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Women on regular dialysis are usually infertile, but contraception should not be neglected. Pregnancy is invariably complicated and poses excessive risks, with an uncertain and low chance of success. Even when therapeutic abortion is excluded, the live birth outcome at best is 19%. Renal transplantation usually reverses abnormal reproductive function and comprehensive pre-pregnancy counseling is essential, with discussion of all implications, including the harsh realities of long-term maternal survival. In this survey of 2,309 pregnancies in 1,594 women, therapeutic abortion was undertaken in 27% of conceptions and the spontaneous abortion rate was 13%. Of the conceptions that continued beyond the first trimester, 92% ended successfully. In most, renal function was augmented in pregnancy, with transient deterioration in late pregnancy (with or without proteinuria). Permanent renal impairment occurred in 15% of pregnancies. There was a 30% chance of developing hypertension, preeclampsia or both. Preterm delivery occurred in 50%, and intrauterine growth retardation in 25% of pregnancies. Despite its pelvic location, the transplanted kidney rarely produced dystocia and was not injured during vaginal delivery. Cesarean section should be reserved for obstetric reasons only. Neonatal complications include respiratory distress syndrome, leukopenia, thrombocytopenia, adrenocortical insufficiency, and infection. No predominant or frequent developmental abnormalities have been described and data on infancy and childhood are encouraging. For the future more work is needed to improve pre-pregnancy assessment criteria, to understand the mechanisms of gestational renal dysfunction and proteinuria, to assess the side effects and implications of immunosuppression in pregnancy, and to elucidate the remote effects of pregnancy on both renal prognosis and the offspring.
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PMID:Dialysis, transplantation, and pregnancy. 195 48

One hundred and sixty eight adult patients with B-cell non-Hodgkin's lymphoma (NHL) and other hematologic malignancies who underwent autologous or allogeneic bone marrow transplantation (BMT) were investigated for the subsequent development of hemolytic-uremic syndrome (HUS). All patients were conditioned with cyclophosphamide and total body irradiation. When examined at 3-month intervals for the first year post-BMT, all patients had uniform measurements of hematocrit (Hct) and serum creatinine. Sixteen patients who initially exhibited Hct and creatinine values that were normal range for the BMT populations developed a sudden decrease in Hct and increase in creatinine between 3 and 11 months post-BMT and fulfilled the clinical and laboratory criteria for HUS. None of these patients had known active cytomegalovirus infection, graft-versus-host disease, or cyclosporine administration. The degree of decrease in Hct and creatinine elevation ranged from solely laboratory abnormalities to a clinically significant syndrome. Twelve of the 16 patients developed acute clinical complications of congestive heart failure, hypertension (HTN), or peripheral edema. Twelve patients required red blood cell support, whereas only four patients required platelet transfusions. Both hemolytic anemia and thrombocytopenia have resolved in virtually all cases. At a mean follow up of 18 months postdiagnosis, creatinine elevations have persisted along with HTN. All patients have survived without life-threatening long-term sequelae. With the increasing use of BMT as a curative modality for patients with hematologic malignancies, it becomes important to prospectively monitor patients for the development of HUS and its potential long-term impact on renal function.
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PMID:Hemolytic-uremic syndrome following bone marrow transplantation in adults for hematologic malignancies. 201 7

Sixteen children (11 male, 5 female) who developed renal venous thrombosis in the neonatal period or early infancy have been followed for periods varying from 29 months to 16 years (median 12 years) after initial diagnosis. Twelve patients presented with a hyperosmolar state, and in 4 the illness was preceded by severe birth asphyxia. The diagnosis was based on the findings of clinical and/or radiological renal enlargement (15 cases), haematuria (16 cases) and elevation in plasma urea (16 cases). Thrombocytopenia (13 cases), red cell fragmentation (13 cases) and oliguria (13 cases) were frequent associated findings. All 16 patients survived the acute illness, 1 patient died 3 years later from an unrelated event. On follow-up evaluation, 11 patients have normal renal function (glomerular filtration rate greater than 80 ml/min per 1.73 m2), 5 developed hypertension, 2 of whom responded to unilateral nephrectomy. Urinary concentrating capacity was subnormal (less than 800 mosmol/kg) in 7 of the 15 cases studied. Follow-up renal imaging studies were undertaken in 14 patients, and the imaging procedure was normal in only 2 of these. Of the remainder, 8 showed unilateral abnormality and 4 bilateral abnormality. Intellectual development was normal in 12 patients, mildly impaired in 1, and severely impaired in 3.
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PMID:Renal venous thrombosis in infancy: long-term follow-up. 202 36

