Hatice Toy

Blue Rubber Bleb Nevus Syndrome: Successful Treatment With Sirolimus

Blue rubber bleb nevus syndrome (BRBNS) is a rare disorder with characteristic vascular malformations of the skin, gastrointestinal system, and, less often, other organ systems. The characteristic cutaneous lesions consist of deep-blue, soft, rubbery blebs, which are easily compressible. The most serious complication is abundant gastrointestinal bleeding. We describe the case of an 8-year-old girl with diagnosed BRBNS who had multiple venous malformations all over her body, importantly, throughout the gastrointestinal tract, mouth, esophagus, stomach, small bowel, and colon. She presented with recurrent massive gastrointestinal bleeding and soft tissue hematoma despite prednisolone and α-interferon therapy. We started low-dose sirolimus as an antiangiogenic agent. The vascular masses were reduced rapidly and there was no gastrointestinal bleeding and muscular hematoma after sirolimus therapy. There was no drug adverse reaction at 20-month follow-up. To the best of our knowledge, this is the first report related to the use of sirolimus in a patient with BRBNS.

Diagnostic and prognostic value of procalcitonin and phosphorus in acute mesenteric ischemia

BACKGROUND In this study, using an animal model of acute mesenteric ischemia (AMI), we investigated the possible use of procalcitonin and phosphorus in the early diagnosis of AMI. METHODS In this study, 21 New Zealand rabbits were used. Subjects were allocated into three groups as Control, Sham and Ischemia. No intervention was performed in the subjects in the Control group. In the subjects in the Sham and Ischemia groups, laparotomy was performed with midline incision. In the Ischemia group, the superior mesenteric artery was found and tied after laparotomy. Blood was drawn from the animals in all groups at 0, 1, 3 and 6 hours, and procalcitonin and phosphorus levels were studied in these samples. RESULTS In the Ischemia group, the increase in the levels of serum phosphorus and procalcitonin was found to be statistically significant compared to the Control and Sham groups (p<0.05). The levels of phosphorus and procalcitonin were detected to increase from the 1st hour after ischemia onset, and the increase continued for the following 6 hours (p<0.05). CONCLUSION Phosphorus and procalcitonin may be important parameters for use in the early diagnosis and prognosis of AMI.

Hepatoblastoma in a child with neurofibromatosis type I.

A major hallmark of NF1 is the development of benign tumors, including peripheral neurofibromas, plexiform neurofibromas, gliomas of the optic tract, other low grade gliomas, and pheochromocytomas. Hepatoblastoma have not been previously reported in patients with neurofibromatosis type 1. We present a case of a 9‐month‐old boy diagnosed with both hepatoblastoma and neurofibromatosis type 1. Hepatoblastoma occurs in association with several well‐described cancer predisposition syndromes, including familial adenomatous polyposis, Beckwith–Wiedemann syndrome, Li‐Fraumeni syndrome, trisomy 18, and glycogen storage disease type I. This paper describes a case of hepatoblastoma diagnosed in association with neurofibromatosis type 1. Pediatr Blood Cancer 2007;49:357–359.

Treatment of plaque-type localized scleroderma with retinoic acid and ultraviolet A plus the photosensitizer psoralen: a case series

© 2007 The Authors 519 JEADV 2008, 22, 499–527 Journal compilation

Merkel cell carcinoma in a child.

Merkel cell carcinoma, a rare tumor of the skin with aggressive behavior, is usually fatal when advanced disease is present. Merkel cell carcinoma occurs mostly in white race between 60 and 80 years of age, however, it can occur in any races and ages. It is extremely rare in children. We present here a Merkel cell carcinoma in a boy. Its features and treatment modalities were discussed in the literature light.

