What Medicines Should Be Avoided with G6PD Deficiency?

Patient Presentation
A 3-day-old male came to clinic for a weight and bilirubin checkup. He was feeding well every 2-3 hours for 30 minutes. He was having several stools but they still had meconium. He would awaken but seemed sleeper for the past 24 hours per his mother, and she thought he looked more jaundiced. The past medical history showed that he was a 38 week gestation, appropriate-for-gestation-age, infant born vaginally to a G2P2 O+ female with no risk factors. He had an uneventful post-natal course and was discharged at 48 hours with a total bilirubin of 11.8 mg/dL. His neonatal screening test was normal. The family history was non-contributory other than both parents were originally from Italy and were now living in the United States. The review of systems was negative.

The pertinent physical exam showed a sleepy-appearing male. His weight was 5% down from birth weight and was 2% down since discharge. He was jaundiced to at least his knees but otherwise appeared normal. His transcutaneous bilirubin was 17.2 mg/dL, and a total serum bilirubin was 17.9 mg/dL (high risk). The diagnosis of a healthy appear male with jaundice was made. The patient was admitted to the hospital for phototherapy. During the admission process the mother said that there were people in her family that had G6PD deficiency, but that she did not. The patient’s sibling had jaundice as an infant treated with phototherapy but the parents were not aware that any additional evaluation had been performed. Further laboratory testing showed the patient’s blood smear to have increased hemolysis and an infant blood type of O+ with a negative direct antibody test. His G6PD biochemical assay was positive for the disease. The infant continued under phototherapy for 36 hours. His rebound bilirubin after phototherapy at 5 days of life was 12 mg/dl (low risk). He was seen in the clinic the following day and another bilirubin was 10.8 mg/dL. The family had already been given information about G6PD and agreed to genetic counseling so an appointment had been made.

Discussion
Glucose 6-phosphate dehydrogenase deficiency (G6PD) is an X-linked disease that has multiple mutations. Each mutation causes a different amount of the enzyme to be produced within cells and therefore not all mutations will produce disease. Glucose 6-phosphate dehydrogenase is an enzyme that is critical to the metabolism of all aerobic cells as it catalyzes the rate-limiting step of the pentose phosphate pathway reducing NADP to NADPH. NADPH is important for nucleic acid replication and therefore cell division. G6PD is the only source of NADPH within the cell, so deficiency makes red blood cells susceptible to hemolysis if they are under oxidative stress. Populations at higher risk for G6PD include those who their ethnic heritage is from the Mediterranean, Africa, Middle East and Asia. Newborn screening testing is not routinely done in the United States. Infants are often identified because of the early timing, persistent or extreme levels of hyperbilirubinemia.

Although the levels of hyperbilirubinemia in this patient were not extreme (i.e. > 20 mg/dL), G6PD is a common reason for extreme hyperbilirubinemia. Causes of severe unconjungated hyperbilirubinemia specifically due to problems with red blood cells can be found here. A review of general causes of neonatal unconjugated hyperbilirubinemia can be found here.

Learning Point
Hemolytic episodes in persons with G6PD can be triggered by infections, severe stress, certain foods (such as fava beans), and certain drugs, including:

  • Antibiotics – chloramphenicol, cotrimoxazole, nitrofurantoin, quinolones, sulfadiazine
  • Antimalarial – dapsone, primaquine, methylene blue, chloroquine, quinine
  • Antipyretics – acetaminophen, aspirin, phenazopyridine, sulfasalazine
  • Other – Isoniazid, Vitamin C, Vitamin K, rasburicase, thiazide diuretics
  • Cardiac – dinitrate, isosorbide
  • Hypoglycemics – tolbutamide, glibenclamide

Favism by proxy, that is exposure to fava (broad) beans through breast milk, can also occur.

For health care providers who do not routinely treat patients with this problem, a consultation with a pharmacist or a geneticist may be necessary, particularly if there is a medical need to use a particular drug such as treatment of malaria.

Questions for Further Discussion
1. How common is G6PD worldwide?
2. What would be the pros and cons of universal neonatal screening for G6PD?

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for this topic: G6PD Deficiency

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Christensen RD, Lambert DK, Henry E, Eggert LD, Yaish HM, Reading NS, Prchal JT. Unexplained extreme hyperbilirubinemia among neonates in a multihospital healthcare system.
Blood Cells Mol Dis. 2013 Feb;50(2):105-9.

Watchko JF, Kaplan M, Stark AR, Stevenson DK, Bhutani VK. Should we screen newborns for glucose-6-phosphate dehydrogenase deficiency in the United States? J Perinatol. 2013 Jul;33(7):499-504.

