Association of thin basement membrane nephropathy with hypercalciuria, hyperuricosuria and nephrolithiasis.

BACKGROUND: Familial persistent microhematuria with normal renal function is the most common presentation of thin basement membrane nephropathy (TBMN). Gross hematuria episodes and loin pain attacks are other manifestations of the disease. On the other hand, it has been shown that hypercalciuria (HC) and hyperuricosuria (HU) can produce both gross or microscopic non-glomerular hematuria, in addition to their role in renal stone formation. METHODS: We studied the prevalence of HC, HU and nephrolithiasis in a group of 27 biopsy-proven TBMN as well as in 19 non-biopsied first-degree relatives with persistent microhematuria and 25 first-degree relatives without microhematuria. A group of 27 patients with IgA nephropathy (IgAN) and persistent microhematuria, and another group of 20 healthy subjects without known renal diseases were selected as control groups. RESULTS: Ten (37%) patients with TBMN and 8 (42%) relatives with microhematuria showed HC and/or HU at presentation; relatives without microhematuria, IgAN patients and normal controls showed a significantly lower prevalence of HC and HU. The prevalence of previous nephrolithiasis among TBMN patients (25%) was significantly higher than in IgAN patients (3%; P < 0.05). Family history of nephrolithiasis was recorded in 14 (51%) of the 27 TBMN families, in contrast with 2 of 27 (7%) with IgAN and 1 of 20 (5%) in normal controls (P < 0.05). The prevalence of nephrolithiasis, gross hematuria bouts and loin pain episodes among TBMN patients and microhematuric relatives showing HC and/or HU at presentation (44%, 44% and 27%, respectively) were significantly higher than those of TBMN patients and microhematuric relatives with normal calcium and uric acid urinary excretions (10%, 7% and 3%, respectively; P < 0.05). At the end of follow-up (8.8+/-4.1 years in TBMN patients and 9.1+/-4.2 years in relatives with microhematuria), all the cases maintained normal renal function. CONCLUSIONS: We found a high prevalence of HC, HU, and nephrolithiasis among TBMN patients and relatives with microhematuria. Our study also shows a significant relationship between the presence of HC and/or HU and the prevalence of nephrolithiasis, gross hematuria bouts and loin pain episodes.

Hilar biliary cysts in hepatic transplantation. Report of three symptomatic cases and occurrence in resected liver grafts.

Hilar cysts are infrequent post-transplant biliary tract complications. Thirteen cases were discovered among 493 consecutive liver transplants (2.6%). Three (0.60%) were symptomatic (obstructive jaundice) while the other ten were found by systematically searching in the hilum in a series of 129 consecutive, resected grafts at retransplantation or autopsy (n = 54). Two types of cysts were detected: in eight grafts (1.6%), these were blind unilocular cavities with viscid mucous content, located adjacent to the biliary tract anastomoses. These had been inadvertently created as a result of the sequestered remnant cystic duct after cholecystectomies and biliary tract reconstructions, where a double-barreled common duct and long cystic duct had been present in the donor liver. These mucoceles ranged from 0.5 to 5.5 cm in diameter (median 1.7 cm). The three symptomatic cases were diagnosed by imaging techniques 3.5 years after transplantation; however, this type of cyst was found as early as the 2nd month post-transplantation when detected in lost liver grafts. Five livers (1%), lost between 5 months and 2.8 years post-transplantation, showed cystically dilated peribiliary glands, sometimes with multilocular, and occasionally multiple, cavities ranging from 0.5 to 2 cm in diameter (median 0.8 cm). This type of cyst was asymptomatic and located adjacent to the left, right, or common hepatic ducts. Threads were found near four cysts, suggesting that surgical injury may have been responsible for obstructing the neck of the glands. With the increasing number of long-term survivors of liver transplantation, unless preventive surgical methods are implemented, the number of symptomatic cysts of these origins can be expected to grow. Transplantation teams should, therefore, be aware of these potential causes of biliary tract complications.

[Small cell anaplastic carcinoma of the colon]. / Carcinoma anaplásico de células pequeñas de localización colónica.

Small-cell anaplastic carcinoma of the colon is a very infrequent tumour (less than 1% of all colorectal neoplasms). Thirty-two cases have been described in the international literature up to 1992. A case, in a 54 year-old patient who underwent successful resection is presented. The importance of this tumour is due to its great aggressivity, and its great tendency to produce early hematogenous and lymph node metastases. It implies a bad prognosis and a survival of around 0% at one year. Because of these facts, treatment must include, beside surgical resection, an aggressive systemic protocol.

[Adenosquamous carcinoma of minor salivary glands. Reporting a case in the tongue]. / Carcinoma adenoescamoso de glándulas salivares menores. Exposición de un caso de localización lingual.

Adenosquamous carcinoma of the salivary glands is an unusual neoplasm not unanimously included in many classifications of salivary gland malignancies. A case sited in the oral cavity, within the tongue is reported. Clinical and histopathologic features are described together with a discussion about treatment and behaviour. We also include an updated of literature relating to this entity.