A rare pediatric case of McCune–Albright syndrome with acute visual disturbance: Case report

Rationale:McCune–Albright syndrome (MAS) is a rare disorder characterized by clinical findings, which includes fibrous dysplasia (FD). FD is a benign tumor that leads to increased rates of bone fracture. In some MAS cases with FD, facial deformities, severe pain, and orbital neuropathies are complicated. Aneurysmal bone cyst (ABC) is a benign bone tumor and rare complication of FD.Patient concerns:A 9-year-old boy was admitted to our hospital because of acute visual disturbance.Diagnosis and interventions:The patient was clinically diagnosed as ABC complicated with MAS, and he underwent surgery.Outcomes:After the surgery, his sight became normal. Recurrence of ABC and visual disturbance was not observed in 3 years. Genetic analysis of a tissue sample from the ABC lesion by next-generation sequencing revealed a somatic activating GNAS mutation.Lessons:To the best of our knowledge, this is the first case report of MAS causing optic neuropathy complicated with ABC. ABC complicated with MAS is extremely rare, but it should be considered as a possible diagnosis in patients with acute visual loss and facial swelling. In addition, our case had OAS, which is an uncommon syndrome and a rare complication in ABC with MAS, and rapid decompression of the ABC was effective in improving the patient''s eyesight.     

Outcome of allogeneic hematopoietic stem cell transplantation for mycosis fungoides and Sézary syndrome

Although allogeneic hematopoietic stem cell transplantation (HSCT) has been reported to provide prolonged remission of relapsed/refractory mycosis fungoides (MF) and Sézary syndrome (SS), its role has not been fully evaluated. Here, the outcomes of allogeneic HSCT for patients with MF/SS were retrospectively evaluated by using the registry database of the Japan Society for Hematopoietic Cell Transplantation. Forty-eight patients were evaluable and enrolled in the analysis. Median age was 45.5 years. Eighteen patients (38%) received myeloablative conditioning, and 33 (69%) received HSCT from an alternative donor. Disease status was complete or partial response in 25% of the patients and relapsed or refractory in the others. At the time of analysis, 18 patients were alive, with a median follow-up of 31.0 months (range, 3.8-31.1). Three-year overall survival (OS) and progression-free survival (PFS) were 30% (95%CI, 16-45%) and 19% (95%CI, 9-31%), respectively. Disease progression was not observed later than 17 months after transplantation. Both disease status and performance status at transplant significantly affected OS and PFS. Although our findings suggest that allogeneic HSCT provides long-term PFS in patients with MF/SS, the timing of transplantation should be decided carefully based on the disease status and the patient's condition in order to improve the outcome.     

An Earlier First Meal Timing Associates with Weight Loss Effectiveness in A 12-Week Weight Loss Support Program

Recent studies have reported that meal timing may play an important role in weight regulation, however it is unknown whether the timing of meals is related to the amount of weight loss. This study aimed to examine the relationship between indices of meal timing and weight loss during weight loss intervention in adults. A 12-week weight loss support program was conducted for 97 adults (age: 47.6 ± 8.3 years, BMI: 25.4 ± 3.7 kg/m²). After the program, body weight decreased by −3.0 ± 2.7%. Only the start of the eating window was positively correlated with the weight change rate in both sexes (men: r = 0.321, p = 0.022; women: r = 0.360, p = 0.014). The participants were divided into two groups based on the start of the eating window as follows: the early group (6:48 ± 0:21 AM) and the late group (8:11 ± 1:05 AM). The weight loss rate in the early group was significantly higher (−3.8 ± 2.7%) than that in the late group (−2.2 ± 2.5%). The present results showed that the start of the early eating window was associated with weight loss and suggested paying attention to meal timing when doing weight loss.     

Adolescents with chronic hepatitis C might be good candidates for direct‐acting antiviral therapy

Three Japanese adolescents with chronic hepatitis C were treated by direct‐acting antivirals (DAAs). No adverse events or laboratory abnormalities were observed during and after DAA therapy, and a sustained virological response was achieved in all cases. The emotional functioning of the patients and their mothers were improved after DAA therapy.     

