- Email: [email protected]
Improved management of brittle-psychosocial diabetes by use of a portable insulin infusion pump
10 0
Clinical and laboratory observations
The Journal of Pediatrics July 1985
hepatic tissue, which m a y be one reason for an elevated level of C R P , H which is synthesized in liver cells. In addition, any kind of tissue necrosis m a y induce a rapid increase in C R P ) 2 In s u m m a r y , a serum C R P concentration > 4 0 m g / L and W B C count >15.0 • 10~/L were frequently recorded in adenovirus infections. In most cases of influenza virus, parainfluenza virus, and R S V - i n d u c e d illnesses, the C R P level was <40 m g / L and t h e W B C count was <15.0 • 109/L, as is typical for a viral disease.
6.
7.
8.
REFERENCES 1. Hanson LA, Jodal U, Salbel K-G, Wadsworth C: The diagnostic value of C-reactive protein. Pediatr Infect Dis 2:87, 1983. 2. McCarthy PL, Frank AL, Ab[ow RC, Masters S J, Dolaa TF: Value of the C-reactive protein test in the differentiation of bacterial and viral pneumonia. J PEDJATR 92:454, I978. 3. Peltola H: C-reactive protein for rapid monitoring of infections in the central nervous system: Lancet 1:980, 1982. 4. Peltola H: C-reactive protein in rapid differentiation of acute epiglottitis from spasmodic croup and acute laryngotracheitis. J PEDIATR 102:713, 1983. 5. Clarke D, Cost K: Use of serum C-reactive protein in
9.
10. 11.
12.
differentiating septic from aseptic meningitis in children. J PEDIATR 102:718, 1983. Sarkkinen HK, Halonen PE, Arstila PP, Salmi AA: Detection of respiratory syncytial, parainfluenza type 2, and adenovirus antigens by radioimmunoassay and enzyme immnnoassay on nasopharyngeal specimens from children with acute respiratory disease. J Clin Microbiol 13:258, 1981. Ruuskanen O, Sarkkinen H, Meurman O, Hurme P, Rossi T, Halonen P, H~nninen P: Rapid diagnosis of adenoviral tonsillitis: A prospective clinical stddy. J PEDIATR 104:725, 1984. Ruuskanen O, Meurman O, Sarkkinen H: Adenoviral diseases in children, a study of 105 hospital cases. Pediatrics, in press. Griffin DE, Hirsch RL, Johnson RT, de Soriano IL, Roedenbeck S, Vaisberg A: Changes in serum C-reactive protein during complicated and uncomplicated measles virus infections. Infect Immun 41:861, t983. Sa[onen E-M, Vaheri A: C-reactive protein in acute viral infections. J Med Virol 8:161, 1981. Spencer M J, Cherry JD: Adenoviral infections. In Feigin RD, Cherry JD, editors: Textbook of pediatric infectious diseases. Philadelphia, 1981, W.B. Saunders Co, pp 1279-1298. Gewurz H, Mold C, Siegel J, Fiedel B: C-reactive protein and the acute phase responses. Adv Intern Med 27:345, 1982.
Improved management of brittle-psychosocial diabetes by use of a portable insulin infusion pump Richard A. Noto, M.D., Lori Ginsberg, M.S., Fima Lifshitz, M.D., David Pelcovitz, Ph.D., and Sandra Kaplan, M.D. M a n h a s s e t and Valhalla, N e w Y o r k
THE USE of a portable insulin infusion p u m p has been effective during inpatient and a m b u l a t o r y m a n a g e m e n t of ketosis-prone insulin-dependent diabetes mellitus? ,2 Its usefulness in treating brittle diabetes, however, is not universally accepted? -9 W e describe the m a n a g e m e n t of
From the Section of Pediatric Endocrinology, Metabolism and Nutrition, Department of Pediatrics, New York Medical College, and the Division of Pediatric Endocrinology, Metabolism and Nutrition, Department of Pediatrics, North Shore University Hospital, Cornell Medical College. Submitted for publication Nov. 7, 1984; accepted Dee. 19, 1984. Reprint requests: Richard A. Noto, M.D.. Department of Pediatrics, Section of Pediatric Endocrinology, Metabolism and Nutrition, New York Medical College, Sunshine Cottage, Valhalla, N Y 10595.
