Cardiopulmonary anatomy and function in long-term survivors of mild to moderate congenital diaphragmatic hernia

Background/purpose

In the last decades, several studies regarding cardiopulmonary sequelae in survivors of congenital diaphragmatic hernia (CDH) have been published, but results often are conflicting, and controversies still exist. The aim of this study was to assess cardiopulmonary anatomic and functional outcome in a group of long-term survivors of CDH of mild to moderate degree.

Methods

Twenty-four children aged 8.15 ± 2.80 years underwent clinical examination with growth assessment, chest radiographs, echocardiography, pulmonary perfusion scintigraphy, static lung volumes measurement, and spirometry.

Results

Mean Z scores of weight for age and height for age were within normal values. Echocardiography showed normal anatomy and function in all but 3 patients with isolated CDH, in whom minor alterations were detected. Mean perfusion to the affected side was significantly lower (45.16 ± 5.30%; P < .0001) but still within normal range. Four children showed a substantial impairment of perfusion to the hernia side. The mean spirometric values and pulmonary volumes were normal. However, a mild restrictive pattern was evident in 6 children (27.3%), an obstructive pattern in 3 (13.6%), and a mixed obstructive and restrictive impairment in 1.

Conclusions

Hypoplastic lungs of mild to moderate CDH survivors continue to cause pulmonary morbidity in some children many years after the correction of the defect. In particular, lung perfusion appears to be impaired in 20% of the patients and pulmonary function in 45%, without any significant cardiac or developmental sequelae. The negative correlation between FEV₁ and duration of ventilation at presentation (r = −0.49; P = .026) may be caused by the consequences of lung hypoplasia, but initial ventilatory management may contribute to increased pulmonary morbidity. Relationship between perfusion and FEF_25-75 (r = 0.61; p = 0.004) could reflect an equivalent degree of reduction in the caliber of distal airways and pulmonary vascular tree.     

Selective decrease in urinary aquaporin 2 and increase in prostaglandin E2 excretion is associated with postobstructive polyuria in human congenital hydronephrosis

This study was undertaken to determine the role of aquaporin 2 (AQP2) in the impaired urinary concentrating capacity observed in patients who underwent pyeloplasty because of congenital unilateral hydronephrosis as a result of pyeloureteral junction disease. Twelve children (mean age, 8 +/- 2 mo) were examined in the study. From day 1 to day 5 after surgery, the urine was collected separately from pyelostomy draining only from the postobstructed kidney and from the bladder catheter draining mostly from the contralateral kidney used as internal control. After pyeloplasty, the postobstructed kidney was characterized by a reduced urinary excretion of AQP2 (approximately 54%) associated with polyuria that persisted from day 1 to day 5 (433 +/- 58 versus 310 +/- 74 ml/24 h at day 1; 326 +/- 44 versus 227 +/- 26 ml/24 h at day 5). In parallel, urine osmolality from the postobstructed kidney was significantly reduced compared with the contralateral kidney (111 +/- 12 versus 206 +/- 49 at day 1; 136 +/- 24 versus 235 +/- 65 mOsm/kg at day 5). Creatinine clearance from the postobstructed kidney was not significantly different compared with the contralateral kidney throughout the 4 d after surgery. However, on day 5, creatinine clearance from the postobstructed kidney became significantly lower. Prostaglandin E2 in the urine from postobstructed kidneys was found to be twofold higher than in the contralateral samples (26.0 +/- 6.7 versus 13.5 +/- 2.5 at day 5). It is concluded that (1) the selective downregulation of AQP2 in postobstructed kidney may account for the higher excretion of hypotonic urine, and (2) the local increase in prostaglandin E2 synthesis in postobstructed kidney may be involved in AQP2 downregulation and in maintaining a GFR similar to that of the contralateral kidney.     

Hyperfunctioning parathyroid tumours in patients with thyroid nodules. Sensitivity and positive predictive value of high-resolution ultrasonography and 99mTc-sestamibi scintigraphy

A series of 112 consecutive patients with primary hyperparathyroidism who underwent both high-resolution neck ultrasonography (US) and 99mTc-sestamibi/99mTc-pertechnetate subtraction scintigraphy (SS) prior to successful parathyroidectomy was reviewed. There were 29 (25.9%) men and 83 (74.1%) women, with a median age of 58 years (range 13-78 years). Patients were divided into two groups, according to the preoperative US findings: group A (87 patients, 77.7%) without thyroid diseases, and group B (25 patients, 22.3%) with either multinodular goitre or a solitary nontoxic thyroid nodule. In group B patients partial or total thyroidectomy was also performed, according to the intraoperative findings and frozen-section examination results. Final histopathology showed 99 (88.4%) solitary parathyroid (PT) adenomas and 3 (2.7%) PT carcinomas, while 10 (8.9%) patients had a multiglandular disease. The sensitivity and positive predictive value (PPV) were (group A vs group B) 79.8% vs 70.8% (P=0.25) and 95.7% vs 94.4% (P=0.58) for US, and 83.3% vs 87.0% (P=0.47) and 95.9% vs 90.9% (P=0.32) for SS respectively. Better but similar (P=not significant) results were obtained in patients with solitary PT tumours: 81.5% vs 77.8% (US) and 85.0 vs 94.1% (SS) sensitivity; 97.1% vs 93.3% (US) and 95.8% vs 88.9% (SS) PPV. Overall, the combination of US and SS was 92.9% sensitive (group A=93.1%, group B=92.0%; P=0.55), and the PPV reached 100% in each group. In conclusion, in patients with primary hyperparathyroidism the results of both US and SS are independent of coexistent thyroid disease, especially in patients with solitary PT tumours.     

