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Behavior in girls with true precocious puberty
EDITORIAL CORRESPONDENCE |
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II.
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I
Heart block with viral myocarditis
Reply
To the Editor." To the report of Mahoney et al., ~ we would like to add our recent experience with complete heart block caused by myocarditis. Unlike the children they described, our patient had no systemic viral prodrome when she presented with syncope. This 3-year-old girl had been in excellent health, with no history of heart disease. While playing she had a sudden, momentary loss of consciousness. Over the ensuing 3 hours four similar episodes occurred, each of approximately 30 seconds duration. Her pulse was irregular, varying from 50 to 90 bpm, and she was afebrile. An ECG showed third-degree A-V block with an idioventricular rhythm, diffuse ST-T wave changes, left anterior hemiblock, and poor R wave progression. Recurrent lapses of consciousness were associated with several 5- tO 8-second episodes of ventricular asystole, which did not respond to atropine. A temporary transvenous pacemaker (VVI) was inserted, and the patient's condition stabilized. Initial laboratory evaluation, including white blood cell count, erythrocyte sedimentation rate, ereatine kinase, chest radiograph, and echocardiogram yielded normal findings. On the fourth hospital day, cardiac catheterization and endomyocardial biopsy were performed. The intracardiac and coronary artery anatomy were normal. Left ventricular function, enddiastolic pressure, and cardiac output were normal. The endomyocardial biopsy demonstrated moderate inflammatory cell infiltrates, which included lymphocytes, plasma cells, and occasional eosinophils; there were several foci of myocyte necrosis. Because of the histologie findings, prednisone therapy was begun, although intermittent return of A-V conduction had already been demonstrated. Over the next 7 days normal sinus rhythm returned. Two months after diagnosis, the ECG was entirely normal. Viral cultures and titers were nondiagnostic. Mahoney et al. ~ state that a Stokes-Adam attack may be the only clinical manifestation of myocarditis. All of their patients had symptoms of a viral syndrome, however. The diagnosis of myoearditis was substantiated by rising viral titers (two patients) or by elevated cardiac enzymes (one patient). Our report emphasizes that myocarditis can present as sudden onset of complete heart block without evidence of viral infection by history, physical examination, or laboratory studies. Further, this case demonstrates the important diagnostic role of endomyocardial biopsy when the cause of symptomatic complete heart block is in doubt. Burt I. Bromberg, M.D. Robert H. Beekman, M.D. Kathleen Heidelberger, M.D. Departments o f Pediatrics and Pathology C. S. Mott Children's Hospital The University o f Michigan Medical School Ann Arbor, M I 48109
To the Editor: 1 agree that sudden onset of complete heart block can occur without clinical evidence of viral infection. The autopsy studies we referred to substantiate this conclusion. Endomyocardial biopsy is becoming more routine in pediatric cardiology practice, and will be an important tool in the diagnosis of myocarditis. Their case also supports the potentially transient nature of a heart block and syncope and the need for a temporary transvenous pacemaker. Larry T. Mahoney, M.D. Assistant Professor Pediatric Cardiology The University o f lowa Hospitals and Clinics Iowa City, 1,4 52242
REFERENCES 1.
790
Mahoney LT, Marvin WJ Jr, Atkins DL, et al. Pacemaker management for acute onset of heart block in children. J PEDIATR 1985;107:207.
