RESUMEN
AIM: Using a high-resolution 3D endoanal ultrasound, we have observed that some perianal fistulas show a hypoechogenic fistula tract surrounded by a well-defined hyperechogenic area with a thin hypoechogenic edge in patients with Crohn's disease ['Crohn's Ultrasound Fistula Sign' (CUFS)], unlike conventional fistula tracks. The study aimed to determine the prevalence of CUFS in a consecutive series of patients with anal fistula. METHOD: Of 157 patients (median age 45, range 14-86 years, 100 males) with perianal fistula were examined with 3D endoanal ultrasound. All 3D volumes were stored and analysed retrospectively by two independent observers blinded to the clinical information of the patients. RESULTS: There were 29 patients with Crohn's disease of whom 20 (69%) showed CUFS. CUFS was absent in 125 (98%) of 128 patients without Crohn's disease. The positive and negative predictive value of CUFS for Crohn's disease was 87% and 93%, respectively. The kappa value of the two independent observers was 0.77, indicating a substantial interobserver agreement. CONCLUSION: This study provides a new 3D endoanal ultrasound criterion, CUFS, of perianal fistula in patients with Crohn's disease. The sign can be used to discriminate a Crohn's from other types of fistula, which may be useful in the management of patients with anal fistula.
Asunto(s)
Enfermedad de Crohn/diagnóstico por imagen , Imagenología Tridimensional , Fístula Rectal/diagnóstico por imagen , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Estudios de Casos y Controles , Enfermedad de Crohn/complicaciones , Endosonografía , Femenino , Humanos , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Fístula Rectal/etiología , Estudios Retrospectivos , Sensibilidad y EspecificidadRESUMEN
BACKGROUND: An audit of all patients with rectal cancer in Sweden was launched in 1995. This is the first report from the Swedish Rectal Cancer Registry (SRCR). METHODS: Between 1995 and 2003, 13 434 patients treated for adenocarcinoma of the rectum were registered with the SRCR; there were approximately 1500 new patients annually. RESULTS: Approximately half had an anterior resection, a quarter an abdominoperineal resection and 15 per cent a Hartmann's procedure. The median 30-day postoperative mortality rate was 2.4 per cent and the overall postoperative morbidity rate was 35.0 per cent. The 5-year cancer-specific survival rate was 62.3 per cent. The 5-year relative survival rate was 70.1 per cent after anterior resection, 59.8 per cent after abdominoperineal resection and 39.8 per cent after a Hartmann's procedure. The crude 5-year local recurrence rate was 9.5 per cent overall, 6.1 per cent after preoperative radiotherapy and 11.4 per cent after surgery alone. For 3868 patients who had a locally curative procedure the local recurrence rate was 7.4 per cent overall, 5.9 per cent for those who had radiotherapy and 10.2 per cent for those who did not. The local recurrence rate was 2.9 per cent (28 of 968) for stage I disease, 7.9 per cent (112 of 1418) for stage II, 13.9 per cent (188 of 1357) for stage III and 8.5 per cent (45 of 532) for stage IV. CONCLUSION: These good population-based results are due, in part, to the nationwide prospective quality assurance registration.
Asunto(s)
Adenocarcinoma/cirugía , Neoplasias del Recto/cirugía , Adenocarcinoma/mortalidad , Adenocarcinoma/radioterapia , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Recurrencia Local de Neoplasia/mortalidad , Complicaciones Posoperatorias/mortalidad , Neoplasias del Recto/mortalidad , Neoplasias del Recto/radioterapia , Sistema de Registros , Análisis de Supervivencia , Suecia/epidemiología , Factores de TiempoRESUMEN
Operative specimens from various parts of gastroduodenal mucosa were analysed for immunoreactive pancreatic secretory trypsin inhibitor (PSTI) using a peroxidase-antiperoxidase method. Normal gastric mucosa exhibited a varying degree of PSTI immunoreactivity, which was more pronounced in the foveolar cells of gastric mucosa of fundus type than in the non-pepsinogen producing antrum-pyloric mucosa. With the exception of metaplastic Paneth cells and some goblet cells, the intracellular content of PSTI was low in gastric mucosa with intestinal metaplasia. These findings may indicate that a PSTI immunoreactive substance has a role in the normal defence of the gastric mucosa.
