ABNORMALITIES
Multiple Exostoses-Mental Retardation
Syndrome (Ale-Calo or M.E.M.R. Syndrome) Description of Two Childhood Cases K.
Kozlowski, Doc. Dr. Med., G. Harrington, M.B., B.S., F.R.A.C.R., A. Barylak, Lek. Med., B. Bartoszewica, Dr. Med.
) HIS
PAPER DESCRIBES two unrelated children with the unique clinical and radiographic features of the rare Ale-Calol or Giedion2~ coined the M.E.M.R. syndrme. name multiple exostoses mental retardation syndrome (M.E.M.R. syndrome). Hall et al.5 presented seven cases at the Fifth Conference of Birth Defects in Baltimore (1972) as the
Langer-Giedion syndrome. Case
Descriptions
Case 1. An eight-year-old girl was admitted to the Prince of Wales Hospital in Sydney. The family had no history of bone disease or mental retardation, and the pregnancy and delivery had been un-
eventful. She was first seen
at the age of two years when multiple malformations were noted. These included lop ears, a bulbous nose. a ~4~ide philt;-um with a median raphe, short columella, full upper lip with inverted margin, absent infraspinatus of the
muscles, umbilical hernia, hyperextensibility fingers, and mental retardation.
Presented at the 2nd Asian and Oceanian Congress Radiology, Manila, The Philippines November 10-14, 1975. From the Department of Radiology, Royal Alexandra Hospital for Children, Sydney, Australia, Department of Sydney, Australia, Radiology Prince of Wales and Department of Radiology, Pediatric Institute of the Medical Academy, Krakow, Poland.
of
Hospital,
During subsequent admissions to the hospital. bilateral urcteral renux was demonstrated and plastic surgery to her tip. nose, and ears was
performed.
Examination at age eight years repeated a healthyy looking girt with peculiar fades (Figs. 1A and l B). Both her height and weight were below the 10th
percentile.
Her shoulder musculature
appeared
scapulae, and she had marked genu vatgum. Her left leg was 2 cm longer than her right leg. She had diminished hearing, difficult to assess because of mental retardation... The otc>Ic>~ic examination showed bulging tympanic mem-
deficient, vith
&dquo;winging&dquo;
branes. Bilateral
of the
myringotomies
were
performed;
secretions were noted. Her development was estimated at being about 15 months behind her chronologic age. Routine blood and urine findings, serum electrolytes, serum alkaline phosphatase, urea, creatinine and serum proteins were normal, as were chromosomal count and E.M.G. R~rdi~~raphic:all~,~: she had multiple large exostoses with secondary deformities, multiple ~piph~&dquo;seal dysplasia and cone-shaped epiphyses in the hands and feet (Figs. 1 C-l E). The multiple exostoses involved the knee joint, upper shaft of the humeri, distal shafts of each radius and ulna, some of the tubular bones of the hands and feet, and a number of ribs. The epiphyseal dysplasia was characterized by hypoplasia and flattening of the epiphyses of the long and tubular bones and small round bones. The changes were no
219
Downloaded from cpj.sagepub.com at Bibliothekssystem der Universitaet Giessen on May 13, 2015
.
l~’i(.s. cxostoses
,l :~- l I3. Multiple mental retarda-
tion syndrome, eiglit-j=earold girl. Peculiar face (after phstic surgery) and lop ears. Shortening of the left leg. Valgus deFormity at the knees and winged
scapulae.
