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Biomedical subjects

G Lock

Publications and source records attributed to G Lock.

69 records · Page 4Linked to original sources

Liver failure in erythropoietic protoporphyria associated with choledocholithiasis and severe post-transplantation polyneuropathy.

In a 58-year-old woman with erythropoietic protoporphyria, asymptomatic liver involvement had been diagnosed 12 years earlier. For more than 20 years the patient had been known to have symptomatic gallstones. A mild polyneuropathy of the lower limbs had been diagnosed several years ago. In December 1992, she presented with colicky upper abdominal pain, dyspepsia and mild jaundice. Diagnosis of beginning cholestasis in erythrohepatic protoporphyria and coincidental choledocholithiasis was made. A causal relation between choledocholithiasis and deterioration of liver function was assumed. Endoscopic extraction of the bile duct stones, however, could not prevent the development of terminal hepatic failure. Biochemically, an excessive protoporphyrinemia and coproporphyrinuria were found. Five weeks after presentation, the patient underwent orthotopic liver transplantation. Immediately after the operation she developed a severe axonal neuropathy with cranial nerve involvement. One year after transplantation, her general condition has markedly improved, but there is still a disabling polyneuropathy. Recently, there were single reports on patients with very similar neurological symptoms following liver transplantation in erythropoietic protoporphyria. This case supports the assumption of a distinct protoporphyrin-induced neural damage in severe hepatic failure.

Alanine Transaminase↗

Conservative treatment of acute hepatic failure.

Treatment of patients with acute liver failure has considerably improved in recent years. Effective treatment of these devastating situations requires early assessment of prognosis and early referral of these patients to specialized centers. The conservative management of extrahepatic complications in intensive care units appears to contribute to a better survival of patients whether or not they are subsequently submitted to a transplantation procedure. Specific hepatocyte-targeted treatment by prostaglandin E2 or similar drugs can be an option in the future. Dramatic progress has been made in the temporary substitution of liver function bridging the time period between liver failure and resumption of hepatocellular function due to liver regeneration. Presently, artificial liver assist devices as well as the extracorporeal perfusion of human or pig livers are evaluated in clinical trials. Initial results indicate that these measures allow to bridge the time until an appropriate donor liver is available. Permanent liver transplantation has been shown to save the lives of many patients suffering from acute liver failure. Selection of patients requiring urgent liver transplantation has been facilitated by the use of prognostic scores specifically adapted to the etiology of underlying liver disease. A temporary auxiliary liver transplantation can be a better treatment option preventing the patient from a life-long dependence upon medical surveillance and drug-induced immunosuppression.

Animals↗

Autonomic and sensorimotor neuropathy in patients with systemic lupus erythematosus and systemic sclerosis.

OBJECTIVE: To determine and compare the prevalence and degree of autonomic (ANP) and sensorimotor neuropathy (SNP) in patients with systemic lupus erythematosus (SLE) and systemic sclerosis (SSc). METHODS: Thirty-one patients with SLE and 19 with SSc were investigated. Pupillary ANP was assessed by pupillometry, cardiovascular ANP using a standardized test battery, and SNP by a standardized clinical examination. RESULTS: In patients with SLE (SSc), 26% (10.5%) had a pathological pupillary latency time and 6.5% (13.7%) had an abnormal maximal pupillary area (p for the difference between latency time and maximal pupillary area in SLE = 0.040). Thus, patients with SLE had more often lesions of the pupillary parasympathetic portion of the autonomic nervous system. Overall prevalence of ANP was not different between the 2 groups; 29.0% in SLE and 21.1% in SSc for pupillary ANP and 9.7 and 15.7% for cardiovascular ANP, respectively. Overall prevalence of SNP was 6.5% in SLE and 21.1% in SSc. Disease activity was correlated with ANP (p = 0.008) and SNP (p = 0.020) in SLE. Kidney involvement was associated with more severe ANP in patients with SSc (p = 0.031). Duration of the disease did not correlate with any type of neuropathy. CONCLUSION: ANP and SNP are often present in SLE and SSc. SLE and SSc differ in the pattern of ANP and SNP, which indicates that different structures and neuropathogenic mechanisms may be involved. Patients with SLE had severe pupillary ANP, probably a sign of central nervous system involvement.

