Elimination of human rabies in Goa, India through an integrated One Health approach.

Dog-mediated rabies kills tens of thousands of people each year in India, representing one third of the estimated global rabies burden. Whilst the World Health Organization (WHO), World Organization for Animal Health (OIE) and the Food and Agriculture Organization of the United Nations (FAO) have set a target for global dog-mediated human rabies elimination by 2030, examples of large-scale dog vaccination programs demonstrating elimination remain limited in Africa and Asia. We describe the development of a data-driven rabies elimination program from 2013 to 2019 in Goa State, India, culminating in human rabies elimination and a 92% reduction in monthly canine rabies cases. Smartphone technology enabled systematic spatial direction of remote teams to vaccinate over 95,000 dogs at 70% vaccination coverage, and rabies education teams to reach 150,000 children annually. An estimated 2249 disability-adjusted life years (DALYs) were averted over the program period at 526 USD per DALY, making the intervention 'very cost-effective' by WHO definitions. This One Health program demonstrates that human rabies elimination is achievable at the state level in India.

Correction of lower limb deformities in children with renal osteodystrophy by guided growth technique.

PURPOSE: Renal osteodystrophy (ROD) may cause severe lower limb deformities in children. The purpose of this study is to evaluate the efficacy of the temporary hemiepiphysiodesis for the correction of lower limb deformities in children with ROD. METHODS: Guided growth correction by hemiepiphysiodesis has been performed in skeletally immature patients with deformities of the lower limbs caused by ROD. The correction of the mechanical axes of the lower limbs and its correction speed have been evaluated. RESULTS: A total of seven patients with ROD, five males and two females, were treated with the above technique. The average age of the patients at their first surgery was 7.8 years (2.9 to 13.6). The average follow-up time 5.2 years (2.3 to 8). There were 13 valgus deformities and one varus deformity of the knee. The measure of the lower limb angular deformity was in the range of 10° to 47°. Restoration of normal mechanical axis was achieved in all patients at the final follow-up. Three patients relapsed and required further hemiepiphysiodesis. The average time for correction was 20 months (7 to 30). The average speed of correction was 0.49° per month for a tibia and 1.73° per month for a femur. There were two minor complications: a screw mobilisation and a screw breakage occurred during removal. CONCLUSION: Guided growth technique by hemiepiphysiodesis is a mini-invasive surgical procedure that has been found to be effective for the correction of misalignment due to ROD in skeletally immature patients. The method has allowed progressive correction of the deformities at any age in childhood. These patients are predisposed to relapse to their deformities, thus a strict follow-up is required.

Receptive music therapy and salivary histamine secretion.

INTRODUCTION: Receptive music therapy (rMT) not only provides a good feeling but also a more effective healing process and mastery of stress. METHODS AND RESULTS: In a preliminary study it could be shown that American Doudouk-music (feel-good music) suppressed salivary histamine secretion in two groups (n = 4) of allergic and non-allergic young volunteers. Stress was induced by eating adverse food/allergenic food during music exposure. There was no response in the vein blood samples and no significant difference between the allergic and non-allergic groups. CONCLUSION: It can be concluded that saliva is an appropriate medium for histamine measurements during music exposure.

Comparison of intra-articular triamcinolone hexacetonide and triamcinolone acetonide in oligoarticular juvenile idiopathic arthritis.

OBJECTIVE: To compare the efficacy and safety of intra-articular triamcinolone hexacetonide (TH) and triamcinolone acetonide (TA) in children with oligoarticular juvenile idiopathic arthritis (JIA). METHODS: One hundred and thirty joints of 85 patients undergoing intra-articular injections were randomly treated with either TH or TA depending on the availability of the drug. The efficacy of both treatments was evaluated prospectively in a blinded fashion. A good response was defined as a decrease in the articular score of > or =60% from baseline. Clinical, laboratory and immunological variables were noted in order to examine possible factors, other than treatment, predictive of the result. RESULTS: Seventy injections were performed using TH and 60 with TA. The two groups were comparable for clinical, immunological and laboratory characteristics. The rate of response was significantly higher with TH than with TA: 81.4% vs 53.3% (P = 0.001) at 6 months, 67.1 vs 43.3% (P = 0.006) at 12 months, and 60 vs 33.3% (P = 0.002) at 24 months. CONCLUSION: At comparable doses TH appeared to be much more effective than TA for intra-articular use, in both short- and long-term follow-up. This result was not affected by disease duration or degree of local and systemic inflammation.

