ISSN: 2394-8418
Journal of Dental Problems and Solutions
Short Communication       Open Access      Peer-Reviewed

Inferior dental nerve symptomatology in dental practice needs exclusion of sickle cell disease

Felix I D Konotey-Ahulu*

Kwegyir Aggrey Distinguished Professor of Human Genetics, University of Cape Coast, Ghana, and Fellow of Ghana Academy of Arts and Sciences. Former Director of Ghana Institute of Clinical Genetics, Korle Bu Teaching Hospital. Former Consultant Physician, Cromwell Hospital London SW5 0TU & 9 Harley Street, London W1G 9AL
*Corresponding author: Felix I D Konotey-Ahulu, MD, Kwegyir Aggrey Distinguished Professor of Human Genetics, University of Cape Coast, Ghana, and Fellow of Ghana Academy of Arts and Sciences. Former Director of Ghana Institute of Clinical Genetics, Korle Bu Teaching Hospital. Former Consultant Physician, Cromwell Hospital London SW5 0TU & 9 Harley Street, London W1G 9AL, E-mail: felix@sicklecell.md
Received: 09 May, 2022 | Accepted: 03 August, 2022 | Published: 04 August, 2022

Cite this as

Konotey-Ahulu FID (2022) Inferior dental nerve symptomatology in dental practice needs exclusion of sickle cell disease. J Dent Probl Solut 9(2): 017-019. DOI: 10.17352/2394-8418.000111

Copyright License

© 2022 Konotey-Ahulu FID. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Definition

As this is the most important part of the article, considerable detail is given at length

Sickle cell disease is defined as two abnormal beta-globin genes at least one of which is the sickle cell gene denoted by the capital letter S [1]. Other Abnormal beta-globin-genes are denoted either by capital letters of the alphabet C D E G K … [2] or by the name of the place where the abnormal haemoglobin was found, for example, Haemoglobins Korle Bu [3], Osu-Christiansborg [4], K Woolwich [5] all of which are qualitative beta-globin gene abnormalities. There can, however, be a quantitative beta-globin gene abnormality which then is called beta-thalassemia and which I denote as beta-thal.

Therefore, the phenotypes that fit the definition of sickle cell disease as stated above “two abnormal beta-globin genes at least one of which is the sickle cell gene” are SS, SC, SD, SE, SG, SK, SKorle Bu [3], SOsu-Christiansborg [4], SK Woolwich [5], Sbeta-Thalassaemia [6], and so on.

The SS phenotype fits the sickle cell disease definition, but it is the only phenotype that the term Sickle Cell Anaemia is especially devoted to [1,2,5,7].

CAUTION: As Normal beta-globin haemoglobin is denoted with the capital letter A, when this Normal gene A is inherited with any of the Abnormal Haemoglobins the resulting phenotype must never be called a disease, but a Trait. Phenotype “AS” is Sickle Cell Trait, not Sickle Cell Disease [1,2,6-8].

Read this again, please, because the greatest confusion that doctors and the media make today in Haematology is to call Sickle Cell Trait Sickle Cell Disease, and Sickle Cell Disease Sickle Cell Trait [9-11].

Sickle cell crisis: The vital difference is important because it is only Sickle Cell Disease (two abnormal genes) that manifests what is called “sickle cell crisis” when under in vivo conditions like dehydration from diarrhea, fever, infections, sepsis, exercise, swimming, trauma, flying in under-pressurized aircraft, cold rainy weather, shock, poor oxygenation the red cells of a person with a sickle cell disease phenotype turn from round to sickle shape while those with sickle cell trait AS behave exactly as persons without any abnormal haemoglobin AA and do not sickle erythrocytes [1,6]. 

Sickling of red cells in Sickle Cell Traits AS occurs only in vitro when a drop of a reducing agent like 2% sodium metabisulphite solution is added to the blood specimen [12,13]. 

Fraudulent Science has been used to call sickle cell disease people sickle cell traits to confuse phenotypes and that fraud has been used by insurance companies to defraud true sickle cell traits who are entirely healthy. It is dangerous to expose such commercial fraud that was why I was once given 4 bodyguards in Philadelphia [14] at an invited lecture in the presence of Nobel Laureate Linus Pauling who had discovered the molecular pathology of the haemoglobin S gene [15].

While the Media including such important broadcasters as BBC, ITV, and CNN often use the term “sickle cell” to apply to both those with Sickle Cell Trait and Sickle Cell Disease, diligent Dental Surgeons and Doctors must never be heard to say to Nurses assisting them “My next patient is Sickle Cell”. Nor was it correct for the main news item recently in London to announce: “Body of the missing 18-year-old African student with Sickle Cell has been found”. Without phenotype qualification, just to describe a person with the S gene as “Sickle Cell” is not only intolerable but dangerously unfair. 

