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Portal hypertension following neonatal cytomegalovirus infection
Volume 94 Number 6
Editorial correspondence
REFERENCE
1. McKeen EA, Bodurtha J, Meadows AT, Douglass EC, and MulvihiU J: Rhabdomyosarcoma complicating multiple neurofibromato~is, J P~DIATR 93:992, 1978.
Portal hypertension following neonatal cytomegalovirus infection To the Editor: The child with noncirrhotic portal fibrosis following neonatal cytomegalic inclusion disease reported by Dresler and Linder 1 is remarkably similar to one whom we have followed. At delivery, after a term gestation, our patient weighed 1,800 gm and had a "blueberry muffin" appearance. Urine culture yielded cytomegalovirus (CMV) on two occasions. She had persistent thrombocytopenia and hypoglycemia, and hepatosplenomegaly with abnormal liver function tests (peak bilirubin 41.4 mg/dl total, 36.8 direct, and SGOT = 470 units). These problems resolved but she grew poorly. At age 12 months she had an upper gastrointestinal hemorrhage. Endoscopy demonstrated gastritis without varices, but varices were detected on two subsequent upper gastrointestinal series. Percutaneous needle biopsy of the liver at age 14 months showed the lobular architecture to be normal but there was central collapse with fine spidery fibrosis along the walls of the sinusoids, so-called cappillarization, similar to the strands of mature collagen in the sinusoids described by Dresler and Linder. The portal areas were normal, but the specimen was insufficient to rule out the subendothelial fibrosis of the portal vein radicles described in the report. Biopsy material did not yield CMV on culture. Both our patient.and the one reported by Dresler and Linder had CMID with moderately severe neonatal hepatitis, followed within 16 months by portal hypertension, associated with an unusual histologic picture of a sinusoidal collagenization in the absence of cirrhosis. We conclude that it is probable that the hepatic lesion was caused by the antecedent CMV infection. These cases demonstrate that, in contrast to the previous impression that neurologic damage is the only significant sequela of congenital CMV infection? permanent hepatic damage can also occur. Since asymptomatic CMV infection in the neonatal period is common ~ and idiopathic portal hypertension and sinusoidal capiUarization are rare, it is likely that only severe, symptomatic CMV hepatitis, such as that occurring in these two patients, results in irreversible liver injury. Caroline A. Riely, M.D. Samuel Kocoshis, M.D. Joyce D. GryboskL M.D. Gerald Klatskin, M.D. Liver Study Unit Departments of Internal Medicine and Pediatrics Yale University School of Medicine 333 Cedar St. New Haven, CT 06510
10 1 1
REFERENCES
1. Dresler S, and Linder D: Noncirrhotic portal fibrosis following neonatal cytomegalic inclusion disease, J PEDIATR 93:887, 1978. 2. Hanshaw, JB: in Rudolph A M, editor: Pediatrics, New York 1977, Appleton-Century-Crofts, pp 520-523. 3. Kuman ML, Nankervis GA, and Gold E: Inapparent congenital cytomegalovirus infection: A foUow-up study, N Engl J Med 288:1370, 1973.
Washout of intranasal DDA VP with swimming To the Editor: During the past three years we have treated all established and new patients with central diabetes insipidus with 1-deamino8-D-arginine vasopressin (DDAVP). Becker and Foley, 1 as well as others, 2 agree that DDAVP is the treatment of choice for children with central diabetes insipidus. The mother of an adolescent boy asked whether the smoking of marijuana might interfere with the effect of the drug because of recent decrease in duration of effect. The patient reported that he noted the decreased duration of effect on the days that he went swimming. We suggested that he use a nose clip. Over the next three months we surveyed our 22 patients receiving this drug for this problem. Twelve of them swam on occasion and five of these 12 had noticed increased diuresis on swimming days. They had swum in the ocean, in small private pools, and in large municipal pooh, ruling out a specific effect of chlorine. Nose plugs were uniformly rejected as uncomfortable. The problem was solved instead by adding a small dose in the afternoon. The mechanism of prolonged antidiuretic effect of DDAVP is apparently gradual absorption of the drug from the nasal mucosa? Water reaching the posterior nares through the nose may be instrumental in washing out the unabsorbed drug. Anticipation of this potential problem in the youngster who swims will prevent unnecessary alarm by patient or parents. William J. Riley, M.D. Arian L. Rosenbloom, M.D. Division of Endocrinology, Dept. of Pediatrics University of Florida Gainesville, FL 32610 REFERENCES
1. Becker DJ, and Foley TP: l-Deamino-8-D-arginine vasopressin in the treatment of central diabetes insipidus in childhood, J PEDIATR 92:1011, 1978. 2. Lampert RP, Blackett PR, and Rennert OM: Management of diabetes insipidus with DDAVP, J PEDIATR 93:896, 1978. 3. Seif SM, Zenser TV, Ciarochi FF, Davis BB, and Robinson AG: DDAVP (1-deamino-8-D-arginine-vasopressin) treatment of central diabetes insipidus-mechanism of prolonged antidiuresis, J Clin Endocrinol Metab 46:381, 1978.
