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[Rheumatologic effects of etretinate].

The effects of chronic hypervitaminosis A and long-term isotretinoin treatment on bone include cortical hyperostosis, ligament calcification and premature epiphyseal closure. Similar effects have now been reported in patients under maintenance treatment with etretinate in high doses. Etretinate, an oral, aromatic, synthetic vitamin A derivative, is widely used in Europe for disorders of keratinization. We report the cases of two patients--one with lamellar ichthyosis, the other with pachyonychia congenita--who developed such bone diseases during treatment with etretinate over 2 and 6 years respectively. The doses ranged from 0.5 to 1 mg/kg/day. Two years after starting treatment (total dose 25 g), the patient with lamellar ichthyosis complained of mechanical pain in the lumbar region and hips. Radiography showed calcification of the extraspinal tendons and ligaments and hyperostosis of the calcaneus bone at the insertion of the plantar ligament. After six years of etretinate treatment (total dose 50 g), the patient with pachyonychia congenita presented with scoliosis and limb length discrepancy. The musculoskeletal abnormalities resembled chronic hypervitaminosis A, with such osseous changes as demineralization, thinning and increased curvature of long bones with osteopenia, and premature closure of the epiphyses. Acroosteolysis was also present. Etretinate has been implicated in the formation of spinal hyperostoses and calcification of extraspinal ligaments in patients who had taken the drug for many years. The occurrence of premature epiphyseal closure in children certainly is a consequence of therapy with relatively high doses of etretinate for six years. But premature epiphyseal closure may also result from trauma to a fragile bone.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Pediatric update #7. The orthopaedic manifestations of head injury in children.

The incidence of significant head injury has been estimated at 220/100,000 children. Over 90% of these will recover with little residual disability. The purpose of this study is to re-examine the small but significant percentage of head injured children with permanent total body involvement to identify patterns of deformity and temporal sequences and to develop preventive treatment regimens. Sixteen head injured children with residual spastic quadriplegia were examined. All exhibited musculoskeletal abnormalities. Hip adduction contractures were most common leading to hip subluxation in eight children, followed by pes equinus, scoliosis, pelvic obliquity, and knee flexion contracture. The average time to onset of permanent foot deformity was 11 months after head injury, for scoliosis an average of 22 months postinjury, and for hip subluxation an average of 31 months postinjury. Hamstring contractures occurred later, at an average of 37 months, but caused the most interference with good seating. The physical problems of the head injured child have some similarities to those of the child with cerebral palsy, but with distinct differences. An aggressive surgical approach to prevention and treatment of fixed deformity in these children is recommended at an early stage with postoperative orthotic management and stable seating in abduction.

Child↗

[Backache in a hospital environment: epidemiologic aspects and the role of various risk factors].

A cross-sectional study was carried out in 1985 among 5941 hospital workers from the Regional and University Hospital Center (CHRU) of Strasbourg on the occasion of the occupational medical examinations. The purpose was to determine the importance of low back pain among hospital staff and to describe the relationship between individual and occupational characteristics and the onset of these symptoms. Our results confirm the risk-factors determined by other investigators: age, sex, length of service, heavy work assignments (nursing aides, stretcherbearers, nurses, etc.) but also underline the role of obesity and previous attacks of low back pain, and invalidate the part musculoskeletal abnormalities play in this pathology.

Adult↗

Low-back pain in industry. An old problem revisited.

Low-back pain is a major occupational health problem. Risk factors predisposing to the development of low-back pain are discussed. These include individual risk factors such as age, sex, anthropometry, musculoskeletal abnormalities, muscle strength and physical fitness, psychological factors, and previous attacks of low-back pain and workplace factors such as heavy work, lifting, bending, and slipping. Various programs for prevention are evaluated. These include selection of workers, education and training regarding lifting methods, design of lifting jobs, and fitness training. Limitations of the various studies of these programs are discussed. Preemployment strength testing and ergonomic job design together appear to offer the greatest promise.

Adult↗

Musculoskeletal problems in blind children.

At a regional school for the blind, 157 children were examined for musculoskeletal abnormalities. The incidence of scoliosis was 9.5 per cent; of foot deformity, 53.5 percent; and of ligament laxity, 25 per cent. Thirty-six children (23 per cent) had evidence of spastic, flaccid, or athetoid cerebral palsy or other disorders of the central nervous system. The orthopaedist should evaluate blind children carefully and repeatedly for evidence of occult cerebral palsy and progressive deformity of the spine or foot.

Abnormalities, Multiple↗

Developmental flatfoot.