very unstable brittle diabetes and psychosocial problems in patients who failed to respond to all other therapies but who improved dramatically after P I I P treatment. CASE REPORTS
Patient1. This 1t ~/2-year-old adopted boy had had IDDM for 2 years. Control had been poor, as reflected by elevated glycosylated hemoglobin values, poor growth (0.1 cm in 3 months), 10 I
1DDM PIIP
Insulin-dependent diabetes mellitus Portable insulin infusion pamp
I
hospitalizations, and six separate emergency room visits for treatment of ketoacidosis. Ten episodes of ketoacidosis had occurred in the 4 months before PIIP therapy. He also had a conduct disorder (socialized aggressive) for which he and his parents were receiving weekly psychotherapy. Before PIIP thera-
Volume 107 Number 1
py he was receiving four insulin injections a day (0.82 to 1.15 ~/kg/24 hr) along with home blood glucose monitoring. During the first 10 weeks of PIIP treatment, control of glycemia was excellent, and glycosylated hemoglobin fell from 17% to 10%. No episodes of ketoacidosis were recorded during the first 4 months of pump treatment. He experienced a growth spurt (5 cm in 4 months), the onset of sexual development, and a significantly improved clinical state of well being. During weeks 10 to 35 of PIIP treatment, control was not ideal, but it was improved compared with the pre-PlIP period. Total glycosylated hemoglobin values ranged from 13.8% to 9.8%. Two hospitalizations were required for treatment of ketoacidosis, one secondary to gastroenteritis and dehydration and one after the funeral of his grandmother. After 7 months of PIIP treatment the patient moved out of the state. PIIP treatment was subsequently terminated after 11 months because he wanted to return to conventional therapy. Patient 2. This 15-3/t2-year-old pubertal girl had had IDDM for 9 years. During the last 2 years glycemic control had been poor, as exemplified by nine hospitalizations for treatment of ketoacidosis, elevated glycosylated hemoglobin values, hypertriglyceridemia, and irregular menstruation. During the 4 months before PIIP treatment, she required four hospitalizations and four separate emergency room visits to treat ketoacidosis. Therapy before PIIP management consisted of two insulin injections a day. She also had a conduct disorder (socialized nonaggressive) for which she and her family were receiving psychotherapy. She had partial eighth nerve deafness resulting from congenital rubella. During the first 13 weeks of PIIP treatment, glycemic control was excellent, and her glycosylated hemoglobin level fell from 17.2% to 7.7% (in the normal range). After 13 weeks of treatment control was not ideal, but total glycosylated hemoglobin values were most often lower than those during the pre-PIIP treatment period, ranging between 10.6% and 14.1%. During the 15 months of treatment the patient required only one hospitalization for ketoacidosis, and that illness was caused in part by pump malfunction. After 15 months pump treatment was discontinued. This decision was related in part to a strong desire to attempt conventional treatment and inpart to poor compliance. Both of these patients required a marked increase in insulin dosage during prolonged emotional stress. During a period of behavorial deterioration, patient 1 needed an insulin increase of 49% (1.06 to 1.58 U/kg/day) to achieve adequate control; patient 2, during a difficult period while her parents were getting divorced, required a 42% (1.17 to 1.70 U/kg/day) increase in insulin dosage. The management and follow-up team in the diabetes clinic consists of an endocrinologist, clinical nurse specialist, nutritionist, podiatrist, psychiatrist, and psychologist. Each member of the team meets with the patient and parents at every clinic visit, after which the team discusses the cases, management, and follow-up at a group meeting. Problems with PIIP treatment have included pump failure and runaway (the pump falsely administering all of its insulin), needle site infection, and hypoglycemia. The two patients received treatment by PIIP (Model AS2C; Auto-Syringe, Hooksett, N.H.) as described by Tamborlane et al. 1
Clinical and laboratory observations
10 1
