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Biomedical subjects

J Hess

Publications and source records attributed to J Hess.

At least 181 records · Page 10Linked to original sources
PubMed1993

Left and right ventricular diverticula, ventricular septal defect and ectopia cordis in a patient with Cantrell's syndrome.

A patient with Cantrell's syndrome is presented with ectopia cordis, ventricular septal defect and a left ventricular as well as a right ventricular diverticulum. One-stage correction was performed with resection of both diverticula. Closure of the ventricular septal defect was complicated due to exposure problems as a result of the malposition of the heart. Although the presentation of Cantrell's syndrome can be variable, one-stage repair is technically feasible and should be considered as the treatment of choice in this rare syndrome.

Diverticulum↗
PubMed1993

Behavioural and emotional problems in children and adolescents with congenital heart disease.

Behavioural/emotional problems were assessed at least nine years after surgical correction for congenital heart disease (ConHD) in childhood. Parents of 144 10-15-year-old ConHD-children completed the Child Behavior Checklist (CBCL) and 179, 11-17-year-old, ConHD-adolescents completed the Youth Self-Report (YSR). On the CBCL and YSR ConHD-children and adolescents obtained significantly higher problem scores than same-aged peers from normative reference groups. No significant differences were found between problem scores for different cardiac diagnostic groups. A negative correlation was found between CBCL total problem scores and IQ-scores of ConHD-children; for YSR total problem scores no such relationship was found.

Adaptation, Psychological↗
PubMed1993

Bovine filensin possesses primary and secondary structure similarity to intermediate filament proteins.

The cDNA coding for calf filensin, a membrane-associated protein of the lens fiber cells, has been cloned and sequenced. The predicted 755-amino acid-long open reading frame shows primary and secondary structure similarity to intermediate filament (IF) proteins. Filensin can be divided into an NH2-terminal domain (head) of 38 amino acids, a middle domain (rod) of 279 amino acids, and a COOH-terminal domain (tail) of 438 amino acids. The head domain contains a di-arginine/aromatic amino acid motif which is also found in the head domains of various intermediate filament proteins and includes a potential protein kinase A phosphorylation site. By multiple alignment to all known IF protein sequences, the filensin rod, which is the shortest among IF proteins, can be subdivided into three subdomains (coils 1a, 1b, and 2). A 29 amino acid truncation in the coil 2 region accounts for the smaller size of this domain. The filensin tail contains 6 1/2 tandem repeats which match analogous motifs of mammalian neurofilament M and H proteins. We suggest that filensin is a novel IF protein which does not conform to any of the previously described classes. Purified filensin fails to form regular filaments in vitro (Merdes, A., M. Brunkener, H. Horstmann, and S. D. Georgatos. 1991. J. Cell Biol. 115:397-410), probably due to the missing segment in the coil 2 region. Participation of filensin in a filamentous network in vivo may be facilitated by an assembly partner.

Amino Acid Sequence↗
PubMed1993

Balloon valvuloplasty for valvular pulmonary stenosis in children over 6 months of age: initial results and long-term follow-up.

Between 1984 and December 1990 balloon valvuloplasty for valvular pulmonary stenosis was planned and performed in 92 children over 6 months of age (range 0.8-17.9 years). Valvuloplasty decreased the invasively determined peak systolic gradient from 61 +/- 34 (mean +/- SD) to 27 +/- 20 mmHg (P < 0.0001). At follow-up cardiac catheterization, performed in 22 patients after 13 +/- 5 months, the gradient was 22 +/- 23 mmHg (ns). Initially the continuous wave Doppler gradient decreased from 61 +/- 23 to 26 +/- 12 mmHg (P < 0.0001). It then remained unchanged both at early follow-up after 0.6 +/- 0.3 years, being 23 +/- 12 mmHg, and at long-term follow-up after 3 +/- 1.7 years (21 +/- 10 mmHg, ns). In the 41 patients treated before the end of 1986 the residual continuous wave Doppler gradient at long-term follow-up after 5.1 +/- 0.8 years was 21 +/- 10 mmHg. Mild pulmonary regurgitation was present in 77% of the patients at early follow-up and in 83% at the last follow-up. Right ventricular hypertrophy on the electrocardiogram was present in 83% of the children before valvuloplasty. At early follow-up it had decreased to 44% with a further decrease to 24% at the last follow-up. Comparison of patients with initial invasive systolic gradients greater and smaller than 50 mmHg revealed no differences between either group with regard to the continuous wave Doppler gradient at last follow-up, the incidence of pulmonary regurgitation and the occurrence of significant complications.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1993

Balloon angioplasty for aortic recoarctation in children: initial and follow up results and midterm effect on blood pressure.

