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Acquired Kinky Hair Syndrome

Pili Multigemini


Presentation

  • Uncombable hair syndrome Pili annulati Monilethrix Loose anagen hair syndrome Menkes kinky hair syndrome Case 4 27 year old white male presents for full body skin exam.[dermcast.tv]
  • Congenital TN may be present at birth, appear within the first year of life, or present as a symptom of an underlying metabolic disorder like argininosuccinic aciduria, Menkes’ kinky hair syndrome, Netherton syndrome, and Laron syndrome at the age of[capillaroscopia.it]
  • Woolly hair may be an inherited as an isolated condition ( OMIM ID #194300 ) or present as part of a syndrome.[dermnetnz.org]
  • Patients presenting with woolly hair must be examined completely to rule out cardiofaciocutaneous and Noonan syndrome. We present a 5-year-old with Type A woolly hair nevus.[ijpd.in]
  • Therefore, to distinguish between the entities it is important to take account of the clinical presentation and also examine a blood film for the presence of abnormal neutrophil granulation, which is present in Chediak-Higashi but not Griscelli syndrome[jcp.bmj.com]
Pseudotumor
  • […] of the gastrointestinal tract in infancy and childhood 508 Benign pneumatosis 509 Reduced intestinal gas 510 Increased distance between the intestinal loops 511 Intraabdominal cysts in children 512 Migratory peritoneal calcifications 514 Peritoneal pseudotumors[books.google.com]
Congestive Heart Failure
  • heart failure 438 Pulmonary edema or congestion with normal or slightly enlarged heart 439 Cor pulmonale General overview 440 Azygos or hemiazygos vein enlargement 441 Asplenia syndrome 442 Systemic hypertension in childhood General overwview 443 Surgical[books.google.com]
Pneumonia
  • […] pattern in children 316 Acute alveolar disease 317 Lobar pneumonia in children 318 Homogeneous lobar or segmental opacification 319 Round pneumonia 320 Pulmonary round lesions in a pediatric leukemic patient 321 Aspiration pneumonia 322 Recurrent chronic[books.google.com]
  • In December 2005, he had pneumonia of the left lung, which was treated by his family physician.[yumpu.com]
Respiratory Distress
  • distress in the newborn 272 Lung disease in the newborn 275 Wetlung disease 276 The airbronchogram pattern in the newborn 277 White lung appearance in the newborn 278 Pulmonary hemorrhage A complication of 279 Bilateral homogenous opacification in neonates[books.google.com]
Aspiration
  • […] lungs in the pediatric age 315 Pneumonia pattern in children 316 Acute alveolar disease 317 Lobar pneumonia in children 318 Homogeneous lobar or segmental opacification 319 Round pneumonia 320 Pulmonary round lesions in a pediatric leukemic patient 321 Aspiration[books.google.com]
Stridor
  • […] density along the inner wall of the upper chest in the newborn 290 The respiratory system of the older infant chilld and youngster 28 Upper airways narrowing or obstruction in infancy and children 291 Retropharyngeal softtissue thickening in children 292 Stridor[books.google.com]
Pleural Effusion
  • effusion 350 Pleural effusion with or without pulmonary pathology 351 Parietal pleural thickening without pleural fluid 352 Localized pleural thickening in children 353 The Mediastinum and the Diaphragm Mediastinum 1 Pneumomediastinum in infancy and[books.google.com]
Hypertension
  • […] pattern 434 Pulmonary veins engorgement 435 Congestive heart failure 438 Pulmonary edema or congestion with normal or slightly enlarged heart 439 Cor pulmonale General overview 440 Azygos or hemiazygos vein enlargement 441 Asplenia syndrome 442 Systemic hypertension[books.google.com]
  • 小川愛子:肺高血圧症における血管リモデリング.Pulmonary Hypertension Update,1(2)244-247,2015 22. 松原広己:慢性血栓塞栓性肺高血圧症.Pulmonary Hypertension Update,1(2)222-227,2015 23.[okayamamc.jp]
Heart Disease
  • disease 444 Indentation on the esophageal wall 466 Inadequate peristalsis of the esophagus or neuromuscular deficit 467 Gastroesophageal reflux in the infantile period 468 Esophagitis 469 Dilated or wide esophagus 471 Intramural pseudodiverticulum of[books.google.com]
Heart Failure
  • failure in infancy and childhood Various entities B 331 Unilateral pulmonary edema 332 Pulmonary hemorrhage in the older infant 333 Honeycomb pattern of the lungs As a late phase of the lung disease 334 Bronchiectasis 335 Pneumatocele 336 A cystlike[books.google.com]
Hepatomegaly
  • […] in the newborn period 516 Hepatomegaly in children General overview 517 Hepatomegaly with portal hypertention and obstructive jaundice 519 Liver calcifications in the pediatric age 520 حقوق النشر[books.google.com]
Alopecia
  • In some patients, APKH developed into androgenic alopecia. There is a decreased anagen to telogen ratio.[hairstylessolutions.blogspot.com]
  • He is currently author of 180 peer-reviewed scientific publications and author or editor of a number of textbooks on hair, including the Springer books Male Alopecia: Guide to Successful Management (2014), Female Alopecia (2013) Aging Hair (2010), and[books.google.com]
  • […] and alopecia Figure 10.68.[5minuteconsult.com]
  • Alopecia --- An Overview Seymour M. Weaver, III, M.D. Board Certified Dermatologist Alopecia is the medical term to describe the loss of hair from the head or body.[blackhairmedicalexpert.com]
  • Continued Traction alopecia and trichotillomania In terms of the mechanical action behind hair loss, traction alopecia and trichotillomania are exactly the same. The hair is plucked out of the skin leaving clear bald patches or diffuse, thin hair.[webmd.com]
Polyneuropathy
  • Reviewing the full spectrum of clinically significant neuropathies, the book contains chapterson common and rare forms including mononeuropathy in the upper and lower extremities, mononeuritismultiplex, diffuse and symmetric polyneuropathies, brachial[books.google.com]
  • Polyneuropathy, Organomegaly, Endocrinopathy, M Protein, andSkin Changes: POEMS Syndrome ( James J. Nordlund ). Urticaria Pigmentosum and Mastocytosis ( James J.NordlundI ). Poikiloderma of Civatte ( Vlada Groysman & NormanLevine ).[amazon.it]
Mononeuropathy
  • Reviewing the full spectrum of clinically significant neuropathies, the book contains chapterson common and rare forms including mononeuropathy in the upper and lower extremities, mononeuritismultiplex, diffuse and symmetric polyneuropathies, brachial[books.google.com]

