Scurvy: historically a plague of the sailor that remains a consideration in the modern intensive care unit

We report the case of the case of a 56 year old female with sepsis on a background of rheumatoid arthritis and steroid use manifesting with overt clinical features of scurvy. Ascorbic acid assays were able to demonstrate severe deficiency and confirm a diagnosis of scurvy. Clinical resolution of signs and symptoms following commencement of vitamin C replacement was rapid. The intensivist and dietitian need to consider this diagnosis even in the first world setting, particularly in the presence of sepsis, inflammatory conditions, steroid use and importantly malnutrition.     

Skin,muscle and joint disease from the 17th century: scurvy

We report three cases of scurvy, with differing musculoskeletal presentations, from a tertiary teaching hospital in Sydney, Australia. Case 1 was a man with cerebral palsy who presented with knee swelling following a minor injury. In Case 2, a patient with thalassaemia major presented with purpuric rash, difficulty walking and distal thigh swelling and ecchymosis. Case 3 was a man with Down’s syndrome who presented with acute ankle arthritis. Scurvy in Cases 1 and 3 were related to abnormal dietary preferences, whereas in Case 2, scurvy was thought to be related to thalassaemia. All three cases responded rapidly to vitamin C replacement. The subjects did not appear malnourished as they had adequate carbohydrate and protein intake.     

On the Influence of Vitamin K-rich Vegetables and Wine on the Effectiveness of Warfarin Treatment

ABSTRACT Thrombotest (TT) values were studied in patients fed an ordinary diet and on continuous and well controlled warfarin therapy because of deep venous thrombosis or pulmonary embolism. The aim was to characterize the effect of single and multiple administrations (daily during one week) of vitamin K₁ (Konakion®), vitamin K-rich vegetables such as spinach and broccoli, and table wine. Single administration of 250 μg vitamin K₁, 250 g spinach, 250 g broccoli and 37.5 cl wine did not result in TT-values outside the therapeutic range. However, when Konakion®, broccoli and spinach were given daily during one week the TT-values tended to rise above the therapeutic limit, requiring dose adjustment. On the basis of this study it appears that excessive intake of vitamin K-rich food and a moderate intake of alcohol on one occasion may be permitted during anticoagulant therapy.     

A case of the Budd Chiari syndrome attributed to a deficit in protein C

We report a case of Budd Chiari syndrome associated with a deficit in protein C, in a young women admitted to hospital for investigation of thromboembolic disease and ascites. This coagulation defect was thought to be an aetiological factor in the suprahepatic vein thrombosis. The diagnosis and treatment of this rare form of the syndrome are discussed.     

Symptomatic hereditary type-II protein C deficiency caused by a missense mutation in exon IX of the protein C gene (Gly381 to Ser)

Summary We report the characterization of the genetic defect in a family with hereditary type-II protein C (PC) deficiency. The propositus is a 28-year-old woman with a history of thrombosis. Her PC activity level (58%) and PC antigen level (115%) are comptible with the diagnosis of type-II PC deficiency. Her asymptomatic sister is also PC deficient. Analysis of the PC gene of the propositus revealed a point mutation (G to A) at nucleotide 8856, which results in the replacement of Gly381 by Ser in the heavy chain of PC. The amino acid change occurs close to the active-site serine at a residue which is highly conserved among the serine proteases. The mutation is also present in the PC gene of the propositus' sister. Her brother, who is asymptomatic, has a normal genotype with respect to the mutation at nucleotide 8856.     

Dietary habits of adolescents living in North America,Europe or Oceania: A review on fruit,vegetable and legume consumption,sodium intake,and adherence to the Mediterranean Diet

AimsThis review aimed at investigating fruit, vegetable and legume consumption, salt/sodium intake, and the adherence to the Mediterranean dietary pattern in adolescents, three key aspects towards the adoption of a healthy diet.Data synthesisThree separate searches were carried out on PubMed and Scopus, using the same procedure, searching for studies published in the previous decade with data on fruit and/or vegetable or legume consumption, salt or sodium intake, and adherence to the Mediterranean Diet assessed using the KIDMED questionnaire. The review included a total of 58 papers, which describe original investigations on healthy adolescents (10–19 years old) living in North America, Europe or Oceania, with a sample size >150 participants. The average fruit and vegetable consumption has been found strongly below the recommended values of 400 grams or 5 portions per day in almost all the examined populations. Very little is known about adolescents' legume consumption. Few available data have been found also for sodium intake and, for the majority of the screened populations, levels were far above the recommended 5 grams per day. Lastly, a medium–low adherence to the Mediterranean Diet has been found for adolescents living in Mediterranean Countries.ConclusionsAdolescents living in North America, Europe or Oceania are far from being compliant with the nutritional recommendations for fruit, vegetables, legumes, and sodium, and they do not follow the principles of the Mediterranean Diet. Educational and behavioural interventions are required to improve adolescents' dietary patterns.     

