Severe Malnutrition Associated with α-Heavy Chain Disease: Response to Tetracycline and Intensive Nutritional Support

A 20-yr-old black male was admitted with a 5-month history of profound weight loss and diarrhea. Appetite and dietary intake had been remarkably well preserved up until the week before admission. The severity of his depletion was evidenced by a body weight of only 38% of standard, multiple electrolyte deficiencies, and reduced metabolic expenditure, protein turnover, protein synthesis, and pancreatic function. Immunological defects included diminished lymphocyte numbers, lymphocyte transformation, gamma-globulin concentration, and cell-mediated immunity. A diagnosis of alpha-heavy chain disease (alpha-HCD) was made on endoscopic duodenal biopsy and serology--lymphoma being excluded by scanning and laparotomy. Treatment consisted initially of intravenous nutrition (because of the extreme malnutrition, severe diarrhea, and malabsorption of fluid, electrolytes, carbohydrates, and fat) and oral tetracycline. Response was dramatic, with a doubling of body weight within 6 wk, and resolution of malabsorption. He was discharged on a normal diet and long-term oral tetracycline (250 mg/day), and at 1-yr follow-up, nutritional status and gut function were normal despite persistence of duodenal mucosal abnormalities and markers of alpha-HCD and bacterial overgrowth. These results suggest that the malabsorption initially identified in this patient was not due simply to the mucosal abnormalities that characterize alpha-HCD, but was more a consequence of the superimposition of nutrient maldigestion and absorption resulting from the extreme state of protein deficiency and its effects on gut and pancreatic function.     

Conduction tissue in congenital heart surgery

The anatomy of the cardiac conduction system in normal and malformed hearts reconstructed from histological serial sections is reviewed. The sinus node and its arterial supply are located in the right superior cavoatrial junction except in hearts with left-sided juxtaposition of the atrial appendages, mirror-image, and isomeric arrangements of the atria. In the presence of normally positioned atrial and ventricular septal structures, the triangle of Koch is a useful guide to the location of the atrioventricular node and the penetrating bundle. In hearts with isolated ventricular septal defects, it is necessary to distinguish between a perimembranous defect and a muscular inlet defect. The conduction axis runs in the area of fibrous continuity at the posteroinferior edge of a perimembranous defect but in the anterosuperior rim of a muscular inlet defect. In most hearts with tetralogy of Fallot, the danger area is the region of aortic-mitral-tricuspid fibrous continuity where the axis penetrates. In hearts with atrioventricular septal defects, the lack of contiguity between atrial and ventricular septum results in a posterior displacement of the nodal triangle and coronary sinus and an elongation of the nonbranching bundle. Hearts with abnormally located atrioventricular nodes and conduction axes are mainly those with atrioventricular discordance, ambiguous atrioventricular connection in presence of left-hand pattern ventricular topology, hearts with univentricular atrioventricular connection to a morphologically left or indeterminate ventricle, and hearts with a straddling tricuspid valve. The location of the atrioventricular node is governed by the orientation of the ventricular septum and by the pattern of ventricular topology.

