Primary hyperoxaluria diagnosed after kidney transplant: A review of the literature and case report of aggressive renal replacement therapy and lumasiran to prevent allograft loss

Primary hyperoxaluria type 1 is a rare inherited disorder caused by abnormal liver glyoxalate metabolism leading to overproduction of oxalate, progressive kidney disease, and systemic oxalosis. While the disorder typically presents with nephrocalcinosis, recurrent nephrolithiasis, and/or early chronic kidney disease, the diagnosis is occasionally missed until it recurs after kidney transplant. Allograft outcomes in these cases are typically very poor, often with early graft loss. Here we present the case of a child diagnosed with primary hyperoxaluria type 1 after kidney transplant who was able to maintain kidney function, thanks to aggressive renal replacement therapy as well as initiation of a new targeted therapy for this disease. This case highlights the importance of having a high index of suspicion for primary hyperoxaluria in patients with chronic kidney disease and nephrocalcinosis/nephrolithiasis or with end stage kidney disease of uncertain etiology, as initiating therapies early on may prevent poor outcomes.     

Impaired renal function due to raised intraabdominal pressure

A 19-year-old male developed renal failure after a laparotomy for liver trauma (urinary output of 30 ml/h, plasma creatinine 220 mol/l). Surgical decompression of the abdomen was performed without any attempt at correcting the underlying pathology. This reduced the intraabdominal pressure (IAP) from 40 to 24 cm H₂O and resulted in a massive diuresis (530 ml/h). Twenty-four hours later the plasma creatinine peaked at 280 mol/l and then returned to within the normal range. This case report confirms that there is a direct relationship between IAP and renal function.     

The renal nerves in the newborn rat

Immunocytochemical methods were used to investigate the distribution of afferent [calcitonin gene-related peptide-(CGRP) immunoreactive and substance P-immunoreactive] nerves and efferent (neuropeptide Y-immunoreactive and dopamine -hydroxylase-immunoreactive) nerves in the kidneys of rats within the 1st day of life. The newborn rat kidney possesses an afferent and efferent innervation. Both afferent and efferent nerves reach the kidney in the same bundles. The afferent sensory fibers predominate overwhelmingly in the renal pelvis and ureter while the efferent fibers clearly predominate in the vasculature. The corticomedullary connective tissue contains both types of innervation with a more prominent afferent innervation (CGRP immunoreactive). Only afferent arterioles of perihilar nephrons were innervated by efferent sympathetic fibers. The distribution and extent of afferent and efferent innervation is consistent with the renal nerves playing a significant role in the transition from fetal to newborn life. The close proximity between afferent and efferent fibers suggests a possible interaction between the two systems.     

慢性肾功能衰竭患者畸变产物耳声发射分析

测定２１例（４２耳）慢性肾功能衰竭（ＣＲＦ）患者的畸变产物耳声发射（ＤＰＯＡＥ）。结果表明：ＣＲＦ患者的耳聋 为耳蜗性聋,表现为高频听力损失,ＤＰＯＡＥ检测可客观、快速地反映耳蜗的病变。且ＤＰＯＡＥ具有频率特异性,有助于对该病患者听力障碍的早期诊断。     

45例肾血管肌脂瘤的治疗

目的：探讨肾血管肌脂瘤的治疗方法。方法：４５例患者中２３例未作特殊治疗;６例超选择性动脉栓塞治疗、７例行肿瘤剜除术或肾部分切除术、９例行全肾切除术。结果：随访２１例,平均随访时间３．６ａ。未作特殊治疗的病例中未发现肿瘤破裂出血,２便肿瘤体积增大但我症状;超选择性动脉栓塞病例无症状复发、无破裂出血、肾功能稳定。手术患者均保持肾功能稳定,无肿瘤复发。结论：对本病采取保守性治疗原则。超选择肾动脉栓塞是简     

Cationic amino acid fluxes beyond the proximal convoluted tubule of rat kidney

To investigate the fluxes of cationic amino acids beyond the proximal convolution, we micropunctured and microperfused superficial tubules of male Wistar rats in vivo et situ. In free-flow micropuncture experiments, the concentrations of endogenous L-arginine⁺, [Arg], and of intravenously infused L-homoarginine⁺, [HoArg], were determined by HPLC. Fluorescein isothiocyanatelabeled inulin was detected on-line in the same tubular fluid samples. To determine undirectional fluxes, radiolabeled Arg and inulin were (1) microperfused through short loops of Henle and (2) microinfused into different tubule segments to measure urinary recovery of the radiolabel. At a mean [Arg]_plasma of 116 mol/l, [Arg] was 9.3 mol/l in the late proximal tubule (LPT), and 35.6 mol/l in the early distal tubule (EDT) corresponding to fractional deliveries (FD) of 0.055 in LPT and 0.078 in EDT. Fractional urinary excretion (FE) of Arg was 0.00033 (P<0.05 vs FD_EDT). Infusion of HoArg (2.5 or 7.5 mol/min) led to respective mean [HoArg]_plasma values of 1.44 and 3.73 mmol/l, and resulted in respective FD_LPT values for HoArg of 0.23 and 0.53, respective FD_EDT values of 0.29 and 0.41, and finally, respective FE values for HoArg of 0.25 and 0.58. When short loops of Henle were microperfused with 1 or 50 mmol/l [¹⁴C]Arg (+[³H]inulin), fractional recovery (FR) of ¹⁴C (relative to inulin) in the EDT was 0.13 and 0.36, respectively. During microinfusion of radiolabeled Arg (1 or 50 mmol/l) and inulin into LPT, the urinary FR of the radiolabel was 0.14, or 0.59, respectively. If 0.007, 1 or 50 mmol/l radiolabeled Arg were microinfused into EDT, the respective urinary FR of the radioactivity was 1.02, 1.10, or 1.01. Microperfusion of microinfusion of 1 mmol/l [¹⁴C]Arg plus 50 mmol/l HoArg resulted in a FR_EDT of ¹⁴C of 0.43 (loop, perfusion) and an FE for ¹⁴C of 0.69. Five conclusions can be drawn. First, cationic amino acids can enter and leave the lumen of short loops of Henle through specific carrier(s) at high rates, although, secondly, net transport is small or absent. Thus, medullary tubule cells can be supplied with Arg from the lumen of short loops of Henle for urea and nitric oxide production. Thirdly, the distal convolution of superficial nephrons and the collecting duct are not permeable to Arg. Thus, fourthly, the difference between FD_EDT and urinary FE of Arg must be explained by an inter-nephron heterogeneity between deep and superficial nephrons. Finally, the process responsible for the different Arg handling in deep nephrons is not accessible to HoArg or, if so, it is saturated at millimolar concentrations.     

The endothelium-derived relaxing factormediated acetylcholine response of the arterial perfusion pressure after cold storage of the isolated rabbit kidney

The vasodilatation induced by acetylcholine (ACh) in a rabbit isolated perfused kidney was abolished when the tissue was exposed to cold ischemia for 72 h in Euro-Collins (EC) solution. This vasodilatation is due to the release of endothelium-derived relaxing factor (EDRF) from renal vasculature as evidenced by the attenuation following methylene blue pretreatment. When kidneys were preserved in EC solution containing UK 38 485, a thromboxane synthase inhibitor, or nicardipine, a calcium channel blocker, ACh-induced vasodilatation persisted after 72 h of cold ischemia. These results were taken as evidence of tissue protective activity of UK 38 485 and nicardipine and have promising implications for cadaveric kidney transplantation.This paper was presented at the 35th World Congress of the International Society of Surgery in Hong Kong in August 1993