Piet Dijkstra - Academia.edu (original) (raw)
Papers by Piet Dijkstra
A 100-year-old anatomical specimen presenting with boomerang-like skeletal dysplasia: Diagnostic strategies and outcome
American Journal of Medical Genetics, Jul 16, 1999
The Museum Vrolik collection of human anatomy comprises 360 recently redescribed specimens with c... more The Museum Vrolik collection of human anatomy comprises 360 recently redescribed specimens with congenital anomalies. The specimen described here dated from 1881 and presented with a general embryonic appearance, disproportionate short stature, brachycephaly, widened cranial sutures, hypertelorism, microphthalmia, bilateral cleft lip and palate, micrognathia, short and curved limbs, polysyndactyly, and abnormal female genitalia. Conventional radiography was hampered by decalcification of the skeleton, due to acidification of the preservation fluid. The use of additional imaging techniques, i.e., mammography, computerized tomography with three-dimensional reconstruction, and magnetic resonance imaging eventually led us to conclude that the condition of our specimen was similar to Piepkorn type skeletal dysplasia, boomerang dysplasia, and a condition described by Carpenter and Hunter [1982: J Med Genet 19:311-315], though none of these diagnoses seemed fully applicable.
Uniform fat suppression in hands and feet through the use of two-point Dixon chemical shift MR imaging
J Magn Reson Imaging, 1999
To assess the potential of two-point Dixon chemical shift magnetic resonance imaging to achieve u... more To assess the potential of two-point Dixon chemical shift magnetic resonance imaging to achieve uniform fat suppression in the distal parts of the extremities. Two-point Dixon chemical shift imaging was performed in 31 consecutive patients clinically suspected to have bone marrow disease. In some patients, Dixon studies were performed before and after the intravenous administration of gadopentetate dimeglumine, and in some patients follow-up examinations were performed, for a total of 64 studies. Areas of interest were the hand, wrist, foot, ankle, and lower leg. There was a special interest in the neuropathic foot and osteomyelitis. The uniformity of fat suppression in the entire field of view, the frequency of displacement artifacts, and the applicability of the technique in routine patient treatment were evaluated. In 64 (100%) Dixon studies, uniform fat suppression was achieved. In 59 (92%) studies, there were no displacement artifacts. In five (8%) studies, displacement artifacts occurred; however, in only one (2%) study did they severely hamper the reading. Thus, in 63 (98%) studies, adequate diagnostic quality was obtained. Two-point Dixon chemical shift imaging is a good technique for achieving uniform fat suppression in the distal parts of the extremities. Because the frequency of displacement artifacts is low, the technique is applicable in a routine clinical setting.
Achondrogenesis…Hypochondrogenesis: the Spectrum of Chondrogenesis Imperfecta a Radiological, Ultrasonographic, and Histopathologic Study of 23 Cases
In the classification of lethal osteochondrodysplasias, achondrogenesis and hypochondrogenesis ha... more In the classification of lethal osteochondrodysplasias, achondrogenesis and hypochondrogenesis have recently received special attention. We describe 23 cases representing the different subtypes. Within the classical type I (Parenti-Fraccaro) two distinct disorders can be recognized: type IA (Houston-Harris) and type IB (Fraccaro). The classical type II (Langer-Saldino) and hypochondrogenesis represent phenotypic variants of one disorder in which type II is the most severe form and hypochondrogenesis the mildest form, while transitional forms exist. It is likely that a basic defect in cellular function of the chondrocyte results in a deficient cartilage matrix and in disorganized enchondral ossification.
Perinatal Lethal Osteogenesis Imperfecta: Radiologic and Pathologic Evaluation of Seven Prenatally Diagnosed Cases
Pediatric Pathology Affiliated With the International Paediatric Pathology Association, Feb 1, 1988
The radiologic and pathologic characteristics of 7 cases of lethal osteogenesis imperfecta (OI), ... more The radiologic and pathologic characteristics of 7 cases of lethal osteogenesis imperfecta (OI), diagnosed prenatally by ultrasound in the 15th to 34th week, are described. They include four variants of the Sillence classification: types IIA, IIB, IIC, and type III. The radiologic criteria that differentiate these types of OI are described. The histopathology of the bones differed only slightly in types IIA, IIB, and III; OI type IIC, however, differed markedly from the other types.