Editorial correspondence
REFERENCE
1. McKeen EA, Bodurtha J, Meadows AT, Douglass EC, and MulvihiU J: Rhabdomyosarcoma complicating multiple neurofibromato~is, J P~DIATR 93:992, 1978.
Portal hypertension following neonatal cytomegalovirus infection To the Editor: The child with noncirrhotic portal fibrosis following neonatal cytomegalic inclusion disease reported by Dresler and Linder 1 is remarkably similar to one whom we have followed. At delivery, after a term gestation, our patient weighed 1,800 gm and had a "blueberry muffin" appearance. Urine culture yielded cytomegalovirus (CMV) on two occasions. She had persistent thrombocytopenia and hypoglycemia, and hepatosplenomegaly with abnormal liver function tests (peak bilirubin 41.4 mg/dl total, 36.8 direct, and SGOT = 470 units). These problems resolved but she grew poorly. At age 12 months she had an upper gastrointestinal hemorrhage. Endoscopy demonstrated gastritis without varices, but varices were detected on two subsequent upper gastrointestinal series. Percutaneous needle biopsy of the liver at age 14 months showed the lobular architecture to be normal but there was central collapse with fine spidery fibrosis along the walls of the sinusoids, so-called cappillarization, similar to the strands of mature collagen in the sinusoids described by Dresler and Linder. The portal areas were normal, but the specimen was insufficient to rule out the subendothelial fibrosis of the portal vein radicles described in the report. Biopsy material did not yield CMV on culture. Both our patient.and the one reported by Dresler and Linder had CMID with moderately severe neonatal hepatitis, followed within 16 months by portal hypertension, associated with an unusual histologic picture of a sinusoidal collagenization in the absence of cirrhosis. We conclude that it is probable that the hepatic lesion was caused by the antecedent CMV infection. These cases demonstrate that, in contrast to the previous impression that neurologic damage is the only significant sequela of congenital CMV infection? permanent hepatic damage can also occur. Since asymptomatic CMV infection in the neonatal period is common ~ and idiopathic portal hypertension and sinusoidal capiUarization are rare, it is likely that only severe, symptomatic CMV hepatitis, such as that occurring in these two patients, results in irreversible liver injury. Caroline A. Riely, M.D. Samuel Kocoshis, M.D. Joyce D. GryboskL M.D. Gerald Klatskin, M.D. Liver Study Unit Departments of Internal Medicine and Pediatrics Yale University School of Medicine 333 Cedar St. New Haven, CT 06510
10 1 1
REFERENCES
1. Dresler S, and Linder D: Noncirrhotic portal fibrosis following neonatal cytomegalic inclusion disease, J PEDIATR 93:887, 1978. 2. Hanshaw, JB: in Rudolph A M, editor: Pediatrics, New York 1977, Appleton-Century-Crofts, pp 520-523. 3. Kuman ML, Nankervis GA, and Gold E: Inapparent congenital cytomegalovirus infection: A foUow-up study, N Engl J Med 288:1370, 1973.
Washout of intranasal DDA VP with swimming To the Editor: During the past three years we have treated all established and new patients with central diabetes insipidus with 1-deamino8-D-arginine vasopressin (DDAVP). Becker and Foley, 1 as well as others, 2 agree that DDAVP is the treatment of choice for children with central diabetes insipidus. The mother of an adolescent boy asked whether the smoking of marijuana might interfere with the effect of the drug because of recent decrease in duration of effect. The patient reported that he noted the decreased duration of effect on the days that he went swimming. We suggested that he use a nose clip. Over the next three months we surveyed our 22 patients receiving this drug for this problem. Twelve of them swam on occasion and five of these 12 had noticed increased diuresis on swimming days. They had swum in the ocean, in small private pools, and in large municipal pooh, ruling out a specific effect of chlorine. Nose plugs were uniformly rejected as uncomfortable. The problem was solved instead by adding a small dose in the afternoon. The mechanism of prolonged antidiuretic effect of DDAVP is apparently gradual absorption of the drug from the nasal mucosa? Water reaching the posterior nares through the nose may be instrumental in washing out the unabsorbed drug. Anticipation of this potential problem in the youngster who swims will prevent unnecessary alarm by patient or parents. William J. Riley, M.D. Arian L. Rosenbloom, M.D. Division of Endocrinology, Dept. of Pediatrics University of Florida Gainesville, FL 32610 REFERENCES
1. Becker DJ, and Foley TP: l-Deamino-8-D-arginine vasopressin in the treatment of central diabetes insipidus in childhood, J PEDIATR 92:1011, 1978. 2. Lampert RP, Blackett PR, and Rennert OM: Management of diabetes insipidus with DDAVP, J PEDIATR 93:896, 1978. 3. Seif SM, Zenser TV, Ciarochi FF, Davis BB, and Robinson AG: DDAVP (1-deamino-8-D-arginine-vasopressin) treatment of central diabetes insipidus-mechanism of prolonged antidiuresis, J Clin Endocrinol Metab 46:381, 1978.