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H Djonlagic

Publications and source records attributed to H Djonlagic.

At least 19 recordsLinked to original sources

Effective long-term control of cardiac events with beta-blockers in a family with a common LQT1 mutation.

The congenital long QT syndrome (LQTS) is characterized by a prolonged QT interval on the surface electrocardiogram and an increased risk of recurrent syncope and sudden cardiac death. Mutations in seven genes have been identified as the molecular basis of LQTS. beta-blockers are the treatment of choice to reduce cardiac symptoms. However, long-term follow-up of genotyped families with LQTS has been rarely reported. We have clinically followed a four-generation family with LQTS being treated with beta-blocker therapy over a period of 23 years. Seven family members were carriers of two amino acid alterations in cis (V254M-V417M) in the cardiac potassium channel gene KCNQ1. Voltage-clamp recordings of mutant KCNQ1 protein in Xenopus oocytes showed that only the V254M mutation reduced the IKs current and that the effect of the V417M variant was negligible. The family exhibited the complete clinical spectrum of the disease, from asymptomatic patients to victims of sudden death before beta-blocker therapy. There was no significant reduction in QTc (556 +/- 40 ms(1/2) before therapy, 494 +/- 20 ms(1/2) during 17 years of treatment; n = 5 individuals). Of nine family members, one female died suddenly before treatment, three females of the second generation were asymptomatic, and four individuals of the third and fourth generation were symptomatic. All mutation carriers were treated with beta-blockers and remained asymptomatic for a follow-up up to 23 years. Long-term follow-up of a LQT1 family with a common mutation (V254M) being on beta-blocker therapy was effective and safe. This study underscores the importance of long-term follow-up in families with specific LQT mutations to provide valuable information for clinicians for an appropriate antiarrhythmic treatment.

Adrenergic beta-Antagonists↗

[Toxic shock syndrome with multi-organ involvement].

HISTORY AND ADMISSION FINDINGS: An 18-year-old school girl was referred for admission by another hospital because of headache, joint pains, fever, vomiting, diarrhoea and orthostatic syncope associated with renal failure. On admission he was somnolent with a blood pressure of 90/60 mmHg, heart rate of 104 beats/min and a slight fever of 39.1 degrees C. A sunburn-like skin rash was noted. INVESTIGATIONS: Laboratory tests indicated low levels of platelets and calcium, increased levels of white cells, C-reactive protein, creatinine, bilirubin, transaminases, creatinekinase and lactate. Chest X-ray demonstrated diffuse shadows, while other imaging revealed a space-occupying lesion, ca. 3 cm in diameter, in the right lowere quadrant of the abdomen. The patient was hypoxic. Microbiology revealed vaginal colonies of Staph. aureus (producing toxic shock syndrome toxin 1 [TSST-1]). Serum antibody titre against TSST-1 was less than 1:25. DIAGNOSIS, TREATMENT AND COURSE: A toxic shock syndrome (TSS) with multi-organ involvement was suspected because of the association of menstruation with the use of tampons. An inserted tampon was removed. At laparoscopy the space-occupying lesion proved to be a haematoma. As bacterial septicaemia could not ne excluded broad-spectrum antibiotics were administered together with symptomatic measures. The patient fully recovered within a week. The characteristic skin desquamation confirmed the diagnosis of TSS. CONCLUSION: In its acute phase the diagnosis of TSS is often uncertain. The initial symptoms are nonspecific and numerous conditions need to be considered in the differential diagnosis. The diagnosis can be confirmed, if at all, only in the convalescent phase by the skin desquamation or a rise in anti-TSST-1 antibody titre. A search for a focus of infection is essential for differentiation from a non-menstrual TSS, even if there is as association with menstruation.

Adolescent↗

Effectiveness of end-tidal carbon dioxide tension for monitoring thrombolytic therapy in acute pulmonary embolism.

