[ADAPTHERA-Statewide cross-sectoral care network for patients with early rheumatoid arthritis shows sustained remission in standard care]. / ADAPTHERA ­ Landesweit transsektorales Versorgungsnetzwerk für Patienten mit früher rheumatoider Arthritis zeigt anhaltende Remissionen in der Regelversorgung.

BACKGROUND/OBJECTIVE: The majority of patients in Germany miss out on the necessity of early diagnosis and initiation of therapy for rheumatoid arthritis (RA) caused by considerable structural deficits in the health care system. The challenge is to reconcile the individual demand for the best possible therapy result with a sustainable expenditure of resources. METHODS: The cross-sectoral regional care network ADAPTHERA aims to improve early RA diagnosis and treatment in Rhineland-Palatinate. The retrospective triage analyses of suspected early onset RA patients was performed by tracing the selection process of all available enquiries (n = 1045). For analysis of the clinical course of the disease, a subset comprising 143 patients with a minimum observation time of 12 months (5 consecutive visits) was available. Clinical and laboratory parameters were collected quarter yearly, self-administered questionnaires were filled out and the treatment was adapted if necessary. RESULTS: A total of 454 patients were included. The mean waiting time was 23.9 (SD = 18) days. The mean observation period in the subcohort was 29.2 (SD = 12.7) months, with about 50% of the patients presenting within 3 months. Almost 75% of the patients were in remission after 2 years. A sustained remission could be described for 74.8% (6 months) and 53.5% (12 months), respectively. Especially patients with rapid remission induction benefited in terms of longer remissions (p = 0.03). A very early stage of the disease (VERA) was associated with a rarely necessary biologic therapy (p = 0.022). DISCUSSION: The approach of a supply network is not a panacea, but it might improve healthcare for patients with early onset RA. In order to minimize resource utilization, a pinpoint referral and accurate triage of potential cases are crucial.

[Endoscopic ultrasound in the long-term follow-up of primary lymphomas of the stomach under conservative therapy]. / Endosonographie im Langzeitverlauf primärer Magenlymphome unter konservativem Therapieansatz.

BACKGROUND AND AIMS: The stomach is the main site of primary extranodular manifestation of non-Hodgkin's lymphomas. Besides clinical staging additional to histological diagnoses, the endoscopic ultrasound (EUS) also becomes more important during follow-up courses of conservative therapy regimens (e. g. H.p. eradication; radiochemotherapy). The aim of the present study was to assess the impact of EUS during long-term observation of primary gastric lymphomas and to outline possible changes of the gastric wall. PATIENTS AND METHODS: Within the scope of 2 prospective multicenter study on primary gastrointestinal non-Hodgkin's lymphomas (GIT-NHL), which were performed at Muenster University Hospital, 26 patients undergoing conservative treatment were examined with endoscopic ultrasound at a three-month interval between 01/1992 and 11/1998. The mean survey period was 28 months (range 7-62). RESULTS: In 22 patients over a period of 21 months (range 4-51) a histological proven complete remission (CR) was found. In 2 patients only a partial remission (PR) was achieved within a time period of seven respectively eight months of survey. Two other patients developed early relapse of non-Hodgkin's lymphoma. In patients with CR the endoscopic ultrasound showed a highly significant decrease of gastric wall thickness 7 and 12 months after therapy was started. On average after 4.6 months enlarged lymph nodes were no more detectable, after 5.5 months thickness of the gastric wall and finally after 6.6 month the previous abolished layering of the gastric wall returned to normal. CONCLUSION: In patients with gastric non-Hodgkin's lymphomas endoscopic ultrasound seems to be the only valid method which demonstrates early changes of the gastric wall and its layering in an appropriate way and might therefore in addition be capable to differentiate between CR, recurrent or refractory non-Hodgkin's lymphoma.

[Destombes-Rosai-Dorfman syndrome: 2 uncommon clinical forms]. / Syndrome de Destombes-Rosai-Dorfman: deux formes cliniques inhabituelles.

