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Risk of SIDS in siblings
EDITORIAL CORRESPONDENCE
Glycogenosis type Ib and periodontitis To the Editor." Periodontitis in children may occur as a sole manifestation without underlying diseases or as a part of the manifestations of systemic diseases such as Papillon-Lef6vre syndrome, hypophosphatasia, cyclic neutropenia, Che~liak-Higashi syndrome and Mac 1/LFA 1 deficiency syndrome. L2 Thus there is an association between periodontitis and quantitative or qualitative disorders of leukocytes, particularly impaired neutrophil chemotaxis. 3 Glycogenosis type Ib is often associated with neutropenia, and impaired neutrophil chemotaxis and bacteriocidal activity? .5 Our 4-yearold patient with glycogenosis type Ib does not have periodontitis, but we have two older patients with impaired neutrophil chemotaxis (Chediak-Higashi syndrome and undetermined cause) and periodontitis. A periodic evaluation for periodontal disease should be recommended in patients with glycogenosis type Ib, as well as for patients with chronic neutropenia or impaired neutrophil chemotaxis. Toshiro Hara, MD Yumi Mizuno, MD Kenshi Okubo, MD Kohji Ueda, MD Department of Pediatrics Faculty of Medicine Kyushu University, Fukuoka, Japan REFERENCES
1.
Klebanoff S J, Clark RA. Checliak-Higashi syndrome. In: Klebanoff S J, Clark RA, eds. The neutrophil! function and clinical disorders. New York: North-Holland Publishing Co, 1978:735-92. 2. Anderson DC, Schmalsteig FC, Finegold M J, et al. The severe and moderate phenotypes of heritable Mac 1, LFA-1 deficiency: their quantitative definition and relation to leukocyte dysfunction and clinical features. J Infect Dis 1985;54:257. 3. Page RC, Baab DA. A new look at the etiology and pathogenesis of early-onset periodontitis: cementopathia revisited. J Periodontol 1985;56:748. 4. Beaudet AL, Anderson DC, Michels VV, Arison W J, Lange AJ. Neutropenia and impaired neutrophil migration in type IB glycogen storage disease. J PEDrATR 1980;97:906. 5. Schaube J, Heyne K. Review: glycogen storage disease type lb. Eur J Pediatr 1983;140:283.
Reply To the Editor. The suggestion by Hara et al. that periodontitis is an expected complication of the neutrophil dysfunction associated with type Ib
952
glycogen storage disease is appropriate. This has not been emphasized in publications, but our two patients with type Ib disease do have periodontitis. Gingivitis is mentioned in some reports. 1 As a corollary of this issue, the finding of periodontal disease in the prepubescent child often indicates an underlying systemic disorder and always warrants careful evaluation of neutrophil function. Arthur L. Beaudet, MD Howard Hughes Medical Institute Institute for Molecular Genetics Department of Pediatrics Donald C. Anderson, MD Department of Pediatrics Baylor College of Medicine Houston, TX 77030 REFERENCES
1.
Ambruso DR, McCabe ERB, Anderson D, et al. Infectious and bleeding complications in patients with glycogenosis Ib. Am J Dis Child 1985;139:691.
Risk of SIDS in siblings To the Editor." Peterson et al. (J PEmATR 1986;108:911-4) concluded that the practical significance of a twofold difference in the risk for sudden infant death syndrome (SIDS) among the siblings of SIDS victims, and that among the siblings of controls matched on maternal age and birth rank, may be neglible, and that siblings of SIDS victims are not at a significant excessive risk to SIDS. I believe these conclusions are unjustified, given the stated purpose of their article. If the desire had been to know whether an observed excessive risk could be explained by a weighting of known risk factors, then the strategy employed was correct. The question they were addressing, however, was not of a research nature, but rather that of the appropriate advice to parents and providers of care. From the latter's perspective, the determination that a certain level of risk is unacceptably high is not likely to be influenced by the explanation for the high risk. If possible, the counsel given to parents should be consistent with the subsequent position taken by the care provider. Although subsequent siblings as a group appear to be subject to a SIDS rate that is four to five times greater than that in the general population, the data of Peterson et al. suggest that it may be helpful to think of this overall risk of recurrence as being made up of a number of significantly different rates. If, after adjustment for the mother's age and parity, the difference between the risk for case and control siblings is neglible, then a specific risk of recurrence can be obtained by referring to a cross-tabulation by mother's age and parity of SIDS rates in the general population. In Oregon, for example, a subsequent sibling born to a teenage
Volume 111 Number 6, part 1
Editorial correspondence
953
T a b l e . Infant mortality rates for S I D S per 1000 live births by m o t h e r ' s age and birth rank: Oregon 1980-1984 Mother's a g e (yr) Birth rank
<20
20-24
1 2 3 >---4 All
4.8 (94) 10.2 (45) 10.8 (6) -5.9 (145)
1.9 3.9 7.0 7.0 3.3
25-29
(64) (96) (52) (13) (225)
1.2 1.6 2.0 3.2 1.7
(28) (41) (25) (19) (113)
>__30 1.0 1.2 1.0 2.4 1.4
(10) (18) (10) (25) (63)
Total 2.2 (196) 2.9 (200) 3.0 (93) 3.1 (57) 2.7 (546)
Data from Health Division, Oregon Department of Human Resources,personal communication, 1987, Number of cases in parenthesis.
