Long-acting Growth Hormone Therapy, Rational and Future Aspects.

Recombinant growth hormone (GH) is administered as daily subcutaneous injections. Daily treatment can be challenging for children/adolescents as well as for parents and/or caregivers (legal representatives, guardians of children in institutional care). Challenges associated with daily treatment may result in missing several doses and non-adherence with treatment leads to inadequate growth response. As an inadequate growth response does not meet criteria for continuing treatment, payers (commercial or public) may decide to end reimbursement. Novel long-acting GH formulations (LAGH) with extended half-life can be administered less frequently and target to improve patient convenience and consequently to improve adherence and responses to treatment. LAGH formulations can restore growth velocity and body composition as effectively as daily treatment, without unexpected adverse effects as reported in randomized clinical trials.

Momordica charantia enhances tendon healing in rats: An experimental study.

Momordica charantia (MC) is a traditional plant widely used since ancient times for wound healing. This study evaluated its potential effects on tendon healing. Adult Male Wistar albino rats (n=32, 8 rats in each group) were anesthetized, and their Achilles tendons were prepared for surgical procedures. Group 1 (Cont= control group) were not subjected to any surgery and were used as a control group for baseline values. Group 2 (PR= primary repair group) underwent primary repair (PR) with a monofilament suture after a full-thickness incision of the Achilles tendon. A full-thickness incision was also made to the Achilles tendon of Group 3 (CT=collagen tube administered group), followed by PR and collagen tube insertion. In Group 4 (MC= Momordica charantia-administered group), 1 ml of MC extract was applied locally on the collagen tube in addition to the surgical procedure applied to Group 3. The Achilles tendons were excised on the postoperative 40th day and examined stereologically, histologically, and bioinformatically. Data showed that the total volume of the collagen fibers was higher in MC and CT groups than in the PR group. The total volume of the tendon was decreased in MC and CT groups than in the Cont group. The ratios between the volumes of the collagen fibers and total tendon in the MC and CT groups were significantly different from PR; but not different from the Cont group. Additionally, MC improved tenoblastic activity, collagen production, and neovascularization. Bioinformatic interactions showed that the proteases of MC could trigger the signals playing a role on vasculogenesis, reducing inflammation, and contributing to tenoblast activation and collagen remodeling. MC extract ameliorates the healing of injured tendon and can provide satisfactory tendon repair. Further works are recommended to explore the healing capacity of MC.

The effect of adjuvant cryotherapy added to well-performed high-speed burr curettage on the long-term surgical outcomes of chondroblastoma cases.

OBJECTIVES: This study aims to investigate the effect of adjuvant cryotherapy added to well-performed high-speed burr curettage on the long-term surgical outcomes of chondroblastoma cases. PATIENTS AND METHODS: Between January 2004 and December 2020, a total of 30 chondroblastoma cases (19 males, 11 females; median age: 18.6 years; range, 9 to 53 years) who were surgically treated were retrospectively analyzed. The pressurized-spray technique was performed using liquid nitrogen. Data including age, sex, radiological appearance, treatment modality, duration of follow-up, skin problems, and recurrence were recorded. All patients received adjuvant liquid nitrogen cryotherapy after extended intralesional curettage with high-speed burr. The bone cavity was filled with an autologous iliac crest bone graft, allograft, or polymethylmethacrylate (PMMA). RESULTS: The median follow-up was 54 (range, 19 to 120) months. The lesion was located around the knee in 16 (53.3%), in the shoulder in seven (23.3%), around the hip in five (16.6%), and in the ankle in two (6.6%) cases. The defect was filled with an autologous iliac crest bone graft in 28 (93.3%), an additional allograft in eight (26.7%), and PMMA in two (6.7%) cases. Local recurrence was observed in only two (6.7%) patients during follow-up. Two (6.7%) patients developed physeal growth arrest. Osteoarthritic changes were observed in two (6.7%) patients (one knee and one hip) due to the periarticular location of the tumor. Three (10%) patients had skin complications. None of the cases had a pathological fracture. CONCLUSION: A well-performed extended intralesional curettage with high-speed burr is the first and essential step in treating chondroblastoma. Adding adjuvant liquid nitrogen cryotherapy with high-speed burr can improve treatment outcomes and significantly reduce the recurrence rate of this disease.

The Effect of Tourniquet Duration on Pain, Bleeding, and Functional Outcomes in Total Knee Arthroplasty.

