Blastomycosis in a young African man presenting with a pleural effusion

Blastomyces dermatitidis is a dimorphic fungus endemic to northwestern Ontario, Manitoba and some parts of the United States. The fungus is also endemic to parts of Africa. Pulmonary and extrapulmonary findings of a 24-year-old African man who presented with weight loss, dry cough and chronic pneumonia not resolving with antibiotic treatment are presented. The unusual occurrence of pulmonary blastomycosis associated with skin lesions and a moderate pleural effusion is reported.     

Occlusion of pseudoaneurysms using human or bovine thrombin using contrast-enhanced ultrasound guidance

Percutaneous thrombin occlusion of pseudoaneurysms complicating invasive coronary interventions has emerged as a useful therapeutic tool. To facilitate thrombin occlusion and make the procedure even more secure, preinjection of echo contrast medium during duplex ultrasound was tested in 132 patients. Complete and immediate occlusion of the pseudoaneurysm was achieved in 99.2% (131 of 132 patients). No complications related to the injection of thrombin were documented. Contrast-enhanced ultrasound-guided thrombin occlusion was a safe and effective therapeutic option. In aneurysms with complex morphology or multiple cavities, preinjection of contrast medium was helpful for documenting the flow pattern.     

Coronary morphologic findings after stent implantation

Clinical studies demonstrated a reduction of acute complications by high-pressure stenting. This study was performed to correlate the histomorphologic changes of the vessel wall after coronary stenting with stent expansion pressure. We studied the effects of intravital and postmortem stenting on coronary morphology in human hearts. Artifact-free analysis and morphometry of the artery segments' cross section was performed after plastic resin embedding and cutting and grinding sectioning. By comparing intra- and postmortem findings we demonstrated that postmortem stent implantation can serve as an adequate model to study the mechanical effects of coronary stenting. A consistent histologic feature was eccentric stent expansion. Larger calcified areas of the vessel wall were not deformed by implanted stents. The highest degree of vessel injury and deformation was apparent in anatomically "nondiseased" or only slightly fibrotic parts of the arterial wall. Dissections were predominantly located directly adjacent to calcified plaques and appeared as "half-moon"-like tears reaching into the arterial media. A statistically significant stent lumen gain was found when the implantation pressure was increased up to 15 atm. Stent symmetry was not influenced by the applied implantation pressure but depended mostly on local coronary morphology. Thus, increasing implantation pressures during coronary stenting seemed to improve the stenting result up to 15 atm. When applying histomorphologic criteria, the higher pressures (>15 atm) did not cause further optimization of stent expansion. Morphometric analysis of stents implanted postmortemly and intravitally revealed comparable results. Postmortem stenting seems to be an appropriate model for studying stent expansion and stenting results in human coronary arteries.     

Skeletal maturation during thyroxine treatment in children with congenital hypothyroidism

Heyerdahl S, Kase BF, Stake G. Skeletal maturation during thyroxine treatment in children with congenital hypothyroidism. Acta Prediatr 1994;83:618–22. Stockholm. ISSN 0803–5253
The aim of this investigation was to study if bone age development (assessed by the Greulich & Pyle atlas) was related to L-thyroxine treatment in 47 children with congenital hypothyroidism, treated early and according to general recommendations. In spite of frequent delay in skeletal maturation at diagnosis, the delay in mean bone age at a mean chronological age of 1.5 years was slight (0.5 months), and 30% of the variation in bone age SD score (SDS) at 1.5 years was accounted for by the dose of L-thyroxine and serum thyroxine during the first year. The children with a bone age within ± 1 SDS had a prescribed mean dose of L-thyroxine per kg body weight from 3 to 12 months of age of 5.4 ± 1.7 pg/kg/day, and their mean serum thyroxine concentration during the first year was 175 ± 29 nmol/l. We conclude that bone age at 1.5 years of age was positively correlated with the dose of L-thyroxine and the serum thyroxine concentration during the first year. This supports the general use of bone age assessments as a complement to other treatment variables in the follow-up of children with congenital hypothyroidism.     

