Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

During the last 3 years 20 patients (median age 8 years) have undergone construction of a continent urinary reservoir at our pediatric urology service. Of the patients 12 had failed exstrophy reconstruction, 7 had myelodysplasia with a neurogenic bladder and 1 had extensive bladder damage as a result of a previous operation. In 5 patients a continent reservoir was constructed after cystectomy performed in early childhood. Techniques of reconstruction included the Mitrofanoff procedure (4 patients), a modification of the Indiana pouch (12), a modification of the Benchekroun procedure (2), the use of the appendix in situ as a continence mechanism (2) and a modified Kropp procedure using a duplicate vagina for catheterization (1). The morbidity from these procedures was acceptable and consisted primarily of chronic diarrhea (4 patients), stone formation within the pouch (5) and the need to revise the continence mechanism (7). All but 1 patient maintained stable or improved upper tracts after diversion. Over-all, 85% of the patients experienced diurnal continence on an intermittent catheterization regimen, while 75% were dry at night. The plicated/intussuscepted ileal nipples required revision because of difficulty with catheterization (7 patients) and urinary leakage. We currently favor the use of the Benchekroun or Mitrofanoff techniques for the ease of construction and minimal requirements for revision.
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PMID:Continent urinary reservoirs in pediatric urological practice. 237

A case report of vaginal calculus formation in a nine-year-old girl with myelodysplasia is presented. Etiologic factors in the formation of such calculi include fistulous communication between the vagina and the bladder, neuropathic urethrovesical dysfunction, anatomic conditions causing vaginal outlet obstruction, and/or vaginal pooling of urine and urease-producing bacterial infection. A correct preoperative diagnosis can be made by using oblique x-ray films and cystoscopy. Surgical treatment is simple and successful.
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PMID:Vaginal calculus in female with myelodysplasia. 635 34

Most patients with advanced or recurrent uterine sarcoma experience disease progression and ultimately die. We present a case of uterine sarcoma with lung metastasis treated with systemic chemotherapy and with no evidence of disease for more than 5 years. A 77-year-old woman underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy for carcinosarcoma of the uterus followed by external pelvic radiotherapy. Ten months later, the tumor recurred in the apex of the vagina and was treated with brachytherapy. After 6 months of remission, she presented with pulmonary metastasis. After four cycles of systemic chemotherapy with cisplatin and ifosfamide, the pulmonary nodules completely disappeared. Currently she is still in complete remission after more than 5 years, but unfortunately she has developed myelodysplastic syndrome. This is the first reported case in the literature of cured metastatic uterine carcinosarcoma to lungs, with long-term survival of 5 years.
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PMID:Cure of metastatic uterine carcinosarcoma to lungs: a case report. 1263 Dec 27

Granulocytic sarcomas are extramedullary tumors (EMD) of malignant myeloid precursor cells. EMD or granulocytic sarcoma of ovary is rare disease. A 15-year-old girl had complaints of abdominal pain and weight loss for 3 months. On physical examination, there were hepatosplenomegaly and a painless mass under the umbilicus. Breast development was grade II. There was no clitoris hypertrophy. Her labia majora were separate and vagina hypoplastic. Hemoglobin level was 9.3 g/dl, white blood cells count 2.8 x 10(6)/1, platelet count 31.6 x 10(9)/1. There were dysplastic features in the blood and bone marrow cells. There were 10 and 22% blasts in the peripheral blood smear and bone marrow, respectively. The levels of serum follicle stimulating and luteinizing hormones were high. An inguinal mass (diameter 9.5 x 7.6) cm was detected on computed tomography. The histopathological diagnosis of this was obtained from laporascopy was composed of ovotestis and there was marked blastic infiltration in this ovotestis which had myeloid markers on flow cytometry. In the immunohistochemical analyses of ovotestis and bone marrow, blasts were positive for LCA, CD-13, CD-33 and CD 68. The cytogenetic analysis of the bone marrow shaved 46 XY karyotype. No response was achieved with combination chemotherapy and the patient died from progressive leukemia. Here we report a rare patient with myelodysplastic syndrome, EMD and hermaphroditism. To our knowledge this is the first case of MDS, EMD and hermaphroditism.
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PMID:Granulocytic sarcoma of the ovotestis: an association of myelodysplastic syndrome and hermaphroditism. 1536 14

It is occasionally difficult to distinguish between Behcet's disease (BD) and pyoderma gangrenous (PG). Our case showed ulcers of the oral, vaginal and perineal areas, and in the ileum, thus resulting in our initial diagnosis of BD. However, the patient showed a continued leukopenia, and she was subsequently diagnosed by bone marrow biopsy as having a myelodysplastic syndrome, which will sometimes accompany PG. In addition, following a hysterectomy, the ulcers of the stump in the vagina and the perineum showed the characteristic findings of a PG-like destructive ulceration. Based on these findings, we finally diagnosed our case to have PG.
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PMID:Case of pyoderma gangrenosum showing oral and genital ulcers, misdiagnosed as Behcet's disease at first medical examination. 1847 29