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Resumen Se revisa la anatomía del sistema de conducción cardiaca en los corazones normales y con malformaciones a la luz de la reconstrucción a partir de cortes histológicos seriados. El nódulo sinusal y su irrigación arterial está ubicado en la unión cavoatrial derecha, excepto en corazones con yuxtaposición izquierda de las auriculillas o con alteraciones en imagen en espejo e isoméricas de las aurículas. En presencia de estructuras septales atriales y ventriculares normalmente ubicadas, el tríangulo de Koch es una guía útil para la localización del nódulo atrioventricular y la penetración del haz. En corazones con defectos septales ventriculares aislados es necesario distinguir entre un defecto perimembranoso y un defecto del tracto muscular de entrada. El haz de conducción avanza en el área de continuidad fibrosa sobre el borde posteroinferior de un defecto perimembranoso, en el filo anterosuperior de un defecto del tracto muscular de entrada. En la mayoría de los corazones con tetralogía de Fallot el área de peligro se encuentra en la región del trígono fibroso aórticomitral y tricuspídeo sobre el cual penetra el haz de conducción. En corazones con defectos septales atrioventriculares la falta de continuidad entre el septo atrial y el ventrícular resulta en un desplazamiento posterior del triángulo de Koch y del seno coronario y en una elongación del haz no ramificado. Corazones con nódulos atrioventriculares y haces de conducción anormalmente ubicados son principalmente aquellos con discordancia atrioventricular, con conexión atrioventricular ambigua en presencia de un patrón izquierdo de arquitectura ventricular, corazones con conexión atrioventricular univentricular a un ventrículo morfológicamente izquierdo o indeterminado y corazones con válvula tricuspídea cabalgante. La ubicación del nódulo atrioventricular está determinada por la orientación del septo ventricular y por el patrón de la arquitectura ventricular.

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Résumé L'anatomie du système de conduction du coeur normal et du coeur siège de malformations reconstituée à partir de coupes sériées histologiques est exposée. Le sinus nodal et sa vascularisation sont situés au niveau de la partie supérieure de la jonction auriculo-ventriculaire droite sauf dans certains cas: coeur avec juxtaposition des auricules du côté gauche, image en miroir et dispositions isomères des oreillettes. Lorsque les cloisons auriculaire et ventriculaire occupent une situation normale, le triangle de Koch constitue un repère utile pour localiser le noyau auriculo-ventriculaire et le faisceau de His. En cas de défects septaux ventriculaires isolés, il est nécessaire de distinguer les défects périmembraneux et les défects musculaires. L'axe nerveux conducteur chemine dans la zone de continuité fibreuse au bord postéroinférieur du défect périmembraneux mais il chemine au bord antéro-supérieur du défect musculaire. En cas de tétralogie de Fallot, le danger se situe souvent au niveau de la zone fibreuse de continuité aortique-mitrale-tricuspide où pénètre l'axe conducteur. En cas de coeur porteur d'un défect septal auriculo-ventriculaire, le défaut de continuité entre la cloison ventriculaire et la cloison auriculaire entraîne le déplacement postérieur du triangle nodal et du sinus coronaire ainsi que l'élongation du faisceau. Les coeurs qui présentent une anomalie du siège des noyaux auriculo-ventriculaires et des nerfs conducteurs sont principalement ceux qui sont le siège d'une anomalie auriculo-ventriculaire, d'une connexion auriculo-ventriculaire ambiguë en cas d'une disposition ventriculaire de type gauche, d'une connexion auriculo-ventriculaire univentriculaire avec une morphologie ventriculaire gauche ou indeterminée, d'une valve tricuspide en fourche. Le siège du noyau auriculo-ventriculaire est fonction de l'orientation du septum ventriculaire et de l'architecture ventriculaire.

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Supported by the British Heart Foundation.     

Short-term chemoprophylaxis with ceftizoximevs. Five-day aminoglycoside with metronidazole in ‘contaminated’ lower gastrointestinal surgery

In a prospective, open, randomized controlled trial, 173 patients requiring surgery for potentially contaminated lower gastrointestinal surgery were allocated to receive either two doses of ceftizoxime (2 gm) with one dose of metronidazole (1.5 gm) or gentamicin 3 mg/kg/day for five days with one dose of metronidazole (1.5 gm). Eighty-nine patients received ceftizoxime and 84 patients received gentamicin. The groups were comparable with respect to diagnosis, procedure, type of anastomosis, and wound closure. The incidence of withdrawal due to failure to respond to the study drug (11.5 percent) was equivalent in the two groups. There was no difference in the overall incidence of postsurgical infection between the ceftizoxime and metronidazole group (22.2 percent) and the gentamicin and metronidazole group (25.7 percent). The incidence of wound infection (ceftizoxime and metronidazole, 6.9 percent; gentamicin and metronidazole, 10 percent) and deep sepsis (ceftizoxime and metronidazole, 15.3 percent; gentamicin and metronidazole, 15.7 percent) was similar.     

