[Acute hepatitis as the leading symptom of secondary syphilis]. / Akute Hepatitis als Leitsymptom einer Sekundärlues.

HISTORY AND CLINICAL FINDINGS: A 44-year-old heterosexual man reported having been jaundiced for 4 days. He drank little alcohol. There was no history of venereal disease. INVESTIGATIONS: Laboratory tests indicated marked cholestasis (alkaline phosphatase 1589 U/I, gamma-GT 449 U/I), but only moderately raised transaminases (GOT 66 U/I, GPT 230 U/I), with a bilirubin level of 4.8 mg/dl. Ultrasonography revealed diffuse parenchymal damage in the liver, while endoscopic retrograde cholangiopancreatography showed no abnormalities. Viral hepatitis was excluded by serological tests. Histology of a liver biopsy showed inflammatory infiltration of the portal areas and of the liver parenchyma. Routine syphilis serology indicated fresh infection with Treponema pallidum. There was a healing painless ulcer in the area of the sulcus coronarius of the genitals. TREATMENT AND COURSE: 2.4 mill. I.U. benzathine benzylpenicillin were administered intramuscularly. This rapidly improved his condition and liver function tests became normal. CONCLUSION: In case of hepatitis of uncertain genesis syphilis should be considered as a possible cause, even in the absence of other signs of the disease.

Spontaneous remission of acute myeloid leukemia after infection and blood transfusion associated with hypergammaglobulinaemia.

Spontaneous remissions of acute myeloid leukemia (AML) have been documented in association with infection as well as blood transfusions. Activation of the immune system including an increased number of NK cells and cytokine release have been implicated in the mechanism of this phenomenon. We have observed spontaneous remissions in two patients with AML (one with a t(8;21)-positive M2, one with M5b), both occurring after infection and blood transfusions. The bone marrow showed a reduction of blast cells from 65% to 2% or 40% to 1%, respectively. Remission was accompanied by a marked polyclonal hypergammaglobulinemia in both cases (IgG values of 6420 and 2160 mg/dl, IgA of 802 and 811 mg/dl, respectively). A concomitant increase in bone marrow plasma cells was observed in both patients. Reduction of AML1/ETO PCR positivity from one-step to two-step PCR (approximately 100-fold) was documented in the patient with a t(8;21), while a regression of lymph node and skin leukemic infiltrations occurred in the patient with M5b. One patient relapsed after 4 months, at a time when his serum immunoglobulin levels had markedly decreased. The other patient is in continuous remission after 14 months. These cases suggest a potential role for a humoral immune response in the mechanism of spontaneous remission.

[Sarcoma botryoides in the pediatric vagina--a soft tissue sarcoma with good prognosis]. / Das Sarcoma Botryoides der kindlichen Vagina--Ein Weichteilsarkom mit guten Heilungschancen.

Although rare, sarcoma botryoides of the vagina in infants is a highly aggressive soft tissue tumour. Former opinions suggesting exenterative surgery and radiation to improve the dismal outcome in these young children, are outdated. Due to balanced therapies (topical tumourectomy, chemotherapy and radiation adjusted to tumour stage) cure of these sarcomas may be expected dependent on tumour stage, localisation and response to chemotherapy. The rate of complete remissions has increased in recent years. We report on an eighteen-month old girl with complete remission of tumour stage IIA who had been treated according to the CWS 91 protocol. Diagnosis and treatment were performed in March 1994 at the age of six months. We observed the patient in complete remission for 8 months (till September 1995).

Gastric carcinoma arising from a hyperplasiogenic polyp with a diameter of less than 2 centimeters.

As there are only few reports on the occurrence of carcinoma in hyperplasiogenic polyps there is at present no general agreement concerning the malignant potency of these polyps in the stomach. In this paper we review the literature and present the unusual case of a patient who developed carcinoma in a hyperplasiogenic polyp with a diameter of 15 x 5 mm. Whereas in nearly all the reported cases cancer developed in polyps with a diameter greater than 2 cm our findings demonstrate that even in smaller polyps malignant changes have to be suspected.

Morphology of the left atrial appendage.

