Monochorionic triplets after single embryo transfer

A 40-year-old patient underwent intracytoplasmic sperm injection and assisted hatching, and a single embryo was transferred. Ultrasonography demonstrated a single gestational sac containing monochorionic tri-amniotic pregnancy. Several factors that have been implicated in the aetiology of monozygotic triple pregnancies after IVF appear to be present in this case. To avoid multiple pregnancies after IVF, it is time to have definite predictive factors for the occurrence of monozygotic multiple pregnancies as well as transferring only a single embryo.     

Newborn outcome after assisted reproductive technology: experiences and reflections from Poland

In spite of much ethical uncertainty and wide variations in obstetric and neonatal outcomes reported in the literature, we have studied the outcome of Polish newborns after assisted reproductive technology (ART). In all of our patients, both obstetrics and neonatal care were performed in the Level Three Center in Poznan, Poland. A total of 45 and 82 newborns were analyzed from the ART and the control group, respectively. We analyzed gestational age, birth weight, Apgar score, duration of hospitalization, newborn mortality, and congenital malformations. We found a far higher rate of mothers over 35 years and with university education in ART compared to controls. The incidence of cesarean section and birth weight <1,500 g was much higher in ART. Newborn mortality was much higher among ART newborns compared to controls and was related to premature delivery of triplet pregnancy. The most important factor resulting in high neonatal mortality in our study group was multiple pregnancy. We consider the lack of regulation in Poland concerning the number of replaced embryos as a main factor influencing newborns' outcome after ART.     

Hypothalamic adipic hypernatraemia syndrome with normal osmoregulation of vasopressin

Adipsic hypernatraemia is an uncommon disorder in childhood caused by a defect in the osmoregulation of thirst, leading to impairment of water homeostasis and chronic hyperosmolality of body fluids. Adipsia is often associated with an abnormality in osmoregulated vasopressin secretion due to the close proximity of the hypothalamic osmoreceptors that control thirst with those regulating vasopressin secretion. Hypothalamic lesions of diverse aetiology (vascular abnormalities, neoplasms, granulomatous diseases, trauma etc.) have been described in this syndrome. We report a 12-year-old boy with evident weight loss due to hypernatraemic dehydration with a selective defect in osmoregulation of thirst and normal vasopressin secretion with no demonstrable structural lesion. To date, only six paediatric patients with this condition have been described in the literature. Conclusion:hypothalamic adipsic hypernatraemia syndrome must be suspected when a dehydrated patient denies thirst. The study of antidiuretic function is necessary because the osmoregulation of vasopressin secretion could be altered.Abbreviations ADH antidiuretic hormone - AVP arginine vasopressin - Posm plasma osmolality     

Engraftment syndrome emerges as the main cause of transplant-related mortality in pediatric patients receiving autologous peripheral blood progenitor cell transplantation

The authors examined data from 166 children who received autologous peripheral blood progenitor cell (PBPC) transplantation to ascertain the incidence of early transplant-related mortality (TRM) and the contributing risk factors. Eleven patients (6.6%) (6 boys, 5 girls) died within 180 days following PBPC infusion. The median age was 4 years (range 2-17). The overall probability of TRM was 6.9 +/- 2% at day +180. On univariate analysis, the status of disease at transplantation (complete remission vs. not in complete remission) was identified as the only pretransplant significant predicting factor for TRM (14% of patients who were not in complete remission died within 180 days after PBPC infusion, whereas only 2% of patients in complete remission died) (relative risk [RR] 1.13, 95% confidence interval [CI] 1.01-1.26, P = 0.01). Age, gender, conditioning, and number of CD34+ cells infused were not significantly associated with TRM. In the postinfusion phase, patients who developed multiorgan dysfunction during the neutropenic period, especially when the lung was the first failing organ (RR 16.1, 95% CI 7.16-36.18, P = 0.0001), and those with engraftment syndrome (RR 2.81, 95% CI 1.49-5.24, P = 0.001) had an increased risk for TRM. On multivariate analysis, development of engraftment syndrome was the only significant variable that influenced TRM. In conclusion, the authors found for the first time that engraftment syndrome emerges as the main cause of TRM after autologous PBPC transplantation in children with malignancies.     

Squamous cell carcinoma of the cervix presenting as lymphangitic carcinomatosis: a case report and review of the literature

BACKGROUND: Lymphangitic carcinomatosis (LC) secondary to carcinoma of the cervix is rare. The presenting symptoms are misleading and nonspecific, which often leads to delayed diagnosis. CASE: We present the case of a 24-year-old woman with dyspnea and lower back pain, who was found to have stage IIIb squamous cell carcinoma of the cervix complicated by pulmonary LC. CONCLUSIONS: Pulmonary LC is a rare but important manifestation of metastatic cervical cancer. Patients present with severe respiratory compromise, which mimics other, more common disease states. Diagnosis can be achieved by transbronchial biopsy. Optimal treatment of the patient with LC is not well defined, but a trial of chemotherapy and/or intravenous steroids may be warranted. Prognosis for this condition is poor.     

