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Biomedical subjects

D Schranz

Publications and source records attributed to D Schranz.

At least 19 recordsLinked to original sources
PubMed2006

Systemic sarcoidosis after cardiac transplantation in a 9-year-old child.

Sarcoidosis is a granulomatous disease of unknown etiology and is only rarely seen in infants and children. We present the case of a 9-year-old boy who developed sarcoidosis with multi-organ involvement 9 years after cardiac transplantation for Shone complex. The patient was on immunosuppressive therapy with tacrolimus and mycophenolate mofetil. He presented with severe respiratory distress due to marked mediastinal lymphadenopathy and bilateral pulmonary infiltrates in association with fatigue, low-grade fever, hepatosplenomegaly and generalized lymphadenopathy. Lymph node histology showed non-caseating epitheloid cell granulomas and giant cells. Initialization of therapy with prednisolone resulted in prompt clinical recovery and resolution of all symptoms except for the development of mild pulmonary fibrosis. Tapering of the steroids led to recurrence of mediastinal lymphadenopathy 5 months after the initial disease, which responded to an increase in steroid dose. The clinical course, the medical management, and the possible role of immunosuppression in the etiology of the disease are discussed.

Child↗
PubMed2005

PTPN11 mutations play a minor role in isolated congenital heart disease.

PTPN11 missense mutations cause approximately 50% of Noonan syndrome, an autosomal dominant disorder presenting with various congenital heart defects, most commonly valvar pulmonary stenosis, and hypertrophic cardiomyopathy. Atrioventricular septal defects and coarctation of the aorta occur in 15% and 9%, respectively. The aim of this study was to determine if PTPN11 mutations exist in non-syndromic patients with these two relevant forms of congenital heart disease. The 15 coding PTPN11 exons and their intron boundaries from subjects with atrioventricular septal defects (n = 24) and coarctation of the aorta (n = 157) were analyzed using denaturing high performance liquid chromatography and sequenced if abnormal. One subject with an atrioventricular septal defect but no other known medical problems had a c.127C > T transition in exon 2, predicting a p.L43F substitution. This mutation affected the phosphotyrosine-binding region in the N-terminal src homology 2 domain and was close to a Noonan syndrome mutation (p.T42A). An otherwise healthy patient with aortic coarctation had a silent c.540C > T change in exon 5 corresponding to p.D180D. Our study showed that PTPN11 mutations are rarely found in two isolated forms of congenital heart disease that commonly occur in Noonan syndrome. The p.L43F mutation belongs to a rare class of PTPN11 mutations altering the phosphotyrosine-binding region. These mutations are not predicted to alter the autoinhibition of the PTPN11 protein product, SHP-2, which is the mechanism for the vast majority of mutations causing Noonan syndrome. Future studies will be directed towards understanding these rare phosphotyrosine binding region mutants.

Adolescent↗
PubMed2005

Perioperative management in pediatric heart transplantation.

Peri- and early postoperative mortality significantly influences the probability of survival following heart transplantation in children. Main causes of death early after transplantation are rejection, non specific graft failure and RV failure due to pulmonary hypertension. Optimal therapy of pulmonary hypertension and aggressive use of assist devices as a bridge to recovery will substantially improve survival in the early period after transplantation. Furthermore, the use of marginal donor organs will be more acceptable because transient myocardial insufficiency may recover during extracorporeal life support.

Child↗
PubMed2005

The first vector-graphics-aided computer tooth model and its animation.

Computer graphics is here to stay and spreading. This article intends to be nothing more than a brief introduction of this new tool in the field of dentistry. We hope that our colleagues will welcome this new development and recognize it as an opportunity to make contact with the basic sciences.

Algorithms↗
PubMed2003

Idiopathic monomorphic ventricular tachycardia originating from the left aortic sinus cusp in children: endocardial mapping and radiofrequency catheter ablation.

