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Francesca MAROTTI

Ricercatore Universitario
Dipartimento di Scienze Mediche e Chirurgiche Materno-Infantili e dell'Adulto

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Pubblicazioni

2020 - Myelomeningocele Repair Combining a Double Cryopreserved Amniotic Membrane Homograft and the Keystone Flap in a 3-Year-Old Child: A Case Report [Articolo su rivista]
Pignatti, Marco; Feletti, Alberto; Sapino, Gianluca; Marotti, Francesca; Pavesi, Giacomo; De Santis, Giorgio
abstract

Early repair in patients affected by myelomeningocele (MMC) is of paramount importance in order to prevent infection, minimize neural tissue damage, and reduce mortality. Treatment must include duraplasty and possibly an adequate soft tissue coverage. Delayed surgery in MMC patients can be more tedious due to the less clear borders between the placode and the skin. Moreover, the risks of wound infection and breakdown increase significantly.

2016 - Effects of nutritional intake on disease severity in children with sickle cell disease. [Articolo su rivista]
Mandese, Valentina; Marotti, Francesca; Bedetti, Luca; Bigi, E; Palazzi, G; Iughetti, Lorenzo
abstract

BACKGROUND: Children with Sickle Cell Disease (SCD) may show growth failure in comparison to healthy peers. Many factors as hematological status, endocrine and/or metabolic dysfunction, and nutritional status, may play an important role in growth failure. The aim of this study was to assess whether impaired growth and nutritional intake can affect SCD severity during childhood. METHODS: We conducted an observational study on children with SCD referring to our clinic for routine follow-up visits in a 6-month period. We collected information on weight, height and body mass index (BMI) and calculated their respective standardized scores (z). The nutritional intake was assessed through the last 24-h recall intake of total calories, macro- (proteins, lipids, carbohydrates) and micronutrients (calcium, iron, phosphorus, vitamins B1, PP, A, C, B2). Disease severity was assessed through total hemoglobin (Hb) and fetal hemoglobin (HbF), and lactic dehydrogenase (LDH) levels, and through the total number and days of hospitalizations, as well as the lifetime episodes of acute chest syndrome (ACS). RESULTS: Twenty nine children (14 males, 15 females) with SCD were enrolled; their mean age was 9.95 years (SD 3.50, min 3.72, max 17.18). Z-weight and z-BMI were significantly directly related to total Hb. Food intake resulted significantly unbalanced in terms of total calorie intake, macro- and micronutrients, especially calcium, iron, vitamin B1 and C. Low intake of calcium and vitamin B1 were significantly inversely correlated with number and days of hospitalizations per year. Protein, lipid, phosphorus, and vitamin PP intakes resulted adequate but were inversely correlated with number and days of hospitalizations. Carbohydrate, lipid, iron, phosphorus, vitamins B1 and B2 intakes were significantly inversely correlated to HbF levels. CONCLUSIONS: This study showed that, in our population, inadequate nutritional intake, weight and BMI have a significant impact on SCD severity indices.

2004 - Bone growth, modeling and remodeling in a supernumerary metatarsal bone associated with segmental gigantism in cutis marmorata telangiectatica congenita [Articolo su rivista]
Marotti, Francesca; Mf, Bertolani; Palumbo, Carla
abstract

Skeletal structure and processes of bone growth, modeling and remodeling were studied in a supernumerary metatarsal surgically removed from a 3-year-old boy affected by Cutis Marmorata Telangiectatica Congenita (CMTC), associated with hypertrophy of the right upper and lower limbs and postaxial hexadactylism of the homolateral hand and foot. No other anomalies were observed. The excess of periosteal growth, due to congenital anomaly, induced an abnormal development of both modeling and remodeling processes. In bone modeling, osteoblast activity on the periosteal surface was not paralleled by osteoclast resorption along the wall of the medullary canal, and this enormously increased the cortical thickness. In bone remodeling, osteoclastic resorption cavities were not refilled by secondary Haversian systems, thus inducing a severe bone loss. While the alteration of bone growth and modeling can be ascribed to the congenital disease, the unbalanced bone remodeling appears mainly to depend on mechanical disuse of the supernumerary metatarsal.

