Metachromatic leukodystrophy radiopaedia

Metachromatic leukodystrophy - Radiopaedi

  1. Metachromatic leukodystrophy (MLD) is the most common hereditary (autosomal recessive) leukodystrophy and is one of the lysosomal storage disorders
  2. Case Discussion. Metachromatic leukodystrophy is the most common leukodystrophy. It can present as late infantile, juvenile, and adults forms. Other differentials initially considered given the acute presentation was ADEM and acute hypoxic-ischemic insult. Clinical and laboratory evaluation confirmed Metachromatic leukodystrophy.. Other MRI findings of affected areas on T2WI may show a.
  3. This is a case of adult onset metachromatic leukodystrophy which, although uncommon, is one of the most common adult leukodystrophies. Of note, the MRS findings are atypical, in that choline is usually depressed. Myo-inositol elevation is characteristic 1-2
  4. This case shows dysmyelinating white matter disease, with the above described conventional and MR spectroscopic criteria are typical of metachromatic leucodystrophy (MLD). References Cheon JE, Kim IO, Hwang YS et-al. Leukodystrophy in children: a pictorial review of MR imaging features

Age, clinical symptoms and MRI tigroid pattern were highly suspicious of metachromatic leukodystrophy. Metabolic analyzes were performed in another tertiary-care center and confirmed the diagnosis Metachromatic leukodystrophy is an inherited condition characterized by the accumulation of fats called sulfatides in cells, especially cells of the nervous system. This accumulation results in progressive destruction of white matter of the brain, which consists of nerve fibers covered by myelin Metachromatic leukodystrophy is an autosomal recessive disorder caused by a deficiency of the lysosomal enzyme arylsulfatase A (, 4 -, 8). This enzyme is necessary for the normal metabolism of sulfatides, which are important constituents of the myelin sheath Metachromatic leukodystrophy is an autosomal recessive lysosomal condition due to arylsulfatase A (ARSA) gene mutations, resulting in deficiency of the enzyme arylsulfatase A (ASA) that leads to accumulation of 3- O -sulfogalactosylceramide (sulfatide) in oligodendrocytes, Schwann cells, and some neurons (29, 30)

Metachromatic leukodystrophy (MLD) is a rare hereditary disease characterized by accumulation of fats called sulfatides. This causes the destruction of the protective fatty layer (myelin sheath) surrounding the nerves in both the central nervous system and the peripheral nervous system Metachromatic leukodystrophy is an inherited disorder characterized by the accumulation of fats called sulfatides in cells. This accumulation especially affects cells in the nervous system that produce myelin, the substance that insulates and protects nerves. Nerve cells covered by myelin make up a tissue called white matter Radiopaedia • CC-by-nc-sa 3.0 • de Metachromatic leukodystrophy (MLD) is the most common hereditary (autosomal recessive) leukodystrophy and is one of the lysosomal storage disorders . It has characteristic imaging features including peri-atrial and to a lesser extent frontal horns leukodystrophy as well as periventricular perivenular.

Mar 12, 2020 - Metachromatic leukodystrophy (MLD) is the most common hereditary (autosomal recessive) leukodystrophy and is one of the lysosomal storage disorders. It has characteristic imaging features including peri-atrial and to a lesser extent frontal horns.. Metachromatic leukodystrophy (MLD) is an autosomal recessive disorder caused by mutations in the ARSA gene, which encodes arylsulfatase A enzyme. Arylsulfatase A deficiency leads to impaired breakdown of sulfatides. Sulfatide accumulation in the central nervous system (CNS) leads to myelin breakdown Playlist by user 'noura-alraeesi' (100 entries) ADVERTISEMENT: Radiopaedia is free thanks to our supporters and advertisers

