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The exact differential diagnosis of iron overload syndromes is mandatory as important therapeutic consequences may derive from a correct diagnosis, especially when hemochromatosis is present. To facilitate diagnostic and therapeutic decisions algorithms and probabilistic calculations based on different frequencies of clinical symptoms and typical laboratory findings of the diseases in question have been proposed. Overestimation and/or underestimation of clinical symptoms and/or laboratory findings in using such calculations, however, may lead to incorrect diagnosis and therapy as demonstrated in this case. We report on a 62-year-old patient with arthralgia, pathologic glucose metabolism, brown skin pigmentation and excessively elevated ferritin and transferrin saturation levels, which initially were interpreted as signs of the assumed underlying disease (hemo-chromatosis) based on a high initial suspicion level and further corroborated by Bayesian probability analysis yielding a probability 99.0 % for the presence of hemochromatosis. Because of this high probability and the patient's wish for treatment phlebotomy was started, but stopped after having obtained negative results of genetic testing and normal quantitative liver iron values. The diagnosis of hemochromatosis had to be revised and symptoms and laboratory findings of this patient were found to be compatible with chronic fatty liver and pathologically altered iron metabolism due to chronic alcohol intake which the patient has initially concealed. The joint pain was explained in terms of chronic degenerative bone destruction, the impaired glucose tolerance seen as the consequence of obesity and the skin pigmentation was ascribed to sun exposure due to the patient's outdoor activities as a hobby farmer not evaluated during initial presentation. The implications and importance of unbiased history taking, critical interpretation of clinical symptoms and laboratory findings in using probabilistic calculations and diagnostic decision analysis are emphasized and the different mechanisms of iron metabolism in hemochromatosis and hemosiderosis are discussed.

Primary cardiac tumors in infancy and childhood are extremely rare. In the young age group the clinical symptoms often are variable and can make diagnosis difficult. Depending on the histological character and especially the localisation of the mass the clinical appearance may lead to the first diagnosis or at least to further investigation. While myocardial tumors (rhabdomyomas and fibromas) mainly cause rhythmic disturbances, the intracavitary tumors (like myxomas) frequently disturb hemodynamics. Echo-cardiography is the main diagnostic technique, followed by angiocardiography, computertomography and nuclearmagneticresonance imaging.

An autoradiographic study of the proliferative response of the cells of the Macula Densa days or months after the removal of 5/6 of the rat's kidney has shown that these cells are capable of division. The index of labelling of these cells is considerably lower than that of the other cells of the distal tubules. This applies of the kidney of normal controls and to the remnant after partial nephrectomy, shortly after operation as well as several months later. The cells of the Macula Densa therefore seem to represent a more stable population in the nephron. They therefore differ from the other cells of the distal tubules not only in appearance and function, but also in the pattern of cell proliferation.

There is evidence that the rarefaction of the capillary bed is typical for the skeletal muscle of spontaneously hypertensive rats. We were therefore interested to learn whether there is also a rarefaction in skeletal muscle of human hypertensives. The number of capillaries was morphometrically analysed and counted in the quadriceps and the pectoralis major muscles of human normotensives (n = 12) and hypertensives (n = 15). The clinical diagnosis and certain pathological criteria, such as blood pressure (with or without antihypertensive therapy), heart weight, left ventricular wall thickness, the state of kidney arterioles and brain, and heart vessels, were used to classify the patients into two groups. The dissected tissue samples were prepared according to the GMA method and the capillary numbers per area were counted using light microscopy (250 x). The quadriceps muscle had a capillary density (per 2.5 mm2) of 442 +/- 51 in normotensives and 277 +/- 41 in hypertensive patients; in the pectoralis major muscle we counted 477 +/- 30 in controls and 232 +/- 28 in hypertensives. The rarefaction in the quadriceps muscle ranged by about 37%, in the pectoralis major muscle by about 51%. It is suggested that the reduction of the capillary surface area caused by the capillary rarefaction reduces the transcapillary fluid exchange and in that way prevents an overperfusion of the terminal vascular bed.