American Journal of Medical Genetics, 2005
pattern profile (MCPP) was determined in 49 radiographs of 40 patients with Rubinstein-Taybi synd... more pattern profile (MCPP) was determined in 49 radiographs of 40 patients with Rubinstein-Taybi syndrome. Two recognizable hand profiles were seen, depending on the configuration of the thumb and on age. Patients with a straight thumb showed a short first proximal phalanx, and short third medial phalanx. Patients with a radially deviated thumb had a short first proximal phalanx. Depending on age, a relatively large (infancy) or markedly short (older patients) first distal phalanx was found. The similarity between the patients was high. A third group of patients did not show a particular hand profile, but only small hand bones. The pattern variability indices were high in all groups of patients.
Prenatal ultrasonographic diagnosis of osteogenesis imperfecta
American Journal of Obstetrics and Gynecology, 1988
Between 1982 and 1986, osteogenesis imperfecta was diagnosed by ultrasound in seven fetuses. The ... more Between 1982 and 1986, osteogenesis imperfecta was diagnosed by ultrasound in seven fetuses. The known heterogeneity of osteogenesis imperfecta was confirmed by the prenatal ultrasonographic findings. Dependent on the type of osteogenesis imperfecta, the appearance of the limbs varied from severely shortened and broad, with very low echogeneity and absent acoustic shadow (type IIA), to only moderately shortened and thin, with almost normal echogeneity and acoustic shadow but clearly visible fractures causing angulation of the bone (types IIC and III). Ultrasonography offers the possibility to detect or exclude the lethal and severe forms of osteogenesis imperfecta early (type IIA) or halfway (types IIB, IIC, and III) through the second trimester. Prenatal diagnosis of the disease allows the option of elective abortion or may prevent unnecessary obstetric intervention.
Uniform Fat Suppression in Hands and Feet through the Use of Two-Point Dixon Chemical Shift MR Imaging
Radiology, 1999
To assess the potential of two-point Dixon chemical shift magnetic resonance imaging to achieve u... more To assess the potential of two-point Dixon chemical shift magnetic resonance imaging to achieve uniform fat suppression in the distal parts of the extremities. Two-point Dixon chemical shift imaging was performed in 31 consecutive patients clinically suspected to have bone marrow disease. In some patients, Dixon studies were performed before and after the intravenous administration of gadopentetate dimeglumine, and in some patients follow-up examinations were performed, for a total of 64 studies. Areas of interest were the hand, wrist, foot, ankle, and lower leg. There was a special interest in the neuropathic foot and osteomyelitis. The uniformity of fat suppression in the entire field of view, the frequency of displacement artifacts, and the applicability of the technique in routine patient treatment were evaluated. In 64 (100%) Dixon studies, uniform fat suppression was achieved. In 59 (92%) studies, there were no displacement artifacts. In five (8%) studies, displacement artifacts occurred; however, in only one (2%) study did they severely hamper the reading. Thus, in 63 (98%) studies, adequate diagnostic quality was obtained. Two-point Dixon chemical shift imaging is a good technique for achieving uniform fat suppression in the distal parts of the extremities. Because the frequency of displacement artifacts is low, the technique is applicable in a routine clinical setting.
Journal of Medical Genetics, 1995
The fragile X syndrome, which often presents in childhood with overgrowth, may in some cases show... more The fragile X syndrome, which often presents in childhood with overgrowth, may in some cases show some diagnostic overlap with classical Sotos syndrome. We describe four fragile X patients with general overgrowth, all of whom are from families with other affected relatives who show the classic Martin-Bell phenotype. Molecular studies of the FMR1 gene in all cases showed the typical full mutation as seen in males affected by the fragile X syndrome. Endocrine studies were unremarkable, except in one case where there were raised levels of insulin-like growth factor-I (IGF-I) and insulin-like growth factor binding protein-3 (IGFBP-3)
The Journal of Hand Surgery: Journal of the British Society for Surgery of the Hand, 1996
Based on our own experiments on piglets from 1983 to 1986 and the clinical experience of Yu Dong ... more Based on our own experiments on piglets from 1983 to 1986 and the clinical experience of Yu Dong Gu from Shanghai, we used, in 199415, supplementary one spinal nerve C7 from the healthy side for the neurotization in total avulsions of the brachial plexus. We studied the sequels of such a procedure and the efficiency of the reinervation in the paralyzed upper extremity.
