Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011849 (diabetes)
 277,896 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Between November 1986 and January 2000, 28 patients with insulin-dependent diabetes mellitus were enrolled in the implanted insulin pump study at Johns Hopkins Hospital. An additional two patients underwent pump implantation under compassionate use guidelines due to apparent resistance to subcutaneously administered insulin uptake. The mean patient age was 44 +/- 10.5 years. Eleven patients (39%) were female and the mean duration of diabetes was 25.7 +/- 8.9 years. Diabetic retinopathy, neuropathy, and nephropathy were present in 43%, 25% and 11 % of patients, respectively. The insulin pumps functioned safely for a total of 189 patient years. Mean pump life was 26 +/- 1.2 months. There was no mortality. Morbidity was limited to pump-site infections [n=y (4%) of all pumps placed], one case of pump migration and skin erosion, and one small bowel obstruction associated with the pump catheter. Mean serum hemoglobin AIC levels before and after pump placement were 9.0 +/- 2.9% and 7.5 +/- 0.7% (P=0.0023), respectively. Correspondingly, the mean daily blood glucose levels decreased from l61 +/- 40 mg/dl before placement to 141 +/- 27 mg/dl after pump placement (P=0.0063). Intraperitoneal delivery of insulin by a mechanical pump appears to be an attractive alternative for the treatment of insulin-dependent diabetes mellitus.
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PMID:Intraperitoneal delivery of insulin via mechanical pump: surgical implications. 1112 19

Newer, minimally invasive catheter-based endovascular technology utilizing stent grafts are currently being evaluated for abdominal aortic aneurysm (AAA) repair. A retrospective review of all (3 years) consecutive, non-ruptured elective AAA repairs was undertaken to document the results of AAA surgical repair in a modern cohort of patients to allow a contemporary comparison with the evolving endoluminal data. One hundred twenty-one AAAs were identified in a male veteran population. Mean age was 68.5 +/-7.7 years. Medical history review showed hypertension in 55%, heart disease in 73.5%, peripheral vascular disease in 21%, stroke and transient ischemic attacks in 22%, diabetes mellitus in 7%, renal insufficiency in 10%, and smoking history in 80%. The AAA size was documented with ultrasound (5.2 +/-1.3 cm, n=40) and computed tomography (5.6 +/-1.3 cm, n=100). Fifty-nine percent had angiography. Intraoperative end points included an operative time of 165 +/-6.3 minutes from incision to dressing placement. A Dacron tube graft was used in 78%, the remaining were Dacron bifurcated grafts. A suprarenal clamp was used in 8% for proximal aortic control with juxtarenal aneurysms. A pulmonary-artery catheter was placed in 69%. A transverse incision was used in 69% of patients and a midline incision was used in the rest. Estimated blood loss was 1505 +/-103 mL; cell saver blood returned 754 +/-53 mL; crystalloid/Hespan 4771 +/-176 mL; banked packed red blood cells 0.75 +/-0.11 U. Time to extubation was, in the operating room (78.5%), on the day of the operation (5.0%), postoperative day (POD) 1 (12.4%), POD2 (1.7%), POD3 (0.8%), and one case was performed with epidural anesthesia only. Postoperative end points included a 30-day mortality rate of 1.6% (two patients). Postoperative morbidity included wound dehiscence 0.8%; sepsis, urinary tract infection, wound infection, leg ischemia, ischemic colitis, and stroke each had an incidence of 1.6%; myocardial infarction, congestive heart failure, pneumonia, re-operation for suspected bleeding, and ileus or bowel obstruction occurred with an incidence of 3.3%. No significant increase in serum creatinine levels was noted. Time to enteral fluids/nutrition was 3.5 +/-0.08 days. Patients were out of bed to a chair or walking by 1.3 +/-0.06 days postoperatively. The length of stay in the intensive care unit (ICU) was 2.0 +/-0.12 days and postoperative hospital stay was 6.6 +/- 0.33 days. Transfusion requirement for the hospital stay was 1.6 +/-0.2 U per patient. This review highlights a cohort of male veteran patients with significant cardiac co-morbidity who have undergone repair with a conventional open technique and low mortality and morbidity rates. This group had rapid extubation, time to oral intake, and ambulation. In addition, ICU and hospital stays were relatively short.
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PMID:Abdominal aortic aneurysm repair. 1156 37

