Monoclonal gammopathy of renal significance (MGRS)-related AL amyloidosis complicated by amyloid myopathy: a case report.

BACKGROUND: Lately, monoclonal gammopathy of renal significance (MGRS) has been defined as a group of renal disorders that are strongly associated with monoclonal protein, including amyloid immunoglobulin light chain (AL) amyloidosis. Amyloid myopathy is rare (1.5% of all patients with amyloidosis) and the prognosis is poor. Furthermore, only approximately 20% of patients with amyloid myopathy are reported to have renal involvement, indicating a lack of data in the literature. CASE PRESENTATION: Here, we report a rare case of MGRS-related AL amyloidosis complicated by amyloid myopathy that presented with muscle weakness in the upper and lower limbs, neck and fingers, and nephrotic syndrome. Blood, urine, and bone marrow examination revealed monoclonal gammopathy of undetermined significance (MGUS) (Bence Jones protein-lambda). Muscle biopsy of the vastus lateralis muscle demonstrated amyloid proteins in the sarcolemma and in the blood vessel walls on Congo red staining, suggesting amyloid myopathy, and tiny inclusions in fibers on modified Gomori trichrome stain. Although we thought they were reminiscent of nemaline bodies, we could not confirm the nature of this structure. Renal biopsy demonstrated amyloid proteins in the mesangial region, part of the capillary walls, and the blood vessel walls on direct fast scarlet staining. As these amyloid proteins were positive for p-component staining and negative for amyloid A staining, ß2-microglobulin, and pre-albumin, and as lambda light chains were positive in the mesangial region, we diagnosed the patient with MGRS-related AL amyloidosis. Although he was treated with melphalan and dexamethasone, his symptoms did not improve. CONCLUSIONS: AL amyloidosis involving the kidneys and muscles has a poor prognosis, and a delayed diagnosis of amyloid myopathy is common because of its rarity and frequent misdiagnosis, which increases organ function deterioration. Therefore, early detection, therapeutic intervention, and careful follow-up are crucial.

Photo-Fenton degradation of non-ionic surfactant and its mixture with cationic or anionic surfactant.

The oxidative degradation of non-ionic surfactants by the photo-Fenton process has been examined. The photo-Fenton degradation kinetics of mixtures of non-ionic surfactant and other type surfactants has been also investigated since mixtures of non-ionic and ionic surfactants are commonly used to utilize their synergistic effects in many practices. Effects of operating parameters such as dosages of Fenton reagents (iron and hydrogen peroxide) and UV light intensity on the degradation of commercial non-ionic surfactant Sannonic SS-90 (polyoxyethylene alkyl ether) were studied. Although the dosages of the Fenton reagents increased the degradation rate up to the optimum dosages, further addition of the reagents could not enhance the degradation rate. Excess dosages of Fe and H(2)O(2) caused excess OH radicals which could be a scavenger of OH radicals and as a result could not enhance the degradation of the surfactant. The increase in UV light intensity resulting in the faster photo-Fenton process or the enhancement of OH radical formation rate led to the increase in degradation rate of non-ionic surfactant. Although the existence of the anionic surfactant (sodium dodecylbenzene sulphonate) would inhibit the degradation of the non-ionic surfactant due to the formation of complex with Fe ion, the existence of cationic surfactant (dodecyltrimethyl ammonium chloride) affected insignificantly the photo-Fenton degradation process of the non-ionic surfactant.

Nephrotic syndrome with acute kidney injury due to focal segmental glomerulosclerosis following long-term treatment with minodronate.

Although bisphosphonates are well known to cause kidney disease, there are very few published cases of focal segmental glomerulosclerosis (FSGS) following treatment with minodronate. Here we report the case of an 86-year-old woman who developed acute kidney injury and nephrotic syndrome after receiving monthly oral minodronate for 24 months. Kidney biopsy revealed cellular variant FSGS. Treatment was initiated with the discontinuation of minodronate followed by intravenous methylprednisolone pulse and prednisolone at 35 mg/day. Subsequently, the patient's renal function gradually worsened, requiring initiation of hemodialysis. However, renal function and proteinuria improved markedly and hemodialysis was withdrawn 1 month after the initiation of steroid therapy. This is, to our knowledge, the first published case of FSGS induced by long-term use of minodronate, and also the first case of cellular variant FSGS induced by bisphosphonates although collapsing variant of FSGS is commonly caused by bisphosphonates. Our study indicates that patients on bisphosphonates should be closely monitored for proteinuria and renal impairment, regardless of the type of bisphosphonate.

Serum ionized magnesium during magnesium sulfate administration for preterm labor and preeclampsia.

OBJECTIVE: The purpose of this study is to estimate the relations between ionized and total Mg levels during MgSO4 administration in patients with preterm labor and preeclampsia. METHODS: Forty-three pregnant patients who were candidates for MgSO4 were studied (preterm labor, 27; preeclampsia, 16). The administration method was intravenous injection of MgSO4 4 g over 30 min followed by 1-2 g/h. Ionized Mg was measured by the selective ion electrode method at bedside, and compared it with total Mg levels. RESULTS: Significant correlation was existed between levels of ionized and total Mg throughout therapy for both preterm labor (ionized Mg=0.19 x total Mg+0.19; r=0.61, p<0.001) and preeclampsia (ionized Mg=0.20 x total Mg+0.14; r=0.60, p<0.001). CONCLUSION: There are correlations between ionized and total Mg levels during administration of MgSO4 for both preterm labor and preeclampsia.

