Peripartum cardiomyopathy (PPCM) is a rare and life-threatening disease of unknown aetiology. The primary objective of this review was to analysed aetiopathogeneses, clinical presentation and ...diagnosis, as well as pharmacological, perioperative and intensive care management and prognosis of this pathology.
We undertook a systematic review of the literature using Medline, Google Scholar and PubMed searches.
Unlike other parts of the world in which cardiomyopathy are rare, dilated cardiomyopathy is a major cause of heart failure throughout Africa. Its aetiopathogenesis is still poorly understood, but recent evidence supports inflammation, viral infection and autoimmunity as the leading causative hypotheses. This diagnosis should be limited to previously healthy women who present with congestive heart failure (CHF) and decreased left ventricular systolic function in the last month of pregnancy or within 5 months after delivery. Recently, introduction of echocardiography has made diagnosis of PPCM easier and more accurate. Conventional treatment consists of diuretics, vasodilators, and sometimes digoxin and anticoagulants, usually in combination. Patients who fail to recover may require inotropic therapy. In resistant cases, newer therapeutic modalities such as immunomodulation, immunoglobulin and immunosuppression may be considered. Prognosis is highly related to reversal of ventricular dysfunction. Compared to historically higher mortality rates, recent reports describe better outcome, probably because of advances in medical care. Based on current information, future pregnancy is usually not recommended in patients who fail to recover normal heart function.
PPCM is a rare but serious form of cardiac failure affecting women in the last months of pregnancy or early puerperium. Its aetiopathogenesis is still poorly understood. Introduction of echocardiography has made diagnosis of PPCM easier and more accurate. Prognosis is highly related to reversal of ventricular dysfunction.
The objective of this work was to review current data about the pathophysiology, clinical features, and treatment of thrombotic microangiopathies.
Thrombotic microangiopathies are microvascular ...occlusive disorders characterized by systemic or intrarenal aggregation of platelets, thrombocytopenia, and mechanical injury to erythrocytes. In thrombotic thrombocytopenic purpura, systemic microvascular aggregation of platelets causes ischemia in the brain and other organs. In the hemolytic-uremic syndrome, platelet-fibrin thrombi occlude predominantly the renal circulation. Thrombotic microangiopathy is a rare disorder whose varied clinical manifestations result from the formation of platelet-rich thrombi within the microvasculature and consequent tissue ischemia. The clinical features are acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. This diagnosis is of considerable importance because of the possible fulminant clinical course. Some atypical forms may be unrecognized. Plasma exchange is the current reference treatment of thrombotic thrombocytopenic purpura. However, in the light of recent publications, either infusions of concentrates of purified enzyme or more intensive immunosuppressive therapy would be more specific.
Post traumatic renal artery thrombosis is rarely described in the literature. This pathology can result from stretch injury to inelastic intima of the renal artery, or by the direct flow to the ...abdomen causing compression injury to the renal artery against the vertebral column. However, the association of this pathology with hematologic diseases (in particular protein C deficit) was never described. We report an observation of a 28-year-old man with an uneventful history who was admitted to the intensive care unit for traumatic head injury associated with post traumatic renal artery thrombosis requiring nephrectomy. The etiologic investigation of this thrombo-embolic complication reveals a protein C deficit. Our patient was improved under treatment. This original observation confirms that post traumatic renal artery thrombosis can be associated with hematologic diseases (in particular protein C deficit).
We describe a large kindred of 6 patients with a slowly progressive autosomal recessive form of giant axonal neuropathy (GAN). The propositus presented with progressive infantile onset of distal ...amyotrophy of 4 limbs, brisk reflexes, diffuse fasciculations, bulbar signs, and deep sensory loss in both lower limbs. The EMG and nerve biopsy showed typical hypertrophic neuritis. In 4 patients, there were giant axons filled with neurofilaments, with normal conduction velocity. In the youngest boy, the neurologic deficit was less severe, and the nerve biopsy revealed only a few unmyelinated axons filled with neurofilaments. These cases appear to represent a different genetic defect from other reported cases of GAN.
Our aim was to study the susceptibility of Streptococcus pneumoniae to antibiotics in patients with pneumococcal meningitis and to search for the prognosis factors in those patients.
We have studied ...retrospectively 31 cases of pneumococcal meningitis. Comparaisons were performed with univariate analysis.
