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Picture of the Month
Felix G. Riepe, MD;
Dirk Eichmann, MD;
Hans C. Oppermann, MD;
Heniz J. Schmitt, MD;
Walter W. Tunnessen, Jr, MD
From the Departments of Pediatrics (Drs Riepe, Eichmann, and Schmitt)
and Diagnostic Radiology (Dr Opperman), Faculty of Medicine, Christian Albrechts
University Kiel, Kiel, Germany; and the American Board of Pediatrics, Chapel
Hill, NC (Dr Tunnessen).
Arch Pediatr Adolesc Med. 2001;155:607-608.
A 15-MONTH-OLD boy had a history of unexplained bleeding from his gums
for several weeks and fever for 2 days. He had been fed only cow's milk and
oatmeal since age 4 months. On physical examination he had almost no spontaneous
movement. His legs were held in a "frog leg" position (Figure 1), were swollen along the long bones, and were tender to
palpation. His skin was dry and pale. Hemorrhages of the gingiva were obvious
as were 2 blood-filled cysts of the lower canine teeth (Figure 2). The tympanic membranes were hyperemic, and evidence of
middle-ear fluid was present. Palpable prominence of the costochondral junctions
of the chest wall was noted.
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Figure 1.
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Figure 2.
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The results of laboratory examinations revealed a normal white blood
cell count, a hemoglobin level of 76 g/L, and a platelet count of 334 x
109/L. The serum calcium, phosphorous, copper, and alkaline phosphatase
levels were normal. Thyroid stimulating hormone, triiodothyronine, and thyroxine
levels were also normal. The serum level of vitamin D was normal, but vitamin
C levels were low, 28 µmol/L (reference range, 45-108 µmol/L).
Chest x-ray film showed a scorbutic rosary at the costochondral junctions
with a "corner" sign noted in the proximal metaphysis of the humerus. Lower
extremity radiographs demonstrated abnormalities (Figure 3).
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Figure 3.
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Denouement and Discussion: Infantile Scurvy
Figure 1. The infant initiated
little spontaneous movement and held his legs slightly flexed and abducted.
Figure 2. The gingiva are spongy
and bleeding with blood-filled cysts in both lower canine teeth.
Figure 3. A radiograph of the
right lower extremity demonstrates osteoporosis with a "corner" sign at the
distal femur and rings of ossification around the proximal tibia epiphysis
and distal femoral epiphysis.
Infantile scurvy (Moeller-Barlow disease) is rarely reported in developed
countries except in association with global malnutrition.1, 2, 3
The rarity of occurrence is responsible for the frequent delayed recognition
of this disorder. A deficiency of vitamin C (ascorbic acid) is responsible
for the manifestations of scurvy. Vitamin C is a cofactor for numerous enzymes
that are critical to several body functions. The most common manifestations
of scurvy are due to the decreased production and increased fragility of collagen,
a result of faulty hydroxylation of proline and lysine in forming a precise
triple-helical collagen structure.4, 5
Vitamin C also plays a number of roles in hematopoiesis, including the promotion
of iron absorption and as a cofactor for the conversion of folic acid to folinic
acid. Humans cannot synthesize vitamin C and must rely on intestinal absorption
from ingested nutrients.
CLINICAL MANIFESTATIONS
Most cases of scurvy are seen between ages 6 and 24 months. Neonatal
scurvy is rare, except in infants of mothers with extreme hypovitaminosis
C.6, 7 Breast milk contains
sufficient amounts of vitamin C unless the mother's diet is deficient in this
vitamin.8 The first clinical manifestations
of scurvy are often associated with acute febrile illnesses that seem to increase
the need for vitamin C.
Initial manifestations of scurvy are vague and include irritability,
decreased appetite, and delayed development. As effects of vitamin C deficiency
progress, affected children lie still with little movement because of generalized
tenderness, most apparent in bones as a result of subperiosteal hemorrhages.
Swelling may be noted along the shafts of long bones. Pseudoparalysis may
be apparent as a result of the bone pain. Infants often hold their legs in
a "frog leg" position and dislike being handled, often refusing to walk.