mother who has had one previous live born child has a risk of recurrence of 10.2 per 1000 live births (Table). It does not seem unreasonable to double this figure if we accept a twofold difference in the SIDS rates for cases and controls after adjustment. The subsequent sibling in question, then, can be estimated to be subject to a rate that is 7.6 (10.2 • 2/2.7) times greater than that in the population as whole. By contrast, subsequent siblings in certain other maternal age/parity categories would appear to have a relative risk of <1.
Philip S. Spiers, PhD American SIDS Institute Portland, OR 97205
Reply To the Editor." Because we found only four SIDS recurrences among 566 next subsequent siblings (six among all 810 subsequent siblings), we chose to interpret our findings conservatively, i.e., applicable to the group as a whole. Extrapolation of our results to subsets, as described by Spiers, probably exceeds the limitations of our data.
Donald R. Peterson, MD Eugene E. Sabotta, MA Janet R. Daling, PhD Department of Epidemiology School of Public Health and Community Medicine University of Washington SC-36 Seattle, WA 98195
gestation. His early course was unremarkable. Feeding residuals on the seventh day of life were followed by apnea, bradycardia, and shock. He required chest compressions, tracheal intubation, and fluids via an umbilical artery catheter. Ampicillin and gentamicin were administered. Radiographs demonstrated pneumoperitoneum and pneumatosis intestinalis. A laparotomy was performed, and his necrotic, perforated left colon was removed. Severe disseminated intravascular coagulation ensued. An aortagram that preceded umbilical artery catheter removal demonstrated multiple iliac artery branch thrombi. Renal failure and progressive disseminated intravascular coagulation led to his death, 13 hours postoperatively. Patient 2, born a year earlier, also the third of triplets, weighing 1.16 kg at 32 weeks gestation. Her first days of life were uneventful, but on the fourth day her gavage feedings were discontinued and ampicillin and gentamicin were administered because of abdominal distention. Hematochezia and pneumatosis intestinalis developed. Initial improvement was followed by cardiovascular collapse and bowel perforation on the ninth day. At operation, multiple perforations were evident in the necrotic jejunum and ileum. Cardiac arrest ensued. In both patients, blood cultures drawn at the time of initial clinical deterioration grew a pure growth of S. epidermidis. Our experience and that of othersa,2 associate bacteremia with this organism with severe necrotizing enterocolitis that requires surgical intervention and is sometimes associated with a fatal outcome. We heartily endorse Gruskay's recommendations to consider vancomycin in the initial management of any form of enterocolitis.
Jerri Curtis, MD, LT, MC, USNR Department of Pediatrics Paul E. Stable, MD, LCDR, MC, USN Neonatology Division Naval Hospital Portsmouth, VA 23708-5000
Necrotizing enterocolitis and
staphylococcal sepsis To the Editor." Recently Gruskay et al. (J PEDIATR1986; 109:520-4) emphasized the frequency with which Staphylococcus epidermidis bacteremia was associated with a characteristically mild form of enterocolitis. Two recent cases at our institution extend this association to include severe, even fatal forms of necrotizing enterocolitis. Patient 1, the third of triplets, weighed 1.32 kg at 32 weeks
REFERENCES 1.
2.
Baumgart S, Hall SE, Campos JE, et al. Sepsis with eoagulase negative staphylococci in critically ill newborns. Am J Dis Child 1983;137:461-3. Noel G J, Edelson PJ. Staphylococcus epidermidis bacteremia in neonates: further observations and the occurrence of focal infection. Pediatrics 1984;74:832-7.