Asunto(s)
Duodeno/inmunología , Mucosa Gástrica/inmunología , Inhibidor de Tripsina Pancreática de Kazal/inmunología , Inhibidores de Tripsina/inmunología , Mucosa Gástrica/patología , Humanos , Técnicas para Inmunoenzimas , Mucosa Intestinal/patología , Metaplasia/inmunologíaRESUMEN
Normal and metaplastic gastrointestinal mucosa obtained at surgical resection were studied by light microscopy, using the unlabelled antibody enzyme method for immunohistochemical staining of lysozyme, pancreatic endoproteases, and pancreatic secretory trypsin inhibitor (PSTI). Paneth cells in the mucosa of normal small intestine, gastric mucosa with intestinal metaplasia, and colonic metaplastic mucosa were found to contain anionic trypsin, cationic trypsin, lysozyme, and PSTI immunoreactivity, but not chymotrypsin and elastase immunoreactivity. Normal gastric and colonic mucosa and some goblet cells in the small intestine showed positive PSTI immunoreactivity but no endoprotease immunoreactivity. The presence of immunoreactive trypsin and immunoreactive PSTI in the Paneth cells, which are of secretory type, probably indicates an important extrapancreatic source of these proteins rather than a storage of endocytosed material.
Asunto(s)
Mucosa Gástrica/enzimología , Mucosa Intestinal/enzimología , Páncreas/enzimología , Inhibidores de Tripsina/metabolismo , Adenoma/enzimología , Colitis Ulcerosa/enzimología , Humanos , Inmunoelectroforesis , Técnicas para Inmunoenzimas , Neoplasias Intestinales/enzimología , Muramidasa/metabolismo , Péptido Hidrolasas/metabolismo , Tripsina/metabolismoRESUMEN
Specimens of normal and neoplastic colonic mucosa from 52 patients were analysed by immunohistochemistry using a monospecific polyclonal antiserum against human pancreatic secretory trypsin inhibitor (PSTI). In normal colonic mucosa PSTI was found in the goblet cells in the basal parts of the crypts. In adenomas of tubular, villous, and tubulo-villous types PSTI was also found in the upper parts of the polyps, usually occurring in the regeneration zone. There was a more intense staining reaction in polyps with increased atypia. Carcinomas of different types and of various grades of differentiation and of in situ type did not contain PSTI. These findings indicate that PSTI could be a marker for adenomatous rather than carcinomatous epithelium in the colon. Furthermore, the absence of the inhibitor in malignant cells might facilitate tissue invasion by malignant cells because of deficient protease inhibition.
Asunto(s)
Colon/química , Neoplasias del Colon/química , Inhibidor de Tripsina Pancreática de Kazal/análisis , Adenoma/química , Adenoma/patología , Biomarcadores de Tumor/análisis , Carcinoma in Situ/química , Neoplasias del Colon/patología , Pólipos del Colon/química , Humanos , Inmunohistoquímica , Mucosa Intestinal/químicaRESUMEN
AIMS: To measure the content of immunoreactive human pancreatic secretory trypsin inhibitor (irPSTI) in colonic carcinoma and adjacent normal colonic mucosa. METHODS: From a stable hybridoma cell line producing monoclonal antibodies specific for human PSTI, a specific enzyme linked immunosorbent assay (ELISA) for human PSTI was developed. In a precipitation assay system these antibodies bound human PSTI in a dose-dependent manner. The specimens were obtained from resectional surgery. RESULTS: The content of irPSTI was 19.9 micrograms/g protein (0.55 micrograms/g tissue wet weight) in colonic carcinoma. In adjacent normal colonic mucosa 43.6 micrograms/g protein (1.12 micrograms/g tissue wet weight) was shown. CONCLUSIONS: The enzymatic degradation of surrounding tissue necessary for tumour cell invasion could be facilitated by this relative deficit of the inhibitor in infiltrative carcinoma.
