Hypouricemia in cirrhosis reflects hemodynamic alterations

In a population of 27 consecutive patients with liver cirrhosis, systemic hemodynamics were investigated and correlated to uric acid concentrations, fractional uric acid excretion, and creatinine clearances. Mean serum uric acid concentration was lower than in normal controls, and this was related to abnormally high uric acid clearances. Uric acid concentrations correlated positively to total peripheral resistances and negatively to cardiac output. Fractional uric acid excretions correlated negatively to total peripheral resistances and positively to cardiac output. There was no correlation between creatinine clearances and any variable of systemic hemodynamics. Serum uric acid concentration and fractional uric acid excretion are dependent of the hemodynamic state in cirrhosis.     

Avascular necrosis of the scaphoid after three-ligament tenodesis for scapholunate dissociation: case report

An unusual complication after tenodesis for scapholunate instability (Brunelli's technique) is described. More than 1 year after the procedure, a fracture of the scaphoid with collapse was observed. Further examination concluded there was avascular necrosis of the scaphoid. The patient was treated with a proximal row carpectomy.     

Multifocal kaposiform haemangioendothelioma

Kaposiform haemangioendothelioma (KHE) is a rare, locally aggressive vascular spindle cell proliferation, with resemblance to Kaposi’s sarcoma. This tumour usually occurs in skin and retroperitoneum of infants and young children and is often complicated by the Kasabach–Merritt phenomenon (KMP). A 3-year-old boy presented with a right submandibular swelling due to lymphadenopathies, a violaceous skin lesion at the left commissure of the lips and an ill-defined lesion in the right thyroid lobe. There were some signs of KMP. Histological examination revealed a typical infiltrative multilobular spindle cell proliferation with slit-like vascular spaces in these three localisations. Immunohistochemical stains showed positivity for CD34 and CD31 and many alpha-smooth muscle actin-positive spindle cells around the vascular spaces. There was no Herpes virus type 8 expression. The presented case is unique in two ways. First, thyroid involvement of KHE has never been described in the literature until now. Secondly, and most remarkably, the multifocal presentation in three anatomically distinct and separated localisations is extremely unusual.     

Extramammary myofibroblastoma is genetically related to spindle cell lipoma

Extramammary-type myofibroblastoma is a rare, benign spindle cell lesion, strictly resembling the breast counterpart, but occurring in extramammary sites, mainly in the inguinal/groin area. In this paper, we describe an extramammary-type myofibroblastoma in the groin of a 37-year-old male patient. The tumor showed a typical morphological and immunophenotypical profile, including staining for both CD34 and desmin. Dual-color interphase florescent in situ hybridization analysis revealed losses of RB/13q14 and FKHR/13q14 loci within tumor cells. The chromosome 13 rearrangements associated with the loss of the 13q14 chromosomal region are typically seen in spindle cell lipoma, and have been previously recognized in mammary myofibroblastoma, providing strong evidence for a pathogenetic link between these lesions.     

Apnea as the sole manifestation of a seizure attributable to meningioangiomatosis of the temporal lobe in an infant

An 8-month-old boy presented with periods of apnea occurring several times daily. Initial clinical and radiologic evaluations could not identify a cause for these episodes. Video electroencephalographic monitoring indicated interictal high-voltage θ and δ activity over the right frontotemporal area. A short ictal run of δ activity over the same region was registered during a brief apneic spell. Cranial magnetic resonance imaging revealed a lesion anteromedial in the right temporal lobe. After epilepsy surgery, anatomopathologic examination indicated meningioangiomatosis. This case demonstrates that a focal lesion in the temporal lobe can present during infancy with apnea as sole seizure manifestation, without other clear clinical evidence.