Parvovirus B19 infection in AIDS patients

We report a 16-year-old boy, born to consanguineous parents, with mental retardation, gait disturbances and dysarthria; brain magnetic resonance showed features consistent with rhombencephalosynapsis. This condition is characterised by a hypoplastic single-lobed cerebellum. The interest of this case is the presence of common ancestors, pointing to an autosomal recessive inheritance of the malformation.     

Hepatitis B and C virus infections in children with congenital coagulation disorders

The prevalence of hepatitis B and C virus infections, transmitted by blood transfusions, was studied in 79 children with congenital coagulation disorders. Twenty nine percent had evidences of hepatitis B virus infection and 52% evidences of hepatitis C virus infection. Older children and those with the higher number of transfusions had the highest rates of infections. It is concluded that children with congenital coagulation disorders constitute a high risk group for hepatitis B and C virus infections.     

Parvovirus B19 quiescence during the course of human immunodeficiency virus infection in persons with hemophilia

One hundred and seventy patients with major lower limb amputation (MLLA) presenting to The National Prosthetic-Orthotic Centre (NPOC) in Khartoum over a 1-year period were studied. There were 141 males and 29 females giving a M:F ratio of 4.9: 1.0, with mean age of 37 years (range 5-72 years). Forty-one patients (24%) underwent amputation of diabetic septic foot, 30 patients (17.6%) underwent amputation as a result of trauma from road traffic accidents and Madura foot, and war injuries accounted for 29 amputations (17%). One hundred and eleven patients had below knee amputation (BKA), 52 had above knee amputation (AKA) and seven patients had Syme's amputation. Diabetic amputees had higher rate of revisional surgery compared with others because of sepsis and/or flap necrosis. Stump pain was reported by amputees with excessive scarring of the stump and those with undue prominence of bony ends. There are two types of prostheses provided by the NPOC for both BKA and AKA: the peg leg and the conventional prostheses. The Syme's amputees were fitted with either simple hoof or articulated prostheses with solid ankle cushion heel (SACH). The peg leg consists of a leather lined side bearing metal socket connected to a rocker base by side steels. It is used by the country natives as it suits different weather and job conditions, particularly farming, and it can be repaired locally. The urban population use the conventional prostheses which is lighter in weight, can be put on and taken off easily and is cosmetically acceptable. However, these prostheses are more expensive and require frequent repair or replacement. The functional outcome of patient's rehabilitation with the prostheses was significantly affected by the level and indication of amputation. Those with BKA and those amputated because of trauma or Madura foot experienced better functional outcome compared with the diabetics, independent of age. 50% of patients with the AKA and 19% of those with BKA reported poor functional outcome. Surgeons should be more involved with the long-term evaluation of functional outcome in such patients, to offer help if feasible and to modify their technique for future procedures.     

Parvovirus B19 as infectious agent in infants

OBJECTIVE: Since its casual discovery and implication as a human pathogen that provokes transitory aplastic crises and infectious erythema, the B19 parvovirus has been related to a wide spectrum of diseases. To better understand this clinical diversity, we reviewed the cases of a serology positive infants admitted to the hospital. PATIENTS AND METHODS: From January 1992 to June 1995, all clinical charts were reviewed and we obtained 15 patients that had positive IgM antibodies by immunoenzyme assay. RESULTS: The mean age was 12.2 months. No sex differences were seen. The incidence was higher in winter months. Over 50% of the patients belong to the last year studied. Clinical findings included 5 cases of arthritis (one juvenile rheumatoid arthritis, one polyarticular syndrome and 3 nonspecific forms), hematology disturbances in 5 cases (1 case of erythrophagocytosis, 1 of thrombocytopenic purpura, 2 of anemia and 1 chronic neutropenia), 3 cases of febrile syndrome, 1 liver dysfunction, and 1 neuromyelitis. Complementary exams were not significant and follow-up in all infants was satisfactory. CONCLUSIONS: The B19 parvovirus, a poorly understood virus, is related to many clinical situations where is true significance remains unknown.     

Seroprevalence of B19 parvovirus infection in patients with acute polyarthritis

Over a period of 29 months, from January 1991 to December 1994, all cases of acute polyarthritis seen at the Rheumatology Service in our Institution were studied to determine the seroprevalence of parvovirus B19 (B19) infection. The variables studied included: age and sex of patients, presence of fever and rash, Anti-B19 IgM and IgE serological determinations (ELISA, Mardix Lab.), follow-up time and final diagnosis. The study included 36 patients (22 women and 14 men, mean age 34 +/- 19 years). Thirteen and seven patients had fever and cutaneous rash, respectively. Anti-B19 IgM serology was positive in 4 patients; in 2 of them IgG seroconversion was confirmed. The mean follow-up time was 14 +/- 9 months. Final diagnoses included undifferentiated polyarthritis, rheumatoid arthritis, B19 polyarthritis, systemic lupus erythematosus, and miscellaneous in 19, 7, 4, 2, and 4 patients, respectively. Seroprevalence of B19 infection in acute polyarthritis in our area was 11%, approximately.     

