Unknown etiology

Articles

• hypertrophic myopathic CIPO
  3 December 2009

  A hypertrophic pattern in VM, dominating the atrophic pattern, is exceedingly rare. As a pattern of injury, hypertrophy is almost absent from the description of visceral myopathy in much of the literature.
  Much of literature on CIP0 and CVM is contained within the clinical and surgical medical specialties and is less well represented in anatomical pathology, and it is possible that the hypertrophic patterns are underreported.
  Clinical synopsis
  hypertrophic pattern of visceral (...)

• neonatal giant cell hepatitis
  15 November 2010

  Synopsis
  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 (...)

• sarcoidosis
  22 September 2003

  Sarcoidosis 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 (...)

• pyoderma gangrenosum
  11 February 2004

  Definition: Pyoderma gangrenosum (PG) is an uncommon, chronic ulcerative condition of the skin that was first described in 1930. It can occur in any age group, but only 4% of the patients are infants or children. An underlying systemic disease is present in approximately 50% of the patients with PG.
  The most common associations include inflammatory bowel disease, arthritis, lymphoproliferative disorders and chronic recurrent multifocal osteomyelitis (CRMO).
  Pyoderma gangrenosum (PG) is a (...)

• adult diffuse nesidioblastosis
  16 May 2008

  Persistent 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 (...)

• autism
  1 October 2003

  Definition: 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 (...)

• eosinophilic granulomatosis with polyangiitis
  8 June 2005

  allergic 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 (...)

• infantile myofibromatosis
  9 February 2006

  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 (...)

• syndromic diarrhea
  10 March 2010

  phenotypic diarrhea; Syndromic diarrhea - Phenotypic diarrhea - Tricho-hepato-enteric syndrome - Intractable diarrhea of infancy with facial dysmorphism - Trichorrhexis nodosa and cirrhosis - Neonatal hemochromatosis phenotype with intractable diarrhea and hair abnormalities - Intractable infant diarrhea associated with phenotypic abnormalities and immune deficiency.

• Sjögren disease
  24 March 2010

  primary 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 (...)

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