Temporal changes in PO2 of R3230AC tumors in Fischer-344 rats

It has been hypothesized that abdominal trauma may be one of the factors involved in membranous obstruction of the inferior vena cava. We present two cases of membranous obstruction of the inferior vena cava associated with trauma. One asymptomatic case, associated with an occult myeloproliferative disorder, developed within 3 years of a violent abdominal trauma. The other case, associated with familial plasminogen deficiency, was discovered at surgery 3 days after a road accident with obvious abdominal trauma, since superimposed extensive thrombosis of the inferior vena cava caused acute Budd-Chiari syndrome. We conclude that underlying prothrombotic conditions are probably necessary for the development of membranous obstruction of the inferior vena cava and that minor trauma may contribute to the development of thrombosis through indirect mechanisms.     

Hepatic vein stenting for Budd-Chiari syndrome

This report describes two patients with Budd-Chiari syndrome with intractable ascites due to a tight hepatic vein stenosis while the other hepatic veins were occluded. Percutaneous transluminal angioplasty of the hepatic vein stenosis followed by insertion of expandable metallic stents reduced the pressure gradient across the stenosis to almost zero. In both patients, ascites disappeared and diuretic therapy could be reduced significantly. This treatment has remained effective for more than 1 yr in one case and 2 yr in the other. These cases demonstrate the feasibility of hepatic vein stenting as a therapy for hepatic venous outflow obstruction. This therapy may be used in selected patients to defer and perhaps avoid shunt-surgery or liver transplantation.     

Myelodysplasia, vasculitis and anti-neutrophil cytoplasm antibodies

A cutaneous or systemic vasculitis occurs in myelodysplasia as well as in myeloproliferative and lymphoproliferative disorders. The most common lesion is a leucocytoclastic vasculitis, with neurological or joint involvement occurring less often. The vasculitis may appear contemporaneously with or precede the clinical onset of the blood dyscrasia. Occasionally the lesions respond dramatically to the use of steroids but in general, patients with vasculitis have a worse prognosis than those with uncomplicated myelodysplasia. Vasculitis and myelodysplasia appear together too often for the association to be coincidental and the vasculitis in most cases cannot be attributed to intercurrent infections, therapeutic agents or a pre-existing rheumatological disorder. While autoantibodies are frequently present in myelodysplasia, and ANA and anti-neutrophil cytoplasm antibodies (ANCA) are found in other vasculitides, neither of these antibodies is associated with the vasculitis of myelodysplasia. There has however been one report of ANCA in Sweet's syndrome a non-vasculitic skin condition that also occurs in the myelodysplastic syndromes.     

Human immunodeficiency virus infection, Part II

The acceptance of highly active antiretroviral therapy (HAART) among patients and health care providers has had a dramatic impact on the epidemiology and clinical characteristics of many opportunistic infections associated with human immunodeficiency virus (HIV). Previously intractable opportunistic infections and syndromes are now far less common. In addition, effective antibiotic prophylactic therapies have had a profound impact on the risk of patients developing particular infections and on the incidence of these infections overall. Most notable among these are Pneumocystis carinii, disseminated Mycobacterium avium complex, tuberculosis, and toxoplasmosis. Nevertheless, infections continue to cause significant morbidity and mortality among patients who are infected with HIV. The role of HAART in many clinical situations is unquestioned. Compelling data from clinical trials support the use of these therapies during pregnancy to prevent perinatal transmission of HIV. HAART is also recommended for health care workers who have had a "significant" exposure to the blood of an HIV-infected patient. Both of these situations are discussed in detail in this article. In addition, although more controversial, increasing evidence supports the use of HAART during the acute HIV seroconversion syndrome. An "immune reconstitution syndrome" has been newly described for patients in the early phases of treatment with HAART who develop tuberculosis, M avium complex, and cytomegalovirus disease. Accumulating data support the use of hydroxyurea, an agent with a long history in the field of myeloproliferative disorders, for the treatment of HIV. Newer agents, particularly abacavir and adefovir dipivoxil, are available through expanded access protocols, and their roles are being defined and clarified.     

