Membranoproliferative glomerulonephritis induced by portosystemic shunt surgery for non-cirrhotic portal hypertension

The liver and spleen both have important phagocytic functions and contain monocytes/macrophages which clear immune complexes. We describe here three patients who presented proteinuria and hematuria 7 to 13 years after portosystemic shunt surgery, which diverted portal venous blood to the systemic circulation. They had hematemesis and/or melena and underwent mesocaval shunt surgery and splenectomy in childhood because of non-cirrhotic portal hypertension with esophageal varices. Renal biopsy specimens revealed findings characteristic of membranoproliferative glomerulonephritis (MPGN) type I. Immunohistologically, these three cases were accompanied by a distinct IgA deposition along with a marked C3 deposition. The IgA observed in these three cases contained not only IgA1 but also IgA2, which is the predominant form of mucosal IgA. On the other hand, of 20 patients with idiopathic MPGN type I with IgA deposition (n = 20), only two were positive for IgA2, and the distribution was focal and segmental. Our study shows that MPGN type I may have developed secondary to portosystemic shunt. This secondary form of MPGN type I may be caused by a reduced clearance of immune complexes in the liver and their deposition in the glomerulus, since a portosystemic shunt routes portal venous blood from the intestinal tract directly to the systemic circulation.     

Spiral autovenous mesocaval shunt in portal hypertension syndrome

An analysis was performed of results of ultrasonic and hormonal investigations in 239 patients with nodular goiter revealed in a screening-type clinical and laboratory examination of the 3371 persons evacuated from the 30-km zone of ChNPP, at their ages randing between 18 to 68 years. In 84.20% of patients in the group who took part in the elimination of effects of the Chernobyl accident (PEEA)--"PEEA + the evacuated"--the diameter of the nodules did not exceed 1 cm, in 6.1% it ranged from 1 to 2 cm, and in 13.41% it exceeded 2 cm. Distribution of nodules in the group "the evacuated" did not differ much from that in the above group. In 20% of patients with nodular goiter, there were functional disturbances concurrent with the autoimmune processes. No effects were detected of small doses of extra irradiation on the pattern of structural-and-functional indices in patients with nodular goiter evacuated from the 30-km zone of ChNPP who took part in EEA.     

Active collagen synthesis by pulmonary arteries in pulmonary hypertension complicated by congenital diaphragmatic hernia

BACKGROUND: Patients with spinal cord injury (SCI) have an increased prevalence of gallstones. AIMS: To study prospectively the incidence of gallstones and gall bladder contractility in patients with SCI. PATIENTS AND METHODS: Thirty six consecutive patients with SCI were studied: 18 patients with SCI above thoracic 10 neuronal segment (> T10) and 18 patients with SCI below T10 (< T10). An equal number each of disease controls (multiple fractures) and healthy controls were also studied. All patients and controls underwent serial ultrasonography to detect development of gallstones and ultrasonographic measurement of gall bladder contractility. RESULTS: A significantly higher number (9/18) of patients with SCI > T10 developed biliary sludge compared with patients with SCI < T10 (2/18), disease controls (2/18), and healthy controls (1/18) (p < 0.05). No patient developed gallstones. The gall bladder fasting volume was significantly decreased in patients with SCI > T10 (20.56 ml; 95% confidence intervals (CI) 19.74 to 21.38) compared with that in patients with SCI < T10 (27.33 ml, 95% CI 26.17 to 28.49; p < 0.05), disease controls (27.92 ml, 95% CI 26.69 to 29.15; p < 0.05), and healthy controls (28.35 ml, 95% CI 27.25 to 29.45; p < 0.05). Gall bladder contractility was normal in patients with SCI as shown by normal gall bladder residual volume and emptying time. CONCLUSIONS: Patients with SCI above T10 have an increased incidence of biliary sludge and a decreased gall bladder fasting volume. Gall bladder contractility is, however, normal.     

Lung transplantation for primary pulmonary hypertension and giant pulmonary artery aneurysm

We report the case of an 18-year-old patient with a giant pulmonary artery aneurysm and primary pulmonary hypertension who was successfully treated with bilateral lung transplantation and complete reconstruction of the pulmonary artery.     

