John P. McNamara, MS, DC
- Associate Professor
- Biomedical Sciences Department
- Jefferson College of Health Sciences
- A Carilion Clinic Affiliate
- Roanoke, Virginia
On cut section allergy testing one year old discount rhinocort uk, the renal pelvis and calyces are dilated and cystic and contain a large stone in the pelvis of the kidney pressure atrophy of renal parenchyma allergy medicine knocks me out order rhinocort with a mastercard. The cystic change is seen to extend into renal p arenchyma allergy symptoms for gluten rhinocort 200 mcg low price, dilated pelvi-calyceal system extends deep into the renal compressing the cortex as a thin rim at the periphery allergy shots for dust mites order rhinocort 200mcg overnight delivery. Unlike polycystic cortex so that a thin rim of renal cortex is stretched over kidney, however, these cysts are communicating with the pelvi-calyceal the dilated calyces and the external surface assumes system. These may arise from renal tubules is the direct continuity of dilated cystic spaces. There is progressive atrophy of these tumours, the kidney may be the site of the secondary tubules and glomeruli alongwith interstitial fibrosis. Cortical Adenoma Cortical tubular adenomas are more common than other benign renal neoplasms. They are frequently multiple and associated with chronic pyelonephritis or benign nephrosclerosis. Microscopically, they are composed of tubular cords or papillary structures projecting into cystic space. The cells of the adenoma are usually uniform, cuboidal with no atypicality or mitosis. Transitional cell papilloma Transitional cell carcinoma Others (squamous cell carcinoma, Medullary interstitial cell tumour is a tiny nodule in the adenocarcinoma of renal pelvis, medulla composed of fibroblast-like cells in hyalinised undifferentiated carcinoma of stroma. These tumours used to be called renal fibromas but renal pelvis) electron microscopy has revealed that the tumour cells are not fibrocytes but are medullary interstitial cells. A third Juxtaglomerular cell malignant renal tumour is urothelial carcinoma occurring more tumour (Reninoma) commonly in the renal pelvis is described in the next section F. Adenocarcinoma of Kidney (Synonyms: Renal cell Oncocytoma carcinoma, Hypernephroma, Grawitz tumour) Oncocytoma is a benign epithelial tumour arising from Hypernephroma is an old misnomer under the mistaken collecting ducts. This cancer comprises 70 to 80% of all renal cancers and Microscopically, the tumour cells are plump with occurs most commonly in 50 to 70 years of age with male abundant, finely granular, acidophilic cytoplasm and preponderance (2:1). These cases have following associations: Mesoblastic nephroma is a congenital benign tumour. Granular cell type 8% Sporadic and familial Abundant acidophilic cytoplasm, marked atypia 4. Chromophobe type 5% Multiple chromosome losses, Mixture of pale clear cells with hypodiploidy perinuclear halo and granular cells 5. The clear cytoplasm of tumour cells is due to form of multiple losses of whole chromosomes i. Both hereditary and patterns: solid, trabecular and tubular, separated by acquired cystic diseases of the kidney have increased risk of delicate vasculature. Adult polycystic kidney disease and multicystic ged in papillary pattern over the fibrovascular stalks. The nephroma is associated with higher occurrence of papillary tumour cells are cuboidal with small round nuclei. These tumours have i) Exposure to asbestos, heavy metals and petrochemical more marked nuclear pleomorphism, hyperchromatism products. The tumour is papillary, granular cell, chromophobe, sarcomatoid and characterised by whorls of atypical spindle tumour cells. It is composed of a single layer of arises from the poles of the kidney as a solitary and cuboidal tumour cells arranged in tubular and papillary unilateral tumour, more often in the upper pole. Cut slow-growing tumour and the tumour may have been section of the tumour commonly shows large areas of present for years before it is detected. The upper pole of the kidney shows a large and tan mass while rest of the kidney has reniform contour. Sectioned surface shows irregular, circumscribed, yellowish mass with areas of haemorrhages and