Aanpak en behandelingsopties voor congenitale melanocytaire naevi

Author(s):  
A. VANNESTE ◽  
M. GARMYN ◽  
M.-A. MORREN

Management and treatment options in congenital melanocytic nevi Congenital melanocytic nevi (CMN) are benign collections of nevus cells in the skin. They are present at birth or arise during the first weeks of life. Depending on the size, they appear in 1 in 100 to 500.000 live births. CMN are associated with a variety of benign conditions such as benign proliferations, certain facial characteristics or subtle endocrine dysfunctions as well as malign developments such as melanoma and neurological complications. The risk for these complications strongly depends on the clinical phenotype. Magnetic resonance imaging (MRI) has a strong value in estimating the risk of these complications. A normal MRI of the central nervous system results in a lower risk of developing melanoma and neurological complications because of the thorough follow-up and early capture. Although there are various treatment modalities, a shift to more conservative treatment is seen. Little is known about the long term prognosis after treatment of CMN. This article tries to give a recommendation for treatment and follow-up of CMN based on the current literature.

2021 ◽  
Vol 9 (1) ◽  
pp. 194
Author(s):  
Frances M. Lee ◽  
Rafael Gaszynski ◽  
Neil Merrett

Hydatid disease (HD) remains endemic in Australia, with estimated incidence highest in rural and indigenous populations. Its recurrence is defined by new active cysts arising after appropriate therapy, and affects anywhere between 2-25% of total cases. Available treatment options include surgical resection, percutaneous drainage, and chemotherapy. Interestingly, their individual contributions to long-term minimisation of recurrence are scarcely described in the literature. We present a unique case of a 27-year-old female with recurrent hepatic HD requiring repeat operations and long-term chemotherapy. Prevention and treatment of recurrent HD requires careful evaluation of a multitude of factors, including disease characteristics, patient attributes, physician expertise and availability of resources. Consistent long-term follow up is required to better ascertain the long-term efficacy of reported treatment modalities for preventing recurrence. Despite a growing body of research looking at treatment of HD, there remains a considerable amount of controversy regarding most effective approach for minimising and preventing its recurrence.


2017 ◽  
Vol 2 (1) ◽  
pp. 43
Author(s):  
Akmal Hisham ◽  
Devananthan Ilenghoven ◽  
Wan Syazli Wan Ahmad Kamal ◽  
Salina Ibrahim ◽  
Shah Jumaat Mohd Yussof

The emergence of highly active antiretroviral therapy (HAART) has revolutionized the prognosis of HIV-infected patients. However, the extended use of HAART is associated with a disfiguring complication termed lipodystrophy, a disorder of body fat maldistribution causing peripheral fat loss (lipoatrophy) and central fat accumulation (lipohypertrophy). Lipoatrophy commonly affects the face, legs, buttocks and arm, whilst lipohypertrophy frequently favours the abdomen, breast and dorsocervical region. To our knowledge, we present only the second documented case in the literature of a labia majora lipohypertrophy in a HIV-positive patient receiving long-term HAART. The severity of labial abnormality caused significant physical and functional morbidities. Labiaplasty with dermolipectomy of the labia majora and excisional lipectomy of the mons pubis was successfully performed. At a 6-month follow-up, patient had no recurrence with resolution of symptoms and resumption of normal activities of daily living (ADL).


2020 ◽  
Vol 16 (8) ◽  
pp. 1022-1043
Author(s):  
Imran Khan ◽  
Sadaf Mahfooz ◽  
Mustafa A. Hatiboglu