The differentiation of the thyroid glands follicular neoplasias into adenomas and carcinomas is currently done using the histological criteria recommended by WHO. This pilot study of 10 human follicular carcinomas and 10 folliculars adenomas demonstrates the possibility of a cytological classification using digital picture processing of high resolution cell images. Giemsa stained paraplast sections were scanned with a Colour-TV-camera, different channels were used with respect to staining and analyzing methods and computed with an image processing system. The computer aided cytophotometric methods detected significant differences in the chromatin arrangement and structure.

The tissue distribution of intravenously injected native and enzyme-treated human gammaglobulin has been investigated in human rectum mucosa. FITC-labelled antibodies to human gammaglobulin were used to locate the injected antibody preparation. Three hours after application of these different gammaglobulin-preparations no enrichment in the mucosa was found. Neither the native gammaglobulin nor the enzyme-treated gammaglobulin showed any concentration in the lamina propria of the large bowel mucosa or an intracellular enrichment in the epithel. This study shows that the quicker elimination of enzyme-treated gamma-globulin from the circulation cannot be explained by an intracellular increase.

A case of human rabies is reported showing a peculiar course in some respects. The lack of a satisfactory history concerning an animal bite 5 months earlier as well as a misleading psychiatric history led to difficulty and delay in reaching a diagnosis. Under intensive care, including mechanical ventilation and cardiac pacing, the disease reached a final stage, which is rarely seen. The clinical course and the pathological findings are described in detail and the etiology and prognosis of the disease are discussed. The latter remains rather hopeless de spite modern intensive care.

Nocardial sepsis occurred after aortic valve replacement in two patients. A septic suture aneurysm of the aortotomy was resected and the prosthesis exchanged in one of them. The other received conservative treatment for sternal osteomyelitis and local mediastinitis. Clinical cure was followed by relapse and death from cerebral infarction, and necropsy revealed a septic suture aneurysm of the aortotomy. Radical surgical revision seems to be necessary for lasting cure in such infections.

In 10 patients with severe pain localised over the dorsal aspect of the lunate, the capsule of the wrist joint was exposed and fenestrated over the scapho-lunate ligament. In two cases flat ganglion cysts were found, too small to be palpated before surgery. In the other 8 cases without macroscopically visible changes, histologically, areas of mucoid degeneration and very small ganglion cysts could be seen in the capsule. All patients were pain free after surgery. It is concluded that very small intracapsular cysts in the area of the dorsal scapho-lunate ligament, the most common origin of ganglia, may irritate the posterior interosseus nerve which is located in this area.

Experimental and clinical investigations (92 patients) of a novel continent colostoma sphincter plasty are presented with particular evaluation of the histology of the autografted sphincter plasty. In

In five infants with autosomal aberrations and diminished resistance to infection (in spite of intact humoral and cellular immune mechanisms) several granulocyte functions (chemotaxis, phagocytosis, intracellular killing and metabolism of killing) were measured. A serum-dependent or a cell-dependent disturbance of phagocytosis of Candida albicans was found in two infants with cat-cry syndrome and one with trisomy 18. In one of these children there was an additional serum dependent defect of the killing of Candida albicans and of Staphylococcus aureus, serum levels of opsonins (IgG, IgM, CH50 and C3) being within normal range. An infant with trisomy 21 showed, in addition to a cellular defect of chemotaxis, a reduced cellular ability of the killing of Staphylococcus aureus and of Escherichia coli in autologous and AB-pool-serum. Phagocytosis of these bacteria remained normal.

The clinical picture of chronic pharyngitis is well defined but insufficiently clarified as to its morphological nature. In our study of 12 children with chronic pharyngitis who underwent adeno-tonsillectomies small pieces of tissue of the posterior wall of the pharynx were microscopically examined. In most instances inflammatory infiltrations were absent. Present was a variably developed hyperplasia of the lymphatic tissues. In the more pronounced hyperplasias there was a lymphoepithelial disintegration that could account for the clinical manifestations and that perhaps could also be considered as a disposing factor for a bona fide pharyngitis. The micromorphological changes were divided into 4 stages.

