International Journal of Impotence Research (2006) 18, 77–81& 2006 Nature Publishing Group All rights reservedORIGINAL ARTICLEEarly use of vacuum constriction device following radicalprostatectomy facilitates early sexual activity and potentiallyearlier return of erectile functionR Raina1,2, A Agarwal1, S Ausmundson1, M Lakin1, KC Nandipati1, DK Montague1, D Mansour2 and CD Zippe11Cente
Tfjd640-03-180265.texPediatric Hematology and Oncology, 23:1–7, 2006Copyright C Taylor & Francis Group, LLCISSN: 0888-0018 print / 1521-0669 onlineDOI: 10.1080/08880010600803214 CONCURRENT DEVELOPMENT OF CROHN DISEASE AND
MYELODYSPLASTIC SYNDROME IN A CHILD: Case Report
and Literature Review
Sergio Carlos Nahas, Caio Sergio Rizkallah Nahas,
and Carlos Frederico Marques
✷ Colon and Rectum Surgery Department, Hospital das Cl´ınicas, Medical School, University of S˜ao Paulo, S˜ao Paulo, Brazil Marcello Rodriguez Borba
✷ Surgery Department of University Hospital, University of S˜ao Paulo, S˜ao Paulo, Brazil Alfredo Salim Helito
✷ Hospital S´ırio-L´ıbanˆes of S˜ao Paulo, S˜ao Paulo, Brazil Vicente Odoni
✷ Pediatric Department of Hospital das Cl´ınicas, Medical School, University of S˜ao Paulo, S˜ao Paulo, Brazil ✷ A small number of cases of Crohn disease associated with myelodysplastic syndromes or leukemia have been reported in adults in the last 25 years in the English-language medical literature. The authors report a case of a 9-year-old boy who developed Crohn disease and myelodysplastic syndrome concurrently. Analysis of his bone marrow showed a chromosome 20 abnormality. Although chro- mosome 20 abnormalities have been reported in a minority of these patients, the significance of this association remains unclear at the present time. Keywords
Crohn diseas, leukemia, myelodysplastic syndromes, pancytopenia An association between Crohn disease (CD) and myelodysplastic syn- dromes (MDS) or leukemia has been suggested on the basis of concomitant findings of these disorders in a total of 24 cases reported in the English- language medical literature [1–11]. Interestingly, all patients reported were adults (mean age of 68 years old, range 28–83). Eng et al. , the first to report such a condition in 4 patients, found clonal abnormalities of chromo- some 20 in 3 cases. Although the association of chromosome 20 abnormalities and MDS has been described , whether such abnormalities account for the association between MDS and CD is unknown. Moreover, since Eng’s Received 30 January 2006; accepted 12 May 2006.
Address correspondence to Dr. Sergio Carlos Nahas, Rua Adma Jafet 50, conj 131/132, Bela Vista, 01308 050 S˜ao Paulo, SP, Brazil. E-mail: email@example.com publication, all subsequent reports have failed to demonstrate chromosome 20 abnormalities in patients with CD and MDS [2–11].
Herein, we report a case of concurrent development of CD and MDS in a child with a chromosome 20 abnormality.
A 9-year-old boy presented with a 1-week history of high fever (39–40◦C) associated with a 1-month history of anorexia, nausea, and intermittent dif- fuse colicky abdominal pain. He had experienced episodes of diarrhea and a 10% weight loss over the last month. On physical examination a right lower quadrant tender mass was palpable. Laboratory data revealed normo- cytic anemia (hemoglobin level, 85 g/L; mean corpuscular volume, 89 u3), leukopenia (leukocyte count, 2.6 × 109/L), and thrombocytopenia (platelet 39count, 110 × 109/L). Reticulocyte count was 1.6%. Erythrocyte sedimenta- 40tion rate was 90 mm/h. He had negative serology for infectious diseases, negative stool cultures, and normal rheumatologic markers.
A colonoscopy was performed because of a clinical suspicion for inflam- matory bowel disease and revealed segmental aphthous ulcerations and lon- gitudinal fissures throughout the cecum and terminal ileum with typical histopathologic features of CD. Prednisolone (1 mg/kg/day) and 5-ASA (1 g/day) were started. With a worsening clinical examination and radio- graphic evidence of pneumatosis, he was taken to the operating room for a right colectomy. Histological examination of the specimen confirmed the diagnosis of CD. Postoperatively, the patient continued to be febrile and was pancytopenic with normal folate and vitamin B12 serum levels. He required 51blood transfusions for his anemia. Fanconi’s anemia was ruled out by a neg- ative diepoxybutane (DEB) test. Hemoglobin electrophoresis showed an in- creased fetal hemoglobin (5.6%). Bone marrow aspiration at 2 weeks after initiation of steroids and 5-ASA revealed trilineage dysplasia with a moderate hypocellularity, and 2% blasts. A peripheral blood smear showed no blasts at that time. Cytogenetic analysis of the bone marrow revealed chromosome 20 abnormalities (monosomy), chromosome 1 derivative duplication, and chromosome 8 trisomy in 8 of a total of 9 cells analyzed. These abnormalities were confirmed by fluorescence in situ hybridization (FISH). The patient had significant improvement of trilineage cell counts after administration of granulocyte-colony stimulating factor (G-CSF) (5 µg/kg) daily for 2 weeks 62and was discharged in satisfactory condition.
