Management of childhood-onset craniopharyngioma in Italy: A multicenter seven year follow-up study of 145 patients

2021 ◽  
Author(s):  
Stefano Zucchini ◽  
Natascia Di Iorgi ◽  
Gabriella Pozzobon ◽  
Stefania Pedicelli ◽  
Maria Parpagnoli ◽  
...  
Keyword(s):  
Surgical Approach ◽  
Age Groups ◽  
Surgery Complications ◽  
Pediatric Endocrinology ◽  
Gh Treatment ◽  
Duration Of Symptoms ◽  
Electrolyte Disturbances ◽  
Post Surgery ◽  
Number Of Patients

Abstract Context Nationwide data on children diagnosed with craniopharyngioma (CP) are not available in Italy. Objective Identify patients’ characteristics, type of surgical approach, complications and recurrences, number of pituitary deficits and number of patients starting GH treatment. Methods Retrospective multicenter collection of 145 patients aged 0-18 years underwenting surgery for CP between 2000-2018, followed-up in 17 Italian centers of Pediatric Endocrinology. Results Age at diagnosis was 8.4±4.1 years. Duration of symptoms was 10.8±12.5 months and headache was most frequent (54%), followed by impaired growth (48%) and visual disturbances (44%). Most lesions were suprasellar (85%) and histology was adamantinomatous in all cases but two. Surgical approach was transcranic (TC) in 67.5% of cases and transphenoidal (TS) in 31.%. TC approach was prevalent in all age groups. Post-surgery complications occurred in 53% of cases with water-electrolyte disturbances most frequent. Radiotherapy was used in 39% of cases. All patients but one presented at least one hormone pituitary deficiency, with TSH deficit as most frequent (98.3%), followed by ACTH (96.8%), AVP (91.1%) and GH (77.4%). BMI significantly increased over time. A hypothalamic disturbance was present in 55% of cases. GH therapy was started during follow-up in 112 patients at a mean age of 10.6 years and 54 developed a recurrence or regrowth of the residual lesion. Conclusions CP is often diagnosed late also in Italy with TC more frequent than TS surgical approach. Post-surgery complications were not rare and hypopituitarism developed almost in all cases. BMI shows a tendency to increase overtime.

Impact Of Age On Toxicity Associated With Chemotherapy For Acute Lymphoblastic Leukaemia (ALL): Results From The UK Prospective Study UKALL2003

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 840-840
Author(s):  
Rachael E. Hough ◽  
Clare Rowntree ◽  
Rachel Wade ◽  
Nicholas Goulden ◽  
Chris Mitchell ◽  
...  
Keyword(s):  
Conflicts Of Interest ◽  
Residual Disease ◽  
Age Groups ◽  
Serious Adverse Events ◽  
Number Of Patients ◽  
Teenagers And Young Adults ◽  
The Uk ◽  
Cure Rates ◽  
The Impact