necrosis. The residual kidney is compressed on one side and shows obliterated calyces and renal pelvis. By the time the tumour is the prognosis in renal cell carcinoma depends upon the detected, it has spread to distant sites via haematogenous extent of tumour involvement at the time of diagnosis. The route to the lungs, brain and bone, and locally to the liver overall 5-year survival rate is about 70%. Clear cells predominate in the tumour while the stroma is composed of fine and delicate fibrous tissue. The sectioned surface shows replacement of almost whole kidney by the tumour leaving a thin strip of compressed renal tissue at lower end (arrow). Cut section of the tumour is gray white, fleshy and has small areas of haemorrhages and necrosis. It is generally solitary and unilateral but to 6 years of age with equal sex incidence. A defect in chromosome 11p13 results in abnormal growth identifiable myxomatous or cartilaginous elements of metanephric blastema without differentiation into normal (Fig. A higher incidence has been seen in monozygotic twins Microscopically, nephroblastoma shows mixture of and cases with family history. These include osteosarcoma, smooth and skeletal muscle, cartilage and bone, fat cells botyroid sarcoma, retinoblastoma, neuroblastoma etc. The most common presenting usually quite large, spheroidal, replacing most of the feature is a palpable abdominal mass in a child. A few abortive tubules and poorly formed glomerular structures are present in it. The tumour rapidly spreads via blood, shorter and runs from the bladder parallel with the anterior especially to lungs. The mucous membrane in female urethra the prognosis of the tumour with combination therapy is lined throughout by columnar epithelium except near the of nephrectomy, post-operative irradiation and chemo bladder where the epithelium is transitional. The other layers therapy, has improved considerably and the 5-year survival and mucous glands are similar to those in male urethra. This is a condition in which the entire primary sites, chiefly from cancers of the lungs, breast and ureter or only the upper part is duplicated. Normally they enter obliquely into the owing to congenital developmental deficiency of anterior bladder, so that ureter is compressed during micturition, thus wall of the bladder and is associated with splitting of the preventing vesico-ureteric reflux. There may be prolapse of the posterior Histologically, ureter has an outer fibrous investing layer wall of the bladder through the defect in the anterior bladder which overlies a thick muscular layer and is lined internally and abdominal wall. The condition in males is often by transitional epithelium or urothelium similar to the lining associated with epispadias in which the urethra opens on the of the renal pelvis above and bladder below. Normally, the persistence of the urachus in which urine passes from the capacity of bladder is about 400 to 500 ml without over bladder to the umbilicus. Micturition is partly a reflex and partly a patent which may be the umbilical end, bladder end, or voluntary act under the control of sympathetic and central portion. Histologically, the greater part of the bladder wall is made Adenocarcinoma may develop in urachal cyst. The superficial epithelial layer is made and has been described already along with its morphologic of larger cells in the form of a row and have abundant consequences (page 681). Inflammation of the tissues of lower eosinphilic cytoplasm; these cells are called umbrella cells. It is lined in the prostatic part by urothelium but elsewhere by stratified columnar epithelium except near its Infection of the ureter is almost always secondary to pyelitis orifice where the epithelium is stratified squamous. Ureteritis is usually mild but urethral mucosa rests on highly vascular submucosa and repeated and longstanding infection may give rise to chronic outer layer of striated muscle. Cystitis get repeated attacks of severe and excruciating pain on 699 distension of the bladder, frequency of micturition and great Inflammation of the urinary bladder is called cystitis. Cystoscopy often reveals a cystitis is rare since the normal bladder epithelium is quite localised ulcer.
