A rare cause of refractory ascites in a child: familial Mediterranean fever

Familial Mediterranean fever (FMF) is hereditary episodic febrile syndrome characterized by acute attacks of fever and serosal inflammation, generally lasting 1–3 days and resolves spontaneously. Apart from abdominal pain, patients may present with variety of abdominal manifestations such as acute peritonitis, mechanical intestinal obstruction, diarrhea, bowel infarction, amyloidosis and small amounts of peritoneal fluid during the acute attacks. A 6-year-old boy was admitted with massive ascites. After extensive laboratory investigations, no causative agent could be identified. On subsequent days, he developed fever and skin eruptions. Acute-phase reactants were increased. A second tomography revealed cystic fluid collection near the anterior side of spleen that invades the stomach. An exploratory was performed and histopathological examination of the all resected specimens revealed mix inflammatory cell infiltrate associated with severe myofibroblast proliferation suggesting chronic inflammatory process on the mesenteric region. A diagnosis of FMF was suspected based on the clinical, laboratory and histopathological findings, and a trial of colchicine therapy initiated. Ascites and other serosal inflammations improved within 1 week without any recurrence during the next 12-month period on colchicine treatment. Atypical presentations of FMF have been increasingly reported. Pediatricians should keep FMF in mind in the differential diagnosis of massive ascites especially in regions where hereditary inflammatory disease are common.     

Chlorine-induced extensive tracheobronchial necrosis concomitantly benzene-induced pancytopenia presented with severe pneumonia

We report a case of 25-year-old woman with severe tracheobronchial necrosis caused by chlorine released from a mixture household cleaning agents. She subsequently exposed benzene while she was fixing the seats with benzene containing gum. The case was found interesting with its history, delayed diagnosis, bronchoscopic features, and fatal outcome. We presented its bronchoscopic and pathological images which has not been shown in the literature up to date.     

Allogeneic Haematopoietic Stem Cell Transplantation as Therapy for Chronic Granulomatous Disease��Single Centre Experience

Chronic granulomatous disease (CGD) is phagocytic cell metabolic disorder resulting in recurrent infections and granuloma formation. This paper reports the favourable outcome of allogeneic transplantation in six high-risk CGD patients. The following donors were used: HLA-matched, related (two) and unrelated (three), and HLA-mismatched, unrelated (one). One patient was transplanted twice using the same sibling donor because of graft rejection at 6 months after reduced-intensity conditioning transplant (fludarabine and melphalan). Myeloablative conditioning regimen consisted of busulphan and cyclophosphamide. Stem cell source was unmanipulated bone marrow containing: 5.2 (2.6-6.5) × 10(8) nucleated cells, 3.8 (2.0-8.0) × 10(6) CD34+ cells and 45 (27-64) × 10(6) CD3+ cells per kilogramme. Graft-versus-host disease prophylaxis consisted of cyclosporine A and, for unrelated donors, short course of methotrexate and anti-T-lymphocyte globulin. Mean neutrophile and platelet engraftments were observed at day 22 (20-23) and day 20 (16-29), respectively. Pre-existing infections and inflammatory granulomas resolved. With the follow-up of 4-35 months (mean, 20 months), all patients are alive and well with full donor chimerism and normalized superoxide production.     

Organ Donation: A Portrait of Family Refusal in Québec

Many studies from around the world have reported different reasons why families refuse organ donation. In Quebec, however, there are no known data on the subject. To enable us to better communicate with families, a research project was conducted from January to December 2007 in hospital centers with personnel who specialize in supporting families. Our objective was to identify the reasons why Quebecers refuse a request for organ donation. The findings demonstrate that knowing the express wishes of the deceased person about organ donation and end-of-life treatment influences the family. When these wishes are not known, the partners of older donors refuse in greater numbers, primarily for familial or circumstantial reasons. Refusal based on religion or ethnicity is rare. Some families approached before neurologic death is diagnosed do not wish to wait until all the criteria for neurologic death are met.     