Roles of substrate availability and infection of resting and activated CD4+ T cells in transmission and acute simian immunodeficiency virus infection

In studies of sexual mucosal transmission and early stages of simian immunodeficiency virus (SIV) and HIV infections, productive infection predominates in CD4⁺ T cell populations, in both ostensibly resting and activated cells. The surprising ability of SIV and HIV to replicate in resting cells in vivo, in contrast to propagation of infection in vitro, suggested a model in which during the early stages of infection these viruses exploit the greater availability of resting cells to maintain unbroken chains of transmission from an infected resting cell to another resting cell nearby. Because immune activation in response to infection provides more activated CD4⁺ T cells, these viruses take advantage of the greater efficiency of virus production and spread in activated cells for propagation and dissemination of infection. In this article, we report the results of experimental tests of this model, including visualization at the light microscopic level and direct analysis of virus production by cells in tissues. Analysis of tissues of rhesus macaques inoculated intravaginally or i.v. with SIV supports the proposed roles of target cell availability, susceptibility, and virus production by infected resting and activated CD4⁺ T cells in mucosal transmission and early infection, and points to a potential role for topical anti-inflammatory agents in moderating the initial propagation of infection.     

Advances in preconception genetic counseling

Preconception counseling offers couples contemplating pregnancy the opportunity to identify and reduce potential pregnancy risks, plan care for known risks, and establish early prenatal care. The goal of preconception counseling is to promote physically and emotionally healthy parents in an attempt to ensure optimal prenatal, intrapartum, and postpartum maternal and fetal health. The advantages of addressing these issues prior to conception include the opportunity of giving recurrence or occurrence risk information for informed decision regarding pregnancy, the accessibility of more reproductive options such as preimplantation genetic diagnosis, and the opportunity to make lifestyle, medical, and behavior alterations for optimal maternal and fetal outcome. This article will discuss advances and updates in preconception genetic counseling including risk assessment and potential intervention for concerns identified through evaluation of family history, ethnicity, maternal age, medical and obstetric history, and associated psychosocial and nursing implications. Significant advances have emerged in the areas of cystic fibrosis carrier screening, first trimester screening, preimplantation genetic diagnosis, and detection and treatment of maternal thrombophilia.     

Therapeutic idiotype vaccines in B lymphoproliferative diseases

The recognition of the surface immunoglobulin protein of the tumour B cells as a specific tumour antigen has prompted the development of vaccination strategies aimed at the induction of humoral and cellular antitumour responses. Results obtained in preclinical models of B lymphoproliferative diseases, as well as in initial clinical trials, have shown the immunogenic potential of the idiotype (Id) when administered in association with proper adjuvants. The definitive evidence for clinical efficacy of this therapeutic approach awaits ongoing randomised Phase III studies. Research efforts at present include identification of new vaccination settings to improve the clinical benefit of vaccine treatment, the establishment of more convenient methods to produce individual Id vaccines and the development of new strategies of vaccination, including genetic vaccination.     

Surgical management of primary hyperparathyroidism: the case for giving up quick intraoperative PTH assay in favor of routine PTH measurement the morning after

OBJECTIVE: To analyze the utility of quick intraoperative parathyroid hormone (PTH) measurement in the surgical management of primary hyperparathyroidism. BACKGROUND DATA: The use of intraoperative PTH monitoring is well established in the surgery of primary hyperparathyroidism. However, some false-negative predictions lead to unnecessary explorations; furthermore, surgeons are becoming increasingly dependent on hormone measurement for intraoperative decisions, which raises concerns about the cost-effectiveness of the method. METHODS: A retrospective analysis of 268 neck explorations performed for primary hyperparathyroidism using intraoperative PTH monitoring from April 2001 to February 2003 was done. We used the criterion of "biologic recovery" of hyperfunctioning tissue, defined as a more than 50% decrease in PTH level from baseline value at 5 minutes after excision to predict the outcome of successful parathyroidectomy documented by normal postoperative serum calcium level. Additionally, we also sampled PTH at 10 minutes, 30 minutes, and the morning after surgery to compare the predictive value of delayed sampling. Patients were classified according to the prediction being concordant or discordant with the outcome. The data were analyzed using a 2 x 2 table construct for each of the sampling times, therefore providing sequential sensitivity, specificity, positive and negative predictive values, and overall accuracy of the predictions. RESULTS: Concordance or overall accuracy of prediction (true positives and negatives) was obtained in 229 cases (85.4%), and discordance or failure of prediction (false positives and negatives) was obtained in 34 cases (12.7%) at T5. On analyzing the iPTH prediction at T10, T30, and D1 among the group of 33 false negatives, we found that 28 (10.4%) patients reached the concordance at 30 minutes, while by the first day 32 patients (12.3%) had achieved concordance. Thus, there was a progressive increase in sensitivity and overall accuracy, but more importantly, in the negative predictive value reaching 88.9% on the day after surgery. CONCLUSIONS: The method of sampling PTH intraoperatively at 5 minutes has a high positive predictive value (99.5%) but a low negative predictive value (19.5%), which can lead to unnecessary explorations and a delay in the operative procedure. The negative predictive value increases substantially at 30 minutes and is best on the day after surgery. We suggest giving up the intraoperative measurement of PTH to adopt the first day postoperative measurement of PTH as a predictor of successful parathyroidectomy.     

Endovascular treatment of a giant intracranial aneurysm with a stent-graft

This report describes a giant intracavernous carotid aneurysm successfully treated by the placement of a single covered stent. A 40-year-old woman was admitted with a progressive diplopia in relation with palsy of the IV and VI cranial nerves. Magnetic resonance imaging revealed an intracavernous giant aneurysm located at the bifurcation between the origin of a trigeminal artery and the intracavernous portion of the right internal carotid artery. A covered stent was successfully placed, and complete exclusion of the aneurysm was confirmed at 11-month follow-up angiography. The use of covered stents in intracranial vascular structures can now be a feasible way of treating selected cases of wide-necked intracranial aneurysms.