Definition: Infantile myofibromatosisis is defined by the presence of multiple infantile myofibromas. Infantile myofibromatosis is a term coined by Chung and Enzinger.
It exclusively affects infants and young children, with 60% of cases noted at birth or shortly thereafter and more than 80% occurring before the age of 2 years.
Infantile myofibromatosis is one of the most commonly occurring tumors of the neonatal period, presenting in both solitary and multicentric forms.
Most solitary (...)
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Unknown etiology
Articles
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infantile myofibromatosis
9 February 2006 -
balanitis xerotica obliterans
26 October 2007Balanitis xerotica obliterans (BXO)
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eosinophilic granulomatosis with polyangiitis
8 June 2005allergic granulomatous vasculitis, Churg-Strauss syndrome; Churg-Strauss disease; EGPA
Cases
Digital Case 198 (HPC:198)
Clinical synopsis
fever
cough
expectorations
asthenia
bilateral pulmonary infiltrates.
Synopsis
eosinophilic vasculitis
eosinophilic perimyocarditis
eosinophilic pericardial effusion
eosinophilic pleural effusion
peripheral blood eosinophilia
pulmonary anomalies pulmonary eosinophilic vasculitis eosinophilic pneumonia (pulmonary eosinophilic (...) -
Rosai-Dorfman disease
16 December 2003sinus histiocytosis with massive lymphadenopathy
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sarcoidosis
22 September 2003Sarcoidosis is a granulomatous disorder of unknown etiology, associated with an accumulation of CD4+ T cells and a TH1 immune response. WP
Images
hepatic sarcoidosis https://twitter.com/Hopkins_GI_Path/status/675402892036173826
cutaneous sarcoidosis https://twitter.com/RyanHickMD/status/731249607909351424
Sarcoidosis: all types of giant cells and endogenous inclusions occur, none are specific https://twitter.com/smlungpathguy/status/879435684540538884
Links
Sarcoidosis in (...) -
autism
1 October 2003Definition: Autism is a neurodevelopmental syndrome defined by deficits in social reciprocity and communication and by unusual repetitive behaviors. Although there is an underlying genetic predisposition, the etiology of autism is currently unknown. A recent increase in prevalence suggests that genetically determined vulnerability to environmental exposure might contribute to the causation of autism.
Autism is a common and genetically heterogeneous disorder, with an estimated heritability (...) -
adult diffuse nesidioblastosis
16 May 2008Persistent hyperinsulinemic hypoglycemia (PHH) in adults that is not caused by an insulinoma is a rare and not well-characterized disease that has been named nesidioblastosis.
Diffuse nesidioblastosis in adult patients with PHH resembles that seen in neonates suffering from PHH. The most important criterion for the diagnosis is the beta-cell hypertrophy.
As approximately 4% of adult patients with PHH are affected by diffuse nesidioblastosis, this disease is not as rare as it has been (...) -
neonatal giant cell hepatitis
15 November 2010Synopsis
extramedullary hematopoiesis (74%), including both myelopoiesis and erythropoiesis
portal and lobular inflammation mild-to-absent in 95% of cases
lobular cholestasis (from mild to moderate) with predominantely canalicular pattern (84%).
bile ducts hypoplastic (32%) but not absent or reduced in numbers.
mild focal ductular proliferation (18%)
portal fibrosis or pericellular fibrosis (30%; advanced in 8%).
Idiopathic (49%),
Secondary
hypopituitarism (16%)
biliary (...) -
Sjögren disease
24 March 2010primary Sjögren’s syndrome; Goujerot-Sjögren disease; Sjögren syndrome
Sjogren’s syndrome causes keratoconjunctivitis sicca and xerostomia. It can also cause other exocrine abnormalities such as vaginal dryness, bronchitis, and affect other organs.
Anti-SS-A (Ro) and anti-SS-B (La) antibodies
Anti-SS-A(Ro) antibodies are found in many autoimmune disorders, such as SLE, subacute cutaneous lupus, neonatal lupus, ANA negative lupus, Sjogren’s syndrome, primary biliary cirrhosis, chronic active (...) -
rheumatoid arthritis
6 October 2003rheumatoid polyarthritis
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