THE EFFECT OF ILOPROST ON RENAL DYSFUNCTION AFTER RENAL I/R USING CYSTATIN C AND β2-MICROGLOBULIN MONITORING

The purpose of this study was to investigate the effect of iloprost, a cytoprotective prostacyclin analog, on renal injury during unilateral renal I/R in rats and to determine whether the levels of serum cystatin C (CyC) and &bgr;2-microglobulin (B2M), as markers of glomerular function, might denote this injury. Thirty-two Wistar rats were randomized into four groups (n = 8) as follows: control (sham laparotomy), renal I/R (60-min left renal ischemia and 120-min reperfusion), renal I/R + iloprost (20 ng kg−1 min−1 infusion during renal I/R period, i.v.), and control + iloprost. Blood and kidney tissue samples were obtained for biochemical and histological analysis from all rats. Serum urea, creatinine, CyC, and B2M levels were evaluated for biochemical analysis. Histopathological changes in renal structure were examined for histological analysis. Serum urea, creatinine, and CyC levels were significantly increased in the renal I/R group. Iloprost treatment decreased these three markers in the renal I/R + iloprost group. &bgr;2-Microglobulin levels were not significantly changed in any group. Histological analyses showed that renal I/R elicited significant renal injury, whereas iloprost significantly decreased I/R-induced renal injury. Serum CyC level is one of the good indicators of acute renal damage due to I/R produced by renal artery occlusion. In contrast, we have shown that there are no significant changes in the levels of serum B2M levels that would make it an accurate diagnostic tool for detecting acute changes in renal injury subject to renal I/R in rats.

The healing effects of tissue glues and healing agent locally applied on esophageal anastomoses.

OBJECTIVE This study aimed to investigate the effects of cyanoacrylate (C), fibrin glue (FG), and natrium hyaluronate (NH) on the healing of esophageal anastomosis (EA). METHODS Twenty-four rabbits were divided equally into 4 groups: primary anastomosis (PA), C, FG, and NH. A 1-cm-length of the cervical esophagus was resected through a cervical incision and then anastomosis was performed. C, FG, and NH were instilled into anastomosis lines in the respective groups. The animals were fed orally on postoperative day 7 on the condition that there was no esophageal leakage. The rabbits were sacrificed 8 weeks later to evaluate bursting pressure (BP), tissue hydroxyproline (HP) levels and wound healing scores (WHSs) in the anastomosis lines. RESULTS BP was significantly higher in the C group than in the PA, FG, and NH groups, and HP was significantly lower than in the other groups. WHSs in the PA and NH groups were lower than in the C and FG groups. CONCLUSIONS C and NH appear to be beneficial in EA healing with respect to increased BP and decreased HP when they are used simultaneously with PA prophylactically to prevent esophageal leakages and stricture.

Late-onset adenosine deaminase deficiency presenting with Heck’s disease

Focal epithelial hyperplasia, also known as Heck’s disease, is a rare but distinctive entity of viral etiology with characteristic clinical and histopathological features. It is a benign, asymptomatic disease of the oral mucosa caused by human papilloma viruses (HPV). Previous studies postulated an association between these lesions and immunodeficiency. Genetic deficiency of adenosine deaminase (ADA) results in varying degrees of immunodeficiency, including neonatal onset severe combined immunodeficiency (ADA-SCID), and milder, later onset immunodeficiency. We report a 12-year-old girl with the late onset-ADA deficiency presenting with Heck’s disease. Our case report should draw attention to the possibility of immunodeficiency in patients with HPV-induced focal epithelial hyperplasia.

Generalized idiopathic acanthosis nigricans treated with acitretin

Generalized idiopathic acanthosis nigricans is a rare form of acanthosis nigricans. This form may be familial or nonfamilial and begins at birth or in early childhood. We report an 18‐year‐old boy with generalized idiopathic acanthosis nigricans who has no family history of acanthosis nigricans. The patient was treated with oral acitretin. Acitretin has been reported rarely as a therapeutic retinoid agent in the disease to date.

The effect of zinc deficiency and zinc supplementation on element levels in the bone tissue of ovariectomized rats: Histopathologic changes

Abstract Objective: Study aimed to determine the effects of zinc supplementation/deficiency on the histological structure and elements levels in bone tissue in ovariectomized rats. Methods: The study included 40 Sprague-Dawley type adult female rats, divided as follows: Control, ovariectomized, ovariectomized + zinc supplemented, ovariectomized + zinc deficient groups. At the end of the study bone tissues (femur) were collected to determine the levels of calcium, phosphorus, magnesium, zinc, iron, aluminium, chrome, lithium, lead, nickel, and manganese. The bone tissue was examined for histopathology. Results: Ovariectomy leaded to significant decrease in magnesium. Zinc supplementation to ovariectomized rats restored the reduced calcium, phosphorus, zinc. However, zinc deficiency in ovariectomized rats further reduced calcium, phosphorus, zinc, and manganese levels. Zinc deficiency in ovariectomized significantly increased Al, Cr, Li, Pb, and Ni levels. Tissue integrity was impaired due to ovariectomy and zinc deficiency. Conclusion: Ovariectomy and zinc deficiency leads significant decreases elements of the bone.