[A rare cause of carpal tunnel syndrome: leprosy. Latency period of decades is possible]. / Seltene Ursache eines Karpaltunnelsyndroms: Lepra. Jahrzehntelange Latenzzeit möglich.

In a 63-year-old German woman with no apparent exposure to leprosy, clinical and histological evidence of tuberculoid leprosy was found. A noteworthy feature of this case is either the very long incubation time of 45 years, or the mode of infection involving an only short encounter in an endemic region. In the event of unclear lesions--on white skin not, typically, hypopigmented, but reddish--accompanied by disordered sensation, we in Europe must also give consideration to the possibility of a sporadic case of leprosy. A particular characteristic of this case was leprous neuritis involving the median nerve which was diagnosed clinically as carpal tunnel syndrome, and scheduled for surgical treatment. Under treatment with dapsone and rifampicin, however, the condition cleared up completely.

Sonography of acute appendicitis in children: 7 years experience.

OBJECTIVE: The objective of this prospective study was to determine the accuracy in diagnosing appendicitis in children by using high-resolution graded compression sonography. MATERIALS AND METHODS: During a 7-year period, 3859 children (mean age 9.9 years) with clinically suspected appendicitis were evaluated by sonography with the graded compression technique. The ultrasound results were correlated with the intraoperative and histopathological findings or clinical outcome. RESULTS: High-resolution sonography was performed in 3859 children. Of the 610 patients who underwent a laparotomy, 494 had histologically proven acute or perforated appendicitis (prevalence 13%). In these children, sensitivity, specificity and overall accuracy of sonography were 90%, 97% and 96%, respectively. The reasons for false-negative and false-positive results are discussed. CONCLUSION: Although the use of ultrasound to diagnose acute appendicitis in children has excellent results, the decision for surgery remains a clinical one because of the continuing false-negative and false-positive results from sonography.

[Appendicitis in childhood]. / Appendizitis im Kindesalter.

From 1989 to 1995 high-resolution ultrasonography (US) was performed in 3,546 children (age: 1-17 years) with clinically suspected appendicitis. A total of 518 patients underwent laparotomy; 420 had histologically proven acute or perforated appendicitis (prevalence 11.8%). In these children, the sensitivity, specificity and overall accuracy of US examination were 90%, 97% and 96% respectively. The use of US in clinically doubtful acute abdomen may allow earlier diagnosis of acute appendicitis; in 1995 the rate of unnecessary appendectomy was reduced to 13%.

[Disorders of the afferent visual pathway in HIV infection. 1. Optic nerve and 2. Visual pathways/visual cortex]. / Erkrankungen der afferenten Sehbahn bei HIV-Infektion. 1. Sehnerv und 2. Sehstrahlung/Sehrinde.

Up to now little involvement of the afferent visual pathway (optic nerve and optic radiation and visual cortex) in HIV infection has been apparent. These results are based on our prospective investigations of 538 HIV-infected individuals, among them 261 patients with full-blown AIDS carried out by the same examiner over a 6-year period (1984-1990). Diseases of the optic nerve were observed in 22/261 (8.4%) of Aids patients but in only 1/227 (0.4%) of patients with earlier stages of HIV infection. Optic neuritis was the most common disease (in 14/261 = 5.4% of Aids patients), for the most part occurring in the course of CMV (cytomegalovirus) retinitis. For this form the prognosis was primarily good under virustatic therapy with ganciclovir in contrast to primary CMC papillitis. Furthermore, a few cases of optic perineuritis, optic neuropathy in basal meningitis, ischemic optic neuropathy and papilloedema with increased intracranial pressure were observed, most of them caused by opportunistic infections of the central nervous system. In single cases the HIV might hypothetically have played a role in the etiology. Visual impairment of the patients varied from subtle disturbances to blindness. Often optic atrophy resulted. Homonymous hemianopsia was the principal sign in diseases of the visual pathway between the lateral geniculate body and the visual cortex (in 10/261 = 3.8% of Aids patients). This symptom resulted from cerebral toxoplasmosis in 7 cases, progressive multifocal leukoencepalopathy (PML) in 2 cases and primary intracerebral malignant lymphoma in 1 case. The visual fields and neuroradiological findings are demonstrated. In 3 cases the homonymous hemianopsia was the first clinical appearance of Aids. Involvement of the afferent visual pathway in HIV infection may be a cause of blindness or visual disturbances despite normal findings on examination of the eyes themselves.