An overview of the clinical entity referred to as developmental flatfoot has been discussed. Specific reference has been made to its occurrence, etiology, identification, pathomechanics, clinical significance, and management rationale. This often overlooked, inconspicuous condition is the most common musculoskeletal abnormality affecting the foot of the child under 6 years of age. Recognition of the fact that the developmental flatfoot is the logical precursor of foot dysfunction, deformity, and resultant disability later in life will allow the practitioner to design a management program for today that will meet the foot health needs of tomorrow.

Biomechanical Phenomena↗

Compressive, invasive referred pain to the shoulder.

The painful shoulder can be the presenting complaint of a wide variety of local, as well as systemic, problems. Compression of the brachial plexus or occlusion of the subclavian vessels caused by narrowing of the thoracic outlet is best diagnosed by a thorough history and physical examination, and by performing the appropriate test maneuvers. Patients who do not respond to an exercise program to strengthen the shoulder girdle may require surgical excision of the first rib to enlarge the thoracic outlet. Referred pain in the shoulder originating from the cervical spine or peripheral nerves can be determined by cervical spine roentgenograms and nerve conduction tests. Neoplasms and parenchymal disease of the lung, and infections of the pleural and subdiaphragmatic areas can refer pain to the shoulder through the phrenic nerve. For those patients with persistent shoulder pain and no discernible musculoskeletal abnormalities who do not respond to standard therapeutic measures, another source of the shoulder pain should be sought.

Diagnosis, Differential↗

[Malignant hyperthermia in a black child. A case report].

A case of malignant hyperthermia in a Black boy is presented. He developed this condition during repair of a cleft palate, with halothane as the triggering agent. The importance of the high incidence of malignant hyperthermia in patients with certain musculoskeletal abnormalities is stressed. Despite a cool and well air-conditioned theatre, the patient's temperature was 41 degree C when the condition was suspected. At that stage general muscle rigidity was present. The patient was successfully treated with procainamide, sodium bicarbonate and hydrocortisone; surface cooling (with ice packs) was instituted and the stomach was washed out with ice-cold Ringer's solution. Over a period of 14 days serum creatine phosphokinase values decreased from 630 IU (on the day of the incident) to 12 IU. A muscle biopsy showed variation in muscle fibre size. Electron microscopical studies showed myofibrillar disruption and folding of the basement membrane. A modified version of Denborough's technique was used for the in vitro exposure of muscle strips to halothane and suxamethonium. Isometric contraction was measured and recorded. A severe contraction followed the exposure of muscle strips to halothane, which confirmed the diagnosis.

Child↗

[Malignant hyperthermia during the 13th general anaesthesia (author's transl)].

We report a case of malignant hyperthermia in a man of 41 years during his 13th general anaesthesia. All previous anaesthetics were quite normal. Musculoskeletal abnormalities and increased CPK-levels are to be found in some members of the patient's family. The combined use of suxamethonium and halothane might have caused the development of malignant hyperthermia. As a concept of the aetiology of the syndrome the case history indicates that it may be stress-related.

Adult↗

A study of a family with inherited disease of cardiac and skeletal muscle. Part I. Clinical, electrocardiographic, echocardiographic, haemodynamic, electrophysiological and electron microscopic studies.

A family consisting of parents and their 6 sons were investigated to elucidate the relationship between a hypertrophic cardiomyopathy, musculoskeletal abnormalities and mental subnormality. The proband was diagnosed as having definite hypertrophic obstructive cardiomyopathy and the remaining family members were shown to have a spectrum of hypertropic non-obstructive cardiomyopathy. Mild muscle weakness was present in 3 sons. All the subjects except for 1 son showed definite signs of electromyographic abnormality, whereas sensory and motor conduction velocities were normal. All the EEGs except for that of the proband were normal. Testicular hypoplasia was present in 3 sons. The inheritance pattern appears to be polygenic autosomal recessive in type. Definite evidence of linkage between hypertrophic cardiomyopathy and HLA awaits further data.

Adolescent↗

Painful spondylolisthesis in a two-year-old child.

Spondylolisthesis is considered one of the common causes of back pain in children. There are far fewer cases reported in children under the age of 4 years. However, all patients described were diagnosed by radiographic examination in a workup for other congenital or musculoskeletal abnormalities. This report describes a 2-year-old girl who presented with low back pain and was subsequently diagnosed with spondylolisthesis.

Child, Preschool↗

[A case of Stickler's syndrome].

The authors presented a case of Stickler's syndrome diagnosed in 56-year-old woman referred to the clinic because of cataract in both eyes. After surgery typical vitreo-retinal changes mainly extensive lattice degenerations, pigmentary lesions and preretinal membranes in vitreous were found. Orofacial and musculoskeletal abnormalities confirmed diagnosis.

Female↗