It may be a primary cutaneous neoplasm or represent skin involvement by a nodal anaplastic large cell lymphoma allergy treatment xerosis order 100 mcg rhinocort otc. There are important differences in biologic behavior and prognosis between primary cutaneous and nodal anaplastic large cell lymphoma allergy shots in dogs generic 100mcg rhinocort free shipping. Typically allergy xyzal buy generic rhinocort 100 mcg online, only exposed skin is affected and it occurs minutes to hours after sun exposure allergy symptoms getting worse purchase rhinocort once a day. Burkitt lymphoma is a highly aggressive hematological malignancy with high proliferation rates. Histopathologic Features: Blastic plasmacytoid dendritic cell neoplasm is characterized by a diffuse, monomorphic infiltrate of medium-sized neoplastic cells with a blastoid morphology. Intratumoral hemorrhage is common and prominent in cases characterized by a bruise-like presentation clinically. Past medical history was significant for an inguinal sarcoma resected 5 years earlier. Sarcomatoid squamous cell carcinoma Synovial sarcoma is a malignant soft tissue tumor which, despite its name, does not arise from synovium. This tumor typically affects young adults and tends to arise in deep soft tissue sites near joints. Biphasic tumors are composed of epithelial and spindle cell components while monophasic cases typically consist of only the spindle cell population. The epithelial cells are characterized by cuboidal to columnar cells organized in cords, nests or glands. Some tumors are poorly differentiated and exhibit round cell morphology with prominent nucleoli. However, there is no involvement of the epidermis and abundant plasma cells are noted. Question 76 the best approach to establish a definitive diagnosis is to obtain: A. The clinical lymphadenopathy is suggestive of cutaneous involvement by a systemic lymphoma. It may help but will not provide definitive diagnosis Clinical Features: Patients are elderly adults and skin lesions may be the first manifestation of the disease. Papules, plaques and tumors are not distinctive and resemble other cutaneous lymphomas Histopathologic Features: Nodular infiltrates of small, medium or large pleomorphic lymphocytes intermingled with reactive cells (plasma cells, eosinophils, histiocytes) are seen. Cutaneous involvement by angioimmunoblastic T-cell lymphoma with remarkable heterogeneous Epstein-Barr virus expression. In between are the clinical forms classified as borderline tuberculoid, borderline, and borderline-lepromatous leprosy. It usually manifests as single or multiple ill defined hypopigmented or slightly erythematous macules, usually on the limbs. Tuberculoid leprosy is a relatively stable form seen in patients with strong immunologic host resistance and a markedly positive lepromin test result. Sensory impairment is an essential feature, and enlarging regional nerves often lead to palsy. Borderline leprosy represents the middle of the spectrum, but it is unstable, with patients quickly upgrading or downgrading to a more stable stage. Cutaneous lesions are larger, usually ill-defined, erythematous or copper-colored, annular patches or plaques. Borderline-lepromatous leprosy has more numerous and poorly defined lesions than borderline-tuberculoid leprosy. The cutaneous lesions are usually symmetric, poorly demarcated, erythematous and hypopigmented macules, patches, and nodules, frequently involving the earlobes and nasal mucosa. Multiple facial 194 nodules, which spare the eyebrows, give a classical leonine appearance. Multiple autoantibodies are frequently detected in lepromatous leprosy, and there is an increased incidence of vitiligo. The clinical features include widespread eruptions of painful, erythematous, and violaceous nodules, often involving the extremities, and associated with systemic symptoms. Indeterminate leprosy is characterized by a superficial and deep perivascular and periadnexal lymphohistiocytic infiltrate, which involves less than 5% of the dermis. In borderline-tuberculoid leprosy, the noncaseating granulomas are less evident, and nerve destruction is less prominent. Lymphocytes and histiocytes infiltrate the nerve, producing laminated perineurium. The foamy histiocytes of leprosy resemble those seen in xanthoma; they are called lepra or Virchow cells. Effacement of the epidermal rete ridges with a distinct Grenz zone is often present along with scattered lymphocytes and plasma cells. At the sites of preexisting lepromatous leprosy, erythema nodosum leprosum shows a mixed dermal infiltrate of lymphocytes and a variable number of neutrophils. The epidermis often shows hyperkeratosis and papillomatosis and is occasionally ulcerated. Most often skin lesions develop in the midst of other systemic symptoms and signs