Behavior in girls with true precocious puberty To the Editor: We read with interest the report by Sonis et al. ~ on behavior problems and social competence in girls with true precocious puberty, and wish to comment in light of our experience. In a study of 100 girls with precocious puberty, we gave parents the child behavior checklist (CBCL). 2 The Hebrew version of the CBCL has been found reliable (Kronbaeh alpha value of the scales .78 to .91) and valid in a large population? We were able to obtain data from 47 girls aged 6 to 11 years (mean 8.3 years) with true (central) idiopathic precocious puberty (PPid). All patients with tumors were excluded from this study, in contradistinction to that of Sonis et al. 1The results were compared with those obtained in a group of 20 healthy girls with no evidence of sexual precocity, matched for age and socioeconomic status. The socioeconomic status (Israeli standards) revealed that 37 patients were from middle class families, seven from lower class, and two from upper class families. In contrast to the data reported by Sonis et al., only 7% of the patients had a total behavior problem score between 71 and 100. Furthermore, there was a difference between patients with PPid and the controls on only five of the 12 scales of the CBCL (internalizing total scale, schizoid, delinquent, externalizing, and total behavior problem scales), as compared with a difference on nine of 12 scales for the subjects described by Sonis et al. Our control group, compared with that of Sonis et al., had lower scores (closer to the 50 T score) on 10 to 12 scales, with a significant difference on five (depression, schizoid, sex problems, delinquency, and aggression). When our group with PPid was compared with that of Sonis et al., ours had lower scores on all 12 scales, with significant differences on eight (internalizing, depressed, social withdrawal, somatic, externalizing, sex problems, aggressive, and total). What may be the cause for these differences between our results and those reported by Sonis et al? Environmental factors, such as cultural Or socioeconomic differences, would not seem to be of
Volume 108 Number 5, Part 1
great importance, because the differences between the proband groups in both studies are much greater than those found between the control groups in both studies. Sonis et al. suggested that these differences m a y be related to the fact that their patients are a very select group who have traveled to Bethesda for treatment. O u r own group is more likely to be representative of the entire population, because distance is no major factor in reaching our center. Furthermore, 11 of the 33 patients in the Sonis group had some kind of central nervous system or hypothalamic problem, and this m a y be one reason for the increased incidence of emotional and behavioral problems; our group comprised only patients with PPid. Further studies are needed on the behavioral aspects of PPid and the correlation with hormone activity and age of onset. These may help to delineate more fully the role of sex hormones in this disorder. IQ studies in a group of 52 girls with precocious puberty revealed a significant superiority in verbal IQ scores, which was interpreted as possible evidence of an effect of sex hormones on brain development, particularly on the left hemisphere, during the prepubertal period. 4
A. Galatzer, M.A. Z. Laron, M.D. Institute o f Pediatric and Adolescent Endocrinology Beilinson Medical Center and Sackler School o f Medicine Tel Aviv University Tel Aviv, Israel REFERENCES
1.
2.
3.
4.
Sonis W A , Comite F, Blue J, et al. Behavior problems and social competence in girls with true precocious puberty. J PED~AT~ 1985; 106:156-160. Achenbach T M , Edelbrock CS. Behavior problems and competencies reported by parents of normal and disturbed children aged four through sixteen. Monogr Soc Res Child Dev 1981;44(serial no. 188):1. Offer R. The validity and reliability of the child behavior checklist (CBCL). M.A. Thesis, Department of Psychology, Bar llan University, R a m a t Gan, Israel, 1983. Galatzer A, Beit Halachmi N, Kauli R, Laron Z. Intellectual functions of girls with precocious puberty. Pediatrics 1984;74:246-249.
O u t c o m e of home mechanical
ventilation To the Editor: W e read with interest and concern the article by Frates et al) on home mechanical ventilation. The authors make an interesting cost, mortality, and morbidity analysis, showing that home mechanical ventilation is 16 times cheaper than hospital care, that registered nurses are of no benefit, and that this treatment modality is reasonably safe. A m o n g these patients, 28% attended a regular school, 23% a special school, and the remainder had education at home. Although the families carry the main responsibility and the child dependent on respiratory therapy is a financial burden, the authors conclude that "a home program is beneficial to the majority of patients and families."
