Thyroid function, sensitivity to thyroid hormones, and metabolic syndrome in euthyroid children and adolescents with Down syndrome.

PURPOSE: Patients with Down Syndrome (DS) showed multiple comorbidities, including thyroid disorders, obesity, and metabolic derangement. Different thyroid hormone (THs) patterns and sensitivity to thyroid hormone indices (STHI) seem to be associated with metabolic disorders. The study's aim was to evaluate the prevalence of metabolic syndrome (MS) in pediatric patients affected by DS, taking into consideration the relationship between the metabolic parameters, THs and STHI. METHODS: We enlisted 50 euthyroid patients with DS (9.03 ± 4.46). Clinical parameters, TSH, FT3, FT4 and the presence of MS were recorded. Indexes of peripheral sensitivity (FT3/FT4 ratio) and central sensitivity (TSH index, TSHI; TSH T4 resistance index, TT4RI; TSH T3 resistance index, TT3RI) were also detected. Thirty healthy subjects were included as a control group. RESULTS: MS was detected in 12% of the subjects with DS. FT3, FT4, and TSH levels were higher in DS than in the control group (p < 0.01); higher levels of FT3/FT4 ratio, TSHI and TT3RI and lower TT4RI values (p < 0.01) were also detected. A significant correlation was detected between FT3 and fasting blood glucose (FBG) (R = 0.46), triglyceride (TG) (r = 0.37), total (r = 0.55) and high density lipoprotein-cholesterol (HDL-C) (r = - 0.38), diastolic blood pressure (DBP) (r = - 0.4); FT3/FT4 ratio and waist circumference (WC) (r = 0.36); TSHI and total (r = 0.30) and HDL cholesterol (r = - 0.31); TT4RI and HDL cholesterol (r = - 0.31); TT3RI and total (r = 0.39) and HDL cholesterol (r = - 032). CONCLUSION: We confirmed a higher MS prevalence in children with DS compared to the control group. A significant association between THs, STHI, and the glucose and lipid metabolism parameters was detected supporting their role in metabolic alterations related to the DS.

The effect of losing federal coverage through the Affordable Care Act on ear tube placements at an urban children's hospital.

PURPOSE: 15-31% of the population in a large Mid-western city is between 100 and 400% of the Federal Poverty Level, thus qualifying for health care coverage under the Affordable Care Act (ACA). Coverage for their children would potentially be available under Children's Health Insurance Program (CHIP) or Medicaid programs. Loss of funding for these programs could be devastating for this community. METHODS: We retrospectively reviewed 1162 charts of pediatric patients with tympanostomy tube (TT) placement pre-ACA from November 2012 to December 2013 and 1606 charts post-ACA from January 2014 to July 2015. We filtered demographics by health insurance (Medicaid/CHIP/Other), residential zip codes, identified race/ethnicity within those zip codes as well as gender and age of patients getting TT during these periods. RESULTS: Bivariate analysis of these demographics between the two periods showed statistical significance (p = 0.0098) between White Hispanic/Latino children receiving ear tubes (pre-ACA = 3.8%, post-ACA = 6.4%). However, there was no statistical significance for insurance enrollment (Medicaid or non-Medicaid) and other races (White-not Hispanic/Latino (nHL), African American, Other/Unknown/Refused) with respect to TT placement. Using pre-ACA period and White nHL females as arbitrary reference, a multivariate logistic regression showed that patients requiring TT surgery were equally likely to be covered on Medicaid either before or after ACA. CONCLUSION: We demonstrated that the pre and post ACA Medicaid coverage for TT surgery did not change. Underserved children did not obtain other forms of insurance during this time. This demonstrates a potentially catastrophic loss of coverage for children should Medicaid/CHIP benefits be lost to sole coverage under the ACA.

Assessing Photoreceptor Structure in Retinitis Pigmentosa and Usher Syndrome.

