Empyema complicated with nephropleural fistula and perinephric abscess.

The coexistence of empyema, nephropleural fistula and perinephric abscess is rare and presents diagnostic challenges, especially when patients exhibit atypical or mild symptoms. This case report details a female in her early 90s who presented with 3 weeks of chest pain and was found to have a left-sided pleural effusion. Further imaging revealed additional findings of a left-sided nephropleural fistula and left perinephric collection. The patient underwent thoracentesis and video-assisted thoracoscopic surgery, with cultures growing Streptococcus Anginosus Subsequently, she underwent a left nephrectomy, with pathology findings consistent with chronic pyelonephritis and a fistulous tract. The patient was discharged on intravenous antibiotics. Follow-up imaging showed improvement. The patient completed the antibiotic course and reported symptom resolution 2 weeks after discharge.

KIF11 Variants Associated With Novel Renal System Involvement-Two Cases That Expand the Phenotypic Spectrum of Microcephaly With or Without Chorioretinopathy, Lymphedema, or Impaired Intellectual Development.

Pathogenic variants in KIF11 are linked to microcephaly with or without chorioretinopathy, lymphedema, or impaired intellectual development (MCLMR). To our knowledge, renal phenotypes have not been described in the literature in association with KIF11-related disorders. This study is a case report of two probands with heterozygous pathogenic variants in KIF11 who presented with the common clinical features of MCLMR but also had additional renal involvement not previously reported as associated phenotypes of MCLMR, elucidating phenotypic expansion of this syndrome.

The cryptonephridial/rectal complex: an evolutionary adaptation for water and ion conservation.

Arthropods have integrated digestive and renal systems, which function to acquire and maintain homeostatically the substances they require for survival. The cryptonephridial complex (CNC) is an evolutionary novelty in which the renal organs and gut have been dramatically reorganised. Parts of the renal or Malpighian tubules (MpTs) form a close association with the surface of the rectum, and are surrounded by a novel tissue, the perinephric membrane, which acts to insulate the system from the haemolymph and thus allows tight regulation of ions and water into and out of the CNC. The CNC can reclaim water and solutes from the rectal contents and recycle these back into the haemolymph. Fluid flow in the MpTs runs counter to flow within the rectum. It is this countercurrent arrangement that underpins its powerful recycling capabilities, and represents one of the most efficient water conservation mechanisms in nature. CNCs appear to have evolved multiple times, and are present in some of the largest and most evolutionarily successful insect groups including the larvae of most Lepidoptera and in a major beetle lineage (Cucujiformia + Bostrichoidea), suggesting that the CNC is an important adaptation. Here we review the knowledge of this remarkable organ system gained over the past 200 years. We first focus on the CNCs of tenebrionid beetles, for which we have an in-depth understanding from physiological, structural and ultrastructural studies (primarily in Tenebrio molitor), which are now being extended by studies in Tribolium castaneum enabled by advances in molecular and microscopy approaches established for this species. These recent studies are beginning to illuminate CNC development, physiology and endocrine control. We then take a broader view of arthropod CNCs, phylogenetically mapping their reported occurrence to assess their distribution and likely evolutionary origins. We explore CNCs from an ecological viewpoint, put forward evidence that CNCs may primarily be adaptations for facing the challenges of larval life, and argue that their loss in many aquatic species could point to a primary function in conserving water in terrestrial species. Finally, by considering the functions of renal and digestive epithelia in insects lacking CNCs, as well as the typical architecture of these organs in relation to one another, we propose that ancestral features of these organs predispose them for the evolution of CNCs.

Disseminated Mycobacterium chelonae infection in kidney transplant patients.

Mycobacterium chelonae (M. chelonae) is a member of the rapidly growing non-tuberous mycobacteria and can cause disseminated tissue infection, particularly, in the limbs. We reviewed medical records of two kidney transplant patients. We describe their background disease and transplantation details, with the use of immunosuppressive medication. We also discuss the presentation of M. chelonae infection and treatment. Both patients received deceased brain-dead donor kidney transplants for end-stage kidney disease. Both developed cutaneous manifestations of M. chelonae, progressing to disseminated infections. Case 1 was on low-dose prednisolone (2 mg) and tacrolimus, whereas, case 2 received varying doses of prednisolone (5-40 mg) and sirolimus. Antibiotics advised by infectious disease specialists were initiated within a month of skin lesion appearance. Effective treatment involved a combination of antibiotics such as clarithromycin, azithromycin, linezolid and tigecycline. These cases underline the efficacy of clarithromycin and azithromycin as long-term antibiotic treatment, with linezolid and tigecycline for management of acute dissemination.

Post nephrectomy metachronous testicular metastasis in a case of clear cell renal cell carcinoma (ccRCC).

