Surviving Patients (surviving + patient)

Distribution by Scientific Domains


Selected Abstracts


Tuberculosis in liver transplant recipients: A systematic review and meta-analysis of individual patient data,

LIVER TRANSPLANTATION, Issue 8 2009
Jon-Erik C. Holty
Mycobacterium tuberculosis (MTB) causes substantial morbidity and mortality in liver transplant recipients. We examined the efficacy of isoniazid latent Mycobacterium tuberculosis infection (LTBI) treatment in liver transplant recipients and reviewed systematically all cases of active MTB infection in this population. We found 7 studies that evaluated LTBI treatment and 139 cases of active MTB infection in liver transplant recipients. Isoniazid LTBI treatment was associated with reduced MTB reactivation in transplant patients with latent MTB risk factors (0.0% versus 8.2%, P = 0.02), and isoniazid-related hepatotoxicity occurred in 6% of treated patients, with no reported deaths. The prevalence of active MTB infection in transplant recipients was 1.3%. Nearly half of all recipients with active MTB infection had an identifiable pretransplant MTB risk factor. Among recipients who developed active MTB infection, extrapulmonary involvement was common (67%), including multiorgan disease (27%). The short-term mortality rate was 31%. Surviving patients were more likely to have received 3 or more drugs for MTB induction therapy (P = 0.003) and to have been diagnosed within 1 month of symptom onset (P = 0.01) and were less likely to have multiorgan disease (P = 0.01) or to have experienced episodes of acute transplant rejection (P = 0.02). Compared with the general population, liver transplant recipients have an 18-fold increase in the prevalence of active MTB infection and a 4-fold increase in the case-fatality rate. For high-risk transplant candidates, isoniazid appears safe and is probably effective at reducing MTB reactivation. All liver transplant candidates should receive a tuberculin skin test, and isoniazid LTBI treatment should be given to patients with a positive skin test result or MTB pretransplant risk factors, barring a specific contraindication. Liver Transpl 15:894,906, 2009. © 2009 AASLD. [source]


A Prospective Observational Study of the Effect of Etomidate on Septic Patient Mortality and Length of Stay

ACADEMIC EMERGENCY MEDICINE, Issue 1 2009
Karis L. Tekwani MD
Abstract Objectives:, Etomidate is known to cause adrenal suppression after single-bolus administration. Some studies suggest that when etomidate is used as an induction agent for intubation of septic patients in the emergency department (ED), this adrenal suppression leads to increased mortality, vasopressor requirements, and length of hospital stay. The authors sought to determine differences in the in-hospital mortality and hospital length of stay (LOS) between septic patients given etomidate and patients given alternative or no induction agents for rapid-sequence intubation in our ED. Methods:, This was a nonrandomized, prospective observational study of all patients meeting sepsis criteria who were intubated in an ED over a 9-month period. Times of patient presentation, intubation, admission, discharge, and/or death were recorded, as well as the intubation agent used, if any, and corticosteroid use. The authors also recorded relevant laboratory and demographic variables to determine severity of illness using the Mortality in Emergency Department Sepsis (MEDS) score. Mortality and survivor LOS between the patients given etomidate and those given alternative or no induction agents were compared. Results:, A total of 106 patients with sepsis were intubated over the study period. Of these, 74 patients received etomidate, while 32 patients received ketamine, benzodiazepines, propofol, or no induction agents. Age in years (median = 78; interquartile range [IQR] = 67 to 83), gender (45% male), MEDS score (median = 13; IQR = 10 to 15), and receipt of supplemental corticosteroids (56%) were statistically similar between the two groups. In-hospital mortality of patients given etomidate (38%; 95% confidence interval [CI] = 28% to 49%) was similar to those receiving alternatives (44%; 95% CI = 28% to 61%). Surviving patients had a median hospital LOS after receiving etomidate of 10 days compared to those receiving alternatives (7.5 days; p = 0.08). Conclusions:, No statistically significant increase in hospital LOS or mortality in patients given etomidate for rapid-sequence intubation was found. Suggestions that the use of etomidate for intubation in the ED be abandoned are not supported by these data. [source]


Intractable recurrent cervical cancer with pelvic bone involvement successfully treated with external hemipelvectomy

