Progressive tubulointerstitial nephritis and chronic cholestatic liver disease

We report the clinical and morphological features of a distinctive hepatorenal disorder in four patients and review the five similar patients in the literature. The main clinical characteristics were early onset of cholestatic liver disease and progressive tubulointerstitial nephritis leading to renal death in early childhood. Liver histology showed disturbed architecture with nodular and acinar formations and portal fibrosis and bile duct proliferation. Histological abnormalities in the kidney were severe interstitial fibrosis and tubular atrophy and dilatation, while the typical features of nephronophthisis were lacking. These clinical and morphological characteristics distinguish our patients from the majority described, as having nephronophthisis and congenital hepatic fibrosis or any other known syndrome with concomitant hepatorenel involvement. We suggest that the association of cholestatic liver disease and progressive tubulointerstitial nephritis represents a new syndrome.     

Properties of visual evoked potentials to onset of movement on a television screen

In 80 subjects the dependence of movement-onset visual evoked potentials on some measures of stimulation was examined, and these responses were compared with pattern-reversal visual evoked potentials to verify the effectiveness of pattern movement application for visual evoked potential acquisition. Horizontally moving vertical gratings were generated on a television screen. The typical movement-onset reactions were characterized by one marked negative peak only, with a peak time between 140 and 200ms. In all subjects the sufficient stimulus duration for acquisition of movement-onset-related visual evoked potentials was 100ms; in some cases it was only 20ms. Higher velocity (5.6°/s) produced higher amplitudes of movement-onset visual evoked potentials than did the lower velocity (2.8°/s). In 80% of subjects, the more distinct reactions were found in the leads from lateral occipital areas (in 60% from the right hemisphere), with no correlation to handedness of subjects. Unlike pattern-reversal visual evoked potentials, the movement-onset responses tended to be larger to extramacular stimulation (annular target of 5°–9°) than to macular stimulation (circular target of 5° diameter).Abbreviation PREP pattern-reversal visual evoked potentials     

P53 overexpression as an indicator of overall survival and response to treatment in osteosarcomas

The p53 gene located at chromosome 17pl3 is found to be altered (allelic loss or other mutation) in multiple human cancers, including osteosarcomas. The mutated gene produces a protein with a prolonged half-life thus rendering it detectable by conventional immunohistochemistry. We examined the correlation between p53 expression and clinical prognosis as well as response to therapy. Twentyone patients with previously untreated and histologically verified highly malignant osteosarcoma were used for this study. Biopsy material taken both prior to the start of COSS 91 protocol and at the time of surgery (ten weeks later) was examined for alterations in p53 protein expression and drug resistance. Two patients who had strong (+++) p53 protein expression and three others who became positive during the chemotherapy had significantly worse prognosis (all of them died within one year) than those who showed no p53 expression both at biopsy and after chemotherapy (all 11 patients are alive, average follow-up time: 3.5 years). All patients who showed any kind of positive p53 protein expression on initial biopsy were non-respon-ders to chemotherapy. In contrast, 69% (9 out of 13) of those who exhibited no p53 expression on initial biopsy were responders or intermediate responders to chemotherapy. We concluded that p53 expression may be a useful prognostic factor in osteosarcomas. The direct correlation between p53 positive expression and resistance to therapy can help in identifying patients who are in need of a more vigorous or different chemotherapeutical protocol.     

Convulsant action of a benzodiazepine receptor agonist/inverse agonist Ro 19-4603 in developing rats

An inverse benzodiazepine receptor agonist Ro 19-4603, administered intraperitoneally, was found to induce two types of motor seizures, i.e. minimal, predominantly clonic and major, generalized tonic-clonic, in rats at all developmental stages studied (7, 12, 18 and 25 days old). The developmental profile of the two types of seizure was different. Minimal seizures could be induced easily in the two youngest groups, whereas there were no marked differences in the induction of major seizures between the age groups. A lethal outcome was more common in 18- and 25-day-old rats than in younger animals. The convulsant action of the benzodiazepine agonist/inverse agonist Ro 19-4603 shows only quantitative changes during post-natal development in the rat.Abbreviations DPPC Diplamitoylphosphat     

Incidence of IDDM during 1988–1992 in Zagreb,Croatia

Summary The objective of this study was to determine the incidence of insulin-dependent diabetes mellitus (IDDM) in the population of Zagreb, Croatia, during 1988–1992. A centralized diabetes registry was the primary source of data, while secondary sources were used to assess ascertainment. A total of 282 new cases of IDDM were diagnosed in the study period, the primary and secondary sources identifying annually 93–100% of the cases. The annual incidence rate ranged from 5.6 per 100,000 to 6.6 per 100,000. Early fatality in persons older than 50 years was the major cause of underascertainment. The incidence peaked in the 10–14 years age group (12.4 per 100,000), and remained stable after age 24 years. Males had a significantly higher incidence in the 5–9 and 24–44 years age groups. In the 45–54 years age group, females had a significantly higher incidence. No seasonality was observed. Despite the war conditions in Croatia, the low overall IDDM incidence rates did not change significantly during the study period.Abbreviations IDDM Insulin-dependent diabetes mellitus - DKA diabetic ketoacidosis     

