Indication of total parathyroidectomy for an Epstein syndrome patient with end-stage renal disease.

Epstein syndrome is an extremely rare genetic disorder characterized by the association of nephritis, deafness and megathrombocytopenia. We present the case of a 21-year-old patient diagnosed with Epstein syndrome and hyperparathyroidism secondary to chronic kidney disease. The main particularity of this case resides in the association between megathrombocytopenia and secondary hyperparathyroidism requiring surgery, which could lead to a series of concerns regarding the intra- and postoperative hemorrhagic risk of the procedure. Nevertheless, both the surgical procedure and the postoperative recovery were uneventful, suggesting that the lower threshold for preoperative thrombocyte count in megathrombocytopenia should be specifically considered on an individual case analysis.

Status thymicolymphaticus: real or fake?

Sudden infant death syndrome (SIDS) is the sudden, unexpected death of an infant less than one year of age that remains unexplained after a full investigation. SIDS is the most frequent cause of death of infants between two weeks and one year of age, explaining 35% to 55% of all deaths in this age group. We report a newborn male who died soon after birth. The newborn was cyanotic, bradycardic at first, and then asystolic; without any vesicular murmur, apneic, low amplitude thorax movements, even under conditions of positive pressure ventilation on the endotracheal tube. The microscopic aspect thymus highlighted a corticomedullary ratio quite high in favor of the cortical, rich in lymphocyte population, with the dilated subcapsular sinuses. In this report, we considered that cardiorespiratory failure, which was the immediate cause of death, could have been caused by the thymus hypertrophy. This hypertrophy can be a complication of an intrapartum preexistent condition, most probably of hepatic nature.

Adaptive and Pathological Reshuffling in the Venous Wall in Special Hemodynamic Conditions-Acquired Arteriovenous Fistula.

Hemodialysis and peritoneal dialysis are the two possible choices in chronic renal disease in the uremic stage. Native arteriovenous fistula is susceptible to complications, some posing vital risk and requiring prompt treatment. We present the case of a patient undergoing hemodialysis on native AVF. An aneurism developed progressively on the arterialized cephalic vein. The AVF was abolished by closing the arteriotomy with a venous patch and excising the aneurismal venous segment. The pathology exam of the surgical specimen showed unequal vessel wall thickness due to hyperplasia of the media and subintimal space, with calcifications within the hyperplastic media.