JOURNAL OF OBSTETRICS AND GYNAECOLOGY RESEARCH (ELECTRONIC), Issue 1 2008
Junzo Hamanishi
Abstract The indication of external hemipelvectomy for lateral recurrent cervical cancer involving the pelvic bone is controversial. We report the second longest surviving patient of recurrent cervical cancer successfully treated by external hemipelvectomy. A 38-year-old woman who had undergone conization for stage Ia1 cervical cancer six years earlier had severe right inguinal pain. A large multicystic recurrent tumor was identified in the right obturator region. After chemotherapy and chemoradiation, the tumor regressed, but soon relapsed. The patient's symptoms flared and the tumor was enlarged involving the right iliac bone. We performed right external hemipelvectomy with amputation of the right lower extremity, right iliac wing and ischiopubic bone. There was no major complication after the operation and the patient was discharged on postoperative day 48. After 27 months of follow-up, she has no complaints and is without evidence of recurrence. In selected cases of intractable lateral recurrent cervical cancer with pelvic bone involvement, relief from tumor-related pain and a possibility of prolonged survival can be expected by external hemipelvectomy. [source]


Plantar pressures in diabetic patients with foot ulcers which have remained healed

DIABETIC MEDICINE, Issue 11 2009
T. M. Owings
Abstract Aims, The recurrence of foot ulcers is a significant problem in people with diabetic neuropathy. The purpose of this study was to measure in-shoe plantar pressures and other characteristics in a group of neuropathic patients with diabetes who had prior foot ulcers which had remained healed. Methods, This was an epidemiological cohort study of patients from diabetes clinics of two Swedish hospitals. From a database of 2625 eligible patients, 190 surviving patients with prior plantar ulcers of the forefoot (hallux or metatarsal heads) caused by repetitive stress were identified and 49 patients agreed to participate. Barefoot and in-shoe plantar pressures were measured during walking. Data on foot deformity, activity profiles and self-reported behaviour were also collected. Results, Mean barefoot plantar peak pressure at the prior ulcer site (556 kPa) was lower than in other published series, although the range was large (107,1192 kPa). Mean in-shoe peak pressure at this location averaged 207 kPa when measured with an insole sensor. Barefoot peak pressure only predicted ,35% of the variance of in-shoe peak pressure, indicating variation in the efficacy of the individual footwear prescriptions (primarily extra-depth shoes with custom insoles). Conclusions, We propose that the mean value for in-shoe pressures reported in these patients be used as a target in footwear prescription for patients with prior ulcers. Although plantar pressure is only one factor in a multifaceted strategy to prevent ulcer recurrence, the quantitative focus on pressure reduction in footwear is likely to have beneficial effects. [source]


Long-term review of driving potential following bilateral panretinal photocoagulation for proliferative diabetic retinopathy

DIABETIC MEDICINE, Issue 1 2009
S. A. Vernon
Abstract Aim To determine the necessity for repeated Driver and Vehicle Licensing Agency (DVLA) visual field testing in people with diabetes who have had bilateral panretinal photocoagulation (PRP) for proliferative diabetic retinopathy. Methods A questionnaire survey was conducted of driving history in a cohort of people with diabetes who had been treated with bilateral PRP for proliferative retinopathy between 1988 and 1990. In addition, all similarly eligible subjects attending the diabetic retinal review clinic over a 12-month period who had had laser between 1991 and 2000 were questioned as to their driving status. Results Forty-five surviving patients from the 1988,1990 cohort were eligible and 25 returned the questionnaire (55%). Eight had never driven and 15 (13 with Type 1 diabetes) still held a valid licence, having passed the DVLA field test on a number of occasions. Neither of the two patients who had stopped driving reported failing the DVLA field test as the reason for stopping. All 12 of the patients directly questioned in the clinic were still driving and had passed at least one repeat DVLA test. Conclusions People with Type 1 diabetes who have no further laser treatment for proliferative diabetic retinopathy can expect to retain their UK driving licence for at least 15 years following small-burn PRP, provided they maintain sufficient acuity. [source]


Disease control, survival, and functional outcome after multimodal treatment for advanced-stage tongue base cancer

HEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 7 2004
James P. Malone MD
Abstract Background. Surgical resection and postoperative radiation for advanced-stage malignancies of the oral cavity, oropharynx, and hypopharynx result in a dismal overall survival of 38%. Patients with carcinoma of the tongue base frequently have advanced disease at the time of presentation, and combined-modality therapy is usually required to achieve cure. Because of the poor survival rates with advanced malignancies with standard therapy, new and innovative approaches continue to be developed in an attempt to have a greater impact on disease control, patient survival, and functional outcome after therapy. This study examines functional outcome, survival, and disease control in patients receiving an intensified treatment regimen with concomitant chemoradiotherapy, surgery, and intraoperative radiotherapy for previously untreated, resectable, stage III and IV squamous cell carcinoma (SCC) of the tongue base. Methods. Forty patients with previously untreated, resectable, stage III and IV squamous cell carcinoma of the tongue base were treated in one of three sequential phase II intensification regimens (IRs). Treatment consisted of perioperative, hyperfractionated radiotherapy (9.1 Gy) with concurrent cisplatin followed by surgical resection with intraoperative radiotherapy boost (7.5 Gy). Postoperative treatment involved concurrent chemoradiotherapy (40 Gy to the primary site and upper neck and 45 Gy to the supraclavicular areas) with cisplatin with or without paclitaxel. Locoregional and distant disease control, 2-year overall, and disease-specific survival rates were calculated. The Performance Status Scale (PSS) for Head and Neck Cancer Patients was administered to 25 of the surviving patients. The effects of the method of surgical reconstruction, surgery involving the mandible and/or larynx, and early versus advanced T stage on PSS score were evaluated with the Wilcoxon rank-sum test. Results. Median follow-up in months for IR1, IR2, and IR3 were 83.6, 75.2, and 26.8. The locoregional control rate was 100%, and the rate of distant metastases was 7.5% for all patients. Two-year overall and disease-specific survival rates for the entire study population were 74.7% and 93.6%, respectively. Mean PSS scores by subscales Eating in Public, Understandability of Speech, and Normalcy of Diet were 55 (range, 0,100), 73 (range, 25,100), and 49 (range, 0,100), respectively. PSS scores were significantly higher in patients with primary closure of the surgical defect, no mandibular surgery, and early T-stage lesions. Conclusions. Although functional outcome may be decreased by certain surgical interventions and advanced T stage, the high rate of locoregional and distant disease control and excellent 2-year disease-specific survival supports an aggressive treatment regimen for advanced tongue base cancer. © 2004 Wiley Periodicals, Inc. Head Neck26: 561,572, 2004 [source]


Adjuvant fractionated high-dose-rate intracavitary brachytherapy after external beam radiotherapy in Tl and T2 nasopharyngeal carcinoma

HEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 5 2004
Jiade J. Lu MD
Abstract Background. The value of high-dose-rate intracavitary brachytherapy (HDRIB) for persistent or recurrent nasopharyngeal carcinoma has been well described; however, the benefit of routine adjuvant fractionated HDRIB following external beam radiation therapy (EBRT) has not been completely determined. The objective of this analysis was to evaluate the outcome of two fractions of adjuvant HDRIB treatment in Tl and T2 nasopharyngeal carcinoma. Methods. Thirty-three consecutive and nonselected patients who had Tl and T2 non-disseminated nasopharyngeal carcinoma were treated according to an IRB approved institutional research protocol between March 1999 and July 2001. By the 1997 AJCC cancer staging classification, 22 patients (67%) had Tl disease and 11 patients (33%) had T2 disease. Seventeen of these patients who had stage I or stage II disease (i.e., NO or Nl) were treated with EBRT followed by two fractions of adjuvant HDRIB (group 1); 16 patients who had stage III or stage IV disease (i.e., N2 or N3) were treated with concurrent cisplatin, EBRT and adjuvant HDRIB and subsequent adjuvant cisplatin and fluorouracil (5-FU) chemotherapy (group 2). EBRT was delivered by daily conventional fractionation to a total dose of 66 Gy to the primary tumor. Nodal disease received 66 Gy if it was less than 3cm in maximum diameter and 70 Gy if larger or there was palpable residual disease after 66 Gy. A total of 10 Gy of HDRIB in 2 equal fractions of 5 Gy spaced 1 week apart was delivered starting 1 week after the completion of EBRT. All patients were assessed for treatment response, local control, survival, and toxicity. Results. The median follow up for all 29 surviving patients is 29 months (range: 17,38 months). One patient died 7 months and one died 18 months after radiation therapy from the effects of distant metastases; two died of unrelated causes. At the time of this analysis, one patient (3%) had persistent local disease and one patient (3%) developed pathologically confirmed local recurrence in the nasopharynx. In addition, one patient (3%) developed recurrence only in a neck node followed by distant metastasis, and two patients (6%) developed distant metastasis without locoregional relapse. The 2-year local control rate at the primary site was 93.6%, and the overall survival and disease-free survival rates were 82% and 74% respectively. All patients experienced some degree of acute and/or late toxicity related to radiation therapy. Ten patients (30%) experienced grade 3 acute and/or late toxicity and six patients (18%) developed grade 4 acute and/or late toxicity. No grade 5 toxicity occurred. No unexpected damage of structures within the HDRIB fields was detected. Conclusions. EBRT supplemented by two fractions of adjuvant HDRIB produced a 93.6% local control rate for Tl and T2 nasopharyngeal cancer at 2 years of follow up, with acceptable rates of acute and late toxicity. Brief adjuvant HDRIB appears to permit dose escalation safely, even in patients who receive chemotherapy concurrently with conventional radiation therapy. This strategy needs to be optimized and then tested in a prospective randomized phase III trial to learn if it can improve outcome. © 2004 Wiley Periodicals, Inc. Head Neck26: 389,395, 2004. [source]


