Neonatal limb gangrene and renal vein thrombosis

A 3-day-old male infant born to a non-diabetic mother was admitted to the University Hospital with gangrene of the right forearm and some gangrenous patches of the left forearm. This was associated with left renal vein thrombosis. Screening tests for coagulation disorders gave normal results. The gangrenous right forearm was amputated and subsequently grafted. The skin lesions of the left forearm were successfully grafted. The renal system recovered well on supportive measures. The course of hospitalization and subsequent follow-up assessment at 3 months of age were satisfactory.     

Muscle weakness and cocontraction in upper limb hemiparesis: relationship to motor impairment and physical disability

The purpose of this article is to describe the relationship between poststroke upper limb muscle weakness and cocontraction, and clinical measures of upper limb motor impairment and physical disability. Electrormyographic (EMG) activity of the paretic and nonparetic wrist flexors and extensors of 26 chronic stroke survivors were recorded during isometric wrist flexion and extension. The root mean square (RMS) of the EMG signal was used as a measure of strength of contraction. A ratio of RMS of antagonist and agonist muscles was used as a measure of cocontraction. Upper limb motor impairment and physical disability were assessed with the Fugl-Meyer motor assessment (FMA) and the arm motor ability test (AMAT), respectively. The strength of muscle contraction was significantly stronger in the nonparetic limb (P < 0.001). The degree of cocontraction was significantly greater in the paretic limb (P < 0.001). The strength of muscle contraction in the paretic limb correlated significantly with FMA (r = 0.62 to 0.87, P < or = 0.001) and AMAT (r = 0.66 to 0.80, P < or = 0.001) scores. Similarly, the degree of cocontraction correlated significantly with FMA (r = -0. 70 to -0.64, P < or = 0.001) and AMAT (r = -0. 72 to -0.62, P < or = 0.001) scores. Muscle weakness and degree of cocontraction correlate significantly with motor impairment and physical disability in upper limb hemiplegia. This relationship may provide insights toward development of specific interventions. However, additional studies are needed to demonstrate a cause and effect relationship.     

Trauma-associated growth of suspected dormant micrometastasis

Background  

Cancer patients may harbor micrometastases that remain dormant, clinically undetectable during a variable period of time. A traumatic event or surgery may trigger the balance towards tumor growth as a result of associated angiogenesis, cytokine and growth factors release.     

Transcatheter arterial chemoembolization in unresectable cholangiocarcinoma: initial experience in a single institution

PURPOSE: Unresectable cholangiocarcinoma carries a dismal prognosis, with median survival times ranging from 6 to 12 months from the time of diagnosis. Palliative therapies have been disappointing and have not been shown to significantly prolong survival. Conversely, transcatheter arterial chemoembolization (TACE) has been effective in prolonging the lives of patients with hepatocellular carcinoma but has not been used against cholangiocarcinoma. Therefore, the purpose of the present study was to assess the safety and efficacy (ie, survival) of TACE in patients with unresectable intrahepatic cholangiocarcinoma. MATERIALS AND METHODS: Seventeen patients with unresectable cholangiocarcinoma were treated with one or more cycles of TACE between 1995 and 2004 at our institution. Follow-up imaging was performed on all patients 4-6 weeks after each TACE procedure to determine tumor response and need for further treatment. Survival was calculated with use of the Kaplan-Meier survival curve. RESULTS: The median survival for 17 patients treated with TACE was 23 months. Two patients with previously unresectable disease underwent successful resection after TACE. The procedure was well tolerated by 82% of the patients, who experienced no side effects or mild side effects that quickly resolved with conservative therapy alone. Two patients had minor complications (12%), which were managed successfully, and one had a major complication that resulted in a fatal outcome. This patient had a rapidly declining course from the time of diagnosis and died shortly after TACE. CONCLUSIONS: The results suggest that TACE was effective at prolonging survival of patients with unresectable cholangiocarcinoma. Therefore, for these patients, TACE may be an appropriate palliative therapy.     

Sjogren's syndrome: concomitant H. pylori infection and possible correlation with clinical parameters

OBJECTIVE: To identify whether or not there are clinical markers that correlate with Helicobacter pylori (H. pylori) infection in patients with Sjogren's syndrome (SS) and its implication to handling this group of patients. METHODS: Four groups of patients were studied. Group 1, 36 patients with primary SS; group 2, 31 patients with secondary SS; group 3, 46 patients with various connective tissue diseases not suffering from sicca symptoms, and group 4, 64 healthy controls. Clinical assessment was done and a score for disease manifestation was given for every SS patient. Serum IgG and IgM antibodies to H. pylori were assessed by ELISA. RESULTS: The prevalence and mean titer of H. pylori infection in patients with SS in group 1 (80.6%) and 2 (71%) were significantly higher than in group 3 (60.9%) and 4 (56.3%) (P < 0.01). There was significant correlation between H. pylori infection and age, disease duration, global score for disease status and C-reactive protein (CRP) in SS patients. On the other hand, there was no significant correlation with body mass index, and erythrocyte sedimentation rate (ESR). CONCLUSION: Patients with SS are more prone to have H. pylori infection in comparison to other connective tissue diseases. Serum antibody titer to H. pylori correlated with index for clinical disease manifestations, age, disease duration and CRP. Assessment of H. pylori infection in older patients suffering from active SS for a relatively long duration is recommended, especially those suffering form primary SS for more than 3 years.     

Intra-familial clinical heterogeneity: absence of genotype-phenotype correlation in primary hyperoxaluria type 1 in Israel

BACKGROUND/AIMS: Primary hyperoxaluria type 1 (PH1) is caused by the deficiency of the liver enzyme alanine:glyoxylate aminotransferase which results in increased synthesis and excretion of oxalate. The clinical manifestations of PH1 are heterogeneous with respect to the age of onset and rate of progression. The aim of this study was to investigate possible relationships between a given genotype, the biochemical profile and the clinical phenotype. METHODS: We conducted a study of 56 patients from 22 families with PH1 from Israel. The clinical and biochemical data were compiled and the genotype was determined for each family. RESULTS: The prevalent phenotype was of early onset with progression to end-stage renal disease during the first decade of life. Fifteen PH1-causing mutations were detected in 21 families: 10 were first described in this patient population. Marked intra-familial clinical heterogeneity was noted, meaning that there was no correlation between a given genotype and the phenotype. CONCLUSIONS: The clinical course of patients with PH1 is not dictated primarily by its genotype. Other genetic and/or environmental factors play a role in determining the ultimate phenotype.