OBJECTIVE: To assess the direct and follow up results of balloon angioplasty for aortic recoarctation with respect to the type of initial operation and to determine the midterm effect on systolic blood pressure. DESIGN: Prospective study of invasive haemodynamic and angiographic data and non-invasive data on upper body blood pressure. SETTING: Tertiary referral centre for paediatric cardiology. SUBJECTS: 24 infants and children (age 0.3-16.2 years, mean 5.9 years) who had had surgical correction for coarctation (end to end anastomosis (14 patients) subclavian flap angioplasty (nine), patch angioplasty (one)). MAIN OUTCOME MEASURES: Peak systolic gradient over the recoarctation and aortic diameters before and directly after angioplasty and at follow up. Upper body blood pressure before and after angioplasty and at latest follow up. RESULTS: Mean peak systolic gradient initially decreased from 35 (15) to 12 (9) mm Hg (p < 0.001) and was 9 (10) mm Hg at follow up after 1.4 (0.5) years. Patients with a subclavian flap repair showed a slight further decrease in the residual gradient at follow up (p < 0.05). The coarctation diameter increased from 5.3 (2.6) to 7.7 (2.5) mm (p < 0.001), and a further increase to 9.3 (2.9) mm (p < 0.01) was present at follow up after 1.4 (0.5) years without significant changes in other aortic diameters. Upper body systolic blood pressure decreased from 138 (24) to 115 (17) mm Hg after balloon angioplasty, and the effect on blood pressure persisted at a mean follow up of 3.7 years. One patient died of ventricular failure. Femoral artery thrombosis occurred in three patients. In one patient a small aneurysm occurred that had not increased at follow up. In one patient restenosis after angioplasty was redilated successfully. In one patient dilatation of a residual stenosis after angioplasty failed. CONCLUSION: Balloon angioplasty for recoarctation is effective and is associated with accelerated growth of the dilated segment at follow up in many patients. The complication rate is acceptable. Midterm follow up shows persistent relief of upper body hypertension in most patients.

Adolescent↗
PubMed1993

Maternal autoantibodies and congenital heart block: no evidence for the existence of a unique heart block-associated anti-Ro/SS-A autoantibody profile.

One of the rare examples of the transfer of autoimmune disease from mother to (unborn) child is the neonatal lupus syndrome. This syndrome comprises the development of fetal heart disease (congenital heart block) or neonatal skin rash and is specifically associated with maternal anti-Ro/SS-A autoantibodies. Previous studies have suggested that especially maternal autoantibody reactivity against the 52 kDa protein of the Ro/SS-A antigen and/or against the La/SS-B antigen is responsible for the development of congenital heart block (CHB). To determine the CHB-associated antibody response in more detail, we analysed the presence of autoantibodies in sera from mothers of children with isolated heart block. All 14 mothers of children with congenital heart block were positive for anti-Ro/SS-A antibodies. Remarkably, their antibody profile, including recognition of different Ro/SS-A proteins and autoantibody levels against these proteins, did not differ from anti-Ro/SS-A positive mothers of healthy children. In contrast, all 8 anti-Ro/SS-A negative mothers had children with acquired heart block. We conclude from our data that maternal anti-Ro/SS-A antibodies are essential for CHB but that fine analysis of this autoantibody response does not predict the occurrence of CHB.

Adult↗
PubMed1993

Dental findings in a patient with biliary atresia.

The dental treatment of a 3-year-old male with biliary atresia is discussed. The patient was scheduled for a liver transplant and was receiving Cyclosporine A. Due to the severity of the dental condition and to reduce the possibility of infection, all twenty primary teeth were extracted. The extracted teeth displayed a marked greenish discoloration of both the crowns and the roots. Histological examination revealed extensive interglobular dentin, a number of faint yellow-brown lines contouring the developmental pattern and a distinctive fluorescent line. The dental and pharmacological considerations relating to the treatment plan for this case are reviewed.

Biliary Atresia↗
PubMed1993

Specific sequelae after Fontan operation at mid- and long-term follow-up. Arrhythmia, liver dysfunction, and coagulation disorders.