Luzzatto L, Seneca E. G6PD deficiency: a classic example of pharmacogenetics with on-going clinical implications. Br J Haematol. 2014 Feb;164(4):469-80.


ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    5. Patients and their families are counseled and educated.
    7. All medical and invasive procedures considered essential for the area of practice are competently performed.
    8. Health care services aimed at preventing health problems or maintaining health are provided.
    9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.

  • Systems Based Practice
    23. Differing types of medical practice and delivery systems including methods of controlling health care costs and allocating resources are known.
    24. Cost-effective health care and resource allocation that does not compromise quality of care is practiced.
    25. Quality patient care and assisting patients in dealing with system complexities is advocated.

    Author

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

  • What is Gianotti-Crosti Syndrome?

    Patient Presentation
    A 4-year-old male came to clinic with a new rash and a low grade fever that had begun the night before. The mother said that the child had a recent sore throat but had been doing well until the last night when she noticed some papules on his upper legs. In the morning the rash had spread to his thighs and knees, buttocks and upper arms including the elbows. He had none on his face or trunk. The spots were becoming more extensive and were now red whereas the night before they were more flesh-colored or slightly pink. She denied new soaps, lotions, detergents or other similar products. He had not and was not taking any medication. There were several children in his daycare with colds. The past medical history was non-contributory.

    The pertinent physical exam showed a well-appearing male with normal vital signs including being afebrile and growth parameters in the 5-10%. He had a mildly erythematous throat, and some shotty cervical and inguinal adenopathy. His skin exam showed multiple pink to red papules that were clustered around the knees and elbows but also were scattered on his upper thighs, upper arms and buttocks. He had none on his face or trunk. There were no excoriations and the papules did not seem to have a central indentation. The physician wasn’t sure what the diagnosis was, so he got a rapid strep test and then found one of his partners. She believed that the diagnosis was Gianotti-Crosti. “Gianotti-Crosti has elements of many things so I always tuck it into the back of my brain when the rash looks different. The keys for me are nothing on the trunk, its usually not pruritic but it can be sometimes, and that the patient looks well. It looks like one of those rashes that you’re pretty sure is benign, but it still bothers you. It’s a viral exantham so you can just watch it.” The patient was discharged with instructions to monitor the patient and in followup the patient had resolution of the rash.

    Discussion
    Infectious exanthams are usually considered when rashes are bilateral, symmetric and relatively widespread. They usually involve the trunk too and have associated systemic symptoms. Gianotti-Crosti syndrome (GCS) or acropapular dermatitis of childhood is often misdiagnosed because it doesn’t follow these rules. A discussion of common viral exanthams can be reviewed here and a differential diagnosis of rashes by pattern and distributions can be reviewed here.

    Dr. Ferdinando Gianotti came from a poor family, underwent several personal tragedies, but entered medicine and created the first department of pediatric dermatology in Italy (possibly in Europe). He became interested in a child with papular eruption that he could not classify and after seeing several other cases over the next few months he published his first case series in 1955. Dr. Agostino Crosti was the head of the Department of Dermatology and together they published another article in 1956.

    Learning Point
    Gianotti-Crosti Syndrome (GCS) usually occurs in children 6 months – 12 years of age. Clusters of cases occur because of various viral and bacterial etiologies.

    It is an acute papular eruption that usually begins on the buttocks and thighs, then the outer arms and later the face. The trunk is notably spared (a key sign). The rash is also asymmetric. The papules are 5-10 mm in size and may occur singly or in clusters and be quite extensive. The papules can be flesh-colored, light to deep red or purpulish in color also. The papules can blister. Generally they are non-pruritic and resolve over several weeks (2-8) and there can be some scaling during this time.

    The rash can mimic other causes such as molluscum contagiosum (especially in single or few lesions that are flesh-colored), a drug rash or even purpura.

    There have been numerous etiologies linked with GCS especially Hepatitis B in Europe and Epstein-Barr virus in the US. Other viruses include Adenovirus, Coxsackie, Cytomegalovirus, Echovirus, Hepatitis A and C, Human Herpes Virus 6, HIV, Influenza A, Influenza H1N1, Mumps, Parvovirus B19, Respiratory Syncytial Virus and Rotavirus. Post vaccination eruption has also been documented with Hepatitis B, Influenza, Japanese B Encephalitis, MMR and Polio vaccines.

    No specific treatment is necessary. Antihistamines can be used for pruritis. If there is jaundice or hepatomegaly, testing for Hepatitis B can be considered, but increased liver function tests are usually more common with Epstein-Barr virus.

