KALLMANN SYNDROME
DESCRIPTION Also known as hypogonadotropic hypogonadism with anosmia, caused by failure of GnRH secretion by the hypothalamus, leading to testicular failure. KAL1, encoding the extracellular glycoprotein anosmin-1, is responsible for the X-linked recessive form of the disease. It is a cause of male infertility due to the defect in the short arm of the X chromosome, and has variable inheritance and penetrance. Anosmia, cleft palate, renal anomalies, microphallus, cryptorchidism, blindness, and deafness are also associated conditions. Testes are typically small. Delayed puberty is often an initial presenting sign.
TREATMENT
• Androgens can virilize but will not promote spermatogenesis.
• hCG with FSH and LH may help fertility.
REFERENCE
Dodé C, Hardelin JP. Kallmann syndrome. Eur J Hum Genet. 2009;17(2):139–146.
KAPOSI SARCOMA, UROLOGIC CONSIDERATIONS
DESCRIPTION A tumor of reticuloendothelial system that presents as a raised, painful papule or ulcer with a bluish hue. In the United States, it is seen most commonly in association with AIDS. Most common site in the GU system is the penis, with a much higher incidence in homosexual males. It may cause urethral obstruction. (See also Section I: “HIV/AIDS, Urologic Considerations.”)
TREATMENT
• Radiation or penectomy (partial or total) aimed at palliation
• Proximal urethrostomy for obstruction not responsive to other treatment
REFERENCE
Woldrich JM, Silberstein JL, Saltzstein SL, et al. Penile Kaposi sarcoma in the state of California. Can J Urol. 2012;19(2):6178–6182.
KARTAGENER SYNDROME (IMMOTILE CILIA SYNDROME)
DESCRIPTION Also called primary ciliary dyskinesia syndrome, this syndrome is characterized by situs inversus, chronic sinusitis, otitis media, airway disease, and immotile sperm leading to infertility. The absence of the inner and outer dynein arm of cilia is the primary pathology. Most men have live but immotile spermatozoa and are infertile, whereas some have motile spermatozoa but immotile cilia. Women have decreased fertility, with <50% completing pregnancy. This is the most common of a group of inherited ciliary defects that lead to respiratory disorders called primary ciliary dyskinesias. ICSI may be used for reproduction, but genetic counseling should be offered.
REFERENCE
Haddad G, Kashgarian M. Primary ciliary dyskinesia (immotile cilia syndrome). In: Kliegman R, et al., eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, PA: Saunders; 2007.
KEGEL EXERCISES
DESCRIPTION 1st described by Arnold Kegel in 1948, these exercises can be used as treatment for both stress and urgency urinary incontinence. Modern cure/improvement rates range from 50–80%. The usual regimen consists of multiple contractions of the pelvic floor muscles ≥3 times a day. Patients can practice by starting and stopping their urinary stream. Biofeedback, electrical stimulation, and cystometry are adjuncts to Kegel exercises.
SYNONYM
Pelvic floor exercises
REFERENCES
Bump RC, Hurt WG, Fantl JA, et al. Assessment of Kegel pelvic muscle exercise performance after brief verbal instruction. Am J Obstet Gynecol. 1991;165(2):322–327.
Park SW, Kim TN, Nam JK, et al. Recovery of overall exercise ability, quality of life, and continence after 12-week combined exercise intervention in elderly patients who underwent radical prostatectomy: a randomized controlled study. Urology. 2012;80(2):299–305.
KELAMI CLASSIFICATION SYSTEM (MODIFIED)
DESCRIPTION A classification system developed by Kelami to define the severity of penile curvature. The system consists of a grading system from 1–3: Grade 1, curvature of ≤30°; grade 2, curvature of 30–60°; and grade 3, ≥60° curvature. (See also Section I: “Penis, Curvature and/or Pain.”)
REFERENCE
Usta MF, Bivalacqua TJ, Jabren GW, et al. Relationship between the severity of penile curvature and the presence of comorbidities in men with Peyronie’s disease. J Urol. 2004;171(2 Pt 1):775–779.
KELLY PLICATION
DESCRIPTION A transvaginal surgical technique to treat female SUI. The fibromuscularis tissue underlying the bladder neck is plicated in the midline through an anterior vaginal wall incision. No longer a commonly used treatment modality for SUI due to the availability of superior treatment modalities and the risk of postoperative voiding dysfunction.
