Pregnancy in a patient with congenital analbuminaemia.

Congenital analbuminaemia is a rare autosomal recessive disorder that is characterised by a severe reduction or total absence of serum albumin. This condition has implications for therapeutics as a large proportion of commonly used drugs are plasma protein bound where albumin is the primary component of plasma protein. This is the first case report of pregnancy in a patient with congenital analbuminaemia in the medical literature. In the absence of drug dosage guidelines for patients with congenital analbuminaemia, a list of drugs which may be required for this patient during pregnancy, delivery and/or emergency situations were compiled by a multidisciplinary team. Our patient suffered from polyhydramnios during her pregnancy which was successfully managed with albumin transfusions and had a normal vaginal delivery with no complications in the intrapartum or postpartum period. The management and unique challenges of pregnancy in a patient with congenital analbuminaemia are discussed.

Chinese herbal medicine in treatment of polyhydramnios:a meta-analysis and systematic review.

OBJECTIVE: To compare the efficacy of Chinese herbal medicine (therapy A) or Chinese herbal medicine plus indomethacin (therapy B) with that of indomethacin alone (therapy C) in treating polyhydramnios. METHODS: Literatures published up to April 2012 were retrieved from PubMed, Embase and Cochrane library, Chinese National Knowledge Infrastructure (CNKI), Chinese Scientific and Technological Periodical Database (VIP), Wangfang, and Traditional Chinese Medicine online. Two researchers collected data independently. The assessment of methodological quality was based on Cochrane handbook and the materials were analyzed with software RevMan 5.1.2. The outcome measure index was relative risk or difference of mean value (95% confidence interval). The following outcomes were evaluated (1) general clinical improvement rate; (2) maximum vertical pocket depth; (3) amniotic fluid index (AFI) value; (4) rate of fetal ductus arteriosus constriction; (5) incidence of adverse events. RESULTS: Based on the search strategy, 5 trails involving 1017 patients were finally included. Compared with therapy C, therapy A decreased the rate of fetal ductus arteriosus constriction (P<0.01). Therapy B was more effective than therapy C in general clinical improvement and decrease of AFI for polyhydramnios (P<0.01). No serious adverse events were reported in therapy A and therapy B. CONCLUSIONS: Compared with therapy C, therapy A and therapy B may appear to be more effective for polyhydramnios. However, the exact effect needs to be confirmed with well-designed large-scale clinical trials.

Meal related glucose monitoring is a method of diagnosing glucose intolerance in pregnancies with high probability of gestational diabetes but normal glucose tolerance by oral glucose tolerance test.

Gestational diabetes mellitus diagnosed by classical oral glucose tolerance test can result in fetal complications like macrosomia and polyhydramnios. Guidelines exist on management of patients diagnose by abnormal oral glucose tolerance test with diet modification followed by insulin. Even patients with abnormal oral glucose tolerance test maintaining apparently normal blood sugars with diet are advised insulin if there is accelerated fetal growth. But patients with normal oral glucose tolerance test can present with macrosomia and polyhydramnios. These patients are labelled as not having gestational diabetes mellitus and are followed up with repeat oral glucose tolerance test. We hypothesise that these patients may have an altered placental threshold to glucose or abnormal sensitivity of fetal tissues to glucose. Meal related glucose monitoring in these patients can identify minor abnormalities in glucose disturbance and should be treated to targets similar to physiological levels of glucose in non pregnant adults.

Indomethacin in pregnancy: applications and safety.

Preterm labor (PTL) is a major cause of neonatal morbidity and mortality worldwide. Among the available tocolytics, indomethacin, a prostaglandin synthetase inhibitor, has been in use since the 1970s. Recent studies have suggested that prostaglandin synthetase inhibitors are superior to other tocolytics in delaying delivery for 48 hours and 7 days. However, increased neonatal complications including oligohydramnios, renal failure, necrotizing enterocolitis, intraventricular hemorrhage, and closure of the patent ductus arteriosus have been reported with the use of indomethacin. Indomethacin has been also used in women with short cervices as well as in those with idiopathic polyhydramnios. This article describes the mechanism of action of indomethacin and its clinical applications as a tocolytic agent in women with PTL and cerclage and its use in the context of polyhydramnios. The fetal and neonatal side effects of this drug are also summarized and guidelines for its use are proposed.

Prenatal pharmacotherapy for fetal anomalies: a 2011 update.

