Outcome of renal transplant in hereditary gelsolin amyloidosis.

Amyloid nephropathy is an unusual manifestation of hereditary gelsolin amyloidosis and may present with proteinuria and progressive renal failure. We report the first case of renal transplantation in a patient with hereditary gelsolin amyloidosis complicated by end-stage renal disease. The patient was a 44-year-old man from the Northwest of Iran who had undergone hemodialysis for 1 year. He finally received a living, unrelated renal transplant. During a 6-year posttransplant period, the patient maintained stable allograft function without proteinuria. No significant infectious or cardiac complications were noted. Although a definite conclusion cannot be reached with a single case, this report may indicate that renal transplantation can be successfully attempted in patients with hereditary gelsolin amyloidosis and amyloid nephropathy. Renal transplantation has been performed in various hereditary, primary, and secondary amyloidoses. A brief review of this topic is presented.

Transplant renal artery stenosis associated with acute cytomegalovirus infection: resolution following ganciclovir administration.

Cytomegalovirus has been implicated in the pathogenesis of transplant renal artery stenosis. However, the clinical course of this infection-associated transplant renal artery stenosis and its response to antiviral treatment is still unclear. We report a patient with transplant renal artery stenosis associated with an acute cytomegalovirus infection, which resolved following ganciclovir administration without the need for surgical or angiographic intervention. Serological testing revealed positive titers of anti- cytomegalovirus IgM and IgG antibodies. Renal allograft Doppler study findings were compatible with critical renal artery stenosis. Renal allograft angiography disclosed a critical circular stenosis. Following the intravenous ganciclovir administration, dramatically decreased Doppler ultrasound parameters along with the absence of parvus/tardus waveform pattern revealed the resolution of the stenosis. Moreover, the serological testing was negative for anti-cytomegalovirus IgM antibody, but anti-cytomegalovirus IgG antibody was positive. This report not only implies the causative possible relationship between acute cytomegalovirus infection and transplant renal artery stenosis, but it also highlights the importance of this complication when managing a renal transplant recipient with signs of allograft arterial stenosis.

The evolution of the study of anatomy in Japan.

The following review focuses on how the study of anatomy in Japan has evolved throughout the centuries; specifically, we investigate anatomical knowledge during the primitive, ancient, feudal, and early modern periods of Japanese history. Early vague and mythical anatomical concepts derived from China prevailed for many centuries in Japan. Kajiwara wrote one of the earliest anatomical works in 1302. As a science, anatomy was the first basic science to be established in Japan, beginning simplistically during the 1600s and flourishing more recently with the onset of Meiji Restoration. As a result, Japan has produced several of the most influential anatomists of the 20th century, including Buntaro Adachi, who added detail to our knowledge of the vascular system and its variations; and Sunao Tawara, who discovered the atrioventricular node. Herein, we discuss the ways in which Japan has added to and promoted the anatomical sciences.

Lateral lakes of Trolard: anatomy, quantitation, and surgical landmarks. Laboratory investigation.

OBJECT: There is scant and conflicting information in the literature regarding the lateral lacunae, or lateral lakes of Trolard. As these venous structures can be encountered surgically, this study aimed at further elucidating their anatomy, identifying surgical landmarks, and associated quantitation. METHODS: Thirty-five adult cadavers were dissected of lateral lacunae. Following quantitation of the lacunae, these structures were measured, as were the distances from them to the coronal and sagittal sutures. RESULTS: A mean of 1.9 lacunae were identified on the right sides and 1.4 lacunae on the left sides. Although there tended to be slightly more lacunae on the right sides, this difference did not reach statistical significance (p > 0.05). The average lengths of the lacunae were 3.2 and 2.0 cm for the right and left sides, respectively. The mean widths of these venous lakes were 1.5 cm for the right sides and 0.8 cm for the left sides. Lacunae were variably positioned but tended to cluster near the vertex of the skull. None were identified posterior to the lambdoid sutures, and only 5 were found to lie anterior to the coronal suture, with 4 of these located on right sides (p < 0.05). When lacunae were identified anterior to the coronal suture, they were generally 5-6 cm from this structure. The majority of lacunae could be identified between the coronal and lambdoid sutures and within 3 cm of the midline. CONCLUSIONS: Although the situation varies, lateral lacunae are concentrated posterior to the coronal suture and anterior to the lambdoid sutures. They are most often found within 3 cm of the sagittal suture. These previously unreported data could be useful to the neurosurgeon in planning surgical procedures that traverse the calvaria.

