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Biomedical subjects

C Opitz

Publications and source records attributed to C Opitz.

At least 37 records · Page 2Linked to original sources
PubMed1998

Bilaterally cleft lip, limb defects, and haematological manifestations: Roberts syndrome versus TAR syndrome.

We report on a 13-year-old patient followed since birth. He is the only offspring of young, non-consanguineous German parents. His mother has an isolated left cleft of lip and a cleft palate. At birth, our patient presented with bilaterally cleft lip/cleft palate, phocomelia of upper limbs with normal hands, and mild symmetrical deficiencies of the long bones of the lower limbs. Haematological evaluation demonstrated a leukaemoid reaction during a urinary tract infection as well as intermittent thrombocytopenia and episodes of marked eosinophilia during the first two years of life. Intellectual development has been normal. Comparison with two similar cases from the literature suggests a non-random phenotypic overlap of Roberts syndrome (MIM 268300) and TAR syndrome (MIM 274000). Such clinical constellations may be key observations to understand the genetic relationship of Roberts syndrome and TAR syndrome in future phenotype-genotype correlations.

Adolescent↗
PubMed1998

Oculo-facio-cardio-dental (OFCD) syndrome.

The association of facial dysmorphy, congenital cataracts, microphthalmia, heart disease, and dental radiculomegaly is very rare. We describe a girl with atrial septal defect, unilateral congenital cataract, unilateral microphthalmia, radiculomegaly of incisor and canine teeth with open apices and other dental crown anomalies. This combination of symptoms clearly represents a distinct syndrome and has recently been described as oculo-facio-cardio-dental (OFCD) syndrome.

Abnormalities, Multiple↗
PubMed1998

[Nager syndrome].

In this publication, Nager syndrome was analyzed in the literature and six patients from our clinic were evaluated in relation to symptoms, etiology and pathogenesis. The diseases to be considered when making a differential diagnosis are pointed out. Clarification of the etiology is still pending. Molecular genetic research in these patients is possibly the key for new findings. A case report illustrates the results of interdisciplinary treatment by the surgeon and orthodontist. Possibilities and problems in relation to therapy are demonstrated.

Abnormalities, Multiple↗
PubMed1998

Impaired ventilatory efficiency in chronic heart failure: possible role of pulmonary vasoconstriction.

BACKGROUND: Patients with chronic heart failure show impairment of ventilatory efficiency, defined as the relation between ventilation and carbon dioxide output. It is caused by ventilation of excess physiologic dead space. We hypothesized a role of active vasoconstriction in the increase of physiologic dead space, presumed to lead to alveolar hypoperfusion. METHODS AND RESULTS: In 57 patients with chronic heart failure (New York Heart Association classification II through IV, ejection fraction 25.6%+/-10.4%) and 7 control subjects, gas exchange at rest and on exercise was compared with hemodynamic measurements and, in a subgroup of 15 patients, with endothelin-1, epinephrine, and norepinephrine levels in the pulmonary and systemic circulation. Ventilatory efficiency at rest (VE/VCO2 ratio) correlated with ventilatory efficiency on exercise (VE vs VCO2 slope). Impairment of ventilatory efficiency correlated strongly negative with exercise tolerance (maximal oxygen uptake: r = -0.67) and cardiac output (r = -0.66) and positive with pulmonary hypertension (mean pulmonary artery pressure: r = 0.69, pulmonary vascular resistance: r = 0.60). None of the vasoconstrictors correlated with reduction of ventilatory efficiency in the subgroup studied. CONCLUSIONS: Impairment of ventilatory efficiency in chronic heart failure is correlated with resting pulmonary artery pressures and associated with the impairment of exercise capacity. An imbalance of pulmonary vascular tone probably leads to both pulmonary hypertension and alveolar hypoperfusion.

Adult↗
PubMed1997

[Median facial dysplasia in patients with lip-jaw-palate clefts. Characteristics and problems in interdisciplinary orthodontic-oral surgery treatment].