Objective The aim of this study was to evaluate the effects of tourniquet use on perioperative blood loss, pain, and functional and clinical outcomes. Patients and methods This is a prospective study that included 80 knees who underwent total knee arthroplasty. The patients were separated into two groups: those with a tourniquet used throughout the entire surgical procedure and those where the tourniquet was only used during the cementation procedure. In the postoperative period, the pain levels of the patients were evaluated using a visual analog scale (VAS), and the functional results were evaluated with knee range of motion measurement, the Western Ontario and Mcmaster Universities Osteoarthritis (WOMAC) index, the Knee Injury and Osteoarthritis Outcome Score (KOOS), the Kujala Patellofemoral Scoring System, and the Oxford Knee Scoring system. The patients were examined in the early postoperative period and again in the 12th week, including possible complications that may develop postoperatively. Results In the early postoperative period, a greater hemoglobin decrease and calculated blood loss values, better functional clinical results, and better knee range of motion were determined in the group with a tourniquet applied only during the cementation, and the swelling in the knee was less (p<0.05). However, the difference between the two groups had disappeared by the postoperative 12th week. There was no significant difference in respect of complications. Conclusion Limiting the duration of tourniquet use during total knee arthroplasty has the significant advantage of providing better functional results with less pain in the early postoperative period.

Analysis of prognostic factors and histopathological response to neoadjuvant chemotherapy in osteosarcoma.

OBJECTIVES: This study aims to examine the clinical results of patients who underwent medical and surgical treatment for osteosarcoma, to determine the overall survival (OS) and disease-free survival (DFS) rates, and to examine the effects of prognostic factors on these rates. PATIENTS AND METHODS: Between January 2005 and January 2020, a total of 64 patients (38 males, 26 females; mean age: 20.9±11.5 years; range, 6 to 70 years) who received medical and surgical treatment for osteosarcoma were retrospectively analyzed. Demographic characteristics, follow-up period, tumor location and size, tumor stage and necrosis rate, metastatic disease, surgical treatments, postoperative complications, local recurrence, and metastasis were recorded. The relationship of these factors with the survival was examined. RESULTS: The median follow-up was 51.6 (range, 3 to 156) months. The most common tumor localization was in the distal femur with 42 (65.6%) patients and the most common histopathological subtypes were conventional osteosarcoma in 50 (78.1%) patients. The OS rates were 91.6% at one year, 65.9% at five years, and 51.6% at 10 years. With the exception of two patients who died during neoadjuvant chemotherapy, all patients underwent surgical treatment. The addition of chemotherapy + radiotherapy in the treatment did not provide any benefits in terms of survival and recurrence compared to the group that was not added, and the five-year OS rate was 79.3% compared to 20.7%, respectively. The overall 10-year survival rates were 83.9% and 37.2% in the group with a good response (≥90%) and poor response (<90%) to treatment (p=0.012). The mean survival time of three patients who presented with pathological fractures was shorter than the others (p>0.05). Surgical margin was ≤2 mm in 27 (42.2%) patients, >2 mm in 30 (46.9%) patients, and surgical margin was positive in five (7.8%) patients. The mean OS in the group with a surgical margin closure of >2 mm was 10.8±1.9 years and was longer than the other groups (p=0.047). CONCLUSION: Metastasis at the time of diagnosis, <90% tumor necrosis, a tumor size of ≥10 cm, and metastasis development were significantly associated with poor survival and were found to be independent prognostic factors. The OS rate in the patient group with Stage III-IV response after neoadjuvant chemotherapy given the cisplatin + doxorubicin protocol was found to be better than those given the European and American Osteosarcoma Studies (EURAMOS) protocol. More research is needed to determine the most optimal chemotherapy protocols in this patient population. In addition, a multidisciplinary approach in treatment is of utmost importance to improve oncological outcomes.

Schwannomatosis of the sciatic nerve: a case report.

Schwannomas are one of the most common peripheral nerve sheath neoplasms. These tumors, which are characteristically slow-growing and encapsulated, can occur in solitary or multiple forms. Although they usually occur sporadically, they can be seen with various genetic tumor predisposition syndromes such as neurofibromatosis type 2 (NF-2) or schwannomatosis. However, schwannomatosis is a relatively rare disease. We present a case of a 22-year-old patient with segmental schwannomatosis of the sciatic nerve and a comprehensive literature review.

