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Pregnancy during hemodialysis: perinatal outcome in our cases.

The perinatal outcome of patients undergoing chronic hemodialysis has been improved in recent years. In this report we review the treatment and outcome of seven pregnancies in women undergoing chronic hemodialysis before and during pregnancy between 2000 and 2002. The hemodialysis schedule was increased from 4 hours twice weekly to 4 hours four to six times weekly. Hemodialysis was performed using a high-flux dialyzer with volume-controlled ultrafiltration. The patients were followed in close collaboration between the obstetrician and the nephrologist. Monitoring of fetal well-being was started after 24 weeks' gestation, using cardiotocography by a nonstress test twice weekly and by weekly Doppler flow measurements. All patients underwent uterine contraction monitoring immediately after the dialysis. The mean gestational age at delivery was 32 weeks (range, 26 to 36 weeks). The causes of preterm delivery were premature contractions, premature rupture of membranes, preeclampsia, and intrauterine growth restriction. The outcomes were two pregnancies complicated by polyhydramnios and six pregnancies, that resulted in live births, all of whom survived. There was one neonatal death. The mean newborn birthweight was 1400 g (range, 420 to 2640 g) and the 1- and 5-minute Apgar scores ranged from 2/8 and 4/10, respectively one infant at 29-weeks gestation experienced respiratory distress syndrome but did well after 12 days. Cesarean section was performed in four pregnancies. The mothers were discharged on postoperative days 3 to 5. It is well known that the management of pregnant patients undergoing chronic hemodialysis is difficult. However, advances in dialysis, obstetrics, and neonatal care have improved the outcomes.

Adult↗

Clinical presentation of genetically defined patients with hypokalemic salt-losing tubulopathies.

PURPOSE: Hypokalemic salt-losing tubulopathies (Bartter-like syndromes) comprise a set of clinically and genetically distinct inherited renal disorders. Mutations in four renal membrane proteins involved in electrolyte reabsorption have been identified in these disorders: the furosemide-sensitive sodium-potassium-chloride cotransporter NKCC2, the potassium channel ROMK, the chloride channel ClC-Kb, and the thiazide-sensitive sodium-chloride cotransporter NCCT. The aim of this study was to characterize the clinical features associated with each mutation in a large cohort of genetically defined patients. PATIENTS AND METHODS: The phenotypic characteristics of 65 patients with molecular defects in NKCC2, ROMK, ClC-Kb, or NCCT were collected retrospectively. RESULTS: ROMK and NKCC2 patients presented with polyhydramnios, nephrocalcinosis, and hypo- or isosthenuria. Hypokalemia was less severe in the ROMK patients compared with the NKCC2 patients. In contrast, NCCT patients had hypocalciuria, hypomagnesemia, and marked hypokalemia. While this dissociation of renal calcium and magnesium handling was also observed in some ClC-Kb patients, a few ClC-Kb patients presented with hypercalciuria and hypo- or isosthenuria. CONCLUSIONS: ROMK, NKCC2, and NCCT mutations usually have uniform clinical presentations, whereas mutations in ClC-Kb occasionally lead to phenotypic overlaps with the NCCT or, less commonly, with the ROMK/NKCC2 cohort. Based on these results, we propose an algorithm for the molecular diagnosis of hypokalemic salt-losing tubulopathies.

Algorithms↗

Prenatal ultrasonographic diagnosis of intracranial teratoma and massive craniomegaly with associated high-output cardiac failure.

Congenital intracranial teratomas are rare and usually fatal. We present prenatal diagnosis of such a case associated with scalp, facial, and body skin edema, hepatomegaly, extramedullary hematopoiesis, polyhydramnios, and a hydropic placenta. These manifestations of high-output cardiac failure were thought to be the result of the large cardiac output required by massive intracranial tumor arteriovenous shunting.

Adult↗

Diabetes mellitus and fertility control: contraception management issues.