Glycogenosis type Ib and periodontitis To the Editor." Periodontitis in children may occur as a sole manifestation without underlying diseases or as a part of the manifestations of systemic diseases such as Papillon-Lef6vre syndrome, hypophosphatasia, cyclic neutropenia, Che~liak-Higashi syndrome and Mac 1/LFA 1 deficiency syndrome. L2 Thus there is an association between periodontitis and quantitative or qualitative disorders of leukocytes, particularly impaired neutrophil chemotaxis. 3 Glycogenosis type Ib is often associated with neutropenia, and impaired neutrophil chemotaxis and bacteriocidal activity? .5 Our 4-yearold patient with glycogenosis type Ib does not have periodontitis, but we have two older patients with impaired neutrophil chemotaxis (Chediak-Higashi syndrome and undetermined cause) and periodontitis. A periodic evaluation for periodontal disease should be recommended in patients with glycogenosis type Ib, as well as for patients with chronic neutropenia or impaired neutrophil chemotaxis. Toshiro Hara, MD Yumi Mizuno, MD Kenshi Okubo, MD Kohji Ueda, MD Department of Pediatrics Faculty of Medicine Kyushu University, Fukuoka, Japan REFERENCES
1.
Klebanoff S J, Clark RA. Checliak-Higashi syndrome. In: Klebanoff S J, Clark RA, eds. The neutrophil! function and clinical disorders. New York: North-Holland Publishing Co, 1978:735-92. 2. Anderson DC, Schmalsteig FC, Finegold M J, et al. The severe and moderate phenotypes of heritable Mac 1, LFA-1 deficiency: their quantitative definition and relation to leukocyte dysfunction and clinical features. J Infect Dis 1985;54:257. 3. Page RC, Baab DA. A new look at the etiology and pathogenesis of early-onset periodontitis: cementopathia revisited. J Periodontol 1985;56:748. 4. Beaudet AL, Anderson DC, Michels VV, Arison W J, Lange AJ. Neutropenia and impaired neutrophil migration in type IB glycogen storage disease. J PEDrATR 1980;97:906. 5. Schaube J, Heyne K. Review: glycogen storage disease type lb. Eur J Pediatr 1983;140:283.
Reply To the Editor. The suggestion by Hara et al. that periodontitis is an expected complication of the neutrophil dysfunction associated with type Ib
952
glycogen storage disease is appropriate. This has not been emphasized in publications, but our two patients with type Ib disease do have periodontitis. Gingivitis is mentioned in some reports. 1 As a corollary of this issue, the finding of periodontal disease in the prepubescent child often indicates an underlying systemic disorder and always warrants careful evaluation of neutrophil function. Arthur L. Beaudet, MD Howard Hughes Medical Institute Institute for Molecular Genetics Department of Pediatrics Donald C. Anderson, MD Department of Pediatrics Baylor College of Medicine Houston, TX 77030 REFERENCES
1.
Ambruso DR, McCabe ERB, Anderson D, et al. Infectious and bleeding complications in patients with glycogenosis Ib. Am J Dis Child 1985;139:691.
Risk of SIDS in siblings To the Editor." Peterson et al. (J PEmATR 1986;108:911-4) concluded that the practical significance of a twofold difference in the risk for sudden infant death syndrome (SIDS) among the siblings of SIDS victims, and that among the siblings of controls matched on maternal age and birth rank, may be neglible, and that siblings of SIDS victims are not at a significant excessive risk to SIDS. I believe these conclusions are unjustified, given the stated purpose of their article. If the desire had been to know whether an observed excessive risk could be explained by a weighting of known risk factors, then the strategy employed was correct. The question they were addressing, however, was not of a research nature, but rather that of the appropriate advice to parents and providers of care. From the latter's perspective, the determination that a certain level of risk is unacceptably high is not likely to be influenced by the explanation for the high risk. If possible, the counsel given to parents should be consistent with the subsequent position taken by the care provider. Although subsequent siblings as a group appear to be subject to a SIDS rate that is four to five times greater than that in the general population, the data of Peterson et al. suggest that it may be helpful to think of this overall risk of recurrence as being made up of a number of significantly different rates. If, after adjustment for the mother's age and parity, the difference between the risk for case and control siblings is neglible, then a specific risk of recurrence can be obtained by referring to a cross-tabulation by mother's age and parity of SIDS rates in the general population. In Oregon, for example, a subsequent sibling born to a teenage
Volume 111 Number 6, part 1
Editorial correspondence
953
T a b l e . Infant mortality rates for S I D S per 1000 live births by m o t h e r ' s age and birth rank: Oregon 1980-1984 Mother's a g e (yr) Birth rank
<20
20-24
1 2 3 >---4 All
4.8 (94) 10.2 (45) 10.8 (6) -5.9 (145)
1.9 3.9 7.0 7.0 3.3
25-29
(64) (96) (52) (13) (225)
1.2 1.6 2.0 3.2 1.7
(28) (41) (25) (19) (113)
>__30 1.0 1.2 1.0 2.4 1.4
(10) (18) (10) (25) (63)
Total 2.2 (196) 2.9 (200) 3.0 (93) 3.1 (57) 2.7 (546)
Data from Health Division, Oregon Department of Human Resources,personal communication, 1987, Number of cases in parenthesis.
