Correlations between biochemical markers in the synovial fluid and severity of rotator cuff disease.

The role of biochemical factors in the onset and natural history of rotator cuff disease is not fully understood, but it is generally recognised that they could induce tendon damage in association with mechanical and vascular factors. In this study, 5 biochemical parameters were analysed (total protein concentration, matrix metalloproteinase (MMP)-2 or gelatinase A, MMP-9 or gelatinase B, type I collagen telopeptides, hyaluronic acid) in the synovial fluid (SF) aspirated from the gleno-humeral joint of 29 patients undergoing surgical therapy for rotator cuff lesions. Four different groups of patients were identified according to the severity of the lesion: partial tear of the rotator cuff, full thickness tear involving 1 tendon and cuff tear arthropathy (CTA). The total SF protein concentration progressively increased with loss of integrity of the rotator cuff, reaching the highest levels in CTA. The absolute enzymatic activity of gelatinases was greater in full thickness tears than in partial tears, while it decreased in CTA. Conversely, the ratio between gelatinases and total protein content reached the highest level in partial tears and then progressively decreased. Collagen I telopeptides were significantly increased in full thickness tears and CTA, whereas the levels of hyaluronic acid decreased with worsening of rotator cuff disease. These findings support the hypothesis that gelatinases, which are involved in physiological tendon remodelling, intervene in the evolution of rotator cuff disease, too. Increased levels of type I collagen telopeptides give evidence that tendon tears are associated with an anatomic loss of tendon tissue and not with simple tendon retraction.

[Celiac disease and the evolution of its diagnosis. Comparison and experience at a hospital pediatric department (1975-1993). (Second part)]. / La malattia celiaca e l'evoluzione nella sua diagnosi. Confronti ed esperienze di un reparto pediatrico ospedaliero (1975-1993). (Seconda parte).

In a period of over 18 years the prominent medical bibliographic marks with regard to definition, diagnosis and examinations of coeliac disease (CD) have been compared and as far as possible reproduced. The results confirm the remarks derivating from wider statistics. From the beginning of 1975 to the first six months of 1993 in Merate Hospital Pediatric Division, 323 patients were submitted to a first jejunal peroral biopsy in 133 cases (41.2%) CD was diagnosed. Since 34 children (25.6%) concluded the ESPGAN diagnostic iter with 3 consecutive biopsies, the reasons why the other patients didn't finish or respect the programs are here examined. Since 1987 a specific anti-gliadin (IgA and IgG) antibodies titrimetry has been available either in the investigation of suspect symptomatology or like control mark during the assessment or after a sure CD diagnosis. Since october 1992 antiendomysium antibodies (EMA or AEA IgA) have been determined only in selected patients. From the examination of 24 subjects now checked with AGA IgA/IgG and EMA and with a first positive biopsy, it is possible to point out that only one jejunal biopsy (or at the most a second one as a control during the gluten challenge) with the guarantee of haematologic patterns doesn't raise doubts about a CD diagnosis. Analogous considerations mainly refer to the atypical CD "late onset" when a constant lack of AGA and EMA during gluten free diet (GFD) or their changes in a non compliance or in gluten challenge, can exclude a following hystological confirmation. By this experience it follows that a specific antigliadin and antiendomysium antibodies investigation is indispensable to the shortening of diagnostic times, to the reduction of an often unwelcome invasive diagnostic method and to the discovery of the "CD iceberg".

[Celiac disease and its diagnostic evolution. Comparisons and experiences in a hospital pediatric department (1975-1992). I]. / La malattia celiaca e l'evoluzione nella sua diagnosi. Confronti ed esperienze di un reparto pediatrico ospedaliero (1975-1992). (Prima parte).