Journal of Controlled Release, 2005
he also initiated a joint program together with these investigators to develop drug delivery syst... more he also initiated a joint program together with these investigators to develop drug delivery systems based on biodegradable polymers.
The Value of Additional Carpal Box Radiographs in Suspected Scaphoid Fracture
INVESTIGATIVE RADIOLOGY, 1997
Carpal Box (CB) radiographs, transverse and longitudinal, produce elongated and magnified views o... more Carpal Box (CB) radiographs, transverse and longitudinal, produce elongated and magnified views of the carpus. These radiographs can be used after carpal injury, in addition to conventional scaphoid x-rays. In this study, the use of CB radiographs was evaluated in patients with possible scaphoid fracture. Seventy-one consecutive patients who presented at the First Aid department from May 1994 to May 1995 were included. All patients were examined for scaphoid fracture after a fall on the out-stretched hand. If a scaphoid fracture was seen on the scaphoid x-rays, patients were immobilized. If the x-rays remained negative or dubious for fracture, additional transverse and longitudinal CB radiographs were obtained. If CB radiographs remained negative or inconclusive, patients were referred for three-phase bone scintigraphy. The results of independent and masked judgment by three different observers were used for an inter- and intraobserver analysis. Twenty of 71 patients initially showed a scaphoid fracture on the conventional scaphoid x-rays, 41 were negative, and 10 inconclusive. All 41 negative patients remained negative on CB radiograph; however, the bone scintigraphy was positive for scaphoid fracture in 11 patients and in 9 patients a hot spot elsewhere in the carpus was found. Of the 10 patients with inconclusive x-rays, 2 showed a clear fracture of the scaphoid on CB radiograph, 5 were negative, and 3 remained inconclusive. The agreement between observers, calculated in kappa values, was highest in CB radiographs. In the diagnosis of scaphoid fracture, Carpal Box radiography is of limited value in patients with clinically suspected scaphoid fracture. In two of 10 patients with initial dubious scaphoid x-ray, bone scintigraphy can be avoided. Furthermore, the reliability of the interpretation of the radiographs is increased by additional Carpal Box radiography.
Radiography of the Carpal Scaphoid
Investigative Radiology, 1992
Human Genetics, 1978
Double translocation heterozygosity t(2;6),t(7;12) in three generations of a Dutch family is desc... more Double translocation heterozygosity t(2;6),t(7;12) in three generations of a Dutch family is described: the segregation of a double translocation in more than one generation has not been previously published. The index case was a 16-year-old mentally retarded boy with partial trisomy 12p who showed several dysmorphic features such as high prominent forehead, flat face, flat and short nose bridge, short nose, dysplastic ears, prominent lower lip, and several skeletal abnormalities. Based on the findings in this patient and those in nine other cases, the existence of a specific trisomy 12p syndrome is postulated.
Perinatal Lethal Osteogenesis Imperfecta: Radiologic and Pathologic Evaluation of Seven Prenatally Diagnosed Cases
Fetal & Pediatric Pathology, 1988
The radiologic and pathologic characteristics of 7 cases of lethal osteogenesis imperfecta (OI), ... more The radiologic and pathologic characteristics of 7 cases of lethal osteogenesis imperfecta (OI), diagnosed prenatally by ultrasound in the 15th to 34th week, are described. They include four variants of the Sillence classification: types IIA, IIB, IIC, and type III. The radiologic criteria that differentiate these types of OI are described. The histopathology of the bones differed only slightly in types IIA, IIB, and III; OI type IIC, however, differed markedly from the other types.