DISCUSSION O u r experience with these two patients has shown t h a t the patient with poorly controlled brittle diabetes and psychosocial problems can achieve stabilization of the illness with the use of a PIIP. In b o t h patients euglycemia was established in the initial 2 m o n t h s of P I I P t r e a t m e n t , but afterward their control was not ideal, although it was significantly better t h a n t h a t a t t a i n e d with conventional therapy. T h e personality disorders of the two patients did not impose barriers to t r e a t m e n t , probably because their psychologic problems did not affect compliance with the t r e a t m e n t regimen. These observations are in contrast to another published report 4 of a patient who used the p u m p to a t t e m p t suicide. No mention is m a d e in t h a t report a b o u t patient selection, frequency of follow-up, a n d psychiatric support; therefore, we c a n n o t assess or explain the failure. T h e improved diabetes control in our two patients is attributed to m a n y factors. Stabilization of the diabetes with P I I P t r e a t m e n t allowed both families to focus attention and energies on patient and family problems and on family therapy r a t h e r t h a n on diabetes m a n a g e m e n t . Once good control was achieved and maintained, psychologic changes ensued t h a t helped support and prolong better management. A n i m p o r t a n t aspect of t r e a t m e n t in these patients was the aggressive approach to the a m b u l a t o r y t r e a t m e n t of hyperglycemia with the pump. A t the time of hyperglycemia, increased insulin dosage up to 0.1 U / k g / h r , freq u e n t monitoring of blood sugar and of urine sugar a n d acetone, a n d oral hydration were used to restore euglycemia. W e believe t h a t this approach averted m a n y episodes of ketoacidosis and subsequent emergency hospitalizations. A n interesting finding in these cases was the enormous insulin dosage required to m a i n t a i n a d e q u a t e control during emotional stress. After resolution of the crisis, the insulin requirements diminished. These findings indicate t h a t successful diabetes m a n a g e m e n t in such patients must include very close metabolic surveillance, frequent followup, and a family t h a t is c o m m i t t e d to P I I P t r e a t m e n t and is capable of insulin dosage regulation. W e have used a P I I P in the therapy of three other adolescents with diabetes. Two h a d very brittle disease, and one had neuropathy. All are doing well with P I I P treatment. W e have no other such patients with psychosocial problems. W e believe t h a t P I I P t r e a t m e n t can be feasible for stabilization of the pediatric and adolescent patient with brittle diabetes a n d psychosocial problems who is compliant with t r e a t m e n t and receives it at a center t h a t offers an intensive t e a m approach to m a n a g e m e n t .
102
Clinical and laboratory observations
The Journal of Pediatrics July 1985
REFERENCES 1. Tamborlane WV, Sherwin RS, Genel M, Felig P: Outpatient treatment of juvenile-onset diabetes with a preprogrammed portable subcutaneous insulin infusion system. Am J Med 68:190, 1980. 2. Mecklenburg RS, Benson JW, Becker NM, Brazel LP, Fredland PN, Metz R J, Nielsen RL, Sannac CA, Steenrod W J: Clinical use of the insulin infusion pump in 100 patients with Type [ diabetes. N Engl J Med 307:513, 1982. 3. Minuchin S, Posman BL, Baker L, et al: A conceptual model of psychosomatic illness in children. Arch Gen Psychiatry 32:1031, 1975. 4. Riley W J, Silverstein JH, Rosenbloom AL, Spillar R, McCallum MH: Ambulatory diabetes management with pulsed subcutaneous insulin using a portable pump. Clin Pediatr 19:609, 1980. 5. Barbosa J, Menth L, Eaton J, Sutherland D, Freier EF,
6.
7.
8.
9.
Najarian J: Long-term, ambulatory subcutaneous insulin infusion versus multiple daily injections in brittle diabetic patients. Diabetes Care 4:269, 1981. Keen H, Pickup JC, Viberti GC, Bilous R, Williams G: Aspects of continuous subcutaneous insulin infusion in diabetes. Diabetes Care 4:54, 1981. Selam JL, Slingeneyer A, Hedon B, Mares P, Beraud J J, Mirouze J: Long-term ambulatory peritoneal insulin infusion of brittle diabetes with portable pumps: Comparison with intravenous and subcutaneous route. Diabetes Care 6:105, 1983. Nathan DM: Successful treatment of extremely brittle, insulin-dependent diabetes with a novel-subcutaneous insulin pump regimen. Diabetes Care 5:105, 1982. Schiffrin A, Desroslers M, Moffatt M, Belmonte MM: Feasibility of strict diabetes control in insulin-dependent diabetic adolescents. J PEDIATR 103:522, 1983.
