Intraoperative flow measurement of native liver and allograft during orthotopic liver transplantation in children.

Hepatic hemodynamic changes during liver transplantation (OLT) in children have not yet been studied. We measured intraoperative portal vein flow (PVF) and hepatic arterial flow (HAF) (mL/min) in 53 children and 58 grafts during OLT. Flows were measured in the native organ and in the allograft. In the native liver, PVF and HAF are similar; after transplantation they return to the physiological situation. No flow differences were seen between whole and partial grafts. Among the 8 (14%) portal vein thromboses, PVF was lower in both the native liver and the graft than in the no thrombosis group (P < .05). PVF <5 mL/min/kg was a risk factor to develop PV thrombosis. No graft loss occurred in 3 cases without PVF at the time of OLTs despite the observation that repermeabilization was not possible. In 4 patients with PVF <5 mL/min/kg, after tying a spontaneous spleno-renal shunt (n = 3) or performing a porto-renal vein anastomosis (n = 1), PVF reached >20 mL/min/kg, avoiding thrombosis. In conclusion, PVF and HAF measurements during pediatric OLT may predict patients at high risk for development of PV thrombosis.

Liver transplantation in childhood with more than 10 years of follow-up: analysis of a single-center experience.

UNLABELLED: We pioneered pediatric liver transplantation (OLT) in Spain (June 1985). The aim of this study was to evaluate the current status of our OLT recipients with more than 10 years follow-up. MATERIALS AND METHODS: The 50 patients with >10 years follow-up had a mean age at OLT of 5.6 years with 60% showing a main indication of biliary atresia. All but one (tacrolimus) received cyclosporine. RESULTS: No patient loss occurred among these patients. Eighteen patients had follow-up >15 years and 12 >20 years. The incidence of acute rejection was 56%; chronic rejection, 16%; and lymphoproliferative disorders, 12%. Seven (14%) required retransplantation at a mean of 4.2 years after the first OLT due in four instances to chronic rejection. After 10 years of follow-up, one patient developed portal vein thrombosis and three biliary strictures. All patients remain on immunosuppression. In 64% cyclosporine was switched to tacrolimus or another agent. One patient developed acute rejection at 19.2 years. In 14% of patients the liver function test is abnormal with serum creatinine is >1.5 mg/dL in 10%; one requires insulin and three, antihypertensive drugs. Noncompliance with medications was detected in 10%. Three recipients had offspring. CONCLUSIONS: OLT was an effective treatment with a good quality of life also on long-term follow-up.

Recurrence of hepatocellular carcinoma after liver transplantation.

Outcome after liver transplantation (OLT) clearly depends on recurrence of hepatocellular carcinoma (HCC). After recurrence, patient outcome will depend on the time and site of appearance. The aim of this study was to analyze the therapeutic implications of tumor recurrence behavior. From October 1988 to December 2005, 685 patients received OLT, including 202 due to HCC (32%). We analyzed 28 recurrences (15.2%) among 184 patients who survived at least 3 months (minimum follow-up 1 year). According to the time of recurrence, we divided the patients into early recurrence (ER < 12 months; n = 9; 32.1%) and late recurrence (LR > 12 months n = 19; 67.9%). Actuarial survivals at 1, 5, and 10 years were 82%, 65%, and 50% and disease-free survival, 80%, 58%, and 46%, respectively. Risk factors for recurrence were: vascular invasion (P < .01), bad differentiation (P < .01), and previous hepatectomy (P < .05). After OLT, ER presented at: 5.7 +/- 2.3 months (range 3-10) vs 33.5 +/- 24.3 months (range 12-103) for LR P < .001). Survival postrecurrence (SPR) was shorter: 3.1 +/- 2.4 (range 1-8) months vs 16.4 +/- 14.2 (range 1-5) months (P < .001). Treatment was offered to one ER (11%) and to eight LR (47.1%; P < .05), achieving in these cases longer SPR: 20.1 +/- 14 vs 6.9 +/- 9 months (P < .05). The most common sites of recurrence were liver (n = 7), lung (n = 7), bone (n = 5), adrenal gland (n = 2), peritoneum (n = 2), lymph node (n = 2), skin (n = 2) or cerebral (n = 1). Early recurrences showed short survivals; no treatment could be offered to these patients. Liver recurrence appeared early. In contrast, most lung recurrences appeared later with the possibility of treatment and longer SPR. Bone recurrence appeared later, usually associated with other locations. Treatment was palliative and prognosis was worse. Skin and lymph node recurrences can be treated curatively with prolonged survival. In conclusion, HCC recurrence was difficult to treat curatively and was only prevented by employing restricted criteria.