The coeliac disease (CD) or gluten-sensitive enteropathy (GSE) is a permanent intolerance to wheat gliadin and to correlated proteins inducing malabsorption and typical damages of the jejunal mucosa (total or subtotal villous atrophy = SVA) in genetically-predisposed individuals ("DQW2"). A large amount of research has been devoted to CD pathogenesis: the most recent studies, thanks to sophisticated and experimental methods, support the pathogenetic immunological theory and the one of direct cytotoxicity. The correct diagnostic procedure for CD, established in 1970 by the European Society for Pediatric Gastroenterology and Nutrition (ESPGAN), suggested three small bowel mucosal biopsies. In the last years, because of the difficulties of such a practice, the necessity of non-invasive diagnostic approaches has developed; such approaches have been verified in absorption tests (one-hour blood xylose, intestinal permeability methods) and in immunogenetic tests (antibodies antigliadin, anti-reticulin, anti-endomysium, anti 90 KD glycoprotein, anti-human jejunum, HLA I/II antigens). The specific MHC antigens establish CD's incidence in several population and in particular situations, as in first-degree relatives and in diseases associated with CD (dermatitis herpetiformis (DH), insulin dependent diabetes mellitus (IDDM) and other auto-immune syndromes). The specific serum antibodies singly used as first level screening if estimated in combination with absorption tests, reach the highest levels of specificity and sensibility in CD diagnosis. It's anyway fundamental the comparison with at least a typical CD histological feature, caused by a challenge with a sufficient gluten to be carried in dubious cases and in non high auxological risk age (ESPGAN 1989). Adolescence is a period of frequent non compliance with a gluten-free diet and of particular psychological and physical problems: the apparent "gluten insensitivity", typical of teen-agers and adults, recalls the definitions of silent CD and latent CD (iceberg like). In the first case the jejunal mucosa is abnormal and the symptomatology isn't evident. In latent CD, genetically restricted, the mucosa is normal but there are minimal markers of inappropriate immunity to gliadin (at intestinal humoral immunity level) and a possible worsening of histological lesions to the third stage under environmental stimuli. This represents a two-stage model CD. That's why CD is still under-evaluated despite recent statistics reporting an increasing incidence (late and atypical forms). Prevalence rates between 1:300 and 1:4,000 and more are quoted in literature. The necessity of a strict gluten-free diet is confirmed by the evident frequency of lymphoma and by the increased risk of malignancy in untreated CD.(ABSTRACT TRUNCATED AT 400 WORDS)

[Dysplasia epiphysealis capitis femoris (Meyer's disease). An emergent pathology from the echographic screening of congenital hip dysplasia]. / Dysplasia epiphysealis capitis femoris (malattia di Meyer). Una patologia emergente dallo screening ecografico della displasia congenita dell'anca.

The authors present a case of Meyer disease. The knowledge of this pathology, emerging from the ultrasonic screening of the hip, is useful to the pediatrician in the differential diagnostic argument.

[Acute hemolytic anemia due to autoantibodies with cholelithic complication. Echotomographic diagnostic importance in a case report]. / Anemia emolitica acuta da autoanticorpi con complicanza colelitiasica. L'importanza diagnostica ecotomografica nella descrizione di un caso.

While the cholelithiasis is very common among chronic hemolytic anemia, it is not in acute. A case of cholelithiasis as a complication of autoimmune acute hemolytic anemia in a 5 years old child is reported. A specific and accurate diagnosis was readily made with grey-scale ultrasound. Serial sonograms were obtained to follow the resolution of this case.

[Primary intestinal lymphangiectasis. A case treated surgically]. / Linfangectasia intestinale primitiva (L.I.P.). Un caso di trattamento chirurgico.

The authors describe a rare case of primary intestinal lymphangiectasis resolved with surgical treatment. Usually the natural course of the disease is relatively mild and medical nutritional treatment can be sufficient. In this case the lymphatic intestinal anomaly was generalized to the entire small intestine but a distal ileal segment was particularly involved. The surgical resection of this intestinal tract resolved the symptomatology.

[Hydronephrosis in childhood. Long and mid-term morphologic and functional results]. / L'idronefrosi in età pediatrica. Risultati morfologici e funzionali a medio-lungo termine.

Hydronephrosis in infancy and childhood is a frequent urinary malformation and is almost always congenital. In over 80% of the cases it is due to ureteropelvic junction obstruction; in about 17%, anomalous renal vessels at the ureteropelvic junction are present. The authors analyze their experience in the surgical treatment of hydronephrosis. 360 hydronephrosis out of 335 patients were operated on from November 1971 to November 1988. 108 patients were less than 1 year old (32.2%). Primary nephrectomy was carried out only in 11 of the 360 hydronephrosis operated on (about 3%). An anterior approach and a simple dismembering pyeloplasty were performed in the majority of the cases. From the radiological point of view there was an impressive amelioration or normalization in about 92% of the cases operated on. From the functional point of view the radionuclide scan showed a normal or slightly reduced renal function in almost 90% of the cases operated on. The preoperative and postoperative radionuclide study showed a moderate increase of the renal function in the majority of the cases controlled. The authors stress the importance of prenatal ultrasound diagnosis to improve long term results of an early surgical treatment.