The need to prevent complications in the woman and fetus mandates that pregnancies in diabetic women always be planned and that safe and effective contraceptives be used at all times until it is determined that pregnancy is a safe and desired option. Pregnancy may aggravate complications of diabetes such as retinopathy and coronary artery disease. A pregnant diabetic woman is also more likely to experience such complications as hypertension, urinary tract infection, polyhydramnios, and cesarean section. Her fetus is at increased risk for congenital malformations, prematurity, stillbirth, neonatal morbidity, and diabetes later in life. Good diabetic control must be maintained before and throughout the pregnancy to minimize the risk of these and other complications. Until such time as good control is achieved and the woman desires pregnancy, a reliable method of contraception should be used. Most recent research supports the use of barrier methods, low-dose monophasic or triphasic oral contraceptives, or progestin-only methods, at least for the short-term. Under some circumstances the intrauterine device may be an appropriate option. Long-term data regarding the use of these methods is lacking. The decision regarding which method of contraception is used should be made by the woman in consultation with her physician.

Contraception↗

Ultrasound in the diagnosis of congenital anomalies.

With high-resolution ultrasound equipment, it is now possible to diagnose certain fetal anomalies in the third trimester and in some cases before the twentieth week of gestation. During a 27 month period 2,548 ultrasound scans were performed in high-risk patients. An anomaly was diagnosed in 10 of 122 second-trimester patients who were at risk for recurrent fetal defects. Fetal deformity was also found in 26 third-trimester patients. Of the 2.8% of patients found to have polyhydramnios 18% were associated with various types of anomaly. With ultrasound it was possible to examine internal fetal anatomy and to identify abnormalities of the fetal cranium, spine, chest, abdomen, and limbs. These anomalies are reviewed here in detail. Based on ultrasonically derived information, second-trimester patients can be offered information concerning the status of their fetuses at risk genetically and physicians can better manage third-trimester patients with diagnosed fetal deformities.

Amniotic Fluid↗

Ultrasound evaluation of amniotic fluid volume. II. The relationship of increased amniotic fluid volume to perinatal outcome.

Qualitative amniotic fluid volume determination is a routine part of fetal biophysical profile score testing. The relationship between polyhydramnios and poor perinatal outcome has been previously documented. We have undertaken a retrospective chart review which relates qualitative amniotic fluid volume as determined at the time of last biophysical profile score assessment to perinatal outcome in 7562 referred high-risk obstetric patients. Gross and corrected perinatal mortality in association with normal qualitative amniotic fluid volume ranged from 4.65/1000 and 1.97/1000, respectively, to 32.9/1000 and 4.12/1000 in association with increased qualitative amniotic fluid volume, respectively. The incidences of major congenital anomaly and fetal macrosomia were significantly related to qualitative amniotic fluid volume.

Amniotic Fluid↗

Fetal ascites associated with ABO incompatibility: case report and review of the literature.

We report a pregnancy complicated by anti-B isoimmunization that resulted in fetal ascites, anemia, hepatomegaly, and polyhydramnios. A previous pregnancy in the same patient was complicated by neonatal ABO incompatibility. A review of the obstetric literature suggests that ABO incompatibility may cause severe fetal anemia, especially in patients with type O blood or a previous history of ABO incompatibility of the newborn.

ABO Blood-Group System↗

Intraamniotic deamino(D-Arg8)-vasopressin: prolonged effects on ovine fetal urine flow and swallowing.

OBJECTIVE: Fetal urine is the main source of amniotic fluid, and alterations in urine production have an impact on amniotic fluid dynamics. Intraamniotic arginine vasopressin is absorbed into fetal plasma, but fetal antidiuretic responses may be obscured by vasoconstrictor (V1 receptor) actions of arginine vasopressin. We determined the effects of intraamniotic administration of a specific V2 receptor agonist, deamino(D-Arg8)-vasopressin, on fetal plasma arginine vasopressin immunoreactivity, fetal urine output, and swallowing. STUDY DESIGN: Six chronically prepared pregnant ewes (129 +/- 1 days' gestation) received a single deamino(D-Arg8) (50 micrograms injection into the amniotic fluid with subsequent measurement of fetal plasma arginine vasopressin immunoreactivity, fetal urine flow and osmolality, and fetal swallowing. RESULTS: Within 30 minutes of deamino(D-Arg8)-vasopressin administration, fetal plasma arginine vasopressin immunoreactivity (4.5 +/- 0.5 to 76.4 +/- 21.9 pg/ml) and urine osmolality (135 +/- 13 to 285 +/- 43 mOsm/kg H2O) significantly increased and urine flow decreased (0.18 +/- 0.01 to 0.05 +/- 0.01 ml/kg per minute). After 48 hours urine flow remained reduced (0.08 +/- 0.03 mg/kg per minute) and osmolality remained elevated (399 +/- 18 mOsm/kg H2O). There were no changes in fetal swallowing, systolic (64 +/- 3 mm Hg) or diastolic (42 +/- 3 mm Hg) blood pressure, or heart rate (169 +/- 6 beats/min). CONCLUSIONS: Intraamniotic deamino(D-Arg8)-vasopressin administration evokes persistent fetal antidiuresis in the absence of arginine vasopressin-induced fetal cardiovascular effects or changes in fetal swallowed volume, indicating its suitability as a potential therapeutic agent in amniotic fluid volume disorders such as polyhydramnios.

