Upper gastro-intestinal tract bleeding in cirrhotic children candidates for liver transplantation

Signs of portal hypertension, history of upper gastro-intestinal tract bleeding episodes and outcome of the latter were recorded in 76 cirrhotic children evaluated for liver transplantation. Fifty-three (70%) had varices and 22 (29%) had experienced upper gastro-intestinal tract bleeding. Of these 22, 19 bled from varices and 3 from ulcers. Non bleeding ulcers were also found in five patients bleeding from varices. Iterative sclerotherapy controlled acute variceal bleeding in all but one patient in whom emergency transplantation was performed. Six of the eight patients with ulcers were successfully treated by the H2 histamine receptor antagonist ranitidine. We conclude that iterative sclerotherapy is efficient to control acute variceal bleeding and prevents recurrent bleeding in children with end-stage liver diseases awaiting liver replacement. Bleeding asymptomatic ulcers are frequent and respond to H2 histamine receptor antagonists.     

Unifocal stricture of the common bile duct in two children: a localized form of primary sclerosing cholangitis

A 4-year-old boy and a 14-year-old girl referred for a cholestatic syndrome were found to have a unifocal stricture of the extrahepatic bile duct. Both radiological (endoscopic-retrograde-choledocopancreatography) and histological findings were suggestive of primary sclerosing cholangitis. There was no past history of chronic inflammatory bowel disease nor any other disorder. Serum autoimmune markers were negative. Complete remission was observed after surgical drainage, and both patients remained symptom-free after a follow-up of 12 months and 8 years, respectively. Such unifocal form of the disease may have a better prognosis than the plurifocal disease. The absence of associated disorders and autoimmunity may also be an indicator of better prognosis in these patients.     

Prevalence and treatment of silent gastro-oesophageal reflux in children with recurrent respiratory disorders

Thirty-six infants and children presenting with recurrent respiratory disorders (RRD) as the sole clinical symptom including bronchial asthma (6), recurrent obstructive bronchitis with or without wheezing (18), chronic nocturnal cough (3), recurrent episodes of pneumonia (3), recurrent pharyngitis (3) and recurrent laryngitis (3) were investigated for associated gastro-oesophageal reflux (GER) by oesophagram, endoscopy and continuous 24 h pH monitoring of the distal oesophagus.The pH monitoring criteria were selected on the basis of a preliminary study comparing statistically measurements of 32 variables recorded in 15 patients who all had clinical, radiological and endoscopic evidence of GER and in 8 asymptomatic controls. Although patients with symptomatic GER differed significantly from the asymptomatic ones for 27 variables examined, 6 variables emerged as having the highest value for discrimination (overlap score 0–1). Among these, the Euler-Byrne index (number of reflux pH<4+4 times the number of reflux episodes of more than 5 min), the percentage of total reflux time and the number of reflux episodes 1 h post-cibal scored 0 (no overlap). GER was considered to be present when at least five of these six parameters were abnormal.The overeall incidence of GER in children with RRD was 41% (15) when detected by oesophagram and 61% (22) when diagnosed by pH monitoring criteria. In the children with bronchial asthma or with recurrent laryngitis, the percentage of reflux time during sleep was about 40 times higher than in asymptomatic controls and 2 times higher than in those with symptomatic GER. Of the 22 patients with RRD and GER, 9 were elected to have fundoplication because of poor response to medical antireflux management. All of these, showed complete (6) or partial (3) resolution of respiratory symptoms. Of the 13 patients in whom medical therapy was continued, 9 showed satisfactory improvement with a mean follow-up time of 1 year. Silent GER might be an important contributing factor to the severity of some common chronic respiratory disorders in children.Abbreviation RRD recurrent respiratory disorders - GER gastro-oesophageal reflux     

