Here is the list of diseases and conditions that start with letter “K“:
- Kabuki syndrome
- Kallmann syndrome
- Kaposi’s sarcoma (KS)
- Kaposiform hemangioendothelioma
- Kawasaki disease (KD)
- Kearns–Sayre syndrome
- Kennedy’s disease
- Keratoconjunctivitis sicca (KCS)
- Keratolytic Winter erythema (KWE)
- Keratosis pilaris
- Kikuchi’s disease
- Klumpke’s paralysis
- Köhler disease
- Korsakoff’s syndrome
- Krause-van Schooneveld-Kivlin syndrome
- Kyasanur forest disease (KFD)
Kabuki syndrome, also previously known as Kabuki makeup syndrome, KMS or Niikawa–Kuroki Syndrome, is a pediatric congenital disorder of suspected genetic origin with multiple congenital anomalies and intellectual disabilities. It is very rare, affecting roughly one in every 32,000 individuals. It was discovered and described in 1981 by two Japanese groups, led by the scientists Niikawa and Kuroki (hence the name). It is named Kabuki Syndrome because of the facial resemblance of affected individuals with white Kabuki makeup, a Japanese traditional theatrical form. On the Kabuki Syndrome listserv, children with this syndrome are called Kabuki Kids, or KKs.
There is a wide range of congenital problems associated with Kabuki syndrome with large differences between affected individuals. Some of the common problems are heart defects (30%), urinary tract anomalies, hearing loss (50%), hypotonia, and postnatal growth deficiency (83%). Other characteristics include skeletal abnormality, joint laxity, short stature, and unusual dermatoglyphic patterns. They often suffer from recurrent ear infections in infancy.
In terms of development, mild to moderate intellectual disability (92%) is a common feature. Also, children with Kabuki syndrome often have distinctive behavioural features. For example, 50% are described as unusually sociable, 30% as engaging in only minimal interaction with others, 74% as liking routine and 87% as having a happy disposition. A few have normal intelligence, most of whom have learning difficulties such as struggling with fine motor, speech skills, and having a good memory.
The facial appearance of individuals with this syndrome include long eyelids with turning up of the lateral third of the lower eyelid, a broad and depressed nasal tip, large prominent earlobes, and a cleft or high-arched palate.
Other clinical features often include scoliosis, short fifth finger, persistence of fingerpads, and X-ray abnormalities of the vertebrae, hands, and hip joints.
Kallmann syndrome is a hypogonadism (decreased functioning of the glands that produce sex hormones) caused by a deficiency of gonadotropin-releasing hormone (GnRH), which is created by the hypothalamus. Kallmann syndrome is also called hypothalamic hypogonadism, familial hypogonadism with anosmia, and hypogonadotropic hypogonadism, reflecting its disease mechanism.
Kallmann syndrome is a form of tertiary hypogonadism, reflecting that the primary cause of the defect in sex-hormone production lies within the hypothalamus rather than a defect of the pituitary (secondary hypogonadism), testes or ovaries (primary hypogonadism). It was described in 1944 by Franz Josef Kallmann, a German-American geneticist. However, others, such as the Spanish doctor Aureliano Maestre de San Juan in 1856, had noticed a correlation between anosmia and hypogonadism.
The best-known person who has Kallmann syndrome is the jazz vocalist Jimmy Scott. In 2004, Canadian writer Brian Brett published a memoir, Uproar’s Your Only Music, about growing up with Kallmann syndrome. Kallmann syndrome’s characteristics: hypogonadotropic hypogonadism (a lack of the pituitary hormones LH and FSH); congenital (present from birth) anosmia (complete inability to smell) or hyposmia (decreased ability to smell); normal stature.
It can occasionally be associated with optic problems, such as colour blindness or optic atrophy, nerve deafness, cleft palate, cryptorchidism, renal agenesis, and mirror movement disorder. However, it is not clear how, if at all, these other problems have the same cause as the hypogonadism and anosmia.
