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Short-term hemodialysis for ethylene glycol poisoning
Volume 109 Number 4
Acute chest syndrome in sickle cell disease To the Editor: Poncz et al. 1 recently reported a large number of children with acute chest syndrome (ACS) in sickle cell anemia with emphasis on possible etiologic factors, and suggested various therapeutic approaches. Transfusions were required more frequently for bacterial pneumonia. We have had a very unusual patient with recurrent severe ACS that responded only to initiation and maintenance of a hypertransfusion program. A 15-year-old black girl with a confirmed HbSS disease, was referred to us in 1983 for help in management of severe chest pain and respiratory distress. She had been hospitalized more than 40 times because of pain crisis and pneumonia. She had received pneumococcal vaccine, and was receiving penicillin prophylaxis. No definitive documentation of bacterial, viral, or Mycoplasma infection had ever been possible during the previous or the present hospitalizations. Bilateral extensive pulmonary infiltrates with sterile pleural effusion and negative cultures, associated with severe respiratory distress that required ventilatory support, prompted us to initiate a partial exchange transfusion to increase the HbA level rapidly. She had a quick, obvious decrease in respiratory distress and gradual disappearance of infiltrates. A hypertransfusion program was started, with monthly phlebotomy and partial exchange transfusions, no acute episodes of respiratory distress developed for 8 months. After extensive discussion among the hematology, pediatric, and pulmonary physicians and the family, the transfusion program was discontinued. A month later the patient was readmitted with severe respiratory distress and extensive bilateral pulmonary infiltrates, which again responded to a partial exchange transfusion. She is again receiving the hypertransfusion regimen with iron chelation therapy, and has had no further episodes. We have been unable to find any references regarding recurrent severe ACS, its response to transfusions, and the role, if any, for a hypertransfusion program. R. P. Warrier, M.B.B.S., M.D., D.C.H. Rafael Ducos, M.D. Lolie C. Yu, M.D. Louisiana State University Medical School Children's Hospital of New Orleans 200 Henry Clay Ave. New Orleans. LA 70118 REFERENCES
1. Poncz M, Kane E, Gill FM. Acute chest syndrome in sickle cell disease: etiology and clinical correlates. J PEDIATR 1985;107:861-6.
Reply To the Editor." We have had a similar patient who had recurrent severe acute chest syndrome necessitating more than 10 admissions in less than
E d i t o r i a l correspondence
73 1
a year. To interrupt this cycle of recurrent ACS in this 3-year-old boy, we transfused packed red cells for 6 months, maintaining hemoglobin S level at <30%. This patient had an apparent excellent response to thi s short course of transfusions, having had only two episodes of ACS in the 3 years since the transfusions. Our patient did not become transfusion dependent, which may reflect his younger age. In recurrent ACS, it is important to be certain that the patient does not have another underlying disease such as asthma or cystic fibrosis. Certainly a controlled prospective trial of red cell transfusions in these patients with recurrent ACS would be of value in understanding better how to manage the rare sickle cell patients with this perplexing problem. Mortimer Poncz, M.D. Frances M. Gill, M.D. The Children's Hospital o f Philadelphia 34th and Civic Center Blvd. Philadelphia, PA 19104