Upregulation of multi drug resistance genes in doxorubicin resistant human acute myelogeneous leukemia cells and reversal of the resistance

The major problem in the treatment of acute myeloid leukemia (AML) patients results from multidrug resistance to administered anticancer agents. Drug resistance proteins, MDR1 and MRP1, which work as drug efflux pumps, can mediate the multidrug resistance of human leukemia cells. In this study, the mechanisms of resistance to doxorubicin-induced cell death in human HL60 AML cells were examined. Continuous exposure of cells to step-wise increasing concentrations of doxorubicin resulted in the selection of HL60/DOX cells, which expressed about 10.7-fold resistance as compared to parental sensitive cells. The expression analyses of MRP1 and MDR1 drug efflux proteins in doxorubicin-sensitive and -resistant HL60 cells revealed that there was an upregulation of MRP1 gene in HL60/DOX cells as compared to parental sensitive cells. On the other hand, while there was no expression of MDR1 gene in parental cells, the expression of MDR1 gene was upregulated in HL60/DOX cells. HL60/DOX cells also showed cross-resistance to cytosine arabinoside (Ara-c). This resistance was reversed by a combination therapy of Ara-c and cyclosporine A. However, the expression levels of CD15 and CD16 surface markers were significantly decreased in HL60/DOX cells.     

Follow-up hemoglobin concentrations in ICU: relationship between diagnostic blood loss and daily fluid balance

To evaluate DeltaHb (daily changes of hemoglobin concentrations) in nonbleeding critically ill patients, and to investigate its relation with diagnostic blood loss (DBL) and fluid balance (FB). Hospital records of 34 nonseptic patients who stayed in respiratory intensive care unit (RICU) at least 72 hours with no evidence of acute bleeding, renal failure and bleeding diathesis, were evaluated retrospectively. Demographics, clinical features, acute physiology assessment and chronic health evaluation (APACHE) II scores, daily Hb levels, DBL and FB were recorded. Correlation statistics was performed between DeltaHb and DBL and FB. We compared the patients with DeltaHb > or =0.5 g/dL/day (group A) and the patients with DeltaHb < 0.5 g/dL/day (group B) in the first three days. The mean age was 55 +/- 14, Hb level was 13.2 +/- 1.7 g/dL at admission and 12.6 +/- 2.3 g/dL at discharge from RICU. DBL was 25.2 +/- 7.4 mL/d, and FB was 251 +/- 1783 mL/d for the first day in intensive care unit. DBL was lesser in subsequent days than in the first day but it wasn't significant. DeltaHb was -0.54 +/- 1.5 g/dL for the first three days, while it was -0.23 +/- 1.5 g/dL for subsequent four days (p= 0.9). DeltaHb in the first three days has no correlation with DBL and FB. Age, sex, APACHE II score, clinical features, DBL and FB were not differed between Group A and Group B. CONCLUSION: No relation was found between DeltaHb and DBL, and also FB; but studies like this are important to indicate that Hb concentrations may decrease in critically ill patients without any reason such as bleeding.     

Two sisters with breast tuberculosis

Breast tuberculosis is a rare form of extrapulmonary tuberculosis. Two sisters diagnosed with mammary tuberculosis and having a family history concerning pulmonary tuberculosis in other siblings were presented in this study; and thereby breast tuberculosis was discussed by evaluating similar cases of breast tuberculosis published in our country. The ultrasonography examinations of the cases (26/F, 27/F) with history of mass, enlargement, pain and discharge complaints were reported as being consistent with fibrotic mastopathy and inflammation. Patients having the diagnosis of breast tuberculosis after the excisional biopsy were undergone HRZE treatment. After the antituberculosis regimen, a complete resolution of the lesion in the breast was observed in our first case which had concurrent pulmonary tuberculosis. The lesion of our second subject who had isolated mammary tuberculosis showed marked improvement. Our cases are still being followed up without any complaints. In conclusion, breast tuberculosis should become a part of differential diagnosis of breast diseases in populations with high incidence of tuberculosis as our country.     

Induction Therapy in Cardiac Transplantation: When and Why?

Induction therapy has continued to be a subject of controversy in heart transplantation for more than 20 years. It is an example of a therapy that is logical, and ought to be better than "doing without." However, a careful review of the evidence suggests otherwise. Except for patients where the benefits clearly outweigh the short and long-term risks, the use of induction therapy should be avoided. In immunosuppression, as in life, there is no "free lunch." Clinicians need to be certain they fully understand what they are ordering when asking for induction therapy to be administered to their patients.