Whipple's disease in Spain: a clinical review of 91 patients diagnosed between 1947 and 2001.

BACKGROUND: To determine the epidemiological, clinical, diagnostic and therapeutic characteristics of Whipple s disease in Spain. PATIENTS AND METHOD: Cases of Whipple s disease reported in the Spanish literature between 1947 and 2001 which meet histological or PCR criteria are reviewed. RESULTS: 91 cases were included, 87.5% of which were male. The maximum incidence was between 40 and 60 years of age (68%). There was no family clustering or susceptibility by profession or surroundings. The most common symptoms and signs were: weight loss (80%), diarrhoea (63%), adenopathies (35%), skin problems (32%), abdominal pain (27%), fever (23%), joint problems (20%) and neurological problems (16%). Arthralgias, diarrhoea and fever were noted prior to diagnosis in 58, 18 and 13% of patients, respectively. Diagnosis was histological in all cases except two, which were diagnosed by PCR. Intestinal biopsy was positive in 94%. Adenopathic biopsies (mesenteric or peripheral) were suggestive in 13% of cases, and treatment was effective in 89%. There were nine relapses, four of which were neurological, although all occurred before the introduction of cotrimoxazole. CONCLUSIONS: Whipple s disease is not uncommon, although it requires a high degree of suspicion to be diagnosed in the absence of digestive symptoms. The most common and most sensitive diagnostic method is duodenal biopsy. PCR is beginning to be introduced to confirm the diagnosis and as a therapeutic control. Initial antibiotic treatment with drugs that cross the blood-brain barrier, such as cotrimoxazole and ceftriaxone, is key to achieving a cure and avoiding relapses.

Enfermedad deWhipple en España. Revisión clínica de 91 pacientes diagnosticados durante 1947-2001 / Whipple’s disease in Spain: a clinical review of 91 patients diagnosed between 1947 and 2001

Fundamento: conocer las características epidemiológicas, clínicas,diagnósticas y terapéuticas de la enfermedad de Whipple enEspaña.Pacientes y método: se revisan los casos de enfermedad deWhipple de la literatura española que cumplen criterios histológicoso de PCR desde 1947 hasta 2001.Resultados: se incluyeron 91 casos. El 87,5% eran hombres.La incidencia máxima fue entre los 40 y 60 años de edad (68%).No hubo agregación familiar ni preferencia por profesión o entornoambiental. Los síntomas y signos más habituales fueron: adelgazamiento(80%), diarrea (63%), adenopatías (35%), cutáneos(32%), dolor abdominal (27%), fiebre (23%), articulares (20%) yneurológicos (16%). Artralgias, diarrea y fiebre se referían previamenteal diagnóstico en el 58, 18 y 13% de los enfermos, respectivamente.El diagnóstico fue histológico en todos salvo en dosque se diagnosticaron por PCR. La biopsia intestinal fue positivaen el 94%. Las biopsias de adenopatías (mesentéricas o periféricas)fueron orientadoras en un 13%. El tratamiento fue eficaz enel 89%. Hubo 9 recidivas, 4 neurológicas, estas antes de la introduccióndel cotrimoxazol.Conclusiones: la enfermedad de Whipple no es tan infrecuente.Se precisa un alto índice de sospecha para diagnosticarlaen ausencia de síntomas digestivos. El método diagnóstico másempleado y más sensible es la biopsia duodenal. Se empieza a introducirla técnica de PCR para confirmar el diagnóstico y comocontrol terapéutico. El tratamiento antibiótico inicial con antibióticosque pasan la barrera hematoencefálica como cotrimoxazol yceftriaxona es determinante para la curación de los pacientes yevitar las recidivas (AU)

