[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.

[Neuropathology of trichopoliodystrophy (Menkes' disease). Anatomoclinical observation]. / Neuropathologie de la trichopoliodystrophie (maladie de Menkes). Une observation anatomo-clinique

The authors describe an anatomo-clinical case of trichopoliodystrophy in many ways similar to cases already published. The original features of the case are as follows: the ultrastructure of the duodenal mucosa is normal; the endoplasmatic reticulum of the putaminal neurons contains many-layered lamellar structures probably composed of protein; type II fibres predominate in the striated skeletal muscle suggesting abnormal neuromuscular maturation.

[Study of neurological manifestations of chronic lymphatic leukemia (lymphosis). Role of associated globulin anomalies (apropos of 8 cases)]. / Contribution à l'étude des manifestations neurologiques des leucémies lymphoïdes chroniques (lymphoses). Role des anomalies globuliniques associées (a propos de huit observations)

The authors emphasize the relative frequency of neurological symptoms in lymphatic leukaemia. Clinically, these cause more or less diffuse encephalitic or multineuritic syndromes, generally a combination of the two. Their pathogenesis is usually connected with lymphoid tissue infiltration into either the meninges or the vascular sheaths of the central nervous system or the sheaths of the roots or of the peripheral nerves. The authors stress the possible function of immunoglobulin abnormalities of the C.S.F. indicative of the presence of the leukaemic process within the nervous system. This pathogenesis prompts the use of therapeutic methods directly attacking leukaemic infiltration of the nervous system (focal cobalt therapy, intrathecal chemotherapy) and the authors have found that these give favourable results.

[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.

[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.

[Mesoblastic nephroma. Apropos of 6 cases]. / Néphromes mésoblastiques. A propos de 6 cas.

6 cases of congenital mesoblastic nephroma removed surgically between 5 days and 2 months 1/2 of age are reported. The authors point out the peculiar histological aspect of this tumor : myomatous or fibromyomatous proliferation which surround in some places normal or dysplastic glomerular or tubular structures and penetrates by digitations in parenchymatous elements without destroying them. At the periphery of the tumor there are some lymphatic capsular ectasia or lymphoid infiltration or cartilaginous islets. In case of a renal tumor of the newborn or infant they emphasize the necessity to search for a congenital mesoblastic nephroma in order to avoid all types of radio therapic or chimiotherapic treatment without histological diagnosis. These forms of therapy are responsible for severe complications heavily impairing a prognosis which in most cases is an excellent one when nephrectomy alone is carried out. In some exceptional cases it seems useful to add other forms of therapy when the tumor proves itself to be of a peculiar aggressivity macro or microscopically.

[Adenomatoid malformation of the lung. A study on thirteen observations (author's transl)]. / Malformation adénomatoïde pulmonaire. A propos de treize observations.

The authors report thirteen observations of CAM of mixed solid and cystic type. They point out that besides a classic form of neonatal diagnosis might be a form late revealed in infancy or adolescence, of possible appearing after the embryonic period. This late form could be more localized. Only histologic examination can confirm the diagnosis whose fundamental criteria is the proliferation of bronchiolar or bronchiole-like structures.

[Pulmonary complications of azotemia. A case of uremic lung]. / Les complications pulmonaires de l'azotémie. A propos d'une observation de poumon urémique

The authors studied the problem of pulmonary complications in acute and chronic uremia. They recalled the main characteristics of haemodynamic oedemas which can accompany the periods of left ventricular insufficiency during the evolution of uremia. They analized the characteristicsof the classic uremic lung representing in fact only a variety of lesional oedema in which the toxic effect of urea is controversial. Ventilatory consequences of chronic uremia were briefly exposed.

[Alpers' infantile cerebral poliodystrophy. A case with abnormal hepatic pyruvate carboxylase]. / Poliodystrophie cérébrale infantile d'Alpers. Un cas avec anomalie de la pyruvate-carboxylase hépatique.

The authors describe an anatomical familial case of progressive infantile cerebral poliodystrophy (Alpers disease), in which the study of enzyme kinetics of hepatic pyruvate carboxylase revealed an abnormal graph reflecting a loss of activity of the enzyme with low concentrations of substrate, This is a new feature in the literature on Alpers disease, and possibly indicates one of the pathogenic mechanisms responsible in this disorder which remains mysterious, although its pathology has been clearly described.

Immunodeficiency diseases. I. T-lymphocyte precursors and T-lymphocyte differentiation in partial Di George syndrome.

Immunological and pathological studies in a case of partial Di George syndrome revealed an absence of parathyroids, a major hypoplasia of thymus but a relatively moderate decrease in peripheral T-lymphocyte numbers and functions. After in vitro incubation with normal thymus extracts, a normal proportion of bone marrow cells was induced to differentiate into cells with characteristics of T lymphocytes, thus establishing the presence of T-cell precursors in the patient's bone marrow.

[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.

[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.

[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.

[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.

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.