Connective tissue nevus misdiagnosed as juvenile localized scleroderma.

BACKGROUND: Connective tissue nevi (CTN) are congenital hamartomas caused by excessive proliferation of dermis components. In children, CTN can mimic juvenile localized scleroderma (JLS), an immune mediated skin disorder that requires aggressive immunosuppression. OBJECTIVES: Aim of our study was to describe a series of pediatric patients with CTN misdiagnosed as JLS and the discerning characteristics between the two conditions. METHODS: Retrospective analysis of children referred to our Center during the last two decades for JLS who received a final diagnosis of CTN. Clinical, laboratory, histopathological and instrumental data (MRI and thermography) were collected and compared with those with JLS. RESULTS: Seventeen patients with mean age at onset 4.6 years entered the study. All came to our Center with a certain diagnosis of JLS (n = 15) or suspected JLS (n = 2). The indurated skin lesions were flat and resembled either circumscribed morphea or pansclerotic morphea. In 14 patients (82.4%) they were mainly localized at the lower limbs and in three (17.6%) at the upper limbs. No patient had laboratory inflammatory changes or positive autoantibodies. Skin biopsies confirmed the diagnosis of CTN: non-familial collagenoma in eleven (64.7%), mixed CTN in four (23.5%) and familial CTN in two (11.8%). Mean age at final diagnosis was 9.5 years, with a mean diagnostic delay of 4.8 years (range 1-15 years). Sixteen patients underwent musculoskeletal MRI that was normal in all except two who showed muscle perifascial enhancement. Thermography was normal in all patients. At our first evaluation, eleven patients (64.7%) were on systemic treatment (methotrexate 11, corticosteroids 7, biologics 2), three (17.6%) on topical corticosteroids and three untreated. CONCLUSIONS: CTN can be misdiagnosed as JLS and therefore aggressively treated with prolonged and inappropriate immunosuppression. The absence of inflammatory appearance of the skin lesions, normal instrumental and laboratory findings and the accurate evaluation of skin biopsy are crucial to address the right diagnosis.

Functional outcomes after transanal total mesorectal excision (TaTME): a random forest analysis to predict patients' outcomes.

PURPOSE: Anorectal, sexual, and urinary dysfunction are common issues after rectal cancer surgery, although seldom explored. The primary aim of this study was to investigate postoperative anorectal functional results. METHODS: Patients with mid/low-rectal cancer treated with transanal TME (TaTME) with primary anastomosis with/without diverting stoma between 2015 and 2020 were reviewed and selected if they had a minimum follow-up of 6 months (from the primary procedure or stoma reversal). Patients were interviewed using validated questionnaires and the primary outcome was bowel function based on Low Anterior Resection Syndrome (LARS) scores. Statistical analyses were performed to identify clinical/operative variables correlated with worse outcomes. A random forest (RF) algorithm was computed to classify patients at a greater risk of minor/major LARS. RESULTS: Ninety-seven patients were selected out of 154 TaTME performed. Overall, 88.7% of the patients had a protective stoma and 25.8% reported major LARS at mean follow-up of 19.0 months. Statistical analyses documented that age, operative time, and interval to stoma reversal correlated with LARS outcomes. The RF analysis disclosed worse LARS symptoms in patients with longer operative time (> 295 min) and stoma reversal interval (> 5.6 months). If the interval ranged between 3 and 5.6 months, older patients (> 65 years) reported worse outcomes. Finally, no statistical difference was documented when comparing the rate of minor/major LARS in the first 27 cases versus others. CONCLUSION: One-quarter of the patients developed major LARS after TaTME. An algorithm based on clinical/operative variables, such as age, operative time, and time to stoma reversal, was developed to identify categories at risk for LARS symptoms.

Duodenal perforation in a SARS-CoV-2-positive patient with negative PCR results for SARS-CoV-2 in the peritoneal fluid.

