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Biomedical subjects

K L Wanderman

Publications and source records attributed to K L Wanderman.

At least 19 recordsLinked to original sources
PubMed1989

Cardiac involvement in glycogen storage disease type III.

Twenty patients with enzymatically proven glycogen storage disease type III (GSD III) aged 3-30 years underwent cardiological evaluation. Seventeen showed subclinical evidence of cardiac involvement in form of ventricular hypertrophy on ECG. Of 16 patients in whom an ECG examination was performed, 13 had abnormal echocardiographic features. Only 2 patients had cardiomegaly on X-ray. The cardiac findings in 1 of the patients, a 25-year-old female with clinically evident cardiomyopathy are described in detail. In view of our findings, patients with established GSD III, should not only be investigated regarding their muscular involvement, but should also undergo a detailed evaluation of their cardiac status.

Adolescent↗
PubMed1988

Cor pulmonale due to adenoidal or tonsillar hypertrophy or both in children. Noninvasive diagnosis and follow-up.

Four children, aged 1 to 3 1/2, were first seen with cor pulmonale, pulmonary edema and severe respiratory distress due to chronic upper airway obstruction secondary to adenoidal or tonsillar hypertrophy or both. Arterial blood gas values, electrocardiograms and chest x-ray films were compatible with cor pulmonale. Echocardiography (four cases) and radionuclide angiography (two cases) showed severe right ventricular and right atrial dilation with reduced right ventricular ejection fraction. Following surgery, all four children improved; their echocardiographic and radionuclide findings returned to normal. Cardiac catheterization, traditionally performed in such cases, was unnecessary. The diagnosis and follow-up of this syndrome are adequately performed noninvasively.

Adenoids↗
PubMed1986

Neuromuscular involvement in glycogen storage disease type III.

Sixteen patients with glycogen storage disease type III (GSD III) aged 3 to 22 years underwent a detailed neuromuscular evaluation. A minimal impairment of skeletal muscle function was presented in eight patients, slight impairment in four and severe impairment in one patient. Serum creatinine phosphokinase (CPK) was elevated in all patients studied. In the nine patients, in whom electromyography (EMG) was performed; six exhibited a myopathic pattern while a "mixed" (neurogenic-myopathic) pattern was present in three. Muscle biopsies performed in 12 patients, revealed in all cases amylo-1,6,-glucosidase deficiency and biochemical as well as morphological evidence of glycogen accumulation. Two brothers suffered from late onset myopathy, which in the older sibling was associated with clinical, EMG and EM findings of a peripheral neuropathy. Fifteen patients had either electrocardiographic and or echographic evidence of cardiomyopathy. Observations based on this patient material suggest a widespread myopathy in GSD III patients with heterogeneous expression, while peripheral nerve involvement is rarely encountered.

Adolescent↗
PubMed1986

Viral myocarditis simulating dilated cardiomyopathy in early childhood: evaluation by serial echocardiography.

Left ventricular dimensions and function were assessed by serial M mode and cross sectional echocardiography in ten infants and young children with heart muscle disease characterised by left ventricular dilatation and impaired systolic function presenting in congestive heart failure, severe respiratory distress, or both. The patients were followed for 8-60 months after their initial admission. The aetiology was probably viral in all cases. One patient died three weeks after diagnosis. Left ventricular size and function returned to normal in only one child; however, various degrees of improvement were found in seven others who were symptom free at follow up. This study demonstrates that serial echocardiography adequately defines this disease in infants and young children and that invasive evaluation is seldom required. Furthermore, it emphasises the fact that even in symptom free children an echocardiographic picture that is indistinguishable from dilated cardiomyopathy may persist; such patients required continued follow up.

Cardiomyopathy, Dilated↗
PubMed1982

Severe rheumatic mitral valve disease in children. Evaluation by echocardiography.

Severe rheumatic mitral stenosis with or without mitral insufficiency is virtually unseen in children in the affluent countries of the western world, but is not uncommon in developing countries. Fifteen cases of rapidly progressive rheumatic mitral valve disease in children are presented to illustrate the value of echocardiography in the diagnosis and evaluation of this lesion. Cardiac catheterization was performed in nine and simply confirmed the diagnosis made noninvasively. Our experience demonstrates that thorough echocardiographic examination in these cases frequently enables the examiner to assess the need for surgical intervention without cardiac catheterization.