Prognosis in cervical insufficiency at less than 32 weeks of gestation.

OBJECTIVE: To identify prenatal events associated with adverse outcome in babies at less than 32 weeks of gestation in cases of cervical insufficiency and preterm labor (PTL)/premature rupture of the membranes (PROM). STUDY DESIGN: A case-control study was performed using a logistic regression model at 17 tertiary hospitals in Japan. Adverse outcome was defined as neonatal death or abnormal cerebral ultrasound scans (intraventricular hemorrhage [IVH] and periventricular leukomalacia [PVL]) prior to discharge from hospital. RESULTS: Data were analyzed for 307 cases (74 for cervical insufficiency and 233 for PTL/PROM). Neonatal death and IVH/PVL were noted in 25 and 29 cases, respectively. A significant association of cervical insufficiency (odds ratio (OR) 1.32, 95% confidence interval (CI) 1.02-1.68), gestational age at delivery (<26 weeks) (OR 4.64, 95% CI 1.73-12.44), and Apgar score <7 at 5 min (OR 3.3, 95% CI 1.42-7.64) with combined neonatal death or IVH and PVL was found in a logistic regression model that controlled for in utero transportation, gestational age on admission, clinical chorioamnionitis, and histopathologic chorioamnionitis. CONCLUSION: Cervical insufficiency is a significant factor related to the occurrence of adverse outcome.

Three novel mutations of the MCT8 (SLC16A2) gene: individual and temporal variations of endocrinological and radiological features.

We performed genetic analysis and clinical investigations for three patients with suspected monocarboxylate transporter 8 (MCT8) deficiency. On genetic analysis of the MCT8(SLC16A2) gene, novel mutations (c.1333C>A; p.R445S, c.587G>A; p.G196E and c.1063_1064insCTACC; p.R355PfsX64) were identified in each of three patients. Although thyroid function tests (TFTs) showed the typical pattern of MCT8 deficiency at the time of genetic diagnosis in all patients, two patients occasionally were euthyroid. A TRH test revealed low response, exaggerated response and normal response of TSH, respectively. Endocrinological studies showed gonadotropin (Gn) deficiency in two adult patients. On ultrasonography, goiter was detected in one patient. Interestingly, pituitary magnetic resonance imaging (MRI) demonstrated atrophy and thinness of the pituitary gland in two patients. Our findings suggest that thyroid status in patients with MCT8 deficiency varies with time of examination, and repeated TFTs are necessary for patients suspected of MCT8 deficiency before genetic analysis. In addition, it is noteworthy that some variations were observed on the TRH test and ultrasonography of the thyroid gland in the present study. Morphological abnormality of the pituitary gland may be found in some patients, while Gn deficiency should be considered as one of the complications.

Diagnostic usefulness of 3 tesla MRI of the brain for cushing disease in a child.

It is sometimes difficult to confirm the location of a microadenoma in Cushing disease. Recently, we experienced an 11-yr-old female case of Cushing disease with hyperprolactinemia. She was referred to our hospital because of decrease of height velocity with body weight gain. On admission, she had typical symptoms of Cushing syndrome. Although no pituitary microadenomas were detected on 1.5 Tesla MRI of the brain, endocrinological examinations including IPS and CS sampling were consistent with Cushing disease with hyperprolactinemia. Oral administration of methyrapone instead of neurosurgery was started after discharge, but subsequent 3 Tesla MRI of the brain clearly demonstrated a 3-mm less-enhanced lesion in the left side of the pituitary gland. Finally, transsphenoidal surgery was performed, and a 3.5-mm left-sided microadenoma was resected. Compared with 1.5 Tesla MRI, 3 Tesla MRI offers the advantage of a higher signal to noise ratio (SNR), which provides higher resolution and proper image quality. Therefore, 3 Tesla MRI is a very useful tool to localize microadenomas in Cushing disease in children as well as in adults. It will be the first choice of radiological examinations in suspected cases of Cushing disease.

Diagnosis of toxic shock syndrome by two different systems; clinical criteria and monitoring of TSST-1-reactive T cells.

Two methods of TSS diagnosis were evaluated: comparison of symptoms with clinical criteria and monitoring for evidence of selective activation of Vbeta2(+) T cells by the causative toxin, TSS toxin-1 (TSST-1). Ten patients with acute and systemic febrile infections caused by Staphylococcus aureus were monitored for increase in TSST-1-reactive Vbeta2(+) T cells during their clinical courses. Nine of the ten patients were diagnosed with TSS based on evidence of selective activation of Vbeta2(+) T cells by TSST-1; however, clinical symptoms met the clinical criteria for TSS in only six of these nine patients. In the remaining patient, clinical symptoms met the clinical criteria, but selective activation of Vbeta2(+) T cells was not observed. Time taken to reach the diagnosis of TSS could be significantly shortened by utilizing the findings from tracing Vbeta2(+) T cells. In vitro studies showed that TSST-1- reactive T cells from TSS patients were anergic in the early phase of their illness. Examining selective activation of Vbeta2(+) T cells could be a useful tool to supplement clinical criteria for early diagnosis of TSS.