Workup

Bowel Distention
  • distention 484 Malabsorption patterns of the small bowel 486 Fixed stiff small bowel loops 487 Destruction of the small bowel mucosa 488 Deformation of the terminal ileum 489 Lesions mimikig regional enteritis 490 Abnormalities of the ileocecal valve[books.google.com]
Atelectasis
  • […] chest in the newborn 283 Unilateral hyperlucent thorax in the newborn 284 Coarse parenchymal markings with irregular bilateral changes and hyperinflation in the newborn 285 Cystlike structures in the lung parenchyma of the newborn 286 Neonatal platlet atelectasis[books.google.com]
Hyperlucent Lungs
  • lung 313 Bilateral underaeration of the lungs in the pediatric age 315 Pneumonia pattern in children 316 Acute alveolar disease 317 Lobar pneumonia in children 318 Homogeneous lobar or segmental opacification 319 Round pneumonia 320 Pulmonary round lesions[books.google.com]
Colonic Stricture
  • stricture or narrowing 496 Segmental colonic narrowing in children 497 Toxic megacolon 498 Distention of the small andor large bowelnonobstructive paralytic ileus 499 Distended large bowel 500 Large bowel obstruction 502 Large retrorectal space 503 Large[books.google.com]
Colitis
  • […] mucosa 488 Deformation of the terminal ileum 489 Lesions mimikig regional enteritis 490 Abnormalities of the ileocecal valve 491 Large bowel 73 Cecal filling defects or deformed cecum 492 Typhlitis 493 Filling defects in the colon Single or multiple 494 Colitis[books.google.com]
Pleural Effusion
  • effusion 350 Pleural effusion with or without pulmonary pathology 351 Parietal pleural thickening without pleural fluid 352 Localized pleural thickening in children 353 The Mediastinum and the Diaphragm Mediastinum 1 Pneumomediastinum in infancy and[books.google.com]

