Cases reported "Autoimmune Diseases"

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1/67. Acquired haemophilia - a study of ten cases.

    Clinico-haematological features in 10 patients with acquired Haemophilia are presented. Three patients had FVIII inhibitors following pregnancy while in six the cause for development of inhibitors could not be determined. One patient had acquired von Willebrand's disease. Lupus anticoagulant coexisted with factor viii inhibitors in three patients. All patients presented with sudden onset of bleeding without any past or family history of a bleeding disorder. factor viii inhibitor levels ranged from 8 to 512 Bethesda units in the nine patients. Immunosuppressive therapy was given to 8 patients, consisting of CVP regimen or corticosteroids with endoxan or cyclosporin. Seven patients had clinical and laboratory responses and one patient did not respond. One patient had severe postpartum bleeding with acute shock which was controlled with FEIBA. diagnosis and management of idiopathic acquired Haemophilia, thus, continues to be a major challenge, and among acquired Haemophilia, postpartum Haemophilia has good prognosis.
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ranking = 1
keywords = pregnancy
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2/67. ovulation induction and early pregnancy loss in a woman susceptible to autoimmune diseases: a possible interrelationship.

    We report a 34-year-old woman with sequentially occurring autoimmune diseases that are possibly triggered by numerous ovulation inductions. At the ages of 26-32 years, she experienced 27 uncontrolled ovulation induction cycles using clomiphene citrate (CC) or CC plus human menopausal gonadotropin plus human chorionic gonadotropin. She became pregnant at the ages of 27, 30 and 31 with subsequent pregnancy loss in the 28th, 8th and 10th week of gestation, respectively. insulin-dependent diabetes mellitus (IDDM) developed at the age of 28. During the second year of ovulation induction, at the age of 27, she developed arthralgia that worsened and became migratory from the age of 31. thrombocytopenia appeared at the age of 33. The diagnosis of systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) was established at the age of 34. To the best of our knowledge, this is the first case of concurrent IDDM, SLE and APS in a patient associated with ovulation inductions. Excessive levels of estradiol achieved during the ovulation inductions could play a role in the expression of multiple autoimmune diseases in the susceptible woman.
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ranking = 5.1607760458723
keywords = pregnancy, gestation
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3/67. Autoimmune neutropenia in pregnant women causing neonatal neutropenia.

    Autoimmune neutropenia (AIN) can occur during pregnancy. However, neonatal neutropenia occurring in an infant born to a mother with AIN has only rarely been documented. Recently, we have experienced two cases of AIN during pregnancy, both of which caused severe yet transient neonatal neutropenia (< 0.3 x 10(9)/l), probably as a result of transplacental maternal anti-neutrophil autoantibodies. The anti-neutrophil antibodies seemed to be against antigens other than NA1/NA2 because the autoantibodies did not bind to neutrophils of specific NA types selectively in the granulocyte indirect immunofluorescence test. Although AIN is a relatively uncommon disease, neonatal neutropenia caused by maternal AIN may not be quite as rare.
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ranking = 2
keywords = pregnancy
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4/67. Changing thyroid status related to pregnancy.

    A case of post-thyroidectomy hypothyroidism is reported. The patient became euthyroid in three consecutive pregnancies, reverting to hypothyroid within three months of delivery on each occasion. The alteration in thyroid status is attributed to pregnancy related changes in antibody titres, though the laboratory data to confirm this are not available.
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keywords = pregnancy
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5/67. In utero development of a warm-reactive autoantibody in a severely jaundiced neonate.

    BACKGROUND: The fetus and neonate are widely considered to be immunologically immature. However, there are rare case reports of RBC alloantibody and autoantibody development. STUDY DESIGN AND methods: This report describes the case of a severely jaundiced full-term boy neonate presenting at birth with an IgG warm-reactive autoantibody. RESULTS: Mother and neonate were both blood group A, D . The mother had a negative antibody screen at 18 weeks' gestation and a negative DAT and antibody screen at the time of delivery. The neonate was born with a strongly reactive DAT (IgG) and a panreactive eluate. The serum also contained a panreactive antibody, and all crossmatches were incompatible. The neonate had a bilirubin of 12.5 mg per dL at birth, which peaked at 22.5 mg per dL. However, there was no overt evidence of hemolysis, as evidenced by normal serial Hct levels and reticulocyte counts. The neonate responded well to phototherapy and did not require either simple or exchange transfusion. The neonate's warm-reactive autoantibody maintained its original strength of reactivity on follow-up testing performed at 2 weeks and 2 months of age. CONCLUSIONS: This report describes a rare case of apparent in utero RBC autoantibody development. The fetal/neonatal immune response to blood group antigens is reviewed.
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ranking = 0.16077604587225
keywords = gestation
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6/67. Autoimmune and pregnancy complications in the daughter of a kidney transplant patient.