[Incidence and pathogenesis of ocular symptoms in HIV infection]. / Zur Inzidenz und Pathogenese okulärer Symptome bei HIV-Infektion.

202 patients with HIV infections of different stages [1 (n = 59): symptomless HIV seropositivity, 2 (n = 64): pre-AIDS (LAS = Lymphadenopathy syndrome, ARC = AIDS related complex), 3 (n = 79): AIDS] were prospectively examined. The findings of the first examination were analyzed statistically in a cross-section-study. In 15 AIDS autopsy cases the eyes were examined histopathologically and with immunohistochemical techniques. Patients with stage 1 had only some Sicca syndromes (3%) and neuroophthalmological signs (dyscoria with neurosyphilis) (2%). 14% of the patients with stage 2 had a microvascular retinal syndrome, 11% a Sicca syndrome and 2% neuroophthalmological signs. Among the AIDS patients (stage 3), however, 61% had a microvascular syndrome of the retina, 24% a retinitis resp. choroiditis as an opportunistic infection (in 15% Cytomegalovirus was the causative organism), 16% had neuroophthalmological symptoms, 14% a Sicca syndrome and 5% Kaposi's sarcoma of the eyelids or conjunctiva. The microvascular retinal syndrome was found to be the most sensitive ocular indicator for an advanced stage of HIV infection. Further changes of the anterior eye segments (especially conjunctival vessel abnormalities and precipitates of the corneal endothelium in Cytomegalovirus retinitis) are demonstrated.

[Experiences with DHPG (ganciclovir)--treatment of cytomegalovirus retinitis in AIDS]. / Erfahrungen mit DHPG (Ganciclovir)--Behandlung der Cytomegalievirus-Retinitis bei AIDS.

Nine patients with unilateral cytomegalovirus (CMV) retinitis were treated with intravenous infusions of the new virustatic drug DHPG (Ganciclovir). The induction dose was 10 mg/kg body weight per day (2-5 weeks). In six cases, a maintenance dose was given thereafter of 5 mg/kg body weight per day. After the induction dose, five patients had visual improvement and satisfactory cicatrization. In one patient, PVR (proliferative vitreous reaction) developed with total tractional retinal detachment. More ocular complications were seen while on longterm therapy: relapses during discontinuation because of leukopenia (three times in two patients), breakthrough (= relapse during maintenance therapy) (one case), serous retinal detachment (one case), and optic atrophy (two cases). The complications caused blindness in two further patients. Only one patient has tolerated maintenance therapy for 22 weeks without having any complications. One patient wanted to have therapy suspended and has remained free of relapse for 28 weeks while on cytostatic therapy. The eyes of two deceased patients were examined histopathologically, immunhistochemically, and ultrastructurally and the findings compared with those of an untreated case. Given at an early stage and without discontinuation, DHPG is an effective means of preventing or delaying blindness caused by cytomegalovirus retinitis in AIDS patients. The directives for an optimal dosage are subject to further prospective randomized clinical studies.

[Kaposi sarcoma of the gastrointestinal tract in AIDS. Morphologic findings and differential diagnostic aspects]. / Kaposi-Sarkom des Gastrointestinaltraktes bei AIDS. Morphologische Befunde, differentialdiagnostische Aspekte.

At autopsies of 20 patients with AIDS, 7 cases with gastrointestinal involvement by Kaposi's sarcoma were observed. The lesions were found in nearly all parts of the digestive canal; although we observed a certain accumulation in the upper gastrointestinal tract. Macroscopically the spectrum of appearances extended from red macules in the mucosal plane to plaquelike indurations of the wall and polypoid, hemorrhagic mucosal nodules. A certain, stage-dependent correlation between the macroscopic presentation and the histologic findings was found. In consideration of differential diagnostic aspects the morphologic findings in different tumor-stages are presented.

