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Biomedical subjects

E Rosenthal

Publications and source records attributed to E Rosenthal.

At least 109 records · Page 6Linked to original sources
PubMed1997

Progressive familial intrahepatic cholestasis among the Arab population in Israel.

BACKGROUND: Progressive familial intrahepatic cholestasis, which constitutes a heterogeneous group of imperfectly delineated syndromes and appears to be inherited as an autosomal recessive condition, has not been hitherto reported from the Middle East, in spite of the high rate of consanguineous marriage in this region. METHODS: Sixteen affected children from six Israeli Arab families were evaluated over 30 years. All were born to consanguineous parents. RESULTS: Jaundice appeared during the first 3 weeks of life in 15 babies. When first referred, 10 had hepatomegaly and nine had splenomegaly. A progression toward cirrhosis was the rule. Serum levels of conjugated bilirubin, liver enzymes, and alkaline phosphatase were raised; gamma-glutamyl transpeptidase levels were normal in all three infants in whom it was examined, but elevated in two siblings of another family at ages 2 and 3 years. No abnormal bile acids were detected in the serum and urine of patients. Histologic examination of the liver showed giant-cell transformation, paucity of intrahepatic bile ducts, cholestasis, fibrosis, or cirrhosis. The pattern of liver pathology differed at times among affected members within the same family. Therapeutic trials with phenobarbital, cholestyramine, or ursodeoxycholic acid were ineffective. Survival of the patients was from 5 to 18 months in four families; in the other two families, three children received liver transplants, and one is awaiting liver transplantation. CONCLUSIONS: Progressive familial intrahepatic cholestasis should be included in the differential diagnosis of infants with cholestatic jaundice of unknown etiology, especially those born to consanguineous Arab parents.

Arabs↗
PubMed1997

Single pass VDD pacing in children and adolescents.

Use of a single pass lead for VDD pacing in complete heart block is well described in adults, but there are only brief reports of its use in children. We have used standard adult size single pass leads in 13 children and adolescents aged 3.7-17.2 years (mean 10.1 years) and weighing 13.5-76 kg (mean 34.8 kg). Congenital complete heart block was present in 7 patients, surgical complete heart block in 5 patients and 2:1 AV block of unknown cause in 1 patient. In four patients, the VDD system was their first pacing system. In nine of the patients, 1-6 previous systems had been used and simultaneous extraction of ventricular leads and/or atrial leads was performed. Leads of four different types were used: Brilliant IMP15Q, Brilliant + IMR15Q, CapSure 5032, and Unipass 425-13 with 4 different generators: Saphir 600, Saphir II 620, Thera VDD 8948, and Unity 292-07. At implantation, via a subclavian vein puncture, excess lead was advanced into the right atrium to allow both atrial sensing and slack for further growth. Ventricular thresholds ranged from 0.2-0.7 V. The minimal atrial amplitude was 0.7-4 mV and the maximum amplitude was 2.5-8 mV. There were no complications. All patients have maintained adequate atrial signals for reliable pacing with follow up of 3-36 months (mean 17.6 months) during which time some have undergone considerable growth. Reliable atrial synchronous ventricular pacing is possible in growing children with complete heart block using a standard adult single pass lead.

Adolescent↗
PubMed1997

VDD pacing in children with congenital complete heart block: advantages of a single pass lead.