Operative treatment in case of a closed rupture of the anterior tibial tendon

Closed rupture of the tibial anterior tendon is a rare clinical entity. Case reports in the literature reveal a total of only 49 cases up to the year 2000. According to these reports, the age group affected is 50 to 70 years old, and there are more men than women affected. Although the functional limitation is quite considerable, late diagnosis is common. An appropriate clinical examination, including an exact history taking, should lead to the right diagnosis. Ultrasound examination and magnetic resonance imaging (MRI) may be helpful. 'Restitutio ad integrum' can only be achieved by operative treatment. If technically possible, reinsertion of the tendon directly into bone or direct tendon repair is preferred. After delayed diagnosis, a secondary reconstruction through tendon transfer or transplantation is often necessary. A 64-year-old woman presented with pain and swelling in the area of the ankle joint 5 months after falling. She showed insecurity in walking, and the heel-walk could not be demonstrated. The distal neurovascular function was intact. The area of the retinaculum showed a swelling, and the tendon was not palpable in comparison with the other forefoot. An intact tendon could not be seen by ultrasound, and MRI confirmed these findings. A complete rupture was noted during the operative revision. The proximal and the distal tendon stumps were found to be thickened and knotted, the proximal stump was also atrophic. An augmented tenoplasty was performed. Afterwards, the tendon was tense in the neutral position. The lower leg was put in a plaster cast for 6 weeks, followed by physiotherapy. Ten months after the operation, the tendon was palpable in the correct position, the dorsal extension was powerful, and the patient did not experience any difficulty. Rupture of the anterior tibial tendon is a rare clinical entity and should be considered in the differential diagnosis of pain in the area of the ankle joint. An early operative treatment is advantageous.     

Surgical view of the treatment of patients with hepatoblastoma: results from the first prospective trial of the International Society of Pediatric Oncology Liver Tumor Study Group

BACKGROUND: Surgical resection is the cornerstone of treatment for patients with hepatoblastoma (HB). The Society of Pediatric Oncology Liver Tumor Study Group launched its first prospective trial (SIOPEL-1) with the intention to treat all patients with preoperative chemotherapy and delayed surgical resection. The objective of this article was to assess the assumed surgical advantages of primary chemotherapy. METHODS: Between 1990 and 1994, 154 patients age < 16 years with HB were registered on SIOPEL-1. The pretreatment extent of disease was assessed, and, after undergoing biopsy, patients were treated with cisplatin 80 mg/m(2) intravenously over 24 hours and doxorubicin 60 mg/m(2) intravenously over 48 hours by continuous infusion (PLADO). Generally, tumors were resected after four of a total of six courses of PLADO. RESULTS: One hundred twenty eight patients underwent surgical resection (13 patients underwent primary surgery, and 115 patients underwent delayed surgery after PLADO). A pretreatment surgical biopsy was performed in 96 of 128 patients (75%). Biopsy complications occurred in 7 of 96 patients (7%). Twenty-two patients showed pulmonary metastases at the time of diagnosis, and 7 patients underwent thoracotomy. Operative morbidity and mortality were 18% and 5%, respectively. Complete macroscopic surgical resection was achieved in 106 patients (92%), including 6 patients who underwent orthotopic liver transplantation. The actuarial 5-year event free survival (EFS) rate for all 154 patients in the study was 66%, and the overall survival (OS) rate was 75%. For the 115 patients who were included in the surgical analysis that followed the exact protocol, the EFS and OS rates were 75% and 85%, respectively. CONCLUSIONS: Biopsy is a safe procedure and should be performed routinely. Preoperative chemotherapy seems to make tumor resection easier. Reresection of a positive resection margin does not necessarily have to be performed, because postoperative chemotherapy showed good results. Resection of lung metastases can be curative if there is local control of the primary tumor; however, results showed that the patient's prognosis was worse. Surgical morbidity or mortality rates were not necessarily higher in large multicenter studies. More importantly, countries of lesser economic status also can contribute effectively to these trials.     

Gabapentin versus lamotrigine monotherapy: a double-blind comparison in newly diagnosed epilepsy

PURPOSE: This randomised, double-blind study compared the newer antiepileptic drugs (AEDs) gabapentin (GBP) and lamotrigine (LTG) as monotherapy in newly diagnosed epilepsy. METHODS: Patients with partial seizures with and/or without secondary generalization or primary generalized tonic-clonic seizures were randomized to either GBP or LTG. During 2- and 6-week titration periods, respectively, GBP dosage reached 1,800 mg/day, and LTG, 150 mg/day. In the subsequent 24-week maintenance phase, the dose could be adjusted based on seizure control or adverse events between 1,200 and 3,600 mg/day for GBP and 100 and 300 mg/day for LTG. The primary end point was time to exit, a composite of efficacy and tolerability. Evaluable patients were used for the primary efficacy analysis, whereas tolerability was examined on an intent-to-treat basis. RESULTS: A total of 309 patients was randomized, and 291 (148 GBP, 143 LTG) were included in the evaluable population. Nineteen patients in each group had an exit event. The median time to exit was 69 days for GBP and 48 days for LTG. The hazard ratio was estimated as 1.043 (90% confidence intervals, 0.602-1.809). Overall, 106 (71.6% of the evaluable population) GBP-treated and 96 (67.1%) LTG-treated patients completed the study. Of those, 80 (75.5%) patients taking GBP and 73 (76.0%) taking LTG remained seizure free during the final 12 weeks of treatment. Only 14 (8.9%) GBP-treated patients and 15 (9.9%) LTG-treated patients withdrew because of study drug-related adverse events. CONCLUSIONS: GBP and LTG monotherapy were similarly effective and well tolerated in patients with newly diagnosed epilepsy.     

