Chronic hypomagnesemia and hypokalemia due to renal wasting in siblings

A 13 year old girl with carpopedal spasm is presented. Investigation values showed hypomagnesemia and hypokalemia. Her younger brother and sister demonstrated the same biochemical abnormalities without any symptoms. Their urinary excretion of magnesium and potassium were inappropriately high compared with their serum levels. Treatment with oral magnesium sulfate failed to correct the abnormalities, but serum levels of magnesium and potassium were just below the lower limits. This familial disease may represent congenital renal wasting of magnesium and potassium.     

A multicenter,randomized, controlled trial of intravenous gamma globulin therapy in children with acute Kawasaki disease

We studied the effect of intravenous, polyethyleneglycol-treated, human immunoglobulin, administered at 200 mg/kg per day (group A: n = 147; male 86, female 61; age < 1 year, 50) or 400 mg/kg per day (group B: n = 152; male 87, female 65; age < l year, 52) for five consecutive days and compared it with freeze-dried, sulfonated human immunoglobulin [group C: n = 152; male 87, female 65; age < 1 year, 51), administered at 200 mg/kg per day for five consecutive days, on the prevention of coronary artery abnormalities in Kawasaki disease. Echocardiograms were interpreted blindly and independently. Proportions of 87.1%, 95.4%, and 82.3% in groups A, B, and C, respectively, had no coronary artery abnormalities. The confidence limits of difference between the proportions of groups A and C, groups B and C, and groups B and A were −4.4% and 10.4%, 7.8% and 15.9%, and 4.0% and 10.8%, respectively. Duration of fever and serum immunoglobulin G (IgG) levels were correlated with the prevalence of coronary artery abnormalities. We concluded that intravenous, polyethyleneglycol-treated, human immunoglobulin and freeze-dried, sulfonated human immunoglobulin had clinically equivalent effects on coronary artery abnormalities, and that five daily doses of 400 mg/kg of intravenous, polyethyleneglycol-treated, human immunoglobulin is more effective than that of 200 mg/kg gamma globulin.     

Case report: Portal-systemic encephalopathy due to a congenital extrahepatic portosystemic shunt

We present a case of portal-systemic encephalopathy due to a congenital splenorenal shunt. A 69 year old woman was admitted to hospital because of recurrent episodes of disturbed consciousness. The present episode had begun 3 months prior to admission. Although the patient demonstrated mildly slurred speech, the remainder of her neurological examination was unremarkable. She showed no clinical signs of portal hypertension and her liver function, except for a serum hepaplastin test of 58% and an ICG retention rate of 28% at 15 min, was normal. Her serum ammonium level was 210 μg/dL. The venous phase of a superior mesenteric arteriogram revealed a splenorenal shunt and narrowing of the portal vein, which was 4 mm in diameter. The histological findings, demonstrated by a needle liver biopsy specimen, were consistent with mild fibrosis and lymphocytic infiltration. Following the diagnosis of a splenorenal shunt in the absence of liver cirrhosis, ligature of the shunt was performed with a splenectomy. The portal vein pressure after ligature of the shunt rose from 12.5 to 18.8 mmHg. This shunt was thought to be of congenital origin. The high preoperative serum ammonia concentration decreased to the normal range postoperatively and the serum hepaplastin test and ICG retention rate similarly improved postoperatively. A follow-up superior mesenteric arteriogram was performed during the venous phase, demonstrating resolution of the shunt and decreased portal vein narrowing. The patient has suffered no further episodes of disturbed consciousness postoperatively.     

Relationship of angiographic finding with neovascular structure detected by immunohistochemical staining of α-smooth muscle actin in small hepatocellular carcinoma

To elucidate the relationship between angiographic features and histological findings, an immunohistological study of α-smooth muscle actin was performed in 106 patients with small hepatocellular carcinoma. Arterial dominance or portal blood paucity were found in 73 patients (68.9%) on digital subtraction angiography, 88 (83.0%) on computerized tomographic arterial portography and 87 (82.1%) on carbon dioxide-enhanced ultrasonography. Among 73 patients with hypervascularity on angiography, 57 (78.1%) had thick-walled, nuclei-rich and slender-shaped vessels (type II), eight (11, 0%) had thin-walled, nuclei-poor and oval-shaped vessels (type I) and the remaining eight had a mixed type of II and I. Conversely, among 33 patients without hypervascularity, five (15.2%) had a type II, 21 (63.6%) had a type I, five had a mixed type and two had no positive vessel. Tumour size, histological classification and amount of non-triadal vessels were also associated with the angiographic appearance of the tumours. Among varied aspects of the cancer including tumour size, tumour multiplicity, microscopic portal invasion, histological classification, amount of α-smooth muscle actin-positive vessels and shape of α-smooth muscle actin-positive vessels, multivariate logistic regression analysis demonstrated that the shape of α-smooth muscle actin-positive vessels was solely associated with angiographic hypervascularity independently (P < 0.0001). Although the existence of non-triadal vessels characterized hepatocellular carcinoma, angiographic hypervascularity was closely associated with the type II vessel. A morphological change of non-triadal vessel from type I to type II was considered to occur in an early stage of hepatocellular carcinoma.     

