This is caused by mutations in one of three possible genes; PADI3, TGM, or TCHH. These genes code for proteins involved in hair shaft formation and improvement often occurs in later childhood. Uncombable hair syndrome is mainly autosomal recessive, but it can also be autosomal dominant because there are other involved genes that have yet to be identified.By early adulthood, phenotypic symptoms of UHS spontaneously improve or disappear. The hair of people with UHS may eventually lie flat and appear near normal in texture by adolescence or early adulthood for unknown reasons. Basmanav et al. have suggested that either different, non-mutated isoforms of the hair-forming enzymes are produced as we age, or that aging-related mechanistic changes in hair such as greater diameter and length help resolve UHS. Symptoms
Coarse hair
Trichodysplasia
White hair
Woolly hair
Patchy baldness

Cause
As one would expect from the name, individuals affected by this disorder have difficulty managing and grooming their hair. Affected individuals usually possess blonde or silver hair that may be present at birth or develop over time. The hair is wiry, frizzy, and stands straight out from the scalp. It is often dry but not fragile or brittle. This is due to several structural abnormalities in the hair shaft. A cross-section of the hair shaft from affected individuals reveals a kidney bean, triangular, or oval diameter, unlike the circular shaft shape displayed in straight hair. This irregularity in the diameter of the hair shaft prevents the hair from lying neatly against adjacent hair fibers.
Care is directed to the dysfunctional organs (dialysis for the kidneys, mechanical ventilation for lungs dysfunction).Mortality rates have been decreasing in the United States. This is likely due to the rapid identification and treatment of the CS. Some studies have suggested that this possibly related to the increased use of coronary reperfusion strategies, like heart stents. Nonetheless, the mortality rates remain high. Multi-organ failure is associated with higher rates of mortality. Signs and symptoms
The presentation is the following:
Anxiety, restlessness, altered mental state due to decreased blood flow to the brain and subsequent hypoxia. Low blood pressure due to decrease in cardiac output. A rapid, weak, thready pulse due to decreased circulation combined with tachycardia. Cool, clammy, and mottled skin (cutis marmorata) due to vasoconstriction and subsequent hypoperfusion of the skin. Distended jugular veins due to increased jugular venous pressure. Oliguria (low urine output) due to inadequate blood flow to the kidneys if the condition persists. Rapid and deeper respirations (hyperventilation) due to sympathetic nervous system stimulation and acidosis. Fatigue due to hyperventilation and hypoxia. Absent pulse in fast and abnormal heart rhythms. Pulmonary edema, involving fluid back-up in the lungs due to insufficient pumping of the heart. Causes
Cardiogenic shock is caused by the failure of the heart to pump effectively. It is due to damage to the heart muscle, most often from a heart attack or myocardial contusion. Other causes include abnormal heart rhythms, cardiomyopathy, heart valve problems, ventricular outflow obstruction (i.e. systolic anterior motion (SAM) in hypertrophic cardiomyopathy), or ventriculoseptal defects.