Adult↗

Non-occlusive mesenteric ischemia.

Non-occlusive disease of the mesentery is still a rather underdiagnosed and underestimated condition. It is associated with circumstances that may compromise circulation or the intake of drugs that may lower mesenteric blood flow. Pathophysiologically, a "low flow syndrome" of mesenteric circulation is followed by vasoconstriction; a reperfusion injury may contribute to the ischemic injury. Histopathological changes vary between superficial localized lesions and transmural gangrene. Diagnosis within the initial 24 hours of the development of symptoms is crucial for prognosis but remains a difficult task. Clinical presentation, laboratory tests and ultrasound lack specificity; the role of duplex ultrasound, tonometry and reflectance spectophotometry is still under evaluation. Mesenteric angiography remains the only reliable diagnostic tool and should be applied early in all patients in whom acute mesenteric ischemia is a real possibility. Therapy is aimed at the rapid correction of predisposing and precipitating factors and an effective treatment of mesenteric vasoconstriction. Treatment of choice is a papaverine infusion into the superior mesenteric artery via an angiography catheter. Patients with peritoneal signs have to be treated surgically.

Algorithms↗

Clinical significance of sonographic findings in outpatient clinics.

In the literature substantial controversy exists about the clinical significance of abdominal screening procedures. Therefore, the aim of our prospective study was to determine the clinical relevance and the differences of sonographic findings in both symptomatic and asymptomatic screening outpatients. We therefore systematically analyzed the sonographic findings in 200 consecutive patients of a medical outpatient unit. The patients were divided into 2 subgroups: group I consisted of 128 patients without specific clinical symptoms in the abdomen (screening group); group II included 72 patients with abdominal symptoms and therefore well-defined sonographic requests. The consequences drawn from the sonographic results were analyzed separately for both patient groups. All findings were subdivided into relevant, prospectively relevant or not relevant, depending on their individual influence on the patients' course. In all cases the entire abdomen and basal parts of the thorax were examined. In the 1st group (n = 128/93 findings) 6 findings (6.5%) were relevant, 24 (26%) prospectively relevant and 63 not relevant (67.5%). In contrast, the 2nd group (n = 72/68 findings) contained 13 relevant (19%), 27 prospectively relevant (40%) and 28 not relevant sonographic findings (41%). The differences between group I and II were statistically significant for the relevant findings (2p < 0.05) and the not relevant findings (2p < 0.01). In conclusion, abdominal ultrasonography provides--even for the screening of the outpatient population--in up to 30% additional clinically acute or prospectively relevant information. Therefore, it completes the physical examination adding a substantial amount of clinical information to the medical record of outpatients.

Abdomen↗

[Neuroborreliosis: progressive encephalomyelitis with cerebral vasculitis].

Encephaloborreliosis is described in a 16 year old patient. Besides the progressive encephalopathy this case presents symptoms and findings of a cerebral vasculitis. During and after antibiotic treatment all clinical symptoms and pathological changes in the cerebrospinal fluid almost completely disappeared.

Adolescent↗

Post-traumatic pseudocyst of the spleen: sclerotherapy with ethanol.

We report a case of successful percutaneous treatment of a chronic post-traumatic splenic pseudocyst using alcohol as the sclerosing agent. A 26-year-old man presented with a symptomatic cystic mass located in the spleen. Aspiration of 300 ml of fluid was only temporarily effective, and therefore a drainage catheter was placed 3 days later. After histopathologic and microbiologic exclusion of a malignant or infectious origin, local sclerotherapy with alcohol was performed because of recurrence after percutaneous drainage. This therapy was repeated six times within 2 weeks. Two weeks later, the remaining volume was determined to be 16 ml. Six months after treatment the cyst was no longer visible. To our knowledge this is the first case of a chronic post-traumatic splenic cyst treated with alcohol. Percutaneous sclerotherapy of a symptomatic post-traumatic splenic pseudocyst may be an alternative to surgical treatment.