E d i t o r i a l correspondence
791
More than 80% of Frate's patients have an untreatable condition, and the majority of these have a progressive disease. We regret that the authors did not analyze the stress on patients, family, and community. We have no doubt that the parents or guardians are capable of learning home ventilation, but how do they really cope with this challenge? The loss of privacy, the limited social lives, the disruption of sleeping patterns, and the difficult schedule make a normal family life impossible. The sibling's activities are curtailed, and the parents m a y refuse to have additional children. 2 We do not advocate premature withdrawal of medical support, and the criteria for deciding w h o will live and who will die need continued reevaluation. But is prolonged, hopeless ventilation therapy a good choice? Should we initiate mechanical ventilation in children who have a well-defined, progressive disease with obligate lethal outcome, such as Duchenne muscular dystrophy or spinal muscular atrophy?
Sergio Faneoni, M.D. Intensive Care Unit Eugen Boltshauser, M.D. Department o f Neurology Children's Hospital Zurich, Switzerland REFERENCES
1.
2.
Frates RC, Splaingard ML, Smith EO, Harrison GM. Outcome of home mechanical ventilation in children. J PEDIATR 1985;I 06:850. Burr BH, Guyer B, Todres ID. H o m e care for children on respirators. N Engl J Med 1983;309:1319.
Reply To the Editor: Our Swiss colleagues ask if we ought to initiate or continue mechanical ventilation in children who have either "untreatable conditions" such as high quadriplegia or a progressively fatal disease such as Duchenne muscular dystrophy. We hoped that our report would provide survival data and management recommendations to guide clinicians in deciding whether they could safely provide ventilation therapy in these children outside of traditional hospital settings. Our data show that m a n y "'untreatable" ventilator-dependent children lived at home for years, with about half the eligible children attending school and a few going on to college and even professional school. W e did not perform a detailed analysis of family stresses; however, our data combined with other studies L2 of this.group of patients indicate that many households were able to cope rather efficiently. We agree that very real constraints are placed on families caring for children with respirators at home. However, our impression is that the addition of the ventilator to prolong a child's life is not as disruptive as many physicians might expect for a patient with progressive neuromuscular disease. Families and other caretakers have often already adjusted to the nonambulatory patient's need for a wheelchair and increasing dependency in activities of daily living. The addition of a ventilator for these patients often signifies "another loss," and
II
II.
IIIII II 84
I
Heart block with viral myocarditis
Reply
To the Editor." To the report of Mahoney et al., ~ we would like to add our recent experience with complete heart block caused by myocarditis. Unlike the children they described, our patient had no systemic viral prodrome when she presented with syncope. This 3-year-old girl had been in excellent health, with no history of heart disease. While playing she had a sudden, momentary loss of consciousness. Over the ensuing 3 hours four similar episodes occurred, each of approximately 30 seconds duration. Her pulse was irregular, varying from 50 to 90 bpm, and she was afebrile. An ECG showed third-degree A-V block with an idioventricular rhythm, diffuse ST-T wave changes, left anterior hemiblock, and poor R wave progression. Recurrent lapses of consciousness were associated with several 5- tO 8-second episodes of ventricular asystole, which did not respond to atropine. A temporary transvenous pacemaker (VVI) was inserted, and the patient's condition stabilized. Initial laboratory evaluation, including white blood cell count, erythrocyte sedimentation rate, ereatine kinase, chest radiograph, and echocardiogram yielded normal findings. On the fourth hospital day, cardiac catheterization and endomyocardial biopsy were performed. The intracardiac and coronary artery anatomy were normal. Left ventricular function, enddiastolic pressure, and cardiac output were normal. The endomyocardial biopsy demonstrated moderate inflammatory cell infiltrates, which included lymphocytes, plasma cells, and occasional eosinophils; there were several foci of myocyte necrosis. Because of the histologie findings, prednisone therapy was begun, although intermittent return of A-V conduction had already been demonstrated. Over the next 7 days normal sinus rhythm returned. Two months after diagnosis, the ECG was entirely normal. Viral cultures and titers were nondiagnostic. Mahoney et al. ~ state that a Stokes-Adam attack may be the only clinical manifestation of myocarditis. All of their patients had symptoms of a viral syndrome, however. The diagnosis of myoearditis was substantiated by rising viral titers (two patients) or by elevated cardiac enzymes (one patient). Our report emphasizes that myocarditis can present as sudden onset of complete heart block without evidence of viral infection by history, physical examination, or laboratory studies. Further, this case demonstrates the important diagnostic role of endomyocardial biopsy when the cause of symptomatic complete heart block is in doubt. Burt I. Bromberg, M.D. Robert H. Beekman, M.D. Kathleen Heidelberger, M.D. Departments o f Pediatrics and Pathology C. S. Mott Children's Hospital The University o f Michigan Medical School Ann Arbor, M I 48109
To the Editor: 1 agree that sudden onset of complete heart block can occur without clinical evidence of viral infection. The autopsy studies we referred to substantiate this conclusion. Endomyocardial biopsy is becoming more routine in pediatric cardiology practice, and will be an important tool in the diagnosis of myocarditis. Their case also supports the potentially transient nature of a heart block and syncope and the need for a temporary transvenous pacemaker. Larry T. Mahoney, M.D. Assistant Professor Pediatric Cardiology The University o f lowa Hospitals and Clinics Iowa City, 1,4 52242
REFERENCES 1.