[Post-necrotizing enterocolitis stenosis of the colon associated with cytomegalovirus infection. Description of a clinical case]. / Stenosi colica post-NEC associata ad infezione da Cytomegalovirus. Descrizione di un caso clinico.

We report a case of an infant with a post-necrotizing enterocolitis stenosis, resolved with operative measures. The histologic finding of Cytomegalovirus in the resected sigmoid colon rise the question if the intestinal stricture was the proper consequence of a primary enteric viral infection or a superinfection in a transitory immunodeficiency after the acute phase of necrotizing enterocolitis.

Birth after direct intraperitoneal insemination with frozen semen of donor.

For the first time, as least as far we known, a pregnancy and a normal baby obtained with DII with frozen semen of donor are reported. Our experience not only shows DII with frozen semen may be used successfully in AID but that it poses interesting hypotheses, to be verified in future, of its use in association with, or as an alternative to, GIFT and IVF/ET in cases of AID failure and as a first approach to an insemination technique in AID in general.

[Hemoglobin H disease. Presentation of a case]. / La malattia da emoglobina H. Presentazione di un caso.

Haemoglobin H (Hb H) disease, the most important clinical form of alpha-thalassaemia, shows remarkable clinical variability. Hb H si an unstable tetramer of beta-globin chains which accumulates because of the lack of adequate numbers of alpha-globin chains and precipitates in the red cells, causing their premature destruction. A case of Hb H disease in a 9-yr-old child, admitted into hospital for acute haemolysis after use of pyrazolone derived, is presented. Haematologic data with synthesis in vitro of globin chains were obtained from the parents and sister. The clinical and haematologic features of this form of haemoglobinopathy are briefly discussed in the light of recent knowledges of his genetic mechanism of transmission.

[Infantile urticaria pigmentosa. Presentation of a case]. / Urticaria pigmentosa infantile. Presentazione di un caso.

The authors describe one case of urticaria pigmentosa in childhood as contribution to the knowledge of this disease prevalently interesting the paediatric dermatology.

[The ureterocele in childhood. Type of treatment and results]. / L'ureterocele in età pediatrica. Tipo di trattamento e risultati.

Ureterocele is an infrequent urinary malformation in pediatric age, but it is frequently clinically severe. Ureterocele in a duplex pyelo-ureteral system often presents very difficult treatment problems not uniformly dealt with. Authors report their experience with 79 ureteroceles observed in 72 children. 27 children were less than one year old. In the authors experience simple ureterocele with a single collecting system doesn't give any surgical problem. On the contrary ectopic ureterocele, almost always associated with a pyeloureteral duplication, frequently gives some problems of surgical treatment. Treatment is always individualized but in our experience the best results have been achieved with unroofing of the ureterocele and upper heminephroureterectomy in one stage, eventually associated with lower ureteral reimplantation. This treatment can be used successfully in the majority of ectopic ureteroceles in pediatric age.

[Posterior urethral valves. Type of treatment and short- and long-term evaluation of renal function]. / Le valvole dell'uretra posteriore. Tipo di trattamento e valutazione a breve ed a lungo termine della funzionalità renale.

In this paper the authors have analyzed the management and the outcome of 81 cases of posterior urethral valves which occurred during the period January 1972 - April 1985. 53 children presented very severe urethral valves (grade 4 according to Hendren), 28 mild valve type. All the children of the first group but two had a dilatation of the U.U.T. Vesico-renal reflux, usually severe, was present in 51 ureters; in another 50 ureters a dilatation without reflux was present. 47% of the children of the first group had a renal function within the limits of normality at the moment of presentation and the remaining 53% a reduced renal function. In 9 patients (8 under 50 days of age) a cutaneous vesicostomy according to Blocksom followed, at the age of 10-18 months, by transurethral valve destruction was done. In 6 infants, in the early part of the series, the valve was removed with a hook via the perineal approach. In 38 patients we performed a transurethral valve destruction with the n. 3 Bugbee electrode. 36 out of 53 children (68%) had exclusively a removal of the valvular obstruction. After the removal of the obstruction, 32 out of 37 non refluxing dilated ureters (86.5%) showed a clear improvement. In 7 out of 29 refluxing ureters a nephrectomy was carried out. In the remaining 22 ureters the reflux vanished in 17 and improved in the other 5. 17 children had other types of operations after valvular removal. 23 ureters in 13 patients were reimplanted, with 3 failures (13%). In the 53 children with very severe valve (grade 4) supravesical diversions were not carried out. In the follow-up of 51 children (from 6 month to about 14 years) the renal function was within the limits of normality in 74% (before the operation it was 47%). The best results were obtained in children diagnosed and treated in the first months of life. Cutaneous vesicostomy showed itself to be a very useful method of treatment in very young babies with severe complications. We observed a slight terminal urethral stricture, easily dilatable, in only 1 child. All the children over the age of 12-13 years were continent. In 28 children with mild valves, as well as transurethral valve destruction, an ureteric reimplantation was carried out with success in 5 children (8 ureters) and a vesical diverticulectomy in another 2.

