Serum creatine kinase isoenzymes in children with osteogenesis imperfecta.

This study evaluates serum creatine kinase isoenzyme activity in children with osteogenesis imperfecta to determine its usefulness as a biochemical marker during treatment with bisphosphonate. The changes of creatine kinase (CK) isoenzyme activity during and after discontinuation therapy were observed. These results could be useful in addressing over-treatment risk prevention. INTRODUCTION: The brain isoenzyme of creatine kinase (CKbb) is highly expressed in mature osteoclasts during osteoclastogenesis, thus plays an important role in bone resorption. We previously identified high serum CKbb levels in 18 children with osteogenesis imperfect (OI) type 1 treated for 1 year with bisphosphonate (neridronate). In the present study, serum CK isoenzymes were evaluated in the same children with continuous versus discontinued neridronate treatment over a further 2-year follow-up period. METHODS: This study included 18 children with OI type 1, 12 with continued (group A) and 6 with ceased (group B) neridronate treatment. Auxological data, serum biochemical markers of bone metabolism, bone mineral density z-score, and serum total CK and isoenzyme activities were determined in both groups. RESULTS: Serum CKbb was progressively and significantly increased in group A (p < 0.004) but rapidly decreased to undetectable levels in group B. In both groups, the cardiac muscle creatine kinase isoenzyme (CKmb) showed a marked decrease, while serum C-terminal telopeptide (CTx) levels were almost unchanged. CONCLUSIONS: This study provides evidence of the cumulative effect of neridronate administration in increasing serum CKbb levels and the reversible effect after its discontinuation. This approach could be employed for verifying the usefulness of serum CKbb as a biochemical marker in patients receiving prolonged bisphosphonate treatment. Moreover, the decreased serum CKmb levels suggest a systemic effect of these drugs.

Clinical and biochemical response to neridronate treatment in a patient with osteoporosis-pseudoglioma syndrome (OPPG).

Osteoporosis-pseudoglioma syndrome (OPPG) is a rare autosomal recessive syndrome characterized by juvenile-onset osteoporosis and ocular abnormalities due to a low-density lipoprotein receptor-related protein 5 (LRP5) gene mutation. Treatment with bisphosphonates, particularly with pamidronate and risedronate, has been reported to be of some efficacy in this condition. We report on a patient with OPPG due to an LRP5 gene mutation, who showed an encouraging response after a 36-month period of neridronate therapy. We report a case of a patient treated with bisphosphonates. Bisphosphonates should be administered in OPPG patients as a first-line therapy during early childhood.

Outcome and technical consideration of conversion total hip arthroplasty after failed fixation of intracapsular and extracapsular hip fractures: Are they really that different?

INTRODUCTION: Conversion Total Hip Arthroplasty (cTHA) is a rescue strategy for proximal femur osteosynthesis failures. However, it is unclear whether cTHAs performed for extra-capsular fracture fixation failures (ECF) or for intra-capsular fracture fixation failures (ICF) share the same complexity and efficacy. The purpose of our study was to compare cTHAs performed on pre-existing ICFs and pre-existing ECFs, focusing on surgical complications and functional outcomes in both groups. METHODS: An observational retrospective study was conducted on cTHA patients, treated between 2014 and 2018, divided into 2 groups: ICF-group and ECF-group. The main outcomes were: type of implant used, duration of surgery, need for transfusions, incidence of complications, functional outcomes. RESULTS: 28 patients were included (15 in the ECF group and 13 in the ICF group); the average follow-up was of 31 ± 17.3 months. No significant differences were identified in terms of the type of implant used and duration of surgery. The number of transfused patients was 4 in the ICF group and 12 in the ECF group (p = 0.02); the average transfused units were 0.4 ± 0.7 in the ICF group and 1.3 ± 0.9 in the ECF group (p = 0.01). The incidence of complications - an infection and a dislocation, both of which occurred in the ICF group - and functional outcomes did not present significant differences. CONCLUSION: The conversion surgery on ECFs patients is technically more difficult for the surgeon and prone to greater blood loss. The outcomes are satisfactory and overlap between the two groups.

Analysis of the risk of a secondary displacement in conservati- vely treated paediatric distal radius metaphyseal fractures. A multicentric study.

