A case of double cystic esophageal duplication in VACTERL syndrome: the first case report and a review of the literature.

Background: An esophageal duplication cyst (EDC) is a rare malformation resulting from the embryonic foregut. VACTERL syndrome is a genetic disorder affecting many systems of the human body. We report the first case of VACTERL syndrome associated to asymptomatic double EDC. Case report: A girl with anorectal malformation and rectovestibular fistula, kidney malformation, and various vertebral defects came to our attention at the time of birth. VACTERL disease was diagnosed. She underwent Peña anoplasty at 4 months of life without complications. MRI was conducted at the age of 2. It accidentally showed a double esophageal duplication (12 mm × 35 mm × 10 mm) at the D7-D9 level. We planned a thoracoscopy; previous intraoperative esophagogastroduodenoscopy showed an external compression of the native esophagus. Two duplicated esophageal lesions were removed. The patient made an uneventful recovery and was completely asymptomatic at long-term follow-up. Conclusions: VACTERL syndrome is still a not well-defined disease. Based on the current literature, this is the first case of a double esophageal duplication in a patient affected by VACTERL syndrome. According to us, the thoracoscopic approach of esophageal duplications can be followed by experts. Complete surgical excision is possible even if the cyst shares a common muscular wall with the esophagus. For this reason, we suggest to close the muscular wall by a simple interrupted suture.

Adrenal pseudocyst mimicking cancer: a case report.

Adrenal cysts are infrequently observed, since less than 500 cases have been reported in Western literature. Adrenal cysts are conventionally divided into four categories: epithelial, parasitic, endothelial, and hemorrhagic. They are characterized by different etiological and pathological features. Some authors suggest that endothelial and hemorrhagic cysts are related and may represent a spectrum of lesions. We report herein the case of an adrenal hemorrhagic pseudocyst that simulated adrenocortical cancer and argue on the clinical clues for a differential diagnosis with other adrenal tumors.

High prevalence of colonic polyps in patients with acromegaly. Influence of sex and age.

BACKGROUND: An association between acromegaly and colonic polyps has been reported, although risk factors are still uncertain. METHODS: Full colonoscopy was performed with a fiberoptic colonoscopy on 31 acromegalic patients, 11 men and 20 women aged 27 to 85 years (mean, 52.2 years), and on 236 subjects, 127 men and 109 women aged 23 to 84 years (mean, 50.1 years), referred for hemorrhoids, who were considered controls. The colonoscopic findings were evaluated in relation to demographic, clinical, and hormonal data pertaining to the two groups. RESULTS: The prevalence of either adenomatous or hyperplastic polyps was higher in acromegalic patients than in controls (38% vs 14% and 26% vs 10%, respectively; P < .001, respectively). Acromegalics with and without colonic adenomas did not differ significantly in growth hormone and insulinlike growth factor I levels or duration of acromegalic disease and its status (activity or remission); however, patients with adenoma were younger (median age, 50.5 vs 59 years; range, 27 to 85 years vs 39 to 66 years; P < .05). An opposite age pattern was observed in the control group. Indeed, the prevalence of adenoma in acromegalic patients was much higher than that in controls among those less than 50 years of age (46% vs 7%, P < .001); the difference was less remarkable at older ages. Adenomatous polyps were more frequently found in male subjects, in both patients and controls (45% vs 33% [not significant] and 19% vs 9% [P < .05], respectively). CONCLUSIONS: Acromegaly may carry an increased risk of colonic adenoma, especially in younger patients, who usually display more aggressive disease. A smaller increase in risk was observed in elderly patients, in whom disease is reportedly milder. We suggest that acromegalic patients should undergo screening colonoscopy.

Dynamics of the binding capacity of plasma sex hormone binding globulin (SHBG) for testosterone and dihydrotestosterone during puberty.

