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A case of profound iron deficiency anemia owing to corrosive esophagitis in a 20-year-old developmentally delayed male
JOURNAL OF ADOLESCENT HEALTH 1994;15:592-594
CASE REPORT
A Case of Profound Iron Deficiency Anemia Owing to Corrosive Esophagitis in a 20-Year-Old Developmentally Delayed Male RICHARD M. BUCHTA, M.D. AND ROBERT BICKERTON
The level of severe compensated iron deficiency anemia incompatible with life is not defined in the pediatric or adolescent literature. A hemoglobin of 3.5 gmldl in an older adolescent with few physical symptoms is distinctly unusual. A case of profound iron deficiency anemia in a ZO-year-old developmentally delayed male is the subject of this brief report. There were only subtle physical findings in spite of this severe anemia. The anemia was the result of corrosive esophagitis associated with a hiatal hernia and reflux. Physicians dealing with developmentally delayed adolescents should be aware of the fact that a severe anemia may develop, and such individuals should be periodically screened for anemia, melena, hematochezia, and hematemesis. KEY WORDS:
Severe iron deficiency anemia Corrosive esophagitis Reflux
mentally delayed male is the subject of this paper. We wish to alert physicians who care for such individuals that a severe anemia may develop with only subtle physical signs and symptoms.
Case Report The patient is a 20-year-old Caucasian male who had an anoxic neurological insult at the age of 10 years (strangulation from an electrical car door). This visit followed fever of two days duration along with some mild personality changes, according to the mother. He also had an occasional episode of brown emesis over the past two months and a twoyear history of chronic abdominal pain and dysphagia. There was no melena, hematochezia or hematemesis.
Past History
introduction The level of severe compensated iron deficiency anemia incompatible with life is not defined in the pediatric or adolescent literature. A hemoglobin of 1.5 gm/dl in an older adolescent with few physical symptoms is disi’ilctly unusual. A case of profound iron deficiency anemia in a 2O-year-old develop-
103-139x/94/$7.00
He was hospitalized for one year (1981-1982) for anoxic encephalopathy with permanent neurological sequelae requiring complete dependent care. In September 1988, an upper GI series sho$ved normal mucosa and peristalsis of the esophagus and stomach. There was a small 2.5 mm shallow ulceration at the apex of the duodenal bulb. He was treated with I-I, receptor anta.gonist. A hemoglobin/hematocrit at that time was 12.6 gm/d1/40.5%. MCV was 93 mm. He is hypersensitive to benzodiazepines. At the time of evaluation, he was taking phenetidine 40 mg q hs, Mylanta 30 cc q 4 hrs pm, simethicone 80 mg pm, tenazapine 15 mg pm, and acetaminophen 650 mg pm. Since the time of the accident he has been cared for by his parents and is responsive to some
0 Society for Adolescent Medicine, 1994 Published by Elsevier Science Inc., 655 Avenue of :he Americas, New York, NY 10010
November 1994
ANEMIA OWING TO ESOPHAGITIS
basic commands, minimally conversational (mostly grunts), with limited purposeful movclment of his extremities. He is on a regular diet.
Family History 1vo specific illnesses, blood disorders.
Phycical
gastrointestinal
diseases
or
Examination
Temperature was 38.3”C. Heart rate was 130/minute. Respiratory rate was 20/minute. Blood pressure was 120/70 mmHg. in a lying position. Examination of th’e head, eyes, ears, nose and throat was normal. The neck revealed no lymphadenopathy. Heart showed regular rhythm with a normal Sl, S2. There was no S3 or S4 sounds. A grade 1 out of 6 systolic ejection murmur was noted. Examination of the lungs revealed some basilar rales. The abdomen was soft with no masse?, organomegaly or tenderness. Genitalia were Tanner stage 3 with testes distended bilaterally., Rectal examination revealed no lesions but stool was trace positive for blood. The skin was pale with a slight yellowish-orange discoloration. He was minimally responsive to verbal commands but was not cooperative for mental status, strength, coordination or sensory examination. Deep tendon reflexes were 3+ and symmetrical bilaterally. There were positive Babinski’s bilaterally and hypertonic extremities with mild contractures.