1999 - Extraction of a rubber bullet from a bronchus after 1 year - Complete resolution of chronic pulmonary damage [Articolo su rivista]
Bertolani, Mf; Marotti, Francesca; Bergamini, Barbara Maria; Pellegrino, M; Balli, R; Buonaura, Pc
abstract

Inhalation of a foreign body (FB) into the bronchial tree rarely occurs asymptomatically and, if leading to recurrent pneumonia, can be very difficult to diagnose, The present report deals with the case of a 10-year-old boy who had three episodes of pneumonia in the left lower lobe caused by the asymptomatic inhalation of a FB 12 months before. Standard thoracic CT, done during the third episode, revealed a slight reduction in the volume of the left lung with air bronchograms, multiple areas of bronchiectasis, and parenchymal consolidation of a segment of the lower lobe, Flexible fiberoptic bronchoscopy revealed a FB at the distal end of the left lower lobar bronchus, surrounded by granulation tissue and fully obstructing the anterior basal segmental bronchus. High-resolution CT (HRCT) images showed an inverted C-shaped image obstructing a bronchus. Removal of the FB was successful only with rigid bronchoscopy under total anesthesia, The FB was an air-pistol rubber bullet that the boy remembered playing with 12 months before. Two months after removal of the FB (ie, 14 months from its asymptomatic inhalation) and treatment with oral steroids, antibiotics, and respiratory physiotherapy, the patient recovered completely, and HRCT showed complete normalization of the lung. We conclude that, when the radiographic density of the FB is greater than the surrounding pulmonary parenchyma, HRCT can reveal the FB, and diagnostic flexible fiberoptic bronchosopy can be avoided.

1997 - Comparison of interrupter resistance with flow/volume method in childREN [Articolo su rivista]
Bertolani, M. F.; Marotti, F.; Bergamini, B. M.; Ferraroni, E.; De Guglielmo, M.; Ganazzi, D.
abstract

Airway resistance measurement with the Interrupter Technique (RINT) has been proposed as a valid method for the evaluation of bronchial obstruction and, being easy to administer, could find its application in the uncooperative child (Ch). In order to evaluate the possible clinical application of RINT (Micro Medical MICROLAB 4000), this technique was compared with the Flow/Volume curve (F/V) (SPIROPRO 2000 Pabysch) in Ch (mean age 109.39±39 months). We studied : A) 54 Ch with normal F/V in baseline conditions; B) 50 asthmatic Ch before and after the exercise test (6' free running); C) 102 asthmatic Ch before and after inhalation of 200 ug salbutamol MDI. RINT was calculated, both with mouthpiece and face mask, as the average of 20 measurements for test A and 10 for tests B and C. Paired t test (significant for p<0.05) and Pearson correlation coefficient (significant for r2 0.50) were calculated. Results : A) RINT vs FEV1, PEF, MEF 25, 50 and 75 were not correlated (r2<0.50) even though r revealed an inverse trend (negative sign). Mean values with mouthpiece were higher than those with face mask (p<0.005). B) The change in mean values of expiratory flows was significant at 6', 12' and 20', while mean RINT change was significant only at 6' whether with mouthpiece or with face mask; r had a negative sign but r2<0.50. C) The bronchodilator test showd significant changes in values (p<0.001) both with RINT and F/V. r had a negative sign but r2 < 0.50. Conclusions : RINT measurements are not generally comparable with F/V curve values. Only the bronchodilator test showed significant changes. The mouthpiece measurements are much lower than those with face mask. The results are extremely non-homogeneous and we conclude that the RINT system used has to be improved before it can be applied in clinical practice. © 1997 Wiley-Liss, Inc.

1997 - Cricoarytenoid arthritis as an early sign of juvenile chronic arthritis [Articolo su rivista]
Bertolani, Mf; Bergamini, Barbara Maria; Marotti, Francesca; Giglioli, P; Venuta, A.
abstract

A 14-month-old girl developed chronic strider and dyspnoea. Four months later she presented arthritis, anterior uveitis and positive ANA. Juvenile chronic arthritis (JCA) was diagnosed. Laryngoscopy demonstrated the presence of cricoarytenoid arthritis (CA). The left vocal cord was adducted and immobile, while the right vocal cord had decreased mobility. Erythema and swelling of the arytenoid cartilage on both sides was seen. Steroid treatment resulted in the resolution of these symptoms and made airway control unnecessary. This case demonstrates that CA may be the first sign of JCA, preceding peripheral arthritis. CA should be considered in, every child with chronic stridor and laryngeal obstruction.