Splenic irradiation in the palliation of patients with lymphoproliferative and myeloproliferative disorders

INTRODUCTION: Splenic irradiation is an accepted mode of treatment for palliation of hypersplenism and splenic pain for patients with lymphoproliferative or myeloproliferative disorders. However, results are conflicting regarding the duration of palliation and the toxicity associated with this treatment. METHODS: Twenty-five patients with lymphoproliferative or myeloproliferative disorders were treated with splenic irradiation for palliation of splenomegaly and pain. The spleen was measured and pain and toxicity were assessed during radiation therapy. RESULTS: Splenomegaly and splenic pain decreased in 60 percent and 91 percent of patients, respectively. Radiation doses higher than 500 cGy appeared to be more effective than lower doses in reducing the spleen size in patients with chronic lymphocytic leukemia. Regression of splenomegaly and pain relief were maintained for less than one year and more than six months, respectively. Acute radiation toxicity resulted in the cessation of radiotherapy in two patients. CONCLUSION: Splenic irradiation is effective in the short-term palliation of splenomegaly and pain and may be most useful in the subset of patients with a life expectancy of less than one year. Terminally ill patients with splenomegaly secondary to lymphoproliferative or myeloproliferative disorders may benefit from splenic irradiation to minimize pain and pressure symptoms in addition to possible reduction of narcotic use.     

The present state of pathophysiology and therapeutic trials in polycythemia vera

Polycythemia vera shares basic features of pathogenesis with other subtypes of the group of chronic myeloproliferative disorders. All myelopoietic cells are derived from one transformed hemopoietic stem cell. Genetic instability of mitotic clonal cells explains the risk of leukemic transformation, which may be enhanced by cytoreductive treatment. Recent data show that erythroid hyperplasia is not due to erythropoietin hypersensitivity, but rather to abnormal stimulation by other cytokine growth factors. Treatment as established by clinical trials has almost normalized life expectancy in older patients, but the optimal strategy for subgroups of patients is still unknown. For younger patients, new and potentially curative approaches should be investigated.     

Antidiarrheal agents for the management of treatment-related diarrhea in cancer patients

The efficacy and use of antidiarrheal agents in patients who diarrhea associated with cancer treatments are reviewed. Diarrhea is common in cancer patients and may interfere with cancer treatment. Diarrhea may be induced by chemotherapy, radiation therapy, surgery, graft-versus-hot disease (GVHD) or infection after bone marrow transplantation, and other causes. The general goal of antidiarrheal therapy is to reduce fluid loss in the stool by inhibiting intestinal secretion, promoting absorption, and decreasing intestinal motility. Antidiarrheal agents may be classified as intestinal transit inhibitors, intraluminal agents, proabsorptive agents, and antisecretory drugs. Opiate agonists are the most commonly used intestinal transit inhibitors; they can be effective in treating cancer treatment-related diarrheas but must be used cautiously. Intraluminal agents include clays, activated charcoal, and cholestyramine; these adsorbents and other binding resins can interfere with the absorption of orally administered antidiarrheals and other drugs and are unlikely candidates for use in most cases of diarrhea in cancer patients. Clonidine, a proabsorptive agent, should be used only in patients with secretory diarrhea refractory to opiate agonist treatment. Octreotide is an antisecretory drug that has shown considerable efficacy in clinical trails as a treatment for diarrhea caused by chemotherapy of GVHD; its use for radiation therapy-induced diarrhea, although not studied clinically, is nevertheless an option. In general, opiate agonists and octreotide appears to offer the most efficacy and flexibility. Opiate agonists and octreotide are effective agents for cancer treatment-related diarrhea.     

Anagrelide, a selective thrombocytopenic agent

The pharmacology, pharmacokinetics, clinical efficacy, adverse effects, and dosage and administration of anagrelide are reviewed. Anagrelide is a selective thrombocytopenic agent with FDA-approved labeling for the treatment of essential thrombocythemia. Clinical trials have shown that the drug may have a role in the treatment of other chronic myeloproliferative disorders, including polycythemia vera, chronic myeloid leukemia, and agnogenic myeloid metaplasia. The mechanism by which anagrelide reduces platelet count is not yet clear. The current hypothesis is that anagrelide affects the late (postmitotic) phases of megakaryocyte development. Anagrelide has a large volume of distribution and is extensively metabolized; less than 1% is recovered unchanged in the urine. Plasma half-life after a 0.5-mg dose is 1.3 hours. Anagrelide's efficacy and safety have been evaluated in open-label, noncomparative trials, in which the response rate was 60-93%. Adverse effects include headache, diarrhea, edema, palpitations, and abdominal pain. Patients with renal or hepatic dysfunction need to be closely monitored for signs of toxicity. The recommended starting dosage is 0.5 mg four times a day or 1 mg twice a day, with dosage adjustment to the lowest effective amount required to reduce and maintain platelet count below 600 x 10(9)/L. The wholesale acquisition price for 0.5-mg capsules is $350 per 100. Whether anagrelide will replace hydroxyurea as first-line therapy in some or all patients remains to be determined. Anagrelide is effective in the treatment of essential thrombocythemia and may have a role in the treatment of other myeloproliferative disorders.     