BACKGROUND: When examining the left atrial appendage by transesophageal echocardiography, differences in size and shape of the left atrial appendage are to be observed. The study was carried out with the aim of investigating the morphology of the left atrial appendage and to find associations with pathologic cardiac findings. METHODS AND RESULTS: In 220 cases (106 female, 114 male, mean age 72 +/- 13 years) a cast of the left atrial appendage was made after the post mortem examination by using synthetic resin. In 198 cases an ECG was available (sinus rhythm n = 143, atrial fibrillation n = 55). The casts were described in respect to course and ramifications of the principal axis. The casts were measured concerning orifice diameters, outline, and volume. Most frequently (42%) the course of the principal axis was angulated below 100 degrees. More than five ramifications of the principal axis were found in 56% of the casts. The volume ranged from 770-19,270 mm3 (mean 5,220 +/- 3,041). When comparing the clinical and autopsy-data of the patients with the morphology of the casts, associations could be found between the volume of the casts and atrial fibrillation (7,060 mm3 as compared to 4,645 mm3 in sinus rhythm, P < 0.01), left ventricular hypertrophy (5,740 mm3 as compared to 4,639 mm3 without hypertrophy, P < 0.01), myocardial scars (5,923 mm3 as compared to 4,891 mm3 without scars, P < 0.05), closed foramen ovale (5,515 mm3 as compared to 4,037 mm3 with patent foramen ovale, P < 0.01), and left atrial appendage thrombi (8,566 mm3 as compared to 5,027 mm3 without thrombi, P < 0.01). CONCLUSION: Left atrial appendages are formations greatly varying in volume and shape. This variability should be considered when interpreting images of the left atrial appendage, and in particular when diagnosing thrombi.

The pancreas as a site of granulomatous inflammation in Crohn's disease.

Several reports on the occurrence of pancreatic dysfunction complicating Crohn's disease have been published during the past few years. Nevertheless, the pathogenesis of these disorders remains controversial. In this report, we describe a patient presenting with extrahepatic cholestasis and a mass in the head of the pancreas. Histological examination showed a granulomatous inflammation caused by Crohn's disease involving the stomach, the duodenum, and the head of the pancreas, clearly showing that the pancreas can also be a localization of Crohn's disease. Granulomatous inflammation could be an important factor in the pathogenesis of pancreatic dysfunction occurring in association with Crohn's disease.

[The high autopsy rate in Vienna]. / Zur hohen Autopsierate in Wien.

In 1983 34% of all persons who died in the Republic of Austria were autopsied. We examined the frequency of postmortem examinations in the capital city, Vienna, between 1983 and 1987 in view of this high overall autopsy rate. The annual autopsy rate in all 9 pathology institutes and the department of legal medicine of the University of Vienna was on average 51 to 53%. There was a slight statistically verifiable decline in 1987. The reason for this high necropsy rate is that Austrian law permits the autopsies without the consent of next of kin if it appears indicated for medical, scientific or educational reasons; further more the fact, that the chairman of the department is at the same time the coroner for the hospital. A high autopsy rate is necessary to maintain a high standard of diagnostic accuracy even in modern medicine. The diagnoses were inaccurate or incomplete in 15% of all cases. The high autopsy rate is the basis for extremely accurate mortality and morbidity statistics and provides the basis for long range public health planning. Medical students develop an insight in disease patterns, which cannot be gained in any other way. The pathology departments of Vienna introduce a new city-wide computer-supported diagnostic file system, which will provide a baseline for comparative scientific studies.

[Endoscopic-diagnostic problems in malignant lymphoma of the upper intestinal tract]. / Endoskopisch-diagnostische Probleme bei malignen Lymphomen des oberen Verdauungstraktes.

A retrospective analysis of 79 patients with malignant lymphoma (ML) of the upper gastrointestinal tract in a series of 842 endoscopically verified malignant tumours is presented. There was a significant difference between carcinomas and ML: in the latter, macrosopic diagnosis was correct in more than 90%, while histologic evaluation of forceps biopsies was false negative in 23.2%. Guided brush cytology provided an unexpected gain of information in 35%. Gastroscopy should be performed even in symptomfree patients with ML. Errors of histologic evaluation can be avoided by wire loop biopsy. Extragastric involvement of ML was successfully demonstrated in 93% by 67-gallium scanning.

[Diagnosis experiences in 188 patients with primary liver cancer]. / Diagnostische Erfahrungen bei 188 Patienten mit primärem Leberkarzinom (PLK).

In Austria primary carcinoma of the liver (plc) is no rare malignancy. From 1973 and 1979, in our department, 188 patients with plc had been registered. By separate evaluation of the results of alpha-1-fetoprotein (AFP)-determination and 67-gallium-liverscintigraphy, a diagnostic accuracy of 81.6%, resp. 78. % could be demonstrated. By combination of both methods, a correct diagnosis of plc was obtained in 95%. Based on the promising results of lobectomies performed in patients with plc, therapeutic nihilism is not longer justifiable.