Amniotic Fluid↗

Mathematic modeling of human amniotic fluid dynamics.

OBJECTIVE: We sought to develop a model quantifying the relative contributions of fetal swallowing and intramembranous flow to amniotic fluid dynamics during human gestation. We then used the model to simulate the impact of absent swallowing on amniotic fluid volume. STUDY DESIGN: The model was developed with published data for normal human amniotic fluid volume and composition, human fetal urine flow rate and composition (11 to 42 weeks), and extrapolated data from ovine lung fluid production. Fetal swallowing and intramembranous flow were calculated with assumptions that (1) swallowed fluid is isotonic to amniotic fluid, (2) intramembranous flow is free water diffusion, and (3) 50% of lung fluid is swallowed. The model was then applied to simulate absent fetal swallowing and variable (0%, 50%) proportions of swallowed lung fluid were used as a representation of esophageal atresia-tracheal fistula variations. RESULTS: Fetal swallowed volume and intramembranous flow linearly increase until 28 to 30 weeks. Daily swallowed volume then exponentially increases to a maximum of 1006 ml/day at term, whereas intramembranous flow continues on a linear trend to reach 393 ml/day at term. With absent swallowing and variable amounts of lung fluid swallowed (0%, 50%), predicted amniotic fluid volume is similar to normal values through 20 weeks, exceeds the 95% confidence interval for normal amniotic fluid volume at 29 to 30 weeks' gestation (approximately 2000 ml), and then exponentially increases. Predicted amniotic fluid osmolality (280 to 257 mOsm/kg) is slightly lower than actual values although within the clinically normal range. CONCLUSIONS: This model indicates that the normal reduction in amniotic fluid volume beginning at 34 weeks results from the marked increase in swallowed volume during the third trimester. Additionally, this model correlates well with the timing of the initial clinical presentation of polyhydramnios observed in some fetuses with conditions that result in absent or reduced swallowing or gastrointestinal atresia. Modeling of amniotic fluid dynamics can predict normal changes in fetal fluid exchange and may aid in understanding of amniotic fluid imbalances.

Amniotic Fluid↗

Relationship between graded degrees of anemia and amniotic fluid volume in the ovine fetus.

OBJECTIVE: Severe fetal anemia is associated with polyhydramnios in both human and ovine fetuses. This study examined the relationship between varying degrees of anemia and amniotic fluid volume in fetal sheep. STUDY DESIGN: Eleven long-term catheterized ovine fetuses at 126 +/- 1 days' gestation (mean +/- SE) were subjected to hemorrhage of 20 to 80 mL daily for 9 consecutive days to produce varying degrees of fetal anemia. Five additional animals served as time control animals. Statistical analysis was by least squares regression and 3-factor analysis of variance. RESULTS: Amniotic fluid volume was 793 +/- 147 mL and did not change with time in the control fetuses. In the fetuses that were subjected to hemorrhage the amniotic fluid volume changed little through the hematocrit range of 40% to 25%. As fetal hematocrit fell below approximately 25%, amniotic fluid volume began to increase. With greater degrees of anemia the amniotic fluid volume increased as an exponential function of hematocrit and approached 2000 mL excess fluid as hematocrit dropped to <15%. According to bivariate regression the increase in amniotic fluid volume was related to fetal hematocrit, PaO(2), and urinary flow rate as well as to plasma and amniotic fluid lactate concentrations. According to multivariate regression only fetal PO(2) and urinary flow rate were significantly related to the increase in amniotic fluid volume. CONCLUSIONS: Although mild anemia was not associated with increased amniotic fluid volume, moderate to severe fetal anemia was associated with an exponential rise in amniotic fluid volume. This rise may have been mediated by a hypoxemia-induced diuresis, by a diuresis related to a lactate-induced osmotic accumulation of fetal fluid, or by both mechanisms.