    BACKGROUND: immunosuppressive agents taken by pregnant organ transplant recipients readily cross the placenta during development of the fetal immune system. There are few data on the long-term implications for the progeny, but evidence from animal studies suggest that second and third generations of organ transplant patients may be at risk for autoimmune disorders and reproductive problems. methods: We present the 23-year-old daughter of a renal allograft recipient exposed to azathioprine 75 mg/day and prednisone 5 mg/day throughout her mother's pregnancy. RESULTS: During the daughter's first pregnancy, she developed multiple autoantibodies, Raynaud's phenomenon, and fetal death occurred at 20 weeks gestation. The second pregnancy was complicated by systemic lupus erythematosus, preeclampsia, and the birth of a preterm male infant. CONCLUSIONS: It is uncertain whether the autoimmune manifestations and obstetric complications in this patient were related to fetal exposure to immunosuppressive drugs. Nevertheless, further studies on the health and pregnancies of adult offspring of transplant patients are warranted.
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ranking = 7.1607760458723
keywords = pregnancy, gestation
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7/67. First male case of lymphocytic hypophysitis in korea.

    Lymphocytic hypophysitis is a rare inflammatory disorder which is caused by autoimmune destruction of the pituitary gland. Almost all reported cases have been in women and the disease is often associated with pregnancy. We describe here the first male case of lymphocytic hypophysitis in korea. The patient presented with headache, impotence, decreased libido, and deteriorated vision. Endocrinologic studies showed panhypopituitarism, and pituitary MRI imaging revealed a homogeneously enhanced pituitary mass with a thickened stalk. Treatment with prednisolone and thyroid hormone for five months was ineffective. Transsphenoidal resection of the pituitary mass was performed successfully with normalization of the visual field defect. Histologic examination revealed diffuse lymphocytic infiltration with dense collagenous fibrosis, consistent with lymphocytic hypophysitis. Lymphocytic hypophysitis should be considered in differential diagnosis even in men with hypopituitarism and an enlarged pituitary gland.
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ranking = 1
keywords = pregnancy
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8/67. Reversible adrenocorticotropin deficiency due to probable autoimmune hypophysitis in a woman with postpartum thyroiditis.

    The natural history and pathogenesis of lymphocytic hypophysitis remain poorly understood. We describe a 34-yr-old woman with postpartum thyroiditis and ACTH deficiency, studied at monthly intervals for 18 months after pregnancy. A significant titer of thyroid peroxidase autoantibodies was detected at 16 weeks gestation, and she was recruited into a prospective study of postpartum thyroid function. Four months postpartum she developed mild hyperthyroidism [free T4 (fT4), 27 pmol/L; TSH, less than 0.2 mU/L] and showed a rise in thyroid peroxidase and thyroglobulin autoantibodies. At 9 months postpartum, serum fT4 and fT3 levels were low normal (8.0 and 1.7 pmol/L, respectively), but TSH was not raised (0.4 mU/L). Subsequent investigation showed a low basal plasma cortisol level (28 nmol/L) in association with undetectable ACTH, and subnormal cortisol responses to depot Synacthen (535 nmol/L at 6 h) and hypoglycemia (peak, 145 nmol/L). FSH, LH, GH, and PRL function and computerized tomography of the pituitary were normal. Retrospective analysis of serum samples taken throughout the postpartum year showed developing hypocortisolemia between 3-9 months postpartum. Each sample was also tested for pituitary autoantibodies using a specific indirect immunofluorescent assay; none was detected. The ACTH deficiency recovered spontaneously, with normal cortisol responses to depot Synacthen (greater than 1380 at 6 h) and hypoglycemia (peak, 590) 14 and 18 months postpartum, respectively. This case illustrates that postpartum pituitary deficiencies are potentially reversible. The pattern of pituitary deficit and postpartum thyroiditis supported a diagnosis of autoimmune hypophysitis.
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ranking = 1.1607760458723
keywords = pregnancy, gestation
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9/67. A case report of neonatal thyrotoxicosis due to maternal autoimmune hyperthyroidism.

    A case of neonatal thyrotoxicosis secondary to maternal autoimmune hyperthyroidism is reported in an infant born at 34 weeks gestation who presented with tachycardia, jitteriness, diarrhea, and a small goiter. propranolol and oxygen were used to treat high-output cardiac failure and transient persistent pulmonary hypertension. The infant's response to propylthiouracil therapy, gradual resolution of cardiac and systemic symptoms, and normaliziation of thyroid studies are described. Thyroid physiology and function and the special considerations in a premature infant are reviewed. An overview of maternal autoimmune hyperthyroidism and the implications for the developing fetus and neonate are presented. The risk factors for, and clinical presentation of, hyperthyroidism are outlined and treatment strategies highlighted. The nursing care of infants with hyperthyroidism is carefully described with an emphasis on the surveillance for and management of multisystem manifestations.
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ranking = 0.16077604587225
keywords = gestation
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10/67. Neonatal alloimmune thrombocytopenia due to anti-P1A1 (anti-HPA-1a): importance of paternal and fetal platelet typing for assessment of fetal risk.

    Neonatal alloimmune thrombocytopenia (NAIT), which usually involves sensitization to P1A1 (HPA-1a), may have devastating complications for the fetus. These may be prevented by antenatal treatment of severe cases with either maternally administered high-dose gamma-globulin and/or repeated intrauterine platelet transfusions. Determination of the paternal platelet phenotype is useful for counseling parents who have had one or more affected pregnancies. This report of an unaffected pregnancy in a woman with a history of previous pregnancies complicated by NAIT illustrates the role of paternal and fetal platelet phenotyping in managing existing pregnancies at risk of NAIT.
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keywords = pregnancy
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