lost to follow-up. We can now report a longer-term follow-up on this child, in order to illustrate the fickle nature of coccidioidomycotic infection and the relative ineffectiveness of our treatment for this condition. The child's condition improved substantially without further treatment after he left Fresno, Calif, but he developed signs of active local and systemic disease in 1966 at the age of 5. He was admitted to a Galveston, Tex, hospital with obvious synovitis of the left knee and drainage from the right hand. His complement-fixation titer was positive at 1:64 dilutions. His con¬ dition improved after an additional adminis¬ 1.8 gm of amphotericin tered intravenously, and he was dis¬ charged home. Recurrent disease in the left knee led to rehospitalization, synovectomy, and intravenous administration of another 150 mg of amphotericin in yet another Texas hospital in March 1968. A month later the knee was ma¬ nipulated under general anesthesia to increase its range of motion. The pa¬ tient was again lost to follow-up. On reexamination in February 1973, he was asymptomatic. There was a foreshortened second metacarpal with a 40° flexion contracture of the metacarpophalangeal joint. The left knee had a full, painless range of motion with no effusion or synovial hypertrophy. Roentgenograms of the knee and hand showed no evidence of active disease. Complement-fixation titers were positive at 1:4 dilutions; creatinine level was 0.8 serum mg/100 ml. Comment. -Although human coccidioidomycosis is highly variable in all its manifestations, spontaneous cure of an established extrapulmonary focus is unreported. Nonethe¬ less, it is hard to convincingly relate this patient's apparent cure to any of the modalities employed in his treat¬ ment. The ineffectiveness of ampho¬ tericin therapy had already been demonstrated. Synovectomy has been proposed as a logical treatment, but can never remove more than a portion of the diseased tissue and infectious organisms. In a large series of pa¬ tients with coccidioidomycotic arthri¬ tis, synovectomy with or without ad¬ ministration of amphotericin failed to control disease activity in the ma¬ jority of cases.2 We do not know whether cure of this case of disseminated coccidioidomycosis after seven years was in was
any way related to treatment. We be¬ lieve that this case offers an excellent example of the unpredictability of hu¬ man coccidioidal infection. It may also offer support to a previously stated hypothesis that mycosis in children may be more benign than disseminated mycosis in adults.3 WILLIAM G. WINTER, JR, MD Orthopedic Service Veterans Administration Hospital Lexington, KY 40506 ROGER K. LARSON, MD Orthopedic and Medical Departments Valley Medical Center Fresno, Calif 1. Ziering WH, Rockas HR: Coccidioidomy-
cosis: Long-term treatment with amphotericin B of disseminated disease in a 3-month-old baby. Am J Dis Child 108:454-459, 1964. 2. Winter WG, Larson RK, Masten M, et al: Coccidioidal arthritis and its treatment\p=m-\1974.J Bone Joint Surg, to be published. 3. Dykes J, Segesman JK, Birsner JW: Coccidioidomycosis of bone in children. Am J Dis Child 85:34-42, 1953.
Unusual Gastrointestinal Manifestations of
Henoch-Sch\l=o"\nleinPurpura The gastrointestinal manifestations of Henoch-Sch\l=o"\nleinpurpura are diverse and, in the absence of the diagnostic rash, can easily be confused with other conditions. The case presented below illustrates the capacity of this entity to masquerade as acute appendicitis and as acute Crohn disease.
