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By X. Cobryn. University of West Florida.

During this period generic lopressor 100 mg overnight delivery, inflammation and LANE discount lopressor 50 mg with mastercard, AND DAWSON, 1990) vascularization occur. Systemic factors can inhibit bone healing includ- process is dependent on the presence of live bone cells ing the following: in a graft material. Cigarette smoking (Glassman, 1998) Contain viable cells with the ability to form bone b. Malnutrition (Mankin, 1990) (osteoprogenitor cells) or the potential to differentiate c. Rheumatoid arthritis Osteogenesis is a property found only in fresh auto- e. Osteoporosis (Kelsey and Hoffman, 1987) genous bone and in bone marrow cells. Nonsteroidal anti-inflammatory medications GAZDAG ET AL, 1995) (Glassman et al, 2000) The physical property of the graft to serve as a scaf- Healing occurs in three distinct but overlapping stages fold for viable bone healing. Inflammatory cells and autografts and allografts, demineralized bone matrix, fibroblasts infiltrate the bone under prostaglandin hydroxyapatite, collagen, and calcium phosphate. This results in the formation of granulation tissue, ingrowth of vascular tissue, and migration of mesenchymal cells. OSTEOINDUCTION (CONNOLLY, 1998; MOHAN Exposed skin cells, bone, and muscle provide the pri- AND BAYLINK, 1991) mary nutrients of this early process. BMPs and demineralized bone matrix are the prin- REPAIR STAGE cipal osteoinductive materials. Autograft and allograft bone also have some osteoin- port vascular ingrowth. This leads to the formation of a soft ET AL, 1989) callus around the repair site. Callus is very weak in the first 4–6 weeks and slowly reabsorbed and simultaneously replaced with requires adequate protection (Kenwright and new viable bone, thus incorporating bone grafts Gardner, 1998).

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Usually an occipitocervical spon- An important task in the differential diagnosis is to rule dylodesis is performed in such cases generic 25 mg lopressor with mastercard. The atlantoaxial out secondary osteoporosis purchase 50 mg lopressor mastercard, which occurs in the follow- instability must also always be borne in mind during ing disorders: hyperthyroidism, hyperparathyroidism, any induction of anesthesia. The second problem that hypogonadism, Cushing syndrome, homocystinuria, occasionally requires treatment is the thoracolumbar vitamin C deficiency, rickets, Lowe syndrome, uremia, kyphosis, which generally leads to spondylolisthesis in Turner syndrome, leukemia, lymphoma and osteogen- this area. Treatment with corticosteroids, for in- further slippage and accentuation of the kyphosis. If stance in juvenile rheumatoid arthritis or Langerhans the coxa vara is very pronounced, a valgization oste- cell histiocytosis, can lead to severe osteoporosis. In some cases the valgus deformity ment, successful results have been obtained with of the rearfoot is severe enough to require a subtalar the bisphosphonate pamidronate (Aredia) [108]. In the event of a fractured extrem- Only isolated reports exist for the autosomal-recessive ity, the possibility of inactivity-induced osteoporosis microcephalic osteodysplastic dysplasia, which occurs should be avoided. The patients show moderately stunted should not be treated with cast fixation, and internal growth (which only becomes apparent during the course plate fixation is also highly inadvisable. A more appro- of growth), have short fingers, are microcephalic but with priate response is to use intramedullary load-bearing normal intelligence, the bones are generally very thin, and implants, provided these are compatible with open coxa vara and abnormally shaped epiphyses are typical epiphyseal plates (e. Overall bone mass decreases as a result of the increased resorption by osteoclasts, although there are no qualitative abnormali- ties. The laboratory tests do not show any major abnormalities apart from a negative calcium balance. This is an ex- tremely rare condition that is probably caused (like osteogenesis imperfecta) by an anomaly of type I collagen as a result of a mutation. The principal symptoms are dull back pain, possibly with symptoms in the extremities, particularly the feet, with a consequent adverse effect on gait. Lateral x-ray of the spine of a 5-year old boy withidiopathic hyperkyphosis.