Endovascular embolization of the gastric veins in portal hypertension complicated by esophagogastric hemorrhages

Through a period from 1981 to 1996 127 roentgen-endovascular embolization of gastric veins were carried out in 95 patients for portal hypertension complicated by esophagogastric bleeding or in its threatening relapse. Group 1 consisted of 73 patients, to whom endovascular treatment was carried out urgently at the background of continuing hemorrhage, in 52 patients--it was delayed and in 21 patients in the nearest posthemorrhagic period. In 73 patients bleeding resulting from embolization was stopped. In the earliest postembolization period recurrence of hemorrhage developed in 6 patients, thus initial hemostatic effect of embolization made up 91.8%, and survival rate--74% (54 from 73 patients of the 1-st group were discharged from the clinic). Group 2 consisted of 22 patients to whom embolization of gastric veins was carried out as an elective procedure. There were bleedings in the anamnesis in the patients of the latter group, and varicose veins of the esophagus with trophic disturbances in esophageal mucosa manifested real treat of the hemorrhage. The survival rate in this group of patients made up 95.5%. The analysis of the results of elective endovascular embolization of gastric veins revealed more favourable results in the nearest postembolization period, than in urgent procedures. Far off results of embolization of gastric veins were followed up in 61 patients (43 patients of the 1st group and 18 patients of the 2nd group). Index of survival up to 6 months made up 87.8%, up to 12 months--80.5%, up to 3 years--57.5% and up to 6 years--19.6%. Hemostatic effect up to 6 months made up 100%, to 12 months--84.6%, to 3 years--54.9%, to 6 years--15.6%.     

Intrasplenic venous flow patterns demonstrated by Doppler ultrasound in patients with portal hypertension

The flow pattern in the splenic vein has been previously reported in patients with portal hypertension, but with no reference to the flow within the intrasplenic venous radicles. Using Doppler ultrasound, this study describes the intrasplenic venous flow direction in 176 adult patients with intrahepatic portal hypertension. In our series, a normal flow pattern was maintained in all except four patients (2.3%) who had either reversed or dual venous drainage patterns resulting in trans-splenic portosystemic shunts. These abnormal patterns are Doppler signs of portal hypertension which might be associated with a higher risk of oesophageal variceal bleeding. It is recommended that the intrasplenic venous flow pattern should be assessed before surgical intervention involving the spleen in patients with portal hypertension.     

Fibrosing mediastinitis causing pulmonary arterial hypertension without pulmonary venous hypertension. Clinical and necropsy observations

Clinical and morphologic observations are described in two patients with severe pulmonary arterial hypertension without pulmonary venous hypertension from fibrosing mediastinitis. In one patient, both main pulmonary arteries and one major pulmonary vein were severely narrowed by dense fibrous tissue; in the second patient, only the right main pulmonary artery was severely narrowed. Both patients had normal intrapulmonary arteries and normal pulmonary parenchyma. Of nine previously described necropsy patients with pulmonary hypertension due to fibrosing mediastinitis, seven had severe narrowing of multiple large pulmonary veins and in six of them the pulmonary hypertension was entirely due to pulmonary venous obstruction. In one other patient, the pulmonary hypertension was due to obstruction of one main pulmonary artery and several large pulmonary veins. Each of these seven previously described patients had severe changes in the small intrapulmonary arteries. Of the other two previously described patients with pulmonary hypertension from fibrosing mediastinitis, one had severe narrowing of only the main right pulmonary artery, and the other, of both main pulmonary arteries. Thus, although pulmonary arterial hypertension in patients with fibrosing mediastinitis is usually due to obstruction of multiple large pulmonary veins and to severe secondary changes in small intrapulmonary arteries, fibrosing mediastinitis can cause severe pulmonary hypertension by obstructing the right or both main pulmonary arteries.     

