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Ghon's complex is a lesion seen in the lung that is caused by tuberculosis. The lesions consist of a calcified focus of infection and an associated lymph node. These lesions are particularly common in children and can retain viable bacteria, so are sources of long-term infection and may be involved in reactivation of the disease in later life.
In countries where cow milk infected with "Mycobacterium bovis" has been eliminated (due to culling of infected cows and pasteurization), primary tuberculosis is usually caused by "Mycobacterium tuberculosis" and almost always begins in the lungs. Typically, the inhaled bacilli implant in the distal airspaces of the lower part of the upper lobe or the upper part of the lower lobe, usually close to the pleura. As sensitization develops, a 1- to 1.5-cm area of gray-white inflammation with consolidation emerges, known as the Ghon focus. In most cases, the center of this focus undergoes caseous necrosis. Tubercle bacilli, either free or within phagocytes, drain to the regional nodes, which also often caseate. This combination of parenchymal lung lesion and nodal involvement is referred to as the Ghon complex. During the first few weeks there is also lymphatic and hematogenous dissemination to other parts of the body.
In approximately 95% of cases, development of cell-mediated immunity controls the infection.
Diagnosis can be achieved through blood cultures, or cultures of other bodily fluids such as sputum. Bone marrow culture can often yield an earlier diagnosis, but is usually avoided as an initial diagnostic step because of its invasiveness.
Many people will have anemia and neutropenia if bone marrow is involved. MAC bacteria should always be considered in a person with HIV infection presenting with diarrhea.
The diagnosis requires consistent symptoms with two additional signs:
- Chest X-ray or CT scan showing evidence of right middle lobe (or left lingular lobe) lung infection
- Sputum culture or bronchoalveolar lavage culture demonstrating the infection is caused by MAC
Disseminated MAC is most readily diagnosed by one positive blood culture. Blood cultures should be performed in patients with symptoms, signs, or laboratory abnormalities compatible with mycobacterium infection. Blood cultures are not routinely recommended for asymptomatic persons, even for those who have CD4+ T-lymphocyte counts less than 100 cells/uL.
A Ghon focus is a primary lesion usually subpleural, often in the mid to lower zones, caused by "Mycobacterium bacilli" (tuberculosis) developed in the lung of a nonimmune host (usually a child). It is named for Anton Ghon (1866–1936), an Austrian pathologist.
It is a small area of granulomatous inflammation, only detectable by chest X-ray if it calcifies or grows substantially (see tuberculosis radiology). Typically these will heal, but in some cases, especially in immunosuppressed patients, it will progress to miliary tuberculosis (so named due to the granulomas resembling millet seeds on a chest X-ray).
The classical location for primary infection is surrounding the lobar fissures, either in the upper part of the lower lobe or lower part of the upper lobe.
If the Ghon focus also involves infection of adjacent lymphatics and hilar lymph nodes, it is known as the Ghon's complex or primary complex. When a Ghon's complex undergoes fibrosis and calcification it is called a Ranke complex.
The Ghon complex undergoes progressive fibrosis, often followed by radiologically detectable calcification (Ranke complex), and despite seeding of other organs, no lesions develop. Although they are often confused, Ranke complex and Ghon complex are not synonymous. The Ranke complex is an evolution of the Ghon complex (resulting from further healing and calcification of the lesion).
The Ghon complex is named after Austrian pathologist Anton Ghon; the Ranke complex is named in honour of German pulmonologist Karl Ernst Ranke.
MAC in patients with HIV disease is theorized to represent recent acquisition rather than latent infection reactivating (which is the case in many other opportunistic infections in immunocompromised patients).
The risk of MAC is inversely related to the patient's CD4 count, and increases significantly when the CD4 count decreases below 50 cells/mm³. Other risk factors for acquisition of MAC infection include using an indoor swimming pool, consumption of raw or partially cooked fish or shellfish, bronchoscopy and treatment with granulocyte stimulating factor.
Disseminated disease was previously the common presentation prior to the advent of highly active antiretroviral therapy (HAART). Today, in regions where HAART is the standard of care, localized disease presentation is more likely. This generally includes a focal lymphadenopathy/lymphadenitis.
Diagnosis usually occurs upon investigation of a cause for already suspected Cushing's syndrome. High levels of cortisol observed in patients with PPNAD are not suppressed upon administration of dexamethasone (dexamethasone suppression test), and upon MRI or CT imaging, the pituitary will show no abnormalities. Measuring ACTH will confirm that the cause of the patients Cushing's syndrome is ACTH independent. The nature of Cushing's syndrome itself is periodic, which can make diagnosing PPNAD increasingly difficult.