A boy developed a nephrotic syndrome at the age of two years, and remained steroid sensitive for twelve years. In this time more than twenty relapses occurred. Two renal biopsies showed minimal changes. Renal function was normal all along. At an age of 14 years he developed an irreversible renal failure. Since this time he remained dependent to dialysis. A third renal biopsy now shows a perimembranous glomerulonephritis with a diffuse interstitial fibrosis.

The connective tissue of a lethal EDS IV case was investigated for the reasons of the manifested disturbances of the arterial wall. This functional disorder was attributed to the mechanical decoupling of elastin and collagen, with the premise of a composite material consisting of cellular, fibrillar, lamellar and other matrix components. A conceivable relation between the manifested deficiency of type III collagen and a disturbed anchoring of elastin is shown. These findings are supported by biochemical, morphological, x-ray and mechanical data.

The diabetic glomerulosclerosis as a consequence of the abnormal metabolic state is characterized by an uniform thickening of the glomerular basement membrane (GBM) and an augmentation of the mesangial matrix. Both alterations begin already few years after the onset of the diabetes and are observed in all glomeruli to the same extent (=diffuse type). Later on, nodular deposits of glycoproteins are additionally found in the mesangium (=nodular type). Only these nodules are morphologically specific for the diabetic disorder. In association with both the diffuse and the nodular glomerulosclerosis, insudation of plasma can be seen in the afferent and efferent vessels and the glomerular capillary loops. The mechanism of the increase in the amount of GBM-material is not known, since contradictory data have been reported with regards to both the chemical composition and metabolism of the GBM in human and experimental diabetes. Some postulate that the abnormal deposition of GBM-mateiral is due to an excessive synthesis (anabolic disorder), others argue that a further decrease in the normally slow breakdown and disposal (catabolic disorder) might be responsible. This review presents the different pathogenetic concepts of the glomerulosclerosis and attemps to explain the possible causes for the current discrepancies.

The course of illness of a male infant who lived for seven months with a diffuse, nesidioblastic hyperplasia of pancreatic islets is described. Before surgical intervention the diagnosis should be ascertained by 1. observation of acetonuria which is always absent after hypoglycemic episodes, 2, the typical constellation of insulin concentration, free fatty acid concentration and beta-hydroxybutyrat during a hypoglycemia (as found by Baker et al. 1976) and/or by simultaneous measuring of glucose-insulin levels under the conditions of fasting as well as of oral leucine, oral glucose and intravenous tolbutamide loading. Therapy with diazoxide should be tried in any case. If all conservative measure fail and relative or absolute hyperinsulinemia is proved, experience shows that an immediate operation is indicated.

The study is concerned with a problem which is to a great extent still surgically unsolved, namely that of the incontinent hollow organ occlusion. We report on our own experiments on animals and on physiologicofunctional studies, which reveal the suitability of the smooth muscles as an ideal material for sphincter replacements. By histologic research it became obvious that autotransplantations of smooth muscles, carried out for the first time by the Department of Surgery of the University of Würzburg, are possible without significant tissue changes. The transplant is soon supplied by vessels which sprout ubiquitously and completely permeate it so that it can take over its function as sphincter. Meanwhile, the experience gained in experiments on animals has been transferred to surgery on humans. Incontinence and incontinence of the preternatural anus were eliminated in the first operations using autologous autotransplanted sphincteroplasties.

One case of five independent tumors is reported: Fibrosarcoma of the right thigh, bilateral carcinoma of breast, papillary carcinoma of thyroid, and basalioma below the left clavicula. A synopsis of literature containing the rare cases of more than three primary tumors is presented. The difficulties concerning localization, origin and registration of multiple primary malignancies and their differentiation to recurrencies and metastases are discussed.

In recent years an increase in benign hepatomata during administration of oral contraceptives has been reported many times. A case is reported here of a 38-year-old woman who took such contraceptives for 8 years. The cardinal symptom was a tumor in the right upper quadrant of the abdomen. Laparotomy revealed several nodules in both lobes of the liver which could be almost completely removed by a wedge-shaped incision. Histologically a multicentric, focal nodular hyperplasia was present. Follow-up examinations after 24 months show no enlargement of the remaining tumor residues.