Significant
Laboratory
Studies
Hemoglobin was 1.5 g&d1 and hematocrit was 5.7%. MCV was 64.3 mm and the MCHC was 28.6 gm/dl. A blood smear revealed moderate schistocytes, marked anicytosis and Frofound hypochromic microcytic anemia with no evidence of basophilit stippling or WBC abnormalities. An arterial blood gas on 35% FIO,, pH was 7.48, PaCO, was 29.6, Pa4 was 161.4. The AADO, was 56, and hemoglobin was 1.8%. Serum ferritin was 7 ng/ml. Serum iron was 21 pg/dl, iron-binding capacity was 403 pg/dl and iron to iron-binding capacity was 5.2%. Platelets were 156,000, prothrombin time was 12.4 set, partial thromboplastin time (PTT) was 30.9 with a control of 29.3 seconds. Reticulocyte count, uncorrected, was 6.4%; corrected 0.8%. Heavy metal screening of blood and urine were negative. Hemoglobin electrophoresis was normal. Direct and indirect Coombs were negative. Total bilirubin was 0.2 mg/dl. Urine hemosiderin was negative. LDH was
593
227 &L. Haptoglobin was 122 kg/dl. WBC cnunt: 3400; 44% segs, 32% bands, 19% lymphs, 5% monos. Blood, urine and bone marrow cultures were negative. Chest and abdominal x-rays revealed mild cardiomegaly and no evidence of bowel obstruction, respectively. Sodium was 135 meq/L, chloride 101 meq/L, phosphate 3.8 mg/dl, potassium 3.6 meq/L, bicarbonate 22 meq/L, calcium 7.9 mg/dl, BUN 4 mg/dl, and creatinine 0.5 mg/dl. A bone marrow biopsy showed a hypocellulai marrow with megaloblastic dyserythropoiesis and absent iron stores. An upper endoscopy revealed moderately severe erosive esophagitis distally, moderate sized hiatal hernia with evidence of reflux, and no evidence of ulceration in the stomach and duodenum. Esophageal biopsy at the gastroesophage31 junction showed acute esophagitis and ulceration. A barium enema revealed no significant abnormalities.
Hospital
Course
Profound iron deficiency anemia, secondary to severe erosive esophagitis was diagnosed. The patient was transfused with 6 U of packed red blood cells. This infusion was given over a period of 24 hours. In retrospect, a much smaller amount of packed red cells would have been sufficient. The gastroenterologist, however, was concerned that should an acute episode of GI bleeding occur, the patient would not have the red blood cell reserve to survive. lt is important to transfuse blood slowly in cases of chronic anemia as heart failure can be precipitated by rapid infusion. Hemoglobin, hematocrit went from 1.5 gm/dl//5.7% to 11.2 gm/d1,//32.8%. A gastric acid pump inhibitor, Emeprizol, was started along with iron replacement therapy with ferrous sulfate. The patient’s fever spontaneously resolved after two days. No infectious etiology was identified and no antibiotics were given. Chest x-rays and cultures of blood, urine and bone marrow were negative. In view of the patient’s age and delayed pubertal development, serum testosterone level was obtwined,which was ~20 &dl. The patient was later treated with testosterone cypionate IM as an outpatient E.ut this was discontinued by parental request. The patient had an unremarkable hospital stay. He was discharged on Emeprizol 20 mg po q day, ferrous sulfate 300 mg po tid, and vitamin C 500 rrg PO q day. On i?,e date of discharge, his hemoglobin was 11.7 grn/dl, hematocrit was 36.8%, MCV was 85.9
594
R.M. BUCHTA
mm, MCHC was 31.8 gm/dl. Reticulocyte count was 1.5%, corrected 1.2%. He was last seen on l/2/92. At that time, his hemoglobin was 14.9 gm/dl, hematocrit was 44.5%, MCV was 91.7 mm, MCHC was 33.5 gm /dl, reticulocyte count was 1.3%, 1.3% corrected.