My Philadelphia Lecture entitled “The Vital Difference between Sickle Cell Disease and Sickle Cell Trait” mentioned how a Black American Sickle Cell Trait man (AS) ran at Olympic Games, Mexico City 7,200 ft above sea level where Oxygen concentration is expected to be thinner than at sea level and managed to beat the whole world [16]. He later wanted to buy a house in New York (sea level) only to be told that his insurance would be 150% the normal rate because he had “Sickle Cell Disease”.

But what has all this got to do with dental practice?

Answer: When any of the above-mentioned circumstances precipitate a sickle cell crisis from in vivo sickled cells blocking tiny vessels supplying tissues, bone, joints, organs like spleen, liver, skin, placenta, eyes, brain, adrenals, spinal cord, and nerves the symptoms and signs in the patient can be numerous and bizarre.

Because I am the Former Director of the Sickle Cell Clinic for the largest number of patients in the world at the Korle Bu Teaching Hospital in Accra I was able to describe usual and unusual signs and symptoms manifesting themselves in more than 1,500 consecutive patients [1]. One very important symptomatology is what I need to draw Dental Practitioners’ attention to.      

Fifty years ago, exactly, I described in Lancet [17] a new physical sign in Clinical Medicine that had fooled not a few brilliant Clinicians even in university teaching hospitals. When sickle cell crisis is severe several bones including the mandible are affected. The inferior dental nerve gives rise to 4 nerves one of which is the mental nerve which during its passage through the mental canal on the medial aspect of the mandible emerges at the mental foramen and becomes infarcted during sickle cell crisis.

The Physical Sign I was first to describe in 1972 was a post-sickle cell crisis persistent burning sensation over the chin, spreading to the inside of the lower lip. The area of paraesthesia shrinks over weeks, and by 3 to 4 months becomes a patch of numbness from what I called “peripheral neuropathy of the mental nerve” resulting from infarction of the nerve during the severe sickle cell crisis – a phenomenon that other clinicians later recognized in 1979, 1980, 1982 and 1984 [18-21].

As some authors just observed it was “first described in Africa” without specifying who first published it where and when, I made sure to codify it in The Lancet as the “Kanumblll Sign” [22]. The Konotey-Ahulu Numb Lower Lip Lancet Sign.