    Questions for Further Discussion
    1. GCS is considered an atypical viral exantham. What other viral exanthams are considered atypical and why?
    2. What are indications for consultation with a dermatologist?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

    Information prescriptions for patients can be found at MedlinePlus for these topics: Skin Conditions and Rashes.

    To view current news articles on this topic check Google News.

    To view images related to this topic check Google Images.

    To view videos related to this topic check YouTube Videos.

    May J, Pollack R. Giannoti-Crosti syndrome associated with type A influenza. Pediatr Dermatol. 2011 Nov-Dec;28(6):733-5.

    Kroeskop A, Lewis AB, Barril FA, Baribault KE. Gianotti-Crosti syndrome after H1N1-influenza vaccine. Pediatr Dermatol. 2011 Sep-Oct;28(5):595-6.

    Biesbroeck L, Sidbury R. Viral exanthems: an update. Dermatol Ther. 2013 Nov-Dec;26(6):433-8.

    Gianotti F, Pesapane F, Gianotti R. Ferdinando Gianotti and the papular acrodermatitis of childhood: a scientist against all the odds. JAMA Dermatol. 2014 May 1;150(5):485.

    DermNet NZ. Available from the Internet at http://dermnetnz.org/viral/gianotti-crosti.html (rev. 12/29/2013, cited 6/24/14).

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    8. Health care services aimed at preventing health problems or maintaining health are provided.
    9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Interpersonal and Communication Skills
    19. The health professional works effectively with others as a member or leader of a health care team or other professional group.

  • Systems Based Practice
    26. Partnering with health care managers and health care providers to assess, coordinate, and improve health care and how these activities can affect system performance are known.

    Author

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

  • What Causes Testicular Pain?

    Patient Presentation
    A 17-year-old male came to clinic with a 3 week history of what he described as intermittent left testicular pain. It occurred only during the day and would occur randomly, lasting for a few seconds to a few minutes. The pain occurred mainly in the lower abdomen but would come into the upper scrotal area. He described it as a pressure-type pain not a stabbing/cutting pain. He said there was no inguinal masses. He denied sexual activity, masturbation, trauma, or excessive exertion. He denied any inguinal swelling, swelling or erythema of the scrotum, testes or penis and no penile discharge. He was an athlete who wore compression clothing but not a protective cup or athletic supporter. The review of systems showed no fever, chills, nausea, emesis, constipation, diarrhea or dysuria.

    The pertinent physical exam showed normal vital signs and growth parameters. His genitourinary examination showed Tanner V pubertal changes with no skin changes, erythema or edema. There was no specific swelling in the inguinal area. His left testicle was slightly higher than the right and was oriented longitudinally. There was no tenderness of the testicle, the epididymis or the anterior or superior area of the testicle. The spermatic cord had no “bag of worms.” Palpation of the area of the inguinal ring re-created the pain. No specific “silk-bag” sign or bulging could be appreciated but the ring seemed slightly larger/looser on the left than on the right. There was a normal cremesteric reflex on both sides. The pallus was normal without discharge. The diagnosis of a probable indirect inguinal hernia with referred intermittent pain was made.

    The work-up included a normal urinalysis and screening for sexually transmitted infections that were normal. The patient was referred to a general surgeon and given instructions on when to seek care sooner if the pain intensified or changed.

    Discussion

    Most inguinal hernias are indirect (i.e. the hernia passes through the internal inguinal ring and down the inguinal canal); only 2% of all hernias in children are direct hernias (i.e. the hernia directly protrudes through the floor of the inguinal canal). Indirect inguinal hernias occur in about 1-5% of infants. They occur on the right side (60%), left side (30%) and bilaterally (10%) and they are more common in premature infants of both sexes. The male : female ratio of inguinal hernias is 4-8 : 1.

    The chief complaint of testicular or scrotal pain always raises concerns. The acute scrotum generally has pain, edema, erythema and warmth. Although acute scrotum is considered a surgical emergency because of the need to treat testicular torsion promptly, most cases are nonemergent. The most common causes of acute scrotum are torsion of the appendix testis or epididymitis depending on the study. One 18 year retrospective of surgically confirmed cases found 9.8% of all urological surgical admissions were for acute scrotum. Of these, 74.7% were for torsion of the appendix testis, 8.4% were for testicular torsion, 5.8% were for tunica vaginalis inflammation, 3.7% were for epididymitis, and 7.2% were for other causes.

    Prompt referral to urology for evaluation and treatment of an acute scrotum should be made. Ultrasound evaluation can be helpful to aid with diagnosis particularly determining how much blood flow there is to the testes. Other ultrasonic findings can include edema of the epididymis (for epididymitis),

    The differential diagnosis of abdominal pain can be found here.
    The differential diagnosis of scrotal swelling can be found here.