The diagnosis of median facial dysplasia is made in cases of a typical deficiency of the median facial structures without concomitant abnormalities of the brain, and presence of unilateral or bilateral cleft lip and palate. The identification and description of this type of cleft lip and palate are important because such patients have severe growth disturbances and tissue deficiencies. These make surgical reconstruction and orthodontic treatment difficult. This is demonstrated in two examples. The clinical and roentgenological findings in the mother of one of the demonstrated patients point to a discrete form of the same anomaly.

Adolescent↗
PubMed1997

[Aerosolized prostacyclin for preoperative evaluation and post-cardiosurgical treatment of patients with pulmonary hypertension].

BACKGROUND: Inhaled nitric oxide (NO) has been shown to selectively lower pulmonary vascular resistance and is applied in patients with pulmonary hypertension (PHT). However, application and monitoring is complex and not always successful ("non-responders"). We evaluated the effect of aerolized prostacyclin (aePGI2) as a therapeutic alternate to NO. PATIENTS AND METHODS: aePGI2 and NO were applied to patients with different causes of pulmonary hypertension (Group 1a: preoperative patients with intracardiac shunting defects and Eisenmenger's disease, n = 30; Group 1b: patients with primary or postoperative PHT, n = 13; Group 2: PHT immediately following surgery for congenital heart disease, n = 6). RESULTS: Pulmonary vascular resistance could be lowered significantly (Group 1a: from 91% of systemic vascular resistance to 58% with NO and 53% with aePGI2; Group 1b: from 20.2 Wood Units*m2 to 13.4 and 11.3; Group 2: from 24.9 Wood Units*m2 to 9.5 and 10.5); cardiac index increased (Group 1b: from 2.96 to 3.55 and 3.96 l/min*m2, Group 2: from 1.57 to 1.89 and 2.00 l/min*m2). CONCLUSIONS: The short-term application of aePGI2 shows a selective pulmonary vasodilation similar to NO. Given adequate monitoring, aePGI2 appears to be useful for the acute treatment of PHT.

Administration, Inhalation↗
PubMed1997

Relationship between haemodynamics and morphology in pulmonary hypertension. A quantitative intravascular ultrasound study.

BACKGROUND: Intravascular ultrasound imaging of the pulmonary arteries has been demonstrated to be a reliable method of quantifying vessel diameter, luminal area and pulsatility. Simultaneous measurement of flow velocity and its response to vasodilators allows the relationship between morphology and functional compromise to be studied, especially endothelial dysfunction. METHODS: In 51 patients (mean age = 49.8 +/- 12.6 years, 17 female) we performed right heart catheterization and simultaneous intravascular ultrasound of pulmonary artery branches. The patients were divided in two groups: group 1 with normal pulmonary artery pressure and pulmonary vascular resistance, and group 2 with pulmonary hypertension (peak pulmonary artery pressure > 30 mmHg and/or mean pulmonary artery pressure > 20 mmHg). Vessel wall and lumen were studied using a 2.9 F intravascular ultrasound catheter with a 30 MHz phased array transducer. Measurement of blood flow velocity was accomplished by a Doppler flow wire (0.018 inch). The maximal flow change during acetylcholine infusion (adjusted to 10(-6); 10(-5), and 10(-4) M concentration in the blood vessel) was measured. RESULTS: There were no significant differences between groups 1 and 2 with respect to age (48.5 +/- 14.3 years vs 50.3 +/- 12.3 years; P = ns), gender (4 female/8 male vs 13 female/26 male; P = ns), luminal area of the vessel segment in which the intravascular ultrasound measurements were obtained (11.8 +/- 6.1 mm2 vs 16.7 +/- 14.3 mm2; P = ns), internal diameter (3.9 +/- 1.2 mm vs 4.2 +/- 1.7 mm; P = ns), and external diameter (6.1 +/- 1.3 mm vs 6.9 +/- 2.1 mm; P = ns). Cross-sectional images of the pulmonary artery wall demonstrated a single ring with high echodensity with a thin inner layer regarded as intima in group 1. In contrast, the majority of patients (n = 35/39) in group 2 demonstrated a thickening of the intimal layer and/or a disturbance of layering of the echogenic arterial wall. The relative wall thickness was higher in group 2 than in group 1 (22.5 +/- 10.4% vs 15.3 +/- 6.5%; P < 0.05). There were no significant correlations between pulmonary artery pressure and wall thickness pulmonary artery pressure and area change in the cardiac cycle, acetylcholine-dependent increase in pulmonary flow and morphological changes in the vessel wall. CONCLUSION: We conclude that intravascular ultrasound is capable of detecting morphological changes in the pulmonary vessel wall in pulmonary hypertension and that vessel wall hypertrophy of small pulmonary segment arteries, as detected by intravascular ultrasound, is not predictive of functional vasodilatory response of resistance vessels of the same vessel area.