Metachromatic leukodystrophy Radiology - radiopaedia

Organizing pneumonia (OP) Case 13. Eosinophilic granulomatosis with polyangiitis. Case 14. Acute pulmonary edema. Case 15. Kaposi sarcoma of lung. Case 16. Spectrum of fibromuscular dysplasia - acute vertebral artery occlusion, internal carotid and renal artery involvement, and renal infarct Metachromatic leukodystrophy | Radiology Case | Radiopaedia.org. This is a case of adult onset metachromatic leukodystrophy which, although uncommon, is one of the most common adult leukodystrophies. Of note, the MRS findings are atypical, in that choline is usually depressed. Myo-inositol elevation is chara.. The radiological findings are typical for Megalencephalic leukoencephalopathy with subcortical cyst (MLC) or Van der Knaap syndrome MLC is a neurodegenerative leukoencephalopathy with genetic mutation affecting astrocyte dysfunction. It is a. Subcortical Leukodystrophy Symptom Checker: Possible causes include Pyruvate Carboxylase Deficiency. Check the full list of possible causes and conditions now! Talk to our Chatbot to narrow down your search Saved from radiopaedia.org. Double cortex syndrome | Radiology Case | Radiopaedia.org. This case illustrates typical and obvious features of double cortex (aka band heterotopia) without obvious lissencephaly. This is likely to be x-linked (female patient) due to mutations of the DXC gene located on the long arm of chromosome X

Metachromatic leukodystrophy Radiology - Radiopaedi

  1. ently in the proband and barely detectable in.
  2. This type of leukodystrophy causes abnormal growth of myelin. The spongy degeneration results in abated tone, regression, enlarged head and limited [6] head control. The condition worsens over time, resulting in stiffness. Life expectancy is limited for Canavan's disease. 3. Metachromatic leukodystrophy (MLD
  3. Metachromatic leukodystrophy (MLD) (OMIM # 250100) is an autosomal recessive lysosomal storage disease with a prevalence of approximately 0.6-1.9 per 100,000, which arises from deficient activity of the enzyme arylsulfatase A (ARSA) (EC . Less commonly, a deficiency in sphingolipid activator protein, saposin B, is the cause
  4. Niemann-Pick disease. Dr Daniel J Bell and Assoc Prof Frank Gaillard et al. Niemann-Pick disease (NPD) is actually a collection of a number of distinct autosomal recessive lysosomal storage diseases. They are divided into two groups of two based on the underlying metabolic deficiency: deficiency of acid sphingomyelinase 1,3,4
  5. Tay-Sachs disease is a hereditary neurodegenerative disorder resulting from excess storage of GM2 ganglioside within the lysosomes of cells. Epidemiology The incidence of the disease is estimated to be 1 in 3,600 in Ashkenazi Jews with a carri..
  6. ant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an autosomal do

Metachromatic leukodystrophy Genetic and Rare Diseases

  1. Baló concentric sclerosis is a rare and severe monophasic demyelinating disease, considered a subtype of multiple sclerosis, appearing as a rounded lesion with alternating layers of high and low signal intensity on MRI, giving it a characteristic 'bullseye' or 'onion bulb' appearance 1,9. On this page: Article: Epidemiology. Clinical presentation
  2. ant cause of debilitating cardioskeletal myopathy and is a lysosomal storage disorder. Epidemiology Although considered rare, the exact incidence is unknown 1. Clinical presentation Danon disease is character..
  3. ant leukodystrophy (ADLD) presenting autonomic dysfunction as the first symptom are reported. We describe detailed MR appearances of the brain in 2 new families and neuropathology in 2 patients and compare the findings with those in other adult-onset leukodystrophies. METHODS: Twenty subjects (12 women and 8 men; age range.
  4. Metachromatic leukodystrophy is the most common form and is caused by arylsulfatase A deficiency. Diffuse symmetric demyelination results, with increased T2/FLAIR signal and global cerebral atrophy. Adrenoleukodystrophy is a peroxisomal disorder due to acyl-CoA synthetase deficiency, which produces cerebral and adrenal cortical dysfunction
  5. Glycogen storage disease (GSD) refers to a number of syndromes which are characterized by a defect in synthesis, metabolism or storage of glycogen. Pathology There are many types of GSD: type I: von Gierke disease type II: Pompe disease type..
Metachromatic leukodystrophy | Image | Radiopaedia

Leukodystrophy in Children: A Pictorial Review of MR

Win an All-Access Pass! Become a new yearly Curie (Radium) or Roentgen (Gold) Radiopaedia Supporter during December and be in the running to win one of four 12-month All-Access Passes. Find out more. Schilder type multiple sclerosis is characterized by 1-2 large demyelinating plaques, which are hyperintense on T2-weighted images, in each cerebral hemisphere, predominantly in the centrum semiovale. Lesions typically are greater than 2 cm in diameter 3. Typically, the cerebral cortex, cerebellum, brainstem, and spinal cord are relatively.