Males present with delayed puberty and may have micropenis (although congenital micropenis is not present in most male KS cases). Females present with delayed puberty (i.e., primary amenorrhea) and lack of secondary sex characteristics, such as breast development.
Treatment is directed at restoring the deficient hormones—hormone therapy (HT). Males are administered human chorionic gonadotropin (hCG) or testosterone. Females are treated with estrogen and progestins.
There are a range of different methods for the delivery of HRT, especially for men. The short acting monthly injection is now less widely used in favour of the longer lasting injection, Nebido, which can last from 3 to 6 months depending on the individual. Daily application gels and patches are also available as are implants inserted every 6 months.
Tablets are not thought to be effective for the treatment of Kallmann syndrome due to their low bio-availablity once processed by the liver, though this can be overcome by using oil filled capsules which allows the testosterone to reach the blood stream in effective doses.
Having Kallmann syndrome can have a profound affect on a person’s life, however it will affect different people in different ways. Age of diagnosis and treatment is a big key to how well an individual patient copes with the condition. For some patients the ability to put a name to the condition and the knowledge that they are not the only person in the world with this condition is very reassuring.
With the key symptom being not going through puberty at the normal age it can produce a huge effect on a person’s social development as well as physical development.
It will vary from person to person but in general men with Kallmann syndrome will have a smaller penile length than the average for the population, which in addition to the lack of testicular development can affect self confidence to such a degree that sexual activity is not even attempted. Most men with Kallmann syndrome can have a normal, active sex life but the confidence required to achieve this is sometimes beyond some men with Kallmann syndrome and they have less sexual activity than other people the same age.
Another aspect of Kallmann syndrome is the social isolation. Since it is such a rare condition a lot of patients with Kallmann syndrome have never even met or talked to a fellow patient. The ability to meet and talk to other people with the condition goes a long way to helping a patient come to terms with the condition.
Kaposi’s sarcoma (KS) is a tumor caused by Human herpesvirus 8 (HHV8), also known as Kaposi’s sarcoma-associated herpesvirus (KSHV). It was originally described by Moritz Kaposi (KA-po-she), a Hungarian dermatologist practicing at the University of Vienna in 1872. It became more widely known as one of the AIDS defining illnesses in the 1980s. The viral cause for this cancer was discovered in 1994. Although KS is now well-established to be caused by a virus infection, there is widespread lack of awareness of this even among persons at risk for KSHV/HHV-8 infection.
Restated, Kaposi’s sarcoma (KS) is a systemic disease which can present with cutaneous lesions with or without internal involvement. Four subtypes have been described: Classic KS, affecting middle aged men of Mediterranean and Jewish descent, African endemic KS, KS in iatrogenically immunosuppressed patients, and AIDS-related KS. The erythematous to violaceous cutaneous lesions seen in KS have several morphologies: macular, patch, plaque, nodular, and exophytic. The cutaneous lesions can be solitary, localized or disseminated. KS can involve the oral cavity, lymph nodes, and viscera. The pathogenesis of KS is still being elucidated, though infection with HHV-8 appears to be associated with KS development. Classic KS tends to be indolent, presenting with erythematous or violaceous patches on the lower extremities. African endemic KS and AIDS-related KS tend to be more aggressive. The AIDS-related KS lesions often rapidly progress to plaques and nodules affecting the upper trunk, face, and oral mucosa. The diagnosis can be made with a tissue biopsy and, if clinically indicated, internal imaging should be done.
Once the diagnosis of KS has been made, treatment is based on the subtype and the presence of localized versus systemic disease. Localized cutaneous disease can be treated with cryotherapy, intralesional injections of vinblastine, alitretinoin gel, radiotherapy, topical immunotherapy (imiquimod), or surgical excision. Extensive cutaneous disease and/or internal disease may require IV chemotherapy and immunotherapy. Discontinuation or reduction of immunosuppressive therapy is recommended when KS arises in the setting of iatrogenic immunosuppression. However, with AIDS-related KS, HAART has been shown to prevent or induce regression of KS. Some AIDS patients have complete resolution of the lesions and prolonged remission while continuing the therapy. Therefore, HAART should be considered first-line treatment for these patients, though they may require other concomitant treatments.
