Neonatal Graves'' disease.

A newborn boy was noted by his mother to have a prominent left eye at birth, but an eye examination was delayed until age 7 months, at which time his ophthalmologist diagnosed exophthalmos. Computed tomography was interpreted as showing mild, diffuse, optic nerve thickening bilaterally suggestive of optic nerve gliomas. Subsequent examination in our clinic revealed pseudoproptosis secondary to retraction of the left upper eyelid. Magnetic resonance imaging demonstrated normal orbital structures. The mother was noted to be clinically hyperthyroid, and abnormal thyroid function tests confirmed the diagnosis. Although the infant was euthyroid, neonatal Graves' ophthalmopathy was diagnosed. He was managed by close observation while his mother was treated for her hyperthyroidism.     

Peritoneal carcinomatosis: preoperative CT with intraperitoneal contrast material.

Thirty-five abdominal computed tomographic (CT) scans of 27 patients with peritoneal metastases from a mucin-producing tumor of the appendix, colon, small bowel, or ovary were retrospectively reviewed. Fifteen scans were obtained of 15 patients after CT with intraperitoneal infusion of contrast material (IP), and 20 scans were obtained of 16 patients with CT without IP. Subsequent exploratory laparotomy revealed that all 27 patients had multi-focal spread of peritoneal metastases. The sensitivity of CTIP and CT without IP for detection of peritoneal metastases at all sites of involvement was 61% and 59%, respectively. For CTIP, the highest sensitivity was in the right subphrenic space (88%), splenic hilum (86%), and left subphrenic space (83%). For CT without IP, the highest sensitivity was noted in the splenic hilum (100%), left subphrenic space (75%), and left paracolic gutter (75%). CTIP and CT without IP had low sensitivity for detection of disease in the greater omentum (50% each) and small-bowel mesentery (38% and 59%, respectively), two areas that had the highest frequency of metastases.     

Infantile free sialuria without lysosomal storage

Except for two reported patients, increased free sialic acid excretion has been associated with lysosomal storage. This is a report of a child with progressive neurologic deterioration and increased excretion of free sialic acid. Although lysosomal storage was absent, nuclear invagination or inclusions were present.     

Recombinant human interferon alpha increases oestrogen receptor expression in human breast cancer cells (ZR-75-1) and sensitizes them to the anti-proliferative effects of tamoxifen

Exposure of ZR-75-1 human breast cancer cells for 48 h to human recombinant interferon alpha (IFN alpha) resulted in increased expression of oestrogen receptors as measured in a whole cell binding assay. This effect was inversely proportional to dose being significant following treatment with 10-100 IU IFN ml-1 and was only observed at a low initial cell plating density. The extent of the increase in oestrogen receptor levels ranged from 1.2- to 7.2-fold following treatment with 10 IU IFN ml-1. No increase in progesterone receptor expression was observed under the same experimental conditions. Concentrations of IFN which increased oestrogen receptor levels had no effect on cell proliferation. IFN (500 IU ml-1) inhibited cell proliferation and the combination of this treatment with tamoxifen (2 microM) had a greater anti-proliferative effect than either drug alone although there was no evidence of synergism. However, a 5-day pretreatment of cells with IFN (10 IU ml-1) markedly sensitised them to the growth-inhibiting effect of a subsequent 6-day exposure to tamoxifen.     

Enteric hyperoxaluria, nephrolithiasis, and oxalate nephropathy: potentially serious and unappreciated complications of Roux-en-Y gastric bypass.