marked
the hips. Cone-shaped or dense present in the phalanges «f’ the hands and feet. The bone age of the hands and feet was retarded. The chest was narrow superiorly with narrow, oblique ribs. The thoracic vertebral bodies showed minimal change in shape and a corona! cleft of T-I1 was present. The skull showed some hypertelorism. The intravenous most
epiphyses
at
were
was normal. Case 2. This 13-year-old boy was admitted to the Pediatric Institute of the Medical Academy in Krakow, Poland in November, 1974 with multiple congenital abnormalities and mental retardation (Figs. 2A and 2B). He was the first child of healthy parents. His mother had been 26 years old, his father 21 years c~id; at the time of delivery. Two younger sisters and one younger brother are normal, Pregnancy and delivery were uneventful. The family had no history of mental retardation or bone disease. He started to talk at the age of four years. His
pydogram
development progressed slowly, according
to
parents. At admission, his weight was cm; head circumference 48
I 1 7 .j
20 kg.; height
his
chest circumHe was coopera-
cm;
ference 57 cnii arm span 117 cm. tive, He had large low set ears, scanty hair, slight proptosis, more marked on the lef’t. He had a bulbous nose, a long phikrum and a receding chin. His trunk was unusually short and he had marked lordosis of the lunibosacral spite. Winged scapulae were present bifateralfy’. There was vagus deformity of the knees, more marked on the left. He had thickening and deformities of the middle phalanf;eal joints of the 2nd to 4th fingers. Exostoses were seen, along the long bones and ribs. His muscle mass was decreased but the neuroogic examination showed no abnormality. He had increased mobility at the joints. The ophihalmologic examination showed proptosis. The dermatogly~bics were abnormal. With the ~~’.I:S.G; test, he showed normal
220
Downloaded from cpj.sagepub.com at Bibliothekssystem der Universitaet Giessen on May 13, 2015
a decreased verbal score. His social dcx.eipnicni. according to the Don scate, was about Six years. Radiographically. he had nT~lltipEe ~~t~stt3~es u-itla
with
performance Full-scale
LO.
was
secondary deformities, multiple epiphysea! dysand plasia, and cone-shaped epiphyses in the hands feet (Figs. 2(~-2H). Secondary deformities at the knee and zla7kle joints with ~fat3t-tenii~~; of some bones were observed. Onlv one exostosis was seen
tlie ribs but severat were palpable at the costochondral junctions. Small exostoses were noted at the growing ends of the shafts ot the illE3lllitT’ bones. was characterized by The el3il>!a~-seul ctva}ala:~iu ot the hypoptasia and flattening of the epiphyses bones. small round and bones tnbt))ar and long
in
were most marked at the proximai femoral and distal tibíalepiphyses. The proximal humeral epiphyses iaere &dquo;4tlrarc>st normal... Coneshaped or dense epiphyses were present in the phalanges .of the hands and feet. The bone age out the hands and feet was retarded. The vertebra) cotumn showed a saginaictett vertebrae of -6, and minima) shape changes in some other thoracic vertebrae. The iower thoracic vertebra! bodies were pear-shaped. The ossthcation centers of the annuiar cartiteginous rings were absent. The skull was normal.
The changes
Comments
‘
‘
Two other disorders from this condition.
.
be differentiated The Tricho-Rhino.
must
Fm. I C.
Mukipte exosMses at the end of the long bones.
FIG. 1 D. Cone-shaped epiphyses are best demonstrated at the proximal phalanges of the second to
fingers. Sclerotic epiphyses in some of the distal phalanges. Exostoses at the distal end of the right
fifth
radius.
221
Downloaded from cpj.sagepub.com at Bibliothekssystem der Universitaet Giessen on May 13, 2015
FtG. I E.
&dquo;
Paralytic chest. Hypoptastic upper ribs are
of)]It]Lle ill 1-)osltl()i). Exostosesattheproximaifett humeral metaphyses.
Ft(;s, 2A-2B.
Multiple
mental retardation syndrome, 13-yearold boy. Peculiar face, scanty hair, and lop ears.
Hyperiordosisandwinged Deformities of the limbs with demonstrable exostoses. Small genitalia.
scapulae.
Downloaded from cpj.sagepub.com at Bibliothekssystem der Universitaet Giessen on May 13, 2015
F)C. 2E. Cone-shaped epiphyses are best demonstrated the prc~xitnat and middle phatac~ges. Szlerc>tic ’ epiphyses of the distal phalanges of the 5th fingers. at the metaphyses of some of the Exostoiic prolii’eratins tubular bones. A large exostosis deforms the distal end of the left radius. Small carpal bones. at
described by Klingmiller and Gicdion,2 consists of thin, slow
Phalangeale syndrome, Ftc. 2C. Mutuptc cxc~stc~ses at the end of the shafts of the long bones ~vith secondary deformities. Hypoptastic epiphyses. _
growing hair, a pear-shaped nose with high philtrum, lop ears, brachyphalangy with
FIG. 2D. Hypc~plastic; irregularly ossified and flat-
tened femoral epiphyses. Shortened and widened femoral necks. Small pelvis.