Cystic fibrosis and the phenotypic expression of autosomal dominant polycystic kidney disease

Recent experiments in cultured cyst epithelial cells from kidneys of patients with autosomal dominant polycystic kidney disease (ADPKD) have shown that the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) is present in the apical surface of these cells and mediates chloride (Cl-) and fluid secretion in vitro. To determine whether the presence of CF with the expression of mutated CFTR proteins modifies cyst formation in ADPKD, we studied a large family with both inherited diseases. ADPKD in this family is linked to PKD1. The family is composed of 26 members; 11 members with ADPKD, 4 members with CF, and 2 members with both diseases. Renal volumes measured by computerized tomography (CT), calculated creatinine clearances, and other clinical parameters in the family members with ADPKD and CF were compared with those in the family members with ADPKD alone, as well as to a large population of patients with ADPKD. The patients with CF and ADPKD, but not the CF heterozygote carriers with ADPKD, had less severe polycystic kidney and liver disease, as indicated by normal renal function; smaller renal volume, even when corrected for height and body surface area; and the absence of hypertension and liver cysts. These observations suggest that the coexistence of CF may reduce the severity of ADPKD.     

Charakteristische Elektrolytveränderungen bei der erblichen Myopathie mit Kardiomyopathie des Syrischen Goldhamsters (Stamm BIO 8262)

Zusammenfassung Bei der erblichen Myopathie und Kardiomyopathie des Syrischen Goldhamsters (Stamm BIO 8262) wurden während verschiedener Lebensabschnitte die Elektrolyte Natrium, Kalium, Kalzium und Magnesium im Serum und mehreren Geweben mit entsprechenden Kontrollen verglichen. Die Elektrolytmessung erfolgte mittels Atomabsorptions-Spektrophotometrie. Als hervorstechendstes Merkmal erwies sich eine hochgradige Kalziumakkumulation in der nekrotisierenden Herz- und Skeletmuskulatur, daneben eine ausgeprägte Natriumerhöhung in der myopathischen Skeletmuskulatur. Die muskuläre Natriumerhöhung wird hauptsächlich auf ein interstitielles Ödem, also eine Folge der Myopathie, bezogen. Dagegen wird die myokardiale sowie muskuläre Kalziumakkumulation als wesentliches Glied im Ablauf der, pathogenetischen Prozesse der Erbkrankheit betrachtet. Sie scheint entscheidend für die Nekrotisierung verantwortlich zu sein.

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Characteristic electrolyte changes in the hereditary myopathy and cardiomyopathy of the Syrian golden hamster (strain BIO 8262)

Summary In hamsters of differing ages suffering from a hereditary myopathy and cardiomyopathy (strain BIO 8262), the electrolytes sodium, potassium, calcium, and magnesium in serum, and several tissues were compared with appropriate controls. The determinations of the electrolytes were performed by atomic absorption spectrophotometry. An enormous accumulation of calcium in the necrotizing heart and skeletal muscle was the noticeable feature besides pronounced elevation of the sodium content in the myopathic skeletal muscle. While the latter refers mainly to an interstitial edema which is a consequence of the myopathy, the calcium accumulation is assumed to be an essential part in the cycle of pathologic events occurring in the hereditary disease: it seems to induce the necrotization.

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Mit 4 Tabellen

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Mit Unterstützung der Deutschen Forschungsgemeinschaft     

Essential myosin light chain as a target for caspase-3 in failing myocardium

Programmed cell death involves the activation of caspase proteases that can mediate the cleavage of vital cytoskeletal proteins. We have recently reported that, in failing cardiac myocytes, caspase-3 activation is associated with a reduction in contractile performance. In this study we used a modified yeast two-hybrid system to screen for caspase-3 interacting proteins of the cardiac cytoskeleton. We identified ventricular essential myosin light chain (vMLC1) as a target for caspase-3. By sequencing and site-directed mutagenesis, a noncanonical cleavage site for caspase-3 was mapped to the C-terminal DFVE(135)G motif. We demonstrated that vMLC1 cleavage in failing myocardium in vivo is associated with a morphological disruption of the organized vMLC1 staining of sarcomeres, and with a reduction in myocyte contractile performance. Adenoviral gene transfer of the caspase inhibitor p35 in vivo prevented caspase-3 activation and vMLC1 cleavage, with positive impact on contractility. These data suggest that direct cleavage of vMLC1 by activated caspase-3 may contribute to depression of myocyte function by altering cross-bridge interaction between myosin and actin molecules. Therefore, activation of apoptotic pathways in the heart may lead to contractile dysfunction before cell death.     

Erfolgreiche Therapie einer Fusarium-assoziierten Keratitis bei multiresistentem Erreger und multimorbidem Patienten

A 75-year-old man (not a contact lens wearer) presented with Fusarium-associated hypopyon keratitis. After several weeks of empirical and subsequently targeted antimycotic treatment, no considerable improvement was observed. However, after sclerokeratoplasty (11.2?×?11.2 mm) combined with prolonged antimycotic therapy a good local result with relapse-free long-term follow-up was achieved.