REFERENCE
Thaweekul Y, Bunyavejchevin S, Wisawasukmongchol W, et al. Long term results of anterior colporrhaphy with Kelly plication for the treatment of stress urinary incontinence. J Med assoc Thai. 2004;87(4):357–360.
KERR KINKS
DESCRIPTION Kinking of the renal pelvis due to a deformity of the pyelocalyceal system, caused by traction of a strictured infundibulum and parenchymal fibrosis of a tuberculous kidney. The deformity leads to obstruction and dilatation of areas not directly affected by tuberculous ulcerations and eventual pressure atrophy of renal tissue.
REFERENCE
Barrie HJ, Kerr WK, Gale GL. The incidence and pathogenesis of tuberculous strictures of the renal pelvis. J Urol. 1967;98:584–589.
KETAMINE ABUSE, UROLOGIC CONSIDERATIONS
DESCRIPTION Ketamine is generally used as a dissociative anesthetic compound by licensed anesthesiologists. When used recreationally, it can cause hallucinations, derealization, and thought disorders. It has seen an increase in use in young adults. Ketamine abuse can cause urinary tract changes. These include findings of bilateral upper tract narrowing, hydronephrosis, and contracted bladder states on urodynamic studies.
REFERENCE
Lai Y, Wu S, Ni L, et al. Ketamine-associated urinary tract dysfunction: an under-recognized clinical entity. Urol Int. 2012;89(1):93–96.
KIBRICK TEST
DESCRIPTION A test designed to evaluate circulating immune factors, as an aid to diagnosing causes of infertility. Dilutions of serum from both partners are combined with semen samples in a medium with an agglutinating gelatin. Agglutination will occur if antibodies in the serum are reactive against the sperm. Controls are usually also run with the samples to prevent errors.
REFERENCE
Kalaydjiev SK, Dimitrova DK, Trifonova NL, et al. The age-related changes in the incidence of ‘natural’ anti-sperm antibodies suggest they are not auto-/isoantibodies. Am J Reprod Immunol. 2002;47(2):65–71.
KIDNEY, METASTASIS TO
DESCRIPTION Kidney metastases may present as a renal mass and grossly appear as a renal primary neoplasm. Discovered most often at autopsy, with an incidence of about 7%. They are frequently asymptomatic, but flank pain, hematuria, or hemorrhage may occur. Common primary tumors are lung (bronchogenic carcinoma most common), ovary, bowel, breast, and lymphoma. Virtually any origin is possible.
REFERENCE
Campbell SC, Lane BR. Malignant renal tumors. In: Wein AJ, et al., eds. Campbell-Walsh Urology. 10th ed. Philadelphia, PA: Saunders; 2012:1413–1474.
KIDNEY, SUPERNUMERARY
DESCRIPTION One of the least common genitourinary anomalies, this mass of renal tissue has no parenchymatous connection with the definitive kidney (unlike a horseshoe kidney). The supernumerary kidney is usually in a caudal position relative to the normal kidney and rarely is in a more cephalad position. The kidney is usually smaller or hypoplastic than a normal kidney and can function normally or not function at all. The ureter can insert into a normal ureter or bladder. It is usually associated with other GU anomalies, such as duplicated renal pelvis, vaginal atresia, and duplicated female urethra. Treatment is unnecessary unless disease is present. (See also Section I: “Renal Ectopia and Renal Fusion Anomalies.”)
REFERENCE
Favorito LA, Morais AR. Evaluation of supernumerary kidney with fusion using magnetic resonance image. Int Braz J Urol. 2012;38(3):428–429.
KLINEFELTER SYNDROME
DESCRIPTION A syndrome characterized by small, firm testes, gynecomastia, and elevated urinary gonadotropins; it is present in 1 out of 600 male births. Usually presents as incomplete virilization, infertility, or rarely as male pseudohermaphroditism. Mental retardation and low bone mineral density (BMD) are associated. A testicular biopsy usually shows sclerosis of tubules. The condition is caused by a nondisjunction of the meiotic chromosome, resulting in XXY karyotype and its variants. FSH is markedly elevated. Azoospermia is traditionally described on semen analysis, but recent series indicate that sperm can be found in over 50% of men with Klinefelter syndrome; thus, these men are not always sterile. Recent evidence suggests that children with Klinefelter syndrome are born with spermatogonia and lose large numbers of germ cells during puberty. No treatment can improve spermatogenesis. (See also Section II: “XXY Syndrome.”)