Discussion If fever !lad not developed in this young adult, he probably would not have been evaluated by a physician, in spite of a hemoglobin of 1.5 gm/dl. Under experimental conditions, abnormalities of cellmediated immunity and ability to kill several types of bacteria are well established (1). However, whether this leads to increased incidence or duration of infection is unknown in such individuals. In our case. the fever was probably on the basis of a viral rather than a bacterial infection, since all cultures were negative and fever receded spontaneously without antibiotics. The only objective findings that could be attributed to anemia in this patient are a heart rate of 130/minute and a pale, yellowish hue to the skin. Subjectively, the mother noticed some mild personality changes, although this would not have been detected by the staff owing to this young man’s severe developmental delay. Children with developmental delay have a higher incidence of reflux esophagitis. They tend to have decreased esophageal motility and to be in bed for longer periods of time, thereby increasing the risk for reflux.
JOURNAL OF ADOLETKENT HEALTH Vol. 15, No. 7
One condition associated with severe iron defisyndrome, which ciency anemia is Plummer-Vinson consists of iron deficiency anemia, dysphagia, esophageal webs and a lung-term risk for esophageal cancer (2). In this condition, the esophagitis is caused by the iron deficiency anemia rather than the reverse. On the other hand, severe corrosive esophagitis and hiatal hernia certainly can result in severe iron deficiency anemia (3,4). Our patient had esophagitis on endoscopy but no evidence of esophageal webs, and therefore cannot be included as a case of Plummer-Vinson syndrome. Our case represents the most severe iron deficiency anemia ever documented in corrosive esophagitis. Physicians dealing with developmentally delayed children should periodically screen for anemia, melena, hematochezia, and hematemesis, especially in patients with chronic abdominal pain and dysphagia.
References Dallman PR. Iron deficiency and the immune Clin Nutr 1987;463329-34.
response. Am J
Seitz ML, Sabatino D. Hummer-Vinson syt!drome in an adolescent. J Adolesc Health 1991;12:279-81. Windsor CWO, Collis JL. Anemia and hiatis hernia. Proc R Sot Med 1968;61:213-5. Fisher M, Katz S, Katzka I. Silent erosive esophagitis with severe iron-deficiency anemia. NY State J Med 1980 October:1740-2.
CASE REPORT
A Case of Profound Iron Deficiency Anemia Owing to Corrosive Esophagitis in a 20-Year-Old Developmentally Delayed Male RICHARD M. BUCHTA, M.D. AND ROBERT BICKERTON
The level of severe compensated iron deficiency anemia incompatible with life is not defined in the pediatric or adolescent literature. A hemoglobin of 3.5 gmldl in an older adolescent with few physical symptoms is distinctly unusual. A case of profound iron deficiency anemia in a ZO-year-old developmentally delayed male is the subject of this brief report. There were only subtle physical findings in spite of this severe anemia. The anemia was the result of corrosive esophagitis associated with a hiatal hernia and reflux. Physicians dealing with developmentally delayed adolescents should be aware of the fact that a severe anemia may develop, and such individuals should be periodically screened for anemia, melena, hematochezia, and hematemesis. KEY WORDS:
Severe iron deficiency anemia Corrosive esophagitis Reflux
mentally delayed male is the subject of this paper. We wish to alert physicians who care for such individuals that a severe anemia may develop with only subtle physical signs and symptoms.
Case Report The patient is a 20-year-old Caucasian male who had an anoxic neurological insult at the age of 10 years (strangulation from an electrical car door). This visit followed fever of two days duration along with some mild personality changes, according to the mother. He also had an occasional episode of brown emesis over the past two months and a twoyear history of chronic abdominal pain and dysphagia. There was no melena, hematochezia or hematemesis.