Fetal therapy can be defined as any prenatal treatment administered to the mother with the primary indication to improve perinatal or long-term outcomes for the fetus or newborn. This review provides an update of the pharmacological therapies that are solely directed at the fetus with anomalies and outlines a future transcriptomic approach. Fetal anomalies targeted with prenatal pharmacotherapy are a heterogeneous group of structural, endocrine, and metabolic conditions, including congenital cystic adenomatoid malformation (CCAM), congenital adrenal hyperplasia, congenital heart block, fetal tachyarrhythmias, inborn errors of metabolism, fetal thyroid disorders, and polyhydramnios. To date, the majority of pharmacotherapies for fetal anomalies have been evaluated only in retrospective, uncontrolled studies. The way forward will be with an evidence-based approach to prenatal pharmacological interventions.

Neuroblastoma presented with polyhydramniosis.

Neuroblastoma is the most common extracranial solid tumor in pediatric age group. Clinical presentation of neuroblastoma is mostly related to origin of the tumor, extent of disease, and the presence of paraneoplastic syndromes. Here the authors report a neonate with neuroblastoma with an atypical presentation, diagnosed with postnatal abdominal ultrasonography performed due to polyhydramniosis in her mother during pregnancy.

Indomethacin-induced colon perforation in Bartter's syndrome.

Bartter's syndrome (BS) is an inherited renal tubular disorder characterized by hypokalemia, hypochloremic metabolic alkalosis, and hyperaldosteronism with normal blood pressure. A 22-year-old woman was referred at 23 week of gestation. Polyhydramnios was detected and the chloride level of the amniotic fluid was high. The mother was treated with indomethacin from 26 to 31 week of gestation. The newborn was delivered at 34 week of gestation. At 8th day of life, indomethacin was also started for the baby. After three days, a colonic perforation developed. Indomethacin-induced colon perforation is uncommon in antenatal Bartter's syndrome. This patient indicates that administration of indomethacin in both antenatal and/or early postnatal period may be associated with colonic perforation.

Acute recurrent polyhydramnios: a combination of amniocenteses and NSAID may be curative rather than palliative.

Acute recurrent polyhydramnios is a rare occurrence characterized by a poor fetal outcome. This is a case report describing a 34-year-old woman presenting with acute recurrent polyhydramnios. Treatment with non-steroidal anti-inflammatory drugs (NSAID) and therapeutic amniocenteses was initiated immediately and resulted in a decreased amniotic fluid production from 30 weeks' gestation. Even after the discontinuation of NSAID treatment, the amniotic fluid production normalized, and the woman delivered a healthy boy at 39 weeks 2 days' gestation. Amniotic prolactin was measured at three occasions using an enzyme-linked immunosorbent assay. As in normal pregnancies, amniotic prolactin levels decreased by 80% from highest to lowest value in this case of resolving acute recurrent polyhydramnios.

Alcohol injection for the intrauterine treatment of chorioangioma in a pregnancy with transfusion resistant fetal anemia: a case report.

Chorioangiomas are usually small, clinically inevident, benign vascular lesions of the placenta, but larger ones may cause serious perinatal and neonatal complications. There is need for in utero intervention in these fetuses. Several interventions are described to relieve pathophysiologic insult on fetus. Alcohol injection is one of the therapeutic interventions. In this case, we present a patient with chorioangioma and early onset of hydrops fetalis and polyhydramnios at 24th week of gestation. Successful intratumoral injection of absolute ethyl alcohol relieved hydrops fetalis and polyhydramnios. Unfortunately, pregnancy ended at 28th week and a 1,330 g fetus was delivered.

Premature closure of the ductus arteriosus: variable response among monozygotic twins after in utero exposure to indomethacin.

Exposure of the fetus to indomethacin by administration of the drug to the mother may cause many side effects, including premature closure of the ductus arteriosus. Hypoxia is a predisposing factor for premature ductal closure and often occurs after maternal indomethacin therapy. We present two sets of monozygotic twins with twin-to-twin transfusion, where in utero ductal closure occurred in the donor twin while the recipient twin appeared unaffected. This selective closure of the ductus arteriosus suggests that the affected twin was predisposed to hypoxia and thus was more susceptible to ductal closure in response to indomethacin exposure.

[The therapeutic role of tocolysis]. / Tocolyse dans les situations pathologiques particulières.