Lansoprazole. An update of its pharmacological properties and clinical efficacy in the management of acid-related disorders

Lansoprazole is a proton pump inhibitor that reduces gastric acid secretion. It has proved effective in combination regimens for the eradication of Helicobacter pylori and as monotherapy to heal and relieve symptoms of gastric or duodenal ulcers and gastro-oesophageal reflux. After initial healing, it may be used to prevent recurrence of oesophageal erosions or peptic ulcers in patients in whom H. pylori is not the major cause of ulceration and to reduce basal acid output in patients with Zollinger-Ellison syndrome. Usual dosages are 15 to 60 mg/day, although dosages of < or = 180 mg/day have been used in patients with hypersecretory states. In patients with duodenal or gastric ulcer, short term lansoprazole monotherapy was similar to omeprazole and superior to histamine H2 receptor antagonists in achieving healing rates > 90%. Lansoprazole was as effective a component of H. pylori eradication regimens as omeprazole, tripotassium dicitrato bismuthate (colloidal bismuth subcitrate) or ranitidine. Lansoprazole was superior to ranitidine in symptom relief and healing of gastro-oesophageal reflux disease and tended to relieve symptoms more rapidly than omeprazole, although initial healing was similar. As maintenance treatment, lansoprazole was similar to omeprazole and superior to ranitidine in relieving symptoms and preventing relapse. Lansoprazole was also superior to ranitidine in healing and relieving symptoms of oesophageal erosions associated with Barrett's oesophagus; healing was maintained for a mean of 2.9 years in > or = 70% of patients. Lansoprazole was also superior to ranitidine in prophylaxis of redilatation of oesophageal strictures. After > or = 4 years of use in patients with Zollinger-Ellison syndrome, lansoprazole 60 to 180 mg/day effectively controlled basal acid output. Dosages may be reduced in some patients once healing and symptom relief has been achieved. Preliminary studies of lansoprazole in patients at risk of aspiration pneumonia or stress ulcers show promise. Although studies show lansoprazole is potentially effective in treating gastrointestinal bleeding, future studies should assess patients' H. pylori status. Lansoprazole has been well tolerated in clinical trials, with headache, diarrhoea, dizziness and nausea appearing to be the most common adverse effects. Tolerability of lansoprazole does not deteriorate with age and the drug is well tolerated in long term use (< or = 4 years) in patients with Zollinger-Ellison syndrome or reflux disease. Thus, lansoprazole is an important alternative to omeprazole and H2 receptor antagonists in acid-related disorders. In addition to its efficacy in healing or maintenance treatment, it may provide more effective symptom relief than other comparator agents.     

Post-traumatic thrombosis of the portal vein

Blunt trauma to the abdomen is an exceptional cause of portal vein thrombosis. To our knowledge, 8 cases have been reported in the literature. When thrombosis of the portal vein occurs, a complete search for all the known main causes must be carried out before entertaining this diagnosis. Other causes may be cirrhosis, tumors and inflammation of the abdomen, coagulation disorders and hematologic diseases including latent myeloproliferative syndrome. We report a case in a 25-year-old man with an uneventful past history who presented with thrombosis of the portal vein after a violent blunt trauma which occurred during a rugby play. In this young man, none of the other potential causes was found, in particular bone marrow culture on medium with low growth-factor concentration allowed us to eliminate a latent myeloproliferative syndrome. The only triggering factor remaining was the recent abdominal trauma. After an 18-month follow-up, no other element has been observed which could have caused thrombosis of the portal vein.     