Short-term hemodialysis for ethylene glycol poisoning To the Editor." We read with interest the article by Rothman et al. 1 concerning the use of early hemodialysis in an infant with acute ethylene glycol poisoning. We have successfully applied this therapy in a 2-year-old girl (body weight 12.5 kg) who had accidentally ingested 30 gm (2.4 gm/kg) of a related toxic agent, diethylene glycol, in combination with 0.7 gm chlorokresol. She was admitted 1 hour after the accident. Although analysis of blood yielded high serum levels of ethytene glycol (37 mg/dl), the main metabolite of diethylene glycol, and chlorokresol (0.27 mg/dl) the patients clinical condition was initially normal. After 3 hours encephalopathy (somnolence, decrease of EEG activity), bepatopathy (increased transaminase activity), nephropathy (increased serum urea and creatinine, erythrocyturia with tubular casts), and metabolic acidosis developed. Immediate therapy included induced vomiting, enforced diuresis, and infusion of ethanol (0.1 gm/kg/hr). Hemodialysis was performed after 7 hours through a Shaldon catheter (right femoral vein) using a capillary dialyzer (AM 06, Ashai, Tokyo; effective surface area 0.6 m 2, priming volume 55 ml). The dialysate contained sodium bicarbonate. The hemodialysis was continued for 4 hours at a mean blood flow rate of 35 ml/min and resulted in a decrease of the ethylene glycol level from 37 to 10 mg/dl. The ethylene glycol level again rose to 12 mg/dl, but after a second hemodialysis 10 hours later dropped below 0.5 mg/dl. The mean clearance of ethylene glycol by hemodialysis was 2.1 ml/min • kg. The serum level of chlorokresol was 0.84 mg/dl after 14 hours, which showed that this substance is not dialysable. After 5 days all clinical and biochemical observations were normal. We thus confirm the conclusion of Rothman that early short-term hemodialysis represents an cffec-
732
Editorial correspondence
The Journal of Pediatrics October 1986
tive method for removing ethylene glycol and related drugs in young children before renal failure occurs.
Amelia Younossi-Hartenstein, M.D. Bernhard Roth, M.D. Children's Hospital Roll lffiand, Ph.D. Guido Sticht, Ph.D. lnstitue of Forensic Medicine University of Cologne D-5000 Cologne, West Germany
into account maternal age, the most widely recognized predisposing factor in the occurrence of Down syndrome. In these studies the incidence of Down syndrome in relatives of patients with Alzheimer disease was calculated and compared with the incidence in the general population. The studies did not determine if the distribution of maternal ages in their study populations was comparable with that of the "general" population. Although the possibility of a relationship between Alzheimer disease and an increased risk of Down syndrome in families is provocative and warrants additional research, the available information is not sufficient to support such an association.
REFERENCES
William G. Wilson, M.D. Division of Medical Genetics Department of Pediatrics University of Virginia Medical Center Charlottesville, VA 22908
1. Rothman A, Normann SA, Manoguerra AS, Bastian JF, Griswold WR. Short-term bemodialysis in childhood ethylene glycol poisoning. J PEDIATR 1986;108:153-5.
REFERENCES
Down syndrome and Alzheimer disease
l.
To the Editor." In his review of the relationship between Alzheimer disease and Down syndrome, t Dr. Fishman states that the risk of Down syndrome in relatives of Alzheimer disease probands "is approximately two and one-half times that compared in control populations." The studies cited to support this statement2'3 did not take
Fishman MA. Will the study of Down syndrome solve the riddle of Alzheimer disease? J P~DtATg 1986;108:627-9. Heston LL. Alzheimer's dementia and Down's syndrome: genetic evidence suggesting an association. Ann NY Acad Sci 1982;396:29-37. Heyman A, Wilkinson WE, Hurwitz BJ, et al. Alzheimer's disease: genetic aspects and associated clinical disorders. Ann Neurol 1983;14:507-15.
CORRECTION In the article by Hass et al. in the August issue of the Journal (J PEDIATR 1986;109:265-9), the Figure legend should read as follows: Distribution of plasma endotoxin levels in immunocompromised children during febrile episodes and after recovery, and in normal children. Column 1 shows all values obtained from febrile patients; dark circles, endotoxin determinations in which simultaneous blood culture yielded pathogen or in which bacterial infection was clinically evident. Column 2 shows highest endotoxin level obtained for each febrile episode; dark diamonds, highest level from those episodes in which cause for fever was identified. These levels do not necessarily coincide with blood culture(s) that yielded pathogen. One picogram of endotoxin standard used is equivalent to 0.0029 EU USP reference standard endotoxin (E. coil 0113:H 10:K negative).