Achondrogenesis…Hypochondrogenesis: the Spectrum of Chondrogenesis Imperfecta a Radiological, Ultrasonographic, and Histopathologic Study of 23 Cases
Fetal & Pediatric Pathology, 1988
In the classification of lethal osteochondrodysplasias, achondrogenesis and hypochondrogenesis ha... more In the classification of lethal osteochondrodysplasias, achondrogenesis and hypochondrogenesis have recently received special attention. We describe 23 cases representing the different subtypes. Within the classical type I (Parenti-Fraccaro) two distinct disorders can be recognized: type IA (Houston-Harris) and type IB (Fraccaro). The classical type II (Langer-Saldino) and hypochondrogenesis represent phenotypic variants of one disorder in which type II is the most severe form and hypochondrogenesis the mildest form, while transitional forms exist. It is likely that a basic defect in cellular function of the chondrocyte results in a deficient cartilage matrix and in disorganized enchondral ossification.
European Journal of Nuclear Medicine, 1993
The use of bone scintigraphy in patients with negative radiographs after carpal injury is widely ... more The use of bone scintigraphy in patients with negative radiographs after carpal injury is widely advocated. However, focally increased activity on the bone scan in the scaphoid or other carpal bones cannot always be radiologically confirmed as a fracture. To confirm scintigraphically suspected carpal fractures, computed tomography (CT) of the wrist was performed in patients with clinically suspected scaphoid fracture and initially negative radiographs. All patients underwent plain radiography, bone scintigraphy and CT. The combination of plain radiographs and CT, as judged by a panel of experienced observers, was used as the reference standard. In 18 patients, 21 out of 22 carpal hot spots on bone scintigraphy could be radiologically confirmed as a fracture. The diagnosis was missed by CT scan in three patients with proven fractures on plain radiographs. We conclude that, in patients with negative initial radiographs following carpal injury, a positive bone scan must be interpreted as a fracture.
Arthritis & Rheumatism, 2003
To describe radiologic features of patients with juvenile idiopathic arthritis (JIA) in a standar... more To describe radiologic features of patients with juvenile idiopathic arthritis (JIA) in a standardized manner, to test the reliability and feasibility of this description, and to correlate these features with clinical signs as a first step in the development of a standardized assessment method.
Sulfasalazine in the treatment of juvenile chronic arthritis: A randomized, double-blind, placebo-controlled, multicenter study
Arthritis & Rheumatism, 1998
To assess the efficacy, tolerability, and safety of sulfasalazine (SSZ) in the treatment of juven... more To assess the efficacy, tolerability, and safety of sulfasalazine (SSZ) in the treatment of juvenile chronic arthritis (JCA). We conducted a 24-week randomized, placebo-controlled, double-blind, multicenter study of patients with active JCA of both oligoarticular and polyarticular onset. Patients were treated with a dosage of 50 mg/kg/day of SSZ (maximum 2,000 mg/day) or placebo. The efficacy variables were joint scores, physician's, parents', and patient's overall assessments, and laboratory parameters of inflammation. Of the 69 patients enrolled, 52 (75%) completed the trial. Six patients (18%) withdrew from the placebo group, and 11 (31%) withdrew from the SSZ group (P = 0.18). In the intention-to-treat analysis of end point efficacy, between-group differences were significant for the overall articular severity score (P = 0.02), all global assessments (P = 0.01), and the laboratory parameters (P < 0.001). Adverse events occurred more frequently in the SSZ group and were the main reason for withdrawal (P < 0.001), but in all instances, these events were transient or reversible upon cessation of treatment. The results of this first placebo-controlled study show that SSZ is effective and safe in the treatment of children with oligoarticular- and polyarticular-onset JCA, although it was not well tolerated in one-third of the patients.
Metacarpophalangeal pattern profile analysis in Sotos and Marfan syndrome
American Journal of Medical Genetics, 1994
Autosomal dominant familial radial luxation, carpal fusion and scapular dysplasia with variable heart defects
American Journal of Medical Genetics, 1996
A family is described with skeletal abnormalities involving the shoulder, elbow, and hand, in com... more A family is described with skeletal abnormalities involving the shoulder, elbow, and hand, in combination with variable cardiac defects including conduction defects and anatomical anomalies. The disorder followed an autosomal dominant pattern of inheritance with apparently full penetrance for the skeletal abnormalities and reduced penetrance for the cardiac defects.
