Assessment and management of itchy skin in pregnancy.

BACKGROUND: Women with rashes or itchy skin during pregnancy will often present initially to the general practitioner. Knowledge of the specific dermatoses of pregnancy will assist in diagnosis, management and, importantly, facilitation of timely escalation of care of conditions that can potentially affect the fetus. OBJECTIVE: The aim of this article is to provide a diagnostic framework for approaching a pruritic rash during pregnancy as well as a helpful summary of management of pregnancy-specific dermatoses. It will assist clinicians in the identification of specific dermatoses that pose fetal risks. DISCUSSION: In addition to considering non-pregnancy specific conditions when approaching pruritus or a pruritic rash in pregnancy, it is important that clinicians also consider pregnancy-specific dermatoses, which have been reclassified into four categories: polymorphic eruption of pregnancy, pemphigoid gestationis, intrahepatic cholestasis of pregnancy (ICP) and atopic eruption of pregnancy. Unlike the other dermatoses, ICP begins with pruritus, and skin changes are secondary. ICP and pemphigoid gestationis are associated with fetal risks such as prematurity and stillbirth.

Burden of illness of progressive familial intrahepatic cholestasis in the US, UK, France, and Germany: study rationale and protocol of the PICTURE study.

Background: Progressive familial intrahepatic cholestasis (PFIC) is an ultra-rare disease with a considerable burden on pediatric patients and their caregivers, impacting quality of life (QoL). The mortality rates highlight a significant need for efficacious treatments. Real-world data on associated costs and QoL are needed to gauge the potential impact of new pharmacological treatments.Methods: Clinical and socio-economic burden of PFIC on patients/caregivers, health systems, and society will be assessed. Patient/caregiver- and physician-level retrospective cross-sectional data will be collected from the US, UK, France, and Germany, for PFIC types 1, 2, 3.A representative sample of physicians will provide clinical and resource utilization information using an electronic Case Report Form (eCRF). Patient/caregiver surveys will collect socio-economic and QoL data, enabling assessment of PFIC impact on QoL. Mean costs (direct medical/non-medical, indirect) will be calculated.The study materials were reviewed by medical professionals and patient representatives and received ethical approval from the University of Chester.Discussion: The study aims to reveal the unmet medical need, disease burden, resource utilization, and costs of PFIC, to raise awareness with policymakers and healthcare professionals, and provide support for the patient/caregiver community. As novel PFIC therapies recently emerged, this study will yield quantifiable data for health technology assessments.

Progressive familial intrahepatic cholestasis and benign recurrent intrahepatic cholestasis: a review.

Progressive familial intrahepatic cholestasis (PFIC) and benign recurrent intrahepatic cholestasis (BRIC) are two rare autosomal recessive disorders, characterized by cholestasis. They are related to mutations in hepatocellular transport system genes involved in bile formation. The differentiation between PFIC and BRIC is based on phenotypic presentation: PFIC is a progressive disease, with evolution to end-stage liver disease. BRIC is characterized by intermittent recurrent cholestatic episodes, with irresistible pruritus, mostly without evident liver damage. Between symptomatic periods, patients are completely asymptomatic. In this article, a short overview of the aetiology, the clinical and diagnostic characteristics and the therapy of both PFIC and BRIC are given.

Magnetic resonance cholangiopancreatography in cholestatic diseases.

BACKGROUND/AIMS: To evaluate the usefulness of magnetic resonance cholangiopancreatography in cholestasis. METHODOLOGY: Sixty-seven patients with cholestasis underwent ultrasound and magnetic resonance cholangiopancreatography, subsequently. The results of ultrasound and magnetic resonance cholangiopancreatography were interpreted by two independent radiologists. RESULTS: According to ultrasonographic findings intra- and extrahepatic cholestasis were diagnosed in 62.7% and 37.3% of patients, respectively. Magnetic resonance cholangiopancreatography revealed intra- and extrahepatic cholestasis in 52.2% and 47.8% of patients at the same group, respectively (p=0.016, kappa=0.789). Endoscopic retrograde cholangiopancreatography was indicated in 37.3% of patients (22.4% diagnostic, 14.9% therapeutic) according to ultrasonographic findings. However, 41.8% of patients had indication for endoscopic retrograde cholangiopancreatography following magnetic resonance cholangiopancreatography in the same group. In 20% of patients who needed diagnostic endoscopic retrograde cholangiopancreatography according to ultrasonographic findings, endoscopic retrograde cholangiopancreatography wasn't indicated after magnetic resonance cholangiopancreatography. Similarly, 8.9% of patients who didn't have indication for endoscopic retrograde cholangiopancreatography according to ultrasonographic findings underwent endoscopic retrograde cholangiopancreatography (p=0.508, kappa=0.72). CONCLUSIONS: Magnetic resonance cholangiopancreatography is valuable in diagnosis of extrahepatic cholestasis in patients suspected with intrahepatic cholestasis according to ultrasonographic findings. Magnetic resonance cholangiopancreatography does not prevent endoscopic retrograde cholangiopancreatography requirement significantly in patients who need diagnostic endoscopic retrograde cholangiopancreatography following ultrasonography.

Metal stents for malignant biliary obstruction.

The main problem in the palliative treatment of malignant biliary obstruction is recurrent jaundice and cholangitis due to clogging of the endoprostheses. Large-bore metal stents, which can be placed using small-sized delivery systems, have been recognized as an important gain. Their use has facilitated the percutaneous drainage procedure. The long-term patency rates of both endoscopically and percutaneously placed metal stents seem to be better than those of conventional stents. Although the long-term economical aspects are in favor of metal stents due ot the decreased need for readmissions and reinterventions, the high initial costs of metal stents constitute the main obstacle to their wide-spread use.

Biliary strictures complicating liver transplantation. Incidence, pathogenesis, management, and outcome.

Six hundred sixty-six patients received 792 liver transplants between February 1, 1984 and September 30, 1991. Biliary reconstruction was by choledochocholedochostomy (CDCD) with T-tube (n = 509) or Roux-en-Y choledochojejunostomy (CDJ) (n = 283). Twenty-five patients (4%) developed biliary strictures. Anastomotic strictures were more common after CDJ (n = 10, 3.5%) than for CDCD (n = 3, 0.6%). Intrahepatic strictures developed in 12 patients. Six patients had occult hepatic artery thrombosis (HAT). The other six patients received grafts in which cold ischemia time exceeded 12 hours. Anastomotic strictures were successfully managed by percutaneous dilation (PD) in five patients (n = 10), operation in three (n = 6), with retransplantation required in two patients. Intrahepatic strictures were managed by PD in seven, retransplantation in one, and expectantly in four patients. Of 25 patients, 19 (76%) are alive with good graft function. In three of six deaths, the biliary stricture was a significant factor to the development of sepsis and allograft failure. The authors conclude that (1) anastomotic strictures are rare after LT; (2) the development of biliary strictures may signify occult HAT; (3) PD is effective for most strictures; and (4) extended cold graft ischemia (less than 12 hours) may be injurious to the biliary epithelium, resulting in intrahepatic stricture formation.