An aggressive and lethal course of Churg–Strauss syndrome with alveolar hemorrhage,intestinal perforation,cardiac failure and peripheral neuropathy

Churg–Strauss syndrome (CSS) is a rare type of necrotizing vasculitis affecting small to medium-sized vessels typically characterized by asthma, lung infiltrates, necrotizing granulomas and hypereosinophilia. Herein, we describe a case of CSS presenting severe and aggressive course. A 35-year-old male patient with weight loss, dyspepsia, dyspnea and hemoptysis was admitted. The laboratory analyses indicated a remarkable eosinophilia, elevated levels of serum total IgE and positive cANCA. Thorax CT findings were suggestive of alveolar hemorrhage. Bronchoalveolar lavage revealed alveolar hemorrhage with eosinophilia and transbronchial lung biopsy showed eosinophilic vasculitis. Cardiac enzymes were increased and murmurs were audible revealing cardiomyopathy proven by echocardiography. Pulse cyclophosphamide and methyl prednisolone was immediately started. On the 21st day, intestinal perforation developed and urgent surgery was performed. During a follow-up, although a radiological improvement was observed in the chest X-ray, cardiac failure, peripheral neuropathy and skin lesions developed and high-dose intravenous immunoglobulin and anti-TNF therapy (adalimumab) were applied. Despite the therapy, he died from heart failure and septicemia at 68th day of therapy.     

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. 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. Subcutaneous leiomyosarcomas have more     

Endoscopic retrograde cholangiopancreatography in hepatic alveolar echinococcosis

Background and Aim:  Hepatic alveolar echinococcosis (HAE) involves both the vascular and biliary structures of the liver. Endoscopic retrograde cholangiopancreatography (ERCP) is said to be an alternative for the diagnosis and treatment of biliary complications of HAE. We present here our experience with ERCP in HAE.
Methods:  We followed 13 patients who underwent ERCP for the treatment of biliary complications of HAE in the endoscopy unit of our clinic at Ataturk University School of Medicine, Erzurum between January 2002 and June 2008.
Results:  Eight men and five women were followed up. Mean age was 43.2 (24–64 years). All patients had non-resectable HAE. Indications for ERCP were biliary fistula in seven patients, obstructive jaundice in five patients and cholangitis in one patient. Endoscopic sphincterotomy (ES) was carried out in 12 patients, and in one patient with biliary leakage, a stent was inserted into the right hepatic branch. ERCP findings were dilated common bile duct, irregular narrowing and distortion of the common bile duct and common hepatic duct, communication with the cystic cavity or biliocutaneous fistula and complete disappearance of the biliary tree above the level of the common hepatic duct or hepatic bifurcation. In patients with biliary leakage, biliary drainage decreased only in two patients after ERCP and in patients with obstructive jaundice, the high bilirubin levels decreased in only one patient.
Conclusion:  ERCP showed structural changes of the external biliary tract and ES has a limited effect on these changes and stents can be used in selected cases.     

Primary hypertrophic pyloric stenosis in the adult: a case report.

A 66-year-old male was admitted with a two-month history of vomiting and weight loss. Endoscopy showed a pyloric obstruction and the patient underwent subtotal gastrectomy with gastro- jejunostomy. The histopathological study of the specimen revealed primary hypertrophic pyloric stenosis without any evidence of duodenal peptic disease. In the adult, this is a rare cause of gastric outlet obstruction of unknown etiology. It is usually recognized by histopathological examination of the specimen after a gastric resection performed to treat gastric outlet obstruction syndrome. However, some endoscopic and radiological signs, such as the cervix sign, or elongation of the pyloric channel, may give clues about the presence of the disease preoperatively. In symptomatic cases, surgery is the preferred treatment modality.     

Enhanced sensitivity to oxidant-induced micronucleus frequency in elderly individuals is not associated with glutathione S-transferase M1 (GSTM1) null genotype in lymphocytes

BACKGROUND: A large number of studies have demonstrated that various kinds of DNA damage accumulate during aging and that oxidative stress possibly contributes to this process. Glutathione S-transferase M1 (GSTM1) can prevent their possible effects on DNA via detoxification of reactive substances that induced oxidative stress. OBJECTIVE: To investigate the relationship between GSTM1 polymorphism and DNA sensitivity to oxidative stress with age, we used micronucleus (MN) frequency as a marker of DNA damage in lymphocytes from young and elderly subjects. Methods: This study was performed in 30 young (age range 20-36 years) and 30 elderly (age range 66-87 years) healthy individuals who were chosen on the basis of their GSTM1 genotype (15 GSTM1 null and 15 GSTM1 positive for each group). Lymphocytes were cultured after Ficoll isolation and treated for 48 h with a 30-muM dose of cumene hydroperoxide (CumOOH), a dose that does not decrease cell viability. RESULTS: There was no significant difference in the MN frequency observed in control cultures from young and elderly individuals. However, the MN frequency in CumOOH-treated cultures was significantly higher in the elderly group than the young group (p < 0.001). No association was found between the GSTM1 phenotype and CumOOH-induced MN frequency. CONCLUSIONS: The results suggest that lymphocytes of elderly individuals are more susceptible to in vitro MN induction by CumOOH. However, this difference in susceptibility is not explained by the lack of GSTM1.