Orthotic Care Based on the Ferrari Concept for Children and Adults with Meningomyelocele

2019 ◽  
Vol 157 (06) ◽  
pp. 695-705
Author(s):  
Tim Schmid ◽  
Elisabeth Strehl ◽  
Regina Trollmann ◽  
Raimund Forst ◽  
Albert Fujak

Abstract Background Even today, myelomeningocele (MMC) is still encountered in clinical medicine and its incidence has not decreased over the last 20 years despite a known reduction in risk due to the use of folic acid supplements. The spectrum of clinical symptoms is extremely broad and, depending on the level of the defect, varies from mild to severe. Subject to the degree of paralysis, patients are reliant on the use of orthopaedic aids and orthoses for the treatment of primary contractures and deformities and the prevention of secondary ones. This forms the basis for attaining or maintaining mobility in many patients. The objective of the study was to determine the practical application of the proposed Ferrari concept for the provision of orthoses for children and adults. Patients and Material The retrospective study comprised medical records of 180 patients (97 m) with an average age of 19.44 years (3 – 52 years, SD 9.3) at the time of investigation. The average duration of treatment was 15.34 years (1 – 38 years, SD 8.96). Data relating to deformities of the vertebral column and lower limbs, provision of hydrocephalus shunts and orthoses, and patient mobility was evaluated. Results Most patients were given systematic treatment with orthoses at an early stage. In 58,9% of cases, it was possible to implement the proposed concept for providing patients with dynamic orthoses, whereby the treatment concept was more difficult to implement with high lumbar lesions than with lower lesions. Moreover, a decrease in the patientsʼ mobility with increasing age was noticeable. Some 42.3% of adult patients were able to walk with marked variations in mobility in relation to the different levels of lesions. Conclusion Taking into consideration the complexity of both the clinical picture and therefore the provision of orthopaedic devices, the result of the implementation of the proposed orthotic concept can be considered positive. Similarly, early commencement of provision of orthoses and hence the possibility of achieving a positive influence on later mobility can be considered a success. The need for individual concepts and further development in order to increase mobility particularly in the case of patients with thoracic or high lumbar lesions is evident. A more comprehensive provision of information to patients regarding orthotic treatment options and their consequences for prophylaxis and quality of life should be an important component of interdisciplinary long-term patient care.

2019 ◽  
Vol 1 (1) ◽  
pp. 16-38
Author(s):  
Mosaad A. Abdel-Wahhab ◽  
Aziza A. El-Nekeety ◽  
Soher E. Aly

Mycotoxins are ubiquitous compounds found in the natural life cycle of food- producing plants. They have a range of diverse chemical and physical properties and toxicological effects on man and animal. Mycotoxins are considered the most important contaminants of the food chain due to their chronic adverse effects on health and the economy. Mycotoxins are known as the 21th century “Great Masquerader” due to its complex natural history involving different tissues and resembling different diseases at each stage in its evolution. Mycotoxins can induce a variety of clinical symptoms including epistaxis, conjunctivitis, coughing, apnea, wheezing, vomiting and nausea. Some mycotoxins induce acute pulmonary hemorrhage, bone marrow failure and pneumonia. Knowledge about these symptoms enables the clinician to ask questions for possible exposure to the main classes of mycotoxins to protect children from sources of such exposure. These sources may include food, clothes, furniture and indoor air at home. Early childhood exposure to mycotoxins may be critical determinants of later health effects. Exposure in utero and through early infancy may additionally be important. Several well-known diseases such as neural tube defects, liver and esophageal cancers are associated with the consumption of mycotoxin-contaminated food. Knowledge of previous short or long term exposure to mycotoxins may help paediatricians to more accurately diagnose and provide treatment options to children and their families. The current review discusses the problems associated with the occurrence of different common mycotoxins in children’s food and the possible halal strategies to counteract these problems.  


