Thin basement membrane nephropathy associated with minimal change disease in a 15-year-old boy

Thin basement membrane nephropathy (TBMN) is characterized clinically by persistent hematuria, minimal proteinuria, normal renal function, another family member with hematuria, and a benign course. Especially in childhood TBMN, proteinuria of any degree is reported to be uncommon. We report on a boy with benign familial hematuria found by urinary screening at 3 years of age who presented with nephrotic syndrome (NS) at 15 years of age. His renal histology showed TBMN associated with minimal change disease (MCD). Treatment with corticosteroid resulted in complete remission of NS in a short period of time, while isolated hematuria persisted during the follow-up period despite this therapy. We speculate, therefore, that the nephrotic range proteinuria is not due to TBMN but rather is the manifestation of associated MCD. Several cases of TBMN with NS have been reported in adults, but it has not yet been reported in children in the literature. To our knowledge, this is the first case of childhood TBMN associated with NS resulting from coincidental MCD.     

Intraperitoneal Rupture of the Ureter as a Cause of Generalized Peritonitis: Report of a Case

We report a rare case of generalized peritonitis caused by nontraumatic, intraperitoneal rupture of the ureter. An 80-year-old woman with a history of bilateral vesicoureteral reflux and long-term urethral indwelling catheter drainage presented with a very distended abdomen. Computed tomography showed massive ascites and intraperitoneal free gas. We performed an emergency laparotomy, assuming a gastrointestinal perforation; but could not find a cause of generalized peritonitis. Postoperatively, she presented with anuria and massive peritoneal drainage. The findings of a cystogram confirmed intraperitoneal ureteral rupture. She was managed successfully with ureteral stenting. The diagnosis of this condition requires a high degree of clinical suspicion, along with radiographic evidence and peritoneal fluid analysis. Image-guided interventions play a crucial role in the management of ureteral urine leaks after a correct diagnosis has been made.     

JPN Guidelines for the management of acute pancreatitis: severity assessment of acute pancreatitis

This article addresses the criteria for severity assessment and the severity scoring system of the Ministry of Health and Welfare of Japan; now the Japanese Ministry of Health, Labour, and Welfare (the JPN score). It also presents data comparing the JPN score with the Acute Physiology and Chronic Health Evaluation (APACHE) II score and the Ranson score, which are the major measuring scales used in the United States and Europe. The goal of investigating these scoring systems is the achievement of earlier diagnosis and more appropriate and successful treatment of severe or moderate acute pancreatitis, which has a high mortality rate. This article makes the following recommendations in terms of assessing the severity of acute pancreatitis: (1) Severity assessment is indispensable to the selection of proper initial treatment in the management of acute pancreatitis (Recommendation A). (2) Assessment by a severity scoring system (JPN score, APACHE II score) is important for determining treatment policy and identifying the need for transfer to a specialist unit (Recommendation A). (3) C-reactive protein (CRP) is a useful indicator for assessing severity (Recommendation A). (4) Contrast-enhanced computed tomography (CT) scanning and contrast-enhanced magnetic resonance imaging (MRI) play an important role in severity assessment (Recommendation A). (5) A JPN score of 2 or more (severe acute pancreatitis) has been established as the criterion for hospital transfer (Recommendation A). (6) It is preferable to transfer patients with severe acute pancreatitis to a specialist medical institution where they can receive continuous monitoring and systemic management.     

Does Resection Line Involvement Affect Prognosis in Early Gastric Cancer Patients? An Italian Multicentric Study

Background Resection line involvement has been indicated as an important prognostic factor for gastric cancer. Its late detection renders the choice of treatment difficult for surgeons. Materials and Methods We describe the multicenter experience of a group of 11 patients with early gastric carcinoma (EGC) and positive resection confirmed at histological examination who did not undergo surgical retreatment for reasons of associated disease, surgical considerations on duodenal stump, or patient refusal. Results The gastric margin was involved in 4 patients, and 7 patients had duodenal resection line involvement. No surgical complications or postoperative deaths were observed. Five and 8-year survival was 100% and 86%, respectively. The only patient who relapsed did not have lymph node involvement and died from liver metastases, without local recurrence. Conclusions It is sometimes difficult to accurately define the resection line in gastric cancer surgery, especially in the early stages of disease, but because of the strongly negative prognostic value of an infiltrated margin, frozen sections are recommended if neoplastic invasion is suspected and a new resection is always recommended if possible. Nevertheless, the good prognosis of resected EGC patients with resection line involvement must be considered before submitting patients with associated diseases to radical surgical retreatment.     

Antenatal magnetic resonance imaging is useful in providing predictive values for surgical procedures in abdominal wall defects

Background

Antenatal magnetic resonance imaging (MRI) is useful for the diagnosis of abdominal wall defects. Its predictive value concerning the possibility of primary closure of the abdominal wall, however, has so far not been reported.

Methods

Between August 2001 and November 2004, antenatal MRI was performed on 9 patients with abdominal wall defects in whom surgical repair was performed immediately after birth. Areas of the abdominal cavity and exteriorized viscera were manually traced from both sagittal and axial MR images, and the data were further transmitted to a Workstation for MRI Volumetry (Advantage Windows 4.1, General Electric Medical Systems, Milwaukee, Wis). We examined the exteriorized ratio (ER), which is calculated by dividing the absolute volume of the abdominal cavity by that of the exteriorized viscera, and evaluated the predictive value by a retrospective comparison with surgical procedure.