Background: Glioblastoma is one of the most aggressive and devastating tumours of the central nervous system with short survival time. Glioblastoma usually shows fast cell proliferation and invasion of normal brain tissue causing poor prognosis. The present standard of care in patients with glioblastoma includes surgery followed by radiotherapy and temozolomide (TMZ) based chemotherapy. Unfortunately, these approaches are not sufficient to lead a favorable prognosis and survival rates. As the current approaches do not provide a long-term benefit in those patients, new alternative treatments including natural compounds, have drawn attention. Due to their natural origin, they are associated with minimum cellular toxicity towards normal cells and it has become one of the most attractive approaches to treat tumours by natural compounds or phytochemicals. Objective: In the present review, the role of natural compounds or phytochemicals in the treatment of glioblastoma describing their efficacy on various aspects of glioblastoma pathophysiology such as cell proliferation, apoptosis, cell cycle regulation, cellular signaling pathways, chemoresistance and their role in combinatorial therapeutic approaches was described. Methods: Peer-reviewed literature was extracted using Pubmed, EMBASE Ovid and Google Scholar to be reviewed in the present article. Conclusion: Preclinical data available in the literature suggest that phytochemicals hold immense potential to be translated into treatment modalities. However, further clinical studies with conclusive results are required to implement phytochemicals in treatment modalities.


2020 ◽  
Vol 13 (11) ◽  
pp. e237097
Author(s):  
Apoorv Sehgal ◽  
Pratyush Shahi ◽  
Avijeet Prasad ◽  
Manoj Bhagirathi Mallikarjunaswamy

A 32-year-old woman presented with progressive pain and swelling of the left wrist for 6 months. Physical examination revealed a firm, tender, oval swelling over the left wrist. X-rays showed a pressure effect on the distal radius and ulna. Magnetic Resonance Imaging (MRI) revealed a well-defined, asymmetrical, dumbbell-shaped soft-tissue lesion involving the interosseous region of the distal forearm and extending until the distal radioulnar joint (DRUJ). Core needle biopsy confirmed the diagnosis of desmoid tumour. Marginal excision of the tumour was done. At the 2-year follow-up, the patient was doing well and had painless and improved left wrist motion. Desmoid tumour involving the DRUJ has not been previously reported. We, through this case, report new observation and discuss the epidemiology, investigation of choice, treatment modalities, and the need for a regular follow-up for appendicular desmoid tumours.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Jeong-Seon Lee ◽  
Joong-Gon Kim ◽  
Soyoung Lee

AbstractChildhood-onset polyarteritis nodosa (PAN) is a rare and systemic necrotising vasculitis in children affecting small- to medium-sized arteries. To date, there have been only a few reports because of its rarity. Thus, we aimed to investigate the clinical manifestations, laboratory findings, treatment, and long-term outcomes in patients with childhood-onset PAN and to evaluate the usefulness of the paediatric vasculitis activity score (PVAS). We retrospectively analysed the data of nine patients with childhood-onset PAN from March 2003 to February 2020. The median ages at symptom onset, diagnosis, and follow-up duration were 7.6 (3–17.5), 7.7 (3.5–17.6), and 7.0 (1.6–16.3) years, respectively. All patients had constitutional symptoms and skin manifestations, while five exhibited Raynaud’s phenomenon. Organ involvement was observed in one patient. The median PVAS at diagnosis was 7 (range: 2–32). Prednisolone was initially used for induction in all patients, and other drugs were added in cases refractory to prednisolone. All patients survived, but three patients with high PVAS at diagnosis experienced irreversible sequelae, including intracranial haemorrhage and digital amputation. In conclusion, early diagnosis and treatment may minimise sequelae in patients with childhood-onset PAN. This study suggests that high PVAS score at diagnosis may be associated with poor prognosis.


2021 ◽  
Vol 10 (2) ◽  
pp. 180
Author(s):  
Frédéric Bouisset ◽  
Jean-Bernard Ruidavets ◽  
Jean Dallongeville ◽  
Marie Moitry ◽  
Michele Montaye ◽  
...  