Pancreatic apudomas

Pancreatic apudomas are not common but are frequently curable. Thus, it is important for every clinician to be fully aware of the varied clinical syndromes that suggest their presence. The availability of specific radioimmunoassays has made confirmation of the diagnosis relatively simple. Advances in the techniques for staining the different cell types have led to the recognition that many of these tumors are mixed, and that the general term pancreatic apudoma is appropriate. Pancreatic endocrine tumors, as examples of nature's experiments, have yielded considerable insight into the possible physiologic effects of the various peptides they produce. It is to be hoped that further study of tumors such as the somatostatinoma and PPoma may yield further information about these enigmatic compounds.

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Résumé Les apudomes pancréatiques sont rares. Ils sont fréquemment curables. Il est donc important de connaître les divers syndromes cliniques qui peuvent en faire soupçonner l'existence. Le diagnostic peut être facilement confirmé par dosage radioimmunologique. L'amélioration des techniques de coloration des divers types cellulaires a montré que bon nombre de ces tumeurs sont des tumeurs mixtes, qui méritent donc l'appellation générale d'apudomes. Les tumeurs pancréatiques endocrines sont de bons exemples d'expérimentation naturelle: elles ont largement contribué à une meilleure connaissance des effets physiologiques des divers peptides qu'elles secrètent. Il faut espérer que l'exploration future de certaines tumeurs, comme le somatostatinome ou le PPome, apportera de nouvelles informations sur ces substances encore énigmatiques.

     

Antibiotic therapy for treatment in relapse of intestinal Crohn's disease

We have undertaken a prospective randomized trial of one month's antimicrobial therapy for patients with symptomatic relapse of Crohn's disease. Criteria for entry included two major symptoms: fever, abdominal pain, diarrhea, weight loss, abdominal mass or complications (excluding perianal disease); and two hematologic abnormalities: hemoglobin, ESR, albumin, C reactive protein, iron, or total iron binding capacity. Patients were monitored for the aforementioned clinical and hematologic (hemoglobin, albumin, CRP) parameters over six weeks and for changes in fecal flora. Randomization was to four groups: metronidazole alone (M), cotrimoxazole alone (C), metronidazole and cotrimoxazole (C plus M), or double placebo (P). Seventy-two patients entered the study (18=M, 16=C 21=C plus M, 17=P). After two weeks, improvement was reported as follows: M=67 percent, C=17 percent, C plus M=71 percent, P=35 percent. In the metronidazole group, two patients required surgery and one had trouble-some side effects. In the cotrimoxazole group, two had side effects. In the combined group (C plus M), four had troublesome side effects and two of the placebo group (P) required operation. By four weeks, there was no difference in response among the groups: (M=44 percent, C=62 percent, C plus M=57 percent, P=41 percent). Antimicrobials had no effect on fecal flora or hematologic parameters. These results indicate that antimicrobials have little therapeutic potential for relapse of intestinal Crohn's disease.     

Melanoma

A retrospective review of 182 cutaneous melanomas seen at King's College Hospital from 1970 to 1987 showed that surgeons saw much thicker melanomas (median 3.64 mm) when compared to dermatologists (median 1.14 mm). Surgeons saw a greater proportion of nodular melanomas, less superficial spreading melanomas, were less likely to make the correct clinical diagnosis, and were more likely to perform wide excisions for thin melanomas (less than 2 mm) when compared to dermatologists. General surgeons see a different group of melanoma patients compared to dermatologists and manage them differently from dermatologists working with plastic surgeons. There is a need for a combined approach for the diagnosis and management of cutaneous melanoma between specialists.