PURPOSE: The purpose of this study was to examine cone photoreceptor structure in retinitis pigmentosa (RP) and Usher syndrome using confocal and nonconfocal split-detector adaptive optics scanning light ophthalmoscopy (AOSLO). METHODS: Nineteen subjects (11 RP, 8 Usher syndrome) underwent ophthalmic and genetic testing, spectral-domain optical coherence tomography (SD-OCT), and AOSLO imaging. Split-detector images obtained in 11 subjects (7 RP, 4 Usher syndrome) were used to assess remnant cone structure in areas of altered cone reflectivity on confocal AOSLO. RESULTS: Despite normal interdigitation zone and ellipsoid zone appearance on OCT, foveal and parafoveal cone densities derived from confocal AOSLO images were significantly lower in Usher syndrome compared with RP. This was due in large part to an increased prevalence of non-waveguiding cones in the Usher syndrome retina. Although significantly correlated to best-corrected visual acuity and foveal sensitivity, cone density can decrease by nearly 38% before visual acuity becomes abnormal. Aberrantly waveguiding cones were noted within the transition zone of all eyes and corresponded to intact inner segment structures. These remnant cones decreased in density and increased in diameter across the transition zone and disappeared with external limiting membrane collapse. CONCLUSIONS: Foveal cone density can be decreased in RP and Usher syndrome before visible changes on OCT or a decline in visual function. Thus, AOSLO imaging may allow more sensitive monitoring of disease than current methods. However, confocal AOSLO is limited by dependence on cone waveguiding, whereas split-detector AOSLO offers unambiguous and quantifiable visualization of remnant cone inner segment structure. Confocal and split-detector thus offer complementary insights into retinal pathology.

Psoas abscess: a rare complication of Crohn's disease.

UNLABELLED: Psoas abscess is a rare complication of Crohn's disease. METHODS AND MATERIALS: We evaluated the incidence of psoas abscess on 312 patients with Crohn's disease, seen at our institution between 1992-2001. RESULTS: We encountered three cases of psoas abscess (0.9%). One patient was managed with ileocolic resection and immediate anastomosis, while in two patients a percutaneous drainage was first performed and then, after 12 days of total parenteral nutrition, a resection of the diseased bowel with immediate reconstruction was carried out. CONCLUSIONS: A correction of the nutritional deficiencies is mandatory. Percutaneous computed-tomography guided drainage of the abscess with intestinal resection with immediate anastomosis, performed after a parenteral hyperalimentation, should be the method of choice in the management of such patients.

Severe gastrointestinal bleeding in Crohn's disease.

INTRODUCTION: Acute gastrointestinal bleeding is rare in Crohn's disease. METHODS: We characterized the clinical features and course of such hemorrhage in patients seen at our institution from 1992 to 2000. RESULTS: Five patients had gastrointestinal bleeding during Crohn's disease. All patients had a known Crohn's disease, with a mean duration of the disease of 6 years. The source of bleeding was identified in four patients (80%). Endoscopy was, in all patients, the first diagnostic procedure. An Hartmann total colectomy with closure of the rectal stump and ileostomy was performed in three patients, while two patients with ileal massive bleeding were treated conservatively. One patient had a recurrence of bleeding from the small bowel one week later but he didn't required surgical treatment. One patient with pancolic Crohn's disease died on 10th postoperative day because of multiorgan failure and septic complications. CONCLUSIONS: Gastrointestinal bleeding is rare in Crohn's disease, with a predilection for site of involvement. The preoperative diagnosis of the site of bleeding is not easy, and enteroscopy should be mandatory in such patients. Surgery is required for half of cases and recurrent haemorrhage should be an appropriate indication for surgery.

[Is it possible to improve survival in patients with Klatskin tumors?]. / E possibile migliorare la sopravvivenza dei pazienti con tumore di Klatskin?

BACKGROUND: This retrospective study was undertaken to evaluate if a high resectability rate could improve the long-term outcome of patients with proximal bile duct cancer. METHODS: Between 1985 and 2001, 50 patients (34 males and 16 females) with proximal bile duct cancer were treated. The tumor site were classified according to Bismuth-Corlette's classification: 9 lesions (18%) were Bismuth type I, 23 lesions (46%) type II, 12 lesions (24%) Type IIIa, 5 lesions (10%) type IIIb and only 1 resected tumor (2%) was type IV. Thirty-six patients (72%) were considered suitable for surgery, while 14 underwent non surgical palliative procedures. Twenty patients had bile duct resection only. Ten patients had Roux-enY cholangiojejunostomy with two or three divided segmental hepatic ducts; in ten, the cholangiojejunostomy was performed with 4 or 5 divided segmental hepatic ducts. Thirteen patients had bile duct resection plus hepatectomy. Despite the curative intention of the operation, only in 19 (52.7%) patients did the histopathological examination reveal tumor-free margins. RESULTS: There was no operative mortality. Overall 1-year, 3-year and 5-year survival of the entire surgical group was 61%, 22.5% and 9% respectively. In the 19 patients treated with curative intent the survival at 1,3, and 5 years was 66%, 30% and 15%, respectively, while in the palliative group it was 45%, 15% and 0%, respectively. CONCLUSION: Only margins free from tumor may guarantee an improvement in long-term outcome. Increasing resectability improves survival and could offer a chance of better 5-year survival.