Abstract Despite the substantial improvements made in the outcomes of paediatric ALL, with ‘cure' rates now in excess of 90%, survival in teenage and young adult (TYA) patients has remained inferior. The reasons for this are likely multifactorial, including tumour biology, toxicity, compliance, access to clinical trials and protocol (adult or paediatric) used. We report the toxicity profiles observed in children, teenagers and young adults treated on the UK intensive, minimal residual disease (MRD) directed ALL protocol, UKALL2003. Of a total of 3126 patients treated, 1520 patients were under 5 years old, 767 were aged 5-9 years, 610 aged 10-15 years and 229 aged 16-24 years, with a median overall follow-up of 4 year and 10 months. The risk of serious adverse events (SAEs) was higher in patients older than 10 years (56% in 10-15 year olds, 53% in 16-24 year olds) compared to those aged 9 or younger (30% in under 5 years and 31% in 5-9 years)(p<0.0001), with no difference in the those aged 16-24 compared to younger teenagers (p=0.5). The incidence (per number of patients in each group) and distribution of toxicities according to age group is summarised in the table.Table 1Age in years<55-910-1516-24AllTotal number of patients1520767610229 NB: 56 pts≥20 years3126Infection n (%)328 (21.6%)130 (17.0%)145 (23.8%)72 (31.4%)675 (21.6%)Asaparaginase n (%)57 (3.8%)57 (7.4%)64 (10.5%)31 (13.5%)209 (6.7%)Methotrexate n (%)100 (6.6%)74 (9.6%)123 (20.2%)33 (14.4%)330 (10.6%)Steroid n (%)54 (3.6%)37 (4.8%)141 (23.1%)52 (22.7%)284 (9.0%)Vincristine n (%)34 (2.2%)11 (1.4%)22 (3.6%)7 (3.0%)74 (2.4%)Other SAEs94 (6.2%)42 (5.5%)90 (14.8%)25 (10.9%)251 (8.0%) The incidence of certain toxicities including viral infection (5.3%), asparaginase hypersensitivity (1.9%) and vincristine neurotoxicity (2.1%) appeared equivalent across all age groups. Avacular necrosis was seen predominantly in adolescents (83% of 147 events in 10-19 year olds) and was rare in those younger than 10 years (n=18) or older than 20 years (n=7). Asparaginase thrombotic events increased in frequency with increasing age (1.5% in under 5 years, 3.3% in 5-9 years, 4.4% in 10-15 years and 8.3% in 16-24 year olds)(p<0.0001). All other toxicities were more frequently observed in over 10 year olds compared to patients aged 9 or younger, with no difference between 16-24 year olds and 10-15 year olds. The impact of age on SAEs associated with intensive ALL chemotherapy varies according to specific toxicities. In general, toxicity is higher in those over 10 years compared to younger patients, with no excess toxicity in those aged 16-24 compared to 10-15 years. However, specific toxicities may increase with increasing age (thrombosis), be restricted to adolescence (AVN) or be unrelated to age (vincristine neurotoxicity, asparaginase hypersensitivity). Disclosures: No relevant conflicts of interest to declare.

Clinical Outcome following a Modified Approach for Psoas Lengthening for Coxa Saltans in Adults

2007 ◽  
Vol 17 (3) ◽  
pp. 150-154 ◽  
Author(s):  
B. Komarasamy ◽  
R. Vadivelu ◽  
C.J. Kershaw
Keyword(s):  
Conservative Treatment ◽  
Surgical Approach ◽  
Psoas Muscle ◽  
Surgical Approaches ◽  
Duration Of Symptoms ◽  
Musculotendinous Junction ◽  
Internal Iliac ◽  
Lateral Cutaneous Nerve ◽  
Hip Flexion

Internal snapping often resolves with conservative treatment but persistent significant symptoms may require surgical treatment. Different surgical approaches have been suggested in the literature with varying results. We describe a modified surgical approach for internal snapping of hip in adults with good results. Patients who failed conservative treatment for internal snapping over 11/2 years were included. A skin crease incision was made just lateral to the ASIS in supine position. The psoas tendon was reached sub-periosteally along the internal iliac surface and a hole was made in periosteum. Then the tendon was hooked into the wound and divided releasing its musculotendinous junction. The patients were allowed to mobilise as able in the postoperative period. There were 8 snapping hips (7 patients, 6 females) with average age of 30 years (17–51 yrs). The mean follow-up was 11 months. The average duration of symptoms before operation was 4.5 years (range 2–10 years). Painful symptomatic clicking was relieved in all patients. Two patients felt slight weakness of hip flexion. One patient had temporary neuropraxia of lateral cutaneous nerve of thigh. The diagnosis is made by ultrasound or examination for a palpable click. Surgical correction of snapping is considered after failure of conservative treatment. Different extrapelvic (medial and iliofemoral) and intrapelvic extraperitoneal approaches have been described with varying results. With our slightly modified intrapelvic and subperiosteal approach through oblique inguinal incision in adults, psoas muscle release at musculotendinous junction seems a safe and effective method and could be used as an alternative surgical approach for treatment of internal snapping of hip in adults.

scholarly journals P265 IBD′s features depending on the age group of patients and the timing of diagnosis of the disease