Suprascapular nerve as a donor for extracranial facial nerve reanimation procedures: a cadaveric feasibility study.

OBJECTIVES: Facial nerve injury with resultant facial muscle paralysis is disfiguring and disabling. To the authors' knowledge, neurotization of the facial nerve using a branch of the brachial plexus has not been previously performed. METHODS: In an attempt to identify an additional nerve donor candidate for facial nerve neurotization, 5 fresh adult human cadavers (10 sides) underwent dissection of the suprascapular nerve distal to the suprascapular notch where it was transected. The facial nerve was localized from the stylomastoid foramen onto the face, and the cut end of the suprascapular nerve was tunneled to this location. Measurements were made of the length and diameter of the supra-scapular nerve. In 2 of these specimens prior to transection of the nerve, a nerve-splitting technique was used. RESULTS: All specimens were found to have a suprascapular nerve with enough length to be tunneled, tension free, superiorly to the extracranial facial nerve. Connections remained tensionless with left and right head rotation of up to 45 degrees . The mean length of this part of the suprascapular nerve was 12.5 cm (range 11.5-14 cm). The mean diameter of this nerve was 3 mm. A nerve-splitting technique was also easily performed. No gross evidence of injury to surrounding neurovascular structures was identified. CONCLUSIONS: To the authors' knowledge, the suprascapular nerve has not been previously explored as a donor nerve for facial nerve reanimation procedures. Based on the results of this cadaveric study, the authors believe that use of the suprascapular nerve may be considered for surgical maneuvers.

Hypophosphatemic encephalopathy in a CAPD patient.

Severe refeeding hypophosphatemia has been reported in infants and adults on continuous ambulatory peritoneal dialysis. We report an episode of transient encephalopathy and cardiac suppression in a diabetic, malnourished end-stage renal disease patient undergoing peritoneal dialysis with a glucose-based solution, which was associated with severe hypophosphatemia. Further investigation revealed an adynamic bone disease. The potential factors precipitating this episode of hypophosphatemia are discussed.

Contributions of the fourth spinal nerve to the brachial plexus without prefixation.

OBJECT: The intradural contributions of the C-4 nerve rootlets have not been previously evaluated for their connections to the brachial plexus. The authors undertook a cadaveric study to evaluate the C-4 contributions to the upper trunk of the brachial plexus. METHODS: The posterior cervical triangles from 60 adult cadavers were dissected. All specimens that were found to have extradural C-4 contributions to the upper trunk of the brachial plexus were excluded from further study. In specimens found to have no extradural C-4 contributions to the brachial plexus a C1-T1 laminectomy was performed. Observations were made of any neural communications between adjacent spinal rootlets, specifically between C-4 and C-5. RESULTS: Nine (15%) of the 60 sides were found to have extradural C-4 contributions to the upper trunk of the brachial plexus. These sides were excluded from further study. No specimen was found to have a postfixed brachial plexus. Of the remaining 51 sides, 11 (21.6%) were found to have intradural neural connections between C-4 and C-5 dorsal rootlets and 1 (1.96%) had a connection between the ventral roots of C-4 and C-5. Communications between these 2 adjacent dorsal cervical cord levels were of 3 types. Type I was a vertical communication between the more horizontally traveling dorsal roots. Type II was a forked communication between adjacent C-4 and C-5 dorsal rootlets. The Type III designation was applied to connections between ventral rootlets. Although communications were slightly more frequent on left sides, this did not reach statistical significance. CONCLUSIONS: In approximately 20% of normally composed brachial plexuses (those with extradural contributions from only C5-T1) we found intradural C4-5 neural connections. Such variations may lead to misinterpretation of spinal levels in pathological conditions of the spinal axis and should be considered in surgical procedures of this region, such as rhizotomy.