790
Mahoney LT, Marvin WJ Jr, Atkins DL, et al. Pacemaker management for acute onset of heart block in children. J PEDIATR 1985;107:207.
Behavior in girls with true precocious puberty To the Editor: We read with interest the report by Sonis et al. ~ on behavior problems and social competence in girls with true precocious puberty, and wish to comment in light of our experience. In a study of 100 girls with precocious puberty, we gave parents the child behavior checklist (CBCL). 2 The Hebrew version of the CBCL has been found reliable (Kronbaeh alpha value of the scales .78 to .91) and valid in a large population? We were able to obtain data from 47 girls aged 6 to 11 years (mean 8.3 years) with true (central) idiopathic precocious puberty (PPid). All patients with tumors were excluded from this study, in contradistinction to that of Sonis et al. 1The results were compared with those obtained in a group of 20 healthy girls with no evidence of sexual precocity, matched for age and socioeconomic status. The socioeconomic status (Israeli standards) revealed that 37 patients were from middle class families, seven from lower class, and two from upper class families. In contrast to the data reported by Sonis et al., only 7% of the patients had a total behavior problem score between 71 and 100. Furthermore, there was a difference between patients with PPid and the controls on only five of the 12 scales of the CBCL (internalizing total scale, schizoid, delinquent, externalizing, and total behavior problem scales), as compared with a difference on nine of 12 scales for the subjects described by Sonis et al. Our control group, compared with that of Sonis et al., had lower scores (closer to the 50 T score) on 10 to 12 scales, with a significant difference on five (depression, schizoid, sex problems, delinquency, and aggression). When our group with PPid was compared with that of Sonis et al., ours had lower scores on all 12 scales, with significant differences on eight (internalizing, depressed, social withdrawal, somatic, externalizing, sex problems, aggressive, and total). What may be the cause for these differences between our results and those reported by Sonis et al? Environmental factors, such as cultural Or socioeconomic differences, would not seem to be of
Volume 108 Number 5, Part 1
great importance, because the differences between the proband groups in both studies are much greater than those found between the control groups in both studies. Sonis et al. suggested that these differences m a y be related to the fact that their patients are a very select group who have traveled to Bethesda for treatment. O u r own group is more likely to be representative of the entire population, because distance is no major factor in reaching our center. Furthermore, 11 of the 33 patients in the Sonis group had some kind of central nervous system or hypothalamic problem, and this m a y be one reason for the increased incidence of emotional and behavioral problems; our group comprised only patients with PPid. Further studies are needed on the behavioral aspects of PPid and the correlation with hormone activity and age of onset. These may help to delineate more fully the role of sex hormones in this disorder. IQ studies in a group of 52 girls with precocious puberty revealed a significant superiority in verbal IQ scores, which was interpreted as possible evidence of an effect of sex hormones on brain development, particularly on the left hemisphere, during the prepubertal period. 4
A. Galatzer, M.A. Z. Laron, M.D. Institute o f Pediatric and Adolescent Endocrinology Beilinson Medical Center and Sackler School o f Medicine Tel Aviv University Tel Aviv, Israel REFERENCES
1.