Acute chest syndrome in sickle cell disease To the Editor: Poncz et al. 1 recently reported a large number of children with acute chest syndrome (ACS) in sickle cell anemia with emphasis on possible etiologic factors, and suggested various therapeutic approaches. Transfusions were required more frequently for bacterial pneumonia. We have had a very unusual patient with recurrent severe ACS that responded only to initiation and maintenance of a hypertransfusion program. A 15-year-old black girl with a confirmed HbSS disease, was referred to us in 1983 for help in management of severe chest pain and respiratory distress. She had been hospitalized more than 40 times because of pain crisis and pneumonia. She had received pneumococcal vaccine, and was receiving penicillin prophylaxis. No definitive documentation of bacterial, viral, or Mycoplasma infection had ever been possible during the previous or the present hospitalizations. Bilateral extensive pulmonary infiltrates with sterile pleural effusion and negative cultures, associated with severe respiratory distress that required ventilatory support, prompted us to initiate a partial exchange transfusion to increase the HbA level rapidly. She had a quick, obvious decrease in respiratory distress and gradual disappearance of infiltrates. A hypertransfusion program was started, with monthly phlebotomy and partial exchange transfusions, no acute episodes of respiratory distress developed for 8 months. After extensive discussion among the hematology, pediatric, and pulmonary physicians and the family, the transfusion program was discontinued. A month later the patient was readmitted with severe respiratory distress and extensive bilateral pulmonary infiltrates, which again responded to a partial exchange transfusion. She is again receiving the hypertransfusion regimen with iron chelation therapy, and has had no further episodes. We have been unable to find any references regarding recurrent severe ACS, its response to transfusions, and the role, if any, for a hypertransfusion program. R. P. Warrier, M.B.B.S., M.D., D.C.H. Rafael Ducos, M.D. Lolie C. Yu, M.D. Louisiana State University Medical School Children's Hospital of New Orleans 200 Henry Clay Ave. New Orleans. LA 70118 REFERENCES
1. Poncz M, Kane E, Gill FM. Acute chest syndrome in sickle cell disease: etiology and clinical correlates. J PEDIATR 1985;107:861-6.
Reply To the Editor." We have had a similar patient who had recurrent severe acute chest syndrome necessitating more than 10 admissions in less than
E d i t o r i a l correspondence
73 1
a year. To interrupt this cycle of recurrent ACS in this 3-year-old boy, we transfused packed red cells for 6 months, maintaining hemoglobin S level at <30%. This patient had an apparent excellent response to thi s short course of transfusions, having had only two episodes of ACS in the 3 years since the transfusions. Our patient did not become transfusion dependent, which may reflect his younger age. In recurrent ACS, it is important to be certain that the patient does not have another underlying disease such as asthma or cystic fibrosis. Certainly a controlled prospective trial of red cell transfusions in these patients with recurrent ACS would be of value in understanding better how to manage the rare sickle cell patients with this perplexing problem. Mortimer Poncz, M.D. Frances M. Gill, M.D. The Children's Hospital o f Philadelphia 34th and Civic Center Blvd. Philadelphia, PA 19104
Short-term hemodialysis for ethylene glycol poisoning To the Editor." We read with interest the article by Rothman et al. 1 concerning the use of early hemodialysis in an infant with acute ethylene glycol poisoning. We have successfully applied this therapy in a 2-year-old girl (body weight 12.5 kg) who had accidentally ingested 30 gm (2.4 gm/kg) of a related toxic agent, diethylene glycol, in combination with 0.7 gm chlorokresol. She was admitted 1 hour after the accident. Although analysis of blood yielded high serum levels of ethytene glycol (37 mg/dl), the main metabolite of diethylene glycol, and chlorokresol (0.27 mg/dl) the patients clinical condition was initially normal. After 3 