Parvovirus B19-antibody serosurvey in 500 developmentally disabled subjects

Inflammatory cytokines (interleukin [IL]-1 and tumor necrosis factor [TNF]) have specific inhibitors (IL-1 receptor antagonist [IL-1Ra] and TNF-soluble receptors), the concentration of which can indicate activation and regulation of this system. We measured IL-1 and IL-1Ra in the cerebrospinal fluid (CSF) of HIV-infected patients and seronegative controls. High IL-1Ra concentrations were found in samples from patients with opportunistic meningoencephalitis, even in the presence of normal cell count and protein content, not in samples from patients with leucoencephalopathies or controls. Therefore, IL-1Ra appears to be a sensitive marker of inflammation in the central nervous system. In contradistinction to previous results obtained from blood measurement, IL-1alpha and IL-1beta remained below detectable levels in all cases, suggesting that IL-1 may be regulated differently in the central nervous system and in the blood.     

Parvovirus B19 in the Czech Republic. Seroepidemiologic study

Two rare cases of strabismus resulting from contracture of the extraocular rectus muscles after retrobulbar anesthesia for cataract surgery are described. Clinical signs in both cases suggested that the development of the impaired function of the lateral and superior rectus muscles followed the same pattern: initial stimulation followed by paretic and restrictive stages. Abnormal enlargement of the muscles was identified by computed tomography (CT) and magnetic resonance imaging (MRI). The data indicate that the strabismus was the result of direct injection of anesthetics into the rectus muscle.     

Changes in blood coagulation in HIV infection

The human immunodeficiency virus (HIV) infection is becoming more complex. Hemostatic abnormalities occur frequently in the patient with HIV. HIV-related thrombocytopenia (Tr-HIV) is the most common hemostatic disorder with a high morbidity and affects patients from every risk group independently of age, sex, or stage of infection. Two mechanisms are responsible for the Tr-HIV: bone marrow failure and immunological disorders, namely, circulating immune complex deposited on the platelet membrane and the production of autoantibodies directed against platelets. The treatment of choice is zidovudine; other available options are not as effective as zidovudine. In addition, there are some abnormalities in the fluid phase of the coagulation cascade which can produce bleeding or thrombosis in the HIV patient. The most common are a prolonged partially activated thromboplastin time test, the production of a lupic anticoagulant and anticardiolipin antibodies, and several abnormalities in the natural-occurring anticoagulants. The thrombotic thrombocytopenic purpura recently associated with HIV has a clinical presentation and treatment alternatives that closely resemble those for the classical disease. The knowledge of these hemostatic abnormalities in the HIV seropositive patient allows a more rational care of these patients.     

Parvovirus B19 infection-related complications in renal transplant recipients: treatment with intravenous immunoglobulin

BACKGROUND: Chronic red cell aplasia can develop in immunocompromised patients including transplant recipients infected with parvovirus B19 (PV B19). Renal involvement with PV B19 infection is not well-recognized. METHODS: We diagnosed erythroid hypoplasia associated with PV B19 infection in three renal transplant recipients; one of them developed de novo collapsing glomerulopathy. These patients were treated with intravenous immunoglobulin (IVIG). RESULTS: In two patients, anemia responded promptly to IVIG therapy. One of them had recurrence of anemia that responded to a second course of IVIG. Despite IVIG treatment, persistent infection with PV B19, recurrent anemia, and de novo collapsing glomerulopathy leading to allograft failure developed in the third patient, who had received the most intense immunosuppression. CONCLUSIONS: These findings indicate that PV B19 infection in transplant recipients can cause chronic red cell aplasia that generally responds to IVIG therapy. In some patients, particularly those who are heavily immunosuppressed, infection may persist despite treatment. As the cellular receptor for PV B19 is expressed in the kidney, persistent infection may result in development of glomerulopathies in these patients.     

The chronocorrection of disorders in the blood coagulation rhythm with kurantil in patients with decompensated rheumatic heart defects

The circadian pattern of hemocoagulation was studied in patients with decompensated rheumatic heart disease (DRHD) concurrent with stages I-II circulatory failure (CF) during complex treatment or medical treatment with disaggregants. Biorhythmological studies demonstrated that in patients with DRHD and CF chronotherapy with curantyl had some advantages over the traditional therapy during complex drug therapy. In these patients, the chronopatterns of circadian rhythms of hemocoagulative parameters tended to normalize under the influence of curantyl chronotherapy, by diminishing the signs of external desynchronization. Advantages of chronotherapy over the traditional treatment found in patients with DRHD and stages I-II CF, as manifested by its clinical effect in shorter periods (on days 4-5) when small daily and course doses of the drug were used. Based on the biorhythmological studies of hemostatic parameters, a method of curantyl chronotherapy was developed for patients with DRHD and stages I-II CF, which may optimize the therapeutical process in patients with this abnormality.     