2020 ◽  
Vol 14 (Supplement_1) ◽  
pp. S282-S282
Author(s):  
M Skalinskaya ◽  
E Skazyvaeva ◽  
M Zhuravleva ◽  
I Bakulin ◽  
D Komarova ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Age Groups ◽  
Severe Form ◽  
Young Age ◽  
Growing Up ◽  
Age Group ◽  
Duration Of Symptoms ◽  
Number Of Patients ◽  
Life Threatening ◽  
History Of

Abstract Background Untimely diagnosis increases the number of severe forms of IBD, which enlarges the possibility of life-threatening complications, extraintestinal presentations, and the patient’s need for surgical treatment. Methods We analysed the data of 1130 patients with an established diagnosis of UC or CD. The dynamics of the timing of diagnosis of IBD, the nature of the disease, the frequency of occurrence of intestinal complications, extraintestinal presentations were evaluated. Results The maximum frequency of start of both UC and CD falls on the young age of patients - from 19 to 38 years. The duration of symptoms before diagnosis is established is 2.3 years (27.4 months) for CD, 1.1 years (12.1 months) for UC. 81.8% of patients with UC are diagnosed no more than 4 years, but in 10.9% of patients this interval was 4–9 years; in 3.6%, 9–13 years; in 1.9%, 13–18 years, and in another 1.8%, more than 20 years. In CD 59.5% of patients fall into the diagnosis interval up to 4 years, in 27.4% this period takes from 4 to 9 years, in 7%—10–15 years, and in 6% more than 6 years. At the age of 18–25 years severe course of IBD is observed in 12% of patients, at the age of 26–30 years—in 18%, 31–40 years—in 9%. Older people are more likely to experience mild IBD, and the percentage of severe and moderate forms is 4% and 29%, respectively, after 60, and less than 0.5% and 19.5%, respectively, after 70 years. Extraintestinal presentations were observed in 43.4% of patients, with 25% of them having more than one of them. In patients with CD, the development of complications in the first 4 years of the disease was noted in 62%, in UC—in 58.9%. In patients with intestinal complications, the diagnosis period was 2.9 years, without them—2.4 years. However, in patients with a history of surgical treatment of IBD, the diagnosis period was less than 1 year. 69, 3% of revealed patients with a history of needing one or more hospitalisations associated with IBD, and 15.8% underwent surgery for IBD. Conclusion Based on the data obtained, it can be concluded that the main part of complications occurs in the first few years after the onset of the disease. With late diagnosis, the probability of developing not only intestinal complications, but also extra-intestinal presentations significantly increase, which reflects a more severe form of the disease. The shorter time of diagnosis of IBD in patients who need surgical treatment is explained by more pronounced symptoms that require urgent medical care. However, the proportion of IBD with severe and moderate form is greater in young age groups, and as the ‘growing up’ groups - the number of patients with mild IBD begins to prevail.

scholarly journals Lumbar disc extrusions reduce faster than bulging discs due to an active role of macrophages in sciatica

2019 ◽  
Vol 162 (1) ◽  
pp. 79-85 ◽  
Author(s):  
N. Djuric ◽  
X. Yang ◽  
A. el Barzouhi ◽  
R. Ostelo ◽  
S. G. van Duinen ◽  
...  
Keyword(s):  
Disc Herniation ◽  
Histological Study ◽  
Lumbar Disc ◽  
Active Role ◽  
Modic Changes ◽  
Macrophage Infiltration ◽  
Herniated Disc ◽  
Duration Of Symptoms ◽  
Post Surgery

Abstract Objective This retrospective observational histological study aims to associate the size and type of disc herniation with the degree of macrophage infiltration in disc material retrieved during disc surgery in patients with sciatica. Methods Disc tissue of 119 sciatica patients was embedded in paraffin and stained with hematoxylin and CD68. Tissue samples were categorized as mild (0–10/cm2), moderate (10–100/cm2), and considerable (> 100/cm2) macrophage infiltration. All 119 patients received an MRI at baseline, and 108 received a follow-up MRI at 1-year. MRIs were reviewed for the size and type of the disc herniations, and for Modic changes in the vertebral endplates. Results Baseline characteristics and duration of symptoms before surgery were comparable in all macrophage infiltration groups. The degree of macrophage infiltration was not associated with herniation size at baseline, but significantly associated with reduction of size of the herniated disc at 1-year post surgery. Moreover, the degree of macrophage infiltration was higher in extrusion in comparison with bulging (protrusion) of the disc. Results were comparable in patients with and without Modic changes. Conclusion Macrophage infiltration was positively associated with an extruded type of disc herniation as well as the extent of reduction of the herniated disc during 1-year follow-up in patients with sciatica. This is an indication that the macrophages play an active role in reducing herniated discs. An extruded disc herniation has a larger surface for the macrophages to adhere to, which leads to more size reduction.