Two cases of Destombes Rosai Dorfman's syndrome are presented. Diagnosis was performed by superficial lymph node biopsy. The first case concerned a nine and half years old girl with cervical adenopathy who developed a compressive mediastinal adenopathy responsible for a right lower lobe atelectasis. Because of local lung suppuration a lobectomy had to be performed. The second case concerned a fourteen years old boy with recurrent fever, diffuse superficial lymph nodes and erythematous skin rash. The two patients showed clinical and biological inflammatory symptoms without any immunodeficiency. No aetiological agent could be identified. Antibiotics and corticoids had no effect but the two patients recovered (after 18 months follow up in case 2). These two particular cases confirm the clinical course heterogeneicity of the syndrome which requires histological diagnosis.

Hepatic tumours during androgen therapy in Fanconi anaemia.

The occurrence of liver tumours in the course of Fanconi anaemia (FA) has been well documented. We present a case, review the literature and conclude that androgen therapy would increase the risk of developing tumours, most of which appear to be benign (adenomas or peliosis) and androgen-dependent, generally decreasing in size after cessation of treatment. Survival of patients is poor, mostly because of the rapid evolution of the tumour. In the absence of an allogenic bone marrow transplantation, administration of haematopoietic growth factors might be effective. As a preventive measure, other types of unsubstituted androgens may be used.

Acute renal failure in a child after chewing of match heads.

A few days after ingestion of 40 match heads, a 3-year-old boy was admitted to hospital with oliguric acute renal failure (ARF) requiring peritoneal dialysis during 9 days. A renal biopsy showed acute tubulointerstitial nephritis; the outcome was rapidly favorable and the child recovered normal GFR. It seems to be the first published case of ARF after match poisoning, probably because of the presence of potassium bichromate.

[Value of the use of a vicryl-collagen tape for the treatment of cleft palate. Experimental study in dogs]. / Intérêt de l'utilisation d'un treillis de vicryl collagène dans le traitement des fentes palatines. Travail expérimental chez le chien.

The purpose of this study was to evaluate the bone forming capacity of a Vicryl/collagene tape, placed in a defect of the palatal bone. In order to decrease the rate of bucco-nasal fistulaes after primary closure of a cleft palate, the authors carried out an experiment on dogs. First of all, we had to demonstrate the good tolerance of the tape in the buccal cavity. The first group of 5 dogs redieved the tape between buccal mucosae and palatal bone. The tolerance was excellent concerning the 12 biopsies on the mucosae after 1 month; 10 were totally normal and 2 revealed signs of inflammation on the medial incision. Concerning the second group, we needed to know the delay of bone formation of a "neo-palatal" defect; for that reason a 2 cm2 bone resection and a histological observation after 1 month and 1,5 months, in the right palatal bone was performed. Then we performed the same resection in 5 other dogs, and let the vicryl tape in the defect before closing the mucosae. The histological results of the second group showed that the ossification began after 1 month, and after 1.5 months the defect was totally covered by bone. The good results we obtained led us to go on with this experiment even if the low number of dogs included in this series does not allow us to a statistical evaluation. Exact responsibility of both, vicryl and collagene, has to be determined by a longer experiment, before realising the device in child's cleft palate.

[Membranous glomerulonephritis in children: 20 cases]. / Glomérulonéphrites extramembraneuses chez l'enfant: 20 observations.

Twenty cases of membranous glomerulonephritis have been diagnosed between 1978 and 1988 in children (13 girls, 7 boys) aged 4 to 15 years, observed for a 5.1 +/- 2.9 year period. The conditions of the diagnosis were: routine urinalysis in 10 cases, edema in eight, and the surveyance of a D-penicillamine treatment in two. All the patients had proteinuria (0.3 to 15 g/24 h) ranging to nephrotic syndrome in nine children. Microscopic hematuria was found in 16 children (80%). Elevated blood pressure was recorded in two cases at the time of diagnosis, and developed in two other cases during the follow-up. One child experienced renal failure at the onset of the disease. Most histological lesions consisted in stage II membranous glomerulonephritis. Immunofluorescence study (18 biopsies/20) always showed granulosus and intensive IgC deposits, associated with IgM and IgA deposits which were less marked; intensive extra-membranous C3 deposits were noted in 11 cases. As to the etiology, D-penicillamine was responsible for two cases (10%) and HBs antigen in one (5%); the nephropathy was considered as idiopathic in the 17 remaining cases (85%). Regarding the evolution: in eight cases (38%) proteinuria disappeared by 54 +/- 28 months; in 10 cases (55%), proteinuria persisted after 41 +/- 31 months; hematuria, which was present at onset, disappeared in most cases (13/17); in one case (5%), end-stage renal failure occurred within 3 years. The patient with initial renal failure has been last sight off.(ABSTRACT TRUNCATED AT 250 WORDS)