Patients who have undergone a Fontan-type operation usually have an elevated systemic venous pressure. To determine the sequelae of this nonphysiologic condition, we evaluated 66 patients 1 to 14 years after a Fontan-type operation. Fifty-one patients were apparently in good clinical condition, and 15 patients had symptoms and were restricted in their daily life. Bicycle exercise capacity, tested in 41 patients, ranged from 50% to 110% (mean 85%) of the predicted value for length. In 16 patients, a decreased capacity (< 85%) was, among others, related to arrhythmias and the presence of protein-losing enteropathy. A 24-hour ambulatory electrocardiogram was available in 56 patients and found to be normal in 32 (57%) patients. Arrhythmias were present in 21 patients, six of whom had symptoms. Three patients had previous pacemaker implantation. One or more abnormalities in liver enzyme and function tests were present in 40 patients (61%) and in coagulation factors in 46 patients (69%). The most pronounced was a protein C deficiency, a known thrombotic risk factor, present in 41 patients. The occurrence of arrhythmias increased with time of follow-up (p < 0.004), the occurrence of protein C deficiency decreased with time (p < 0.0001), and the occurrence of abnormal liver enzyme and function tests was not related to time of follow-up. With regard to age at operation, arrhythmias did not occur in patients who underwent operation at a mean age of 4 +/- 1.9 years (standard deviation), in contrast to patients who underwent operation at a mean age of 7.6 +/- 4 years (standard deviation) (p < 0.001). The occurrence of the two other types of sequelae was not related to the age at operation. With regard to the type of operation, only patients with a valved right atrium-to-pulmonary artery connection had a higher prevalence of arrhythmias than patients with a nonvalved or direct right atrium-to-pulmonary artery connection (p < or = 0.001). The latter patients also had a higher prevalence of protein C deficiency (p < or = 0.001). No relationship was found among the other types of operation, the underlying structure, or the hemodynamic condition measured at rest and the presence of arrhythmias, abnormal liver enzyme and function tests, or protein C deficiency. This point survey shows that even patients with an apparently good clinical condition are at risk for arrhythmias, abnormal liver enzyme and function tests, and coagulation factor abnormalities. Serial statement of affairs is recommended to ensure that adequate preventive measures can be taken.

Adolescent↗
PubMed1993

[Herpes paronychia].

Herpetic whitlow is a rare viral infection, that should be treated nonoperatively and managed by a hand surgeon. There is a high risk of infection for dentists and other medical personal. The authors present a case, describe findings, possible complications, and treatment.

Animals↗
PubMed1992

Short- and midterm results of balloon valvuloplasty for valvular aortic stenosis in children.

During a 27-month period, 21 consecutive children (aged 0.1 to 15.7 years) with isolated valvular aortic stenosis underwent percutaneous transfemoral balloon valvuloplasty. Ten children had undergone earlier surgical valvulotomy. The indication for treatment was ST-T-segment changes at rest or during bicycle-ergometry, a continuous-wave Doppler-derived transvalvular gradient greater than 60 mm Hg or syncope, or a combination. Mean peak systolic left ventricular pressure decreased from 165 +/- 19 to 131 +/- 19 mm Hg (p less than 0.001). Mean end-diastolic left ventricular pressure did not change significantly (12 +/- 3 vs 11 +/- 5 mm Hg). Mean peak systolic valve gradient decreased from 71 +/- 23 to 22 +/- 11 mm Hg (p less than 0.001). Mean cardiac index remained unchanged (2.9 +/- 0.8 vs. 3.0 +/- 0.7 liters.min-1.m-2). Aortic valve regurgitation on angiography appeared or increased in 9 patients (up to grade 3 in 3 children). Noninvasive follow-up studies were performed for 2 to 4.2 years (mean 2.8). ST-T changes on the electrocardiogram at rest or during exercise were present in 6 patients before balloon valvuloplasty and had disappeared in all at 6-month follow-up. Reoccurrence of ST-T changes after a longer follow-up was associated with severe valve regurgitation. Syncope was not observed after balloon valvuloplasty. The continuous-wave Doppler gradient decreased from 94 +/- 36 to 49 +/- 15 mm Hg (p less than 0.001). After a follow-up of 2 to 4.2 years (mean 2.8) it remained unchanged (43 +/- 13 mm Hg; p = not significant).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1992

Vasopressin and oxytocin in brain areas of rats selectively bred for differences in behavioral performance.