Adult↗

Auditory event-related cerebral potentials (P300) in hepatic encephalopathy--topographic distribution and correlation with clinical and psychometric assessment.

BACKGROUND/AIMS: Early cognitive disturbances in patients with cirrhosis (Ci) are difficult to assess. Therefore, we evaluated the role of topographic auditory evoked cerebral potentials (P300-EP). METHODOLOGY: Prospective longitudinal study. SETTING: Tertiary clinical care institution. PARTICIPANTS: 45 patients with cirrhosis were compared to 22 healthy subjects. MAIN OUTCOME MEASURES: Hepatic Encephalopathy (HE) was assessed using the clinical grading, standardized psychometric tests, and auditory evoked P300-EP by multichannel EEG recordings. RESULTS: In the patients, the mean P300 peak latency was significantly increased (386.7 +/- 26.7 versus 318.6 +/- 22.2 ms in controls, p < 0.00001). Even in patients with cirrhosis but no HE (n = 18) the P300 peak latency was abnormally prolonged (> 384 ms) in 8 cases (44%). In addition, P180 peak latency was significantly longer in patients with liver cirrhosis as compared to controls (p = 0.021). The maximal P300 amplitude was significantly lowered in patients with liver cirrhosis in the frontocentral and central cortical regions (FZ: p < 0.008; Cz: p < 0.04). Liver function and etiology of liver disease were not related to the increased peak latencies of the P300 and P180 peaks. CONCLUSIONS: P300-EP is a sensitive measure to detect functional cognitive impairment in cirrhotic patients with subclinical HE and clinically apparent HE. Typical changes include latency prolongation and decreased central peak amplitude. Some 40% of patients with no clinical evidence of HE and normal psychometric tests show abnormal results during P300 testing, which is likely to reflect early impairment of cognitive function. Auditory evoked P300 potentials are more sensitive than psychometric testing alone.

Adult↗

Modified serum pancreolauryl test in chronic pancreatitis: evaluation in comparison to endoscopic retrograde pancreatography.

BACKGROUND/AIMS: The aim of this study was to assess the applicability and accuracy of the modified serum pancreolauryl test (sPLT) in patients with chronic pancreatitis (cP). METHODOLOGY: We compared the results of a modified serum pancreolauryl test to morphological changes as detected by endoscopic retrograde pancreatography (ERP) in 60 patients with a history suggesting chronic pancreatitis were compared. Serum fluorescein was measured 30, 60, 120, 150, 180 and 240 minutes after the ingestion of fluoresceindilaureat, a standardized breakfast, and i.v. administration of secretin (1 U/kg) and metoclopramide (10 mg). Furthermore, the results of sPLT and ERP were compared to the findings on abdominal ultrasonography. RESULTS: Forty of 60 patients suffered from cP according to ERP criteria. With a fluorescein cut-off point of 4.5 micrograms/ml, sPLT reached a sensitivity of 68% and a specificity of 50%. According to the ROC curve, the optimal cut-off point was at a fluorescein level of 4.1 micrograms/ml; however, predictive accuracy was only slightly improved at this point. In the subgroup of patients with advanced pancreatic duct changes (n = 23), however, sPLT reached a sensitivity of 87% with 16 patients showing a peak fluorescein concentration below 2.5 micrograms/ml. CONCLUSION: Like other indirect pancreatic function tests, modified sPLT provides good recognition of patients with advanced cP but poor identification of patients with mild or moderate cP, leading to an unsatisfying overall performance of the test.

Adult↗