Jean Kemeny - Academia.edu (original) (raw)
Papers by Jean Kemeny
Revue du Rhumatisme, 2008
Journal of Neuropsychiatry and Clinical Neurosciences, 2000
SIR: Dementia with Lewy bodies (DLB) is a lately identified concept based on a clinical and patho... more SIR: Dementia with Lewy bodies (DLB) is a lately identified concept based on a clinical and pathological correlation: progressive disabling mental impairment with peculiar features such as hallucinations, fluc- tuating cognition or motor parkin- sonism, and cortical with or with- out subcortical Lewy bodies. 1 We report briefly two cases with un- usual features of DLB. Case Reports
Revue De Medecine Interne - REV MED INTERNE, 1984
Mots cl6s : maladie de von Recklinghausen, cardiomyopathie hypertrophique, phacomatose.
Revue De Medecine Interne - REV MED INTERNE, 1983
European Journal of Endocrinology, 1987
Two patients, aged 32 and 35 years, presented with gynaecomastia and a unilateral testicular tumo... more Two patients, aged 32 and 35 years, presented with gynaecomastia and a unilateral testicular tumour which proved to be a Leydig cell tumour. Pre-operative samples taken at 08.00 h on different days showed marked elevation of plasma oestradiol in the first patient, and very slight irregular oestradiol elevation in the second, plasma oestrone within the normal range in both patients, reduced plasma testosterone in the first patient and reduced or normal testosterone in the second, and low or low-normal serum LH and FSH in both patients. One of the patients received an oral dose of 100 mg of clomiphene citrate for 3 consecutive days which induced a rise in LH and FSH and a decrease in the 17-hydroxyprogesterone/androstenedione ratio. These data suggest the inhibiting effect of endogenous hyperoestrogenism on testicular steroidogenesis owing to both the reduction of gonadotropin secretion and a direct local negative effect on C 17,20-lyase. After human chorionic gonadotropin stimulation, oestradiol response was increased and abnormally prolonged, a finding which may be helpful when diagnosing a feminizing Leydig cell tumour; testosterone reached normal values. After removal of the tumoural testis, gynaecomastia regressed within a few days, gonadotropins increased, oestrogens dropped, testosterone and 5 alpha-dihydrotestosterone normalized in one patient but remained low in the other at day 30. The Leydig cells outside the tumour appeared morphologically normal, but the count gave evidence of juxtatumoural Leydig cell hyperplasia in areas where the tumour was well encapsulated while showing a significant reduction at a distance from the tumour and in the contralateral testis by comparison with control testes.(ABSTRACT TRUNCATED AT 250 WORDS)
European Archives of Oto-Rhino-Laryngology, 2002
Laryngeal chondrosarcoma is a rare tumor that is known for its indolent course and its tendency t... more Laryngeal chondrosarcoma is a rare tumor that is known for its indolent course and its tendency to ultimate recurrence. According to the actually adopted classification, 95% of the reported cases are of a low-grade type. A consensus recognizing conservative surgery as the most reasonable treatment for these lesions has almost been reached. However, fear of jeopardizing the patency of the laryngeal airway as a result of a wide cricoid excision and also the fear of repeated recurrences could still push some surgeons to perform a total laryngectomy in the case of laryngeal chondrosarcoma. After a brief review of the literature, we will present five cases of laryngeal chondrosarcoma that were treated and followed at the Clermont-Ferrand University Medical Center over the last two decades. These cases exhibit many of the clinicopathologic features of the tumor and illustrate the pitfalls of diagnosis and treatment. In light of this presentation, we will discuss the widely accepted management as well as a newly suggested treatment modality for this disease.