Aetiology of membranous glomerulonephritis: a prospective study of 82 adult patients.

Eighty-two consecutive Caucasian adults (52 males, 30 females, aged 17-86 years) with membranous glomerulonephritis were prospectively evaluated for possible aetiological factors 1-4 weeks after renal biopsy. Presumed causes were identified in 17 patients (21%) as follows: drugs in five (D-penicillamine 3, captopril 1, fenoprofen 1); malignancy in four; chronic thyroiditis in three; systemic lupus erythematosus (SLE) in two; secondary syphilis in one; hepatitis B virus (HBV) infection in one and non-insulin-dependent diabetes mellitus in one patient. Except for age (patients with secondary membranous glomerulonephritis were older), clinical presentation and histological stage distribution did not differ between the secondary and the primary groups. Ten out of the 17 patients with secondary membranous glomerulonephritis (59%) achieved complete clinical remission within 12 months. The incidence of associated conditions in adults with membranous glomerulonephritis in this study corresponds with that reported in the few previous series. Although membranous glomerulonephritis is deemed to be idiopathic in most cases, it seems warranted to search for medication, malignancy, SLE, HBV infection, syphilis and thyroiditis as possible aetiological factors. Further evaluation should be orientated by the clinical context. An improved outcome of membranous glomerulonephritis may be expected insofar as the underlying condition is controlled.

[Hemolytic uremic syndrome and febrile urinary Escherichia coli infection]. / Syndrome hémolytique et urémique et infection urinaire fébrile à Escherichia coli.

The authors report on 2 cases of hemolytic uremic syndrome in 2 infants during a febrile Escherichia coli urinary tract infection. They discuss the role of urinary tract infection in the occurrence of hemolytic uremic syndrome and the possible role of specific Escherichia coli strains.

Hemoglobin Pierre-Bénite [beta 90(F6)Glu----Asp], a new high affinity variant found in a French family.

Hemoglobin Pierre-Bénite [beta 90(F6)Glu---Asp] is a new high affinity variant (P50 = 21.5 mm Hg), with normal heme-heme interaction, found in a French family. It was difficult to detect by conventional electrophoretic methods. However, the high performance liquid chromatography profile of its tryptic peptides contained an additional peak. Amino acid analysis of the corresponding peptide and determination of its sequence allowed us to identify the mutation. No instability was found. Mutations previously recorded in position 90 of the beta-chain display a positive charge shift and a reduced affinity for oxygen, whereas Hb Pierre-Bénite shows no charge shift and increased affinity for oxygen.

[Hypertrophic kidneys in utero and neonatal renal failure caused by diffuse mesangial sclerosis]. / Gros reins in utero et insuffisance rénale néonatale par sclérose mésangiale diffuse.

The authors report an unusual mode of onset of diffuse mesangial sclerosis in a newborn. Fatal neonatal renal failure, and not infantile nephrotic syndrome, was the main symptom after birth. In utero, ultrasonography revealed hypertrophy and hyperechogenicity of the kidneys associated with oligoamnios.

[Glomerular nephropathy in the Bardet-Biedl syndrome]. / La néphropathie glomérulaire du syndrome de Bardet-Biedl.