Clinical differences in chronic granulomatous disease in patients with cytochrome b-negative or cytochrome b-positive neutrophils Ron S. Weening, M.D., Ph.D.,* Lesley H. Adriaansz, Corry M. R. Weemaes, M.D., Ph.D., Ren~ Lutter, and Dirk Roos, Ph.D. Amsterdam
and Nijmegen, The Netherlands
THE HIGH SUSCEPTIBILITY tO infection in patients with chronic granulomatous disease is caused by the inability of their phagocytes (neutrophils, monocytes, macrophages, and eosinophils) to kill ingested microorganisms; C G D phagocytes fail to generate bactericidal oxygen products (e.g., hydrogen peroxide) during phagocytosis, t-3 At least two modes of inheritance have been recognized in CGD: an X-linked form and an autosomal recessive form. 3,4 Segal et al? reported a close correlation between the lack of an optical cytochrome b signal and the X-linked inheritance of CGD. They suggested that patients with the X-linked
From the Central Laboratory of The Netherlands Red Cross Blood Transfusion Service, incorporating the Laboratory of Experimental and Clinical Immunology, University of Amsterdam; the Department of Pediatrics, Academic Medical Center, Amsterdam; and the Department of Pediatrics, Sint Radboud Hospital, University of Nijmegen. Submitted for publication June 20, 1984; accepted Dec. 11, 1984. Reprints not available. *Any clinician who has patients with CGD under his or her care who wishes to join The International Study Group of CGD may obtain detailed information from Dr. R: S. Weening, P.O. Box 9190, 1006 AD Amsterdam. The Netherlands.
form of C G D lack cytochrome b in their phagocytes, whereas in autosomal recessive C G D this cytochrome is present but may be nonfunctional. Clinical differences between patients with the X-linked C G D and those with the autosomal recessive form have not been reported, but it has been suggested that the autosomal form leads to less severe symptoms than the X-linked form. z,3 We retrospectively compared the clinical data in seven patients with X-linked C G D and cytochrome b I
CGD
Chronic granulomatous disease
]
negative neutrophils with that in five patients with the autosomal cytochrome b-positive form of CGD.
METHODS Patients were considered to have C G D if (1) the clinical history was characteristic of CGD, j-3 (2) isolated neutrophils failed to kill Staphylococcus aureus intracellularly, 6 and (3) activation of the neutrophils with serum-treated zymosan particles or phorbol myristate acetate did not result in increased oxygen consumption 6 or hydrogen peroxide production. 6 Cytochrome b was measured in suspensions of isolated neutrophils as the sodium di-
Clinical and laboratory observations
The Journal of Pediatrics July 1985
hepatic tissue, which m a y be one reason for an elevated level of C R P , H which is synthesized in liver cells. In addition, any kind of tissue necrosis m a y induce a rapid increase in C R P ) 2 In s u m m a r y , a serum C R P concentration > 4 0 m g / L and W B C count >15.0 • 10~/L were frequently recorded in adenovirus infections. In most cases of influenza virus, parainfluenza virus, and R S V - i n d u c e d illnesses, the C R P level was <40 m g / L and t h e W B C count was <15.0 • 109/L, as is typical for a viral disease.
6.
7.
8.
REFERENCES 1. Hanson LA, Jodal U, Salbel K-G, Wadsworth C: The diagnostic value of C-reactive protein. Pediatr Infect Dis 2:87, 1983. 2. McCarthy PL, Frank AL, Ab[ow RC, Masters S J, Dolaa TF: Value of the C-reactive protein test in the differentiation of bacterial and viral pneumonia. J PEDJATR 92:454, I978. 3. Peltola H: C-reactive protein for rapid monitoring of infections in the central nervous system: Lancet 1:980, 1982. 4. Peltola H: C-reactive protein in rapid differentiation of acute epiglottitis from spasmodic croup and acute laryngotracheitis. J PEDIATR 102:713, 1983. 5. Clarke D, Cost K: Use of serum C-reactive protein in
9.
10. 11.
12.
