[A case of IAHS (infection associated hemophagocytic syndrome) successfully treated with etoposide].

This report details a case of infection associated hemophagocytic syndrome (IAHS). A 20-year-old female was admitted to our hospital with persistent high fever in July, 1994. Physical examination revealed high body temperature (40 degrees C), marked hepatosplenomegaly and no superficial lymph node swelling. Laboratory examination revealed leukopenia and abnormal liver function on admission. Serum ferritin levels were surprisingly elevated. The coagulation tests showed high FDP and D-dimer. Specific viral antibody titers were not elevated such as Epstein-Barr virus or Cytomegalovirus. Bone marrow examination revealed histiocytic hyperplasia with hemophagocytosis, and the histiocytes were well matured. We diagnosed IAHS. Corticosteroids were administered on the 3rd hospital day (methylprednisolone 1 g/day, 3 days), but persistent high fever and laboratory findings did not improve. So we tried etoposide (etoposide 200 mg/day, 5 days) therapy on the 13th hospital day. After administration of etoposide, she failed to recover from severe leukopenia and suffered from meningitis. We administered G-CSF, gamma-globulin and antibiotics for intensive supportive therapy. As the leukocyte count increased, her symptoms and laboratory data improved. There was no hemophagocytosis in her bone marrow before discharge. Recently, etoposide is said to be effective for reactive monocytic proliferation. Administration of etoposide was very effective for IAHS, although corticosteroids, were ineffective.

Pulmonary arteriovenous fistula associated with unequal branching of the pulmonary artery system.

A 60-year-old woman was admitted to our hospital with shortness of breath and was diagnosed to have pulmonary arteriovenous fistula with unequal branching of the pulmonary artery system, as assessed by pulmonary angiography. The concomitant occurrence has not been reported previously. Although the etiology of these abnormalities is unknown, these abnormalities are considered to have occurred during an early stage of fetal development. The analysis of a concomitant occurrence of these abnormalities may be useful in elucidating the etiology of pulmonary arteriovenous fistula.

[Dynamics of pleural liquid in hydrothorax].

The basics of pleural liquid dynamics are summarized. The normal pleural cavity contains a small amount of pleural liquid (0.1-0.3 ml/kg). Its protein concentration is about 1.0 g/dl and its pH is 7.6. The normal flow of pleural liquid is gravity dependent, and pleural liquid flows from the costal to the mediastinal region. In experimental hydrothorax, the pleural liquid was removed mainly via lymphatics. The turnover of the pleural liquid is rapid, and it depends on the area of contact between pleural liquid and pleural and on the blood flow to the pleura.

[Detection of intratumoral DNA heterogeneity in primary lung cancer using a multiple sampling method].

The nuclear DNA contents of 18 freshly resected specimens of primary lung cancer tissue were determined by flow cytometry with a multiple sampling method. A DNA aneuploidy pattern was observed in all cases. In three cases, a diploid DNA pattern was observed along with abnormal DNA stem lines. Twelve cases (66%) had two or more abnormal DNA stem lines, and were classified as DNA multiploidy. The frequencies of DNA aneuploidy and DNA multiploidy were higher than in previously reported studies. Intratumoral DNA heterogeneity was observed in 10 cases (55%). Three of these were a mixture of DNA diploidy and DNA aneuploidy, and the other seven were a mixture of DNA aneuploidy and DNA multiploidy. If multiple sampling had not been performed in these cases, their ploidy patterns would have been misinterpreted. We conclude that multiple sampling is important in determining the precise DNA ploidy patterns of primary lung cancers.

Production of basic fetoprotein in human peripheral lymphocytes during blastic transformation.

To clarify the significance of basic fetoprotein (BFP) in lymphocytes, we investigated whether BFP is produced in lymphocytes during blastic transformation. Peripheral blood lymphocytes obtained from 14 adults were cultured under the stimulation of lectins. The concentration of BFP in the culture medium (extracellular BFP) was estimated serially. The incorporation of [6-3H]-thymidine was assayed simultaneously. The intracellular BFP was measured by dual flow cytometry for DNA and BFP. A lymph node was studied immunohistochemically. Serum BFP was measured in four cases of lymphocytic leukaemia. In two cases, dual staining was performed. The intracellular BFP of the mitogen-stimulated lymphocytes was increased within 24 h. The extracellular BFP was increased exponentially from 72 h. The extracellular BFP at 96 h did not correlate with the [3H]-thymidine incorporation. The intracellular BFP increase began in G1 phase. Immunostaining showed that the B cells also produced BFP. The serum BFP level in leukaemia was high in 1 of 4 cases and the leukaemic cells in two cases showed high intracellular BFP content. These observations indicate that BFP is produced in activated human lymphocytes and in lymphocytic leukaemic cells. The production of BFP during blastic transformation will be a useful new in vitro model for studying the biological role of BFP, and BFP labelling may offer some new possibilities for the study of lymphocytes.

