[Sickle cell trait complications: A case series of 6 patients]. / Complications du trait drépanocytaire : à propos d'une série de 6 cas.

INTRODUCTION: Patients with sickle cell trait (SCT) are commonly considered as asymptomatic carriers. However, some clinical manifestations may occur. METHODS: Here we present a retrospective descriptive study about SCT subjects with at least one complication diagnosed in a sickle cell disease referral center, in Paris, between 2008 and 2019. We also performed a literature review on the complications of SCT subjects. RESULTS: Six patients (between 19 and 65 years old) were included. SCT was already known only for 4 of them at the time of the complication. Four patients presented with a splenic infarct after a stay in high altitude or a plane trip, one of them was associated with papillary necrosis; one patient had isolated papillary necrosis, and the last one had splenic sequestration. These complications happened for most of them after exposure to an unusual situation of hypoxia or deshydratation. Five out of 6 patients had a marked elevated C reactive protein. CONCLUSION: SCT may cause acute ischemic complications in a context of prolonged hypoxia or dehydration. The most commonly reported are the splenic infarct and the renal papillary necrosis. A study of hemoglobin should be considered in these clinical situations in patients with compatible ethnic origin.

Renal papillary necrosis in patients with sickle cell disease: How to recognize this 'forgotten' diagnosis.

INTRODUCTION: Renal papillary necrosis is not commonly seen in daily practice, but can have severe consequences when it is not diagnosed in time. It is known to be associated with sickle cell hemoglobinopathies; however a wide range of etiologies are possible, and it is therefore not the first diagnosis clinicians consider in patients with sickle cell disease who present with hematuria. METHODS: A literature search was performed to summarize the current knowledge about renal papillary necrosis associated with sickle cell disease. These findings are illustrated with a case of a 9-year old girl with sickle cell disease who was referred with painless gross hematuria. RESULTS: Typical radiologic signs for renal papillary necrosis are necrotic cavities that fill with contrast, small collections of contrast peripheral to the calyces in the papillary region (ball-on-tee sign), calcification of the papillary defect, filling defects, hydronephrosis, blunted papillary tip, clefts in the renal medulla filled with contrast, hyperattenuated medullary calcifications, non-enhanced lesions surrounded by rings of excreted contrast, and clubbed calyces. DISCUSSION: This study focuses on the pathophysiology of renal papillary necrosis associated with sickle cell disease, the possible symptoms, as well as the diagnostic steps, with a special interest in particular presentation on old (retrograde pyelography) and new (computed tomography) gold standard in radiologic imaging, and the management for this pathology. CONCLUSION: This study aims to remind clinicians of this "forgotten" diagnosis and what signs to look for in pediatric patients with sickle cell disease who present with hematuria. In pediatric cases radiation protection is important, therefore knowing what radiologic signs can be found on retrograde pyelography can lead to early identification of this pathology without having to proceed to computed tomography.

Mucormycosis of the transplanted kidney with renal papillary necrosis.

The occurrence of renal allograft mucormycosis is uncommon, but its association with renal papillary necrosis has not been reported. We describe such an association in a patient who survived on peritoneal dialysis after nephrectomy and antifungal therapy.

Bilateral ureteric obstruction secondary to renal papillary necrosis.

A 2-year-old male presented to hospital with a 5-day history of vomiting and pyrexia. He was initially treated with non-steroidal medication as an anti-pyretic. Initial investigations demonstrated a raised urea and creatinine and he was treated with intravenous fluids. Within 24 h he became anuric with progressive renal insufficiency. Ultrasound scan demonstrated minimal bilateral hydronephrosis with debris in the lower pole calyces. The bladder was empty. Cystoscopy and retrograde contrast imaging revealed bilateral ureteric obstruction. Double J stents were inserted and his renal function returned to normal within 4 days. We believe the aetiology to be renal papillary necrosis and bilateral ureteric obstruction secondary to the administration of ibuprofen in association with dehydration.

[Diagnostic image (394). A man with a tissue fragment in the urine. Papillary necrosis]. / Diagnose in beeld (394). Een man met een weefselbrok in de urine. Necrose van een nierpapil.

A 54-year-old man with chronic renal failure due to diabetic nephropathy presented with macroscopic haematuria and excretion of necrotic tissue in the urine as a sign of papillary necrosis.

Indinavir-associated toxicity mimicking urinary tuberculosis in a patient with AIDS.

This case reported to a patient with AIDS who presented persistent sterile leukocyturia and hematuria, lower back pain, bladder suffering symptoms, and renal papillary necrosis which were thought to be secondary to urinary tuberculosis but were demonstrated to be indinavir-associated side effects. The intention of this report is to remind medical professionals involved in the care of HIV+ patients of this possible association in order to avoid unnecessary investigation and to stress the need of careful periodical assessment of renal function and urinalysis in patients treated with indinavir.

Indinavir-associated toxicity mimicking urinary tuberculosis in a patient with AIDS

This case reported to a patient with AIDS who presented persistent sterile leukocyturia and hematuria, lower back pain, bladder suffering symptoms, and renal papillary necrosis which were thought to be secondary to urinary tuberculosis but were demonstrated to be indinavir-associated side effects. The intention of this report is to remind medical professionals involved in the care of HIV+ patients of this possible association in order to avoid unnecessary investigation and to stress the need of careful periodical assessment of renal function and urinalysis in patients treated with indinavir.

An unusual cause of renal allograft dysfunction: graft papillary necrosis.