OBJECTIVES: Distal radius fractures represent one of the most fre-quent injuries in children. The treatment of choice is a closed reduction followed by immobilisation in plaster cast; the immediate recourse to osteosynthesis with Kirschner wires is only reserved for certain cases. The displacement rate reported in the literature is 21-39%. The aim of this study is to retrospectively evaluate the risk factors for a secondary displacement of metaphyseal radius fractures in a paediatric population treated in three different centres. MATERIALS AND METHODS: The initial treatment for all 360 patients examined was a closed reduction under general anaesthesia and im-mobilisation in an above elbow cast for 4 weeks. The pre-operative displacement, residual post-reduction displacement and possible di-splacement at 7 and 14 days of follow-up were all assessed clinically and radiographically. RESULTS: A loss of reduction was reported in 102 cases; 51 under-went an additional reduction procedure - some followed by osteo-synthesis - while in the remaining 51 cases, the loss of reduction was acceptable in relation to the expectation of remodelling. The most statistically significant variable for the occurrence of a secondary displacement is a severe primary displacement. The association with the ulna fracture is not significantly correlated. The quality of the plaster cast is important for maintaining the reduction. There are a few things to consider as indicators for a second procedure: age, time elapsed from moment of fracture, fracture site and the absence of an acceptable reduction. CONCLUSIONS: In our experience, a reduction followed by osteo-synthesis with Kirschner wires should be considered the treatment of choice in fractures with a high risk of secondary displacement, namely those with severe initial displacement or unsatisfactory reduction.

A proposal of rehabilitative approach in the rare disease "De Barsy Syndrome": case report.

De Barsy syndrome is an autosomal recessive condition characterized by an progeroid appearance with distinctive facial features and cutis laxa. Ophthalmological, orthopedic, and neurological anomalies are generally also present. This syndrome is rare and the complex therapeutic management, from a surgical but also rehabilitative point of view, has not been recognized. The aim of this paper is to describe a possible rehabilitative protocol, after an orthopedic surgical treatment, in a child with De Barsy Syndrome. A 6-year-old boy was born with a congenital bilateral hip dysplasia associated with bilateral congenital foot deformity (vertical talus). Moreover, he showed stereotypic dyskinetic movements and psychomotor delay with cognitive impairment and absent language; the sitting position was maintained with orthoses to support the trunk control and the standing position was not acquired. He was treated with pinstripe knee-highs for the foot and double nappy for the hips. At 19 months old, he underwent a two stage surgical approach for a bilateral pronated valgus foot with severe talonavicular subluxation. Satisfactory hip range of motion was achieved by conservative treatment alone. Afterwards, for the foot laxity and the flat-pronated foot corrective shoes were prescribed. The main rehabilitative goals were: attention improvement, visual exploration for foot-eye and hand-eye coordination, encourage the essential prerequisites of language, controlling the upright position using support, improving hip-knee-foot relationship, improving load transfer between the right and left sides of the body, and bimanual coordination. The rehabilitation process lasted six months, three times a week, for a time from 30 minutes to 60 minutes per session. The results were encouraging and the patient acquired the possibility of sitting with the indicated postural system, the possibility of assuming an upright position and taking a few steps with the aid of rollator with a postural stabilization system for the pelvis.

The treatment of femoral fractures in children with cerebral palsy.

OBJECTIVE: The purpose of this study is to retrospectively evaluate a group of children affected by cerebral palsy with a recent femoral fracture, and to analyse the results and complications in relation to the treatment used. MATERIALS AND METHODS: The analysis was performed on 36 children (21 M, 15 F, 8-14 years old) with cerebral palsy (7 diplegia, 28 tetraparesis, 1 hemiplegia) with a metaphyseal or a diaphyseal femoral fracture. The patients were subdivided into two groups according to their Gross Motor Function Classification System (GMFCS) level: level 2-3 (9 patients) and level 4-5 (27 patients), evaluating the presence of complications and malunions for each group at the end of each follow up. RESULTS: The fractures were displaced in 24 patients and nondisplaced in 12 patients. In 26 cases the treatment involved a closed reduction and immobilisation in a long leg hip spica cast for 7 weeks, while in 10 cases the treatment involved an open reduction-internal fixation (ORIF) followed by a 3-week period in a plaster coated fracture bandage. CONCLUSIONS: Taking into consideration the maximum possible recovery of function, an ORIF is preferable to prevent malunion, particularly in distal metaphysis and distal shaft fractures. In the GMFCS level 2-3 patients, surgery has allowed to recover, or at least maintain, the pre-fracture functional level, while in patients with GMFCS level 4-5, it has allowed to reduce the immobilisation times and prevent the development of decubitus lesions.

