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Hypogammaglobulinemia and elevated sweat chloride values
Volume 102 Number 1
diarrhea but who were not acidotic also did not develop methemoglobinemia (1.3 _+ 0.5%, n = 9, compared with 1.2 _+ 0.7% in 10 age-matched controls). However, all five young infants tested in the past year who had acidosis and diarrhea did have methemoglobinemia (6.2 _+ 2.6%). Based on these preliminary findings, it appears that an oxidant stress in conjunction with acidosis is necessary to produce methemoglobinemia in these infants. The contribution of acidosis to methemoglobinemia is also being studied. Methemoglobin reduction rates of normal cord and adult erythrocytes were assayed at pH 7.40 and 7.00. ~At pH 7.40 adult erythrocytes reduced methemoglobin at a rate (mean _+ SD) of 3.7 + 1.5% per hour, whereas at pH 7.00 the rate was 1.6 + 0.8% per hour (n = 10, P < 0.01). Cord blood (F hemoglobin content of 51 _+ 5%, mean _+ SD) had reduction rates of 4.9 _+ 1.6% per hour at PH 7.40 and 2.8 _+ 1.4% per hour at pH 7.00 (n = 8, P < 0.05). Preliminary results of isoelectric focusing studies show that both hemoglobins A and F are oxidized. Although the cause and physiopathology of methemoglobinemia in acidotic infants less than 3 months of age with diarrhea is unknown, one hypothesis is that the agent associated with tlle diarrhea produces an oxidant stress that causes methemoglobin formation. Because of concomitant acidosis, methemoglobi n reduction is impaired and methemoglobin levels rise. Once the oxidant stress diminishes and acidosis is corrected, the infants readily reduce methemoglobin to normal levels. We agree that this phenomenon is more prevalent than anticipated and should be part of the evaluation of "cyanotic" infants with diarrhea. No specific treatment for the methemoglobinemia seems indicated, unless levels are very high, because the condition is transient and self-correcting with restoration of fluid and electrolyte balance. Elizabeth H. Danish, M.D. Department o f Pediatrics Cleveland Metropolitan General Hospital 3395 Scranton Road Cleveland, OH 44109
Editorial correspondence
163
normal. Sweat sodium concentrations (pilocarpine iontophoresis) were 106, 92, and 107 mEq/L, respectively, on three different occasions. He had had recurrent giardiasis with lactose intolerance. A jejunal biopsy showed mild inflammation and shortening of intestinal villi. Both the malabsorption symptoms and the histologic abnormalities responded to eradication of Giardia lamblia. Pancreatic insufficiency could not be demonstrated (normal fecal fat excretion and normal amylase concentration in duodenal juice). Serum immunoglobulins were assayed repeatedly. IgG values varied between 510 and 950 mg/dl; IgM varied between 20 and 49 mg/dl, but lgA was always undetcctable. Despite the finding of elevated sweat sodium concentration a11d hypogammaglobulinemia, our patient has not had serious lung disease. In addition, none of the other conditions known to be associated with raised sweat electrolytes could explain this intriguing fact? We are convinced that our patient with hypogammaglobulinemia has abnormal sweat sodium concentration unrelated to cystic fibrosis. The patients of Corkey and Gelfand, as well as our observation, present another cause of misleading diagnosis of cystic fibrosis in patients with hypogammaglobulinemia. Nelson A. Rosario, M.D. Romolo Sandrini ]Veto, M.D. Assako Nitta, M.D. Leide P. Marinoni, M.D. Department o f Pediatrics Federal University o f Parana Parana, Brazil REFERENCES 1.
2.
Corkey CWB, Gelfand EW: Hypogammaglobulinemia and antibody deficiency in patients with elevated sweat chloride concentrations, J PEmA'rR 100:420, 1982. Wood RE, Boat TE, Doershuk CF: Cystic fibrosis, Am Rev Respir Dis 113:833, 1976.
REFERENCE I.
Beutler E, Baluda MC: Methemogtobin reduction: Studies of the interaction between cell populations and of the role of methylene blue, Blood 22:323, 1963.
