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Biomedical subjects

D Christmann

Publications and source records attributed to D Christmann.

At least 37 records · Page 2Linked to original sources

Adrenoleukodystrophy. Value of contrast-enhanced MR imaging.

In its early stage adrenoleukodystrophy (ALD) is characterized by hypodensity at CT and signal abnormalities at RMI (low-intensity signal on T1-weighted sequences, high-intensity signal on T2-weighted sequences) in the white matter of the parieto-occipital region and the splenium of the corpus callosum. These CT and RMI abnormalities are suggestive of ALD in children with progressive alteration of the superior brain functions, but they are not specific of the disease. The authors present two cases of ALD and underline the almost pathognomonic value of contrast-enhanced ribbons found at the periphery of low-intensity signal plaques after gadolinium injection. These areas of blood-barrier disruption on a background of inflammation and active demyelination appear, on T2-weighted sequences, as ribbons of low-intensity signal within plaques of high-intensity signal. MRI is also superior to CT in detecting abnormalities located in the posterior fossa, notably lesions of the auditory fibres.

Adrenoleukodystrophy

[Magnetic resonance imaging of the brain of newborn infants].

The authors report their experience of cerebral magnetic résonance imaging (MRI) in the neonatal period. MRI offers many advantages compared to CT scan or ultrasonography in the study of malformations, tumors, infections and anoxic-ischemic brain injury. However, MRI is limited by the duration of the examination the need for total immobility which is achieved in the neonate via administration of chloral hydrate, and lack of accessibility. MRI is a non invasive method for following in vivo brain development during infancy.

Brain

[Late diagnosis of congenital argininemia during administration of sodium valproate].

Congenital hyperargininaemia is a rare condition transmitted as an autosomal dominant trait. Following a one-year free interval, repeated vomiting, psychomotor regression and spastic paraparesis with talipes equinus progressively develop. The diagnosis, confirmed by arginine assays in blood and urine, is probably often missed. We report a case of homozygous arginase deficiency belatedly diagnosed at the age of 18 years, when treatment with sodium valproate (VPA) was instituted. This female patient presented with psychomotor regression since the age of 15 months and with paraparesis since she was 3 years' old. These symptoms rapidly became worse. At the age of 18 years, when she was bed-ridden, she was hospitalized for subintrant tonic seizures. EEG showed generalized, continuous spike-wave discharges at the rate of 3.5 c/s. Treatment with VPA was instituted. Five days later, she went into a state of stupor. Blood ammonia level was elevated at 362 mumol/l. VPA was discontinued, and this was followed by a regression of disturbances of consciousness and by a decrease in arterial ammoniaemia, although the ammonia levels remained high, fluctuating between 40 and 100 mumol/l. Several months after VPA treatment was interrupted, the patient had a second episode of stupor, and her ammoniaemia was 500 mumol/l. Serum amino acid chromatography showed hyperargininaemia at 501 mumol/l (N = 30-150 mumol/l). The diagnosis of arginase deficiency was confirmed by the rise of arginine in red cells, cerebrospinal fluid and urine and, above all, by the finding of a deeply depressed arginase activity in erythrocytes. In all cases of intolerance to VPA, arterial ammoniaemia should be measured after withdrawal of VPA, some time after the acute episode.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

[Renal manifestations in dermatomyositis and polymyositis].

Renal involvement is usually considered to be rare in dermatomyositis and polymyositis. The aim of this prospective study was to reevaluate the renal changes which may occur in these two conditions. Twenty patients, 12 with dermatomyositis and 8 with polymyositis of the adult type, were investigated to determine the incidence, the severity and course of renal disease. The 24 hour proteinuria was over 0.1 g in 14 cases and greater than 1 g/24 hours in 6 cases, including one patient with the nephrotic syndrome. Microscopic haematuria was noted in 3 cases and pyuria in 8 cases. Endogenous creatinine clearance was significantly decreased (p less than 0.001) in patients with dermatomyositis compared with a control group of subjects paired for age, sex, weight and serum creatinine concentration. Renal biopsy (5 cases) showed minimal glomerular lesions (2 cases), endocapillary proliferation and extramembranous glomerulonephritis (1 cases) and amyloid deposits (1 case). Although the proteinuria, haematuria and pyuria responded to steroid therapy (except in 1 case), the creatinine clearance remained decreased during the course of steroids.

Adult

[Meningoradiculitis after a tick bite. Study of 31 cases].

A retrospective study covering a period of 20 years identified reports on 31 cases of meningoradiculitis of the Garin-Bujadoux-Bannwarth type (MRGBB). Clinical, biological, electromyographic characteristics and course of the disease were studied. The most recent cases (n = 8) in 1984 and 1985 had serological tests for Borrelia Burgdorferi and half of the cases had negative results. Conversely, in some patients with meningoradiculitis, even in the absence of a tick bite or of migrating chronic erythema, serology was positive for Borrelia Burgdorferi antigen. The efficacy of antibiotic therapy against pain and on the quality and time of functional recovery justifies the use of this therapy under these two circumstances.

Bites and Stings

[Malignant diffuse peritoneal mesothelioma: clinical and therapeutic aspects. Apropos of a case with gelatinous ascites].

Peritoneal mesothelioma ranks second in frequency after pleural mesothelioma. A well-established clinical entity since 1960, mesothelioma is increasingly frequent due to improvements in diagnostic methods and to the fairly wide industrial use of asbestos, a well-known carcinogenic substance for mesothelial cells. The diagnosis of malignant peritoneal mesothelioma rests on a corpus of convergent data. Treatment consists of surgery combined with radiotherapy and chemotherapy. The mean survival from the time of diagnosis is 8-10 months.