Report of a Case.\p=m-\A6-year-old boy had a four-day history of periumbilical pain and vomiting. He had had an upper-respiratory tract infection the week prior to development of symptoms, and a temperature of 38.3 C (101 F) orally on the day prior to presentation. He had no history of
diarrhea, hematochezia, trauma,
or uridid he have a past history of weight loss, unexplained fevers, or substantial abdominal pain. Physical examination disclosed an enervated boy with decreased moisture of his buccal mucosa, normal skin turgor, a soft but diffusely tender abdomen with voluntary guarding in all four quadrants, bilateral rebound tenderness greater on the right, and a diffusely tender rectum. A guaiac-negative stool was obtained. Laboratory examination showed a white blood cell count of 13,700/cu mm with 72% polymorphonuclear leukocytes and 3% band forms, and a hemoglobin level of 12.9 gm/100 ml. Roentgenograms of the abdo¬ men and chest were normal, as were re¬ sults of urinalysis, urine and throat cul¬ tures, and serum electrolyte levels. The child underwent laparotomy that evening and five segments of inflamed, thickened, and edematous bowel were
nary tract
symptoms,
nor
found in the jejunum; the largest affected area was about 8 cm long, and another area 2 to 3 cm in length disclosed periserosal fat; six involved lymph nodes showed nonspecific adenitis on histological examination. To gross inspection, the ap¬ pendix, ileum, and ascending colon were uninvolved, and the postoperative diag¬ nosis was atypical acute regional enteritis. The patient had an uneventful postoper¬ ative course until one week later when he developed periumbilical pain and hemato¬ chezia. On proctoscopy, the rectal mucosa was studded with patchy, superficial ul¬ cérations with surrounding erythema and a central exúdate with normal intervening mucosal tissue; stool samples were nega¬ tive for bacterial pathogens, ova and para¬ sites, and amoebae. Rectal biopsy failed to show pathological changes. These symptoms resolved the following day and three days later the boy developed bilateral ankle swelling and petechiae over the anterior part of both tibiae and on the buttocks. All petechiae disappeared after four days and no hematuria or hyperten¬ sion developed on subsequent examina¬ tions. A series of roentgenograms of the upper-gastrointestinal tract and small bowel taken at the time of the skin erup¬ tion was completely normal.
Comment— This patient had ab¬ dominal pain, vomiting, and a leuko¬ cytosis, all of which can be found in acute appendicitis and in acute Crohn disease as well as in Henoch-Schön¬ lein purpura. However, the latter diagnosis could not be made in the ab¬ sence of the telltale rash. The review by Rodriguez-Erdmann and Levitan1 states that 14% of patients will show gastrointestinal features of anaphylactoid purpura before appearance of the rash. In this condition the bowel is edematous with submucosal and subserosal hemorrhages, while in acute Crohn disease the terminal part of the ileum is hyperemic and edema¬ tous.2 Furthermore, periserosal fat is usually seen in Crohn disease. Thus, one can see how at operation the bowel appearance could be mis¬ leading. Finally, although HenochSchönlein purpura has been reported to involve any part of the alimentary tract,3 rectal involvement is not com¬ mon and is more often seen with Crohn disease. A case similar to this in an adult was reported by Yentis.4 Philip Sunshine, MD, of Stanford University manuscript and aided in its prepa¬
reviewed the ration.
GORDON L. KLEIN, MD SAM STAFFORD III, MD Department of Pediatrics Naval Regional Medical Center Mountain Boulevard Oakland, CA 94627
Downloaded From: http://archpedi.jamanetwork.com/ by a University of British Columbia Library User on 06/17/2015
1. Rodriguez-Erdmann F, Levitan R: Gastrointestinal and roentgenologic manifestations of Henoch-Schonlein purpura. Gastroenterology
54:260-264, 1968.
2. Spiro HM: Clinical Gastroenterology. New York, Macmillan Co, 1970. 3. Handel J, Schwartz S: Gastrointestinal
manifestations of the Schonlein-Henoch syndrome: Roentgenologic findings. Am J Radiol 78:643-652, 1957. 4. Yentis I: Henoch-Schonlein purpura mimicking acute appendicitis and Crohn's disease. Br J Radiol 46:555-556, 1973.
Nutritional Supplementation in Fibrosis
Cystic
Sir.\p=m-\Thevalue of nutritional supplementation in cystic fibrosis (CF) has been raised in the February issue of
the Journal (129:165, 1975). The results of one year of "complete" dietary supplementation in patients with CF receiving enzyme replacement were described as promising, but "not definitive." When experimental and control groups were compared, the progression of changes in the chest roentgenograms was similar in both groups, and no significant difference was found in the rates of increase of height. Duke Medical Center and the National Institutes of Health have analyzed the maximum achieved heights
of 60 persons with CF 20 years old.1 Both parameters were markedly skewed toward the lower percentile bands (Figure). No significant differences were found among patients whose condition was diagnosed early in life, in those with and
weights
over
more severe
pulmonary
or
gastroin¬
testinal impairment, or among those in whom pancreatic enzyme and/or nutritional supplementation had been employed. Nutritional supplementa¬ tion has yet to be proven a significant long-term influence on the morbidity or mortality of CF. CLIFFORD W. LOBER, MD Department of Medicine Mayo Clinic Rochester, MN 55901 1. Lober CW, Wood RE, di Sant'Agnese PA, et al: Patterns of presentation of cystic fibrosis of the pancreas seen in patients over age 20. Presented at the 40th annual meeting of the American College of Chest Physicians, New Orleans, Nov 5,1974. 2. Vaughan VC III: Growth and development, in Nelson WE, Vaughan VC, McKay RJ (eds): Textbook of Pediatrics. Philadelphia, WB Saunders Co, 1969, p 15. 3. Montoye HJ, Epstein FH, Kjelsberg MO: The measurement of body fatness: A study in total community. Am J Clin Nutr 16:417-427,1965.