[Seidlmayer's "cockade" purpura. Report of a case]. / La porpora "a coccarda" di Seidlmayer. Presentazione di un caso.

Among hemorrhagic disorders affecting infants and depending on a vessel increased permeability, Seidlmayer "cockade purpura" is characteristic for its typical cutaneous manifestations. Owing to the rarity of this disease, we report a recently observed case.

[Prolonged Q-T syndrome (Romano-Ward syndrome). Description of a case diagnosed in infancy]. / La sindrome del Q-T lungo (S. di Romano-Ward). Descrizione di un caso diagnosticato nella prima infanzia.

A case of prolonged Q-T interval syndrome without deafness (Romano-Ward syndrome) is reported. A 2-month-old female was seen in consultation because of a near-miss event (syncopal attack). An EKG showed a long Q-T interval. Successful therapy was achieved with propanolol.

[Infantile gluteal granuloma. Case report]. / Granuloma gluteale infantile. Contributo casistico.

A 5 months old infant having "granuloma gluteale infantum" (G.G.I.) is reported. One or more tumorous red-purple nodules on gluteal or on genitocrural area are the usual cutaneus injuries of G.G.I. The histologic aspect resembles pyogenic granulomas. The exact pathogenesis of G.G.I. is still to be defined; nevertheless the use of plastic diaper covers and topical fluorinated steroid preparations seems to have great influence.

[Visceral leishmaniasis. Practical considerations on a clinical case]. / La Leishmaniosi viscerale. Considerazioni pratiche su un caso clinico.

A male infant with profound anemia and liver and spleen enlargement was affected by visceral Leishmaniasis. Diagnostic tests and therapeutical approach to this parasitic infection, very rare in our Region, are discussed.

[Acute hydrops of the gallbladder in Kawasaki's disease. Presentation of a case diagnosed with echotomography]. / Idrope acuto della colecisti in corso di malattia di Kawasaki. Presentazione di un caso diagnosticato con ecotomografia.

Acute hydrops of the gallbladder is described in a patient who had mucocutaneous lymph node syndrome. Diagnosis was readily made with gray-scale ultrasound. Serial sonograms were obtained to follow the resolution of the hydrops once supportive medical therapy was started. The role of surgery in acute hydrops of the gallbladder is for complications of the hydrops and not for distension alone.

[A case of Flaiani-Basedow-Graves disease]. / Un caso di malattia di Flaiani-Basedow-Graves.

The authors report a typical case of hyperthyroidism with alopecia in a 5 years old female child with family precedents. We punctualize the present etiopathogenetic theory and the therapeutical possibilities.

[Long-term non-deforming urinary infections in childhood. 2. Proposed treatment and results]. / Le infezioni urinarie di lunga durata non malformativa in età pediatrica. II. Proposte di trattamento e risultati.

In the second part of the Authors work, they report the results obtained in the treatment of 85 female children with recurrent non malformative urinary tract infections. The first group of 15 female children had been treated with phenoxybenzamine and urinary chemyotherapycs with little satisfaction. The other two groups, both of which consisted of 35 children, one group was treated with only chemyotherapycs and the other with chemyotherapycs associated to oxybutynin chloride. The best results were obtained in the last group regarding the improvement of the symptoms and earlier and higher incidence of sterilization of the urine. At the same time in this group the maximum cystometric capacity and vesical compliance were brought back to normal and the detrusorial instability disappeared.