223
Downloaded from cpj.sagepub.com at Bibliothekssystem der Universitaet Giessen on May 13, 2015
deformities of the
shaped epiphyses.
and
fingers,
Slow mental
wedgeelevel«pr~3ent
is often present. About 80 instances of this entity have been reported Dominant and recessive forms apparently occur, although some authors suggest that only one type and one mode of inheritance exists-the autosomal dominant with variables expressivity.
Nfultiple
(similar
to
the Tricho-Rhino-Phaiangcaie muscular hypoplasia, with hyper-
syndrome), extensibiHty at
the
exostoses, and
epiphyseal dN-splasi~t.
~eripyerai a~~ints, mtt~tiple
References 1. Ale, G., and Calo, S.: Su di
caso di disostosi Peri-
un
ferica associata con esostosi osteogeniche
multiple
ed iposomia disuniforme e disarmonica. Radiol. Diagn. (Bologna) 34: 376, 1961.
exostoses
represents a common skeletal disorder characterized by multiple osteochondromas, especially in the long bones. Radial head dislocation, N’I>idelung’s deformity, skeletal fusion, other deformities, and n~ali~naz~t degeneration are not uncommon complications. About two-thirds of the patients have a similarly affected parent. The disease is inherited in about half the children of an affected parent. The most important signs of the Ale-Calo or M.E.M.R. syndrome in our two patients are mental retardation, microcephaly, shortened stature with asymmetry, peculiL~r facies
Ann.
2. Giedion, A.: Das Tricho-Rhino-Phalangeale syndrom. Helv. 21: Paediatr. Acta. 475, 1966. 3. —: Acrodysplasias. Progr. Pediatr. Radiol. Vol. 4. From Intrinsic Diseases of Bones. Karger.
Basel, 1973, pp. 325-43.
4. —: Personal communication (1974). 5. Hall, B. D., Langer, L. O., Giedion, A., Smith, D. W., Cohen, M. M., Beals, R. K., and Brandner, M.: Langer-Giedion Syndrome. The Fifth Conference on
the Clinical Delineation of Birth Defects,
1972.
6. Klingmüller, G.: Uber eigentümliche Konstitutionsanomalien bei 2 Schwestern und ihre Beziehung zu neueren entwicklungspathologischen Befun-
den, Hautarzt. 7: 105, 1956. 7. Kozlowski, K., and Rupprecht, E.: Klinik und Röntgenbild der Osteochondrodysplasien und Mukopolysaccharidosen. Berlin, Akademic
Verlag,
1972.
BOOKSHELF
The Hyperkinetic Child: A of
Medical, Educational,
Bibliography
and
Child Abuse: Intervention
Behavioral
NANCY B. EBERLING and DEBORAH A. HILL,
Studies
Eds.
ANN WINCHELL. Westport, Conn., Greenwood Press, 1975. 178 pp. $11.00. CAROL
A crossindexed listing of 1974 literature citations, from the 1950s through June 1974, pre-
pared by a professional librarian. Quick Reference to Pediatric Emergencies DLEMER J. PASCOE, and MOSES GROSSMAN, Ed. Philadelphia, J. B. Lippincott Co., 1975.
pp. soft cover $17.00. Presents basic principles, clinical picture, and, especially, management concisely and clearly for hundreds of emergency problems where quick action is needed. The appendixes list valuable information as doses of emergency drugs, and treatment of complications of diagnostic and therapeutic procedures. ~Nill fit conveniently in the pediatric medical bag. An invaluable handy reference. 421
and Treatment
Acton, Mass., Publishing Sciences Group,
Inc., 1975. 182 pp. $15.00. a
The period of hospitalization for treatment of physically abused child is but a small fraction
of the
complexity of that child’s problem. Both protection and management of the neglected and abused child and his family all mainly on the
social worker. Here is a most informative set of presentations, prepared mainly by social workers though with some chapters by physicians, nurses and lawyers, dealing with prevention, etiology, intervention and treatment, emotional reactions to the problems, legal issues, the dynamics of separation and placement. &dquo;Often, the child’s injury can be interpreted as an oblique plea for help-albeit pathetically desperate.&dquo; This collection of papers, easy to read and rich in anecdotes, should be required reading for all advanced students of pediatrics, whether undergraduates, residents, or active in practice.