Thrombotic microangiopathy as a complication of medicinal leech therapy.

Medicinal leeches (hirudo medicinalis) are increasingly used as a useful therapeutic option in various medical and surgical settings. The potential complications associated with this therapy include infections with Aeromonas species, bleeding, anemia, and allergic reactions. We describe a patient who developed thrombotic microangiopathy and acute renal failure following leech therapy. It is proposed that the series of nonhuman proteins within leech saliva may be capable of injuring the endothelium.

The relationship between the internal jugular vein and common carotid artery in the carotid sheath: the effects of age, gender and side.

The internal jugular vein is often used for central venous catheter placement. The variations in the location of this vein along the major neck vessels (in the carotid sheath) may account for unsuccessful cannulations or iatrogenic arterial injuries. The aim of this study was to delineate the relation of the internal jugular vein and common carotid artery in the lower neck, and to assess the effects of age, gender and side on these anatomical structures. Two-dimensional ultrasonographic examinations of the right and left supraclavicular triangle were performed in 219 adult individuals who had no history of neck surgery or known pathology. The location of the internal jugular vein in relation to the common carotid artery was recorded. An anterolateral location of the internal jugular vein was the most common configuration observed on both sides (84% right side and 91.8% left side) followed by the lateral (14.2% right and 6.4% left) and anterior (1.4% right and 1.8% left) locations. A single case of a medial internal jugular vein was observed on the right side (0.23% of both sides). Subjects with a laterally located internal jugular vein were older than those with an anterolateral configuration (P<0.01). No gender differences were found with regard to these two configurations (P=0.867). The laterally located internal jugular vein was more frequent on right sides (P=0.007). Such information may be potentially useful for clinicians who are managing critically ill patients or patients undergoing hemodialysis.

Thrombotic microangiopathy in the early post-renal transplant period.

The aim of this study was to identify cases of post-renal transplant thrombotic microangiopathy in a single transplant center over a period of five years. In a retrospective study, we reviewed the renal biopsy specimens of 57 renal transplant recipients with allograft dysfunction. The presence of fibrin thrombi within the glomerular capillaries or arterioles was used to define thrombotic microangiopathy. Systemic thrombotic microangiopathy was justified with the presence of thrombocytopenia and evidence of microangiopathic hemolysis. Patients with the biopsy findings compatible with thrombotic microangiopathy but without any systemic findings were categorized as having localized thrombotic microangiopathy. Four out of 57 patients had systemic thrombotic microangiopathy, while two had localized disease. The characteristics of each patient are discussed. Post-transplant thrombotic microangiopathy constitutes 10.5% of cases of early renal allograft dysfunction. A high index of suspicion is needed for diagnosing this entity as a potential cause of post-kidney transplant allograft dysfunction. Further studies with a greater number of patients may be required to highlight the risk factors for post-renal transplant thrombotic microangiopathy.

Parvovirus B19 microepidemic in renal transplant recipients with thrombotic microangiopathy and allograft vasculitis.

Parvovirus B-19 (B-19) can lead to various clinical scenarios in renal transplant recipients. Here, we report a B-19 microepidemic that occurred between January and March 2007, involving renal transplant recipients from a single center in Tabriz, Iran. We observed 6 patients in whom there was a temporal association between active B-19 infection and thrombotic microangiopathy and intrarenal small and medium-sized vessel vasculitis. Patients typically presented with deteriorating renal allograft function and anemia, and laboratory findings revealed thrombotic microangiopathy. Ultimately, extensive endothelial injury and renal allograft vasculitis that mimicked a vascular rejection ensued. In conclusion, B-19-related thrombotic microangiopathy may precede allograft vasculitis in renal transplant recipients. A high index of suspicion is required for early diagnosis and treatment of B-19 infection. To the best of our knowledge, this series represents the first report of B-19-related renal allograft vasculitis in the English literature.

Calcium and phosphorus metabolism in stable renal transplant recipients.

