Autosomal dominant polycystic kidney disease in pregnancy complicated by twin gestation and severe preeclampsia: a case report

BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD), an autosomal dominant genetic disorder with a reported prevalence of 1 in 1,000, may be associated with hypertensive disease in pregnancy. The evaluation of a pregnant woman with an adult-onset genetic disorder is complex and involves counseling about inheritance, prenatal diagnosis and management of the current pregnancy. CASE: A 33-year-old woman presented for obstetric care with a history of hypertension and ADPKD for 6 years. The patient had secondary infertility, which was treated by in vitro fertilization. The case was complicated by twin gestation and superimposed severe preeclampsia, leading to preterm cesarean delivery at 26 weeks' estimated gestational age. CONCLUSION: Because of the heritable nature of ADPKD and the long-term risk of end-stage renal disease requiring dialysis and/or renal transplantation, the evaluation and counseling of women with ADPKD who are pregnant or considering pregnancy should include a discussion of the modes of inheritance, natural history, available prenatal diagnostic options, and pregnancy risks and management options. Specific counseling issues in this case include the genetic concepts of variable expression and penetrance and the medical management of chronic hypertension and preeclampsia.     

A tale of two prior probabilities--avoiding the false positive antenatal diagnosis of autosomal recessive polycystic kidney disease.

We describe a case in which the typical ultrasound diagnosis of infantile polycystic kidney in a woman with no family history of renal disease was not confirmed by histology following termination of the pregnancy. This is contrasted with the situation in another couple who were known carriers of autosomal recessive polycystic kidney disease and where the prenatal ultrasound diagnosis was confirmed histologically. When prior genetic risk is low, the possibility of a normal or less severe outcome must be discussed with parents when fetal ultrasound shows large, echogenic kidneys but normal amniotic fluid volume.     

Enfermedad de Berger y gestación

IgA glomerulonephritis or Berger's disease is a kidney disease characterized by immunoglobulin A deposition in the mesangium of renal glomeruli. During pregnancy, key prognostic factors, such as blood pressure and renal function, should be monitored closely to avoid complications, both maternal and fetal. If these factors are regulated, pregnancy is entirely feasible.     

妊娠合并肾脏疾病28例的产科处理

目的探讨妊娠合并肾脏疾病的产科处理。方法对妊娠合并肾脏疾病28例病例的产科处理做回顾性分析。结果28例妊娠合并肾脏疾病中合并肾炎的发病率最高(20/28),妊娠并发症中妊娠期高血压疾病发生率最高(10/28)。24例肾功能代偿期孕妇均定期接受产科检查,除1例孕13周时行人工流产术外,其余23例均足月正常分娩,母儿预后良好;3例合并妊娠期高血压疾病子痫前期(重度)、肾功能不全(氮质血症期),除1例早产外,另2例剖宫产终止妊娠,母儿预后良好;另有1例孕期未进行产前检查,孕24周合并妊娠期高血压疾病子痫前期(重度),胎儿宫内发育迟缓,肾功能不全(尿毒症期),以剖宫产终止妊娠,胎儿死亡。结论妊娠结局与妊娠合并肾脏疾病中肾功能的分期和有无妊娠并发症密切相关;孕期检查和适时、适当的产科处理对于围生儿、孕妇的预后至关重要。     

Hyperreactio luteinalis in a woman with high-risk factors. A case report

BACKGROUND: Hyperreactio luteinalis is a rare condition characterized by multicystic and bilateral ovarian enlargement associated with high maternal human chorionic gonadotropin serum levels. CASE: A case of spontaneous twin pregnancy, polycystic kidney and thyrotoxicosis was treated conservatively. CONCLUSION: In this case, hyperreactio luteinalis was associated with twin pregnancy in a woman with preexisting renal failure. The association of other endocrinopathies, such as hyperthyroidism and diabetes mellitus, creates a clinical problem that could be quite hazardous in pregnancy if this association is not detected.     

Minimal change disease in pregnancy

Abstract

Background: New onset minimal change disease (MCD) is rare in pregnancy with the potential for serious complications including acute kidney injury (AKI).

Case: A case of MCD was diagnosed at 19 weeks gestation by renal biopsy. Within one month of starting steroids, the patient experienced normalization of renal function and resolution of nephrotic syndrome, although hemodialysis was needed as a temporizing measure.

Conclusion: The differential diagnosis for new onset proteinuria in pregnancy should include MCD. In selected cases, renal biopsy can be used to confirm diagnosis, and when indicated, hemodialysis should be instituted while awaiting a response to steroid therapy.     