Mineralocorticoid receptor function in patients with posttraumatic stress disorder

OBJECTIVE: The study examined whether enhanced limbic mineralocorticoid receptor activity resulting in negative glucocorticoid feedback could contribute to the diminished basal and stress-induced cortisol output reported in patients with posttraumatic stress disorder (PTSD). METHOD: The effects of acute antimineralocorticoid (spironolactone) versus placebo pretreatment on levels of plasma cortisol at baseline and after stimulations with corticotropin-releasing hormone (CRH) and on adrenocorticotropic hormone (ACTH) level were measured in 12 PTSD patients and 12 healthy comparison subjects. RESULTS: Spironolactone significantly elevated basal cortisol and ACTH concentrations as well as cortisol secretion after CRH stimulation, but no differential effect between PTSD patients and comparison subjects was detected. CONCLUSIONS: The results indicate intact, but not enhanced, mineralocorticoid receptor function in PTSD. The study's experimental conditions did not allow determination of whether other compensatory factors might have masked the putative mineralocorticoid receptor changes.     

Detection of somatostatin receptor-positive tumours using the new 99mTc-tricine-HYNIC-D-Phe1-Tyr3-octreotide: first results in patients and comparison with 111In-DTPA-D-Phe1-octreotide

Indium- 111 labelled DTPA-D-Phe1-octreotide (DTPA-OC, OctreoScan) has been introduced into clinical routine for the detection of somatostatin receptor (SSTR)-positive tumours, which are predominantly of neuroendocrine origin. Potential further applications in other SSTR-positive cancers (e.g. small cell lung cancer, breast cancer, melanoma) have been limited mainly by the restricted availability and the high radionuclide costs. Previous attempts to introduce technetium-99m labelled analogues of octreotide have not been very successful in terms of the labelling procedure, in vivo biodistribution and/or tumour detection capabilities. The aim of this study was to assess the performance of the new 99mTc-labelled analogue HYNIC-D-Phe1-Tyr3-octreotide (HYNIC-TOC), using tricine as co-ligand, for the detection of SSTR-positive tumours in patients in comparison with 111In-DTPA-OC. Overall, 13 patients were examined using 99mTc-tricine-HYNIC-TOC. Twelve patients had proven SSTR-positive tumours, while one patient presented with an SSTR-negative tumour. In 9 of the 13 patients both tracers (99mTc-tricine-HYNIC-TOC and 111In-DTPA-OC) were used. Serial whole-body scans, spot views and/or single-photon emission tomography studies were performed. Images were qualitatively and semi-quantitatively (ROI analyses) evaluated. The biodistribution of 99mTc-tricine-HYNIC-TOC in patients showed high physiological uptake in kidneys, moderate uptake in liver and spleen and little uptake in the gut. The tracer showed predominantly renal and negligible hepatobiliary excretion. Known SSTR-positive tumour sites showed rapid and intense tracer accumulation. 99mTc-tricine-HYNIC-TOC demonstrated rapid tissue uptake within the first hour after injection and had basically no significant clearance (<20%) from normal or tumour tissue thereafter. In contrast, 111In-DTPA-OC showed continuous clearance from normal tissues as well as renal and very little hepatobiliary excretion. Nevertheless, the patterns of accumulation of 99mTc-tricine-HYNIC-TOC in tumours and normal organs were comparable to those of 111In-DTPA-OC. A lesion-by-lesion comparison showed comparable tumour detection capabilities in intrahepatic tumour sites and superior capabilities of 99mTc-tricine-HYNIC-TOC in respect of extrahepatic lesions. In conclusion, 99mTc-tricine-HYNIC-TOC shows promise as a tracer for SSTR imaging, given its favourable clinical characteristics (specific and high receptor affinity, good biodistribution, renal excretion, low radiation exposure, high imaging quality, on-demand availability) and cost-effectiveness. 99mTc-tricine-HYNIC-TOC allows earlier diagnosis (10 min-4 h) compared with 111In-DTPA-OC (4-24 h).