224
Downloaded from cpj.sagepub.com at Bibliothekssystem der Universitaet Giessen on May 13, 2015
v
Multiple Exostoses-Mental Retardation
Syndrome (Ale-Calo or M.E.M.R. Syndrome) Description of Two Childhood Cases K.
Kozlowski, Doc. Dr. Med., G. Harrington, M.B., B.S., F.R.A.C.R., A. Barylak, Lek. Med., B. Bartoszewica, Dr. Med.
) HIS
PAPER DESCRIBES two unrelated children with the unique clinical and radiographic features of the rare Ale-Calol or Giedion2~ coined the M.E.M.R. syndrme. name multiple exostoses mental retardation syndrome (M.E.M.R. syndrome). Hall et al.5 presented seven cases at the Fifth Conference of Birth Defects in Baltimore (1972) as the
Langer-Giedion syndrome. Case
Descriptions
Case 1. An eight-year-old girl was admitted to the Prince of Wales Hospital in Sydney. The family had no history of bone disease or mental retardation, and the pregnancy and delivery had been un-
eventful. She was first seen
at the age of two years when multiple malformations were noted. These included lop ears, a bulbous nose. a ~4~ide philt;-um with a median raphe, short columella, full upper lip with inverted margin, absent infraspinatus of the
muscles, umbilical hernia, hyperextensibility fingers, and mental retardation.
Presented at the 2nd Asian and Oceanian Congress Radiology, Manila, The Philippines November 10-14, 1975. From the Department of Radiology, Royal Alexandra Hospital for Children, Sydney, Australia, Department of Sydney, Australia, Radiology Prince of Wales and Department of Radiology, Pediatric Institute of the Medical Academy, Krakow, Poland.
of
Hospital,
During subsequent admissions to the hospital. bilateral urcteral renux was demonstrated and plastic surgery to her tip. nose, and ears was
performed.
Examination at age eight years repeated a healthyy looking girt with peculiar fades (Figs. 1A and l B). Both her height and weight were below the 10th
percentile.
Her shoulder musculature
appeared
scapulae, and she had marked genu vatgum. Her left leg was 2 cm longer than her right leg. She had diminished hearing, difficult to assess because of mental retardation... The otc>Ic>~ic examination showed bulging tympanic mem-
deficient, vith
&dquo;winging&dquo;
branes. Bilateral
of the
myringotomies
were
performed;
secretions were noted. Her development was estimated at being about 15 months behind her chronologic age. Routine blood and urine findings, serum electrolytes, serum alkaline phosphatase, urea, creatinine and serum proteins were normal, as were chromosomal count and E.M.G. R~rdi~~raphic:all~,~: she had multiple large exostoses with secondary deformities, multiple ~piph~&dquo;seal dysplasia and cone-shaped epiphyses in the hands and feet (Figs. 1 C-l E). The multiple exostoses involved the knee joint, upper shaft of the humeri, distal shafts of each radius and ulna, some of the tubular bones of the hands and feet, and a number of ribs. The epiphyseal dysplasia was characterized by hypoplasia and flattening of the epiphyses of the long and tubular bones and small round bones. The changes were no
219
Downloaded from cpj.sagepub.com at Bibliothekssystem der Universitaet Giessen on May 13, 2015
.
l~’i(.s. cxostoses
,l :~- l I3. Multiple mental retarda-
tion syndrome, eiglit-j=earold girl. Peculiar face (after phstic surgery) and lop ears. Shortening of the left leg. Valgus deFormity at the knees and winged
scapulae.
