (carpal, dental or collarbone x-rays). All such techniques are criticized as they are often arbitrary, do not take into account ethnic variations, and are based on reference materials that are outdated, invasive and may procure harm to the individuals whose age is assessed. They also generate a margin of error that makes them inaccurate to use. There is a debate about the risks and ethics associated with the use of X-rays for non-medical purposes versus the benefits of more accurate age assessments in the interest of justice. It appears that in European countries many individuals carrying out age assessment do not have sufficient training or are not sufficiently independent enough to be carrying out such assessments. Moreover, there is a lack of standardized approach between countries or even within the same country. Only some countries clearly indicate a margin of error in the results of age assessment examinations but there is no consensus - within and among countries - about the width of such margins in relation to each exams applied. It has been advised that the expert report should give the degree of age probability to allow Magistrate to interpret the age assessment results on the 'balance of probabilities' and give the detainee the right to the rule of the 'benefit of the doubt'. It also addresses concerns rested in the convention of the Rights of the Child.

Splenic Abscess: A Rare Complication of the UVC in Newborn.

Splenic abscess is one of the rarest complications of the UVC in a newborn and it is hypothesized that it could be due to an infection or trauma caused by a catheter. The case that is being reported presented with abdominal distension and recurrent desaturation with suspicion of neonatal sepsis versus necrotizing enterocolitis. However, the final diagnosis was splenic abscess as a complication of an inappropriate UVC insertion which was discovered by abdominal ultrasound. The patient was given broad spectrum antibiotics empirically and the symptoms were resolved without any surgical intervention. Such cases and controlled studies need to be reported in order to identify further causes and risk factors associated with splenic abscess in a patient with UVC which can eventually help us adopt preventive strategies to avoid such complications.

Effects of the anti-receptor activator of nuclear factor kappa B ligand denusomab on beta thalassemia major-induced osteoporosis.

INTRODUCTION: Osteoporosis represents the second most common cause of endocrinopathy in patients with beta thalassemia major (BTM). Some drugs proved effective to reduce vertebral and non-vertebral fracture risk. Denosumab is a fully human monoclonal antibody to the receptor activator of nuclear factor kappa B ligand (RANKL), a member of the tumor necrosis factor receptor superfamily essential for osteoclastogenesis. The efficacy and safety of denosumab in BTM-induced osteoporosis has not been tested. OBJECTIVE: To evaluate the efficacy and safety of anti-RANKL on the biochemical and radiological parameters of bone mineralization in patients with BTM-induced osteoporosis. DESIGN: The study population was selected using the random sampling method from the patient's database of our thalassemia clinic. Transfusion-dependent BTM patients above 18 years with no history of treatment with bisphosphonates were randomly selected. Bone mineral density (BMD) of the lumbar spine (LS) and right femoral neck (FN) were measured by dual energy X-ray absorption (DEXA) scan using a calibrated method. Independent factors likely to be associated with low bone mass were determined and included in the analysis to ascertain possible associations. PATIENTS AND METHODS: We studied 30 patients with BTM-induced osteoporosis as per World Health Organization criteria (T Score of less than - 1.0 being defined as osteopenic and a T Score of less than - 2.5 being referred as osteoporotic). 19 males and 11 females aged between 18 and 32 years, with full pubertal development (Tanner's stage 5) at the time of the study. Their mean serum ferritin concentration was 3557 ng ± 1488 ng/ml. Every patient underwent DEXA scan as a baseline and after 12 months of denosumab therapy. Biochemical evaluation including serum concentrations of creatinine, Na, K, calcium, phosphorus, parathormone, bone specific alkaline phosphatase and type 1 collagen carboxy telopetide (ICCT) using enzyme-linked immunosorbent assay (Nordic Bioscience Diagnostics A/S) was done at baseline, after a month and then every 3 months for 12 months after starting denosumab. 60 mg of denosumab was administered subcutaneously twice yearly for a year. The mean BMD T Scores at baseline were -2.7 at the LS and -2.1 at the FN. RESULTS: Denosumab therapy for a year was associated with a significant increase in BMD of 9.2% (95% confidence interval [CI], 8.2-10.1) at the LS and 6.0% (95% CI, 5.2-6.7) at the FN. Denosumab treatment decreased serum ICCT levels by 56% at 1 month and normalized them in all patients at 1 year. Significant correlations were found between BMD T Score before and 1 year after denosumab in LS (r = 0.752, P < 0.001) and FN (r = 0.758 P < 0.001), respectively. The most common side effects were pain in the back and extremities (12%) and nausea (10%). Asymptomatic hypocalcaemia occurred in two patients. CONCLUSION: Denosumab therapy for a year significantly increased BMD density at LS and FN of patients with BTM and was associated with a rapid and sustained reduction in ICCT levels. Further studies are required to confirm long-term effects of this therapy.

Growth and endocrine disorders in thalassemia: The international network on endocrine complications in thalassemia (I-CET) position statement and guidelines.

The current management of thalassemia includes regular transfusion programs and chelation therapy. It is important that physicians be aware that endocrine abnormalities frequently develop mainly in those patients with significant iron overload due to poor compliance to treatment, particularly after the age of 10 years. Since the quality of life of thalassemia patients is a fundamental aim, it is vital to monitor carefully their growth and pubertal development in order to detect abnormalities and to initiate appropriate and early treatment. Abnormalities should be identified and treatment initiated in consultation with a pediatric or an adult endocrinologist and managed accordingly. Appropriate management shall put in consideration many factors such as age, severity of iron overload, presence of chronic liver disease, thrombophilia status, and the presence of psychological problems. All these issues must be discussed by the physician in charge of the patient's care, the endocrinologist and the patient himself. Because any progress in research in the field of early diagnosis and management of growth disorders and endocrine complications in thalassemia should be passed on to and applied adequately to all those suffering from the disease, on the 8 May 2009 in Ferrara, the International Network on Endocrine Complications in Thalassemia (I-CET) was founded in order to transmit the latest information on these disorders to the treating physicians. The I-CET position statement outlined in this document applies to patients with transfusion-dependent thalassemia major to help physicians to anticipate, diagnose, and manage these complications properly.