A single pass lead for VDD pacing in complete heart block is well described in adults but there are only brief reports of its use in children. We used standard adult size single pass leads in ten children and adolescents aged 3.7-17.2 years (mean 9.9 years) and weighing 13.5-76 kg (mean 35.4 kg) with congenital complete heart block. One patient had coexisting congenital heart disease and had undergone surgery. A 2:1 atrioventricular block in one patient was presumed to be congenital in origin. In four patients, the VDD system was their first pacing system. In six of the patients, 1-4 previous systems had been used and simultaneous extraction of ventricular leads (6) and/or atrial leads (2) was performed. Four different types of lead were used: Brilliant IMP15Q (Vitatron); Brilliant + IMR15Q (Vitatron); CapSure 5032 (Medtronic); and Unipass 425-13 (Intermedics) with four different generators: Saphir 600 (Vitatron); Saphir II 620 (Vitatron); Thera VDD 8948 (Medtronic); and Unity 292-07 (Intermedics). All leads were introduced via a subclavian vein puncture and the atrial dipole was placed low in the right atrium to provide slack for further growth while maintaining atrial sensing. Ventricular thresholds ranged from 0.2-0.8 V. The minimal atrial amplitude was 0.7-4 mV and the maximum amplitude was 2.5-8 mV. There was one early microdisplacement and the lead was repositioned. Over a follow-up period ranging from 1-39 months (mean 20.4 months), all patients have maintained low ventricular pacing thresholds and adequate atrial signals for reliable pacing at rest and with exercise. During this time some have undergone considerable growth. The patient with coexisting congenital heart disease died suddenly at 3 years, but the pacing system had no fault at autopsy. The standard adult size single pass lead provides a simple means to enable reliable atrial synchronous ventricular pacing in growing children with complete heart block.

Adolescent↗
PubMed1997

Use of an atrial loop to extend the duration of endocardial pacing in a neonate.

Use of an atrial loop has been proposed as a means of extending the longevity of endocardial pacing systems in small children who require ventricular pacing. A few reports have demonstrated the effectiveness of this method at reducing the number of interventions in infants and small children, but there is little reported experience in neonates. Permanent endocardial ventricular demand pacing was performed in a 3.4-kg neonate. The generator was placed in a prepectoral pocket and a redundant loop of lead was left in the atrium to cater for further growth. At 32 months he weighs 13.6 kg and the loop has uncoiled leaving additional lead slack for further growth.

Endocardium↗
PubMed1997

Iatrogenic atrioventricular bypass tract following a Fontan operation for tricuspid atresia.

A 16 year old female with tricuspid atresia had undergone a Fontan operation at four years of age. Two years later she first presented with a narrow complex tachycardia which could only be partially controlled on flecainide in high doses. On electrophysiological study, the tachycardia was found to be due to atrioventricular re-entry within the surgical right atrial to right ventricular outflow tract anastomosis. Radiofrequency ablation at this site abolished the arrhythmia and she is now symptom-free on no medication.

Adolescent↗
PubMed1997

The limits of history-taking in geriatric depression.

The authors assessed the limits of reliable history-taking in depressed elderly patients (N = 20) with some cognitive impairment. Each subject and an informant was interviewed with structured instruments by two trained raters. An expert panel formed consensus judgments after reviewing information reported by the patients, the informants, and each of the clinical raters. Intraclass correlation between the two raters was 0.99 for the duration of depressive episodes and 0.88 for age at onset. The raters agreed on the duration of major depressive episodes in 85% of cases and on age at onset in 80% of cases. The duration of previous depressive episodes and age at depression onset cannot always be determined reliably even when informants and structured interviews are used. Greater difficulties may be encountered in patients with minor depression or chronic intermittent depression and early-onset depression. Clinicians should obtain history from as many reliable sources as possible and critically evaluate this information while considering the entire clinical picture. The aggregate kappa statistic can provide a clinically meaningful way of assessing interrater reliability of psychopathological constructs for which several definitions are used.

Age of Onset↗
PubMed1996

Implantation of Palmaz stents in branch pulmonary arteries using Olbert balloons.

Twenty-three patients, ranging in weight between 4.9 and 74 kg, underwent stent implantation in branch pulmonary arteries. At first, high-pressure balloons were used for stent implantation but, because of stents slipping off the balloons, subsequently in eight patients, aged 2-18 years and weighing 11-72 kg, Olbert balloons were used. Fifteen stents were implanted in 11 vessels in these 8 patients. No instance of stent slippage occurred during introduction through the sheath or when the stent was passed around the curves of the right heart. In one patient, the fully expanded stent slipped over the wire into the main pulmonary artery. This occurred because of undersizing of the stent. The stent was re-advanced into position and expanded using a larger balloon. No significant complications occurred with these balloons. The use of Olbert balloons for stent implantation in branch pulmonary arteries is recommended.

Adolescent↗
PubMed1996

Medium-term results of experimental stent implantation into the ductus arteriosus.

