Hypoparathyroidism and pseudohypoparathyroidism: etiology, laboratory features and complications

ABSTRACT Objectives To identify a clinical profile and laboratory findings of a cohort of hypoparathyroidism patients and determine the prevalence and predictors for renal abnormalities. Materials and methods Data from medical records of five different visits were obtained, focusing on therapeutic doses of calcium and vitamin D, on laboratory tests and renal ultrasonography (USG). Results Fifty-five patients were identified, 42 females and 13 males; mean age of 44.5 and average time of the disease of 11.2 years. The most frequent etiology was post-surgical. Levels of serum calcium and creatinine increased between the first and last visits (p < 0.001 and p < 0.05, respectively); and serum levels of phosphate decreased during the same period (p < 0.001). Out of the 55 patients, 40 had USG, and 10 (25%) presented with kidney calcifications. There was no significant difference in the amount of calcium and vitamin D doses among patients with kidney calcifications and others. No correlation between serum and urinary levels of calcium and the presence of calcification was found. Urinary calcium excretion in 24h was significantly higher in patients with kidney calcification (3.3 mg/kg/d) than in those without calcification (1.8 mg/kg/d) (p < 0.05). Conclusions The reduction of hypocalcemia and hyperphosphatemia suggest an effectiveness of the treatment, and the increase in serum creatinine demonstrates an impairment of renal function during follow-up. Kidney calcifications were prevalent in this cohort, and higher urinary calcium excretion, even if still within the normal range, was associated with development of calcification. These findings suggest that lower rates of urinary calcium excretion should be aimed for in the management of hypoparathyroidism.

A Case of Secondary FSGS due to Chronic Chloride Diarrhea

Congenital chloride diarrhea (CLD) is a rare autosomal recessive disease that is difficult to diagnose. CLD requires early treatment to correct electrolyte imbalance and alkalosis and to prevent severe dehydration. Renal injury is clearly associated with defective electrolyte balance induced by CLD, particularly during the first months or years of life. A 7-year-old boy was diagnosed with CLD following detection of a homozygous mutation (c.2063-1G>T) in SLC26A3 at 6 months of age. During treatment with electrolyte supplements, mild proteinuria was detected at 8 months of age, and is still present. Renal biopsy showed the presence of focal renal dysplasia, with metaplastic cartilage and mononuclear cell infiltration, calcification, and fibrosis in the interstitium. Up to two-thirds of the glomeruli exhibited global obsolescence, mostly aggregated in the dysplastic area. In nondysplastic areas, the glomeruli were markedly increased in size and severely hypercellular, with increased mesangial matrix, and displayed segmental sclerosis. The marked glomerular hypertrophy with focal segmental glomerulosclerosis suggested a compensatory reaction to the severe nephron loss or glomerular obsolescence associated with renal dysplasia, with superimposed by CLD aggravating the tubulointerstitial damage.

Renal Complications and Their Prognosis in Korean Patients with Middle East Respiratory Syndrome-Coronavirus from the Central MERS-CoV Designated Hospital

Some cases of Middle East Respiratory Syndrome-Coronavirus (MERS-CoV) infection presented renal function impairment after the first MERS-CoV patient died of progressive respiratory and renal failure. Thus, MERS-CoV may include kidney tropism. However, reports about the natural courses of MERS-CoV infection in terms of renal complications are scarce. We examined 30 MERS-CoV patients admitted to National Medical Center, Korea. We conducted a retrospective analysis of the serum creatinine (SCr), estimated glomerular filtration rate (eGFR), urine dipstick tests, urinary protein quantitation (ACR or PCR), and other clinical parameters in all patients. Two consecutive results of more than trace (or 1+) of albumin and blood on dipstick test occurred in 18 (60%) (12 [40%]) and 22 (73.3%) (19 [63.3%]) patients, respectively. Fifteen (50.0%) patients showed a random urine ACR or PCR more than 100 mg/g Cr. Eight (26.7%) patients showed acute kidney injury (AKI), and the mean and median durations to the occurrence of AKI from symptom onset were 18 and 16 days, respectively. Old age was associated with a higher occurrence of AKI in the univariate analysis (HR [95% CI]: 1.069 [1.013-1.128], P = 0.016) and remained a significant predictor of the occurrence of AKI after adjustment for comorbidities and the application of a mechanical ventilator. Diabetes, AKI, and the application of a continuous renal replacement therapy (CRRT) were risk factors for mortality in the univariate analysis (HR [95% CI]: diabetes; 10.133 [1.692-60.697], AKI; 12.744 [1.418-114.565], CRRT; 10.254 [1.626-64.666], respectively). Here, we report renal complications and their prognosis in 30 Korean patients with MERS-CoV.

