1/62. Hormonal dependency in darier disease.Variation in the severity of keratosis follicularis relative to states of homonal change was noted in eight women with the disease. Observations included frequent onset near puberty, puberty, exacerbation during menses, and improvement while patients are pregnant or on oral contraceptive agents.It is proposed that states of relative estrogen excess are associated with quiescence of this skin disorder. In support of this hypothesis, three postpubertal women with Darier desease were observed to undergo significant improvement with the use of estrogen-dominant birth control pills.- - - - - - - - - - ranking = 1keywords = near (Clic here for more details about this article) |
2/62. chondrodysplasia punctata stemming from maternal lupus erythematosus.The finding of stippled epiphyses on a neonatal radiograph generates a wide differential diagnosis, including genetic and teratogenic causes. We report the case of a male infant with stippled epiphyses evident on neonatal radiographs in whom a typical rash of lupus erythematosus developed. The skin abnormalities in the infant resulted in a diagnosis of systemic lupus erythematosus in his mother. Over a 3-year follow-up period, the child has demonstrated strikingly short stature, midface hypoplasia, anomalous digital development, slow resolution of the stippled epiphyses, and near normal cognitive development. The differential diagnosis of chondrodysplasia punctata and the literature supporting maternal lupus as one cause are reviewed.- - - - - - - - - - ranking = 1keywords = near (Clic here for more details about this article) |
3/62. Outcome of a baby born from a mother with acquired juvenile hypothyroidism having undetectable thyroid hormone concentrations.We report a baby born from a mother with strongly positive thyroid stimulation blocking antibody (TSBAB) and nearly undetectable T4 level. This case is a unique model of nearly complete absence of thyroid hormones during fetal and early neonatal life in humans. The infant girl was born by cesarean section, because of fetal bradycardia, after 41 weeks gestation and received mechanical ventilation for 3 days. The TSH level was more than 120 microU/mL in the neonatal thyroid screening. At age 17 days, the results of a thyroid function study showed undetectable free T3 and free T4 concentrations, TSH 550 microU/mL, and TSH receptor antibody (TRAB) 87%. thyroxine at a dose of 30 microg/day was started at age 17 days. The patient required thyroxine treatment until age 8 months. The brain magnetic resonance image at age 2 months revealed reduced brain size. Her auditory brain stem response was absent at age 2 months. The audiogram at age 4 yr revealed sensorineural deafness of 70 dB. When she was 6 yr of age, motor development remained the same as that at age 4 months. Her height was 106 cm (- 1.5 SD). The results of thyroid function study of the mother 23 days after delivery showed undetectable free T3 and free T4, TRAB 84%, and TSBAB 83%. In conclusion, the outcome of severe thyroid hormone deficiency in utero and early in human neonatal life was normal physical growth, fetal distress resulting in cesarean section, difficulty in the onset of breathing, permanent deficit in auditory function, brain atrophy, and severely impaired neuromotor development despite the start of an adequate dose of thyroxine replacement during the neonatal period.- - - - - - - - - - ranking = 2keywords = near (Clic here for more details about this article) |
4/62. Suicidal paracetamol poisoning of a pregnant woman just before a delivery.The aim of the paper is to present a case of self-poisoning with paracetamol, overdosed just before a delivery. A 21-year-old woman was admitted to Obstetric and gynecology Ward of local hospital in the second stage of physiological delivery, more than 6 hours after she had ingested 19 g of acetaminophen for self-poisoning. She delivered a normal infant weighing 3520 g who had Apgar scores of 10, and then both infant and mother were sent in an emergency ambulance to the nearest poison centre. blood samples for toxicological examination were taken on admission to toxicological intensive care unit i.e. 11 hours post maternal ingestion. acetaminophen levels of both patients were above the acetaminophen overdose nomogram line and the antidote treatment, i.v. N-acetylcysteine was administered according to the protocol: the mother within 11 hours post-ingestion and approximately 4 hours after a delivery; the neonate within 11 hours post maternal ingestion and 4 hours of life. Higher paracetamol concentration in the blood of infant compared to the mother's was noted in the first and then control toxicological examination performed within 35 hours post maternal ingestion. Peak maternal aspartate aminotransferase (AST) activity was 326 U/L within 35 hours and alanine aminotransferase (ALT) activity was 262 U/L within 56 hours post-ingestion. The highest neonatal enzyme activity was noted within 11 hours post maternal ingestion of paracetamol, and the elevation was not high. Except moderate anaemia in the mother, no clinical or biochemical symptoms of renal, cardiovascular or CNS injury were stated in the mother or infant. Normalisation in the maternal enzymes activity was stated within 226 hours, while in the neonatal within 58 hours post maternal ingestion. The woman recovered without sequelae and was discharged from hospital on the 11th day following paracetamol overdosing. No evidence of the liver injury was found in the infant either.- - - - - - - - - - ranking = 1keywords = near (Clic here for more details about this article) |
5/62. Anesthetic management for cesarean section in a pregnant woman with impending acute liver failure--a case report.Acute liver failure includes a constellation of clinical conditions resulting from rapid loss of hepatocyte function. The anesthesiologist may often be asked to anesthetize a patient with acute liver failure before the etiological entity of disease can be completely clarified. Most of the previous reports were concentrated in obstetric or hepatological periodicals. Reports about anesthesia for such patients are scanty. We present a case about a near term pregnant woman with a complication diagnosed as impending acute liver failure, who received total intravenous anesthesia for emergent cesarean section. Pros and cons of different anesthetic techniques for this condition are discussed.- - - - - - - - - - ranking = 1keywords = near (Clic here for more details about this article) |
