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Biomedical subjects

K Dohmen

Publications and source records attributed to K Dohmen.

At least 37 records · Page 2Linked to original sources

Post-infantile giant cell hepatitis in an elderly female patient with systemic lupus erythematosus.

A 69-year-old Japanese female was admitted because of general fatigue. Laboratory data showed elevation of serum total bilirubin, transaminase, gamma-glutamyl transpeptidase, and creatinine levels. An immunological study revealed hypergammaglobulinemia, low titer of complement, and high titers of antinuclear antibody, anti-DNA antibody, and circulating immune complexes. Antibodies to parainfluenza virus 3 were positive. Histology of the liver disclosed numerous giant cell hepatocyte transformations with the lobular architecture being slightly distorted by portal inflammation and fibrosis. These findings led us to make a diagnosis of giant cell hepatitis associated with systemic lupus erythematosus. Prednisolone was effective in improving the anemia and the serum immunoglobulin, immune complex, and antinuclear antibody levels. The addition of cyclosporine to the initial corticosteroid therapy was also beneficial in decreasing the transaminase level and in improving liver histology. The patient died of acute pneumonitis and renal failure on the 166th day after admission. Parainfluenza virus 3 and autoimmune mechanisms were thus considered to be the causes of the giant cell hepatitis.

Aged↗

[Two cases of systemic lupus erythematosus associated with fatty liver and Basedow's disease].

We present two cases of systemic lupus erythematosus (SLE) associated with both Basedow's disease and fatty liver. The first case is a 46-year-old Japanese female who was admitted because of high fever and general fatigue. She had been diagnosed as having Basedow's disease and treated with thiamazole for over 4 years. Since thiamazole-induced lupus was unlikely because of high titer anti-nuclear antibody and anti-DNA antibody and low levels of complements, a diagnosis of SLE was made. The upper abdominal ultrasound study and the specimen obtained by liver biopsy performed before initiating steroid therapy demonstrated marked fatty liver. SLE itself is considered as an etiology of fatty liver in this case. The second case was a 25-year-old Japanese female with SLE. She had been treated with prednisolone for 13 years and was complicated with Basedow's disease 10 years later. Fatty liver was also demonstrated in this patient on ultrasonography, and was thought to be resulted from long-term steroid hormone administration.

Adult↗

Retroperitoneal fibrosis associated with scirrhous gastric cancer.

A case of retroperitoneal fibrosis associated with scirrhous gastric cancer is reported. A sixty two-year-old Japanese female was admitted because of acute renal failure. The patient's serum creatinine level showed 3.2 mg/dl while the blood urea nitrogen level was 23 mg/dl. An ultrasound study of the upper abdomen revealed bilateral hydronephrosis. Drip infusion pyelography revealed a dilated right renal pelvis without ureteral obstruction. The left kidney was not opacified, suggesting a functional disorder. Gastrography and gastrofiberscopy revealed scirrhous gastric cancer. Signet ring cell carcinoma was later demonstrated histologically by biopsy specimens. CT demonstrated a prominent thickening of the gastric wall and hydronephrosis, although no prevertebral soft tissue masses were observed. A total gastrectomy was performed with failure to surgically decompress the ureters because fibrous plaque had firmly enveloped the retroperitoneal structures. Biopsy specimens of the retroperitoneum revealed an invasion of the tumor cells and prominent fibrosis. As an etiology of renal failure, ureteral stenosis resulting from secondary retroperitoneal fibrosis was also considered.

Acute Kidney Injury↗

[Effectively treated hypertension with minocycline hydrochloride infusion into the cyst in a patient with a multi-septated massive hepatic cyst].

We present a successfully treated case of a multi-septated massive hepatic cyst with repeated injection of minocycline hydrochloride (MINO). A 57-year-old Japanese female complaining of right back pain, hypochondralgia and hypertension had a multi-septated massive hepatic cyst, 25 cm in diameter. Multiple cysts of various sizes were also seen in liver and kidneys. In order to reduce the size of the massive hepatic cyst to relieve the complaints, we performed the reduction therapy of the cyst. After a pig tail catheter was inserted into the cyst, the cystic fluid was aspirated and then a total of 3900 mg of MINO was injected. Red-brownish, serous cystic fluids were obtained. Cytology and bacterial culture were negative, but the LDH (3, 336 IU/l) and CA19-9 (751,500 U/l) concentrations were very high. After the 9 series of the therapy, the cyst was minified on CT and the patient's symptoms were relieved. Furthermore high blood pressure was improved. Thus, the therapy of size-reduction for a massive hepatic cyst is revealed to be very safe and useful.

Cysts↗

Nodular regenerative hyperplasia of the liver with portal vein thrombosis and hyperplasia of the adrenal gland.

Nodular regenerative hyperplasia (NRH) of the liver is an uncommon entity of unknown origin and pathogenesis. We report here a case of NRH of the liver which was associated with portal vein thrombosis and adrenal hyperplasia. A 48-year-old man who was admitted for further examination of portal hypertension and splenomegaly, died of acute myocardial infarction. Autopsy revealed an enlarged heart with occluded coronary arteries and fresh multiple necrosis in the myocardium. The spleen and the liver were enlarged. In the liver diffusely distributed nodules of regenerative hyperplasia were noted. Organized thrombi of the portal vein and adenomatous hyperplasia of the left adrenal gland were also noted. Antinuclear antibody was positive in the serum.