OBJECTIVE: In acute massive pulmonary embolism with hemodynamic instability, monitoring of pulmonary artery pressure can be used to assess the efficacy of thrombolytic therapy. As a noninvasive alternative to pulmonary artery catheterization, we investigated the efficacy of continuous monitoring of end-tidal CO2 tension. DESIGN: In 12 patients with massive pulmonary embolism who required mechanical ventilation, mean pulmonary arterial pressure (MPAP) and end-tidal carbon dioxide tension (ETCO2) were registered continuously during thrombolytic therapy. PaCO2, cardiac index as estimated by thermodilution catheter and respiratory ratio of arterial oxygen tension and inhaled oxygen concentration (PaO2/FIO2) were determined every 60 mins. MEASUREMENTS AND MAIN RESULTS: Before thrombolysis, MPAP (34.5+/-9.8 mm Hg) and the difference between PaCO2 and ETCO2 (10.1+/-4.7 mm Hg) were markedly increased compared with normal values. Continuously monitored MPAP was related to ETCO2 for both all patients (r2 = .42; p < .001) and individually (mean r2 = .92; range, .79-.98; p < .001). In ten survivors, the mean cardiac index and PaO2/FIO2 increased during therapy from 1.7+/-0.4 to 2.8+/-0.6 L/min x m2 and 125+/-27 to 285+/-50 mm Hg (p < .01, respectively). In these patients, the difference between PaCO2 and ETCO2 decreased from 9.8+/-4.5 to 2.8+/-0.9 mm Hg (p < .001). Recurrent embolism was detected in two patients by sudden reduction of ETCO2. CONCLUSIONS: Analysis of ETCO2 allows monitoring of the efficacy of thrombolysis and may reflect recurrent embolism. Thus, on the basis of this small study, analysis of ETCO2 appears to be useful for noninvasive monitoring in mechanically ventilated patients with massive pulmonary embolism.

Acute Disease↗

[Sensorimotor polyneuropathy and systemic amyloidosis as paraneoplastic symptoms of a carcinoid-like well differentiated carcinoma of the breast].

HISTORY AND ADMISSION FINDINGS: A 75-year-old woman was admitted because of weakness and paraesthesias in both legs. 15 years earlier a chest radiogram had shown numerous round foci of uncertain cause. Physical examination confirmed muscular weakness in all limbs, especially the legs, as well as abnormal superficial and deep sensory perception. INVESTIGATIONS: Electromyography registered a patchy pattern of intentional muscular activity and marked denervation activity but largely normal nerve conduction. There was no evidence of inflammatory disease in the laboratory tests, including the CNS. DIAGNOSIS, COURSE AND TREATMENT: The neurological findings indicated progressive axonal sensorimotor polyneuropathy. A paraneoplastic cause was suspected, but search for a primary tumour was unsuccessful. The patient developed a urinary infection from which she died in septic shock. A carcinoid-like well-differentiated carcinoma of the breast with hematogenous metastases to liver, spleen and lung was discovered at autopsy. It also revealed severe systemic AA-type amyloidosis, involving liver, spleen heart and kidneys. In addition there was a severe progressive axonal neuropathy and marked neurogenic atrophy of the peripheral skeletal musculature. No amyloid deposition was seen. CONCLUSION: In case of amyloidosis and polyneuropathy of uncertain cause a paraneoplastic pathogenesis should be considered in the differential diagnosis, even in the absence of proven malignancy.

Aged↗

Production of reactive oxygen species by central venous and arterial neutrophils in severe pneumonia and cardiac lung edema.

OBJECTIVE: In pneumonia the influx of neutrophils to the lungs is thought to be of primary importance with regard to host defence and to complications like the adult respiratory distress syndrome. We wanted to evaluate the neutrophil function in patients in acute respiratory failure who required admission to the intensive care unit. DESIGN: We determined the luminolenhanced chemiluminescence (CL) of neutrophils isolated both from central venous and arterial blood. In addition, the plasma-concentrations of alpha 1-proteinase inhibitor, (alpha 1PI), alpha 2-macroglobulin (alpha 2PI) and elastase-alpha 1PI-complex (elastase) were determined by chemiluminescence immunoassay, and the intracellular elastase content of blood neutrophils was determined using immuno activation assay. PATIENTS: 28 Patients, 18 with acute pneumonia (group 1) and 10 with cardiac pulmonary edema (group 2). MEASUREMENTS AND RESULTS: In group 1, luminol enhanced CL was significantly higher than in group 2 (mean 87.7 vs 30.4 x 10(6) counts per minute, p < 0.01). The production of reactive oxygen species was significantly higher in central venous than in arterial neutrophils in the patients with pneumonia (p < 0.03). In patients with pulmonary edema there was no such difference. The plasma concentration of elastase in group 1 was significantly higher than in group 2, that of alpha 2PI were significantly lower. The intracellular elastase content of neutrophils was lower in group 1 than in group 2. In group 1, there was a trend for a correlation between lower intracellular elastase content and a higher elastase plasma concentration. There were no central venous-arterial differences with regard to leukocyte count, cell differential or protein concentration in either group. CONCLUSION: The central venous-arterial differences in neutrophil production of reactive oxygen species support the concept of compartmentalization of activated neutrophils from the systemic to the pulmonary compartment.

Adult↗

Impact of early accelerated dose tissue plasminogen activator on in-hospital patency of the infarcted vessel in patients with acute right ventricular infarction.