Adult Leukodystrophies: A Step-by-Step Diagnostic Approach

Metachromatic Leukodystrophy - NORD (National Organization

Metachromatic leukodystrophy: MedlinePlus Genetic

Metachromatic leukodystrophy. This condition causes an accumulation of fats in cells. Pancreatobiliary malunion. This condition is a cause of bile duct obstruction. Outlook #5 Exclusion of leukodystrophy (Adrenoleukodystrophy, metachromatic leukodystrophy, etc.).Genetic criteria NOTCH3 mutations localize in exons 2-24 and result in the gain or loss of cysteine residues in the epidermal growth factor-like repeat domain. Cysteine-sparing variants should be carefully evaluated by skin biopsy and segregation studie Tan AP (2017) Pediatric MRI Brain: Normal or abnormal, that is the question. Radiol Diagn Imaging 1: DOI: 10.15761/RDI.1000112. Helen M.Branson(2013) Normal Myelination: A Practical Pictorial Review

metachromatic leukodystrophy pacs - Suche für Radiologe

  1. Gallbladder polyps have only rarely been described in children, in whom they occur either as a primary disorder or in association with other conditions, including metachromatic leukodystrophy, Peutz-Jeghers syndrome, or pancreatobiliary malunion . Polypoid gallbladder lesions can be categorized as benign or malignant . Benign lesions have been.
  2. Metachromatic leukodystrophy, adult form Metachromatic leukodystrophy, juvenile form Metachromatic leukodystrophy, late infantile form Mild Canavan disease Mills syndrome Monomelic amyotrophy [se-atlas.de
  3. ance 25 CT: diffuse hypodensity [radiopaedia.org
  4. [radiopaedia.org] Show info. Metachromatic Leukodystrophy. Six years after diagnosis, the other control has a slowly progressive course with spastic dystonic quadriplegia, epilepsy, dysphagia, continual drooling and incontinence. [ncbi.nlm.nih.gov
  5. GM1 gangliosidoses; Other names: GM1 gangliosidosis: Specialty: Endocrinology : The GM1 gangliosidoses are caused by a deficiency of beta-galactosidase, with resulting abnormal storage of acidic lipid materials in cells of the central and peripheral nervous systems, but particularly in the nerve cells

Mar 12, 2020 - Central nervous system manifestations of systemic lupus erythematosus (CNS lupus) describe a wide variety of neuropsychiatric manifestations that are secondary to systemic lupus erythematosus (SLE) in the central nervous system (CNS). For a gene.. See what RADIOLOGY IMAGING AND DIAGNOSIS (tauqeeranjum) has discovered on Pinterest, the world's biggest collection of ideas Jul 26, 2019 - This patient has characteristic and near pathognomonic features of tuberous sclerosis. An easy diagnosis if one is aware of the entity Adult Metachromatic Leukodystrophy & Cerebellar Ataxia & Cluster Headache Symptom Checker: Possible causes include Myelocerebellar Disorder. Check the full list of possible causes and conditions now! Talk to our Chatbot to narrow down your search

Pelizaeus-Merzbacher Disease Symptom Checker: Possible causes include Hypomyelinating Leukodystrophy Type 5. Check the full list of possible causes and conditions now! Talk to our Chatbot to narrow down your search Antoine Henri Becquerel (15 December 1852 - 25 August 1908) was a French physicist, Nobel laureate, and the first person to discover evidence of radioactivity. For work in this field he, along with Marie Skłodowska-Curie and Pierre Curie, [2] received the 1903 Nobel Prize in Physics Radiology Signs Exhibition. 3,177 likes · 29 talking about this. Educatio The word 'lacunar' in 'lacunar infarct' refers to minor damages with a diameter of 1 to 15 millimeters that are called 'lacunes'. They appear to be small holes in the deeper brain areas, for example the areas of the brain stem or of the basal ganglia. They are also often seen in the fiber web (internal capsule) of white matter which is. Jun 11, 2014 - Patient history and MR findings are in keeping with acute phase of hemiconvulsion-hemiplegia epilepsy syndrome. Aggressive anticonvulsive therapy is important to prevent/milden developing hemiatrophy and permanent epilepsy. Possible development..