REFERENCE
Paduch DA, Fine RG, Bolyakov A, et al. New concepts in Klinefelter syndrome. Curr Opin Urol. 2008;18(6):621–627. Review.
KLIPPEL–TRENAUNAY–WEBER SYNDROME
DESCRIPTION Klippel–Trenaunay–Weber syndrome was 1st described by French physicians in 1900. It consists of cutaneous vascular malformations in combination with soft tissue and bone hypertrophy. The defects are present at birth and most commonly involve the lower extremities. The vascular lesions have a propensity for hemorrhage. In a study of 214 patients by Husmann et al., 30% had genital cutaneous involvement. Of these patients, 36% developed intractable bleeding. Surgical excision of these vascular malformations was associated with significant blood loss.
REFERENCE
Husmann D, Rathburn SR, Driscoll DJ. Klippel-Trenaunay syndrome: incidence and treatment of genitourinary sequelae. J Urol. 2007;177(4):1244–1249.
KOCK POUCH AND HEMI-KOCK NEOBLADDER
DESCRIPTION A Kock continent catheterizable urinary reservoir (pouch) is created from 70–80 cm of small bowel. The mid 45-cm portion is folded into a U-shaped configuration and opened along its antimesenteric border, and the adjoining edges of the U are sutured together. The resulting U patch is folded again from top to bottom to form a reservoir. The 17-cm end limbs are intussuscepted and stapled to create nipple valves at each end. The ureters are anastomosed in the proximal afferent limb, where the nipple prevents reflux and the efferent limb is used to create a continent stoma, which is catheterized to empty the pouch.
The hemi-Kock neobladder is an orthotopic neobladder constructed based on the theme of the Kock pouch. In this diversion, a single intussuscepted ileal nipple valve is used to create a nonrefluxing ureteroileal anastomosis. The remainder of the pouch is made from a detubularized ileum, which is configured into a pouch and anastomosed to the urethra. It is not currently a recommended form of urinary diversion due to complications related to intussuscepted nipple valves.
REFERENCE
Hautmann RE, Abol-Enein H, Hafez K, et al.; World Health Organization (WHO) Consensus Conference on Bladder Cancer. Urinary diversion. Urology. 2007;69(1 Suppl):17–49.
KOYLE STENT
DESCRIPTION The Koyle diaper stent (Cook Medical Inc., Bloomington, IN, USA) is used for stenting the urethra after hypospadias repair. It has an 8-Fr circumference in the fossa navicularis to minimize distal meatal or urethral stenosis while providing excess tubing externally to allow drainage into a 2nd or outside diaper while keeping the inside diaper dry to allow healing.
REFERENCE
Koyle MA. Hypospadias: A 30 year personal journey. Scand J Surg. 2011;100(4):250–255.
KRUGER STRICT SPERM MORPHOLOGY
DESCRIPTION Some fertility experts use the test to decide between intrauterine insemination (IUI) and in vitro fertilization (IVF) although the test is controversial. This test examines sperm morphology more in-depth than the standard WHO method. Freshly ejaculated sperm are smeared on a slide and stained. Sperm are judged as normal based on the following criteria:
• Head must be oval in shape with smooth contours, 5–6 μm in length and 2.5–3.5 μm wide with the acrosome taking up 40–70% of the head.
• Neck and mid-piece must have no abnormalities and a cytoplasmic droplet (a remnant from sperm production) if present must not be larger than 1/2 the size of the head.
• Tail must not be coiled or bent and should not have a droplet at the end.
After 200 individual sperm are counted at 1,000 X, the percent normal forms are calculated. The IUI prognosis is based on the following scale:
REFERENCE
Ghirelli-Filho M, Mizrahi FE, Pompeo AC, et al. Influence of strict sperm morphology on the results of classic in vitro fertilization. Int Braz J Urol. 2012;38(4):519–528.