A 43-year-old nondiabetic man, 5 years post-renal transplantation, presented complaining of oliguria, fever and dysuria of 1-day duration. Graft ultrasound did not reveal any obstructive changes. Graft function did not improve in spite of 3 days of antibiotics. On the fourth day he passed fleshy material in urine subsequent to which his urine output improved and fever recovered. His graft function settled near to the previous baseline. Histological analysis of the material revealed necrosed renal papillary tissue. Renal papillary necrosis in allograft is uncommon and generally reported in the immediate postoperative phase, but it can still occur later in transplant follow-up. It is a potentially treatable cause for acute allograft dysfunction and should be suspected in transplant patients presenting with acute pyelonephritis but not getting relief from antibiotic therapy.

Acute renal papillary necrosis with complete bilateral ureteral obstruction in a child.

A 9-year-old girl presented with apparent meningococcal septicemia and developed acute renal failure after 48 hours of treatment with antibiotics and analgesics. Early ultrasound scanning demonstrated mild bilateral hydronephrosis and hydroureter. Intravenous urography showed slow contrast uptake with delay nephrogram and no contrast entering the bladder. Repeat ultrasonography revealed bilateral papillary irregularity and echogenic debris in the distal ureters. Bilateral double-J stents were inserted cystoscopically, resulting in prompt polyuria and a return of normal renal function. Although rare, recognition of sloughed papilla in papillary necrosis causing ureteral obstruction can lead to early management with no long-term sequelae.

[Diagnosis and conservative treatment of acute pyelonephritis in diabetes mellitus].

AIM: To raise efficacy of conservative treatment of patients with acute pyelonephritis (AP) which developed in the presence of diabetes mellitus (DM). MATERIAL AND METHODS: Elderly patients (n = 218, 182 females and 36 males) with DM and AP enered the study. DM type 1 and 2 were diagnosed in 41 and 177 of them. Pyelonephritis was diffuse-purulent, pyodestructive, calculous, pyocalculous. RESULTS: Conservative treatment was made in 160 patients, surgical treatment--in 58. Lethal outcomes (14 cases) were registered as a rule in patients with bilateral pyodestructive pyelonephritis. CONCLUSION: AP is a severe complication of DM. The severity of DM decompensation and metabolic disorders is proportional to AP severity. AP in diebetics runs often an asymptomatic course and the diagnosis is difficult. Therapeutic policy is individual with consideration of a clinical course. Positive results were achieved in 80% patients.

Insuficiencia renal aguda asociada a papilitis necrotizante: reporte de un caso clínico / Acute kidney failure associated to papillary necrosis: a clinical case report

La papilitis necrotizante es una complicación de diversas patologías que tienen en común el desarrollo de algún grado de isquemia renal, las más importantes son la Diabetes Mellitus, la nefropatía por antiinflamatorios no esteroidales y la infección urinaria, estando esta última condición presente en más de 2/3 de los casos. Su espectro clínico es muy amplio, desde casos asintomáticos hasta casos de insuficiencia renal aguda obstructiva y muerte, de no mediar tratamiento oportuno. Este artículo presenta un caso clínico de papilitis necrotizante, con el fin de ilustrar las características clínicas principales de esta patología, de alta mortalidad y probablemente subdiagnosticada en nuestro medio.

[Wegener's granulomatosis in the head and neck region]. / Morbus Wegener im Kopf-Hals-Bereich.

Wegener's granulomatosis (WG) is defined as a granulomatous inflammation of the upper and lower respiratory tract and systemic vasculitis of small and medium sized vessels which is often accompanied by a necrotizing glomerulonephritis. The etiology of the disease is still unknown. In former times untreated WG usually ended deadly. Immunosuppressive therapy made WG a treatable disease with a chronically relapsing course. Therefore an early diagnosis of WG is of utmost importance. WG usually starts as a limited and localized organ manifestation in the upper respiratory tract and it generalizes, if untreated, with pulmonary and renal involvement. Symptoms in the head and neck region are observed in up to 95% of the patients with WG. Sinusitis, crusting of the nose, development of a saddle nose, middle and inner ear symptoms and subglottic stenosis are common manifestations. Due to the early and common manifestation of WG in the head and neck region the otorhinolaryngologist plays an important role for the early diagnosis and the fast initiation of immunosuppressive therapy but also during follow-up for activity assessment.

99mTc ethylene dicysteine scintigraphy for diagnosing cortical defects in acute pyelonephritis: a comparative study with 99mTc dimercaptosuccinic acid.

AIM: To evaluate the clinical usefulness of early cortical phase images in the diagnosis of acute pyelonephritis and to compare the measurements of differential renal function obtained by both 99mTc ethylene dicysteine (99mTc-EC) and 99mTc dimercaptosuccinic acid (99mTc-DMSA). METHODS: Forty-three children who had undergone both 99mTc-EC and 99mTc-DMSA studies within 5 days of acute infection were studied. Cortical images of 99mTc-EC were obtained by the sum of the renogram frames achieved between the first 60-120 s of the renogram study. DMSA and EC images were visually interpreted using four points of semiquantitative ratings: 0, normal; 1, mild hypoactivity; 2, moderate hypoactivity with partial loss of margins; 3, marked hypoactivity with loss of cortical margins. Values for the differential renal function were obtained for both studies. RESULTS: DMSA detected a total of 109 lesions in 36 patients and EC detected 90 lesions with a sensitivity of 82.5%. 99mTc-EC successfully detected moderate-to-severe cortical lesions but was less effective with mild lesions (sensitivity 60%). Bland-Altman analysis demonstrated good agreement among the results for differential renal function (95% CI -0.26 to 0.96). CONCLUSION: 99mTc-EC early phase images detected most of the cortical lesions. Its sensitivity depends on the severity of the lesions. EC provided reliable information in the estimation of differential renal function.