BACKGROUND: Idiopathic repetitive monomorphic ventricular tachycardia with an inferior axis and left bundle branch block pattern typically originates from the superior right ventricular outflow tract. When indicated, radiofrequency catheter ablation is usually safe and effective. However, a left ventricular origin has been described recently in adult patients in whom ablation attempts in the right ventricular outflow tract were unsuccessful. Experience in pediatric patients is limited. PATIENTS AND METHODS: Since 1998, 13 young patients suffering from symptomatic ventricular tachycardia episodes with an inferior axis and left bundle branch block pattern underwent an electrophysiological study and radiofrequency catheter ablation. In 2 patients, age 13 and 15 years, no endocardial local electrograms preceding the surface ECG QRS complex could be recorded within the right ventricular outflow tract during ventricular ectopy. Detailed mapping within the left ventricular outflow tract and in the aortic root revealed local electrograms 25 and 53 ms earlier than the QRS complex and a 11/12 and 12/12 lead match during pacing inferior and anterior to the ostium of the left main coronary artery in the left aortic sinus cusp. Earliest activation was recorded 10 and 12 mm away from the coronary artery ostium identified angiographically. In each of the patients, one single radiofrequency current application (60 degrees C, 30 W, duration 30 and 60 s, respectively) resulted in complete cessation of ventricular ectopy. Subsequent selective injection into the left coronary artery did not reveal any abnormalities. During follow-up (2 and 34 months) off any antiarrhythmic drugs, both of the patients are in continuous normal sinus rhythm. CONCLUSION: In young patients with symptomatic idiopathic ventricular tachycardia originating from the left aortic sinus cusp, radiofrequency catheter ablation was safe and effective.

Adolescent↗
PubMed2003

Cardiac troponin I: a marker of acute heart rejection in infant and child heart recipients?

Acute rejection of the donor heart is a major cause of mortality in infant heart transplant recipients. The early diagnosis of acute cardiac rejection (ACR) is crucial. Non-invasive methods have shown poor sensitivity in detecting rejection when compared to endomyocardial biopsies (EMB). We assessed troponin I as a new marker to diagnose cardiac rejection. Serum cardiac troponin I (cTNI) levels were retrospectively analysed in 25 heart transplant patients (ages, 2 wk to 13 yr; mean age, 3 months) presenting 36 acute rejections. In early post-operative rejection and initially elevated cTNI levels, rejection was associated with a second increase of serum cTNI concentrations in 21% of the patients (p = 0.15). If cTNI levels were in normal range before ACR an elevation was monitored in 59% of the rejection periods (p < 0.05). In 25% of the cases (n = 9) cTNI levels remained in normal range during the rejection episode (<0.6 ng/mL), in 22% (n = 8) cTNI levels did not exceed pathological values from 0.6 to 1.5 ng/mL and in 53% (n = 19) the measured levels went beyond 1.5 ng/mL. Maximum concentrations of cTNI were measured mostly 12 d from the moment rejection was suspected (day 1) in patients (median day 3). However, cTNI levels were elevated for 2-43 d after ACR was diagnosed (median 10 d). Twenty per cent of the patients with grade 3 rejection (ISHLT) and 75% of the patients with grade 4 rejection had a corresponding elevated cTNI level (p = 0.013). No false-positive elevations of cTNI were documented. The present data demonstrate that cTNI is a not a sensitive but a specific marker of ACR in children.

Acute Disease↗
PubMed2003

Stenting of the ductus arteriosus and banding of the pulmonary arteries: basis for various surgical strategies in newborns with multiple left heart obstructive lesions.

OBJECTIVE: To present an institutional experience with stent placement in the arterial duct combined with bilateral banding of the pulmonary artery branches as a basis for various surgical strategies in newborns with hypoplastic left heart obstructive lesions. DESIGN: Observational study. SETTING: Paediatric heart centre in a university hospital. PATIENTS: 20 newborns with various forms of left heart obstructive lesions and duct dependent systemic blood flow. INTERVENTIONS: Patients underwent percutaneous ductal stenting and surgical bilateral pulmonary artery banding. Atrial septotomy by balloon dilatation was performed as required, in one premature baby by the transhepatic approach. MAIN OUTCOME MEASURES: Survival; numbers of and reasons for palliative and corrective cardiac surgery. RESULTS: One patient died immediately after percutaneous ductal stenting. One patient died in connection with the surgical approach of bilateral pulmonary banding. Stent and ductal patency were achieved for up to 331 days. Two patients underwent heart transplantation and two patients died on the waiting list. Ten patients had a palliative one stage procedure with reconstruction of the aortic arch and bidirectional cavopulmonary connection at the age of 3.5-6 months. There was one death. One patient is still awaiting this approach. Two patients received biventricular repair. In one, biventricular repair will soon be provided. CONCLUSIONS: Stenting the arterial duct combined with bilateral pulmonary artery banding in newborns with hypoplastic left heart or multiple left heart obstructive lesions allows a broad variation of surgical strategies depending on morphological findings, postnatal clinical conditions, and potential ventricular growth.

