Pregnancy outcomes in women with classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency

OBJECTIVE: Despite earlier detection, treatment, and surgical advances, fertility prognosis in women with classical 21-hydroxylase deficiency (21-OHD) is still low, especially in the salt-wasting (SW) form. PATIENTS AND METHODS: We analysed the course and outcome of four pregnancies in two simple virilizing (SV) and one SW patient. RESULTS: The evaluation of carrier status indicated that all three fathers had two normal CYP21 genes. During the pregnancy, the dose of prednisolone was increased in one of the SV patients and the SW patient. In the SW patient who developed pre-eclampsia, the dose of fludrocortisone was also increased. Three patients gave birth to a total of four healthy girls who were heterozygotes for 21-OHD with normal genitalia (one by vaginal delivery and three by Caesarean section). Family studies revealed that the mother of the SW patient has nonclassical 21-OHD. CONCLUSION: Improving a low birth rate in females with SW 21-OHD remains a problem and new approaches are required. If the mother has 21-OHD (even nonclassical 21-OHD), pre-conception counselling and paternal genotyping are advisable and prenatal dexamethasone therapy should be considered.     

Upper extremity arterial injuries: Factors influencing treatment outcome

Objective

The aim of the study was to identify factors influencing surgical treatment outcome following upper extremity arterial injuries.

Methods

This 15-year study (January 1992 to December 2006) included 167 patients with 189 civilian, iatrogenic or military upper extremity arterial injuries requiring surgical intervention. Patient data were prospectively entered into a vascular trauma database and retrospectively analysed.

Results

The most frequently damaged vessel was the brachial artery (55% of injuries), followed by the axillary (21.7%), antebrachial (21.2%) and subclavian (2.1%) arteries. Three primary amputations (1.8%) were performed because of extensive soft-tissue destruction and signs of irreversible ischaemia on admission. Seven secondary amputations (4.2%) were due to graft failure, infection, anastomotic disruption or the extent of soft-tissue and nerve damage. Fasciotomy was required in 9.6% of cases. Operative mortality was 2.4% (four deaths). Early graft failure, compartment syndrome, associated skeletal and brachial plexus damage and a military mechanism of injury were found to be significant risk factors for limb loss (p < 0.01).

Conclusion

Although careful physical examination should diagnose the majority of upper extremity arterial injuries, angiography is helpful in detailing their site and extent. Prompt reconstruction is essential for optimal results. Nerve trauma is the primary cause of long-term functional disability.     

Correction: Compressive strength evaluation of thin occlusal veneers from different CAD/CAM materials,before and after acidic saliva exposure

In the present study are depicted valuable observations for practitioners, obtained from an in vitro study which aims to evaluate the compressive strength of occlusal veneers fabricated from 3 type of restorative materials, before and after 1 month of acidic artificial saliva exposure (pH = 2.939). In this context, 90 extracted human molars were prepared to receive computer-aided design/computer-aided manufacturing (CAD/CAM) occlusal veneers. The restorative materials considered in this study were: Cerasmart; Straumann Nice and Tetric CAD. The occlusal veneers were designed, milled and cemented with an adhesive dual-cure resin cement. From all the extracted human molars, only sixty specimens were immersed in acidic artificial saliva, for 1 month, at 37 °C ± 1 °C and part of this specimens were also thermo-cycled, between 5 and 55 °C ± 2 °C, before compressive strength test. The results showed a lower compressive strength for both the samples exposed to acidic artificial saliva as well as for the samples exposed to acidic artificial saliva and thermo-cycled. Scanning electron microscopy (SEM) showed that after compressive strength, all the specimens non-exposed to acidic artificial saliva, present extensive cracks formation at the surface of the restorations, and after exposure to acidic artificial saliva for 1 month, the surface damage was characterized by longitudinal and profound fractures of the restoration, as well as the fracture of the tooth structure. Between CAD/CAM materials tested, nanoceramic resin shows more favorable fracture patterns, both before and after acidic artificial saliva exposure.

     

Determination of glycated hemoglobin in patients with advanced liver disease

AIM: To evaluate the glycated hemoglobin (HbA(1c)) determination methods and to determine fructosamine in patients with chronic hepatitis, compensated cirrhosis and in patients with chronic hepatitis treated with ribavirin. METHODS: HbA(1c) values were determined in 15 patients with compensated liver cirrhosis and in 20 patients with chronic hepatitis using the ion-exchange high performance liquid chromatography and the immunoassay methods. Fructosamine was determined using nitroblue tetrazolium. RESULTS: Forty percent of patients with liver cirrhosis had HbA(1c) results below the non-diabetic reference range by at least one HbA(1c) method, while fructosamine results were either within the reference range or elevated. Twenty percent of patients with chronic hepatitis (hepatic fibrosis) had HbA(1c) results below the non-diabetic reference range by at least one HbA(1c) method. In patients with chronic hepatitis treated with ribavirin, 50% of HbA(1c) results were below the non-diabetic reference using at least one of the HbA(1c) methods. CONCLUSION: Only evaluated in context with all liver function parameters as well as a red blood count including reticulocytes, HbA(1c) results should be used in patients with advanced liver disease. HbA(1c) and fructosamine measurements should be used with caution when evaluating long-term glucose control in patients with hepatic cirrhosis or in patients with chronic hepatitis and ribavirin treatment.     

Is There any Benefit from Expanding the Criteria for the Resection of Hepatocellular Carcinoma in Cirrhotic Liver? Experience from a Developing Country

Background

Patients with large-size (>10?cm) hepatocellular carcinoma (HCC) in Child B cirrhosis are usually excluded from curative treatment, i.e., hepatic resection, because of marginal liver function and poor outcome. This study was designed to evaluate the feasibility of the radiofrequency (RF)-assisted sequential “coagulate-cut liver resection technique” in expanding the criteria for resection of large HCC in cirrhotic livers with impaired liver function.

Methods

Forty patients with Child-Pugh A or B cirrhosis underwent liver resection from December 1, 2001 to December 31, 2008. Of these, 20 patients (13 Child-Pugh A and 7 Child-Pugh B) with advanced stage HCC (stage B and C according to Barcelona-Clinic Liver Cancer Group) underwent major liver resection. The two groups were comparable in terms of patient age, liver cirrhosis etiology, tumor number, and size.

Results

All resections were performed without the Pringle maneuver. There was no significant difference found between the two groups regarding resection time, perioperative transfusion, postoperative complications, hospital stay, and day 7 values of hemoglobin and liver enzymes. Likewise, there was no significant difference found in the overall survival between Child A and Child B patients who underwent major liver resection

Conclusions

RF-assisted sequentional “coagulate-cut liver resection technique“ may be a viable alternative for management of patients with advanced HCC in cirrhotic liver with impaired function.