Villous duodenal adenoma with II and III grade dysplasia and carcinoma in situ treated with cephalic duodenopancreatectomy

BACKGROUND: Villous duodenal adenomas are rare, tend to malignant alteration and recurrency if surgical procedures are not sufficiently radical. They are mostly localized in the second portion of the duodenum and close to a papilla and are prone to malignant alteration. CASE REPORT: In this paper we presented a 79-year-old female patient already operated on twice elsewhere due to obstructive jaundice caused by villous duodenal adenoma, using, firstly, cholecystectomy and T-tube drainage of the choledoclus, and, then, an unsuccessful attempt of radical surgery--duodenopancreatectomy. The patient was some time wearing a T-tube drainage, and, then, submitted to endoscopic papillotomy, endoscopic stenting, and, finally to the insertion of a self-expandable metallic stent which got clogged three months later causing chalangitis. Three years following the first surgery, the patient presented to our institution and submitted to cephalic duodenopancreatectomy. Histology confirmed villous duodenal adenomas with II and III grade dysplasia and carcinoma in situ. The limph nodes were tumour-free. The postoperative recovery was somewhat prolonged due to cardiologic difficulties and a mild infection of the wound. CONCLUSION: Villous duodenal adenoma is curable if treated correctly and in a proper time. Radical treatment delay increases the risk for malignant alteration which was the case with our patient who was submitted to it three years after the histologic confirmation of the diagnosis.     

Validation of the Serbian version of inflammatory Rasch‐built overall disability scale in patients with chronic inflammatory demyelinating polyradiculoneuropathy

Inflammatory Rasch‐built overall disability scale (I‐RODS) seems to be a valid activity measure for use in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Our aim was to translate and validate the I‐RODS for use in CIDP patients from Serbia. Study comprised 83 patients diagnosed with CIDP. I‐RODS was translated and cross‐culturally validated using the standard guidelines. Following scales were also administered: Medical Research Council (MRC) sum score, Inflammatory Neuropathy Cause and Treatment (INCAT) sensory and disability scores, Krupp's Fatigue Severity Scale, Beck Depression Inventory, Visual Analogue Scale for pain, and health survey‐36 (SF‐36) as a quality of life measure. According to the I‐RODS, significant proportion of our patients reported that “running” (51%), “dancing” (41%), and “standing for hours” (40%) were impossible tasks to perform, while “teeth brushing” (94%), “eating” (88%), and “reading a newspaper/book” (82%) were noted as the easiest items. Patients with more muscle weakness (lower MRC sum score) and more severe INCAT sensory score had lower I‐RODS score (P < .01). Also, patients with fatigue, depression and pain had lower I‐RODS scores (P < .01). I‐RODS score correlated with the INCAT disability score (P < 0.01) was 78 ± 19 compared to 51 ± 30 in patients with INCAT >1 (P < .01). I‐RODS score correlated with the total SF‐36 score (rho = +0.73, P < .01), as well as with all SF‐36 domain scores. Serbian version of the I‐RODS seems to be a valid activity measure for use in CIDP patients. I‐RODS was able to assess different levels of disability, it was in association with impairment measures, INCAT disability scale and quality of life. Further studies are needed to assess its responsiveness.     

Quality of life in adult patients with limb–girdle muscular dystrophies

Although limb–girdle muscular dystrophies (LGMD) can cause permanent disability, to date there are no studies that examined quality of life (QoL) in these patients. Our aim was to evaluate QoL in patients with LGMD, and to identify the most significant predictors of QoL. The study comprised 46 patients with diagnosis of limb–girdle muscular weakness. QoL in patients was evaluated using two scales—SF-36 questionnaire and the Individualized Neuromuscular Quality of Life questionnaire (INQoL). Following scales were also applied: Epworth Sleepiness Scale (ESS), Hamilton Scale for Depression (HamD), and Krupp’s Fatigue Severity Scale (FSS). Mean SF-36 score was 52.4 ± 23.5, and physical composite score was worse than mental. Total INQoL score was 46.1 ± 20.4, with worst results obtained for weakness, fatigue and independence, while social relationships and emotions showed better results. Significant predictors of worse SF-36 score in LGMD patients were higher fatigue level (β = ? 0.470, p < 0.01) and use of assistive device (β = ? 0.245, p < 0.05). Significant predictors of worse INQoL score were higher fatigue level (β = 0.514, p < 0.01) and presence of cardiomyopathy (β = ? 0.385, p < 0.01). It is of special interest that some of the identified factors that correlated with worse QoL in LGMD patients were amenable to treatment.     