Plasma sex hormone binding globulin binding capacity (SHBG-b.c.) has been evaluated in 203 normal subjects (114 males and 89 females) aged 3 to 51 years. The subjects were divided into groups: prepubertal, early pubertal (Tanner's Stages 1 and 2), late pubertal (Tanner's Stages 4 and 5) and adult. In both sexes, plasma mean values of SHBG binding capacity both for dihydrotestosterone (DHT) and testosterone (T) were significantly higher in prepubertal subjects, falling during puberty to adult levels. During pubertal development DHT-BG binding capacity and T-BG binding capacity showed different plasma values with respect to sex and phase of puberty. Our data do not support an absolute relationship between sex hormones and SHBG binding capacity, but suggest other mechanisms as well: (a) SHBG modifies its physicochemical properties during puberty, or (b) the binding capacity is the result of a pool of proteins which modifies its composition during pubertal evolution.

Third-line chemotherapy with mitomycin C and lonidamine in advanced bladder cancer. Partial response in a patient with skin and lung metastases.

Skin metastases from transitional cell carcinoma are quite rare. The present case report describes the results of a combination of mitomycin C and lonidamine administered as third-line chemotherapy in a patient with pulmonary and skin involvement from bladder cancer. The partial response obtained suggests that further testing should be carried out on the activity of this association in a second-line approach.

Plasma bile pigment conjugation modalities in icterus syndromes of various origin.

Plasma azopigments derived from conjugated bilirubin were analyzed by thin-layer chromatography according to HEIRWEGH et al. in 14 cases of obstructive jaundice and in 11 of acute hepatitis. The chromatographic patterns were compared with those obtained from azopigments derived from 8 normal bile samples. The plasma pigment patterns did not differ from those of the bile in number and chromatographic mobility of the spots. However, the quantitative percentages of the plasma azopigments were significantly modified: the alpha 0 fraction (free azodipyrrolic pigment) increased in both icteric syndromes, while the delta fraction (mainly glucuronide azopigment) decreased. Moreover, the behavior of two closed components of the delta group showed significant differences in both icteric syndromes. It can be postulated that the synthesis of bilirubin diconjugates decreases both in hepatocellular and cholestatic jaundice, while monoglucuronidated as well as saccharide and glucoside conjugates increase. In cholestatic jaundice the conjugation with glucuronic acid mainly takes place in the normal way, whereas compounds with different features are formed in hepatitis.

Twenty-four hour profile of blood pressure in patients with acromegaly. Correlation with demographic, clinical and hormonal features.

Cardiovascular events are frequently reported in patients with acromegaly and they are usually related to arterial hypertension. Aim of the present study was to assess the 24-hour profile of blood pressure (BP) and heart rate (HR) in patients with active acromegaly and to correlate them with clinical and hormonal data. Sixteen patients and 16 healthy, age and sex matched subjects underwent ambulatory blood pressure monitoring by means of a portable automatic device (SpaceLabs monitor 90207, Kontron) with measurements every 20 minutes for 24 hours. The presence of the nocturnal fall was assessed by the calculation of the night-day systolic and diastolic ratio. The mean 24-hour diastolic BP was significantly higher in acromegalic patients than in controls (79.1+/-11.5 mmHg vs 70.8+/-5.3 mmHg, p<0.05) and the circadian diastolic profile was flatten. In fact, 10/16 patients were defined as nondippers while this figure was 2/16 in the control group (62% vs 12%, p<0.01). Also the mean 24-hour systolic BP was higher in acromegalic patients than in controls (124.8+/-17.2 mmHg vs 114.1+/-8.6 mmHg, p<0.05). The circadian systolic profile paralleled that of diastolic and was flatten, without a significant nocturnal fall. Ten out of 16 patients were nondippers compared to 2/16 controls (62 vs 12%, p<0.01). No significant correlation was found between mean 24-hour BP, either diastolic or systolic, and demographic or hormonal characteristics of the patients. HR patterns did not differ between patients and controls and were characterized by a prominent nocturnal fall.