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Schematic view of Goldthwait operation: The patellar cheap 12.5mg lopressor otc, tendon is split lengthwise and the lateral half is pulled over the ▬ Ventralization of the tibial tuberosity according to medial half and fixed on the medial side discount lopressor 100 mg visa, while the lateral retinacula Maquet. The advantage of this method is that it can be performed even if the apophyseal plate has not yet closed. The dis- advantage is a much higher recurrence rate compared to bone-based transpositions. In the procedure of medialization of the tibial tuberos- ity according to Roux and Hauser, a bone frag- ment with the complete patellar tendon attachment is chiseled out of the tibia, relocated laterally and fixed with one or more screws (⊡ Fig. This is particularly indicated for patients with a patella alta (the height of the patella is known to be a common predisposing factor). A disadvantage of distalization of the tendon attachment is a possible increase in pressure in the patellofemoral groove. To avoid this drawback, Maquet proposed the ventraliza- tion of the attachment. However, we would explicitly discourage this measure since the anterior transfer of the tibial tuberosity frequently produces severe symptoms at this protruding site. Other complications of the transposition of the tu- berosity include recurrent lateral dislocations, overcor- ⊡ Fig. Schematic view of the Roux-Hauser operation: The distal rections with medial dislocations and a low patella attachment of the patellar ligament is chiseled out with a block of (patella baja) as a result of scarring of the patellar tendon bone and fixed with a screw in a more medial (and possibly distal). Unless a pro- riceps muscle is detached from the femur and transposed nounced patella alta is present, the operation should be medially (⊡ Fig. Ideally, the Elmslie for congenital and habitual dislocations, and occasionally method should be used in which only the proximal part also for neuromuscular dislocations of the patella. We of the tuberosity is transposed, while the distal part is left recently followed up 7 patients with 9 congenital disloca- in place. Before surgery, an exten- Corrections involving the proximal extensor mechanism sion deficit of 20–50° had been present in all knees. At the The following procedures can be performed on the proxi- latest follow-up, 6 knees could be fully extended, while the mal extensor mechanism: other 3 showed a deficit of 10–20°. If indicated, therefore, ▬ Insall procedure for distalization of the vastus media- quadriceps transfer is a useful procedure. Two options for correcting the patellofemoral groove were recently proposed: The Insall procedure for distalization of the vastus medialis Elevation of the lateral femoral condyle by osteotomy attachment on the patella is indicated if the inser- according to W. This situation is Deepening of the intercondylar sulcus (trochleaplasty) usually linked with a patella alta.

For parents the realization that the child’s growth will » small is beautiful purchase 12.5 mg lopressor with visa. purchase lopressor 12.5 mg without a prescription.. The way in which this »crisis« is managed is hugely important for the child’s future. The greater the parents’ self reproach, the greater Classification the risk that the child will be spoilt and incapable, in later Classifying such a heterogeneous group as the congenital life, of living independently despite having the necessary disorders of the musculoskeletal system is not a simple intellectual abilities. But such a classification is needed in order treating doctors to avoid stirring up any feelings of guilt to create a common basis for professional discussions. The family and pregnancy The »Committee on Nomenclature on Intrinsic Diseases history must be taken with extreme sensitivity. Specific of Bones« of the European Society of Paediatric Radiology details should only be questioned if they are actually rel- was the first body to undertake this classification in 1971 evant to the diagnostic process. Seemingly trivial routine (»Paris Nomenclature«), which has since been revised questions (such as the administration of drugs or the several times, most recently in 2001. This primarily drinking of alcohol during pregnancy) can very easily clinically oriented classification was recently restructured lead to lifelong (unjustified) self reproach on the part of to take account of the findings of molecular genetics the mother. This international classification currently comprises 36 groups, including 33 with generalized disorders (os- » Childhood illnesses of the soul first manifest teochondrodysplasias) and 3 with localized deficiencies themselves in adults « (dysostoses). While the gene defect in most disorders is (Hans Weigel) already known [2, 5, 8], the gene product still remains Many patients with hereditary illnesses are perfectly nor- unclear in many cases. Some may show outstanding ar- of genetics, these gaps are also expected to be closed in tistic talent. The recently deceased Michel Petrucciani, whose »Committee on Nomenclature on Intrinsic Diseases small stature was associated with osteogenesis imperfecta, of Bones« was one of the greatest jazz pianists of this time. Even Osteochondrodysplasias the powerful figures of the world were not always tall and 1. Attila, the king of the Huns, King Charles III of Thanatophoric dysplasia Naples and Sicily and Napoleon were all said to be small Achondroplasia in stature. Short-rib dysplasias ▬ Asphyxiating thoracic dysplasia (Jeune syn- drome) ▬ Chondroectodermal dysplasia (Ellis-van-Creveld syndrome) 5. Type II collagenopathies ▬ Achondrogenesis II and hypochondrogenesis ▬ Spondyloepiphyseal dysplasia ▬ Kniest syndrome ▬ Stickler syndrome 648 4. Type XI collagenopathies Maffucci syndrome ▬ Otospondylomegaepiphyseal dysplasias Fibrous dysplasia, (monostotic, polyostotic, Mc- 10. Multiple epiphyseal dysplasia Fibrodysplasia ossificans progressiva ▬ Multiple epiphyseal dysplasia Cherubism ▬ Pseudoachondroplasia 32.

