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Natural history of childhood lipoid nephrosis
Yolume Number
Fig. and
86
Annotations
1
1. Examples monitoring
of the degree of correlation in simultaneously lead (upper tracing) ECG. See text for explanation.
and shape of QRS and P waves, though the comolexes were not identical. “No cerrelatign” describes ho similarity in form and/or direction (Fig. 1). In the “V1-like” lead, “excellent cwrelation” was found in 52 patients (86.6 per cent), “acceptable csrrelution” in 7 (11.6 per cent), and “1~) wrre&‘on” in only 1 case (1.7 per cent). “Excellenl correldion” was present in 49 patients (81.6 per cent) with the “aVp-like” lead, “acceptable correlation” in 9 (15 per cent), and “no correlation” in 2 (3.4 per cent). In the cases in which “no correZation”was found, the QRS complex in the conventional lead was always isodiphasic, thus probably explaining why small changes in the electrode position resulted in significant differences in the shape of the complexes recorded by the monitoring lead. The almost insignificant number of patients in whom lack of correlation was found when comparing monitoring and conventional leads, confirms the postulate that the basic diagnostic conclusions derived from the conventional ECG can be safely and reliably applied to the features obtained in a properly placed bipolar chest lead. In setting up a continuous ECG monitoring system, the adoption of a standard method of electrode placement seems advisable, since such a method
Natural
history
conventional
lead
(Zozuer kacing)
allows easier interpretation of ECG data by the unit’s staff, and provides adequate information for more sophisticated, computerized systems of arrhythmia detection as well. N. Cristal, M.D. Coronary Care Unit Neges Central Hospital Beer-Sheva, Israel I want to thank Mrs. R. Hoffman, R.N., for technical assistance in the preparation of this work. REFERENCES 1. 2.
3.
4.
of childhood
Recent studies have shown that the vast majority of children with the nephrotic syndrome have minima1 histological changes by renal biopsy.’ This type of renal disease, variously termed “nil” disease,
recorded
139
Stock, J. P. P.: New frontiers in arrhythmias, Br. Heart J. X&809, 1971. Desanctis, R. W., Block, P., and Hutter, A. M.: Tachyarrhythmias in myocardial infarction, Circulation 45:681, 1972. Marriott, H. J. L., and Fogg, E.: Constant monitoring for cardiac dysrhythmias and blocks, Mod. Concepts Cardiovasc. Dis. 39:103, 1970. Cristal, N., Gueron, M., and Hoffman, R.: “V, like” and “aVF like” leads for continuous electrocardiographic monitoring, Br. Heart J. 34:696, 1972.
lipoid nephrosis
epithelial cell disease, and lipoid nephrosis, is characterized by complete remissions induced by glucosteroid therapy. Sixty-one children with the nephrotic syndrome defined by heavy proteinuria, hy-
140
Am. Heart J. July, 1973
Annotations
100
80
I,
27
24
23
11
12
13
14
E v, VJ I, a
60
I
2
3
4
5
6
YEAR Fig. 1. The percentage of the relapsing by the bar and the number of patients
7
8
OF
FOLLOW-UP
9
10
group in complete remission during each remaining in the group is indicated at the
poalbuminemia, and edema and who had no known etiology for their disease formed the basis of our report.8 Clinical criteria used in selecting these patients were: minimal follow-up period of 5 years, age of onset 12 to 72 months, and complete remission due to initial steroid therapy. White and colleagues’ have demonstrated that these clinical characteristics will define a homogenous group of patients with lipoid nephrosis. Follow-up was 10 years for 51 patients, 15 years for 21 patients, and the average period of observation was 13.7 years. There were 10 patients who experienced only the initial episode and 51 patients who had one or more recurrences. There were 47 boys and 14 girls and the mean age of onset was 37 months. Clinical features during the initial episode, such as hematuria, transient hypertension, and azotemia, were not helpful in predicting whether an individual patient would follow a relapsing or non-relapsing course. The most helpful feature in determining the likelihood of a relapsing course was the duration of remission after the initial episode. Twenty-two patients were without relapse during the first year after onset, and of these, 12 (55 per cent) subsequently had at least one relapse. Of the 13 patients who were in remission during the first two consecutive years from onset, 23 per cent subsequently relapsed, while only one of eleven patients without relapse during the first three consecutive years had a later recurrence. All 10 patients who did not relapse during the first four consecutive years after onset remained in remission until the end of follow-up. These data suggest that the relapsing form of lipoid nephrosis is evident during the first two years of the disease and that complete resolution or “cure” is likely in the absence of a recurrence during the first three
15
year of follow-up top of each bar.
