Presentation
The present case report is about a 6 year old boy from south India who has been diagnosed as Jaffe-Lichtenstein syndrome (JLS). [ipinnovative.com]
Abstract The case of a 12-year-old girl with Jaffé-Lichtenstein-Uehlinger syndrome is presented. [journals.viamedica.pl]
Covering pathologic conditions by clinical appearance, Oral Pathology: Clinical Pathologic Correlations, 6th Edition uses an atlas-style format to help you identify, diagnose, and plan treatment for oral disease presentations. [books.google.com]
It is our ambition to present a complete survey of all medical phenomena named for a person, with a biography of that person. Disclaimer: Whonamedit? does not give medical advice. [whonamedit.com]
Acronym LIKNS Synonyms SCAR19 Spinocerebellar ataxia, autosomal recessive, 19 Keywords Disclaimer Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. [uniprot.org]
Entire Body System
- Recurrent Respiratory Infections
31 HP:0006070 21 long-tract signs 31 HP:0002423 22 c1-c2 subluxation 31 HP:0003320 Symptoms via clinical synopsis from OMIM®: 57 (Updated 05-Apr-2021) Respiratory: recurrent respiratory infections Hematology: neutropenia Skeletal Spine: c1-c2 subluxation [malacards.org]
infections Frequent infections Frequent, severe infections Increased frequency of infection infections, recurrent Predisposition to infections Susceptibility to infection [ more ] 0002719 Recurrent respiratory infections Frequent respiratory infections [rarediseases.info.nih.gov]
- Single Transverse Palmar Crease
transverse palmar crease 31 HP:0000954 15 recurrent infections 31 HP:0002719 16 increased susceptibility to fractures 31 HP:0002659 17 hirsutism 31 HP:0001007 18 decreased circulating iga level 31 HP:0002720 19 hypoplasia of dental enamel 31 HP:0006297 [malacards.org]
transverse palmar crease 0000954 Synophrys Monobrow Unibrow [ more ] 0000664 Ulnar deviation of finger Finger bends toward pinky 0009465 Showing of 23 | Last updated: 2/1/2021 Find a Specialist Find a Specialist If you need medical advice, you can look [rarediseases.info.nih.gov]
Respiratoric
- Rib Tenderness
Symptoms Bone cyst Facial bone pain Facial bone swelling Facial bone tenderness Fractures Leontiasis ossea appearance Long bone pain Long bone swelling Long bone tenderness Rib pain Rib swelling Rib tenderness Scoliosis Skull pain Skull swelling Skull [fr.wikidoc.org]
Musculoskeletal
- Chest Deformity
Bone manifestations are consequences of fibrous lesions that can lead to pathologic fractures, limping, unequal limp length, chest deformity, skull asymmetry, leontiasis-like appearance, scoliosis. [accessanesthesiology.mhmedical.com]
Bone manifestations are consequences of fibrous lesions that can lead to pathologic fractures, limping, unequal limp length, chest deformity, skull asymmetry, leontiasis-like appearance, and scoliosis. [accesspediatrics.mhmedical.com]
unequal limp length, skull asymmetry, leontiaslike appearance, scoliosis, lordosis, and chest deformity. [whonamedit.com]
Skin
- Hirsutism
[…] the respiratory system 31 HP:0002086 12 ulnar deviation of finger 31 HP:0009465 13 synophrys 31 HP:0000664 14 single transverse palmar crease 31 HP:0000954 15 recurrent infections 31 HP:0002719 16 increased susceptibility to fractures 31 HP:0002659 17 hirsutism [malacards.org]
Dental cavities Tooth cavities Tooth decay [ more ] 0000670 Decreased circulating IgA level 0002720 Downturned corners of mouth Downturned corners of the mouth Downturned mouth [ more ] 0002714 Epicanthus Eye folds Prominent eye folds [ more ] 0000286 Hirsutism [rarediseases.info.nih.gov]
Face, Head & Neck
- Downturned Corners of the Mouth
corners of mouth Downturned corners of the mouth Downturned mouth [ more ] 0002714 Epicanthus Eye folds Prominent eye folds [ more ] 0000286 Hirsutism Excessive hairiness 0001007 Hypoplasia of dental enamel Underdeveloped teeth enamel 0006297 Increased [rarediseases.info.nih.gov]
Neurologic
- Long Tract Signs
[…] infections 31 HP:0002719 16 increased susceptibility to fractures 31 HP:0002659 17 hirsutism 31 HP:0001007 18 decreased circulating iga level 31 HP:0002720 19 hypoplasia of dental enamel 31 HP:0006297 20 metacarpophalangeal joint contracture 31 HP:0006070 21 long-tract [malacards.org]
Hypoplasia of dental enamel Underdeveloped teeth enamel 0006297 Increased susceptibility to fractures Abnormal susceptibility to fractures Bone fragility Frequent broken bones Increased bone fragility Increased tendency to fractures [ more ] 0002659 Long-tract [rarediseases.info.nih.gov]
Workup
Management of suspected heavy metal intoxication involves referral for diagnostic workup. [dentalcare.com]
The workup of a potentially battered child is extremely serious, and involves significant legal and social investigations of the parents. [rad.washington.edu]
Thyroid function tests Serum calcium testing to screen for hyperparathyroidism Erythrocyte sedimentation rate and C-reactive protein measurement are nonspecific screening tests for inflammation See Workup for more detail. [emedicine.medscape.com]
Treatment
Full-color photomicrographs help you identify pathologic elements and provide correct diagnoses and treatment plans. Boxes and tables offer clear, at-a-glance information on the clinical features, diagnosis, and treatment for many conditions. [books.google.com]