Results

In the primary closure group (n = 5), mean values of ER were 0.33 ± 0.31 from axial and 0.45 ± 0.31 from sagittal MR images. In contrast, in the staged closure group (n = 4), mean values of ER were 1.39 ± 0.40 from axial and 1.34 ± 0.42 from sagittal MR images. There was a significant difference (P < .05) between the 2 groups for both sets of images. The ER obtained from antenatal MRI correlated well with surgical procedure.

Conclusions

The ER might be useful for antenatal counseling, planning for delivery, and prediction of the most likely surgical procedure.     

Bladder preservation for locally advanced bladder cancer by transurethral resection, systemic chemotherapy and radiation

Twenty-three out of 31 patients with clinical T2-4a N0 M0 bladder cancer and given a trial of trimodality therapy including transurethral resection (TUR), systemic chemotherapy and radiation between 1991 and 2002 completed this therapy. The other 8 dropped out because of insufficient clinical effect. Local bladder recurrence was seen in 3 patients and the bladder preservation rate was 64.5%. Nineteen of the 23 patients showed a complete histological response on a subsequent TUR specimen, the other 4 were not examined for histological response. Thirteen of the 19 patients showed a complete histological response after maximal TUR and systemic chemotherapy, while 6 did after TUR, chemotherapy and radiotherapy. Bladder cancer was T2 in, 15, T3 in 1, and T4a in 3 patients. The CR rate for T2 cancer was significantly higher than that for T3-4a cancer. The 5-year disease-specific survival of the 23 patients treated with preservation therapy was 67.1%. Some of the patients with locally advanced bladder cancer may benefit from this preservation therapy.     

Scintigraphic diagnosis of primary and metastatic bone tumour by technetium-99m diphosphonate

Summary Diagnosis by serial bone scan was performed on 134 patients with bone tumours using the radioactive isotopes ^99m TcEHDP, ^99m TcBleo and ⁶⁷ Ga. ^99m TcEHDP bone scan was found to be a highly sensitive screening test in patients with skeletal metastases from malignant bone tumours.Malignant bone tumours demonstrated a marked uptake of all three radiopharmaceuticals. Benign bone tumours did not reveal any abnormal accumulation. The exceptions however were myeloma which showed a decreased uptake in the lesion compared to the background radioactivity and also giant cell tumours which showed an abnormal uptake of all three bone-seeking radiopharmaceuticals.Bone scanning using ^99m TcEHDP combined with ^99m TcBleo and ⁶⁷ Ga is a safe, simple and sensitive method for the detection and evaluation of bone tumours. It should, however, be emphasised that there are several pitfalls in the interpretation of bone scans. These include fractures in benign bone tumours and chronic osteomyelitis.

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Résumé Le diagnostic par scintigraphie a été effectué sur 134 patients atteints de tumeurs osseuses, en utilisant les isotopes radioactifs: ^99m Tc-EHDP, ^99m Tc-Bléo et ⁶⁷ Ga.La scintigraphie au ^99m Tc-EHDP s'est révélée être une méthode de dépistage efficace chez les patients présentant des métastases osseuses de tumeurs malignes.Les tumeurs malignes des os fixent de façon particulièrement marquée ces trois radioisotopes. Par contre, les tumeurs osseuses bénignes ne montrent aucune accumulation anormale de ces produits. Les exceptions sont le myélome, qui présente une fixation diminuée au niveau de la lésion par rapport à la radioactivité de fond, et les tumeurs à cellules géantes qui manifestent une accumulation anormale des trois isotopes radioactifs.La scintigraphie au ^99m Tc-EDHP, en association avec le ^99m T-Bléo et le ⁶⁷ Ga, est une méthode sûre, simple et sensible pour la détection et le diagnostic des tumeurs osseuses. Cependant, il faut souligner qu'il existe plusieurs pièges dans l'interprétation de la scintigraphie, notamment les fractures sur tumeurs osseuses bénignes et l'ostéomyélite chronique.

     

The incidence of antithyroid antibodies in relatives of patients with thyroid disorders (author's transl)]

In order to elucidate a predisposition for so-called autoimmune thyroid disorders such as Graves' disease, chronic thyroiditis and myxedema, the incidence of thyroid antibodies was studied in relatives of patients with thyroid disorders. The relatives studied were all limited within a two degree relationship of the patients. Serum antibodies to thyroglobulin and thyroid microsome were determined by susing the method of a hemagglutination test. The following results were obtained; 1) Thirty six of 103 relatives (35%) were found to have such antibodies, and sixteen (7.0%) of 230 hospital controls were positive. This difference was significant (P less than 0.001). 2) There was no difference in the incidence among relatives of patients with Graves' disease and those chronic thyroiditis. 3) With regard to age, the incidence in the more than 20 years old group was significantly greater than that in the under 19 years old group. 4) There was no difference in the incidence between male and female. These results suggested that the relatives of patients with so-called autoimmune thyroid disorders may have a predisposition for thyroid disorders.     

Peculiar congenital parieto-occipital head tumor: report of 3 cases.

3 infantile cases of congenital parieto-occipital midline soft tumor, the clinical and pathological findings of which did not correspond precisely to a known congenital head tumor, are reported. One of them probably belongs to McLaurin's type of encephalocele with heterotopic glial rest, and the remaining two to McLaurin's type of meningocele without central connection. The types of anomaly shown by the present cases should generally be called 'atretic cephalocele' or 'rudimentary cephalocele'.