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management rheumatology amyloidosis antinxp2 antinxp2dm calcifications clincial dermatology dermatomyositis encephalopathy infant lichen neurology photo posterior pres radiology reversible sjogrens stages
Plain radiography of the abdomen revealed calcification of both adrenal glands. A homozygous mutation in LIPA
confirmed the diagnosis ... deficiency, or Wolman’s disease ... and gut, which causes ... #clincial #peds ... #infant #NEJM #
Amyloidosis - Summary Group of disorders associated with extracellular deposition of fibrils formed from low-molecular-weight proteins in
Clinical suspicion ... the extent of disease ... Amyloidosis Clinical ... : • Nephrotic syndrome ... Carpal tunnel syndrome
Lichen Planus- ...On examination, numerous purple papules were noted on the anterior surface of the forearms
confirming the diagnosis ... Although the cause ... an autoimmune disease ... #NEJM #clinical ... #derm #wickhams
Posterior Reversible Encephalopathy Syndrome (PRES) Overview Clinico-Radiological Syndrome, characterized by: • Headache • Seizures • Altered mental
Shock, Autoimmune disease ... epilepticus Treatment ... the underlying cause ... • Treatment of ... until cause identified
Syphilis - Clinical Manifestations and Treatment 1) Primary Syphilis • Direct lesion contact during sex is responsible
Syphilis - Clinical ... responsible for most cases ... loss • Skin: Rash ... Albuminuria, nephrotic syndrome ... Syphilis #stages #diagnosis
Sjogren's Syndrome Overview Epidemiology: • F > M: 9:1 • 5-6th Decades (can be any age) Autoimmune exocrinopathy multisystemic disease
salivary and lacrimal glands ... Diagnosis: • Dry ... Hypocomplementemia • Can cause ... salivary ducts Clinical ... Adenocarcinoma Treatment
Anti-NXP-2 Dermatomyositis What? DM with generalized subcutaneous edema Pts have more myalgias, more severe weakness, and an increased prevalence
Violaceous papules or plaques ... auricular papules Clinical ... Muscle atrophy may cause ... had more severe disease ... positive patients Treatment
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