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By X. Cobryn. University of West Florida.

During this period generic lopressor 100 mg overnight delivery, inflammation and LANE discount lopressor 50 mg with mastercard, AND DAWSON, 1990) vascularization occur. Systemic factors can inhibit bone healing includ- process is dependent on the presence of live bone cells ing the following: in a graft material. Cigarette smoking (Glassman, 1998) Contain viable cells with the ability to form bone b. Malnutrition (Mankin, 1990) (osteoprogenitor cells) or the potential to differentiate c. Rheumatoid arthritis Osteogenesis is a property found only in fresh auto- e. Osteoporosis (Kelsey and Hoffman, 1987) genous bone and in bone marrow cells. Nonsteroidal anti-inflammatory medications GAZDAG ET AL, 1995) (Glassman et al, 2000) The physical property of the graft to serve as a scaf- Healing occurs in three distinct but overlapping stages fold for viable bone healing. Inflammatory cells and autografts and allografts, demineralized bone matrix, fibroblasts infiltrate the bone under prostaglandin hydroxyapatite, collagen, and calcium phosphate. This results in the formation of granulation tissue, ingrowth of vascular tissue, and migration of mesenchymal cells. OSTEOINDUCTION (CONNOLLY, 1998; MOHAN Exposed skin cells, bone, and muscle provide the pri- AND BAYLINK, 1991) mary nutrients of this early process. BMPs and demineralized bone matrix are the prin- REPAIR STAGE cipal osteoinductive materials. Autograft and allograft bone also have some osteoin- port vascular ingrowth. This leads to the formation of a soft ET AL, 1989) callus around the repair site. Callus is very weak in the first 4–6 weeks and slowly reabsorbed and simultaneously replaced with requires adequate protection (Kenwright and new viable bone, thus incorporating bone grafts Gardner, 1998).

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Usually an occipitocervical spon- An important task in the differential diagnosis is to rule dylodesis is performed in such cases generic 25 mg lopressor with mastercard. The atlantoaxial out secondary osteoporosis purchase 50 mg lopressor mastercard, which occurs in the follow- instability must also always be borne in mind during ing disorders: hyperthyroidism, hyperparathyroidism, any induction of anesthesia. The second problem that hypogonadism, Cushing syndrome, homocystinuria, occasionally requires treatment is the thoracolumbar vitamin C deficiency, rickets, Lowe syndrome, uremia, kyphosis, which generally leads to spondylolisthesis in Turner syndrome, leukemia, lymphoma and osteogen- this area. Treatment with corticosteroids, for in- further slippage and accentuation of the kyphosis. If stance in juvenile rheumatoid arthritis or Langerhans the coxa vara is very pronounced, a valgization oste- cell histiocytosis, can lead to severe osteoporosis. In some cases the valgus deformity ment, successful results have been obtained with of the rearfoot is severe enough to require a subtalar the bisphosphonate pamidronate (Aredia) [108]. In the event of a fractured extrem- Only isolated reports exist for the autosomal-recessive ity, the possibility of inactivity-induced osteoporosis microcephalic osteodysplastic dysplasia, which occurs should be avoided. The patients show moderately stunted should not be treated with cast fixation, and internal growth (which only becomes apparent during the course plate fixation is also highly inadvisable. A more appro- of growth), have short fingers, are microcephalic but with priate response is to use intramedullary load-bearing normal intelligence, the bones are generally very thin, and implants, provided these are compatible with open coxa vara and abnormally shaped epiphyses are typical epiphyseal plates (e. Overall bone mass decreases as a result of the increased resorption by osteoclasts, although there are no qualitative abnormali- ties. The laboratory tests do not show any major abnormalities apart from a negative calcium balance. This is an ex- tremely rare condition that is probably caused (like osteogenesis imperfecta) by an anomaly of type I collagen as a result of a mutation. The principal symptoms are dull back pain, possibly with symptoms in the extremities, particularly the feet, with a consequent adverse effect on gait. Lateral x-ray of the spine of a 5-year old boy withidiopathic hyperkyphosis.