Diagnosis of PPNAD can be difficult to determine preoperatively as CT scan findings can be variable ie appear normal or suggest unilateral adrenal lesions therefore impeding the correct diagnosis. NP-59 scintigraphy may be particularly useful in identifying the bilateral nature of the disease.
Gene studies are not necessary for diagnosis as there are clear gross and histological diagnostic markers, as the nodules can usually be seen clearly in both cases A positive family history of PPNAD has been shown to be associated with abnormal histological findings, e.g. mitotic figures, which may further hinder diagnosis. At the point where abdominal CT scanning and pituitary fossa MRI show no clear abnormalities, adrenalectomy may be performed.
Primary inoculation tuberculosis (also known as "Cutaneous primary complex," "Primary tuberculous complex," and "Tuberculous chancre") is a skin condition that develops at the site of inoculation of tubercle bacilli into a tuberculosis-free individual.
Surgical correction is recommended when a constriction ring results in a limb contour deformity, with or without lymphedema.
Cardiac myxomas can be difficult to manage surgically because of recurrence within the heart, often far away from the site of the initial tumor.
At the beginning of the surgery a tourniquet will be applied to the limb. A tourniquet compresses and control the arterial and venous circulation for about 2 hours. The constriction band must be dissected very carefully to avoid damaging the underlying neurovasculature. When the constriction band is excised, there will be a direct closure. This allows the fatty tissue to naturally reposition itself under the skin.
“With complete circumferential constriction bands, it is recommended that a two-stage correction approach be used. At the first operation, one-half of the circumference is excised and the other one-half can be excised after three to six months. This will avoid any problems to the distal circulation in the limb, which may already be compromised. Lymphedema, when present, will significantly improve within a few weeks of the first surgery.”
For the direct closure of the defect after dissecting a constriction band there are two different techniques:
1. Triangular flaps; For this technique the circumference between the two borders must be measured. Depending on the difference the number of triangular flaps can be decided. With a triangular flap you can create more skin.
2. Z/W-plasty; “Z-plasty is a plastic surgery technique that is used to improve the functional and cosmetic appearance of scars. It can elongate a contracted scar or rotate the scar tension line. The middle line of the Z-shaped incision (the central element) is made along the line of greatest tension or contraction, and triangular flaps are raised on opposite sides of the two ends and then transposed.”
In rare cases, if diagnosed in utero, fetal surgery may be considered to save a limb that is in danger of amputation or other deformity. This operation has been successfully performed on fetuses as young as 22 weeks. The Melbourne's Monash Medical Centre in Australia, as well as multiple facilities in the United States of America, have performed successful amniotic band release surgery.
After diagnosis, it is important for patients to be continually monitored. The most common treatment for PPNAD is bilateral laparoscopic adrenalectomy; the process by which both adrenal glands are removed by a small incision.
Patients who have received this treatment will be prescribed mineralocorticoid and glucocorticoid steroids as they are no longer being naturally produced.
This is a treatment which has been used and refined since 1984.
At CT scans, bile duct hamartomas appear as small, well-defined hypo- or isoattenuating masses with little or no enhancement after contrast administration. At MRI, they appear hypointense on T1-weighted images, iso- or slightly hyperintense on T2-weighted images, and hypointense after administration of gadolinium based contrast-agent. On imaging, multiple hamartomas may look similar to metastases or microabscesses.
The ring 20 abnormality may be limited to as few as 5% of cells, so a screen for chromosomal mosaicism is critical. Newer array technology will not detect the ring chromosome and the standard metaphase chromosome analysis has been recommended. A karyotype analysis examining at least 50 cells should be requested to properly detect mosaicism.
An odontoma (also termed odontome) is a benign tumour of odontogenic origin (i.e. linked to tooth development). Specifically, it is a dental hamartoma, meaning that it is composed of normal dental tissue that has grown in an irregular way.
The average age of people found with an odontoma is 14. The condition is frequently associated with one or more unerupted teeth. Though most cases are found impacted within the jaw there are instances where odontomas have erupted into the oral cavity.
Odontomas are thought to be the second most frequent type of odontogenic tumor worldwide (after ameloblastoma), accounting for about 20% of all cases within this relatively uncommon tumor category which shows large geographic variations in incidence.