Elevated leukocyte count in cerebrospinal fluid of patients with chronic inflammatory demyelinating polyneuropathy

Cerebrospinal fluid (CSF) examination is often part of the diagnostic work‐up of a patient suspected of having chronic inflammatory demyelinating polyneuropathy (CIDP). According to the European Federation of Neurological Societies and the Peripheral Nerve Society (EFNS/PNS) criteria, an elevated protein level without pleocytosis (leukocytes <10 cells/µl) is supportive of the diagnosis CIDP. It is unclear how many CSF leukocytes are compatible with the diagnosis CIDP and how extensive the diagnostic work‐up should be in patients with a demyelinating neuropathy and pleocytosis. We performed a retrospective study at two tertiary neuromuscular referral clinics and identified 14 out of 273 (6%) patients with CIDP with elevated CSF leukocytes (≥10 cells/µl). All these patients met the EFNS/PNS criteria for definite or probable CIDP. Eight patients (57%) presented with a subacute onset and four patients with an antecedent infection. Most patients responded well to therapy, and eight patients are currently in remission. In four patients, lumbar puncture was repeated. A spontaneous decrease in leukocytes before start of treatment was found in three patients. Our data indicate that a mild to moderate pleocytosis in CSF does not exclude the diagnosis of CIDP, especially in patients with a subacute onset of disease.     

What have we learned from surgical treatment of gastric adenocarcinoma? A comparison of two periods

In January 1992, a standardized and more radical surgical approach in gastric cancer treatment and a standardized pathohistological workup of specimens was introduced at our institution. The aim of our retrospectively designed study was to compare the results of two consecutive groups of altogether 348 patients who underwent a total or subtotal gastrectomy for gastric cancer (period A: 162 patients operated between 1992 and 1996; period B: 186 patients operated between 1997 and 2000). The two groups of patients were comparable in age, sex, their general condition, and the proportion of potentially curable (R0) resections. There was a significant difference between the groups with regard to tumor site (fewer distal and more proximal stomach tumors in period B), type of operation (more total gastrectomies in period B), extent of lymphadenectomy (more D2 and D3 in period B), average number of affected lymph nodes (higher in period B), average number of examined nodes (higher in period B), and in UICC stage (less stage II in period B). UICC classification was impossible in 19 patients overall, but there were fewer non-classified patients in period B. The incidence of surgical complications (15.6% vs. 18.7%) and average hospital stay (14.72 days vs. 14.70 days) was not significantly different in the two groups. The drop in overall mortality rate (potentially curable and non-curable resections) in period B almost reached statistical significance (period A: 8%, period B: 6.5%; p = 0.57). The 5-year survival for all R0 resected patients (279) in 9 years was 31%. The median survival was significantly longer in period B (941 days) than in period A (570 days) for R0 and non-curable (R2) resections together (p=0.0132) as well as for R0 resections alone (period A: 844 days, period B: 1176 days; p = 0.0127). Comparison of the two consecutive groups of patients shows that after a defined period of systematic surgical work our results improved, reflected in the higher number of extracted lymph nodes, the higher number of total gastrectomies, and the longer median survival in the second period.     

Rupture of the ureteropelvic junction in childhood: long-term results

Between 1971 and 1982, five children were admitted to our hospital due to rupture of the ureteropelvic junction. In one patient with an extremely late diagnosis, 165 days after trauma and urinoma formation, we performed a nephrectomy. In four patients, one with early recognition during laparotomy and three with delayed diagnosis 104, 35, and 33 days after trauma with resulting urinoma, we carried out a primary anastomosis. Long-term follow-up ranged from 6 to 17 years with regular radiologic examinations disclosing none of the usual long-term complications such as ureteral stenosis, hydronephrosis, urinary tract infection or calculus.     

Disseminated subcutaneous fat necrosis and elbow joint arthritis as a complication of pancreatitis

BACKGROUND: Intraabdominal fat necrosis of the retroperitoneum, mesenthery and omentum is a frequent complication of acute pancreatitis. Very rarely, during the disease multiple aseptic subcutaneous fat necrosis, polyarthritis, polyserositis, vasculitis, subcutaneous nodi and eosinophylia, isolated or in combination, may appear. They are known as "pancreatic disease syndrome". CASE REPORT: We presented a female patient, 43-year-old, in whom in the course of acute interstitial biliary pancreatitis had occur red multiple localized aseptic necrosis of subcutaneous fat tissue of extremities appeared mostly around the talocrural and wrist joints requiring multiple incision, as well as aseptic elbow joints arthritis requiring puncture of one elbow joint. The symptoms were followed by a prolonged febrility that settled within several weeks. CONCLUSION: Localized disseminated fat necrosis around joints, arthritis of major joints, alone or with some of other symptoms of the "pancreatic disease syndrome" have to be considered as a probable sign of pancreatitis, even in the abscence of major abdominal symptoms.     

Amifampridine safety and efficacy in spinal muscular atrophy ambulatory patients: a randomized,placebo-controlled,crossover phase 2 trial

Journal of Neurology - Spinal muscular atrophy (SMA) is an autosomal recessive disease where a deficient amount of SMN protein leads to progressive lower motor neuron degeneration. SMN-enhancing...