Blunt liver injury: from non-operative management to liver transplantation.

Therapeutic options for blunt hepatic trauma include both non-operative and operative management. We have reviewed our experience of the management of blunt hepatic trauma, from non-operative to liver transplantation. A total of 72 patients with blunt hepatic injury observed at the first surgical unit of Padua in a 3-year period (1998-2000) were analysed; we also included a patient who had a liver transplant in 1993 for severe liver trauma. Twenty-nine patients (39.7%) were treated conservatively, with a 93% success rate; 60.3% were treated surgically. Suture hepatorraphy was the most common procedure performed (52.3%). Advantages of non-operative management in our experience were the reduced need for transfusion (1.1U versus 4.3U) with 92% of patients not needing transfusion, and a reduced stay in the intensive care unit; there was no liver-related mortality. The overall morbidity in surgical patients was 30%, with 16% liver-related complications. Twelve surgical patients (27.2%) died, with a liver-related mortality of 18.2%. A large number of patients may present with an associated endo-abdominal injury, even in low-grade liver trauma, requiring rapid laparotomy. In high-grade hepatic trauma, the evolution toward liver failure is an indication for liver transplantation.

Laparoscopic treatment of simple hepatic cysts and polycystic liver disease.

BACKGROUND: The authors present their experience in the laparoscopic management of hepatic cysts and polycystic liver disease (PLD). METHODS: Between January 1996 and January 2002, 16 patients underwent laparoscopic liver surgery. Indications were solitary giant cysts (n = 10) and PLD (n = 6). Data were collected retrospectively. RESULTS: Laparoscopic fenestration was completed in 15 patients. Median operative time was 80 min. There was no deaths. Complications occurred in four patients: one patient with a solitary liver cyst experienced diarrhea, while a pleural effusion, a bleeding from the trocar-insertion site, and ascites occurred in three patients with PLD. Median follow-up was 34 months. There was one asymptomatic recurrence (11%) in one patient with a solitary cyst. Two patients with PLD had a symptomatic recurrence of a liver cyst. CONCLUSION: Laparoscopic fenestration could be the preferred treatment of solitary liver cysts and PLD. Adequate selection of patients and type of cystic liver together with a meticulous surgical technique are recommended.

Risk stratification using the Society of Thoracic Surgeons Program.

In an era of progressive cost containment and public scrutiny, the wisdom of aggressive surgical therapy for high-risk candidates has been questioned. At our center in the previous 24 months, 728 patients with coronary artery disease were entered into The Society of Thoracic Surgeons national database, and the hospital outcomes plus length of stay were analyzed. Patients were separated according to the predicted mortality based on the groupings in The Society of Thoracic Surgeons database: 0 to 5% (453 patients); 5% to 10% (126 patients); 10% to 20% (96 patients); 20% to 30% (17 patients); and 30% and greater (36 patients). There was a close correlation with the predicted rates of mortality. Importantly, the preoperative risk stratification demonstrated a strong correlation with the significant morbidity and excessive length of stay in the highest-risk groups (predicted risk of 20% to > or = 30%). The incidences of the most common complications in the group with the highest predicted risk (> or = 30%) were 28%, renal failure; 33%, ventilator dependence; and 17%, cardiac arrest. In addition, at short-term follow-up (6 to 8 months), a 24.3% mortality was identified in patients with a predicted mortality that exceeded 20%. These data quantify the risks and morbidities associated with the care of seriously ill patients with coronary artery disease and demonstrate the need for professional and public discussions focusing on the association of a high preoperative risk status and the consumption of resources.