Symptoma is a Digital Health Assistant & Symptom Checker. Patients and doctors enter symptoms, answer questions, and find a list of matching causes - sorted by probability. Symptoma empowers users to uncover even ultra-rare diseases Dysmyelinating-leukodystrophy Symptom Checker: Possible causes include Autosomal Recessive Spastic Paraplegia Type 35. Check the full list of possible causes and conditions now! Talk to our Chatbot to narrow down your search Jun 2, 2014 - This Pin was discovered by Dr Who. Discover (and save!) your own Pins on Pinteres A cerebral cavernous malformation (CCM) is a blood vessel malformation, consisting of a tangle of, in particular, veins in the brains. A cavernous malformation can also be found in the spinal cord See what Usha Shankar (ushaksdr) has discovered on Pinterest, the world's biggest collection of ideas

Metachromatic leukodystrophy (MLD—OMIM 250100) is a severe neurodegenerative metabolic disorder, also classified as a lysosomal storage disorder. It is caused by deficient activity of arylsulfatase A CEO and Editor of Radiopaedia.org, a collaborative online radiology resource Progressive familial intrahepatic cholestasis (PFIC) is a group of inherited conditions; each condition is caused by a faulty gene. In children with PFIC, cells in the liver cannot release bile, a fluid that helps the body digest food. As a result, bile builds up in the liver, leading to symptoms such as jaundice, itching, impaired growth, and. 1120 ELSTER AND RICHARDSON AJNR:11, November/December 1990 A B c D No patient in this series had symptoms or signs referable to the midbrain, as determined by review of a three-page medical question The purpose of this study was to evaluate the clinical features and the characteristics of MR images of patients with end-stage hepatic failure. We reviewed the MR findings and clinical features of 31 consecutive patients (20 men, 11 women=31, mean age 58.7 years) who had been diagnosed with clinical hepatic encephalopathy. Associations between the lesion locations on each MR sequence were.

Metachromatic leukodystrophy is the most common hereditary disorder of myelin formation and maintenance that results in diffuse symmetrical demyelination, with increased signal on T2-W fluid-attenuated inversion recovery (FLAIR) MR images, global cerebral atrophy and a thin corpus callosum Child male 3 years old with metachromatic leukodystrophy (A): axial T2 FLAIR images of the brain revealed bilateral symmetrical confluent areas of signal change in periventricular white matter with characteristic tigroid appearance, (B & C) MRS at long and short TR shows high myoinositol (mI) level (thick white arrow) and Glx (thin white arrow. Kim TS, Kim IO, Kim WS, et al. MR of childhood metachromatic leukodystrophy. AJNR 1997; 18:733-738 [Google Scholar] 29. Barkovich AJ. Pediatric neuroimaging, 4th ed. Philadelphia, PA: Lippincott Williams & Wilkins, 2005:83 [Google Scholar] 30. Given CA 2nd, Santos CC, Durden DD. Intracranial and spinal MR imaging findings associated with. Notably, four PRES patients had known leukodystrophies, two with metachromatic leukodystrophy and two with adrenoleukodystrophy. All four received cyclosporine after bone marrow transplantation. The cortical and subcortical white matter edema was readily discernible from the underlying deep white matter inflammation (Fig. 7A , 7B ) Vanishing White Matter Disease (VWM) is a genetic disorder that affects the nervous system and causes neurologic symptoms. Although initially recognized as a disease of young children, it is now known that the disease has a highly variable course with a wide range in severity. A striking feature of VWM is that in addition to a generally slow.

brain stem nuclei, and the cerebellum, such as metachromatic leukodystrophy, dentatorubropallidoluysian atrophy, and the spinocerebellar ataxias. Conclusions: Neuropsychiatric symptoms are integral to a thorough phenomenological account of hyperkinetic movement disorders. Drawing on moder Multidetector CT and MR imaging of diffuse liver disease are discussed, with emphasis on their roles in evaluation of specific parenchymal hepatic diseases, which can be categorized as storage, vas.. leukoencephalopathy: [ loo″ko-en-sef″ah-lop´ah-the ] any of a group of diseases affecting the white substance of the brain. The term leukodystrophy is used to denote such disorders due to defective formation and maintenance of myelin in infants and children z Tigroid pattern • Spares subcortical U fibers. • Low T2 thalamus.MLD Metachromatic leukodystrophy • Early involvement of the subcortical U fibers. • Typically involve posterior limb of internal capsule. PMD Pelizaeus merzbacher disease . 42. z Reverse Tigroid pattern Pachygyria Frontal cortical thickening (double arrow) 43

