Noncirrhotic portal hypertension and nodular regenerative hyperplasia of the liver in dogs with mucopolysaccharidosis type I

Seventy six consecutive patients with T2-4, N0-1, M0 primary breast cancer (BC) received a median of 3 cycles of CMF (cyclophosphamide, methotrexate, 5-fluorouracil) regimen. Tamoxifen was concomitantly administered in patients with estrogen receptor positive (ER+) BC. Ki67 antigen was evaluated immunohistochemically in tumor specimens obtained before chemotherapy and at mastectomy. At post chemotherapy evaluation, tumor shrinkage greater than 50% was obtained in 60 patients (78.9%), 21 of them being complete responders (27.6%). As a whole, primary chemotherapy significantly decreased the number of Ki67 positive cells. More than 50% decrease in Ki67 expression was observed in 78.9% of patients attaining a clinical complete response (CR), in 44.7% of patients with partial remission (PR) and in 50.0% of non-responders, while an increase (>25%) in Ki67 expression was found in 5.3%, 18.4% and 18.7% of patients with CR, PR and non-response, respectively. Both CR and PR rates were superimposable in patients with ER+ and ER- primary BC, while the reduction in Ki67 expression was mainly found in ER+ cases. Patients with increased Ki67 expression from baseline, at the end of primary chemotherapy, had a shorter disease-free interval (70 months) with respect to patients with no change (88+ months) or decrease (87+ months), p<0. 05. To conclude, the activity of CMF + tamoxifen in primary BC does not seem superior to that expected administering CMF alone. The reduction in Ki67 expression, as a whole, correlated with clinical CR, but some individual discrepancies between tumor shrinkage and Ki67 pattern have been observed. The Ki67 reduction mainly confined to the ER+ primary BC suggests that tumor response in this subset may be linked to the reduction in proliferation activity, whereas other mechanisms such as apoptosis might be responsible for the tumor shrinkage in ER- tumors. Since the increase in proliferation activity after primary chemotherapy was associated with a greater recurrence rate and lower disease free interval, irrespective of tumor response, changes in proliferation activity after primary chemotherapy may represent a potentially available parameter that, in addition to the tumor response, can discriminate patients who would benefit from the cytotoxic treatment from patients who would not.     

Intrahepatic bile duct injury and nodular regenerative hyperplasia of the liver in a patient with polyarteritis nodosa

Although involvement of the hepatic vasculature in patients with polyarteritis nodosa is not unusual, biliary manifestations are very rare. We describe a patient with polyarteritis nodosa presenting with a febrile cholestatic anicteric syndrome. Histological examination of the liver revealed necrotizing arteritis of small hepatic arteries associated with significant lesions of intrahepatic bile ducts of the sclerosing cholangitis type, i.e. fibrous collar around the ducts, periductal inflammation and ductal proliferation. Concomitant nodular regenerative hyperplasia was found, a condition which has rarely been described in association with polyarteritis nodosa. We think that hepatic arteritis compromising arterial blood flow to the liver was responsible for the most likely ischemic nature of the bile duct injury and the nodular regenerative hyperplasia seen in our patient.     

Coexpression of IL-6 and soluble IL-6R causes nodular regenerative hyperplasia and adenomas of the liver

Studies with tumor necrosis factor p55 receptor- and interleukin-6 (IL-6)-deficient mice have shown that IL-6 is required for hepatocyte proliferation and reconstitution of the liver mass after partial hepatectomy. The biological activities of IL-6 are potentiated when this cytokine binds soluble forms of its specific receptor subunit (sIL-6R) and the resulting complex interacts with the transmembrane signaling chain gp130. We show here that double transgenic mice expressing high levels of both human IL-6 and sIL-6R under the control of liver-specific promoters spontaneously develop nodules of hepatocellular hyperplasia around periportal spaces and present signs of sustained hepatocyte proliferation. The resulting picture is identical to that of human nodular regenerative hyperplasia, a condition frequently associated with immunological and myeloproliferative disorders. In high expressors, hyperplastic lesions progress with time into discrete liver adenomas. These data strongly suggest that the IL-6/sIL-6R complex is both a primary stimulus to hepatocyte proliferation and a pathogenic factor of hepatocellular transformation.     

Diffuse vascular ectasia of the antrum, duodenum, and jejunum in a patient with nodular regenerative hyperplasia. Lack of response to portosystemic shunt or gastrectomy

The case is reported of a 70 year old man who presented with severe anaemia because of chronic gastrointestinal blood loss. This loss was ascribed to vascular ectasia resembling the gastric antral vascular ectasia syndrome but extended to include the antrum, the duodenum, the jejunum, and, possibly, the cardiac area. This condition was associated with portal hypertension as a result of nodular regenerative hyperplasia. Consecutive treatments including sucralfate, prostaglandin E2, propranolol, organic nitrates, pentoxyphilline, corticosteroids, endoscopic sclerotherapy, portosystemic shunt, total gastrectomy, proved ineffective.     