Cardiac Catheterization↗
PubMed2002

Predictors of treatment failure during highly active antiretroviral therapy (racing trial).

PURPOSE: To determine factors associated with virological failure during long-term treatment with the triple combination of saquinavir soft gel capsule, zalcitabine and zidovudine. METHOD: Open-label, prospective, multicentre study undertaken in private practices and the outpatient department of the Auguste-Viktoria-Hospital. A total of 95 patients with plasma HIV RNA > 5000 copies/ml who had received no more than 6 months pre-treatment with NRTIs and no prior PI therapy received saquinavir soft gel, zalcitabine and zidovudine for 52 weeks, before being randomly assigned to either remain on therapy or switch to nelfinavir, lamivudine and zidovudine for further 52 weeks. RESULTS: Combination therapy with saquinavir, zalcitabine and zidovudine was found to be effective and well tolerated, with virological response to therapy maintained for up to 2 years. In patients responding to therapy, switching to a novel triple regimen did not result in a virological or immunological worsening, but it did not confer an additional clinical benefit. Factors predictive of early treatment failure (virological failure within 16 weeks of treatment initiation) included high viral load and presence of RT mutations at baseline (OR: 0.30, 95% CI 0.11 0.83 and OR 0.13, 95% CI 0.03 0.52, respectively), with baseline viral load and the development of genotypic mutations on therapy being predictive of late treatment failure (16 52 weeks; OR: 0.15, 95% 0.05 0.46 and OR: 0.26, 95% CI < 0.001 1.16, respectively). Plasma saquinavir concentration < 50 mg/ml at 4 weeks was also found to be an independent risk factor for both early and late treatment failure (OR: 1.80, 95% CI 1.23 2.64 and OR: 1.16, 95% CI 0.84 1.60, respectively). CONCLUSIONS: While antiretroviral drug resistance appears to be a principal cause of treatment failure, other factors such as inadequate drug plasma concentrations also play a role.

Adult↗
PubMed2002

GAD65 and IA-2 autoantibodies are common in a subset of siblings of Sardinian Type 2 diabetes families.

UNLABELLED: Type 1 diabetes in Sardinia is very common in children, and we hypothesized that Latent Autoimmune Diabetes of Adult (LADA) might constitute a significant proportion of diabetes in adult Sardinian subjects. Since Type 2 diabetes is a familial disorder, we tested this hypothesis by investigating the prevalence of GAD65 and IA-2 autoantibodies (Ab) in Type 2 diabetes multiplex families of Sardinian ancestry enrolled in the Study Group for the Genetics of Diabetes in Sardinia. METHODS: A total of 684 individuals were ascertained from 252 Sardinian Type 2 diabetes multiplex families with 2.4 affected siblings per family comprising 190 families with two affected, 37 with three, 15 with four, 7 with five, and 3 with six, in addition to 80 unaffected siblings. Controls were household contacts representing 204 healthy spouses of affected siblings. Diagnosis was at 35-69 years of age and insulin was not given in the first 4 years after diagnosis. GAD65Ab and IA-2Ab were determined in standard radioligand binding assays. RESULTS: Among affected siblings GAD65Ab were positive in 8.8% of insulin-treated (n = 137; P = 0.0006), in 2.5% of non-insulin-treated (n = 467), and in 1.2% of non-diabetic siblings (n = 80) compared with 0.5% of controls (n = 204). IA-2Ab was positive in 6.6% insulin-treated (P = 0.04), 2.1% non-insulin-treated, and 2.5% non-diabetic siblings compared with 1.5% of controls. CONCLUSION: A high frequency of GAD65Ab and IA-2Ab as markers of Type 1 diabetes was found among Type 2 diabetes siblings from Sardinian multiplex families despite excluding those who had been treated with insulin during the first 4 years of disease. Our data support the hypothesis that LADA may be common in Sardinian Type 2 diabetes and stress the importance of investigating markers of Type 1 diabetes in studies of Type 2 diabetes.