Kaposiform hemangioendothelioma (also known as “Infantile kaposiform hemangioendothelioma” ) is an uncommon vascular tumor, first described by Zukerberg, Nickoloff, and Weiss in 1993, that affects infants and young children, with rare cases having also been reported in adults.
Kawasaki disease (KD), also known as Kawasaki syndrome, lymph node syndrome and Mucocutaneous lymph node syndrome, is an autoimmune disease that manifests as a systemic necrotizing medium-sized vessel vasculitis and is largely seen in children under 5 years of age. It affects many organ systems, mainly those including the blood vessels, skin, mucous membranes and lymph nodes; however, its most serious effect is on the heart where it can cause severe coronary artery aneurysms in untreated children. Without treatment, mortality may approach 1%, usually within 6 weeks of onset. With treatment, the mortality rate is less than 0.01% in the U.S. There is often a pre-existing viral infection that may play a role in its pathogenesis. The conjunctival and oral mucosa, along with the epidermis (skin), become erythematous (red and inflamed). Edema is often seen in the hands and feet and one or both of the cervical lymph nodes are often enlarged. Also, a remittant fever, often 40℃ (104°F) or higher, is characteristic of the acute phase of the disease. In untreated children, the febrile period lasts on average approximately ten days, but may range from 5 to 25 days. The disorder was first described in 1967 by Dr. Tomisaku Kawasaki in Japan.
Kawasaki disease often begins with a high and persistent fever that is not very responsive to normal treatment with paracetamol (acetaminophen) or ibuprofen. The fever may persist steadily for up to two weeks and is normally accompanied by irritability. Affected children develop red eyes because of non-suppurative conjunctivitis, iritis and bilateral anterior uveitis. Inflammation of the mucous membranes in the mouth, along with erythema (redness), edema (swelling) with fissures (cracks in the lip surface), desquamation (peeling) and exsudation of the lips are also evident. The oropharynx mucosa has enanthema and the tongue maintains an unusual red appearance termed “strawberry tongue” (marked erythema with prominent gustative papillae). Keratic precipitates (detectable by a slit lamp but usually too small to be seen by the unaided eye), and swollen lymph nodes may also be present and can be the first manifestation of the disease. Rashes occur early in the disease, and the cutaneous rash observed in patients with KD is non-specific, polymorphic, non-itchy and normally observed up to the 5th day of fever. Cutaneous exanthema may comprise macular-papular erythematous and fissure lesions, the most common type, in addition to urticariform type rash, purpuric, multiform-like erythema. and peeling of the skin in the genital area, hands, and feet (especially around the nails and on the palms and soles) may occur in later phases.
Like all autoimmune diseases, the cause of Kawasaki disease is presumably the interaction of genetic and environmental factors, possibly including an infection. The specific cause is unknown, but current theories center primarily on immunological causes for the disease. Evidence increasingly points to an infectious etiology, but debate continues on whether the cause is a conventional antigenic substance or a superantigen.
Kawasaki disease can only be diagnosed clinically (i.e. by medical signs and symptoms). There exists no specific laboratory test for this condition. It is difficult to establish the diagnosis, especially early in the course of the illness, and frequently children are not diagnosed until they have seen several health care providers. Many other serious illnesses can cause similar symptoms, and must be considered in the differential diagnosis, including scarlet fever, toxic shock syndrome, juvenile idiopathic arthritis, and childhood mercury poisoning (infantile acrodynia).
Kearns–Sayre syndrome (abbreviated KSS) also known as oculocraniosomatic disease or Oculocraniosomatic neuromuscular disease with ragged red fibers is a mitochondrial myopathy with a typical onset before 20 years of age. KSS is a more severe syndromic variant of chronic progressive external ophthalmoplegia (abbreviated CPEO), a syndrome that is characterized by isolated involvement of the muscles controlling eyelid movement (levator palpebrae, orbicularis oculi), and those controlling eye movement (extra-ocular muscles). This results in ptosis and ophthalmoplegia respectively. KSS involves a triad of the already described CPEO, as well as bilateral pigmentary retinopathy, and cardiac conduction abnormalities. Other areas of involvement can include cerebellar ataxia, proximal muscle weakness, deafness, diabetes mellitus, growth hormone deficiency, hypoparathyroidism, or other endocrinopathies. In both of these diseases, muscle involvement may begin unilateral but always develops into a bilateral deficit, and the course is progressive.