Ascites

[Epirubicin in combination chemotherapy of metastasized breast cancer (VEC) and advanced ovarian cancer (PEC)].

Epirubicin was applied in combination with vincristine and cyclophosphamide (VEC) against metastatic cancer of the breast in 14 patients, against advanced cancer of the ovaries. Epirubicin was applied in combination with cisplatin and cyclophosphamide (PEC) in 12 patients. Treatment of metastatic cancer of the breast achieved retrogression in 64%, the tumor was brought to a standstill in 12%. Treatment of cancer of the ovaries achieved complete retrogression in 75%, the tumor was brought to a standstill in 8.3%. There was no incidence of considerable myelo- or cardiotoxic side effects resulting in a necessary interruption of treatment. The achieved results of this treatment correspond to those with doxorubicin combinations, the toxicity, however, is less.

Antineoplastic Combined Chemotherapy Protocols

[Regression of IgA kappa monoclonal gammopathy in IgA lambda Kahler's disease].

The authors report the case of a patient with a stable IgA kappa gammapathy over several years which finally evolved to an IgA lambda myeloma during which the initial gammapathy regressed. As the two gammapathies probably arose from two different cellular populations, the regression of the IgA kappa dysglobulinaemia is an additional argument in favour of the hypothesis that benign monoclonal gammapathies are the result of cellular hyperplasia.

Humans

[Bacteriologic surveillance of patients with infectious endocarditis. Value and limitations of the determination of minimal inhibitory concentration and serum bactericidal activity].

This is a retrospective study of the records of 17 patients who suffered from infectious endocarditis with positive blood cultures, and concerns the possibilities of predicting immediate infectious signs in patients:--by testing each of the antibiotics used for the minimal inhibitory concentration;--by determining the peak of the serum bactericidal activity of patients. All the patients were under observation, as outpatients, for more than a year and proved completely cured of their infectious endocarditis. A satisfactory minimal inhibitory concentration does not necessarily prevent an infectious evolution in the immediate future. The determination of the peak serum bactericidal activity does not reflect faithfully and instantaneously the anti-infectious action of the treatment; this is shown by the excellent and rapid evolution 13 times out 20 although serum bactericidal activity was considered insufficient (less than 1/8). This level of 1/8 is not a guarantee of therapeutic efficiency. Finally the antiinfectious treatment has been, in the majority of cases, determined by clinical signs and in very few cases by serum bactericidal activity results.

Adult

[Stromal endometriosis. A rare semimalignant tumor with a tendency to recur. Clinical follow-up over 17 years].

The authors report on a female patient with stromal endometriosis. Clinical observation at the authors' clinic extended over an unusually long period, i.e., 17 years. After describing the histology, clinical symptoms, and therapeutic possibilities, the disease course, the repeated operations necessary, and the histological findings are presented in detail. In spite of the rarity of stromatosis it should be included in the differential diagnosis when diagnosing uterine sarcoma. After histological confirmation and the completion of surgical treatment of a stromatosis, recurrence and malignancy must be considered as possibilities even many years later.

Adult

First case of acquired functional C1(-) INH deficiency: association with angioedema during Churg and Strauss vasculitis.

This report deals with the first case of acquired functional C1(-) INH deficiency with normal antigenic C1(-) INH level which was detected in a young girl with angioedema and Churg and Strauss vasculitis. This complement abnormality was associated with slightly depressed levels of CH50, C4 and C2, but a normal level of C3, and high levels of total IgE and IgM rheumatoid factors. Finally, most of these abnormalities disappeared after corticosteroid therapy and clinical improvement.

Adolescent

[Acute fatal non-occlusive intestinal ischemia during treatment with a cardiotonic glucoside].

After presenting the case of a 70 year old woman who presented a massive mesenteric infarction as a result of non-occlusive intestinal ischaemia ( NOII ), the authors review the principal features of this particular form of intestinal ischaemia. This condition is seen principally in elderly people with a syndrome of low cardiac output, resulting in marked mesenteric vasoconstriction. It is a complication of either severe decompensated heart disease treated with digitaloids or to a state of hypovolaemic or septic shock. The diagnosis of NOII should be suggested by the combination of abdominal signs with a state of shock and/or treated heart disease. The diagnosis is confirmed by selective mesenteric arteriography which reveals a patent but spastic vessel. The treatment is initially medical, consisting of the correction of haemodynamic disturbances and the in situ injection of vasodilator products, which may need to be completed by a surgical operation. However, the prognosis of NOII remains serious, particularly because of the frequent delay in making the diagnosis.

Acetyldigitoxins

[Study of genetic markers of hepatitis B virus in 204 persons working in hospitals].

In this study we have shown that 20% of the staff of the University Hospital Center at Nancy have at least one genetic marker for hepatitis B virus. We believe that, given this relatively high incidence of infection, members of a hospital staff at high risk for infection should be screened by serologic tests in order to immunize only susceptible individuals. Conversely, in populations with low incidences of infection, immunization without prior serologic tests is preferable as it is less expensive.

France

Syndesmo-odontoid joint and calcium pyrophosphate dihydrate deposition disease (CPPD).

Twenty-seven patients with definite or probable CPPS have been investigated with systematic frontal and lateral tomograms of the cervicooccipital region. In 12 cases there were calcifications of the syndesmo-odontoid region. These calcifications are of two different types: linear calcifications (5 cases) and mottled calcifications (7 cases). The anatomic location of these calcifications is the transverse ligament or other structures of the syndesmo-odontoid joint. Although this type of calcification has already been described in other rheumatic diseases, it must lead to search for CPPD in the usual locations.

Aged