Double intracranial tumours of maldevelopmental origin--teratoma at the pineal region and an epidermoid cyst in the fourth ventricle.

A 21-year-old man with double intracranial tumours of maldevelopmental origin, teratoma at the pineal region and an epidermoid cyst in the fourth ventricle, is reported. The tumours were removed totally by multiple operations; the epidermoid cyst was resected by suboccipital craniectomy with the patient in prone position and the teratoma was removed via occipital transtentorial approach with the patient in sitting position. Maldevelopmental tumours located within the cranial cavity with different histological types such as the present case have not sofar been described. Histogenesis of these tumours is confusing because teratoma often contains tissues of epidermoid cyst. Etiological considerations for these tumours are presented and appropriate operative procedures for tumours simultaneously occurring in the pineal and the fourth ventricle are also discussed.

Treatment of multiple intracranial aneurysms in the anterior circulation--case report.

A 69-year-old female underwent surgery for subarachnoid hemorrhage due to a ruptured intracranial aneurysm. Nine additional aneurysms were found. Four of the latter nine aneurysms were clipped in a second operation, and two were wrapped.

Intimal fibroelastic thickening--its etiological significance in juvenile cerebral infarction. Case report.

Recurrent occlusive stroke of the vertebrobasilar system is reported in a 16-years-old man. Multiple arterial occlusions, segmental stenosis, or irregular dilatation were found in the angiography involving both the intra- and extracranial vertebro-basilar arteries. Surgical specimen of the extracranial vertebral artery with localized irregular lumen disclosed fibroelastic thickening of the intima with mural thrombus, which were thought to be an embolic source. Etiological significance of this intimal lesion in juvenile cerebrosvascular accident is discussed.

[A case of persistent trigeminal artery aneurysm treated by proximal obliteration of feeding vessels in both intra- and extracranial compartments].

The authors described a case with a saccular aneurysm of the persistent trigeminal artery (PTA) treated with intra- and extracranial proximal obliteration of the feeding arteries. PTA aneurysm is a rare disorder found only 14 cases in the literature. There have been only 4 cases treated by surgical procedure; 2 underwent direct surgery and the remaining 2 had carotid ligation. Both of the cases treated by direct obliteration of the aneurysm sustained diplopia and sensory disturbance around the orbit as postoperative neurological deficits. We thought another choice of treatment to reduce the risk of rupture of the aneurysm avoiding postoperative complications. Clinical presentation of the present case was rt-oculomotor palsy, which disappeared before the operation. Cerebral angiography disclosed an intracavernous sinus aneurysm with a broad neck arising from the rt-internal carotid-PTA junction. Bilateral posterior cerebral arteries were perfused by the rt-internal carotid artery was compressed manually, lt-vertebral angiography showed the aneurysm and the internal carotid artery simultaneously via the PTA as retrograde filling. Because the direct surgery might cause damage to the cranial nerves passing through the cavernous sinus, we employed proximal obliteration of the feeding vessels. The operation was done by the following steps: ligation of the cervical portion of the rt-internal carotid artery, rt-superficial temporal artery-middle cerebral artery anastomosis, clipping of the PTA at the basilar side.(ABSTRACT TRUNCATED AT 250 WORDS)

The direct inhibitory action of phentolamine on the contraction of rabbit basilar artery.

The effects of phentolamine on rabbit basilar artery were studied and compared with those on other smooth muscles. The drug showed a dose-dependent depressing effect on high-potassium-induced contracture of rabbit basilar artery. The depressing effect was stronger on tonic than on phasic contraction. When phentolamine was applied during high-potassium-induced tonic contraction, dose-dependent relaxation was observed. The degree of phentolamine-induced-relaxation of high-potassium-induced tonic contraction was not affected by surgical denervation of the basilar artery, although catecholamine content was almost completely lost, as indicated by chemical and histochemical experiments. The relaxed tension caused by phentolamine was reversed by addition of Ca in a dose-dependent manner. Verapamil also showed a dose-dependent relaxing effect on high-potassium-induced contracture, with this relaxed tension being reversed by the addition of Ca in a dose-dependent manner. The relaxing effect of phentolamine on rabbit basilar artery was stronger than on rabbit aorta or guinea-pig taenia coli. Phentolamine depressed serotonin- or histamine-induced contraction at a concentration more than 10 times higher than the concentration for depression of noradrenaline-induced contraction. The depression of high-potassium-induced contracture and reversal by additional Ca, especially in denervated preparations, indicates that phentolamine acts directly on smooth muscle in a similar manner to that of Ca antagonists.