Balloon dilation is disappointing in maintaining patency of the arterial duct. In neonatal lambs, stent implantation in the arterial duct results in significantly larger ducts with greater pulmonary blood flow than balloon dilation. Little is known, however, about the duration of duct patency after stent implantation. The outcome of stent implantation into the arterial duct in 12 lambs was observed over a period of 1 to 24 months. Stents (Wallstent in 9, Tower stent in 3) were implanted after recanalizing the occluded duct at 2 to 7 days of age. Heparin was given only during the procedure, but no further anticoagulants were used. Angiographic or postmortem evaluations were made at 1, 1.5, 2, 3, 4, 6, 12, 16, and 21 months in a subgroup of 9 lambs who did not undergo reinterventions. The duct was patent in all these except for one studied at 16 months. Neointima initially developed in the center of the stent before extending toward the orifices, eventually burying the metal strands in contact with the wall. From 4 to 6 months onward stenoses were present inside some of the stents. When the stent did not protrude into the aorta, neointima extended over the duct orifice. Metal strands that were not in contact with the duct wall were incompletely covered with endothelial cells, platelets, and fibrin strands, but no thrombi were noted. Late balloon dilation of the stented duct was performed in two lambs increasing the pulmonary artery blood flow. In one lamb the neointimal lining was successfully removed at 14 months with an atherectomy catheter. Stent implantation into the arterial duct can maintain patency up to 21 months and could be considered as an alternative to neonatal systemic to pulmonary artery shunt operations. Neointimal proliferation and stenosis formation, however, is a major limitation that may eventually lead to a reduction in the pulmonary artery blood flow.

Animals↗
PubMed1996

Translocation t(3;17)(q23;q21): a new translocation in acute lymphoblastic leukaemia.

We report on two adult patients with CD10+ positive acute lymphoblastic leukaemia (ALL) who presented with similar clinical and laboratory features and with a new chromosomal translocation: t(3;17)(q23;q21). This translocation may be involved in the formation of a new chimaeric transcription factor. Both patients shared several poor prognostic factors at presentation and an adverse clinical course. The t(3;17)(q23;q21) translocation may therefore predict a poor outcome in ALL.

Adult↗
PubMed1996

Outcome of isolated congenital complete heart block diagnosed in utero.

OBJECTIVE: To establish identifiable prenatal factors in fetal heart block which might predict death in utero, the need for intervention, or the probability of pacemaker requirement. SETTING: Tertiary referral unit for fetal echocardiography. SUBJECTS: 36 fetuses with congenital complete heart block and structurally normal hearts identified between 1980 and 1993. METHODS: Maternal anti-Ro antibody status was documented. Prenatal variables examined included absolute heart (ventricular) rate, change in rate, and development of hydrops fetalis. Postnatally, heart rate, need for pacing, and the indications for pacing were detailed. RESULTS: Of the total of 36 patients, there are 24 survivors; 11 are paced. Of those fetuses which died, two were electively aborted for severe hydrops, seven died in utero, two were immediate postnatal deaths, and one was an unrelated infant death. The trend was for the heart rate to decrease during fetal life and postnatally. Fetuses with deteriorating cardiac function did not always show the lowest heart rates. Bradycardia of less than 55 beats/min in early pregnancy or rapid decrease in heart rate prenatally were poor prognostic signs. Hydrops was also associated with bad outcome, 10 out of the 12 hydropic fetuses dying (83%). Of 10 fetuses presenting with a heart rate above 60/min, nine survived of whom three required pacing. Of seven presenting with heart rates of 50/min or less, only three survived and two of these required pacing. Of the two fetuses with negative maternal anti-Ro antibody status one died in utero and one required heart transplantation after pacemaker insertion. CONCLUSIONS: Isolated complete heart block identified in fetal life does not always have a good prognosis. An individual heart rate does not accurately predict the outcome in utero or the need for postnatal pacing. Regular, careful monitoring during pregnancy is required in order to optimise care and timing of any interventions.

Cardiac Pacing, Artificial↗
PubMed1996

Evolving use of embolisation coils for occlusion of the arterial duct.