Past History
introduction The level of severe compensated iron deficiency anemia incompatible with life is not defined in the pediatric or adolescent literature. A hemoglobin of 1.5 gm/dl in an older adolescent with few physical symptoms is disi’ilctly unusual. A case of profound iron deficiency anemia in a 2O-year-old develop-
103-139x/94/$7.00
He was hospitalized for one year (1981-1982) for anoxic encephalopathy with permanent neurological sequelae requiring complete dependent care. In September 1988, an upper GI series sho$ved normal mucosa and peristalsis of the esophagus and stomach. There was a small 2.5 mm shallow ulceration at the apex of the duodenal bulb. He was treated with I-I, receptor anta.gonist. A hemoglobin/hematocrit at that time was 12.6 gm/d1/40.5%. MCV was 93 mm. He is hypersensitive to benzodiazepines. At the time of evaluation, he was taking phenetidine 40 mg q hs, Mylanta 30 cc q 4 hrs pm, simethicone 80 mg pm, tenazapine 15 mg pm, and acetaminophen 650 mg pm. Since the time of the accident he has been cared for by his parents and is responsive to some
0 Society for Adolescent Medicine, 1994 Published by Elsevier Science Inc., 655 Avenue of :he Americas, New York, NY 10010
November 1994
ANEMIA OWING TO ESOPHAGITIS
basic commands, minimally conversational (mostly grunts), with limited purposeful movclment of his extremities. He is on a regular diet.
Family History 1vo specific illnesses, blood disorders.
Phycical
gastrointestinal
diseases
or
Examination
Temperature was 38.3”C. Heart rate was 130/minute. Respiratory rate was 20/minute. Blood pressure was 120/70 mmHg. in a lying position. Examination of th’e head, eyes, ears, nose and throat was normal. The neck revealed no lymphadenopathy. Heart showed regular rhythm with a normal Sl, S2. There was no S3 or S4 sounds. A grade 1 out of 6 systolic ejection murmur was noted. Examination of the lungs revealed some basilar rales. The abdomen was soft with no masse?, organomegaly or tenderness. Genitalia were Tanner stage 3 with testes distended bilaterally., Rectal examination revealed no lesions but stool was trace positive for blood. The skin was pale with a slight yellowish-orange discoloration. He was minimally responsive to verbal commands but was not cooperative for mental status, strength, coordination or sensory examination. Deep tendon reflexes were 3+ and symmetrical bilaterally. There were positive Babinski’s bilaterally and hypertonic extremities with mild contractures.
Significant
Laboratory
Studies
Hemoglobin was 1.5 g&d1 and hematocrit was 5.7%. MCV was 64.3 mm and the MCHC was 28.6 gm/dl. A blood smear revealed moderate schistocytes, marked anicytosis and Frofound hypochromic microcytic anemia with no evidence of basophilit stippling or WBC abnormalities. An arterial blood gas on 35% FIO,, pH was 7.48, PaCO, was 29.6, Pa4 was 161.4. The AADO, was 56, and hemoglobin was 1.8%. Serum ferritin was 7 ng/ml. Serum iron was 21 pg/dl, iron-binding capacity was 403 pg/dl and iron to iron-binding capacity was 5.2%. Platelets were 156,000, prothrombin time was 12.4 set, partial thromboplastin time (PTT) was 30.9 with a control of 29.3 seconds. Reticulocyte count, uncorrected, was 6.4%; corrected 0.8%. Heavy metal screening of blood and urine were negative. Hemoglobin electrophoresis was normal. Direct and indirect Coombs were negative. Total bilirubin was 0.2 mg/dl. Urine hemosiderin was negative. LDH was
593
227 &L. Haptoglobin was 122 kg/dl. WBC cnunt: 3400; 44% segs, 32% bands, 19% lymphs, 5% monos. Blood, urine and bone marrow cultures were negative. Chest and abdominal x-rays revealed mild cardiomegaly and no evidence of bowel obstruction, respectively. Sodium was 135 meq/L, chloride 101 meq/L, phosphate 3.8 mg/dl, potassium 3.6 meq/L, bicarbonate 22 meq/L, calcium 7.9 mg/dl, BUN 4 mg/dl, and creatinine 0.5 mg/dl. A bone marrow biopsy showed a hypocellulai marrow with megaloblastic dyserythropoiesis and absent iron stores. An upper endoscopy revealed moderately severe erosive esophagitis distally, moderate sized hiatal hernia with evidence of reflux, and no evidence of ulceration in the stomach and duodenum. Esophageal biopsy at the gastroesophage31 junction showed acute esophagitis and ulceration. A barium enema revealed no significant abnormalities.
