2-Year Natural Decline of Cardiac Sympathetic Innervation in Idiopathic Parkinson Disease Studied with 11C-Hydroxyephedrine PET.

The objective of this study was to detect regional patterns of cardiac sympathetic denervation in idiopathic Parkinson disease (IPD) using 11C-hydroxyephedrine (11C-HED) PET and determine the denervation rate over 2 y. METHODS: We obtained 62 cardiac 11C-HED PET scans in 39 patients (30 men and 9 women; mean age ± SD, 61.9 ± 5.9 y), including 23 patients with follow-up scans at 2 y. We derived 11C-HED retention indices (RIs; mL of blood/min/mL of tissue) reflecting nerve density and integrity for 480 left ventricular (LV) sectors. We compared IPD patients with 33 healthy controls using z score analysis; RI values ≤ 2.5 SDs were considered abnormal. We expressed global and regional LV denervation as the percentage extent of z score severity and severity-extent product (SEP) on 9-segment bullseye maps and decline in cardiac sympathetic innervation as the 2-y difference in SEP (diff-SEP). RESULTS: Baseline 11C-HED PET in the 39 IPD patients revealed an RI mean of 0.052 ± 0.022 mL of blood/min/mL of tissue. In comparison with data from normal controls, 12 patients had normal 11C-HED PET, 5 showed mild denervation (percentage extent < 30%), and 22 had moderate to severe denervation (percentage extent > 30%, z score ≤ 2.5 SD). In the 23 paired PET scans, worsening cardiac denervation (global diff-SEP > 9) occurred in 14 of 23 (60.9%) patients over 2 y, including percentage LV abnormality (59% increasing to 66%), z-severity (-2.4 down to -2.5), and SEP (-195 to -227) (P = 0.0062). We found a mean annual decline of 4.6% ± 5.6 (maximum, 13%) in 11C-HED retention from a baseline global RI mean of 0.0481 ± 0.0218 to 0.0432 ± 0.0220 (P = 0.0009). At baseline, 5 patients with normal uptake had no interval change; 3 with mild denervation developed interval decline in lateral and inferior segments (diff-SEP -82 to -99) compared with anterior and septal segments (-65 to -79), whereas the reverse pattern occurred in 15 patients with severe baseline denervation. CONCLUSION: Progressive decline in cardiac sympathetic neural integrity in IPD patients occurs at a modest rate over 2 y on 11C-HED scans with marked heterogeneity and a regional pattern of involvement and decline.

An Unusual Case of Adult-Onset Still's Disease with Hemophagocytic Syndrome, Necrotic Leukoencephalopathy and Disseminated Intravascular Coagulation.

Case. A 34-year-old African-American female with a history of adult-onset Still's disease presented to an outside hospital with oligoarthritis. She experienced a generalized tonic-clonic seizure en route via ambulance, was intubated upon arrival, and transferred to the intensive care unit for treatment of suspected pneumonia and sepsis. She subsequently developed generalized cutaneous desquamation that progressed despite the cessation of antibiotics and other potential offending drugs which required transfer to our hospital's burn unit. She was suspected to have reactive hemophagocytic syndrome based on her clinical presentation of fever, rash, polyarthritis, elevated liver enzymes, coagulopathy, splenomegaly, normocytic anemia, thrombocytopenia, hypertriglyceridemia, hyperferritinemia, and hemophagocytosis visualized in bone marrow biopsy specimen. Magnetic resonance imaging demonstrated necrotic demyelination of the deep white matter and corona radiata. The patient developed multiorgan dysfunction and DIC without any other attributable etiology. Despite aggressive broad spectrum therapy and high dose of steroids she progressively deteriorated and eventually expired. Conclusion. Previous publications have highlighted the prevalence of necrotic leukoencephalopathy in children with familial hemophagocytic syndrome. Our patient demonstrated some uncommon features complicating her HLH including DIC and necrotic leukoencephalopathy, which are very rare entities in AOSD.

What are the clinical implications of nodular gastritis? Clues from histopathology.