Patients with normal or borderline sweat tests present a diagnostic challenge. In spite of the availability of genetic analysis and measurement of nasal potential difference, there is still uncertainty in diagnosing cystic fibrosis in some patients. CA 19-9 is a tumor-associated antigen whose levels were previously found to be elevated in some cystic fibrosis patients. We investigated whether serum CA 19-9 levels can contribute to establishing the diagnosis of cystic fibrosis in patients with a borderline sweat test, and evaluated the influence of different clinical variables on CA 19-9 levels. Serum CA 19-9 levels were measured in 82 cystic fibrosis patients grouped according to their genotype and in 38 healthy individuals. Group A included 50 patients who carried two mutations previously found to be associated with a pathological sweat test and pancreatic insufficiency (DeltaF508, W1282X, G542X, N1303K, and S549R). Group B included 13 compound heterozygote cystic fibrosis patients who carried one mutation known to cause mild disease with a borderline or normal sweat test and pancreatic sufficiency (3849+10kb C-->T, 5T). Group C included 38 normal controls. Nineteen cystic fibrosis patients carried at least one unidentified mutation. An association between CA 19-9 levels and age, pulmonary function, pancreatic status, sweat chloride, previous pancreatitis, serum lipase, meconium ileus, distal intestinal obstruction, liver disease, and diabetes was investigated. The distribution of CA 19-9 levels was significantly different between the three groups ( p<0.01); high CA 19-9 levels were found in 60% (30/50) of group Apatients and in 46.6% (6/13) of group B patients, but in only 5.2% (2/38) of the controls. CA 19-9 levels were inversely related to forced expiratory volume in 1 s, while no association was found with the other clinical parameters examined. Our findings suggest that the serum CA 19-9 in cystic fibrosis patients originates in the respiratory system, and has a useful ancillary role, particularly when diagnostic uncertainty exists. Hence, the diagnosis of cystic fibrosis should be considered in patients with borderline sweat tests and high CA 19-9 levels, but normal levels do not exclude cystic fibrosis.
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PMID:Serum CA 19-9 levels as a diagnostic marker in cystic fibrosis patients with borderline sweat tests. 1459 87

Human disease is a result of interactions between each person's genetic program and the environment. Individual genes responsible for many pediatric diseases have been discovered, but there is still considerable variability in phenotype within a given disorder. While some of the variability is undoubtedly caused by environmental factors, genes other than the primary disease gene likely modify phenotypes. These gene modifiers alter the effects of the primary disease gene and therefore result in phenotypic variability. Studying gene modifiers will therefore likely lead to a better understanding of pathophysiology and to development of new treatments. Statistical and genetic methods used to identify gene modifiers range from simple association studies to complex family studies, each with its own strengths. The search for gene modifiers in cystic fibrosis (CF), a common mendelian disorder, provides a good example of an application of the latest approaches. Potential modifiers of CF are chosen to reflect the known pathophysiology of CF, including genes involved in the host response to infection and CF complications such as intestinal obstruction, diabetes, and liver disease. A region on chromosome 19 that modifies intestinal obstruction in CF has been identified based on a region of synteny in the mouse; however, the specific gene has not been completely characterized. While a number of other putative gene modifiers have been investigated in CF, none are clearly substantiated. To date, only a few gene modifiers in pediatric disease have been conclusively identified, but we believe that future research will open the doors for new knowledge and treatment.
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PMID:Gene modifiers in pediatrics: application to cystic fibrosis. 1536 69