An Evaluation of Endoscopic Images from Over 15 Years Prior to the Diagnosis of Autoimmune Gastritis: A Report of Three Patients

We herein describe three patients whose endoscopic images from over 15 years prior to their diagnosis of autoimmune gastritis (AIG) were available for review. All patients had corpus-dominant atrophic gastritis at the time of the diagnosis of AIG. Previous endoscopic images without severe atrophy showed erythema restricted to the fundic mucosa. These findings are suggestive of ongoing gastritis in patients with AIG. Initial endoscopy in Patient 2 showed multiple hyperplastic polyps that decreased in size and number over the course of 15 years. In this patient, circular wrinkle-like patterns and remnant oxyntic mucosa were visible after the atrophy had become quite prominent.     

An approach to study the viral safety of plasma-derived products in previously treated, non-infected patients

Using the polymerase chain reaction (PCR), we designed a study concept to evaluate the safety of plasma derivatives in previously treated patients who are non-infected by the specific viruses studied. Several product lots can be studied in a single patient, with a study period for each lot of 3 months. In the present study 19 patients were included for treatment with Baxter Hyland Immuno's PCR-screened factor VIII concentrate Immunate (n=7), factor IX concentrate Immunine (n=10), the by-passing agent FEIBA plus Immunine (n=1), and the protein C concentrate Ceprotin (n=1). PCR testing for hepatitis B, C or HIV genomic material in patient samples was done as well as serological testing. All patients remained negative for the tested markers. All seven Immunate patients completed three treatment periods with three different lots of the study drug. The median study period was 282 days and the median dose 115 000 units, with a median of 115 exposure days. Five of the 10 Immunine patients completed three treatment periods and four patients, two treatment periods. One Immunine patient was discontinued from the study for reasons unrelated to the study drug administration. The median study period was 305 days and the median total dose 82 200 units, with a median of 88 exposure days. Our study presents a new design to approach the evaluation of viral safety of new plasma derivatives in previously treated, non-infected patients (NIPs) and offers several advantages over the currently recommended studies using testing for serological markers of infection in previously untreated patients (PUPs).     

Lack of sequence variations in the C4b-BP β-chain in patients with type III protein S deficiency bearing the Ser 460 to Pro mutation: description of two new intragenic isomorphisms in the C4b-BP β-chain gene (C4BPB)

Type III protein S (PS) deficiency, characterized by low levels of free PS and normal total PS levels, is often associated with the Ser 460 to Pro substitution. However, some patients bearing this mutation have normal PS levels, suggesting that another gene defect may account for this phenotype. We postulated that this defect was located in the C4b-BP β-chain gene (C4BPB) and searched for a mutation in the coding regions of this gene in 35 propositi with type III PS deficiency and bearing the Ser 460 to Pro mutation. No mutations explaining the phenotype of type III PS deficiency were identified. We did, however, find two frequent nucleotide changes, one being located in the donor splice site of intron d and the second in the codon corresponding to Asn 137. We used these two polymorphisms to establish C4BPB gene haplotype in five informative type III PS-deficient families and exclude a role of the C4BPB gene in this phenotype of three of them. Finally, increased C4b-BP β-chain levels were not responsible for the phenotype of type III PS deficiency as the C4BPB haplotype did not correlate with C4b-BP β-chain levels.     

The “angina-producing” myocardial segment: An approach to the interpretation of results of coronary bypass surgery

The first 153 cases of saphenous vein aortocoronary bypass surgery performed at The Johns Hopkins Hospital were reviewed. Eighty-eight percent of the 140 late survivors reported significant symptomatic improvement. Seventy-one unselected patients consented to complete reevaluation at a mean interval of 6.1 months postoperatively. Vein bypass patency in this group was 66 percent. Eighty-two percent of these 71 patients had improved performance on electrocardiographic stress testing. There were no significant differences between hemodynamic status on pre- and postoperative studies regardless of the status of vein bypass patency. Repeat coronary angiography revealed a 30 percent incidence of new total occlusions of the intrinsic coronary circulation. Segmental wall motion on ventriculography was improved in 12 percent, unchanged in 50 percent and decreased in 38 percent of the segments analyzed. Localized electrocardiographic changes compatible with myocardial damage developed in the immediate postoperative period in 45 of these patients (63 percent); in 38 of the 45, these changes corresponded to new angiographic abnormalities. Physiologic mechanisms underlying symptomatic improvement were sought by identification of an “angina-producing” myocardial segment. In most instances, alleviation of angina could be related to either: (1) a patent bypass graft into an unchanged intrinsic coronary artery with presumed increased blood flow to the distal coronary segment, or (2) occlusion of the bypass graft and the corresponding coronary artery with probable infarction of previously ischemic myocardium.Current criteria for the selection of patients undergoing coronary bypass surgery are reviewed in the light of these findings. It is concluded that relief of disabling angina is the major indication for surgery. Prevention of myocardial infarction and improvement in left ventricular function are at present less reliable objectives.