OBJECTIVES: This study sought to elucidate the status of calcium, phosphorus, and parathyroid hormone in patients following kidney transplant. MATERIALS AND METHODS: In this cross-sectional study, 20 renal transplant recipients were evaluated. For each patient, age, sex, time since transplant, and body weight were recorded. Inclusion criteria were age > 14 years and good allograft function defined as a serum creatinine level < 132.6 micromol/L for at least 6 months after transplant. Exclusion criteria were immunosuppressive therapy other than the standard triple regimen (cyclosporine, prednisolone, and mycophenolate mofetil or azathioprine) and use of any drug known to alter calcium hemostasis. Levels of 24-hour urine calcium, phosphorus, creatinine, and uric acid, as well as concentrations of hemoglobin, serum creatinine, calcium, and phosphorus were measured. To obtain a mean value of serum intact parathyroid hormone in transplant recipients at our center, serum intact parathyroid hormone levels were additionally quantitated in another group of 30 renal transplant recipients. RESULTS: The mean hemoglobin level was 135.6 +/- 17.7 g/L, the mean serum creatinine level was 105.0 +/- 15.3 micromol/L, and the mean serum calcium and phosphorus levels were 2.25 +/- 0.17 mmol/L (normal range, 2.02-2.60 mmol/L) and 1.28 +/- 0.24 mmol/L (normal range, 0.81-1.61 mmol/L), respectively. The mean serum intact parathyroid hormone level was 33.17 +/- 14.67 ng/L (normal range, 10-60 ng/L). Mean 24-hour urine calcium and phosphorus values were 2.32 +/- 1.68 mmol/day (normal, 2.49-6.24 mmol/day) and 19.77 +/- 8.31 mmol/day (normal, 12.91-41.98 mmol/day), respectively. A positive correlation was found between serum calcium and alkaline phosphatase levels (r = +0.71, P = .006). Hemoglobin level was negatively correlated with serum phosphorus level (r = -0.65, P = .003) and sex (r = -0.57, P = .003) and positively correlated with urine creatinine levels (r = +0.69, P = .001). CONCLUSIONS: Renal transplant recipients with stable allograft function may have normal serum calcium, phosphorus, and intact parathyroid hormone levels. However, presence of hypocalciuria and elevated serum alkaline phosphatase levels might imply impaired calcium metabolism in these patients.

Concomitant pulmonary tuberculosis and tuberculous appendicitis in a recipient of a renal transplant: a case report.

INTRODUCTION: Tuberculosis is still a serious infection among recipients of renal transplants. Although the ileocecal region is the most affected part in intestinal tuberculosis, acute tuberculous appendicitis is quite a rare entity. We report a case of concomitant pulmonary tuberculosis and tuberculous appendicitis in a recipient of a renal transplant. CASE PRESENTATION: A 27-year-old Iranian woman, who had been the recipient of a renal transplant five years earlier, presented with a two-week history of coughing, fever and weight loss. The cause of her end-stage renal disease was chronic pyelonephritis. There were fine crackles noted during a chest examination, and a plain chest radiography showed fine miliary nodules throughout her entire lung fields. Sputum and bronchial aspirate examination was positive for acid-fast bacilli, suggestive of Mycobacterium tuberculosis infection. A chest computed tomography scan revealed widespread miliary nodules, compatible with miliary tuberculosis. She developed severe abdominal pain and abdominal surgery disclosed a perforated appendicitis. Histopathological examination of the resected appendix revealed widespread caseating epithelioid granulomas, suggestive of tuberculosis. CONCLUSION: Our case report highlights a rare presentation of tuberculosis in a patient who has undergone renal transplant. Such unusual presentation of tuberculosis, particularly among patients receiving potent immunosuppressive protocols, should be considered by clinicians.

A testimony to the history of heart and lung transplantation: English translation of Demikhov's paper, "Transplantation of the Heart, Lungs and other Organs".