of active disease although rarely the skin may be the first sign of disease. The pathologic correlate to these ulcerations can include granulomatous vasculitis but more often the changes are inflammatory, with a mixture of acute and granulomatous inflammation without obvious vasculitis. Mucosal lesions were not appreciated, but a few papules were seen around the mouth. Sometimes the intensity of interface alteration results in subepidermal vesiculation. The biopsy shows a predominantly intraepidermal blister with reticular degeneration of keratinocytes and necrosis of the blister roof, features described in hand-foot-mouth disease. Parvovirus B19 causes erythema infectiosum and is the most commonly implicated virus in papular-pruritic gloves and socks syndrome. These cases are usually caused by Coxsackie virus serotype A16 and enterovirus type 71. These cases are more commonly seen 201 in adult patients, may be widespread rather than limited in lesion distribution, are sometimes severe enough to require short hospitalizations, and may result in shedding of the nail (onychomadesis) during recovery. Muir-Torre syndrome presents as multiple sebaceous neoplasms, multiple adenomatous polyps and improved survival despite the diagnosis of one or more visceral adenocarcinomas. Superficial angiomyxoma shows spindled and stellate cells upon an often delicately and well-vascularized more basophilic matrix. Solitary fibrous tumor is a circumscribed lesion with alternating cellular and hypocellular regions. Despite a variable appearance which raises a broad differential diagnosis, this tumor is considered benign with only a rare risk of recurrence. Pleomorphic lipoma lacking mature fat component in extensive myxoid stroma: a great diagnostic challenge. Three other classical patterns include the spongiotic, pemphigus-like, and Hailey-Hailey disease-like changes. This particular case showed focal acantholytic dyskeratosis with numerous cornoid lamellae which, in combination with clinical findings, supported a diagnosis of Grover disease, porokeratotic variant. Grover Disease: A Reappraisal of Histopathological Diagnostic Criteria in 120 Cases.
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It If such an exudate is absorbed allergy forecast ventura purchase rhinocort discount, the detached retina may produces an absolute feld defect starting in the upper nasal well become spontaneously replaced allergy testing northampton ma buy discount rhinocort 200mcg online. For this reason such detachments habitu Retinoschisis can be confused with retinal detachment ally cause an extensive separation of the retina allergy medicine upset stomach 200mcg rhinocort with amex, particu and is differentiated from it by the presence of an absolute larly in the lower part of the eye where the fuid tends to feld defect as well as by the immobility and transparency gravitate allergy medicine knocks me out generic 100mcg rhinocort with mastercard. A rhegmatog enous retinal detachment occurs when a tear in the retina Juvenile Retinoschisis leads to fuid accumulation with a separation of the neuro Juvenile retinoschisis is a hereditary disorder in which there sensory retina from the underlying retinal pigment epithe is a splitting of the retina in the nerve fbre layer with the lium. In the frst retinal folds radiating from the foveal centre in a petalloid place, the presence of a break designates a detachment pattern. Those involving more occurs when subretinal fuid accumulates in the potential than a quadrant of the circumference are called space between the neurosensory retina and the underlying giant retinal tears. Depending on the mechanism ora serrata causes a large tear known as retinal dialysis. A of subretinal fuid accumulation, retinal detachments tradi dialysis may be large, in which case the choroid is seen tionally have been classifed into rhegmatogenous, trac through it and the edge of the detached retina is sharply tional and exudative. Fluid vitreous has seeped through the tear into the subretinal space, elevating the retina into a bullous detachment. A round retinal tear is surrounded by a small retinal detachment in the inferior retina. By preliminary examina Predisposing Factors tion with the mirror alone, a difference in the nature of these include myopia, previous intraocular surgery such as the refex as the eye is turned in various directions will at aphakia or pseudophakia, a family history of retinal detach once arrest attention, while examination with the indirect ment, trauma and infammation. Eventually, and sometimes rap idly, the detached portion of retina assumes a different tint Clinical Features from the normal fundus. During slight movements of the eye the folds show retain its functions, which may be only partially impaired oscillations and