Keloid (also known as a “keloidal scar“) is a type of scar, which depending on its maturity, is composed of mainly either type III (early) or type I (late) collagen. It is a result of an overgrowth of granulation tissue (collagen type 3) at the site of a healed skin injury which is then slowly replaced by collagen type 1. Keloids are firm, rubbery lesions or shiny, fibrous nodules, and can vary from pink to flesh-coloured or red to dark brown in colour. A keloid scar is benign, non-contagious, and sometimes accompanied by severe itchiness and pain, and changes in texture. In severe cases, it can affect movement of skin. Keloids should not be confused with hypertrophic scars, which are raised scars that do not grow beyond the boundaries of the original wound.
Keloids expand in claw-like growths over normal skin. They have the capability to hurt with a needle-like pain or to itch without warning, although the degree of sensation varies from patient to patient.
If the keloid becomes infected, it may ulcerate. Removing the scar is one treatment option, however may result in more severe consequences i.e. the probability that the resulting surgery scar will also become a keloid is high, usually greater than 50%. Laser treatment has also been used with varying degrees of success.
Keloids form within scar tissue. Collagen, used in wound repair, tends to overgrow in this area, sometimes producing a lump many times larger than that of the original scar. Although they usually occur at the site of an injury, keloids can also arise spontaneously. They can occur at the site of a piercing and even from something as simple as a pimple or scratch. They can occur as a result of severe acne or chickenpox scarring, infection at a wound site, repeated trauma to an area, excessive skin tension during wound closure or a foreign body in a wound. Keloids can sometimes be sensitive to chlorine. Keloid scars can grow, if they appear at a younger age, because the body is still growing.
Kennedy’s disease or X-linked spinal and bulbar muscular atrophy (SBMA) or spinobulbar muscular atrophy or X-Linked bulbo-spinal atrophy is a neuromuscular disease associated with mutation of the androgen receptor (AR). Because of its endocrine manifestations related to the impairment of the AR, it can be viewed as a variation of the disorders of the androgen insensitivity syndrome (AIS). It is also related to other neurodegenerative diseases caused by similar mutations, such as Huntington’s disease and the spinocerebellar ataxias. Kennedy’s disease is named after W. R. Kennedy, a neurologist who was among the first to describe this disease.
Kennedy’s disease patients have muscle cramps and progressive weakness due to degeneration of motor neurons in the brain stem and spinal cord. Ages of onset and severity of manifestations in affected males vary from adolescence to old age, but most commonly develop in middle adult life. The latest onset was described in a male of 84 years of age. KD does not usually compromise longevity. The syndrome has neuromuscular and endocrine manifestations.
Keratoacanthoma is a relatively common low-grade malignancy that originates in the pilosebaceous glands and closely resembles squamous cell carcinoma (SCC). In fact, strong arguments support classifying KA as a variant of invasive SCC. The pathologist often labels KA as “well- differentiated squamous cell carcinoma, keratoacanthoma variant”. KA is characterized by rapid growth over a few weeks to months, followed by spontaneous resolution over 4–6 months in most cases. KA reportedly progresses, although rarely, to invasive or metastatic carcinoma; therefore, aggressive surgical treatment often is advocated. Whether these cases were SCC or KA, the reports highlight the difficulty of distinctly classifying individual cases. Diagnosis is best done with the clinical exam and history. It presents as a fleshy, elevated and nodular lesion with an irregular crater shape and a characteristic central hyperkeratotic core. Usually the patient will notice a rapid growing dome-shaped tumor on sun-exposed skin. Skin keratoacanthoma whole slide.