The indications for initiating total parenteral nutrition (TPN) were prospectively evaluated in 100 consecutive patients at a tertiary referral hospital with a long-standing Nutritional Support Service to illustrate the reasons why the parenteral route was chosen at this unique institution in terms of patient population. Sixty male and 40 female patients, average age 59 +/- 17 years (range 22-86 years), were classified a priori as to the underlying reasons for initiation of TPN. The study was conducted by a Nutrition Support Service at this hospital without pediatric, trauma, or burn services specializing in the care of patients with diabetes mellitus. Of the 100 patients, 63% were from the surgical service; 24% had diabetes mellitus. Their mean weight (118 +/- 29% of ideal), body mass index (25 +/- 6 kg/m(2)), and serum albumin (2.8 +/- 0.7 g/dL) indicated a reasonable body composition with a moderate systemic inflammatory response. Six patients received preoperative TPN for an average of 5 +/- 3 days with a variety of diagnoses including malignancy, Crohn's disease, bowel obstruction, and gastrointestinal bleeding. The underlying reasons for initiating nutritional support were related to three factors that largely determine the need for involuntary feeding: preexisting protein calorie malnutrition, actual or anticipated semistarvation for a prolonged period, and the presence of a systemic inflammatory response. The choice of TPN was based on anticipated or proven intolerance to full enteral feeding. The duration of time before initiation of TPN postoperatively was 6 +/- 5 days, which reflects our policy that initially well-nourished patients who are experiencing a systemic inflammatory response should not undergo more than 5 to 7 days of inadequate feeding. The duration of TPN overall was 11 +/- 10 days, which primarily illustrates the dramatic reduction in length of hospital stay that has occurred throughout the health care system and the willingness to provide TPN in alternative settings including transitional care units, rehabilitation hospitals, and for short-term care, the patient's home. The most common specific reasons identified for initiating TPN rather than enteral nutrition were ileus (25%), an underlying acid-base or electrolyte/mineral disorder (13%) requiring correction, and the convenience of TPN because a central venous catheter was in place (12%). The usual indication for nutritional support at this tertiary referral and specialty hospital was actual or impending protein calorie malnutrition. TPN was chosen for a variety of reasons related to actual or anticipated tolerance to enteral feeding. This audit demonstrates that our TPN practice has evolved in relation to time of initiation and duration of feeding, which reflect a clearer appreciation of the risks and benefits of TPN.
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PMID:Indications for total parenteral nutrition in the hospitalized patient: a prospective review of evolving practice. 1553 43

Inguinal hernia repair is performed in more than 600,000 cases every year in the United States. However, the true prevalence may be even higher. Many groin hernias are not diagnosed, e.g., Sportmans' hernia, or are asymptomatic. The etiology of classic inguinal hernia, Sportsman's hernia or traumatic hernia may be different. The hernia repair is performed in agreement with a classification of the hernia, e.g., Nyhus classification. According to recent randomized controlled trials and meta-analyses open-mesh repair demonstrates several advantages in comparison to laparoscopic procedures. Laparoscopic procedures require more time and cost more, show a potential for serious complications and may be followed by an increased rate of recurrence. There may be a faster reconvalescence after laparoscopic procedures. However, there may be also a selection bias. Laparoscopic procedures are associated with specific complications, e.g., pneumomediastinum, pneumothorax, gas extravasation, trocar injuries, intraabdominal adhesions, bowel obstruction, which are rarely or never seen in open-mesh repair. In the United States we could observe an uncoupling of hernia repair from classification. In more than 90% of cases the treatment was open-mesh. In many hernia studies the hernias were classified as direct or indirect, primary or recurrent. The existing classifications are based on anatomical findings in relation to the development of the hernia: posterior floor integrity, enlarged interior ring and size of the hernia. However, the size of the hernia may not always be associated with the severity of the hernia and it may be difficult to estimate. The outcome of hernia repair may be influenced by other factors. There may be differences in the presentation of the hernia to the surgeon based on the damage done to the surrounding tissue in the inguinal canal, e.g., external ring, aponeurosis of the external oblique, inguinal ligament, which is most often accompanied by severe adhesions. Further factors influencing outcome of hernia repair may be patient-related factors, e.g., constipation, ASA classification, diabetes, smoking. A classification should be simple to use and easy to remember: (A) indirect hernia, (B) direct hernia, (C) scrotal or giant hernia, (D) femoral hernia. A and B can be classified as (0) uncomplicated, (1) posterior floor defect, (2) posterior floor defect plus defect in the anterior part of the inguinal canal. All four types (A-D) may be either primary or recurrent. In this classification combined femoral, indirect and/or direct hernias can be categorized by using the types A, B, C, or D as in a modular construction system. The category "other" is reserved for rare types of hernia, e.g., obturator hernia, Spieghelian hernia. Aggravating factors are included: Diabetes, obesity, age above 65, constipation, ASA III or more and cigarette smoking. This classification may be helpful to evaluate outcome of hernia repair with regard to patient related factors and the increased demands for the surgeon and the staff. In some health care systems the general belief is that all hernias are equal and be managed equally. However, groin hernias may be complex and need individual treatment.
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PMID:Inguinal Hernia: classification, diagnosis and treatment--classic, traumatic and Sportsman's hernia. 1585 79