In Reply.\p=m-\Loberreported data on 60 persons with cystic fibrosis (CF) over 20 years of age, while our population
Percentile distribution of maximum achieved heights and weights of 60 persons over age 20 with cystic fibrosis. Percentiles determined by comparison with Iowa growth charts up to age 182; extrapolated beyond age 18 by comparison with figures published by Montoye et al.3
MAXIMUM ACHIEVED HEIGHTS AND WEIGHTS
of 63 individuals contained only three who were over 15 years old. The median age of death in patients with CF is 12 years. It is not realistic to compare patients who have survived to adulthood with a younger group. He did not state the type of nutritional supplementation that was of no significance among his patients. Our experience with nutritional supplements other than the amino acid\x=req-\ glucose polymer mixture has been equally poor. The diet of predigested nutrients was given to patients in our clinic whose clinical condition was severe and for whom the prognosis in terms of survival to age 20 was not good. Our data on growth rates were similar to those of Lober in that both heights and weights were skewed toward the lower end, and height was less affected than weight. Increased rates of linear growth were observed in eight of 13 children under 12 years of age at the time the dietary supple¬ ment was begun. These children had failed to grow in height during the previous year. Patients who continue to take the nutritional supplement have not shown further deterioration in pulmonary status. It is not likely that advanced pulmonary changes in patients with severe manifestations of the disease are reversible, but they might be prevented or minimized. Long-term studies of the effects of the predigested dietary supplement, begun early in life, should be under¬ taken to assess its value in decreasing morbidity and mortality in CF. HELEN K. BERRY, MA Institute for Developmental Research Children's Hospital Research Foundation Cincinnati, OH 45229 Lumbar Punctures and
1%
10% 25%
50% 75% 90% 99%
Percentile Band
100%
Meningitis
Sir.\p=m-\Itis hard for me to discern exactly what hypothesis Fischer et al were testing in their recent article, "Lumbar Punctures and Meningitis" (129:590-592, 1975). In their cases 1 through 3 there is no evidence that the lumbar puncture itself contributed in any way to the subsequent manifestation of meningitis in these bacteremic children. It is reasonable to expect that certain microorganisms, after seeding the bloodstream, may infect the meninges. In newborns, essentially every organism\p=m-\ Escherichia coli with capsular anti-
Downloaded From: http://archpedi.jamanetwork.com/ by a University of British Columbia Library User on 06/17/2015
any way related to treatment. We be¬ lieve that this case offers an excellent example of the unpredictability of hu¬ man coccidioidal infection. It may also offer support to a previously stated hypothesis that mycosis in children may be more benign than disseminated mycosis in adults.3 WILLIAM G. WINTER, JR, MD Orthopedic Service Veterans Administration Hospital Lexington, KY 40506 ROGER K. LARSON, MD Orthopedic and Medical Departments Valley Medical Center Fresno, Calif 1. Ziering WH, Rockas HR: Coccidioidomy-
cosis: Long-term treatment with amphotericin B of disseminated disease in a 3-month-old baby. Am J Dis Child 108:454-459, 1964. 2. Winter WG, Larson RK, Masten M, et al: Coccidioidal arthritis and its treatment\p=m-\1974.J Bone Joint Surg, to be published. 3. Dykes J, Segesman JK, Birsner JW: Coccidioidomycosis of bone in children. Am J Dis Child 85:34-42, 1953.
