iosr-JDMS   Volume-7 ~ Issue-2

Abstract: We present a case of a patient with myelodysplastic syndrome where in impreciation persists for case analysis . The cytogenetic study revealed Philadelphia negative with multiple breakpointS and isolated monosomy of chromosome 5 revealing the likely consideration for myelodysplastic myeloproliferative syndrome terminating to acute myelogenous leukaemia (unclassified RAEB category).There is absolute paucity of presentation in the world literature of cases of myelodysplastic syndrome having atrial fibrillation as the initial presentation.

[1]. Roberts WC , Bodey GP , Wertlake PT. The heart in acute leukaemia. A study of 420 autopsy cases. Am J Cardiol. 1968;21(3):388-412.
[2]. Jost E, Lorenzen J, Haage P, Bos G, Beelen D, Galm O et al. Heart and muscle involvement by extra-medullary myeloid leukaemia : a case report and review of the literature. Leuk Lymphoma. 2005;46(12):1819-24.
[3]. Hatake K, Saito K, Saga T, Akashi N, Doishita K. A case of acute myelogenous leukaemia with advanced atrioventricular block and pericardial effusion caused by leukaemic cell infiltration. Jpn J Med 1982;21(2):115-19
[4]. Ottaviani G, Matturri L, Rossi L, Jones D. Sudden death due to lymphomatous infiltration of the cardiac conduction system. Cardiovasc Pathol 2003;12(2):77-81.
[5]. Wikipedia.Myelodysplastic Syndrome
[6]. Greenberg P, Cox C, LeBeau MM, Fenaux P, Morel P, Sanz G et al. International scoring system for evaluating prognosis in myelodysplastic syndromes. Blood. 1997;89(6):2079-88.
[7]. Oliva EN, Dimitrov BD, Benedetto F, D Angelo A, Nobile F. Haemoglobin level threshold for cardiac remodeling and quality of life in myelodysplastic syndrome. Leuk Res. 2005;29(10):1217-19.
[8]. Greenberg PL. Myelodysplastic syndromes:iron overload consequences and current chelating therapies. J Natl Compr Canc Netw. 2006; 4(1):91-96.
[9]. Athens JWJ. Complications of haematopoietic neoplasms. Wintrobes Clinical Haematology. Vol 2.Edited by Lee GR. Philadelphia , Lea and Febiger ; 1993 : 1792 – 1842 .
[10]. Bincoletto C, Saad ST, Soares da Silva E, Queiroz ML. Autonomous proliferation and bcl-2 expression involving haematopoeitic cells in patients with myelodysplastic syndrome. Br J Cancer. 1998;78(5):621-24.

Abstract: Lupus manifesting as an ascites is extremely rare as aptly assessed by the figures available in world literature. We report a case which clinically and cytologically mimicked a malignant process. Further investigations and conservative approach taken in the case led to definitive diagnosis of florid peritoneal reaction associated with systemic lupus erythematosus. The clinical presentation is discussed in the context of other similar rare reports.

Key words : Fibrosis, sclerosing peritonitis, florid ascites, systemic lupus erythematosus.

1]. Kaklamonis P, Vayopoulos G, Stamatelos G, Dadinas G,Tsokos GC. Chronic lupus peritonitis with ascites. Ann Rheum Dis. 1991; 50 : 176-177.
[2]. Pepels M.J.A.E. Peters F.P.J. Mebis J.J.I.R., Ceelen Th L. Hoofwijk A.G.M., Erdkamp F.C.G. Sclerosing peritonitis : an unusual cause of ascites in patients with systemic lupus erythematosus. Netherlands Journal of Medicine. 2006; 64(9): 346-349.
[3]. Finney AL, Spagndo DV, Crawford GP, Shilkin KB. Pseudosarcomatous sclerosing peritonitis. Case report of an unusual form of chronic lupus peritonitis. International Journal of Surgical Pathology. 1996; 4(2) : 121-128.
[4]. Wilkins K, Holffman G. Massive ascites in systemic lupus erythematosus. J Rheum. 1985; 12 : 571-574.
[5]. Mier A, Weir M. Ascites in systemic lupus erythematosus. Ann Rheum Dis. 1985; 44 : 778-779.
[6]. Miller MH, Urowitz MB, Gladmann DD, Tozman ECS. Chronic adhesive lupus serositis as a complication of systemic lupus erythematosus. Arch Intern Med. 1984; 144 : 1863-64.
[7]. Schousboe J, Koch A, Chang R. Chronic lupus peritonitis with ascites : review of literature with a case report. Semin Arthritis Rheum. 1988; 18 : 121-126.
[8]. Garosi G, Di Paolo N. Morphological aspects of peritoneal sclerosis. J Nephro. 2001; 14 (4) : 530-8.
[9]. Kelly JK, Hwary WS. Idiopathic refractile sclerosing mesenteritis and its differential diagnosis. AM J Surg Pathol. 1989; 13 : 513-521.
[10]. Dehn TCB, Lucas MG Wood RFM. Idiopathic sclerosing peritonitis. Postgrad Med J. 1985; 61 : 841-841.

Abstract: We report a case with infrequent cause of myopathy related to hypothyroidism who presented to us with pseudohypertrophic mass on left calf with serological findings consistent with diagnosis of hypothyroidism. The syndrome of pseudohypertrophic etiology (Hoffman's syndrome) remains obscure. The case is discussed and reviewed briefly.

Keywords: Pseudohypertrophy, myopathy, hypothyroidism.

1. Vasconcellos LF Peixoto MC, de Oliveira TN, Penque G, Leite AC. Hoffman's Syndrome : pseudohypertrophic myopathy as initial manifestation of hypothyroidism. Case report. Arq Neuropsiquiatr. 2003 Sep; 61(3B) : 851-4. Epub; 2003 Oct.28 [PubMed].

2. Deepak S, Harikrishnan, Jayakumar B. Hypothyroidism presenting as Hoffman's syndrome. J Indian Med Assoc 2004; 102 : 41-2.

3. Mastropasqua M, Spagna G, Baldini V, Tedesco I, Paggi A. Hoffman's Syndrome : Muscle Stiffness, Pseudohypertrophy and Hypothyroidism. Case Report . Horm Res 2003; 59 : 105-108.

4. Ono S, Inouye K, Mannen T. Myopathology of hypothyroid myopathy : some new observations. J Neurol Sci 1987; 77 : 237-248.
5. McKeran RO, Slavin G, Ward P, Paul E, Mair WGP. Hypothyroid myopathy : a clinical and pathological study. J Pathol 1980; 132

Abstract: Knowledge of embryology ,teratology , clinical genetics and diagnostic ultrasonography have paved way to identity the anomalies and treat them successfully in pre/postnatal phase .Tremendous advancement in the imaging technique and availability of sophisticated instruments have made prenatal fetal surgery to repair many of the anomalies possible – an unimaginable dream come true. In this era where family planning has been accepted by all. It is imperative to take measures to identify anomalies during prenatal phase, so that in cases of major anomalies, MTP can be advised to avoid economic burden and domestic difficulties to the parents. The present study was done on 50 still born and aborted fetus around age group of 26 weeks to 40 weeks obtained from Gandhi Hospital Secunderabad. Observations found are Gastrointestinal tract anomalies are 5, Central nervous system anomalies were 6, Urogenital system anomalies are 1 in number, normal foetus are 40 cases. The primary importance to examine the malformed foetus is to derive more information which can be used to counsel parents in respect of future pregnancies.

Keywords: Anencephaly, Spinabifida, Imperforate anus, Gastroschisis, Congenital anomalies, Foetus.

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