Amniotic Fluid↗

Prenatal diagnosis of twin-twin transfusion syndrome complicated with hydrops fetalis at 14 weeks of gestation.

Twin-twin transfusion syndrome (TTTS) may complicate multiple pregnancy. Monochorionic discordant twins with oligohydramnios and polyhydramnios may be diagnostic. Hydrops fetalis is particularly ominous. All the signs can appear independently at any stage of gestation. However, TTTS with hydrops fetalis in early pregnancy is rare. We report here a case of TTTS complicated with hydrops fetalis diagnosed at 14 weeks of gestation. Our case may be one of the earliest cases of the prenatal diagnosis of TTTS complicated with hydrops fetalis in the literature.

Abortion, Induced↗

Diabetes mellitus in pregnancy in an African population.

OBJECTIVES: To compare the pregnancy outcome among diabetic and non-diabetic Nigerian women. METHODS: A retrospective case record review of 200 pregnant diabetic patients and control was carried out over a 10-year period (1990-1999) at the Maternity unit of the University of Nigeria Teaching Hospital Enugu, Nigeria. RESULTS: The prevalence of diabetes mellitus among pregnant mothers was 1.7%. Pre-gestational diabetes mellitus accounted for 39% of cases while gestational diabetes was responsible for 61% of them. Late antenatal booking and poor control of diabetes mellitus were common features, while maternal and fetal morbidity was high. Hypertension, vulvovaginitis, premature labor, polyhydramnios and ketoacidosis were significantly higher among diabetic mothers than controls. The perinatal mortality was also higher among diabetics than controls (12.5% vs. 3.5%) with stillbirth being the major contributor. Patients with gestational diabetes were at increased risk of fetal macrosomia than controls (28.7% vs. 5.5%). The overall cesarean section rate was high (36%) among diabetics with previous cesarean section and cephalopelvic disproportion being the commonest indications. CONCLUSIONS: Health education and provision of modern affordable methods of management of diagnosed cases such as uristix and hemastix will improve maternal and fetal outcome in pregnant diabetics in Africa.

Adolescent↗

Prenatal diagnosis of osteogenesis imperfecta type II.

OBJECTIVE: To characterize the prenatal sonographic features of osteogenesis imperfecta (OI) type II. DESIGN: Descriptive (case series). SETTING: Department of Obstetrics and Gynecology, Faculty of Medicine, Maharaj Nakorn Chiang Mai Hospital, Chiang Mai University. SUBJECTS: Six fetuses with prenatal diagnosis of OI were evaluated. RESULTS: Six fetuses were prenatally diagnosed as OI type II in five mothers without familial history of the disease. One mother had two consecutive pregnancies complicated with this condition. The first five cases were classified as OI type IIA, while the last one was OI type IIB. All of subtype A exhibited typical triad of bone shortening, diffuse hypomineralization and multiple fractures of long bones including beaded ribs whereas the subtype B showed shortening of only femurs, normal bone echodensity and isolated fractures of long bones. The postnatal radiography and autopsy confirmed the prenatal diagnosis in all cases. Other findings may occasionally be found, including polyhydramnios, oligohydramnios, hydrop fetalis and small for gestational age. CONCLUSION: The triad of bone shortening, decreased bone density and numerous fractures including beaded ribs permits a confident diagnosis of OI type IIA. Furthermore, sonographic features may differentiate the subtype of OI type II, depending on degree of bone shortening and echodensity.

Female↗

Management of diabetic pregnant patients in a tertiary center in the developing world.

OBJECTIVES: To audit our insulin regimen in our antenatal diabetics. METHODS: 170 pregnant diabetic patients were retrospectively analyzed. RESULTS: The perinatal mortality rate was 37/1000, vs. a perinatal mortality rate of 30/1000 in our setting. Cesarean section rate was 49%. Strict blood-glucose control reduced the incidence of macrosomia and polyhydramnios. Routine amniocentesis for fetal-lung maturity was not practiced. CONCLUSION: Satisfactory results in pregnant diabetic patients are achievable with an established insulin regimen in a developing country.

Adult↗

First trimester sonographic diagnosis of holoprosencephaly.