A 100-year-old anatomical specimen presenting with boomerang-like skeletal dysplasia: Diagnostic strategies and outcome
American Journal of Medical Genetics, Jul 16, 1999
The Museum Vrolik collection of human anatomy comprises 360 recently redescribed specimens with c... more The Museum Vrolik collection of human anatomy comprises 360 recently redescribed specimens with congenital anomalies. The specimen described here dated from 1881 and presented with a general embryonic appearance, disproportionate short stature, brachycephaly, widened cranial sutures, hypertelorism, microphthalmia, bilateral cleft lip and palate, micrognathia, short and curved limbs, polysyndactyly, and abnormal female genitalia. Conventional radiography was hampered by decalcification of the skeleton, due to acidification of the preservation fluid. The use of additional imaging techniques, i.e., mammography, computerized tomography with three-dimensional reconstruction, and magnetic resonance imaging eventually led us to conclude that the condition of our specimen was similar to Piepkorn type skeletal dysplasia, boomerang dysplasia, and a condition described by Carpenter and Hunter [1982: J Med Genet 19:311-315], though none of these diagnoses seemed fully applicable.
Uniform fat suppression in hands and feet through the use of two-point Dixon chemical shift MR imaging
J Magn Reson Imaging, 1999
To assess the potential of two-point Dixon chemical shift magnetic resonance imaging to achieve u... more To assess the potential of two-point Dixon chemical shift magnetic resonance imaging to achieve uniform fat suppression in the distal parts of the extremities. Two-point Dixon chemical shift imaging was performed in 31 consecutive patients clinically suspected to have bone marrow disease. In some patients, Dixon studies were performed before and after the intravenous administration of gadopentetate dimeglumine, and in some patients follow-up examinations were performed, for a total of 64 studies. Areas of interest were the hand, wrist, foot, ankle, and lower leg. There was a special interest in the neuropathic foot and osteomyelitis. The uniformity of fat suppression in the entire field of view, the frequency of displacement artifacts, and the applicability of the technique in routine patient treatment were evaluated. In 64 (100%) Dixon studies, uniform fat suppression was achieved. In 59 (92%) studies, there were no displacement artifacts. In five (8%) studies, displacement artifacts occurred; however, in only one (2%) study did they severely hamper the reading. Thus, in 63 (98%) studies, adequate diagnostic quality was obtained. Two-point Dixon chemical shift imaging is a good technique for achieving uniform fat suppression in the distal parts of the extremities. Because the frequency of displacement artifacts is low, the technique is applicable in a routine clinical setting.
Achondrogenesis…Hypochondrogenesis: the Spectrum of Chondrogenesis Imperfecta a Radiological, Ultrasonographic, and Histopathologic Study of 23 Cases
In the classification of lethal osteochondrodysplasias, achondrogenesis and hypochondrogenesis ha... more In the classification of lethal osteochondrodysplasias, achondrogenesis and hypochondrogenesis have recently received special attention. We describe 23 cases representing the different subtypes. Within the classical type I (Parenti-Fraccaro) two distinct disorders can be recognized: type IA (Houston-Harris) and type IB (Fraccaro). The classical type II (Langer-Saldino) and hypochondrogenesis represent phenotypic variants of one disorder in which type II is the most severe form and hypochondrogenesis the mildest form, while transitional forms exist. It is likely that a basic defect in cellular function of the chondrocyte results in a deficient cartilage matrix and in disorganized enchondral ossification.
Perinatal Lethal Osteogenesis Imperfecta: Radiologic and Pathologic Evaluation of Seven Prenatally Diagnosed Cases
Pediatric Pathology Affiliated With the International Paediatric Pathology Association, Feb 1, 1988
The radiologic and pathologic characteristics of 7 cases of lethal osteogenesis imperfecta (OI), ... more The radiologic and pathologic characteristics of 7 cases of lethal osteogenesis imperfecta (OI), diagnosed prenatally by ultrasound in the 15th to 34th week, are described. They include four variants of the Sillence classification: types IIA, IIB, IIC, and type III. The radiologic criteria that differentiate these types of OI are described. The histopathology of the bones differed only slightly in types IIA, IIB, and III; OI type IIC, however, differed markedly from the other types.