Background: to determine the epidemiological, clinical, diagnosticand therapeutic characteristics of Whipple’s disease in Spain.Patients and method: cases of Whipple’s disease reportedin the Spanish literature between 1947 and 2001 which meet histologicalor PCR criteria are reviewed.Results: 91 cases were included, 87.5% of which were male.The maximum incidence was between 40 and 60 years of age(68%). There was no family clustering or susceptibility by professionor surroundings. The most common symptoms and signswere: weight loss (80%), diarrhoea (63%), adenopathies (35%),skin problems (32%), abdominal pain (27%), fever (23%), jointproblems (20%) and neurological problems (16%). Arthralgias, diarrhoeaand fever were noted prior to diagnosis in 58, 18 and13% of patients, respectively. Diagnosis was histological in all casesexcept two, which were diagnosed by PCR. Intestinal biopsywas positive in 94%. Adenopathic biopsies (mesenteric or peripheral)were suggestive in 13% of cases, and treatment was effectivein 89%. There were nine relapses, four of which were neurological,although all occurred before the introduction of cotrimoxazole.Conclusions: Whipple’s disease is not uncommon, althoughit requires a high degree of suspicion to be diagnosed in the absenceof digestive symptoms. The most common and most sensitivediagnostic method is duodenal biopsy. PCR is beginning to beintroduced to confirm the diagnosis and as a therapeutic control.Initial antibiotic treatment with drugs that cross the blood-brainbarrier, such as cotrimoxazole and ceftriaxone, is key to achievinga cure and avoiding relapses (AU)

Porfiria cutánea tarda: asociación con mutaciones HFE, hepatitis virales, alcohol y otros factores de riesgo en Guipúzcoa, País Vasco / No disponible

Objetivo: estudiar la frecuencia de las mutaciones en el genHFE (C282Y, H63D, S65C) en un grupo de 54 pacientes conporfiria cutánea tarda (PCT) y en un grupo de controles sanos (donantesde sangre) en Guipúzcoa. También analizar su relación conlos virus de la hepatitis B y C (VHB, VHC), alcohol y otros factoresde riesgo reconocidos.Métodos: el análisis de las mutaciones se hizo mediante PCR.Se compararon las frecuencias alélicas y genotípicas. Se determinaronla probabilidad y el test de Chi cuadrado.Resultados: no encontramos asociación entre C282Y y PCT(5,76 vs. 5% controles). Se observó una alta frecuencia alélica enla mutación H63D en PCT (34,25%), pero sin ser estadísticamentesignificativa (controles 29,31%), debido a la alta prevalencia deesta mutación en la población vasca. La mutación S65C fue menoren PCT que en controles. Encontramos una idéntica presenciade H63D en heterocigosis en ambos grupos (38,8 vs. 38,8%).La asociación con el VHC se objetivó en el 35,18% de los pacientesy la infección por VHB en el 7,4%. Un 55,55% de los pacientestenía un hábito alcohólico de más de 60 g etanol día. Todoseran negativos para el virus de la inmunodeficiencia humana (VIH)y 1 de las 5 mujeres con PCT tomaba estrógenos.Conclusión: las mutaciones C282Y y H63D no tienen un papelrelevante en los pacientes con PCT en Guipúzcoa. Los factoresexternos (consumo importante de alcohol y VHC) parecen jugarun papel fundamental en el desarrollo de la PCT en nuestrapoblación

Aim: to study the frequency of HFE gene mutations (C282Y,H63D, S65C) in a group of 54 sporadic PCT patients and in agroup of healthy controls (blood donors) from Guipúzcoa, Spain.We studied the association of PCT with HCV, HBV, alcohol abuse,and other established risk factors.Methods: the analysis of mutations was made by PCR. Allelicand genotypic frequencies were compared. Probability was determinedand a Chi-squared test was performed.Results: no association was observed between C282Y mutationand PCT (5.76 vs. 5% in controls). A high H63D mutationfrequency was observed in PCT (34.25%) but was not statisticallysignificant (controls 29.31%) because of the high prevalence ofthis mutation in the Basque general population. The S65C mutationwas lower in PCT than in controls. There is a similar presencefor H63D heterozygosis in PCT (38.8 vs. 38.8%). HCV associationwas observed in 35.18% of patients with PCT. HBVinfected 7.4% of patients. Heavy alcohol intake (> 60 g/day) waspresent in 55.55% of patients. No HIV-infected patients were detected.The study of other risk factors revealed only one of the fivewomen with PCT taking estrogens.Conclusion: our results found no relevant role for C282Yand H63D mutations. External factors such as HCV and alcoholcould be determinant in the development of PCT in the Basquepopulation