BMJ ◽  
2020 ◽  
pp. m3544 ◽  
Author(s):  
Ju Dong Yang ◽  
Julie K Heimbach

ABSTRACT Hepatocellular carcinoma is one of the leading causes of cancer related death in the world. Biannual surveillance for the disease in patients with cirrhosis and in high risk carriers of hepatitis B virus allows early stage cancer detection and treatment with good long term outcomes. Liver ultrasonography and serum α fetoprotein are the most commonly used surveillance tests. If suspicious results are found on the surveillance test, multiphasic computed tomography or magnetic resonance imaging should be undertaken to confirm the diagnosis of hepatocellular carcinoma. If radiologic tests show inconclusive results, liver biopsy or repeat imaging could be considered for confirmation of hepatocellular carcinoma. Management of the disease is complex. Patients should be evaluated by a multidisciplinary team, and the selection of treatment should consider factors such as tumor burden, severity of liver dysfunction, medical comorbidities, local expertise, and preference of patients. Early stage hepatocellular carcinoma is best managed by curative treatment, which includes resection, ablation, or transplantation. Patients with intermediate stage disease often receive locoregional treatment. Systemic treatment is reserved for patients with advanced disease. Several positive, phase III, randomized controlled trials have expanded the systemic treatment options for advanced hepatocellular carcinoma with promising long term outcomes, especially trials using combination treatments, which could also have eventual implications for the treatment of earlier stage disease.


2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Yang Zhang ◽  
Zhigang Huang ◽  
Neil Gross ◽  
Jugao Fang ◽  
Xiaohong Chen ◽  
...  

Primary laryngeal carcinosarcoma is a rare type of malignancies, and the standard therapeutic protocol for its treatment has yet to be established. This retrospective study analyzed the clinical and pathological characteristics, risk factors, treatment options, and prognosis of 13 patients with primary laryngeal carcinosarcomas. This case series included 11 males and 2 females with an age range from 32 to 78 years at diagnosis. The initial clinical symptoms included hoarseness, dyspnea, and foreign body sensation in the throat. The primary tumor sites were at the glottis, supraglottis, and larynx. All these patients were active or passive smokers, and more than half of them were alcohol addictive. The surgical treatment for laryngeal carcinosarcomas included CO2 laser surgery in seven cases, partial laryngectomy and neck dissection in three cases, and total laryngectomy and neck dissection in three cases. Among them, seven cases received postoperative radiotherapy. After follow-up with a mean of 31.15-month, four cases died. Primary laryngeal carcinosarcoma is a rare but more aggressive malignancy. Contralateral lymph node metastasis can occur at the early stage of this disease. A treatment combining surgery and postoperative radiotherapy is strongly recommended.


2020 ◽  
Vol 29 (R1) ◽  
pp. R107-R116 ◽  
Author(s):  
Rexxi Prasasya ◽  
Kristen V Grotheer ◽  
Linda D Siracusa ◽  
Marisa S Bartolomei

Abstract Temple syndrome (TS) and Kagami-Ogata syndrome (KOS) are imprinting disorders caused by absence or overexpression of genes within a single imprinted cluster on human chromosome 14q32. TS most frequently arises from maternal UPD14 or epimutations/deletions on the paternal chromosome, whereas KOS most frequently arises from paternal UPD14 or epimutations/deletions on the maternal chromosome. In this review, we describe the clinical symptoms and genetic/epigenetic features of this imprinted region. The locus encompasses paternally expressed protein-coding genes (DLK1, RTL1 and DIO3) and maternally expressed lncRNAs (MEG3/GTL2, RTL1as and MEG8), as well as numerous miRNAs and snoRNAs. Control of expression is complex, with three differentially methylated regions regulating germline, placental and tissue-specific transcription. The strong conserved synteny between mouse chromosome 12aF1 and human chromosome 14q32 has enabled the use of mouse models to elucidate imprinting mechanisms and decipher the contribution of genes to the symptoms of TS and KOS. In this review, we describe relevant mouse models and highlight their value to better inform treatment options for long-term management of TS and KOS patients.