Adult↗
PubMed2001

[Cardiac troponin I after heart surgery corrective operation in infancy and childhood].

BACKGROUND: Perioperative myocardial damage is an important determinant for postoperative cardiac function and recovery. Cardiac troponin I (cTNI) is a specific marker for myocardial damage. The aim of our study was to evaluate pre- and postoperative cTNI levels, the pattern of elevation in the first four postoperative days and the prognostic value after pediatric cardiac operation. METHODS: Cardiac troponin I levels were measured in 115 children mean age 36 +/- 45 months (range 4 days to 189 months) undergoing elective operation of a congenital heart defect. Routine measurements were made preoperatively, immediately after cardiopulmonary bypass and serially 8, 18, 42, 90, 138 hours thereafter. Data from 13 patients undergoing surgery without cardiopulmonary bypass served as controls. Postoperative cTNI levels were correlated with intra- and postoperative parameters (such as duration of aortic crossclamping, cardiopulmonary bypass time and need for postoperative inotropic support). RESULTS: All preoperative cTNI levels were in the normal range. Postoperatively, the highest median cTNI levels were found in patients after repair of tetralogy of Fallot (TOF), atrioventricular septal defect (AVSD) and implantation of a homo- or xenograft. Postoperative cTNI levels correlated significantly with duration of cardiopulmonary bypass and aortic crossclamping, operative approach (ventriculotomy versus atriotomy) and inotropic support (p < 0.0001). Peak cTNI levels were found immediately after surgery in 77.4% of our patients, 8 hours postoperative in 13.9% and at 18 hours after the surgery in 5.2% of the patients. In three children cTNI continued to increase; a secondary increase was found in one patient. Two of these children died, two had a prolonged postoperative recovery. CONCLUSION: The postoperative level of cardiac troponin I could be used as a marker of perioperative myocardial injury caused by ischemia and operative trauma. Peak levels usually could be obtained immediately after surgery, but a further increase of cTNI during the following 18 hours may occur and is not necessarily related to impaired recovery. However still increasing cTNI levels after 18 hours postoperatively and a secondary increase as well may be used as indicators of poor outcome.

Adolescent↗
PubMed2001

Heart transplantation in children and infants: short-term outcome and long-term follow-up.

Since 1988, 82 heart transplants have been performed in 80 infants and children. Diagnoses pretransplant were: hypoplastic left heart syndrome (HLHS) (n = 43); cardiomyopathy (n = 19); endocardial fibroelastosis (n = 6); and other complex congenital heart diseases (n = 12). Age at transplantation was < 1 yr in 61 patients. Overall survival rate was 79% at 1 yr and 73% at 5 and 10 yr. To date, 20 patients have died after transplantation. Causes of death were: rejection (eight patients); right ventricular failure (four patients); transplant coronary artery disease (TCAD) (two patients); and other causes (six patients). In the majority of patients somatic growth is not impaired, and renal function is reduced (but stable) in all patients. Two patients developed post-transplant lymphoproliferative disease, which was treated successfully. Major long-term morbidity is neurologic deficit - severe in three patients and minor in six. TCAD was present or suspected in six surviving patients. We conclude that heart transplantation in infants and children can be performed with good early and late results. Quality of life is excellent in most patients. TCAD, however, will become an increasing problem in the long term.

Child↗
PubMed2000

Safety and efficacy of interventional occlusion of patent ductus arteriosus with detachable coils: a multicentre experience.