[Porphyria cutanea tarda. An analysis of HFE gene mutations, hepatitis viruses, alcohol intake, and other risk factors in 54 patients from Guipúzcoa, Basque Country, Spain]. / Porfiria cutánea tarda: asociación con mutaciones HFE, hepatitis virales, alcohol y otros factores de riesgo en Guipúzcoa, País Vasco.

AIM: To study the frequency of HFE gene mutations (C282Y, H63D, S65C) in a group of 54 sporadic PCT patients and in a group of healthy controls (blood donors) from Guipúzcoa, Spain. We studied the association of PCT with HCV, HBV, alcohol abuse, and other established risk factors. METHODS: The analysis of mutations was made by PCR. Allelic and genotypic frequencies were compared. Probability was determined and a Chi-squared test was performed. RESULTS: No association was observed between C282Y mutation and PCT (5.76 vs. 5% in controls). A high H63D mutation frequency was observed in PCT (34.25%) but was not statistically significant (controls 29.31%) because of the high prevalence of this mutation in the Basque general population. The S65C mutation was lower in PCT than in controls. There is a similar presence for H63D heterozygosis in PCT (38.8 vs. 38.8%). HCV association was observed in 35.18% of patients with PCT. HBV infected 7.4% of patients. Heavy alcohol intake (> 60 g/day) was present in 55.55% of patients. No HIV-infected patients were detected. The study of other risk factors revealed only one of the five women with PCT taking estrogens. CONCLUSION: Our results found no relevant role for C282Y and H63D mutations. External factors such as HCV and alcohol could be determinant in the development of PCT in the Basque population.

[Fasciola hepatica. study of a series of 37 patients]. / Fasciolasis hepatobiliar. Estudio de una serie de 37 pacientes.

BACKGROUND: To analyze the clinical, bacteriologic, diagnostic and therapeutic features of patients with Fasciola hepatica (FH) in our hospital. PATIENTS AND METHOD: We reviewed all the patients with a diagnosis of fascioliasis from 1975 to 1999 in the Aranzazu Hospital in San Sebastián (Guipúzcoa, Spain). Diagnosis was made by direct vision of adult parasites during surgery and/or the presence of ova in stool examinations and/or positive serologic tests in patients with symptoms consistent with parasitosis. RESULTS: Thirty-seven patients were included (23 men and 14 women), aged 19 to 71 years. Ingestion of watercress was confirmed in 27. Seven cases occurred as part of familiar outbreaks. Thirty-two were in the liver invasive stage and in 5 the biliary tree was invaded. The most common features were eosinophilia (91.8%), malaise and weight loss (75.6%), elevated alkaline phosphatase (74.2%), and abdominal pain (72.9%). Adult worms in the biliary ducts were observed in 3 patients and ova in feces were observed in 6. In 13 of 27 patients indirect hemagglutination test was ( 1/1,280. Data significant to confirmation of liver involvement were provided by laparoscopy in 12 of 13 patients and by imaging techniques in 13 of 31 patients. Four patients had cholelithiasis and of these, 2 also showed adult parasites in the common bile duct. Three patients underwent surgery. Therapy with dehydroemetine and/or bithionol was followed by complete remission in 30 patients, although 6 required repeat treatment cycles. The remaining 4 patients were cured by praziquantel. CONCLUSIONS: Most of the patients in these series reported consumption of watercress and all patients showed the symptoms typical of parasite disease. Imaging techniques proved to be of great utility in confirming the diagnosis of hepato-biliary disease. In most of the patients therapy with dehydroemetine and/or bithionol (in one or several cycles) was followed by complete remission.