The mean age was 36.7 +/- 20.5 years (ranged: 9 and 78 years). The sex ratio was 3,4. The susceptibility of Streptococcus pneumoniae to penicillin G was affected in 10 cases (33% of isolated pneumococcus. The MIC to penicillin G was > or =2 in only one case. The hospital mortality was 26% (8/31). With univariate analysis, factors associated with death were: age > or =55 years (Ss p= 0,006, OR: 17.2 IC95%: 2.3-134), albuminorachie > or = 7 g/l (p = 0.002, OR: 22; IC95%: 1.9-2.51), shock (p = 0.031, OR: 6.7; IC95%: 1.05-42) and Glasgow Coma Score (GCS) < or =8 (p = 0.001, OR: 20; IC95%: 2.68-149).
No susceptibility to penicillin G is not associated with a worse outcome in patients with pneumococcal meningitis. An age > or =55 years, albuminorachie > or =7 g/l shock and Glasgow Coma Score < or =8 at admission were determinant of the prognosis in our study.
To analyze the clinico-biological manifestations, identify the causes and evaluate the outcome of patients with severe thrombotic microangiopathies admitted in a Tunisian intensive care unit.
...Retrospective study over a period of 10 years (1995-2004) in an intensive care unit.
Were included in this study 9 cases with a mean age of 29.2+/-9 years (range 15-44 years). Fever was observed in 5 patients, neurological impairment in 5 and digestive manifestations in 6. Haemolytic anaemia, thrombocytopenia and acute renal failure were observed in 100% of the cases. In our study, the aetiologies of thrombotic microangiopathies were: complicated pregnancy in 6 cases, systemic lupus erythematosus in 1 case. In contrast, no aetiology was found in 2 patients. Plasma exchange was performed in 5 patients, while 4 patients received only plasma infusion. After an average stay of 18+/-12.5 days, evolution was marked by the death 3 patients.
The incidence of severe thrombotic microangiopathies is rare in Tunisian ICU. The clinical manifestations are not specific. Despite the improvement in the outcome by exogenous plasma supply, thrombotic microangiopathies with severe organ dysfunctions leading to hospitalization in the intensive care unit are associated with a high mortality rate.
A histomorphometric analysis of the superficial peroneal nerve was made in 15 cases of leprosy. Thirteen patients presented clinical signs of leprous neuropathy, while the other two showed only ...cutaneous signs of leprosy. The presence of M. leprae in all the nerves sampled, and the appearance of the histologic lesions, made it possible to confirm the diagnosis of leprosy and to specify the type of leprosy in each case, even in the absence of clinical signs of leprous neuropathy. Correlation of the histomorphometric results with the duration of development of the disease and with the time elapsing before treatment showed, in the beginning stage, a considerable reduction in myelinated and unmyelinated never fibres and a proliferation of Schwann cells, as well as segmental demyelination and axonal degeneration of the teased fibres. When treated early, the evolution of the lepromatous type (LL) appears favourable, with apparent regeneration of the nerve fibres. When treatment is not instituted early, gradual loss of nerve fibres, axonal degeneration of all the teased fibres, proliferation of the Schwann cell processes devoid of axons, and increase in endoneural connective tissue lead to a severe degeneration of the nerve. This unfavorable development appears to progress faster in the absence of treatment or when treatment is irregular. In the borderline lepromatous (BL) and borderline tuberculoid (BT) types, nerve degeneration appears to be more rapid than in the type LL.
OBJECTIVE: To report the clinical findings and the genetic linkage mapping of an autosomal recessive cerebellar ataxia associated to peripheral neuropathy, showing an early onset cerebellar ataxia ...with retained tendon reflexes (EOCA) phenotype. BACKGROUND: EOCA is a clinical syndrome delimited by Harding distinguished from Friedreich's ataxia (FA) mainly by the preservation of tendon reflexes. Molecular genetic study of patients with EOCA has demonstrated genetic heterogeneity. A form of autosomal recessive spastic ataxia has been described in Charlevoix Saguenay area in Quebec (ARSACS); the gene responsible has been mapped to chromosome 13q. METHODS: Genetic linkage analysis was performed on 18 members of a large family including 8 of 9 members with EOCA. After exclusion of FA and ataxia with vitamin E deficiency loci as well as loci of autosomal dominant cerebellar ataxias, we performed a linkage analysis to markers of 13q11‐12 region. RESULTS: The 9 affected members of this family showed stereotyped clinical features with cerebellar ataxia, pyramidal syndrome, and a variable degree of axonal peripheral neuropathy. Linkage was detected between the disease locus and the microsatellite marker D13S232. Surrounding markers to D13S232 confirmed the linkage and showed the homozygosity of the affected members. CONCLUSION: The family reported here showed the same locus as autosomal recessive spastic ataxia Charlevoix Saguenay disease.