Adolescent↗
PubMed1981

Systolic time intervals in adolescents. Normal standards for clinical use and comparison with children and adults.

Systolic time intervals were measured in 147 healthy 13-19-year-old adolescents to derive regression equations for clinical use in this age group and to determine to what extent they differ from those of children and adults. Stepwise regression analysis showed that heart rate was the only variable consistently and significantly related to electromechanical systole (QS2), left ventricular ejection time (LVET) and preejection period (PEP). None of the other variables tested (age, height, weight, body surface area, blood pressure, hemoglobin, hematocrit and serum electrolytes), when used alone, were relevant variables in relation to QS2, LVET or PEP. The PEP/LVET ratio in adolescents was essentially independent of heart rate, as in children and adults. The regression lines for QS2, LVET and PEP in adolescents fall between those for children and adults, indicating that there is a distinct tendency toward lengthening of all the systolic time intervals with age, independent of changes in heart rate. The PEP lengthens proportionately more than the LVET, resulting in a progressive increase in the mean PEP/LVET ratio from childhood (0.30) to adolescence (0.32) and to adulthood (0.345). The precise hemodynamic factors underlying these changes with increasing age remain to be determined.

Adolescent↗
PubMed1981

Choice of electrocardiographic leads for recording the earliest QRS onset in noninvasive measurements.

A significant error may be introduced in intervals measured from the onset of the QRS if an electrocardiographic lead that does not record the earliest deflection is used. To ascertain to what extent the commonly used leads can be relied on to show the earliest QRS onset, 100 normal subjects and 219 patients with heart disease were studied by means of simultaneous recording of three leads: a right precordial lead chosen to show an rS configuration, lead II, and another limb lead chosen to show a qR configuration. Lead II most frequently showed a delayed QRS onset--in 34% of normal subjects and 36% of the patients. In the other limb lead the initial QRS deflection was delayed in 24% of the normal subjects and 23% of the patients. The QRS onset in the right precordial lead was never delayed in the normal subjects; however, it was delayed in this lead in 6% of the patients. The delays in each of the leads ranged from 5-20 msec. We conclude that while a right precordial lead is by far the most reliable single lead that can be used for interval measurements, simultaneous recording of a right precordial lead and a limb lead assures that recording of the earliest QRS onset in all cases.

Electrocardiography↗
PubMed1979

Coexistence of hypertrophic obstructive cardiomyopathy and mitral annular calcification: proposed etiologic relationship.

A case of combined hypertrophic obstructive cardiomyopathy and mitral annular calcification is reported. The characteristic features of both entities were clearly demonstrated by thorough noninvasive examinations. It is proposed that the association of the two conditions is not coincidental but that mitral annular calcification, by narrowing of the left ventricular outflow tract, can result in the development of hypertrophic obstructive cardiomyopathy. Mitral annular calcification should possibly be added to the other causes of pressure overload of the left ventricle--such as hypertension and valvular or discrete subvalvular aortic stenosis--as a potential etiology of secondary hypertrophic obstructive cardiomyopathy.

Aged↗
PubMed1978

Systolic time intervals in children: normal standards for clinical use.

Systolic time intervals were measured in 253 normal children, with careful attention to precise recording and measuring techniques, to derive regression equations which could be used as reliable standards for clinical assessment of left ventricular performance in this age group. Using stepwise regression analysis, a highly significant correlation was found for electromechanical systole (QS2) and for left ventricular ejection time (LVET) with heart rate alone in both males and females, and age was not found to be a relevant variable. Similarly, for the preejection period (PEP) in males, heart rate was the only relevant variable. PEP in females showed a small but significant influence of age in addition to heart rate; in practice, this small influence can be neglected. Regression equations relating each of the systolic time intervals to heart rate alone were therefore derived for clinical use. The PEP/LVET ratio was found to be only weakly correlated with heart rate in males and with age in females, and in practice can be considered to be independent of these variables. The mean value of the PEP/LVET ratio and its standard deviation (0.30 +/- 0.04) can therefore be used for all children. We found that satisfactory recording of systolic time intervals can be obtained with little difficulty in almost all children using the techniques described. The method is therefore practical for clinical use in children, as it is in adults, and the normal standards derived in this study can serve as a reliable basis for its application.

Adolescent↗