American Journal of Kidney Diseases, 2010
In alkaptonuria, the absence of homogentisic acid oxidase results in the accumulation of homogent... more In alkaptonuria, the absence of homogentisic acid oxidase results in the accumulation of homogentisic acid (HGA) in the body. Fatal disease cases are infrequent, and death often results from kidney or cardiac complications. We report a 24-year-old alkaptonuric man with severe decreased kidney function who developed fatal metabolic acidosis and intravascular hemolysis. Hemolysis may have been caused by rapid and extensive accumulation of HGA and subsequent accumulation of plasma soluble melanins. Toxic effects of plasma soluble melanins, their intermediates, and reactive oxygen side products are increased when antioxidant mechanisms are overwhelmed. A decrease in serum antioxidative activity has been reported in patients with chronic decreased kidney function. However, despite administration of large doses of an antioxidant agent and ascorbic acid and intensive kidney support, hemolysis and acidosis could not be brought under control and hemolysis led to the death of the patient.
The American Journal of Dermatopathology, 1991
Nature Medicine, 2003
PrP sc accumulation in sympathetic ganglia of vCJD, as shown by immunohistochemistry. (a) Prion p... more PrP sc accumulation in sympathetic ganglia of vCJD, as shown by immunohistochemistry. (a) Prion protein immunostaining of paraffin-embedded tissue fixed in 10% formalin, using a monoclonal antibody to PrP, amino acids 142-160 (12F1O; Commissariat à l'Energie Atomique) . Sections were stained with avidin-biotin horseradish peroxidase complex procedure, using nickeldiaminobenzidine as a cosubstrate. PrP sc immunostaining is visible as black precipitate. Sections were counterstained with Harris hematoxylin. This immunostaining PrP procedure was optimized to detect PrP sc accumulation as florid plaques in vCJD isocortex and vacuolar deposits around spongiform change in sporadic CJD isocortex. In vCJD, strong PrP sc immunoreactivity is seen as membrane-associated deposits in sympathetic neurons of celiac and stellate ganglia. This staining was not observed in the ganglia of sporadic CJD and control cases. (b) Tyrosine hydroxylase and PrP immunostaining of adjacent sections of a celiac ganglion from a vCJD case. Neurons immunoreactive for PrP sc were heavily stained using a polyclonal antibody to tyrosine hydroxylase (Pel-Freez Biologicals). Scale bar, 40 µm.
Revue du Rhumatisme, 2008
Journal of Neuropsychiatry and Clinical Neurosciences, 2000
SIR: Dementia with Lewy bodies (DLB) is a lately identified concept based on a clinical and patho... more SIR: Dementia with Lewy bodies (DLB) is a lately identified concept based on a clinical and pathological correlation: progressive disabling mental impairment with peculiar features such as hallucinations, fluc- tuating cognition or motor parkin- sonism, and cortical with or with- out subcortical Lewy bodies. 1 We report briefly two cases with un- usual features of DLB. Case Reports
Revue De Medecine Interne - REV MED INTERNE, 1984
Mots cl6s : maladie de von Recklinghausen, cardiomyopathie hypertrophique, phacomatose.
Revue De Medecine Interne - REV MED INTERNE, 1983
European Journal of Endocrinology, 1987
Two patients, aged 32 and 35 years, presented with gynaecomastia and a unilateral testicular tumo... more Two patients, aged 32 and 35 years, presented with gynaecomastia and a unilateral testicular tumour which proved to be a Leydig cell tumour. Pre-operative samples taken at 08.00 h on different days showed marked elevation of plasma oestradiol in the first patient, and very slight irregular oestradiol elevation in the second, plasma oestrone within the normal range in both patients, reduced plasma testosterone in the first patient and reduced or normal testosterone in the second, and low or low-normal serum LH and FSH in both patients. One of the patients received an oral dose of 100 mg of clomiphene citrate for 3 consecutive days which induced a rise in LH and FSH and a decrease in the 17-hydroxyprogesterone/androstenedione ratio. These data suggest the inhibiting effect of endogenous hyperoestrogenism on testicular steroidogenesis owing to both the reduction of gonadotropin secretion and a direct local negative effect on C 17,20-lyase. After human chorionic gonadotropin stimulation, oestradiol response was increased and