Liver transplantation in patient with Berardinelli-Seip syndrome: A literature review and case report.

BACKGROUND: Berardinelli-Seip syndrome is an infrequently seen and potentially fatal genetic disorder characterized by the absence of adipose tissue. Herein, we report a first-in-literature liver transplant done on a 7-year-old girl because of liver cirrhosis caused by the Berardinelli-Seip syndrome. CASE REPORT: Physical examination showed prominent subdermal fat tissue loss and mild muscle hypertrophy, giving her a slim appearance, hirsutism, thick hair, a large head in contrast to the body, low anterior hairline, icterus, prominent facial contours, prominent mandibula, loss of buccal fat, low set ears, and large limbs. After the diagnosis, she admitted to our clinic because of variceal esophageal bleeding and increasing liver enzymes. Transplantation decision was made and orthothopic liver transplantation done by the surgery team. DISCUSSION: Common causes of death in Berardinelli-Seip syndrome patients are infections and liver cirrhosis. The mean age of the patients was 27.1 at the time of death. There is no any established cure for congenital lipodystrophies so far. However, some symptomatic treatment methods are found to be helpful. The main point of the case report to be discussed is the liver transplantation done by our surgical team. There are no examples of any transplantation in Berardinelli-Seip syndrome patients, but several reports can be found of patients with kidney or liver failure. CONCLUSION: Berardinelli-Seip syndrome is a rare disorder with no cure but a chance of improving lifestyle and life expectancy. The transplantation option should be considered in young patients after a multidisciplinary review.

Metastasis to the stomach: a systematic review.

Background: This study reviews the literature on gastric metastases (GM) in terms of diagnosis, treatment, and outcomes. The goal of this study was to provide clinicians with a reliable and beneficial source to understand gastric metastases arising from various primary tumors and to present the growing literature in an easily accessible form. Methods: Articles published in English language from implementation of MEDLINE and Cochrane databases until May 2022 were considered for the systematic review. Articles other than English language, letters to the editor, posters, and clinical images were excluded. Hematogenous and lymphogenic metastases were included whereas direct tumoral invasion and seeding were excluded. Articles and abstracts were analyzed and last selection was done after cross-referencing and by use of defined eligibility criteria. Results: In total 1,521 publications were identified and 170 articles were finally included totaling 186 patients with GM. The median age of patients was 62 years. Gynecologic cancer was the most common cancer type causing GM (67 patients), followed by lung cancer (33 patients), renal cancer (20 patients), and melanoma (19 patients). One of the main treatment methods performed for metastasis was resection surgery (n=62), sometimes combined with chemotherapy (ChT) or immunotherapy. ChT was the other most used treatment method (n=78). Also, immunotherapy was amongst the most preferred treatment options after surgery and ChT (n=10). Conclusions: As 172 case reports were screened in the systematic review from different journals, heterogeneity was inevitable. Some articles missed important information such as complete follow-up or clinical information. Moreover, since all of the included articles were case reports quality assessment could not be performed. Among 172 case reports reviewed, resection surgery was performed the most and was sometimes combined with ChT and immunotherapy. Further research about what type of treatment has the best outcomes for patients with gastric metastases is needed.

Global Burden of Disease: Digestive Disease Alarm in South Caucasus.

Digestive disease-caused death rates are significantly high in the South Caucasus region. The latest Global Burden of Disease (GBD) data are a subject of discussion and should lead to serious steps to be taken. Azerbaijan, Armenia, and Georgia are three countries in the region with similar cultures but different roots. The problem seems to affect every country in the region with slightly different rates. It is crucial to start investigations into the detailed cause and to take serious steps in order to prevent digestive disease-caused deaths in the region. This letter aims to arouse awareness of the problem in the region.

Spontaneously Resolved Penile Cyst in a Fetus: A Case Report and Review of the Literature.

Cysts are uncommon congenital lesions of the fetal penis. Fetal penile cysts can develop when epithelial cells become entrapped during the fusion of the labial scrotal folds. The spectrum of diagnoses varies from simple epidermal inclusion cysts and megalourethra to hypospadias. In our case, we present a penile cyst that appeared between the 16th and 24th weeks. There was no other congenital anomaly, and since the mother did not accept an amniocentesis, we could not learn the karyotyping findings. As there were no other signs of congenital anomalies on ultrasound, we decided only to follow up, and at the 24th week's control ultrasound, the cyst was completely resolved with no other imaging findings.

Rectus Sheath Hematoma Secondary to Domestic Violence.

Rectus sheath hematoma (RSH) is an infrequent condition that occurs when epigastric arteries bleed into the rectus sheath and sometimes acts like an acute abdomen. In view of the fact that it is a rare case without specific clinic signs, misdiagnosis and use of invasive manipulations for patients are possible. A 27-year-old woman applied to our clinic with abdominal pain, weakness, and nausea. Acute abdominal pain was in the periumbilical region and physical examination revealed tenderness, rebound phenomenon, and local mass in the right periumbilical region. Rectus sheath hematoma was identified by magnetic resonance imaging. The cause in this case was abdominal trauma due to domestic violence. After the legal procedures, symptomatic treatment was prescribed to the patient. Interestingly in this case, we did not get to the diagnosis through history, we reached the history after diagnosis. Besides the treatment of the patient, maybe our team prevented further violence against a woman.