Disseminated herpes simplex virus (HSV) infection usually manifests in the immunocompromised. However, anecdotal examples of visceral HSV disease and viremia have complicated type I diabetes. A case of a 53-year-old type I diabetic patient with bowel obstruction one week subsequent to bronchitis is reported. At laparotomy, a perforated segment of ileum was associated with an adhesive peritoneal band. HSV cytopathic atypia and HSV immunohistochemical staining were confined to fibrocytes and mesothelial cells without involvement of the epithelium. Dissemination of symptomatic HSV pneumonia was verified by histology, immunohistochemistry, in situ hybridization, polymerase chain reaction and direct fluorescence antibody. Intravenous acyclovir resolved symptoms. This is a novel documentation of HSV complicating ileal adhesive band disease. Furthermore, this case indicates that the HSV cytopathic effect is not unique to the epithelium. Disseminated infection can manifest in myofibrocytes and mesothelium, distinguishing it from standard epithelial atypia of localized HSV infection.
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PMID:Herpes simplex virus type II infection of ileum mesothelium: a case report and review of the literature. 1599 70

Nutrition therapy is vital to the overall management of lung transplant recipients. The objective of this review is to outline the current applications of pre- and posttransplant nutrition management of the adult lung transplant recipient. Pretransplant nutrition therapy decisions are based on cause of end-stage lung disease, transplant indications, and pretransplant nutritional status. Maintaining adequate nutrient stores is the major goal of nutrition therapy for patients awaiting transplantation. In the posttransplant course, several gastrointestinal (GI) complications such as gastroesophageal reflux, gastroparesis, and distal intestinal obstruction syndrome complicate nutritional recovery. Long-term nutrition therapy for lung transplant recipients is aimed at management of common comorbid conditions such as obesity, diabetes mellitus, hypertension, osteoporosis, and hyperlipidemia. Lung transplantation outcomes are steadily improving; however, much has yet to be explored to improve the nutrition management of these patients in both the pre- and posttransplantation course.
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PMID:Current nutrition practices in adult lung transplantation. 1621 58

In France the median life expectancy of patients with cystic fibrosis (CF) is 36 years and one third of patients are adults. Respiratory disorders are the main determinants of survival during adulthood, although gastrointestinal complications can also have a major impact on quality of life and nutritional status. We reviewed gastro-intestinal and pancreatic manifestations of CF in adults. Some complications (diabetes, distal intestinal obstruction syndrome and acute pancreatitis) are more frequent in adulthood than in childhood. Clinical and therapeutic aspects of specific conditions (such as distal intestinal obstruction syndrome) or atypical presentations (including appendicular complications) are presented herein.
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PMID:[Digestive complications in adults with cystic fibrosis]. 1651 88

Pancreatic surgery in children is a rare occurrence, and this unfamiliarity can be associated with the assumption of significant morbidity and mortality. The indication for pediatric pancreatic surgery and its relationship to postoperative complications and mortality was evaluated. Patients with pancreatic disease requiring surgical intervention from 1992 to 2004 at a tertiary referral center were retrospectively reviewed. Disorders were divided into 3 categories: 1) pancreatitis, 2) trauma, and 3) tumors. Sixty-two patients (28 males and 34 females), average age was 9.5 years (range, 1 week-18 years), underwent 72 operations. Thirty-seven procedures in 30 category I patients, 18 procedures in 15 category II, and 17 operations in 17 category III. There was only one death. A total of 33.9 per cent of the patients had postoperative complications that included: infection (11%), pseudocyst (6%), diabetes mellitus (5.6%), pancreatic fistula (3%), bowel obstruction (1.3%), extracellular fluid (1.3%), pleural effusion (1.3%), and recurrent abdominal pain (13%) (all in category I patients). There was equivalent morbidity between all 3 groups but unique differences with in the categories. Recurrent abdominal pain characterized category I patients, fistulas were more common in category II, and diabetes mellitus was primarily related to near total excisions in category III. Pancreatic surgery in children is associated with a very low mortality (1.6%) and morbidity equal to that of adult patients. Unique types of morbidities occur with each category of disease state.
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PMID:Pancreatic disorders in children: relationship of postoperative morbidity and the indication for surgery. 1687 89


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