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The Shaken Baby Syndrome Myth renamed "Abusive Head Trauma" or "Non-Accidental Injury"
1. SBS
"MYTH" WEBSITE SUMMARY SUBJECT: Infantile Scurvy Articles (Barlow's disease) Infantile Scurvy Articles (Barlow's disease) "During
one trial, the prosecution stated that infantile scurvy was no longer
Metabolic
rib injuries Munch Med Wochenschr.
1968 Mar 1;110(9):535-40.Links Scherzer
E. Arch Fr Pediatr. 1982 Aug-Sep;39(7):453-4.Links Bachy A, Hendrickx E, Vandercam J, Gérard P, Dochain J. Pediatr
Radiol. 1976 Aug 20;4(4):251-3.Links An
Esp Pediatr. 1991 Mar;34(3):243-6.Links Burches Greus E, Lecuona López C, Ardit Lucas J, Aguilar Bacallado F, García Vicent C, Tomás Ratés C, Alvarez Angel V, Donderis Folgado P. Servicio de Pediatría, Hospital General Universitario de Valencia. Ryoikibetsu
Shokogun Shirizu. 1998;(21 Pt 2):405-7.Links Mase K. East
Mediterr Health J. 2001 Nov;7(6):1070-2.Links Kerman University of Medical Sciences Kerman Darman Hospital, Kerman, Islamic Republic of Iran. Ann
Dermatol Venereol. 2000 May;127(5):510-2. Boulinguez S, Bouyssou-Gauthier M, De Vençay P, Bedane C, Bonnetblanc J. Service de Dermatologie, CHU Dupuytren, 87042 Limoges Cedex. INTRODUCTION: The risk of vitamin C deficiency is underestimated in industrialized countries and is only disclosed in rare cases of severe scurvy. CASE REPORT: We report three cases of scurvy presenting with ecchymotic purpura and hemorrhagic ulcerations of the lower limbs. Vitamin C supplementation led to rapid improvement of the skin lesions. DISCUSSION: Clinical diagnosis of low-grade deficiency can be difficult. Biological diagnosis requires special care in sample taking and transport. Turk
J Pediatr. 1998 Apr-Jun;40(2):249-53.Links Department of Pediatrics, Dr. Sami Ulus Children's Hospital, Ankara, Turkey. An 18-month-old girl presented with irritability, epistaxis, spongy appearance of the gums perifollicular papules with follicular hyperkeratosis, ecchymosis, painful swollen knees and scorbutic rosary. Her diet consisted mainly of wheat flour. X-ray of the knees showed findings compatible with scurvy. Ascorbic acid level was below 0.003 g/L. Ascorbic acid therapy resulted in a dramatic clinical improvement. Scurvy is an uncommon disease in our society today. It is important to recognize the signs and symptoms of scurvy because it is easily treated with vitamin C replacement. Int
J Dermatol. 2007 Feb;46(2):194-8. We report
a 10-month-old boy with inflammatory and necrotic gingival lesions,
fever, irritability, and pseudoparalysis of the legs. Laboratory examinations
revealed moderate anemia and skeletal X-rays showed osteopenia, scorbutic
rosary at the costochondral junctions, and "corner sign"
on the proximal metaphyses of the femora. The boy had been fed only
with diluted cow's milk. He had never taken solid food, vitamin C,
or iron complement. Seventy-two hours after starting oral vitamin
C supplementation, there was significant improvement in the patient's
gingival lesions and general health. The clinical presentation and
laboratory and imaging findings, together with the dramatic response
to ascorbic acid intake, allowed us to confirm the diagnosis of infantile
scurvy. Scurvy, a dietary disease due to the deficient intake of vitamin
C, is uncommon in the pediatric population. In an infant who has never
received vitamin C, the combination of gingival lesions, pseudoparalysis,
and irritability strongly suggests a diagnosis of scurvy. The clinical
picture, together with the laboratory data, radiological studies,
and therapeutic response to vitamin C administration, confirmed the
diagnosis. Comment
in: Am
J Clin Dermatol. 2007;8(2):103-6.Links Pediatric Dermatology, University of Miami, Miami, Florida 33125, USA. cynthiajburk@yahoo.com Ascorbic acid (vitamin C) is necessary for the formation of collagen, reducing free radicals, and aiding in iron absorption. Scurvy, a disease of dietary ascorbic acid deficiency, is uncommon today. Indeed, implementation of dietary recommendations largely eradicated infantile scurvy in the US in the early 1900s. We present a case of an otherwise healthy 2-year-old Caucasian girl who presented with refusal to walk secondary to pain in her lower extremities, generalized irritability, sleep disturbance, and malaise. The girl's parents described feeding the patient an organic diet recommended by the Church of Scientology that included a boiled mixture of organic whole milk, barley, and corn syrup devoid of fruits and vegetables. Physical examination revealed pale, bloated skin with edematous, violaceous gums and loosening of a few of her teeth. Dermatologic findings included xerosis, multiple scattered ecchymoses of the extremities, and perifollicular hemorrhage. Laboratory and radiographic evaluation confirmed the diagnosis of scurvy. The patient showed dramatic improvement after only 3 days of treatment with oral ascorbic acid and significant dietary modification. In this case report, we revisit the old diagnosis of scurvy with a modern dietary twist secondary to religious practices. This case highlights the importance of taking a detailed dietary history when evaluating diseases involving the skin. Rev
Clin Esp. 1996 Aug;196(8):570.Links Rodríguez Cuartero A. Pediatrics.