[Role of mutilating surgery in the treatment of non-chemosensitive pediatric soft tissue sarcomas. Experience of the Italian Cooperative Group Studies RMS-79 and RMS-88]. / Ruolo della chirurgia mutilante nel trattamento dei sarcomi delle parti molli non chemiosensibili in età pediatrica. Esperienza degli studi Cooperativi Italiani RMS-79 ed RMS-88.

UNLABELLED: Aim of the study was to evaluate the role of mutilating surgery in the patients with non chemosensitive soft tissue sarcomas (STS) registered in the Italian Studies. Between 1979 and 1995, 114 patients (pts) with "non chemosensitive" localized STS were enrolled in the cooperative studies RMS-79 (33) and RMS-88 (81). Median age: 89 months (range 1-194). HISTOLOGY: fibrosarcoma 29, Malignant Perpheral Nerve Sheath Tumors (MPNST) 40, malignant fibrous histiocytoma 5, hemangiopericytoma 6, leiomyosarcoma 4, others 20, STS nos 10. The cases were classified according to the IRS staging system as Gr. I 46, Gr. II 24, Gr. III 44. Twelve out of 114 pts (7%), 5/33 (14%) in the first study and 7/81 (8%) in the second, underwent mutilating surgery: 8 pts (of whom 3 were < 2 y of age) had a fibrosarcoma and 4 a MPNST. The mutilating procedure was carried out at diagnosis in 6 cases (4 in RMS-79 and 2 in RMS-and 88) and achieved radicality in 5/6 cases. It was performed after ineffective chemotherapy (CT) in 5 pts (1 in RMS-79 and 4 in RMS-88). One pt (RMS-88) underwent mutilation after multiple local recurrences treated with CT, radiotherapy and conservative excisions (RMS-88) and died. The procedures consisted of 5 amputations, 4 demolitive excisions of extremities with functional impairment, 1 nephrectomy, 1 excision of deferents with spermatic vesicae and 1 mastectomy. OUTCOME: At present 6/12 pts, 5 with fibrosarcoma and 1 with MPNST, are alive with no evidence of disease (NED), 4 of the first and 2 of the second study. Of the 5 Gr. I patients, 4 are alive (NED) and 1 died of 2nd tumor; 1 Gr. II pt is alive NED; of the 5 Gr. III pts 1 is alive NED and 4 died (3 of metastatic spread and 1 of 2nd tumor); the pt amputated after repeated local relapses (Gr. II) is dead of pulmonary metastases. CONCLUSIONS: In the RMS-79 study the mutilations were frequent and were performed at diagnosis in several cases; this trend decreased in the 2nd study in which chemotherapy was attempted in most of the patients. Probably the timing of mutilating procedures was not always adequate (too early or too late). The outcome of pts undergone mutilations is similar to that of the whole group of 114 pts, whose overall survival at 10 years is 64% (C.I. 60-77). Only fibrosarcomas and MPNST probably requires a more aggressive surgical behaviour. At present we avoid initial demolitive surgery; it should be planned without delay after the first local relapse or after initial ineffective CT and RT.

Primary re-excision: the Italian experience in patients with localized soft-tissue sarcomas.