Focal nodular hyperplasia of the liver: a rational approach to treatment

Focal nodular hyperplasia of the liver in a 3 year old child has been successfully treated by ligation of the hepatic artery branches supplying the lesion. The prominence of the abnormal vasculature in the lesion and its possible aetiological involvement is stressed. Because of the risks of partial hepatectomy, hepatic artery ligation is suggested as the treatment of choice for focal nodular hyperplasia.     

Oral contraceptive intake in women with focal nodular hyperplasia of the liver

This study examines the risk associated with oral contraceptive (OC) use in women with focal nodular hyperplasia (FNH). A total of 216 women (mean age, 36.2 years) with FNH were studied during 1989-98. The studied women were separated into five groups: no OC use (n = 28); high-dose OC use (50 mcg ethinyl estradiol, n = 46); low-dose OC use (30 mcg or less ethinyl estradiol, n = 98); low-dose and high-dose OC use (n = 33); pure progestagen use (n = 11). In each group, the mean diameter and the number of lesions per patient were assessed via magnetic resonance imaging (MRI). Findings revealed no differences between the five groups as to the number and the size of the lesions. The data showed that neither the intake nor the type of OC influenced the size and number of FNHs. A total of 128 women were followed up with serial MRI done after a mean of 23 months: 89 discontinued OCs, 14 remained without OCs, and 25 had taken or remained on low-dose OCs. In those who discontinued OCs, the FNH had decreased in size in two lesions and increased in size in one lesion. Despite continuation of OCs, the largest FNH disappeared 2 years after the first diagnosis, whereas the other FNH remained unchanged. Moreover, during this follow-up study, 12 women became pregnant; no increase in lesion size was seen during pregnancy. These findings indicate that low-dose OCs can be maintained in young women with FNH.     

A case of progressive multiple focal nodular hyperplasia with alteration of imaging studies

Focal nodular hyperplasia (FNH) of the liver is a lesion characterized by a well circumscribed region of hyperplastic liver tissue with stellate fibrosis. The pathogenesis of the lesion is unknown but various authors consider that FNH may be a response to a preexisting vascular abnormality. We experienced a case of progressive multiple FNH, in which the hemodynamic change as shown by imaging modalities, may support this hypothesis. The patient, a 38-yr-old woman, was found by chance to have multiple portal venous shunts and multiple FNH in both lobes of her liver. Because of their benign characteristics, we followed the nodules periodically without any special treatment. After about 4 yr, the nodules increased both in size and number. In addition, digital subtraction angiography showed that the diameter of the artery had become larger. The hemodynamic change revealed by imaging studies in this case supports the hypothesis that one of the pathogens of FNH is a secondary hepatocellular response to arterial hyperperfusion caused by some vascular malformations.     

Focal nodular hyperplasia of the liver and hypercholesterolemia in a child with VACTERL syndrome

Ten cases of acute renal failure (ARF) were seen in the period from July 1990 to August 1991 in the Nephrology Department of the SIMS Hospital, Srinagar. All were males in the age group of 18-28 years and in apparent good health when apprehended by the police. There was alleged history of physical torture of different types. All had been beaten on the buttocks, back and limbs; in addition, 2 cases had been given repeated electric shocks and 1 case put to 'sit-and-stand' exercise for about 3 h. The interval between the first day of torture till they came to our observation varied from 4 to 11 days. The main clinical features at the time of presentation were generalized aches and weakness (10), oligoanuria (9), vomiting (8), hypertension (6), acidosis (10), facial puffiness and pedal edema (6), fever and shivering (3), pulmonary edema (2), stupor (4), and hyperkalemia (5). All the cases had an established ARF (serum creatinine 668-1,997 mumol/l and serum urea 21.8-71.8 mmol/l) when first seen. Muscle enzymes, creatine phosphokinase, lactic dehydrogenase and serum glutamic oxaloacetic transaminase were all significantly raised indicating rhabdomyolysis. All showed evidence of myoglobin casts in urine. Nine had oliguric and 1 had nonoliguric ARF. All except the 1 case with nonoliguric ARF were managed with peritoneal dialysis and/or hemodialysis. All recovered. Early recognition of ARF is important since the main attention in such cases is directed towards the surgical aspect.     

Obstructive jaundice due to compression of the common hepatic duct by right hepatic artery--a case associated with the absence of the lateral segment of the left hepatic lobe

A functionalized derivative of the mu opioid agonist carfentanil was synthesized (NH2-carfentanil) and showed high specific activity when radiolabeled with iodine. [127I]NH2-carfentanil displayed high affinity and pronounced mu-binding selectivity with a delta/mu selectivity ratio of over 1200. The ability of [125I]NH2-carfentanil to interact in vivo with opioid receptors was determined in mouse brain using ex vivo binding techniques. Twenty minutes after intraperitoneal injection, 0.1% of the [125I]NH2-carfentanil injected into the mouse was present in the brain. [125I]NH2-carfentanil specific binding was inhibited by co-injection of naloxone or morphine while naltrindole, a delta-selective antagonist, was unable to displace the bound radioligand. Autoradiographic experiments revealed a heterogeneous distribution of [125I]NH2-carfentanil specific binding sites, maximal binding occurred in areas with high densities of mu receptors. Peripherally administered iodo-NH2-carfentanil selectively labelled central mu opioid receptors in mouse indicating great potential for single photon emission computed tomography studies.     

