Endoscopic nasobiliary drainage: an effective treatment option for benign recurrent intrahepatic cholestasis (BRIC)

Benign recurrent intrahepatic cholestasis (BRIC) is characterised by recurrent episodes of jaundice, severe pruritus and low or normal serum -glutamyltransferase activity lasting from several weeks to months. BRIC is an autosomal recessive disorder caused by the mutation in either of the two hepatic transporter genes—ATP8B1 or ABCB11 gene. The disease is very well known for episodic flare of jaundice with cholestatic symptoms that are spontaneous or perpetuated by acute insults, followed by self-recovery. There is no proven medical therapy and rarely does it progress to progressive familial intrahepatic cholestasis (PFIC) or biliary cirrhosis. BRIC may be associated with nephrolithiasis, diabetes or pancreatitis. Here, we report a case of BRIC with spontaneous flare and further complicated by drug-induced liver injury with disabling cholestastic symptoms, who underwent endoscopic nasobiliary drainage and was completely relieved of the distressing symptoms.

http://ift.tt/2pgifTf

Congenital talipes equinovarus and congenital vertical talus secondary to sacral agenesis

Sacral agenesis is a rare congenital defect which is associated with foot deformities such as congenital talipes equinovarus (CTEV) and less commonly congenital vertical talus (CVT). We report a 3-year-old Caucasian girl who was born with right CTEV and left CVT secondary to sacral agenesis. Her right foot was managed with a Ponseti casting method at 2 weeks, followed by an Achilles tenotomy at 4 months. The left foot was initially managed with a nocturnal dorsi-flexion splint. Both feet remained resistant and received open foot surgery at 10 months producing plantigrade feet with neutral hindfeet. At 19 months, she failed to achieve developmental milestones and examinations revealed abnormal lower limb reflexes. A full body MRI was performed which identified the sacral agenesis. We advocate early MRI of the spine to screen for spinal defects when presented with resistant foot deformities, especially when bilateral.

http://ift.tt/2pHKhd7

Oesophageal stent placement to treat a massive iatrogenic duodenal defect after laparoscopic cholecystectomy

Iatrogenic duodenal injury occurring during laparoscopic cholecystectomy (LC) is managed surgically, though rarely a large, persistent fistula is refractory to surgical interventions. We present the case of a 40-year-old woman transferred to our centre following elective LC for a reported perforated duodenal ulcer. An uncontained leak was found to originate from a 1.5 cm duodenal defect, with no evidence of ulceration. A duodenostomy tube was placed. One month after abdominal closure, the patient continued to have a persistent, large duodenal fistula. A through-the-scope covered oesophageal stent was placed under endoscopic and fluoroscopic guidance. Five weeks later, it was successfully retrieved and no subsequent extravasation of contrast from the duodenum was noted. Unrecognised iatrogenic duodenal injuries sustained during LC can be catastrophic. In cases of massive duodenal defects and high-output biliary fistula uncontrolled after surgical intervention, endoscopic-guided and fluoroscopic-guided placement of a fully covered oesophageal stent may be lifesaving.

http://ift.tt/2pgv8Nh

Psoriasiform papules, condyloma lata, lung nodules and hepatitis: the enormous variability of secondary syphilis manifestations

Description

A 44-year-old man, married, consulted his general practitioner with a 5-day history of fever, myalgia, headache and dry cough. Physical examination demonstrated wheezes in lung auscultation and liver function tests revealed cholestatic hepatitis with no increase in inflammatory markers; abdominal ultrasound was normal and thorax tomography showed small areas of subpleural consolidation (figure 1) and infracentimetric mediastinal lymph nodes suggestive of infection. A hepatotropic viral infection was presumed and he was treated symptomatically with no improvement. He was admitted to the hospital 2 weeks later presenting with perianal condylomata lata and psoriasiform papules affecting the trunk, genitalia, palms and soles (figure 2). Serological markers of hepatitis A, B and C and HIVs were negative and alpha 1-antitrypsin deficiency, autoimmunity and iron or copper overload were excluded. Both serum venereal disease research laboratory (VDRL) and treponemic tests were positive, the former with a titre of 1:128, and...