Hospital
Course
Profound iron deficiency anemia, secondary to severe erosive esophagitis was diagnosed. The patient was transfused with 6 U of packed red blood cells. This infusion was given over a period of 24 hours. In retrospect, a much smaller amount of packed red cells would have been sufficient. The gastroenterologist, however, was concerned that should an acute episode of GI bleeding occur, the patient would not have the red blood cell reserve to survive. lt is important to transfuse blood slowly in cases of chronic anemia as heart failure can be precipitated by rapid infusion. Hemoglobin, hematocrit went from 1.5 gm/dl//5.7% to 11.2 gm/d1,//32.8%. A gastric acid pump inhibitor, Emeprizol, was started along with iron replacement therapy with ferrous sulfate. The patient’s fever spontaneously resolved after two days. No infectious etiology was identified and no antibiotics were given. Chest x-rays and cultures of blood, urine and bone marrow were negative. In view of the patient’s age and delayed pubertal development, serum testosterone level was obtwined,which was ~20 &dl. The patient was later treated with testosterone cypionate IM as an outpatient E.ut this was discontinued by parental request. The patient had an unremarkable hospital stay. He was discharged on Emeprizol 20 mg po q day, ferrous sulfate 300 mg po tid, and vitamin C 500 rrg PO q day. On i?,e date of discharge, his hemoglobin was 11.7 grn/dl, hematocrit was 36.8%, MCV was 85.9
594
R.M. BUCHTA
mm, MCHC was 31.8 gm/dl. Reticulocyte count was 1.5%, corrected 1.2%. He was last seen on l/2/92. At that time, his hemoglobin was 14.9 gm/dl, hematocrit was 44.5%, MCV was 91.7 mm, MCHC was 33.5 gm /dl, reticulocyte count was 1.3%, 1.3% corrected.
Discussion If fever !lad not developed in this young adult, he probably would not have been evaluated by a physician, in spite of a hemoglobin of 1.5 gm/dl. Under experimental conditions, abnormalities of cellmediated immunity and ability to kill several types of bacteria are well established (1). However, whether this leads to increased incidence or duration of infection is unknown in such individuals. In our case. the fever was probably on the basis of a viral rather than a bacterial infection, since all cultures were negative and fever receded spontaneously without antibiotics. The only objective findings that could be attributed to anemia in this patient are a heart rate of 130/minute and a pale, yellowish hue to the skin. Subjectively, the mother noticed some mild personality changes, although this would not have been detected by the staff owing to this young man’s severe developmental delay. Children with developmental delay have a higher incidence of reflux esophagitis. They tend to have decreased esophageal motility and to be in bed for longer periods of time, thereby increasing the risk for reflux.
JOURNAL OF ADOLETKENT HEALTH Vol. 15, No. 7
One condition associated with severe iron defisyndrome, which ciency anemia is Plummer-Vinson consists of iron deficiency anemia, dysphagia, esophageal webs and a lung-term risk for esophageal cancer (2). In this condition, the esophagitis is caused by the iron deficiency anemia rather than the reverse. On the other hand, severe corrosive esophagitis and hiatal hernia certainly can result in severe iron deficiency anemia (3,4). Our patient had esophagitis on endoscopy but no evidence of esophageal webs, and therefore cannot be included as a case of Plummer-Vinson syndrome. Our case represents the most severe iron deficiency anemia ever documented in corrosive esophagitis. Physicians dealing with developmentally delayed children should periodically screen for anemia, melena, hematochezia, and hematemesis, especially in patients with chronic abdominal pain and dysphagia.
References Dallman PR. Iron deficiency and the immune Clin Nutr 1987;463329-34.
response. Am J
Seitz ML, Sabatino D. Hummer-Vinson syt!drome in an adolescent. J Adolesc Health 1991;12:279-81. Windsor CWO, Collis JL. Anemia and hiatis hernia. Proc R Sot Med 1968;61:213-5. Fisher M, Katz S, Katzka I. Silent erosive esophagitis with severe iron-deficiency anemia. NY State J Med 1980 October:1740-2.