American Journal of Medical Genetics, 2005
pattern profile (MCPP) was determined in 49 radiographs of 40 patients with Rubinstein-Taybi synd... more pattern profile (MCPP) was determined in 49 radiographs of 40 patients with Rubinstein-Taybi syndrome. Two recognizable hand profiles were seen, depending on the configuration of the thumb and on age. Patients with a straight thumb showed a short first proximal phalanx, and short third medial phalanx. Patients with a radially deviated thumb had a short first proximal phalanx. Depending on age, a relatively large (infancy) or markedly short (older patients) first distal phalanx was found. The similarity between the patients was high. A third group of patients did not show a particular hand profile, but only small hand bones. The pattern variability indices were high in all groups of patients.
Prenatal ultrasonographic diagnosis of osteogenesis imperfecta
American Journal of Obstetrics and Gynecology, 1988
Between 1982 and 1986, osteogenesis imperfecta was diagnosed by ultrasound in seven fetuses. The ... more Between 1982 and 1986, osteogenesis imperfecta was diagnosed by ultrasound in seven fetuses. The known heterogeneity of osteogenesis imperfecta was confirmed by the prenatal ultrasonographic findings. Dependent on the type of osteogenesis imperfecta, the appearance of the limbs varied from severely shortened and broad, with very low echogeneity and absent acoustic shadow (type IIA), to only moderately shortened and thin, with almost normal echogeneity and acoustic shadow but clearly visible fractures causing angulation of the bone (types IIC and III). Ultrasonography offers the possibility to detect or exclude the lethal and severe forms of osteogenesis imperfecta early (type IIA) or halfway (types IIB, IIC, and III) through the second trimester. Prenatal diagnosis of the disease allows the option of elective abortion or may prevent unnecessary obstetric intervention.
Uniform Fat Suppression in Hands and Feet through the Use of Two-Point Dixon Chemical Shift MR Imaging
Radiology, 1999
To assess the potential of two-point Dixon chemical shift magnetic resonance imaging to achieve u... more To assess the potential of two-point Dixon chemical shift magnetic resonance imaging to achieve uniform fat suppression in the distal parts of the extremities. Two-point Dixon chemical shift imaging was performed in 31 consecutive patients clinically suspected to have bone marrow disease. In some patients, Dixon studies were performed before and after the intravenous administration of gadopentetate dimeglumine, and in some patients follow-up examinations were performed, for a total of 64 studies. Areas of interest were the hand, wrist, foot, ankle, and lower leg. There was a special interest in the neuropathic foot and osteomyelitis. The uniformity of fat suppression in the entire field of view, the frequency of displacement artifacts, and the applicability of the technique in routine patient treatment were evaluated. In 64 (100%) Dixon studies, uniform fat suppression was achieved. In 59 (92%) studies, there were no displacement artifacts. In five (8%) studies, displacement artifacts occurred; however, in only one (2%) study did they severely hamper the reading. Thus, in 63 (98%) studies, adequate diagnostic quality was obtained. Two-point Dixon chemical shift imaging is a good technique for achieving uniform fat suppression in the distal parts of the extremities. Because the frequency of displacement artifacts is low, the technique is applicable in a routine clinical setting.
Journal of Medical Genetics, 1995
The fragile X syndrome, which often presents in childhood with overgrowth, may in some cases show... more The fragile X syndrome, which often presents in childhood with overgrowth, may in some cases show some diagnostic overlap with classical Sotos syndrome. We describe four fragile X patients with general overgrowth, all of whom are from families with other affected relatives who show the classic Martin-Bell phenotype. Molecular studies of the FMR1 gene in all cases showed the typical full mutation as seen in males affected by the fragile X syndrome. Endocrine studies were unremarkable, except in one case where there were raised levels of insulin-like growth factor-I (IGF-I) and insulin-like growth factor binding protein-3 (IGFBP-3)
The Journal of Hand Surgery: Journal of the British Society for Surgery of the Hand, 1996
Based on our own experiments on piglets from 1983 to 1986 and the clinical experience of Yu Dong ... more Based on our own experiments on piglets from 1983 to 1986 and the clinical experience of Yu Dong Gu from Shanghai, we used, in 199415, supplementary one spinal nerve C7 from the healthy side for the neurotization in total avulsions of the brachial plexus. We studied the sequels of such a procedure and the efficiency of the reinervation in the paralyzed upper extremity.
