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Background Herein, a 3-year-old boy presented with hidden-onset isolated proteinuria was reported. The disease was induced by COQ8B (previously termed ADCK4) compound heterozygous variants, including c.[271C > T] and c.[737G > A], which were inherited from his father and mother, respectively. Case presentation The patient visited our clinic due to non-nephrotic range proteinuria for 3 months, but no obvious abnormality was detected in the vital signs or laboratory test results. Renal histopathology revealed mitochondrial nephropathy, which manifested as mild glomerular abnormalities under light microscope, together with mitochondrial proliferation and hypertrophy and crowded arrangement under electron microscope. As suggested by whole exome sequencing, the patient inherited the COQ8B compound heterozygous variants from both of his parents who showed normal phenotype. After literature review, it was confirmed that one of the variant site (c.[271C > T]) had not been reported among the East Asian populations so far. Conclusions Steroid-resistant nephrotic syndrome and focal segmental glomerulosclerosis are the most common phenotypes and renal histopathological manifestations of COQ8B variant. Nonetheless, our case shows that such variant may have hidden and mild clinical manifestations at the early onset. Therefore, early diagnosis will help to identify children at the early disease stage who have opportunity to benefit from oral coenzyme Q10 supplementation.

Introduction. The existence of proteinuria may be overlooked by applying the test strips. The aim of this study has been to determine the discrepancy between the findings of proteinuria detected by test strips when compared to the results of its testing with the sulfosalicylic acid. Material and Methods. The study sample consisted of 1106 subjects, who were divided into the proteinuria positive (test strips showed the presence of isolated proteinuria), and proteinuria negative group (microscopic examination revealed the presence of >10 fresh red blood cells/?L, and/or >1 dysmorphic erythrocyte/?L, and/or >10 leukocytes/?L, and/or> 1 cylinder, and/or >1 nonsquamous epithelial cells/?L, and/or >100 bacteria/?L). Both groups had the urine tested with sulfosalicylic acid. The chemical and microscopic examination of the urine was done by the analyzer LabUMat-UriSed. Results. Proteinuria was confirmed with the sulfosalicylic acid test in 96.5% of subjects from group 1 and in 85.3% of subjects from group 2. Among the patients with the negative finding of proteinuria on the test strip and with the positive sulfosalicylic acid test there was a significantly higher number of those with pathological findings of erythrocytes, leukocytes, bacteria and cylinders in the urine when compared to those of the same group with negative sulfosalicylic acid test. Conclusion. Sulfosalicylic acid test should be performed in cases of pathological microscopic findings in the urine in case of the presence of >10 fresh erythrocytes/?L and/or >1 dysmorphic eryth?rocyte/?L and/or >10 leukocytes/?L and/or >1 cylinder (except hya?line) and/or >1 nonsquamous epithelial cells/?L and/or >100 bacteria/?L even if the test strip examination is negative for proteinuria.

We performed a prospective, longitudinal study of pregnant women presenting to their first obstetrics visits to characterize the changes in spot urine protein-to-creatinine (UPCR) and albumin-to-creatinine ratios (UACR) in normotensive pregnancies, as well as identify clinical characteristics associated with isolated proteinuria and preeclampsia. We measured spot urinary albumin, protein, and creatinine at the first prenatal visit, end of the second trimester, and at delivery. In the normotensive pregnancies ( n = 142), we found that from the beginning of pregnancy to delivery, UACR increased by a median [interquartile range (IQR)] of 14.7 mg/g Cr (3.74–51.8) and UPCR by 60 mg/g Cr (30–130) ( P < 0.001 for both changes). Isolated proteinuria (defined as UPCR > 300 mg/g Cr in the absence of hypertension) was identified in 19/142 (13.4%) normotensive pregnancies. Increases in systolic and diastolic blood pressure from early pregnancy to delivery and increases in UACR from early to midpregnancy were associated with isolated proteinuria at delivery. Twelve women developed preeclampsia. Nulliparity, early, and midpregnancy diastolic blood pressures were strongly associated with the development of preeclampsia, but early changes in UACR were not. In conclusion, women who develop isolated proteinuria at delivery have a larger increase in blood pressure than women without proteinuria and have a “microalbuminuric” phase earlier in gestation, unlike women who develop preeclampsia. These findings suggest a different mechanism of urine protein excretion in women with isolated proteinuria as compared with women with preeclampsia, where proteinuria has a more abrupt onset.