Primary re-excision (PRE) is a wide, non-mutilating procedure carried out in patients with soft-tissue sarcomas (STS) when microscopic residuals are left after initial excision or when there are insufficient data on its completeness. The aim of this study was to evaluate the role of PRE in patients enrolled in two consecutive Italian studies between January 1988 and September 1999. Of 126 patients with grade IIa tumors. 53 underwent PRE (23 rhabdomyosarcomas [RMS] and 30 non-RMS STS [NRSTS]). The primary sites were the extremities in 20, paratesticular 15, trunk 9, head-neck-non-parameningeal (HNnPM) 6, bladder 1, other sites in 2; the tumor (T) status was T1a in 30, T1b in 10, T2a in 9, and T2b in 4; the median interval between primary surgery and PRE was 36 days. Of the 53 patients, 45 had complete histologic excision of the tumor (residuals were found in 21/45 specimens) and subsequently received chemotherapy (CT) alone: 39/45 are in their first complete remission (CR) with a median follow-up of 53 months; 6/45 (3 RMS, 3 NRSTS) relapsed, 4 locally (2 extremities, 2 trunk), and 1 of these died of progressive disease, and 2 with metastatic spread died of their disease. In 8/53 cases (HNnPM 4. extremities 2, bladder 1, trunk 1) PRE did not achieve complete removal of the residuals (3 T1a, 2 Tlb, 2 T2a, 1 T2b); these patients were treated with CT and/or radiotherapy (RT); 1 also underwent further surgery. PRE was able to achieve or confirm complete excision in 45/53 patients, and 39 maintained the first CR without RT. The histologic types and the presence of residuals at PRE did not predict the failures; PRE was effective especially in extremity, trunk, and paratesticular sites, whereas its role was uncertain in large sarcomas over 5 cm in size.

Spinal involvement in Pyle's disease.

BACKGROUND: Pyle's disease is a rare skeletal dysplasia involving the metaphyses of long bones. To date, spinal involvement has been only rarely described in the literature. OBJECTIVE: To show that spinal changes, which are an expression of the same growth defect of the long bones, are an important and constant sign of the disease in the families studied. MATERIALS AND METHOD: The radiographic skeletal changes in five patients have been observed. RESULTS: The pathognomonic metaphyseal widening of tubular bones (known as the 'Erlenmeyer flask sign') was associated with the spinal changes in all cases. Radiographic findings in the spine varied from moderate platyspondyly to the bodies having the appearance of a biconcave lens. This may be attributed to two main causes: (1) a defect in the modelling process of the vertebrae (comparable to that observed in the metaphyses) and (2) chronic pathological fractures secondary to osteoporosis (a typical feature of Pyle's disease). CONCLUSIONS: The finding of platyspondyly of varying severity widens the spectrum of radiographic findings in this disease and can assist in diagnosis.

Pseudomalignant myositis ossificans in children. Description of a case and review of the literature.

The authors report a rare case of pseudomalignant myositis ossificans occurring during childhood. A female aged 10 years with no previous history of trauma came to their observation with findings of pain and progressive swelling in the gluteal region. The severity of the clinical findings and the absence of characteristic ossification in the x-rays obtained at the onset of the disease suggested that neoplastic pathology be excluded. Diagnosis (bone scan, CT scan, MRI, angiography, biopsy) and the progression of the disease, until its complete clinical resolution, are discussed in light of a review of the literature (44 cases of pseudomalignant myositis ossificans during pediatric age). All of the elements that may be of help in diagnosis and capable of avoiding surgical procedures that are either untimely or improper are emphasized.

Voluntary hip dislocation in Down's syndrome: report of two cases.

Two cases of monolateral voluntary hip dislocation in Down's syndrome are reported. No surgical procedure was permitted by the family in either case, thus showing the natural evolution of the joint morphology. In the case observed a year after the onset of hip dislocation CT Scan already showed an initial smoothing of the posterior acetabular wall. In the other case, after a 9 year follow-up, the voluntary hip dislocation was followed by progressive subluxation and fixed dislocation at the end. This suggests that only early surgical treatment can prevent this outcome.