OBJECTIVE: To assess the efficacy of early accelerated dose tissue plasminogen activator on in-hospital patency of the infarct related artery in patients with inferior myocardial infarction with and without right ventricular involvement. DESIGN: Single centre prospective assessment before discharge of infarct related vessel patency after early thrombolysis. SETTING: Tertiary cardiac referral centre at a university hospital. PATIENTS AND METHODS: 90 consecutive unselected patients with acute myocardial infarction, of whom 35 (39%) had electro-cardiographic evidence of right ventricular involvement (ST segment elevation greater than 0.1 mV in right precordial lead V4R), were studied. All patients received accelerated dose tissue plasminogen activator 100 mg within six hours from the onset of symptoms and had control angiography before discharge. MAIN OUTCOME MEASURES: Infarct related coronary artery patency using the Thrombolysis in Myocardial Infarction (TIMI) grading system before discharge. Incidence of prolonged systemic hypotension, sinus bradycardia, complete atrioventricular block, and ventricular tachyarrhythmia during early hospitalisation. RESULTS: Despite aspirin and bolus heparinisation before thrombolysis and high dose heparinisation thereafter for at least 48 hours the infarct related artery was more likely to be occluded (TIMI 0 or 1 flow) in patients with right ventricular involvement than in those without (69 v 29%, P < 0.001), as shown by control angiography performed a mean of 12.8 days after thrombolysis. These findings may be explained, at least in part, by predominant involvement of the proximal right coronary artery (66 v 31%, P < 0.05) and a low cardiac output syndrome, being indirectly reflected by a high incidence of prolonged hypotension (26 v 7%, P = 0.02), bradycardia (34 v 14%, P = 0.03), and complete atrioventricular block (37 v 5%, P = 0.0001). CONCLUSION: Primary angioplasty should be considered as the treatment of choice in patients with acute inferior infarction with right ventricular involvement because of the high failure rate of thrombolysis.

Coronary Angiography↗

[Severe metabolic alkalosis with a consciousness disorder].

HISTORY AND CLINICAL FINDINGS: A 47-year-old man in a reduced general condition, presumed to be a chronic alcoholic, was hospitalised in a sleepy state and impaired level of consciousness (Glasgow Coma Scale 8). There were no focal neurological deficits, but all proprioceptor reflexes were weak. Body temperature was 36.8 degrees C, blood pressure 90/60 mm Hg, and heart rate 80/min. INVESTIGATIONS: Biochemical tests showed sodium concentration reduced to 121 mmol/l, potassium to 1.83 mmol/l, chloride to 55 mmol/l and, on the next day, phosphate to 0.11 mmol/l. Blood gas analysis demonstrated a noncompensated respiratory alkalosis (pH 7.69, bicarbonate 39.5 mmol/l and a base excess of 20 mmol/l. TREATMENT AND COURSE: The impaired consciousness was thought to be due to the marked alkalosis in combination with hypophosphataemia. The alkalosis was completely removed within 48 hours by administration of Ringer's solution and potassium chloride concentrate, without sodium chloride Phosphate deficit was neutralised with KH2PO4 infusion. Normal consciousness was restored. CONCLUSIONS: Even severe hypochloraemic alkalosis can be quickly reversed with infusion of chloride without sodium Successful treatment with chloride alone excludes alkalosis induced by mineralocorticoids.

Acute Disease↗

Accelerated ST-segment reduction after thrombolytic therapy with recombinant tissue plasminogen activator (rtPA) compared to urokinase.

Effects of therapy with urokinase (UK) and with recombinant tissue plasminogen activator (rtPA) were compared in patients with acute myocardial infarction (AMI). To achieve homogenous therapeutic conditions the comparison was restricted to patients having their first AMI and to cases of clinically successful thrombolytic therapy (defined by non-invasive criteria, such as a 50% decrease in elevated ST-segment in the worst load of a 12 lead ECG within 300 min after onset of thrombolytic therapy, complete pain resolution during thrombolytic therapy, and later confirmed by angiography 10 days after AMI). Effects of UK and rtPA on continuous multilead ST-segment analysis and cardiac proteins (creatine kinase and its isoenzyme CK-MB, aspartate transaminase and hydroxybutyrate dehydrogenase) were analyzed during 24 hours following onset of therapy. Continuous ST analysis showed a faster resolution of the elevated ST-segments after thrombolytic therapy with rtPA than with UK(p < 0.01). Accelerated idioventricular rhythms (p < 0.05) occurred sooner following rtPA than UK treatment. The wash-out of creatine kinase was increased (p < 0.01) after rtPA. Although both drugs induced comparable, angiographically controlled reperfusion, the results suggest that the process of reperfusion was accelerated during thrombolysis with rtPA compared to UK. Thrombolytic therapy of AMI with rtPA may hence improve myocardial salvage.

