Transthyretin amyloidosis with cardiomyopathy after domino liver transplantation: Results of a cross-sectional study

Domino liver transplantation (DLT) has been used widely in patients with hereditary amyloid transthyretin (ATTR) amyloidosis. New-onset polyneuropathy in recipients of DLT has been reported, but there are few cases of cardiac involvement reported. We aimed to perform a cross-sectional study for ATTR amyloidosis with cardiomyopathy (ATTR-CM) in DLT recipients. We evaluated 23 living DLT recipients a median of 9 years since DLT at 2 referral centers with a systematic cardiac evaluation, including bone scintigraphy. Median age was 72 years, 91% had hypertension, 35% had diabetes mellitus, 67% had chronic renal failure, and 8 patients (35%) developed new-onset polyneuropathy. Only 13% had a normal electrocardiogram and a normal echocardiography, and most of them showed some conduction disturbance or increase in left ventricular wall thickness, but only 1 patient with a Glu89Lys mutation developed ATTR-CM diagnosed by bone scintigraphy and endomyocardial biopsy. None of the recipients of a DLT with Val30Met mutation showed cardiac involvement by bone scintigraphy. In conclusion, DLT from Val30Met donors seems to be safe regarding the development of ATTR-CM. Evaluation of cardiomyopathy in DLT recipients is challenging due to concomitant comorbidities and in this context, bone scintigraphy can be helpful to evaluate ATTR-CM.     

Robotic transanal total mesorectal excision: Is the future now?

Total mesorectal excision (TME) is the standard surgical treatment for the curative radical resection of rectal cancers. Minimally invasive TME has been gaining ground favored by the continuous technological advancements. New procedures, such as transanal TME (TaTME), have been introduced to overcome some technical limitations, especially in low rectal tumors, obese patients, and/or narrow pelvis. The earliest TaTME reports showed promising results when compared with the conventional laparoscopic TME. However, recent publications raised concerns regarding the high rates of anastomotic leaks or local recurrences observed in national series. Robotic TaTME (R-TaTME) has been proposed as a novel technique incorporating the potential benefits of a perineal dissection together with precise control of the distal margins, and also offers all those advantages provided by the robotic technology in terms of improved precision and dexterity. Encouraging short-term results have been reported for R-TaTME, but further studies are needed to assess the real role of the new technique in the long-term oncological or functional outcomes. The present review aims to provide a general overview of R-TaTME by analyzing the body of the available literature, with a special focus on the potential benefits, harms, and future perspectives for this novel approach.     

Exposición al radón y cáncer genitourinario en mineros

ObjectiveTo assess the association between exposure to radon and genitourinary cancer in a mining population through a systematic review of the scientific literature.MethodA systematic review of the scientific literature was carried out in MEDLINE (PubMed), combining MeSH (Medical Subject Heading) terms and free terms. We applied a specific scale to assess the quality of the included studies.ResultsWe included 17 studies; all were cohort studies with the exception of one which was a pooling of data. All studies included analysed the relationship between exposure to radon and genitourinary cancer. While some studies point towards an association between radon exposure and genitourinary cancer, especially kidney cancer, others do not find such association.ConclusionsThe included studies showed great heterogeneity. It cannot be concluded that there is an association between exposure to radon and genitourinary cancer. More research is needed on this topic, designing studies with higher statistical power, better control of confounders, and preferably prospective.     

Role of endomyocardial biopsy in the diagnosis and management of cardiomyopathies

Abstracts

Role of endomyocardial biopsy in the diagnosis and management of cardiomyopathies     

Calcitriol oral pulse therapy in children with renal osteodystrophy

A 6-month protocol of oral pulse calcitriol was used in nine uraemic children (2–14 years old) on dialysis who presented with renal osteodystrophy. Calcitriol was administered twice a week, 4 g per dose for patients over 30 kg and 3g for patients less than 30 kg. Plasma levels of parathyroid hormone, calcium, phosphorus and alkaline phosphatase were carefully controlled during the study. Parathyroid hormone levels decreased by 68% and 56% by the 2nd and 6th months of treatment in seven patients, while they remained unchanged in two patients with focal segmental glomerulosclerosis and massive proteinuria. Eight hypercalcaemic episodes from 77 determinations were observed, all of them recovered after 1 week of vitamin D withdrawal. We conclude that oral calcitriol pulse therapy is a good alternative for renal osteodystrophy in uraemic children. Careful monitoring of plasma parathyroid hormone and calcium is needed during follow-up when using this approach in paediatric patients.     