Unusual Gastrointestinal Manifestations of
Henoch-Sch\l=o"\nleinPurpura The gastrointestinal manifestations of Henoch-Sch\l=o"\nleinpurpura are diverse and, in the absence of the diagnostic rash, can easily be confused with other conditions. The case presented below illustrates the capacity of this entity to masquerade as acute appendicitis and as acute Crohn disease.
Report of a Case.\p=m-\A6-year-old boy had a four-day history of periumbilical pain and vomiting. He had had an upper-respiratory tract infection the week prior to development of symptoms, and a temperature of 38.3 C (101 F) orally on the day prior to presentation. He had no history of
diarrhea, hematochezia, trauma,
or uridid he have a past history of weight loss, unexplained fevers, or substantial abdominal pain. Physical examination disclosed an enervated boy with decreased moisture of his buccal mucosa, normal skin turgor, a soft but diffusely tender abdomen with voluntary guarding in all four quadrants, bilateral rebound tenderness greater on the right, and a diffusely tender rectum. A guaiac-negative stool was obtained. Laboratory examination showed a white blood cell count of 13,700/cu mm with 72% polymorphonuclear leukocytes and 3% band forms, and a hemoglobin level of 12.9 gm/100 ml. Roentgenograms of the abdo¬ men and chest were normal, as were re¬ sults of urinalysis, urine and throat cul¬ tures, and serum electrolyte levels. The child underwent laparotomy that evening and five segments of inflamed, thickened, and edematous bowel were
nary tract
symptoms,
nor
found in the jejunum; the largest affected area was about 8 cm long, and another area 2 to 3 cm in length disclosed periserosal fat; six involved lymph nodes showed nonspecific adenitis on histological examination. To gross inspection, the ap¬ pendix, ileum, and ascending colon were uninvolved, and the postoperative diag¬ nosis was atypical acute regional enteritis. The patient had an uneventful postoper¬ ative course until one week later when he developed periumbilical pain and hemato¬ chezia. On proctoscopy, the rectal mucosa was studded with patchy, superficial ul¬ cérations with surrounding erythema and a central exúdate with normal intervening mucosal tissue; stool samples were nega¬ tive for bacterial pathogens, ova and para¬ sites, and amoebae. Rectal biopsy failed to show pathological changes. These symptoms resolved the following day and three days later the boy developed bilateral ankle swelling and petechiae over the anterior part of both tibiae and on the buttocks. All petechiae disappeared after four days and no hematuria or hyperten¬ sion developed on subsequent examina¬ tions. A series of roentgenograms of the upper-gastrointestinal tract and small bowel taken at the time of the skin erup¬ tion was completely normal.
Comment— This patient had ab¬ dominal pain, vomiting, and a leuko¬ cytosis, all of which can be found in acute appendicitis and in acute Crohn disease as well as in Henoch-Schön¬ lein purpura. However, the latter diagnosis could not be made in the ab¬ sence of the telltale rash. The review by Rodriguez-Erdmann and Levitan1 states that 14% of patients will show gastrointestinal features of anaphylactoid purpura before appearance of the rash. In this condition the bowel is edematous with submucosal and subserosal hemorrhages, while in acute Crohn disease the terminal part of the ileum is hyperemic and edema¬ tous.2 Furthermore, periserosal fat is usually seen in Crohn disease. Thus, one can see how at operation the bowel appearance could be mis¬ leading. Finally, although HenochSchönlein purpura has been reported to involve any part of the alimentary tract,3 rectal involvement is not com¬ mon and is more often seen with Crohn disease. A case similar to this in an adult was reported by Yentis.4 Philip Sunshine, MD, of Stanford University manuscript and aided in its prepa¬
reviewed the ration.
GORDON L. KLEIN, MD SAM STAFFORD III, MD Department of Pediatrics Naval Regional Medical Center Mountain Boulevard Oakland, CA 94627
Downloaded From: http://archpedi.jamanetwork.com/ by a University of British Columbia Library User on 06/17/2015
1. Rodriguez-Erdmann F, Levitan R: Gastrointestinal and roentgenologic manifestations of Henoch-Schonlein purpura. Gastroenterology
54:260-264, 1968.
2. Spiro HM: Clinical Gastroenterology. New York, Macmillan Co, 1970. 3. Handel J, Schwartz S: Gastrointestinal
manifestations of the Schonlein-Henoch syndrome: Roentgenologic findings. Am J Radiol 78:643-652, 1957. 4. Yentis I: Henoch-Schonlein purpura mimicking acute appendicitis and Crohn's disease. Br J Radiol 46:555-556, 1973.