is shown
years after onset. In contrast to patients with a non-relapsing course, once a patient had experienced even one relapse, the likelihood of predicting complete resolution, even after extended periods in remission, was uncertain. For example, of 31 patients in remission for five years at some time during the course of illness after a relapse, seven (22 per cent) subsequently had a recurrence. Sufficient data were available on 45 of the 51 cases with a relapsing course for long-term evaluation of the frequency and pattern of relapses. While only 23 per cent remained free of relapse during the first year, approximately one-half of the group was in remission during each of the subsequent six years. Although the proportion in remission gradually increased with time, 20 per cent of the group had relapses as long as 15 years after onset (Fig. 1). Similarly, during the first year after onset, the group averaged more than one relapse per patient, but thereafter the relapse rate was less and a distinct trend toward a decreasing rate was evident after the tenth year of disease. The clinical status of all 61 patients at the end of the study was evaluated by complete history, physical examination, urinalysis, blood urea nitrogen (BUN), and serum creatinine. The ten patients who had only the initial episode each had a normal BUN, serum creatinine, and blood pressure at the completion of follow-up. Of the 51 patients with a relapsing course, 25 had been in remission for a minimum of two consecutive years; 22 were continuing to experience relapses: 13 of these were steroid responsive, 6 were steroid dependent, and 3 were steroid resistant. Of these 47 patients, all had normal BUN, serum creatinine, and blood pressure except the three who were steroid resistant. Of these
Volume Number
141
Annotations
86 1
three, all had persistent proteinuria; two had mildly elevated BUN (20 and 30 mg. per 100 ml., respectively) and serum creatinine (2.7 and 3.0 mg. per 100 ml., respectively) and the remaining patient had an elevated blood pressure (lSO/lOO mm. Hg). Four patients had died: three of these were steroid resistant and had renal insufficiency at the time of death, the fourth was steroid responsive and died of fluid and electrolyte complications during a relapse. These data indicate that the nephrotic syndrome in preschool age children is not a benign disease despite the high responsiveness to treatment and the nature of the histologic lesion. Although the incidence of progression to renal failure remains low and for most children there is a marked reduction in the frequency of relapses by the time they reach
adolescence, recurrences will be experienced by the majority of patients for many years. Norman .I. Siegel, M.D. Assistant Professor of Pediatrics Yale University School of Medicine
333 Cedar St. New
Haven,
Corm.
06510
REFERENCES 1. White, R. H. R., Glashow, E. F., and Mills, R. J.: Clinicopathological study of nephrotic syndrome in childhood, Lancet 1:1353, 1970. 2. Siegel, N. J., Goldberg, B., Krassner, L. S., and Hayslett, J. P.: Long-term follow-up of children with steroid-responsive nephrotic syndrome, J. Pediatr. 81:251, 1972.