Clinical results and hemodynamic changes of portal venous system in transjugular intrahepatic portosystemic stent shunt

We describe on a case of a patient with Mayer-v. Rokitansky-Küster-syndrome the formation of a vagina by a laparoscopic modification of the Vecchietti-procedure. A further simplification of this procedure is the use of a laparoscopic needle-holder to tense the strings above the abdominal wall. We even abandoned the use of a prothesis after finishing the tension phase. Instead, we allowed early intercourse on the third day of extraction of the vaginal phantom.     

Bidirectional cavopulmonary shunt in patients with anomalies of systemic and pulmonary venous drainage

BACKGROUND: Bidirectional cavopulmonary shunt and Fontan repair are now commonly performed in patients with a variety of forms of complex single ventricle, including those with anomalies of systemic or pulmonary venous return. These anomalies are ideally dealt with during bidirectional cavopulmonary shunt, thereby minimizing the complexity of the eventual Fontan procedure. METHODS: Between March 1990 and December 1995, 36 patients with anomalous systemic or pulmonary venous drainage underwent bidirectional cavopulmonary shunt. A combination of anomalous systemic and pulmonary venous drainage was present in 12 patients, whereas 19 patients had anomalous drainage only from the systemic circulation and 5 patients had isolated anomalies of pulmonary venous return. Visceral heterotaxy syndrome was diagnosed in 18 patients. The median age at operation was 11 months, and bidirectional cavopulmonary shunt was the first surgical procedure performed in 10 of these patients. Techniques of repair are described. RESULTS: There were two early deaths and one bidirectional cavopulmonary shunt was taken down, for mortality and failure rates not significantly different than those for all patients undergoing bidirectional cavopulmonary shunt during this time period (n = 117). At a mean follow-up of 19.9 months, there have been three late deaths and 11 patients have undergone Fontan completion. Actuarial survival was 87% at 1 year and 81% at 3 years. Among all patients undergoing bidirectional cavopulmonary shunt during this time period, neither heterotaxy syndrome nor anomalies of systemic or pulmonary venous return were significantly associated with decreased survival or poor outcome. CONCLUSIONS: Bidirectional cavopulmonary shunt can be performed in patients with anomalous systemic or pulmonary venous drainage, including those with visceral heterotaxy syndrome, with morbidity and mortality rates that do not differ significantly from those achieved in all patients undergoing bidirectional cavopulmonary shunt. In this report, we describe our experience with this group of patients, primarily focusing on outcomes and technical issues that pertain to the use of bidirectional cavopulmonary shunt as a preparatory procedure for the extracardiac conduit Fontan operation.     

Differential regulation of hepatic arterial and portal venous vascular resistance by nitric oxide and carbon monoxide in rats

Nitric oxide (NO), a gaseous mediator that accounts for the biological activity of endothelium-derived relaxing factor, has been shown to play an important role in the reduction of basal vascular tone in multiple vascular beds, including the hepatic circulation. On the other hand, recent studies have provided first evidence that endogenously generated carbon monoxide (CO) may exert vasodilatory effects in the hepatic portal vein and within sinusoids. Thus, we defined the differential role of NO and CO in the regulation of vascular resistance in the two inflows to the liver in the normal rat in vivo. Male Sprague-Dawley rats were anesthetized with pentobarbital sodium and surgically instrumented in order to study the change in hepatic arterial (Rha) and portal venous vascular resistance (Rpv) in response to intravenous bolus administration of either the NO-synthase inhibitor N(omega)-nitro-L-arginine methyl ester (L-NAME) (1 mg/kg; n = 7 animals) or of tin protoporphyrin-IX (SnPP-IX) (50 micromol/kg), a specific inhibitor of the CO-generating enzyme heme oxygenase (n = 8 animals). While L-NAME caused a substantial increase in Rha, Rpv increased only slightly under these conditions. In sharp contrast, SnPP-IX did not affect Rha, but caused a profound increase in Rpv. In conclusion, Rha and Rpv are differentially regulated by NO and CO in the normal rat liver in vivo, i.e., NO serves as a potent vasodilator in the hepatic arterial circulation, but exerts only a minor vasodilatory effect in the portal venous vascular bed. In contrast, while there is no intrinsic CO-mediated vasodilation in the hepatic artery, CO acts to maintain portal venous vascular tone in a relaxed state.     