A skin biopsy must be performed to confirm the diagnosis. Unfortunately, a shave biopsy will often reveal only keratin fragments. A deep punch biopsy will often reveal a well differentiated, mildly atypical, squamous cell suggestive of an actinic keratosis or a squamous cell carcinoma. Only when the pathologist has access to the entire lesion (not practical in many circumstances) can a correct diagnosis be rendered. From a practical standpoint (insurance reimbursement), the correct diagnosis should be dictated as “well differentiated squamous cell carcinoma, keratoacanthoma variant”. This is especially important for facial and nasal KA’s, as it allows the surgeon to treat the tumor with the proper respect it deserves, with margin-controlled surgery like Mohs surgery.
On the trunk, arms, and legs, electrodesiccation and curettage often suffice. Excision of the entire lesion is often required if one wants to confirm the clinical diagnosis of keratoacanthoma. On the nose and face, Mohs surgery allows for good margin control with minimal tissue removal; unfortunately, many insurance companies require the correct diagnosis of a malignancy before allowing such procedure. Recurrence after electrodesiccation and curettage is frequent, and usually can be identified and treated promptly with either further curettage or surgical excision. Allowing the KA to grow and necrose spontaneously is not acceptable in today’s standard of care.
Keratoconjunctivitis sicca (KCS), also called keratitis sicca, xerophthalmia or dry eye syndrome (DES) is an eye disease caused by eye dryness, which, in turn, is caused by either decreased tear production or increased tear film evaporation. It is found in humans and some animals. The phrase “keratoconjunctivitis sicca” is Latin, and its literal translation is “dry inflammation of the cornea and conjunctiva”. A variety of approaches can be taken to treatment. These can be summarised as: avoidance of exacerbating factors, tear stimulation and supplementation, increasing tear retention, and eyelid cleansing and treatment of eye inflammation.
Keratoconus is a degenerative disorder of the eye in which structural changes within the cornea cause it to thin and change to a more conical shape than its normal gradual curve.
Keratoconus can cause substantial distortion of vision, with multiple images, streaking and sensitivity to light all often reported by the patient. It is typically diagnosed in the patient’s adolescent years and attains its most severe state in the twenties and thirties. If afflicting both eyes, the deterioration in vision can affect the patient’s ability to drive a car or read normal print. In most cases, corrective lenses are effective enough to allow the patient to continue to drive legally and likewise function normally. Further progression of the disease may require surgery including intrastromal corneal ring segments, corneal collagen cross-linking, or corneal transplantation. However, despite the disease’s unpredictable course, keratoconus can often be successfully managed with little or no impairment to the patient’s quality of life.
Keratoconus affects around one person in a thousand. It seems to occur in populations throughout the world, although it occurs more frequently in certain ethnic groups such as South Asians. The exact cause of keratoconus is uncertain, but has been associated with detrimental enzyme activity within the cornea. A genetic link seems likely, as the incidence rate is greater if a family member has been diagnosed. The progression of keratoconus is rapid in patients having Down syndrome.
People with early keratoconus typically notice a minor blurring of their vision and come to their clinician seeking corrective lenses for reading or driving. At early stages, the symptoms of keratoconus may be no different from those of any other refractive defect of the eye. As the disease progresses, vision deteriorates, sometimes rapidly. Visual acuity becomes impaired at all distances, and night vision is often quite poor. Some individuals have vision in one eye that is markedly worse than that in the other eye. The disease is often bilateral, though asymmetrical in many patients. Some develop photophobia (sensitivity to bright light), eye strain from squinting in order to read, or itching in the eye, but there is normally little or no sensation of pain.
Keratolytic Winter erythema (KWE), also known as erythrokeratolysis hiemalis, Oudtshoorn disease and Oudtshoorn skin, is a rare autosomal dominant skin disease of unknown etiology which causes erythema (redness) and keratolysis (peeling) of the skin on the palms and soles. Onset, increased prominence and severity usually occurs during winter. It is a type of genodermatosis.
The name “Oudtshoorn skin” derives from the town of Oudtshoorn in the Western Cape province of South Africa, where the disorder was first described. It is one of several genetic disorders known to be highly prevalent among the Afrikaner population.