2019 ◽  
Vol 20 (22) ◽  
pp. 5765 ◽  
Author(s):  
Rajesh M. Jagirdar ◽  
Andreas Bozikas ◽  
Sotirios G. Zarogiannis ◽  
Maria Bartosova ◽  
Claus Peter Schmitt ◽  
...  

Encapsulating peritoneal sclerosis (EPS) is a life-threatening complication of long-term peritoneal dialysis (PD), which may even occur after patients have switched to hemodialysis (HD) or undergone kidney transplantation. The incidence of EPS varies across the globe and increases with PD vintage. Causative factors are the chronic exposure to bioincompatible PD solutions, which cause long-term modifications of the peritoneum, a high peritoneal transporter status involving high glucose concentrations, peritonitis episodes, and smoldering peritoneal inflammation. Additional potential causes are predisposing genetic factors and some medications. Clinical symptoms comprise signs of intestinal obstruction and a high peritoneal transporter status with incipient ultrafiltration failure. In radiological, macro-, and microscopic studies, a massively fibrotic and calcified peritoneum enclosed the intestine and parietal wall in such cases. Empirical treatments commonly used are corticosteroids and tamoxifen, which has fibrinolytic properties. Immunosuppressants like azathioprine, mycophenolate mofetil, or mTOR inhibitors may also help with reducing inflammation, fibrin deposition, and collagen synthesis and maturation. In animal studies, N-acetylcysteine, colchicine, rosiglitazone, thalidomide, and renin-angiotensin system (RAS) inhibitors yielded promising results. Surgical treatment has mainly been performed in severe cases of intestinal obstruction, with varying results. Mortality rates are still 25–55% in adults and about 14% in children. To reduce the incidence of EPS and improve the outcome of this devastating complication of chronic PD, vigorous consideration of the risk factors, early diagnosis, and timely discontinuation of PD and therapeutic interventions are mandatory, even though these are merely based on empirical evidence.


2012 ◽  
Vol 23 (4) ◽  
pp. 205-213 ◽  
Author(s):  
Anna Sotnikova ◽  
Elisabeth Steinmann ◽  
Vanessa Wendisch ◽  
Gabriele Gerber-von Müller ◽  
Ulrich Stephani ◽  
...  

Several studies have demonstrated that behavioural therapy oriented interventions exert a positive influence on the clinical course of the attention-deficit hyperactivity disorder (ADHD). However, the long-term effects of the behavioral treatment in ADHD, especially those on neuronal mechanisms underlying this disorder, have been studied insufficiently. Functional MRI (Go-NoGo paradigm) was carried out in 9 children with ADHD before and 1.5 years after a response cost and token-based training. In the follow-up, patients were still characterized by a significant increase in activation in the anterior cingulate and in the precentral gyrus compared with recordings done before the training. It seems likely that the behavioural training elicits stable neuronal changes in children with ADHD which correspond with an improvement of neuropsychological functioning and clinical symptoms.


2009 ◽  
Vol 67 (2b) ◽  
pp. 553-558 ◽  
Author(s):  
Andrei F. Joaquim ◽  
Charles A. Sansur ◽  
David K. Hamilton ◽  
Christopher I. Shaffrey

We present a literature review of the diagnosis and treatment of acquired lumbar spinal stenosis (LS), with a brief description of new surgical techniques. LS is the most common cause of spinal surgery in individuals older than 65 years of age. Neurogenic claudication and radiculopathy result from compression of the cauda equina and lumbosacral nerve roots by degenerated spinal elements. Surgical decompression is a well established treatment for patients with refractory, or moderate to severe clinical symptoms. However, the variety of surgical options is vast. New techniques have been developed with the goal of increasing long term functional outcomes. In this article we review lumbar decompression and fusion as treatment options for LS but also present other recent developments. Prospective long term studies are necessary to know which procedures would result in optimal patient outcome.