Introduction The majority of women with preeclampsia have increased proteinuria. However, the 2013 Task Force on Hypertension in Pregnancy suggested that the degree of proteinuria is no longer a severe feature of preeclampsia due to a minimal correlation between proteinuria levels and pregnancy outcome. This change has brought about even more uncertainty regarding the clinical significance of isolated proteinuria in the development of pre-eclampsia. Objective To investigate the association between the severity of isolated proteinuria and later development of severe preeclampsia and other placental mediated complications. Methods Pregnant women with new onset proteinuria levels exceeding 300 mg in 24-h urine collection, who were referred to our institution between 2014 and 2017 were approached. Exclusion criteria included immediate diagnosis of preeclampsia, chronic renal disease or chronic hypertension. 104 women met inclusion criteria and were followed throughout pregnancy and until after delivery. Results Overall, 29 of 104 (28%) women developed severe preeclampsia. The rate of severe preeclampsia was significantly higher in women with proteinuria ⩾3 g⧹24 h (57.1%) compared to women with proteinuria ± 2 for women with ⩾3 g ⧹ 24 h, versus 36.4 ± 1 for women with 3 g ⧹ 24 h, (p 0.01). The median time interval (days) between the diagnosis of proteinuria and presentation of severe preeclampsia was not significantly different between women with ⩾ 3 g ⧹ 24 h (15) and 3 g ⧹ 24 h (17). Discussion Isolated proteinuria > 3 g ⧹ 24h is a significant risk factor for severe preeclampsia and HELLP syndrome with a rapid and earlier progression to clinical disease.

To examine maternal and neonatal outcomes of isolated proteinuria and define maternal characteristics for progression to pre-eclampsia.Retrospective cohort study. Data from all hospitalized pregnant women between 2009 and 2014 with new onset isolated proteinuria of over 300 mg/24 h at admission were obtained. Follow-up was performed from the time of admission to the hospital to the time of discharge postpartum. Obstetrical, maternal and neonatal outcomes were obtained.Ninety-five pregnant women diagnosed with new onset isolated proteinuria were followed to term. Thirteen women developed pre-eclampsia during pregnancy and eight developed pre-eclampsia postpartum. Maternal characteristics for progression to pre-eclampsia were greater maximal values of proteinuria. Earlier pre-eclampsia onset was associated with early-onset proteinuria and multiple gestation. Although greater values of proteinuria were associated with increased risk for intrauterine growth restriction and lower Apgar scores, maternal outcome was favorable, regardless of pre-eclampsia progression. Isolated proteinuria progressing to pre-eclampsia was associated with late pre-eclampsia onset and favorable maternal and neonatal outcomes.A significant proportion of women with new onset isolated proteinuria will develop pre-eclampsia. In these women, close follow-up is recommended until after delivery.

Idiopathic carpotarsal osteolysis is characterized by gradual lysis and resorption of bones, occurring mainly on hands and feet. It may be sporadic or hereditary and either form can manifest renal involvement. Nephropathy is seen more frequently and is more severe in the sporadic form. We present herein two new cases of unrelated boys with the sporadic form associated with nephropathy. One of the patients had focal segmental glomerulosclerosis. The other patient revealed isolated proteinuria.

SETTING Twenty-one of 209 children admitted to the intensive care unit with meningococcal septicemia developed oliguric acute renal failure necessitating renal replacement therapy. PATIENTS Twelve survivors underwent renal assessment at a median of 4.2 yrs postpresentation. RESULT Two had abnormal glomerular filtration rate, proteinuria, and hypertension; one had isolated proteinuria; and one had an isolated renal parenchymal defect on DMSA scan. CONCLUSION Long-term follow-up of this population is recommended.