lopressor 25 mg with mastercard

Schematic view of Goldthwait operation: The patellar cheap 12.5mg lopressor otc, tendon is split lengthwise and the lateral half is pulled over the ▬ Ventralization of the tibial tuberosity according to medial half and fixed on the medial side discount lopressor 100 mg visa, while the lateral retinacula Maquet. The advantage of this method is that it can be performed even if the apophyseal plate has not yet closed. The dis- advantage is a much higher recurrence rate compared to bone-based transpositions. In the procedure of medialization of the tibial tuberos- ity according to Roux and Hauser, a bone frag- ment with the complete patellar tendon attachment is chiseled out of the tibia, relocated laterally and fixed with one or more screws (⊡ Fig. This is particularly indicated for patients with a patella alta (the height of the patella is known to be a common predisposing factor). A disadvantage of distalization of the tendon attachment is a possible increase in pressure in the patellofemoral groove. To avoid this drawback, Maquet proposed the ventraliza- tion of the attachment. However, we would explicitly discourage this measure since the anterior transfer of the tibial tuberosity frequently produces severe symptoms at this protruding site. Other complications of the transposition of the tu- berosity include recurrent lateral dislocations, overcor- ⊡ Fig. Schematic view of the Roux-Hauser operation: The distal rections with medial dislocations and a low patella attachment of the patellar ligament is chiseled out with a block of (patella baja) as a result of scarring of the patellar tendon bone and fixed with a screw in a more medial (and possibly distal). Unless a pro- riceps muscle is detached from the femur and transposed nounced patella alta is present, the operation should be medially (⊡ Fig. Ideally, the Elmslie for congenital and habitual dislocations, and occasionally method should be used in which only the proximal part also for neuromuscular dislocations of the patella. We of the tuberosity is transposed, while the distal part is left recently followed up 7 patients with 9 congenital disloca- in place. Before surgery, an exten- Corrections involving the proximal extensor mechanism sion deficit of 20–50° had been present in all knees. At the The following procedures can be performed on the proxi- latest follow-up, 6 knees could be fully extended, while the mal extensor mechanism: other 3 showed a deficit of 10–20°. If indicated, therefore, ▬ Insall procedure for distalization of the vastus media- quadriceps transfer is a useful procedure. Two options for correcting the patellofemoral groove were recently proposed: The Insall procedure for distalization of the vastus medialis Elevation of the lateral femoral condyle by osteotomy attachment on the patella is indicated if the inser- according to W. This situation is Deepening of the intercondylar sulcus (trochleaplasty) usually linked with a patella alta.

For parents the realization that the child’s growth will » small is beautiful purchase 12.5 mg lopressor with visa. purchase lopressor 12.5 mg without a prescription.. The way in which this »crisis« is managed is hugely important for the child’s future. The greater the parents’ self reproach, the greater Classification the risk that the child will be spoilt and incapable, in later Classifying such a heterogeneous group as the congenital life, of living independently despite having the necessary disorders of the musculoskeletal system is not a simple intellectual abilities. But such a classification is needed in order treating doctors to avoid stirring up any feelings of guilt to create a common basis for professional discussions. The family and pregnancy The »Committee on Nomenclature on Intrinsic Diseases history must be taken with extreme sensitivity. Specific of Bones« of the European Society of Paediatric Radiology details should only be questioned if they are actually rel- was the first body to undertake this classification in 1971 evant to the diagnostic process. Seemingly trivial routine (»Paris Nomenclature«), which has since been revised questions (such as the administration of drugs or the several times, most recently in 2001. This primarily drinking of alcohol during pregnancy) can very easily clinically oriented classification was recently restructured lead to lifelong (unjustified) self reproach on the part of to take account of the findings of molecular genetics the mother. This international classification currently comprises 36 groups, including 33 with generalized disorders (os- » Childhood illnesses of the soul first manifest teochondrodysplasias) and 3 with localized deficiencies themselves in adults « (dysostoses). While the gene defect in most disorders is (Hans Weigel) already known [2, 5, 8], the gene product still remains Many patients with hereditary illnesses are perfectly nor- unclear in many cases. Some may show outstanding ar- of genetics, these gaps are also expected to be closed in tistic talent. The recently deceased Michel Petrucciani, whose »Committee on Nomenclature on Intrinsic Diseases small stature was associated with osteogenesis imperfecta, of Bones« was one of the greatest jazz pianists of this time. Even Osteochondrodysplasias the powerful figures of the world were not always tall and 1. Attila, the king of the Huns, King Charles III of Thanatophoric dysplasia Naples and Sicily and Napoleon were all said to be small Achondroplasia in stature. Short-rib dysplasias ▬ Asphyxiating thoracic dysplasia (Jeune syn- drome) ▬ Chondroectodermal dysplasia (Ellis-van-Creveld syndrome) 5. Type II collagenopathies ▬ Achondrogenesis II and hypochondrogenesis ▬ Spondyloepiphyseal dysplasia ▬ Kniest syndrome ▬ Stickler syndrome 648 4. Type XI collagenopathies Maffucci syndrome ▬ Otospondylomegaepiphyseal dysplasias Fibrous dysplasia, (monostotic, polyostotic, Mc- 10. Multiple epiphyseal dysplasia Fibrodysplasia ossificans progressiva ▬ Multiple epiphyseal dysplasia Cherubism ▬ Pseudoachondroplasia 32.

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