Plasma Cholecystokinin Responses after Ingestion of Liquid Meal and Intraduodenal Infusion of Fat, Amino Acids, or Hydrochloric Acid in Man: Analysis with Region Specific Radioimmunoassay

A highly sensitive and precise radioimmunoassay system for plasma cholecystokinin (CCK) was developed with the anli-CCK-8 specific antiserum which raised against N-terminal amino acids residue of sulfated CCK-8 and reacted with CCK-8, CCK-33, and CK-39 but not with gastrin and its related peptides. Mean concentration of the fasting plasma CCK determined with this method using CCK-8 as standard was 12.9 ± 5.9 pg/ml in normal subjects (n = 26), and in patients with hepatic cirrhosis it was significantly higher (36.7 ± 16.9 pg/ml, n = 9, p < 0.01) than in normal subjects. In six young healthy volunteers, intraduodenal infusion of fat caused a significant increase ( p < 0.05) of plasma CCK from a basal level of 8.0 pg/ml to a peak of 43.0 ± 12.0 pg/ml at 20 min after starting of infusion. In the same subjects, a significant increase of plasma CCK was also observed by amino acids infusion, but no elevation of plasma CCK level was found during intraduodenal acidification.     

Radiological Demonstration of Spontaneous Rupture of a Pancreatic Pseudocyst into the Portal System

A rare case of a pancreatic pseudocyst that ruptured into the portal system is reported. Diagnosis was made preoperatively by a combination of radiological examinations. Details of the endoscopic retrograde cholangiopancreatogram, computed axial tomography and ultrasonically guided percutaneous transhepatic portography are presented.     

Pure laparoscopic right hepatectomy in the semi-prone position using the intrahepatic Glissonian approach and a modified hanging maneuver to minimize intraoperative bleeding

Purpose

Although laparoscopic liver resection has been widely adopted, performing a pure laparoscopic right hepatectomy remains a challenging procedure. The aim of this report is to evaluate the efficiency of a pure laparoscopic right hepatectomy (PLRH) in the semi-prone position using the intrahepatic Glissonian approach and a modified hanging maneuver.

Methods

Pure laparoscopic right hepatectomy was performed in the semi-prone position with the use of an intrahepatic Glissonian approach and modified hanging maneuver for patients with primary liver cancer (n = 3) and metastatic liver cancer (n = 1).

Results

The intraoperative total blood loss was only 95?C140 g (mean: 126.2 g). None of the patients required a blood transfusion, and no serious complications were encountered. The durations of the surgeries ranged from were 308 to 445 min (mean: 394.8 min). The postoperative hospital stay was 8?C11 days (mean 9.5 days).

Conclusion

Pure laparoscopic right hepatectomy in the semi-prone position using the intrahepatic Glissonian approach and a modified hanging maneuver is thus considered to be a safe modality, which minimizes intraoperative bleeding.     

Totally laparoscopic colectomy with intracorporeal anastomosis achieved using a laparoscopic linear stapler: experience of a single institute

Purpose  

Laparoscopic colonic surgery is now widely accepted. We assessed the safety and effectiveness of using a total intracorporeal surgical strategy to perform intracorporeal functional end-to-end anastomosis with an endoscopic linear stapler to treat colon cancer.     

Is “functional end-to-end anastomosis” really functional? A review of the literature on stapled anastomosis using linear staplers

Purposes

Anastomosis is one of the basic skills of a gastrointestinal surgeon. Stapling devices are widely used because stapled anastomosis (SA) can shorten operation times. Antiperistaltic stapled side-to-side anastomosis (SSSA) using linear staplers is a popular SA technique that is often referred to as “functional end-to-end anastomosis (FEEA).” The term “FEEA” has spread without any definite validation of its “function.” The aim of this review is to show the heterogeneity of SA and conventional hand-sewn end-to-end anastomosis (HEEA) and to advocate the renaming of “FEEA.”

Methods

We conducted a narrative review of the literature on SSSA. We reviewed the literature on ileocolic and small intestinal anastomosis in colonic cancer, Crohn’s disease and ileostomy closure due to the simplicity of the technique.

Results

The superiority of SSSA in comparison to HEEA has been demonstrated in previous clinical studies concerning gastrointestinal anastomosis. Additionally, experimental studies have shown the differences between the two anastomotic techniques on peristalsis and the intestinal bacteria at the anastomotic site.

Conclusions

SSSA and HEEA affect the postoperative clinical outcome, electrophysiological peristalsis, and bacteriology in different manners; no current studies have shown the functional equality of SSSA and HEEA. However, the use of the terms “functional end-to-end anastomosis” and/or “FEEA” could cause confusion for surgeons and researchers and should therefore be avoided.