The distress of dying
In contrast to many subjective reports, few objective assessments’-3 have been made on the distress of dying. Doctors spend little time with dying patients so the observations recorded by nurses can be of particular value in providing a more comprehensive assessment than most doctors make alone. For this reason charts were devised that enable nurses to record the distress of dying in a manner similar to their recording of temperature (TPR) charts. The factors to be assessed include pain, respiratory distress at rest, awareness of dying, depression, anxiety, vomiting, and incontinence. Pain is recorded on a vertical chart graded 0 to 10 with the absence of pain recorded as 0 and very severe pain as 10. A similar principle is used for recording the other features of distress. The charts provide a useful pictorial image, the significance of which is easily assessed. In extracting data for statistical analysis, one has the problem of taking and analyzing from diurnal linear charts information recorded in a manner similar to that normally used for temperature charts. One can take the highest, lowest, or average figures, though where the aim is to relieve distress, the highest figures are most important and these are the ones used. Data was obtained about 50 consecutive deaths occurring at home or hospital during a 12 month oeriod. in the Llanidloes area of Wales. Significant differences occurred between deaths at home and in hospital for three reasons. Patients dying at home were (1) more likely to be fully alert shortly before death (P < O.OS), (2) less likely to be suffering from vomiting, incontinence, or bedsores (P < O.OOl), and ($) $s; li$ly to have unrelieved physical distress . . It is possible that the presence of an alert mind and the absence of symptoms requiring constant
nursing care determined the site of death of these patients. Most people dying at home were free from vomiting, incontinence, and bedsores, whereas 28 per cent of all patients were troubled at some time by vomiting, 30 per cent had bedsores, 30 per cent had fecal incontinence, 2 per cent a colostomy, 20 per cent required an indwelling catheter, and another 46 per cent had urinary incontinence. Only 26 per cent had none of the above symptoms. Twenty-two per cent revealed an awareness that they might die, 10 per cent expected to die soon, 10 per cent thought they would probably die, and 2 per cent thought they might possibly die. Fiftyfour per cent experienced pain of varying severity and 26 per cent had severe or very severe pain. Only 20 per cent had no respiratory distress at rest; 26 per cent had severe or very severe respiratory distress, though in some of these instances the respiratory distress was mitigated by the fact that they were stuporose or comatose. Fifty-six per cent experienced some anxiety and 40 per cent had depression. At times the anxiety of 14 per cent was so severe, they could be described as fearful. The results can be compared with those reported by Hinton’ and Exton-Smith,* though the three surveys do differ in method, type of patient studied, and environment. In Llanidloes consecutive deaths in a community were assessed by trained nurses. The 220 patients in Exton-Smith’s survey2 all died in a geriatric unit. His information was obtained “from personal observation and close enquiry from the nursing staff.” Hinton’s study was confined to a teaching hospital. He interviewed 102 dying patients and later questioned the sister or staff nurse about the state of the patient in the last few hours of life. On average, the oldest patients were in Exton-Smith’s groupg and the youngest in Hinton’s.’ The cause of death differed in the three surveys,
Fig. and
86
Annotations
1
1. Examples monitoring
of the degree of correlation in simultaneously lead (upper tracing) ECG. See text for explanation.
and shape of QRS and P waves, though the comolexes were not identical. “No cerrelatign” describes ho similarity in form and/or direction (Fig. 1). In the “V1-like” lead, “excellent cwrelation” was found in 52 patients (86.6 per cent), “acceptable csrrelution” in 7 (11.6 per cent), and “1~) wrre&‘on” in only 1 case (1.7 per cent). “Excellenl correldion” was present in 49 patients (81.6 per cent) with the “aVp-like” lead, “acceptable correlation” in 9 (15 per cent), and “no correlation” in 2 (3.4 per cent). In the cases in which “no correZation”was found, the QRS complex in the conventional lead was always isodiphasic, thus probably explaining why small changes in the electrode position resulted in significant differences in the shape of the complexes recorded by the monitoring lead. The almost insignificant number of patients in whom lack of correlation was found when comparing monitoring and conventional leads, confirms the postulate that the basic diagnostic conclusions derived from the conventional ECG can be safely and reliably applied to the features obtained in a properly placed bipolar chest lead. In setting up a continuous ECG monitoring system, the adoption of a standard method of electrode placement seems advisable, since such a method
Natural
history
conventional
lead
(Zozuer kacing)
allows easier interpretation of ECG data by the unit’s staff, and provides adequate information for more sophisticated, computerized systems of arrhythmia detection as well. N. Cristal, M.D. Coronary Care Unit Neges Central Hospital Beer-Sheva, Israel I want to thank Mrs. R. Hoffman, R.N., for technical assistance in the preparation of this work. REFERENCES 1. 2.
3.