The outcome of pregnancy in patients with isolated proteinuria is believed to be favorable. However, whether women with isolated proteinuria are at risk for progressing to preeclampsia has not been extensively studied.The amount of proteinuria is thought to increase in the early third trimester, irrespective of whether preeclampsia has been diagnosed. A dipstick urinalysis has a poor sensitivity (ranging from 22 to 86%) for the detection of significant proteinuria (or = 0.3 g/day). Measurements of the levels of circulating angiogenic factors such as soluble fms-like tyrosine kinase 1, soluble endoglin, vascular endothelial growth factor, and placental growth factor suggest that gestational proteinuria is a mild variant of preeclampsia. In one study, women with isolated proteinuria (or = 0.3 g/day) were found to be more likely to progress to preeclampsia than women with isolated hypertension. A considerable number of women with eclampsia exhibited proteinuria alone during their last antenatal visit performed within a week prior to their first convulsion.The outcome of women with a retrospective diagnosis of gestational proteinuria is generally favorable. However, a considerable number of women with isolated proteinuria develop hypertension and progress to preeclampsia. Therefore, the statement that the 'outcome of pregnancy in patients with isolated proteinuria is favorable' is misleading. Physicians should be aware of this type of preeclampsia when counseling patients. One possible explanation for the difficulty in diagnosing this form of preeclampsia might be the low sensitivity of the dipstick urinalysis technique for the detection of significant proteinuria.

Objectives In 2013 the American Task force on Hypertensive diseases defined new criteria for preeclampsia (pet) without proteinuria and excluded the severity of proteinuria from criteria defining pet with severe signs. Still, the majority of women presenting with pet also exhibit proteinuria. It remains disputed whether isolated proteinuria is part of the pet continuum or whether it is a physiological alternation in kidney function in pregnancy. Our aim was to examine maternal and neonatal outcomes of isolated proteinuria and define risk factors for progression to pet. Methods Retrospective cohort study. Data from all hospitalized pregnant women with isolated proteinuria of over 300 mg/24 h at admission between 2009 and 2014, was obtained. We followed this study group from the time of admission to the hospital to the time of discharge postpartum, obtaining maternal and neonatal outcomes. Results During the 5 year period 99 women were diagnosed with isolated proteinuria. 13 women developed pet during pregnancy, and an additional 8 developed pet postpartum (21%). No risk factors for proteinuria were found, however the earlier the diagnosis of proteinuria the higher were the maximal values of proteinuria reached. Risk factors for progression of pet were primiparity (p = 0.01) and greater values of proteinuria (p = 0.005). Among women who developed pet, an earlier diagnosis was made for those with early onset proteinuria (p = 0.002), well established risk factors for pet (p = 0.01) and multiple gestations (p = 0.002). Neonatal outcome was equivocal regardless of progression to pet and levels of proteinuria. Conclusions To date this is the largest series of women with isolated proteinuria followed to term. We conclude that isolated proteinuria is a risk factor for pet, with greater chances of developing pet among primiparous women with higher values of proteinuria. While proteinuria itself is negligible, chances of pet are high and close follow-up is recommended until after delivery. Disclosures S. Shinar: None. A. Schwartz: None. M. Ram-Weiner: None. M. Kupferminc: None. A. Many: None.

In 376 children, between 6 months and 5 years of age, with suspected urinary tract infection, 4 parameters of a routine urine examination were correlated with culture reports. In diagnosing urinary infection, the sensitivity and specificity of proteinuria was 79 and 80% respectively, that of bacteriuria 78 and 96% and that of pyuria10 wbc/hpf 80 and 82% respectively. 61% among the culture positive groups had all these three parameter present, as against only 0.5% in the culture negative group (P0.001). All these 3 parameters were absent in 70% in the culture negative group, as against 8% in the culture positive group (P0.001). Bacteriuria in association with either proteinuria or Pyuria10 Wbc/hpf had 98% specialty is diagnosis. In diagnosis UTI, Pyuria10 wbc/hpf was significantly more specific (82:66.6) than the conventional5 wbc/hpf. Isolated proteinuria, isolated pyuria, isolated bacteriuria and microscopic haematuria were not features of urinary tract infection in children. Urine culture can be more selectively done if the routine urinalysis is well interpreted. In the absence of dependable culture facilities, a routine urine examination can be reliably used in the diagnosis of UTI in children.