There is no widely accepted histopathological definition for nodular gastritis. In this study we aim to uncover the pathologic entity responsible for the nodular appearance and to find clues about the clinical implications of nodular gastritis. Antral biopsy specimens of 160 patients with nodular gastritis and 133 patients without nodular gastritis were examined by an experienced pathologist for dysplasia, foveolar hyperplasia, inflammatory activity, intraepithelial lymphocytosis, intestinal metaplasia, and lymphoid follicle/aggregate formation, and comparative analysis was performed between the two groups of patients. The presence of intraepithelial lymphocytosis was more frequent in patients with nodular gastritis (P < 0.05). There was no difference between the two groups regarding the other pathological features such as presence of dysplasia, inflammatory activity, intestinal metaplasia, lymphoid hyperplasia, and Helicobacter pylori (H. pylori) infection. Increase of intraepithelial lymphocytes may contribute to formation of macroscopical nodules in this peculiar type of gastritis. Nodular gastritis would not indicate a new therapeutic approach in addition to the current measures for Helicobacter pylori infection.

Xanthoma disseminatum in a child with cranial bone involvement.

Xanthoma disseminatum is a normolipemic xanthomatosis classified in the group of non-Langerhans cell histiocytic disorders. Classic clinical findings are cutaneous and mucosal xanthomas and diabetes insipidus. Ocular, liver, bone marrow, respiratory, skeletal, and central nervous system involvement may accompany the classic findings of the disease. Skeletal involvement is a rare manifestation of the disease in children. We report on a 5-year-old boy with cranial bone involvement, proptosis and xanthomatous cutaneous lesions, treated successfully with systemic corticosteroids.

Intraneural hemangioma of digital nerve diagnosed with MR imaging.

Hemangioma may occur at any location. Although the imaging findings of hemangioma are well known, it may be difficult to differentiate this entity from other soft-tissue tumors. Intraneural hemangiomas are extremely rare. We present a girl with a painful swelling in the fourth web space of her right hand. Since it was painful, the referring clinician considered the possibility of a glomus tumor. However, demonstration of dilated feeding or draining vessels suggested the diagnosis of hemangioma. At surgery a hemangioma of the ulnar digital nerve of the ring finger was resected marginally.

Benign mixed epithelial and stromal tumor of the kidney.

A 51-year-old, perimenopausal, female patient with 1-month history of right flank pain who was diagnosed with a renal mass and underwent nephron-sparing partial nephrectomy is presented. The renal mass was found to be a benign, biphasic tumor composed of an epithelial component, consisting of ducts of variable size scattered within a mesenchymal component, composed of spindle cells arranged in sheets and fascicles. No atypia, mitosis, or necrosis was found. The spindle component shows desmin, smooth muscle actin, and estrogen and progesterone receptor positivity immunohistochemically. The diagnosis of benign mixed epithelial and stromal tumor of the kidney is rendered. No recurrent disease has been detected during 2 years of follow up.

A novel approach for preventing esophageal stricture formation: sphingosylphosphorylcholine-enhanced tissue remodeling.

Using a new class of intracellular 2nd messengers to prevent stricture formation after caustic ingestion, sphingosylphosphorylcholine (SPC) has a wide spectrum of activity in cell growth regulation and signal transduction. Caustic esophageal burns were created with 15% NaOH in an experimental rat model. Control group animals (n = 10) had esophageal burns with no treatment, whereas the SPC group (n = 10) had esophageal burns gavaged with SPC for 7 days. Efficacy of treatment was assessed in 28 days by contrast esophagograms, histopathologic evaluation, and biochemically by tissue hydroxyproline (OHP) content. Contrast esophagograms demonstrated that SPC significantly prevented stricture formation. Obvious collagen deposition was present in submucosa, muscularis mucosa, and muscular layers in the control group compared with the SPC group. The damage to the esophageal wall on histopathologic examination was significantly lower in the SPC group (p < 0.05). Tissue OHP contents were significantly lower in the SPC-treated group (3.0 +/- 0.1 microg/mg) compared with the control group (4.3 +/- 0.2 microg/mg) (p < 0.05). We conclude that SPC improves healing following caustic esophageal burns. Furthermore, SPC is effective in preventing caustic esophageal strictures. These effects of SPC occur through its proliferative and specifically its remodeling effects on wound healing.

Isolated late penile metastasis of an adenocarcinoma of the rectum.

Penile metastatic tumors are relatively infrequent and very rare compared to extraurogenital tumor metastasis. We present an interesting rare occurrence of a late penile metastasis on an adenocarcinoma of the rectum.

