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]

At present, the World Health Organization recognizes four variants of these lesions, namely: bone-cement dysplasia, fibrous dysplasia, ossifying fibroma and Familial gigantiform cementoma. [scielo.br]

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]

• Fever

  No fever; characteristic X-ray. The disorder is usually sporadic, but familial cases have been reported. It was described in 1942 by Lichtenstein and Jaffe who reported 15 cases. Schlumberger subsequently reported 67 additional cases. [whonamedit.com]

  The simple type presents with RUQ pain and recurrent attacks of cholangitis with fever and jaundice. The periportal fibrosis type may present with pain or signs of portal hypertension, including hematemesis from esophageal varices 1-3. [radiopaedia.org]

  This can develop into a fever and chills, and the person may have a headache and become dizzy or agitated. The face, eyes, and lips can also swell. [medicalnewstoday.com]

  In Letterer-Siwe disease, a disorder affecting predominantly children under 3 years of age, multiple bone lesions in the skull and facial bones are associated with a rapidly progressive clinical course, with fever, malaise, hepatosplenomegaly, lymphadenopathy [rrnursingschool.biz]

• Camping

  It has now become evident that fibrous dysplasia is a genetic disease caused by somatic activating mutation of the Gsα subunit of G protein-coupled receptor resulting in upregulation of cAMP. [head-face-med.biomedcentral.com]

  Camp, Basilar invagination of the skull — so called platybasia. Surg. Gyn. Obstetr. 74 (1942), 751–754. Google Scholar 8. D'Erico, A., Surgical treatment of hydrocephalus associated with spina bifida (Arnold-Chiari deformity). Yale J. [link.springer.com]

  Griffiths CEM, Camp R, Barker JNWN. Psoriasis. In: Burns TBS, Cox N, Griffiths C, (editors). Rook’s Textbook of Dermatology. Italy: Blackwell Science. 2004; pp. 35.46-35.47. 27. Ostensen M, Forger F. [medichub.ro]

• 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]

• Pathologist

  […] kyphoscoliosis hypogonadism or cryptorchidism ocular malformations cardiovascular malformations giant cell granuloma of the jaw History and etymology It is named after Mario Campanacci, Italian physician (1932-1999), and Henry Lewis Jaffe, American pathologist [radiopaedia.org]

  […] usually associated with pain and the lesions keep increasing in size while FD lesions are generally painless and stop growing in size at bone maturity. [8] Establishing the diagnosis of FD requires close cooperation between clinician, radiologist and pathologist [mjdrdypu.org]

• Osteoporosis

  […] related to Lichtenstein Syndrome: 31 (show all 22) # Description HPO Frequency HPO Source Accession 1 recurrent respiratory infections 31 HP:0002205 2 carious teeth 31 HP:0000670 3 abnormal facial shape 31 HP:0001999 4 anteverted nares 31 HP:0000463 5 osteoporosis [malacards.org]

  Homepage Rare diseases Search Search for a rare disease Lichtenstein syndrome Disease definition Lichstenstein syndrome is characterised by frequent infections associated with osteoporosis, a tendency for fractures and osseous anomalies. [orpha.net]

  المحتويات Chapter 18 The Spectrum of Pediatric Osteoporosis 439 Chapter 19 Osteogenesis Imperfecta 511 Chapter 20 Sclerosing Bone Dysplasias 541 Chapter 21 Parathyroid Disorders 557 Chapter 22 Fibrous Dysplasia 589 Chapter 23 Nutritional Rickets 625 Chapter [books.google.com]

  Skull x-ray : thickening of the diploe ; osteoporosis circumscripta (cotton wool appearance). [amboss.com]

• Flushing

  The skin can appear flushed and become itchy and burning. This can develop into a fever and chills, and the person may have a headache and become dizzy or agitated. The face, eyes, and lips can also swell. [medicalnewstoday.com]

  • Markedly increased the risk for adverse skin events (including flushing, pruritus, acanthosis nigricans, and other types of skin rash). • Increased the risk for other adverse events: • Atrial fibrillation • Gastrointestinal events (including nausea [onlinejacc.org]

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]

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]

There are two main types of clinical studies: Clinical trials determine if a new test or treatment for a disease is effective and safe by comparing groups receiving different tests/treatments. [rarediseases.info.nih.gov]

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]

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]

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]

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]

I am professor in genetic epidemiology. 2006 Professor in genetic epidemiology. [ki.se]

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]

Journal Journal ID (nlm-ta): Pan Afr Med J Journal ID (iso-abbrev): Pan Afr Med J Journal ID (publisher-id): PAMJ Title: The Pan African Medical Journal Publisher: The African Field Epidemiology Network ISSN (Electronic): 1937-8688 Publication date (Electronic [scienceopen.com]

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]

[…] 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]

Odontogenic infection necessitated multiple tooth extractions, which were performed under the preventive protocol for ONJ. The postoperative period was uneventful, and the patient remains under close clinical follow-up. [oooojournal.net]