Journal of Controlled Release, 2005
he also initiated a joint program together with these investigators to develop drug delivery syst... more he also initiated a joint program together with these investigators to develop drug delivery systems based on biodegradable polymers.
The Value of Additional Carpal Box Radiographs in Suspected Scaphoid Fracture
INVESTIGATIVE RADIOLOGY, 1997
Carpal Box (CB) radiographs, transverse and longitudinal, produce elongated and magnified views o... more Carpal Box (CB) radiographs, transverse and longitudinal, produce elongated and magnified views of the carpus. These radiographs can be used after carpal injury, in addition to conventional scaphoid x-rays. In this study, the use of CB radiographs was evaluated in patients with possible scaphoid fracture. Seventy-one consecutive patients who presented at the First Aid department from May 1994 to May 1995 were included. All patients were examined for scaphoid fracture after a fall on the out-stretched hand. If a scaphoid fracture was seen on the scaphoid x-rays, patients were immobilized. If the x-rays remained negative or dubious for fracture, additional transverse and longitudinal CB radiographs were obtained. If CB radiographs remained negative or inconclusive, patients were referred for three-phase bone scintigraphy. The results of independent and masked judgment by three different observers were used for an inter- and intraobserver analysis. Twenty of 71 patients initially showed a scaphoid fracture on the conventional scaphoid x-rays, 41 were negative, and 10 inconclusive. All 41 negative patients remained negative on CB radiograph; however, the bone scintigraphy was positive for scaphoid fracture in 11 patients and in 9 patients a hot spot elsewhere in the carpus was found. Of the 10 patients with inconclusive x-rays, 2 showed a clear fracture of the scaphoid on CB radiograph, 5 were negative, and 3 remained inconclusive. The agreement between observers, calculated in kappa values, was highest in CB radiographs. In the diagnosis of scaphoid fracture, Carpal Box radiography is of limited value in patients with clinically suspected scaphoid fracture. In two of 10 patients with initial dubious scaphoid x-ray, bone scintigraphy can be avoided. Furthermore, the reliability of the interpretation of the radiographs is increased by additional Carpal Box radiography.
Radiography of the Carpal Scaphoid
Investigative Radiology, 1992
Human Genetics, 1978
Double translocation heterozygosity t(2;6),t(7;12) in three generations of a Dutch family is desc... more Double translocation heterozygosity t(2;6),t(7;12) in three generations of a Dutch family is described: the segregation of a double translocation in more than one generation has not been previously published. The index case was a 16-year-old mentally retarded boy with partial trisomy 12p who showed several dysmorphic features such as high prominent forehead, flat face, flat and short nose bridge, short nose, dysplastic ears, prominent lower lip, and several skeletal abnormalities. Based on the findings in this patient and those in nine other cases, the existence of a specific trisomy 12p syndrome is postulated.
Perinatal Lethal Osteogenesis Imperfecta: Radiologic and Pathologic Evaluation of Seven Prenatally Diagnosed Cases
Fetal & Pediatric Pathology, 1988
The radiologic and pathologic characteristics of 7 cases of lethal osteogenesis imperfecta (OI), ... more The radiologic and pathologic characteristics of 7 cases of lethal osteogenesis imperfecta (OI), diagnosed prenatally by ultrasound in the 15th to 34th week, are described. They include four variants of the Sillence classification: types IIA, IIB, IIC, and type III. The radiologic criteria that differentiate these types of OI are described. The histopathology of the bones differed only slightly in types IIA, IIB, and III; OI type IIC, however, differed markedly from the other types.
