Cases reported "Puerperal Disorders"

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1/8. The time course of the change in antibody titres in herpes gestationis.

    The time course of the change in antibody titres was examined postpartum after treatment in two patients with herpes gestationis. The first patient, a 29-year-old woman seen first in the 32nd week of her first pregnancy, had an exudative erythema, and developed an itchy erythema with small tense vesicles on the trunk and legs after delivery in the 40th week of pregnancy. The second patient, a 28-year-old woman seen first in the 28th week of her first pregnancy, had an itchy exudative erythema, small tense vesicles and crusts on the legs. After a Caesarean section in the 40th week of pregnancy performed because of cardiac complications in the fetus, the skin lesions extended to the trunk and extremities. Direct immunofluorescence revealed linear depositions of IgG and C3 at the basement membrane zone (BMZ) and indirect immunofluorescence was positive at the epidermal side of the BMZ in 1 mol/L NaCl-split skin in both cases. In patient 1, prednisolone, 20 mg/day, administered 4 months after delivery, gave rapid improvement (within 1 week) of the skin lesions; in patient 2, minocycline, 200 mg/day, administered 2 weeks after delivery, gave improvement within 2 weeks. immunoblotting against epidermal extracts revealed the presence of antibodies directed to the 180 kDa bullous pemphigoid antigen in both sera. Indirect immunofluorescence and immunoblot were positive for at least 2 months in patient 1 and for 5 months in patient 2 after disappearance of the skin lesions.
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2/8. Penicillin-induced haemolytic anaemia associated with microangiopathy.

    The development of a penicillin-induced haemolytic anaemia was studied in a patient with microangiopathy following an average daily dose of five million units of penicillin g (total dose 46 million units). It has been proposed that the combination of the benzylpenicilloyl hapten with exposed or altered erythrocyte antigens, induces an autoimmune response. In this particular case, it is suggested that erythrocyte membrane damage has predisposed to immune drug-mediated red cell damage.
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3/8. Epithelioid haemangioendothelioma of the sphenoid bone.

    The authors report a case of cranial EH occurred in the left sphenoid bone that was totally excised without transfusion. A 26-year-old woman presented with a 1-year history of progressing exophthalmos in the left eye. A cranial X-ray showed a mixed osteolytic and sclerotic expansile mass lesion in the left sphenoid bone. Neuroradiologic imaging study revealed a left temporosphenoidal extra-axial expansile mass lesion with heterogeneous enhancement after contrast enhancement associated with destruction and erosion of the temporal and sphenoid bones. The cerebral parenchyma and orbital contents were compressed without any sign of infiltration. Bone scan showed hot uptake in the left orbital region. Angiogram demonstrated marked neoplastic angiogenesis from the middle meningeal artery and other branches of left external carotid artery, for which preoperative embolisation was tried in vain. Cranio-orbito-zygomatic approach was undergone in two stages because of the patient's religious belief (patient was Jehovah's witness) and profuse bleeding during the first surgery. Two weeks after initial operation, second surgery was carried out with total excision of the residual mass in the left orbital ridge. Histopathological examination revealed typical epithelioid cell cords or nests in myxoid stroma with a positive immunoreactivity to factor viii-related antigen. The authors report an unusual case of EH involving sphenoid and temporal bone in a young woman. Preoperative embolisation is thought to be absolutely essential before removal. Because there is no convincing data to advise radiation/chemotherapy, total resection and close follow-up may be reasonable.
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4/8. Postpartum acute exacerbation of chronic hepatitis c with complete response to interferon-alpha.