Chronic renal disease in pregnancy

The purpose of this review was to examine the impact of varying degrees of renal insufficiency on pregnancy outcome in women with chronic renal disease. Our search of the literature did not reveal any randomized clinical trials or meta-analyses. The available information is derived from opinion, reviews, retrospective series, and limited observational series. It appears that chronic renal disease in pregnancy is uncommon, occurring in 0.03-0.12% of all pregnancies from two U.S. population-based and registry studies. Maternal complications associated with chronic renal disease include preeclampsia, worsening renal function, preterm delivery, anemia, chronic hypertension, and cesarean delivery. The live birth rate in women with chronic renal disease ranges between 64% and 98% depending on the severity of renal insufficiency and presence of hypertension. Significant proteinuria may be an indicator of underlying renal insufficiency. Management of pregnant women with underlying renal disease should ideally entail a multidisciplinary approach at a tertiary center and include a maternal-fetal medicine specialist and a nephrologist. Such women should receive counseling regarding the pregnancy outcomes in association with maternal chronic renal disease and the effect of pregnancy on renal function, especially within the ensuing 5 years postpartum. These women will require frequent visits and monitoring of renal function during pregnancy. Women whose renal disease is further complicated by hypertension should be counseled regarding the increased risk of adverse outcome and need for blood pressure control. Some antihypertensives, especially angiotensin-converting enzyme inhibitors and angiotensin-receptor blockers, should be avoided during pregnancy, if possible, because of the potential for both teratogenic (hypocalvaria) and fetal effects (renal failure, oliguria, and demise).     

Recurrent excessive dilatation of the efferent urinary tract in pregnancy. Case report

Case report on a patient with an extensive dilatation of renal pelvis and ureter during the first and the following two pregnancies. Operative drainage was performed of renal pelvis on both sides in the 24th and 28th week of the first pregnancy. The second pregnancy ended with spontaneous abortion in the 27th week of pregnancy. Percutaneous punction nephrostomy with successful course of the third pregnancy. Drainage of renal pelvis by punction nephrostomy is indicated in case of co-existence between dilatation of renal pelvis and severe illness and destruction renal parenchyma and of septic pyelonephritis in pregnancy. This procedure should also be done in case of a solitary kidney and excessive dilatation of the renal pelvis. An operative approach is recommended in renal abscess.     

Hemolytic uremic syndrome in twin pregnancy at 32 weeks gestation with HELLP syndrome. Case report]

Renal failure occurring in pregnancy or post partum is an unusual but well-described complication. Acute renal failure seems to be associated more often with HELLP syndrome rather than with pre-eclampsia or chronic hypertension. Probable overlapping of HELLP and hemolytic uremic syndrome in pregnancy or postpartum should be taken into consideration when treating pregnant women who show signs of proteinuria, hypertension, hematuria, increase of reticulocytes, decrease of haptoglobin with thrombocytopenia and microangiopathic hemolytic anemia. Our case refers to a 32 year old woman at 32 weeks gestation in twin pregnancy who presented with HELLP syndrome and renal failure. Immediately postpartum oliguria was noted and the laboratory analyses suggested the coexistence of HELLP and hemolytic uremic syndrome. In patients with gestosis and/or HELLP syndrome presenting oliguria combined with a decrease of hemoglobin level not due to intraoperative hematic leaks it is always necessary to ask for haptoglobin dosage. In treating hemolytic uremic syndrome it is very important to use a high dosage of plasma and sometimes plasmapheresis. HELLP syndrome contributes to various complications which are sometimes responsible for kidney or maternal mortality. In treating these patients early diagnosis combined with a specific treatment can considerably reduce kidney and maternal mortality.     

妊娠合并慢性肾病与不良妊娠结局

育龄期女性约3%合并慢性肾脏疾病,这些患者不良妊娠结局风险明显高于健康人群。基础肾脏功能、是否合并系统性疾病、慢性高血压以及蛋白尿均对妊娠结局有重要的影响。文章阐述合并慢性肾病患者不良妊娠结局发生情况,并简要介绍改善此类患者妊娠结局的措施。     

Urinoma prenatal y función renal posnatal

Urinoma consists of extravasated urine contained by Gerota's fascia. On ultrasound examination, this entity appears as an echo-negative image between the kidney and the renal fascia without continuity with the ureteropelvic junction or the renal parenchyma. We diagnosed a urinoma in a woman at 24 weeks of pregnancy, which was totally resorbed and disappeared at 28 weeks of pregnancy; in the postnatal stage, a dysplastic kidney was found. We reviewed 40 cases and found ipsilateral kidney involvement in 80.6% of cases. Poor prognostic factors for postnatal kidney function were female fetus, diagnosis in the second trimester, ureteropelvic junction stenosis, intrauterine resorption and visualization of a dysplastic kidney.     