Adrenal Glands↗

Sibling cases of primary biliary cirrhosis associated with polymyositis, vasculitis and Hashimoto's thyroiditis.

Primary biliary cirrhosis (PBC) was diagnosed in two siblings, a 42-year-old male and his 49-year-old sister. Furthermore, they were complicated with three types of autoimmune diseases: polymyositis, Hashimoto's thyroiditis and vasculitis of the right axillary artery. Although PBC is often associated with other autoimmune diseases, reports of the association with polymyositis and vasculitis syndrome are rare. Familial clustering of PBC with autoimmune diseases is also rare.

Adult↗

Primary papillary adenocarcinoma in the third portion of the duodenum.

We herein report a rare case of papillary adenocarcinoma which occurred in the third portion of the duodenum. The patient was a 62-year-old Japanese male who was admitted due to vomiting and right lower abdominal pain. No abnormal findings were found in the laboratory examinations. After a diagnosis of primary duodenal carcinoma was made by radiologic, endoscopic and ultrasonographic studies, a pancreatoduodenectomy was performed. The histology of the resected specimen revealed papillary adenocarcinoma, with invasion reaching to the pancreatic body. Some characteristic features of the disease are also reviewed.

Adenocarcinoma, Papillary↗

[A case of Fitz-Hugh-Curtis syndrome confirmed by laparoscopy].

A 25-year-old Japanese female was admitted to the Department of Surgery in Kyushu Koseinenkin Hospital because of serious right hypochondralgia. Gastrofiberscopy, abdominal ultrasonography, intravenous pyelography and irrigoscopy did not reveal the origin of the pain, and she was introduced to the Department of Internal Medicine. Because enzyme immunoassay of the uterine cervical specimen in the Department of Urology showed positive chlamydial antigen, we suspected her of perihepatitis induced by Chlamydia trachomatis (Fitz-Hugh-Curtis syndrome). Laparoscopy revealed typical violin string adhesions between the anterior surface of the liver and the corresponding parietal peritoneum, and the diagnosis was confirmed. After an administration of Ofloxacin was started, the symptom disappeared completely. It is considered to be important to remember this syndrome when examining a young women with right hypochondralgia.

Adult↗

[A case of primary biliary cirrhosis with negative anti-mitochondrial antibody anteceded by rheumatoid arthritis].

We report a case of primary biliary cirrhosis with negative anti-mitochondrial antibody which were anteceded by rheumatoid arthritis. The patient was a 46-year-old female who was admitted due to low grade fever and elevated serum alkaline phosphatase (ALP) and gamma-glutamyl transpeptidase (gamma-GTP) levels. She had been diagnosed as having erythema nodosum and rheumatoid arthritis 6 years before. Her family history disclosed that her mother had rheumatoid arthritis and her sister systemic lupus erythematosus. On admission, she had moderately elevated erythrocyte sedimentation rate, and elevated serum ALP, gamma-GTP and IgM levels. Anti-mitochondrial antibody and anti-pyruvate dehydrogenase complex antibody were negative but anti-nuclear antibody was positive. However, the histology of liver showed chronic non-suppurative destructive cholangitis. AMA was always negative and serum ALP and bilirubin levels remained constant during the following two years. The pathogenesis of primary biliary cirrhosis with negative anti-mitochondrial antibody is discussed.

Arthritis, Rheumatoid↗

[Five cases of Crow-Fukase syndrome].

We presented five cases of Crow-Fukase syndrome. Plasma cell hyperplasia or dyscrasia in bone marrow were recognized in all cases and localized bone lesion was seen in three cases. Thyroid dysfunction was seen in three cases; hyperthyroidism in one case and hypothyroidism in two cases, which was considered to be one of the characteristics though it has seldom been described in this disease. Two of four cases treated with prednisolone had good responses but two cases treated with interferon had no effect.

Adult↗

A case of prominent hepatic cholestasis developing to hepatic failure in lambda-AL amyloidosis.

We report a case of lambda-AL amyloidosis which manifested prominent hepatic cholestasis. The patient was a 71-year-old Japanese male who was admitted to our hospital because of abdominal distension and jaundice. Laboratory examination revealed a marked deterioration of liver function with cholestasis. Gastric biopsy revealed amyloid deposition. Under a diagnosis of primary amyloidosis he was treated with corticosteroid and dimethylsulfoxide. However, jaundice progressed, renal function deteriorated rapidly, and he died two weeks after admission. Autopsy revealed a profound deposition of lambda-AL amyloid not only in the liver but also in the kidneys, spleen, lungs, heart and intestine.

Aged↗

Giant metastatic VIPoma in the liver.

A case of giant metastatic liver VIPoma manifesting WDHA syndrome is described. The patient was a 33-year-old Japanese male who was admitted because of persisting watery diarrhea and weight loss. Laboratory examinations revealed marked hypokalemia, mild hypercalcemia and highly elevated plasma vasoactive intestinal peptide (VIP) and glucagon levels. Intensive imaging examinations disclosed two large tumors in the liver but not in other organs, including the pancreas. Laparoscopy disclosed a slightly elevated purplish-colored large tumor on the surface of the apparently normal liver. At operation, however, a small hard nodule was observed in the tail of the pancreas. Histologically, it was revealed to be a VIPoma resembling a carcinoid or an islet cell tumor, and was identical to the liver tumors. Thus, this neoplasm was considered to be a VIPoma of pancreatic origin, with metastatic lesions in the liver.

Adult↗