    Learning Point
    The differential diagnosis of testicular/scrotal pain includes:

    • Testes
      • Testicular torsion – usually has unilateral severe scrotal pain and may be associated with nausea, emesis, elevation of testes, absence of cremesteric reflex, horizontal positioning of testes, and scrotal edema. Occurs most often in neonates and adolescent boys.
      • Epididymitis – usually has an insidious onset, painful epididymis, and normal placement of testes and cremesteric reflex. It may also be associated with scrotal edema or have dysuria. Common pathogens in younger males are adenovirus, enterovirus and Mycoplasma. In adolescents, Neisseria gonorrhea and Chlamydia trachomatis may occur. E. coli is also common if there are associated anatomic abnormalities.
      • Torsion of the appendix testis – occurs often in school age boys, and has painful superior area point tenderness. There may be a blue-dot sign.
      • Torsion of embryonic remnant of testis or epididymis
      • Tunica vaginalis swelling
      • Trauma
      • Orchitis – classically associated with mumps virus but also associated with sexually transmitted diseases or HIV.
      • Fournier gangrene
      • Testicular infarction
      • Testicular tumor – usually painless
      • Hydrocoele – usually painless
      • Varicoele – usually painless
    • Referred pain
      • Appendicitis
      • Hernia
      • Irritation of innervation of the genitourinary structures – ilioinguinal, iliohypogastric, genitofemoral, pudendal
      • Urolithiasis
    • Other
      • Henoch Schonlein purpura
      • Skin problems – rash, insect bites

    Questions for Further Discussion
    1. What testing should be considered for an acute scrotum?
    2. What is the time period over which a testicle may become non-viable if there is testicular torsion?
    3. What is the treatment for epididymitis?

    Related Cases

      Symptom/Presentation: Pain

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

    Information prescriptions for patients can be found at MedlinePlus for this topic: Testicular Disorders.

    To view current news articles on this topic check Google News.

    To view images related to this topic check Google Images.

    To view videos related to this topic check YouTube Videos.

    Yang C Jr, Song B, Liu X, Wei GH, Lin T, He DW. Acute scrotum in children: an 18-year retrospective study. Pediatr Emerg Care. 2011 Apr;27(4):270-4.

    Boettcher M, Bergholz R, Krebs TF, Wenke K, Treszl A, Aronson DC, Reinshagen K. Differentiation of epididymitis and appendix testis torsion by clinical and ultrasound signs in children. Urology. 2013 Oct;82(4):899-904.

    Munden MM, Williams JL, Zhang W, Crowe JE, Munden RF, Cisek LJ. Intermittent testicular torsion in the pediatric patient: sonographic indicators of a difficult diagnosis. Am J Roentgenol. 2013 Oct;201(4):912-8.

    Van Heurn LW, Pakarinen MP, Wester T. Contemporary management of abdominal surgical emergencies in infants and children. Br J Surg. 2014 Jan;101(1):e24-33.

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    5. Patients and their families are counseled and educated.
    7. All medical and invasive procedures considered essential for the area of practice are competently performed.
    8. Health care services aimed at preventing health problems or maintaining health are provided.
    9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.

  • Systems Based Practice
    24. Cost-effective health care and resource allocation that does not compromise quality of care is practiced.

    Author

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

  • How Common Is Developmental Dysplasia of the Hip in Infants With Breech Presentation?

    Patient Presentation
    A 1-week-old female came to clinic for her health supervision visit. Breastfeeding was going well and mild clinical jaundice had resolved. The past medical history showed a full-term female with no prenatal risk factors who had been born by Cesarean section for breech presentation. The family history was negative for orthopaedic issues.

    The pertinent physical exam showed an alert infant. Her weight was 3.26 kg (50%), length of 48% (50%) and head circumference of 35 cm (90%). Her examination was notable for erythema toxicum neonatorum on her cheeks and a normal hip examination. The diagnosis of a healthy female was made and routine parent education was given. The physician did schedule a screening hip ultrasound because of the breech delivery. The patient’s clinical course at 1 month found her to be in good health with growth parameters consistent with birth. The radiologic evaluation of a hip ultrasound showed developmental dysplasia of the left hip. The patient was sent to Orthopaedics who placed her in a Pavlik harness. At her 2 month check up she was doing well with only minimal intermittent skin irritation from the harness.

    Case Image

    Figure 115 – Coronal views from a hip ultrasound exam show a normal right hip (above) with the right femoral head well centered in the right acetabulum and a dysplastic left hip (below) with the left femoral head displaced laterally out of the shallow left acetabulum.