The American neurosurgeon Harvey Cushing in 1914 reported a patient with a pituitary tumour that he had operated on. Post mortum finding were suggestive of Carney complex. this condition had yet to be described. In 2017 archived tissue from this operation were subjected to DNA sequencing. This revealed a Arg74His (Arginine to Histidine: Guanine (G)-> Adenosine (A) transition in the second codon position of the 74 codon in the protein) mutation in the PRKAR1A gene confirming the diagnosis of Carney complex. Cushing's paper appears to be the first report of this complex.
When treated early, that is, before the onset of pulmonary hypertension, a good outcome is possible in patients with Shone’s syndrome. However, other surgical methods can be employed depending upon the patient’s medical background. The single most important determinant of poor outcome during the surgical management of patients with Shone's syndrome is the degree of involvement of the mitral valve and the presence of secondary pulmonary hypertension.
The Mazzotti reaction, first described in 1948, is a symptom complex seen in patients after undergoing treatment of onchocerciasis with the medication diethylcarbamazine (DEC). Mazzotti reactions can be life-threatening, and are characterized by fever, urticaria, swollen and tender lymph nodes, tachycardia, hypotension, arthralgias, oedema, and abdominal pain that occur within seven days of treatment of microfilariasis. The Mazzotti reaction correlates with intensity of infection; however, there are probably multiple infection intensity-dependent mechanisms responsible for mediating this complex reaction.
The phenomenon is so common when DEC is used for the treatment of onchocerciasis that this drug is the basis of a skin patch test used to confirm that diagnosis. The drug patch is placed on the skin, and if the patient is infected with the microfilaria of "O. volvulus", localized pruritus and urticaria are seen at the application site.
A case of the Mazzotti reaction has been reported after presumptive treatment of schistosomiasis and strongyloidiasis with ivermectin, praziquantel and albendazole. The patient had complete resolution of symptoms after intravenous therapy with methylprednisolone.
Ashman phenomenon, also known as Ashman beats, describes a particular type of wide QRS complex, often seen isolated that is typically seen in atrial fibrillation. It is more often misinterpreted as a premature ventricular complex.
It is named for Richard Ashman (of New Orleans) (1890 –1969), after first being described by Gouaux and Ashman in 1947.
Clinically, it is often asymptomatic by itself and considered benign in nature.
MR Imaging is best suited to evaluate patients with Shone's complex. Routine blood tests should be done prior to cardiac catheterization. The surgeons will repair the mitral valve and al the partial surgical removal of supramitral ring is done. This surgical method is preferred to the valve replacement procedure.
Classifying cardiac lesions in infants is quite difficult, and accurate diagnosis is essential. The diagnosis of Shone’s complex requires an ultrasound of the heart (echocardiogram) and a cardiac catheterization procedure, that is, insertion of a device through blood vessels in the groin to the heart that helps identify heart anatomy.
Though BLSII is an attractive candidate for gene therapy, bone marrow transplant is currently the only treatment.
AIDS-related complex, or ARC, was introduced after discovery of the HIV (Human Immunodeficiency Virus) when the medical community became aware of the inherent difficulties associated with treating patients suffering from an advanced case of HIV which gave rise to the term Acquired Immune Deficiency Syndrome (AIDS). The necessity for doctors to quickly and accurately understand the special needs of unknown patients suffering from AIDS in an emergency room situation was addressed with the creation of the term ARC.
ARC is "A prodromal phase of infection with the human immunodeficiency virus (HIV). Laboratory criteria separating AIDS-related complex ( ARC) from AIDS include elevated or hyperactive B-cell humoral immune responses, compared to depressed or normal antibody reactivity in AIDS; follicular or mixed hyperplasia in ARC lymph nodes, leading to lymphocyte degeneration and depletion more typical of AIDS; evolving succession of histopathological lesions such as localization of Kaposi's sarcoma, signaling the transition to the full-blown AIDS."
Clinical use of this term was widely discontinued by the year 2000 in the United States after having been replaced by modern laboratory criteria.
Generalized lentiginosis is a cutaneous condition that will occasionally present without other associated abnormalities. It may be caused by carney complex, LEOPARD syndrome or Peutz–Jeghers syndrome.
Sensenbrenner syndrome (OMIM #218330) is a rare (less than 20 cases reported by 2010) multisystem disease first described in 1975. It is inherited in an autosomal recessive fashion, and a number of genes appear to be responsible. Three genes responsible have been identified: intraflagellar transport (IFT)122 (WDR10), IFT43 — a subunit of the IFT complex A machinery of primary cilia, and WDR35 (IFT121: TULP4)
It is also known as Sensenbrenner–Dorst–Owens syndrome, Levin Syndrome I and cranioectodermal dysplasia (CED)