[So-called nephrogenic adenoma (adenomatous metaplasia). A rare tumorous adenopapillary lesion of the urothelium in the urinary bladder in a 6-year-old boy following injury]. / Sogenanntes nephrogenes Adenom (adenomatöse Metaplasie). Seltene tumorartige adenopapilläre Läsion des Urothels in der Harnblase bei einem 6-jährigen Knaben nach Trauma.

A 6-year-old boy developed an extensive adenopapillary proliferation of the urothelium of the urinary bladder--a nephrogenic adenoma (adenomatous metaplasia)--2 years after he had been injured in an accident, which was followed by operation and medical treatment. Histologically this lesion is characteristic. Like other epithelial tumors of the urinary tract, it is very rarely found in children. A dysontogenetic or metaplastic pathogenesis has been suggested. Once more is known about them, adenomas may be diagnosed more often. The clinical picture and therapy are briefly discussed and a review of the literature is given.

[Eye involvement in AIDS]. / Augenbeteiligung bei AIDS.

Between 1984 and 1987 (over two-and-a-half years) 30 hospitalized patients with HIV infections of different degrees of severity were ophthalmologically examined. Ocular involvement was found in 17 patients (approx. 57%). In 16 of these 17 patients with pathologic ophthalmologic findings (approx. 94%), AIDS was already fully developed. Ocular involvement is therefore a sign of poor prognosis. Fourteen patients had a microvascular retinal syndrome and four patients had infectious (chorio-)retinitis (causative organisms: cytomegalovirus in three cases, Cryptococcus neoformans in one). Further findings included sicca syndrome with superficial punctate keratitis in two cases, keratitis in one patient with generalized mucocutaneous candidiasis, Kaposi's sarcoma of the eyelids in two cases, Kaposi's sarcoma of the conjunctiva in one case, papilledema with cryptococcal meningitis in one case, and atypical hordeolum in one case. Morphologic and pathogenetic aspects of the ophthalmologic findings, their importance and course in AIDS patients, and therapeutic problems are discussed.

The TRP4 gene of Saccharomyces cerevisiae: isolation and structural analysis.

The TRP4 gene of Saccharomyces cerevisiae, encoding the anthranilate phosphoribosyl transferase, was isolated and subcloned by functional complementation in yeast. A 2 kb fragment containing information for a polypeptide of 380 amino acids and the 5'- and 3'-flanking regions was sequenced. The TRP4 transcript was identified and mapped with S1 nuclease. Homologies to two prokaryotic genes encoding the same function, and sequences potentially involved in transcription start and termination and in regulation of TRP4 gene expression are discussed.

Arrangement of genes TRP1 and TRP3 of Saccharomyces cerevisiae strains.

The tryptophan biosynthetic genes TRP1 and TRP3 and partly also TRP2 and TRP4 have been compared by the technique of Southern hybridization and enzyme measurements in twelve wild isolates of Saccharomyces cerevisiae from natural sources of different continents, in the commonly used laboratory strain S. cerevisiae X2180-1A and in a Kluyveromyces marxianus strain. We could classify these strains into four groups, which did not correlate with their geographical distribution. In no case are the TRP3 and TRP1 genes fused as has been found in other ascomycetes. Two strains were found which, in contrast to strain X2180-1A, show derepression of gene TRP1. Two examples are discussed to demonstrate the usefulness of Southern hybridizations for the identification of closely related strains.

Purification and characterization of the indole-3-glycerolphosphate synthase/anthranilate synthase complex of Saccharomyces cerevisiae.

The indole-3-glycerolphosphate synthase/anthranilate synthase complex from Saccharomyces cerevisiae was purified to apparent homogeneity. The native complex with Mr approximately equal to 130 000 consists of two different subunits, the TRP2 gene product with Mr = 64 000 and the TRP3 gene product with Mr = 58 000. The larger polypeptide was identified as anthranilate synthase and is active in vitro with ammonia as cosubstrate without need of complex formation. The smaller polypeptide carries both glutamine amidotransferase activity and indole-3-glycerolphosphate synthase activity. Various steady-state kinetic parameters as well as the amino acid composition of the two polypeptides were determined.

Health impact of radioactive debris from the satellite Cosmos 954.