[Ehlers-Danlos syndrome associated with acute pancreatitis]. / Síndrome de Ehlers-Danlos asociado a pancreatitis aguda.

Ehlers-Danlos syndrome is an inherited connective tissue disease characterized by articular laxity, hyperelastic skin and tissue fragility. The syndrome is genetically, biochemically and clinically heterogeneous and several well-defined subtypes have been identified. We describe a patient with the type IV variant of this syndrome who developed acute spontaneous pancreatitis.

An anterograde degeneration study of the distribution of regenerating rat myelinated fibers in the silicone chamber model.

Specificity of reinnervation after a peripheral nerve lesion has given rise to considerable controversy. As a contribution to solving this issue we have evaluated the specificity of reinnervation of the peroneal nerve after a complete transection of the sciatic nerve repaired with an 8 mm silicone tube, leaving a 4 mm gap between the nerve stumps. Our findings reveal unspecificity of reinnervation of the distal peroneal branch. This lack of specificity is shown by a random distribution of fibers originating from both proximal branches at the level of the tube and at distal peroneal and tibial branches, argue against specificity of regeneration in this model.

Estado del arte de la participacion en salud ocupacional de los trabajadores en Colombia desde 1976 / State of Art of teh occupational health workers participation in Colombia since 1976

Tradicionalmente la persona encargada de la salud ocupacional, se siente responsable de controlar los factores de riesgo a los cuales está expuesto el trabajador quien asume un papel pasivo. Aun cuando este tipo de intervención es necesaria, se requieren cambios de actitud del trabajador y la organización laboral, que contribuyan al empoderamiento del trabajador respecto a su salud. Para esto son esenciales los procesos participativos. Por lo anterior se considera importante realizar una recapitulación crítica de la participación en salud de los trabajadores, conscientes de la necesidad de un cambio en los procesos participativos, que los involucren como sujetos activos en la determinación de procesos que promuevan, mejoren y conserven su salud, pues son ellos los que poseen la vivencia y conocimiento acerca de las condiciones de trabajo y las repercusiones en ella. Con este estado del arte, se busca recopilar la información existente, teniendo en cuenta los antecedentes históricos, tanto de la participación comunitaria como la de los trabajadores; un marco de referencia que contempla la conceptualización de la participación; las formas y tipos de participación; la intervención de la educación como proceso de participación; la participación vista como un derecho y deber; los modelos de participación; el marco constitucional y legal y los actores sociales en nuestro contexto. Finalmente, en una fase reflexiva - crítica de la documentación se pretende analizar los fenómenos hallados, con el fin de develar como se presentan y los intereses y las tendencias de los mismos. Como último aporte se elabora una base de datos de la participación, útil para próximas investigaciones relacionadas con el tema

AIDS-associated cholangiopathy in a series of ten patients.

OBJECTIVE: To present the clinical, biological, radiologic and cholangiographic findings in patients with clinical suspicion of AIDS associated cholangiopathy in our hospital. PATIENTS AND METHODS: We have revised the clinical charts of 10 patients admitted in our hospital from 1991 to 1995. RESULTS: Ultrasonography and/or abdominal CT were carried out on all the patients. Biliary tract dilatation was observed in 11 cases. From the 12 ERCP, biliary tract was fulfilled in 11. In 5 cases papillary stenosis was diagnosed, sclerosing cholangitis in 2, normal biliary tract in 3 and acute cholangitis in one case. Sphincterotomy was done in 5 patients, with clinical improvement. In one case, another sphincterotomy was needed because of reestenosis. CONCLUSIONS: ERCP is very important in the diagnosis of AIDS associated cholangiopathy. Endoscopic sphincterotomy relieves abdominal pain in these patients. Cholangiopathy occurs in very immunocompromised HIV positive patients. Survival is very short.

Treatment of achalasia with botulinum toxin.

