Skeletal maturation in individuals with Down's syndrome: comparison between PGS curve, cervical vertebrae and bones of the hand and wrist.

INTRODUCTION: This study was conducted with the aim of adapting the methods developed by Martins and Sakima to assess skeletal maturation by cervical vertebrae in the pubertal growth spurt (PGS) curve. It also aimed to test the reliability and agreement between those methods and the method of hand and wrist radiograph when compared two by two and all together.  METHODS: The sample comprised 72 radiographs, with 36 lateral radiographs of the head and 36 hand-wrist radiographs of 36 subjects with Down's syndrome (DS), 13 female and 23 male, aged between 8 years and 6 months and 18 years and 7 months, with an average age of 13 years and 10 months.  RESULTS AND CONCLUSIONS: Results revealed that adapting the methods developed by Martins and Sakima to assess skeletal maturation by cervical vertebrae in the PGS curve is practical and useful in determining the stage of growth and development of individuals. The stages of maturation evaluated by cervical vertebrae and ossification centers observed in radiographs of the hand and wrist were considered reliable, with excellent level of agreement between the methods by Hassel and Farman as well as Baccetti, Franchi and McNamara Jr and Martins and Sakima. Additionally, results revealed an agreement that ranged between reasonable to good for the three methods used to assess the skeletal maturation, showing statistical significance.

Musculoskeletal and cutaneous sarcoidosis: exuberant case report.

Sarcoidosis is a multisystem granulomatous disease of unknown cause. The osteoarticular involvement in sarcoidosis is rare and is often associated with cutaneous and long-standing chronic multisystem disease. More common in black women, osseous sarcoidosis is difficult to diagnose, with an incidence of 3 to 13%. The most characteristic radiological clinical picture evidences rounded, well-defined cysts, with no periosteal reaction and without peripheral sclerosis. The small bones of hands and feet are the most frequently involved sites. This report aims to demonstrate a rare case of osteoarticular sarcoidosis with characteristic clinical presentation, and highlight the importance of detecting osteoarticular involvement in this pathology.

New software for cervical vertebral geometry assessment and its relationship to skeletal maturation--a pilot study.

OBJECTIVES: In the present study, we developed new software for quantitative analysis of cervical vertebrae maturation, and we evaluated its applicability through a multinomial logistic regression model (MLRM). METHODS: Digitized images of the bodies of the second (C2), third (C3) and fourth (C4) cervical vertebrae were analysed in cephalometric radiographs of 236 subjects (116 boys and 120 girls) by using a software developed for digitized vertebrae analysis. The sample was initially distributed into 11 categories according to the Fishman's skeletal maturity indicators and were then grouped into four stages for quantitative cervical maturational changes (QCMC) analysis (QCMC I, II, III and IV). Seven variables of interest were measured and analysed to identify morphologic alterations of the vertebral bodies in each QCMC category. RESULTS: Statistically significant differences (p < 0.05) were observed among all QCMC categories for the variables analysed. The MLRM used to calculate the probability that an individual belonged to each of the four cervical vertebrae maturation categories was constructed by taking into account gender, chronological age and four variables determined by digitized vertebrae analysis (Ang_C3, MP_C3, MP_C4 and SP_C4). The MLRM presented a predictability of 81.4%. The weighted κ test showed almost perfect agreement (κ = 0.832) between the categories defined initially by the method of Fishman and those allocated by the MLRM. CONCLUSIONS: Significant alterations in the morphologies of the C2, C3 and C4 vertebral bodies that were analysed through the digitized vertebrae analysis software occur during the different stages of skeletal maturation. The model that combines the four parameters measured on the vertebral bodies, the age and the gender showed an excellent prediction.

Comparative analysis between three methods of bone estimating age in individuals with down syndrome by mode of the hand and wrist ray.

The wrist and hand region has been the most commonly used for estimating age and osseous development due to the great number of ossification centers. The aim was to determine which method, Tanner & Whitehouse's (TW3), Greulich & Pyle's (GP) or Eklof & Ringertz's, more closely relates to the chronological age in subjects with Down syndrome with chronological ages between 61 and 180 months, using wrist and hand radiographs. The sample consisted of 85 radiographs, 52 of males and 33 of females. Eklof & Ringertz's method was computerized (Radiomemory). Greulich & Pyle's atlas was used and compared with the wrist and hand radiographs. For the TW3 method, 13 ossification centers were evaluated; for each one of them, there are seven or eight development stages to which scores are assigned; these scores are then added and the results are transformed into osseous age values. No statistically significant differences were observed between the male and female genders for methods TW3 and GP, contrasting with the observed differences for the Eklof & Ringertz method. Correlation (r2) between osseous and chronological ages was 0.8262 for TW3 and 0.7965 for GP, while for the method of Eklof & Ringertz, it was 0.7656 for females and 0.8353 for males. The author concluded that the osseous age assessment method that better related to the chronological age was the TW3, followed by Greulich & Pyle's and Eklof & Ringertz's.