OBJECTIVE: The coronavirus disease 2019 (COVID-19) pandemic has significantly affected health care organizations globally. Many aspects of this disease, as well as the risks for patients treated with multiple drug regimens to control severe COVID-19, are unclear. During emergency surgery for SARS-CoV-2-positive patients, the risk of SARS-CoV-2 exposure and transmission to the surgical staff has yet to be determined. PATIENTS AND METHODS: In this report, we describe a SARS-CoV-2-positive patient with severe respiratory syndrome treated with multiple doses of IL-6 inhibitors who presented with a perforated duodenal ulcer and underwent emergency surgery. During and after surgery, we tested for SARS-CoV-2 at the ulcer site and in the peritoneal fluid. RESULTS: The history of the patient allows for two possible interpretations of the pathogenesis of the duodenal ulcer, which could have been a stress ulcer, or a gastrointestinal ulcer associated to the use of IL-6 inhibitors. We also noticed that the ulcer site and peritoneal fluid repeatedly tested negative for SARS-CoV-2. Therefore, we reviewed the pertinent literature on gastrointestinal bleeding in patients with COVID-19 and on SARS-CoV-2 detection in the peritoneal fluid of surgical patients and discussed possible prevention strategies for bleeding and the actual risk of infection for the surgical staff. CONCLUSIONS: The first implication of this case is that the relation between repeated administration of IL-6 inhibitors and upper gastrointestinal bleeding and perforation must be investigated, and that the threshold for administering prophylactic proton pump inhibitors therapy should be carefully considered for patients with severe COVID-19. The second implication is that further testing should be performed on the peritoneal fluid of COVID-19 patients undergoing emergency surgical procedures to clarify the discordant results for the presence of SARS-CoV-2 in the peritoneal cavity and the possible risk of transmission to the surgical staff.

Efficacy of pirfenidone for idiopathic pulmonary fibrosis: An Italian real life study.

BACKGROUND: In this retrospective Italian study, which involved all major national interstitial lung diseases centers, we evaluated the effect of pirfenidone on disease progression in patients with IPF. METHODS: We retrospectively studied 128 patients diagnosed with mild, moderate or severe IPF, and the decline in lung function monitored during the one-year treatment with pirfenidone was compared with the decline measured during the one-year pre-treatment period. RESULTS: At baseline (first pirfenidone prescription), the mean percentage forced vital capacity (FVC) was 75% (35-143%) of predicted, and the mean percentage diffuse lung capacity (DLCO) was 47% (17-120%) of predicted. Forty-eight patients (37.5%) had mild disease (GAP index stage I), 64 patients (50%) had moderate IPF (stage II), and 8 patients (6.3%) had severe disease (stage III). In the whole population, pirfenidone attenuated the decline in FVC (p = 0.065), but did not influence the decline in DLCO (p = 0.355) in comparison to the pre-treatment period. Stratification of patients into mild and severe disease groups based on %FVC level at baseline (>75% and ≤75%) revealed that attenuation of decline in FVC (p = 0.002) was more pronounced in second group of patients. Stratification of patients according to GAP index at baseline (stage I vs. II/III) also revealed that attenuation of decline in lung function was more pronounced in patients with more severe disease. CONCLUSIONS: In this national experience, pirfenidone reduced the rate of annual FVC decline (p = 0.065). Since pirfenidone provided significant treatment benefit for patients with moderate-severe disease, our results suggest that the drug may also be effective in patients with more advanced disease.

Side effects of anabolic androgenic steroids abuse.

Long-term side effects of high doses of anabolic androgenic steroids self-administration were evaluated in this study. Twenty male bodybuilders, voluntarily starting steroid self-administration, were followed every 6 months over 2 years. Physical examination, haematological, metabolic and endocrine variables, semen analysis, hepatic and prostate ultrasound and echocardiographic evaluations were performed. LH values (baseline 3.43 +/- 1.75) were suppressed at 18 (1.98 +/- 1.99) (p = 0.026) and 24 (2.43 +/- 2.17) (p = 0.026), and FSH (3.95 +/- 2.01) at 6 (3.01 +/- 2.16) (p = 0.031), 12 (2.45 +/- 2.54) (p = 0.029), 18 (2.02 +/- 2.29) (p = 0.032) and 24 (3.42 +/- 2.64) (p = 0.032) months and SHBG (34.11 +/- 10.88) values significantly lowered at 12 (24.81 +/- 12.49) (p < 0.05), 18 (21.28 +/- 11.15) (p < 0.01), 24 months (25.42 +/- 11.16) (p < 0.01). A significant decrease in spermatozoa count (p < 0.01), and fertility index (p = 0.01) occurred. HDL-cholesterol (baseline 56.94 +/- 13.54) was reduced at 18 (41.86 +/- 14.17) (p < 0.01) and 24 (43.82 +/- 18.67) (p < 0.05) months and Apo A-1 at 12 (p < 0.001), 18 (p = 0.05) and 24 (p = 0.05) months. The most important long-term adverse effects were lower fertility and the impairment of lipid profile associated with an increased cardiovascular risk.

Serum cardiac troponin T after repeated endurance exercise events.