Fluoroscopic freehand and electromagnetic-guided targeting system for distal locking screws of humeral intramedullary nail.

PURPOSE: The current techniques used to lock distal screws for the nailing of long bone fractures expose the surgeons, radiologists and patients to a hearty dose of ionizing radiation. The Sureshot™ Distal Targeting System is a new technique that, with the same results, allows for shorter surgery times and, consequently, less exposure to radiation. MATERIALS AND METHODS: The study was performed on 59 patients (34 males and 25 females) with a simple humerus fracture diagnosis, type 1.2.A according to the AO classification, who were divided into two groups. Group 1 was treated with ante-grade intramedullary nailing with distal locking screws inserted with a freehand technique. Group 2 was treated with the intramedullary nail using the Sureshot™ Distal Targeting System. Two intra-operative time parameters were evaluated in both groups: the time needed for the positioning of the distal locking screws and the time of exposure to ionizing radiations during this procedure. RESULTS: Group 2 showed a lower average distal locking time compared to group 1 (645.48″ vs. 1023.57″) and also a lower average time of exposure to ionizing radiation than in group 1 (4.35″ vs. 28.96″). CONCLUSION: The Sureshot™ Distal Targeting System has proven to be equally effective when compared to the traditional techniques, with the added benefits of a significant reduction in both surgical time and risk factors related to the exposure to ionizing radiation for all the operating room staff and the patient.

Assessment and therapeutic choice in septic arthritis of the hip in an intravenous drug abuser: case report at 14 years follow-up and review of literature.

INTRODUCTION: Osteoarticular infections are found frequently in drug addicted individuals, representing one of the main reasons for their hospitalization. Through inoculation, the pathogenic agents can enter the individual's system directly through the skin or parenterally, transmitted, that is, through syringes and other objects used during such practice. In these particular conditions, or when the medical history is vague, a warranted suspicion and the execution of targeted research can help in the diagnosis of high-risk patients such as addicts. DISCUSSION: With this paper, the Authors are presenting a case of septic arthritis in the hip joint, in a drug addicted patient with the habit of injecting narcotics into the femoral vein, in correspondence of the anatomical region known as the triangle of Scarpa. Following an examination of the bacterial culture samples taken by arthrocentesis, the S. Aureus infection was identified and a targeted antibiotic therapy (coxacillin and aminoglycosides) was prescribed. After one year, with clinical examination and medical scans resulting negative for infection, there was a remaining deformity of the femoral head and, therefore, a total hip arthroplasty (THA) was performed. The 14 year post-operative clinical examination and medical scan check-up showed a complete articular functionality and recovery of normal daily and work related activities.

Apophyseal and epiphyseal knee injuries in the adolescent athlete.