6/62. Gestational thyrotoxicosis with acute wernicke encephalopathy: a case report.A 35-year-old hyperthyroid woman who developed nausea, vomiting, tachycardia, nystagmus and mental disturbance, was referred to our hospital with a suspected diagnosis of thyroid storm. However, the thyroid gland was only slightly palpable, bruits were not audible, and exophthalmos was not present. serum levels of thyroid hormone were increased, but TSH receptor antibodies were negative. Echography and color flow doppler ultrasonography revealed a slightly enlarged thyroid gland and a slightly increased blood flow, both of which were much less milder than those expected for severe hyperthyroid Graves' disease. Under the diagnosis of hyperthyroidism due to gestational thyrotoxicosis associated with wernicke encephalopathy, vitamin B1 was administered on the first day of admission. Her consciousness became nearly normal on the second day except for slight amnesia. Her right abducent nerve palsy rapidly improved, but horizontal and vertical nystagmus, diminished deep tendon reflexes and gait ataxia improved only gradually. MRI findings of the brain were compatible with acute wernicke encephalopathy. We concluded that history taking and physical findings are important to make a differential diagnosis of gestational thyrotoxicosis with acute wernicke encephalopathy from Graves' thyroid storm, and that wernicke encephalopathy should be treated as soon as possible to improve the prognosis.- - - - - - - - - - ranking = 1keywords = near (Clic here for more details about this article) |
7/62. Anaesthetic management of a parturient with a colloid cyst of the third ventricle.A colloid cyst in the third ventricle near the foramen of Monroe can obstruct cerebrospinal fluid (CSF) flow from the lateral ventricles. Any change in the CSF pressure on either side of the cyst can lead to displacement and thus precipitate acute hydrocephalus. Management of the confinement of a patient with a colloid cyst must therefore aim to minimize changes in CSF pressure. We describe our management of a patient with a small colloid cyst who was permitted to labour with the assistance of patient-controlled epidural analgesia. The available alternatives are discussed.- - - - - - - - - - ranking = 1keywords = near (Clic here for more details about this article) |
8/62. Acardiac twin with preserved brain.The fatal acardiac syndrome is a rare complication of monochorionic twinning and is reported in 1 of 35,000 births. It is caused by arterioarterial and venovenous placental anastomoses leading to circulatory predominance of 1 twin. The donor 'pump' twin provides circulation for itself and for the recipient acardiac twin. The acardiac twin is usually grossly abnormal with severe reduction anomalies of the upper part of the body. We report a twin pregnancy, where a recipient twin initially by ultrasound was misdiagnosed as dead. In the third trimester the supposedly dead twin presented as an edematous acardiac twin without peripheral reduction defects and a nearly normally developed brain. An acardiac twin with a nearly normal external appearance and an almost normally developed brain, nourished by a surviving twin brother, has not previously been described in the literature.- - - - - - - - - - ranking = 2keywords = near (Clic here for more details about this article) |
9/62. Management of respiratory deterioration in a pregnant patient with severe kyphoscoliosis by non-invasive positive pressure ventilation.The problem of kyphoscoliosis in combination with pregnancy is uncommon and published cases are rare. Until now, little and controversial information on the outcome, optimal management and course of pregnancy in patients with kyphoscoliosis has been available. The majority of maternal deaths seem to be attributed to cardiorespiratory failure, while obstetric complications account for relatively few complications. We present the case of a 34-year old pregnant woman with congenital kyphoscoliosis and a forced vital capacity (FVC) of about one liter. A further deterioration of lung function was expected. In fact, severe limitations in exercise capacity (bed rest), fatigue and hypersomnolence, as well as a severe increase in pulmonary hypertension occurred during the second and third trimester. Nasal intermittent positive pressure ventilation (NIP-PV) with bilevel positive airway pressure (BiPAP) was started in the 20th week of gestation and adapted throughout pregnancy. Nasal BiPAP was well-tolerated and corrected exercise tolerance, fatigue and nocturnal oxygen desaturations. At 32 weeks of gestation, the patient was admitted for an elective Caesarean section under combined spinal-epidural anaesthesia with ongoing NIPPV, and delivered a healthy baby. Home nocturnal ventilatory support was continued as nocturnal episodic desaturations were also assessed during the postpartum period. At time of discharge, the patient's exercise capacity and lung function were nearly equal to levels before pregnancy. We conclude that pregnancy in selected kyphoscoliotic patients with severe limitations in lung function is relatively safe for both the mother and the child when NIPPV is used for overcoming respiratory deterioration and for preventing further cardiorespiratory failure.- - - - - - - - - - ranking = 1keywords = near (Clic here for more details about this article) |
10/62. Linear scleroderma "en coup de sabre" coexisting with plaque-morphea: neuroradiological manifestation and response to corticosteroids.A 24 year old woman in the 33rd week of pregnancy developed progressive neurological complications with right sided hemiparesis in association with the occurrence of linear scleroderma "en coup de sabre" (LSCS) and pre-existing plaque-morphea, already being treated by balneophototherapy. Further progression of neurological symptoms led to a caesarean section with the delivery of a healthy child. Brain magnetic resonance imaging (MRI) showed focal T2 signal increases in the left frontoparietal region directly adjacent to the area of LSCS. cerebrospinal fluid analysis revealed oligoclonal bands, suggesting an intracerebral inflammatory process. Subsequent pulsed corticosteroid treatment led to a remission of neurological symptoms and to a marked resolution of the MRI lesions.- - - - - - - - - - ranking = 5keywords = near (Clic here for more details about this article) |
| | Next -> |