On the basis of a review of the literature and five case histories, the pathophysiological and clinical features in pregnant women with haemolysis, elevated liver enzymes and thrombocytopenia which together constitute the HELLP syndrome (haemolysis, elevated liver enzymes and low platelet count) are illustrated. This syndrome describes a complicated obstetric course with greatly increased neonatal and maternal morbidities. Hypertension/proteinuria are common but are not obligatory. The condition should be suspected in pregnant women with pain under the right rib margin and unexplained jaundice. The diagnosis is verified by the blood picture, liver enzyme count and a blood smear. Women with verified HELLP syndrome and gestational age greater than 34 weeks should be delivered immediately. Women with the same syndrome and gestational age less than 32 weeks should be delivered if the condition cannot be rapidly controlled. In exceptional cases cesarean section may be necessary. On the basis of the coagulation status, the defective plasma components may be supplied (e.g. fresh frozen plasma and antithrombin-III). Plasmapheresis and specific pharmacological intervention must be considered as experimental at present, although these present promising therapeutic possibilities.
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PMID:[Hemolysis, elevated liver enzymes and thrombocytopenia: the HELLP syndrome--a manifestation of severe pre-eclampsia]. 204 40

We describe a 14 year old boy with antiphospholipid syndrome who initially presented at the age of 10 with recurrent loin pain, fever, weight loss, leucocytosis, thrombocytopenia, hypertension and haematuria. He had primary adrenal insufficiency with bilaterally enlarged adrenals on computed tomographic (CT) scan consistent with adrenal infarction. Renal and liver biopsies showed microthrombi in the glomerular capillaries and hepatic sinusoids respectively. The case is unusual in that hypertension rather than hypotension was dominant and a CT scan was consistent with bilateral adrenal infarction without haemorrhage. He represented with evidence of persistent hypertension with glomerulosclerosis and glomerular microthrombi on repeat renal biopsy. He continues to have permanent adrenal insufficiency with complete atrophy of his adrenals.
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PMID:Addison's disease, hypertension, renal and hepatic microthrombosis in 'primary' antiphospholipid syndrome. 206 36

Thrombotic thrombocytopenic purpura (TTP) is a syndrome that occurs mainly in adults with multiorgan microvascular thrombosis consisting of thrombocytopenia, microangiopathic hemolytic anemia, neurologic symptoms, renal involvement, and fever. The female to male ratio is 3:2, and peak incidence occurs in the 3rd decade of life. Clinical signs are the consequence of hyaline thrombosis and occlusion of capillaries and arterioles. Renal ailment manifests itself in hematuria and proteinuria with azotemia and even overt renal failure. In severe disease, azotemia is typical of hemolytic uremic syndrome (HUS). TTP was first described in 1925 by Moschcowitz. The clinical picture of TTP consists of a prodromal phase, a viruslike disease occurring in up to 40% of patients. 60% have neurologic disturbances, 90% have purpura initially, and fever occurs in all. Anemia is often severe with hemoglobin values of 7-9 gm/dl, renal involvement in 90%, and renal failure in 40-80% of patients. Clinical variants include the acute and fulminant variety mortality, the chronic form, and the relapsing form. Predisposing factors and triggering agents are autosomal recessive inherited traits in acute idiopathic TTP, systemic diseases, tumor antigens, pregnancy and puerperium, viruses (endotoxins for HUS), and possibly oral contraceptives and hypertension. Therapy includes corticosteroids (prednisone 100-400 mg/day); heparin for postpartum HUS; and antiplatelet agents (Dextran 70, aspirin, and dipyridamole in high doses). The infusion of PGI2 is controversial; splenectomy is also questionable; and vincristine, azathioprine, and cyclophosphamide have unproven efficacy. Fresh-frozen plasma exchange is the method of choice as it produces survival in 90%. Others are iv immunoglobulins, vitamin E, and dialysis and renal transplant. Platelet transfusions are contraindicated because of sudden death and decreased survival.
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PMID:Thrombotic thrombocytopenic purpura and related disorders. 210 74