4.
of childhood
Recent studies have shown that the vast majority of children with the nephrotic syndrome have minima1 histological changes by renal biopsy.’ This type of renal disease, variously termed “nil” disease,
recorded
139
Stock, J. P. P.: New frontiers in arrhythmias, Br. Heart J. X&809, 1971. Desanctis, R. W., Block, P., and Hutter, A. M.: Tachyarrhythmias in myocardial infarction, Circulation 45:681, 1972. Marriott, H. J. L., and Fogg, E.: Constant monitoring for cardiac dysrhythmias and blocks, Mod. Concepts Cardiovasc. Dis. 39:103, 1970. Cristal, N., Gueron, M., and Hoffman, R.: “V, like” and “aVF like” leads for continuous electrocardiographic monitoring, Br. Heart J. 34:696, 1972.
lipoid nephrosis
epithelial cell disease, and lipoid nephrosis, is characterized by complete remissions induced by glucosteroid therapy. Sixty-one children with the nephrotic syndrome defined by heavy proteinuria, hy-
140
Am. Heart J. July, 1973
Annotations
100
80
I,
27
24
23
11
12
13
14
E v, VJ I, a
60
I
2
3
4
5
6
YEAR Fig. 1. The percentage of the relapsing by the bar and the number of patients
7
8
OF
FOLLOW-UP
9
10
group in complete remission during each remaining in the group is indicated at the
poalbuminemia, and edema and who had no known etiology for their disease formed the basis of our report.8 Clinical criteria used in selecting these patients were: minimal follow-up period of 5 years, age of onset 12 to 72 months, and complete remission due to initial steroid therapy. White and colleagues’ have demonstrated that these clinical characteristics will define a homogenous group of patients with lipoid nephrosis. Follow-up was 10 years for 51 patients, 15 years for 21 patients, and the average period of observation was 13.7 years. There were 10 patients who experienced only the initial episode and 51 patients who had one or more recurrences. There were 47 boys and 14 girls and the mean age of onset was 37 months. Clinical features during the initial episode, such as hematuria, transient hypertension, and azotemia, were not helpful in predicting whether an individual patient would follow a relapsing or non-relapsing course. The most helpful feature in determining the likelihood of a relapsing course was the duration of remission after the initial episode. Twenty-two patients were without relapse during the first year after onset, and of these, 12 (55 per cent) subsequently had at least one relapse. Of the 13 patients who were in remission during the first two consecutive years from onset, 23 per cent subsequently relapsed, while only one of eleven patients without relapse during the first three consecutive years had a later recurrence. All 10 patients who did not relapse during the first four consecutive years after onset remained in remission until the end of follow-up. These data suggest that the relapsing form of lipoid nephrosis is evident during the first two years of the disease and that complete resolution or “cure” is likely in the absence of a recurrence during the first three
15
year of follow-up top of each bar.
is shown
years after onset. In contrast to patients with a non-relapsing course, once a patient had experienced even one relapse, the likelihood of predicting complete resolution, even after extended periods in remission, was uncertain. For example, of 31 patients in remission for five years at some time during the course of illness after a relapse, seven (22 per cent) subsequently had a recurrence. Sufficient data were available on 45 of the 51 cases with a relapsing course for long-term evaluation of the frequency and pattern of relapses. While only 23 per cent remained free of relapse during the first year, approximately one-half of the group was in remission during each of the subsequent six years. Although the proportion in remission gradually increased with time, 20 per cent of the group had relapses as long as 15 years after onset (Fig. 1). Similarly, during the first year after onset, the group averaged more than one relapse per patient, but thereafter the relapse rate was less and a distinct trend toward a decreasing rate was evident after the tenth year of disease. The clinical status of all 61 patients at the end of the study was evaluated by complete history, physical examination, urinalysis, blood urea nitrogen (BUN), and serum creatinine. The ten patients who had only the initial episode each had a normal BUN, serum creatinine, and blood pressure at the completion of follow-up. Of the 51 patients with a relapsing course, 25 had been in remission for a minimum of two consecutive years; 22 were continuing to experience relapses: 13 of these were steroid responsive, 6 were steroid dependent, and 3 were steroid resistant. Of these 47 patients, all had normal BUN, serum creatinine, and blood pressure except the three who were steroid resistant. Of these
Volume Number
141
Annotations
86 1
three, all had persistent proteinuria; two had mildly elevated BUN (20 and 30 mg. per 100 ml., respectively) and serum creatinine (2.7 and 3.0 mg. per 100 ml., respectively) and the remaining patient had an elevated blood pressure (lSO/lOO mm. Hg). Four patients had died: three of these were steroid resistant and had renal insufficiency at the time of death, the fourth was steroid responsive and died of fluid and electrolyte complications during a relapse. These data indicate that the nephrotic syndrome in preschool age children is not a benign disease despite the high responsiveness to treatment and the nature of the histologic lesion. Although the incidence of progression to renal failure remains low and for most children there is a marked reduction in the frequency of relapses by the time they reach
adolescence, recurrences will be experienced by the majority of patients for many years. Norman .I. Siegel, M.D. Assistant Professor of Pediatrics Yale University School of Medicine
333 Cedar St. New
Haven,
Corm.