Recently Dr. Rowe made a hypothesis according to which small areas of myocardial necrosis can be caused by microvascular spasm, related to high catecholamine concentrations and other mechanisms, following extraordinary unremitting endurance exercises or due to the cumulative effect of several endurance events. It was this last suggestion which prompted us to investigate 25 top cyclists, taking part in the 77th Giro d'Italia. Blood samples were obtained the day before the start of the competition and once a week thereafter until the end. We measured myoglobin, lactic dehydrogenase, total creatine kinase, creatine kinase isoenzyme MB and serum cardiac troponin T (Tn-T), a highly sensitive and specific method for the detection of myocardial injury. While at measuring time points which followed we found a significant increase in the serum indicators of muscle damage, compared with their values at the beginning of the race, creatine kinase isoenzyme MB did not rise significantly and cardiac Tn-T was found in the serum of only 5 athletes, repeatedly in some cases, but always below the cut off values considered as indicating myocardial ischemia. On the basis of the behaviour of creatine kinase isoenzyme MB and, above all, of cardiac Tn-T, we can conclude that heavy endurance exercises, repeated daily for 22 days, as was the case in our study, do not seem able to produce, in top athletes, permanent heart damage by means of acute myocardial injury.

Lipoprotein(a) and exercise.

Plasma levels of lipoprotein(a), total cholesterol, triglycerides, HDL cholesterol, LDL cholesterol, apoprotein A1 and apoprotein B were assessed in 10 healthy, untrained volunteers subjected to a bicycle ergometric exercise equal to 50% of individual VO2max, followed by increasing loads until muscular exhaustion. Blood samples were taken before the exercise, immediately afterwards and then at 12-hourly intervals for a 72 hours period. Subsequently, the same parameters were evaluated for 8 long-distance runners during the XXIII New York Marathon, with blood samples being taken before and after the race, and then after one month of detraining. After the exercise, lipoprotein(a) in untrained subjects began to decrease significantly from the 24th hour on and remained lower than baseline levels up till the 72nd hour. After detraining, lipoprotein(a) in marathon runners increased significantly both with respect to basal values and especially to post-race values. Modifications of the other metabolic parameters evaluated in both tests were negligible and predictable. In the two groups of subjects examined, no correlation was found between lipoprotein (a) and the anthropometrical data and metabolic parameters considered.

Lipoprotein (a): effect of detraining.

Plasma levels of lipoprotein (a), total cholesterol, triglycerides, HDL-cholesterol, LDL-cholesterol, apo-protein Al and apoprotein B were evaluated for 8 long-distance runners during the XXIII New York Marathon, with blood samples being taken before and after the race, and after one month of detraining. After detraining lipoprotein (a) increased significantly both with respect to basal values and especially with respect to immediately post-race values. Negligible and predictable modifications of the other metabolic parameters evaluated, were observed. No correlation was found between lipoprotein (a) and the anthropometrical data and metabolic parameters considered.

[Chlordesmethyldiazepam and prolactinemia during surgical stress]. / Clordemetildiazepam e prolattinemia in corso di stress chirurgico.

The per-operative plasma prolactin levels in 16 male patients undergoing plastic-reconstructive surgery were studied. 8 patients received chlordesmethyldiazepam 0.1 mg/kg-1 i.v. (group C), 8 sodium thiopental 5 mg/kg-1 i.v. (Group T) in anaesthesia induction. All patients were premedicated by diazepam 0.15 mg/kg-1 by mouth 90 min. before anaesthesia induction and atropine 0.007 mg/kg-1 i.v. just before induction, and received succinylcholine 1 mg/kg-1 i.v. and an endotracheal tube for mechanically controlled ventilation. Anaesthesia maintenance was assured by isoflurane 0.5-2.5% in a N2/O2 (2/1) gas mixture. Blood samples were collected from each patient at the following times; 24 h before surgery (t0); 30 min after skin incision (t1); 30 min after extubation (t2). Significant variation of blood prolactin levels are not been shown.

[Effects of chlordemethyldiazepam on blood cortisol during surgical stress]. / Effetti del clordemetildiazepam sulla cortisolemia in corso di stress chirurgico.

The pre-operative plasma cortisol levels in 16 male patients undergoing plastic-reconstructive surgery were studied. 8 patients received chlordesmethyldiazepam 0.1 mg/kg-1 i.v. (group C), 8 sodium thiopental 5 mg/kg-1 i.v. (group T) in anaesthesia induction. All patients were premedicated by diazepam 0.15 mg/kg-1 by mouth 90 min before anaesthesia induction and atropine 0.007 mg/kg-1 i.v. just before induction, and received succinylcholine 1 mg/kg-1 i.v. and an endotracheal tube for mechanically controlled ventilation. Anaesthesia maintenance was assured by isoflurane 0.5-2.5% in a N2O/O2 (2/1) gas mixture. Blood samples were collected from each patient at the following times: 24 h before surgery (t0); 30 min after skin incision (t1); 30 min after extubation (t2). Significant variations of blood cortisol levels have not been shown.