differentiating septic from aseptic meningitis in children. J PEDIATR 102:718, 1983. Sarkkinen HK, Halonen PE, Arstila PP, Salmi AA: Detection of respiratory syncytial, parainfluenza type 2, and adenovirus antigens by radioimmunoassay and enzyme immnnoassay on nasopharyngeal specimens from children with acute respiratory disease. J Clin Microbiol 13:258, 1981. Ruuskanen O, Sarkkinen H, Meurman O, Hurme P, Rossi T, Halonen P, H~nninen P: Rapid diagnosis of adenoviral tonsillitis: A prospective clinical stddy. J PEDIATR 104:725, 1984. Ruuskanen O, Meurman O, Sarkkinen H: Adenoviral diseases in children, a study of 105 hospital cases. Pediatrics, in press. Griffin DE, Hirsch RL, Johnson RT, de Soriano IL, Roedenbeck S, Vaisberg A: Changes in serum C-reactive protein during complicated and uncomplicated measles virus infections. Infect Immun 41:861, t983. Sa[onen E-M, Vaheri A: C-reactive protein in acute viral infections. J Med Virol 8:161, 1981. Spencer M J, Cherry JD: Adenoviral infections. In Feigin RD, Cherry JD, editors: Textbook of pediatric infectious diseases. Philadelphia, 1981, W.B. Saunders Co, pp 1279-1298. Gewurz H, Mold C, Siegel J, Fiedel B: C-reactive protein and the acute phase responses. Adv Intern Med 27:345, 1982.
Improved management of brittle-psychosocial diabetes by use of a portable insulin infusion pump Richard A. Noto, M.D., Lori Ginsberg, M.S., Fima Lifshitz, M.D., David Pelcovitz, Ph.D., and Sandra Kaplan, M.D. M a n h a s s e t and Valhalla, N e w Y o r k
THE USE of a portable insulin infusion p u m p has been effective during inpatient and a m b u l a t o r y m a n a g e m e n t of ketosis-prone insulin-dependent diabetes mellitus? ,2 Its usefulness in treating brittle diabetes, however, is not universally accepted? -9 W e describe the m a n a g e m e n t of
From the Section of Pediatric Endocrinology, Metabolism and Nutrition, Department of Pediatrics, New York Medical College, and the Division of Pediatric Endocrinology, Metabolism and Nutrition, Department of Pediatrics, North Shore University Hospital, Cornell Medical College. Submitted for publication Nov. 7, 1984; accepted Dee. 19, 1984. Reprint requests: Richard A. Noto, M.D.. Department of Pediatrics, Section of Pediatric Endocrinology, Metabolism and Nutrition, New York Medical College, Sunshine Cottage, Valhalla, N Y 10595.
very unstable brittle diabetes and psychosocial problems in patients who failed to respond to all other therapies but who improved dramatically after P I I P treatment. CASE REPORTS
Patient1. This 1t ~/2-year-old adopted boy had had IDDM for 2 years. Control had been poor, as reflected by elevated glycosylated hemoglobin values, poor growth (0.1 cm in 3 months), 10 I
1DDM PIIP
Insulin-dependent diabetes mellitus Portable insulin infusion pamp
I
hospitalizations, and six separate emergency room visits for treatment of ketoacidosis. Ten episodes of ketoacidosis had occurred in the 4 months before PIIP therapy. He also had a conduct disorder (socialized aggressive) for which he and his parents were receiving weekly psychotherapy. Before PIIP thera-
Volume 107 Number 1
py he was receiving four insulin injections a day (0.82 to 1.15 ~/kg/24 hr) along with home blood glucose monitoring. During the first 10 weeks of PIIP treatment, control of glycemia was excellent, and glycosylated hemoglobin fell from 17% to 10%. No episodes of ketoacidosis were recorded during the first 4 months of pump treatment. He experienced a growth spurt (5 cm in 4 months), the onset of sexual development, and a significantly improved clinical state of well being. During weeks 10 to 35 of PIIP treatment, control was not ideal, but it was improved compared with the pre-PlIP period. Total glycosylated hemoglobin values ranged from 13.8% to 9.8%. Two hospitalizations were required for treatment of ketoacidosis, one secondary to gastroenteritis and dehydration and one after the funeral of his grandmother. After 7 months of PIIP treatment the patient moved out of the state. PIIP treatment was subsequently terminated after 11 months because he wanted to return to conventional therapy. Patient 2. This 15-3/t2-year-old pubertal girl had had IDDM for 9 years. During the last 2 years glycemic control had been poor, as exemplified by nine hospitalizations for treatment of ketoacidosis, elevated glycosylated hemoglobin values, hypertriglyceridemia, and irregular menstruation. During the 4 months before PIIP treatment, she required four hospitalizations and four separate emergency room visits to treat ketoacidosis. Therapy before PIIP management consisted of two insulin injections a day. She also had a conduct disorder (socialized nonaggressive) for which she and her family were receiving psychotherapy. She