OBJECTIVE: To describe our experience with sonographic diagnosis of fetal holoprosencephaly in first trimester. SUBJECTS: A total of three fetuses with early prenatal diagnosis of holoprosencephaly were sonographically evaluated and followed up. RESULTS: The study revealed that all showed monoventricular cavity, fused thalami, no falx and cavum septum pellucidum. All of them were correctly diagnosed sonographically in the first trimester. Extracranial anomalies had also been identified in all three fetuses and all of them had facial abnormalities. Cytogenetic studies were successfully carried out in only one case. No polyhydramnios was demonstrated in all cases. CONCLUSION: This small series indicates that holoprosencephaly can be diagnosed in the first trimester. The most valuable clue to the diagnosis is the demonstration of the single ventricle.

Adult↗

Prenatal sonographic features of sacrococcygeal teratoma.

OBJECTIVE: To describe the potential role of ultrasound in prenatal diagnosis of fetal sacrococcygeal teratoma (SCT). SUBJECTS: A total of four fetuses with prenatal diagnosis of SCT were sonographically evaluated and followed-up. RESULTS: Fetal SCT was diagnosed at 13, 17, 26 and 27 weeks of gestation. Three of them had predominantly solid tumors with scattered cystic areas, whereas the other one had an entirely cystic tumor. One had a rapid growth tumor and finally developed early hydrops. Three had polyhydramnios and delivered prematurely. The diagnosis was posnatally confirmed in all cases. In the case with the large tumor, a cesarean section was done to avoid traumatic delivery. Two cases survived and the tumors were successfully resected, whereas the others died due to heart failure secondary to hydrops in one case and prematurity in the other one. CONCLUSION: This small series suggests that SCT be readily diagnosed in utero, possibly as early as first trimester. The important clue was an abnormal mass in the sacrococcygeal area. The prenatal diagnosis can contribute to changes in clinical decision.

Female↗

Pyloric atresia: 15-year review from a single institution.

BACKGROUND: Pyloric atresia (PA) is a rare pathology. Calder presented the first pyloric atresia case in 1749 and Touroff, Sussman, Meltz, and their colleagues presented the first successful operation in 1940. PA has 3 types of anatomic variations: (1) type A, pyloric membrane or web; (2) type B, the pyloric channel is a solid cord; and (3) type C, in which there is a gap between the stomach and duodenum. Associated anomalies also have been described. Epidermolysis bullosa (EB) and intestinal anomalies occur most often with this condition. METHODS: Charts of 16 cases of congenital PA, aged 1 to 30 days and admitted to our department between 1986 and 2001, were studied retrospectively in regard to sex, prenatal diagnosis, presence of polyhydramnios, time of admission, pathology, type of operation, associated anomalies, and mortality rate. RESULTS: In the study group, the male to female ratio was 5:3, the mean birth weight was 2,312 g and the mean age of admission was 6.5 days. The distribution of the anatomic variations was type A in 9 (56.3%) and type B in 7 (43.7%) of cases. Associated anomalies were present in 7 cases (43.8%). Familial occurrence was a prominent feature of our series. Seven of 16 cases occurred in 3 families. We performed 9 web excisions together with Heineke Mikulicz (H-M) pyloroplasty, 5 atresia excisions and gastro-duodenostomy, and 2 H-M pyloroplasties alone. Stamm gastrostomy was supplemented in 3 cases. The overall mortality rate (n = 9) was 56.3%. CONCLUSIONS: Pyloric atresia can be managed successfully if it is diagnosed early. In this group of patients, congenital anomalies or septicemia are the main causes of mortality.

Abnormalities, Multiple↗

Prenatal management of congenital cystic adenomatoid malformation of the lung.

In utero evolution and postnatal outcome were studied for 18 cases of congenital adenomatoid malformation (CAM) diagnosed by prenatal ultrasound. Five were macrocystic CAM, 9 were microcystic, and 4 were homogeneously hyperechogenic. Three fetuses presented with hydrops. Pulmonary amniotic shunting was performed in 3 patients. Outcome was the following: 4 were aborted, 1 died neonatally, and 13 survived. Four of these infants required no surgery in the neonatal or postneonatal period. In three of these, the size of the mass had decreased spontaneously in utero. Outcome did not appear to be related to the anatomic type of CAM nor to the presence of moderate polyhydramnios, but was related to the degree of mediastinal compression and to the existence of hydrops. A clearer understanding of the natural evolution of CAM is useful to determine the indications for in utero therapy.

Cystic Adenomatoid Malformation of Lung, Congenita↗