marked
the hips. Cone-shaped or dense present in the phalanges «f’ the hands and feet. The bone age of the hands and feet was retarded. The chest was narrow superiorly with narrow, oblique ribs. The thoracic vertebral bodies showed minimal change in shape and a corona! cleft of T-I1 was present. The skull showed some hypertelorism. The intravenous most
epiphyses
at
were
was normal. Case 2. This 13-year-old boy was admitted to the Pediatric Institute of the Medical Academy in Krakow, Poland in November, 1974 with multiple congenital abnormalities and mental retardation (Figs. 2A and 2B). He was the first child of healthy parents. His mother had been 26 years old, his father 21 years c~id; at the time of delivery. Two younger sisters and one younger brother are normal, Pregnancy and delivery were uneventful. The family had no history of mental retardation or bone disease. He started to talk at the age of four years. His
pydogram
development progressed slowly, according
to
parents. At admission, his weight was cm; head circumference 48
I 1 7 .j
20 kg.; height
his
chest circumHe was coopera-
cm;
ference 57 cnii arm span 117 cm. tive, He had large low set ears, scanty hair, slight proptosis, more marked on the lef’t. He had a bulbous nose, a long phikrum and a receding chin. His trunk was unusually short and he had marked lordosis of the lunibosacral spite. Winged scapulae were present bifateralfy’. There was vagus deformity of the knees, more marked on the left. He had thickening and deformities of the middle phalanf;eal joints of the 2nd to 4th fingers. Exostoses were seen, along the long bones and ribs. His muscle mass was decreased but the neuroogic examination showed no abnormality. He had increased mobility at the joints. The ophihalmologic examination showed proptosis. The dermatogly~bics were abnormal. With the ~~’.I:S.G; test, he showed normal
220
Downloaded from cpj.sagepub.com at Bibliothekssystem der Universitaet Giessen on May 13, 2015
a decreased verbal score. His social dcx.eipnicni. according to the Don scate, was about Six years. Radiographically. he had nT~lltipEe ~~t~stt3~es u-itla
with
performance Full-scale
LO.
was
secondary deformities, multiple epiphysea! dysand plasia, and cone-shaped epiphyses in the hands feet (Figs. 2(~-2H). Secondary deformities at the knee and zla7kle joints with ~fat3t-tenii~~; of some bones were observed. Onlv one exostosis was seen
tlie ribs but severat were palpable at the costochondral junctions. Small exostoses were noted at the growing ends of the shafts ot the illE3lllitT’ bones. was characterized by The el3il>!a~-seul ctva}ala:~iu ot the hypoptasia and flattening of the epiphyses bones. small round and bones tnbt))ar and long
in
were most marked at the proximai femoral and distal tibíalepiphyses. The proximal humeral epiphyses iaere &dquo;4tlrarc>st normal... Coneshaped or dense epiphyses were present in the phalanges .of the hands and feet. The bone age out the hands and feet was retarded. The vertebra) cotumn showed a saginaictett vertebrae of -6, and minima) shape changes in some other thoracic vertebrae. The iower thoracic vertebra! bodies were pear-shaped. The ossthcation centers of the annuiar cartiteginous rings were absent. The skull was normal.
The changes
Comments
‘
‘
Two other disorders from this condition.
.
be differentiated The Tricho-Rhino.
must
Fm. I C.
Mukipte exosMses at the end of the long bones.
FIG. 1 D. Cone-shaped epiphyses are best demonstrated at the proximal phalanges of the second to
fingers. Sclerotic epiphyses in some of the distal phalanges. Exostoses at the distal end of the right
fifth
radius.
221
Downloaded from cpj.sagepub.com at Bibliothekssystem der Universitaet Giessen on May 13, 2015
FtG. I E.
&dquo;
Paralytic chest. Hypoptastic upper ribs are
of)]It]Lle ill 1-)osltl()i). Exostosesattheproximaifett humeral metaphyses.
Ft(;s, 2A-2B.
Multiple
mental retardation syndrome, 13-yearold boy. Peculiar face, scanty hair, and lop ears.
Hyperiordosisandwinged Deformities of the limbs with demonstrable exostoses. Small genitalia.
scapulae.
Downloaded from cpj.sagepub.com at Bibliothekssystem der Universitaet Giessen on May 13, 2015
F)C. 2E. Cone-shaped epiphyses are best demonstrated the prc~xitnat and middle phatac~ges. Szlerc>tic ’ epiphyses of the distal phalanges of the 5th fingers. at the metaphyses of some of the Exostoiic prolii’eratins tubular bones. A large exostosis deforms the distal end of the left radius. Small carpal bones. at
described by Klingmiller and Gicdion,2 consists of thin, slow
Phalangeale syndrome, Ftc. 2C. Mutuptc cxc~stc~ses at the end of the shafts of the long bones ~vith secondary deformities. Hypoptastic epiphyses. _
growing hair, a pear-shaped nose with high philtrum, lop ears, brachyphalangy with
FIG. 2D. Hypc~plastic; irregularly ossified and flat-
tened femoral epiphyses. Shortened and widened femoral necks. Small pelvis.