Gingival hemorrhages may occur along with spongy, hemorrhagic swellings
of the mucous membranes overlaying teeth. Petechial hemorrhages may occur
in the skin spontaneously or, more commonly, below the site of tourniquet
application, the Rumpel-Leede sign, as a manifestation of capillary fragility.
Swelling may be palpated along the costochondral junctions of the rib cage,
resembling the rosary seen in rickets.
RADIOGRAPHIC FINDINGS
Changes in the long bones, particularly around the knee, are most diagnostic
of scurvy. The earliest finding is generalized demineralization with a ground-glass
appearance to the bones. The cortex is thinned. A white line, so-called Fränkel
sign, is apparent at the ends of metaphyses, representing widening of the
provisional zones of calcification and its increased density. The epiphyseal
centers show central rarefaction and are also surrounded by a white line of
calcification, referred to as the Wimberger ring or halo-ossification center.
Transverse bands of diminished density adjacent to the Fränkel sign are
known as scurvy lines. Lateral metaphyseal spurs (corner sign) between the
provisional zone of calcification and the cortex are the result of metaphyseal
infarctions. The corner sign is more pathognomonic of scurvy than the other
bony findings. Irregular calcification and widening of the costochondral junctions
results in the scorbutic rosary. Periosteal hemorrhages cannot be detected
on radiography in scurvy until they become calcified during healing.
DIFFERENTIAL DIAGNOSIS, DIAGNOSIS, AND TREATMENT
Bleeding manifestations and bone pain may suggest acute leukemia. Bone
pain and refusal to walk may lead to consideration of osteomyelitis, septic
arthritis, and rheumatic disorders.
The diagnosis of scurvy is based on a combination of clinical and radiographic
findings. A dietary history compatible with insufficient intake of vitamin
C should be present. Accurate laboratory measurement of vitamin C levels is
difficult because of the instability of vitamin C.
Healing occurs rapidly with the oral administration of 100 to 200 mg/d
of vitamin C. As healing occurs, the intake of vitamin C may be reduced to
50 mg/d until complete clinical and radiologic resolution has taken place.9
AUTHOR INFORMATION
Accepted for publication January 14, 2000.
Reprints: Felix G. Riepe, MD, Klinikum der Christian-Albrechts Universitit,
Klinik far Allgemeine Pädiatrie, Schwanenweg 20, 24105 Kiel, Germany.
REFERENCES
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1. Yilmaz S, Karademir S, Ertan U, et al. Scurvy: a case report. Turk J Pediatr. 1998;40:249-253.
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2. Najera-Martinez P, Rodriquez-Collado A, Gorian-Maldonado E. Scurvy: a study of 13 cases. Bol Med Hosp Infant Mex. 1992;49:280-285.
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3. Young LW, Schiliro G, Russo A. Radiological case of the month: scurvy: almost historic, but not quite. AJDC. 1979;133:323-324.
4. Gershoff SN. Vitamin C (ascorbic acid): new roles, new requirements? Nutr Rev. 1993;51:313-326.
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5. Halliwell B. Ascorbic acid: hype, hoax, or healer [editorial]? Am J Clin Nutr. 1997;65:1891-1892.
FREE FULL TEXT
6. Clow CL, Laberge C, Scriver CR. Neonatal hypertyrosinemia and evidence for deficiency of ascorbic acid
in arctic and subarctic peoples. CMAJ. 1975;113:624-626.
ABSTRACT
7. Gómez DB, Warmann S, Scholl S, Glüer S. Neonatal scurvy [in German]. Monatsschr Kinderheilkd. 1999;147:570-572.
FULL TEXT
8. Macy IG. Composition of human colostrum and milk. AJDC. 1949;78:589-594.
9. Moran JR, Greene HL. The B vitamins and vitamin C in human nutrition. AJDC. 1979;133:308-314.
SECTION EDITOR: WALTER W. TUNNESSEN, JR, MD
THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES
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