Spondylocostal dysplasia (Jarcho-Levin syndrome).

The authors report 3 cases of rare spondylocostal dysplasia (Jarcho-Levin syndrome) associated with multiple visceral anomalies (V.A.C.TE.R.L. syndrome). The difficulty encountered in treating the disease because of the complexity and multiplicity of the visceral anomalies, and the extent and type of associated vertebral deformities is emphasized. The cases described revealed a moderate amount of progression, and were able to be treated non-surgically. It is also emphasized that even when there is moderate scoliosis associated costal deformities may cause anti-cosmetic thoracic gibbosity.

Monoarticular juvenile chronic arthritis of the shoulder. Report of a case and review of the literature.

In 20% of the cases Juvenile Chronic Arthritis (JCA) has a monoarticular onset. Usually the inflammatory process spreads out to other joints with pauciarticular or polyarticular course. Very rarely the disease persists in one joint only and this is in about 70% of the cases, the knee. We describe a case of Monoarticular JCA with isolate and persistent involvement of the shoulder never reported in the literature. The clinical, pathologic features and different diagnoses are discussed.

A 20-year follow-up study of a case of surgically treated massive osteolysis.

Massive osteolysis of the humerus occurred in a 19-year-old male. The lesion was successfully treated with an autogenous fibular shaft transplant. During the 20-year follow-up period, humerus function was restored. Roentgenograms showed incorporation of the graft without any recurrence of the disease. This experience and reports in the literature show that only predominantly cortical autogeneic bone grafting has been successful. This could be due to the intrinsic characteristic of cortical bone. In fact, it seems that cortical bone shows greater resistance to erosion than cancellous bone to the lymphangiomatous osteolytic tissue. Therefore, autogenous bone grafting could be considered as a reasonable alternative to radiotherapy, particularly in young people with monostotic disease localized in bones that are easily operable and have no soft-tissue involvement.

[Septic arthritis in children]. / Considerazioni sull'artrite settica del bambino.

Seventy-five cases of children affected with septic arthritis for a total of 85 joints were followed-up after an average of 6 years and 9 months and evaluated according to Rigault clinical and radiographic criteria. Prognosis of the disease was related to age (less favorable results during neonatal age), to the joint affected (the hip and shoulder obtained the worst results, the knee the best), and to delay in diagnosis. Early and suitable surgical treatment associated with systemic antibiotic therapy is required.

[Torsional defects of the lower limb: an evaluation of the reliability of the results obtained by CT]. / Difetti torsionali dell'arto inferiore: valutazione dell'attendibilità dei risultati ottenibili con TC.

The torsional angle was calculated goniometrically of 9 femurs and 2 tibio-peroneal groups, and then compared to the values obtained from CT scans of the corresponding bones. The results show how the methods adopted up to now have been inaccurate and have yielded only gross definitions of the axis of the femoral neck. As far as this feature is concerned, an error of +/- 6.5 degrees can be estimated, with limits of confidence of 95%, after applying a more accurate system of measurement to the images and considering statistical interferences. After pointing out the advantages of this method, we suggest that the test performed in vivo be systematically proposed to all those patients in whom a more accurate diagnosis would lead to a different medical/surgical therapy considering that: 1) the inevitable error such a measurement bears can be contained between acceptable limits; 2) the radiation dose sufficient to distinguish the bone from the other surrounding tissues is much reduced with respect to the dosage necessary to perform a common CT of the inferior limbs. It comprises one topogram and four tomograms, with a total dose to the patient of approximately 10-20 mGy.

Primary clavicular localisation of Ewing's tumour treated by total cleidectomy. Case report and review of the literature.

The authors report a case of Ewing's sarcoma localised in the clavicle followed up 5 years after total cleidectomy. There was no recurrence and excellent function of the shoulder, which justifies this method as it may be the treatment of choice for this rare localisation as opposed to the less reliable partial cleidectomy.