Hypogammaglobulinemia and elevated sweat chloride values To the editor." The article by Corkey and Gelfar~d ~ is certainly of interest to those caring for patients with cystic fibrosis. We have recently seen an 18-year-old white man, who came to our attention because of growth retardation. There was a history of chronic diarrhea and recurrent upper respiratory infection. Physical examination was unremarkable except that height and weight were well below the third percentile. Thyroid, pituitary, and adrenal gland functions were normal. A radiologic study showed no abnormalities of his chest, but revealed bilateral maxillary sinusitis. Arterial blood gases and spirometry were
Association of diabetes mellitus
and hemolytic-uremic syndrome To the Editor." We read with interest the articles by Andreoli and Bergstein ~ and by Burns et al? regarding the association between insulindependent diabetes mellitus and the hemolytic-uremic syndrome. We have encountered a similar example of this association; others were reported previously by Loo et al? and Adelman et al. 4 Although in most of the patients reported there has been no pathologic correlation between pancreatic structural damage and hyperglycemia, the documentation of seven cases of this association would suggest that a causal relationship is present. Details in each of the seven patients are shown in the table. The average age (5.3 years) was higher than the total population of patients seen with hemolytic-uremic syndrome, and the presence of bloody diarrhea was also somewhat more prominent. Peritoneal dialysis was commenced in six of the seven patients prior to the onset of hyperglycemia. When present, measurements of amylase were
diarrhea but who were not acidotic also did not develop methemoglobinemia (1.3 _+ 0.5%, n = 9, compared with 1.2 _+ 0.7% in 10 age-matched controls). However, all five young infants tested in the past year who had acidosis and diarrhea did have methemoglobinemia (6.2 _+ 2.6%). Based on these preliminary findings, it appears that an oxidant stress in conjunction with acidosis is necessary to produce methemoglobinemia in these infants. The contribution of acidosis to methemoglobinemia is also being studied. Methemoglobin reduction rates of normal cord and adult erythrocytes were assayed at pH 7.40 and 7.00. ~At pH 7.40 adult erythrocytes reduced methemoglobin at a rate (mean _+ SD) of 3.7 + 1.5% per hour, whereas at pH 7.00 the rate was 1.6 + 0.8% per hour (n = 10, P < 0.01). Cord blood (F hemoglobin content of 51 _+ 5%, mean _+ SD) had reduction rates of 4.9 _+ 1.6% per hour at PH 7.40 and 2.8 _+ 1.4% per hour at pH 7.00 (n = 8, P < 0.05). Preliminary results of isoelectric focusing studies show that both hemoglobins A and F are oxidized. Although the cause and physiopathology of methemoglobinemia in acidotic infants less than 3 months of age with diarrhea is unknown, one hypothesis is that the agent associated with tlle diarrhea produces an oxidant stress that causes methemoglobin formation. Because of concomitant acidosis, methemoglobi n reduction is impaired and methemoglobin levels rise. Once the oxidant stress diminishes and acidosis is corrected, the infants readily reduce methemoglobin to normal levels. We agree that this phenomenon is more prevalent than anticipated and should be part of the evaluation of "cyanotic" infants with diarrhea. No specific treatment for the methemoglobinemia seems indicated, unless levels are very high, because the condition is transient and self-correcting with restoration of fluid and electrolyte balance. Elizabeth H. Danish, M.D. Department o f Pediatrics Cleveland Metropolitan General Hospital 3395 Scranton Road Cleveland, OH 44109
Editorial correspondence
163
normal. Sweat sodium concentrations (pilocarpine iontophoresis) were 106, 92, and 107 mEq/L, respectively, on three different occasions. He had had recurrent giardiasis with lactose intolerance. A jejunal biopsy showed mild inflammation and shortening of intestinal villi. Both the malabsorption symptoms and the histologic abnormalities responded to eradication of Giardia lamblia. Pancreatic insufficiency could not be demonstrated (normal fecal fat excretion and normal amylase concentration in duodenal juice). Serum immunoglobulins were assayed repeatedly. IgG values varied between 510 and 950 mg/dl; IgM varied between 20 and 49 mg/dl, but lgA was always undetcctable. Despite the finding of elevated sweat sodium concentration a11d hypogammaglobulinemia, our patient has not had serious lung disease. In addition, none of the other conditions known to be associated with raised sweat electrolytes could explain this intriguing fact? We are convinced that our patient with hypogammaglobulinemia has abnormal sweat sodium concentration unrelated to cystic fibrosis. The patients of Corkey and Gelfand, as well as our observation, present another cause of misleading diagnosis of cystic fibrosis in patients with hypogammaglobulinemia. Nelson A. Rosario, M.D. Romolo Sandrini ]Veto, M.D. Assako Nitta, M.D. Leide P. Marinoni, M.D. Department o f Pediatrics Federal University o f Parana Parana, Brazil REFERENCES 1.
2.
Corkey CWB, Gelfand EW: Hypogammaglobulinemia and antibody deficiency in patients with elevated sweat chloride concentrations, J PEmA'rR 100:420, 1982. Wood RE, Boat TE, Doershuk CF: Cystic fibrosis, Am Rev Respir Dis 113:833, 1976.
REFERENCE I.
Beutler E, Baluda MC: Methemogtobin reduction: Studies of the interaction between cell populations and of the role of methylene blue, Blood 22:323, 1963.
Hypogammaglobulinemia and elevated sweat chloride values To the editor." The article by Corkey and Gelfar~d ~ is certainly of interest to those caring for patients with cystic fibrosis. We have recently seen an 18-year-old white man, who came to our attention because of growth retardation. There was a history of chronic diarrhea and recurrent upper respiratory infection. Physical examination was unremarkable except that height and weight were well below the third percentile. Thyroid, pituitary, and adrenal gland functions were normal. A radiologic study showed no abnormalities of his chest, but revealed bilateral maxillary sinusitis. Arterial blood gases and spirometry were
Association of diabetes mellitus
and hemolytic-uremic syndrome To the Editor." We read with interest the articles by Andreoli and Bergstein ~ and by Burns et al? regarding the association between insulindependent diabetes mellitus and the hemolytic-uremic syndrome. We have encountered a similar example of this association; others were reported previously by Loo et al? and Adelman et al. 4 Although in most of the patients reported there has been no pathologic correlation between pancreatic structural damage and hyperglycemia, the documentation of seven cases of this association would suggest that a causal relationship is present. Details in each of the seven patients are shown in the table. The average age (5.3 years) was higher than the total population of patients seen with hemolytic-uremic syndrome, and the presence of bloody diarrhea was also somewhat more prominent. Peritoneal dialysis was commenced in six of the seven patients prior to the onset of hyperglycemia. When present, measurements of amylase were