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Larsen's Syndrome


Presentation

  • Three cases of Larsen's syndrome in siblings are presented to illustrate a paradigm for the age-related treatment of deformity. Each child presented at a different age, with differing degrees of deformity and growth disturbance.[ncbi.nlm.nih.gov]
Disturbance of Growth
  • Each child presented at a different age, with differing degrees of deformity and growth disturbance. Both growth and remodeling potential influenced the choice of treatment and outcome.[ncbi.nlm.nih.gov]
Short Nails
  • nails 38. multiple carpal ossification center 39. wedged vertebrae 40. delayed ability to walk 41. webbed toes (2nd and 3rd toes) 42. elasticity in the skin 43. elasticity of the muscles 44. elasticity of the nerves 45. elasticity of the tendons 46.[tawnymullins77.blogspot.com]
  • nails – – – – – – – Airway abnormality – – – – – – – Cardiac defect – – – – – – – Cryptorchidism – – NA – – NA – Spinal cord compression – – – – – – – Skeletal phenotype Short stature – – – – – Joint laxity Cylindrical fingers – Pectus deformity – –[bmcmedgenet.biomedcentral.com]
Neglect
  • Treatment of bilateral hip dislocations is best neglected. Finally, the spinal status of these children must be monitored throughout their life.[ncbi.nlm.nih.gov]
  • The possibility of a sensorineural hearing impairment as an additional part of this syndrome has been rather neglected in the past. Method: Case report and literature review.[cambridge.org]
Hyperreflexia
  • After anterior and posterior cervical fusion, his preoperative symptoms of weakness, gait dysfunction, and hyperreflexia have improved at 9-month follow-up.[ncbi.nlm.nih.gov]
Cervical Cord Compression
  • Sumitani S, Kameda K, Sone S, Minami R: A case of Larsen syndrome with severe cervical cord compression. No To Hattatsu 1994;26:345–348. Topley JM, Varady E, Lestringant GG: Larsen syndrome in siblings with consanguineous parents.[karger.com]

Workup

  • Special features in the radiologic workup should be sought after so as to plan staged surgical procedures. Conventional principles of planning of fusion levels are inadequate. Early treatment is advocated, as the neurologic compromise is reversible.[ncbi.nlm.nih.gov]

Treatment

  • Neither the natural history nor the optimal treatment of cervical spine anomalies in Larsen's syndrome has been clearly defined.[ncbi.nlm.nih.gov]

Prognosis

  • Prognosis LS does not affect life-expectancy. The documents contained in this web site are presented for information purposes only.[orpha.net]
  • The prognosis of children who have LS is variable and most affected persons have moderate symptoms that can be treated, allowing for a relatively normal life span. In severe forms of LS the prognosis can be poor.[jcnonweb.com]
  • Prognosis The effects of the syndrome vary markedly from person to person. Therefore, prognosis is based on the findings in a given individual.[encyclopedia.com]
  • What is the general prognosis for Larsen Syndrome? If Larsen Syndrome is caught early, most individuals can lead healthy lives with normal lifespans.[checkorphan.org]
  • [ edit ] While Larsen syndrome can be lethal if untreated, the prognosis is relatively good if individuals are treated with orthopedic surgery, physical therapy, and other procedures used to treat the symptoms linked with Larsen syndrome. [7] See also[en.wikipedia.org]

Etiology

  • These novel congenital anomalies further support the etiology of Larsen's syndrome as a generalized mesenchymal disorder, since they arise from a defective process of embryonal induction involving mesenchymal tissue.[ncbi.nlm.nih.gov]
  • Their etiology is not precisely century with the first clinical recognition of known, so we have included chapters that dis symptoms and patterns for diagnosis of car cuss many aspects of congenital cardiac mal diovascular diseases.[books.google.com]
  • Etiology LS is due to missense mutations or small in-frame deletions in the FLNB gene (localized to 3p14.3) that encodes cytoskeletal protein filamin B.[orpha.net]
  • Its Etiology and Natural History’, Journal of Bone & Joint Surgery, December 1978, vol. 60, no. 8, p. 1113-1116.[physio-pedia.com]
  • M92.61 - Juvenile osteochondrosis of tarsus, right ankle M92.62 - Juvenile osteochondrosis of tarsus, left ankle Sindig-Larsen-Johansson Disease ICD-9 732.4 (juvenile osteochondrosis of the lower extemity, exlcuding foot) Sindig-Larsen-Johansson Disease Etiology[eorif.com]

Epidemiology

  • Epidemiology Larsen's syndrome with the same frequency develops both in men and in women. By very rough estimates, this disease occurs in a newborn of a hundred thousand. This, fortunately, is a rarity.[acikgunluk.net]
  • Summary Epidemiology The birth prevalence of Larsen syndrome in Europe is 1/250,000 live births.[orpha.net]
  • Juvenile osteochondrosis of tarsus, right ankle M92.62 - Juvenile osteochondrosis of tarsus, left ankle Sindig-Larsen-Johansson Disease ICD-9 732.4 (juvenile osteochondrosis of the lower extemity, exlcuding foot) Sindig-Larsen-Johansson Disease Etiology / Epidemiology[eorif.com]
  • […] anterior knee pain at the inferior pole of patella at the proximal patella tendon attachment similar to Osgood-Schlatter's disease which is at the distal attachment of the patella tendon differnt from Jumper's Knee which is tendonitis of the patella tendon Epidemiology[orthobullets.com]
  • Epidemiology Frequency United States The incidence is not known. International The prevalence is estimated to be 0.4 cases per 100,000 people in Sweden. [19] The prevalence elsewhere is not known.[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • […] which is at the distal attachment of the patella tendon differnt from Jumper's Knee which is tendonitis of the patella tendon Epidemiology demographics more common in adolescence Location patellar tendon insertion at the inferior pole of the patella Pathophysiology[orthobullets.com]
  • Pathophysiology Osteochondroses all involve a defect in ossification.[patient.info]
  • Tracheomalacia and bronchomalacia in children: Pathophysiology, assessment, treatment and anaesthesia management. Paediatr Anaesth 2003;13:3-11. 6. Saricaoglu F, Dal D. Cardiac arrest in a patient with Larsen syndrome under sevoflurane anesthesia.[joacp.org]
  • The ichthyosis (usually evident at birth) may be seen in some patients after the first year of life. [1] Pathophysiology Sjögren-Larsson syndrome (SLS) is due to deficient activity of fatty aldehyde dehydrogenase (FALDH), an enzyme required to oxidize[emedicine.medscape.com]

Prevention

  • The surgeon who undertakes the correction or prevention of these deformities must face the problem of and, it is hoped, use, growth potential from open physes and the progressive loss of remodeling capacity with advancing age.[ncbi.nlm.nih.gov]
  • How Can You Prevent SLJ Syndrome? The most important thing you can do to prevent getting SLJ is to stop doing an activity that causes pain in your knee at the first signs of irritation. Then, try to limit your activity until the pain goes away.[kidshealth.org]
  • Learn more about how to prevent ACL injuries with our ACL Injury Prevention Program A discoid meniscus is an abnormality of the structure of the meniscus.[childrensortho.com]

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