2.
3.
4.
Sonis W A , Comite F, Blue J, et al. Behavior problems and social competence in girls with true precocious puberty. J PED~AT~ 1985; 106:156-160. Achenbach T M , Edelbrock CS. Behavior problems and competencies reported by parents of normal and disturbed children aged four through sixteen. Monogr Soc Res Child Dev 1981;44(serial no. 188):1. Offer R. The validity and reliability of the child behavior checklist (CBCL). M.A. Thesis, Department of Psychology, Bar llan University, R a m a t Gan, Israel, 1983. Galatzer A, Beit Halachmi N, Kauli R, Laron Z. Intellectual functions of girls with precocious puberty. Pediatrics 1984;74:246-249.
O u t c o m e of home mechanical
ventilation To the Editor: W e read with interest and concern the article by Frates et al) on home mechanical ventilation. The authors make an interesting cost, mortality, and morbidity analysis, showing that home mechanical ventilation is 16 times cheaper than hospital care, that registered nurses are of no benefit, and that this treatment modality is reasonably safe. A m o n g these patients, 28% attended a regular school, 23% a special school, and the remainder had education at home. Although the families carry the main responsibility and the child dependent on respiratory therapy is a financial burden, the authors conclude that "a home program is beneficial to the majority of patients and families."
E d i t o r i a l correspondence
791
More than 80% of Frate's patients have an untreatable condition, and the majority of these have a progressive disease. We regret that the authors did not analyze the stress on patients, family, and community. We have no doubt that the parents or guardians are capable of learning home ventilation, but how do they really cope with this challenge? The loss of privacy, the limited social lives, the disruption of sleeping patterns, and the difficult schedule make a normal family life impossible. The sibling's activities are curtailed, and the parents m a y refuse to have additional children. 2 We do not advocate premature withdrawal of medical support, and the criteria for deciding w h o will live and who will die need continued reevaluation. But is prolonged, hopeless ventilation therapy a good choice? Should we initiate mechanical ventilation in children who have a well-defined, progressive disease with obligate lethal outcome, such as Duchenne muscular dystrophy or spinal muscular atrophy?
Sergio Faneoni, M.D. Intensive Care Unit Eugen Boltshauser, M.D. Department o f Neurology Children's Hospital Zurich, Switzerland REFERENCES
1.
2.
Frates RC, Splaingard ML, Smith EO, Harrison GM. Outcome of home mechanical ventilation in children. J PEDIATR 1985;I 06:850. Burr BH, Guyer B, Todres ID. H o m e care for children on respirators. N Engl J Med 1983;309:1319.
Reply To the Editor: Our Swiss colleagues ask if we ought to initiate or continue mechanical ventilation in children who have either "untreatable conditions" such as high quadriplegia or a progressively fatal disease such as Duchenne muscular dystrophy. We hoped that our report would provide survival data and management recommendations to guide clinicians in deciding whether they could safely provide ventilation therapy in these children outside of traditional hospital settings. Our data show that m a n y "'untreatable" ventilator-dependent children lived at home for years, with about half the eligible children attending school and a few going on to college and even professional school. W e did not perform a detailed analysis of family stresses; however, our data combined with other studies L2 of this.group of patients indicate that many households were able to cope rather efficiently. We agree that very real constraints are placed on families caring for children with respirators at home. However, our impression is that the addition of the ventilator to prolong a child's life is not as disruptive as many physicians might expect for a patient with progressive neuromuscular disease. Families and other caretakers have often already adjusted to the nonambulatory patient's need for a wheelchair and increasing dependency in activities of daily living. The addition of a ventilator for these patients often signifies "another loss," and