2014 ◽  
Vol 63 (2) ◽  
pp. 252-257 ◽  
Author(s):  
Tamalee Roberts ◽  
John Ellis ◽  
John Harkness ◽  
Deborah Marriott ◽  
Damien Stark

This article reports long-term infection and treatment failure in 18 symptomatic individuals infected with Blastocystis spp. Patients were initially treated with either metronidazole, iodoquinol or triple combination therapy consisting of nitazoxanide, furazolidone and secnidazole. Following treatment, resolution of clinical symptoms did not occur and follow-up testing revealed ongoing infection with the same subtype. Patients then underwent secondary treatment with a variety of antimicrobial agents but remained symptomatic with Blastocystis spp. still present in faeces. Sequencing of the SSU rDNA was completed on all isolates and four subtypes were identified in this group: ST1, ST3, ST4 and ST5. This study highlights the lack of efficacy of several commonly used antimicrobial regimens in the treatment of Blastocystis and the chronic nature of some infections. It also demonstrates the need for further research into treatment options for Blastocystis infection.


Author(s):  
Sabrina Giglio ◽  
Giovanni Montini ◽  
Francesco Trepiccione ◽  
Giovanni Gambaro ◽  
Francesco Emma

AbstractRenal tubular acidosis (RTA) comprises a group of disorders in which excretion of hydrogen ions or reabsorption of filtered HCO3is impaired, leading to chronic metabolic acidosis with normal anion gap. In the current review, the focus is placed on the most common type of RTA, Type 1 RTA or Distal RTA (dRTA), which is a rare chronic genetic disorder characterized by an inability of the distal nephron to secrete hydrogen ions in the presence of metabolic acidosis. Over the years, knowledge of the molecular mechanisms behind acid secretion has improved, thereby greatly helping the diagnosis of dRTA. The primary or inherited form of dRTA is mostly diagnosed in infancy, childhood, or young adulthood, while the acquired secondary form, as a consequence of other disorders or medications, can happen at any age, although it is more commonly seen in adults. dRTA is not as “benign” as previously assumed, and can have several, highly variable long-term consequences. The present review indeed reports and summarizes both clinical symptoms and diagnosis, long-term outcomes, genetic inheritance, epidemiology and current treatment options, with the aim of shedding more light onto this rare disorder. Being a chronic condition, dRTA also deserves attention in the transition between pediatric and adult nephrology care, and as a rare disease it has a place in the European and Italian rare nephrological diseases network.


2021 ◽  
Vol 27 ◽  
pp. 107602962110269
Author(s):  
Wenxu Jin ◽  
Guanfeng Yu ◽  
Jingyong Huang ◽  
Kangkang Lu ◽  
Chongqing Huang

The aim of this study is to explore the timing and method of endovascular intervention for iliac vein compression syndrome (IVCS) with thrombus. Data from 111 patients with IVCS, complicated acute deep vein thrombosis (DVT), or post-thrombotic syndrome (PTS) who underwent endovascular interventions were analyzed retrospectively. Patients were divided into Group A (DVT group), including 56 patients with IVCS and iliofemoral DVT, with or without femoropopliteal DVT, with sudden lower limb swelling, and Group B (PTS group) included 55 patients with IVCS and PTS, including 18 with lower extremity wet ulcers and 32 with lower limb infections. Interventional therapies were used to treat the thrombus and eliminate stenosis and occlusion of the iliac vein. In both groups, clinical symptoms in the lower limbs after surgery were reduced significantly, and PTS incidence was low during long-term follow-up. The cumulative patency rate was 75.2% in the DVT group and 88.6% in the PTS group. Comprehensive interventional therapies are safe and effective in patients with IVCS and thrombi. Long-term efficacy in the PTS group tended to be better than that in the DVT group.


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