had partial eighth nerve deafness resulting from congenital rubella. During the first 13 weeks of PIIP treatment, glycemic control was excellent, and her glycosylated hemoglobin level fell from 17.2% to 7.7% (in the normal range). After 13 weeks of treatment control was not ideal, but total glycosylated hemoglobin values were most often lower than those during the pre-PIIP treatment period, ranging between 10.6% and 14.1%. During the 15 months of treatment the patient required only one hospitalization for ketoacidosis, and that illness was caused in part by pump malfunction. After 15 months pump treatment was discontinued. This decision was related in part to a strong desire to attempt conventional treatment and inpart to poor compliance. Both of these patients required a marked increase in insulin dosage during prolonged emotional stress. During a period of behavorial deterioration, patient 1 needed an insulin increase of 49% (1.06 to 1.58 U/kg/day) to achieve adequate control; patient 2, during a difficult period while her parents were getting divorced, required a 42% (1.17 to 1.70 U/kg/day) increase in insulin dosage. The management and follow-up team in the diabetes clinic consists of an endocrinologist, clinical nurse specialist, nutritionist, podiatrist, psychiatrist, and psychologist. Each member of the team meets with the patient and parents at every clinic visit, after which the team discusses the cases, management, and follow-up at a group meeting. Problems with PIIP treatment have included pump failure and runaway (the pump falsely administering all of its insulin), needle site infection, and hypoglycemia. The two patients received treatment by PIIP (Model AS2C; Auto-Syringe, Hooksett, N.H.) as described by Tamborlane et al. 1
Clinical and laboratory observations
10 1
DISCUSSION O u r experience with these two patients has shown t h a t the patient with poorly controlled brittle diabetes and psychosocial problems can achieve stabilization of the illness with the use of a PIIP. In b o t h patients euglycemia was established in the initial 2 m o n t h s of P I I P t r e a t m e n t , but afterward their control was not ideal, although it was significantly better t h a n t h a t a t t a i n e d with conventional therapy. T h e personality disorders of the two patients did not impose barriers to t r e a t m e n t , probably because their psychologic problems did not affect compliance with the t r e a t m e n t regimen. These observations are in contrast to another published report 4 of a patient who used the p u m p to a t t e m p t suicide. No mention is m a d e in t h a t report a b o u t patient selection, frequency of follow-up, a n d psychiatric support; therefore, we c a n n o t assess or explain the failure. T h e improved diabetes control in our two patients is attributed to m a n y factors. Stabilization of the diabetes with P I I P t r e a t m e n t allowed both families to focus attention and energies on patient and family problems and on family therapy r a t h e r t h a n on diabetes m a n a g e m e n t . Once good control was achieved and maintained, psychologic changes ensued t h a t helped support and prolong better management. A n i m p o r t a n t aspect of t r e a t m e n t in these patients was the aggressive approach to the a m b u l a t o r y t r e a t m e n t of hyperglycemia with the pump. A t the time of hyperglycemia, increased insulin dosage up to 0.1 U / k g / h r , freq u e n t monitoring of blood sugar and of urine sugar a n d acetone, a n d oral hydration were used to restore euglycemia. W e believe t h a t this approach averted m a n y episodes of ketoacidosis and subsequent emergency hospitalizations. A n interesting finding in these cases was the enormous insulin dosage required to m a i n t a i n a d e q u a t e control during emotional stress. After resolution of the crisis, the insulin requirements diminished. These findings indicate t h a t successful diabetes m a n a g e m e n t in such patients must include very close metabolic surveillance, frequent followup, and a family t h a t is c o m m i t t e d to P I I P t r e a t m e n t and is capable of insulin dosage regulation. W e have used a P I I P in the therapy of three other adolescents with diabetes. Two h a d very brittle disease, and one had neuropathy. All are doing well with P I I P treatment. W e have no other such patients with psychosocial problems. W e believe t h a t P I I P t r e a t m e n t can be feasible for stabilization of the pediatric and adolescent patient with brittle diabetes a n d psychosocial problems who is compliant with t r e a t m e n t and receives it at a center t h a t offers an intensive t e a m approach to m a n a g e m e n t .