UNLABELLED: In order to define the safety and efficacy of Cook detachable coils for interventional closure of patent ductus arteriosus, we performed a retrospective analysis of all patients who underwent cardiac catheterization in seven centres for intended interventional occlusion of patent arterial duct. From January 1995 until March 1998, cardiac catheterization for intended interventional occlusion of patent arterial duct was performed in 317 consecutive children. Successful placement of at least one coil was achieved in 282 children (89%). The mean diameter of the ductus in children treated with Cook detachable coils was 1.65 mm, mean fluoroscopy time was 10.7 min. Occlusion rates were 62% 10 min after the procedure, 82% at the time of discharge, 91% at 4 months and 95% at late follow-up (2 years). In children with a ductus diameter of < or = 2.5 mm the rate of successful coil deployment was 94% with a 98% occlusion rate at late follow-up. Complications occurred in 11 procedures (3.5%) including haemolysis (3 patients), embolization of a coil to the pulmonary artery (7 patients, 2.3%) and inability to release a coil (1 patient). CONCLUSION: In our opinion, Cook detachable coils are safe and effective especially in the treatment of persistent ductus arteriosus with a diameter < or = 2.5 mm. Due to the low costs these coils appear to be superior to other devices in this subgroup of patients.

Adolescent↗
PubMed1999

[Reopening of a persistent left superior vena cava in the early postoperative period following bidirectional cavopulmonary anastomosis--treatment by coil embolization].

Diagnosis of systemic venous drainage is mandatory for patients with congenital heart disease planned for cavopulmonary anastomosis or any Fontan-type palliation. Preexisting venous channels as the persistent left superior vena cava are common in cardiac anomalies and may lead to postoperative deterioration and cyanosis. We describe a 6 month old boy with a complex imbalanced atrioventricular septal defect who developed cyanosis in the very early postoperative period, following bidirectional cavopulmonary anastomosis. It was caused by ineffective lung perfusion due to a reopened persistent left superior vena cava with drainage to the coronary sinus.He underwent coil embolization of the persistent left superior vena cava with retrievable coils and cyanosis improved. Coil embolization is an effective alternative to secondary surgery, especially for hemodynamically compromised patients in the postoperative period.

Angiography↗
PubMed1999

Reduced inotropic support after aprotinin therapy during pediatric cardiac operations.

BACKGROUND: Several reports indicate that aprotinin treatment before and during cardiopulmonary bypass (CPB) might have a protective effect on the myocardium. We evaluated the hemodynamic effects of perioperative aprotinin treatment. METHODS: We conducted a randomized, double-blind, placebo-controlled trial in 34 infants (mean age, 2.5 years) who had cardiac operations. Half of the patients received high-dose aprotinin therapy. There were no significant differences between the aprotinin and placebo groups with respect to age, weight, sex, aortic cross-clamp time, and CPB time. The following data were recorded at arrival in the intensive care unit 6, 12, 24, and 48 hours after termination of CPB: heart rate, blood pressure, left atrial pressure, central-peripheral temperature difference, arterial-central venous oxygen saturation difference, urine output, serum creatinine, lactate and neutrophil elastase levels, the Doppler echocardiographic factors shortening fraction and preejection period/left-ventricular ejection time, and cumulative doses of catecholamines (epinephrine), enoximone, and furosemide. RESULTS: No hemodynamic variable showed any significant difference between aprotinin and placebo groups. Urine output, creatinine, lactate, and elastase levels, as well as the cumulative doses of furosemide and epinephrine were not significantly different. Twelve hours after CPB 10 patients in the placebo group and 4 in the aprotinin group had received enoximone (p<0.05). The placebo group had received significantly larger doses of enoximone than the aprotinin group at arrival in the intensive care unit (0.13+/-0.05 versus 0 mg/kg), 12 hours after CPB (0.58+/-0.14 versus 0.18+/-0.09 mg/kg), 24 hours after CPB (1.11+/-0.24 versus 0.42+/-0.16 mg/kg), and 48 hours after CPB (1.61+/-0.40 versus 0.86+/-0.28). At 6 hours the difference did not reach statistical significance. CONCLUSIONS: Clinical and hemodynamic status of the aprotinin-treated patients was similar to that of the placebo-treated patients in the first 48 hours after CPB. The placebo group, however, required significantly more inotropic support by enoximone than the aprotinin group to achieve this goal.

Adolescent↗