the retinal vessels are seen coursing over for a considerable period. After a few lour secondary to intraretinal oedema and the normal cho weeks, a retinal detachment may present with more fxed roidal pattern of vessels is no longer seen. It has a convex folds, retinal thinning, intraretinal cysts, subretinal fbrosis confguration, and moves freely with eye movements unless and demarcation lines. Even though At the edges of the detachment a considerable degree of they represent areas of increased retinal adhesion to the pigmentary disturbance may appear, as well as white spots retinal pigment epithelium, it is not uncommon for subreti of exudation, haemorrhages and greyish-white lines due to nal fuid to spread beyond the lines. More than half of all retinal breaks are located in the upper temporal quadrant, although any quadrant may be affected. These include eral retinal degenerations that could lead on to a retinal symptoms suggestive of vitreoretinal traction, a history of break. Since many holes are in the extreme periphery, full mydriasis is necessary, and for this purpose the indirect method of oph thalmoscopy, using strong illumination, is more useful and effective than the direct. Sometimes such a lesion is rendered visible only by pressing gently on the sclera near the ora ser rata with a scleral indentor. The retinal periphery should also A B be examined using a Goldmann three-mirror fundus lens, which provides a magnifed view of the ora and its environs through the slit-lamp microscope. A careful drawing showing the position of retinal holes, pathological lesions, retinal ves sels and other landmarks, is made of the fundus. Changes in posture may reveal a retinal tear that has hitherto been hidden by a retinal fold. The fluid has tracked down further nasal implying the break is slightly to the nasal side. Subsequently, the retina and choroid are approximated a retinal detachment are as follows: to allow development of chorioretinal adhesions by using methods of external or internal tamponade. The of breaks that are clustered within 1 clock hour in the supe surgical options include pneumatic retinopexy, scleral buck rior two-thirds of the fundus. The surgical goals are to identify and to close all postoperatively the patient is positioned so that the bubble retinal breaks with minimum iatrogenic damage. This is tamponades the retinal break against the pigment epithe achieved by good indirect ophthalmoscopy followed by the lium. A chorioretinal adhesion is achieved by Chapter | 20 Diseases of the Retina 335 applications of laser or cryotherapy to the edges of the reti procedures such as paracentesis or vitrectomy to allow ade nal break. In non-drainage surgery, subretinal fuid Scleral buckling or external plombage (Fig. A vitrectomy with removal of the vitre the buckle and the circulation of the central retinal artery is ous from the margins of the breaks and the vitreous base is not compromised. The subretinal fuid is drained to relieve the pull on the underlying retinal periphery. Complications that may result from drainage of in the eye to tamponade the retina internally. Sulphur hexafuoride is an inert gas of high molecu tive, but needs close monitoring of the intraocular pressure lar weight, low water solubility and low diffusion coeff during surgery and in the immediate postoperative period. Patients with an intraocular gas bubble should not fy in non-pressurized aircraft. Silicone oil offers certain advantages over gas in the treatment of selected complicated retinal detachments. Visual rehabilitation is faster with silicone oil than with gas tamponade, and laser therapy of retinal defects can also be done more easily than with a gas bubble in the vitreous. The detachment edges of which are radially striated, looking as if frayed becomes total, the photoreceptors start to degenerate within (Fig. Usually the patches are contiguous with the a couple of weeks, impairing visual recovery and compli disc; occasionally they are isolated, but rarely far from cated cataract and iridocyclitis follow. If glau ple, when the vitreous, retina and choroid are grossly coma or optic atrophy causes the fbres to degenerate, the degenerated especially in the presence of multiple vitreous medullary sheaths disappear and no trace of the abnor bands, when there is high myopia and if the detachment has mality remains. It is important to be able to diagnose ment surgery is the proliferation and contraction of mem such fbres, since they may be mistaken for exudates, as branes on both surfaces of the detached retina and on the in hypertensive retinopathy. In cases that can be treated without the use of silicone oil there is a 50% chance See Chapter 18, the Lens. The following varieties of phakomatosis Retinochoroidal Dystrophies have prominent