Anticomplementary activity in serum samples from patients with acute parvovirus B19 infection

Of 65 serum samples submitted for diagnostic purposes which proved to be anti-complementary by complement fixation test, 49 were parvovirus B19 IgM positive. Forty four of the 49 serum samples were from patients with arthropathy. Acute parvovirus B19 infection should be suspected when a patient has symptoms of disease of the joints and the serum is anticomplementary.     

Mycobacterial infection in patients with hematologic disorders

In patients with hematologic disorders, cellular immunity may be suppressed by the disease itself or by treatment with adrenal corticosteroids, immunosuppressants, or anticancer agents. Miliary tuberculosis developing in such compromised hosts is cryptic, and thus its diagnosis is difficult to make. Miliary tuberculosis will be fatal if it cannot be detected. Therefore, the possibility of mycobacterial infection should always be kept in mind when treating patients with hematologic disorders. However, such patients often have a poor prognosis, even if they have already been diagnosed with miliary tuberculosis. Prophylactic medication has been proved to be effective against mycobacterial infection with hematologic disorders. Thus, the importance of prophylactic medication should be emphasized.     

Epilepsy and disorders of cortical development in children with congenital cytomegalovirus infection

INTRODUCTION: Neuroimaging and experimental studies have related cytomegalovirus (CMV) to certain neuronal migration disorders. MATERIAL AND METHODS: To define the electroclinical picture of children with epilepsy associated with disorders of cortical development (DCD) and congenital CMV infection, we conducted a clinical, electroencephalographic and neuroradiological study of 10 children with this condition. RESULTS: Eighty per cent of them had dismorphic traits, or malformations outside CNS. All showed other neuroradiological signs (cerebral calcification, white matter damage, porencephaly). Six patients with bihemispheric DCD (agyria-pachigyria, 2; 'poligyria', 1; schizencephaly, 1; bilateral opercular DCD, 2) showed: Tetraparesis, severe or profound mental deficiency, early onset epilepsy (mean age at onset: 11 months) with spasms, tonic seizures, partial seizures, and multifocal paroxysms or unusual diffuse sharp Alfa-Beta EEG activity. One child developed Epilepsia Partialis Continua. Children with bilateral opercular DCD evolved to a continuous spike and wave (SW) electrical status during wakefulness and sleep, linked to a worsening of psychomotor derangement. Four patients with unilateral hemispheric DCD (pachigyric or 'poligyric') showed: Congenital hemiparesis, mild intellectual deficiency, motor seizures (orofacial, hemiclonic, generalized) beginning in the third year of live, atypical absences with focal phenomena, frequent focal rhythmic SW discharges during wakefulness, and continuous SW status during sleep (CSWS). CONCLUSIONS: A wide spectrum of DCD due to congenital CMV infection is documented. Characteristic electroclinical pictures related to the extent and topographical distribution of the DCD are recognized, which may lead to an appropriate diagnosis and prognosis.     

Diagnosis and therapy of blood coagulation disorders in intrahepatic cholestasis

The reduction of prothrombin level below 5% in a patient with intrahepatic cholestasis of pregnancy is reported. The necessity of controlling the Quick level or better factors II, VII, IX and X is discussed. A well-timed Vitamin K therapy in all cases with impaired secretion of bile during pregnancy is recommended.     

Transjugular liver biopsy is safe and diagnostic for patients with congenital bleeding disorders and hepatitis C infection

The prevalence of chronic hepatitis C virus (HCV) infection among patients with severe congenital bleeding disorders is as high as 98%. Advances in HCV treatment currently result in sustained virological response rates of > or =50%. Recent recommendations have reaffirmed that liver biopsy, which provides a direct histological assessment of liver inflammation and fibrosis, is still important for accurate diagnosis and therapeutic decision making. Percutaneous liver biopsy is a simple, standardized procedure that can be performed rapidly and relatively inexpensively, and has been safely performed in patients with congenital coagulopathies. However, the safety and efficacy of the transjugular approach (transjugular liver biopsy, TJLB), recommended for patients with acquired coagulopathies, has only been minimally studied in the congenital bleeding diathesis population. We now report our institutional experience with TJLB in 13 such adult patients (mean age 33 years) with severe/mild haemophilia A/B (10); von Willebrand disease (1); factor V deficiency (1) and factor XIII deficiency (1). Data were collected by retrospective chart review and the TJLB was performed according to institutional protocol as described. Haemostasis prophylaxis was given for 1-5 days. Patients were hospitalized for < or =48 h and all tolerated the procedure without bleeding. Three patients experienced self-limited abdominal discomfort; one episode was accompanied by transient transaminaemia. Diagnostic specimens were obtained from all patients and were instrumental in the therapeutic decision-making process. We suggest that with a co-ordinated multidisciplinary approach to care, TJLB is a safe, effective and potentially cost-effective alternative to the percutaneous approach in the congenital bleeding disorders population.