Pneumonia: The Demented Patient's Best Friend?

JOURNAL OF AMERICAN GERIATRICS SOCIETY, Issue 10 2002
Discomfort After Starting or Withholding Antibiotic Treatment
OBJECTIVES: To assess suffering in demented nursing home patients with pneumonia treated with antibiotics or without antibiotics. This study should provide the first empirical data on whether pneumonia is a "friend" or an "enemy" of demented patients and promote a debate on appropriate palliative care. DESIGN: Prospective cohort study. SETTING: Psychogeriatric wards of 61 nursing homes in the Netherlands. PARTICIPANTS: Six hundred sixty-two demented patients with pneumonia treated with (77%) or without (23%) antibiotics. MEASUREMENTS: Using an observational scale (Discomfort Scale,Dementia of Alzheimer Type), discomfort was assessed at the time of the pneumonia treatment decision and periodically thereafter for 3 months or until death. (Thirty-nine percent of patients treated with antibiotics and 93% of patients treated without antibiotics died within 3 months.) Physicians also offered a retrospective judgment of discomfort 2 weeks before the treatment decision. In addition, pneumonia symptoms were assessed at baseline and on follow-up. Linear regression was performed with discomfort shortly before death as an outcome. RESULTS: A peak in discomfort was observed at baseline. Compared with surviving patients treated with antibiotics, the level of discomfort was generally higher in patients in whom antibiotic treatment was withheld and in nonsurvivors. However, these same patients had more discomfort before the pneumonia. Breathing problems were most prominent. Shortly before death from pneumonia, discomfort increased. Discomfort was higher shortly before death when pneumonia was the final cause of death than with death from other causes. CONCLUSION: Irrespective of antibiotic treatment, pneumonia causes substantial suffering in demented patients. Adequate symptomatic treatment deserves priority attention. [source]


Specificity and reliability of prognostic indexes in intensive care evaluation: the spontaneous cerebral haemorrhage case

JOURNAL OF EVALUATION IN CLINICAL PRACTICE, Issue 2 2009
Alberto Barbieri PhD MD
Abstract Objective, To determine the reliability of a generic index such as Simplified Acute Physiology Score II (SAPSII), compared with a specific one Intra Cerebral Haemorrhage score (ICH score), as an intensive care unit (ICU) outcome predictor when evaluating a general facility that frequently treats a specific type of patients , those with spontaneous cerebral haemorrhage. Methods, The study cohort consisted of a random sample of patients (81) admitted to Modena's Policlinico Teaching Hospital's ICU with spontaneous ICH over a 24-month period. Main outcome measure, SAPSII, ICH score, overall mortality. Results, The mean ICH score for the 32 surviving patients was 3.41 ± 1.012 while for the 49 deceased patients was of 4.24 ± 0.855 (P = 0.000). The mean SAPSII value for the 32 surviving patients was 49.09 ± 16.58 while for the 49 deceased patients was 49.51 ± 15.93. SAPSII, ICH scores were analysed for mortality, by receiver operating characteristic curves: the area under the curve was significant for ICH, not-significant for SAPSII. Conclusions, Regional quality controls use generic prognostic indexes (SAPSII) in relation to mortality and outcome to assess ICUs, which is appropriate when dealing with a general facility when there is not a predominant type of patient, but it may bias the evaluation if the population with specific pathologies (ICH), not included in the general index, is statistically considerable, leading to an incorrect criticality assessment, an inappropriate strategic plan and the subsequent inefficient resource allocation. [source]