Neurospot 1. Neurospot 17.06.2016 THORSANG CHAYOVAN R3/AJ. NUTTHA SANGHAN 2. Quiz 1: Mount Fuji • Bilateral subdural hypertensive pneumocephalus • Compression of the frontal lobes • Neurosurgical emergency • Differentiation between hypertensive VS non-hypertensive • Hypertensive pneumocephalus afters • Subdural hematomas drainage • Skull base, paranasal sinuses and posterior. Introduction to Neuropathology. Introduction to the histology and pathology of the nervous system. Brief overview of most common brain cancers and histological changes of neurons and glial cells. emerges as anterior rootlets from the anterior lateral sulcus and conveys motor output from visceral and somatic motor neurons The Radiopaedia.org blog received 32,675 page views, which was 13.6 and 10.7 times greater than AJNR and AJR page views, respectively, and 6.0 times greater than both journal articles combined. and the cerebellum, such as metachromatic leukodystrophy, dentatorubropallidoluysian atrophy, and the spinocerebellar ataxias. Neuropsychiatric.

Metachromatic leukodystrophy Radiology Reference Article

Megalencephalic leukoencephalopathy with subcortical cysts, also known as Van der Knaap disease, refers to a rare inherited autosomal recessive disease characterized by diffuse subcortical leukoencephalopathy associated with white matter cystic degeneration.. Epidemiology. The age at symptoms manifestations ranges from birth to 25 years, with a median age of 6 months In metachromatic leukodystrophy, MRI reveals a hyperintense signal in the periventricular and central white matter on T2 weighed images, which may initially be limited to parieto-occipital region and contrast enhancement is not a feature of this disease1,6,8,9. Canavan disease is characterized by a combination of macroencephaly, extensive.

Leukoencephalopathies and metabolic diseases comprise a great number of heterogeneous disorders. Diagnosis of these disorders may be challenging at times, requiring sophisticated laboratory investigations. Magnetic resonance imaging (MRI) is useful in supporting diagnosis even though it bears problems of specificity. Quantitative MRI techniques, providing information on cerebral metabolites. Differential diagnostic considerations for acute central nervous system demyelination in children include acute disseminated encephalomyelitis (ADEM), multiple sclerosis (MS), optic neuritis, transverse myelitis, neuromyelitis optica (Devic disease), and various infectious, metabolic, and rheumatologic conditions ( table 1 ) Macrocephaly, and Leukodystrophy. If you liked this article maybe you will also find interesting the following in-depth articles about other rare diseases, like Microphthalmia and Ectodermal dysplasia, related diseases and genetic alterations Fever and Macrotia, related diseases and genetic alteration Wu C, Sun Q, Fan D. Serial Magnetic Resonance Imaging Changes in a Patient With Late-Onset Cobalamin C Disease With a Misdiagnosis of Metachromatic Leukodystrophy. JAMA Neurol 2018; 75:374. Motte J, Kneiphof J, Straßburger-Krogias K, et al. Hereditary defect of cobalamin metabolism with adolescence onset resembling multiple sclerosis: 41-year. Nov 12, 2019 - Explore shams iqubali's board Radiology, followed by 527 people on Pinterest. See more ideas about radiology, radiology imaging, radiography

Metachromatic leukodystrophy - VisualD

The inner capsula. The capsula interna is an important nerve, fiber bundle between the two brain hemispheres and therefore an important connection to the cerebral cortex, the corpus callosum and the little brain (cerebellum). It is also an important link connecting the thalamus to the prefrontal cortex. The capsula interna contains nerves which. Demyelination describes a loss of myelin with relative preservation of axons. Demyelination results from diseases that damage myelin sheaths or the cells that form them. Demyelination diseases should be distinguished from those in which there is a failure to form myelin normally (sometimes described as dysmyelination) Radiopaedia • CC-by-nc-sa 3.0 • de Canavan disease , also known as spongiform degeneration of white matter (not to be confused with Creutzfeldt-Jakob Disease ) or aspartoacylase deficiency , is a leukodystrophy clinically characterized by megalencephaly, severe mental and neurological deficits, and blindness This causes electricity transmission. Possible physical problems after lightning strike or an electric shock. Heart attack or irregular heartbeat. Respiratory arrest. Loss of consciousness to coma. Perforated eardrums. Eye injuries like cataract. Temporary paralysis legs / feet are blue. Impairment in sensory perception for example stinging.