102
Clinical and laboratory observations
The Journal of Pediatrics July 1985
REFERENCES 1. Tamborlane WV, Sherwin RS, Genel M, Felig P: Outpatient treatment of juvenile-onset diabetes with a preprogrammed portable subcutaneous insulin infusion system. Am J Med 68:190, 1980. 2. Mecklenburg RS, Benson JW, Becker NM, Brazel LP, Fredland PN, Metz R J, Nielsen RL, Sannac CA, Steenrod W J: Clinical use of the insulin infusion pump in 100 patients with Type [ diabetes. N Engl J Med 307:513, 1982. 3. Minuchin S, Posman BL, Baker L, et al: A conceptual model of psychosomatic illness in children. Arch Gen Psychiatry 32:1031, 1975. 4. Riley W J, Silverstein JH, Rosenbloom AL, Spillar R, McCallum MH: Ambulatory diabetes management with pulsed subcutaneous insulin using a portable pump. Clin Pediatr 19:609, 1980. 5. Barbosa J, Menth L, Eaton J, Sutherland D, Freier EF,
6.
7.
8.
9.
Najarian J: Long-term, ambulatory subcutaneous insulin infusion versus multiple daily injections in brittle diabetic patients. Diabetes Care 4:269, 1981. Keen H, Pickup JC, Viberti GC, Bilous R, Williams G: Aspects of continuous subcutaneous insulin infusion in diabetes. Diabetes Care 4:54, 1981. Selam JL, Slingeneyer A, Hedon B, Mares P, Beraud J J, Mirouze J: Long-term ambulatory peritoneal insulin infusion of brittle diabetes with portable pumps: Comparison with intravenous and subcutaneous route. Diabetes Care 6:105, 1983. Nathan DM: Successful treatment of extremely brittle, insulin-dependent diabetes with a novel-subcutaneous insulin pump regimen. Diabetes Care 5:105, 1982. Schiffrin A, Desroslers M, Moffatt M, Belmonte MM: Feasibility of strict diabetes control in insulin-dependent diabetic adolescents. J PEDIATR 103:522, 1983.
Clinical differences in chronic granulomatous disease in patients with cytochrome b-negative or cytochrome b-positive neutrophils Ron S. Weening, M.D., Ph.D.,* Lesley H. Adriaansz, Corry M. R. Weemaes, M.D., Ph.D., Ren~ Lutter, and Dirk Roos, Ph.D. Amsterdam
and Nijmegen, The Netherlands
THE HIGH SUSCEPTIBILITY tO infection in patients with chronic granulomatous disease is caused by the inability of their phagocytes (neutrophils, monocytes, macrophages, and eosinophils) to kill ingested microorganisms; C G D phagocytes fail to generate bactericidal oxygen products (e.g., hydrogen peroxide) during phagocytosis, t-3 At least two modes of inheritance have been recognized in CGD: an X-linked form and an autosomal recessive form. 3,4 Segal et al? reported a close correlation between the lack of an optical cytochrome b signal and the X-linked inheritance of CGD. They suggested that patients with the X-linked
From the Central Laboratory of The Netherlands Red Cross Blood Transfusion Service, incorporating the Laboratory of Experimental and Clinical Immunology, University of Amsterdam; the Department of Pediatrics, Academic Medical Center, Amsterdam; and the Department of Pediatrics, Sint Radboud Hospital, University of Nijmegen. Submitted for publication June 20, 1984; accepted Dec. 11, 1984. Reprints not available. *Any clinician who has patients with CGD under his or her care who wishes to join The International Study Group of CGD may obtain detailed information from Dr. R: S. Weening, P.O. Box 9190, 1006 AD Amsterdam. The Netherlands.
form of C G D lack cytochrome b in their phagocytes, whereas in autosomal recessive C G D this cytochrome is present but may be nonfunctional. Clinical differences between patients with the X-linked C G D and those with the autosomal recessive form have not been reported, but it has been suggested that the autosomal form leads to less severe symptoms than the X-linked form. z,3 We retrospectively compared the clinical data in seven patients with X-linked C G D and cytochrome b I
CGD
Chronic granulomatous disease
]
negative neutrophils with that in five patients with the autosomal cytochrome b-positive form of CGD.
METHODS Patients were considered to have C G D if (1) the clinical history was characteristic of CGD, j-3 (2) isolated neutrophils failed to kill Staphylococcus aureus intracellularly, 6 and (3) activation of the neutrophils with serum-treated zymosan particles or phorbol myristate acetate did not result in increased oxygen consumption 6 or hydrogen peroxide production. 6 Cytochrome b was measured in suspensions of isolated neutrophils as the sodium di-