Long-term quality of life of postoperative rectal cancer patients

JOURNAL OF GASTROENTEROLOGY AND HEPATOLOGY, Issue 5 2002
CHISATO HAMASHIMA
AbstractBackground: The long-term quality of life (QOL) of postoperative rectal cancer patients has not been previously investigated in Japan and may vary depending on the surgical technique used (i.e. with or without a stoma). Methods: The Kanagawa Cancer Registry was used to select 348 rectal cancer patients who underwent surgery at the St Marianna University Hospital between 1978 and 1997. Of these, 164 surviving patients were sent a postal survey consisting of the Japanese EuroQol instrument with an additional questionnaire on present symptoms and lifestyle. Results and conclusions: One hundred and ten responses were received, including 38 from stoma patients. The presence of a stoma did not affect the QOL of the male patients, while it did affect that of the female patients. The QOL of the long-term survival group was associated with several items concerning lifestyle, symptoms and usual activity, and the association did not depend on the presence of a stoma. The long-term QOL could be recognized according to the characteristics of rectal cancer patients, independent of the presence of a stoma. © 2002 Blackwell Publishing Asia Pty Ltd [source]


Isolated liver transplantation in infants with end-stage liver disease due to short bowel syndrome,

LIVER TRANSPLANTATION, Issue 7 2006
Jean F. Botha
Infants with short bowel syndrome (SBS) and associated liver failure are often referred for combined liver/intestinal transplantation. We speculated that in some young children, nutritional autonomy would be possible with restoration of normal liver function. Features we believed to predict nutritional autonomy include history of at least 50% enteral tolerance, age less than 2 yr, and no underlying intestinal disease. This report documents our experience with liver transplantation alone in children with liver failure associated with SBS. Twenty-three children with SBS and end-stage liver disease, considered to have good prognostic features for eventual full enteral adaptation, underwent isolated liver transplantation. Median age was 11 months (range, 6.5 to 48 months). Median pretransplant weight was 7.4 kg (range, 5.2 to 15 kg). All had growth retardation and advanced liver disease. Bowel length ranged from 25 to 100 cm. Twenty-three children underwent 28 isolated liver transplants. There were 14 whole livers and 14 partial grafts (five living donors). Seventeen patients are alive at a median follow-up of 57 months (range, 6 to 121 months). Actuarial patient and graft survival rates at 1 yr are 82% and 75% and at 5 yr are 72% and 60%, respectively. Four deaths resulted from sepsis, all within 4 months of transplantation, and 1 death resulted from progressive liver failure. Two allografts developed chronic rejection; both children were successfully retransplanted with isolated livers. Of 17 surviving patients, three require supplemental intravenous support; the remaining 14 have achieved enteral autonomy, at a median of 3 months (range, 1 to 72 months) after transplantation. Linear growth is maintained and, in many, catch-up growth is evident. Median change in z score for height is 0.57 (range, ,4.47 to 2.68), and median change in z score for weight is 0.42 (range, ,1.65 to 3.05). In conclusion, Isolated liver transplantation in children with liver failure as a result of SBS, who have favorable prognostic features for full enteral adaptation, is feasible with satisfactory long-term survival. Liver Transpl 12:1062,1066, 2006. © 2006 AASLD. [source]


Current role of liver transplantation for the treatment of urea cycle disorders: A review of the worldwide English literature and 13 cases at Kyoto University