Bombesin inhibits growth hormone response to insulin-induced hypoglycemia in humans.

Intravenous administration of bombesin (5 ng/kg/min X 2.5 h) significantly reduced growth hormone (GH) response to insulin-induced (0.15 U/kg, i.v.) hypoglycemia in 8 male volunteers without affecting its basal plasma levels. These data, together with the presence of bombesin-like immunoreactivity in the human brain, suggest that--as in experimental animals--this neurogastrointestinal peptide may be of physiological significance in the control of GH secretion also in man.

Inhibition of gastric emptying and secretion by pirenzepine and atropine in rats.

The effects of pirenzepine and atropine on gastric emptying, gastric secretion and heart rate were studied in rats. Both drugs inhibited gastric emptying and secretion dose dependently and increased pulse rate. In the gastric secretory studies both compounds displayed potencies which were not very dissimilar (ID50 were 8.1 mumol X kg-1 and 1.4 mumol X kg-1 for pirenzepine and atropine respectively, potency ratio 6); pirenzepine was however decidedly less potent than atropine in inhibiting gastric emptying (potency ratio 36 on a molar basis) and in increasing heart rate (potency ratio 125). These data, in accordance with results of clinical trials, indicate that pirenzepine-unlike atropine-can inhibit acid secretion without appreciably affecting gastric motility and cardiac function.

[A simple method for the measurement of human gastric emptying: standardization of operative parameters]. / Un semplice metodo per la misura dello svuotamento gastrico nell'uomo: standardizzazione dei parametri operativi.

The radioisotopic techniques used for the measurement of gastric emptying in man require complex and expensive scintiscanners or gamma-cameras. In this investigation, a simple method utilizing a single and inexpensive detector was evaluated; in addition, the suitability of 99Tcm sulphur colloid as a label for solid meals was assessed. The procedure was found to be accurate and its reproducibility high. The calculated absorbed dose to the gut was very low. Both in vitro and in vivo studies indicate that the radiocolloid used is a food non adsorbable marker for determining gastric emptying of solids. In a group of 50 healthy volunteers the emptying half-time (t1/2) ranged between 40 and 110 min, with a mean of 71.7 +/- 10.1 min, the male subjects exhibiting a significant lower t1/2 than females. In 5 volunteers with t1/2 within the normal range, the effect of drugs acting on gastric motility was evaluated. Hyoscine N-butyl bromide (20 mg) significantly delayed gastric emptying of solids, whereas metoclopramide (20 mg) was ineffective in these subjects. On the contrary, this drug was able to accelerate gastric emptying only in some patients with abnormally delayed emptying. On the whole, the method is simple, inexpensive and reproducible. It may be of great clinical value to investigate abnormalities of gastric function and to perform a better pharmacological approach to the medical treatment.

Effect of acute and chronic cimetidine administration on glucose tolerance and insulin secretion in man.

The effect of acute and chronic cimetidine administration on glucose tolerance and insulin secretion was studied in healthy male volunteers. Cimetidine was administered intravenously (4 mg X kg-1 followed by 0.7 mg X kg-1 X h-1) in acute studies and by oral route (1 g/die for 4 weeks) in long-term studies. Oral (100 g) or intravenous (0.5 g X kg-1) glucose was used as a stimulus for insulin secretion in both studies. Neither acute nor chronic cimetidine administration modified insulin secretion and glucose tolerance. These data are consistent with the idea that H2-receptors are not involved in the insulinogenic effect of glucose.

[Acute bacterial nephritis in diabetics (author's transl)]. / Nefrite acuta batterica in diabetico.

The AA. describe an unusual form of severe grahm negative bacterial infection of the renal parenchima that occurs infrequently in adult patients, most of whom are diabetics. During the acute phase, the kidney becomes smoothly enlarged, with marked impairment of contrast material excretion. The urographic and echographic findings simulated a renal neoplasm; on the contrary the selective renal angiography excluded the presence of a neoplastic blood circulation. This bacterial infection produces an inflammatory infiltrate surrounding and occluding the interlobular arteries in the cortex, with secondary ischemy. A precious and appropriate antibiotic therapy causes a rapid return of renal function; on the contrary this process may produce a globally small kidney in the long run.