Wistar rats were selectively bred over 10 generations for differences in performance in a footshock-motivated brightness discrimination (BD) test in a Y-maze. High behavioral performance (Wis/HBP) and low behavioral performance (Wis/LBP) rat lines were obtained which differ significantly in all behavioral components tested: frequency of correct responses, number of trials to criterion, response latency (HBP less than LBP), and frequency of freezing behavior (HBP less than LBP), the latter suggesting differences in emotionality. In Wis/LBP rats, furthermore, the normal increase in behavioral performance between the training and the relearning session, which indicates the formation of a memory trace, disappeared during selection. In male breeders sampled during selection of the two lines (Wis/HBP: n = 17; Wis/LBP: n = 21), both arginine vasopressin (AVP) and oxytocin (OXT) contents were measured by radioimmunoassay in the motor cortex, septum/striatum, hippocampus, hypothalamus, medulla oblongata and posterior pituitary. Compared with the Wis/HBP rats, the Wis/LBP rats contained less AVP in the hippocampus (3.1 +/- 0.58 vs 8.3 +/- 1.4 pg/mg wet wt., mean +/- S.E.M., P less than 0.001), but more AVP in the medulla (1.7 +/- 0.20 vs 1.1 +/- 0.18 pg/mg, P less than 0.05). In contrast, no significant differences between the lines were detected with respect to OXT concentrations. In the Wis/LBP rats, moreover, the hippocampal AVP content decreased during selection (r = -0.645, P less than 0.01), while the acquisition response latency increased (r = 0.549, P less than 0.01). As a consequence, a significant, albeit weak, negative correlation (r = -0.483, P less than 0.05) was observed between the individual hippocampal AVP content and the response latency during acquisition. Thus, the results confirm the view that genetically determined differences in the hippocampal content of endogenous AVP may contribute to an individual's level of emotionality and behavioral performance.

Animals↗
PubMed1992

Processing by OmpT of fusion proteins carrying the HlyA transport signal during secretion by the Escherichia coli hemolysin transport system.

A fusion gene (ces-hlyAs) was constructed by ligating the genetic information for the C-terminal 60 amino acids (hlyAs) of Escherichia coli hemolysin (HlyA) to the ces gene for a cholesterol esterase/lipase (CE) from a Pseudomonas species. Part (about 30%) of the expressed fusion protein CE-HlyAs was secreted in E. coli carrying hlyB and hlyD genes. Following the insertion between the reporter gene and hlyAs of a linker sequence that contains the information for potential cleavage sites for the outer membrane protease OmpT, two different fusion proteins (PhoA-HlyAs and CE-HlyAs) were shown to be cleaved by OmpT between the two parts during HlyB/HlyD-mediated secretion. Processed PhoA and CE accumulated in the supernatant. The efficiency of cleavage by OmpT was considerably improved by increased ompT gene dose. It was further shown that OmpT preferentially recognizes potential cleavage sites within the linker sequence.

Amino Acid Sequence↗
PubMed1992

Biocompatibility of retrograde filling materials in the ferret canine. Amalgam and IRM.

Periapical tissue response to retrograde fillings of amalgam and IRM were compared in the mandibular canine of the adult male ferret. Teeth were cleaned and shaped with a standard technique and obturated with gutta-percha. The root apex was then exposed and retrofillings were placed. The animals were grouped according to observation periods of 5, 10, and 15 weeks. At the proper time the animals were killed and the lower canine tooth along with the surrounding bone was removed. The tissue blocks were examined clinically, radiographed, and prepared for histologic examination. The clinical and radiographic examination indicated both materials to be well tolerated by periapical tissues. Microscopic examination of amalgam specimens showed a decrease in inflammation and the formation of a fibrous capsule over the 15-week period. IRM specimens showed persistent inflammation and slower healing potential.

Alveolar Process↗
PubMed1992

Management of fixed subaortic stenosis: a retrospective study of 57 cases.