LIVER TRANSPLANTATION, Issue 11 2005
Daisuke Morioka
To address the current role of liver transplantation (LT) for urea cycle disorders (UCDs), we reviewed the worldwide English literature on the outcomes of LT for UCD as well as 13 of our own cases of living donor liver transplantation (LDLT) for UCD. The total number of cases was 51, including our 13 cases. The overall cumulative patient survival rate is presumed to be more than 90% at 5 years. Most of the surviving patients under consideration are currently doing well with satisfactory quality of life. One advantage of LDLT over deceased donor liver transplantation (DDLT) is the opportunity to schedule surgery, which beneficially affects neurological consequences. Auxiliary partial orthotopic liver transplantation (APOLT) is no longer considered significant for the establishment of gene therapies or hepatocyte transplantation but plays a significant role in improving living liver donor safety; this is achieved by reducing the extent of the hepatectomy, which avoids right liver donation. Employing heterozygous carriers of the UCDs as donors in LDLT was generally acceptable. However, male hemizygotes with ornithine transcarbamylase deficiency (OTCD) must be excluded from donor candidacy because of the potential risk of sudden-onset fatal hyperammonemia. Given this possibility as well as the necessity of identifying heterozygotes for other disorders, enzymatic and/or genetic assays of the liver tissues in cases of UCDs are essential to elucidate the impact of using heterozygous carrier donors on the risk or safety of LDLT donor-recipient pairs. In conclusion, LT should be considered to be the definitive treatment for UCDs at this stage, although some issues remain unresolved. (Liver Transpl 2005;11:1332,1342.) [source]


Kufor Rakeb Disease: Autosomal recessive, levodopa-responsive parkinsonism with pyramidal degeneration, supranuclear gaze palsy, and dementia

MOVEMENT DISORDERS, Issue 10 2005
David R. Williams MBBS, FRACP
Abstract Kufor Rakeb disease is an autosomal recessive disorder characterized by subacute, juvenile-onset, levodopa-responsive parkinsonism, pyramidal signs, dementia, and a supranuclear gaze palsy. It was originally described more than a decade ago, and linkage analysis identified a locus on chromosome 1p36 that was previously assigned PARK9. We have further characterized the clinical picture and specifically re-assessed the response to levodopa in the original family, in the northern highlands of Jordan. In the 4 surviving patients, there has been a narrowing of the therapeutic window for levodopa with the emergence of peak-dose dyskinesias with increased spasticity and cognitive decline. Several new features were identified, including facial-faucial-finger mini-myoclonus, visual hallucinations, and oculogyric dystonic spasms. © 2005 Movement Disorder Society [source]


Is Sanfilippo type B in your mind when you see adults with mental retardation and behavioral problems?,

AMERICAN JOURNAL OF MEDICAL GENETICS, Issue 3 2007
Ute Moog
Abstract Sanfilippo type B is an autosomal recessive mucopolysaccharidosis (MPS IIIB) caused by deficiency of N -acetyl-,- D -glucosaminidase, a lysosomal enzyme involved in the degradation of heparan sulfate. It is characterized by neurologic degeneration, behavioral problems, and mental decline. Somatic features are relatively mild and patients with this disorder can reach late adulthood. It is the most common subtype of MPS in the Netherlands and probably underdiagnosed in adult persons with mental retardation (MR). In order to increase knowledge on the adult phenotype and natural history in Sanfilippo type B, we present the clinical data of 20 patients with this disorder. Sixteen of them were followed for one to three decades. Six died between 28 and 69 years of age, mainly from pneumonia and cachexia; the surviving patients were 18,63 years old. Apart from the youngest, they had lost mobility at 36,68 years. Most had developed physical problems, in particular in the 4th,6th decade of life: cardiac disease (cardiomyopathy, atrial fibrillations), arthritis, skin blistering, swallowing difficulties requiring feeding by a gastrostomy tube, and seizures. The course of the disease was dominated in most of them by challenging behavioral problems with restlessness, extreme screaming and hitting, difficult to prevent or to treat pharmaceutically. Even in absence of knowledge of the history of an elderly patient with MR, the presence of behavioral problems should prompt metabolic investigation for MPS. © 2007 Wiley-Liss, Inc. [source]


Efficacy of Living Donor Liver Transplantation for Patients With Methylmalonic Acidemia