Asunto(s)
Colon/química , Neoplasias del Colon/química , Inhibidor de Tripsina Pancreática de Kazal/análisis , Anticuerpos Monoclonales , Anticuerpos Antineoplásicos , Ensayo de Inmunoadsorción Enzimática , Humanos , Mucosa Intestinal/químicaRESUMEN
Brunner's glands (duodenal glands) in humans are located mainly in the two proximal thirds of the duodenum. They are known to produce and secrete mucin. In recent years, human Brunner's glands have also been shown to express immunoreactivity toward epidermal growth factor-urogastrone (EGF-uro) and lysozyme. These proteins are considered to have a protective function within the gastrointestinal canal. Human pancreatic secretory trypsin inhibitor (PSTI) was recently identified in Brunner's glands. This present study was done by an immunohistochemical method, using monospecific polyclonal antibodies against human PSTI and human lysozyme, respectively. McManus/Alcian blue mucin staining was used to clarify the distribution of mucin. We found immunoreactive PSTI (irPSTI) in seven out of ten specimens. Lysozyme and mucin were present in all ten. While virtually all cells were stained for lysozyme and mucin, irPSTI was restricted to separate lobules and to cells in the ducts.
Asunto(s)
Glándulas Duodenales/enzimología , Inhibidor de Tripsina Pancreática de Kazal/metabolismo , Glándulas Duodenales/metabolismo , Humanos , Inmunohistoquímica , Mucinas/metabolismo , Muramidasa/metabolismoRESUMEN
The aim of this study was to prove the production and secretion of pancreatic secretory trypsin inhibitor (PSTI) in human small intestine. To achieve this we analyzed the content of immunoreactive PSTI (irPSTI) in rinsing fluid from isolated small intestine, using the urea method to estimate the volume of epithelial lining fluid recovered. IrPSTI, measured by an enzyme-linked, immunosorbent assay (ELISA), was present in both free and complexed form. The free PSTI showed intact biologic activity, binding trypsin in stable complexes. The complexed PSTI was dissociated on acidification. With the reverse transcriptase polymerase chain reaction (RT-PCR) and Southern blot hybridization, PSTI mRNA was demonstrated in the mucosa of the ileum. These findings indicate that PSTI is produced and secreted in the small intestinal epithelium and may be part of defence system in intestinal mucosa.
Asunto(s)
Intestino Delgado/metabolismo , Inhibidor de Tripsina Pancreática de Kazal/biosíntesis , Adulto , Anciano , Southern Blotting , Cromatografía en Gel , Cartilla de ADN/química , Ensayo de Inmunoadsorción Enzimática , Femenino , Humanos , Mucosa Intestinal/citología , Mucosa Intestinal/metabolismo , Intestino Delgado/citología , Masculino , Persona de Mediana Edad , Reacción en Cadena de la Polimerasa , ARN Mensajero/análisis , Inhibidor de Tripsina Pancreática de Kazal/genética , Inhibidor de Tripsina Pancreática de Kazal/metabolismoRESUMEN
Secretory leukocyte protease inhibitor (SLPI) is the dominant protease inhibitor in the mucus secretions of the repiratory and genital tracts, and local production seems likely, as immunoreactive SLPI has been found in the corresponding mucosa. To our knowledge, SLPI has not been previously demonstrated in intestinal epithelia or secretions. In an earlier study, however, we found surprisingly high levels of SLPI in peritonitis exudate from patients with gastrointestinal perforations. This study extends these observations by demonstrating the presence of immunoreactive SLPI in intestinal mucosa. In the small intestine, SLPI was present in Paneth cells and in scattered mucosa cells of goblet-type. In normal mucosa of the large bowel, SLPI was also found in scattered cells of goblet-type in the epithelium. In addition, immunoreactive SLPI was frequently found in colonic adenomas. The findings in this study raise several interesting questions on the possible role of SLPI in the gut epithelial defense against inflammatory assaults.