Inflammatory Bowel Disease Presenting in Pregnancy

A case of inflammatory bowel disease (IBD) presenting in pregnancy is described. Despite previous reports of severe fulminating disease in this type of patient, this woman did well with an uncomplicated course; she responded to standard medical therapy and there were no fetal complications. IBD should not be a contraindication to pregnancy unless the disease is poorly controlled. Pregnancy does not increase the risk of relapse of IBD, but should this occur it is more likely in the first trimester or in the postpartum period. Treatment of IBD in pregnancy should be much the same as in the nonpregnant woman. Corticosteroids and sulphasalazine are safe in pregnancy and are the mainstays of medical treatment. Surgery should proceed for the usual indications of toxic megacolon and perforation. In the group requiring surgery fetal mortality is considerable but the maternal outcome is improving. Patients presenting with IBD in pregnancy may have more severe disease but recent reports suggest that the outcome for mother and infant in this group is improving.     

Pre- and peripartal management of a woman with McArdle disease: a case report

McArdle disease or glycogen storage disease (GSD) type V is a rare autosomal recessive inherited disorder in skeletal muscle metabolism leading to exercise intolerance, muscle cramps and in some cases to rhabdomyolysis and acute renal failure due to elevated serum myoglobin levels. Albeit the uterine smooth muscle is not affected, pregnancy and delivery can be physically strenuous and may require specific anesthesiologic care. However, data on pregnancy progress and outcome and on special implications linked to anesthesia in women with McArdle’s disease is scarce, thus posing a challenge to pre- and peripartal management. We report a case of a pregnant woman with Morbus McArdle who was monitored during her pregnancy and delivered a healthy male via cesarean section under spinal anesthesia. Pregnancy, delivery and recovery were uneventful. Our findings, combined with a literature review, lead to the conclusion that uncomplicated pregnancy and delivery can be expected.     

妊娠高血压综合征患者肾脏穿刺结果分析

目的：探讨妊娠高血压综合征(妊高征)患者行肾脏穿刺活检的安全性及了解肾脏病理改变与临床表现的关系。方法：对2000年8月至2002年2月13例妊高征患者于产后2周内(9例)及2周后(4例)采用16G弹射针B超引导下行肾脏穿刺活检，标本分别进行光镜、免疫荧光及部分行透射电镜检查。结果：妊高征肾脏的病理改变表现多样，其中表现为毛细血管内增生性肾小球肾炎3例，膜性肾病改变3例，肾小球轻微病变2例，系膜增生性肾小球肾炎2例，妊娠性肾病2例，局灶性硬化性肾小球肾炎1例。结论：妊高征患者肾脏穿刺活检是安全可行的，妊高征患者的肾脏病理改变可表现为各种病理类型，与临床病情无相关性，符合继发性肾病综合征改变。应对有肾脏损害的妊高征患者作相应随访，以发现妊娠诱发的肾脏疾病。     

Pregnancy in a woman with chronic renal failure--the case of two successfully completed pregnancies and the review of the literature

Together with the prolongation and improving the quality of life of young women with chronic renal failure (CRF), procreation becomes an important issue. Pregnancies in women on renal replacement therapy are associated with an increased risk of health complications, both for mothers and for fetuses. Medical management of pregnant women with CRF is a great challenge and requires a close co-operation of nephrologists, transplantologists, gynecologists and neonatologists. The complexity of problems in these particular pregnancies has urged us to describe the case of a woman with CRF who successfully delivered two babies. We also review the current state of knowledge on the topic. The first pregnancy five years after renal transplantation, was completed with the delivery of term newborn with Tetralogy of Fallot. Also the second pregnancy on hemodialysis therapy was finished by the birth of a healthy neonate at term. The described case indicates that the gynecologists should be prepared for the challenge of the care for pregnancies in women suffering from chronic renal failure on renal replacement therapy.     

