Seven cases of chronic sensorimotor polyneuropathy due to amyloidosis, from 7 different families, are described, in addition to the pathology in a sibling of 1 case. The age of onset ranged from 55 to 72 years. Cardiac involvement, intermittent diarrhoea and syncopal attacks were a frequent occurrence. Motor conduction velocity showed a moderate degree of slowing in 5 of 6 cases studied and marked slowing in 1. Amyloid deposits were seen in nerve biopsy material of all 8 subjects and in rectal mucosa from 1. Immunohistochemical identification revealed AF (transthyretin-derived)--amyloid in all 8 instances, confirming the presence of type 1 familial amyloid neuropathy. The genealogical data supported this analysis. Six of the 8 cases originated in a small area of the north-west coast of County Donegal in Ireland. The remaining cases also originated in the same county.

Aged↗

PubMed1987

Mucin production in cervical intraepithelial neoplasia and in stage 1b carcinoma of cervix with pelvic lymph node metastases.

Sections from cervical intraepithelial neoplasia (CIN 3) and stage 1b carcinoma of the cervix were stained with periodic acid-Schiff (PAS) and alcian blue to identify the presence of intracellular mucin. One out of seven specimens of CIN 3 demonstrated intracellular mucin. In a series of 33 patients with stage 1b carcinoma of the cervix with pelvic lymph node metastases, PAS demonstrated intracellular mucin staining patterns that were similar in both tumour and lymph node in most patients. In the subgroup of 23 patients classified as having squamous carcinoma, 8 (35%) demonstrated intracellular mucin to some degree and only 2 (25%) of those 8 were alive after 3 years compared with 13 of the other 15 (87%) who did not show any mucin staining (P less than 0.01).

Adenocarcinoma↗

PubMed1987

Spirochaetosis of the human rectum associated with an intraepithelial mast cell and IgE plasma cell response.

In two patients presenting with mild intestinal symptoms, rectal spirochaetosis was the only morphological abnormality diagnosed by light microscopy. A re-evaluation of the morphological changes using electron microscopy and immunohistochemistry showed certain unusual features: the microorganisms were observed within epithelial cells and in subepithelial macrophages; there were numerous partially degranulated intraepithelial mast cells; and there was a marked increase in the proportion of IgE plasma cells within the lamina propria. Mucosal penetration by the organisms may be responsible for the unusual immune response. In one patient, treatment with antibiotics eliminated the spirochaetes and resulted in a clinical improvement. Spirochaetes should not always be considered as harmless commensals in the colon.

Adult↗

PubMed1987

Immunocytochemical analysis of HLA class II (DR) antigens in liver disease in man.

The in situ distribution of the major histocompatibility (HLA) class II (DR) antigens was studied in 113 liver biopsy specimens and five livers obtained at necropsy, using monoclonal antibody CR3/43. In 20 normal livers HLA-DR antigens were not detected in bile duct epithelium, hepatocytes, or portal vein endothelium. Normal arteriolar, sinusoidal and central venous endothelium often expressed HLA-DR. Kupffer cells always expressed these antigens. HLA-DR positive spindle cells were identified in the connective tissue of portal tracts, large hepatic veins, and liver capsule: most shared antigens common to all leucocytes and reacted with the histiocytic maker EBM11. Bile duct epithelium expresses HLA-DR in primary biliary cirrhosis, large duct obstruction, and drug induced cholestasis, indicating that HLA-DR positive spindle cells are phenotypically similar to histiocytes.

Antibodies, Monoclonal↗

PubMed1987