Issues in the monopharmacotherapy and polypharmacotherapy of obsessive-compulsive disorder

Polypharmacotherapy is again becoming common place in clinical practice. Obsessive-compulsive disorder (OCD) as a single primary diagnosis is responsive exclusively to the serotonin reuptake inhibitors (SRIs) and this fact forms the major evidence supporting a central role for 5-HT (serotonin) in the pathogenesis of the disorder. Presently, the highly potent serotonin reuptake inhibitors clomipramine, fluoxetine, fluvoxamine, and paroxetine are the only agents approved by the Food and Drug Administration (FDA) for OCD, but there is evidence that other SRIs, such as sertraline, are also effective. Because OCD is often treatment refractory and highly comorbid with other psychiatric disorders, the use of polypharmacotherapy can be justified. Other serotonergic medications such as lithium, buspirone, trazodone, or fenfluramine may be useful as adjuvant treatments in treatment-refractory OCD and adjuvant antipsychotics are useful in tic disorders, personality disorders, and psychotic disorders. The usefulness of polypharmacotherapy should be tempered by adverse effects including the serotonin syndrome, withdrawal phenomena, extrapyramidal side effects, and drug-drug interactions.     

Changes in the activities of antioxidant enzymes of the lymphoid organs of 21-day pregnant rats due to administration of fish oil by gavage

Budd-Chiari syndrome (BCS) due to membranous obstruction of the hepatic vein and the inferior vena cava is rare in children. We report a child with BCS that had a membranous obstruction at the level of the hepatic veins. The web was successfully dilated percutaneously by balloon catheters. Symptoms and signs of obstruction improved without any complication. As percutaneous catheterization is an effective, safe and repatable procedure, we recommend this technique for treatment of children and adults with BCS due to membranous obstruction of the hepatic veins.     

Depressive symptoms associated with gonadotropin-releasing hormone agonists

The gonadotropin-releasing hormone (GnRH) agonists are a relatively new class of drugs that are potentially effective in treating disorders that are aggravated either by estrogen or testosterone. GnRH agonists are effective in the treatment of endometriosis, as well as other disorders, such as advanced prostrate cancer, precocious puberty and uterine leiomyomata. While the GnRH agonists reduce the extent of the endometrial lesions and the occurrence of pelvic pain associated with endometriosis, these agents are associated with physical and psychiatric side effects. The adverse effects of these agents are consistent with the physiological effects of ovarian suppression, such as vasomotor instability, vaginal dryness, and headaches. Preliminary results of a prospective, double-blind placebo-controlled study and an open label trial indicates that depressive mood symptoms increase in women treated with GnRH agonist therapy for endometriosis. Additional evidence suggest that sertraline effectively manages depressive mood symptoms associated with GnRH agonist therapy. The reason for the decline in mood on GnRH agonists is postulated to be associated with the decline in estrogen levels. Effective treatment strategies for depressive mood symptoms in women on GnRH agonists therapy may offer insight into the mechanisms of action of estrogen on mood.     

Use of transjugular intrahepatic portosystemic shunt as a bridge to transplantation in fulminant hepatic failure due to Budd-Chiari syndrome

We report a case of fulminant hepatic failure in a 55-yr-old man due to Budd-Chiari syndrome in the setting of polycythemia rubra vera. The patient presented with acute hepatic failure, which rapidly progressed to grade IV hepatic encephalopathy. Placement of a transjugular intrahepatic portosystemic shunt resulted in marked improvement of the encephalopathy and stabilized the liver failure. Subsequently, he underwent successful nonemergent orthotopic liver transplantation. Transjugular intrahepatic portosystemic shunt placement is a safe, effective, therapeutic option to bridge patients with fulminant Budd-Chiari to liver transplantation.     

Recent developments in the pharmacotherapy of attention-deficit/hyperactivity disorder (ADHD).

In comparison to other mental health disorders in pediatric populations, there seems to be compelling evidence-based support for both the efficacy of stimulant medication for children and adolescents with attention deficit/hyperactivity disorder (ADHD) and associated symptoms. Tricyclic antidepressants provide an alternative when stimulants are found to be ineffective or associated with too many adverse effects, particularly for children who have comorbid conditions including anxiety and depression. Finally, the alpha-adrenergic agents, although not approved for the management of ADHD, have been widely employed clinically, particularly for the treatment of impulsivity, overactivity, and aggression. Behavior therapy has been demonstrated to be effective for many of the functional impairments associated with ADHD. Research efforts are needed to examine these therapies either alone or in combination on the long-term outcome of children with ADHD. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Minimizing alcohol exposure of the breastfeeding infant

A 50-year-old woman developed an acute febrile dermatosis on two occasions concurrently with recurrent Crohn's disease of the colon. Based on the presence of painful erythematous plaques on both hands and forearms, on the leukocytosis with excess bands in peripheral blood, on the histology showing dermal infiltration by mature granulocytes, and on the prompt response to steroids, the diagnosis was made of Sweet's syndrome associated with Crohn's disease. Sweet's syndrome is thought to be a hypersensitivity reaction that leads to parainflammatory (e.g., infections, autoimmune disorders, vaccinations) and paraneoplastic (myeloproliferative disorders, solid malignancy) associations, with a frequency of 10-30%. The association of Sweet's syndrome with Crohn's disease is very rare, but the gastroenterologist should readily differentiate it; it is important to be aware that such patients may have a nonspecific elevated activity index owing to the underlying dermatosis.     