AMERICAN JOURNAL OF TRANSPLANTATION, Issue 12 2007
D. Morioka
Application of liver transplantation to methylmalonic acidemia (MMAemia) is controversial because MMAemia is caused by a systemic defect of methylmalonyl-CoA mutase. The clinical courses of seven pediatric patients with MMAemia undergoing living donor liver transplantation (LDLT) were reviewed. Serum and urinary methylmalonic acid (MMA) levels were found to be significantly decreased after LDLT, whereas serum and urinary MMA levels did not return to normal in any patient. One patient died of sepsis 44 days after LDLT. The other six patients are currently doing well. All patients had preoperative history of acute metabolic decompensation and/or metabolic stroke. However, no episode of acute metabolic decompensation or metabolic stroke was observed postoperatively in any surviving patients. In the preoperative period, all patients showed lethargy and cognitive deficit, both of which were eradicated after LDLT in all surviving patients. Preoperatively, all patients were subjected to dietary protein intake restriction and tube feeding, and were administered several metabolism-correcting medications. The metabolism-correcting medications being administered remained mostly unchanged after LDLT, whereas protein restriction was liberalized and tube feeding became unnecessary in all surviving patients. In addition, physical and neurodevelopmental growth delay remained in all surviving patients during the observation period, which ranged from 4 to 21 months with a median of 10.5 months. [source]


Long-term mortality and retinopathy in type 1 diabetes

ACTA OPHTHALMOLOGICA, Issue thesis1 2010
Jakob Grauslund
The incidence of type 1 diabetes is increasing in Denmark as well as the rest of the world. Due to diabetes-related micro- and macrovascular complications, the morbidity and the mortality is higher among type 1 diabetic patients. The aim of this thesis was to examine a population-based cohort of 727 type 1 diabetic patients from Fyn County, Denmark, with an onset of diabetes before 1 July 1973 in order to: 1,Evaluate the all-cause mortality rates and the influence of sex, duration of diabetes and calendar year of diagnosis in a 33-year follow-up (Paper I). 2,Examine glycaemic regulation, lipids and renal dysfunction as risk factors for all-cause mortality, cardiovascular mortality and IHD (Paper II). 3,Estimate the prevalence of DR as well as the 25-year incidence of PDR and associated risk factors in long-time surviving patients (Paper III). 4,To compare the grading of DR between ETDRS seven standard field 30° stereoscopic colour films and nine field 45° monoscopic digital colour images in long-term surviving patients (Paper IV). In the years 1973,2006 an overall MR of 22.3 per 1000 person-years was found. Furthermore a relative mortality of 3.4 was found as compared to the general population in Denmark. The relative mortality was especially high for patients aged 30,39 (SMR 9.8). There was a tendency towards a better survival for patients diagnosed after 1964. This was especially seen for men. Diabetes was the most common cause of death for those who died in the group. In 1993,1996 blood samples were drawn and glycaemic regulation, lipids and renal markers were subsequently used as predictors of all-cause mortality, cardiovascular mortality and ischaemic heart disease. Glycaemic regulation, dyslipidaemia and creatinine were all significantly associated with all three endpoints. Furthermore, variations in glycaemic control were also identified as a risk factor for overall mortality. Two hundred and one patients were examined for diabetic retinopathy in 1981,1982 and 2007,2008. At follow-up, 97.0% had DR and 42.9% of all patients without PDR at baseline developed this during the follow-up period. Patients who had had a poor glycaemic regulation as well as those who had NPDR at baseline were more likely to develop PDR than the remaining patients. On the other hand, other risk factors such as high blood pressure and proteinuria did not predict PDR. In the comparative study between ETDRS seven standard field 30° stereoscopic colour films and nine field 45° monoscopic digital colour images, 43 eyes of 43 patients were examined in 2008. A poor correlation was found between the two methods: only 29.3% were graded alike. In the remaining, the level of DR was graded higher in the digital photos. Among these, PDR was detected in three eyes using digital photos but remained undetected on all films. This suggests that digital photos with wide fields are the best way to detect DR in long-term type 1 diabetic patients. Overall, it is concluded that mortality is still higher among type 1 diabetic patients. This depends, among other things, on glycaemic regulation, lipid status and, partly, renal dysfunction. Diabetic retinopathy is almost universal in long-term type 1 diabetic patients, and almost half of all patients will develop PDR in 25 years. Nine field digital photos provide the best grading of retinopathy in long-term type 1 diabetic patients. [source]