An autopsy case of hemolytic uremic syndrome after treatment with antineoplastic agents for advanced gastric carcinoma is reported. A 70 year-old woman underwent partial gastrectomy for gastric carcinoma on April 16, 1987 (P0H0S2N4, Stage IV). She was treated with Mitomycin C (MMC), UFT, OK-432 and PSK as post operative chemotherapy. Total doses were 60 mg of MMC, 33.9 g of UFT, 55 KE of OK-432 and (507 g) of PSK. She suffered from occult blood in urine in September 1987, thrombocytopenia and anemia in October, edema and hypertension in November and died due to acute renal failure and pulmonary failure on December 5, 1987. It seemed that the cause of death was hemolytic uremic syndrome induced by antineoplastic agents.
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PMID:[A case report of hemolytic uremic syndrome (HUS) induced by antineoplastic agents]. 212 Mar 75

Sixty-one of 375 women with hypertension and preeclampsia in a 48-month period had thrombocytopenia. By postpartum day 3, 90% of these women had rising platelet counts, and by day 4, 59 of 61 women had counts greater than 100,000/mm3. The two exceptions had other pathologic conditions in addition to preeclampsia. Thrombocytopenia associated with preeclampsia should resolve by postpartum day 4.
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PMID:The natural history of thrombocytopenia associated with preeclampsia. 153 66

Autoimmune disorders such as SLE and ITP occur more commonly in young women and are the most common complications in pregnancy. There is considerable controversy concerning the risk to the mother and fetus, and the optimal prepartum management for minimizing that risk. 1. SLE is an autoimmune disorder in which IgG antibodies such as anti dsDNA-IgG, anticardiolipin IgG, and anti SS-A/Ro IgG are produced. Lupus nephropathy accompanied by diminished serum complement (CH50) and a rise in antibodies against dsDNA is a frequent clinical problem during pregnancy, which represents the adverse effect of hypertension or superimposed toxemia and causes fetal death or intrauterine fetal growth retardation. Habitual abortion or fetal death is common in a case with high anticardiolipin IgG titre. Anti SS-A antibodies are often found in the infants of antibody-positive mothers, and the deposition of antibodies in the perinodal region cause congenital heart block. IgG or immune complexes crossing the placenta directly injures the cardiac conduction system. In these cases which have high titre crossing the placenta directly injuries the cardiac conduction system. In these cases which have high titre of autoimmune antibodies, corticosteroid therapy should be started. 2. Management of ITP in pregnancy involves the consideration of three issues: 1) treatment of maternal thrombocytopenia, 2) prediction of fetal thrombocytopenia, 3) obstetrical management. ITP increases the risk for postpartum bleeding of sufficient severity to require blood transfusion. In most of these cases, maternal platelet counts are found to be less than 30,000/mm3. Women who have symptomatic severe steroid-unresponsive ITP may benefit from intravenous IgG(IvIgG) given as elective treatment.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Pregnancy complicated with autoimmune diseases]. 223 Apr 13

The question of whether the HELLP syndrome exists as a distinct entity or is part of a spectrum of pregnancy complications, which have in common hemolysis, elevated liver enzymes, and thrombocytopenia, has long been a source of speculation and debate among obstetricians and internists. A review of the literature indicates a definite need for a uniform definition, diagnosis, and management of this syndrome. Patients manifesting this syndrome usually are seen before term (less than 36 weeks' gestation) complaining of malaise (90%), epigastric or right upper-quadrant pain (90%), and nausea or vomiting (50%), and some will have nonspecific viral-syndrome-like symptoms. Hypertension and proteinuria may be absent or slight. Thus some of these patients may have a variety of signs and symptoms, none of which are diagnostic of classic preeclampsia. In consideration of the high maternal and perinatal mortality and morbidity reported with the presence of this syndrome, I recommend that all pregnant women having any of these symptoms should have a complete blood cell count with platelet and liver enzyme determinations irrespective of maternal blood pressure.
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PMID:The HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets): much ado about nothing? 240 34


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