Clinical Classification, Screening, and Diagnosis in Beta-Thalassemia and Hemoglobin E/Beta-Thalassemia.

This article reviews the classification of beta-thalassemia syndromes, correlating clinical severity and genotype in the earlier classification, and broadening it recently based on clinical severity and transfusion status. The classification is dynamic, and individuals may progress from transfusion-independent to transfusion-dependent. Early and accurate diagnosis prevents delays in instituting treatment and comprehensive care, and precludes inappropriate and potentially harmful interventions. Screening can inform risk in an individual and subsequent generations when partners may be carriers as well. This article discusses the rationale for screening of the at-risk population. In the developed world, a more precise genetic diagnosis must be considered.

Inpatient Complications After Transsphenoidal Surgery in Cushing's Versus Non-Cushing's Disease Patients.

OBJECTIVE: Transsphenoidal surgery (TSS) harbors a potential for hypopituitarism, cerebrospinal fluid (CSF) leaks, and other complications. We utilized the Nationwide Inpatient Sample Database (NIS) to compare inpatient complication rates between Cushing's disease (CD) and non-Cushing's disease (NCD) patients undergoing TSS. METHODS: Inpatient hospitalization data for 960 CD and 12 110 NCD patients who underwent TSS between 2002 and 2010 were accessed. Demographic information, outcomes, and complication rates were evaluated. RESULTS: Patients with CD had a female predilection (81.7%) and were younger (40.5 ± 14.4 years) than NCD patients (47.8% female; 52.1 ± 16.3 years) (P < .001). Length of stay and total charges did not differ between groups. Patients with CD had significantly greater postoperative diabetes insipidus rates (14.0% vs 9.6%, P < .001) and urinary/renal complications (1.7% vs 0.9%, P = .027). After adjusting for possible confounders, the relationship between urinary/renal complications and CD status strengthened. There was no difference in rates of CSF leak and iatrogenic pituitary disorders overall. CONCLUSION: No differences were noted in the rate of early CSF leaks between postoperative TSS CD and NCD patients. Postoperative diabetes insipidus did not significantly differ between groups after adjusting for confounders. Only odds of urinary/renal complications in CD patients was significant after adjustment.

Transsphenoidal surgery for malignant pituitary lesions: an analysis of inpatient complications.

BACKGROUND: Fewer than 4% of pituitary tumors are malignant lesions. These tumors predominantly represent metastatic disease from elsewhere. This study evaluates inpatient complications, demographics, and hospitalization characteristics of patients who underwent transsphenoidal surgery (TSS) for malignant pituitary lesions. METHODS: The Nationwide Inpatient Sample was evaluated for TSS patients from 1998 to 2010. Demographics, hospitalization characteristics, and complications were evaluated among patients with malignant lesions and compared to those with benign tumors. RESULTS: There were 17,425 inpatient records, 1.0% of which involved malignant pituitary tumors. There was no difference in age between these cohorts (p = 0.378). Patients with malignant tumors had greater length of stay (6.7 days vs 4.5 days, p = 0.003) and higher trending charges ($55,371 vs $40,550 p = 0.091). The most common postoperative complications among patients with malignant lesions included diabetes insipidus (DI) (17.9%), fluid/electrolyte abnormalities (14.0%), neurological complications (5.6%), cerebrospinal fluid (CSF) rhinorrhea (2.2%), and iatrogenic pituitary disorders (2.2%). Patients with malignant lesions had a significantly greater rates of postoperative DI and fluid/electrolyte abnormalities (odds ratio = 2.0 and 1.7, respectively), whereas no statistical difference was noted in the rates of CSF rhinorrhea (p = 0.372). CONCLUSION: In this analysis of inpatient hospitalizations for TSS patients, malignant pituitary disease was associated with a greater rate of postoperative DI and fluid/electrolyte abnormalities, but no differences in the rates of postoperative CSF rhinorrhea and other complications were found. Patients with malignant pituitary lesions undergoing TSS had significantly longer hospitalizations and higher trending charges than those with benign lesions. This analysis is, however, subject to the limitations of the database.

Characterization of transsphenoidal complications in patients with acromegaly: an analysis of inpatient data in the United States from 2002 to 2010.