A case of Bardet-Biedl syndrome (BBS) with kidney involvement and renal failure is reported. Light microscopy demonstrates fibrosis of 40% of glomeruli, altered tubules and interstitial fibrosis; no cystic formation is present and immunofluorescence studies are negative. In electron microscopy, the glomerular basement membrane (GBM) looks twisted and uniformly thickened with segmental effacement of the trilaminar architecture; fibrillary material is accumulated close to the inner layer of the GBM. Intermittent peritoneal dialysis is initiated 2 years later; death occurs, after one year of dialysis, due to a bleeding duodenal ulcer. Chronic renal failure seems to be the most frequent cause of death in BBS and several mechanisms are involved. Tubulo-interstitial lesions and renal cysts have been well documented. Glomerular damage with early ultrastructural changes of the GBM may be implicated in the occurrence of renal failure. Further studies are needed to define the incidence and the specificity of the GBM abnormalities in BBS.

[Generalized BCG infection, with a favorable outcome, in a 3-month-old immunocompetent infant]. / Infection généralisée à BCG d'évolution favorable chez un nourrisson de 3 mois sans déficit immunitaire reconnu.

A 3 month-old infant vaccinated with BCG at birth presented with granulomatous hepatitis with BCG isolated in the liver. Splenomegaly, infiltrates in both pulmonary apices and hilar adenopathies were simultaneously present. No immune deficiency could be found. Complete recovery followed specific polychemotherapy. Parenchymal calcifications appeared in the liver, spleen, lungs and mesenteric ganglia.

[Anatomical study of the kidneys of newborn infants dying after a septic state]. / Etude anatomique des reins de nouveau-nés décédés au décours d'un état septique.

Over a 6 years period, 51 autopsies have been performed in infants dead after severe sepsis. One third of them had renal damages, consisting mainly in haemorrhagic infiltration. Pathological and clinical data are not always well correlated, but some parameters may enhance a poorer renal prognosis, such as the duration of shock and anuria, or exchange transfusion. The clinical presentation of the infection and the encountered germs are the same in both groups, with or without renal damage. A better management of intensive haemodynamic cares in these neonates increases their survival rate and raises the problem of chronic renal failure or hypertension in this situation. It seems very useful to state precisely the surveillance and protection of the renal function in such patients.

[Bone angiomatoses in children. Apropos of 3 personal cases]. / Les angiomatoses osseuses de l'enfant. A propos de 3 observations personnelles.

Three observations of skeletal angiomatosis are reported: one diffuse and two focal forms (Gorham's disease). The analysis of these cases and a review of the literature enhance the clinical and radiological presentation of this uncommon disease. The authors point out the interest of indirect lymphoscintigraphy and dwell on the gravity of its course, in spite of therapeutic attempts.

Extended intestinal aganglionosis in siblings.

In this paper we report two cases of extended intestinal aganglionosis in siblings, a condition that could be considered a severe variety of Hirschsprung's disease.

[Diffuse cortical nephroblastomatosis. Apropos of a case]. / La néphroblastomatose corticale diffuse. A propos d'une observation.

The authors report a case of massive bilateral, and asymmetric nephroblastomatosis which was treated successfully by nephrectomy and chemotherapy. The authors discuss the clinico-pathologic significance of the present case and insist upon the requirement of a treatment (i-e-chemotherapy) in every case.

[Cancer of the thyroid in children. 14 cases]. / Le cancer du corps thyroïde chez l'enfant. Quatorze observations.

Fourteen cases of thyroid gland carcinoma in children (mean age: 10 years) were reviewed. Scintigraphy showed a cold nodule in 10 and diffuse heterogeneous uptake indicating undifferentiated carcinoma in 3. The clinical risk of these tumours resides in alterations of the thyroid gland and in lymph node involvement. The favourable long-term prognosis in children justifies a conservative surgical treatment. Pulmonary metastases respond well to radioactive iodine. TSH secretion must be completely suppressed by continuous hormonal treatment. Anticancerous irradiation is fraught with potential dangers.

Data retrieval and analysis for radioimmunoassays.

A data retrieval and analysis system is described here which allows one to simplify and automate the collection of data for radioimmunoassays. The resulting data can be collected in a data summary format to provide the operator with clear, concise results. The software utilizes a Logit function to do regression analysis for results expressed in concentration of antibody, be it IgG, IgM or other, per milliliter of serum. This system allows the operator to do analysis on thousands of serum samples.