In twin pregnancies, the use of beta-adrenergics is associated with a significantly higher incidence of cardiovascular complications, and calcium channel blockers as well as oxytocin antagonists currently appear as first line agents. After extreme preterm delivery of the first twin and in selected patients, the birth of second twin may be delayed with a mean gain of 10-50 days. In cases of symptomatic placenta previa with mild-to-moderate bleeding, tocolytic agents may be associated with a prolongation of pregnancy and increased birth weight without significant impact on frequency or severity of bleeding. Calcium channel blockers are the drugs of choice in the event of diabetes. Indomethacin is a potent tocolytic, in particular in patients with polyhydramnios. However, it may cause oligohydramnios, premature closure of the ductus arteriosus and intrauterine fetal death when high doses are administered for a duration exceeding 48 to 72 hours, particularly beyond 32 weeks' gestation. The neonatal complications of indomethacin occur frequently. Tocolysis appears to reduce the failure rate of external cephalic version at term.

[Prenatal Bartter's syndrome. Report of two cases]. / Le syndrome de Bartter anténatal. A propos de deux cas.

Antenatal Bartter Syndrome (ABS) is a rare autosomic recessive tubulopathy characterized by idiopathic hydramnios, fetal polyuria and elevated levels of amniotic chloride. It is related to mutations affecting several transporters in the loop of Henle e.g. the Na-K-2Cl cotransporter, the chloride channel CLC-NKB and the potassium channel ROMK. We report two cases of ABS in siblings born to consanguineous parents (first cousins). The first pregnancy showed hydramnios of unknown etiology at week 23. Two amnio drains were performed at weeks 26 and 27. The baby was born in week 29 and developed polyuria with hyponatremia, hypokalemia and hyperaldosteronism. After eliminating diabetes insipidus and adrenal insufficiency, ABS was diagnosed. The baby was treated with 0.5 mg/kg/d indomethacine, which controlled the polyuria and the hydroelectrolytic disorder. The second pregnancy showed idiopathic hydramnios at week 24. The elevated amniotic chloride level (above 112 mmole/l) led to the antenatal diagnosis of ABS. The mother was treated with 1 mg/kg/d indomethacine until week 31 in order to stabilize the hydramnios. Two amnio drains at weeks 31 and 33 allowed the pregnancy to be prolonged until week 34. A genetic study of the family showed homozygosity of the NKCC2 gene marker suggesting its implication in the disease.

Indomethacin therapy in the treatment of polyhydramnios due to placental chorioangioma.

A 26-year-old primigravida presented with acute polyhydramnios at 30 weeks gestation. Ultrasonography revealed a large placental chorioangioma with severe hydramnios. No anomalies were detected in the fetus. Preterm labor started with respiratory distress and indomethacin, 25 mg was given every 6 hours. The patient showed a good response with improvement of the hydramnios and respiratory symptoms. A normal infant with no neonatal complications was delivered 3 weeks later.

Renal tubular dysgenesis-like lesions and hypocalvaria. Report of two cases involving indomethacin.

We describe a case of twins with twin-to-twin transfusion syndrome (TTS) who were found to have renal tubular dysgenesis (TRD)-like lesions and hypocalvaria attributed to indomethacin treatment of the mother for acute polyhydramnios. History of pregnancy, postnatal clinical course, pathological findings of the kidneys, and the skulls are presented and discussed. These findings include incompletely differentiated proximal tubules in the kidneys and hypoplastic calvaria in both twins. The renal tubular lesions were more marked in the donor than in the transfused twin, probably due to the greater degree of ischemia in that twin. This seems to be in favor of a vascular etiology of the renal defects. However, the fact that similar renal lesions and hypocalvaria were also present in the transfused twin seems to indicate that indomethacin played a role in their onset. This so-called kidney-skull connection has never been reported in conjunction with indomethacin therapy.

Indomethacin therapy in hydramnios.

AIM: The use of indomethacin in treatment of hydramnios was evaluated. SUBJECTS & METHODS: Twelve patients with symptomatic hydramnios were treated with indomethacin (2.2- 3.0 mg/kg body weight/day). RESULTS: The treatment was started at a gestational age of 31.17-/+7.94 weeks and continued for 3.74-/+2.3 weeks. Eleven patients responded to the therapy both subjectively and objectively and pregnancies were prolonged by 4.6-/+3.1 weeks (range 0.1-10 weeks). Five women had term deliveries. Six patients had a favourable perinatal outcome. Four patients who had a known congenital anomaly in the foetus, delivered stillborn babies or had an early neonatal death. One patient who did not follow up after commencing therapy delivered a full-term stillbirth. One patient delivered within 1 day of starting therapy. Indomethacin therapy caused no maternal complications. CONCLUSION: Indomethacin was effective in the management of hydramnios and preventing it's complications.