2001 Sep;108(3):E55. Department of Paediatrics, Hospital for Sick Children and University of Toronto, Toronto, Canada. michael.weinstein@sickkids.on.ca Scurvy has been known since ancient times, but the discovery of the link between the dietary deficiency of ascorbic acid and scurvy has dramatically reduced its incidence over the past half-century. Sporadic reports of scurvy still occur, primarily in elderly, isolated individuals with alcoholism. The incidence of scurvy in the pediatric population is very uncommon, and it is usually seen in children with severely restricted diets attributable to psychiatric or developmental problems. The condition is characterized by perifollicular petechiae and bruising, gingival inflammation and bleeding, and, in children, bone disease. We describe a case of scurvy in a 9-year-old developmentally delayed girl who had a diet markedly deficient in vitamin C resulting from extremely limited food preferences. She presented with debilitating bone pain, inflammatory gingival disease, perifollicular hyperkeratosis, and purpura. Severe hypertension without another apparent secondary cause was also present, which has been previously undescribed. The signs of scurvy and hypertension resolved after treatment with vitamin C. The diagnosis of scurvy is made on clinical and radiographic grounds, and may be supported by finding reduced levels of vitamin C in serum or buffy-coat leukocytes. The response to vitamin C is dramatic. Clinicians should be aware of this potentially fatal but easily curable condition that is still occasionally encountered among children. Radiologic
diagnosis of Moller-Barlow disease (scurvy). Apropos of a case] Abstract
[A case of purpura, or the return to natural diseases] Scurvy:
a forgotten disease. Abstract
A case of scurvy presenting with cutaneous and articular signs. Tolone
C, Santinelli R, Salvi V, Toraldo R, Di Lena C, D'Avanzo M. Wang YZ. Brickley
M, Ives R. Ramar S,
Sivaramakrishnan V, Manoharan K. De DC,
Bhattacharyya AK. Monatsschr
Kinderheilkd. 1984 Apr;132(4):240-1. Infantile scurvy, today a rare disease, can be diagnosed either by recognition of a characteristic constellation of clinical features, or on the correct interpretation of nearly pathognomonic radiological signs. Dystrophy, recurrent infections, fever, anemia, hemorrhagic effusions, hematuria, tenderness of the legs, pseudo-paralysis and "frog position" and costochondral swelling or angulation are frequent signs. Radiologically, thickening of the epiphyseal plate ("scurvy line") and subepiphyseal rarefication , general osteoporosis and subperiostal hemorrhage are important diagnostic clues. Arch
Pediatr Adolesc Med. 2001 May;155(5):607-8. Med
J Aust. 1970 Jan 24;1(4):187. Pediatr
Ann. 1983 Dec;12(12):894-903.Links An
Esp Pediatr. 1991 Mar;34(3):243-6.Links Burches Greus E, Lecuona López C, Ardit Lucas J, Aguilar Bacallado F, García Vicent C, Tomás Ratés C, Alvarez Angel Donderis Folgado P. Servicio
de Pediatría, Hospital General Universitario de Valencia. Z
Arztl Fortbild (Jena). 1966 Nov 15;60(21):1221-3.Links Int
Z Vitaminforsch Beih. 1957;27(3):265-74. : Pediatr Int. 2002 Feb;44(1):37-42.Click here to read Links Comment
in: Unexplained
subdural hematoma in young children: is it always child abuse? Department of Paediatrics, Faculty of Medicine, 6/F Clinical Science Building, Prince of Wales Hospital, The Chinese University of Hong Kong, Shatin, Hong Kong. b300875@mailserv.cuhk.edu.hk BACKGROUND:
In the published reports of the developed society, subdural hematoma
and/or retinal hemorrhages, in the absence of documented history of
major trauma, should be considered diagnostic of child abuse. Many
people used the above criteria for diagnosis, but subsequently found
that retinal hemorrhages were more common in non-accidental injuries
(NAI). To what extent is the proposed pathognomonic association between
unexplained subdural hematoma/retinal hemorrhages and child abuse
a self-fulfilling prophecy? METHODS: Clinical details of nine
children under 2 years with unexplained subdural hematoma admitted
to Prince of Wales Hospital between 1995 and 1998 were reviewed.
RESULTS: Four had no other physical signs of
injury, five
had retinal hemorrhages and
one had multiple bruises over the body.
Following multidisciplinary case conferences for seven children, a
diagnosis of NAI was concluded in four cases, but
in no case could the abuser be definitely identified. Clinical
outcome was poor with seven children showing either profound disability
(n = 5) or evidence of developmental delay (n = 2). CONCLUSION: In
this series, NAI were not established in three of the seven cases.