UNLABELLED: We studied the efficacy of local injections of botulinum toxin in the treatment of patients with achalasia. Four patients diagnosed of achalasia using manometric, radiologic and endoscopic criteria, were treated with botulinum toxin (80 U) injected directly into lower esophageal sphincter (LES), via a sclerotherapy injector. Response to treatment was assessed by changes in symptom scores and LES pressure. All determinations were repeated after 10, 30, 90, 120 and 180 days of treatment. The patients improved after the initial injection. This improvement was accompanied by improved relaxation of the LES. Two patients relapsed after 30 and 65 days and the other two patients remained symptom-free 5 months after treatment. CONCLUSION: Botulinum toxin is probably a safe and effective alternative for the treatment of achalasia and should be considered in patients in whom pneumatic dilation has failed or who are poor surgical candidates. Long-term evaluation of the safety and efficacy of botulinum toxin in the treatment of achalasia is required.

[Primary herpetic esophageal infection in an immunocompetent patient]. / Primoinfección herpética esofágica en paciente inmunocompetente.

Herpetic esophageal primary infection is not a common event in immunocompetent patients. The case of a 27-year-old immunocompetent woman who developed herpetic esophagitis involving the whole esophagus as a manifestation of primary herpetic infection is presented. The endoscopic appearance initially suggested esophageal candidiasis, which is not an exceptional event.

[Gastrointestinal hemorrhage secondary to heterotopic jejunal pancreas]. / Hemorragia digestiva secundaria a páncreas heterotópico yeyunal.

A case of heterotopic pancreas located in the small intestine is herewith presented. The 19-years male patient had been admitted three times since 1991 for an anemic syndrome secondary to melena. The origin of the hemorrhage was not determined with the studies performed (intestinal transit, opaque enema, esophagogastroscopy, colonoscopy and gammagraphy with technetium 99). On the last admission a vascular lesion low flow was observed on arteriography of the upper mesenteric artery on the mesenteric edge of the proximal jejunum. Following laparotomy a small tumor was found in the jejunum and 5 cm of the intestine including the tumor was resected. The anatomic pathologic results demonstrated a heterotopic pancreas.

[Dubin-Johnson syndrome. Presentation of 3 cases. Review of the national literature]. / Síndrome de Dubin-Johnson. Presentación de tres casos. Revisión de la literatura nacional.

Three patients, two male and one female, 42, 64 and 20 years old respectively, with a Dubin-Johnson syndrome are reported. Both men referred jaundice since several years and in the woman's case, the onset of the illness took place during the last term of her second pregnancy. In two patients, liver aspect and it's biopsy were diagnostic. In the other, who was hospitalized because of a myocardial infarction, a hepatic gammagraphy with Tc 99 HIDA was made. No case was associated with biliary lithiasis and only one patient had other members in his family with the illness.

[Laparoscopic findings in liver fascioliasis. Study of 13 cases]. / Hallazgos laparoscópicos de la fasciolasis hepática. Estudio de 13 casos.

The laparoscopic findings in 13 patients with liver fasciolasis are described. Diagnosis was made in three cases in base of the presence of ova Fasciola Hepatica in the patient's faeces and in 10 cases, because they fulfilled the following conditions: ingestion of fresh watercress, eosinophilic count exceeding 30%, positive serologic tests, eosinophilic granulomas with Charcot-Leyden crystals in liver biopsy and good response to treatment with dihidroemetine or bithionol. Nine cases (68.2%) showed hepatomegaly. In 12 of 13 (92.3%) lesions suggestive of hepatic distomatosis were found. Hepatic nodules of different sizes and shapes could be seen in 10 patients (76.9%), Glisson capsule was involved in five cases (38.4%) and peritoneum in three (23%). The latter was always affected with the liver but, on the other hand, liver capsule was found affected alone in two cases. In our experience, laparoscopy with liver biopsy is an important method for diagnosis of abdominal fasciolasis.