06510
REFERENCES 1. White, R. H. R., Glashow, E. F., and Mills, R. J.: Clinicopathological study of nephrotic syndrome in childhood, Lancet 1:1353, 1970. 2. Siegel, N. J., Goldberg, B., Krassner, L. S., and Hayslett, J. P.: Long-term follow-up of children with steroid-responsive nephrotic syndrome, J. Pediatr. 81:251, 1972.
The distress of dying
In contrast to many subjective reports, few objective assessments’-3 have been made on the distress of dying. Doctors spend little time with dying patients so the observations recorded by nurses can be of particular value in providing a more comprehensive assessment than most doctors make alone. For this reason charts were devised that enable nurses to record the distress of dying in a manner similar to their recording of temperature (TPR) charts. The factors to be assessed include pain, respiratory distress at rest, awareness of dying, depression, anxiety, vomiting, and incontinence. Pain is recorded on a vertical chart graded 0 to 10 with the absence of pain recorded as 0 and very severe pain as 10. A similar principle is used for recording the other features of distress. The charts provide a useful pictorial image, the significance of which is easily assessed. In extracting data for statistical analysis, one has the problem of taking and analyzing from diurnal linear charts information recorded in a manner similar to that normally used for temperature charts. One can take the highest, lowest, or average figures, though where the aim is to relieve distress, the highest figures are most important and these are the ones used. Data was obtained about 50 consecutive deaths occurring at home or hospital during a 12 month oeriod. in the Llanidloes area of Wales. Significant differences occurred between deaths at home and in hospital for three reasons. Patients dying at home were (1) more likely to be fully alert shortly before death (P < O.OS), (2) less likely to be suffering from vomiting, incontinence, or bedsores (P < O.OOl), and ($) $s; li$ly to have unrelieved physical distress . . It is possible that the presence of an alert mind and the absence of symptoms requiring constant
nursing care determined the site of death of these patients. Most people dying at home were free from vomiting, incontinence, and bedsores, whereas 28 per cent of all patients were troubled at some time by vomiting, 30 per cent had bedsores, 30 per cent had fecal incontinence, 2 per cent a colostomy, 20 per cent required an indwelling catheter, and another 46 per cent had urinary incontinence. Only 26 per cent had none of the above symptoms. Twenty-two per cent revealed an awareness that they might die, 10 per cent expected to die soon, 10 per cent thought they would probably die, and 2 per cent thought they might possibly die. Fiftyfour per cent experienced pain of varying severity and 26 per cent had severe or very severe pain. Only 20 per cent had no respiratory distress at rest; 26 per cent had severe or very severe respiratory distress, though in some of these instances the respiratory distress was mitigated by the fact that they were stuporose or comatose. Fifty-six per cent experienced some anxiety and 40 per cent had depression. At times the anxiety of 14 per cent was so severe, they could be described as fearful. The results can be compared with those reported by Hinton’ and Exton-Smith,* though the three surveys do differ in method, type of patient studied, and environment. In Llanidloes consecutive deaths in a community were assessed by trained nurses. The 220 patients in Exton-Smith’s survey2 all died in a geriatric unit. His information was obtained “from personal observation and close enquiry from the nursing staff.” Hinton’s study was confined to a teaching hospital. He interviewed 102 dying patients and later questioned the sister or staff nurse about the state of the patient in the last few hours of life. On average, the oldest patients were in Exton-Smith’s groupg and the youngest in Hinton’s.’ The cause of death differed in the three surveys,