Sneddon's syndrome: clinical and laboratory analysis of 10 cases

Sneddon's syndrome is characterized by livedo reticularis and cerebrovascular lesions. We report the cases of women (mean age, 36.2 +/- 8.1 years) diagnosed with Sneddon's syndrome based on the presence of livedo reticularis and characteristic cerebrovascular findings. Seven of these patients had cerebral infarcts on cranial computed tomography scan. Antiphospholipid antibodies were positive in 6 of these cases. Three cases had abnormal levels of antithrombin III. Analyses of chromosome 6 revealed no abnormalities. In 3 of the cases, investigation of the pedigrees revealed autosomal dominant traits. Two cases had epilepsy, and 3 had migraine. One case with migraine also had myasthenia gravis. In addition, we detected inferior altudinal hemianopia in 2 cases, cognitive functional disorder in 3 and depression in 2. Based on these findings, the entire vascular, haematologic, neurologic, and dermatologic systems should be evaluated in patients diagnosed with Sneddon's syndrome.     

Chronic subdural haematoma following caesarean section under spinal anaesthesia

Intracranial subdural haematoma is a rare complication of spinal anaesthesia. This report describes the case of a 31-year-old woman who presented with post partum headache following spinal anaesthesia for caesarean section. Bilateral haematomata were evacuated via burr-holes performed under total intravenous anaesthesia and the patient made a complete and uneventful recovery. The recognized causes of subdural haematoma are discussed.     

Renal resistive indexes and some renal functions in liver cirrhotic children

BACKGROUND: The aim of the present study was to investigate renal vascular resistive changes in children with different stages of liver cirrhosis without obvious renal failure. METHODS: Twenty-nine children (14 girls, 15 boys, mean age 11.6 years) with cirrhosis and 20 healthy children (mean age 10.3 years) were investigated for renal vascular resistance with Doppler ultrasonography, urinary sodium, N-acetyl-beta-D glucosaminidase (NAG) and microalbuminuria excretion. RESULTS: The measurements of renal resistive indexes (RRI) were significantly higher in cirrhotic patients than the control group (0.69 +/- 0.07 vs 0.62 +/- 0.02, P < 0.0001). RRI measurement was found to be increased in decompensated cirrhotic patients than in compensated cirrhotic patients (0.73 +/- 0.05 vs 0.67 +/- 0.08, P < 0.0001). A significant positive relationship was observed between RRI and child score (r = 0.53). Urine NAG/Cr ratio was significantly higher in cirrhotic patients than in the control subjects (P < 0.001). Microalbumin concentrations were increased in the patients with decompensated cirrhosis than in the controls (P = 0.02). Patients with ascites and portal hypertension showed increased RRI values. CONCLUSIONS: We conclude that patients with cirrhosis are at risk of renal deterioration, which can not be detected by serum urea, creatinine, and glomerular filtration rate. The increase of RRI is associated with the progress of hepatocellular disease, and also the development of ascites and portal hypertension. Elevated urinary sodium excretion, elevated urinary NAG/Cr ratio and microalbuminuria might have a prognostic value especially in patients with Child scores> 6. Hence, monitoring RRI is a non-invasive means of studying early renal hemodynamic alteration in childhood cirrhosis.     

Preeclampsia: Is it because of the asymptomatic,unrecognized renal scars caused by urinary tract infections in childhood that become symptomatic with pregnancy?

Preeclampsia is an important disease of pregnancy whose exact etiology is still unknown despite continuing developments in medicine. Although most commonly it is believed to be caused by a defective placentation, in this paper, we hypothesize that the primary underlying problem in the development of preeclampsia can be in kidneys in a greater proportion of cases than it is believed today. The increased intravascular volume and the increased work load of kidneys together with the resulting glomerular hypertrophy may precipitate nephrotic syndrome, which in this case is called “preeclampsia” in a previously affected kidney. Urinary tract infections in childhood leaving silent, unrecognized small scars in the kidneys may be the underlying renal cause which disrupts its silence with an increased work load of kidneys prominently occurring after the midtrimester. The histopathologic finding in kidneys with renal scars after childhood urinary tract infections and in preeclampsia is focal segmental glomerulosclerosis in the majority of cases and this similarity strengthens our hypothesis.     

Cardiovascular risk assessment in children and adolescents with congenital solitary kidneys

Patients with solitary kidneys (SKs) are at risk of hypertension (HT) and associated end‐organ damage. The authors aimed to evaluate whether children with congenital SKs (CSKs) have higher office, ambulatory, or central blood pressure (BP), increased arterial stiffness or left ventricular mass index, or any risk for arrhythmia. With this purpose, patients with CSK and healthy controls being followed up between January 2018 and June 2019 were enrolled in the study. Demographic, biochemical, and office blood pressure (BP) data were recorded. Then, ambulatory blood pressure monitoring (ABPM) and measurements of central BP (cBP), pulse wave velocity (PWV), and augmentation index (AIx@75) were obtained. Ventricular repolarization parameters were acquired by 12‐lead electrocardiography. Left ventricular mass index (LVMI) and abdominal aortic stiffness parameters including strain, pressure strain elastic modulus (Ep), and normalized Ep (Ep*) were calculated with echocardiographic measurements. Finally, 36 children with CSK and 36 healthy controls were included. Serum creatinine, uric acid, total cholesterol levels, ABPM parameters, cBP levels, and PWV values were significantly higher, and eGFR levels were significantly lower in the CSK group. VR parameters, abdominal aortic stiffness indices, and LVMI were similar between the groups. CSK increased the risk of HT in ABPM (HT_ABPM) by 6 times. PWV was significantly correlated with Ep and Ep* in cases with CSK. Determination of cBP and PWV along with 24‐hour ABPM would be a useful tool in children with CSK.     

Recurrent Left Ventricular Apical Ballooning Syndrome in a Patient with Pheochromocytoma

Transient left ventricular apical ballooning syndrome is characterized by reversible left ventricular wall motion abnormalities, chest pain or dyspnea, ST-segment elevation, and mild elevation of cardiac enzyme levels in the absence of obstructive coronary artery disease. The pathophysiology of the syndrome is still unknown. The probable mechanism is supposed to be a catecholamine discharge. We report the case of a 66-year-old woman with recently diagnosed pheochromocytoma who presented with chest pain and ST-segment elevation. Coronary angiography revealed normal coronaries and apical dyskinesia at ventriculography. A similar episode of chest pain occurred 4 years ago with same angiographic findings and reversible inferobasal akinesia. In-hospital course was uneventful and the patient was discharged from the hospital 4 days later with treatment of aspirin 1 × 100 mg, metoprolol 1 × 50 mg, lisinopril 1 × 10 mg, and atorvastatin 1 × 20 mg. At 2 years follow-up after the event, the patient remained asymptomatic.     

CerebralDermabacter hominis abscess

Summary Dermabacter species are commonly isolated from the skin and other body sites but rarely show pathogenicity in humans. A case ofDermabacter hominis cerebral abscess is reported which presented as a contrast-enhancing intracranial mass in a renal tranplant patient.