William D. Plant

Management of comorbidities in older patients with cystic fibrosis

Several key advances have been made in the treatment and management of people with cystic fibrosis in the past two decades. Substantial improvements in survival have resulted from the introduction of key drugs, coordinated care packages, improved nutritional support, and the intensive use of antibiotics. The age profile of people with cystic fibrosis has changed greatly during this time-some countries now have more adult than paediatric patients with the disease. With their increasing age and more advanced lung disease, several important sequelae (both pulmonary and extrapulmonary) occur in these adult patients including pulmonary disease, cystic fibrosis-related diabetes, renal disease, metabolic bone disease, cancers, drug allergies and toxic effects, and complications associated with lung transplantation.

Gitelman's syndrome in pregnancy: case report and review of the literature

Gitelmans syndrome (GS), a rare renal disorder, results in hypokalaemia, hypomagnesaemia, hypocalciuria and a metabolic alkalosis. It is unclear if an alteration in management is necessary or beneficial during pregnancy. A 32-year-old woman with GS was managed in her second pregnancy. Antenatally, the patient required 39 (principally day case) admissions to the hospital for intravenous (IV) therapy and received a cumulative total of 47 l of IV 0.9% saline solution, 47 doses of 20 mmol magnesium chloride and 46 doses of 80 mmol potassium chloride. She delivered a 2940-g female infant in excellent condition by caesarean section. We would suggest that close attention to maternal weight gain during pregnancy is an easily available clinical tool to assess adequacy of fluid and electrolyte repletion in this condition.

Do patient-reported measures of symptoms and health status predict mortality in hemodialysis? An assessment of POS-S Renal and EQ-5D

Introduction Experience with the use of patient‐reported outcome measures such as EQ‐5D and the symptom module of the Palliative care Outcome Scale—Renal Version (POS‐S Renal) as mortality prediction tools in hemodialysis is limited.

Peri-Lung Transplant Renal Issues in Patients With Cystic Fibrosis

journal.publications.chestnet.org Financial/nonfi nancial disclosures: The authors have reported to CHEST that no potential confl icts of interest exist with any companies/organizations whose products or services may be discussed in this article . Correspondence to: Constantine A. Manthous, MD, FCCP, Department of Internal Medicine, The Hospital of Central Connecticut, 100 Grand St, New Britain, CT 06050; e-mail: cmanthous@ thocc.org

A rare complication of transitional cell carcinoma of the renal pelvis: parathyroid hormone-related peptide-induced hypercalcaemia

We describe a rare occurrence of parathyroid hormone-related peptide (PTHrp) associated hypercalcaemia with a recurrence of transitional cell carcinoma of the renal pelvis. Our patient presented with serum calcium of 3.9 mmol/L, PTH of 5 ng/L and a PTHrp of 9.8 pmol/L (<2 pmol/L). He had no evidence of metastatic disease. His hypercalcaemia responded to bisphosphonate therapy. He chose to be treated conservatively and died 5 weeks after presentation. This is the seventh such case described in the literature. PTHrp-induced hypercalcaemia is associated with a grave prognosis, with a mean survival of 65 days from presentation.

Haemodialysis-induced syncope due to Chiari II malformation

Spina bifida (SB) is associated with chronic kidney disease as a result of vesicoureteric reflux. A proportion of patients progress to end-stage kidney disease (ESKD). Haemodialysis (HD) is probably the most common modality in ESKD, as intra-abdominal malformations and previous surgery can make peritoneal dialysis more challenging. The Chiari malformations also frequently occur in these patients. We report a case of recurrent syncope induced by HD in a patient with SB and the Chiari II malformation. Sparse data exist on the complications of HD in this patient population and on the approach to the management of dialysis-induced syncope in these individuals.

Intussusception of the small bowel in an adult associated with nephrotic syndrome.

Sir, Here, we report our observations of a 39-year-old female who presented with progressive lower limb oedema over a 2-week period. She had no significant medical or family history. Physical examination revealed a right-sided pleural effusion and bilateral lower limb swelling. Laboratory investigations confirmed the clinical suspicion of nephrotic syndrome: albumin 14 g/L, creatinine 60 μmol/L, cholesterol 8.8 mmol/L, urinary protein–creatinine ratio (PCR) 997 mg/mmol. Complements were normal, and autoimmune serology was negative. Percutaneous renal biopsy was performed and demonstrated features consistent with minimal change disease. She was commenced on fluid and salt restriction and high-dose loop diuretics. She also received prednisolone 1 mg/kg orally [1]. Five days post-renal biopsy, she developed acute left-sided colicky abdominal pain. On physical examination, her abdomen was tender in the left iliac fossa with no signs of peritonism. The abdominal film was unremarkable. Abdominal ultrasound showed an iliocolic intussusception (Figure 1). Fig. 1 Transverse section of intussuception. Bull’s-eye sign/target/crescent-in-doughnut. A. Intussicepiens. Concentric rings of alternating hypoechoic and hyperechoic layers. B. Returning limb of intussuceptum. C. Mesentery of intussuceptum. Central ... She remained clinically and biochemically nephrotic at this time. She entered the remission phase of nephrotic syndrome between Days 7 and 10 after the initiation of therapy which coincided with the complete resolution of her abdominal pain. Gastrointestinal disturbances are frequently encountered in the course of nephrotic syndrome. The differential diagnosis considered included renal vein thrombosis, peptic ulcer disease and subacute bowel obstruction. Fortuitously, at the time of ultrasonography, the patient developed an episode of colicky abdominal pain, and the intussusception could be demonstrated. Ultrasonography is the diagnostic tool of choice to detect intussusception, although it can be operator dependent or limited by body habitus. Intussusception causes ‘telescoping’ of the bowel due to a lead point in the bowel, which in this case is due to incoordinate gut motility and bowel wall oedema. Intussusception is not infrequently described in the paediatric literature, but the usual cause in adults is secondary to a bowel tumour, which acts as a lead point for the invagination of the bowel [2]. Treatment of the underlying nephrotic syndrome resulted in resolution of the intussusception without the need for any intervention [3,4]. Infusions of albumin have also been described [5]. We conclude that nephrologists should consider intussusception in the differential diagnosis of abdominal pain in the setting of nephrotic syndrome as early recognition may improve prognosis. Conflict of interest statement. None declared.