Keratosis pilaris (KP, also follicular keratosis), more commonly known as chicken skin, is a common, autosomal dominant, genetic follicular condition that is manifested by the appearance of rough bumps on the skin. It most often appears on the back and outer sides of the upper arms (though the lower arms can also be affected), and can also occur on the thighs, hands, and tops of legs, flanks, buttocks, or any body part except glabrous skin (like the palms or soles of feet). Less commonly, lesions appear on the face, which may be mistaken for acne. Keratosis pilaris is completely harmless; however the condition can contribute to or exacerbate depression and anxiety.
Worldwide, KP affects an estimated 40% of the adult population and approximately 50%-80% of all adolescents. It is more common in women than in men. There are several different types of keratosis pilaris, including keratosis pilaris rubra (red, inflamed bumps which can be on arms, head, legs), keratosis pilaris alba (rough, bumpy skin with no irritation), keratosis pilaris rubra faceii (reddish rash on the cheeks), and related disorders. While KP resembles goose bumps, it is characterized by the appearance of small rough bumps on the skin. As a result, many people with keratosis pilaris do not know they have it, and it is often confused with acne.
While there is no cure for keratosis pilaris, there are palliative treatments available. The efficacy of these treatment methods is directly related to the individual’s commitment and consistency of use.
Creams containing the acid form of vitamin A, Tretinoin, have been shown to help. Most commonly sold under the trade name Retin-A, it is a topical retinoid medically approved in the treatment of acne. This medicine works by increasing the cell turnover rate of the outer layer of the skin, decreasing the amount of the keratin in the skin. As a result, the surface layer of the skin becomes thinner and pores are less likely to become blocked, reducing the occurrence of symptoms related to acne. While keratosis pilaris is not acne, some believe this action may be of benefit to those with KP as well.
Kernicterus is damage to the brain centers of infants caused by increased levels of unconjugated bilirubin. This may be due to several underlying pathologic processes. Newborn babies are often polycythemic. When they break down the erythrocytes, one of the byproducts is bilirubin, which circulates in the blood and causes jaundice. Alternately, Rh incompatibility between mother and fetus may cause hemolysis of fetal red blood cells, thereby releasing unconjugated bilirubin into the fetal blood. Since the fetal blood brain barrier is not fully formed, some of this released bilirubin enters the brain and interferes with normal neuronal development. Kernicterus may also be found in infants as a symptom of Crigler-Najjar syndrome type I, a hereditary hyperbilirubinemia resulting in a decreased ability to excrete bilirubin that is fatal within 18 months of life. Other inherited genetic disorders that can contribute to the development of hyperbilirubinemia are Gilbert’s syndrome and G6PD deficiency, especially if they are present concurrently.
In adults and older children, jaundice is harmless in and of itself. However, the tissues protecting the brain (the blood-brain barrier) are immature in newborns. Bilirubin penetrates the brain and is deposited in cell bodies (gray matter), especially the basal ganglia, causing irreversible damage. Depending on the level of exposure, the effects range from clinically unnoticeable to severe brain damage and even death.
Some medications, such as the antibiotic co-trimoxazole (a combination of trimethoprim/sulfamethoxazole) may induce this disorder in the baby, either when taken by the mother or given directly to the baby, due to displacement of bilirubin from binding sites on serum albumin. The bilirubin is then free to pass into the Central Nervous System, because the baby’s blood-brain barrier is not fully developed. The only effective way at preventing kernicterus is to lower the serum bilirubin levels either by phototherapy or exchange transfusion.
Symptoms of kernicterus in infants include abnormalities of tone, including hypertonia or hypotonia, lethargy, a distinct, high-pitched cry, and arching of the back (retrocollis or opisthotonus). In children and adults, mild kernicterus manifests through movement and language processing disorders. Furthermore, it mimics autism-spectrum disorders and is often misdiagnosed as such.