[Peripheral cholangiocarcinoma: results of surgical treatment]. / Colangiocarcinoma periférico: resultados del tratamiento quirúrgico.

INTRODUCTION: Peripheral cholangiocarcinoma (PC) is an uncommon primary hepatic tumor that represents 10% of hepatic resections for primary malignant tumors in our experience. PATIENTS AND METHODS: From 1988 to 2004, 29 patients with a diagnosis of PC were treated in our unit. One patient was treated with chemoembolization and the remainder underwent surgery. In 7 patients, hepatectomy was not performed due to the presence of an extrahepatic tumor or massive hepatic invasion. The resectability index was 75%. Twenty-one patients underwent radical excision of PC and comprised the study group. RESULTS: The mean age was 60 years with a slight predominance of women. Sixty-two patients were symptomatic and tumoral markers were elevated in 58%. PC developed in normal liver in 15 patients, in cirrhotic liver in 2 patients and in the context of chronic hepatitis in 4 patients. The mean tumoral size was 7 cm (between 1.6 and 13 cm). Multiple tumors were found in 3 patients, invasion of the hepatic hilum lymph nodes was found in 8 patients and vascular invasion was observed in a further 8 patients. Major hepatectomy was performed in 90% of the patients; radical lymphadenectomy of the hepatic hilum was performed in 15 patients and excision of the extrahepatic biliary tract followed by Roux-en-Y hepaticojejunostomy in 4 patients. Operative mortality occurred in 3 patients (14%); one cirrhotic patient died 4 days after surgery from cardiovascular causes and 2 patients died from liver failure after extensive hepatectomies that included resection of the inferior vena cava and suprahepatic veins. Complications occurred in 33% of the patients. Ten patients (47%) died. Of these, 6 died from tumoral recurrence. Tumoral recurrence occurred in 9 patients (5 hepatic and 4 extrahepatic). Hepatic recurrences were treated with radiofrequency ablation in 2 patients and chemotherapy in 5 patients. The median survival was 11 months. Actuarial survival at 1, 3 and 5 years was 60%, 47% and 47% respectively. Disease-free survival at 1, 3 and 5 years was 50%, 31% and 31% respectively. In univariate analysis, significant risk factors for mortality were lymphatic invasion and a resection margin of less than 1 cm. In multivariate analysis, negative factors for tumoral recurrence were lymphatic invasion, satellitosis, and poor tumoral delimitation. CONCLUSION: Surgical treatment of PC through radical hepatic resection with margins of more than 1 cm in patients without nodal invasion provides good results with a 5-year survival of 79%.

Analysis of discarded livers for transplantation.

The aims were to study the causes of nonacceptance of a liver for transplantation after exploration by the donor surgical team and to compare donor characteristics of transplanted and discarded livers. All donor harvesting procedures performed by our unit from 1988 to 2004 were retrospectively studied. Donors were divided in those accepted and transplanted and those discarded by the donor surgical team. The causes of rejection were classified as hepatic and nonhepatic. Donor characteristics of accepted, transplanted livers were compared with those rejected for hepatic reasons. Seven hundred fifty four donor liver procurements were performed: 628 livers were accepted and transplanted (TL), 126 (17.5%) were discarded owing to extrahepatic (n = 16) or hepatic causes (n = 110). Extrahepatic causes were: technical (5.6%), and incidental tumors infection (7.2%). Hepatic causes were: chronic disease or cirrhosis (26.4%), ischemic or septic liver (16.8%), and steatosis (44%). Univariate analysis of donor characteristics showed a significant difference in older age, diabetes, alcohol intake, arterial hypertension, abnormal liver ultrasound (US) exam, and abnormal liver function tests in the group of discarded livers. Obesity and the finding of steatosis in US exam were the only two factors that maintained statistical significance upon multivariate analysis.

Portacaval shunt and inferior vena cava preservation in orthotopic liver transplantation.