mother who has had one previous live born child has a risk of recurrence of 10.2 per 1000 live births (Table). It does not seem unreasonable to double this figure if we accept a twofold difference in the SIDS rates for cases and controls after adjustment. The subsequent sibling in question, then, can be estimated to be subject to a rate that is 7.6 (10.2 • 2/2.7) times greater than that in the population as whole. By contrast, subsequent siblings in certain other maternal age/parity categories would appear to have a relative risk of <1.
Philip S. Spiers, PhD American SIDS Institute Portland, OR 97205
Reply To the Editor." Because we found only four SIDS recurrences among 566 next subsequent siblings (six among all 810 subsequent siblings), we chose to interpret our findings conservatively, i.e., applicable to the group as a whole. Extrapolation of our results to subsets, as described by Spiers, probably exceeds the limitations of our data.
Donald R. Peterson, MD Eugene E. Sabotta, MA Janet R. Daling, PhD Department of Epidemiology School of Public Health and Community Medicine University of Washington SC-36 Seattle, WA 98195
gestation. His early course was unremarkable. Feeding residuals on the seventh day of life were followed by apnea, bradycardia, and shock. He required chest compressions, tracheal intubation, and fluids via an umbilical artery catheter. Ampicillin and gentamicin were administered. Radiographs demonstrated pneumoperitoneum and pneumatosis intestinalis. A laparotomy was performed, and his necrotic, perforated left colon was removed. Severe disseminated intravascular coagulation ensued. An aortagram that preceded umbilical artery catheter removal demonstrated multiple iliac artery branch thrombi. Renal failure and progressive disseminated intravascular coagulation led to his death, 13 hours postoperatively. Patient 2, born a year earlier, also the third of triplets, weighing 1.16 kg at 32 weeks gestation. Her first days of life were uneventful, but on the fourth day her gavage feedings were discontinued and ampicillin and gentamicin were administered because of abdominal distention. Hematochezia and pneumatosis intestinalis developed. Initial improvement was followed by cardiovascular collapse and bowel perforation on the ninth day. At operation, multiple perforations were evident in the necrotic jejunum and ileum. Cardiac arrest ensued. In both patients, blood cultures drawn at the time of initial clinical deterioration grew a pure growth of S. epidermidis. Our experience and that of othersa,2 associate bacteremia with this organism with severe necrotizing enterocolitis that requires surgical intervention and is sometimes associated with a fatal outcome. We heartily endorse Gruskay's recommendations to consider vancomycin in the initial management of any form of enterocolitis.
Jerri Curtis, MD, LT, MC, USNR Department of Pediatrics Paul E. Stable, MD, LCDR, MC, USN Neonatology Division Naval Hospital Portsmouth, VA 23708-5000
Necrotizing enterocolitis and
staphylococcal sepsis To the Editor." Recently Gruskay et al. (J PEDIATR1986; 109:520-4) emphasized the frequency with which Staphylococcus epidermidis bacteremia was associated with a characteristically mild form of enterocolitis. Two recent cases at our institution extend this association to include severe, even fatal forms of necrotizing enterocolitis. Patient 1, the third of triplets, weighed 1.32 kg at 32 weeks
REFERENCES 1.
2.
Baumgart S, Hall SE, Campos JE, et al. Sepsis with eoagulase negative staphylococci in critically ill newborns. Am J Dis Child 1983;137:461-3. Noel G J, Edelson PJ. Staphylococcus epidermidis bacteremia in neonates: further observations and the occurrence of focal infection. Pediatrics 1984;74:832-7.