Is there a secular trend regarding puberty in children with down syndrome?

Introduction: There are very few studies on the age of onset and end of puberty in children with Down syndrome (DS). Also, data regarding the course of puberty in these children compared to their healthy peers is limited. Moreover, there is limited information regarding the effects of factors such as obesity and hypothyroidism on the puberty process in children with DS. Our aim in our study is to determine whether the pubertal development of children with DS differs from their healthy peers and from previous studies conducted with DS children. Methods: The medical records of DS children were examined retrospectively. The anthropometric measurements and the age of onset of pubertal stages, and menarche were recorded. The patients' age at puberty onset, the puberty processes, and age at menarche were compared with their healthy peers and previously published data on children with DS. Results: Of the 140 Down syndrome patients followed in our clinic, 51 of whom with puberty constituted the study group. The mean age of onset of puberty was 10.3 ± 1.0 years in our group (10.0 ± 0.8 years for girls, 10.6 ± 1.2 years for boys, respectively). Obesity occurred in 46% of pubertal girls with DS. The age of menarche in girls with DS was 11.8 ± 0.7 years. The menarche age of girls with DS was significantly different from healthy girls. In the DS boys, only the Tanner V stage ages were different from the healthy children. True- precocious-puberty was detected in three children. Conclusion: Although breast development begins later in females with DS than in their healthy peers; menarche is detected earlier than in their peers and a tendency towards obesity in the whole population. While the age of pubertal onset was similar to healthy children in male patients, our findings suggest that their puberty duration is longer.

Evaluation of prognostic factors and oncological outcomes in patients with limb-localized myxoid liposarcoma.

OBJECTIVES: This study aims to evaluate the prognostic factors and treatment outcomes of patients with extremity-localized myxoid liposarcoma (MLS). PATIENTS AND METHODS: Between January 2001 and October 2019, a total of 43 patients (29 males, 14 females; mean age: 56.3±11.4 years; range, 34 to 76 years) who were histopathologically diagnosed with MLS in our clinic were retrospectively analyzed. Data including demographic characteristics, tumor localization, tumor volume and length, histopathological characteristics, the surgery and chemotherapy (CT)/radiotherapy (RT) applied, survival rates, and complications such as local recurrence and metastasis were recorded. The treatment results and potential prognostic factors were identified. The overall survival (OS) and cancer-specific survival (CSS) rates were evaluated. RESULTS: The mean follow-up was 106.8±54.1 (range, 29 to 204) months. The mean tumor size was 11.4±6.5 (range, 4.7 to 36) cm. Tumor localization was determined as lower extremity in 76.7% of cases and upper extremity in 23.2%. The patients were divided into two groups according to the type of RT they received as follows: the patients who underwent neoadjuvant RT + wide surgical resection (n=14, 32.5%) and patients who underwent extensive surgical resection + adjuvant RT (n=29, 67.4%). To four patients who developed distant metastasis and to two who developed local recurrence, adjuvant CT was applied. In the whole cohort, the OS rate was 87.1% at five years and 73.2% at 10 years. The five and 10-year CSS rates were 83.5% and 66.4%, respectively. Local recurrence developed in 12 (27.9%) and distant metastasis in four (9%) patients. In the multivariate analysis, high tumor grade, R2 margins, and metastasis were found to be independent risk factors for OS. Although wide resection provided significantly good local control, neoadjuvant RT and adjuvant CT were not found to be prognostic factors for OS or CSS (p>0.05). CONCLUSION: High tumor grade, R2 margins, and metastasis are independent risk factors for increased OS and OSS. Surgery with CT and neo/adjuvant RT is not an independent risk factor for OS or CSS. Despite patients with a larger tumor size and neurovascular proximity, similar disease-free survival rates can be achieved in the patients receiving neoadjuvant RT. Neoadjuvant RT can be considered in lesions close to neurovascular structures or in large lesions, with a high risk of wound complications.

The Terrible Triad of the Elbow Accompanied by Capitellum and Humerus Shaft Fracture: A Rare Case Report.