It is highly uncommon for solid tumours to metastasise to the testis. Here, we report a case of metachronous testicular metastasis from clear cell renal cell cancer (RCC) in a male patient 3 years after left radical nephrectomy. Ultrasound of the scrotum showed a 3.5 cm × 4 cm left testicular mass with normal serum tumour markers. The patient underwent left high inguinal orchidectomy, which revealed metastatic renal cell carcinoma. CT of the chest, abdomen and pelvis showed multiple liver secondaries. Cabozantinib was started for metastatic RCC, and the patient showed no evidence of disease progression in a follow-up of 1 year.

ANCA-negative granulomatosis polyangiitis: an airway emergency.

Granulomatosis polyangiitis (GPA) is an autoimmune condition causing inflammation of small blood vessels. It is a rare disorder that may affect various parts of the body. The diagnosis is often based on clinical examination, laboratory investigations and tissue biopsy. In about 10-20% of patients, the anti-neutrophilic cytoplasmic antibody (ANCA) can be negative, and histology maybe inconclusive, which can lead to diagnostic uncertainty. Failure to treat vasculitis can lead to morbidity and even mortality. We present a case report of a gentleman who was presented with an airway emergency with inflammation of the nasal cavity and subglottic involvement amounting to airway stenosis. His ANCA was negative and tissue biopsy from the subglottis was inconclusive. He underwent urgent dilatation of his airway, local therapies to the nose and was commenced on 10 cycles of cyclophosphamide. A follow-up of over 4 years has not shown any relapse of his disease clinically or biochemically. We discuss the clinical findings, diagnostic dilemma and multidisciplinary management of this life-threatening condition.

Measuring insect osmoregulation in vitro: A reference guide.

Osmoregulation is influenced by a wide variety of biotic and abiotic variables, and maintenance of systemic osmoregulatory homeostasis is critical to insect fitness. Because insects are so small, accurately quantifying renal organ function is technically challenging, and often requires specialized equipment. On top of this, nearly a century of toiling in the laboratory has led to a wide and still growing variety of methods that can be difficult for novice researchers to disentangle. Here, we provide a reference guide for the most used in vitro approaches in the study of insect osmoregulation, including the Ramsay assay, Ussing chamber, epithelial potential measurement, scanning ion-selective electrode technique, and hindgut assays. Along the way, we highlight the history of each methodological innovation.

Concurrent existence of cold agglutinin disease and ANCA-associated vasculitis with fulminant pulmonary-renal syndrome poses significant treatment challenges.

We present a case of a man in his 40s with pulmonary-renal syndrome due to myeloperoxidase-positive antineutrophil cytoplasmic antibodies-associated vasculitis and concurrent cold agglutinin disease, a combination that has not yet been described in the literature. The fulminant course of the disease, including the need for kidney replacement therapy and mechanical ventilation posed a significant treatment challenge due to haemolytic complications.

ANCA negative pulmonary vasculitis: a challenging diagnosis.

A man in his 40s with end-stage kidney disease due to IgA nephropathy and receiving peritoneal dialysis presented with a 1-week history of breathlessness, cough and nosebleeds. CT scan of the chest revealed ground glass changes while blood tests indicated elevated inflammatory markers and a negative vasculitis screen. This included negative ANCA and anti-GBM antibodies. Initial treatment for suspected atypical pneumonia with antibiotics yielded no clinical improvement.Over the course of the admission, his symptoms progressively worsened, leading to oxygen dependency with a FiO2 of 40% and episodes of haemoptysis. Suspicions of pulmonary vasculitis arose due to clinical deterioration, prompting consultation with a tertiary vasculitis centre. It was subsequently concluded that the clinical and radiological findings correlated with ANCA-negative pulmonary vasculitis or a rare case of IgA-associated pulmonary capillaritis. Treatment with methylprednisolone and rituximab led to significant improvement, allowing rapid oxygen withdrawal. The patient was discharged with a tapering prednisolone regimen.

Mathematical modelling of the influence of ACE I/D polymorphism on blood pressure and antihypertensive therapy.

The angiotensin-converting enzyme (ACE) gene (ACE) insertion/deletion (I/D) polymorphism raises the possibility of personalising ACE inhibitor therapy to optimise its efficiency and reduce side effects in genetically distinct subgroups. However, the extent of its influence among these subgroups is unknown. Therefore, we extended our computational model of blood pressure regulation to investigate the effect of the ACE I/D polymorphism on haemodynamic parameters in humans undergoing antihypertensive therapy. The model showed that the dependence of blood pressure on serum ACE activity is a function of saturation and therefore, the lack of association between ACE I/D and blood pressure levels may be due to high ACE activity in specific populations. Additionally, in an extended model simulating the effects of different classes of antihypertensive drugs, we explored the relationship between ACE I/D and the efficacy of inhibitors of the renin-angiotensin-aldosterone system. The model predicted that the response of cardiovascular and renal parameters to treatment directly depends on ACE activity. However, significant differences in parameter changes were observed only between groups with high and low ACE levels, while different ACE I/D genotypes within the same group had similar changes in absolute values. We conclude that a single genetic variant is responsible for only a small fraction of heredity in treatment success and its predictive value is limited.