hours encephalopathy (somnolence, decrease of EEG activity), bepatopathy (increased transaminase activity), nephropathy (increased serum urea and creatinine, erythrocyturia with tubular casts), and metabolic acidosis developed. Immediate therapy included induced vomiting, enforced diuresis, and infusion of ethanol (0.1 gm/kg/hr). Hemodialysis was performed after 7 hours through a Shaldon catheter (right femoral vein) using a capillary dialyzer (AM 06, Ashai, Tokyo; effective surface area 0.6 m 2, priming volume 55 ml). The dialysate contained sodium bicarbonate. The hemodialysis was continued for 4 hours at a mean blood flow rate of 35 ml/min and resulted in a decrease of the ethylene glycol level from 37 to 10 mg/dl. The ethylene glycol level again rose to 12 mg/dl, but after a second hemodialysis 10 hours later dropped below 0.5 mg/dl. The mean clearance of ethylene glycol by hemodialysis was 2.1 ml/min • kg. The serum level of chlorokresol was 0.84 mg/dl after 14 hours, which showed that this substance is not dialysable. After 5 days all clinical and biochemical observations were normal. We thus confirm the conclusion of Rothman that early short-term hemodialysis represents an cffec-
732
Editorial correspondence
The Journal of Pediatrics October 1986
tive method for removing ethylene glycol and related drugs in young children before renal failure occurs.
Amelia Younossi-Hartenstein, M.D. Bernhard Roth, M.D. Children's Hospital Roll lffiand, Ph.D. Guido Sticht, Ph.D. lnstitue of Forensic Medicine University of Cologne D-5000 Cologne, West Germany
into account maternal age, the most widely recognized predisposing factor in the occurrence of Down syndrome. In these studies the incidence of Down syndrome in relatives of patients with Alzheimer disease was calculated and compared with the incidence in the general population. The studies did not determine if the distribution of maternal ages in their study populations was comparable with that of the "general" population. Although the possibility of a relationship between Alzheimer disease and an increased risk of Down syndrome in families is provocative and warrants additional research, the available information is not sufficient to support such an association.
REFERENCES
William G. Wilson, M.D. Division of Medical Genetics Department of Pediatrics University of Virginia Medical Center Charlottesville, VA 22908
1. Rothman A, Normann SA, Manoguerra AS, Bastian JF, Griswold WR. Short-term bemodialysis in childhood ethylene glycol poisoning. J PEDIATR 1986;108:153-5.
REFERENCES
Down syndrome and Alzheimer disease
l.
To the Editor." In his review of the relationship between Alzheimer disease and Down syndrome, t Dr. Fishman states that the risk of Down syndrome in relatives of Alzheimer disease probands "is approximately two and one-half times that compared in control populations." The studies cited to support this statement2'3 did not take
Fishman MA. Will the study of Down syndrome solve the riddle of Alzheimer disease? J P~DtATg 1986;108:627-9. Heston LL. Alzheimer's dementia and Down's syndrome: genetic evidence suggesting an association. Ann NY Acad Sci 1982;396:29-37. Heyman A, Wilkinson WE, Hurwitz BJ, et al. Alzheimer's disease: genetic aspects and associated clinical disorders. Ann Neurol 1983;14:507-15.
CORRECTION In the article by Hass et al. in the August issue of the Journal (J PEDIATR 1986;109:265-9), the Figure legend should read as follows: Distribution of plasma endotoxin levels in immunocompromised children during febrile episodes and after recovery, and in normal children. Column 1 shows all values obtained from febrile patients; dark circles, endotoxin determinations in which simultaneous blood culture yielded pathogen or in which bacterial infection was clinically evident. Column 2 shows highest endotoxin level obtained for each febrile episode; dark diamonds, highest level from those episodes in which cause for fever was identified. These levels do not necessarily coincide with blood culture(s) that yielded pathogen. One picogram of endotoxin standard used is equivalent to 0.0029 EU USP reference standard endotoxin (E. coil 0113:H 10:K negative).