Aged↗
PubMed1997

Maxilla dimension in patients with unilateral and bilateral cleft lip and palate. Changes from birth until palate surgery at age three.

This study traces the changes in maxillary dimension in 44 unilateral cleft lip and palate (UCLP) patients and 28 bilateral cleft lip and palate (BCLP) patients from birth up to the time of palate surgery at age 3. Measurements on dental casts were carried out at 4 defined registration stages. The results revealed a continuous reduction in cleft dimension parallel to an increase in the transversal and sagittal maxillary dimensions. The inclination of the palatal slope displayed such a high degree of variability that no statement regarding the development in this variable could be made. Differences between the 2 types of cleft patients were found only in the maxillary sagittal dimension. Wearing an orthopedic plate after surgery appears to prevent transversal collapse in the anterior region and to counteract external transversal and sagittal forces. Only temporary inhibition of growth in the sagittal dimension of the maxilla was observed after lip surgery.

Aging↗
PubMed1997

Unilateral cleft lip and palate. Relationship between morphology of the dentition and functional parameters of the tongue.

The relationship between orthodontic and logopedic findings was evaluated with statistical contingency analysis. The investigation was focused on selected dentomorphologic parameters and oral function/malfunction of 100 patients between 3 and 7 years of age with unilateral cleft lip and palate. For further classification into contingency tables "normal" and "abnormal" attributes were defined. Interdependencies between observed findings and their probable frequencies were calculated and represented in diagrams by means of statistical parameters such as the contingency coefficient. Scatterplots of the calculated coefficients showed a characteristic pattern corresponding to clinical experience. While overjet and transversal occlusion are strongly associated with oral functions, overbite tends not to be associated with oral functions. The contingency coefficient calculated from sagittal and transversal orthodontically relevant conditions of occlusion and incorrect tongue position as well as incorrect swallowing tends to be stronger than that of "abnormal" morphologic parameters and an incorrect tongue position during speech. The longitudinal study of lip position and the 3 investigated morphologic parameters revealed strongly associated contingency coefficients.

Child↗
PubMed1996

Cephalometric investigations concerning the geometry of the viscerocranium of human anencephalic fetuses.

Cephalometric investigations were carried out on 16 macerated skulls of anencephalic fetuses aged around 6 months and compared with the values obtained from a group of 18 normally developed specimens. The skull geometry of the anencephalic fetuses was different from normal proportions: anencephalic fetuses showed a clear mandibular prognathism, and a maxilla tilted in anterior direction. The values describing length of maxilla and length of the anterior cranial base, and NS-Ba angle were found to be within the normal range. From the results it can be concluded that the defective development of the forebrain seems to have no effects on the length development of the anterior cranial base although it does influence the form of the surrounding bony structures. The formation of the mandibular prognathism cannot be brought into direct connection with the defective brain development.

Anencephaly↗
PubMed1992

[A contribution to the controversial discussion on a preoperative orthodontic treatment for infants with unilateral cheilognathopalatoschisis].