The terrible triad of the elbow consists of radial head fracture and coronoid process fracture in addition to posterior dislocation of the elbow. It indicates high-risk complications such as instability, malunion, nonunion, and proximal radioulnar synostosis. We describe a rare case that was admitted to the emergency service with a terrible triad of the elbow with additional capitellum fracture, lateral collateral ligament (LCL) injury, and ipsilateral humeral shaft fracture. We treated the patient urgently by performing osteosynthesis of the humeral shaft fracture, radial head fracture, coronoid fracture, capitellum fractures, and repair of the LCL rupture. The terrible triad of the elbow also can be accompanied by adjacent column fractures, including the humeral shaft. In such complex cases, preoperative planning should be done well, and the entire anatomy should be demonstrated with additional imaging. Optimal treatment of all the fractured bones and ligaments is critical for early rehabilitation. The main aim of surgery is to acquire desired results by starting an early rehabilitation, including joint movement.

Evaluation of prognostic factors affecting survival in chondrosarcoma treatment and comparison with literature.

OBJECTIVES: The aim of this study was to identify the demographic characteristics of chondrosarcoma (CS) and prognostic factors affecting survival. PATIENTS AND METHODS: A total of 87 patients (45 males, 42 females; median age: 51.3 years; range, 19 to 77 years) with CS treated in our clinic between January 2007 and June 2020 were retrospectively analyzed. Demographic characteristics, whether it was primary/secondary, tumor location, histopathological features, tumor grade and stage, clinical follow-up period, surgical treatment methods, use of radiotherapy and chemotherapy, and the presence of local recurrence and metastasis in the postoperative period were recorded. The relationship of these factors with prognosis was analyzed and survival rates were compared. RESULTS: Histological subtype, tumor grade, pathological stage and presence of metastasis were defined as independent predictors in both overall survival and disease-free survival analysis of CS. Overall and disease-free five-year and 10-year survival rates were found to be the highest in the clear cell chondrosarcoma group. While mortality increased in the first five years in the patient groups with histological Grade 2 and 3, all groups were followed in a balanced manner over time. The mortality rate in the group with metastatic disease (M2) was approximately four times higher than the other groups at 10-year follow-up. According to the surgical margins, we found that the five-year survival rates of the R1 (marginal resection) and R2 (residual tumor) groups were similar, with the highest rate being in the R0 (wide resection) group with 78.3%. In multivariate analysis, only grade and stage had a significant association with disease-specific survival. Surgical resection combined with adjuvant radiotherapy was found to increase survival in both overall and disease-free survival of patients with dedifferentiated chondrosarcoma compared to other treatments. CONCLUSION: Histological subtype, grade, stage and presence of metastasis were the independent prognostic factors for survival in CS. However, marginal resection was a risk factor for local recurrence (LR), but there was no significant difference in overall survival in patients with or without LR. Although it is not significant, radiotherapy could increase survival in dedifferentiated CS variants.

Avascular Necrosis of the Type II Accessory Navicular Bone: A Rare Case Report.

The accessory navicular bone (ANB) is one of the most common accessory ossicles of the foot. Fewer than 1% of ANBs are symptomatic, and most of the symptomatic ANBs are type II ANBs. Avascular necrosis of the type II ANB is an uncommon cause of symptomatic accessory navicular syndrome and also a rarely reported condition in the podiatric medical literature. This rare disorder must be distinguished from other painful conditions of the ANB and should be considered in differential diagnoses. We present a case of avascular necrosis of the type II ANB with sclerosis on radiographs and magnetic resonance images in a 46-year-old woman.

Extra-abdominal desmoid fibromatosis: An evaluation of clinical factors affecting local recurrence rates.

OBJECTIVE: The aim of this study was to determine the prognostic factors for recurrence in patients with extra-abdominal desmoid tumors (EDTs) treated surgically. METHODS: This single-institution, retrospective study included patients with a histologically-proven extra-abdominal desmoid tumor between 2007 and 2018. The demographic characteristics (age, sex) of the patients, tumor characteristics (region, size, proximity to neurovascular structures, margins), treatment management (surgery and/or adjuvant radiotherapy), and clinical results were analyzed. The effects of these possible prognostic factors on overall and disease-free survival rates and the risk of local recurrence were evaluated. RESULTS: Evaluation was made of 22 patients (16 females, 6 males) with a mean age at diagnosis of 34.7 years (range = 22-76 years). The mean follow-up was 104 months (range = 4.8-168). Tumor localization was in the upper extremity in 4 patients (18.1%), the lower extremity in 11 (50.0%), and the trunk in 7 (31.8%). The mean tumor size (maximum diameter) was 5.2 cm (range = 0.6-13 cm; median = 5.8 cm), and the mean tumor volume was 181.3 ± 531.4 ml. All the 22 patients were treated surgically along with adjuvant radiotherapy (RT) administered to 8 in addition to surgery for the primary treatment of the tumor. Following primary surgery, resection margins were R0 in 11 patients, R1 in 9 and R2 in 2. Local recurrence (LR) developed in 6 patients (27.2%) during the follow-up period. Recurrence-free survival rate (RFS) was 90.9% at one year, 74.1% at 5 years, and 61.7% at 10 years. During the follow-up, no patient died, and distant metastasis was not detected. Tumor length, resection margins, and adjuvant RT were observed to influence the risk of local recurrence (P < 0.05). CONCLUSION: The results of this study have demonstrated that tumor size ≥ 5 cm and the presence of microscopic or macroscopic positive surgical margins can increase the risk of LR, and adjuvant RT can reduce the development of LR in the management of EDT.