retinal features. The cerebellum, medulla, Inverse retinitis pigmentosa spinal cord, kidneys and adrenals are also affected with Progressive cone dystrophy angiomatoses and cysts. Sometimes they Fundus favimaculatus are large like balloons; at other times small and miliary (see Grouped pigmentation of the Chapter 32, Ocular Manifestations of Systemic Disorders). Whit the retina, corresponding to those related to the nerves ish fecks surround the ovoid zone of atrophy, when differ of the lids and orbit (see Chapter 32, Ocular Manifestations ential diagnosis from fundus favimaculatus, which is often of Systemic Disorders). There duce bilateral and usually symmetrical lesions in the ab is no leakage of dye. The fundus picture in pearance of the visual elements and the pigment epithelium individuals of the same family is often similar and examina in the centre of the retina. Dominant Foveal Dystrophy this is a progressive tapetoretinal dystrophy of the central retina. Histological studies show a pro gressive degeneration of the neuroepithelium and pigment epithelium. It is due to a Butterfy-Shaped Pigment Dystrophy primary dystrophy of the retinal cones. Vitelliform Dystrophy of the Fovea Fundus Flavimaculatus Vitelliform dystrophy of the fovea is known as Best dis ease. White or yellowish-white deep retinal fecks good and the neuroepithelium is unaffected.
It may require treatment with anti-inflammatory agents allergy forecast edmonton alberta generic rhinocort 100 mcg, steroids and regular infusions of immunoglobulin (see section 6 allergy united buy discount rhinocort online. Onset of symptoms may occur between three weeks and two months but tend to respond within two weeks once the drug is stopped allergy forecast charlotte cheap rhinocort online amex. If there is doubt over diagnosis or management allergy testing companies discount rhinocort 100 mcg free shipping, refer to Dr Clarissa Pilkington (tel 0207 829 7887) at Great Ormond Street Hospital for Children. It is accompanied by chronic salt depletion and sometimes failure to thrive without severe dehydration. It can also present acutely often as part of heat stroke so is commoner in hot weather when there has been inadequate salt and fluid replacement with dehydration. Principal findings are hypokalaemic hypochloraemic metabolic alkalosis, sometimes with hyponatraemia. This may be preceded by anorexia, nausea, vomiting, fever and weight loss, in the acute setting this can be mistaken for infective gastroenteritis. Judging degree of dehydration in an acute presentation can be hard, the classic clinical signs of dehydration (sunken eyes, loss of skin turgor) are not always apparent and a comparison of acute presentation weight with last clinic weight is helpful. Check venous sample in blood gas machine for bicarbonate, or venous blood for Cl, Na and K. In the more chronic, indolent presentation treatment is with sodium +/ potassium chloride supplements, which may be required for many months or long term. After salt replacement, the metabolic abnormality resolves and weight gain follows rapidly. Unexplained failure to thrive should always have urinary electrolytes checked, a spot urine + Na <20 mmol/l indicates low total body sodium that needs correcting. A serum potassium at the lower end of the normal range may still be associated with body depletion. It is quite usual for a newborn screened infant under 3 months to have low urine Na levels and normal range is less well defined, so it should not be used to guide sodium supplementation in this age group (see salt supplement recommendations in section 7. The age of telling them may vary and occasionally is problematic if parents are reluctant for the issue to be discussed. We would encourage parents to tell their sons as early as possible, and we would wish to ensure they are informed by 8-12 years. It is important to stress to them that infertility is not the same as impotence and that sexual performance is unaffected (although the volume of ejaculate is 152 Clinical guidelines for the care of children with cystic fibrosis 2017 Care must be taken with oral contraception due to effect of short term courses of antibiotics, but long term ones. This has been highlighted by the survey carried out at the Brompton, Great Ormond Street and Royal London hospitals, where we found 1 in 3 girls aged 11-17 years answering the survey had a problem at times. For many (if not all) girls this is rather embarrassing and many do not want to talk to their parents about it, and especially not to male doctors! It is more likely they will discuss this with female members of the team (nurse specialists, physiotherapists). Please note that, although it is less common, stress urinary incontinence may also occur in males and for some patients (boys and girls) faecal