The effects of verapamil vs. allopurinol on intestinal ischemia/reperfusion injury in rats. "An experimental study".

BACKGROUND/AIMS: Although studies have reported that xanthine oxidase inhibitors or calcium channel blockers attenuate the ischemia-reperfusion injury in several organ systems, no comparative study exists on the significance of each of these pathways. To study this, in anesthetized Wistar Albino rats, a surgical model for intestinal ischemia-reperfusion injury was employed. METHODOLOGY: In experimental animals, after laparotomy, the superior mesenteric artery was occluded for 30 min, followed by a 2-h period of reperfusion; control rats underwent only a sham laparotomy procedure. One group of experimental animals was pretreated intraperitoneally with the calcium channel blocker verapamil (0.3 mg/kg), another group with the xanthine oxidase inhibitor allopurinol (100 mg/kg), the third group received no pretreatment. Plasma lactate, malondialdehyde and glutathione levels as well as intestinal tissue malondialdehyde and glutathione levels were measured to assess for possible protective effects. Histologic evaluation of the extent of injury was also performed. RESULTS: Irreversible tissue damage was depicted in the untreated group, and partially in the allopurinol pretreatment group by histologic examination. Ischemia-reperfusion injury was reversible in the verapamil group. The laboratory results also supported these findings. CONCLUSIONS: Protective effects of verapamil on ischemia-reperfusion injury have been found to be significantly (p<0.0001) more effective compared to allopurinol.

Effect of different enteral nutrients on bacterial translocation in experimental obstructive jaundice.

Obstructive jaundice leads to bacterial translocation (BT) by disruption of the gut barrier, intestinal microecology, and impaired host immune defence. The objective of the present study is to investigate the effects of different enteral nutrients on BT that is induced by obstructive jaundice in rats. Eighty male Wistar-Albino rats were randomly assigned into 4 groups. Group 1: 20 rats underwent laparotomy, common bile duct (CBD) was not actually ligated and transected, but sham ligation of CBD was performed. Groups 2-4: 60 rats underwent laparotomy, CBD ligation and transection. Group 1 and 2 rats were given rat chow, group 3 rats were fed a glutamine and arginine supplemented enteral diet, and group 4 rats were fed an arginine, m-RNA and omega-3 supplemented enteral diet, an immunonutrient. Rats in groups 3 and 4 had significantly less BT to mesenteric lymph nodes compared to rats in group 2 (p = 0.001). These findings suggest that oral administration of an arginine and glutamine supplemented diet and immunonutrition reduce BT in rats with obstructive jaundice.

A 34-week-old male fetus with short rib polydactyly syndrome (SRPS) type I (Saldino-Noonan) with pancreatic cysts.

A 34-week-old male fetus (first diagnosed at 28 weeks of gestation) with short rib polydactyly type I Saldino-Noonan syndrome is presented in this study. In the postmortem examination of the fetus, pancreatic dysplasia, multiple cysts and multicystic dysplastic kidneys, omphalomesenteric cyst, ascites, malrotation, micropenis, undescended testes, bilateral inguinal hernia and hydrops were observed. The parents were first-degree cousins. One male and one female sibling had similar findings and both had died after birth. Only a four-year-old healthy daughter was alive. We believe these findings will be helpful in the differential diagnosis of further lethal skeletal dysplasia cases.

Evaluation of the Helicobacter pylori stool antigen test (HpSA) for the detection of Helicobacter pylori infection and comparison with other methods.

BACKGROUND/AIMS: Several invasive and non-invasive methods are available for the detection of H. pylori infection. The accuracy of anti-H. pylori antibodies in serum is low. There is a need for a quick, inexpensive and reliable non-invasive test to detect H. pylori. The aim of this study was to evaluate the enzyme immunoassay for the detection of H. pylori antigen in stool in the Turkish population and compare it to other methods. METHODOLOGY: 50 patients who were admitted to Hacettepe University Department of Internal Medicine, Division of Gastroenterology with the symptom of dyspepsia for whom the indication of upper gastrointestinal endoscopy was present were included in the study. With their permission stool samples were taken. The patients were evaluated with histology, culture, serology, rapid urease test and HpSA (Helicobacter pylori Stool Antigen test). Forty-one patients had gastritis and biopsies were taken from those. RESULTS: Excluding HpSA if three of the rest of four methods were positive, patients were accepted as H. pylori positive. Nineteen patients were positive for H. pylori, 22 were negative. HpSA was positive in 16 of 19. The sensitivity and specificity of the methods were as follows: histology 100% sensitive, and 86% specific, culture 63% and 100%, HpIgG 58% and 73%, rapid urease test 89% and 82%, respectively. The results were as 84% and 82% for HpSA. Comparing with the 'Gold Standard' histology using McNemar's test Kappa results were as 0.610, 0.181, 0.610, 0.708 for culture, HpIgG, Rapid Urease Test and HpSA, respectively. CONCLUSIONS: HpSA is a cheap, effective method for the diagnosis of H. pylori infection in the Turkish population.