Vladimir Petrovich Demikhov, a Soviet physiologist and surgeon, made meticulous attempts at reviving heart and lung transplantation in the last century. Herein, we present an English translation of Demikhov's 1969 paper of Transplantation of the Heart, Lungs and other Organs (published in Eksperimental'naia Khirurgiia i Anesteziologiia), which represented a synopsis of his experiences with experimental transplantation. A brief account of Demikhov's life and contributions to transplant medicine is also given.

Facial palsy and its management in the Kitab al-Hawi of Rhazes.

More than 1000 years ago, Rhazes practiced rudimentary neurology. This monumental physician wrote more than 200 books in his lifetime and died a blind pauper in the 10th century AD. His Kitab al-Hawi (Liber Continens) was one of the most famous and detailed medical texts of the ancient world. Here, we discuss the life of Rhazes and provide perhaps the first English translation of his writings dealing with facial palsy (Lagveh) and its treatment. It is the contributions of early clinicians/scholars such as Rhazes on which we base our current medical knowledge.

Wrong theories on the origin of blood vessels: Polybus and De Natura Hominis.

Polybus of Cos (approximately 400 B.C.) was the son-in-law and the successor of Hippocrates. He is credited with founding the school of Dogmatism, and writing "The Nature of Man" which was important in advancing the theory of the four body humors (humoralism). Some earlier scholars negated Polybus' role as an independent medical figure. However, Corpus Aristotelicum quoted him as having a unique theory regarding the body vasculature which stated that this system was composed of four pairs of blood vessels originating from the head and that these supplied the whole body. In an interpretation of this theory, we opined that numerological mysticism might have been the common motive for both Hippocrates' humoralism and Polybus' theory of the vasculature. A discussion on this issue is presented.

The Aristotelian account of "heart and veins".

The exploration of the cardiovascular (CV) system has a history of at least five millennia. The model of the heart and veins represented by Aristotle (384-322 B.C.) is one of the earliest and accurate descriptions of the CV system. With his own specific metaphysical approach, Aristotle discussed why there might be a vascular tree composed of two vessels and also why these vessels must extend throughout the entire body. Herein, the authors present a history of the original account of the CV system based on the studies and teachings of Aristotle who made detailed observations and experimented upon animals and human corpses to explore the anatomy of the heart and vessels and thus provided the basis for modern CV medicine. The Aristotelian CV model consisted of two related but slightly dissimilar passages based on experimentation and tradition, which could be perceived as the morphology and metaphysical accounts of physiology, respectively. Restricted by his own methodology of dissecting dead animals, Aristotle was the first to describe the anatomy of the heart and blood vessels. A thorough reading of his Historia Animalium showed that he was able to morphologically delineate the right atrium in addition to three distinct heart cavities corresponding to the left atrium and right and left ventricles. The authors conclude that when interpreting Aristotelian doctrine, the methodology and terminology should be taken into account in order to prevent potential misconceptions. It is the early work of such scientists as Aristotle on which we base our current understanding of the CV system.

Marie-François Xavier Bichat (1771-1802) and his contributions to the foundations of pathological anatomy and modern medicine.

Marie-François Xavier Bichat (1771-1802) was a prominent French anatomist during a time of revolution and one of the founders of French scientific medicine. He conducted several experimental studies, which laid the foundation for modern physiology. Based on autopsy findings, Bichat introduced 21 tissues as the basic elements of organs. His name is carried on by several eponyms in anatomy and histology; Bichat's fossa (pterygopalatine fossa), Bichat's protuberance (buccal fat pad), Bichat's foramen (cistern of the vena magna of Galen), Bichat's ligament (lower fasciculus of the posterior sacroiliac ligament), Bichat's fissure (transverse fissure of the brain) and Bichat's tunic (tunica intima vasorum). This paper deals with the life and works of this early pioneer in anatomy and physiology.

Richard Lower (1631-1691) and his early contributions to cardiology.

Although little is written of Richard Lower he was perhaps the most influential English physiologist of the seventeenth century after Harvey. He is most remembered for his works on transfusion and the function of the cardiopulmonary system (Tractatus de Corde). Herein, we review the life of this important historical medical figure and present his little known posthumously published appendix regarding the function and morphology of the heart as published in John Browne's Myographia Nova.