scholarly journals Surgical Treatment of Lateral Epicondylitis

2017 ◽  
Vol 5 (3_suppl3) ◽  
pp. 2325967117S0012 ◽  
Author(s):  
Martin Kroslak ◽  
George A.C. Murrell
Keyword(s):  
Tennis Elbow ◽  
Surgical Techniques ◽  
Surgical Excision ◽  
Secondary Outcome ◽  
Sham Operation ◽  
Elbow Pain ◽  
Duration Of Symptoms ◽  
Post Surgery ◽  
Number Of Patients ◽  
Significant Difference

Objectives: A number of surgical techniques for managing tennis elbow have been described, one of the commonest (Nirschl & Pettrone. J Bone Joint Surg Am, 61(6A): 832-839) involves excising the affected portion of extensor carpi radialis brevis (ECRB) origin. The results of this technique, as well as most other described surgical techniques for this condition, have been reported as excellent, yet none have been compared with placebo surgery. Methods: This study was a prospective, randomised, double blinded, placebo controlled clinical trial investigating the surgical excision of the macroscopically degenerated portion of ECRB (Nirschl technique; n=13) compared with a sham operation (skin incision and exposure of ECRB alone; n=13) to manage tennis elbow, in patients with tennis elbow for more than 6 months who failed at least two non-surgical modalities. The primary outcome measure was defined as patient rated frequency of elbow pain with activity at 6 months post-surgery. Secondary outcome measures included patient rated frequency and severity of pain, functional outcomes, range of motion, epicondyle tenderness and strength at 6 months and >12 months post-surgery. Results: The two groups were matched for age, gender, hand dominance and duration of symptoms. Both the surgery and placebo procedures improved patient rated pain frequency and severity, elbow stiffness, difficulty with picking up objects, twisting motions and overall elbow rating over 6 months and at >12 months (p<0.01). Both procedures also improved epicondyle tenderness, pronation-supination range, grip strength and modified ORI-TETS at 6 months (p<0.05). No significant difference was observed between the groups in any parameter at any stage. No side effects or complications were reported. The study was stopped before the calculated number of patients were enrolled, as a post-hoc analysis showed over 6500 patients would need to be recruited in each group to see a significant difference between the groups at 26 weeks, when measuring patient rated frequency of elbow pain with activity. Conclusion: This study indicates that the surgical excision of the degenerative portion of ECRB offers no additional benefit over and above placebo surgery for the management of chronic tennis elbow. [Figure: see text]

scholarly journals Reduced Risk of Atrial Fibrillation Following Cholecystectomy: A Nationwide Population-Based Study

2021 ◽  
Vol 13 ◽  
Author(s):  
Tung Ching Ho ◽  
Yu-Ching Chen ◽  
Che-Chen Lin ◽  
Hsu-Chih Tai ◽  
Cheng-Yu Wei ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
Gallstone Disease ◽  
Age Groups ◽  
Population Based ◽  
Research Database ◽  
Population Based Study ◽  
Number Of Patients ◽  
Taiwan National Health Insurance ◽  
Using Data