http://ift.tt/2pHMtBm

Penoscrotal haematoma after cardiac catheterisation

Scrotal haematoma is an extremely rare complication after cardiac catheterisation (CC) with only few cases reported in literature. We report a 56-year-old patient who developed large scrotal haematoma after CC via transfemoral approach requiring blood transfusion due to haemodynamic instability. After an uneventful elective procedure, he was discharged with a collagen plug-based vascular closure device (Angio-Seal). He developed sudden onset, excruciating groin pain with scrotal swelling and shock. Bleeding was stopped with manual compression over the femoral artery and 2 units of packed red blood cells were transfused. CT angiogram revealed scrotal haematoma without active bleeding. Testicular blood supply remained intact. Scrotal swelling improved with conservative management and patient got discharged 3 days later in a stable condition. The review of literature suggests that penoscrotal haematoma is unusual after CC but may result from arterial injury or bleeding into the fascial planes. Majority of patients require observation but surgery is indicated in selected cases for vascular complications.

http://ift.tt/2pgsG9p

Emphysematous Salmonella aortitis with mycotic aneurysm

Description

A 53-year-old man with history of poorly controlled diabetes mellitus presented with left chest pain radiated to the left shoulder for 3 weeks. On examination, he had low-grade fever, tachypnoea, regular pulse rate at 90/min and blood pressure 140/90 mm Hg. His cardiovascular and chest examinations were otherwise unremarkable.

Initial blood test showed leucocytosis and elevated C reactive protein level. Chest radiograph revealed abnormal soft tissue density silhouette with proximal descending aorta with air-filled collection (figure 1, arrow).

Figure 1

Chest radiograph reveals abnormal soft tissue density silhouette with proximal descending aorta with air-filled collection (arrow).

Contrast-enhanced CT of the aorta (CTA) revealed 1.3x1.5 cm outpouching lesion protruded from the anterior aspect of the proximal descending thoracic aorta (figure 2A, asterisks) with periaortic collection containing multiple air pockets (figure 2A, arrows) surrounding the aortic arch. Salmonella group D bacteraemia was confirmed...

http://ift.tt/2pHM5mx

Non-Allergic Rhinitis: Position paper of the European Academy of Allergology and Clinical Immunology

Abstract

This EAACI position paper aims at providing a state-of-the-art overview on non-allergic rhinitis (NAR). A significant number of patients suffering from persistent rhinitis are defined as non-allergic non-infectious rhinitis (NANIR) patients, often denominated in short as having NAR. NAR is defined as a symptomatic inflammation of the nasal mucosa with the presence of minimal 2 nasal symptoms like nasal obstruction, rhinorrhoea, sneezing, and/or itchy nose, without clinical evidence of endonasal infection and without systemic signs of sensitization to inhalant allergens. Symptoms of NAR may have a wide range of severity, and be either continuously present and/or induced by exposure to unspecific triggers, also called nasal hyperresponsiveness (NHR). NHR represents a clinical feature of both AR and NAR patients. NAR involves different subgroups: drug-induced rhinitis, (non-allergic) occupational rhinitis, hormonal rhinitis (including pregnancy rhinitis), gustatory rhinitis, senile rhinitis and idiopathic rhinitis (IR). NAR should be distinguished from those rhinitis patients with an allergic reaction confined to the nasal mucosa, also called 'entopy' or local allergic rhinitis (LAR).

We here provide an overview of the current consensus on phenotypes of NAR, recommendations for diagnosis, a treatment algorithm and defining the unmet needs in this neglected area of research.

This article is protected by copyright. All rights reserved.

http://ift.tt/2pNA7pQ