New uses for old vitamins. The treatment of myelodysplastic disorders

The treatment of myelodysplastic disorders with vitamins A and D or derivatives and nutrients has been less than satisfactory. Combinations of vitamin D3 and 13-CRA with or without cytosine arabinoside appear to offer no advantage over any of the single agents alone. Until other vitamin D derivatives are developed that are effective but do not cause hypercalcemia, vitamin D3 cannot be recommended for the treatment of MDS. 13-CRA has been shown to be effective in some patients with MDS; however, it cannot be recommended as standard therapy because of the conflicting data cited above. Further clinical trials with 13-CRA perhaps in combination with vitamin E or colony-stimulating factors are clearly indicated for this disease for which we have no effective therapy.     

Two-step procedure in Budd-Chiari syndrome with severe intrahepatic vena cava stenosis: vena cava stenting and portocaval shunt

Budd-Chiari syndrome is characterized by hepatic venous outflow obstruction, which often leads to death as a result of portal hypertension and liver failure. Venous decompressive shunt surgery and liver transplantation represent efficient surgical treatments of Budd-Chiari syndrome. In the case presented here, severe intrahepatic compression of the inferior vena cava (IVC) was caused by the hypertrophic caudate lobe. A mere portocaval shunt was not feasible because of a large pressure gradient across the intrahepatic stenosis. A two-step procedure with preoperative radiological dilation and stenting of the intrahepatic IVC followed by a portocaval shunt was successfully performed. Consequently, liver transplantation and its subsequent immunosuppression could be avoided.     

Hyperkinetic syndrome (attention deficit-/hyperactivity disorder) in adulthood

The clinical picture of adult hyperkinetic syndrome (HKS) or attention deficit/hyperactivity disorder is nearly unknown in Germany. It can be estimated, that approximately one third of affected children also show symptoms as adults. In the combined type of the syndrome symptoms of inattention as well as of hyperactivity and impulsivity are present, a predominantly inattentive or hyperactive-impulsive type is possible. Retrospective diagnosis of HKS in childhood can be difficult. Disorganization, emotional disturbances and stress intolerance are common in adults with HKS as well as residual symptoms of learning disorders like dyslexia, dyscalculia and dysgraphia. In differential diagnosis especially affective, anxiety and antisocial personality disorders have to be considered, for which on the other side a frequent comorbidity with HKS is known. There is strong evidence for genetic transmission. Neurobiological findings revealed dysregulation of neurotransmitters. For treatment stimulants as pemoline and methamphetamin are effective, in addition tricyclic antidepressants or beta blockers; positive effects are probable for moclobemide, bupropion, fluoxetine and venlafaxine.     

Prostate cancer masquerading as a zygomatic posttraumatic mass

INTRODUCTION: Idiopathic hypereosinophilic syndrome is an uncommon disease often associated with diverse non-specific skin manifestations. Mucosal ulcerations suggest a myeloproliferative from with poor prognosis due to possible progression to malignant hemopathy or visceral complications. CASE REPORT: A 28-year-old man presented idiopathic hypereosinophilia with isolated mucosal ulcerations involving the buccal and genital areas. Laboratory results (hematology, CD25) suggested a myeloproliferative form. Treatment with alpha interferon (18 months) led to regression of the mucosal lesions and a decrease in the markers of eosinophil toxicity. There was no visceral involvement. DISCUSSION: Immunosuppression with/without high-dose alpha interferon is usually used for the treatment of hypereosinophilic syndrome. In our case favorable outcome was obtained with lower doses of alpha interferon than those reported in the literature. There was objective decrease in eosinophil toxicity (regular counts of hypodense eosinophils, CD25 or interleukin 2 soluble receptor) and no progression (malignant hemopathy, mortal visceral involvement).