Asunto(s)
Adenoma/metabolismo , Neoplasias del Colon/metabolismo , Mucosa Intestinal/metabolismo , Proteínas/metabolismo , Inhibidores de Serina Proteinasa/metabolismo , Adenoma/patología , Anciano , Neoplasias del Colon/patología , Femenino , Humanos , Inmunohistoquímica , Mucosa Intestinal/patología , Intestino Delgado , Masculino , Persona de Mediana Edad , Proteínas Inhibidoras de Proteinasas Secretoras , Inhibidor Secretorio de Peptidasas LeucocitariasRESUMEN
This study has shown that ileal and colonic mucosa contained roughly 1.2 microg/g of irPSTI. irPSTI from gastrointestinal mucosa eluted in a similar way to that of native PSTI after chromatographic separation and inhibited trypsin in a 1-1 molar way. The PSTI immunoreactive material was localized in the Paneth cells and the goblet cells in the small and large intestine. In normal gastric mucosa it was found in the foveolar cells while the acid and pepsinogen producing cells lacked PSTI immunoreactive material. In gastric mucosa with intestinal metaplasia a marked deficiency of PSTI was found. These findings indicate that gastrointestinal mucosa could be an additional source of irPSTI. Further studies are needed to elucidate if PSTI is involved in the defence of the gastrointestinal mucosa.
Asunto(s)
Mucosa Gástrica/enzimología , Mucosa Intestinal/enzimología , Páncreas/metabolismo , Inhibidores de Tripsina/metabolismo , Humanos , InmunohistoquímicaRESUMEN
The treatment of rectal cancer has changed significantly during the last 30 years. With improved surgical technique and the introduction of preoperative radiotherapy sphincter preserving surgery is now predominant and the rate of local recurrence has been reduced substantially. However, new therapy concepts may also introduce an increased risk of complications. A register to monitor quality control in rectal cancer treatment in Sweden was established in 1995. It covers over 95 per cent of the patients with rectal cancer reported to the Swedish National Cancer Registry. Collection of data and validation are done by six regional oncology centres under supervision of surgeons appointed by the hospitals involved. The results are then collated to a nationwide quality register, enabling regions to compare themselves with other regions, and hospitals with other hospitals.
Asunto(s)
Garantía de la Calidad de Atención de Salud , Neoplasias del Recto/terapia , Análisis Costo-Beneficio , Recolección de Datos/economía , Humanos , Recurrencia Local de Neoplasia , Cuidados Preoperatorios , Garantía de la Calidad de Atención de Salud/economía , Radioterapia Adyuvante , Neoplasias del Recto/economía , Neoplasias del Recto/radioterapia , Neoplasias del Recto/cirugía , Sistema de Registros , SueciaRESUMEN
Gastro-intestinal mucosa obtained at surgical resection was studied by light microscopy using the unlabelled antibody enzyme method for immunohistochemical staining of lysozyme, anionic trypsin, cationic trypsin and pancreatic trypsin inhibitor (PSTI). Paneth cells, identified by their content of lysozyme, contained anionic trypsin, cationic trypsin and PSTI-like immunoreactivity. The demonstration of immunoreactive trypsin and immunoreactive PSTI is a further indication of the resemblance between Paneth cells and the pancreatic acinar cells.
Asunto(s)
Mucosa Gástrica/metabolismo , Mucosa Intestinal/metabolismo , Intestino Delgado/citología , Páncreas/citología , Células Epiteliales , Epitelio/metabolismo , Mucosa Gástrica/citología , Histocitoquímica , Humanos , Técnicas para Inmunoenzimas , Mucosa Intestinal/citología , Intestino Delgado/metabolismo , Muramidasa/inmunología , Coloración y Etiquetado , Inhibidores de Tripsina/inmunologíaRESUMEN
Active human pancreatic cationic trypsin labelled with 131I was administered into the duodenum in nine healthy individuals pretreated with "cold", i.e. non-radioactive iodine. Radioactivity in plasma was measured during 72 hours and was present in plasma after 15 minutes with a maximum 1 hour after administration. The recovered radioactivity in plasma was characterized using dialysis and gel-filtration and the radioactive iodine was found to be in the form of free 131I. Our results are in agreement with recent experimental studies indicating the presence of de-iodinases in the intestine. Furthermore, it was found that pretreatment with "cold" iodine prevented isotope binding to circulating plasma proteins.