BACKGROUND: Neither the presence nor prevalence of enteric hyperoxaluria has been recognized after Roux-en-Y gastric bypass (RYGBP). We have noted a high rate of oxalate nephrolithiasis and even 2 patients with oxalate nephropathy in this patient population postoperatively. Our aim was to determine the frequency of the occurrence and effects of enteric hyperoxaluria after RYGBP. METHODS: Retrospective review of all patients at our institution diagnosed with calcium oxalate nephrolithiasis or oxalate nephropathy after standard (n = 14) or distal (n = 9) RYGBP. The mean postoperative follow-up was 55 months. RESULTS: A total of 23 patients (14 men and 9 women; mean age 45 years; mean preoperative body mass index 55 kg/m(2)) developed enteric hyperoxaluria after RYGBP, defined by the presence of oxalate nephropathy (n = 2) or calcium oxalate nephrolithiasis (n = 21) and increased 24-hour excretion of urinary oxalate and/or calcium oxalate supersaturation. Enteric hyperoxaluria was recognized after a mean weight loss of 46 kg at 29 months (range 2-85) after RYGBP. Two patients developed renal failure and required chronic hemodialysis. Of the 21 patients with nephrolithiasis, 14 had no history of nephrolithiasis preoperatively, and 19 of 21 required lithotripsy or other intervention. Of the 23 patients, 20 tested had increased oxalate excretion, and 14 of 15 tested had high urine calcium oxalate supersaturation. CONCLUSION: Enteric hyperoxaluria, nephrolithiasis, and oxalate nephropathy must be considered with the other risks of RYGBP. Efforts should be made to identify factors that predispose patients to developing hyperoxaluria.     

Voice prosthesis insertion after endoscopic balloon-catheter dilatation in case of a stenotic hypopharyngo-oesophageal junction

Stenosis of the hypopharyngo-oesophageal junction can be a rare complication of laryngectomy and/or partial pharyngectomy and makes the insertion of voice prosthesis extremely difficult. This study describes the authors’ experiences gained by endoscopic balloon-catheter dilatation of hypopharyngo-oesophageal stenoses prior to implantation of voice prostheses in four cases. In two patients a single balloon-catheter dilatation resulted in wide enough pharyngo-oesophageal lumen on the long run. The average prosthesis wearing-times were 6.8 months in case 1 and 4.6 months in case 2, corresponding to the published literature data. In case 3, repeated dilatation of the pharyngo-oesophageal transition had proved to be unsuccessful despite taking every effort with the endoscopic balloon-catheter method. Having excised the stenotic segment, reconstruction with pectoralis major myocutaneous flap (PMMF) was indicated. Eighteen months later, a repeated restenosis was observed and a free jejunal flap needed to be performed as a final solution. In case 4, the insertion was carried out into a previously dilated jejunal free flap, which became gradually ischemic and stenotic since the major head-and neck procedure was carried out that resulted in prosthesis rejection after just 1 week. The authors emphasize that correct indication of pedicled and free flaps in head and neck reconstruction is a prerequisite from the aspect of prevention of pharyngo-oesophageal strictures. Endoscopic balloon-catheter dilatation is a safe and established method for dilatating hypopharyngo-oesophageal stenoses of different origin. The procedure provides maximum patient benefit with minimal trauma and morbidity; moreover, facilitates insertion of voice prostheses. However, a single balloon-catheter dilatation cannot always result in wide enough oesophageal lumen on the long run (case 3). Insertion of a voice prosthesis into a previously dilated ischemic jejunal segment is challenging and avoidable due to risks of complications.     

Buccal mucosal graft for urethral reconstruction.

Urethral reconstruction with graft substances, such as skin and bladder mucosa, has been previously used when primary anastomosis cannot be achieved. However, stricture and meatal prolapse are associated with these grafts. We report the use of buccal mucosa for the reconstruction of urethral defects in 3 patients. One patient with failed operation for hypospadias received tube buccal mucosal graft for urethral replacement. Two patients with urethral necrosis and stricture received onlay buccal mucosal graft. All patients were disease-free during follow-up (range, 12 to 49 months; mean, 36 months). One patient had a pinhole fistula that was successfully managed with simple repair. This technique appears to be useful for urethral reconstruction when a local graft is not available, even in patients with complicated conditions.