The Effectiveness and Feasibility of Laparoscopic Re-sleeve Gastrectomy.

BACKGROUND: Weight regain following laparoscopic sleeve gastrectomy (LSG) may be due to dilation of the gastric reservoir. Laparoscopic re-sleeve gastrectomy (LrSG) is among the revisional surgery options. OBJECTIVES: We aimed to investigate the effectiveness of LrSG for weight loss after a 12- and 24-month follow-up period. SETTING: Bariatric surgery center in Baku/Azerbaijan. METHOD: From June 2016 to June 2019, a total of 34 LSG patients with weight regain, underwent LrSG. We prospectively followed outcomes data were BMI changes, excessive weight loss, changes in laboratory values, and the presence of complications. RESULTS: The mean age at revision surgery was 36 ± 7.09 (range, 22-51) years, and the mean body mass index (BMI) before LrSG was 40 ± 5.2 kg/m2. The mean time between the primary and revision surgery was 50 ± 7.8 months. The main reasons for the revisions were weight regain and inadequate weight loss. The mean BMI value decrease at the 12th and 24th months were 27.7 ± 2 and 24.3 ± 1.02, which were statistically significant (p < 0.05). Analyses of hemoglobin A1C (A1C) values showed that the differences at the baseline, 12th and 24th months were statistically significant (95% 1.96 to 3.39, p < 0.001 and 95% CI 0.34 to 2.08, p = 0.005, respectively). CONCLUSIONS: In patients with weight regain or inadequate weight loss after LSG, LrSG may be a feasible and safe revisional procedure in a selected group of patients. Larger studies that compare other revisional surgery options (LRYGB, OAGB, duodenal switch, single anastomosis duodeno-ileal bypass) with LrSG are required.

Surgical management of insulinomas at the Azerbaijan Medical University: a retrospective study of 21 cases over a 10-year period

Background/aim: This study represents the first report that evaluates the experience gathered from diagnosis, surgical treatment and outcome of insulinoma patients from Azerbaijan. Materials and methods: We retrospectively review of insulinoma patients for a 10-year period. Collected data included patient demographics, laboratory and imaging tests, detailed surgical reports, histopathological examination of resected specimens, and clinical follow-up. Results: Twenty-one insulinoma patients were identified. Male patients comprised 52.4%; mean age was 44 years. Mean time to diagnosis was 14 months; 61% patients had ≥3 medical referrals due to hypoglycemia-related symptoms. Diagnosis sensitivity of CT, MRI and US was 85%, 80%, and 55%, respectively. The mean glucose, insulin, C-peptide levels were 35.7 ± 9.5 mg/dL, 33.5 ± 21.9 µU/mL, and 3.74 ± 1.88 ng/mL, respectively. Pancreatic head and tail were the most frequent tumor locations; mean tumor size was 1.5 ± 0.7 cm. No statistical association was found between the tumor size and preoperative glucose, C-peptide and insulin levels. Distal pancreatectomy and enucleation were the most common surgical procedures. Local tumor recurrence rate was 14%. There was no mortality. Conclusions: To prevent delayed diagnoses, physicians should be familiar with the typical symptoms of these rare tumors.

Comparison of Weight Loss in Sleeve Gastrectomy Patients With and Without Antrectomy: a Prospective Randomized Study.

BACKGROUND: Laparoscopic sleeve gastrectomy (LSG) restricts gastric volume to achieve weight loss. We aimed to compare the efficacy of LSG with and without antrectomy for achieving weight loss. METHODS: The prospective randomized study comprised 127 obese patients that underwent either LSG with antrectomy (2 cm to pylorus) (group 1) or LSG without antrectomy (6 cm to pylorus) (group 2), using 36 Fr and 32 Fr bougies, respectively. Patients were examined at 3-, 6-, 12-, and 24-month intervals for body mass index (BMI) measurements. RESULTS: Overall, 66 (51%) and 57 (49%) of patients were assigned to groups 1 and 2, respectively. The mean BMI of group 1 patients were 49.5 ± 8.01, 35.8 ± 5.40, 31.3 ± 4.9, 26.7 ± 4.02, and 22.9 ± 4.01 at the baseline, 3rd, 6th, 12th, and 24th month, respectively. The decreases in BMI were statistically significant. The mean BMI of group 2 patients were 46.7 ± 7.06, 39.3 ± 6.04, 32.4 ± 5.01, 26.6 ± 3.76, and 21.6 ± 3.70 at baseline, 3rd, 6th, 12th, and 24th month, respectively. The differences were also statistically significant. When compared with group 2, group 1 patients showed significantly lower BMI values on the 3rd month. Other differences were not statistically significant. CONCLUSION: LSG with or without antrectomy is safe and effective for weight loss. Larger studies are required to identify patients likely to benefit from LSG with antrectomy.