Did we underdiagnose child abuse in these cases? Despite a
magnitude of opinion to the contrary, the issue of whether "trivial"
head injury can cause subdural hemorrhages and/or retinal hemorrhages
is yet unresolved. Clearly much more information on this
very sensitive and serious issue is required and these data should
be collected with an open mind. [Vitamin
C deficiency--scurvy and Moeller-Barlow's disease] Eur
J Pediatr. 2004 Jul;163(7):420-1. Epub 2004 Apr 9.Click here
to read Links Eye Department, Guys Hospital, London, UK. J
R Soc Med. 2000 Nov;93(11):591-2. Department of Ophthalmology, Great Ormond Street Hospital for Children, Great Ormond Street, London WC1N 3JH, UK. isabelle-re@dial.pipex.com Clin
Pediatr (Phila). 1966 Apr;5(4):230-7. Winckelmann
G. Thromb Diath Haemorrh Suppl. 1968;30:145-5. Winckelmann
G.Dtsch Zahnarztl Z. 1968 Dec;23(12):1422-4.Links Spier
JP. Br Med J (Clin Res Ed). 1983 Dec 17;287(6408):1862-3. Med
Arts Sci. 1967 Jan-Apr;21(1):9-22.Links Spier JP.
[Infantile
scurvy, Barlow's disease.] 1: Pol
Przegl Radiol. 1956 Jul-Aug;20(4):217-22. POLANSKA B. PMID: 13388806 [PubMed - indexed for MEDLINE] : Cah Coll
Med Hop Paris. 1968 Feb;9(2):188-94.Links Blancher G. PMID: 5745278 [PubMed - indexed for MEDLINE] 1: Pediatr
Radiol. 1976 Feb 13;4(2):122-3. A five year old girl presented with short femora, cupped distal metaphyses and flexion deformity of the knees suggesting previous epiphyseo-metaphyseal damage. The aetiology was suggested by finding a history of previous infantile scurvy with separated distal femoral epiphyses. The rarity of this complication and possible contributing factors are discussed. PMID: 967570 [PubMed - indexed for MEDLINE] 1: Indian
Pediatr. 1999 Oct;36(10):1067 PMID: 10745326 [PubMed - indexed for MEDLINE] 1: Pol
Przegl Radiol. 1956 Jul-Aug;20(4):217-22.Links POLANSKA B. PMID: 13388806 [PubMed - indexed for MEDLINE] RIBS Mase K.
1: Am
J Phys Anthropol. 2006 Feb;129(2):163-72. Institute of Archaeology and Antiquity, School of Historical Studies, University of Birmingham, Edgbaston, Birmingham B15 2TT, UK. M.B.Brickley@bham.ac.uk Recent investigations of human skeletal material from the historic St. Martin's cemetery, England, found a range of abnormal lesions in six infants that are almost certainly related to scurvy. Porous and proliferative bone lesions affecting the cranial bones and scapulae were found, and this paper presents images obtained using both macroscopic and scanning electron microscope examination of the lesions. Previous work on infantile scurvy (Ortner et al., 1997-2001) relied heavily on changes at the sphenoid, which is often missing in archaeological bone, so the identification of changes attributable to scurvy on other cranial bones and the scapulae is encouraging. The ability to recognize changes related to scurvy on a range of bones will ensure an enhanced potential for recognition of this disease in future research involving archaeological bone. Research on historical documents from Birmingham dating to the eighteenth and nineteenth centuries, combined with the probable cases of scurvy identified, supports the view that the paucity of cases of infantile scurvy from the archaeological record reflects a lack of understanding and recognition of bone manifestations, rather than a lack of occurrence in this period. Changes linked to scurvy were only found in infants from the poorer sections of the community from St. Martin's, and this is almost certainly linked to patterns of food consumption and may be related to shortages of potatoes, due to blight, experienced during this period. PMID: 16323179 [PubMed - indexed for MEDLINE] 1: Istanbul Tip Fak Mecmuasi. 1959;22:186-208. Related Articles, Links [Severe scurvy (Moeller-Barlow disease) associated with a bone disorder.] [Article in Turkish] TUMAY SB. http://archpedi.ama-assn.org/cgi/content/full/155/5/607 (go to external site for photos) Vol. 155
No. 5, May 2001 TABLE OF CONTENTS Felix G.
Riepe, MD; Dirk Eichmann, MD; Hans C. Oppermann, MD; Heniz J. Schmitt,
MD; Walter W. Tunnessen, Jr, MD 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.
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).
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.
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.
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
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.
1. Yilmaz
S, Karademir S, Ertan U, et al. Scurvy: a case report. Turk J Pediatr.
1998;40:249-253. ISI | PUBMED SECTION EDITOR: WALTER W. TUNNESSEN, JR, MD THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES Vitamin
C Deficiency and Depletion in the United States: The Third National
Health and Nutrition Examination Survey, 1988 to 1994 Dianne Jacobs Thompson Est. 2007 Also http://truthquest2.com (alternative medicine featuring drugless cancer treatments) Author publication: NEXUS MAGAZINE "Seawater--A Safe Blood Plasma Substitute?"
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