Distribution of PCB Congeners, DDE, Hexachlorobenzene, and Methylsulfonyl Metabolites of PCB and DDE Among Various Fractions of Human Blood Plasma

The concentrations of chlorinated biphenyls (CBs), 1,1-bis(4-chlorophenyl)-2,2-dichloroethene (DDE), hexachlorobenzene (HCB), and the methylsulfonyl metabolites of CBs (MeSO₂-CBs) and DDE (MeSO₂-DDE) were determined in human plasma samples and in the fractions obtained by ultracentrifugation of plasma into very-low-density (VLDL), low-density (LDL), high-density (HDL) lipoprotein and lipoprotein depleted (LPDP) fractions (containing primarily albumin). The concentrations of triacylglycerols, cholesterol, phospholipids, and apolipoprotein B (apoB) were determined. The organochlorine compounds were associated with all fractions, but predominantly with the LPDP fraction. On an average 44% of CBs, 61% of MeSO₂-CBs, 73% of DDE, 77% of MeSO₂-DDE, and 45% of HCB were distributed in the LPDP fraction. A tendency to greater association of 3-methylsulfonyl substituted than of corresponding 4-methylsulfonyl substituted chlorobiphenyls to the LPDP fraction was noticed. Among the lipoprotein fractions, LDL was the main carrier of HCB, DDE and CBs. MeSO₂-DDE was predominantly found in HDL and MeSO₂-CBs were distributed equally among the LDL and HDL fractions. Calculating the concentrations of organochlorine compounds in relation to the content of apoB, the levels were about 10 times higher in VLDL than in LDL. Received: 24 April 1998/Accepted: 24 April 1999     

Unintentional human skeletal imaging with99mTc-methylene diphosphonate 45 months beyond expiration

Eight patients were inadvertently administered, and imaged with, 99mTc-labeled MDP which was 45 months expired. Two cases are presented. All patients were subsequently imaged with normal MDP. The images obtained with expired MDP were clinically acceptable. No differences in scan abnormalities were observed compared with normal MDP for any of the patients. None of the patients suffered any side effects attributable to the expired MDP.     

Prognostic factors in fetal hydronephrosis: a multivariate analysis

With the increasing use of obstetric echography fetal hydronephrosis has been reported more frequently. The purpose of this study was to identify prognostic factors associated with adverse outcome, such as renal failure and death, in fetal hydronephrosis. One hundred and forty-eight children with fetal hydronephrosis were admitted, submitted to a systematic protocol, and prospectively followed. Prognostic factors associated with fetal echography and clinical and laboratory findings on admission were studied. The median follow-up was 39 months. The analysis was conducted in two steps. In a univariate analysis, variables associated with adverse outcome were identified by the Kaplan-Meier method. The variables that were significantly associated with adverse outcome were then included in a multivariate analysis. This analysis, using the multivariate Cox’s model, was performed to identify variables that were independently associated with a worse prognosis. Only variables that remained independently associated with adverse outcome were included in the final model. After final adjustment by Cox’s multivariate model, three variables were identified as independent predictors of adverse outcome: oligohydramnios, prematurity, and glomerular filtration rate lower than 20 ml/min. Thus, in the presence of oligohydramnios, prematurity, and abnormal renal function, the medical team must plan appropriate follow-up for infants at health centers prepared to investigate and treat uropathies in newborns. Received: 24 August 1998 / Revised: 7 December 1998 / Accepted: 11 December 1998     

Breast Carcinoma in Childhood and Adolescence: Case Report and Review of the Literature

Secretory breast carcinoma is a rare tumor originally described in children and adolescent women with a characteristic morphology and a controversial choice of treatment. We report an additional case of a 4-year-old girl with a breast tumor diagnosed as a secretory carcinoma without involvement of the axillary lymph nodes. The therapy consisted of simple mastectomy and low axillary dissection. She presented with a local recurrence near the surgical scar 8 months later, and a wide elliptical excision of the scar and underlying tissue was performed with subsequent radiotherapy of the surgical bed. This tumor has a relatively benign behavior and rarely metastasizes. A literature review revealed only 22 cases of breast carcinoma in childhood and adolescence. ▪     

Bilateral macular hemorrhage after laser in situ keratomileusis

· Background: This is the first report of a bilateral submacular hemorrhage after LASIK surgery in an extreme myo pic patient. A 31-year-old man underwent bilateral surgery for correction of –16.75+0.75×70° and –16.50+0.50×55°. · Methods: Case report. · Results: One day after surgery the patient’s uncorrected visual acuity was in the 20/50 range OU and by 17 days after surgery his visual acuity had declined to 20/200 range. Fundus examination showed multifocal subretinal macular and posterior pole hemorrhages. Fluorescein angiography showed some macular lesions compatible with lacquer cracks. · Conclusions: Preoperative and postoperative fundus examination is important to detect this phenomenon. Patients should be informed of this rare complication. Received: 2 June 1998 Revised version received: 16 November 1998 Accepted: 17 November 1998