Achondrogenesis…Hypochondrogenesis: the Spectrum of Chondrogenesis Imperfecta a Radiological, Ultrasonographic, and Histopathologic Study of 23 Cases
Fetal & Pediatric Pathology, 1988
In the classification of lethal osteochondrodysplasias, achondrogenesis and hypochondrogenesis ha... more In the classification of lethal osteochondrodysplasias, achondrogenesis and hypochondrogenesis have recently received special attention. We describe 23 cases representing the different subtypes. Within the classical type I (Parenti-Fraccaro) two distinct disorders can be recognized: type IA (Houston-Harris) and type IB (Fraccaro). The classical type II (Langer-Saldino) and hypochondrogenesis represent phenotypic variants of one disorder in which type II is the most severe form and hypochondrogenesis the mildest form, while transitional forms exist. It is likely that a basic defect in cellular function of the chondrocyte results in a deficient cartilage matrix and in disorganized enchondral ossification.
European Journal of Nuclear Medicine, 1993
The use of bone scintigraphy in patients with negative radiographs after carpal injury is widely ... more The use of bone scintigraphy in patients with negative radiographs after carpal injury is widely advocated. However, focally increased activity on the bone scan in the scaphoid or other carpal bones cannot always be radiologically confirmed as a fracture. To confirm scintigraphically suspected carpal fractures, computed tomography (CT) of the wrist was performed in patients with clinically suspected scaphoid fracture and initially negative radiographs. All patients underwent plain radiography, bone scintigraphy and CT. The combination of plain radiographs and CT, as judged by a panel of experienced observers, was used as the reference standard. In 18 patients, 21 out of 22 carpal hot spots on bone scintigraphy could be radiologically confirmed as a fracture. The diagnosis was missed by CT scan in three patients with proven fractures on plain radiographs. We conclude that, in patients with negative initial radiographs following carpal injury, a positive bone scan must be interpreted as a fracture.
Arthritis & Rheumatism, 2003
To describe radiologic features of patients with juvenile idiopathic arthritis (JIA) in a standar... more To describe radiologic features of patients with juvenile idiopathic arthritis (JIA) in a standardized manner, to test the reliability and feasibility of this description, and to correlate these features with clinical signs as a first step in the development of a standardized assessment method.
Sulfasalazine in the treatment of juvenile chronic arthritis: A randomized, double-blind, placebo-controlled, multicenter study
Arthritis & Rheumatism, 1998
To assess the efficacy, tolerability, and safety of sulfasalazine (SSZ) in the treatment of juven... more To assess the efficacy, tolerability, and safety of sulfasalazine (SSZ) in the treatment of juvenile chronic arthritis (JCA). We conducted a 24-week randomized, placebo-controlled, double-blind, multicenter study of patients with active JCA of both oligoarticular and polyarticular onset. Patients were treated with a dosage of 50 mg/kg/day of SSZ (maximum 2,000 mg/day) or placebo. The efficacy variables were joint scores, physician's, parents', and patient's overall assessments, and laboratory parameters of inflammation. Of the 69 patients enrolled, 52 (75%) completed the trial. Six patients (18%) withdrew from the placebo group, and 11 (31%) withdrew from the SSZ group (P = 0.18). In the intention-to-treat analysis of end point efficacy, between-group differences were significant for the overall articular severity score (P = 0.02), all global assessments (P = 0.01), and the laboratory parameters (P < 0.001). Adverse events occurred more frequently in the SSZ group and were the main reason for withdrawal (P < 0.001), but in all instances, these events were transient or reversible upon cessation of treatment. The results of this first placebo-controlled study show that SSZ is effective and safe in the treatment of children with oligoarticular- and polyarticular-onset JCA, although it was not well tolerated in one-third of the patients.
Metacarpophalangeal pattern profile analysis in Sotos and Marfan syndrome
American Journal of Medical Genetics, 1994
Autosomal dominant familial radial luxation, carpal fusion and scapular dysplasia with variable heart defects
American Journal of Medical Genetics, 1996
A family is described with skeletal abnormalities involving the shoulder, elbow, and hand, in com... more A family is described with skeletal abnormalities involving the shoulder, elbow, and hand, in combination with variable cardiac defects including conduction defects and anatomical anomalies. The disorder followed an autosomal dominant pattern of inheritance with apparently full penetrance for the skeletal abnormalities and reduced penetrance for the cardiac defects.