Prefabrication of a free peripheral nerve graft following implantation on an arteriovenous pedicle.

Extensive nerve injuries frequently necessitate the use of long autografts, and sources of expendable donor nerves are limited. It is for these cases that nerve transplantation would have its greatest potential. However, regeneration in the rejected allograft fails because of a lack of the positive neurotropic and neurotrophic influences physiologically provided by viable Schwann cells. This report aims to show the feasibility of vascularization of the peripheral nerve by prefabrication. The study was designed to vascularize an autogenous nerve graft segment by using an arteriovenous bundle in the rabbit. A 3.5-cm segment of sciatic nerve was harvested and implanted in between the femoral vessels, and was isolated from secondary revascularization by a custom-made tube. A peripheral nerve graft was prefabricated by implantation on the vascular pedicle, and neovascularization was evaluated by microangiography and histology. The graft exhibited early neovascularization on day 2, and numerous new capillaries were noted to restore primarily perineurial blood flow on day 7, then all along the graft on day 14. The viability of the Schwann cells was preserved, and the structural integrity of the graft was maintained. This is a preliminary report on secondary vascularization of a segment of an autogenous nerve to maintain the viability of Schwann cells and the integrity of the conduit. In the future, with the concomitant use of host immunosuppression or with more advanced pre-treatment methods, nerve allografts could be revascularized by vascular bundles. The current tempo of medical research will hopefully enable the use of fresh nerve allografts that are rendered less immunogenic by more refined techniques.

Primary leiomyosarcomas of the skin.

We report four cases of cutaneous, and one of subcutaneous, leiomyosarcoma with the results of histological examinations and immunohistochemical studies. Subcutaneous leiomyosarcomas have more likelihood of metastases than the cutaneous type. Only one of our cutaneous leiomyosarcomas recurred after 18 months and the patient with a subcutaneous lesion had lung metastases after five years.

Synthesis and anti-inflammatory activity of 1-acylthiosemicarbazides, 1,3,4-oxadiazoles, 1,3,4-thiadiazoles and 1,2,4-triazole-3-thiones.

Sixteen 1-(2-naphthyloxyacetyl)-4-substituted-3-thiosemicarbazide, 2-(2-naphthyloxymethyl)-5-substitutedamino-1,3,4-oxadiazole, 2-(2-naphthyloxymethyl)-5-substitutedamino-1,3,4-thiadiazole and 5-(2-naphthyloxymethyl)-4-substituted-1,2,4-triazole-3thione derivatives have been prepared and evaluated as orally active anti-inflammatory agents with reduced side-effects. The structures of the compounds were confirmed by IR and 1H NMR spectral data and microanalysis. The anti-inflammatory and ulcerogenic activities of the compounds were compared with naproxen, indomethacin and phenylbutazone. In carrageenan-induced foot pad edema assay, 2-(2-naphthyloxymethyl)-5-methylamino-1,3,4-oxadiazole, 5-(2-naphthyloxymethyl)-4-methyl-1,2,4-triazole-3-thione and 5-(2-naphthyloxymethyl)-4-ethyl-1,2,4-triazole-3-thione showed an interesting anti-inflammatory activity. In the air-pouch test, 1,3,4-oxadiazole and 1,2,4-triazole-3-thione derivatives reduced total number of leukocytes of the exudate that indicates excellent inhibition of prostaglandin production. Side effects of the compounds were examined on gastric mucosa, liver and stomach and none of the compounds showed significant side effects compared with reference nonsteroidal anti-inflammatory drugs (NSAIDs).