Background: Gallstone disease (GD) is associated with a high risk of cardiovascular disease. However, it is unknown whether GD contributes to atrial fibrillation (AF). We aimed to investigate the association between GD and AF.Methods: We performed a population-based cohort study using data from the Taiwan National Health Insurance Research Database between 2001 and 2011. A GD cohort of 230,076 patients was compared with a control cohort consisting of an equal number of patients matched for age, sex, cardiovascular and gastrointestinal comorbidities.Results: In total, 5,992 (49.8/10,000 person-years) patients with GD and 5,804 (44.5/10,000 person-years) controls developed AF. GD increased AF risk with a hazard ratio (HR) of 1.20 [95% confidence interval (CI), 1.16–1.25]. In patients with GD but without cholecystectomy, the HR of AF reached 1.57 (95% CI = 1.50–1.63). After cholecystectomy, the HR of AF significantly decreased to 0.85 (95% CI = 0.81–0.90). Among the three age groups with GD (&lt;45, 45–64, and ≥65 years), the adjusted HRs of AF were 1.59 (95% CI = 1.08–2.33), 1.31 (95% CI = 1.18–1.45), and 1.18 (95% CI = 1.13–1.22), respectively. Compared with patients with a CHA2DS2-VASc score equal to 0, the HRs of AF risk among total cohort patients and a score equal to 1, 2, 3, and ≥ 4 were 1.28 (95% CI = 1.15–1.43), 2.26 (95% CI = 2.00–2.56), 3.81 (95% CI = 3.35–4.34), and 5.09 (95% CI = 4.42–5.87), respectively.Conclusion: This population-based longitudinal follow-up study showed that patients with GD had an increased AF risk. Moreover, cholecystectomy was related to reduced AF risk. Cardiovascular checkups may be necessary for patients with GD, especially those who are young and have other typical risk factors.

R415 – Recurrence of Nasopharyngeal Angiofibroma (NPF) Study

2008 ◽  
Vol 139 (2_suppl) ◽  
pp. P182-P182
Author(s):  
Vardhan Vishnu ◽  
R. Martha
Keyword(s):  
Prospective Study ◽  
Recurrence Rate ◽  
Surgical Approach ◽  
Statistical Difference ◽  
Duration Of Symptoms ◽  
Nasopharyngeal Angiofibroma ◽  
Appropriate Treatment ◽  
One Year ◽  
The Right

Problem Study of correlation of recurrence of NPF with its histopathologic appearance. Methods A retrospective and prospective study of 184 NPF cases identified, treated in Government Ear Nose Throat Hospital, and followed over a period of 26 years (1981–2006) and their histopathological slides are preserved, their recurrence rate is documented. The histopathology of NPF is varied - composed of vascular and fibrous components and their proportion varied. In our study we will undertake the review of histopathology slides that are already preserved and would also like to study the new recurrent cases in the coming 6 months. Results All recurrences were observed with in one year of of treatment and the recurrence rate was 19.66%. Recurrence had no correlation with age of patient, duration of symptoms, or surgical approach but correlated with stage of tumor at presentation (p less than 0.05). Preoperative embolisation was done in 16.85% cases and did not show any statistical difference in recurrence rate between the embolised and non embolised. Conclusion The correlation between the histopathological appearance and the recurrence rate will help in choosing the right approach for surgery and also postoperative follow-up to detect the recurrence at the earliest. Significance Histopathological detail can help in knowing the prognosis in terms of chances of recurrence and also guide for follow up at close intervals and advocate the appropriate treatment at the earliest.

Screening for familial ovarian cancer—management and outcome of women with moderate to high risk of developing ovarian cancer

2006 ◽  
Vol 16 (Suppl 1) ◽  
pp. 86-91
Author(s):  
G. C. Rieck ◽  
K. Lim ◽  
M. T. Rogers ◽  
E. France ◽  
J. R. Gray ◽  
...  
Keyword(s):  
Breast Cancer ◽  
Ovarian Cancer ◽  
High Risk ◽  
Metastatic Breast ◽  
Prophylactic Surgery ◽  
Cancer Management ◽  
Post Surgery ◽  
Number Of Patients ◽  
Screening Clinic