The aim was to study the advantages of the use of a temporary portacaval shunt (PCS) with inferior vena cava (IVC) preservation during the piggyback technique for the anhepatic phase of orthotopic liver transplantation (OLT) performed in cirrhotic patients. Two groups of cirrhotic patients who underwent OLT with piggyback technique were compared; one with a PCS (n = 57) and the other, without PCS (n = 54). Patients with fulminant hepatitis, retransplantation, portal thrombosis, and previous portosystemic shunts were excluded. In both groups graft reperfusion was achieved by simultaneous arterial and venous revascularization. Donor, recipient, and surgical characteristics were similar in both groups. The PCS group had a significantly higher portal venous flow (PVF) than the no-PCS group (773 +/- 402 mL/min vs 555 +/- 379 mL/min, P = .004). Therefore, two subgroups were studied; the high PVF subgroup A (>800 mL/min), mean 1099 +/- 261 mL/min, and the low PVF subgroup B (<800 mL/min), mean 433 +/- 423 mL/min. Subgroup A, who were treated with PCS, required fewer blood transfusions and displayed better postoperative renal function; whereas, no differences were observed among subgroup B patients with versus without PCS. In conclusion, the use of a temporary PCS with piggyback technique during OLT in cirrhotics has advantages in patients who still maintain a high portal venous flow.

The major acute phase serum protein in pigs is homologous to human plasma kallikrein sensitive PK-120.

A major acute phase protein (pig-MAP) has been isolated from the sera of pigs after turpentine injection. The protein is the pig counterpart of a recently cloned human serum protein denominated PK-120, which is a putative substrate for kallikrein [Nishimura et al., 1995 FEBS Lett. 357, 207-211]. The protein exists in other mammalian species and it is also an acute phase protein, at least in the rat. Pig-MAP shows homology, as PK-120, with the heavy chain 2 (HC-2) of the inter-alpha-trypsin inhibitor superfamily but does not possess trypsin inhibitory activity.

Unrecognized Tourette syndrome in adult patients referred for psychogenic tremor.

BACKGROUND: The diagnosis of Tourette syndrome may be overlooked in patients with severe psychopathologic disorder but mild motor manifestations of Tourette syndrome. OBJECTIVE: To describe 4 patients with long-lasting general psychopathologic disorder and previously unrecognized mild motor and phonic tics exacerbated during adulthood by the onset of tremor; all of the patients had been referred for the evaluation of psychogenic tremor. SUBJECTS: Four adult patients, with previous psychiatric diagnoses of depression (2 cases), generalized anxiety disorder (3 cases), malingering (1 case), and conversion disorder (3 cases). METHODS: Single case studies. RESULTS: Clinical interviews disclosed that the 4 patients had positive family histories of Tourette syndrome, and all had mild motor and phonic tics that had started before the age of 18 years. On neurologic examination, 2 patients had bilateral postural tremor of the hands that varied in frequency, rhythmicity, and amplitude, and the other 2 had resting tremor mimicking parkinsonism. All 4 patients described involuntary somatic sensations of the affected limbs, which they attempted to alleviate by executing movements. No consistent positive placebo response was observed, but in all patients tremoric movements improved with haloperidol. CONCLUSIONS: These cases illustrate an unusual movement disorder (tremor as a "tic equivalent") in adults with Tourette syndrome and emphasize that cases of the syndrome with mild tics often go unrecognized, precluding adequate treatment.

Prospective study of new-onset seizures in patients with human immunodeficiency virus infection: etiologic and clinical aspects.

OBJECTIVE: To determine the frequency and etiologic and clinical aspects of new-onset seizures in patients with human immunodeficiency virus (HIV) infection. DESIGN: A prospective survey of an HIV-infected patient cohort. SETTING: Outpatients and inpatients in a university hospital in Barcelona, Spain. PATIENTS: Five hundred fifty HIV-infected patients recruited over 1 year. MAIN OUTCOME MEASURE: Analysis of new-onset seizures, with detailed medical history and appropriate workup. RESULTS: Seventeen HIV-infected patients (3%) had a new-onset seizure during the study period. Fourteen (82%) of 17 patients had acquired immunodeficiency syndrome diagnosed according to the 1993 CDC Expanded AIDS Definition. Mean latency (+/-SD) between diagnosis of HIV infection and the first seizure was 60.7+/-37.6 months. Seizure cause was drug toxicity in 8 patients (47%) and intracranial lesion in 6 patients (35.3%). Two patients had seizures related to metabolic derangements. No cause was found in 1 case. The first seizure was generalized in 12 patients (70.6%), simple partial motor seizure in 2 (11.8%), and simple partial seizure evolving to generalized seizure in 3 (17.6%). We found partial seizures in 66.6% of patients who had intracranial lesions. Most patients were treated with phenytoin, which was well tolerated and effective in controlling seizures. CONCLUSIONS: New-onset seizures are infrequent in patients with HIV. In most cases a definite or probable cause is identified, which is usually related to toxic and/or metabolic factors. Most seizures are generalized, and partial seizures suggest a focal cerebral lesion.