This paper describes a program of laboratory and field studies undertaken to assess the health impact of radioactive debris from the re-entry of the Soviety nuclear-powered satellite, Cosmos 954, on 24 January 1978. It was estimated that about one-quarter of the reactor core descended over Canada's Northwest Territories in the form of submillimetre particles. The other three-quarters apparently remained as fine dust in the upper atmosphere. Each particle contained megabecquerel quantities of the fission products 95Zr, 95Nb, 103Ru, 106Ru, 141Ce and 144Ce, as well as traces of other fission and activation products. Laboratory tests indicated that these radionuclides would not dissolve significantly in drinking water supplies or in dilute acids. Contamination of air, drinking water, soil and food supplies was not detected. The dose equivalent to the GI tract for an individual who might have inhaled or ingested a particle could have been as high as 140 mSv.

Expression of an artificial yeast TRP-gene cluster in yeast and Escherichia coli.

All five tryptophan biosynthetic genes of Saccharomyces cerevisiae were unified on plasmid pME554, which is based on 2 micrometer DNA and pBR322 sequences allowing for autonomous replication in yeast and E. coli. Homologous and heterologous expression of this artificial yeast TRP-gene cluster was studied. Plasmid pME554 allowed for nearly normal growth of a yeast strain bearing auxotrophic mutations in all five TRP-genes. The plasmid-borne genes TRP2 to TRP5 were expressed and regulated normally in the frame of the general control. Gene TRP1, carried on an EcoRI/Bg/II fragment lacking the ARS1 function, was expressed poorly and did not respond to the general control like the chromosomally-borne TRP1 gene. Plasmid pME554 allowed for poor growth of E. coli strain W3110 tna- delta trpEA2 on minimal medium. Marked stimulation was observed, however, when anthranilic acid or indole were added. Accordingly, poor expression of the first Trp-enzyme anthranilate synthase and the last enzyme tryptophan synthase was found, whereas the other three genes were moderately well expressed in E. coli.

Structure and function of the TRP3 gene of Saccharomyces cerevisiae: Analysis of 3'- and 5'-deletions in vivo and in vitro.

Two sets of deletions, entering the TRP3 gene of Saccharomyces cerevisiae from the 3'- and the 5'-end were constructed. Complementation analysis with chromosomal trp3A, trp3B and trp3C mutations was done by introducing the 3'- and 5'-truncated gene on a multicopy 2 µm-vector. The N-terminal glutamine amido transferase function is encoded by a DNA fragment of 600-700 bp, and the C-terminal indole-3-glycerol-phosphate synthase function by a DNA fragment of about 900 bp, whereas both functions together are encoded by a contiguous DNA fragment of about 1,500 bp. The bi functional TRP3-peptide thus could be dissected into two catalytically independent peptides in vivo.For the indole-3-glycerol-phosphate synthase activity, independent catalytic activity was also demonstrated in vitro: deletions entering the TRP3 gene from the 5'-end, and lacking large parts of the sequence coding for the glutamine amidotransferase function, still are able to ex press a peptide exhibiting functional indole-3-glycerol phosphate synthase activity in vitro. Deletion plasmids pME505·De1C102·2µm and DelC10·2µm exhibited shorter TRP3 transcripts according to the deleted DNA-fragments (150 and 426 by respectively) but yielded peptides of invariable Mr of 35,000 d. Transcription and translation of these peptides, which probably represent the independently folding indole-3-glycerol-phosphate synthase core are discussed.

Transfer of 226Ra, 210Pb and uranium from soil to garden produce: assessment of risk.

A study of gardens contaminated by uranium processing wastes was carried out in the town of Port Hope, Ontario, during the summer of 1976. Concentrations of 226Ra, 210Pb, and total uranium were measured in soils and in garden produce. Highest concentrations were found in root and stem vegetables and, for radium, also in leafy vegetables. Fruit generally showed the lowest values. Uptake was consistent with a linear dependence on soil concentration. Mean concentration factors of 1.1 X 10(-3), 0.37 X 10(-3), and 0.075 X 10(-3) were obtained for 226Ra, 210Pb, and uranium, respectively. The highest estimated dose to an individual who ingested produce from a contaminated garden was 68 mrem/yr to bone surface from 226Ra. The implications of these findings for other communities affected by nuclear fuel-cycle operations are discussed.