OBJECTIVES: In the context of pediatric sports injuries, the epiphyseal and apophyseal knee fractures represent rather peculiar lesions. The most frequently involved anatomical area is the knee. The peculiar function of the physis and the need to preserve their integrity, makes choosing what treatment methods to employ very important. Objective of this study is to assess the kind and the effectiveness of the most suitable treatment in the apophyseal and epiphyseal knee lesions occurring in the adolescents. MATERIALS AND METHODS: From 2006 to 2011, were treated 41 patients (34 M-7 F) between the ages of 10 and 15, with a diagnosis of traumatic knee injury caused by sports activities. Traumatic physeal fractures of the distal femur, the proximal tibia and its anterior tuberosity and the avulsion of the intercondylar eminence were the lesions that occurred most frequently. The treatment belonged to the type of lesion: closed reduction or percutaneous fixation with K-wires/ screws and a femoral-podalic plaster cast, ORIF with K-wires/screws, arthroscopic reduction and internal fixation using absorbable screws. All the patients were given the POSNA questionnaire at the end of the follow up. RESULTS: The follow up was on average 5 years (4-10 years). We considered as excellent the results obtained in 26 patients, as fair in 12 patients, in 1 case the result obtained was considered as poor. 2 caseswere lost during follow up. The average POSNA score at the end of the follow-up was 98.51. Any early complications recorded were the following: in 1 case infection of the K-wires 32 days after pinning and a reported compression of the popliteal neurovascular bundle, due to a displaced tibial physeal fracture. CONCLUSIONS: Since sport during childhood and adolescence is now practiced more and more frequently, also at a competitive level, thesekinds of fractures in children between the ages of 10 and 15 have been occurring more often, especially in male patients. Sports traumatology of the knee in this age group is characterized by a typology of injuries that are very particular. The knowledge of the anatomy and physiology of children, with an appropriate diagnostic assessment, is essential to identify the most appropriate treatment options for each specific injury. As the nucleus of proximal tibial growth plate progressively closes from posterior to medial side, in patients between 11 and 13 years of age with an apophyseal displacement of the tibial tuberosity, you should always perform a CT exam, to exclude an intra-articular physeal fracture.

A study of 44 patients with subtrochanteric fractures treated using long nail and cerclage cables.

OBJECTIVE: This study aims to demonstrate that the use of long cephalomedullary nail and cerclage cables represents a good strategy in order to reduce the high risk of nonunion of the most displaced subtrochanteric fractures. METHODS: This retrospective study examines 44 patients with average follow-up of 23 months, with subtrochanteric fracture type SH IIB, IIC, IIIA, IIIB treated by the same operator and with the same nail and cerclage cables. The clinical results which are derived from THRS have been reported. RESULTS: Clinical and radiological consolidation occurred in all 44 cases, without re-intervention. The average evaluation derived from the THRS was 48 which corresponds to good, according to the scale. CONCLUSIONS: Considering the anatomic reduction achieved in all patients and the clinical results, we can define the use of long cephalomedullary nail and cerclage cables as the most useful technique in the armamentarium of a trauma surgeon for the treatment of the subtrochanteric fractures.

[Use of nomegestrol acetate in the treatment of menstruation disorders. Our experience in 56 cases]. / Utilizzo del nomegestrolo acetato nella terapia delle irregolarità mestruali. Esperienza di 56 casi.

OBJECTIVE: To investigate tolerability and efficacy of nomegestrol acetate, a new 19-nor-progesterone derivative, in the treatment of oligomenorrhea and amenorrhea; to compare this drug with didrogesteron, a widely-used spatial isomer of progesterone. METHODS: 56 women, aged 22 to 50, affected by menstrual disorders or premenstrual syndrome, entered the study. They were divided in two homogeneous groups by randomization. In the first group nomegestrol acetate was administered 5 mg daily per os; in the second group patients were treated by the usual didrogesteron dose, that is 10 mg twice a day per os; both treatments were administered for 10 days from day 16, during three consecutive menstrual cycles. Efficacy, compliance and biological tolerance were evaluated. RESULTS: 24 patients in the group treated by nomegestrol acetate and 22 in the group treated by didrogesteron completed the study. After the first cycle of therapy, nomegestrol acetate showed a higher efficacy in reducing menstrual loss; results are statistically homogeneous in the two groups at the end of the study. There were no reports of pain in both the groups. Evaluation of biological and clinical parameters at the end of the study did not show significant modifications in any subject. CONCLUSIONS: Nomegestrol acetate is an innovative and efficient molecule in the treatment of menstrual disorders. A good response to the drug is evident in a short time and with very low doses; its good compliance, if compared with other progesteron-derivatives, indicates nomegestrol acetate as a first-line therapy for polymenorrhea, olygomenorrhea and premenstrual syndrome.

[Diagnostic accuracy in transvaginal echography in benign endometrial diseases and its comparison with hysteroscopic biopsy]. / Accuratezza diagnostica dell'ecografia transvaginale nell'approccio alle patologie endometriali benigne e suo confronto con l'isteroscopia con biopsia mirata.