Over the next 2 years, G-CSF treatments were given at 6, 12, and 18 months for recurrent pancytopenia. Bone marrow biopsy prior to starting each additional G-CSF treatment showed dyshematopoietic abnormalities similar to the first bone marrow examination but with 3, 3, and 17% blasts, respectively. In addition to increased bone marrow blasts, the peripheral Crohn Disease and Myelodysplastic Syndrome blood smear showed 7% blasts, suggestive of a diagnosis of refractory ane- mia with excess blasts in transformation (RAEB-2). At 22 months the pa- tient progressed to an acute myeloid leukemia, requiring treatment with chlorodeoxyadenosine and citarabine (one cycle). A bone marrow aspira- tion performed 2 weeks after completion of treatment showed complete re- mission. As of this manuscript (at 24 months follow-up), the patient’s disease DISCUSSION
MDS is a bone marrow stem-cell disorder characterized primarily by pe- ripheral cytopenias and hypocellular dysplastic bone marrow, although oc- casionally it can be found to be associated with hypercellular or normocel- lular bone marrow. Based on the French–American–British (FAB) classifica- tion , MDS includes refractory anemia (type I), refractory anemia with ringed sideroblasts (type II), chronic myelomonocytic leukemia (type III), refractory anemia with excess blasts (type IV), and refractory anemia with excess blasts in transformation (type V). More recently, the classification of the myeloid neoplasms had been refined by the World Health Organization (WHO), utilizing not only morphologic findings but also all available information, including genetic, immunophenotypic, biologic, and clinical features, to define specific disease entities. Because of the lack of specific data in the previous literature reports, we find it necessary to use the origi- nal FAB classification. We have included in Table 1, however, our suggested categorization of these case reports according to the WHO classification.
A total of 21 cases of CD associated with one of five different types of MDS described above have been reported in the indexed English-language medical literature [1–9]. In addition, 3 cases of CD in association with other leukemias apart from the FAB classification have been reported [10, 11].
A pathophysiologic link between CD and MDS has been suggested on the basis of a common immunologic impairment. It is unknown whether underlying immunologic alterations account for the development of these diseases or whether the additional immunosuppressive status caused by one disease predisposes to the development of the other. In 1992, Eng et al.  were the first to find chromosome 20 abnormalities in the bone marrow cells of 3 of the 4 patients with coexistent MDS and CD. Unlike the frequent occurrence of clonal chromosomal abnormalities in the bone marrow cells of patients with MDS, karyotypic abnormalities in intestinal cells have not been seen in patients with CD. The chromosome 20 abnormality is described in 5% of primary MDS in the elderly population . However, since Eng’s publication, all other reports of patients with coexisting CD and MSD or CD and leukemia have failed to report that abnormality (except ours) (see MDS usually develop in patients over 60 years of age. CD is typically 110 diagnosed in younger persons, with a suggested second peak after 60 years. 111In this case, curiously, both conditions were present in a 9-year-old boy, while 112all other reported cases of coexistence of both conditions involved older 113patients (mean age 68 years; range 28–83). Gumruk et al.  described one 114case of pyoderma gangrenosum in a 11-month-old girl with MDS who had 115a normal cytogenetic study of her bone marrow and did not have intestinal 116abnormalities.
MDS/leukemia and CD, these disorders were simultaneously diagnosed 119in 10 other patients in addition to ours (cases 5, 6, 10–14, 16, 17, 21, 25) 120[2, 3, 6, 7, 9]. CD clearly antedated (at least one year) MDS/leukemia 121in 10 patients (cases 7–9, 15, 18–20, 22–24) [3–5, 7, 8, 11, 17], whereas 122MDS antedated those of CD in the remaining 4 patients (1–4) . All 24 123previous cases reported had CD involvement of either colon and/or ileum. 124As for the treatment of CD, most patients received steroids and/or 5-ASA. 125Although cases of acute leukemia have been described in patients receiving 126immunosuppressives with or without corticosteroids, acute leukemia as a 127direct effect of either 5-ASA or prednisone has not been documented.
In the majority of cases, CD was clinically managed. Four patients re- 129 quired an operation because of failure of medical treatment (cases 19–23). 130Specific therapy for MDS/leukemia varied among the 20 patients for whom 131that kind of information was reported. Eleven patients received no specific 132therapy. However, 4 patients with MDS (2 with FAB type IV, and 2 with FAB 133type V), and 3 with non-FAB leukemia required antineoplastic medications. 134Two other patients received synthetic testosterone (Danazol) (Table 1).
Due to the fact that most of these disorders affect mainly the elderly, 136 aggressive treatment is difficult in most cases, leaving only expectant ther- 137apy with supportive measures. In the present report, despite progression to 138myeloid leukemia, the young age of onset may have contributed to a good 139initial response to the chemotherapeutic agents. However, the relative short 140follow-up period after treatment of myeloid leukemia does not allow us to 141make further statements regarding outcome.
In conclusion, although the coexistence of MDS and CD has been ex- 143 clusively reported in adults and elderly, the diagnosis of MDS should also be 144considered in children with CD and persistent peripheral cytopenia. The sig- 145nificance of an association between chromosome 20 abnormalities with the 146development of concomitant conditions (MDS and CD) remains unclear.
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Lesvoorbereiding De Buren Beginsituatie : De kinderen weten wat “buren” zijn. De meeste kinderen zagen hun ouders vriendelijk met de buren omgaan. Ze beseffen echter niet welke waarde de Islam hecht aan een goede relatie met de buren. Doelstellingen : De leerlingen kunnen verwoorden dat een moslim respect moet hebben voor zijn buren. De leerlingen gaan spontaan hun buren goed behandelen