Kikuchi’s disease, also known as histiocytic necrotizing lymphadenitis and Kikuchi-Fujimoto disease, is a rare, non-cancerous enlargement of the lymph nodes. It was first described in Japan by Dr Masahiro Kikuchi in 1972 and independently by Y. Fujimoto.
Kikuchi’s disease is a very rare disease and mainly seen in Japan. Isolated cases are reported in America, Europe and Asia. It is mainly a disease of young adults (mean age, 20–30 years), with a slight preponderance towards females. The cause of this disease is not known although infectious and autoimmune etiologies have been proposed. Course of the disease is generally benign and self-limiting. Lymphadenopathy most often resolves over several weeks to six months. Recurrence rate is about 3%. Mortality is extremely rare and usually due to hepatic, respiratory, or cardiac failure.
Klumpke’s paralysis (or Klumpke’s palsy or Dejerine-Klumpke palsy) is a variety of partial palsy of the lower roots of the brachial plexus. The brachial plexus is a network of spinal nerves that originates in the back of the neck, extends through the axilla (armpit), and gives rise to nerves to the upper limb.
Klumpke’s paralysis is a form of paralysis involving the muscles of the forearm and hand, resulting from a brachial plexus injury in which the eighth cervical (C8) and first thoracic (T1) nerves are injured “either before or after they have joined to form the lower trunk. The subsequent paralysis affects, principally, the intrinsic muscles of the hand and the flexors of the wrist and fingers”. Forearm pronators and wrist flexors may be involved, as may dilators of the iris and elevators of the eyelid (both of which may be seen in the case of associated Horner’s Syndrome). The classic presentation of Klumpke’s palsy is the “claw hand” where the forearm is supinated and the wrist and fingers are hyperextended. If Horner syndrome is present, there is miosis (constriction of the pupils) in the affected eye.
Symptoms include claw hand, paralysis of intrinsic hand muscles, and ulnar nerve distribution numbness. Involvement of T1 may result in Horner’s syndrome, with ptosis, and miosis.
Köhler disease (also spelled “Kohler”) is a rare bone disorder of the foot found in children between six and nine years of age. The disease typically affects boys, but it can also affect girls. It was first described in 1908 by Alban Köhler (1874–1947), a German radiologist. It is caused when the navicular bone temporarily loses its blood supply. As a result, tissue in the bone dies and the bone collapses. When treated, it causes no long term problems. As the navicular bone gets back to normal, symptoms typically abate.
Sufferers experience pain and swelling in the middle part of the foot and usually limp as a result. Patients that walk with a limp tend to walk with increased weight on the lateral side of the foot. Also, there can be tenderness over the navicular. Patients often complain of pain over the apex. An X-ray of both feet is used to diagnose disease. The affected foot tends to have a sclerotic and flattened navicular bone.
It is believed that this condition may be the result of an abnormal strain acting on a weak, un-ossified navicular bone. Ossification refers to the hardening of bone. In boys this takes place around 24 to 30 months and in girls around 18 to 24 months. As the child grows, more weight is put on the foot. If the navicular bone ossifies slower than the bones surrounding it, then those bones may compress the navicular and it’s blood vessels, causing it to lose blood supply. However, there are no known causes.
Korsakoff’s syndrome (also called Korsakov’s syndrome, Korsakoff’s psychosis, or amnesic-confabulatory syndrome), is a neurological disorder caused by the lack of thiamine (vitamin B1) in the brain. The syndrome is named after Sergei Korsakoff, the neuropsychiatrist who popularized the theory.
It was once assumed that anyone suffering from Korsakoff’s syndrome would eventually need full time care. This is still often the case, but rehabilitation can help regain some, often limited, level of independence. Treatment involves the replacement or supplementation of thiamine by intravenous (IV) or intramuscular (IM) injection, together with proper nutrition and hydration. However, the amnesia and brain damage caused by the disease does not always respond to thiamine replacement therapy. In some cases, drug therapy is recommended. If treatment is successful, improvement will become apparent within two years although recovery is slow and often incomplete.
