In this paper, we describe two siblings with Juvenile Hyaline Fibromatosis (JHF) who were diagnosed at the age of 34 and 29 years respectively. JHF is a very congenital disease, mainly diagnosed in the first few years of life, with less than 40 published cases in literature. All the main clinical features of this syndrome, which may be summarised as multiple subcutaneous tumours, marked gingival hypertrophy, flexion contractures and osteolytic lesions were present in both of these cases. Clinical, radiological and histological differential diagnosis of JHF were made. Recent information about histopathology, treatment and prognosis of JHF was also reviewed.

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Background/Aim: The aim of this study is to investigate if there is a diagnostic expressive difference in the angle of His, upper and lower esophageal diameter (UED and LED), wall thickness of cervical esophagus (WTCE) measured by cervical and transabdominal ultrasonography (USG) between children with (+) and without gastroesophageal reflux (GER–). Methods: 50 children were separated into 2 groups. Group 1 was the control group (n = 30) consisting of children who had no symptoms of GER and had no GER detected in USG. Group 2 was the study group (n = 20) consisting of children with complaints related to GER, and who were GER+ by USG and esophageal pH monitoring. The USG examinations were completed after having observed 3 episodes of reflux or after 30 min if no reflux was detected. The angle of His, UED, LED and WTCE were measured. In 24-hour esophageal pH monitoring, acid contact to the proximal or distal probe greater than 5.0% of the total time below pH 4 was accepted as pathologic reflux. Results: The age range of the 50 children (30 boys) was 4–13 years. Between the 2 groups there were no differences with regard to age and gender. Values of UED, LED and WTCE were statistically higher in group 2 compared with group 1. The angle of His was higher in the control group, but this was not statistically significant. Conclusion: Our study is the first in the literature that shows that measuring UED, LED and WTCE by USG, which is a noninvasive, readily available repeatable, cheap and fast technique, has a high diagnostic value in children with suspected GER.

PurposeIn this manuscript we report one pediatric surgeon's experience in childhood inguinal hernia repair.MethodsFrom 2005 to 2008, 402 children with inguinal hernias were operated on by one surgeon. A retrospective survey of their charts was carried out to evaluate the demographics and clinical aspects of these patients.ResultsThe ages ranged from 20 days to 16 years with a male-to-female ratio of 2.5:1. 64.9% right, 27.1% left, and 7.9% bilateral hernias. Hydroceles were present in 6.2% assosiated hernias. Incarceration occurred in 8.7% of children. An opposite-side hernia developed in 5.7%. 5.3 percent of patients with a hernia repair on the right side later developed a hernia on the left side, and 8.2% of patients with a hernia repair on the left side later developed one on the right side. 4.5 percent of all male patients in this series and 8.6% of female patients developed an opposite-side hernia. While overall recurrence rate was 1.2%, our recurrence rate was 0.25%. There was a 0.24% wound infection rate, and 1 (0.24%) testicle was atrophic at follow-up.ConclusionIn this study, in the recurrence of childhood hernia, the general surgeon's intervention was the prominent cause. It is suggested by the study that inguinal hernias on the contralateral side becomes symptomatic within the first six months following initial operation.Therefor, close observation is needed in that time.

Mechanical bowel cleansing is considered to be necessary prior to colorectal surgery, some radiological or endoscopic procedures, and for fecal disimpaction. Traditional bowel cleansing (TBC) with cathartics and enemas is a method of mechanical bowel cleansing for patients who have restrictive factors for whole-bowel irrigation (WBI), such as excessive fecal impaction, cardiac, hepatic or renal disorders. In this experimental study, TBC with hyperosmolar agents was evaluated in terms of their effects on colonic flora and bacterial translocation (BT). Sprague-Dawley rats were divided into 6 groups. The animals, except controls, were not fed for 72 h but received tap water ad libitum. During this period, lactulose, lactitol, sodium phosphate (NaP), polyethylene glycol 3350 (PEG3350) and physiological saline gavages were administered to the rats in groups 1–5, respectively, once a day. All animals except controls (group 6) received enemas with 15 ml of warm saline twice a day. The cecum, mesenteric lymph nodes (MLNs) and portal vein blood were sampled for microbiological and histopathological analysis. The highest level of coliform bacterial overgrowth and BT to MLNs were observed in the lactulose group, although the others, except the saline and control groups, also caused some degree of BT. Portal vein cultures were negative for all groups. Histopathological damage was not associated with cecal bacterial count and BT. As a result of this study, PEG3350 seems to be safer and more effective than lactulose, lactitol and NaP during TBC.