Abnormal uterine bleeding is one of the main indications for hysteroscopy in peri-menopausal age. Transvaginal ultrasound allows for an accurate study of the endometrium through the evaluation of thickness, homogeneity. The aim of this retrospective study was the evaluation of transvaginal ultrasound versus hysteroscopy in diagnostic approach to abnormal uterine bleeding in peri- and post-menopause. 130 Women referred to the Department of Obstetrics and Gynecology of the University of Pavia for abnormal uterine bleeding entered the study; all them underwent transvaginal ultrasound and hysteroscopy with directed biopsy, with histological diagnosis of benign endometrial disease. On the basis of endometrial thickness measured at ultrasound, premenopausal patients were divided into two groups, post menopausal patients into three groups, and for each group a comparison between hysteroscopic findings, histological diagnosis and echographic aspect was performed. A 100% correspondence between ultrasound, hysteroscopy and histology was found in the group of subjects with endometrial atrophy, with an endometrial thickness of 2.9 +/- 0.68 at ultrasound; in the remaining groups sensibility and specificity of transvaginal ultrasound seem to be lower. In our experience a cut-off of 4 mm can be established for endometrial thickness measured by transvaginal ultrasound. Values that are below this cut-off point are diagnostic for endometrial atrophy, precluding the need for more invasive examinations: a medical treatment can then be administered. On the contrary, an endometrial thickening of 4 mm or more requires an hysteroscopic examination with directed biopsy and histological diagnosis.

[Preliminary experience with a new tumor marker in obstetrics and gynecology: UGP (Urinary Gonadotropin Protein)]. / Esperienze preliminari con un nuovo marcatore tumorale in ostetricia e ginecologia: UGP.

AIM: To evaluate the use of UGP (urinary gonadotropin protein) as a tumor marker in gynaecologic and obstetric malignant diseases. MATERIALS AND METHODS: The study was carried out in the division of Gynaecology and Obstetrics of the Ospedali Riuniti in Bergamo. 63 patients, with obstetric or gynaecologic benign or malignant diseases, entered the study. 66 healthy volunteers were examined as a group-control. In both the groups UGP levels were determined in morning urine, using an immunoenzymatic commercial kit. RESULTS: Results, expressed in fmol UGP/ml of urin, show that UGP is produced by several neoplasms, but the false-positive percentage is still high; a higher precision can be obtained with an accurate choice of the cut-off value and with a standardization of the analytical technics. Besides, the contemporary determination of UGP and CA 125 levels reduces the possibility of false-positive and false-negative results. CONCLUSIONS: More studies must be carried out to confirm the value of UGP as a tumor marker in obstetrics and gynaecology. Anyway, this recently purified protein can already be useful, in combination with the usual tumor markers, in the prompt diagnosis and management of primary neoplasms or recurrences, with a higher sensibility in comparison with traditional clinical and radiological examinations.

[Clinical characteristics of HCV infection in pregnancy: the role of sexual transmission]. / Caratteristiche cliniche dell'infezione da HCV in gravidanza: il ruolo della trasmissione sessuale. Analisi di 178 casi.

BACKGROUND: There is no uniformity of opinions about the possibility of sexual transmission of hepatitis-C-virus infection. Moreover the infection during pregnancy is often underestimated. METHODS: One hundred and seventy-eight anti-HCV-positive pregnant women were investigated to evaluate the incidence of HCV infection and the possibility of sexual transmission of the disease to the partners. RESULTS: 126 patients out of 178 (70.8%) were positive for viral infection at PCR. In 96 patients (53.9%) HCV-positivity was detected for the first time in the actual pregnancy. 147 male partners out of 178 were checked for HCV-positivity and in 31 of them (21.1%) HCV antibodies were found. CONCLUSIONS: The results underline the importance of a screening for HCV-positivity in every pregnant, searching for anti-HCV antibodies also in patients not reporting risk factors. ALT values seem to be of little importance in the monitoring of the pathology. Sexual transmission of HCV virus from woman to man seems to occur rarely.

[Combined use of goserelin acetate and human menopausal gonadotropin in the induction of follicular growth in a program of fertilization in vitro and embryo transfer]. / Uso combinato del Goserelin acetato e della gonadotropina umana menopausale nell'induzione della crescita follicolare in un programma di fertilizzazione in vitro e embrio-transfer.