A Case of Hepatoblastoma in Adult

A rare case of hepatoblastoma in a 60-year-old man is reported.The first sign of his illness was metastasis found as a tumoron the left fifth rib. There were no laboratory or angiographicfindings that might differentiate hepatoblastoma from hepatocellularcarcinoma. In this case, the diagnosis was made by histologicalexamination at autopsy, which revealed mesenchymal tumor tissuesin addition to the epithelial ones. The incidence of hepatoblastomain people over 15 years old is 0.056% of that of hepatocellularcarcinoma in Japan.     

Molecular evaluation of the SRY gene for gonads of patients with mixed gonadal dysgenesis

AIM: To determine whether the SRY gene is present in the gonads of patients with mixed gonadal dysgenesis (MGD). METHODS: Molecular analysis was performed in three patients with MGD. Polymerase chain reactions were used to test for the presence of the SRY gene in the peripheral lymphocytes, testes and streak gonads. RESULTS: Chromosome analysis revealed 45,XO/46,XY in two patients, and 46,XY in the third patient. In the peripheral lymphocytes and testicular tissue, the SRY sequences were positive in all cases. However, the SRY sequence was detected in the streak gonad in only two of the three patients. CONCLUSIONS: It is interesting that we identified both SRY-positive and SRY-negative streak gonads. Although the SRY gene has a very important role in testicular differentiation, genes other than the SRY gene might also influence the development of the indifferent gonad in MGD.     

Laparoscopic nephropexy: Treatment outcome and quality of life

BACKGROUND: The recent introduction of laparoscopic procedures has markedly altered urological surgery. Laparoscopic nephropexy has attracted the attention of urologists as a treatment for nephroptosis. Herein, we describe our experiences and quality-of-life outcome of laparoscopic nephropexy and discuss its indications and surgical techniques. METHODS: From May 1998 to February 2002, six female patients, ranging in age from 20 to 64 years (median age 39.8 years), with symptomatic nephroptosis underwent laparoscopic nephropexy. Mean preoperative downward kidney displacement was 2.25 vertebral bodies (range 2-2.5) and all affected kidneys were tilted at orthostasis. One patient underwent nephropexy through the transperitoneal approach and the remaining patients underwent nephropexy through the retroperitoneal approach. To evaluate surgical results, postoperative follow-up interview (pain visual analog scale and the short-form 36 (SF-36) health survey questionnaire) and objective examinations were performed. RESULTS: All procedures were accomplished without complication. Postoperative intravenous pyelography correctly confirmed fixed kidney in both supine and erect positions. All patients reported an improvement of symptoms approximately 1 month after nephropexy and no symptoms have recurred during the follow-up period (range 6.3-50.7 months). On the SF-36, two domains, including role limitations due to emotional problems (RE) and mental health (MH), exhibited significant improvement postoperatively (P = 0.0405 and P = 0.0351, respectively). CONCLUSIONS: Laparoscopic, in particular retroperitoneoscopic, nephropexy yields excellent outcomes and greatly improves general health-related quality of life, particularly mental status, as a minimally invasive treatment for symptomatic nephroptosis.     

Interstitial laser coagulation for management of benign prostatic hyperplasia: Long-term follow-up

AIM: We evaluated the long-term results of transurethral interstitial laser coagulation in the treatment of benign prostatic hyperplasia (BPH) with up to 9 years of follow up at the Kurashiki Central Hospital and determined the patient characteristics that predict a favorable outcome. METHODS: From December 1993 to May 1997, a total of 82 patients were enrolled in the present study. Subjective and objective voiding parameters were collected from medical records and a self-administered questionnaire was sent to the patients. Kaplan-Meier plots were constructed to assess the risk of retreatment. RESULTS: The mean follow-up period was 48.4 months (range, 3-108 months). A total of 59 patients (72%) did not need any additional treatment at 12 months and 30 patients (37%) did not require additional treatment during the entire follow-up period. A total of 29 patients (35%) were retreated during follow-up. Transurethral prostate resection (TURP) was performed in 18 patients (22%). The remaining 11 patients (13%) were offered additional pharmacotherapy. The minimum and median retreatment-free durations were 3 and 14 months, respectively. Seven patients died and 17 were lost to follow-up. Men aged 71 years or older had greater likelihood of requiring retreatment than those younger than 71 years (P = 0.0397). No significant differences were noted in the other baseline characteristics. Among postoperative parameters, a rate of decrease of the International Prostate Symptom Score of the patient of lower than 60% and a rate of decrease in patient quality of life of lower than 50% at 3 months were associated with greater likelihood of retreatment (P = 0.0083 and P = 0.0006, respectively). CONCLUSIONS: Interstitial laser coagulation is effective for the treatment of BPH. Good long-term results and an acceptably low retreatment rate render this modality an effective alternative to TURP, especially for younger patients. Short-term improvement of subjective symptoms was predictive of favorable long-term outcome.     

Acute hepatitis C transmitted by needlestick accident despite short duration interferon treatment

Hepatitis C virus (HCV) transmission by needlestick accidents involving hospital employees has become an important problem. The present report is of a case of acute hepatitis C that developed after a needlestick injury, despite short duration interferon treatment performed just after the accident in a trial effort to prevent HCV transmission. Nosocomial infection of HCV in medical employees is reviewed, and the current prospects for protecting them from HCV transmission after needlestick accident are discussed.