Generally, no treatment is required in asymptomatic cases. [dovemed.com]
Conclusions Due to insufficient clinical studies on the treatment of psoriasis in pregnancy, there is no treatment guide. [medichub.ro]
[…] were specific treatment targets compared. [onlinejacc.org]
It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. Our staff consists of biologists and biochemists that are not trained to give medical advice. [uniprot.org]
Prognosis
Prognosis - Lichtenstein syndrome Not supplied. Treatment - Lichtenstein syndrome Not supplied. Resources - Lichtenstein syndrome Not supplied. [checkorphan.org]
The prognosis is dependent upon the severity of the disorder Currently, there are no known methods to prevent Fibrous Dysplasia The types of Fibrous Dysplasia include: Monostotic Fibrous Dysplasia: When only a single bone is involved Polyostotic Fibrous [dovemed.com]
Treatment and prognosis Prognosis is generally poor. If disease is localized, segmentectomy or lobectomy may be offered. In diffuse disease management if generally with conservative measures; liver transplantation may be an option 1. [radiopaedia.org]
Surgical procedures are required for correction of deformities, prevention of pathologic fractures, or eradication of symptomatic lesions. [9] Treatment of malignant transformation is based on the subtype of sarcoma, but the prognosis tends to be worse [emedicine.medscape.com]
Etiology
Although great progress has been made in understanding epidemiology and etiology of the syndrome, much about the syndrome remains mysterious. [books.google.com]
Syringomyelia, scoliosis and idiopathic Arnold-Chiari malformations: a common etiology. Rev Neurol. 1996;24(132):937-59. Royo-Salvador MB. [raredr.com]
Etiology of fibrous dysplasia and McCune-Albright syndrome. Int J Oral Maxillofac Surg 1999;28:366-71. 5. Menon S, Venkatswamy S, Ramu V, Banu K, Ehtaih S, Kashyap VM. Craniofacial fibrous dysplasia: Surgery and literature review. [mjdrdypu.org]
The development of symptoms in people older than 40 years does not exclude irritable bowel syndrome but should prompt a closer search for an underlying organic etiology. [emedicine.medscape.com]
Epidemiology
Although great progress has been made in understanding epidemiology and etiology of the syndrome, much about the syndrome remains mysterious. [books.google.com]
Epidemiology Second most prevalent skeletal disease after osteoporosis Sex : ♂ > ♀ (1.2:1) Age of onset : > 55 years References: [1] [2] [3] Epidemiological data refers to the US, unless otherwise specified. [amboss.com]
MalaCards integrated aliases for Lichtenstein Syndrome: Name: Lichtenstein Syndrome 57 20 58 70 Neutropenia Immunoglobulin Deficiency Peculiar Facies and Bony Anomalies 20 Characteristics: Orphanet epidemiological data: 58 lichtenstein syndrome Inheritance [malacards.org]
I am professor in genetic epidemiology. 2006 Professor in genetic epidemiology. [ki.se]
To elucidate the relative contribution of risk factors of drug, disease, and host to fat redistribution, large epidemiologic studies using multivariate analysis were reviewed. [journals.lww.com]
Pathophysiology
Pathophysiology, evaluation, and treatment. J Bone Joint Surg Am. 2005;87:1848-1864. http://www.ncbi.nlm.nih.gov/pubmed/16085630 Lietman SA, Ding C, Levine MA. [rarediseases.org]
Pathophysiology Caused by sporadic mutation of the GNAS1 gene that encodes the alpha subunit of the stimulatory G protein (G1 medullary bone is replaced by fibrous tissue, which appears radiolucent on radiographs). [accesspediatrics.mhmedical.com]
Pathophysiology PDB is an idiopathic disease. PDB is associated with a high rate of bone remodeling. [amboss.com]
Pathophysiology Traditional theories regarding the pathophysiology of irritable bowel syndrome (IBS) may be visualized as a three-part complex of altered gastrointestinal (GI) motility, visceral hyperalgesia, and psychopathology. [6] More recently, it [emedicine.medscape.com]
The narcotic bowel syndrome: clinical features, pathophysiology, and management. Clin. Gastroenterol. Hepatol. 5, 1126–1239 (2007). 9. Agostini, S. et al. [nature.com]
Prevention
[…] pain * Long bone tenderness * Rib swelling * Rib pain Causes - Lichtenstein syndrome Haematoma Lung cancer Leiomyoma Cyst Bronchial adenoma Xiphoid process Chronic osteomyelitis (type of Osteomyelitis) Paget\'s disease Osteochondroma Fibrous dysplasia Prevention [checkorphan.org]
The prognosis is dependent upon the severity of the disorder Currently, there are no known methods to prevent Fibrous Dysplasia The types of Fibrous Dysplasia include: Monostotic Fibrous Dysplasia: When only a single bone is involved Polyostotic Fibrous [dovemed.com]
There are several options for treating and preventing red man syndrome. Drugs that release histamine in the body can also result in red man syndrome. [medicalnewstoday.com]
Expanded section on bisphosphonate-related osteonecrosis of the jaws helps you effectively manage patients taking bisphosphonates to prevent bone loss, including information on oral complications, risk factors, and benefits. [books.google.com]