BACKGROUND: Transsphenoidal surgery (TSS) is a common procedure for a variety of pituitary lesions. This procedure can be associated with complications related to the surgery or specific pathology. In this study, we evaluate inpatient postoperative complications among patients who underwent TSS for growth hormone adenomas using a nationally representative database, and compare patient characteristics and complications to patients who underwent TSS for other benign pituitary neoplasms. METHODS: Analysis of the Nationwide Inpatient Sample revealed 13,070 TSS patients (including 892 with acromegaly) between 2002 and 2010. Complication rates, outcomes, patient demographics, hospital stay, and total charges were evaluated among TSS patients with and without acromegaly. RESULTS: There was an increase in TSS performed in both cohorts from 2002 to 2010. Acromegaly patients were younger, had shorter hospital stays, and incurred fewer charges. Acromegaly patients had a lower occurrence of postoperative urinary/renal complications (0.2% vs 1.1%), thromboembolic events (0% vs 0.4%), fluid/electrolyte abnormalities (5.7% vs 9.1%), and iatrogenic hypopituitarism (0.3% vs 1.1%) compared to other TSS patients (all p < 0.05). After adjusting for age, acromegalic patients maintained a statistically lower occurrence of fluid/electrolyte abnormalities (p = 0.007). Cerebrospinal fluid leak occurrence in acromegaly patients was 2.6% vs 1.7% in non-acromegaly patients, a result that did not reach significance (p = 0.054). CONCLUSION: Upon comparison of inpatient hospitalizations for patients undergoing TSS for growth hormone adenomas and other benign pituitary neoplasms, acromegaly patients had a significantly lower occurrence of postoperative fluid/electrolyte abnormalities. Acromegaly patients had shorter hospitalizations and subsequently fewer total charges.

Impact and cost of care of venous thromboembolism following pituitary surgery.

OBJECTIVES/HYPOTHESIS: In 2008, the Centers for Medicare and Medicaid Services discontinued reimbursement for postoperative venous thromboembolism (VTE) events such as deep venous thrombosis and pulmonary embolism, citing them as preventable postoperative complications. We examined the impact of postoperative VTE on patients undergoing pituitary surgery. METHODS: The Nationwide Inpatient Sample (NIS) was evaluated for patients undergoing pituitary resection from 2002 to 2010. Patient demographics, comorbidities, length of stay, hospital charges, and postoperative complications were analyzed. RESULTS: Eighty-seven patients who underwent pituitary surgery developed a VTE. Patients who underwent pituitary surgery that developed VTE were older (55.9 ± 15.2 years) than those who did not develop VTE (50.1 ± 17.2 years) (P = 0.002). VTE occurred at a significantly higher rate in patients with coagulopathy, peripheral vascular disorder, and weight loss (P < 0.05). VTE was associated with increased rates of postoperative neurological, pulmonary, cardiac, urinary, renal, hemorrhage, fluid and electrolytes, diabetes insipidus, and cerebrospinal fluid rhinorrhea complications (P < 0.01)-as well as increased mortality rate (P < 0.001), length of stay (P < 0.001), and cost of care (P < 0.001). CONCLUSIONS: Analysis of the data from the NIS database showed that risk factors for the development of VTE following pituitary surgery include older age, preexisting coagulopathy, peripheral vascular disorder, and weight loss. Patients who developed postoperative VTE had a longer length of hospital stay, higher hospital charges, and increased morbidity and mortality.

Transsphenoidal surgery and diabetes mellitus: An analysis of inpatient data and complications.

OBJECTIVES/HYPOTHESIS: Transsphenoidal surgery (TSS) has emerged as the standard approach for pituitary resection due to its minimally invasive nature. There has been little analysis examining the impact of diabetes mellitus (DM) on patients undergoing TSS. In this study, we characterize DM's association with postoperative TSS complications. In addition to analysis of associated charges and patient demographics, we performed comparison of complication rates between DM and non-DM patients who have undergone TSS in recent years. METHODS: The Nationwide Inpatient Sample, a database encompassing nearly 8 million inpatient hospitalizations, was evaluated for patients undergoing TSS from 2002 to 2010. RESULTS: Of 12,938 TSS patients, 2,173 (16.8%) had a DM diagnosis. The non-DM cohort was younger (50.1 y ± 16.6SD vs. 56.8 y ± 14.1; P < 0.001) and had shorter hospitalizations and lesser charges. DM patients had a greater incidence of pulmonary, cardiac, urinary/renal, and fluid/electrolyte complications, and had a lesser incidence of diabetes insipidus (P < 0.05). Upon controlling for age, the greater incidence of pulmonary and fluid/electrolyte complications was present only among patients < 60 years of age. Higher occurrence of cerebrospinal fluid rhinorrhea was noted among black diabetics when compared to non-DM blacks. CONCLUSIONS: DM is associated with greater length of stay and hospital charges among TSS patients. DM patients undergoing TSS have a significantly greater incidence of pulmonary and fluid/electrolyte complications among patients under the age of 60, and greater risk for urinary/renal complications across all ages. Despite a theoretical concern due to an impaired wound-healing in DM patients, association with cerebrospinal fluid rhinorrhea was only noted among black diabetics. LEVEL OF EVIDENCE: 2C.