OBJECTIVE: To assess the outcome of arterial duct occlusion with coils chosen according to the duct morphology. DESIGN: Retrospective study. SETTING: Paediatric cardiology centre. PATIENTS: Coil occlusion was attempted in 57 patients aged 0.5 to 15 (median 3.7) years and weighing 5-59 (median 14) kg between January 1991 and December 1995. A residual leak was present in 8 patients after umbrella closure and in 4 patients after duct ligation. METHODS: Coils of 4 different types were implanted through 4 or 5 F femoral artery catheters. Platinum or Interlocking Detachable 0.018 inch coils were deployed completely inside tubular ducts. Gianturco or PDA controlled release 0.038 inch coils were implanted to straddle short, post ligation and post umbrella ducts. RESULTS: Coil implantation was successful in 54/57 patients. At 1 year the cumulative occlusion rate was 53/57 ducts (93%) on an intention to treat analysis. A single coil was implanted in 37 (69%), 2 coils in 10 (19%), 3 coils in 3 (5%) and 4 coils in 4 (7%) of the 54 successful procedures. Duct occlusion was documented at the end of the procedure in 31%, by the following day in 83%, by 6 weeks in 87%, by 6 months in 96%, and by 1 year in 98%. Coil embolisation occurred in 6/58 procedures (10%), with a 50% rate in the first year of implantation (1/2 patients) falling to 7% in the last year (3/42 patients). All embolised coils were easily retrieved. CONCLUSIONS: Occlusion of small to moderate size arterial ducts, including residual post umbrella or post ligation ducts, was readily accomplished by coils selected according to the duct anatomy. This has both cost and practical benefits.

Adolescent↗
PubMed1995

Therapeutic trial of sympathomimetics in three cases of complete heart block in the fetus.

BACKGROUND: A total of 36 fetuses with isolated congenital complete heart block and structurally normal hearts have been seen in the department of fetal echocardiography since 1980. Although the prognosis is good in the majority of cases, those who develop intrauterine cardiac failure have a high mortality. The aim of this study was to investigate the contribution to management of sympathomimetic therapy by comparing two possible agents administered to the mothers. METHODS AND RESULTS: The effect of two sympathomimetic agents, isoprenaline and salbutamol, was compared in three patients with isolated complete heart block. Fetal heart rate and indexes of cardiac function were monitored during therapy. Maternal cardiovascular status was also regularly assessed. Dosage of isoprenaline increased from 1 to 12 micrograms/min, and salbutamol increased from 4 to 64 micrograms/min during the trial. No significant change was detected with isoprenaline therapy, but all fetuses showed an increase in heart rate and improvement in ventricular function with salbutamol. Salbutamol was maintained until delivery in one case with evidence of cardiac failure, with resolution of fetal hydrops. All three delivered in good condition close to term. Two of three required pacing in the neonatal period. CONCLUSIONS: We conclude that salbutamol can be effective in the treatment of fetal complete heart block and should be considered in patients with this condition where there is evidence of deteriorating cardiac function.

Albuterol↗
PubMed1995

A multifunctional Urechis caupo protein, PAPS synthetase, has both ATP sulfurylase and APS kinase activities.

The synthesis of 3'-phosphoadenosine-5'-phosphosulfate (PAPS) from inorganic sulfate and ATP requires two enzymes, ATP sulfurylase (SUL) and adenosine-5'-phosphosulfate kinase (KIN). In bacteria, fungi, yeast and plants, the two enzymes are present on separate polypeptide chains. We have identified the first animal gene coding for these enzymes. In the marine worm, Urechis caupo (Uc), both SUL and KIN are present on a single polypeptide chain. This protein, which we call PAPS synthetase (PAPSS), is able to complement yeast mutants lacking either enzyme. The Uc PAPSS mRNA is present in oocytes, but is not translated until after fertilization. At least three adult tissues, gut, ceolomocytes and body wall, also contain the mRNA, but at lower concentrations than are found in embryos. Partial sequences of a similar gene from Caenorhabditis elegans (Ce) were detected in a search of the GenBank expressed sequence tag database. Comparison of these Uc and Ce PAPSS sequences with the sequences of cloned genes from non-animal organisms strongly suggests that the animal genes evolved through the fusion of the SUL- and KIN-encoding genes from lower organisms.

Amino Acid Sequence↗