A longitudinal study of 39 casts of pre-operative orthodontically treated unilateral cleft lip and palate children from birth to palate operation was carried out with a special coordinate measuring technique to quantify changes in the maxilla. A significant reduction in width of the alveolar cleft was found. The anterior alveolar arch width remained constant, while the posterior region slightly increased. These results seem to justify treatment with the infant appliance. After measuring casts from 39 preoperatively treated and casts from 62 untreated patients prior to lip operation, a comparison of the mathematical average values between the two groups revealed few differences. This can be explained by referring to the original morphological findings and the different measurements during the preoperative orthodontic treatment, which lead to different changes in segment position prior to lip operation. In a cross section examination this difference could not be found. A sub-classification of the subject matter in primary and secondary clefts revealed that between both types clear differences in the resulting parameters can be observed. This leaves the question, whether the width of the cleft palate is caused by tissue deficiency or by an embedded tongue. Furthermore, how will the orthodontic appliance therapy influence the growth? A greater dislocation of the segments in secondary clefts may be expected in cases where the rest position of the tongue appears caudal. Improved measuring methods to record cleft morphology and more emphasis on the study of soft tissue reactions may help to understand the differing results after preoperative or orthodontic treatment.

Cleft Lip↗
PubMed1991

[Maxillofacial growth in difficult conditions: on orthodontic rehabilitation of patients with cleft lip-jaw-palate].

The orthodontic treatment of cleft palate patients extends from birth to youth and is divided into three phases: pre-operative phase, deciduous dentition, mixed and permanent dentition. Therapeutic concepts for each particular phase are presented. Information is also provided on research into the growth of the jaw and face area in cleft palate patients compared with a control group.

Child↗
PubMed1990

[Follow-up observation in case of cherubism].

It is described the development of cherubism by a boy during the time of 10 years. All symptoms of this syndrome could be shown. In progress of the illness the lateral mandibular-swellings be reduced, but the roentgenological alterations continue to exist in change figure, extension and position. Additional the bone-structure in the upper incisivi-region disintegrated. Nearly all teeth of the second dentition are afflicted with the cystical changes of the jaw and lead to their loss.

Cherubism↗
PubMed1989

[Normal and pathologic development of the secondary palate--a new concept].

Wether the secondary palate develops in the normal or pathological manner is determined by the following processes: Within the contact-epithelium of each palate process there is a still invisible differentiation boundary between the potential oral and nasal epithelium. It extends from rostral to pharyngeal. A genetically determined and timed regular separation of nasal and oral epithelium and an initial epithelial shift along these differentiation boundaries allow the well-timed "primary" fusion of mesenchyme. A great oral part of the contact-epithelium in the thick primordia of the human hard palate is removed by fragmentation (and formation of epithelial pearls). This leads additionally to the retarded "secondary" mesenchymal fusion. Depending on the absence or only the retardation of the epithelium separation and shift the results are the open and the covered clefts. All normal and pathological developments can occur along the differentiation boundary in total extension or only partially.

Cell Differentiation↗
PubMed1989

[3-dimensional recording of changes in the infant jaw with unilateral cheilognathopalatoschisis with special reference to the results of preoperative orthodontic therapy].

The changes of jaws of cleft palate children were examined from birth until after the operative lip closure by the aid of a method of coordinate measurement technique. The examination resulted in a significant reduction of the anterior, medial and posterior cleft width during the preoperative treatment period. The anterior and posterior width of alveolar crest arch remained nearly constant in the examination period. The translation of the anterior poles of the palate segments in the case of the unilateral clefts were more distinct in the transversal direction than in sagittal direction. Values of slope were constant or became little smaller after the orthopedic treatment. After the lip closure we found end-to-end relation resulting from guided growth of the segments having constant arch dimensions. A collapse of the segments could not be observed. Altogether we can say the preoperative orthopedic therapy would give the cleft palate good suppositions for the further development.

Cleft Lip↗
PubMed1989

[Three-dimensional recording of changes in infant jaws with bilateral lip-maxilla-palate clefts with special reference to the results of preoperative orthodontic therapy].

Bilateral clefts are more difficult to treat than unilateral ones. The most important step during the orthopedic therapy is the retrusion of the premaxilla between the lateral segments of the maxilla. Thus the anterior cleft width, as opposed to the medial and posterior cleft width, did not become smaller. The position of premaxilla is mainly shown in the sagittal measurement values. The values of slope also changed in this sample. The maxilla became more flat. The preoperative orthodontic therapy can also be seen as advantageous in the case of bilateral clefts.

Child, Preschool↗