223
Downloaded from cpj.sagepub.com at Bibliothekssystem der Universitaet Giessen on May 13, 2015
deformities of the
shaped epiphyses.
and
fingers,
Slow mental
wedgeelevel«pr~3ent
is often present. About 80 instances of this entity have been reported Dominant and recessive forms apparently occur, although some authors suggest that only one type and one mode of inheritance exists-the autosomal dominant with variables expressivity.
Nfultiple
(similar
to
the Tricho-Rhino-Phaiangcaie muscular hypoplasia, with hyper-
syndrome), extensibiHty at
the
exostoses, and
epiphyseal dN-splasi~t.
~eripyerai a~~ints, mtt~tiple
References 1. Ale, G., and Calo, S.: Su di
caso di disostosi Peri-
un
ferica associata con esostosi osteogeniche
multiple
ed iposomia disuniforme e disarmonica. Radiol. Diagn. (Bologna) 34: 376, 1961.
exostoses
represents a common skeletal disorder characterized by multiple osteochondromas, especially in the long bones. Radial head dislocation, N’I>idelung’s deformity, skeletal fusion, other deformities, and n~ali~naz~t degeneration are not uncommon complications. About two-thirds of the patients have a similarly affected parent. The disease is inherited in about half the children of an affected parent. The most important signs of the Ale-Calo or M.E.M.R. syndrome in our two patients are mental retardation, microcephaly, shortened stature with asymmetry, peculiL~r facies
Ann.
2. Giedion, A.: Das Tricho-Rhino-Phalangeale syndrom. Helv. 21: Paediatr. Acta. 475, 1966. 3. —: Acrodysplasias. Progr. Pediatr. Radiol. Vol. 4. From Intrinsic Diseases of Bones. Karger.
Basel, 1973, pp. 325-43.
4. —: Personal communication (1974). 5. Hall, B. D., Langer, L. O., Giedion, A., Smith, D. W., Cohen, M. M., Beals, R. K., and Brandner, M.: Langer-Giedion Syndrome. The Fifth Conference on
the Clinical Delineation of Birth Defects,
1972.
6. Klingmüller, G.: Uber eigentümliche Konstitutionsanomalien bei 2 Schwestern und ihre Beziehung zu neueren entwicklungspathologischen Befun-
den, Hautarzt. 7: 105, 1956. 7. Kozlowski, K., and Rupprecht, E.: Klinik und Röntgenbild der Osteochondrodysplasien und Mukopolysaccharidosen. Berlin, Akademic
Verlag,
1972.
BOOKSHELF
The Hyperkinetic Child: A of
Medical, Educational,
Bibliography
and
Child Abuse: Intervention
Behavioral
NANCY B. EBERLING and DEBORAH A. HILL,
Studies
Eds.
ANN WINCHELL. Westport, Conn., Greenwood Press, 1975. 178 pp. $11.00. CAROL
A crossindexed listing of 1974 literature citations, from the 1950s through June 1974, pre-
pared by a professional librarian. Quick Reference to Pediatric Emergencies DLEMER J. PASCOE, and MOSES GROSSMAN, Ed. Philadelphia, J. B. Lippincott Co., 1975.
pp. soft cover $17.00. Presents basic principles, clinical picture, and, especially, management concisely and clearly for hundreds of emergency problems where quick action is needed. The appendixes list valuable information as doses of emergency drugs, and treatment of complications of diagnostic and therapeutic procedures. ~Nill fit conveniently in the pediatric medical bag. An invaluable handy reference. 421
and Treatment
Acton, Mass., Publishing Sciences Group,
Inc., 1975. 182 pp. $15.00. a
The period of hospitalization for treatment of physically abused child is but a small fraction
of the
complexity of that child’s problem. Both protection and management of the neglected and abused child and his family all mainly on the
social worker. Here is a most informative set of presentations, prepared mainly by social workers though with some chapters by physicians, nurses and lawyers, dealing with prevention, etiology, intervention and treatment, emotional reactions to the problems, legal issues, the dynamics of separation and placement. &dquo;Often, the child’s injury can be interpreted as an oblique plea for help-albeit pathetically desperate.&dquo; This collection of papers, easy to read and rich in anecdotes, should be required reading for all advanced students of pediatrics, whether undergraduates, residents, or active in practice.
224
Downloaded from cpj.sagepub.com at Bibliothekssystem der Universitaet Giessen on May 13, 2015
v