1996 - A study of childhood febrile convulsions with particular reference to HHV-6 infection: Pathogenic considerations [Articolo su rivista]
Bertolani, Mf; Portolani, Marinella; Marotti, Francesca; Sabbattini, Am; Chiossi, C; Bandieri, Mr; Cavazzuti, Gb
abstract

Most febrile convulsions (FC) in infants occur during a viral infection, particularly in children of less than 3 years of age: human herpesvirus 6 (HHV-6) has an Important pathogenic role. To evaluate the link between this and other viruses and FC, a group of 65 children (mean age 18.46 months: SD+/-9.19) with a first episode of simple FC (G1) was compared with 24 children (mean age 19.29 months, SD+/-13.17) with a febrile syndrome but without FC (G2). Virological study showed the following infections: HHV-6 in 23/65 of G1 and in 12/24 of G2, adenoviruses (ADV) in 9/65 of G1 and in 0/24, of G2, syncytial respiratory virus (SRV) in 3/28 of G1 and in 0/2 of G2. HSV-1 in 6/65 of G1 and in 1/24 of G2, cytomegalovirus (CMV in 2/65 of G1 and in 0/24 of G2 and HHV-7 in 1/42 of GI and in 1/13 of G2. Children in G1, statistically compared with G2, were significantly more Likely to have a family history of FC and circulating granulocytes, while IgM and alpha 2-globulin were less probable. Some cytokines (IL 1 beta, TNF beta and GM-CSF) were found in 24 children in G1 and 12 in G2; no differences were found between the two groups. In the light of our data and of the recent literature, the possibility that the cytokines may act on the nervous system cannot be excluded. Among the HHV-6-infected children, those suf fering from convulsions were statistically more likely to have a family history of FC and ISM, while IgA were less likely. In G1, 57 cases were followed up over 2 years: 9 of them had a second episode of FC. Virological diagnosis at the first episode of FC revealed HHV-6 infection in 3 cases, 2 of these being due to viral reactivation. We underline the important role of HHV-6 infection in FC and postulate a relation ship between family history and the immunity of the patient; this is confirmed by the loss of statistical significance in the reduction of IgM in G1 compared with G2 with no family history of FC. The reactivation of FC by HHV-6 is a possibility to be borne in mind, an increased number of cases would be needed to confirm this hypothesis.

1996 - Oral pathology in young people with insulin dependent diabetes mellitus [Articolo su rivista]
Bertolani, M. F.; Forese, S.; Marotti, F.; Miselli, V.; Giannotti, S.; Tassinari, M.; Bondi, M.
abstract

The oral pathology that affects young people with insulin dependent diabetes mellitus (IDDM) is little known, but represents a non-marginal aspect of the disease and is important for a good quality of life. We have studied the oral health and the composition of the saliva of a group of young people of age ranging from 5 to 17 years (mean 11.74; SD ± 3.17), 15 boys and 8 girls who have been affected by IDDM for a minimum of 26 to a maximum of 72 months (mean 41.22; SD ± 41.14). The IDDM group was compared to 24 healthy subjects of the same age (p = 0.33) ranging from 5 to 16 years (mean 10.79; SD ± 3.44), 14 boys and 10 girls. Salivary composition, during fast, of IDDM differentiates from the saliva of healthy subjects for a more alkaline pH and for a higher concentration of glucose. Among bacteria notoriously related to caries, Lactobacilli are significantly higher in IDDM and Streptococcus mutans is extremely variable but higher on average, also if without statistical significance, than healthy subjects. The presence of Candida albicans is similar in the two groups. Salivary IgA are higher in the IDDM. Odonto-stomatological health reveals in the IDDM a higher approximal plaque and sulcus bleeding indexes; the latter is related to mean HbA(1c) of the last 12 months. In one case of IDDM there are wide gingival pouches and in another one there is loss of bone bearing with dental mobility. In IDDM decay indexes are slightly lower, probably in relation to the diet of the diabetic patient; gingivitis and tartar are more frequent in diabetics. These data, that need to be confirmed by an increase of the number of cases, show, in the young diabetic, a good dental health with precocious periodontal disease inversely proportional to the glycemic balance probably due to the damage of the connective and vascular tissues in IDDM. It follows that an antibacterial prevention and periodical odonto-stomatological examinations are necessary.