Five percent to ten percent of ovarian cancers are hereditary. Individual genetic risk of developing ovarian malignancy is discussed in women. Currently, prophylactic surgery is advised to women with a moderate to high risk of developing ovarian cancer. Workload and outcome of the multidisciplinary familial ovarian screening clinic in South Wales were assessed. This was an observational study of 145 women registered with the Familial Ovarian Screening Clinic between January 1998 and December 2003. The data were retrieved from the medical notes. Yearly follow-ups were investigated with a transvaginal scan and CA125 level. Post-surgery women were followed up with yearly CA125 estimations: 46.9% fell into moderate-risk and 50.3% into high-risk category. The median age was 42 (SD 10.4), 71.7% were pre menopausal, and 10.3% had a personal history of breast cancer and 1.4% colon cancer. Whereas 36.5% opted for surgery, the remaining women (but two) opted for annual follow-up. Histology of the women who had surgery showed three cases of malignancies (fallopian tube carcinoma, atypical ovarian epithelial cells, and metastatic breast cancer). Seven women developed breast cancer during the observation period. The follow-up period is too short to come to a final conclusion as to the benefits of yearly screening in this group of women. In our series, a significant number of patients developed malignancies, despite prophylactic surgery.

scholarly journals SUN-201 Adrenal Insufficiency After Treatment for Vaping Associated Pulmonary Injury

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Melinda Pierce ◽  
Anne Griffiths
Keyword(s):  
Adrenal Insufficiency ◽  
High Dose ◽  
Pediatric Endocrinology ◽  
Acth Stimulation ◽  
Post Discharge ◽  
Prednisone Therapy ◽  
Number Of Patients ◽  
First Time ◽  
Dose Prednisone

Abstract Background: In 2019 a record number of patients were admitted to our children’s hospital with e-cigarette or vaping-associated lung injury (EVALI). The majority of patients were treated with high-dose prednisone therapy including 3 days of 1000 mg daily followed by a 4 week wean. Because of the concern for iatrogenic adrenal insufficiency, all patients were then placed on a 3-4 weak hydrocortisone taper followed by ACTH stimulation testing. The purpose of this study is to document the incidence of iatrogenic adrenal insufficiency following a 2 months glucocorticoid wean. Methods: All patients seen by the Pulmonary team for EVALI who also received high dose prednisone therapy were referred to Pediatric Endocrinology for ACTH testing. A low dose (1 mcg) ACTH stimulation test was performed with cortisol measurements at baseline, 30 minutes, and 60 minutes. A passing result was any cortisol over 18 ug/dl. Results: An anticipated 20-25 patients will have had ACTH stimulation testing by the time of abstract presentation. Of those that have already completed testing, three of four patients failed the first time. Discussion: High-dose prednisone therapy for EVALI is associated with significant rates of iatrogenic adrenal insufficiency, even after slow hydrocortisone wean. Structures need to be implemented to teach patients about adrenal insufficiency prior to hospital discharge and organize adequate post-discharge follow-up until HPA function returns.

scholarly journals Osteomas of the craniofacial region: A case series and review of literature

2013 ◽  
Vol 46 (03) ◽  
pp. 479-485 ◽  
Author(s):  
Sagar Gundewar ◽  
Deepak S. Kothari ◽  
Nitin J. Mokal ◽  
Amol Ghalme
Keyword(s):  
Surgical Approach ◽  
Clinical Presentation ◽  
Age Groups ◽  
Case Series ◽  
Surgical Excision ◽  
Frontal Bone ◽  
Review Of Literature ◽  
Operative Complications ◽  
Histological Confirmation

ABSTRACT Objective: 0 To discuss the clinical presentation, diagnosis and management of osteomas involving the craniomaxillofacial region. Materials and Methods: This study was conducted from June 2004 to March 2012 at our institute. A total of 12 cases between the ages of 10 and 50 years were managed with surgical excision and reconstruction. The criteria used to diagnose osteoma included radiographic and clinical features and histological confirmation of the specimen. The total follow-up period ranged from 6 to 24 months. Results: Out of 12 osteomas, 10 were peripheral and 2 were centrally located. Mandible involvement was seen in six patients, four involved the orbit, one the frontal bone and one the frontal bone with the skull base. All patients undergoing excision and reconstruction had a favourable aesthetic and functional outcome. There were no recurrences and no post-operative complications. Conclusion: Osteomas affect all age groups with no sex predilection and are usually clinically asymptomatic till they become large in size. Surgical excision and appropriate reconstruction is the mainstay of management. Surgery is indicated when lesion is symptomatic or actively growing and the surgical approach for exposure of the lesion should be case specific.

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