Treatment

  • Keeps you up-to-date with the latest hot-topic developments including new biologic agents used in the treatment of various diseases such as psoriasis, eczema and melanoma.[books.google.com]
  • In those patients the syndrome eventually progressed to severe AGA that did not respond to treatment.[alopeciaworld.com]
  • Both drugs have shown to produce kinking of hair in some people, which may reverse itself with the cessation of the drug treatment.[hairstylessolutions.blogspot.com]
  • The description of this new mutation and the response of the patient to Cu-His treatment will contribute to the growing body of knowledge about treatment response in Menkes disease.[bmcpediatr.biomedcentral.com]

Prognosis

  • In typical GS, hypogammaglobulinemia and B-cell depletion are observed. 1–2) Preventing infection improves the prognosis of GS and, to date, cyclical gamma globulin replacement therapy is considered necessary.[yumpu.com]
  • Trichorrhexis Nodosa Prognosis This non-contagious hair problem can be successfully cured within 4 years, if mechanical and chemical damages do not occur again.[medicalsubstance.com]
  • Tinea capitis, alopecia areata, and traction alopecia Hair will regrow, may take months There is a poorer prognosis with alopecia universalis. Telogen ef... Alkhalifah A, Alsantali A, Wang E, et al. Alopecia areata update: part II. Treatment.[5minuteconsult.com]
  • The prognosis in Menkes disease is associated with early detection, early initiation of treatment and with the preservation of some ATP7A activity, which is necessary for Cu-His treatment response.[bmcpediatr.biomedcentral.com]
  • Hori YS, Fukuhara T, Aoi M, Namba Y : Eosinopenia in Children following Traumatic Intracranial Hemorrhage Is Associated with Poor Prognosis and Prolonged Hospital Admission. Pediatric Neurosurgery, 51(2)57-60, 2016 3.[okayamamc.jp]

Etiology

  • […] interstitial and granulonodulo alveolar pattern 324 Interstitialreticular pulmonary pattern 326 Disseminated interstitial pneumonitis 327 The miliary pattern of pneumonia 328 Short linear andor wedgeshaped densities 329 Pulmonary edema in infancy and childhood Etiology[books.google.com]
  • […] be an isolated finding or may present as part of a syndrome, such as Menkes kinky hair syndrome, trichothiodystrophy, or argininosuccinic aciduria. 20-22 Acquired TN, resulting from repeated trauma to the hair shaft, is much more common. 17 Specific etiologies[jddonline.com]

Epidemiology

  • ABSTRACT:Cortexolone 17α-propionate (clascoterone) is a novel topical androgen antag... more Skin Cancer Epidemiology and Sun Protection Behaviors Among Native American....[jddonline.com]
  • Zhang W: Epidemiological and aetiological studies on hair casts. Clin Exp Dermatol 1995;20:202–207. Gummer CL: Bubble hair: A cosmetic abnormality caused by brief, focal heating of damp hair fibres. Br J Dermatol 1994;131:901–903.[karger.com]
Sex distribution
Age distribution

Pathophysiology

  • Part II: The Pathophysiology of Pigmentary Disorders. Section 3: An Overview of Human Skin Color and itsDisorders. 26 A More Precise Lexicon for Pigmentation, PigmentaryDisorders, and "Chromatic" Abnormalities ( James J.[amazon.it]
  • The pathophysiological mechanism resulting in this morphological abnormality has been examined using electron microscopic and histochemical methods, and is thought to be a consequence of “softening” of the hair cortex, possibly secondary to abnormal composition[jcp.bmj.com]

Prevention

  • All cases eventually evolved into androgenetic alopecia within a two to nine-year period, and 5% Minoxidil was ineffective in preventing the eventual hair loss. The effects of finesterinde (Propecia) have not been recorded in cases of APKH.[hairstylessolutions.blogspot.com]
  • The question of prevention is of particular importance, since hereditary deafness has not been and is not likely to be amenable to any kind of therapy.[jamanetwork.com]
  • Genetic mechanisms control the incorporation of copper into apoproteins and the processes that prevent toxic accumulation of copper in the body. Copper absorbed in excess of metabolic requirements is excreted through bile.[merckmanuals.com]
  • In typical GS, hypogammaglobulinemia and B-cell depletion are observed. 1–2) Preventing infection improves the prognosis of GS and, to date, cyclical gamma globulin replacement therapy is considered necessary.[yumpu.com]
  • Treatment is prevention of repetitive physical and chemical insults to the hair. [1], [6] References 1. Martin AM, Sugathan P.[ijdpdd.com]

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