Abstract. Inflammatory and infectious disorders of the spine and spinal cord are less common in children than in adults. They are classified according to the involved spinal compartments into those predominantly affecting (i) the spinal cord, (ii) the nerve roots and meninges, and (iii) the vertebrae, discs, and epidural space Subcortical U-fibers, also known as short association fibers, represent connections between adjacent gyri of the brain, located within the cortex or immediately deep to it in the very outer parts of the subcortical white matter. They are among the last parts of the brain to myelinate, as late as the third or fourth decade and have very slow myelin turnover

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The subdural space (or subdural cavity) is a potential space that can be opened by the separation of the arachnoid mater from the dura mater as the result of trauma, pathologic process, or the absence of cerebrospinal fluid as seen in a cadaver. ( wikipedia.org) In the cadaver, due to the absence of cerebrospinal fluid in the subarachnoid space. Gaucher disease is an inherited condition characterized by insufficient levels of the enzyme glucocerebrosidase, also called beta-glucosidase.. It's named for the French physician, Philippe Gaucher, who first described the condition.. Glucocerebroside is a glycolipid, which is a molecule containing both sugar and fat, that's included in the membrane of many different cells

Xanthoma Xanthomas are caused by the deposition of the lipids in dermal macrophages, forming yellow-pink lesions visible to the eye. They are associated with hyperlipidemia.. Types of Xanthomas Caused by Hyperlipidemia By Chris Gromisch View Comments Anonymous commented 6 years ago. Pseudoxanthoma elasticum facts Pseudoxanthoma elasticum (PXE) is a rare disorder involving degeneration of the. Onset of is less painful and dramatic as compared to gout. 50% of cases affect the knees, while other cases involve the elbows, wrists, hips and shoulders. More common in elderly. Aspirate, fluid-analysis, NSAIDs, wt mgt, exercise, ambulation aids. shams iqubali

Metachromatic leukodystrophy | Radiology Case

Neurodegenerative Learning Pathway Radiopaedia

Subdural space.The subdural space (or subdural cavity) is a potential space that can be opened by the separation of the Hence, the subdural space is referred to as potential or artificial space.Subdural space.Diagrammatic representation of a section across the top of the skull, showing the membranes of the brain, etc Haines, Duane E.; Harkey, H. Louis; Al-Mefty, Ossama (1993) Demyelination is incorrectly often equated to multiple sclerosis, whereas in reality it is a generic pathological term simply describing, as the word suggests, the loss of normal myelin around axons in the central nervous system.This should be distinguished from dysmyelination where the formation of normal myelin is absent.. Primary. Primary demyelinating disorders include An enzyme that catalyzes the hydrolysis of cerebroside 3-sulfate (sulfatide) to yield a cerebroside and inorganic sulfate. A marked deficiency of arylsulfatase A, which is considered the heat-labile component of cerebroside sulfatase, has been demonstrated in all forms of metachromatic leukodystrophy (LEUKODYSTROPHY, METACHROMATIC). EC

Metachromatic Leukodystrophy Differential Diagnose

The term concussion originates from the latin verb 'concutere' meaning: dash together or shake violently.. A concussion is a 'mild' form of brain injury following a blow to the head or after a fall in which the brains are shuffled back and forth nephritis hereditary. Web. Medical Information Search. Analytical, Diagnostic and Therapeutic Techniques and Equipment Stay current on the developments and research surrounding neonatal anemia from the entirely new chapter on Blood and Hematopoietic System highlights. [books.google.com] 1 2 Diamond-Blackfan Anemia 10 2 Diamond-Blackfan Anemia 11 2 Diamond-Blackfan Anemia 12 2 Diamond-Blackfan Anemia 3 2 Diamond-Blackfan Anemia 4 2 Diamond-Blackfan Anemia 5 2 Diamond-Blackfan Anemia 6 2 Diamond-Blackfan Anemia.

Presentation1, radiological imaging of pediatric