Although recommended by several investigators, the benefit of early surgery in patients with fixed subaortic stenosis has not been proved. Findings were reviewed of 57 patients with isolated fixed subaortic stenosis, including 27 surgically treated patients, with special emphasis on the occurrence of aortic regurgitation during a mean follow-up period of 6.7 years. The number of patients with aortic regurgitation increased preoperatively in the total group (23% at diagnosis to 54% after 3.7 years of follow-up). The prevalence of aortic regurgitation in the 27 surgically treated patients was higher (81%) than that in the nonsurgically treated group but remained unchanged after a mean postoperative period of 4.7 years. In all patients but one, aortic regurgitation remained of minor hemodynamic significance. One patient died during follow-up. After surgery, 15 patients (55%) showed a relapse; 11 redeveloped a subvalvular pressure gradient greater than 30 mm Hg and discrete subvalvular ridges (range 6 months to 24 years after surgery, mean 7 years). In those patients with fixed subaortic stenosis, follow-up did not reveal any benefit from early surgery. The unpredictable course and sometimes very severe progression of this disease make frequent and careful follow-up necessary.

Adolescent↗
PubMed1992

Transoesophageal echocardiographic evaluation of atrial morphology in children with congenital heart disease.

In a prospective study, atrial morphology was evaluated by both transoesophageal and precordial echocardiography in 86 unoperated children with congenital heart disease (age range = 0.2 to 14.8 years, mean = 3.8 years) to determine what advantages, if any, might be inherent in the transoesophageal approach. The information derived from both ultrasound approaches was correlated and compared to information obtained during subsequent cardiac catheterization (78 patients) and, or, surgical inspection (53 patients). Atrial appendage morphology and hence atrial situs was determined by transoesophageal echocardiography in every case (82 solitus, two right atrial isomerism, two left atrial isomerism). In addition, the transoesophageal approach indicated left juxtaposition in four patients, compared to only one by precordial examination. Probe patency of the foramen ovale was correctly predicted in 21 patients by transoesophageal imaging, but in only 10 by precordial imaging. In two children significant secundum defects, undetected by the precordial route, were identified. Multiple atrial septal defects were correctly defined in four patients by transoesophageal study but in only one by precordial study. Sinus venosus defects were documented in four by the transoesophageal approach, but in only one by the precordial. Primum defects were equally well documented (nine patients) by either technique, but the associated valve leaflet morphology was better documented by transoesophageal study in 5/9. A subtotal cor triatriatum was diagnosed in one child only by transoesophageal investigation. Transoesophageal echocardiography allows a much more detailed evaluation of atrial morphology than precordial imaging even in infants. It provides direct diagnosis of atrial situs, detection of juxtaposed atrial appendages and improved demonstration or definitive exclusion of atrial septal defects.

Adolescent↗
PubMed1992

Protrusion of the device: a complication of catheter closure of patent ductus arteriosus.

OBJECTIVE: To assess the medium term results of percutaneous transvenous closure of patent ductus arteriosus, in particular with regard to protrusion of the device with or without turbulence of the bloodflow. DESIGN: Clinical examination and echocardiographic study (cross sectional Doppler, and colour Doppler examination) within 24 hours of and at least 6 months after implantation (range 6-26 (mean 15) months). SETTING: Multicentre study at the departments of paediatric cardiology of three academic hospitals. Tertiary clinical care of the first group of patients in the Netherlands treated by the percutaneous transvenous method. PATIENTS: 36 patients (12 male, 24 female) mean age 8.2 years, (range 1.7-58.3), mean weight 25.5 kg (range 11-67.8 kg). The total group consisted of 46 patients. In one the implantation had failed and nine others were not available for regular follow up. All 36 patients underwent non-surgical closure of the patent ductus arteriosus with a Rashkind double umbrella prosthesis. MAIN OUTCOME MEASURES: Diagnosis or exclusion of protrusion of the Rashkind device with or without turbulence of the blood flow with follow up of changes in protrusion and turbulence. RESULTS: In 17 patients the prosthesis protruded into an arterial lumen: the aorta in 13 and the (left) pulmonary artery in four, with turbulence in seven and two cases respectively. After six months the aortic protrusion disappeared in three, including one who had had turbulent blood flow. At the end of follow up the prosthesis still protruded into the aorta in 10 but in three the turbulence had vanished. In two of the three remaining patients with turbulence in the descending aorta the degree of turbulence had decreased. There was no lessening of turbulence in the four patients in whom the device protruded into the pulmonary artery. CONCLUSIONS: The Rashkind double umbrella can protrude into the descending aorta and the left pulmonary artery without causing turbulent blood flow. Turbulence and the protrusion itself can disappear. Endocarditis prophylaxis may be required for as long as the device causes turbulence.

Adolescent↗