Motion of the cerebellar tonsils in Chiari type I malformation studied by cine phase-contrast MRI.

We studied the effects on CSF dynamics at the foramen magnum and the clinical significance of the abnormal tonsillar motion in 14 patients with Chiari type I malformation and 14 control subjects using cine phase-contrast MRI. Dynamic MRI consisted of axial and sagittal cine phase-contrast sequences. CSF and tonsillar motion were qualitatively and quantitatively evaluated, and the subarachnoid space at the foramen magnum measured. In Chiari patients, cine phase-contrast MRI detected the abnormal pulsatile motion of the cerebellar tonsils, which produced a selective obstruction of CSF flow from the cranial cavity to the spine. The amplitude of the tonsillar pulsation and the severity of the arachnoid space reduction were associated with the symptom of cough-strain headache, but not with the presence of syringomyelia. The finding of abnormal valve dynamics of the cerebellar hernia revealed by cine phase-contrast MRI conforms to the pathophysiologic mechanisms suggested in pressure register studies and opens a new possibility in the presurgical assessment of Chiari patients with exertional symptoms.

Arterial line filtration during cardiopulmonary bypass. Neurologic, neuropsychologic, and hematologic studies.

One hundred patients who underwent elective cardiac operations were randomized into two groups. Group 1 had a 20 micron nylon screen filter in the arterial line. In Group 2 no filter was used in the arterial line. Neurologic and neuropsychologic examination of the patients was performed with seven psychometric tests of the Wechsler Memory Scale and the Wechsler Adult Intelligence Scale. These tests were conducted before and a mean of 10 days after the operation. Platelet counts and hemoglobin and total protein levels were measured at the onset and at the end of cardiopulmonary bypass. Two patients died during their hospital stay. Neurologic damage with focal signs was found in the postoperative examination in six patients (three in each group). Twenty-eight patients (28.5%) exhibited signs of mild cerebral dysfunction as assessed by the presence of archaic (primitive) reflexes. Fifteen were in Group 1 and 13 in Group 2 (no significant difference). Sixty patients (61%) had decrements greater than one standard deviation in at least one of the three tests that showed most decreased postoperative scores. There were 32 in Group 1 and 28 in Group 2 (no significant difference). When each test was analyzed individually, there were no significant differences between the two groups. Platelet counts decreased, during cardiopulmonary bypass, a mean of 33% in Group 1 and 34.5% in Group 2 (no significant difference). Both hemoglobin and total protein levels remained virtually unchanged. These results indicate that the addition of a small-pore filter in the arterial line does not prevent the neurologic and neuropsychologic disturbances frequently encountered after cardiopulmonary bypass. The filter itself does not appear to have a direct effect on the blood components. Routine use of an arterial line filter remains questionable.

Outcome of 28 split liver grafts.

UNLABELLED: Our aim is to present our experience with split liver transplantation. From 1992-2002, 14 livers were split to obtain 28 grafts that were transplanted to 12 adults and 16 children. Ex situ splitting was performed in all cases. The left graft consisted of the left lateral segment (segments II-III) in 11 cases and the left lobe in three, depending on the size of the pediatric recipient. Pediatric recipients were of mean age 3, 4 years; mean weight 13 kg; six emergency cases for fulminant hepatic failure or urgent retransplantation and seven of 10 elective cases for biliary atresia. Postoperative mortality rate was 31% (five cases), including four of six emergency cases and one elective case (10%). The main cause was multiorgan failure. Technical complications were: one arterial thrombosis, one portal vein thrombosis, and four biliary complications. Eleven patients are alive and well. Adult recipients were of mean age 53 years. The indications were hepatocellular carcinoma in six cases, liver cirrhosis of various etiologies in five, and one recurrence of hepatitis C in a graft. Two patients died during the postoperative period from sepsis after retransplantation for primary nonfunction of the split graft and multiorgan failure with sepsis. One-year actuarial survival was 84%. CONCLUSIONS: The results of split liver transplantation in elective cases are similar to whole liver transplantation, whereas patient survival among emergency cases is low due to the critical condition of the patients.

Oral ulcerations as the first sign of a foramen magnum meningioma.

We report the case of a patient with oral ulcerations that were likely traumatic in origin caused by biting of the tongue. Physical examination showed only deviation, fasciculations, and hemiatrophy of the right side of the tongue. Neurologic examination disclosed an isolated XII nerve palsy. A magnetic resonance image showed a lesion in the posterior fossa displacing the brain stem at a bulbar level. This was consistent with the diagnosis of a meningioma, later confirmed by the histopathologic study. It is very unusual that a meningioma produces a selective involvement of the XII nerve. Early diagnosis of a foramen magnum meningioma is important, both to improve prognosis and to avoid neurologic sequelae.

[Thoracic stab wounds]. / Heridas torácicas por arma blanca.

Stab wounds are the most common cause of open chest wounds in our setting, with an incidence far higher than either wounds caused by firearms or bull horns. We describe a series of 49 patients, 44 (89.8%) men and 5 (10.2%) women. Mean age was 31 years. The 49 patients had suffered 72 stab wounds to the chest, of which 30 (41.6%) were penetrating and 42 (58.3%) were non penetrating. The lesions observed were 11 (22.4%) cases of pneumothorax, 10 (20.4%) of hemopneumothorax, 6 pulmonary lesions, 2 heart wounds and 1 extensively damaged diaphragm. Twenty-four patients with non penetrating wounds and 8 with penetrating wounds were treated conservatively. It was subsequently necessary to drain the chest of only one. Of the remaining penetrating wounds, drains were inserted in six immediately and 11 underwent surgery. Complications developed in only 9 cases. One patient died as a result of abdominal lesions resulting from stab wounds directly to the abdomen. We are in favor of conservative management. Indications for more aggressive intervention are hypovolemic shock, cardiac tamponade or significant loss of fluid through the thoracic drain.

[Anatomo-clinical study of Behçet's syndrome with involvement of the central nervous system]. / Etude anatomo-clinique d'un syndrome de Behçet, avec atteinte du système nerveux central.

The authors report a patient with Behçet disease who developed severe central neurological disturbances. The M.R.I. was largely coincident with the distribution of the lesions verified in the neuropathological examination, while CT only shows an hypodense lesion in basal ganglia with contrast enhancement. The neurological symptoms did not respond to a treatment with prednisone and azathioprine. The main histopathological findings are similar to those described by previous observers. Reactive histiocytic infiltration with severe lymphocytic phagocytosis, probably related to immunosuppressor treatment, was also observed.

[Hilar and peripheral cholangiocarcinomas]. / Colangiocarcinomas hiliares y periféricos.

Cholangiocarcinoma is the most frequent malignant neoplasm of the bile ducts. It continues to be a diagnostic and therapeutic challenge because of its form of growth and intimate relation with hepatic hilus structures. While cholangiocarcinomas situated distal to the biliary confluence have well-defined characteristics and treatment, cholangiocarcinomas situated in or proximal to the biliary confluence are problematic. A study was made of the so-called intrahepatic cholangiocarcinomas by establishing two types, peripheral and hilar. Their features and therapeutic options are analyzed and published results are reviewed.

[Progressive aphasia without dementia]. / Afasia progresiva sin demencia.

INTRODUCTION: Slowly progressive aphasia without generalized dementia is considered as a degenerative disorder that can be differentiated of others well-known neurodegenerative disorders. CLINICAL CASE: We present a case report of a patient with slowly progressive aphasia, characterized by a progressive anomia and without generalized dementia. The patient was evaluated in the last four years: a neuropsychological assessment, a neurological exploration and structural (MRI) and functional (SPECT) neuroimaging were performed. CONCLUSION: This case is interesting on account of a selective implication of the left temporal hemisphere is showed in the pathogenesis of this disorder.

[Multiple sclerosis epidemiological situation update: pertinence and set-up of a population based registry of new cases in Catalonia]. / Situación epidemiológica actual de la esclerosis múltiple: pertinencia y puesta en marcha de un registro poblacional de nuevos casos en Cataluña.

INTRODUCTION: The first epidemiological studies on multiple sclerosis (MS) around the world pictured a north to south latitudinal gradient that led to the first genetic and environmental pathogenic hypothesis. MS incidence seems to be increasing during the past 20 years based on recent data from prospective studies performed in Europe, America and Asia. This phenomenon could be explained by a better case ascertainment as well as a change in causal factors. The few prospective studies in our area together with the increase in the disease in other regions, justifies an epidemiological MS project in order to describe the incidence and temporal trends of MS. DEVELOPMENT: A prospective multicenter MS registry has been established according to the actual requirements of an epidemiological surveillance system. Case definition is based on the fulfillment of the McDonald diagnostic criteria. The registry setting is the geographical area of Cataluna (northeastern Spain), using a wide network of hospitals specialized in MS management. CONCLUSION: Recent epidemiological studies have described an increase in MS incidence. In order to contrast this finding in our area, we consider appropriate to set up a population based registry.