Splenic artery aneurysm: a rare complication of autosomal dominant polycystic kidney disease.

Autosomal dominant polycystic kidney disease (ADPKD) is an important cause of renal dysfunction. It is the most common genetic disorder leading to end-stage kidney disease requiring dialysis. ADPKD is a multisystem disease and is linked to several extra renal abnormalities. Splenic artery aneurysms are rare in the general population. ADPKD is associated with cerebral artery aneurysms. However, splenic artery aneurysms are not a well-recognised complication of ADPKD. We report an unusual case of a splenic artery aneurysm found incidentally on abdominal CT imaging of a woman with known ADPKD.

Bilateral renal artery revascularisation in heart failure.

Renal artery stenosis can complicate the management of heart failure with reduced ejection fraction, as it is a conventional contraindication to the use of ACE inhibitors. We report a case in which bilateral renal artery revascularisation allowed the safe reintroduction of enalapril (and subsequently sacubitril valsartan) in a patient with severe left ventricular systolic dysfunction. There is a role for renal artery angioplasty in selected patients to allow optimal medical therapy for patients with heart failure due to impaired systolic function.

Hypokalaemia associated with excessive cola-flavoured drinks consumption.

We present a case involving a male patient in his 30s who was admitted to hospital due to recurrent episodes of hypokalaemia over the past 5 years. His medical history revealed hypertension, attention deficit hyperactivity disorder (ADHD), autism, and paranoia. He was taking citalopram, ramipril, amlodipine, and pramipexole. Tests indicated normal levels of aldosterone/renin ratio and plasma metanephrines. On reviewing his dietary history, it was noted that he consumed 3 to 3.5 L of cola-flavoured drinks on a daily basis. Normal potassium levels were achieved after a significant reduction in cola-flavoured drinks intake and potassium replacement. Subsequent outpatient clinic follow-up revealed that normal potassium levels were maintained even after the patient ceased taking potassium replacement tablets. Given the rarity of hypokalaemia associated with fizzy drinks, the underlying mechanism for this association remains unclear. In this case report, we attempt to provide a possible explanation for the involved mechanisms.

Significance of genetic analysis in adult patients with inherited chronic kidney disease.

Genetic focal segmental glomerulosclerosis (FSGS) is an important but underestimated cause of inherited proteinuric chronic kidney disease (CKD) in adults. We discuss a case of familial CKD due to inverted formin 2 (INF2) gene mutation, where three siblings had disparate phenotypic presentations ranging from CKD with subnephrotic proteinuria to nephrotic-range proteinuria with collapsing FSGS on kidney biopsy over a period of 8 years. The youngest sibling was the index case. The family agreed to undergo genetic testing only after two more siblings were diagnosed with kidney disease. This case highlights how clinical heterogeneity, absence of family history in the index case, initial lack of specific biopsy-proven diagnosis and reluctance to undergo genetic testing can delay the diagnosis of genetic kidney disease in adults.

Clinical and Microbiological Profiles of Urinary Tract Infections in Febrile Children Aged Six Months to Five Years Attending a Tertiary Care Hospital in India.

Background Urinary tract infection (UTI) is one of the most common causes of fever in the pediatric age group. The study was designed to study the clinical profile, etiologic microorganisms, and antibiogram patterns. Methods The present study is a hospital-based cross-sectional study done over a study period of one and a half years. Results Females comprised a higher proportion of the study subjects. Increased urinary frequency and urgency were the most prevalent presenting symptoms in the study population, seen in 39 (39%) and 20 (20%) of the children, respectively. Escherichia coli was found to be the most common causative organism in 45 (45%) children followed by Klebsiella in 22 (22%) children. The organisms showed maximum sensitivity to linezolid (88%) followed by levofloxacin(78%), and piperacillin-tazobactam(76%). Cotrimoxazole(16%) and cefixime(9%) showed the maximum resistance. The outcome was favorable for the majority of the patients treated at par with the antibiogram. Eleven (11%) of the children were found to have anatomical abnormalities in their genitourinary system, and it was found to be significantly associated with recurrence (P value=0.05). Conclusions UTI as one of the leading causes of fever and has to be dealt with a high index of suspicion while evaluating for cases of fever without a focus on children. The antibiogram of the underlying organisms needs to be followed while treating cases of UTI to ensure prompt recovery and avoid the emergence of antimicrobial resistance. This also highlights the need for periodic surveillance of the local prevalence of organisms and their antimicrobial susceptibilities to tailor proper management. Children with anatomical abnormalities in their renal system need to be followed up carefully for chances of recurrence.