    Discussion
    Developmental dysplasia of the hip (DDH) includes several abnormalities where the femoral head and the acetabulum are not aligned normally or grow abnormally. This includes hips that are dislocatable, dislocated, dysplastic and subluxed. The natural history and pathophysiology are poorly understood.

    Screening can lead to earlier identification, but there are high rates of spontaneous resolution in the newborn period that require no intervention. Resolution rates are 60-80% if identified by physical examination and 90% resolve if identified by ultrasound examination in studies. Physical examination screening includes the Barlow and Ortolani examinations. Barlow examination is the adducting of a flexed hip with posterior force to identify a dislocatable hip. Ortolani examination is the abducting of a flexed hip with anterior force to relocate a dislocated hip. Some people remember Barlow is “back” (gentle force to push the femoral head backward) and Ortolani is “out” (the femoral head is out of place and is realigned with the gentle anterior force). These maneuvers can be difficult to perform in a manner that provides consistent accuracy. A review of other physical examination signs can be found here.

    Ultrasound examination is another screening and diagnostic technique. In some studies ultrasound has a high false-positive rates which could lead to unnecessary treatment and followup. It is felt that this is due to operator dependence. In other studies ultrasound has high rates of intraobserver and inter-observer reliability. Screening recommendations differ by different professional groups and can be found at the National Guideline Clearinghouse link in the To Learn More section below.

    DDH treatment includes abduction devices such as the most commonly used Pavlik harness or less commonly a hip spica cast. Surgical procedures which are even less common involve reduction of the femoral head into the acetabulum and potentially other procedures on the acetabulum, femur or hip adductor tendons. If DDH is left untreated problems such as pain, osteoarthritis, leg length discrepancies, and gait abnormalities may occur. Even if treated there still exists the possibility of hip deformity and osteoarthritis in later life.

    Learning Point
    DDH incidence is between 1.5-20 per 1000 births. Of patients identified with DDH only 10-27% have risk factors for DDH other than female gender. Risk factors include:

    • *Breech delivery
    • *Family history of DDH
    • *Female gender
    • Congenital anomalies
    • Interuterine postural deformities
    • Oligohydramnios
    • Primiparity
    • High birth weight

    *These are the most consistent risk factors for DDH.

    Risks for clinical hip instability or DDH are:

    • Breech females – 84 per 1000
    • Family history positive females – 24 per 1000
    • Breech males – 18 per 1000
    • Females without risk factors – 14 per 1000
    • Males without risk factors – 3 per 1000

    Questions for Further Discussion
    1. What is the Graf classification?
    2. What are indications for surgical intervention for DDH?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

    Information prescriptions for patients can be found at MedlinePlus for this topic: Hips Injuries and Disorders
    and at Pediatric Common Questions, Quick Answers for this topic: Developmental Dysplasia of the Hips

    To view current news articles on this topic check Google News.

    To view images related to this topic check Google Images.

    To view videos related to this topic check YouTube Videos.

    Lehmann HP, Hinton R, Morello P, Santoli J. Developmental dysplasia of the hip practice guideline: technical report. Committee on Quality Improvement, and Subcommittee on Developmental Dysplasia of the Hip. Pediatrics. 2000 Apr;105(4):E57.

    Shipman SA, Helfand M, Moyer VA, Yawn BP. Screening for Developmental Dysplasia of the Hip: A Systematic Literature Review for the US Preventive Services Task Force. Pediatrics 2006;117;e557

    US Preventive Services Task Force. Screening for Developmental Dysplasia of the Hip: Recommendation Statement. Pediatrics. 2006:117(3); 898 -902.

    Mahan ST, Kasser JR. Does Swaddling Influence Developmental Dysplasia of the Hip? Pediatrics. 2008;121(1);177-178.

    Fox AE, Paton RW. The relationship between mode of delivery and developmental dysplasia of the hip in breech infants: a four-year prospective cohort study. J Bone Joint Surg Br. 2010 Dec;92(12):1695-9.

    American Academy of Orthopaedic Surgeons. Developmental Dislocation (Dysplasia) of the Hip (DDH). Developmental Dislocation (Dysplasia) of the Hip (DDH)
    Available from the Internet at http://orthoinfo.aaos.org/topic.cfm?topic=a00347 (rev. 10/13, cited 6/17/14).

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    5. Patients and their families are counseled and educated.
    7. All medical and invasive procedures considered essential for the area of practice are competently performed.
    8. Health care services aimed at preventing health problems or maintaining health are provided.
    9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.

  • Systems Based Practice
    24. Cost-effective health care and resource allocation that does not compromise quality of care is practiced.

    Author

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital