A. Rohner

Acute pancreatitis and normoamylasemia. Not an uncommon combination.

A consecutive series of 352 attacks of acute pancreatitis (AP) was studied prospectively in 318 patients. AP was ascertained by contrast-enhanced CT scan in all but four cases in which diagnosis was made at operation or autopsy. Sixty-seven of these cases (19%) had normal serum amylase levels on admission (i.e., less than 160 IU/L, a limit that includes 99% of control values), a figure considerably higher than generally admitted. When compared to AP with elevated serum amylase, normoamylasemic pancreatitis was characterized by the following: (1) the prevalence of alcoholic etiology (58% vs. 33%, respectively, p less than 0.01), (2) a greater number of previous attacks in alcoholic pancreatitis (0.7 vs. 0.4, p less than 0.01); and (3) a longer duration of symptoms before admission (2.4 vs. 1.5 days, p less than 0.005). In contrast AP did not appear to differ significantly in terms of CT findings, Ransons score, and clinical course, when comparing normo- and hyperamylasemic patients, although there was a tendency for normoamylasemic patients to follow milder courses. Serum lipase was measured in 65 of these normoamylasemic cases and was found to be elevated in 44 (68%), thus increasing diagnostic sensitivity from 81% when amylase alone is used to 94% for both enzymes. A peritoneal tab was obtained in 44 cases: amylase concentration in the first liter of dialysate was greater than 160 IU/L in 24 cases (55%), and lipase was greater than 250 U/L in 31 cases (70%). Twelve of these 44 cases had low peritoneal fluid and plasma concentrations for both enzymes. Thus little gain in diagnostic sensitivity was obtained when adding peritoneal values (96%) to serum determinations. AP is not invariably associated with elevated serum amylase. Multiple factors may contribute to the absence of hyperamylasemia on admission, including a return to normal enzyme levels before hospitalization or the inability of inflamed pancreases to produce amylase. Approximately two thirds of cases with normal amylasemia were properly identified by serum lipase determinations. AP does not appear to behave differently when serum amylase is normal or elevated, and should therefore be submitted to similar therapeutic regimens in both conditions.

Value of Contrast-Enhanced Computerized Tomography in the Early Diagnosis and Prognosis of Acute Pancreatitis A Prospective Study of 202 Patients

Two hundred two patients admitted with the clinical suspicion of acute pancreatitis underwent computerized tomography scanning within 36 hours of admission. The diagnostic value of the computerized tomography findings was excellent, with a sensitivity of 92 percent and a specificity of 100 percent. One hundred seventy-six patients with acute pancreatitis defined according to the overall clinical course were included in the prognostic study. The pancreatitis was fatal in 21 patients, severe in 47 patients, and mild in 108 patients. The computerized tomography findings were classified into the following three groups on the basis of the extent of phlegmonous extrapancreatic spread: Group I, no phlegmonous extrapancreatic spread (100 patients, none died); Group II, phlegmonous extrapancreatic spread in one or two areas (28 patients, mortality rate 4 percent); and Group III, phlegmonous extrapancreatic spread in three or more areas (48 patients, mortality rate 42 percent) (p less than 0.0001). The following three scores from prognostic clinical and laboratory data were also obtained: Score 1, zero or one positive sign (82 patients, none died); Score 2, two to four positive signs (54 patients, mortality rate 13 percent); Score 3, five or more positive signs (40 patients, mortality rate 35 percent) (p less than 0.001). The combination of computerized tomography findings and prognostic signs had the best predictive value. Patients in Group III, Score 3 (24 patients) or Group III, Score 2 (19 patients) had mortality rates of 58 percent and 32 percent, respectively, and complications developed in all of the survivors. In addition, all except two acute pancreatitis patients in whom pancreatic abscess developed were found in Group III (p less than 0.0001). Furthermore, for Group III patients, the prediction of death associated with abscesses was enhanced by the number of prognostic signs. The mortality rate increased from 17 percent for Score 2 patients to 81 percent for Score 3 patients (p = 0.0078). As a result of this study, we recommend early computerized tomography for all Score 2 and Score 3 patients, since it allows prompt recognition of patients at high risk for systemic and local complications. Adequate therapy can then be directed to the group of patients to whom it is best suited. Serial computerized tomographies should be reserved for those patients presenting with phlegmonous extrapancreatic spread.

Early prediction of acute pancreatitis: Prospective study comparing computed tomography scans, Ranson, Glasgow, acute physiology and chronic health evaluation II scores, and various serum markers

The aim of this study was to assess the predictability of the outcome of acute pancreatitis using the Ranson, Glasgow, and Acute Physiology and Chronic Health Evaluation (APACHE) II scores, the computed tomography (CT) scan, and several serum markers. Altogether, 137 consecutive patients with acute pancreatitis confirmed by CT scan were prospectively included. Blood samples were obtained daily for 6 days. The predictive value of each parameter was studied by univariate and multivariate analyses comparing mild and severe pancreatitis. A total of 111 attacks were graded as mild (81%) and 26 as severe (19%). Ranson (p=0.3) and APACHE II (p=0.049) scores appeared insufficiently predictive in the univariate analysis. Pancreatic imaging by CT scan was insufficiently predictive (p>0.05), whereas the presence of extrapancreatic fluid collections was more indicative of outcome (p<0.05). With the univariate analysis, the four most reliable serum markers were pancreatic amylase (p<0.001), neutrophil elastase (p<0.05), albumin (p<0.002), and C-reactive protein (p<0.001). Results became homogeneous when the CT results were added; serum albumin plus extrapancreatic fluid collections (negative predictive value 92%–96% and positive predictive value 67%–100%) comprised the best indicator of severity. None of the parameters tested achieved sufficient predictability when used alone. Serum albumin plus extrapancreatic fluid collections comprise the best indicator of severity at the time of admission.RésuméLe but de cette étude a été d’évaluer dans la pancréatite aiguë la valeur prédictive des scores de Ranson, de Glasgow et d’APACHE II, la tomodensitométrie (TDM) et plusieurs marqueurs sériques. On a inclus dans cette étude de façon prospective 137 patients consécutifs ayant une pancréatite aiguë confirmée par TDM. On a prélevé du sang tous les jours pendant six jours. La valeur prédictive de tous les paramètres a été comparée par analyse uni- et multivariée. En ce qui concerne la gravité de la pancréatite, 111 crises ont été classées «modérées» (81%) et 26, «évères» (19%). La prédiction de gravité par des scores de Ranson (p=0.3) et d’APACHE II (p=0.049) n’étaient pas significativement discriminative en analyse univariée. L’imagerie du pancréas par TDM n’était pas prédictive de façon significative (p τ; 0.05) alors que la présence de collections liquidiennes extra pancréatiques était plus prédictive en ce qui concerne l’évolution (p<0.05). En analyse univariée, les quatre marqueurs les plus fiables étaient ï’amylase d’origine pancréatique (p<0.001), l’élastase neutrophile (p<0.05), l’albumine (p<0.002) et la CRP (p<0.001). Les résultats étaient plus homogènes lorsqu’on a ajouté des données supplémentaires provenant de la TDM: la combinaison d’albumine dans le sérum + collections liquidiennes extrapancréatiques (valeur predictive negative allant de 92 à 96% et valeur predictive positive, de 67 à 100%) était le meilleur indicateur de sévérité. Utilisé seul, aucun des paramètres testés n’était suffisamment prédictif. La combinaison d’albumine dans le sérum + collections liquidiennes extra pancréatiques est le meilleur indicateur de sévérité au moment de l’admission.ResumenEl objetivo de este trabajo fue averiguar el valor diagnóstico en pancreatitis agudas de: las punctuaciones de Ranson, Glasgow y APACHE II, la tomografía axial computarizada (CT) y de diversos marcadores séricos. Se estudiaron prospectivamente 137 pacientes con pancreatitis aguda cuyo diagnóstico fue confirmado mediante CT. El valor diagnóstico de todos los parámetros estudiados se comparó entre pacientes con una pancreatitis leve o grave, mediante análisis uni y multivariante. Se registraron 111 casos de pancreatitis leves (81%) y 26 graves (19%). Las puntuaciones de Ranson (p=0.3) y del APACHE II (p=0.049) no tienen suficiente valor diagnóstico en un análisis uni-variante. La imagen del páncreas obtenida con CT tampoco tuvo valor diagnóstico suficiente (p<0.05) mientras que la existencia de colecciones líquidas extrapancreáticas tuvieron más valor por lo que al pronóstico se refiere (p<0.05). En un análisis univariante los marcadores séricos más fiables fueron la amilasa pancreática (p<0.001) la elastasa de los neutrófilos (p<0.05) la albúmina (p<0.002) y la proteína C reactiva (CRP) (p<0.001). Los resultados se homogeneizaron al añadirse a estos parámetros séricos las imágenes obtenidos por CT. Así, la albúmina sérica + colecciones líquidas extrapancreáticas (valor diagnóstico negativo entre el 92–96% y positivo en el 67–100% de los casos) constituyeron el mejor indicador por lo que a la gravedad se refiere. Ninguno de los parámetros estudiados tienen, por sí solos, valor diagnóstico o pronóstico alguno. La albúmina sérica + colecciones líquidas extrapancreáticas constituyen, al ingreso, el mejor indicador de la gravedad del cuadro pancreático.

Surgery for adult polycystic liver disease

BACKGROUND Occasionally patients with adult polycystic liver disease (APLD) have symptoms. For these patients surgery may represent a valuable therapeutic option to relieve symptoms. METHODS From September 1977 to August 1993 at our institution, 10 women with APLD were examined and surgically treated. They underwent a partial hepatic resection together with cyst fenestration. The surgical outcome and long-term follow-up were retrospectively analyzed. RESULTS Postoperative morbidity consisted of one case of pneumonia, and one case of acute pancreatitis with deep vein leg thrombosis. One patient died after acute Budd-Chiari syndrome developed as a result of liver collapse after fenestration of a posterior cyst. In the long term six of nine patients were symptom free. Late surgical complications included acute cholecystitis (one patient), small bowel obstruction (one), and incisional hernia (two). CONCLUSIONS A combined surgical approach of hepatic resection and cyst fenestration has proved feasible for patients with highly symptomatic APLD. Extensive fenestration of posterior cysts should be avoided; transverse hepatic resection (frontal hepatectomy) up to the costal margin is proposed. This therapy provides good results at long-term follow-up.

Surgical pitfalls in a patient with type IV Ehlers-Danlos syndrome and spontaneous colonic rupture: Report of a case

PURPOSE: This paper intends to stress the importance of early diagnosis and discuss surgical treatment of Type IV Ehlers-Danlos syndrome (EDS-4), an autosomal dominant connective tissue disease characterized by typical features of the face and extremities, inappropriate and easy bruising, and extreme tissue fragility, which may lead to dramatic and often fatal complications, mostly spontaneous arterial or intestinal rupture. METHODS: We report the case of a 41-year-old female who presented with spontaneous perforation of the sigmoid colon. RESULTS: The patient was seen over a nine-year period, during which time she required six operations and presented with a great number of surgical complications including stenosis of an end-colostomy, repeated subocclusive episodes caused by intraperitoneal adhesions, and enterocutaneous fistulas, finally ending with an ileostomy and short bowel syndrome. It is only after a difficult laparotomy for ovarian cyst excision, marked by numerous adhesions and friable bowel, that the diagnosis of EDS-4 was considered and established. CONCLUSIONS: In case of “idiopathic” spontaneous perforation of the colon in a young adult, features of EDS-4 should be thoroughly looked into and, if found, skin fibroblast culture with collagen Type III analysis performed. The surgical treatment of choice consists of subtotal colectomy and permanent endileostomy. In case of patient refusal, a second-stage ileorectal anastomosis can be performed but carries the high risk of anastomotic leakage.

Can serum and peritoneal amylase and lipase determinations help in the early prognosis of acute pancreatitis

Serum and peritoneal amylase and lipase levels were determined at an early stage in 73 patients with acute pancreatitis confirmed by computed tomography (CT scan), surgery, and/or postmortem. Each patient was given an enzymatic score (ES), which reflects the predominance of the serum or peritoneal concentration of the two enzymes, as the case may be. This score can thus be either 0, 1, or 2; ES = 0 if neither enzyme is predominant in the peritoneal fluid, ES = 1 if amylase or lipase alone are predominant therein, and ES = 2 if both enzymes are predominant. This enzymatic score appears to be a good indicator of severity of disease, being as it is directly and significantly related to mortality rate, prognostic score as proposed by Ranson, and incidence of extrapancreatic spreads as demonstrated by CT scan. In 38 patients (including two fatalities) with an enzymatic score of 0 or 1, mortality was 5%, whereas in 35 patients (10 fatalities) with ES = 2, mortality was 29% (p less than 0.01).

Colon cancers and peritoneal mesothelioma occurring 29 years after abdominal radiation for testicular seminoma. A case report and review of the literature

Secondary malignancies represent an increasing problem for long survivors of primary malignancies treated by chemo- and/or radiotherapy. The occurrence of secondary myelodysplasia and leukaemias after treatment for Hodgkin’s disease is well established. Secondary solid tumors are mostly observed following radiation therapy. We report the case of a patient who presented 3 abdominal solid malignancies within 3 years, 29 years after abdominal radiotherapy for a testicular seminoma, namely 2 colon cancers and a peritoneal mesothelioma. Both types of cancer are independently reported in the literature to be more frequent in patients with a history of abdominal radiation than in the general population. To our knowledge there is no other reported case with 3, nearly simultaneously occurring separate solid tumors, which could all be related to former abdominal irradiation. Such a radiotherapy-related long-term side effect should be taken into account when considering adjuvant radiotherapy in patients with low-risk stage I testicular seminoma.

Determination of pancreatic carboxypeptidase A in human blood serum

A method has been designed for the assay of pancreatic carboxypeptidase A in blood serum. It uses Z-Gly-Phe as the substrate and fluorimetric determination of the released phenylalanine in an amino acid analyser, which yields a measure of free carboxypeptidase A. In addition, the sum (free carboxypeptidase A + procarboxypeptidase A) can be determined on a second portion preincubated with trypsin, which converts the proenzyme to the active form. Determinations made in fifteen healthy individuals showed the presence of a measurable concentration of free carboxypeptidase A. In acute pancreatitis, total carboxypeptidase A is raised. An increase in circulating proenzyme is observed in some cases. Data from 46 patients show a good correlation between total carboxypeptidase A, lipase and immunoreactive trypsin. Differential determination of procarboxypeptidase A and free carboxypeptidase A provides an interesting new tool for the diagnosis of pancreatic disorders.

Spontaneous Retroperitoneal Hemorrhage due to Adrenal Metastasis for Non-Small Cell Lung Cancer Treated by Radiation Therapy

Adrenal hemmorhage is not a rare event. It is mostly bilateral, consecutive to anticoagulation therapy, bleeding diathesis and trauma ( I ) . and also observed in severe stress situations with excessive adrenocortical stimulation, such as acute myocardial infarction, congestive heart failure, and specticemia (2). Massive unilateral hemorrhage is uncommon, but is a recognized complication of primary adrenal tumors, among which pheochromocytoma is most commonly encountered (3, 4). Adrenal involvement by metastatic neoplasms is a frequent occurence in some malignancies such as carcinoma of the lung (2, 5). These lesions are usually discovered incidentally during routine cancer work-ups or in postmortem studies (6). Hemorrhagic complications are exceedingly rare, but can be dramatic and are mostly managed surgically. We report here one case of retroperitoneal hemorrhage secondary to metastatic involvement of the left adrenal by a non-small cell carcinoma of the lung, which was successfully treated by transfusion and radiation therapy. Case repor/. A 56-year-old woman was admitted in June 1993 for investigation of unexplained weight loss and development of paresthesia and pains in her left arm and leg. Clinical examination was positive for a right axillary adenopathy and hypoesthesia of her left lower and upper limbs with a homolateral Babinski sign. Routine laboratory test was within normal ranges. Chest x-ray and thoracic CT-scan revealed a left upper lobe nodule (2 cm in diameter). Bronchoscopy showed a stenosis of a segmentary apical bronchus in the left lung and biopsy was positive for a poorly differentiated squamous cell carcinoma. A left adrenal mass of 5.4 cm in diameter was present on abdominal CT-scan being consistent with metastasis (Fig. la). A cerebral CT-scan showed 3 right hemispheric lesions. During her stay at hospital the patient suddenly presented with diffuse abdominal pain and nausea complicated by an abrupt hemoglobin drop on July 5th (Table 1). requiring transfusion of 4 blood units on the same day. On clinical examination, left upper abdominal tenderness and fever ( 38.4-C) were recorded. A second abdominal CT-scan revealed a change in radiodensity on the left adrenal mass with massive bilateral retroperitoneal infiltration predominating on the left side compatible with hemorrhage (Fig. I b and c). The diagnosis of spontaneous hemorrhage from the left adrenal metastasis was made and was coroborated by a transient rise of the platelets and the bilirubin over the following days (Table). No abnormality in prothrombin time, partial thromboplastin time and fibrinogen level was recorded. A radiation treatment of the adrenal was promptly started with a total dose of 30 G y in 10 daily fractions. The patient’s condition improved and she did not require further blood transfusion. She was discharged shortly thereafter and treated as an outpatient by radiotherapy and corticosteroi‘ds for her cerebral metastasis. Her condition deteriorated progressively and she died at home 2 months later. A postmortem examination was not performed Discussion. Spontaneous massive hemorrhage from neoplastic lesions is a relatively uncommon problem. It can arise from tumors, primary or metastatic, infiltrating the mucosa of the gastro-intestinal, genito-urinary and respiratory tracts (7). Spontaneous bleeding from an extraluminal lesion is an infrequent event, which has been described in metastatic melanoma, hepatoma, glioblastoma and, more rarely, in the context of hepatic metastasis from various tumors (8, 9). Although the hemorrhage in an extraluminal site is often self-contained it can induce acute symptoms because of the compression of the surrounding normal tissues, such as convulsions in cases of cerebral lesions (10). The retroperitoneum is an expandable space which can easily accomodate large amounts of blood, leading to significant blood loss and shock. The clinical manifestations of spontaneous retroperitoneal hemorrhage (SRH) are variable and nonspecific, the most frequently presenting features being abdominal pain, a tender flank mass and signs of hemorrhagic shock (Lenk’s triad) ( 1 I ). Among the many causes of SRH. tumors come far behind ruptured vascular aneurysms (1). Benign as well as primary malignant tumors have been reported to cause SRH (12, 13). Renal cell carcinoma (RCC) is admitted to be the first renal cause of SRH although the occurrence of such an event is still rare. A large review involving 309 cases of RCC reported only one case of SRH ( I ) . Benign angiomyolipomas of the kidney areconsidered to be thesecond most frequent tumoral cause of SRH (14). In the adrenals, pheochromocytomas, which are highly vascular tumors, are known to cause SRH. However, SRH due to retroperitoneal metastatic disease as in our patient is exceedingly rare. In the English medical literature

Early Prediction in Acute Pancreatitis: the Contribution of Amylase and Lipase Levels in Peritoneal Fluid

CONTEXT Predicting the severity of acute pancreatitis early in the course of the disease is still difficult. OBJECTIVE The value of amylase and lipase levels in serum and peritoneal fluid might be of value in predicting the course of acute pancreatitis. DESIGN Prospective study. PATIENTS One-hundred and sixty-seven patients with acute pancreatitis as confirmed by computed tomography scan within 24 hours of admission were studied. MAIN OUTCOME MEASURES Each patient was given an enzymatic score which reflected the predominance of serum or peritoneal levels of amylase and/or lipase. Enzymatic score was 0 if neither enzyme was predominant in the peritoneal fluid, 1 if amylase or lipase alone were predominant and 2 if both enzymes were predominant. The predictive value of the enzymatic score or computed tomography scan for a severe attack was determined. RESULTS One-hundred and thirty-three attacks were graded as mild (79.6%) and 34 were considered as severe (20.4%). The frequency of severe acute pancreatitis significantly increased as the enzymatic score increased (5.4%, 12.5%, and 31.7% in 0, 1, and 2 enzymatic score patients, respectively; P<0.001). An enzymatic score greater than 0 predicted a severe outcome in 32 of 34 patients (sensitivity 94.1%, specificity 26.3%), whereas an enzymatic score of 2 predicted a severe attack in 26 of 34 patients (sensitivity 76.5%, specificity 57.9%). Edema on computed tomography scan was found in 97 of 129 mild attacks (specificity 75.2%) and necrosis in 25 of 33 severe attacks (sensitivity 75.8%), whereas all patients with severe attacks exhibited extrapancreatic acute fluid collection (sensitivity 100%, specificity 34.9%). CONCLUSIONS Peritoneal dialysis is less predictive and more cumbersome than a computed tomography scan in the early prediction of acute pancreatitis.