Conditions resulting in the vitamin deficiency and its effects include chronic alcoholism and severe malnutrition. Alcoholism may be an indicator of poor nutrition, which in addition to inflammation of the stomach lining, causes thiamine deficiency. Other causes include dietary deficiencies, prolonged vomiting, eating disorders, or the effects of chemotherapy. It can also occur in pregnant women who have a form of extreme morning sickness known as hyperemesis gravidarum. Mercury poisoning can also lead to Korsakoff’s syndrome. It has also been caused by centipede (mukade) bites in Japan.
Krause-van Schooneveld-Kivlin syndrome is a hereditary syndrome that mainly affects the eyes, growth and development of the individual. It is also known as Krause-Kivlin syndrome or Peters-plus syndrome. Features of this syndrome include Peters anomaly, leukoma, central defect of Descemet’s membrane, and shallow anterior chamber with synechiae between the iris and cornea. It is associated with short limb dwarfism and delayed mental development.
Krause-van Schooneveld-Kivlin syndrome is listed as a “rare disease” by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Krause-van Schooneveld-Kivlin syndrome, or a subtype of Krause-van Schooneveld-Kivlin syndrome, affects fewer than 200,000 people in the United States.
Kwashiorkor is an acute form of childhood protein-energy malnutrition characterized by edema, irritability, anorexia, ulcerating dermatoses, and an enlarged liver with fatty infiltrates. The presence of edema caused by poor nutrition defines kwashiorkor. Kwashiorkor was thought to be caused by insufficient protein consumption but with sufficient calorie intake, distinguishing it from marasmus. More recently, micronutrient and antioxidant deficiencies have come to be recognized as contributory. Cases in the developed world are rare.
The defining sign of kwashiorkor in a malnourished child is pedal edema (swelling of the feet). Other signs include a distended abdomen, an enlarged liver with fatty infiltrates, thinning hair, loss of teeth, skin depigmentation and dermatitis. Children with kwashiorkor often develop irritability and anorexia. Victims of kwashiorkor fail to produce antibodies following vaccination against diseases, including diphtheria and typhoid. Generally, the disease can be treated by adding food energy and protein to the diet; however, it can have a long-term impact on a child’s physical and mental development, and in severe cases may lead to death.
In dry climates, marasmus is the more frequent disease associated with malnutrition. Another malnutrition syndrome includes cachexia, although it is often caused by underlying illnesses. These are important considerations in the treatment of the patients. Kwashiorkor can lead to death. People can recover from the illness by having a gradual build up of nutrients, but they will not grow properly and will probably be quite small.
Kyphosis, also called hunchback, is a common condition of a curvature of the upper back. It can be either the result of degenerative diseases (such as arthritis), developmental problems (the most common example being Scheuermann’s disease), osteoporosis with compression fractures of the vertebrae, and/or trauma.
In the sense of a deformity, it is the pathological curving of the spine, where parts of the spinal column lose some or all of their lordotic profile. This causes a bowing of the back, seen as a slouching back and breathing difficulties. Severe cases can cause great discomfort and even lead to death.
The Milwaukee brace is one particular body brace that is often used to treat kyphosis in the US. Modern CAD / CAM braces are used in Europe to treat different types of kyphosis. These are much easier to wear and have better in-brace corrections than reported for the Milwaukee brace. Since there are different curve patterns (thoracic, thoracolumbar and lumbar) different types of braces are in use. The advantages / disadvantages of different braces are discussed in a recent review article.
Surgical treatment can be used in severe cases. In patients with progressive kyphotic deformity due to vertebral collapse, a procedure called a kyphoplasty may arrest the deformity and relieve the pain. The procedure is serious and consists of fusion of the abnormal vertebrae.
Kyasanur forest disease (KFD) is a tick-borne viral hemorrhagic fever endemic to South Asia. The disease is caused by a virus belonging to the family flaviviridae, which also includes yellow fever and dengue fever. There are a variety of animals thought to be reservoir hosts for the disease, including porcupines, rats, squirrels, mice and shrews. The vector for disease transmission is Haemaphysalis spinigera, a forest tick. Humans contract infection from the bite of nymphs of the tick.