Nutritional Supplementation in Fibrosis
Cystic
Sir.\p=m-\Thevalue of nutritional supplementation in cystic fibrosis (CF) has been raised in the February issue of
the Journal (129:165, 1975). The results of one year of "complete" dietary supplementation in patients with CF receiving enzyme replacement were described as promising, but "not definitive." When experimental and control groups were compared, the progression of changes in the chest roentgenograms was similar in both groups, and no significant difference was found in the rates of increase of height. Duke Medical Center and the National Institutes of Health have analyzed the maximum achieved heights
of 60 persons with CF 20 years old.1 Both parameters were markedly skewed toward the lower percentile bands (Figure). No significant differences were found among patients whose condition was diagnosed early in life, in those with and
weights
over
more severe
pulmonary
or
gastroin¬
testinal impairment, or among those in whom pancreatic enzyme and/or nutritional supplementation had been employed. Nutritional supplementa¬ tion has yet to be proven a significant long-term influence on the morbidity or mortality of CF. CLIFFORD W. LOBER, MD Department of Medicine Mayo Clinic Rochester, MN 55901 1. Lober CW, Wood RE, di Sant'Agnese PA, et al: Patterns of presentation of cystic fibrosis of the pancreas seen in patients over age 20. Presented at the 40th annual meeting of the American College of Chest Physicians, New Orleans, Nov 5,1974. 2. Vaughan VC III: Growth and development, in Nelson WE, Vaughan VC, McKay RJ (eds): Textbook of Pediatrics. Philadelphia, WB Saunders Co, 1969, p 15. 3. Montoye HJ, Epstein FH, Kjelsberg MO: The measurement of body fatness: A study in total community. Am J Clin Nutr 16:417-427,1965.
In Reply.\p=m-\Loberreported data on 60 persons with cystic fibrosis (CF) over 20 years of age, while our population
Percentile distribution of maximum achieved heights and weights of 60 persons over age 20 with cystic fibrosis. Percentiles determined by comparison with Iowa growth charts up to age 182; extrapolated beyond age 18 by comparison with figures published by Montoye et al.3
MAXIMUM ACHIEVED HEIGHTS AND WEIGHTS
of 63 individuals contained only three who were over 15 years old. The median age of death in patients with CF is 12 years. It is not realistic to compare patients who have survived to adulthood with a younger group. He did not state the type of nutritional supplementation that was of no significance among his patients. Our experience with nutritional supplements other than the amino acid\x=req-\ glucose polymer mixture has been equally poor. The diet of predigested nutrients was given to patients in our clinic whose clinical condition was severe and for whom the prognosis in terms of survival to age 20 was not good. Our data on growth rates were similar to those of Lober in that both heights and weights were skewed toward the lower end, and height was less affected than weight. Increased rates of linear growth were observed in eight of 13 children under 12 years of age at the time the dietary supple¬ ment was begun. These children had failed to grow in height during the previous year. Patients who continue to take the nutritional supplement have not shown further deterioration in pulmonary status. It is not likely that advanced pulmonary changes in patients with severe manifestations of the disease are reversible, but they might be prevented or minimized. Long-term studies of the effects of the predigested dietary supplement, begun early in life, should be under¬ taken to assess its value in decreasing morbidity and mortality in CF. HELEN K. BERRY, MA Institute for Developmental Research Children's Hospital Research Foundation Cincinnati, OH 45229 Lumbar Punctures and
1%
10% 25%
50% 75% 90% 99%
Percentile Band
100%
Meningitis
Sir.\p=m-\Itis hard for me to discern exactly what hypothesis Fischer et al were testing in their recent article, "Lumbar Punctures and Meningitis" (129:590-592, 1975). In their cases 1 through 3 there is no evidence that the lumbar puncture itself contributed in any way to the subsequent manifestation of meningitis in these bacteremic children. It is reasonable to expect that certain microorganisms, after seeding the bloodstream, may infect the meninges. In newborns, essentially every organism\p=m-\ Escherichia coli with capsular anti-
Downloaded From: http://archpedi.jamanetwork.com/ by a University of British Columbia Library User on 06/17/2015