Surgical treatment of ventricular septal defect complicated by pulmonary hypertension at high altitude

Home care providers have a professional and legal obligation to help prevent their elderly patients from being abused and neglected by family members and other home care providers. The elderly are often in a vulnerable situation because they depend on family members or others to help with personal care, housekeeping chores, and money management. A recent article in a major newspaper illustrates the problem. It reported that Mr. X, who was 84 years old, had been without food or water while he lay curled in the trunk of his car for 2 days before he was found by the police. When found, he reported that he saw daylight only when his housekeeper lifted the car trunk lid to ask him if her forgery of his check looked authentic. After he was rescued, he acknowledged that he was confused about why his housekeeper, who had befriended him, had turned on him. He expressed concern for her and hoped she would get a break in her sentencing. "She didn't kill me," he said. This situation is not that unusual. Elder abuse and neglect is a major public health problem in the United States, with most cases hidden from public scrutiny. The National center on Elder Abuse reports that cases of domestic abuse against the elderly increased from 117,000 in 1986 to 241,000 in 1994, and that represented only a fraction of older Americans who were abused and neglected in their homes. The Center estimates that 818,000 elderly Americans were victims of various types of domestic abuse in 1994. They believe the rise in reported cases illustrates a growing pattern of violence and neglect among the nation's expanding elderly population.     

Differential effects on portal and effective hepatic blood flow. A comparison between transjugular intrahepatic portasystemic shunt and small-diameter H-graft portacaval shunt

OBJECTIVE: This study was undertaken to determine the effects of transjugular intrahepatic portasystemic shunt (TIPS) and small-diameter prosthetic H-graft portacaval shunt (HGPCS) on portal and effective hepatic blood flow. SUMMARY BACKGROUND DATA: Mortality after TIPS is higher than after HGPCS for bleeding varices. This higher mortality is because of hepatic failure, possibly a result of excessive diminution of hepatic blood flow. METHODS: Forty patients randomized prospectively to undergo TIPS or HGPCS had effective hepatic blood flow determined 1 day preshunt and 5 days postshunt using low-dose galactose clearance. Portal blood flow was determined using color-flow Doppler ultrasound. RESULTS: Treatment groups were similar in age, gender, and Child's class. Each procedure significantly reduced portal pressures and portasystemic pressure gradients. Portal flow after TIPS increased (21 mL/second +/- 11.9 to 31 mL/second +/- 16.9, p < 0.05), whereas it remained unchanged after HGPCS (26 mL/second +/- 27.7 to 14 mL/second +/- 41.1, p = n.s.). Effective hepatic blood flow was diminished significantly after TIPS (1684 mL/minute +/- 2161 to 676 mL/minute +/- 451, p < 0.05) and was unaffected by HGPCS (1901 mL/ minute +/- 1818 to 1662 mL/minute +/- 1035, p = n.s.). CONCLUSIONS: Both TIPS and HGPCS achieved significant reductions in portal vein pressure gradients. Portal flow increased after TIPS, although most portal flow was diverted through the shunt. Effective hepatic flow is reduced significantly after TIPS but well preserved after HGPCS. Hepatic decompensation and mortality after TIPS may be because, at least in part, of reductions in nutrient hepatic flow.     

Middle cerebral artery aneurysm complicated by tuberculosis

Pentazocine, a kappa opioid receptor agonist, induced catalepsy in mice suggesting thereby that it might possess postsynaptic striatal D 2 dopamine (DA) receptor blocking activity. However, our other findings, that pentazocine pretreatment did not antagonise the cage climbing behaviour induced by the directly acting DA agonist apomorphine in mice and actually potentiated the stereotyped behaviour induced by the indirectly acting DA agonist methamphetamine in mice, indicate that pentazocine does not possess postsynaptic striatal and mesolimbic D 2 DA receptor blocking activity. Pretreatment with naloxone, an antagonist of opioid receptors, antagonised pentazocine-induced catalepsy. This suggests the possible involvement of opioid mechanisms in the induction of catalepsy by pentazocine in mice.