Asunto(s)
Radioisótopos de Yodo/metabolismo , Tripsina/metabolismo , Adulto , Cromatografía en Gel , Diálisis , Humanos , Absorción Intestinal , Yodo , Radioisótopos de Yodo/sangre , Persona de Mediana Edad , Páncreas/metabolismo , Tripsina/sangreAsunto(s)
Canales de Calcio/genética , Enfermedades Cerebelosas/genética , Discapacidad Intelectual/genética , Migraña con Aura/genética , Adulto , Atrofia , Enfermedades Cerebelosas/patología , Salud de la Familia , Humanos , Discapacidad Intelectual/patología , Persona de Mediana Edad , Fenotipo , Mutación PuntualRESUMEN
AIM: Mortality after curative surgery for rectal cancer is increased if surgery is not performed within a week of completed short course radiotherapy. A link to the suppression of leucocytes after neoadjuvant radiotherapy has been suggested. This study investigates the effects of radiotherapy on peripheral leucocyte counts, complications and survival. METHOD: Patient data variables from a retrospective database (Local and National Swedish Registries) of a total of 926 consecutive patients treated for rectal cancer disease at two surgical units (1993-2004) were analysed for leucocyte counts and mortality. In all 310 patients received radiotherapy. Mean follow-up time was 2.8 years. RESULTS: There was a marked suppression of leucocytes in the irradiated groups coupled with a reduction in leucocyte response to surgery (p<0.05) compared to non-irradiated patients. Long course radiotherapy resulted in a better postoperative leucocyte response. Irradiated patients with a low post/preoperative leucocyte ratio had higher complication rates. No association between leucocyte response and survival was seen in the irradiated group. CONCLUSIONS: Postoperative leucocytosis is impaired after neoadjuvant radiotherapy, independent of latency period to surgery. Irradiated patients with a suppression of leucocyte response had significantly higher complication rates. The true extent of survival could not be measured in radiotherapy groups due to the short median follow-up period.
Asunto(s)
Recuento de Leucocitos , Neoplasias del Recto/sangre , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Terapia Neoadyuvante , Neoplasias del Recto/mortalidad , Neoplasias del Recto/radioterapia , Neoplasias del Recto/cirugía , Análisis de Supervivencia , Adulto JovenRESUMEN
OBJECTIVE: To describe and classify endosonographic obstetric sphincter defects at 1 week, 3 months and 1 year after primary repair, and to relate the endosonographic results to anal sphincter pressure and to symptoms of anal incontinence over time. METHODS: Forty-one women who had suffered a third- or fourth-degree perineal tear at delivery underwent anal endosonography and anal manometry 1 week, 3 months and 1 year after primary suture of the tear. The extent of the endosonographic defects was described using defect scores ranging from 0 (no defect) to 16 (maximal defect), the score taking into account the location and the longitudinal and circumferential extent of the defect. The women answered a questionnaire with regard to bowel function 1 and 4 years after delivery, the degree of incontinence being expressed as a Wexner score. RESULTS: Some 90% (37/41) of the women had endosonographic defects at 1 week, 3 months and 1 year. The endosonographic defect scores increased significantly between the first and second examinations and then remained unchanged. At 1 year there was a negative correlation between endosonographic sphincter defect score and sphincter pressure. At 1 and 4 years, 54% (22/41) and 61% (25/41) of the women, respectively, had a Wexner score >/= 1. There was a positive correlation between the endosonographic sphincter defect score at 1 week, 3 months and 1 year and the Wexner incontinence score at 1 and 4 years. The endosonographic sphincter defect score at 1 week was the variable that was most predictive of the Wexner score at 4 years (r = 0.48, P = 0.002). CONCLUSION: The higher the endosonographic sphincter defect score after primary repair of an obstetric sphincter tear the lower the sphincter pressure and the higher the risk of anal incontinence.