abnormally prolonged, a finding which may be helpful when diagnosing a feminizing Leydig cell tumour; testosterone reached normal values. After removal of the tumoural testis, gynaecomastia regressed within a few days, gonadotropins increased, oestrogens dropped, testosterone and 5 alpha-dihydrotestosterone normalized in one patient but remained low in the other at day 30. The Leydig cells outside the tumour appeared morphologically normal, but the count gave evidence of juxtatumoural Leydig cell hyperplasia in areas where the tumour was well encapsulated while showing a significant reduction at a distance from the tumour and in the contralateral testis by comparison with control testes.(ABSTRACT TRUNCATED AT 250 WORDS)
European Archives of Oto-Rhino-Laryngology, 2002
Laryngeal chondrosarcoma is a rare tumor that is known for its indolent course and its tendency t... more Laryngeal chondrosarcoma is a rare tumor that is known for its indolent course and its tendency to ultimate recurrence. According to the actually adopted classification, 95% of the reported cases are of a low-grade type. A consensus recognizing conservative surgery as the most reasonable treatment for these lesions has almost been reached. However, fear of jeopardizing the patency of the laryngeal airway as a result of a wide cricoid excision and also the fear of repeated recurrences could still push some surgeons to perform a total laryngectomy in the case of laryngeal chondrosarcoma. After a brief review of the literature, we will present five cases of laryngeal chondrosarcoma that were treated and followed at the Clermont-Ferrand University Medical Center over the last two decades. These cases exhibit many of the clinicopathologic features of the tumor and illustrate the pitfalls of diagnosis and treatment. In light of this presentation, we will discuss the widely accepted management as well as a newly suggested treatment modality for this disease.
American Journal of Kidney Diseases, 2010
In alkaptonuria, the absence of homogentisic acid oxidase results in the accumulation of homogent... more In alkaptonuria, the absence of homogentisic acid oxidase results in the accumulation of homogentisic acid (HGA) in the body. Fatal disease cases are infrequent, and death often results from kidney or cardiac complications. We report a 24-year-old alkaptonuric man with severe decreased kidney function who developed fatal metabolic acidosis and intravascular hemolysis. Hemolysis may have been caused by rapid and extensive accumulation of HGA and subsequent accumulation of plasma soluble melanins. Toxic effects of plasma soluble melanins, their intermediates, and reactive oxygen side products are increased when antioxidant mechanisms are overwhelmed. A decrease in serum antioxidative activity has been reported in patients with chronic decreased kidney function. However, despite administration of large doses of an antioxidant agent and ascorbic acid and intensive kidney support, hemolysis and acidosis could not be brought under control and hemolysis led to the death of the patient.
The American Journal of Dermatopathology, 1991
Nature Medicine, 2003
PrP sc accumulation in sympathetic ganglia of vCJD, as shown by immunohistochemistry. (a) Prion p... more PrP sc accumulation in sympathetic ganglia of vCJD, as shown by immunohistochemistry. (a) Prion protein immunostaining of paraffin-embedded tissue fixed in 10% formalin, using a monoclonal antibody to PrP, amino acids 142-160 (12F1O; Commissariat à l'Energie Atomique) . Sections were stained with avidin-biotin horseradish peroxidase complex procedure, using nickeldiaminobenzidine as a cosubstrate. PrP sc immunostaining is visible as black precipitate. Sections were counterstained with Harris hematoxylin. This immunostaining PrP procedure was optimized to detect PrP sc accumulation as florid plaques in vCJD isocortex and vacuolar deposits around spongiform change in sporadic CJD isocortex. In vCJD, strong PrP sc immunoreactivity is seen as membrane-associated deposits in sympathetic neurons of celiac and stellate ganglia. This staining was not observed in the ganglia of sporadic CJD and control cases. (b) Tyrosine hydroxylase and PrP immunostaining of adjacent sections of a celiac ganglion from a vCJD case. Neurons immunoreactive for PrP sc were heavily stained using a polyclonal antibody to tyrosine hydroxylase (Pel-Freez Biologicals). Scale bar, 40 µm.