Relevance to dentistry
  1. Do not think this Kanumblll Sign is related to previously given local anesthesia       
  2. As Sickle Cell Disease is found commonly in Greeks, Turks [23], Middle Easters, Asians, and their offspring abroad Sickling Test and quantitative Haemoglobin Electrophoresis need doing to diagnose haemoglobin phenotype not just for Black patients. Note that Sickle Cell beta-Thalassaemia on electrophoresis shows A and S bands with S always greater than A, (written “SA”) while in Sickle Cell Trait the Normal Haemoglobin A is always greater than S (written “AS”) [1 ,24]   
  3. Exclude sickle cell disease phenotype before general anesthesia on the patient.
  4. Patients with Kanumblll Sign will frequently first go to the Dentist for help, without associating it to the sickle cell crisis they have just recovered from.
  5. Identify sickle cell disease patients otherwise accidents could occur in Dental Surgery. Do not use the Sickling Colour Test for diagnosis because it cannot distinguish Sickle Cell Haemoglobin C Disease (SC) from Sickle Cell Trait (AS)! 
  6. When SC is written in Capitals it means Haemoglobins S and C together comprising Sickle Cell Haemoglobin C Disease (two abnormal haemoglobins) and the SC must not be interpreted as S for the sickle, and C for cell [13].
  1. Konotey-Ahulu FID. The Sickle Cell Disease Patient. Natural History from a clinico-epidemiological study of the first 1550 patients of Korle Bu Hospital Sickle Cell Clinic. The Macmillan Press Ltd, London and Basingstoke, 1991 and reprinted 1992. Reprinted 1996 by Tetteh-A’Domeno Company, Watford WD1 7NF
  2. Lehmann H, Kynoch PAM. Human Haemoglobin variants and their characteristics. North Holland Publishing Company. Amsterdam. 1976
  3. Konotey-Ahulu FI, Gallo E, Lehmann H, Ringelhann B. Haemoglobin Korle-Bu (beta 73 aspartic acid replaced by asparagine) showing one of the two amino acid substitutions of haemoglobin C Harlem. J Med Genet. 1968 Jun;5(2):107-11. doi: 10.1136/jmg.5.2.107. PMID: 5722880; PMCID: PMC1468514.
  4. Konotey-Ahulu FI, Kinderlerer JL, Lehmann H, Ringelhann B. Haemoglobin Osu-Christiansborg: a new beta-chain variant of haemoglobin A ( beta52 (D3) aspartic acid leads to asparagine) in combination with haemoglobin S. J Med Genet. 1971 Sep;8(3):302-5. doi: 10.1136/jmg.8.3.302. PMID: 5097135; PMCID: PMC1469179.
  5. Ringelhann B, Konotey-Ahulu FI, Talapatra NC, Nkrumah FK, Wiltshire BG, Lehmann H. Haemoglobin K Woolwich (alpha 2, beta 2 132 lysine leads to glutamine) in Ghana. Acta Haematol. 1971;45(4):250-8. doi: 10.1159/000208632. PMID: 4999133.
  6. Boyo AE, Cabannes R, Conley CL, Lehmann H, Luzzatto L, Milner PF, Ringelhann B, Weatherall DJ, Barrai I, Konotey-Ahulu FID and Motulsky AG. Geneva WHO Scientific Group on Treatment of Haemoglobinopathies and Allied Disorders. (Technical Report) 1972; 509:83.
  7. Serjeant GR. The clinical features in adults sickle cell anaemia in Jamaica. West Indian Med J. 1970 Mar;19(1):1-8. PMID: 5502002.
  8. Konotey-Ahulu FI. The sickle cell diseases. Clinical manifestations including the "sickle crisis". Arch Intern Med. 1974 Apr;133(4):611-9. PMID: 4818434.
  9. Konotey-Ahulu FID. Sickle Cell Trait Misinformation and Disinformation 2011. www.sicklecell.md/blog/?p=108 
  10. Konotey-Ahulu FID. Further Communication on "Sickle Cell Trait Misinformation and Disinformation” and Sickle Cell Terminology: Disease or Disorder?  2012; www.sicklecell.md/blog/?p=127 
  11. Konotey-Ahulu FID. Sickle Cell Trait Confusion: Is It Deliberate? Or Is This Ignorance? http://blog.sicklecell.md/sicklecell/sickle-cell-trait-confusion-is-it-deliberate-or-is-this-ignorance/
  12. DALAND GA, CASTLE WB. A simple and rapid method for demonstrating sickling of the red blood cells; the use of reducing agents. J Lab Clin Med. 1948 Sep;33(9):1082-8. PMID: 18880907.
  13. Konotey-Ahulu FI. Detecting sickle haemoglobin. Br Med J. 1972 Oct 28;4(5834):239. doi: 10.1136/bmj.4.5834.239. PMID: 5082577; PMCID: PMC1786510.
  14. Konotey-Ahulu FID.  Four bodyguards and the perils of unmasking scientific truths. BMJ 2007; 335:210-211 http://blog.konotey-ahulu.com/blog/_archives/2007/8/8/3146254.html http://www.bmj.com/cgi/content/full/335/7612/210 | http://www.bmj.com/cgi/reprint/335/7612/210.pdf
  15. PAULING L, ITANO HA, et al. Sickle cell anemia a molecular disease. Science. 1949 Nov 25;110(2865):543-8. doi: 10.1126/science.110.2865.543. PMID: 15395398.
  16. Lehmann H. Sickle Cell Trait and flying. The Times. London. 1972.
  17. Konotey-Ahulu FID. Mental nerve neuropathy: a complication of sickle cell crisis. Lancet. 1972; 2(7773):388 [Constitutes discovery of a new physical sign in Clinical Medicine] http://www.bmj.com/cgi/reprint/1/5793/177-a.pdf
  18. Kirson LE, Tomaro AJ. Mental nerve paresthesia secondary to sickle-cell crisis. Oral Surg Oral Med Oral Pathol. 1979 Dec;48(6):509-12. doi: 10.1016/0030-4220(79)90295-0. PMID: 292954.
  19. Friedlander AH, Genser L, Swerdloff M. Mental nerve neuropathy: a complication of sickle-cell crisis. Oral Surg Oral Med Oral Pathol. 1980;49(1):15-7. doi: 10.1016/0030-4220(80)90025-0. PMID: 6243181.
  20. Seeler RA, Royal JE. Mental nerve neuropathy in a child with sickle cell anemia. Am J Pediatr Hematol Oncol. 1982 Summer;4(2):212-3. PMID: 7114403.
  21. Hammersley N. Mandibular infarction occurring during a sickle cell crisis. Br J Oral Maxillofac Surg. 1984 Apr;22(2):103-14. doi: 10.1016/0266-4356(84)90022-6. PMID: 6231948.
  22. Konotey-Ahulu FI. Sickle-cell disease and the patient. Lancet. 2005 Jan 29-Feb 4;365(9457):382-3. doi: 10.1016/S0140-6736(05)17818-0. PMID: 15680445.
  23. Ringelhann B, Konotey-Ahulu FID. Hemoglobinopathies and thalassemias in Mediterranean areas and in West Africa: Historical and other perspectives 1910 to 1997 - A Century Review. Atti dell’Accademia dell Science di Ferrara ( Milan) 1998;74: 267-307
  24. Konotey-Ahulu FI. Sickle-cell trait and altitude. Br Med J. 1972 Jan 15;1(5793):177-8. doi: 10.1136/bmj.1.5793.177-a. PMID: 5007853; PMCID: PMC1787128.
 

Order for reprints


Article Alerts

Subscribe to our articles alerts and stay tuned.


Creative Commons License This work is licensed under a Creative Commons Attribution 4.0 International License.