Cytomegalovirus Colitis in Immunocompetent and Immunocompromised Children: A 2-Center Study.

BACKGROUND: Data about cytomegalovirus (CMV) colitis in children are scarce. We aimed to describe the characteristics of childhood CMV colitis in terms of risk factors, clinical symptoms, diagnosis, therapeutic approaches, and outcomes. METHODS: Inflammatory bowel disease (IBD) and non-IBD patients with CMV colitis diagnosed by histology and tissue CMV PCR at 2 tertiary centers between January 2017 and November 2019 were studied. Clinical and laboratory data were retrieved from medical records. Underlying conditions, immune status, response to therapy and outcomes were described and followed up to 6 months after diagnosis. RESULTS: A total of 16 children (8 non-IBD, 7 ulcerative colitis and 1 Crohn's disease) with CMV colitis were included. All patients had persistent diarrhea (bloody in 13 cases). There was a significant age difference between IBD and non-IBD children (P < 0.05). The final diagnosis in 1 patient was immunodeficiency with a mutation in JAK1 gene. Three children were categorized as apparently immunocompromised and 4 children as apparently immunocompetent. Ulcer was not visible in 2 children from the non-IBD group. The mean fecal calprotectin level of IBD children was significantly higher than that of non-IBD children (376.12 ± 231.21 µg/g vs. 160.96 ± 69.94 µg/g, P < 0.05). After follow-up, 1 patient died because of another reason. Ganciclovir was used in 14 of 16 children for 3 weeks and the treatment was continued with valganciclovir in selected 6 children. CONCLUSIONS: CMV colitis is a rare but overlooked cause of prolonged diarrhea in immunocompetent and immunocompromised children. CMV colitis might present without any ulcer formation at colonoscopy in infants.

Relationship Between Lactate Levels and Length of Hospital Stay in Infants with Lower Respiratory Tract Infection.

Background: Increased lactate concentrations are directly related to the severity of shock and mortality rates. There are limited data regarding the prognostic value of lactate among lower respiratory tract infections. We aimed to investigate the impact of lactate levels on admission on the clinical outcomes of children with lower respiratory tract infections. Methods: We performed a retrospective study of hospitalized patients aged 1-12 months. We recorded data on patient demographics, clinical, laboratory, treatment, and outcomes. The primary outcome measure was the length of hospital stay, and the secondary outcome was transfer to the pediatric intensive care unit (PICU) and/or mortality rates. Results: A total of 304 infants were included in the study. There were 198 infants with lactate levels of >2 mmol/L. Lactic acidosis was present in 6 infants, with a mean hospital stay of 8 ± 3 days. Only 1 (0.3%) patient required intubation, and 5 (1.6%) were transferred to the PICU. The overall mortality rate was 0%. Lactate levels (≤2 and >2 mmol/L) were not related to the length of hospital stay, transfer to PICU/discharge, and the need for intubation (P = 0.16, 0.8, and 0.46, respectively). The length of hospital stay was not correlated with lactate levels on admission (r = 0.01, P = 0.84), pCO2 (r = 0.03, P = 0.52), pH (r = 0.07, P = 0.19), C-reactive protein (r = 0.06, P = 0.28), and oxygen saturation (r = -0.02, P = 0.72). Conclusions: Lactate levels on admission did not predict the length of hospital stay in children with lower respiratory infections and were not related to the need for transfer to the intensive care unit. We suggest using lactate levels in combination with clinical, laboratory, and physical examination findings as predictors of disease severity.