SummaryThirty cases with proteinuria either asymptomatic or associated with hypertension were selected through rigid Criteria. 17 patients are normotensive and 13 have a blood Pressure at one or multiple control over 140-90 mmHg. Renal biopsies on such patients emphasize the frequency of vascular lesions. Arteriolar lesions were the only abnormality in twenty one cases. Glomerular lesions were observed in only 8 patients. In 14 patients, with a mean age of 21, vascular lesions are noticed in the absence of hypertension. Our own observation and data of the litterature are in favour of a primary arteriolar disease.

To elucidate the morphological basis of glomerular haematuria, morphometric analysis of the glomerular basement membrane (GBM) and lamina densa (LD) was performed on silver impregnated samples for electron microscopy. The cases studied consisted of 3 groups: group A, normal controls, being from donors for kidney transplantation; group B, haematuric; and group C, non-haematuric cases with isolated proteinuria. Qualitative analysis revealed that gap formation, splitting, segmental and diffuse thinning of the GBM occur preferentially in haematuric cases. The morphometry of the GBM and LD yielded increased mean values of the GBM and of LD thickness in groups B and C. The coefficient of variation (CV, SD/mean) for the GBM and LD, however, was the highest in group B among the 3 groups, suggesting the most irregular GBM and LD in group B. In addition, CV was significantly higher in cases with splitting, segmental attenuation and gap of the GBM than cases without. The findings suggest that the irregularity of the GBM rather than its mean thickness is clearly associated with splitting and ultimately with haematuria via the gaps produced.

Presentation of the Forum is made possible by grants from Smith Kline & French Laboratories, Hoechst-Roussel Pharmaceuticals Inc., CIBA Pharmaceutical Company, GEIGY Pharmaceuticals, and Boehringer Ingelheim Ltd.

Proteinuria was found in at least one of four specimens in 10.7% and in at least two of four specimens in 2.5% of 8,954 schoolchildren, ages 8 to 15 years. To determine the risk of renal disease in isolated proteinuria, the screening program was followed by a systematic clinical evaluation of the proteinuric children. After those with both proteinuria and hematuria were excluded, none of the remaining children was found to have an overt renal disease. Despite urinary protein concentrations in excess of 1,000 mg/dl and protein excretion rates of up to 1 gm in 24 hours, proteinuria proved to be transient or intermittent in every child when a large enough number of urine samples was tested. Children with the highest protein excretion rates (more than 6 mg/hour/m 2 at night or more than 20 mg/hour/m 2 during the day) and those with the most persistent patterns of proteinuria underwent renal function studies, intravenous pyelography, and renal biopsy. No significant abnormalities was found. Mild nonspecific changes were seen in 12 of 28 biopsies, with mesangial deposits in four. The results show that if hematuria and other signs have been excluded, a benign renal morphologic picture is almost invariably to be expected in intermittent proteinuria; renal biopsy, therefore, is not indicated.

The present work comprises a follow-up study, on average four years after, of 128 cases displaying isolated proteinuria of persistent (31 cases), intermittent (55 cases) or transient type (42 cases) in a general health survey. The check-up examination included a screening with questionnaire and analysis of morning urine sent in and also a clinical examination of cases displaying pathological urine findings or suspect case histories of urinary tract disease in the screening. In the clinical examination 89 individuals took part (20 cases with persistent, 45 with intermittent and 24 with transient proteinuria). Of these 89 cases only four (4.5%) had proteinuria in all three urine examinations which were performed. All these four cases had persistent proteinuria at the primary examination. Sixty-eight cases (76%) did not have proteinuria on any occasion during the follow-up, six of whom had persistent proteinuria at the primary examination. Only 11 (12%) of the 89 clinically investigated displayed signs of urinary tract disease. Seven of these 11 cases with urinary tract disease belonged to the group with persistent proteinuria at the primary examination.

A four month old baby presented one morning a quiet, hypotonic, areflexive coma without any signs of localization, which vanished without any sequelae. Hepatic cytolysis and an isolated proteinuria with a maximum at about three days after the initial neurological problems completed the picture. A new interview with the parents looking for a toxic cause revealed without any doubt poisoning by tetrachloro-ethylene. Following this particular case, the main signs of poisoning by halogenated aliphatic hydrocarbons are recalled. One should always have in mind the possibility of poisoning at all ages, even with young babies.