Neurosarcoidosis diagnosed during pregnancy by thoracoscopic lymph node biopsy. A case report

BACKGROUND: Sarcoidosis is a multiorgan-system granulomatous disease causing respiratory complaints in most patients. Eye involvement, most commonly granulomatous uveitis, is seen in 5% of black patients with sarcoidosis. Neurosarcoidosis is also rare, affecting 5% of patients with sarcoidosis. Thoracoscopic lymph node biopsy in pregnancy has never before been reported. CASE: An otherwise-healthy, 25-year-old woman, gravida 2, para 1001, presented at 25 weeks' gestation with a painful facial palsy and visual defects. Ophthalmologic examination revealed uveitis, and a chest radiograph revealed asymmetric hilar adenopathy. At 28 weeks' gestation, the patient underwent thoracoscopic lymph node biopsy, which confirmed the diagnosis of sarcoidosis. The symptoms were stabilized with therapeutic corticosteroids, and the patient delivered a healthy neonate at term. CONCLUSION: The diagnosis of sarcoidosis remains one of exclusion and requires a high index of suspicion. Tissue confirmation is often necessary, especially when patients have extrathoracic complaints. For patients without other lesions amenable to biopsy, thoracoscopic lymph node biopsy can be considered despite pregnancy.     

Prenatal diagnosis of the hemodynamics of fetal renal disease by color Doppler ultrasound

OBJECTIVE: Hemodynamic analysis of the fetal renal artery elucidated the function of the renal glomerulus and renal tubule in normal growth fetus and was weighed against fetal renal disease. DESIGN: The subjects were fetuses from pregnant women who gave informed consent. There were 6 cases of polycystic kidney, 4 cases of hydronephrosis and 33 cases of fetuses presenting with normal growth. A longitudinal study was performed for normal growth fetuses. Using maximum systolic velocity (V(max)), pulsatility index (PI) and resistance index (RI), the blood flow was measured initially at 20-24 weeks of pregnancy and every 4 weeks thereafter. The measurement was performed 5 times in total. Also, for fetal renal disease, the measurement was performed using the same indexes. RESULTS: In 2 cases of polycystic kidney, which led to death due to postpartum afunctional kidney, V(max) indicated the lower level of less than mean -1.5 SD. In 1 case of single hydronephrosis, the single afunctional kidney was observed postpartum due to blood flow disruption. In 7 cases of normal renal function after birth, it indicated the lower level in some gestational ages but was generally in the normal range. CONCLUSIONS: Using indexes to evaluate the glomerulus and renal tubule of fetal renal disease, mean -1.5 SD of V(max) can be considered to be the lower limit in the normal range and expected to be an important factor for the final outcome.     

A rare case of early onset nephrotic syndrome in pregnancy

Nephrotic syndrome occurs very rarely, about 0.012-0.025% of all pregnancies. Here, we report a rare case of early onset nephrotic syndrome developing de novo in the 17th week of pregnancy. A renal biopsy was done and the specimens revealed typical features of focal segmental glomerulosclerosis. The patient had a progressive clinical course of disease despite steroid treatment. Suffering from severe intrauterine growth restriction, the fetus died in utero. After delivery, steroid treatment was continued. The patient had normal renal function with a decrease in proteinuria in the second and fifth month postpartum. This report points out the poor fetal prognosis associated with an early onset nephrotic syndrome. Pregnant patients with early onset nephrotic syndrome should be carefully evaluated for the presence of chronic renal disease, and primary renal pathology should be included in the differential diagnosis of massive proteinuria in early pregnancy.     

Superimposed toxemia,abruptio placentae,hypofibrinogenemia, acute renal shutdown,and paralytic ileus complicating a case of prepregnancy hypertension

This case exemplifies the numerous problems that can occur in a pregnant woman with prepregnancy hypertension on which is suddenly superimposed toxemia of pregnancy. An alteration in the clotting mechanism was constantly watched for. Acute renal shutdown was one of the complications we were concerned with after the episode of bleeding and the period of shock which might cause damage to the pituitary, brain, and kidney. Paralytic ileus that developed after the anuria possibly could be explained by bleeding into the peritoneal cavity. It is not an uncommon factor. Ruling out whether or not the patient had a volvulus of the sigmoid was another problem.     

Complete septate uterus,obstructed hemivagina,and ipsilateral renal anomaly: pregnancy course complicated by a rare urogenital anomaly

Background  Abnormalities of the female reproductive tract are associated with a multitude of intrapartum complications. In the case that follows, we report the complicated pregnancy course of a woman diagnosed postpartum with the uncommon syndrome of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) associated with a complete septate uterus. Case  A 19-year-old nulliparous woman with a history of a right nephrectomy secondary to multicystic dysplastic kidney disease presented with premature rupture of membranes at 30 weeks’ gestation. After failed induction of labor at 34 weeks’ gestation, a uterine anomaly was discovered at the time of cesarean section. Magnetic resonance imaging performed postpartum revealed a complete septate uterus, longitudinal vaginal septum, and obstructed right hemivagina. Conclusion  The OHVIRA syndrome is classically associated with uterus didelphys, not complete septate uterus as in this woman. This case illustrates the potential for intrapartum complications in women with abnormalities of the urogenital tract.