Lege Artis Medicinae

[SUCCESSFUL TREATMENT OF WHIPPLE'S DISEASE IN A PATIENT WITH LYMPH NODE ENLARGEMENT]

SOMOGYI Ágota, SZABÓ Tamás, KISHÁZI Péter, KISS Erika, ARATÓ Gabriella, PÁL Katalin, MADÁCSY László

OCTOBER 20, 2004

Lege Artis Medicinae - 2004;14(10)

[INTRODUCTION - Intestinal lipodystrophy, Whipple’s disease is an uncommon, chronic, systemic bacterial infection. It occurs predominantly in Caucasian males older than 40 years. The gastrointestinal tract is the most frequently involved organ, with clinical manifestations such as abdominal pain, malabsorption syndrome with diarrhea and weigth loss. Patient may present with low grade fever or fever of unknown origin, arthritis, lymphadenopathy, skin hyperpigmentation, endocarditis, pleuritis and peripheral and central neurological manifestations. Due to the wide variability of symptoms, the clinical diagnosis is very difficult and it is often made only years or even decades after the initial presentation. CASE REPORT - A 51-year-old Caucasian race man was admitted to the hospital with weigth loss and signs of subileus, referred for suspected lymphoma. After the exploratory laparotomy and lymphadenectomy the histological and the electron microscopical diagnosis was Whipple’s disease which was confirmed with histology from deep duodenal biopsy. Trimethoprim and sulfamethoxazole therapy for 6 months resulted in complete clinical and molecular biological healing. CONCLUSION - Clinical signs of Whipple’s dease are non-specific and may mimic Crohn’s disease, coeliac disease, amyloidosis, macroglobulinaemia, histoplasmosis, infection with non-tuberculotic mycobacterium in AIDS patients and lymphomas. Therefore, differential diagnosis is of critical importance. The natural evolution of the disease without treatment is always fatal. Trimethoprim and sulfamethoxazole for at least 1 year is usually considered adequate to eradicate the infection.]

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[LONG-TERM RESULTS OF ENDOSCOPIC SPHINCTEROTOMY - EFFECTS OF THE TRANSECTION OF BILE PAPILLA]

DÖBRÖNTE Zoltán

[The abolishment of the choledochoduodenal pressure gradient due to endoscopic sphincterotomy results in the enhancement of the enterohepatic circulation of the bile salts, in the reduction of the cholesterol saturation index and in the modification of the gallbladder function: the reduced gallbladder storage time and the increased ejection fraction facilitates gallbladder emptying. On the contrary, bacterial colonisation of the bile ducts due to duodenobiliary reflux plays a causative role in the increased risk of pigment stone formation. However, when the biliary tree is well-drained, no clinically relevant chronic inflammation develops, furthermore there is no evidence for an increased cancer risk caused by the duodenobiliary reflux. Long-term complications may occur in about 12%, as the recurrence of common bile duct stones, post-EST papillary stenosis, and biliary symptoms caused by retained gallbladder stones. Risk factors for recurrence of bile duct stones are juxtapapillary duodenum diverticulae and persistently dilated bile ducts being the main reason for papillary restenosis and sphincterotomies are mainly performed because of papillary stenosis. In cases of retained gallbladder with stones patency of the cystic duct and contractility of the gallbladder are important predictive factors of late gallbladder complications as it was confirmed by our investigations. Accordingly, small gallbladder stones may pass spontaneously after EST. The indication of a cholecystectomy following EST should be considered individually, particularly in elderly patients. As 30-year-experience confirms, EST is a safe and effective treatment of choledocholithiasis and papillary stenosis even in the long term, and also in young patients. Regular follow-up of patients with high risk for recurrent biliary symptoms is recommended to detect late complications and treat them endoscopically in time.]

Lege Artis Medicinae

[THE SIGNIFICANCE OF THE CARE OF CHILDREN WITH CLEFT LIP AND PALATE IN THE GENERAL PRACTICE]

HIRSCHBERG Jenő

[The cleft lip and palate (i.e. facial cleft) is a frequent and distorting abnormality. The basics of the successful management are the early introduction of therapy and a well-trained team with all relevant specialists included (surgeon, otolaryngologist, orthodontist, speech therapist) as well as good collaboration with the parents and general practitioners being also an important factor. The author with his co-workers has performed more than 6000 surgeries in about 3500 children with facial cleft in the last 45 years and has treated 60-70 patients annuallly with velopharyngeal insufficiency without cleft. According to his experience and international data he summarizes the etiology, pathomechanism of facial clefts and discusses its symptoms, functional consequences and the surgical and conservative solutions are suggested. The recent Hungarian prevalence is 1:500. Specific prevention does not exist, the 5-6% recurrent cleft risk may be decreased to half by administration of folic acid. The generally accepted timing of the lip plasty is the 3-month age. The palatoplasty may be performed in one or two stages, but closure of the velum should be made before the development of speech by all means. The logopedic treatment (speech therapy) should be started, if the speech disorder is already obvious and the child is able to cooperate with the speech therapist. If conservative therapy is unsuccessful, (velo)pharyngoplasty is proposed at the age of 5. The orthodontic treatment should begin in mixed dentition, major nose correction and oral surgery are allowed only after puberty. Just because of a cleft the infant does not aspirate, the brestfeeding is beneficial and could be performed in most cases. Regular hearing control is recommended because of frequent ear and hearing problems. It is suggested to provide the parents with written instruction about outcome, prognosis and timetable of management, which could be helpful also for the general practitioners.]

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[The author deals with the current situation and new trends of vaccinology by focusing on the interests of practitioners. The main topics are the changes of antigens (such as pertussis, measles, or poliomyelitis) to provide better efficacy and milder reactogenity or less adverse events. Purifying the vaccines, like thiomersal and human proteins free vaccines is another proven method to achieve better safety. New antigens e.g. Rota, Lyme, meningococcus B are in the pipeline of vaccinology. The aim of producing a combined vaccine is to achieve immunity against more diseases with less inconvenience for the patient, while achieving higher vaccine coverage (DPT-Hib-HBV-IPV). The epidemiological and clinical experiences will influence the current vaccine schedule such as revaccinations of MMR, and remove the need for revaccinations of BCG and hepaB. The special target groups of immunizations are the elderly and patients with chronic disease. Groups of specialists are working on the vaccine recommendation guidelines for certain risk groups. At the same time, with the successful eradication of polio in Europe the practitioners now have to face the antivaccination movement, as well. The main tools to convince people about the benefit of vaccinations are health education and information.]

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[REVERSAL]

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