1993 - Blackfan-Diamond disease with ambiguous genitalia [Articolo su rivista]
Bertolani, M. F.; Baroncini, A.; Marotti, F.; Scalera, E.
abstract

The Authors describe a case of Blackfan-Diamond anemia with ambiguous genitalia and other minor anomalies. They point out the elements of differential diagnosis with other precocious erythroblastopenic conditions and suppose a recessive inheritance of the disease because of family consanguinity in two generations.

1993 - Pulmonary arteriovenous fistula: a case in childhood [Articolo su rivista]
Bertolani, M. F.; Forese, S.; Muratori, G.; Marotti, F.; Bertolani, M.; Calandra Buonaura, P. L.
abstract

The authors describe a rare case of congenital, bilateral arteriovenous fistulas of the lung, without other symptoms of hemorrhagic telangiectasis. The disease was diagnosed in a ten-year-old child, because of a chronic hypossiemia state. The angiography with digitalized technique defined, at the best, the vascular anomalies; the examen cannot be substituted by other imaging methods. First a therapeutic embolisation in angiography was tried without success because of the dimension of the arteriovenous shunt. Then a local excisional therapy was carried out, on one lung. One year after the operation, the oxygenative and clinical conditions of the patient are excellent.

1990 - Osteogenesi imperfetta. Primi dati sulle alterazioni strutturali riscontrate nei tessuti mineralizzati di un dente [Articolo su rivista]
Zaffe, Davide; Marotti, Francesca; A., Cheli
abstract

Struttura e grado di mineralizzazione sono stati analizzati nello smalto e nella dentina del 2° dente premolare permanente dell'emimandibola sinistra in una paziente affetta da osteogenesi imperfetta tipo III (sindrome di Ekman-Lobstein). Dopo una breve descrizione del caso clinico, con particolare riferimento alle patologie dentaria e scheletrica, vengono riportati i risultati preliminari dello studio istopatologico. Essi dimostrano la presenza di difetti di mineralizzazione sia nello smalto che nella dentina. Nello smalto, le zone ipocalcificate si trovano solamente lungo la giunzione con la dentina, come in genere si riscontra nei denti normali; esse risultano, tuttavia, più estese di quelle fisiologiche. Non sono, pertanto, da ritenersi difetti primari dell'amelogenesi ma secondari alla dentinogenesi imperfetta. Al contrario, nella dentina, le zone ipocalcificate appaiono disseminate nelle regioni sia mantellare che circumpulpare. Rispetto alla dentina normocalcificata, queste zone osservate a luce polarizzata presentano le fibre collagene irregolarmente intrecciate. I risultati ottenuti sono brevemente discussi in relazione sia ai processi di deposizione dello smalto e della dentina sia alla resistenza fisica dei denti di pazienti affetti da osteogenesi imperfetta tipo III.By means of the light microscope, under ordinary and polarized light, and the microradiography the structure and degree of mineralization of enamel and dentin were studied in the permanent 2nd premolar tooth of the left emimandible in a patient afìfected by type III osteogenesis imperfecta (Ekman-Lobstein syndrome). After a brief description of the clinical case, with particular reference to skeletal and dental pathologies, the preliminary results of the histopathological analysis are reported. They show the existence of defects of mineralization in both enamel and dentin. In the former, the hypocalcified areas are only located close to the enamel-dentin junction, as it usually occurs in normal teeth; however, they look broader than physiological ones. Thus they do not seem to be primary defects of amelogenesis but secondary ones, due to dentinogenesis imperfecta. On the contrary, in dentin, the hypocalcified areas are scattered throughout both mantle and circumpulpal regions. In the hypocalcified areas, compared with normally mineralized dentin, collagen fibers appear to be irregularly interwoven under polarized light. The results obtained are briefly discussed in relation to the processes of enamel and dentin deposition, and to the physical resistance of the teeth in type III osteogenesis imperfecta.