Landry Guillian-Barre Strohl syndrome in pregnancy: use of high-dose intravenous immunoglobulin.
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Biomedical subjects
Publications and source records attributed to M Seoud.
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Bernard-Soulier Syndrome is a rare hereditary thrombocytopathy that manifests clinically by excessive bleeding out of proportion to the degree of thrombocytopenia. Only eight reports of Bernard-Soulier Syndrome in pregnancy have been previously described. In some patients the pregnancy course was smooth while in others post-partum haemorrhage was the most common complication. We present a primiparous female who had immediate and delayed post-partum haemorrhage that was managed conservatively. Review of the literature is also included.
Serum erythropoietin (EPO), hematocrit and hemoglobin levels were determined in 17 female patients with stage II breast cancer before, during and at the conclusion of non-nephrotoxic chemotherapy. Serum EPO levels were determined using the ELISA technique. No irradiation was given to any patient. The hemoglobin and hematocrit levels remained stable. However, a statistically significant increase in EPO was noted (p<0.05). The possible factors involved in this increase are reviewed, however the exact mechanism remains to be elucidated.
OBJECTIVE: To evaluate the relationship between the hematocrit (HCT), serum erythropoietin (EPO) and insulin-like growth factor-1 (ILGF-1) levels in breast cancer patients receiving non-nephrotoxic chemotherapy. METHODS: Seventeen patients with stage II breast cancer were included. All received 6 cycles of non-nephrotoxic chemotherapy (cyclophosphamide, 5-fluorouracil and doxorubicin or methotrexate with or without tamoxifen). Insulin-like growth factor-1 and EPO levels were determined before and at the end of therapy. Serum EPO levels were determined by Enzyme linked- immunosorbant assay (ELISA) while those of ILGF- I by radioimmunoassay (RIA). RESULTS: A significant drop in mean HCT from 37.41%+/-0.77% to 35.18%+/-0.70%, associated with a significant decline in ILGF-1 levels from 92.1+/-15.48 ng/ml to 52.75+/-10.5 ng/ml at the end of the treatment was noted. This association became significant when patients receiving tamoxifen were excluded (r=0.69, p=0.02). The mean serum EPO levels increased significantly from 13.64+/-0.55 U/l to 19.44+/-3.18 U/l and correlated negatively with ILGF-1 level (r=-0.46, p=0.05). There was no significant relation between the serum EPO levels and HCT (r=-0.26, p=0.32). CONCLUSION: The current data show that ILGF-1 may play an important role in erythropoiesis and it correlates better than EPO with HCT in breast cancer patients receiving non-nephrotoxic chemotherapy.
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The atypical reaction of a patient to the news of her terminal illness raises a number of questions about care for the dying.
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BACKGROUND: Familial recurrent molar pregnancies are exceedingly rare. The genetic basis for recurrent moles is not well understood, and its association with major human lymphocytic antigen histocompatibility is debatable. The purpose of this report is to present a family with extensive intermarriage and recurrent molar pregnancies with some emphasis on the result of the human lymphocytic antigen-typing. CASE: Two sisters, both married to first-degree cousins, had three and five pathologically confirmed molar pregnancies, respectively. A second-degree cousin, also married to her first-degree cousin, is also reported to have had five consecutive moles. Chromosomal analysis and human lymphocytic antigen-typing on the two sisters and their spouses was performed. Human lymphocytic antigen-typing was compared to a cross-sectional sample of our population. This showed a high incidence of unusual human lymphocytic antigens in these family members. CONCLUSION: In families with extensive intermarriage and recurrent molar pregnancies, patients and their spouses may have unusual human lymphocytic antigen histocompatibility, which supports the possibility of a strong genetic predisposition expressed at the level of major histocompatibility class I and II gene translation.
Bernard-Soulier Syndrome is a rare autosomal recessive bleeding disorder characterized by a normal or low platelets count, the presence of giant platelets, and a prolonged bleeding time. Only five pregnant patients with this disease have been previously reported. Most of the complications seem to occur in the intrapartum or postpartum period. In some patients the disease can go unrecognized until the third or fourth decade. Our patient had two uneventful pregnancies, one pregnancy with early postpartum hemorrhage, and another pregnancy with intrapartum and late postpartum bleeding. Although the optimum mode of delivery is not clear yet, the vaginal route should be considered unless otherwise obstetrically indicated. Management of active bleeding episodes is also debatable; it includes platelets transfusions, desmopressin (DDAVP), antifibrinolytic therapy, and ecbolic agents for postpartum hemorrhage.
Brucellosis in pregnancy is a rare entity. It has been associated with an increased risk of spontaneous abortion in animals and possibly humans. Among six patients with brucellosis in pregnancy, one requested termination of the pregnancy in the first trimester, one had a spontaneous abortion at 12 weeks that could not be directly related to brucellosis, and four continued their pregnancies with no significant increase in maternal or neonatal morbidity or mortality.
Fourteen cases of typhoid fever complicating pregnancy are presented. The diagnosis was confirmed by blood cultures in 13 patients and by a rising Widal titer in one. Stool cultures were positive in only two out of five patients; urine cultures in 12 patients and cervical cultures in five patients were all negative. The clinical presentation was similar to the description in older reports, except for the absence of relative bradycardia. Hypothermic response to antipyretics was frequently observed. Patients were treated with either chloramphenicol, ampicillin, or amoxicillin, with satisfactory response. Typhoid fever diagnosed in the latter part of the second trimester and third trimester and treated early did not seem to alter the neonatal outcome.
Routine sonography prior to genetic amniocentesis revealed the presence of a large complex mass with pulsating channels over the anterior fetal chest wall. This led to early termination of pregnancy in a fetus affected with the rare Klippel-Trenaunay-Weber Syndrome.
Hydatidiform mole is a benign trophoblastic neoplasia characterized by an abnormal development of the embryo and proliferation of placental villi. Using microsatellite markers amplified by the polymerase chain reaction, we have performed a genetic study on eight independent molar tissues occurring in two sisters. Karyotype and genotype data demonstrate a diploid and biparental constitution in seven of the analyzed moles suggesting a common mechanism underlying the etiology of the various molar pregnancies in this family. The data reported here suggest that complete and partial hydatidiform moles are not always separate entities and that women with familial recurrent hydatidiform moles are homozygous for an autosomal recessive mutation.
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.
Desmoid tumors of the female pelvis are rare. The efficacy of the available treatment modalities in improving survival and decreasing recurrence remains controversial. A 32-year-old woman presented with an asymptomatic large ischeorectal mass. Computed tomography scan revealed a large tumor adherent to the pubic bone and impinging on the bladder neck and the rectum. Aggressive surgical removal of the mass including partial osteotomy of the pubic bone was followed by radiotherapy. The patient is still alive 6 years later with no evidence of disease. Aggressive surgical management followed by radiotherapy is an acceptable means of treatment of locally invasive desmoid tumor of the female pelvis.