Toshinao Yamano

ANTITHYROID ANTIBODIES IN ALPORT'S SYNDROME

Two families in which more than two members had Alports syndrome were examined. Serum-antithyroid-antibodies were found in all those with Alports syndrome and some of their relatives. These included three cases of Alports syndrome with nephritis and/or nerve deafness and two normal people who can be regarded as carriers of Alports syndrome from one family, and one case of the syndrome with nephritis and deafness from the other family. One case of Alports syndrome had symptoms of hypothyroidism, and biopsy specimens from another case were characteristic of chronic thyroiditis. The results indicate that serum-anti-thyroidantibodies, together with nephritis and deafness, are a sign of Alports syndrome. The results accord with transmission of the syndrome as an autosomal dominant trait and suggest that Alports syndrome may be caused by an immunological abnormality.

A case of vasculitic cholecystitis associated with Schönlein-Henoch Purpura in an adult

SummaryA case of Schönlein-Henoch Purpura (SHP) in a 32 year-old female, showing gastrointestinal manifestations including acute vasculitic cholecystitis was reported. In the course of hospitalization urgent laparotomy was performed because of the severe abdominal pain. The gallbladder was inflamed with a brownish-red edematous wall and subserosal hemorrhage, and was resected. Histological examination of the resected gallbladder specimen revealed leucocytoclastic vasculitis. The patient was treated with prednisolone postoperatively, and symptoms abated over two weeks. Acute cholecystitis with SHP is extremely rare, and as far as the authors know this is the second case of this disorder documented by histological examination. Patients with acute abdomen associated with SHP should be managed with consideration of the complications of acute cholecystitis.

Gastroscopic biopsy of the stomach for the diagnosis of amyloidosis

Summary1.Amyloid deposition was investigated in the biopsied specimens obtained from the stomachs of suspected cases of amyloidosis. The subjects were seven cases of suggestive amyloidosis with clinical symptoms of muscle rigidity, masked face, macroglossia and B-J proteinuria. The stomach of an autopsied amyloidosis patient was also investigated.2.Mucosal tissue was obtained from fundus, corpus and antrum of the stomach using gastrofiberscope, Olympus GFB2. And amyloid in the tissue was detected by H & E, Congo red staining and polarization microscopy. Rectal biopsy was also done in the majority of the cases to compare with stomach biopsy.3.Positive results were obtained with gastric biopsy in all seven cases and in five of the 5 cases by rectal biopsy. Thus, gastric and rectal biopsy yielded the same results so far as detection of amyloid deposition was concerned.4.Biopsy specimens from several sites of the stomach gave similar results in regard to the amyloid deposition. Tissue deposition of amyloid in the stomach wall showed a somewhat high incidence in the walls of intramucosal capillaries and in the walls of blood vessels of lamina muscularies mucosa. Gastroscopic biopsy of the stomach proved to be useful as a routine technique for the definite diagnosis of the disease.

Plasma apolipoprotein H (β2-glycoprotein I) phenotype frequencies in a Japanese population

SummaryWe determined plasma apolipoprotein H (β2-glycoprotein I) levels in 300 healthy adult individuals and evaluated the frequencies of the BgN and BgD alleles in a Japanese population. These results were then compared with the previous reports. The plasma apo H levels in the subjects showed bimodal distribution: 274 subjects were in the range 15.6–33.2 mg/dl and were considered to be homozygous for BgN (phenotype NN), and 26 subjects were found in the range 9.6–14.8 mg/dl and were presumably heterozygous for BgN and BgD (phenotype ND). In this study, no sample below 5 mg/dl (phenotype DD) was found. Mean plasma apo H levels in NN and ND groups were 22.1±1.6 mg/dl and 12.5±1.6 mg/dl, respectively. The gene frequencies of BgN and BgD in a Japanese population were 0.957 and 0.043, respectively. These results were similar to gene frequencies of BgN and BgD in Caucasoids.