Correlation between hand/wrist and panoramic radiographs in severe secondary hyperparathyroidism.

OBJECTIVES: Hand/wrist and dental radiographs are important for osteoporosis analysis in secondary hyperparathyroidism (SHPT). This study evaluated whether a correlation exists between the effects of the disease on the hands and jaws, and investigated the association between osteoporosis progression in the hands and parathyroid hormone (PTH) levels in chronic kidney disease (CKD) patients. MATERIALS AND METHODS: Four panoramic radiographic parameters (mental index, mandibular cortical index, trabecular bone pattern, and calcification/resorption) and four corresponding hand/wrist radiographic parameters (metacarpal cortical thickness, phalangeal cortical index, trabecular bone pattern, and calcification/resorption) were applied to investigate possible correlation between the effects of SHPT on the jaws and hands/wrists, by Spearman's correlation coefficient. PTH levels and the hand/wrist radiographic parameters were also tested by spearman's correlation coefficient (p < 0.05). The presence of brown tumors, vascular calcifications, and acroosteolysis on the hands was also evaluated. RESULTS: Mandibular cortical index was strongly correlated with the phalangeal cortical index (p = 0.000). Phalangeal cortical index and trabecular bone pattern of hand/wrist correlated with PTH levels (0.002 and 0.000, respectively). Brown tumors occurred in four CKD patients, while both vascular calcifications and acroosteolysis were observed in 19 patients. CONCLUSION: There is a significant correlation between the morphological changes caused by secondary hyperparathyroidism in hand and jaw bones. The morphological status can be assessed using the mandibular cortical index, besides the phalangeal cortical index. The latter correlates well with parathyroid hormone levels of advanced chronic kidney disease. CLINICAL RELEVANCE: Panoramic images reveal morphological changes in the jaw bone, indicating likewise changes in the hand/wrist in severe secondary hyperparathyroidism. The severity of the bone changes may be a reflection of the parathyroid hormone levels in advanced chronic kidney disease.

Prevalence of radiological findings among cases of severe secondary hyperparathyroidism.

CONTEXT AND OBJECTIVE: Patients with end stage renal disease (ESRD) and secondary hyperparathyroidism (HPT2) are prone to develop heterotopic calcifications and severe bone disease. Determination of the sites most commonly affected would decrease costs and patients' exposure to X-ray radiation. The aim here was to determine which skeletal sites produce most radiographic findings, in order to evaluate hemodialysis patients with HPT2, and to describe the most prevalent radiographic findings. DESIGN AND SETTING: This study was cross-sectional, conducted in one center, the Hospital Universitário Clementino Fraga Filho (HUCFF), in Rio de Janeiro, Brazil. METHODS: Whole-body radiographs were obtained from 73 chronic hemodialysis patients with indications for parathyroidectomy due to severe HPT2. The regions studied were the skull, hands, wrists, clavicles, thoracic and lumbar column, long bones and pelvis. All the radiographs were analyzed by the same two radiologists, with great experience in bone disease interpretation. RESULTS: The most common abnormality was subperiosteal bone resorption, mostly at the phalanges and distal clavicles (94% of patients, each). 'Rugger jersey spine' sign was found in 27%. Pathological fractures and deformities were seen in 27% and 33%, respectively. Calcifications were presented in 80%, mostly at the forearm fistula (42%), abdominal aorta and lower limb arteries (35% each). Brown tumors were present in 37% of the patients, mostly on the face and lower limbs (9% each). CONCLUSION: The greatest prevalence of bone findings were found on radiographs of the hands, wrists, lateral view of the thoracic and lumbar columns and femurs. The most prevalent findings were bone resorption and ectopic calcifications.

[Genetic and ophthalmological assessment of patients with type II Stickler syndrome]. / Avaliação genética e oftalmológica de pacientes com síndrome de Stickler tipo II.

PURPOSE: To diagnose, evaluate and describe the clinical, genetic and ophthalmic characteristics of a family with type II Stickler syndrome. METHODS: X-rays for bone age, clinical and genetic evaluation were performed in all patients with ocular alterations. The Stickler syndrome diagnosis was established after correlating these examinations. RESULTS: Type II Stickler syndrome was found in 11 patients. The most important ocular findings were: high myopia (80%), lens subluxation (70%), exotropia (50%) and vitreoretinal abnormalities (80%) including vitreous cavity (50%). The clinical genetic examination disclosed that 30% of the patients had micrognathia, 50% hearing loss, 40% nasal depression and 60% high palate. Seven cases had articular hypermotility and long fingers and arthropathy was present in 3 cases. CONCLUSION: Diagnosis of the Stickler syndrome is difficult due to its phenotypic variability and the existence of other genetic syndromes with similar characteristics. Hand and wrist radiographs are of particular importance in the diagnosis of this syndrome.