Positive predictive value of 99mTc sestamibi scintimammography in patients with non-palpable,mammographically detected,suspicious, breast lesions

The purpose of this study was to analyse whether the use of Tc sestamibi scintimammography improves the positive predictive value of X-ray mammography. A series of 73 women (median age 51 years, range 35-79 years) with non-palpable, mammographically suspicious, breast lesions was reviewed. There were 41 (56.2%) pre-menopausal, and 32 (43.8%) post-menopausal women. All patients underwent sestamibi scintimammography prior to open breast biopsy. Definitive histology showed breast cancer (pT1a=1 (1.9%), pT1b=47 (90.4%), pT1c=4 (7.7%)) in 52 (71.2%) patients, and benign breast lesions in 21 (28.8%). Patients with cancer were significantly older (P <0.01), while the greatest dimension (size) of the excised lesion did not differ (8.47+/-1.51 vs 8.30+/-1.53 mm; P =0.66) between the two groups. Overall, the positive predictive values of mammography and sestamibi scintimammography were 71.2% and 95.7%, respectively (P =0.004). Patients with false positive mammography were significantly younger than those in whom cancer was diagnosed correctly (45.35+/-7.56 vs 53.96+/-10.60 years; P =0.001), while age did not affect the sensitivity of sestamibi scintimammography, which reached 100% in patients with breast lesions > or =8 mm in size. In this subgroup the positive predictive value of mammography, sestamibi scintimammography, and mammography+sestamibi scintimammography together were 63.4%, 95.1% (P =0.001), and 97.6%, respectively, and the majority of the patients with benign lesions (13 of 15 (86.7%)) could have avoided biopsy. It is concluded that the use of Tc sestamibi scintimammography in conjunction with mammography may potentially reduce unnecessary surgical procedures, and should be performed in all patients with mammographically suspicious breast lesions of 8 mm or greater in size.     

Noninvasive parathyroid imaging in primary hyperparathyroidism

Several studies suggested that preoperative localization of abnormal parathyroid (PT) glands may be useful in reducing operative time facilitating parathyroidectomy, especially in patients with ectopic PT glands. At present, noninvasive techniques used to evaluate patients with primary HPT include (1) 99mTc-sestamibi scintigraphy, (2) high-resolution neck ultrasonography, (3) CT scanning, and (4) magnetic resonance imaging (MRI). The sensitivity and positive predictive value of each technique range from 70% to 90%, and a combination of two of more tests may significantly improve the results. In the minimally-invasive era both radioguided and video-assisted parathyroidectomy require an accurate preoperative localization of the abnormal PT glands, and PT imaging should be obtained before surgery in all patients with primary hyperparathyroidism, with the aim of reducing operative time and hospital stay.     

Effect of hyperprolactinemia and age on the hypogonadism of uremic men on hemodialysis

Primary hypogonadism has been commonly reported among uremic men on hemodialysis, characterized by low testosterone levels, increased luteinizing hormone and sometimes follicle-stimulating hormone levels. Little is known about the influence of hyperprolactinemia and age on this hypogonadism. In 149 hemodialysis patients and in 60 healthy subjects the serum levels of testosterone (T), gonadotropins (LH and FSH) and prolactin (PRL) were assessed through radioimmunoassay. Mean +/- SD hormone levels were: T 274 +/- 125 ng/100 ml, lower than controls; LH 44.7 +/- 46.1 mlU/ml and FSH 17.6 +/- 18.4 mIU/ml, both higher than controls. PRL 31.3 +/- 49.4 ng/ml, higher than controls. A positive correlation between LH and FSH, a negative correlation between PRL and both T and LH was found. Moreover T and FSH were correlated with age only in the normoprolactinemic patients. These data suggest: a common damaging mechanism by uremia on both interstitial and tubular structures of the testis; a central antigonadal influence of hyperprolactinemia even if a direct action on the testis cannot be excluded; a worsening action of age on the gonadal function of these patients.     

Scintigraphic patterns of adrenocortical carcinoma: morpho-functional correlates.

OBJECTIVE: Adrenocortical scintigraphy has demonstrated clinical utility in the morpho-functional characterization of adrenal tumors. The aim of this study was to identify possible relationships between the scintigraphic pattern and endocrine and/or morphological data in a series of adrenocortical carcinomas. DESIGN AND METHODS: Twenty-one patients with adrenocortical carcinoma (11 nonfunctioning and 10 hormone-secreting) were investigated with 75Se-methyl-nor-cholesterol scintigraphy. Clinical, hormonal, radiological, and pathological data were analyzed. RESULTS: The adrenal mass showed no radiocholesterol uptake in 18 cases (11 nonfunctioning and 7 functioning lesions). Contralateral normal adrenal gland was visualized in all patients with nonfunctioning tumors, whereas classic bilateral nonvisualization was observed in the 7 cases with hyperfunctioning masses. Three patients with cortisol-producing carcinomas showed radiotracer uptake by the mass, without visualization of the contralateral gland. At histology, the tumors were shown to be undifferentiated adrenocortical carcinomas; they had an aggressive clinical behavior. CONCLUSIONS: Radiocholesterol scintigraphy has an important role in diagnosing adrenocortical carcinomas, which typically are not visualized. However, 30% of hypersecreting adrenocortical carcinomas show an atypical increased tracer uptake, not predictive of the biochemical and histological features of the tumor.     

The role of FDG PET/CT or PET/MRI in assessing response to neoadjuvant therapy for patients with borderline or resectable pancreatic cancer: a systematic literature review

Annals of Nuclear Medicine - The aim of the present systematic review is to examine the role of fluorodeoxyglucose (FDG) positron emission tomography (PET) associated with computed tomography (CT)...