Incidence and mechanisms of aminoglycoside resistance inPseudomonas aeruginosa serotype O11 isolates

Summary Mechanisms of resistance to five aminoglycoside antibiotics: gentamicin (G), tobramycin (T), netilmicin (N), amikacin (A) and isepamicin (I), were assessed in 16 clinical isolates ofPseudomonas aeruginosa serotype O11, originating from five hospitals in Bratislava. All isolates werein vitro highly resistant to all mentioned aminoglycoside antibiotics (MIC>32 mg/l). Thirteen isolates produced three aminoglycoside-modifying enzymes (AGME), responsible for resistance to the respective aminoglycosides: AAC(6)-I (T, N, A); APH (2) (G, T); APH (3)-VI (I). In addition to this, in four isolates a production of AAC(3)-II (G, T, N) was observed. In three isolates no production of AGME was observed. The strains studied were isolated mainly from urine. Several isolates were able to transfer aminoglycoside resistance by bacterial conjugation toP. aeruginosa 1008 rif^r recipient. The transconjugants from these transfers expressed the same resistance pattern and nearly the same mechanisms of resistance as the donor strains.

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Inzidenz und Mechanismus der Aminoglykosid-Resistenz beiPseudomonas aeruginosa O11-Isolaten

Zusammenfassung Bei 16 klinischen Isolaten vonPseudomonas aeruginosa Serotyp O11 aus fünf Krankenhäusern in Bratislava wurde der Mechanismus der Resistenz gegen fünf Aminoglykosid-Antibiotika untersucht; Gentamicin (G), Tobramycin (T), Netilmicin (N), Amikacin (A) und Isepamicin (I). Alle Isolate warenin vitro hochresistent gegen alle genannten Aminoglykosid-Antibiotika (MHK-Werte >32 mg/l). 13 der Isolate bildeten drei Aminoglykosid-modifizierende Enzyme (AGME), die für die Resistenz gegenüber den entsprechenden Aminoglykosiden verantwortlich waren: AAC(6)-I (TNA): APH (2) (G, T): APH (3)-VI (I). Zusätzlich wurde bei drei Isolaten die Produktion von AAC(3)-II (G, T. N) beobachtet. In drei Isolaten fand sich keine Produktion von AGME. Die untersuchten Stämme waren überwiegend aus Urin isoliert worden. Mehrere der Isolate waren fähig, die Aminoglykosid-Resistenz durch bakterielle Konjugation auf denP. aeruginosa 1008 rif^r Rezipienten zu übertragen. Die Transkonjugate von diesen Übertragungen exprimierten dasselbe Resistenzmuster und wiesen nahezu denselben Resistenzmechanismus auf wie die Spenderstämme.

     

An external approach without tracheotomy for submucosal cordectomy with laterofixation for bilateral abductor paralysis of the vocal folds

There are numerous surgical procedures now available to manage bilateral abductor paralyses of the vocal folds. These procedures have various success rates but mostly do not offer reliable and predictable postoperative results, and usually require ,tracheotomy. The technique described in this report is based on a trial study to obtain a safe airway for an affected patient and avoid tracheotomy, if possible. Submucosal cordectomy was used with lateral fixation of one vocal fold and preservation of the arytenoid. The procedure was performed by using continuous intravenous anesthesia, which does not require intubation of the patient's airway. Seven patients were operated with this technique with excellent postoperative results. The glottal airway was largely improved in all patients, with only minor temporary complications.     

Isolated hydatid cyst of the adrenal gland

Hydatide disease of the adrenal gland is extremely rare, even in disseminated disease. Isolated cyst of the gland is even more rare. Only 9 of such cases seem to have been described so far. We present a 49 year old woman in whom a hydatide cyst of the right gland was found during the investigation for the dull pain in the area. She was successfully operated. Hydatide etiology was confirmed at operation and by histology. She had an uneventful recovery. The preoperative pain disappeared postoperatively.     

Diverticulum of the stomach

Gastric diverticula are very rare and usually asymptomatic diseases. Clinical symptoms may include haemorrhage, mild discomfort and pain in upper abdomen. They are most frequently diagnosed incidentally during X-Ray examinations. In symptomatic diverticulum of gastric fundus was diagnosed roentgenographically and endoscopically and was successfully cured surgically.