Successful living donor liver transplantation for classical maple syrup urine disease

MSUD is an autosomal recessive condition characterized by a deficiency in the enzyme, BCKDH, which catalyzes the breakdown of BCAAs. If left untreated, MSUD can result in mental retardation, central nervous system disorders, and even death. Most patients with MSUD are treated with a restricted protein diet and milk from which BCAAs have been removed. LT has been shown effective in patients with MSUD. This report describes the case of a 15‐month‐old boy who received a liver graft from his mother. Transplantation was successful, and the patient was then able to ingest a normal diet. Despite episodes of acute rejection, chylous ascites, and high fever (40 °C), he has shown no evidence of MSUD recurrence. These findings indicate that patients with MSUD can be successfully treated by LDLT, even when the donor is a heterozygous carrier of a mutated BCKDH gene.     

Endolymphatic space size in patients with Meniere’s disease and healthy controls

Conclusions: The incident ratios of cochlear and/or vestibular endolymphatic hydrops (EH) were significantly higher in the affected ears of patients with Meniere’s disease (MD) than in the ears of healthy controls. There were no significant differences between controls and the contralateral ears of MDs. Objectives: The aim was to compare the incidence ratios of EH between unilateral/bilateral MD and controls using 3T magnetic resonance imaging (MRI) with intravenous gadolinium. Methods: A total of 41 patients were diagnosed with MD: 32 with unilateral MD (uMD) and nine with bilateral MD (bMD). Fifteen healthy volunteers were enrolled as controls. The patients underwent 3T MRI 4?h after intravenous injection of gadolinium. Results: Cochlear EH was present in 3.3% of 30 ears of 15 controls, 6.3% of 32 contralateral (contra) ears of 32 uMDs, 62.5% of 32 affected ears of 32 uMDs, and 55.6% of 18 affected ears of nine bMDs. Vestibular EH was observed in 6.7% of control ears, 3.1% of contra-uMD ears, 65.6% of affected uMD ears, and in 55.6% of affected bMD ears. Either cochlear or vestibular EH was present in 10.0% of control ears, 6.3% of contra-uMD ears, 81.3% of affected uMD ears, and 44.4% of affected bMD ears.     

Use of high‐flow nasal cannula in neonates: Nationwide survey in Japan

High‐flow nasal cannula is a new modality of respiratory support and is increasing in popularity despite the lack of supporting evidence. We investigated the prevalence of its use in tertiary neonatal units in Japan. A paper‐based survey was conducted. The response rate was 83%. High‐flow nasal cannula was used in 46/80 units (58%), of which 96% used the high‐flow nasal cannula without guidelines. It was used for several indications, including weaning off nasal continuous positive airway pressure and post‐extubation respiratory support. The main perceived benefits of the cannula included better access to the neonate and reduced risk of nasal trauma. This survey found that high‐flow nasal cannula is used without clear criteria and that clinical practice varies across neonatal units in Japan. Its use in neonates needs to be urgently evaluated.     

Extracapillary proliferation and arteriolar hyalinosis are associated with long-term kidney survival in IgA nephropathy

Background

The Oxford classification of IgA nephropathy consists of four markers as prognosticators. We retrospectively examined the relevance of extracapillary proliferation involving cellular and fibrocellular crescents (Ex) and arteriolar hyalinosis (A) on the long-term outcome of renal function.

Methods

A total of 314 Japanese patients who were diagnosed with IgA nephropathy, with 12 months or more of follow-up period were included in this study. A total of 186 patients were with UP ≥ 0.5 g/day. Patients with diabetes mellitus or severe kidney injury (eGFR < 30 ml/min/1.73 m²) were excluded. The presence of Ex and A were scored 0 in the absence, and 1 in the presence, of each lesion. The end point was determined as a 50 % reduction in initial eGFR or end-stage renal disease defined as eGFR < 15 ml/min/1.73 m².

Results

In univariate analyses, the kidney survival rate was significantly lower in patients with Ex1 and A1 if UP ≥ 0.5 g/day. In the patients with UP < 0.5/day, none of the clinical and pathological parameters was determined as a risk factor. In the multivariate model including pathological parameters, Ex1 and A1 were independent risk factors for renal outcome if UP ≥ 0.5 g/day. In those patients treated with RAS-blocker or treated before introduction of methylprednisolone pulse therapy, Ex was the only independent risk factor. In multivariate analysis including clinical parameters, eGFR alone was a risk factor, due to strong correlation with other parameters.

Conclusion

Ex and A would be associated with the renal outcome of the patients with UP ≥ 0.5 g/day.

     

Death and kidney allograft dysfunction after bacteremia

Background

Some studies have reported causal associations between bacteremia and mortality or allograft loss in kidney transplant recipients (KTR). However, few studies have assessed the clinical course of kidney function and the risk of acute allograft rejection after bacteremia.

Methods

We retrospectively reviewed 902 kidney transplants performed at Nagoya Daini Red Cross Hospital between January 1, 2002 and March 31, 2014. Forty-five living donor kidney transplant recipients with single bacteremia were included. We analyzed death, change in kidney function, and development of acute allograft rejection 12 months after bacteremia according to the following groups: primary source of bacteremia (urinary tract or other sources), site of acquisition (community acquired or nosocomial), severity (not meeting the systemic inflammatory response syndrome criteria and sepsis or severe sepsis and septic shock), empiric antibiotic use (appropriate or inappropriate), and baseline kidney function (estimated glomerular filtration rate ≤44.7 or ≥44.8 ml/min).

Results

Urinary tract infection (UTI) was the leading cause of bacteremia (68.9 %), and Escherichia coli was the most common pathogen. Three cases (6.7 %) died of infection that caused bacteremia within 12 months. Pneumonia accounted for two-thirds. Kidney function declined 1 week after bacteremia (P < 0.05), particularly in severe cases. Thereafter, kidney function was comparable to baseline level in each group (P ≥ 0.05). Severe UTI was associated with subsequent acute allograft rejection (P = 0.03).

Conclusions

Pneumonia in KTR should be managed with caution. Kidney function generally returned to baseline level after bacteremia. However, severe UTI may be associated with subsequent acute allograft rejection.