Background: Available data comparing long-term prognosis according to the type of acute coronary syndrome (ACS) are scarce, contradictory, and outdated. Our aim was to compare short- and long-term mortality in ST-elevated (STEMI) and non-ST-elevated myocardial infarction (non-STEMI) ACS patients. Methods: Patients presenting with an inaugural ACS during the year 2006 and living in one of the three areas in France covered by the Monitoring of Trends and Determinants in Cardiovascular Disease (MONICA) registry were included. Results: A total of 1822 patients with a first ACS—1121 (61.5%) STEMI and 701 (38.5%) non-STEMI—were included in the study. At the 28-day follow-up, the mortality rates were 6.7% and 4.7% (p = 0.09) for STEMI and non-STEMI patients, respectively, and after adjustment of potential confounding factors, the 28-day probability of death was significantly lower for non-STEMI ACS patients (Odds Ratio = 0.58 (0.36–0.94), p = 0.03). At the 10-year follow-up, the death rates were 19.6% and 22.8% (p = 0.11) for STEMI and non-STEMI patients, respectively, and after adjustment of potential confounding factors, the 10-year probability of death did not significantly differ between non-STEMI and STEMI events (OR = 1.07 (0.83–1.38), p = 0.59). Over the first year, the mortality rate was 7.2%; it then decreased and stabilized at 1.7% per year between the 2nd and 10th year following ACS. Conclusion: STEMI patients have a worse vital prognosis than non-STEMI patients within 28 days following ACS. However, at the 10-year follow-up, STEMI and non-STEMI patients have a similar vital prognosis. From the 2nd year onwards following the occurrence of a first ACS, the patients become stable coronary artery disease patients with an annual mortality rate in the 2% range, regardless of the type of ACS they initially present with.


2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii390-iii391
Author(s):  
Aaron Mochizuki ◽  
Anna Janss ◽  
Sonia Partap ◽  
Paul Fisher ◽  
Yimei Li ◽  
...  

Abstract INTRODUCTION Medulloblastoma is one of the most common malignant brain tumors in children. To date, the treatment of average-risk (non-metastatic, completely resected) medulloblastoma includes craniospinal radiation therapy and adjuvant chemotherapy. Modern treatment modalities and now risk stratification of subgroups have extended the survival of these patients, exposing the long-term morbidities associated with radiation therapy. METHODS We performed a single-arm, multi-institution study, seeking to reduce the late effects of treatment in patients with average-risk medulloblastoma prior to advances in molecular subgrouping. To do so, we reduced the dose of craniospinal irradiation by 25% to 18 gray with the goal of maintaining the therapeutic efficacy as described in CCG 9892 with maintenance chemotherapy. RESULTS 28 patients aged 3–30 years were enrolled across three institutions between April 2001 and December 2010. Median age at enrollment was 9 years with a median follow-up time of 11.7 years. The 3-year relapse-free (RFS) and overall survival (OS) were 78.6% (95% CI 58.4% to 89.8%) and 92.9% (95% CI 74.4% to 98.2%), respectively. The 5-year RFS and OS were 71.4% (95% CI 50.1% to 84.6%) and 85.7% (95% CI 66.3% to 94.4%), respectively. Toxicities were similar to those seen in other studies; there were no grade 5 toxicities. CONCLUSIONS Given the known neurocognitive adverse effects associated with cranial radiation therapy, studies to evaluate the feasibility of dose reduction are needed. In this study, we demonstrate that select patients with average-risk medulloblastoma may benefit from reduced craniospinal radiation dose of 18 gray without impacting relapse-free or overall survival.


2017 ◽  
Vol 23 (14) ◽  
pp. 1950-1954 ◽  
Author(s):  
Jinhua Zhang ◽  
Fang Liu ◽  
Yiqi Wang ◽  
Ying Yang ◽  
Yuehong Huang ◽  
...  

Understanding the characteristics of neuromyelitis optica spectrum disorder (NMOSD) with recurrent short partial transverse myelitis (SPTM), which is very rare, contributes to the differential diagnosis of multiple sclerosis (MS). We present two Chinese aquaporin-4 immunoglobulin G (AQP4-IgG)-seropositive NMOSD cases who had at least twice SPTM during 4 and 6 years of follow-up, respectively. Their SPTMs have been mild and responded well to corticosteroids just like in the case of MS. The findings highlight the need of searching for serum AQP4-IgG (cell-based assay strongly recommended) in patients with recurrent SPTM and suggest that those patients may have a mild acute attack phase and favorable long-term prognosis.


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