OBJECTIVE: to investigate the efficacy of a gonadotropin-releasing hormone analogue (GN-RH-a) in combination with human menopausal menotropin (hMG) for in-vitro fertilization. METHODS: 30 infertile women aged 32 to 37 years received a combined treatment with a long-acting slow-releasing Gn-RH-a and hMG to perform ovarian stimulation in a program of in-vitro fertilization. Serum levels of Follicle Stimulating Hormone (FSH), Luteinizing Hormone (LH), 17-beta-estradiol (E2), Progesterone (P), were evaluated and transvaginal ultrasonographic examinations were performed during the treatment to assess the ovarian volume, the mean number and diameter of growing follicles and the endometrial morphology and thickness. Oocyte retrieval was performed by transvaginal-ultrasound-guided approach, 24-36 hours after the administration of human chorionic gonadotropin (hCG). RESULTS: our data suggest that the combined use of Gn-RH-a and exogenous gonadotropins is associated with a more uniform ovarian response and with the absence of premature LH discharge. Moreover, the Gn-RH-a as polymer implant provides a controlled delivery per day over a one-month period and avoids the inconvenience of a daily administration. CONCLUSIONS: this kind of Gn-RH-a formulation, in in-vitro fertilization programs, appears very effective in inducing a reversible hypogonadic state, easy to manage and well tolerated by the patient. Its association with exogenous gonadotropins appears to be effective in increasing the success rate of good quality oocyte retrieval.

Capsuloplasty for the treatment of recurrent anterior dislocation of the shoulder: long-term evaluations of the Putti-Platt method and Bankart method with anchors.

The authors compare two methods used for the treatment of recurrent anterior shoulder dislocations: Putti-Platt capsulomyoplasty, based on an overlapping suture of the free margins of the joint capsule and of the subscapularis muscle, and the Bankart method that uses anchors, based on the repair of the glenoid labrum, that is kept in site, together with the medial flap of the joint capsule, by suturing with the anchors previously stabilized to the neck of the glena. In this method, the subscapularis muscle is re-approximated without any overlapping or shortening. The Bankart method with anchors thus allows us to obtain anatomical repair of the lesion, with doubtless advantages for mobility, function and stability of the shoulder; this is confirmed by the comparison with results obtained out of 34 patients in this study, 15 of which submitted to the Putti-Platt method, 19 to the Bankart method with anchors. The latter had reduction of external rotation which in none of the cases exceeded 25% (a reduction that at times achieved 50% in patients submitted to the Putti-Platt method (p = 0.001); difficulties in work or sports were minimum or absent (p = 0.003), recovery time was more rapid, without compromising joint stability (p = 0.069); these data allow us to consider the Bankart method with anchors the surgical method that is most indicated for the treatment of anterior glenohumeral instability.

Flat foot: a comparison of surgical methods.

The authors present a comparative review of cases concerning the results obtained in the treatment of idiopathic flat foot: the methods compared were Castaman exosinotarsal arthrorisis and Giannini endosinotarsal arthrorisis. Between 1990 and 1198 a total of 80 patients, of which 40 with calcaneo-stop, and 40 with Giannini screws, were submitted to surgery at the Orthopaedic and Traumatology Department of the University of Rome "La Sapienza". The clinical, radiographic and functional results were classified as good, fair and poor. The authors agree on the effectiveness of both methods based on the results obtained and the facility of implantation techniques, however emphasizing the minor injury of the anatomical structures of the subtalar joint when the calcaneo-stop method with Castaman conical screws was used.

Epiphysiolysis of the hip: relationship between etiopathogenesis and hormone status.

The authors present a review of the literature on the etiology of epiphysiolysis of the hip and an original study on the hormone status of these patients. By examining eleven values including hormones, vector proteins, and hormonal transport, the authors related the data obtained to the anagraphical age of the patient, to his or her skeletal age, to the development of sexual features, and to the radiologic evidence of pathology. In light of the results obtained, despite the fact that the study only included 23 patients, the modified equilibrium between SHBG and Somatomedin C, would seem to lead to an accelerated turnover in the growth plate. The increase in BMI directly correlated with low levels of SHBG is also an indication of the increase in body weight that certainly contributes to slippage of the femoral head.