Results of Open Surgical Repair of Chronic Juxtarenal Aortic Occlusion

PURPOSE: The aim of study was to review the results of open surgical repair (OSR) of chronic juxtarenal aortic occlusion (JRAO). MATERIALS AND METHODS: We retrospectively reviewed the results of OSR performed in 47 patients (male, 92%; mean age, 59.9+/-9.3 years [range, 44-79]) with chronic JRAO during the past 21 years. In order to reduce intraoperative renal ischemic time (RIT), we excised a portion of the occluded segment of the infrarenal aorta without proximal aortic clamping. We then performed suprarenal aortic clamping with both renal arteries clamped, removed the proximal aortic thrombus cap, confirmed both renal artery orifices, and moved the suprarenal aortic clamp to the infrarenal aorta to allow renal perfusion and standard aortoiliac reconstruction. We investigated early (<30 days) postoperative surgical morbidity (particularly renal function), operative mortality, and longterm patient survival. We conducted risk factor analysis for postoperative renal insufficiency. RESULTS: The mean intraoperative RIT was 10.7+/-5.5 minutes (range, 3-25), including 6 patients who underwent concomitant pararenal aortic thromboendarterectomy. Postoperatively, five (11%) patients had transient renal insufficiency, one had pneumonia, and one patient had an acute myocardial infarction. However, there was no operative mortality or newly developed dialysis-dependent renal failure. Postoperative follow up was available in 36 (77%) patients for a mean period of 6.3 years (range, 1 month-17 years). Kaplan Meier calculations of patient survival at 5 and 10 years after surgery were 91.2% and 83.6%, respectively. CONCLUSION: We have experienced short RIT, acceptable early postoperative results and long-term survival after OSR of chronic JRAO.

Renal Manifestations and Imaging Studies of Kawasaki Disease

PURPOSE: The aim of this study was to verify renal inflammation following Kawasaki disease (KD) using single photon emission computed tomography along with Technetium-99m dimercaptosuccinic acid scintigraphy (DMSA renal SPECT). METHODS: From March 2011 to October 2011, 15 patients diagnosed with KD at the National Health Insurance System Ilsan Hospital were enrolled in the study. All patients underwent DMSA renal SPECT to evaluate renal involvement during the acute phase of KD. Urine beta2-microglobulin (beta2-MG), a marker of renal proximal tubular dysfunction, was also measured to assess renal damage. RESULTS: All 15 patients had normal renal function test results. However, microscopic hematuria and pyuria were observed in 13% and 33% of the patients, respectively. Moreover, urine beta2-MG was elevated in 46% of the patients. In addition, patients were divided into two groups based on beta2-MG level: those with an increased beta2-MG level, and those with a normal beta2-MG level. No significant differences were found between these two groups in clinical characteristics, laboratory, sonography, and echocardiography findings. All patients' DMSA renal SPECT scans were normal. CONCLUSION: Our study showed that mild abnormalities in the urinalysis and elevated urine beta2-MG were the only findings of renal involvement in KD. However, no aggressive renal manifestations were detected on DMSA renal SPECT.

High levels of serum mannose-binding lectin are associated with the severity of clinical signs of leptospirosis

The clinical heterogeneity observed in leptospirosis may be associated with host factors or bacteria virulence. Human serum mannose-binding lectin (MBL) recognizes many pathogens, and low levels of this lectin are associated with susceptibility to infection. MBL is also implicated in the modulation of the inflammatory process. We determined the levels of serum MBL during leptospirosis infection. A double-antibody sandwich ELISA was used to detect the immunoreactive serum MBL. The ELISA plates were coated with monoclonal antibody to MBL and bound MBL or recombinant human MBL were detected by rabbit anti-human MBL serum. HRPO-conjugated goat anti-rabbit antibody was used for detection of the reaction. Two groups of patients seen at referral hospitals in Recife, PE, Brazil, were divided according to the year of infection, 2001 (N = 61) or 2002 (N = 57) and compared in terms of disease severity and levels of serum MBL. A group of healthy volunteers (N = 97) matched by age, gender, and ethnic background was used as control. Patients infected in 2001 had more severe outcomes than those infected in 2002, including jaundice, hemorrhage, respiratory alteration, and renal complication (P = 0.0009; chi-square test). The frequency of patients producing serum MBL >1000 ng/mL was higher in the 2001 group than in the 2002 and control groups (P < 0.01), suggesting an association of MBL level with disease severity. The involvement of MBL and genetic variation of the MBL2 gene should be further evaluated to establish the role of this lectin in the pathogenesis of leptospirosis.