Asunto(s)
Canal Anal/lesiones , Endosonografía , Incontinencia Fecal/etiología , Complicaciones del Trabajo de Parto/diagnóstico por imagen , Adulto , Canal Anal/diagnóstico por imagen , Canal Anal/patología , Endosonografía/métodos , Incontinencia Fecal/patología , Femenino , Estudios de Seguimiento , Humanos , Manometría , Complicaciones del Trabajo de Parto/patología , Embarazo , Factores de TiempoRESUMEN
OBJECTIVES: To obtain reference data representative of normal findings at anal endosonography in pregnant and non-pregnant women. To determine intraobserver and interobserver agreement in the detection of endosonographic anal sphincter defects in asymptomatic women. METHODS: Twenty-five non-pregnant nulliparous women and 25 non-pregnant parous women (age range, 20-67 years) and 47 pregnant women (age range, 21-39 years) underwent anal manometry and anal endosonography. The endosonographic internal and external sphincter thickness and sphincter length were measured online. Endosonographic sphincter defects were measured and classified offline from videotapes by two independent examiners using an endosonographic defect score ranging from 0 (no defect) to 16 (maximal defect), the score taking into account the location and the longitudinal and circumferential extension of the defect. RESULTS: Endosonographic sphincter thickness and length did not differ between non-pregnant nulliparous and parous women and did not change substantially with age. The anal sphincter was thicker and the anal resting pressure area and manometric sphincter length were greater in pregnant than in non-pregnant women of the same age (20-39 years). There was good intra- and interobserver agreement with regard to detection of endosonographic anal sphincter defects (kappa > or = 0.70). Eighteen (19%) women had endosonographic sphincter defects but in only four (4%; 4/97) cases were they moderate or large (defect score, 7-10). Ten (20%) of the non-pregnant women reported minor gas incontinence and one reported minor incontinence for both gas and liquid stool. The frequency of incontinence did not differ between women with and without sphincter defects. CONCLUSIONS: Reference data representative of normal findings at anal endosonography have been established for non-pregnant women and for nulliparous women in the third trimester of pregnancy. Small endosonographic sphincter defects and minor gas incontinence are common in women without known sphincter trauma. They seem to be unrelated to each other and may be regarded as normal variants.
Asunto(s)
Canal Anal/diagnóstico por imagen , Endosonografía/normas , Ultrasonografía Prenatal/normas , Adulto , Factores de Edad , Anciano , Femenino , Humanos , Persona de Mediana Edad , Variaciones Dependientes del Observador , Paridad , Embarazo , Tercer Trimestre del Embarazo , Valores de ReferenciaRESUMEN
The serum levels of immunoreactive pancreatic secretory trypsin inhibitor (irPSTI) and immunoreactive cationic trypsin (irCT) were measured in 14 patients admitted for severe acute attacks of ulcerative colitis. Patients with acute colitis had significantly higher irPSTI levels in serum (35 micrograms/l) as compared with controls (11.5 micrograms/l), while the serum level of irCT was within normal limits. The normal colonic mucosa recently has been shown to contain irPSTI favoring the theory of an extrapancreatic origin of the increased serum levels of irPSTI in patients with severe active ulcerative colitis.
Asunto(s)
Colitis Ulcerosa/sangre , Inhibidores de Tripsina/sangre , Enfermedad Aguda , Adulto , Anciano , Colitis Ulcerosa/enzimología , Colitis Ulcerosa/inmunología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Tripsina/sangre , Tripsina/inmunología , Inhibidores de Tripsina/inmunologíaRESUMEN
Faecal samples from 16 patients with acute attacks of ulcerative colitis, 7 with quiescent disease, and 8 healthy subjects were studied with regard to extractable amounts of casein digestion capacity, immunoreactive anionic trypsin, cationic trypsin, chymotrypsin, pancreatic elastase, and granulocytic elastase. Patients with acute attacks of colitis had significantly higher levels of casein digestion, pancreatic elastase, and granulocytic elastase in faecal samples than patients with quiescent disease and controls. The non-specific proteolytic activity in faecal extracts from patients with acute colitis was mainly due to the pancreatic proteases anionic elastase, cationic elastase, and anionic trypsin to the granulocytic proteases elastase and neutral protease. These active proteases may cause further destruction of the already damaged mucosa found in patients with severe ulcerative colitis.
Asunto(s)
Colitis Ulcerosa/enzimología , Endopeptidasas/metabolismo , Heces/enzimología , Granulocitos/enzimología , Adulto , Anciano , Cromatografía en Gel , Quimotripsina/metabolismo , Femenino , Humanos , Masculino , Persona de Mediana Edad , Elastasa Pancreática/metabolismo , Radioinmunoensayo , Tripsina/metabolismoRESUMEN
A 31-year-old man with a primary attack of severe acute alcohol-induced pancreatitis presenting with a low to normal amylase activity in serum is described. The diagnosis was confirmed surgically and, further, by studies of immunoreactive trypsin in serum, which was elevated. Analysis of pancreatic isoamylase in serum during the convalescence showed very low activity. The patient is thought to represent a case of pancreatic isoamylase deficiency.