incontinence may be an issue. Transplant assessment Almost all assessments are now carried out at Great Ormond Street Hospital for Children and referrals should be made to Drs Helen Spencer or Paul Aurora. An exception would occur in the case of an adolescent approaching transition to the adult service, in which case, the assessment should be done here, liaising with the adult team. Contact Dr Su Madge, Nurse Consultant, extension 4053 at Royal Brompton Hospital, for the booklet listing investigations. Once complete, return these to Dr Martin Carby or Dr Anna Reed, Consultants in Respiratory & Transplant Medicine, at Harefield Hospital. Traditionally, children fulfilling these criteria would be likely to have a median life expectancy of 2 years, but this may not be the case anymore. Contra-indications the following contra-indications differ between centres, and may be subject to change over time with the availability of. The decision will be influenced by the presence of multiple problems within an individual child. Major fi Other organ failure (excluding hepatic when a lung/liver transplant could be considered). It is therefore important when discussing the issues with the family and child, that as well as the potential benefits, the following negative points should be addressed (these will be addressed at the assessment meetings, but should be raised early with families): 1. Due to a shortage of donors about 30% of patients will die before organs become available. The time spent waiting for organs will be extremely stressful (uncertainty, false alarms etc). After the operation, invasive procedures including bronchoscopy and biopsies are likely to be required. In addition, unless complete eradication of reservoirs of infection has been successful (which almost never occurs due to chronic infection of sinuses), there is potential for bacterial infection of the transplanted lungs, which may make ongoing antibiotic therapy and physiotherapy necessary. Transplantation has little impact on the non-pulmonary manifestations of the disease (ie, enzyme replacement and other therapies need to be continued), although there may be nutritional benefits in the medium term. As a result potential transplant candidates can be identified more easily, be formally assessed more quickly and duplication of investigations will be avoided. When specific results are not available but have been requested please mark as awaited. Serial lung function tests are very helpful and should be included when available. Any questions about this proforma or its use can be addressed by contacting the transplant co-ordinators at the hospital to which you intend to send the referral. Current Exercise Capacity Exercise tolerance (distance) Formal 6 minute walk test performedfi The on-call paediatric respiratory SpR at Royal Brompton Hospital will advise over the exact choice, which is usually ceftazidime and tobramycin. It is also important that chest physiotherapy is strictly adhered to during the admission. Parents should also receive the vaccine (but we do not routinely give to siblings). If however we are informed late in the course of the illness or the child really has mild chicken pox only with a few spots then aciclovir is not warranted. They need to be advised to fill in the medical information in great detail so that there is no risk of the company not reimbursing a potential claim. All their medications (including for an extra week) plus suitable stand-by course of oral antibiotics. Sunblock is needed if taking ciprofloxacin, doxycycline or voriconazole (and for 4 weeks after course has finished). In Europe (except for Cyprus, Gibraltar), the voltage for the nebuliser is not a problem (220v) and a standard travel plug adapter is all that is needed. Discuss this with our Physiotherapy Department (extension 8088) well in advance of the holiday. This consists of breathing 15% O2 at sea level which is the equivalent O2 concentration in the plane at altitude. It should be performed in patients with: fi a history of oxygen requirement during chest exacerbations. This is especially important during long haul flights when the children are likely to sleep. Patients whose SpO2 is normally < 92% will definitely need oxygen, and those usually on home oxygen will need an increased flow rate. Oxygen is usually available at a flow rate 2 or 4 l/min and is not humidified, arrangements can be made through the travel agents, but adequate time is needed to do so. The management of a dying child needs to be flexible so as to cater for individual family needs and reviewed at least twice daily to accommodate changes in needs. We would encourage an honest and open approach at all times, although we would also consider the wishes of the child and his or her family about sharing information.