Adult↗

Treatment of a patient with severe digitoxin intoxication by Fab fragments of anti-digitalis antibodies.

A massive digitoxin (DGTX) intoxication in a 36-year-old man (35 mg DGTX) was treated by prolonged and repeated i.v.-infusions of Fab fragments of anti-digitalis antibodies (FAB). Blood and urine samples were collected over a 98 h period for monitoring the efficacy and adequacy of FAB treatment. DGTX concentrations were determined after protein precipitation (release of FAB-bound and protein-bound DGTX) in unprocessed serum and urine samples, and after aliquots of these samples had been dialysed in vitro against DGTX-free buffer (elimination of DGTX not bound to FAB). The difference in DGTX concentration between the unprocessed and dialysed samples was the amount of DGTX bound to plasma proteins and the small fraction of unbound DGTX being relevant for the therapeutic and toxic effects of the drug. Before FAB therapy was started, the total serum DGTX concentration was 535 nmol/l. The first FAB infusion (320 mg) was started 11 h after drug ingestion. Since this amount of FAB was insufficient to bind all DGTX present in the serum, cardiac DGTX toxicity (total AV-block) persisted. During a second FAB infusion (400 mg) the patient reverted to regular AV-conduction. At this time most of the DGTX in serum was FAB-bound. Toxic symptoms (sinus arrest) reappeared twice and were accompanied by increasing amounts of non-antibody-bound DGTX in the serum. Additional application of FAB (2 x 80 mg) resulted in the immediate disappearance of arrhythmia. During FAB-treatment total DGTX serum concentrations and renal DGTX clearance rose, indicating redistribution of drug from tissue to serum and urinary elimination of FAB-bound DGTX, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Clostridial sepsis with massive intravascular hemolysis: rapid diagnosis and successful treatment.

A 61-year-old man developed a pyrescia accompanied by a massive intravascular hemolysis after abdominal surgery (Whipple's operation) of a pancreatic adenocarcinoma. Abdominal ultrasound and the abdominal CT-scan showed marked aerobilia and multiple liver abscesses. Laboratory tests demonstrated the presence of the Thomsen-Friedenreich cryptantigen (TCA) on the membranes of the patient's erythrocytes. The enzymatic cleavage of N-acetyl-neuraminic acid usually covering the TCA may lead to a life threatening intravascular hemolysis. Since Clostridial bacteriae typically synthesize neuraminidase, the presumptive diagnosis of Clostridial sepsis complicated by massive hemolysis was made. Immediate antibiotic therapy including penicillin G and metronidazole stopped hemolysis within a few hours and the patient servived. On the following day, microbiological examination identified Clostridium perfringens in the patient's blood cultures. Clostrial sepsis should be suspected in patients with underlying infections and/or malignant diseases, particularly of the gastrointestinal or genitourinary tract, who present with septic shock and acute intravascular hemolysis. Whereas microbiological specification of the organism is time consuming, the relatively simple agglutination test with anti-TCA peanut lectin can provide a rapid presumptive diagnosis. The immediate onset of an appropriate antimicrobial therapy is of central importance and might be life-saving.

Adenocarcinoma↗

Morphologic alterations in the long Q-T syndrome. Light and electron microscopic observations in the conduction system and in sympathetic trunks.

Post-mortem examinations of 2 patients with long Q-T syndrome revealed marked focal fibrosis and lipomatosis of the conduction system as well as focal round cell ganglionitis of both sympathetic trunks. The patients, young women of different nationality displaying syncopal attacks and a long Q-T interval in the ECG died suddenly of ventricular arrhythmias. A family study of one of them revealed Q-T prolongation in 4 generations. The ultrastructure of the conductive tissue and the ventricular myocardium showed no specific alterations indicative of a primary metabolic defect. Ganglionitis of the sympathetic trunks has not yet been reported in the long Q-T syndrome. Although the etiology of the inflammatory changes is uncertain a chronic viral infection, noninfectious toxic alterations or an autoimmunopathy are among the plausible causes discussed.

Adult↗

[Pulmonary effect of inhaling leather-impregnation sprays (author's transl)].

After inhaling a leather-impregnation spray in a confined space, coughing fits and dyspnoea occurred in a 40-year-old man and an 20-year-old woman, previously in good health. The cardinal symptoms on admission to an emergency ward were of interstitial pulmonary oedema in the X-ray but without increased pulmonary arterial pressure. Administration of corticosteroids both by aerosol and intravenously improved the acute symptoms within a few hours. Two subsequent chest X-rays demonstrated complete healing. The acute symptoms were caused by the impregnation spray producing an alveolitis or toxic lung oedema in both cases. The early topical administration of corticosteroids would appear to be the most important preventive and protective measure.

Adult↗