    We describe a patient with chronic hepatitis c who had severe postpartum acute exacerbation of the disease, with marked aminotransferase elevations and jaundice. The viral genotype was 2a, and the patient had a low viral load. Neither superinfection with another hepatotropic virus nor autoantibodies were evident. Markedly increased serum concentrations of T-helper (Th) 1-type cytokines and cytokine receptors, including interleukin (IL)-8, tumor necrosis factor (TNF)-alpha, soluble TNF receptor (sTNFR)-p55, sTNFR-p75, and soluble Fas antigen (sFas), as well as that of the Th 2-type cytokine, IL-10, were present. Complete biochemical and virologic response was achieved with interferon (IFN)-alpha treatment, which decreased cytokine elevations while favoring Th 1 dominance. Acute exacerbation of hepatitis c may occur when cellular immune responses are activated, as in late pregnancy and in the postpartum period. Treating such acute exacerbations immediately with IFN may be highly efficacious.
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5/8. Postpartum renal failure in a patient with membranoproliferative glomerulonephritis type II.

    The previously reported detrimental effects of pregnancy on the course of membranoproliferative glomerulonephritis type II (MPGN type II) are limited and are usually considered to be mild. Based on these reports, a 19-year-old female with the diagnosis of MPGN type II who had stable renal function (creatinine 0.9 mg/dl) and a mild nephrotic syndrome with hypertension for 5 years of close follow-up was advised to complete her pregnancy. After a full-term pregnancy, complicated only by moderate nephrotic syndrome, a healthy female infant was born. Two weeks after delivery, the patient presented with acute renal failure and malignant hypertension, without evidence of hemolysis of hepatic failure. Immunologic parameters, including, C3, C4, antinuclear antibodies, circulating immune complexes as well as antibodies to glomerular basement membrane antigen and tubular basement membrane antigen were negative. peritoneal dialysis was initiated and a renal biopsy was performed which showed MPGN type II with 50% crescents. Despite pulse therapy with methylprednisolone, renal function did not improve, resulting in the need for chronic dialysis. Although no specific nephritogenic mechanism was shown, the course of this patient should be considered when counseling female patients with MPGN type II, regarding the possibility of pregnancy exacerbating their disease, or resulting in rapidly progressive renal failure.
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6/8. Post-partum thyroiditis and goitrous (Hashimoto's) thyroiditis are associated with HLA-DR4.

    Using new panels of HLA-DR typing sera, we found an increase in the prevalence of HLA-DR4 in 21 patients with Hashimoto's thyroiditis (57%) and 32 patients with post-partum thyroiditis (53%) compared to controls (21%). Hashimoto's thyroiditis was previously found to be associated with HLA-DR5 and Dw5. We feel that the doubts raised by this study warrant the study of DR antigens in thyroid autoimmune disease using large panels of specific sera.
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7/8. Puerperal acquired factor viii inhibitor causing a von Willebrand-like syndrome in a patient with anti-dna antibodies.

    A 30-year-old Malay lady, with no previous or family history of bleeding, presented with severe gum bleeding 25 days post-partum. The factor viii:c was 0.03 iu/ml with evidence of a slow-acting factor viii inhibitor. von willebrand factor antigen (VWF:age) varied from less that 0.05 to 0.17 iu/ml, and there was absent ristocetin-induced platelet aggregation. Anti-nuclear and anti-dna antibodies were present, but there were no other features of systemic lupus erythematosus. There was some clinical response to cryoprecipitate and tranexamic acid, and slight improvement with corticosteroid. Fifteen months later, the patient has no active bleeding problem, and her VWF-ag is increasing spontaneously. However, factor viii:c is less than 0.01 iu/ml and her factor viii inhibitor titre is still > 20 Bethesda units/ml.
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8/8. Graves' hyperthyroidism after postpartum thyroiditis.

    Two Caucasian patients are described who had destructive postpartum thyroiditis (PPT) before the subsequent onset of Graves' hyperthyroidism (GH). HLA class II DQ typing in these two subjects identified putative susceptibility alleles previously detected in GH and PPT. Although PPT destructive thyroiditis preceding the development of GH is relatively uncommon, the occurrence of both these syndromes in the same patient suggests the possibility of an etiological role for thyroid antigen release and genetic susceptibility as pathogenic factors in the development of Graves' disease.
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