A case of bilateral optic neuropathy and recurrent transverse myelopathy associated with perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA)

The purpose of this study was to evaluate skeletal and dental effects of bionator headgear combination appliances on patients in development period with Class II, division 1 malocclusion. The comparison of computerized X-ray cephalometric measurements between the 26 treated children and 26 untreated children was made. The results showed that ANB angle was significantly reduced and horizontal mandibular growth development tended to be normal in the treated group. It was suggested that the bionator headgear combination appliance can restrain the maxillary growth early and promote the forward mandibular growth which contribute the functional jaws correction.     

A case of idiopathic iliopsoas hematoma associated with liver cirrhosis

We report a rare case of intratracheal thyroid ectopia in the setting of papillary thyroid carcinoma, resulting in the preoperative clinical impression of an aggressive, high-stage tumor. A 24-year-old opera singer presented with complaints of a gagging or choking sensation. The results of computed tomography revealed a mass in the left thyroid lobe with multiple small calcifications consistent with papillary thyroid carcinoma as well as a soft tissue mass in the adjacent left tracheal lumen thought to be direct invasion by the thyroid tumor. A total thyroidectomy was performed including excision of half of the first and second tracheal cartilages and the lower portion of the hemicricoid cartilage. The final histological findings revealed that the intratracheal component was composed of benign thyroid tissue and strands of benign thyrocytes coursed through the first tracheal membrane. Intratracheal thyroid ectopia is a rare symptomatic occurrence with a striking female predisposition. We have identified 23 cases of intratracheal thyroid ectopia from the literature. They occur most often at the level of the cricoid, usually posteriorly with a slight predisposition for the left side. Continuity between the intratracheal component and the thyroid lobe may be seen. Clinicians and pathologists must be aware of this entity to avoid mistaking it for evidence of thyroid invasion by a malignant neoplasm.     

A case report of multiple hepatic hilar cyst associated with hepatocellular carcinoma

Two types of cDNA encoding gonadotropin beta subunits (GTH beta) were isolated from a cDNA library prepared from pituitary gland of Baikal omul (Coregonus autumnalis migratorius Georgi). The nucleotide sequences of cDNA were determined. The CTHI beta and GTHII beta cDNAs code for polypeptides of 137 and 142 amino acids, respectively. Both of them include a putative signal peptide of 24 amino acids. The predicted amino acid structures of omul gonadotropins were compared with those of other vertebrate species.     

Biliary stricture due to mucosal hyperplasia of the common bile duct: a case report

A 71-year-old man with a tumorous lesion of the common bile duct is presented. Although histological examination of tumor biopsy specimens taken at the time of duodenoscopy or percutaneous transhepatic choledochoscopy showed mucosal hyperplasia, cancer could not be ruled out; therefore, the patient underwent pancreatoduodenectomy. However, histology showed nodular mucosal hyperplasia and an ectopic pancreas adjacent to the papilla of Vater, which was suspected to have contributed to the development of the lesion by stimulating the mucosa of the papilla and the distal common bile duct.     

Anesthetic aspects of heart tamponade due to amebic liver abscess. A case report

The problem of tamponade due to rupture of an amoebic abscess, although rare, is extremely complex, and in the planning of the anaesthetic technique the following factors should receive attention: (i) the low cardiac output in which bradycardia, vasodilation, high inflation pressures and excessive fluid load must be avoided: (ii) pulmonary complications in which a double-lumen endotracheal tube should be considered; and (iii) sepsis.     

Chronic recurrent multifocal hyperostosis of the long bones associated with hyperphosphatemia--a case report

BACKGROUND: JM216 is a new oral platinum complex with dose-limiting toxicity myelosuppression, now undergoing phase II evaluation. PATIENTS AND METHODS: JM216 was evaluated as first line therapy in non-small-cell lung cancer. Seventeen patients received 120 mg/m2/day for five days repeated every three weeks. RESULTS: Toxicity was manageable, the commonest side-effects being nausea, vomiting, diarrhoea, constipation and asthenia. Myelososuppression was generally grade < 2 and there were no cases of neutropenic sepsis or bleeding. Thirteen patients were fully evaluable for response. No sustained objective responses were reported. One patient was reported as stable disease had a partial response after three courses but was progressing again after four. An additional five patients had stable disease (46.2%). CONCLUSIONS: Although some patients may have had useful palliation, JM216 did not appear to have significant antitumour activity in non-small-cell lung cancer.